A 68-year-old man presented with a 3-week history of abdominal discomfort, nausea and skin eruption on his entire body. There was no past medial history of hepatolithiasis, recurrent peptic ulcer, abdominal operation, allergic disease, generalized skin eruption or food sensitivity. The laboratory investigations revealed a white blood cell count of 12.86 × 103/µl. There was peripheral eosinophilia ranging from 7% to 52% (normal: 0-7.2%). The total IgE was 593 IU/ml (normal: 0-170 IU/ml). Stool examination and an enzyme-linked immunosorbent assay (ELISA) test to detect parasite infestation showed negative results. The blood urea nitrogen (BUN) was 40 mg/dl (normal: 10-26 mg/dl) and the serum creatine was 2.1 mg/dl (normal: 0.6-1.3 mg/dl). An ultrasound scan for evaluation of acute renal failure yielded negative results except for pneumobilia. Because of the absence of prior sphincterotomy, a surgical bypass procedure or recent endoscopic retrograde cholangiopancreatography (ERCP), we inferred the existence of enterobiliary fistula. The acute renal failure was slightly improved and we then performed multidetector computed tomography (MDCT) for further evaluation. MDCT revealed diffuse submucosal thickening from the antrum of the stomach to the duodenal bulb and a bezoar at the first portion of duodenum. But enterobiliary fistula was not clearly detected (). Gastrointestinal endoscopy showed large ulcers with a geographic pattern from the antrum of the stomach to the duodenal bulb (). The duodenum revealed severe stricture involving the second portion and a soft bezoar at the first portion of the duodenum. MRCP demonstrated that there was pneumobilia in the gall bladder and common bile duct (CBD). In addition, cholecystoduodenal fistula and a suspected choledocoduodenal fistula were observed (). The results of the UGI were similar to those of the MRCP, and the other findings were mucosal fold thickening and nodularity involving the antrum and severe stricture involving the second portion of the duodenum (). Biopsy samples were obtained at the ulcer bed, and they showed marked eosinophilic infiltration (). We adopted the criteria shown in for evaluating the tissue eosinophilia; severe eosinophilia (over 70 eosinophils/× 400 field) was demonstrated in the lamina propria of the gastric mucosa. The diagnosis was enterobiliary fistula complicating eosinophilic gastroenteritis. After steroid therapy, there was interval improvement of both the ulcer and duodenal luminal narrowing on the follow up endoscopy.


A 44-year-old female patient presented with a four-month history of a non-tender, hard mass in her right lower abdominal wall. She had no previous history of any significant illness. Ultrasonography revealed a well-defined, hypoechoic mass in the rectus muscle of the abdominal wall. Abdominal computed tomography (CT) defined a 3.6 × 2.5cm sized ovoid mass in the right rectus muscle. The preoperative surgeon's diagnosis was desmoid tumor, and the mass completely excised. Upon gross examination, the mass was poorly demarcated from the surrounding skeletal muscle tissue and measured 3.6cm across its largest dimension. The cut surface showed a solid grayish-white appearance with focal fibrotic changes (). The tumor was composed of large polygonal cells arranged in cord or sheets; these were divided by slender fibrous tissue septa which were interdigitated with the surrounding skeletal muscle fibers. Lymphoid aggregates were noted at the periphery of the tumor. The large cells had an abundant eosinophilic granular cytoplasm and poorly defined cellular borders (). The nuclei were round to oval with small nucleoli. There was no significant cytologic atypia; mitotic features and necrosis were not observed. The granules in the cytoplasm were positive for Periodic acid Schiff (PAS) staining. Upon immunohistochemical staining, the granular cytoplasm was strongly reactive for S-100 protein (), CD68 and calretinin, but was negative for c-kit, CD34, smooth muscle actin, desmin and vimentin. Ultrastructural examination revealed numerous membrane-bound vacuoles in the cytoplasm that were presumably lysosomes, which were filled with electron-dense materials, fragmented rough endoplasmic reticulum and myelin components ().


A 53-year-old woman with a two-and-a half year history of inflammatory joint disease was admitted to hospital for progressive right leg weakness and difficulty in walking. The diagnosis of seronegative RA had been established 2.5 years earlier and she had been given daily combination therapy including 2000 mg sulphasalasine and 250 mg chloroquine diphosphate.
The patient complained of muscle weakness in her right leg, and also complained of bilateral pain and limited ranges of motion in her finger, wrist, elbow and knee joints, with morning stiffness lasting more than an hour. Her history didn't include pelvic operation, trauma, alcohol or drug abuse, or any predisposing metabolic disease such as diabetes mellitus, not was there a family history of any arthritic disorders.
Examination revealed symmetric synovial thickening of wrists, metacarpophalangeal and proximal interphalangeal joints. Limited and painful ranges of motion were noted in her right and left metacarpophalangeal, elbow, wrist and knee joints. Ritchie articular index was 11. Bilateral dorsal interosseous muscle atrophies and ulnar deviations were observed. Right quadriceps and iliopsoas muscles showed grade 3 weakness on the Medical Research Council (MRC) scale. Sensory examination revealed sensory loss in the right medial leg and thigh. Patellar tendon reflex was absent in the right side. Thigh adductors and all other muscle groups were normal bilaterally. The remainder of the neurological examination was normal. There was no palpable mass at the inguinal region.
Laboratory results were as follows; erythrocyte sedimentation rate (ESR) was 32 mm/h (Westergren method), haemoglobin was 11.8 g/L and white cell count was 3800/mm3. C-reactive protein (CRP) and rheumatoid factor (RF) were negative. All other laboratory parameters were normal.
Radiographs revealed bilateral erosive arthritis of hands and wrists (). Magnetic resonance imaging (MRI) of lumbar discs showed posterior bulging, including L4-5 and L5-S1 segments. Anteroposterior and lateral radiographs of the pelvis, right hip and lumbosacral region showed no abnormalities. Pelvic ultrasound was normal. In nerve conduction study (NCS), the right femoral motor amplitude and conduction velocity were found to be decreased. The left femoral motor and bilateral saphenous nerve conductions were normal. Electromyography (EMG) revealed positive sharp waves and fibrillation potentials in the right vastus lateralis muscles with a moderate decrease in recruitment and a slight enlargement of the motor unit potentials. The thigh adductors, tibialis anterior, and gluteus medius were normal.
In light of these clinical findings, a diagnosis of a partial right femoral neuropathy was confirmed. The patient was started on rehabilitation, and 20 mg daily oral methylprednisolone and vitamin B (including B1and B6) were added her present therapy. After two months this therapy resulted in a significant improvement of both walking and muscle strengths of quadriceps and iliopsoas muscles (MRC 5/5). EMG and NCS were unable to be performed after treatment, because the patient refused both tests.


A 55-year-old male patient presented to us with history of
sudden onset metamorphopsia in the left eye. He was operated
in the same eye four months back for vitreous hemorrhage
secondary to ocular penetration following peribulbar
anesthesia for cataract surgery (both the surgeries were done
elsewhere). He was a known case of open angle glaucoma
under treatment with timolol maleate 0.5% eye drops twice
a day and dorzolamide eye drops thrice a day over the past
three years.
On examination, the best-corrected visual acuity in the
right eye was finger counting (FC) three feet, <N36 and in the
left eye was 20/120, N18. The intraocular pressure (IOP) was
14 mmHg in either eye with applanation tonometer. Fundus
examination showed retinal pigment epithelium (RPE) atrophy
at the macula in the right eye and a large scar running across
the macula bordered by fluid and hemorrhage at its distal end
in the left []. There was an advanced glaucomatous optic
neuropathy (cup disc ratio - 0.9:1 with pale neuro-retinal rim)
in either eye.
On fundus fluorescein angiography (FFA) of the left eye,
there was a linear hypofluorescence running across the macula
corresponding to the needle tract through the RPE. At its distal
edge was found a hyperfluorescent area with leakage suggestive
of a CNVM [, inset]. Patient was subjected to standard
fluence PDT for the same. There was persistence of CNVM one
month following PDT []. At this point of time patient
was subjected to combination therapy of a standard fluence
PDT with intravitreal bevacizumab (2.5 mg/0.1 ml, Off-label)
injection immediately after the PDT, which was uneventful. Six
months following the treatment visual acuity had stabilized to
20/60, N12 with no leakage on FFA [].


A 60-year-old man was admitted because of visual disturbances and ocular pain following penetrating keratoplasty in the right eye for a corneal chemical burn.
On slit lamp examination, we observed a feather-like corneal opacity, corneal infiltrations, and epithelial defects. Fungal keratitis was suspected and corneal scrapings were taken that grew C. parapsiolosis. Subconjuctival amphotericin B (1 mg), topical amphotericin B 0.125%, and levofloxaxin 0.5% were subsequently administered every hour for 16 weeks. We discontinued use of the eye solutions upon observing signs of clinical improvement and stabilization. However, 5 days later the lesion recurred. The patient was restarted on and maintained on a topical amphotericin B treatment (0.125% twice daily) for 1 year. In addition, levofloxaxin 0.5% (three times daily) and fluorometholone 0.1% (twice daily) were administered for 1 year. The lesion did not progress, but there was no change in corneal infiltration. On examination one year later, an elevated corneal lesion and increased corneal infiltration with a yellow color were observed despite the topical amphotericin B treatment. Seven days later anterior chamber hypopyon and vitreous opacity on a B-scan were observed (). We immediately performed an intravitreal injection of amphotericin B 5 µg/0.1mL and vancomycin 1 mg/0.1mL. The next day there were still no signs of clinical improvement. Intravitreal voriconazole 80 µg/0.1mL and ceftazidime 2 mg/0.1mL were injected and topical voriconazole 1% was administered every 2 hours. Oral voriconazole 200 mg (twice daily) was also administered for 7 days. The patient discontinued the oral voriconazole because of abdominal pain, dry mouth, and scaling of the oral mucosa. The topical voriconazole was tolerated despite complaints of ocular burning. The patient was treated topically for 6 weeks, after which the corneal lesion and endophthalmitis had improved. The corneal opacity and endophthalmitis remained as complications of the lesion (). The patient subsequently underwent another penetrating keratoplasty and demonstrated a visual acuity of 0.1 at 10 months follow-up with no signs of recurrence.


A 60-year-old man was referred to us because of a lack of improvement after a month of empiric treatment with topical amphotericin B for fungal keratitis. On our initial examination we observed a feathered corneal infiltration and a corneal ulcer (). Corneal scrapings were taken. We started treatment with topical amphotericin B 0.125% and moxifloxacin 0.5%. After 10 days the culture revealed no growth and the corneal epithelium appeared healed; however, there was no change in corneal infiltration (). Because of a lack of improvement in the corneal lesion a month later, a new treatment regimen was initiated with topical voriconazole 1% administered every hour. The patient complained of ocular burning but tolerated the treatment. Clinical improvement was observed two weeks later with healing of the corneal epithelium. Six weeks later we observed a decreasing density of the infiltrate and healing of the corneal epithelium; the topical voriconazole was decreased to twice daily. The patient was treated topically for 13 weeks. Following completion of the therapy, complete healing of the corneal epithelium and resolution of the corneal infiltrate were observed; however, the corneal opacity persisted ().


