A 54-year-old female, with 12 years of schooling, noted the sudden onset of transient numbness and weakness of the left extremities in January 2012. She was diagnosed with suffering from moyamoya disease. On June 10, she again suffered from transient left hemiparesis. On July 20, she was admitted to our hospital for rehabilitation.
A neurological examination on admission revealed no abnormal findings. Brain magnetic resonance imaging (MRI) showed ischemic lesions confined to the right pre- and postcentral gyri and the medial frontal cortex on diffusion-weighted images (DWI) and fluid-attenuated inversion recovery (FLAIR) images (Fig. ). An analysis of single-photon emission computed tomography (SPECT) images was performed using the easy Z-score imaging system (eZIS) [] and voxel-based stereotactic extraction estimation (vbSEE) software programs []. Hypoperfused regions were mainly observed in the right hemisphere, such as the pre- and postcentral gyri, superior and inferior parietal lobules, supramarginal gyrus, angular gyrus, superior, middle, and inferior temporal gyri, and superior and middle frontal gyri (Fig. ).
The general neuropsychological assessments performed were the MMSE, the Kohs Block test, WAIS-III, Wechsler Memory Scale-Revised, Behavioral Assessment of the Dysexecutive Syndrome, the Trail Making test, Clinical Assessment for Attention, and the Behavioral Inattention test, the results of which were all within normal ranges.
The tactile recognition in the upper extremities was assessed 40 days after the onset of cerebral infarction. The elementary sensations for pain, temperature, light touch, vibration, and position sensations were all preserved. The discriminative sensations for topographic sensation (tactile localization) in 19 body parts were correct for 19/19 points on the right side and 7/19 on the left side. The 2-point discrimination using the Spearman esthesiometer was 3 mm at the tips of the fingers of both hands; no tactile distinction was observed. Hylognosia (perception of weight, texture, and quality of materials) was assessed by requesting the blindfolded patient to feel different stimuli in each hand.
Weight discrimination was assessed using 3 boxes of equal size, weighing 50, 56, and 62 g. The patient was asked to arrange the boxes by weight (lightest to heaviest). Three trials were performed for each hand, and the patient was able perform the task correctly with both hands. Texture discrimination was assessed by presenting the patient with 3 types of sandpaper of Japanese Industrial Standards (JIS) 600 (maximum particle size <72 µm), 800 (maximum particle size <67 µm), and 1,000 (maximum particle size <63 µm). Three trials were performed for each hand, and the patient was able to perform the task correctly with both hands.
Discrimination of the quality of materials was assessed by presenting the patient with 5 kinds of material: metal, wood, plastic, sponge, and cloth. Ten trials were performed for each hand. The patient was able to discriminate the quality of materials with no errors with both hands.
Morphagnosia (perception of size and shape) was assessed by requesting the blindfolded patient to feel different stimuli in each hand. Size discrimination was assessed with a wooden bar of 4 lengths (2, 4, 6, and 8 cm) with a diameter of 1 cm. The patient was asked to align the bars by length (shortest to longest). Three trials were performed for each hand, and the patient was able to perform the task correctly with both hands. Discrimination of 2-dimensional shapes was assessed using 6 palm-sized figures made of cardboard with a thickness of 0.2 mm (square, circle, triangle, star, pentagon, and hexagon). Ten trials were performed for each hand, and the number of correct answers was as follows: 10/10 in the right hand and 3/10 in the left hand. The response time was 2–3 s in the left hand and 10–15 s in the right hand, and the patient correctly identified the circle and square. Discrimination of 3-dimensional shapes was assessed using 5 palm-sized figures made of wood (cube, globe, cuboid, column, and triangular pyramid). Ten trials were performed for each hand and the number of correct answers was as follows: 10/10 in the right hand and 2/10 in the left hand. The patient only correctly identified the globe in the left hand.
Recognition of real objects was assessed using 15 household utensils (toothbrush, pencil, eraser, candle, scissors, glasses, stapler, fork, button, wrist watch, comb, nail clippers, cup, iron nail, hammer, and ballpoint pen). The patient correctly recognized all of the real objects (15/15 in both the right and left hands). The recognition of objects by the right hand was complete and immediate (within 2–3 s), but the response for the left hand took 10–15 s, particularly for the pencil, ballpoint pen, and candle, which were similar in shape and size. Regarding the scissors, the patient commented that they felt cold and finally recognized that. Regarding the fork, she commented that it had a sharp end, and then recognized the fork; these statements suggest that the patient was using the information from hylognosia and size to identify the objects. After the recognition trials for the real objects with the left hand, the patient was asked to draw the tested objects to evaluate whether or not she could recognize the items; she was able to draw all test items (Fig. ).


A 60-year-old right-handed nonsmoker female was seen in the Hull Headache Clinic for recurrent episodes of flashing lights, which started in the left visual field and progressed over 1 hour to cover most of the visual field. This lasted for 10–15 minutes and was followed by a severe left-sided headache associated with nausea, photophobia, and phonophobia but without autonomic features or other neurological deficits. The attacks occurred 1 day after she had endocardial pulmonary vein cryoballoon ablation for paroxysmal atrial fibrillation using an interatrial septal puncture with inguinal approach. The venous access during the procedure was difficult and eventually gained with two left femoral vein punctures in the left and a single right femoral vein puncture in the right. SL-1 long sheath was used with BRK transseptal needle to perform the puncture. Once access was gained into the left atrium, 10,000 units of heparin was administered. The activated clotting time (ACT) was kept above 350 seconds. The venogram demonstrated four separate pulmonary veins, and all veins were isolated using a 28 mm cryoballoon.
The patient had daily attacks for 2 weeks, which slowly improved, and eventually, she was left with five to six episodes of migraine with aura per month, lasting for 2 hours when taking paracetamol. In the last 5 years, the patient experienced similar left periocular and temporal headaches once a month lasting for 3–4 days, associated with nausea, photophobia, and phonophobia (migraine without aura). Before that, she used to get the attacks during menstrual cycles only. There was a family history of migraines (father). The patient has a history of paroxysmal atrial fibrillation and hypertension. Her regular medications are rivaroxiban, propafenone, and ramipril. On examination, her weight was 114 kg, and the blood pressure was 136/69 mmHg. The neurological examination and fundoscopy were normal. Her plasma viscosity, C-reactive protein, complete blood count, serum electrophoresis, and kidney and liver functions were within normal limits. Her autoimmune screen showed increased anti-ds-DNA to 9 IU/L (range 0–8) without clinical significance. The MRI head showed minimal T2/FLAIR hyperintensities related to small vessel ischemia without evidence of large blood vessel disease and vessel ischemic changes in the occipital lobes. The MRI diffusion-weighted imaging (DWI) and apparent diffusion coefficient (ADC) did not show abnormalities. The MRI head has been performed 5 months after the onset of migraine aura. Our patient was not keen on migraine prophylaxis but opted to optimize her acute attack analgesic treatment while avoiding analgesic overuse. Written informed consent was obtained from the patient for publication of her medical data for this case report.