A 57-year-old man was referred to our department for decreased visual acuity in the left eye that had lasted for one month. Prior to admission, he was diagnosed with diabetic retinopathy in both eyes and underwent one laser treatment in the left eye at a local clinic. His medical history revealed that he had suffered a left cerebral infarction 1 year ago. Our department planned to treat him with a trabeculectomy for neovascular glaucoma in his left eye. He had experienced amaurosis fugax in the left eye and a fluorescein angiogram revealed mild diabetic retinopathy in the left eye, while the right eye was normal. We diagnosed ischemic ocular syndrome in the left eye, and a preoperative neck angio CT scan showed bilateral carotid artery stenosis () and obstruction of the left middle cerebral artery. He was transferred to the Department of Neurology and received a bilateral carotid artery stent. The next day, he presented with a superior visual field defect in his right eye. Upon examination, the Snellen acuity with correction was 1.0 in the right eye with FC 20 cm in the left eye with an intraocular pressure (IOP) of 13/32 mmHg. Right fundoscopy revealed ischemic retina whitening along the inferior temporal arcades sparing the fovea. A fluorescein angiogram showed choroidal hypoperfusion and no filling of the retinal artery or vein in the inferotemporal posterior pole (). The visual field defect involved the superior nasal quadrant and partial superior temporal field in the right eye ().
Two months later, right corrected visual acuity was 1.0, while the right visual field recovered slightly after that.


A 64-year-old man received left carotid artery stent insertion for severe left carotid artery stenosis () in the Department of Neurology. He had not experienced ocular symptoms in either eye before insertion of the stent. The following day, he complained of acute onset of a left naso-inferior visual field defect. On initial examination, his visual acuity with correction was 0.8/0.16 (OD/OS) and the IOP was 18/15 mmHg. The visual field defect involved the inferior nasal quadrant field and partial inferior temporal field in the left eye. Left fundoscopy revealed ischemic retina whitening at the superior posterior pole except for the fovea (). A fluorecein angiogram showed delayed filling and the emboli in the superior temporal artery of his right eye (). He was treated with anticoagulant medication in Neurology and was observed in Ophthalmology.
Two months later, the right visual acuity and the visual field defect had not changed.


A 3-month-old male infant presented with bilateral leukokoria. Birth history of the child was unremarkable. On initial examination, the size of the cornea looked smaller. The child was referred for ocular ultrasound examination, with a clinical suspicion of retinoblastoma. An ultrasound study was performed with a high-frequency transducer operating at 10 MHz (L5, GE Medical Systems, USA). Gray scale evaluation revealed an echogenic band in the posterior segment of both globes extending from the posterior surface of the lens capsule to the optic disc. The axial length of the globes was 16 mm, which was suggestive of micro-ophthalmia. Color doppler examination revealed the presence of blood flow in the band []. Spectral analysis of this blood vessel showed arterial waveforms []. Hyperechoic foci were seen in both the posterior segments adjacent to the artery, suggestive of associated vitreous hemorrhage []. No calcification was seen.
Computed tomography (CT) of the orbits (Lightspeed, GE Medical Systems, USA) revealed diffusely hyperdense vitreous in both globes, suggestive of hemorrhage []. No solid mass or calcification was seen on either side. Subtle linear densities were seen in the vitreous of both globes, representing hyaloid artery in Cloquet's canal [].
Based on the above findings, the patient was diagnosed with bilateral PHPV. Further investigation failed to reveal any history of similar disorder in the family of the patient. The parents were advised to get him examined under general anesthesia, so that appropriate management could be decided. However, the parents were unwilling for a procedure under anesthesia and refused for the examination. Leukokoria was thought to be due to vitreous hemorrhage.


A 32-year-old female visited our hospital due to her nonspecific neck discomfort for several years. Sonography was performed on an Acuson unit with a 15-MHz linear transducer (Acuson, Mountain View, CA), and a 0.5 cm sized hypoechoic nodule with ill-defined margins and a taller shape was found in the right portion of the thyroid gland. It was suspected of malignant nodule. Another 1.8 cm sized anechoic cystic lesion with oval shape and well-defined margin was also noted at the posterior aspect of the mid-portion of the thyroid, and it was superior to the suspicious malignant nodule ().
To rule out thyroid cancer, the patient underwent sonography-guided fine needle aspiration of the suspicious malignant nodule. At the same time, we also aspirated the cystic lesion in right portion of the thyroid gland. The aspirated fluid was measured as 1 cc and showed as a thick yellowish pus-like fluid material. The cystic lesion was thought of parathyroid cyst or other complicated cyst, so aspirated fluid was sent for cytology cultures for bacteria and a check of the parathyroid hormone (PTH) level. After aspiration, the cystic lesion collapsed. The cytology results of the suspicious malignant nodule were consistent with a papillary cancer; there were no follicular cells and a few squamous cells in the cytology results of the cystic lesion. The PTH level of cystic fluid showed to be normal and there were no bacteria on culture. The patient underwent right total thyroidectomy for removal of the cancer. Histopathologic examination about the suspicious malignant nodule on US revealed micropapillary carcinoma, the same as the preoperative cytology report. The 1.8 cm sized cystic lesion superior to the cancer was lined by ciliated pseudostratified columnar epithelial cells, and its diagnosis was a branchial cleft cyst ().


A 1-yr-old girl presented with a salivary discharge at the facial skin of her right cheek. CT sialography was done and a sialo-cutaneous fistula from an accessory parotid gland was diagnosed (). She also had a skin tag on the tragus of the right ear. At that time, surgical excision was considered at a later age. At the age of five, she visited the outpatient clinic for definitive treatment. The clinical examination demonstrated a 1 mm-sized opening located at the facial skin 1 cm lateral to the angle of mouth. The salivary discharge was clear and serous. The salivary flow rate was calculated by a cotton ball test. It revealed that the flow from the accessory gland was about 10% of the total flow of a parotid gland. According to our previous experience (), fistulectomy was scheduled, but aesthetic considerations for the young girl made the surgical plan change to more conservative management. Under general anesthesia, the 6 cm-long fistula tract was identified with a lacrimal probe. Intraoperative ultrasonography was done to demarcate the accessory parotid gland. Although the accessory gland was too small to be clearly identified, 16 IU of botulinum toxin was injected to the accessory gland under ultrasound guidance. After thin adhesive film was attached on the buccal skin around the fistula opening to prevent spillage of corrosive agent from unwanted skin injury, about 2 mL of 40% TCA solution was injected into the tract with a small metal suction tip (). Thirty seconds later, all the solution was removed with suction and squeezing. This procedure of TCA cauterization was repeated twice. There was no immediate complication postoperatively.
After several days, a tensely inflated sialocele formed and the opening was found to be completely closed up. Aspiration was done with long subcutaneous tunneling from behind so as not to make an iatrogenic sialo-cutaneous fistula. After 4 sessions of weekly aspiration, the sialocele did not recur. During one year follow-up, there was no more salivary leakage or sialocele. Moreover, the aesthetic result was very satisfactory.


A 29-year-old man ingested metformin in a suicide attempt. The patient consumed the entire remaining contents of his father’s prescription metformin bottle that originally contained 100 tablets of 850 mg each. The father stated that the bottle had contained at least three-quarters of its original contents, putting the ingested dose between 64 and 85 grams. The patient also consumed ethanol, but denied any other co-ingestants. The parents discovered the overdose around 6:30 a.m., about 5 ½ hours post-ingestion, when the patient began complaining of vomiting, diarrhea, thirst, abdominal pain and bilateral leg pain. Paramedics were called, who found the patient to be agitated with a fingerstick glucose level of 180 mg/dL.
The patient had a history of psychosis and depression, including prior suicide attempts by drug ingestion. He was not taking any prescribed medications, having discontinued olanzapine and sertraline several months earlier. The patient had no personal history of diabetes, despite the family history of type II diabetes in his father, who was taking no other anti-diabetic medications than metformin. The patient admitted to daily ethanol and tobacco use, but denied any current or past use of illicit drugs. He had no surgical history or known allergies.
Vital signs on arrival to the Emergency Department (ED) were temperature of 35.2°C (rectal), pulse of 113 beats/min, blood pressure of 129/59 mmHg, respirations at 28 breaths/min with 100% saturation via pulse oximetry on room air. The patient was awake and oriented x4, but agitated and slightly confused (GCS=14). Pupils were equal and reactive at 4mm and the oral mucous membranes were dry. Other than tachycardia, the heart and lung exams were unremarkable. The abdomen was mildly tender to palpation diffusely, but soft and without guarding or rebound. The patient had been incontinent of feces prior to arrival, but the stool was guaiac-negative and the patient had normal rectal tone. Electrocardiographic monitoring demonstrated a narrow-complex sinus tachycardia. A repeat fingerstick glucose upon arrival to the ED was 364 mg/dL. The patient became increasingly combative, attempting to bite and spit at the ED staff. He was placed in 4-point soft restraints, had a respiratory protection mask placed over his mouth, and was medicated with intravenous lorazepam 2 mg, dolasetron 12.5 mg, morphine sulfate 4 mg, and hydration with 0.9% saline. Within 45 minutes, the patient was no longer combative, and the physical restraints were removed.
Laboratory evaluation from blood samples obtained about one-half hour after ED arrival are shown in . Notably, the serum glucose level was 707 mg/dL associated with an elevated anion-gap metabolic acidosis. Arterial blood gas analysis showed: pH 7.10, pCO218.8 mmHg, and pO2 133.1 mmHg. The serum lactate level was above our clinical laboratory’s reporting range (>11.1 mmol/L). Even accounting for ethanol’s contribution to total osmolality, the calculated osmolal gap was highly elevated at >20 mOsm/kg. Given the presence of hyperglycemia with severe metabolic acidosis, the possibility of diabetic ketoacidosis was briefly considered; however, the serum β-hydroxybutyrate level was within normal limits (2.4 mg/dL [0.5–3.0]) and ketonuria was absent. Urinalysis showed glycosuria and moderate hemoglobin, but no calcium oxalate crystalluria, blood cells, or signs of infection. A urine drug screen by enzyme-multiplied immunoassay technique was negative for opiates, cocaine, phencyclidine, amphetamines, propoxyphene, benzodiazepines, barbiturates, and methylenedioxymethamphetamine.
The patient was admitted to the medical intensive care unit service. Due to lack of ICU bed availability, the patient physically remained in the ED for several more hours. The nephrology service was initially contacted one hour after the patient’s arrival for consideration of emergent hemodialysis.
Repeat laboratory analysis on serum samples obtained four hours after ED arrival showed decreasing bicarbonate (6 mEq/L) and continued severe hyperglycemia (672 mg/dL), despite continued IV hydration with 0.9% saline boluses. The nephrology fellow was at the bedside discussing the benefits and risks of dialysis, when the patient was witnessed to become unresponsive, vomit, aspirate, and go into pulseless electrical activity (PEA) with a heart rate of 29/min. The patient was resuscitated with chest compressions, endotracheal intubation, 2 mg IV epinephrine, 1 mg IV atropine, and 4 ampules (176 mEq) of 8.4% sodium bicarbonate, resulting in a heart rate of 110/min and blood pressure 121/88 mmHg. An intra-resuscitative ABG showed: pH 6.95, pCO2 55.7 mmHg, pO2 88.1 mmHg, and bicarbonate 12.4 mEq/L. The patient was started on a bicarbonate drip (150 mEq NaHCO3 in 1L 5% dextrose at 200 cc/hr) and transferred to the ICU.
The patient developed non-sustained episodes of ventricular tachycardia, and then his blood pressure fell to 72/44 mmHg. A dopamine drip was started, but by the time the blood pressure stabilized the patient was found to be coagulopathic (INR 3.08; fibrinogen 113 mg/dL [156–400]; d-dimer >8.0 mcg/mL [<0.5]) and insertion of a hemodialysis catheter was delayed until the patient was treated with four units of fresh frozen plasma. The concurrent platelet count was 390 k/mm3 and the hematocrit was 47.7%. An echocardiogram showed mildly decreased left ventricular function, although serial cardiac enzymes showed progressive elevation of the CK-MB fraction and troponin I (up to 1.321 ng/mL [<0.03]). Hemodialysis was begun twelve hours after ED arrival. Three hours later, the patient again developed PEA and was resuscitated. Several more episodes of PEA recurred, and the patient expired about 19 hours after ED arrival. The last set of laboratory results, drawn five hours prior to death, showed a serum lactate level still >11.1 mEq/L, bicarbonate 12 mEq/L, and glucose 327 mg/dL. The hyperglycemia persisted despite treatment with an insulin drip (3 units regular insulin/hr) and the hemodialysis. No measurements of serum lipase or amylase were made during the patient’s hospitalization.


A 48-year-old female presented with diminution of vision in both eyes of one-week duration. She was admitted with fever, headache and arthralgia in the local hospital, two weeks prior to the onset of blurring of vision. She was investigated thoroughly and was diagnosed to be suffering from chikungunya fever. The investigations were as follows. Erythrocyte sedimentation rate 61 mm in 1 h (Westergren's method), hemoglobin: 12.5 gm%, total count: 14000 cells / cubic mm. Differential count showed polymorphs 62%, lymphocytes 28%, eosinophils 8% and basophils 2%. Platelet count was 1.8 lakhs / cubic mm. Random blood sugar was 240 mg%. Diabetic status was first detected when admitted with fever and antidiabetic medicines were started. Blood urea was 30 mg%, serum creatinine was: 0.8 mg/dL. SGOT was 42 U/L, SGPT was 67 U/L. ELISA test for HIV and VDRL test were negative. Chikungunya (Card) IgM ELISA test was positive. Polymerase chain reaction (PCR) test confirmed the presence of CHIKV infection. Chikungunya RNA real time PCR based on Taqman probes with an amplification product of 76 bp fragment was done. shows the real time Chikungunya virus (CHIKV) RNA PCR report. Both these were done from serum sample one week after the fever started. Lack of hemorrhagic manifestations, normal platelet count and absence of epidemic of dengue in the area were against a diagnosis of dengue fever. She was given symptomatic treatment, which included antipyretic analgesic tablets like paracetamol and diclofenac sodium. She was discharged once the fever subsided. After two weeks of the discharge from the hospital she complained of defective vision of both eyes and was referred to an ophthalmologist. On ophthalmic examination, her best-corrected visual acuity was 20/80 N18 in the right eye and 20/60 N6 in the left eye. Slit-lamp examination revealed no evidence of inflammation in the anterior chamber in both eyes. Intraocular pressure by applanation tonometry was 16 mmHg in both the eyes. There was no relative afferent pupillary defect and color vision testing using Ishihara's pseudoisochromatic chart was normal. Fundus examination by indirect ophthalmoscopy and slit-lamp biomicroscopy revealed few vitreous cells, optic disc edema, intra-retinal hemorrhages, peripapillary cotton wool spots and areas of retinitis with macular star in both eyes [Fig. and ]. Visual field analysis by Humphrey field analyzer showed bilateral centrocaecal scotoma. Fluorescein angiography was characterized by leakage from disc margins and from peripapillary vessels and blocked fluorescence due to retinal hemorrhage. The area of retinitis showed staining in the late phase of the angiogram [Fig. and ]. A diagnosis of bilateral neuroretinitis was made. Since there was no fever and optic nerve head involvement she was started on a course of systemic steroids. Tablet Prednisolone 1 mg / kg body weight was started and tapered over six weeks. Fasting blood sugar was 142 mg% on starting the steroid therapy and her diabetes mellitus was simultaneously controlled in consultation with a physician. At the end of two months the best-corrected visual acuity was 20/30, N6 in right eye and 20/20, N6 in the left eye. Fundus examination revealed bilateral resolution of disc edema with decrease in the retinal hemorrhages and cotton wool spots [Fig. and ]. Repeat Humphrey visual field analysis showed improvement of scotomas seen.


A 54-year-old Korean male was first referred to our department on October 4, 2001, with a history of hoarseness, difficulty in swallowing, and progressive generalized hardening of the body that had progressed over the past two years. The skin on his face and distal extremities was indurated, and several brownish and shiny papules were noted on the forehead and perioral area. On the trunk and proximal extremities, the skin was normal in elasticity, but the underlying muscles were indurated, and almost all of the joints were restricted in motion (, ). Quantitative analyses of serum protein revealed concentrations of immunoglobulin G (IgG) of 1,780 mg/dl and kappa (κ) light chain of 532 mg/dl (the normal ranges are 700-1,600 mg/dl and 170-370 mg/dl, respectively). Serum immunofixation showed a characteristic pattern of monoclonal IgG gammopathy, particularly of κ paraprotein. Laryngoscopic examinations revealed granulomatous materials deposited in the vocal cords, and nerve conduction studies showed polyneuropathy of the sensory nerves. Impaired left ventricular systolic function and thickening of the left ventricular walls with restrictive filling were noted upon echocardiography. There were no remarkable findings in various studies, including a bone marrow aspiration study, abdominal ultrasonography, and esophagography. The skin biopsy specimens showed a deposition of amyloid materials in the dermis, subcutaneous tissues, and in the connective tissue septae of the skeletal muscle by Congo-red stain (), and it produced a characteristic apple-green birefringence when viewed under a polarized light source ().
After failure to control the symptoms with systemic steroids, colchicine, or cyclophosphamide, monthly high-dose intravenous immunoglobulin (hdIVIg) was infused for three courses of treatment (each time giving 0.4 g/kg/day for five days); the patient noted improvement in subjective symptoms such as tightness of legs and dysphagia, and the range of motion of his joints began to improve as early as three weeks after the first treatment (, ). However, the elevated IgG and κ light chain levels did not decrease, despite the symptomatic changes, and the symptoms progressed with the discontinuation of hdIVIg therapy. Although the patient had no apparent renal dysfunction, the concern of potential renal involvement in systemic amyloidosis and the possibility that hdIVIg would enhance that risk made us decide to discontinue further hdIVIg therapy. To clear the excess serum immunoglobulin, which was constantly elevated despite the hdIVIg therapy, monthly TPE was performed, and the normalized serum immunoglobulin levels and the symptoms and signs, including dysphagia, tightness of legs, induration of skin, and the limited range of motion, were maintained in an improved state for more than two years.


A 20-year-old female patient, who had taken synthetic thyroxine (100 µg/day) for Hashimoto's thyroiditis and overt hypothyroidism, was admitted to our hospital with symptoms of dizziness and generalized weakness in October 1997. Eight months prior, she had been diagnosed with primary hypothyroidism and had begun treatment with synthetic thyroxine (50 µg/day). Upon admission to our hospital, the patient had aggravated dizziness with accompanying nausea and vomiting. She has five siblings, none of whom has any autoimmune disorder.
On physical examination, the patient's blood pressure was 100/70 mmHg, body temperature, 36.8℃, respiration rate, 20 breaths/minute, and pulse rate, 110/minute. She weighed 51 kg and measured 151 cm height (body mass index 22.4 kg/m2).
She looked pale and acutely ill, and had anemic conjunctiva and icteric sclera. Exophthalmoses were not present. The thyroid gland was slightly enlarged and had a hard consistency on palpation, without tenderness. The abdomen was soft and flat, and the liver and spleen were not palpable.
The patient's initial hemoglobin (5.2 g/dL) and hematocrit (15.1%) levels were severely low. Her white blood cell count (WBC) was 9,150/µL (68% polymorphonuclear leukocytes, 18% lymphocytes, 12% monocytes and 2% metamyelocytes), and her platelet count was 576,000/µL. The reticulocyte count was 21.2%, the corrected reticulocyte count was 7.0% and the reticulocyte index was 2.8. Peripheral blood smear analysis revealed spherocytosis, macrocytosis, poikilocytosis, and polychromasia with hemolytic features, and adequate white blood cell and platelet counts (). Levels of both serum calcium and phosphorus were within normal ranges. Total bilirubin, alkaline phosphatase, aspartate transferase and alanine transferase in the serum were 61.2 µmol/dL (reference range [RR], 5.1-17), 87 IU/L (RR, 30-120), 45 and 62 U/L (RR, 5-45), respectively. Lactate dehydrogenase concentration was highly increased to 748 IU/L (RR, 30-170 IU/L), and serum haptoglobin was decreased to 11.8 ng/dL (RR, 50-320 ng/dL). Direct/indirect Coombs' tests and a hemosiderin test yielded strongly positive results. Antinuclear antibody (ANA), antiplatelet antibody, acidified sucrose lysis (Ham's) test and osmotic fragility test were all negative. Abdominal ultrasonography revealed that the patient had a normal sized spleen and liver. According to bone marrow aspiration and biopsy, the patient showed erythroid hyperplasia, which is consistent with hemolytic anemia.
The thyroid function tests performed during synthetic thyroxine (100 µg/day) treatment showed that the patient was at a euthyroid state. However, she had elevated levels of antithyroid antibodies [antithyroglobulin antibody 3,100 U/mL (RR, 0-60) and antimicrosomal antibody 541 U/mL (RR, 0-60)]. The technetium-99m scan () indicated that she had a slightly enlarged thyroid with an inhomogeneous uptake pattern (2.8%). Based on the above results, the patient was diagnosed as having autoimmune hemolytic anemia and Hashimoto's thyroiditis. Her HLA genotypes, HLA-A*020101/A*2602, HLA-B*270502/B*5401, HLA-Cw*0102/Cw*020202, HLA DRB1*0404/DRB1*0405, and DQB1*0302/DQB1*0401 were determined as previously described.
We started the patient on an oral administration of prednisolone (1 mg/kg) and continued synthetic thyroxine medication (100 µg/day). Her hemoglobin level increased and stabilized within two weeks after prednisolone treatment. She had regular follow-up with prednisolone (10 to 30 mg/day) and synthetic thyroxine (100 µg/day) for two years and five months. In April 2000, she complained of frequent epistaxis episodes and an increased amount of menstrual bleeding with her regular cycle. She denied ingestion of any drugs including alcohol or herbs during that time period. She had also adhered to the prescribed administration of prednisolone and synthetic thyroxine. Physical examination did not show any clues indicating systemic lupus erythematosus (SLE) such as arthritis, photosensitivity, oral ulcer, skin rash or psychosis. At that time, she had moderate and persisting thrombocytopenia (75,000-90,000/µL), and repeated ANA test results were negative for serum platelet autoantibody. Hepatosplenomegaly was not found by abdominal ultrasonography. Bone marrow biopsy showed normal findings, except a mildly increased number of megakaryocytes. Serum calcium, phosphorus and albumin were 2.3 mmol/L (RR 2.2-2.6), 1.3 mmol/L (RR, 1.0-1.4) and 4.1 g/dL (3.2-5.3), respectively. Her fasting serum glucose level was 4.8 mmol/L. On urinalysis, neither proteinuria nor microscopic hematuria were found.
Considering the occurrence of thrombocytopenia, and regardless of the prednisolone treatment for AIHA and a normal bone marrow biopsy, we concluded that she had refractory idiopathic thrombocytopenic purpura and autoimmune hemolytic anemia, called Evans' syndrome, associated with Hashimoto's thyroiditis. The severity of hemolytic anemia fluctuated with prednisolone dosage, so we were not able to lower her prednisolone dosage below 20 mg/day. In July 2000, the patient had shown aggravated hemolytic anemia, despite a prednisolone dosage of over 30 mg/day. We concluded that the patient was in a steroid dependent and steroid refractory state, and so carried out a laparoscopic splenectomy.
After the operation, the patient's hemoglobin level and platelet count were restored to 11.1 g/dL and 461,000/µL, respectively. We could then lower her prednisolone dosage to 10 mg/day. Currently, she is still taking 10 mg of prednisolone per day due to her steroid-dependent AIHA and ITP condition, and has regular follow-ups at our hospital's outpatient clinic.