A 32-year-old male presented to our outpatient department with complaint of repetitive shedding of skin since birth. Peeling of skin was present all over the body [Figures and ], sparing palms and soles []. Symptoms aggravate on exposure to water. Family history was present; one of his sibling had the same condition. There was no seasonal variation. On dermatological examination, superficial peeling was present. On rubbing of normal looking skin, it peeled off easily without any pain and erythema. Underlying skin was not inflamed. General and systemic examination revealed no abnormality. Routine hemogram and urine analysis were normal. Histopathological examination revealed epidermal separation in stratum corneum, thinning of epidermis, and loss of rete ridges. Mild lymphocytic infiltrate was present in epidermis. Dermis was normal []. He was prescribed emollient along with urea. After 2 weeks, patient was partially relieved symptomatically. However, the patient then discontinued treatment.


On January 6, 2011, a 62-year-old male was referred to our hospital because of a pancreatic mass that was found on ultrasound during a routine health examination at the local hospital. His abdomen was soft, non-tender, and non-distended, with no evidence of a palpable or pulsatile mass. There was no history of weight loss or trauma and no family history of significant disease. Abnormal laboratory results included an international normalized ratio of 1.16 (normal range: 0.85-1.15) and a thrombin time of 21.7 s (normal range: 14.5-21.5). Other laboratory results, which included tumor markers, were normal. The ultrasound showed a well-defined, low-density lesion measuring 3.7 cm × 2.3 cm in the neck and body of the pancreas (Figure ). No blood flow signal was detected within the lesion on color Doppler ultrasound (Figure ). An unenhanced computed tomography (CT) scan revealed a well-marginated and hypodense mass measuring 2.8 cm × 1.9 cm in the pancreatic neck and body (Figure ). On contrast-enhanced CT, the mass was slightly and heterogeneously enhanced (Figure ). Endoscopic ultrasound-fine needle aspiration (EUS-FNA) was also performed. However, we failed to acquire a tumor sample. Although imaging results were obtained, the mass in the pancreatic neck and body was still unable to be accurately diagnosed.
The patient consented to the performance of a laparotomy, and the mass was observed to originate from the pancreatic neck and body. We performed a spleen-preserving distal pancreatectomy with preservation of the splenic artery and vein. Intraoperative frozen pathology revealed a pancreatic schwannoma. Macroscopically, the mass was observed in the pancreatic neck and body, gray-white in color and 3.3 cm × 2.8 cm in size. Microscopically, the tumor cells were spindle-shaped and had a palisading arrangement with no atypia, which are results that are compatible with a benign tumor. Both hypercellular and hypocellular areas were visible (Figure ). Immunohistochemically, the tumor cells were strongly positive for S-100 protein (Figure ), but negative for SMA, CD34 and CD117. The tumor was definitively diagnosed as a schwannoma of the pancreatic neck and body. After surgery, the patient recovered smoothly and was discharged from the hospital 10 d later. The patient was followed for 72 mo and has been doing well without any further complications.


A 31-year-old woman was admitted to our department in order to undergo a laparoscopic cholecystectomy. She had a medical history of symptomatic cholelithiasis with an episode of acute cholecystitis 6 months before and several other episodes of biliary colic before and after this. The rest of her medical history was unremarkable. No preoperative assessment of patient's biliary anatomy with magnetic resonance cholangiopancreatography (MRCP) had been performed. The operation started laparoscopically with division of adhesions between the gallbladder and the greater omentum. During the dissection of the cystic duct and the cystic artery in the hepatocystic triangle, a transected bile duct with small caliber was noticed. The operation was converted to open. It proceeded with retrograde dissection of the gallbladder from the liver bed, identification, dissection, ligation and division of the cystic duct and the cystic artery and removal of the gallbladder from the operating field. During the cholecystectomy, it was recognized that the initially transected bile duct, which was 2 mm in diameter, came out of the hepatoduodenal ligament, ran along the common hepatic duct (CHD) and drained into the cystic duct, in proximity to the neck of the gallbladder. In order to identify the exact nature of the injury, an intraoperative cholangiography was performed by injecting an iodine-containing contrast medium through the common bile duct (CBD). The intraoperative cholangiography revealed that the aberrant bile duct was originated from the right posterior sectoral duct (RPSD). The RPSD continued its typical course up to its confluence with the right anterior sectoral duct (RASD) and the formation of the right hepatic duct (RHD) (Fig. ). The small caliber of the aberrant bile duct and the fact that it drained less than a liver segment led to the decision of its ligation (Fig. ). A drain was placed under the surface of the liver. The postoperative course was uneventful and the patient was discharged on the third postoperative day and remains free of symptoms 6 months later. The histopathological examination of the gallbladder revealed the presence of stones and chronic inflammation of its wall. Figure depicts the patient's biliary tree along with the part of the extrahepatic biliary tree that was removed during the operation (the gallbladder, part of the cystic duct and part of the aberrant duct). An informed consent was taken from the patient in order to publish the aforementioned information.


A 47-year-old Caucasian male with a history of substance abuse presented with nausea, vomiting, watery diarrhea, abdominal pain, anuria, fatigue and paresthesia in his extremities for 4 days. His medications included trimethoprim-sulfamethoxazole and clindamycin for rashes on his arms. On examination, he was hypotensive (67/48 mmHg), tachycardic (HR 115) and altered. Petechiae were present on all extremities and abdominal tenderness was elicited on palpation. Initial labs revealed leukocytosis (WBC 35.2 × 103/μL), anemia (Hg 11.5 g/dL), acute kidney injury (BUN 64 mg/dL, Cr 5.3 mg/dL) and thrombocytopenia (platelets 21 × 103/μL). Since the patient was taking clindamycin and experiencing watery diarrhea, we suspected septic shock secondary to Clostridium difficile-associated diarrhea. Surprisingly, the stool test for Clostridium difficile returned negative along with negative stool (Escherichia coli 0157:H7, Shigella, Campylobacter, etc.), blood and sputum cultures.
On further investigation, he was found to have microangiopathic hemolytic anemia evident by elevated lactase dehydrogenase (4 473 U/L), decreased haptoglobin (<10 mg/dL), indirect hyperbilirubinemia (2.2 mg/dL) and schistocytes on peripheral smear (Fig. ). The patient underwent 5 days of therapeutic plasma exchange for presumed TTP. However, his altered mental status, leukocytosis and thrombocytopenia were not improving with plasma exchange therapy. Multiple tests and procedures were performed to work up an extensive list of differential diagnoses without success, including infective endocarditis, hemophagocytic lymphohistiocytosis and mixed cryoglobulinemia (Table ). After completion of plasma exchange therapy, ADAMTS13 levels obtained prior to plasma exchange returned mildly decreased (28%), which did not support the diagnosis of TTP. Since the patient was not improving with therapeutic plasma exchange, we decided to provide supportive care only. After four days, his thrombocytopenia, altered mental status, and leukocytosis began to improve so he was transferred to the floor. Upon further history taking, he admitted to injecting Opana intravenously 1–2 days prior to becoming ill. Renal biopsy revealed acute tubular injury with cortical necrosis and collapsing glomerulopathy. There were no morphologic features to suggest hepatitis C-related glomerular disease or HIV-associated nephropathy. The patient was eventually discharged from the hospital on Day 17 with hemodialysis-dependent end-stage renal disease.