A 25-yr-old female patient visited the habitual abortion clinic in Hanyang University Hospital because of her history of recurrent abortions. She had been pregnant 3 times and all pregnancies were resulted in spontaneous abortions during 8-10 weeks of gestation. All of the 30 metaphases analyzed showed karyotype of 47,XX,-11,+fis(11)(p10),+fis(11)(q10) (). Both arms of the broken chromosome 11 appeared to be telocentric chromosomes. Chromosome studies on all her family members (father, mother and a younger brother) revealed normal karyotypes.
Six months later, she became pregnant. A prenatal cytogenetic test was performed by chorionic villus sampling at 10th week of pregnancy. The fetus had normal female karyotype, 46,XX, and she delivered a healthy female baby. The karyotype of the baby was reconfirmed by peripheral blood chromosome analysis.
Chromosome analysis of 72 hr lymphocyte cultures was performed with conventional GTG-banding techniques. To confirm the chromosome aberration of fis (), primed in situ labeling (PRINS) with the oligonucleotide primer specific for centromere of chromosome 11 () and fluorescence in situ hybridization (FISH) with whole chromosome painting probe for chromosome 11 (Chromoprobe TM-M11, Cytocell, Cambridge, U.K.) were studied. In all the metaphases scored (more than 30), 47 chromosomes were found. Chromosome 11 was divided into one smaller and one larger telocentric chromosome (). FISH analyses showed that no other chromosomes were involved (). It was evident that both fragments possessed centromeric heterochromatins in different amounts of the FISH positive band, i.e. the telocentric 11p has much larger centromeric heterochromatin than that of the telocentric 11q (). Centric fission of chromosome 11 has resulted in two stable telocentrics. There was no tendency to fuse again or to associate with acrocentrics as verified in 50 additional metaphases. Three distinct fluorescent spots were detected with the PRINS reaction in more than 100 interphase nuclei.


Patient 2, who had a homozygous missense mutation (c.1076C>T [p.359Pro>Leu]) at exon 8, was a 47-yr-old female at the time of her muscle biopsy. At her age of 30, she first noticed difficulty in climbing stairs, which progressed to limitations in daily activities by age 44. At presentation, she had difficulty in rising up from chair and could not stand up by herself from sitting position. Her muscle power was reduced to MRC grade 1 in leg adduction, grade 2 in hip flexion, hip extension, and leg abduction, grade 3 in shoulder elevation, elbow flexion, knee flexion and knee extension, and grade 4 in elbow extension and wrist flexion. Her deep tendon reflexes were lost in limbs except for the ankle jerks. She had no affected family members, and her serum CK level was mildly elevated (496 IU/L). The ECG and chest radiography were normal. Muscle biopsy showed a marked fiber size variation, markedly increased adipose connective tissue, many fibers with internal nuclei, scattered necrotic and regenerating fibers, some fibers undergoing fiber splitting, and type I fiber predominance ().
The patient 3 had been wheelchair-bound for one year when he first presented to us at age 35. He became symptomatic with gait and running difficulty when he was 13 yr old. Since then, his disease was progressively worsened. He started to use canes at age 32 and became wheelchair-bound at age 34. He had a profound muscle weakness on examination. His muscle power was graded as 1 in leg elevation, leg extension, leg adduction, and leg abduction, 2 in distal leg muscles, 3 in upper extremity muscles, and 4 in quadriceps muscles. He also had generalized muscle atrophy, especially in shoulder, pelvic girdle, and intrinsic hand muscles. His deep tendon reflexes were lost except for the knees. He had no affected family members, and serum CK was slightly elevated to 500 IU/L. His ECG and chest radiography were unremarkable. Although he showed loss of immunoreactivity against all the three sets of anti-calpain 3 antibodies, we were not able to find any CAPN3 mutations. His muscle biopsy showed a marked fiber size variation, markedly increased adipose connective tissue, many fibers with internal nuclei, scattered necrotic and regenerating fibers, many fibers with rimmed vacuoles, and type I fiber predominance ().


A 32-yr-old woman with no relevant or significant medical history presented with severe left calf pain that had persisted for the past 10 yr. The symptom was constant and worsened with menstruation, standing or walking for more than 30 min. The pain was relieved by elevating the leg in the sitting position or massaging the calf muscles. The pain was not associated with any progressive neurologic symptoms or signs. She reported that none of the medications she had tried provided any significant pain relief. Intramuscular electrical stimulation for 4 months and repeated muscle injections with lidocaine were also ineffective. Alternative treatments including acupuncture and Korean herbal medications had been tried many times without much success.
The patient's pain drawing showed a pain pattern limited to the left calf, especially in the medial side, which indicated a referred pain pattern of medial gastrocnemius or soleus muscle (). Physical examination revealed severe tenderness on the medial side of the left calf muscles. Signs of calf swelling were equivocal. Deep tendon reflexes were normal in both sides. Muscle strength of both lower extremities was normal. Sensation in both lower extremities was intact. The straight leg raising test was normal. Conventional radiography of the left leg showed several mottled calcifications within the muscles but did not reveal erosions of tibia or fibula (). To rule out a possible vascular deformity, femoral angiography was performed and a hemangioma was suggested in the left calf (). Magnetic resonance imaging (MRI) of the left lower extremity was performed for further evaluation of these abnormalities and showed an enhancing soft-tissue mass in the medial aspect of the soleus muscle ().
The patient was referred to an orthopedic surgeon and underwent excisional biopsy. Operative findings revealed a mass consisting of several irregular fragments of muscular soft tissue, measuring 6×6×4 cm. The final pathologic diagnosis was intramuscular hemangioma with fatty overgrowth isolated to the soleus muscle, which was in concordance with the MRI findings (). Within 1 month, the patient reported 90% pain relief and had discontinued all pain medications. Even with prolonged walking of more than 30 min, calf pain did not occur and the subject was able to resume mountain hiking 3 months following the operation.


A 42-year-old woman had presented to the local clinic with jaundice of two months duration and she was suspected of having periampullary cancer; she was then referred to us and admitted to our hospital for further evaluation. The physical examination revealed no abnormality. The serum total bilirubin level was 3.1 mg/dl and the aspartate aminotransferase/alanine aminotransferase levels were 80/180 U/L. Abdominal CT showed a cystic dilatation of the common hepatic duct and a marked dilatation of the intrahepatic bile duct (). Abdominal ultrasonography also revealed these lesions, but there was a partial thin septum-like structure within the upper aspect of the cystic dilated common hepatic duct (). However, neither definite septation nor an enhancing portion within the dilated duct was noted, and so this was considered to be a choledochal cyst. Percutaneous transhepatic biliary drainage (PTBD) was done through the left hepatic duct, and the subsequent tubogram showed obstruction of the left hepatic duct by a large filling defect. On endoscopic retrograde cholangiopancreatography (ERCP), a well-demarcated filling defect was seen in the common hepatic duct and this did not communicate with the bile duct. The distal portion of the common hepatic duct showed a beaking appearance near this mass and the left hepatic duct was not opacified (). Any anomalous pancreaticobiliary ductal union was not found. Before surgery, magnetic resonance cholangiopancreatography (MRCP) was performed, and it showed a well-defined cystic mass extending from the left hepatic duct to the common hepatic duct with a definite thin wall displaying a dark signal intensity. Magnetic resonance cholangiopancreatography also showed a bile duct variation; the right posterior segmental duct drained into the left hepatic duct (). The patient underwent resection of the extrahepatic biliary tract including the cystic mass, and hepaticojejunostomy was next performed. During surgery, the cystic mass was noted to arise from the common hepatic duct, yet it had no communication with this duct. Pathologically, this unilocular cystic mass arose from the serosal surface of the common hepatic duct, which was bulging into the common hepatic duct (). It was lined by a single layer of tall columnar mucin-secreting cells with focal evidence of mucinous secretion (). Our case was diagnosed as a biliary mucinous cystadenoma in the common hepatic duct. The patient has been alive with no tumor recurrence for one year after surgery.


The patient was a 50-year-old male when he was diagnosed with prostatic adenocarcinoma with bone metastases. Initial treatment included orchiectomy and stillbestrol administration. One year after diagnosis, due to advancement of this disease in spite of orchiectomy, the patient was started on monthly intramuscular injections of leuprolide 7.5 mg and was maintained on that medication for the next 6 years. At the age of 57, he presented to the hospital with pelvic pain and was found to have recurrence of this disease. The patient was then treated with pelvic radiation, and was started on mitoxantrone and hydrocortisone. A subsequent prostatic biopsy revealed SCC admixed with a small component of typical prostatic adenocarcinoma. The immunohistochemistry confirmed the presence of neuroendocrine differentiation, but the tumor was negative for prostatic acid phosphates, calcitonin, and ACTH. A PSA level was markedly elevated (2180 ng/ml).
The patient was treated for his SCC with vincristine and estramustine as an outpatient with once weekly treatments. One month later, he was readmitted to the hospital with mental status changes, a lumbar puncture was performed, and the spinal fluid studies revealed meningeal carcinomatosis, for which he was treated with intrathecal methotrexate. He was also found to have a hypodense lesion of his left parietal lobe consistent with metastatic disease. He subsequently developed disseminated intravascular coagulation (DIC) and expired shortly thereafter.