A 61-year-old male with hypertension suffered from sudden onset of poor vision and superior visual field defect in the right eye within 1 day. His BCVA was counting fingers at 20 cm oculus dexter and 20/20 oculus sinister. Ophthalmologic examinations revealed quiet conjunctiva and clear cornea in both eyes. Fundoscopy showed pale whitish retina in the inferior quadrant with fovea involved []. A large embolus was found at the optic disc within the proximal inferior temporal artery. Hence, BRAO was diagnosed. Initially, anterior chamber paracentesis was performed in his right eye. Topical brimonidine tartrate 0.15%, oral pentoxifylline, and aspirin were given. Hyperbaric oxygen therapy was also performed but showed limited improvement after 3 days. Hence, surgery was suggested and he agreed after 2 days.
Therefore, 25-gauge pars plana vitrectomy, posterior hyaloid separation from the retina under active suction, blocked retinal artery massage with a 25-gauge soft tip backflush needle (Alcon Grieshaber-Switzerland/Alcon Labs, Inc., Fort Worth, TX, USA) [], and bloodletting by 25-gauge end-grasping forceps (Alcon Grieshaber-Switzerland/Alcon Labs, Inc., Fort Worth, TX, USA) were undertaken 5 days after the onset. When bloodletting was induced, the intraocular pressure was increased to 60 mmHg for 2 min, and the 25-gauge soft-tip backflush needle was used to remove intravitreal blood [, Supplemental Digital Content]. Because the embolus was too large, we could not dislodge the embolus to the distal segment of retinal artery intraoperatively.
Fundoscopy showed reperfused retina with a smaller embolus postoperatively []. His BCVA improved to 20/200 1 month and 20/25 2 months postoperatively. The spectral-domain optical coherence tomography (Heidelberg Engineering, Heidelberg, Germany) showed hyperreflectivity of the inner retina with obvious optical shadowing effect preoperatively []; decreased hyperreflectivity, optical shadowing, intact ellipsoid zone, and external limiting membrane postoperatively []. However, visual field examination still revealed the corresponding defect at the right upper quadrant [] and persisted in the every 6-month recheck. His central vision remained 20/25 28 months postoperatively. He refused our suggestion about fluorescein angiography because he was satisfied with the improvement of his vision.


A 51-year-old healthy, heterosexual, monogamous man presented for evaluation of an asymptomatic red lesion on his foreskin and glans penis. He had no history of sexually transmitted disease or HIV-associated risk factors. He presented with a 2-year history of intermittent pruritus at the site of the rash on his penis. He had been previously treated with clotrimazole 1% cream without improvement.
Clinical exam revealed an uncircumcised male. Retraction of the foreskin demonstrated a confluent, erythematous, erosive, mucosal plaque on the lateral and dorsal glans penis, extending to involve the coronal and adjacent prepuce (Fig. ). The differential diagnosis included autoimmune bullous disease, BCP, candidiasis, dermatitis, erythroplasia of Querat, and lichen planus.
Microscopic examination of the biopsy from the glans penis revealed a submucosa with a band-like infiltrate composed of abundant lymphocytes and plasma cells (Fig. ). Parakeratosis and occasional neutrophils were also evident in the superficial epithelium. Correlation of the patient’s history, lesional morphology, and pathologic changes established a diagnosis of BCP.
After the biopsy was performed, but prior to receiving results, the biopsy site and surrounding affected mucosa were treated with mupirocin 2% ointment three times daily. In addition, although not previously effective, clotrimazole 1% was applied twice daily. When the patient was contacted by telephone with the biopsy results 12 days later, he commented that there had been a dramatic improvement of his dermatosis with the new topical therapy. He stopped the antifungal cream and continued with mupirocin ointment.
Treatment with topical mupirocin resulted in the lesion’s clearance within 6 weeks of use. When he subsequently discontinued therapy, the lesion began to reappear. At his return visit, 3 months later, there was near complete resolution with focal residual erythematous plaques (Fig. ). The residual lesions promptly resolved within a month once treatment with mupirocin 2% ointment was reinitiated.


A 14-year-old female presented to Peking Union Medical College Hospital in 2014 with frequent episodes of febrile seizures since she was 6 months old. At age 6, her seizure attacks became more frequent. A computed tomography scan detected bilateral basal ganglia calcification. She was diagnosed with epilepsy and given oxcarbazepine for seizure management; her seizure episodes decreased. At age 11, the patient had hypocalcemia (1.62 mmol/L, normal range: 2.13–2.70 mmol/L), hyperphosphatemia (3.70 mmol/L, normal range: 0.81–1.45 mmol/L), and an inappropriately low parathyroid hormone (PTH) level (22 pg/ml, normal range 12–65 pg/ml). Her serum calcium was still low one year later. She was given calcium (600 mg/d) and calcitriol (0.25 μg/day) supplements and her tetany relieved. At age 11, the patient also started complaining of bilateral hearing loss, which gradually deteriorated. When she presented at age 14, she had some difficulty communicating without a hearing aid but was still able to manage daily life.
The patient was born through normal vaginal delivery. Her parents were nonconsanguineous and had no family history of HDR syndrome. She had normal developmental milestones and had an average grade at school. The patient was 147 cm (–2 standard deviations [SDs]) tall and weighed 38 kg –1 SD to –2 SD). There were no facial or other skeletal deformities present. Both Trousseau and Chvostek signs were negative.
The results of laboratory tests on the patient’s first presentation to our hospital were serum calcium 1.87 mmol/L, serum phosphorus 2.35 mmol/L, serum magnesium 0.99 mmol/L (normal range: 0.70–1.10 mmol/L), and 25-hydroxy-vitamin D (27.1 ng/ml, normal range: 8.0–50.0 ng/ml). Serum albumin, glucose, electrolytes, creatinine, complete blood count, and urinalysis were normal. Blood cortisol, adrenocorticotropic hormone, thyroid hormone, and thyrotropin were also normal. Renal ultrasound revealed no anomalies. Bone mineral density (BMD) was 0.84 g/cm2 in the femoral neck and 1.20 g/cm2 in the lumbar spine (L1–L4). Ophthalmologic examination was normal. The patient was diagnosed with idiopathic hypoparathyroidism. Calcitriol 0.25 μg/d, cholecalciferol 300,000 U/week, and calcium 1200 mg/d were prescribed, and the doses of oxcarbazepine were gradually reduced. She had symptomatic relief with only mild residual limb numbness. One year after initial presentation, the patient had bilateral mixed deafness of both low and high frequencies; thus HDR syndrome was considered, and genetic testing was performed for definitive diagnosis.
Blood samples were collected from the patient and her parents. Genomic DNA was isolated from peripheral blood leukocytes using a DNA extraction kit (Omega, D3494-03 Blood DNA Midi Kit). Six pairs of DNA primers designed by Oligo Primer Analysis software v. 7 (Molecular Biology Insights, Inc., Colorado Springs, Colorado, USA) were used to sequence the GATA3 gene in blood samples. The primers used for amplification are displayed in . Sequencing was performed using the chain termination method on an automatic sequencer (Applied Biosystems 3730 Genetic Analyzer).
A single de novo nucleotide deletion in exon 2 of the GATA3 gene (c.286delT) was detected in this patient. This mutation was not found in the patient's parents and 100 unrelated healthy individuals. Functional assessment of this mutation was assessed using mutation taser (). The mutation was predicted to cause the 96th amino acid of the GATA3, tryptophan, to be substituted by glycine, resulting in a frameshift (p.W96Gfs*99) mutation that yielded a truncated 193 amino acids protein with the last 97 amino acids being different from the normal GATA3. Nonsense-mediated mRNA decay of the mutated protein was likely. This variant was not found in the Database of Short Genetic Variation (), Exome Variant Server (), or the Ensemble Genome Browser Database (asia.ensembl.org/index.html). The sequencing results of the proband and her parents are shown in .


A 53-year-old Kasakh lady with German predecessors presented to her community doctor and was found to have worsening renal function. At this time, she had no symptoms such as raised blood pressure, haematuria, flank pain or peripheral oedema. Incidentally, she noted having a loss of gustatory perception over time.
She had been diagnosed with a pathological glucose tolerance test and arterial hypertension 3 years earlier. She is morbidly obese with a body mass index (BMI) of 61 kg/m2. Her past medical history includes tuberculosis in 1984, cholecystolithiasis and hypothyroidism. Her medication includes an angiotensin-converting enzyme inhibitor, a betablocker and thyroxine.
Due to worsening renal function (estimated glomerular filtration rate MDRD 36.5 mL/min), proteinuria 113 mg/mmol creatinine (1000 mg/g creatinine) and evidence of dysmorphic erythrocytes in the urine sediment (11 per high power field), a renal biopsy was performed and showed glomerular, tubulointerstitial and vascular AA deposits as well as immunglobulin A (IgA)-associated mesangioproliferative glomerulonephritis Oxford Classification M0 E1 S1 T2.
Serum amyloid A level was raised with 81 mg/L (81 μg/mL). C-reactive protein (CRP) level was 60 mg/L (6.3 mg/dL). Immunological screening was unremarkable, including negative antineutrophil cytoplasmic antibodies (ANCA), rheumatic factor, anti-cyclic citrullinated peptide (anti-CCP) antibodies, myositis panel antibodies, anti-extractable nuclear antigen antibodies (ENA) and normal complement factors. Antinuclear antibodies (ANA) titer was slightly raised with 1:160. Total serum Immunoglobulin E level was elevated at 440 IE/mL. Serum IgA level and IgG subclass differentiation, including IgG4 levels, were normal. Testing for HLA-B27 was negative. A genetic predisposition for autoinflammatory syndromes such as cryopyrin-associated autoinflammatory syndrome (CAPS) and tumour necrosis factor receptor-associated periodic syndrome (TRAPS) was ruled out.
However, interferon-gamma-release assay was positive, indicating either previous or acute infection with mycobacterium tuberculosis. Bronchoscopy, gastric fluid aspiration and bone marrow biopsy were performed and showed no evidence of mycobacterium tuberculosis infection. During the clinical course, interferon-gamma-release assay became negative.
Genetic investigations revealed a homozygous presence of the SAA1alpha/SAA1.1/Val52(GTC)/Ala57(GCG) allele, consistent with a genetic predisposition for AA amyloidosis. Sequencing analysis of all four protein coding exons of the LECT2 gene showed heterozygous presence of a G polymorphism at cDNA position 172 (c.172A > G) leading to the replacement of isoleucine by valine (p.Ile58Val/I58V; legacy name I40V). No other mutation was detected in the LECT2 gene. Other genetic causes for amyloidosis were ruled out.
Therefore, biopsy specimens from the kidney were further analysed. Immunostaining was carried out using antibodies as described in detail elsewhere []. Renal amyloid deposits showed strong and even immunostaining with antibodies directed against amyloid P-component and AA amyloid. In addition, some deposits also showed strong immunostaining for LECT2, while the surrounding tissue was entirely immunonegative for LECT2 (Figure ).
Due to raised inflammatory markers, treatment with colchicine at a dose of 0.5 mg twice daily was started. After 6 weeks, serum amyloid A level was normalized with 6.2 mg/L (6.2 μg/mL) and CRP level was 15 mg/L (1.5 mg/dL). Therapy was well tolerated. Gustatory taste had recovered. Exercise tolerance and general well-being improved.


A 28-year-old Caucasian female presented to our institution with a 1-month history of a mildly tender, palpable lump in the upper outer quadrant of the right breast. The patient did not have any personal or family history of breast carcinoma. Her past medical history was only significant for prior biopsies of benign skin nevi. Targeted ultrasound of the area of palpable concern in the right breast upper outer quadrant demonstrated a corresponding 1-cm solid mass (). A core needle biopsy was performed. Histologically, tumor was characterized by sheets and nests of highly pleomorphic, mitotically active neoplastic cells infiltrating the breast parenchyma, with focal comedonecrosis ( and ). An initial diagnosis of poorly differentiated breast carcinoma was made. Further studies showed the tumor to be negative estrogen receptor, progesterone receptor, and HER-2/neu oncogene, consistent with a triple-negative invasive ductal carcinoma.
During pre-operative workup, an additional solid mass was identified in the inferior right breast. Core needle biopsy of this second mass showed cytologically similar neoplastic cells, with a prominent single-cell growth pattern. In addition, the tumor appeared to be well circumscribed with a thin rim of lymphoid tissue on one edge, suggestive of a replaced intra-parenchymal lymph node ( and ). At this point, considering the patient’s age and multi-centricity of the disease, together with the somewhat unusual growth pattern of neoplastic cells in the second biopsy specimen, immunohistochemical stains were performed. The neoplastic cells were positive for cytokeratin AE1/3 (patchy), pan-melanoma, tyrosinase, and SOX-10 and negative for CK7, CAM5.2, and GATA-3 (). The same immunohistochemical panel was then performed on the tissue specimen from the first mass, with similar results. Therefore, the initial diagnosis of breast ductal carcinoma was revised to melanoma. In light of this diagnosis, the patient’s prior skin nevi biopsy results were reviewed and the absence of malignancy confirmed. In addition, thorough skin and eye examination failed to reveal any evidence of primary melanoma.
Subsequent positron emission tomography–computed tomography (PET/CT) scan showed widespread metastatic disease to liver, lungs, and bones. Brain magnetic resonance imaging (MRI) demonstrated left temporal lobe metastasis, which was also histologically confirmed (). Genetic counseling was recommended, and the patient was tested with a gene panel for hereditary cancers, which was negative for germ line mutations. Targeted next-generation sequencing was performed, which revealed that the neoplastic cells harbored somatic BRAF p.V600E and PTEN p.R130Efs*4 mutations. Of note, TERT gene promoter mutation analysis was not performed.
In view of her advanced disease, the patient was enrolled in a clinical trial and was started on a combination of ipilimumab plus nivolumab, which was later changed to a combination of dabrafenib plus trametinib due to severe neurotoxicity (quadriparesis). This second chemotherapy course was complicated by severe neutropenia and hemorrhagic transformation of a temporal lobe lesion requiring craniotomy. Unfortunately, her disease continued to progress, and she died 8 months after the initial diagnosis.