The patient was a 69-year-old male with a past medical history of hypertension and diabetes mellitus who presented to the hospital institution with urinary retention, acute renal failure (BUN, 101 mg/dl; creatinine, 8.5 mg/dl), and bilateral hydronephrosis. The PSA and the alkaline phosphatase level were 3205 ng/ml and 123 U/l, respectively. A bone scan showed diffuse uptake in the ribs and lumbar spine consistent with the metastatic disease. No biopsy was performed during this admission, and the patient was treated with leuprolide and flutamide for presumed prostatic adenocarcinoma.
Three months later, the patient was admitted with symptoms of volume overload and urinary retention. A prostatic biopsy sample revealed SCC of the prostate with no identification of typical prostatic adenocarcinoma. The tumor was positive for cytokeratins and chromogranin A, and was negative for PSA and prostatic acid phosphatase. The serum PSA level during this admission was 209 ng/ml. The patient was subsequently discharged with a Foley catheter in place, and received treatment with cyclophosphamide, vincristine, and carboplatin as an outpatient.
He was readmitted eight months later with pelvic pain. A CT of the abdomen and pelvis revealed a large pelvic soft tissue mass arising from the prostate causing ureteral obstruction, and a liver mass consistent with metastatic disease. A transurethral resection revealed mixed SCC and a poorly differentiated adenocarcinoma. He received pelvic radiation therapy following that admission, but represented two weeks later with lower extremity weakness. Due to his poor condition, he was deemed not to be a candidate for further treatment, and was subsequently discharged with hospice care. Of note, during the patient's last admission, his PSA level was undetectable.


A 78-year-old male with a past medical history of coronary artery disease, peripheral vascular disease, and prostatic adenocarcinoma status post resection and adjuvant chemotherapy with leuprolide one year earlier was admitted to the hospital for a right-below-the-knee amputation due to complications of his vasculopathy. During the postoperative period, he developed acute urinary retention and subsequently a TURP procedure was performed with the pathology revealing high-grade SCC. The tumor was positive for chromogranin A and synaptophysin, while stains for prostate-specific antigen and prostate-specific acid phosphatase were negative.
The patient represented to the hospital one month later with abdominal pain, fevers, and elevated liver function tests. A CT of the abdomen and pelvis revealed a single iliac lymph node and irregularity of the bladder wall, thought to represent invasion from the primary prostatic carcinoma. There was no evidence of a metastatic disease involving the liver. He was started on antibiotics for the provisional treatment of suspected cholangitis, but his condition deteriorated and he subsequently expired during that admission. The patient's infection was felt unlikely to be related to his underlying malignancy.


The patient was an 80-year-old male and his past medical history was significant for prostatic adenocarcinoma, diagnosed 12 years earlier []. He had been treated with external beam radiation, followed by a transurethral resection nine years later, and subsequently a bicalutamide treatment. The current admission was precipitated by multiple episodes of urinary obstruction and hematuria that started 10 days prior to admission.
On admission, he was afebrile with a physical examination significant for 3+ bilateral lower extremities pitting edema and bibasilar lung crackles. Laboratory values included a WBC of 22 900 /ul, with 25% bands and 10% eosinophils. Serum creatinine was 2.6 mg/dl, which increased from his baseline of 1.1 mg/dL. Urinalysis revealed 3+ leukocyte esterase, >100 RBC, and >100 WBC. Liver function tests revealed an elevated total bilirubin of 3.0 mg/dl, and a renal ultrasound showed bilateral hydronephrosis.
On the second day in hospital the patient underwent cystoscopy to evaluate the etiology of the obstructive renal failure and for prostate biopsy, which revealed poorly differentiated carcinoma. On day three, a liver ultrasound revealed marked heterogeneity concerning for metastatic disease. A left-sided percutaneous nephrostomy was performed on day four to relieve the obstructive hydronephrosis. On day nine, an abdominal MRI demonstrated diffuse metastatic disease of the liver and therefore a liver biopsy was performed. On hospital day 11, the carcinoembryonic antigen (CEA) was found to be 4155 U/ml, but PSA was undetectable. A noncontrast CT of the chest, abdomen, and pelvis revealed multiple bilateral pulmonary nodules consistent with diffuse metastases as well as enlarged lymph nodes in the chest and retroperitoneum. The liver biopsy demonstrated metastatic high-grade neuroendocrine carcinoma with mixed small and large cell features; the same histology was seen on subsequent review of the prostate biopsy []. He continued to become more encephalopathic and edematous, and was deemed not to be a candidate for chemotherapy given his extensive metastatic disease. He expired on hospital day 17 from presumed sepsis.


The patient was a 73-year-old male with a past history of hypertension, dyslipidemia, and diabetes mellitus. He presented to his urologist with multiple urinary tract infections over the preceding two years. The PSA was normal, but a nodule was detected in the left side of the prostate by ultrasound. The patient had a core biopsy performed revealing SCC without surrounding adenocarcinoma. The tumor was positive for cytokeratins, chromogranin A, and synaptophysin.
He was then referred to oncology clinic where he had a CT of his chest/abdomen/pelvis, MRI of the brain and spine, and a PET scan performed, all of which failed to reveal evidence of metastatic disease. The patient received four rounds of chemotherapy with etoposide and carboplatin, along with radiation treatment to the prostate. The patient enjoyed normal activities of daily living for 18 months following treatment, but during routine restaging was found to have extensive metastatic disease involving the brain, lungs, liver, and hilar lymph nodes. After these findings were discovered, the patient received high-dose corticosteroids to treat the vasogenic edema surrounding the brain metastasis. Upon discharge, the patient was scheduled to start treatment with topotecan.


A 34-year-old man who for nine years had been undergoing hemodialysis for chronic renal insufficiency, and 20 days earlier had fallen over, visited our hospital complaining of pain in both knees. Physical examination revealed mild tenderness in the suprapatellar area, the popliteal fossa of the left knee and the infrapatellar area of the right, and in both knee joints movement was limited. Laboratory data included a serum calcium level of 9.0 (normal range, 8.2-10.7) mg/dL, phosphorous of 5.8 (normal range, 2.5-4.5) mg/dL, and alkaline phosphatase of 550 (normal range, 30-110 IU/L. Serum protein was 5.9 (normal range, 6.4-8.5) g/dL, and serum BUN and creatinine were 86 (normal range, 7-20) and 14.2 (normal range, 0.7-1.4) mg/dL, respectively. Intact parathyroid hormone (PTH) was 1941 (normal range, 10-65) pg/mL, serum 1,25-dihydroxyvitamin D was 4.1 (normal range, 20-60 pg/mL, and serum aluminum was 35.8 (normal range, < 60) ug/L.
Plain radiographs of both knee joints showed arterial calcification of pipe-stem appearance in both lower legs, and fine calcifications in the suprapatellar area of the left knee () and the infrapatellar area of the right. The bony density of the distal femur and proximal tibia was slightly increased. US revealed focal thickening, decreased echogenicity with obliteration of the normal fibrillar pattern, and scattered calcifications with posterior shadowing at the distal insertion site of the left quadriceps tendon and the proximal insertion site of the right patellar. In the right knee, there was also complete discontinuity of this tendon (). Sagittal T1-weighted MR imaging (TR/TE = 600 ms/35 ms) of the left knee demonstrated obliteration of the normal laminated appearance and focal thickening, with inhomogeneously increased signal intensity from the insertion site of the quadriceps tendon to the base of the patella (). Sagittal T2-weighted MR imaging (TR /TE = 2500 ms/80 ms) revealed slightly increased signal intensity in the thickened area, and T2-weighted MRI also showed an area of abnormal increased signal intensity within the popliteus muscle of the left knee (). Sagittal and coronal T1- and T2-weighted MRI also demonstrated multiple vertical lines (medullary streaks) of low signal intensity in the metaphyses and diaphyses of the distal femur and proximal tibia. The lines were rough and irregular ().
For further evaluation of the nature or cause of the vertical lines seen on MR images, CT scans of both femora were obtained. Unenhanced scans showed coarse and thickened trabeculae in the metaphyses of both distal femora, with a moderate amount of joint effusion in the suprapatellar bursae (). We believe that the vertical lines of bone marrow of the femur and tibia revealed by MR imaging were related to the thickened trabeculae seen on CT scans.


A 29 yr old woman at 27+5 weeks gestation (gravida 1, para 0) was hospitalized due to dyspnea (NYHA IV) which had started 10 days before. The patient was healthy in the past, and the prenatal care findings of the local clinic were nonspecific. According to physical examination, dyspnea was aggravated at the inspiratory phase and right decubital position. Blood pressure was 120/80 mmHg, and her pulse rate evidenced tarchycardia of 120 beats/min. Both of her neck veins were engorged.
Routine blood tests and chest radiography were performed, but resulted in no peculiarity. Transthoracic echocardiography revealed a 5 cm in diameter echogenic mass spreading from the anterior of the left atrium to the mitral leaflet and mitral annulus, severe eccentric mitral regurgitation coupled with severe stenosis, and severe pulmonary hypertension (). The fetus was at a size consistent with IUP 27+2 weeks, according to obstetric sonography, and appeared to be normal. The patient was given intramuscular dexamethasone, 5 mg every 12 hr for 4 doses.
The operation on the intracardiac mass was carried out via median sternotomy. Continuous fetal heart rate and uterine monitoring were performed using cardiotocography throughout the procedure. On the area connecting the sternal body and the xiphoid process, we found a tumor mass about 2 cm in diameter. A diagnosis by frozen section identified the mass as sarcoma. We began normothermic cardiopulmonary bypass. The mass originating from the atrioventricular wall was found to have spread all the way to the leaflet and annulus of the anterior of the mitral valve, so we were compelled to settle for an incomplete resection of the mass (). We also performed a mitral valve replacement and a modified DeVega tricuspid annuloplasty. There was no evidence of fetal compromise during the operation according to cardiotocographic monitoring.
Permanent histopathological examination revealed a undifferentiated pleomorphic sarcoma (). The tumor was immunoreactive for vimentin, but immunohistochemical stains for cytokeratin, myoglobin, desmin, SMA, S-100 and CD34 were negative.
After operation, the patient exhibited stable vital signs and the cardiotocographic monitoring presented nonspecific findings. However, she developed preterm contraction at 29+5 weeks gestation, and we had to deliver a 1,230 g baby by Caesarean section, whose condition was stable, with Apgar scores of 5 at 1 min and 7 at 5 min. The operative findings of both ovaries were nonspecific, and postoperative staging with whole body computerized tomography was negative. We, therefore, concluded that the cardiac mass had indeed been primary sarcoma.
The patient then received systemic chemotherapy, with adriamycin, ifosfamide and darcarbazine. Although the patient exhibited neutropenia during 3 of the 8 courses of chemotherapy, she recovered without any particular complications. two months after chemotherapy, the patient was again hospitalized for dyspnea and severe abdominal distension. The results of examinations showed that global function was normal and the mitral prosthetic valve functioned well on echocardiography. However, computed tomography found a huge solid mass in the patient's right pelvic cavity (). After laparotomy, additional masses were observed. Therefore, we performed a right oophorectomy for the 30 cm in diameter right ovarian mass, and an omentectomy for the 20 cm in diameter omental mass, both of which were diagnosed as metastatic sarcoma (). ten months after the initial clinical onset, the patient died suddenly. Autopsy was not performed. The infant is presently alive and healthy.


A 71-yr-old man presented with abdominal distension and anorexia that had lasted for 10 days. His past medical history was unremarkable except for prostate surgery for benign prostate hyperplasia six years previously. He did not smoke or drink alcoholic beverages. His blood pressure was 140/90 mmHg, the pulse rate 70 beats/min, and he weighed 55 kg. The patient was alert and oriented. No jugular vein distension was found and a cardiopulmonary examination was unremarkable. The abdomen was markedly distended, with taut skin and a circumference of 85 cm. There was shifting dullness and fluid waves evident on palpation. There was no pretibial edema. His white blood cell count was 5,500 cells/µL, hemoglobin was 10.5 g/dL and platelet count was 165,000/µL. The prothrombin time was 11.2 sec (international normalized ratio, INR, 1.0) and the activated partial thromboplastin time was 28 sec. The serum alkaline phosphatase level was 109 U/L, total bilirubin 1.0 mg/dL, aspartate aminotransferase (AST) 70 U/L, alanine aminotransferase (ALT) 18 U/L, amylase 60 mg/dL, and total cholesterol 185 mg/dL. The total protein level was 5.8 g/dL and albumin 3.3 g/dL. Urinalysis results were normal. Hepatitis B and C serologies were negative. His initial chest radiograph showed normal without findings of cardiomegaly or pulmonary edema.
Samples of ascitic fluid showed elevated total protein (3.5 g/dL) and a low serum-to-ascites albumin gradient (SAAG; 0.8 g/dL). These were not characteristics of portal hypertension. The white blood cell count in the fluid was 81/µL, and 84% of the cells were lymphocytes. Gram staining and cytology were negative. Bacterial, fungal and mycobacterial cultures were also negative.
Given these negative findings, we performed imaging studies to evaluate the possible cause of the ascites. Ultrasonography and computed tomography (CT) of the abdomen revealed massive ascites and a normal-sized liver and spleen ().
Esophagogastroduodenoscopy (EGD) disclosed no evidence of portal hypertension such as esophageal varices or gastropathy. A laparoscopic biopsy of the peritoneum and liver was performed to rule out any common cause of high protein, low SAAG ascites such as peritoneal malignancies, tuberculosis or infections. Laparoscopy showed that the surface of the liver was slightly irregular and the peritoneum was normal in appearance. A microscopic examination of the liver biopsy revealed accumulations of yellow bile pigment in the hepatocytes, suggesting intrahepatic cholestasis without findings of liver cirrhosis (). Histology of the peritoneum yielded nonspecific findings.
We then evaluated the possibility of a cardiogenic origin. Echocardiography showed normal sized cardiac chambers, and the patient's left ventricular ejection fraction was 54%. However, there was no evidence of congestive heart failure as the cause of the ascites.
To prevent activation of the renin-angiotensin-aldosterone system and sodium retention from a vicious cycle of ascites, we prescribed an aldosterone antagonist (spironolactone 50 mg daily) and an inhibitor of proximal renal tubule sodiumabsorption (furosemide 20 mg daily). However, the patient's response to diuretics was poor; his body weight decreased by only 1 kg and the abdominal circumference by only 2 cm after diuretic therapy for 10 days.
The patient was discharged with a prescription for 20 mg furosemide and 50 mg spironolactone daily, because of his poor financial status. He did not attend the outpatient clinic for another month, when he was readmitted because of his increased abdominal girth. Compared with discharge, his body weight had increased by 3 kg and the abdominal circumference by 5 cm. The ascitic fluid showed elevated total protein and a low SAAG value, as found previously. We decided to evaluate some relatively unusual causes of ascites. We were interested in the high protein component of the ascites fluid, so we performed thyroid function testing. These showed the following values: T3 0.14 nM/L (normal 0.92-2.78 nM/L); T4 6.0 nM/L (normal 58-140 nM/L); free T4 0.13 pM/L (normal 10.3-35 pM/L); and thyroid-stimulating hormone level (TSH) 70.3 mU/L (normal 0.4-5 mU/L). These findings necessitated further thyroid tests. The antimicrosomal antibody titer was 1:102,400 (normal, <1:100) and the antithyroglobulin antibody titer was negative. Ultrasonography revealed atrophic change to both thyroid lobes (), and a technetium-99m thyroid scan revealed only two small nodular foci in the thyroid bed ().
Thyroid hormone replacement therapy was started with gradually increasing doses of levothyroxine, from 0.05 mg to 0.15 mg daily to treat the patient's hypothyroidism. Over the following three months, he became euthyroid with complete resolution of his ascites, and the liver function tests returned to normal. His body weight decreased by 9 kg and the abdominal circumference was reduced by 28 cm. There has been no recurrence of ascites and his euthyroid condition was maintained over three years of follow-up with levothyroxine 0.15 mg daily. The latest follow-up thyroid function studies showed a free T4 level of 19.3 pM/L and a TSH of 2.7 mU/L.


A 55-year-old man with a previous diagnosis of chronic otomastoiditis in his left ear was admitted to our hospital with complaints of a 1-month history of progressive posterior cervicalgia. He also presented with dysphasia and repetitive aspirations three months before his admission. His neurologic examination revealed diffuse paravertebral tenderness, neck motion limitation to the left side, left facial nerve palsy (House-Brackmann grade 4) and severe ipsilateral hearing loss. The findings of a cerebellar function test were unremarkable. There was no previous history of surgical operation. Due to the progressive and severe nature of his symptoms, he underwent magnetic resonance imaging (MRI) of the brain with a 1.5T MR unit. MRI imaging disclosed a homogenously enhancing solid mass occupying the clivus, extending posterolaterally into the epidural space of posterior fossa beyond temporal bone, and anteriorly to prevertebral space at cervicomedullary junction (). Its intracranial portion occupying the posterior fossa engulfed intraosseous segment of left intra cranial artery (ICA). There was no connection between the mass and the sellar or suprasellar regions. The size and location of the pituitary gland were unremarkable. It was mainly isointense to the brain cortex on both T1-weighted and T2-weighted images (). On high resolution bone algorithm CT images revealed that permeative bone destruction was excessively demonstrated in clivus, occipital condyle, petrous and mastoid portions of left temporal bone (). However, there was no calcification or cystic change observed within the lesion. Carotid and vertebral angiography revealed that the tumor was avascular and moved left vertebral artery toward the medial direction (). The differential diagnosis included chordoma, sarcoma and metastasis of skull base.
The patient underwent resection of the mass via suboccipital craniectomy using the left transcondylar approach and C1 hemilaminectomy. The tumor mass began to bulge out when the occipital condyle was drilled. The intracranial segment of the vertebral artery was displaced posteriorly and medially by the tumor mass. The vertebral artery adhered to the surrounding soft tissue and tumor mass itself. The glossopharyngeal, vagal and hypoglossal nerves were intermingled with the tumor mass, which was rubbery and of hard consistency and moderate vascularity. A dural incision was made after the tumor mass was partially removed, but there were no signs of a definite tumor in the intracranial space. The pathological results demonstrated irregular nests of multistratified squamous cells with peripheral palisading of the nuclei and keratin ().
His symptoms, however, were not improved. Then, the patient was referred for radiotherapy at a dose of 72 Gy (40 fractions), however, the radiation therapy was stopped after the administration of the 36 Gy dose because of deterioration of his general condition. One 1 month later, he died of aggravation of aspiration pneumonia which did not respond to antibiotic treatment.


A 49-year-old woman was hospitalized for further evaluation of dysphagia. Esophagoscopy revealed a huge mass in the lower esophageal portion. Abdominal CT examination demonstrated a huge low-attenuated submucosal mass in the esophagus. Additional heterogeneous highly-attenuated masses were evident in the uterus and vulvar region. While diagnosis of gastrointestinal stromal tumor or leiomyoma is typical from such CT observations, preferred according to conventional CT, a malignancy cannot be fully excluded. Appropriately, the patient was referred to the nuclear medicine department for 18F-FDG PET/CT to differentiate the esophageal and genital masses as malignant or benign. After a 6 hours fast, the patient was intravenously administered 370 MBq 18F-FDG. At the time of injection, serum glucose level was 97 mg/dl. The patient was instructed to rest comfortably for 60 min while whole body PET/CT images were obtained using a Gemini Dual PET/CT scanner (Philips Medical Systems, Amsterdam, The Netherlands). Seven frames (2.5 min/frame) of emission PET data were acquired in a three-dimensional (3D) mode after non-contrast CT scans from the base of the skull to the upper thigh (tube rotation time of 1 second per revolution, 120 kV, 50 mA, 6.5 mm per rotation, and acquisition time of 43.52 second for a scan length of 856.5 mm). Emission PET images were reconstructed with non-contrast CT using 3D-Row-Action Maximum-Likelihood Algorithm reconstruction (field of view = 576 mm, slice thickness = 4 mm, matrix size = 144×144, voxel size = 4.5×4.5×4 mm). Standardized uptake value (SUV) was calculated based on the injected dose and patient body weight. PET/CT demonstrated intensely increased 18F-FDG uptake with 3.8 of maximal SUV in the esophagus, 6.13 in the uterus, and 2.81 in the vulvar mass (), which was indicative of high probabilities of malignancy. Each mass was excised. Pathological testing confirmed that each mass was benign leiomyomatosis (). The patient's son was remarkable for Alport syndrome, and had undergone removal of an esophageal leiomyomatosis nine years previously. However, the patient showed no specific abnormal sign of Alport syndrome.


A 43-year-old man developed unremitting pain in the lower back and the right leg, which especially increased while walking. Subsequently, the patient was admitted to our hospital. The patient received bed rest for more than one month. Magnetic resonance (MR) and emission computed tomography (ECT) images demonstrated the presence of L3, S2 and S3 vertebral lesions that were metastatic tumors. Computed tomography (CT) images demonstrated the presence of metastatic tumors that involved the right acetabulum and the femoral head () that were associated with lung cancer (as determined by surgical pathology). Based on these clinical findings, the patient was diagnosed with multiple metastatic bone tumors. The patient received radiation therapy and experienced pain relief for one month. However, the patient experienced recurrent pain and the pain had seriously affected sleep and the daily activities of the patient. The pain was insensitive to drug administration. Furthermore, side effects of opiate use such as effects on the gastrointestinal tract, had become apparent. Due to the short life expectancy of the patient, a surgical procedure was not performed. Consequently, a percutaneous procedure was considered due to the potential ability to provide prompt pain relief and functional improvement. The patient was informed about the possible benefits and risks of the treatment. Written informed consent was obtained from the patient and his family.
Following detailed analyses of the lesions and adjacent structures, we developed a modified procedure for the treatment of the case. First, PVP was performed on the L3 lesion, and subsequently backache was alleviated. Second, nine days later, POP was performed on the right acetabulum and femoral head. Prior to the performing the procedure, it was essential to review the radiology films of the patient to avoid causing injury to important vessels and nerves. We administered local anesthesia to the patient and placed the patient in the prone position on the uniplane digital subtraction angiography (DSA, AXIOM Artis dTA, Siemens, Erlangen, Germany) operating table. A fluoroscope draped with a sterile sleeve was positioned to allow an anteroposterior (AP) view. A small dermatotomy incision was made with a scalpel blade. Thereafter, a bone puncture needle (13 G, Cook, Bloomington, IN) was used; the needle penetrated the neck of the upper portion of a femur lever attached to the femur flank along the femur neck axis (). We adjusted the cephalopod and left-right angle according to positions depicted on sagittal and axial images until the needle attained the lesion, as seen for all fluoroscopy projections. After removal of the needle, commercially available polymethyl methacrylate (PMMA) (Simplex P, Stryker Howmedica Osteonics, East Rutherford, NJ) was carefully injected into the lesion under continuous fluoroscopic monitoring via lateral and AP projections in order to ensure adequate lesion filling and to avoid PMMA leakage. When bone cement appeared at the neck of the femur cerebral cortex edge, injection was ceased. A total of 5 ml PMMA was injected into the right femur head. Post-procedural fluoroscopic observations showed optimal filling of the lesion with no evidence of PMMA extravasation (). Subsequently, we used a bone puncture needle to penetrate the right acetabulum. For successful penetration, 6 ml of PMMA was injected into the acetabulum. Immediate post-procedural X-ray photographs demonstrated good distribution of cement in the lesion and there was no evidence of PMMA extravasation (). Blood pressure, an electrocardiogram and pulse oximetry waveforms of the patient were monitored and were observed to be steady during the procedure.
The patient experienced considerable pain relief and functional enhancement, and the patient was able to walk with the aid of crutches on the following day. The patient was evaluated prior to and following the procedure for reduction of pain intensity using a numerical rating scale (NRS) with scores ranging from 0 (no pain) to 10 (worst pain imaginable) (). The NRS score decreased from 8 to 2 within 48 hours following POP. CT scans acquired three months following the procedure revealed a stable femur head with no further tumor growth and an unchanged location of the instilled cement (). The patient has continued to be pain-free without medication and has experienced no limitations in activity.