A 12-year-old, left limb dominant boy came to our department with complaints of pain in right groin with limp since 2 months. On routine investigations, his white blood cell count was within normal range, and erythrocyte sedimentation rate was raised with positive C-reactive protein ratio. Antistreptolysin O titer and rheumatic factors were in normal range.
On clinical examination, no swelling or raised local temperature was observed. There was deep tenderness over right groin region.
On X-ray sclerotic shadow was observed in ischiopubic region of a size of cherry extending toward obturator foramen without any periosteal reaction or soft tissue shadows () On computed tomography (CT) scan enlarged unfused IPS with lytic lesion was observed. There was no evidence of periosteal reaction or any other inflammatory changes (, , ).
On magnetic resonance imaging (MRI), there was hyperintense signal on T2 with fat saturation and hypointense signal on T1. Fibrous bridging with fusiform swelling and signal alteration of the adjacent soft tissue was also observed ( and ).
The patient was treated conservatively with administration of nonsteroidal anti-inflammatory drugs and strict bed rest for 3 weeks, following which patient was improved and had no symptoms on further follow-up visits after 3 months and 6 months. No recurrence was noted on any follow-up visits.


A 77-year old woman suffering from hypertension with history of isteroannessectomy for uterine fibroma was admitted to the cardiological unit following an episode of syncope due to severe transitory bradycardia. During hospitalization, an aortic angio-TC scan revealed the presence of a 6 cm aortic aneurysm in the post-isthmic tract extended to the descending aorta and an infrarenal aortic ectasia (29 mm) (). The arrhythmia was treated with the implantation of Pace Maker (PM), and the previous chronic therapy was integrated with the introduction of beta-blocker and a calcium antagonist.
We performed, after two months, a surgical intervention for the endovascular exclusion of the thoracic aortic aneurysm (landing zone 1) after the debranching of both the left common carotid artery (LCCA) and the left subclavian artery (LSA) and their revascularization from a bypass coming from right common carotid artery (RCCA).
The RCCA was isolated through a right laterocervical incision, while LSA and LCCA through the supraclavicular one with scalenotomy and phrenic nerve conservation. Using a 8 mm Dacron prosthesis, we manufactured a carotid-carotid right–left and carotid-subclavian sequentially by-passes; during the entire procedure, we monitored EEG and PESS. We performed termino-lateral anastomosis between RCCA and prosthesis, lateral-terminal anastomosis between prosthesis and LCCA and terminal-lateral anastomosis between prosthesis and LSA. The proximal stump of LCCA was ligated and sutured. Under controlled hypotension produced by cardiac pacing, we deployed Backup Meir guidewire Captivia Valiant (44-44 200). During the release, there was a ‘sail effect’ causing the dislocation of the prosthesis about 4 cm far from the origin of innominate artery. For this reason, we proceeded to proximal relining with another Captivia Valiant (44-44-200). The procedure ended with the occlusion of LSA positioning Amplatzer Plug. The completion angiography was optimal (). The final oversize in the proximal and distal landing were 10% and 15%, respectively. In the second post-operative day, the patient developed a right laterocervical tumefaction. The neck and thoracic CT scan showed an important hematoma enclosing and compressing the RCCA and the origin of bypass extended to the posterior cervical triangle. This study also showed a type III endoleak (EL) in the concavity of aortic arch rising from the overlapping zone of the two prostheses ( and ). We decided to reopen the previous right surgical incision in the neck and evacuate hematoma. No active blood losses were found. EL III was treated with balloon angioplasty. Completion angiography showed a good result. The patient was scheduled for an accurate follow-up at our institute with CT scan after six months, then annually and DopplerUS of bypass every six months. Six-month CT scan was optimal. Eighteen months’ control indeed presented a regular patency of bypass, the persistent absence of EL but the appearance of intraluminal ulcerated thrombus ascending on the external side of the endoprosthesis. A deep ulcer in the thrombus perfused its caudal part. At this level, aortic diameter was increased from 51 to 61 mm (). This finding led to another endovascular procedure on the patient which was planned to extend the distal aortic coverage with a tapered Captivia Valiant (44-40-150). The oversize was 10%. The procedure was performed after two weeks from the previous CT scan. The last control was scheduled in September 2016 after 21 months from the first treatment. It showed the reduction of covered aortic diameter and no leak was revealed. However, it presented the enlargement of suprarenal tract untreated (which reached the size of 50 mm) and the presence of endoluminal thrombus with an irregular surface due to ulceration. Also, the infrarenal tract of aorta was increasing in diameter (40 mm) (). The patient still remained asymptomatic, and she has no limitation to her life. We decided to observe the evolution of the present lesion with a close CT scan.


A 64-year-old man with a history of angina pectoris and thrombocytopenia underwent CABG procedures at the age of 33 and 55 years. During the second CABG, the RGEA was used as a graft for the right coronary artery.
Nine years after the second CABG, he was admitted to our hospital with hematemesis. An upper gastrointestinal endoscopy showed a lesion with a hemorrhagic central ulcerative area and elevated margins that were poorly differentiated from the surrounding tissue on the posterior wall of the antrum. A diagnosis of poorly differentiated adenocarcinoma was confirmed by biopsy. No other apparent metastases to other organs were identified. On the basis of a diagnosis of advanced gastric cancer localized at the gastric angle, we performed distal gastrectomy with D2 LN dissection.
The patient underwent coronary arteriography preoperatively. Based on confirmation of a patent RGEA (Fig. a), the stenotic native coronary artery was revascularized (Fig. b) using bare-metal stents (BMS) to avoid cardiac ischemia due to intraoperative manipulation of the RGEA (Fig. c–e). Definitive gastric surgery was performed 4 weeks after revascularization. The location of the RGEA was easily confirmed intraoperatively (Fig. a). Indigo carmine dye was administered into the greater curvature of the gastric angle, adjacent to the tumor, to observe the lymph stream, through which the infrapyloric lymph ducts could also be observed (Fig. b). We detached the adipose tissue bearing lymphatic tissue along the outermost layer of nerves that twine around the surface of the pancreas and anterior superior pancreaticoduodenal vein [] (Fig. c). After the lymphatic tissue was removed along the outermost layer of nerves, the remaining skeletonized RGEA was preserved (Fig. d). Histopathologically, the tumor was 35 × 25 mm2, type 3, por2, pT3, pN0. Heparin treatment was initiated on postoperative day (POD) 1. However, it was interrupted from PODs 6 to 13 because of a transient hematic discharge from the drainage tube, and antiplatelet therapy was reinitiated on POD 15. The patient was discharged on POD 18. Over the 41-month follow-up period, he did not experience any recurrence of the gastric cancer or cardiac ischemia.


A 26-year-old woman presented with scalp hair loss that had been noticed for 1 month. She also reported nonpruritic scaly plaques on the scalp. She denied a history of hair pulling. Eleven years before, she had been diagnosed as having systemic lupus erythematosus by the presence of malar rash, polyarthritis, proteinuria, positive antinuclear antibody (ANA), and positive anti-double stranded DNA (dsDNA) and was currently in a period of remission. She has been treated with chloroquine for 10 years, and the current dosage was 250 mg 4 times a week. She reported no family history of psoriasis.
Physical examination showed multiple scaly erythematous alopecic plaques on the frontal and both parietal regions of the scalp (Fig. ). Hair pull test was positive and revealed all telogen hairs. There was no nail, mucosal, or other skin lesions. Other physical examinations were unremarkable.
Initially, the thick silvery scales were obtained for potassium hydroxide examination but no organisms were seen. Dermoscopic evaluation showed decreased hair density, increased vellus hairs, diffuse white scales, and numerous groups of dotted vessels distributed on the scalp (Fig. ). A 4-mm punch biopsy was done on the alopecic scaly erythematous plaque of the scalp for routine histopathologic examination. Histopathologic findings included superficial perivascular infiltration mainly composed of neutrophils, psoriasiform epidermal hyperplasia, parakeratosis, dilated tortuous blood vessels, decreased number of terminal hair, and sebaceous gland atrophy (Fig. ). Correlating the clinical, dermoscopic, and histopathologic findings, the diagnosis of psoriatic alopecia was established.
Treatment in our case included topical desoximethasone 0.25% scalp lotion to apply the scalp lesions twice daily, tar shampoo, and olive oil to the scalp before shampooing. After 3 weeks of treatment, she reported significant improvement in reduction of scales and stabilization of hair shedding (Fig. ). She achieved 75% hair regrowth by 3 months after therapy and had no recurrence of alopecia at the 1-year follow-up (Fig. ).


A 55-year-old male was referred to our hospital for endoscopic resection of two synchronous lesions of the distal rectum. On endoscopy, at 5 cm above the dentate line, we observed a 20-mm local recurrent adenoma on the scar of a previous endoscopic mucosal resection of a laterally spreading tumor (pseudo-depressed type) with high-grade dysplasia and a 25-mm laterally spreading tumor (mixed type), with no lifting sign, previously biopsied with histology positive for adenocarcinoma in situ (Fig. ). Rectal endoscopic ultrasound and magnetic resonance imaging showed no lymphatic or metastatic disease of both lesions. Because of the lesions’ characteristics and histology, we used the FTRD to achieve simultaneous R0 resections during the same colonoscopy. The lateral margins of the lesions were marked respectively with argon plasma coagulation. The over-the-scope clip (OTSC) device was mounted on the tip of the colonoscope (CFQ-145I Olympus). The first target lesion was grasped and pulled into the cap by the grasping forceps. Then, another FTRD was mounted on the tip of the colonoscope (CFQ-145I Olympus) and a second endoscopic full-thickness resection (EFTR) was performed (Fig. ). The total procedure time was less than 30 min. No complications occurred, and after 24 h the patient was discharged. Histology of the non-lifting sign lesion showed adenocarcinoma with submucosa infiltration (sm1 sec. Kikuchi), no lymphovascular invasion, and a negative muscular layer. Histology of the local recurrent adenoma on the scar revealed a carcinoma in situ. The endoscopic follow-up after 3 months showed spontaneous OTSC dislocation with normal scars, negative for adenoma relapse.


We described the clinical course of a 64-year-old Chinese man who was admitted to the hospital with septicemia and necrotic soft tissue inflammation of the right lower limb.
Fourteen days prior, a 64-year-old man, whose only known comorbidity was diabetes mellitus history, sought medical attention at another institution. He presented with pain, swollen, deformity of the right leg caused by falling from a height without any open trauma or neurovascular problem. The comminuted tibia fracture was confirmed with radiographs of the affected leg. Approximately 8 days after the injury, he underwent an uncomplicated routine MIPO surgery of the tibia fracture (Fig. ), when the blood glucose levels near normal and the swelling subsided. And the patient as usual received a single dose of cefazolin preoperatively for a prophylactic antibiotic coverage.
On the second postoperative day, the patient had fever as well as pain and redness around the distal operative incision of the leg, despite postoperative antibiotics (cefazolin) were used for prevention. More seriously, the incision started discharge of foul-smelling white exudate the following day. Cellulitis was initially suspected, the sutured wound was opened immediately, and intravenous antibiotic (cefmenoxime) therapy was given empirically. However, the erythema, edema, and then serous-filled bulla progressed to the right proximal leg and distal foot over a 12-hour period. The patient was promptly taken to the operating room for emergency exploratory operation the same day; soft tissue necrosis and pus accumulation beneath the fascia were discovered when the leg was opened. And the patient underwent debridement of the right leg and foot without involving the thigh. On the second day of debridement surgery, the leg infection progressed, with the erythema extending to the distal portion of the right thigh.
The patient was then transported to our unit, which was the level-I trauma center in our region, 4 hours after the sudden onset of excruciating painful swelling in the right thigh and right inguinal area in association with generalized malaise and profound weakness in another institution. On admission to our emergency department, the patient had a temperature of 37.8°C, a blood pressure of 90/45 mm Hg, a pulse rate of 134 beats per minute, and a respiratory rate of 32 breaths per minute. Physical examination of the lower limb showed the erythema and swelling progressed to involve the right inguinal area and the scrotum. The right leg, which was cold, cyanotic, and with a pulse absent in the dorsal artery of the foot, was detected with crepitus and sensory and motor deficits. There was a large area cutaneous deficiency on the right foot, which was caused by first debridement, and desquamation from the derma was noticed on the medial aspect of the right ankle (Fig. ). Laboratory abnormal findings included a hemoglobin level of 69.10 g/L, and a white blood cell count of 2.99 × 109/L with 81.71% neutrophils; a platelet count of 34 × 109/L; a serum potassium level of 5.33 mmol/L; serum creatinine 94.50 μmol/L; a serum urea nitrogen level of 10.73 mmol/L; and a serum glucose level of 19.38 mmol/L. In addition, evolving disseminated intravascular coagulation (prothrombin time 20.70 s, international normalized ratio 1.86, D-dimer level 1.02 mg/L, etc.) and a serum sodium level of 126 mmol /L indicated a NF. As we did not detect the result of C-reactive protein in the emergency room, the laboratory risk indicator for necrotizing fasciitis (LRINEC) score may be 5 or greater. No radiographs, computed tomography, or magnetic resonance imaging were obtained upon presentation because of the hemodynamic instability.
A tentative diagnosis of NF with septic shock was made, and vancomycin and meropenem were given intravenously on an empirical basis. Urgent consultation of doctors including multidisciplinary reached a consensus that emergency amputation must be considered for saving life. Immediately, the patient was transferred to the operating room. Nevertheless, the patient's condition deteriorated rapidly and he died of septic shock and multiple organ failure before surgery can take place. And further investigation of the thigh in the operating room showed prominent skin blistering and bloody bulla with partial necrosis 3 hours after admission (Fig. ). Histological examination of the surgical specimens confirmed the diagnosis of NF by showing necrosis of superficial fascia and subcutaneous fat, with dense infiltration of leucocytes and thrombosis of cutaneous vessels (Fig. ). Additionally, cultures of surgical specimens verified the pathogen to be Staphylococcus aureus, which was sensitive to methicillin.