In November of 1999, a 62-year-old woman with a history of diabetes mellitus and hypothyroidism, was admitted to the hospital with right renal cell carcinoma. The initial blood pressure was 120/70 mmHg. The electrocardiogram (ECG) showed normal sinus rhythm. The initial echocardiography showed normal regional wall motion and normal left ventricular systolic function. There was a 5×4 cm solid mass at the lower pole of the right kidney on the abdominal CT scan, suggesting a renal cell carcinoma. Angioembolization of the right renal cell carcinoma was performed on the 25th of November 1999. A right nephrectomy was planned for the following day. The preoperative blood pressure was 160/100 mmHg in the operating room, and the blood pressure increased to 230/110 mmHg after induction of anesthesia. The ECG changes included T wave inversions and ST segment depression in leads V1-6, which were new ECG findings. The operation was canceled.
Echocardiography was performed immediately and showed impaired wall motion of all basal and mid-ventricular segments of the left ventricle (). The creatine kinase-MB (CK-MB) level was elevated to 66.4 ng/mL by the next day. The ST-T abnormality was normalized in 24 hours. The Echocardiography was repeated after two weeks and showed normalization of the left ventricular wall motion. The coronary angiography showed normal coronary arteries on the 11th of December 1999 procedure. The renal cell carcinoma was treated with interferon from December 1999 to March 2000. The size of the right renal cell carcinoma was markedly reduced after the interferon treatment. Follow-up abdominal CT scanning was performed in April and November of 2000 (). A left adrenal mass was identified. The size of the mass was about 2 cm. The right renal cell carcinoma and the left adrenal mass were mildly enlarged by March 2002. The size of the left adrenal mass was about 2.5 cm ().
The patient had no subjective symptoms and was lost to follow up. In December 2003, the patient visited the emergency room (ER) with dizziness. The initial blood pressure was 150/70 mmHg. T-wave inversions were noted on the inferior ECG leads. Echocardiography showed impaired wall motion of the apical and mid-segments of the left ventricle and severely decreased LV systolic function (). The peak level of cardiac troponin I was 3.1 ng/mL. Technetium-99m methoxyiso-butylisonitrile (Tc-99m MIBI) single emission computerized tomography (SPECT) revealed no evidence of inducible ischemia. Echocardiography was repeated after one week, the LV systolic function and regional wall motion abnormalities had fully recovered. In September 2004, the left adrenal mass of the patient was enlarged () and she was diagnosed with a pheochromocytoma. The 24 hour urine metanephrine was elevated at 3.478 mg/day, and the iodine-131 meta-iodobenzylguanidine (I-131 MIBG) adrenal scan was compatible with a pheochromocytoma (). The patient had a successful resection of the left adrenal tumor and right kidney in December 2004. Microscopic examination confirmed that left adrenal tumor was a pheochromocytoma and the right renal tumor was a renal cell carcinoma.


A 21-yr-old female patient presented with a sudden onset of low abdominal pain. With the clinical impression of an ovarian cyst, she was transferred from a local GYN clinic to our hospital. Gynecologic ultrasonography revealed a large posterior pelvic mass with an irregular echogenicity measuring 9.7×4.2 cm in size (). The patient had no specific past medical history with a parity of 0-0-0-0. A considerable amount of fluid was present in the cul de sac. The uterus and both adnexae were unremarkable. There was no ultrasonographic evidence of lymphadenopathy. Pelviscopy showed a large, thin walled, partly cystic, pelvic mass measuring approximately 10 cm in diameter. The mass was adherent to the internal and external iliac vessels and the right pelvic wall. It was not possible to remove the tumor completely. The retroperitoneal mass was partly removed by pelviscopy and samples were submitted for frozen section analysis.
The submitted specimen for frozen section analysis consisted of multiple irregular fragments of soft tissue weighing approximately 30 g. The tumor was soft and grayish-tan with focal areas of hemorrhage (). On frozen section, the possibility of epithelioid smooth muscle tumor or paraganglioma was suspected. Microscopically, the masses were characterized by a haphazard proliferation of smooth muscle cells (LAM cells) arranged in fascicular, trabecular, and papillary patterns around a ramifying network of endothelium-lined spaces. The cells were plump or epithelioid with abundant eosinophilic cytoplasm and bland nuclei with no mitotic activity. These cells invaded into fat tissue surrounding the lesion. The vascular spaces were empty or sometimes filled with eosinophilic material (). Aggregations of lymphocytes were between the LAM cells, representing vestiges of preexisting lymph nodes (). Occasional foci of hemorrhage and necrosis were present.
Most of the LAM cells showed a positive reaction for alphasmooth muscle actin (Dako Co., Carpinteria, CA, U.S.A.), vimentin (Zymed, San Francisco, CA, U.S.A.), desmin (Bio-Genex, San Ramon, CA, U.S.A.), and HMB-45 (Dako) (). S-100 protein (DiNonA Inc. Seoul, Korea), cytokeratin (Dako), chromogranin (Zymed), and CD 34 (Immunotec, Marseille, Cedex, France) were negative. Estrogen (Immunotec) and progesterone (Novocastra Lab. Ltd. Newcastle, U.K.) receptors were observed in only 10% of the LAM cells. Ki-67 (Zymed) was positive in less than 5% of the tumor cells. Clinical evidence of tuberous sclerosis was not present. Evaluation for the TSC gene was not performed.
After operation, the patient had continuous oozing of chylous ascitic fluid of approximately 1,500 to 2,000 mL per day. There was no clinical or radiological evidence of pulmonary involvement at the time of the initial diagnosis. Chylous pleural effusion was also identified two months after the operation. Chest CT scans showed diffuse, small, thin walled cystic lesions in both lung parenchymas with pleural effusion (). The patient was treated with tamoxifen and medroxyprogesterone, but the response was limited. Even after chest tube insertion and pleurodesis, she continued to drain approximately 800 to 2,300 mL of chylous fluid per day. In the terminal stages of the disease, the patient suffered severe respiratory distress and died six months after initial operation.


An 8-year old girl visited a local clinic with a chief complaint of headache, vomiting, and seizure. She was found to be hypertensive upon admission for treatment of persistent nausea, vomiting, visual disturbance, and seizure, even with medication. She was referred to our department for further evaluation of hypertension. A family history of hypertension or renal disease were absent. History of drug abuse, surgery, trauma, malignancy, or renal biopsy was otherwise unremarkable.
On physical examination, she was 127 cm tall (50 percentile) with a weight of 21 kg (5 percentile) and body mass index (BMI) of 13.02. Blood pressure in both upper and lower extremities were 160/100 mmHg (right) and 170/100 mmHg (left), respectively. She had no hematuria or abdominal and frank bruits. Chest radiography, echocardiogram, and abdominal ultrasound were unrevealing. Results from brain magnetic resonance imaging (MRI) raised suspicion of reversible posterior leukoencephalopathy syndrome ().
Laboratory investigations showed normal biochemistry parameters, urinalysis, thyroid function tests, 24 hour urinary excretion of protein, and catecholamine. adrenocorticotropic hormone (ACTH) stimulation test was normal. Serum renin was 22.6 ng/mL/hr (normal, 0.24-4.7 ng/mL/hr) and serum aldosterone was 68.33 ng/dL (normal 0.75-15.0 ng/dL). Serum epinephrine and norepinephrine were normal.
Kidney color Doppler sonography (US) showed neither stenosis nor obstruction in the renal artery. Abdominal computed tomography (CT) angiography () and renal dimercaptosuccinic acid (DMSA) single photon emission computed toraphy (SPECT) () demonstrate decreased nephrogram and radioactivity of the lower pole of the left kidney.
Diagnostic catheterization was performed, and there was no stenosis in either renal artery. Left renal angiogram showed multiple arteriovenous fistula and early filling of the left renal vein compared with the right, indicating the presence of an arteriovenous shunt (). Renal vein renin levels were obtained from both sides, as well as from the inferior vena cava. Serum renin level increased to 18.02 ng/mL/hr at the left inferior segmental vein, compared with 4.58 ng/mL/hr and 4.02 ng/mL/hr at the left anterosuperior segmental vein and right renal vein.
Diethylenetriamene pentaacetate (DTPA) nuclear renal scan and metaiodobenzylguanidine (MIBG) SPECT were normal.
We did not perform arterial embolization because lesions were multiple and there were some reports of spontaneous regression. The patient's blood pressure was well controlled by atenolol and enalapril and systolic blood pressure was maintained at 100-110 mmHg, and the patient is symptom free.