A 65-year-old woman with abdominal pain presented to a local hospital. She had undergone extrahepatic bile duct resection, cholecystectomy, and choledochojejunostomy at our hospital 2 years 9 months previously for treatment of type Ic PBM according to the Todani classification []. At the local hospital, physical examination revealed epigastric pain and computed tomography (CT) showed dilation of the main pancreatic duct (Fig. ). The patient improved with 4 days of bed rest at the local hospital. She then presented to our hospital to determine the cause of the abdominal pain and dilation of the main pancreatic duct.
Laboratory data on admission to our hospital were as follows: white blood cell count, 6,200/μL; red blood cell count, 404 × 104/μL; hemoglobin, 12.4 g/dL; hematocrit, 38.8%; platelets, 28.6 × 104/μL; total bilirubin, 0.3 mg/dL; aspirate aminotransferase, 22 IU/L; alanine aminotransferase, 20 IU/L; alkaline phosphatase, 317 IU/L; γ-glutamyl transferase, 33 IU/L; amylase, 110 U/L; blood urea nitrogen, 19 mg/dL; creatinine, 0.60 mg/dL; C-reactive protein, 0.04 mg/dL; carcinoembryonic antigen (CEA), 3.9 ng/mL, and carbohydrate antigenic determinant 19-9 (CA19-9), 15 U/mL. The complete blood cell count was within the reference range, and hepatic, biliary, and pancreatic enzyme concentrations were not elevated. The concentrations of tumor markers, including CEA and CA19-9, were normal.
Gastrointestinal endoscopy revealed a tumor of the papilla of Vater, and adenocarcinoma of the papilla of Vater was diagnosed by biopsy (Fig. ). Magnetic resonance imaging (MRI) and CT revealed dilation of the main pancreatic duct. However, the tumor at the papilla of Vater was not clear on MRI. There was no obvious accumulation of fluorodeoxyglucose on whole-body fluorodeoxyglucose-positron emission tomography. There was also no distant metastasis. We then performed pylorus-preserving pancreaticoduodenectomy and pancreaticojejunostomy with a diagnosis of carcinoma of the papilla of Vater. Intraoperatively, we performed adhesiolysis of the abdominal wall, liver, and small intestine and found that the tumor at the papilla of Vater was approximately 1 cm in diameter. We removed the pancreatic head and duodenum and performed pancreaticojejunostomy and gastrojejunostomy. The pathological diagnosis was moderately differentiated tubular adenocarcinoma of the papilla of Vater with no metastasis to the lymph nodes (pT2N0M0 stage IB, International Union against Cancer, UICC). The main part of the tumor was present at the common channel (Fig. ). The specimen showed p53 expression in the cancer tissue and atypical epithelium of the retained bile duct (Fig. ). The patient remained in good health with no recurrence for 3 years after the second operation.


A 66 year-old female patient who suffered from an unhealing wound and pus discharge after tooth extraction since 2013 had undergone several debridement surgeries and was diagnosed as Paget's disease of mandible with osteomyelitis. The bony lesions were also confirmed in skull (), pelvis, both femora and tibiae (). Both medial and lateral cortices of the femur were thickened and multiple looser zones were observed in the simple radiogram and technecium 99m whole body bone scan. Slight external expansion of lateral cortex was recognized in the middle one third of right femoral shaft. She was prescribed risedronate due to migratory pain in both lower limbs from 2015. After 1 year of taking risedronate, right thigh pain developed again and did not subside for 3 months.[] She stopped taking risedronate and visited our clinic for the evaluation of thigh pain and further treatment.
She was afebrile but still had a draining sinus at the inferior aspect of chin due to mandible osteomyelitis. The alkaline phosphatase level had been within normal range consistently for 2-year. Bone specific alkaline phosphatase level (26.8 U/L) was not elevated. Levels of calcium, albumin, 25-hydroxy-vitamin D were normal. White blood cell count, procollagen type I N-terminal propeptide (C1NP) and N-terminal telopeptide (NTx) were within normal range.
Both femora showed thickening and prominence of primary compression and tension trabeculae in the proximal femur. Coxa vara and grade II anterolateral femoral bowing was noted.[] A beak like protrusion replaced smooth external expansion of the lateral cortex in the femur which was recognized three years ago. Looser zone which was initially noted in the middle of expansion became more distinct and looked like a dreaded black line at the moment.[] In order to control the thigh pain and prevent impending complete fracture of AFF like lesion, we decided to perform prophylactic femoral nailing using new nailing technique. Nailing procedure was uneventful and nail was inserted in external rotation to accommodate anterolateral femoral bowing.[] Careful follow up of left femur is also necessary.


A 69-year-old Caucasian male presented to the emergency department with complaints of fever, cough, and dyspnea on exertion for two days prior to presentation. His past medical history was significant for Diabetes mellitus type 2, hypertension, bioprosthetic aortic valve (AV), heart failure with preserved ejection fraction, chronic obstructive pulmonary disease, obstructive sleep apnea, morbid obesity (BMI 64 kg/m2), recurrent nephrolithiasis (status post bilateral lithotripsy and ureteral stent placement), benign prostatic hypertrophy, and adenocarcinoma of colon status post partial colectomy. Twelve days prior to presentation he had undergone cystoscopy with cannulation for the right ureter for laser lithotripsy and stone basket retrieval of kidney stone followed by right ureteral stent placement. He was taking oral amoxicillin for streptococcal pharyngitis diagnosed prior to the procedure and also following the procedure. He was unemployed, had no history of smoking, alcohol or illicit drug use, and family history was only notable for obesity and diabetes. His vital signs on presentation included temperature of 39.6°C, heart rate of 110 bpm, respiratory rate of 20 breaths per minute, blood pressure of 140/70 mm Hg, and oxygen saturation of 91% on room air. Physical examination revealed normal heart sounds without any murmur. There were no peripheral stigmata of infective endocarditis (IE) such as Janeway’s lesions or splinter hemorrhages; nor was there evidence of immunologic phenomena, e.g., Roth spots or Osler’s nodes. The reminder of the physical examination was unremarkable. Laboratory investigations revealed white blood cell (WBC) count of 17.86 cells/mm3 with left shift, Procalcitonin 10.31 IU, and lactic acid 4.2 mg/dL. Chest X ray was not suggestive of pneumonia or heart failure. EKG showed sinus tachycardia. Urinalysis demonstrated both RBCs and WBCs on microscopic exam, but no leukocyte esterase or nitrites. Blood and urine cultures were obtained in the emergency department (ED) and our patient was admitted to the hospital with a diagnosis of sepsis and started on empiric antibiotic treatment with intravenous vancomycin and piperacillin/tazobactam.
Despite a positive microscopic urinalysis for WBC, the urine culture was no growth (possibly attributable to the ongoing oral antibiotic treatment). After 18 hours both sets of blood cultures drawn on admission grew Gram positive cocci in clusters (see ), which were ultimately identified as Aerococcus urinae in the microbiology laboratory by Vitek® 2 testing (bioMérieux, Marcy l’Etoile, France) prompting performance of a transthoracic echocardiogram (TTE). The screening TTE did not show any vegetation. Transesophageal echocardiogram (TEE) however revealed a 1.77 cm x 1.42 cm vegetation on the prosthetic aortic valve (see ). Although no peripheral stigmata of infective endocarditis (IE) were noted on exam, based on the results of the TEE and blood cultures, our patient did meet Duke Diagnostic Criteria of definite IE [].
Agar diffusion antibiotic susceptibility testing by ETEST® 2 (bioMérieux) revealed a penicillin susceptible strain of Aerococcus: (PCN-G MIC < 0.023 mcg/mL). Antimicrobial treatment was changed to a combination of intravenous (IV) penicillin G and gentamicin. Antimicrobial susceptibility results were only available for three other drugs: ETEST revealed MICs of 0.50 mcg/mL and 24 mcg/mL for Vancomycin and Levofloxacin, respectively. A ceftriaxone Kirby-Bauer disk demonstrated a 31 mm zone of inhibition around the ceftriaxone disk consistent with the susceptibility of the organism to this antibiotic. Gentamicin susceptibility testing was not performed.
Although the initial urine culture on admission was negative as well as a repeat urine culture following adjustment of antibiotic treatment (again while the patient was receiving ongoing antibiotics), two critical factors led the treatment team to the consensus that the original source of infection was the urinary tract with subsequent systemic dissemination and progression to IE: the patient had been on oral amoxicillin treatment for streptococcal pharyngitis prior to admission; and the patient had a history of hydronephrosis requiring ureteral stent placement.
Morbidity and mortality associated with medical therapy alone for treatment of prosthetic AV Aerococcus IE is not known, but due to the size of the vegetation in our patient, the cardiovascular surgery service (CVS) was consulted. He was considered high risk for repeat aortic valve replacement by CVS and recommended medical therapy with IV antibiotics. Once our patient was stable, he was discharged to the skilled nursing facility to complete IV antibiotic treatment. Our patient ultimately completed a full six-week course of combination IV penicillin G and gentamicin antibiotic treatment for bioprosthetic aortic valve endocarditis. He was seen back in the infectious disease office following completion of treatment and repeat blood cultures were obtained which were negative.
Despite negative cultures and achieving an apparent cure of Aerococcus endocarditis, our patient continued to develop serious medical conditions following completion of the six-week course of antibiotics. Three months after his initial presentation he developed an incarcerated ventral hernia requiring urgent surgical repair. One month following the urgent hernia repair he underwent elective ureteroscopy with extraction of a left kidney stone and ureteral stent exchange. He received amoxicillin for endocarditis prophylaxis pre- and post-operatively, and tolerated both procedures well without any complications. Unfortunately, our patient ultimately developed progressive heart failure and eventually died one year following his original presentation with Aerococcus endocarditis.