A 65-yr-old woman presented to the emergency room with right knee arthralgia. The patient had suffered from pain in the right knee for 4 hr. The patient had been well until about 4 yr earlier, when liver cirrhosis developed. Two years ago, the patient received a diagnosis of hepatocellular carcinoma, which was managed with 4 times of percutaneous ethanol injection therapy. The admitting temperature was 38.8℃, pulse rate 84/min, respiration rate 20/min, and blood pressure 100/60 mmHg. On physical examination, the patient appeared icteric. There were no abnormal findings in the chest. The abdomen was distended; a fluid wave and a mild splenomegaly were noted, but the liver was not palpable. The right knee joint was swollen, and the tenderness was noted in palpation. Laboratory investigations revealed a hemoglobin of 10.6 g/dL, a white cell count of 9,100/µL with 75.5% polymorphoneuclear leukocytes (PMNs), and a platelet count of 78,000/µL. The erythrocyte sedimentation rate (ESR) was 98 mm/hr. The prothrombin time was 24.2 sec (3.2 in international normalized ratio), and the partial thromboplastin time was 56.9 sec (vs. a control of 35 sec). The blood chemistry were AST 91 IU/L, ALT 75 IU/L, alkaline phosphatase 181 IU/L, total bilirubin 7.24 mg/dL, direct bilirubin 3.39 mg/dL, albumin 2.8 g/dL, BUN 17.5 mg/dL, creatinine 0.9 mg/dL, sodium 126 mEq/L, and potassium 4.5 mEq/L. The hepatitis B surface antigen was positive. Antihepatitis C viral antibody was negative. Alpha-fetoprotein was 189.1 ng/mL.
Aspiration of the ascites revealed a xanthochromic fluid with total WBC count 620/µL (PMN 490/µL), red blood cell 250/µL, and albumin 0.7 g/dL. Serum ascites albumin gradient was 2.1 and ascites culture was negative. Aspiration of the right knee joint revealed a turbid fluid with a white cell count of 22,900/µL (95% PMNs) and Gram stain showed Gram-negative bacilli. The patient received treatment with cefotaxime 4 g/day intravenously. In addition, the patient received arthroscopic irrigation with closed tube drainage. Klebsiella pneumoniae grew subsequently from the joint fluid and the blood. Susceptibility of the isolate was determined with the disk diffusion method and it was susceptible to cephalothin, gentamicin, amikacin, ampicillin-sulbactam, ticarcillin-clavulata, piperacillin-tazobactam, cefozitin, ciprofloxacin, imipenem, trimethoprim-sulfamethosazole, aztreonam, and ceftazidime, as well as to cefotaxime, the antibiotics that the patient was on. The patient responded to the antibiotic therapy and closed tube drainage. The condition of the patient slowly improved, and subsequently, joint-fluid cultures were sterile.
Abdominal computerized tomography showed multi-nodular hepatocellular carcinoma (a 2.8 cm enhanced nodule in the anterior superior segment of right lobe, and a 2.8 cm enhanced nodule in the medial segment of left lobe) with the intact portal vein.
Despite resolution of the infection, the patient's liver function was deteriorated. The patient developed progressive jaundice, azotemia, and hepatic coma and died on the thirty-first day after the admission. Autopsy was refused.


A 23-yr-old Korean woman presented with asymptomatic dark brown macules on the left leg for 2 yr. One or two dark brown macules first appeared and spreaded gradually without any preceding erythematous or scaly skin eruption. There was no history of prolonged sun exposure or trauma on the lesion site. On examination, she had linear streaks of dark brown macules from the left ankle, across the calf and to the thigh, consistent with the pattern of Blaschko's lines (). There was no oral or nail involvement. Skin biopsy showed atrophic epidermis, basal hydropic degeneration with sparse perivascular lymphohistiocytic infiltrates, and numerous melanophages (). These findings were consistent with LPP.


A 16-yr-old Korean woman presented with linear dark brown pigmentation on the left arm for one year. She stated that recurrent asymptomatic erythematous papules first appeared and then the lesion had developed into dark brown macules during 1-2 weeks. Examination showed two linear streaks of dark brown macules from the left dorsum of hand to the upper arm probably related to Blaschko's lines (). There was no accompanying oral or nail change. Skin biopsy showed orthokeratosis, focal basal liquefaction, a sparse perivascular inflammatory infiltrate, and pigmentary incontinence, confirming the diagnosis of LPP ().


A 23-yr-old woman who had complained of exacerbation of cough and dyspnea after inhalation therapy with a nebulizer solution was referred to our outpatient clinic. She had been diagnosed with asthma 3 yr previously and treated with a metered dose of a short-acting b2-agonist inhaler as needed. She had no history of anaphylaxis, urticaria, or drug allergy.
On admission, a complete blood count, blood chemistry, and serologic tests were normal. Chest radiographs revealed no abnormalities. Her white cell count was 7,400/µL with 5% eosinophils and the serum total IgE was 256 IU/mL by the EIA method. On spirometry, her forced vital capacity and forced expiratory volume in 1 sec were 3,520 mL (90% of the predicted value) and 2,700 mL (86% of the predicted value), respectively. She underwent skin prick tests with a panel of 10 common inhalant allergens and histamine (Allergopharma Co., Hamburg, Germany) and serial dilution of nebulizer solution containing BAC (Ventolin®, GlaxoSmithKlein, U.K.). The skin prick test was positive only for house dust mites species (Dermatophagoides farinae and D. pteronyssinus). Subsequently, an intradermal skin test with a serial dilution of her nebulizer solution was performed. A positive reaction was observed with 1:10 solution (wheal: 4×5 mm in diameter, flare: 18×20 mm). About 10 min later, she presented with dizziness, palpitations, and dyspnea. Her blood pressure was 70/30 mmHg, and her pulse was 120-140 per minute. Electrocardiography showed no abnormal findings, except for tachycardia. She was resuscitated with a subcutaneous injection of epinephrine (0.5 mg), an intravenous infusion of saline, and oxygen. She recovered fully 3 hr later. After obtaining oral informed consent, two healthy women without atopy or allergies underwent intradermal skin tests with the same nebulizer solution. Both control subjects were negative on tests. One month later, we conducted a bronchial provocation test with BAC following the method used by Asmus et al. (). The patient inhaled 3 mL of 0.9% NaCl solution containing 600 µg of BAC (benzalkonium chloride, Sigma, St. Louis, U.S.A.). The solutions were prepared using aseptic techniques and stored at 2℃ in an Eppendorf tube. The solutions were allowed to warm to room temperature immediately before use. Each 3 mL dose was inhaled using normal tidal breathing through a DeVilbiss 646 nebulizer (DeVilbiss Co., Somerset, PA, U.S.A.). Spirometry was performed at the beginning of the test and 15 min after inhalation of each dose began. On BAC bronchial challenge, a drop in FEV1 of 23% was noted after inhalation of 1,800 µg BAC. The patient refused skin test with BAC since she was aware of her anaphylactic reaction.


A 73 year-old-man was admitted to the hospital with general weakness and poor appetite, which had persisted for 2 months. He also complained of mild dyspnea on exertion, and cough. One week before admission, he had been evaluated at another hospital, where chest X-rays showed multiple cavitary lung lesions.
The patient had no history of blood transfusion, IV drug use, same-sex intercourse, or recent travel. The patient had a 2-year history of diabetes mellitus and hypertension. He had a 70 pack-year smoking history and social alcohol drinking. He had no history of frequent illness, fever or weight loss. On admission, his blood pressure was 110/90 mmHg, pulse rate 94 beats/min, respiratory rate 22/min and body temperature 36.2℃. On physical examination, coarse crackles were audible in both lower lung fields. There were no skin lesions, lymphadenopathy, or splenomegaly.
Laboratory studies showed whole blood cells 7,300/mm3 (neutrophil 86.4%, lymphocyte 7%, monocyte 5.2%, eosinophil 1.0%, basophil 0.4%), hemoglobin 11.5 g/dl, platelet count 464,000/mm3, blood glucose 109 mg/dl, total bilirubin 0.5 mg/dl, aspartate aminotransferase (AST) 19 IU/L, alanin aminotransferase (ALT) 25 IU/L, lactate dehydrogenase (LDH) 448 IU/L, HbA1C 6.4%. Urinalysis and microscopic examinations were normal.
A sputum gram stain and bacterial culture revealed no organisms. Direct examinations and sputum culture were negative for acid-fast organisms. Multiple thick-walled cavitary lesions, with nodules and consolidations in both lungs, were noted on chest radiography (). Multiple patch consolidations and multiple large pulmonary nodules with cavitary formations and severe wall thickening of subsegmental bronchi in the whole lung field were noted on the chest CT scan (). Fiberoptic bronchoscopy with transbronchial lung biopsy was performed for purposes of identification of any possible causative pathogen. On bronchoscopy, there were white exudate-covered nodules in the orifice of the anterior segment of the right upper lobe. An endobronchial biopsy was also performed. Histologic findings of the endobronchial biopsy showed moderately differentiated squamous cell carcinoma. Histologically, the transbronchial lung biopsy revealed multinodular peribronchial granulomas with central necrosis. Percutaneous needle aspiration cytology revealed yeast-form fungi, consistent with cryptococci (). A cerebrospinal fluid (CSF) cryptococcal antigen test, an India ink staining, and a fungal culture were all negative. Serum cryptococcal antigen was positive (1 : 16 titer).
Although the patient's CD4+ T-lymphocyte count had significantly decreased (127 × 106/L), HIV antibodies were negative according to both enzyme-linked immunosorbent assay (ELISA) and western blot tests. Also, HIV antigen (P24 Ag) was not detectable. His immunoglobulin electrophoresis profile was within the normal range. Initially, amphotericin B (0.5 mg/Kg/day) was infused for 15 days. After switching from intravenous amphotericin to oral fluconazole, the patient was discharged on day 32, and remained on fluconazole (400 mg/day). After 4 weeks of fluconazole therapy, coughing and dyspnea had lessened, and chest radiography showed decreased consolidations and multiple lung cavitary nodules. In order to treat the lung cancer, a right upper lobectomy was performed. The pathologic staging was IA (T1 N0 M0). He was discharged, receiving fluconazole. After a 10-month follow-up, the patient continued to have a decreased CD4+ T-lymphocyte count (172 × 106/L). An additional HIV test proved to be negative. The patient was otherwise well, and continued fluconazole treatment.


A 42-yr-old man was referred to us by his local physician after a 1.5 hr episode of oral bleeding. His history was positive for a sore throat for 3 months that had been treated intermittently with antibiotics. There was no prior history of oral bleeding. An examination revealed bilateral tonsillar hypertrophy with hyperemia, and a blood clot in the lower pole of the right tonsil. However, no active bleeding was seen. The white blood cell count, hemoglobin, prothrombin/partial thromboplastin levels and bleeding time were normal. The blood clot was removed and the lower pole of the right tonsil was cauterized with silver nitrate. Oral antibiotics were prescribed and the patient was scheduled for a tonsillectomy.
Seven days later the patient presented to the emergency room because of spitting fresh blood from the mouth for 1 hr. His tonsils were congested and fresh blood was seen oozing from the lower pole of the right tonsil (). His hemoglobin level fell from 14.9 g/dL to 11.2 g/dL. The patient was admitted to the hospital and an emergency tonsillectomy was performed.
On postoperative day 4, the patient presented to the emergency room with a history of bright red bleeding from the mouth for a few hours. His hemoglobin level was 6.9 g/dL and he received 2 units of packed red blood cells. In the operating room, diffuse oozing from the tonsillar beds was cauterized with bipolar diathermy hemostasis under general anesthesia. The hemoglobin level was 9.5 g/dL (post-transfusion). On postoperative day 9, the patient complained of fresh bleeding from the mouth. The bleeding was controlled by bipolar diathermy hemostasis under local anesthesia. The hemoglobin level was 6.7 g/dL and the patient received 2 units of packed red blood cells.
During hospitalization, further evaluations for a bleeding disorder were carried out. However, there was no clinical or laboratory evidence of a hematological or clotting disorder. On postoperative day 14, the patient was discharged from the hospital.