A previously healthy 31-year-old man experienced a sudden onset of severe left loin pain radiating into the left lower abdomen during a side-movement, while performing a workout of the abdominal muscles in a fitness club. He collapsed and was immediately brought to our emergency department. Vital signs were blood pressure 130/75 mmHg, heart rate 64/min, height 185 cm and weight 85 kg. The clinical examination was unremarkable. Relevant laboratory parameters were a serum creatinine of 1.2 mg/dL, blood–urea nitrogen of 15 mg/dL, creatinine kinase of 24 IU/L (25°C) and a normal urinalysis. Abdominal CT revealed the absence of perfusion of the lower and middle pole of the left kidney, corresponding to an 80% perfusion deficit (A). Subsequent angiography demonstrated a dissection and thrombotic occlusion of the left renal artery (B). Multi-step local lysis was performed within 6 h and on the following day, using 7 mg of recombinant tissue–plasminogen activator (rt-PA), respectively. As estimated by final angiography, perfusion could be essentially restored (C). On Day 2, LDH rose to 1950 U/L (25°C), demonstrating tissue damage, and serum creatinine was 1.5 mg/dL but reached a steady state at 1.3 mg/dL during the next days. Subsequently, blood pressure remained normal and the patient was discharged on oral phenprocoumon for 6 months without antihypertensive medication. At 6-month follow-up, blood pressure was 110/75 mmHg, serum creatinine was 1.0 mg/dl and creatinine clearance was 95 mL/min. Doppler ultrasonography showed a renal arterial resistive-index of 0.6 bilaterally, indicating the absence of re-occlusion or re-stenosis. The lysed kidney had shrunk by 2 cm in length but ∼50% of its function was preserved, as demonstrated by scintigraphic excretory testing (73% on the right, 27% on the left kidney). At 5-year follow-up, blood pressure was 125/75 mmHg, serum creatinine was 1.1. mg/dl and creatinine clearance was 131 mL/min. Renal ultrasound and Doppler sonography showed an unchanged situation.


A 71-year-old male presented with dysphagia over a one year period. He initially underwent fiber optic endoscopy that revealed an anterior mass in the midline projecting into the middle pharynx []. Plain cervical radiography revealed OALL extending from the C4 to C7 levels with beak-like OALL maximal at C4-5 []. The magnetic resonance (MR) scan and computed tomography (CT) scan both confirmed marked anterior OALL projecting into the middle/distal pharynx. There was minimal cervical cord compression at the C3-4 disc level due to OPLL [Figures and ]. The maxillofacial surgeons performed lateral video fluoroscopy (LVF) documenting a disturbance in the oesophageal phase, and reflux at the level of the beak-like projection []. The patient was initially conservatively managed and was prescribed a Kampo medicine (Hange-koboku-To, TJ-16).
With increasing symptoms (e.g., frequent cough/hoarseness), the patient was reassessed the next year for surgery. There was difficulty during intubation due to the severe beak-like deformity and it was performed using enhanced direct video larygoscopy (McGRATH MAC, Medtronics, USA). A microscopic anterior cervical approach was utilized for removal of the OALL. It was largely removed with a bone rongeur and then smoothed using a high-speed drill [Figure and ] and ultrasonic bone curette (SONOPET, Stryker, USA, ). The OALL was removed at C4-5 level and extended to C3-4 as more pathology was found intraoperatively. Postoperatively, the patient's dysphagia resolved immediately, and his hoarseness improved. Adequate decompression of the OALL was confirmed on the lateral plain radiograph [] and CT [Figures and ] studies. Additionally, the LVF study one-month later confirmed improved swallowing [].


A 13-year-old boy presented with a history of collision of a two-wheeler with a wall 24 h previously with one episode of gross haematuria and persistent right hypochondriac pain. Examination showed pulse of 104/min and blood pressure of 110/70 mmHg. Abdominal examination revealed right hypochondriac tenderness and guarding. Investigations showed haemoglobin of 9.4 g/dL, elevated liver transaminases and normal urine examination. Computed tomography (CT) scan showed normal left kidney, devascularisation of lower two-third of right kidney with haematoma with perfusion of only upper polar parenchyma [], urinary extravasation, non-visualisation of right ureter, liver laceration and perisplenic collection. Angiographic reconstruction [] showed right single renal artery with only upper polar parenchymal branches being intact and anomalous low insertion of the left renal vein to inferior vena cava (IVC).
Due to the abdominal guarding, extensive nonviable parenchyma and urinary extravasation, decision for exploration and right nephrectomy was taken. Initially, left flank semi-oblique position was given. First umbilical and left-sided abdominal ports were placed to allow spleen and visceral inspection. Later, position was changed to right flank semi-oblique to proceed with right nephrectomy. Laparoscopy (video at ) showed liver laceration that was not actively bleeding along with perisplenic collection, but no splenic injury. A large right perirenal non-expanding haematoma was seen haematoma after reflecting colon []. Kocherization of duodenum allowed interaortocaval dissection and control of right renal artery at its origin before haematoma manipulation []. Low insertion of left renal vein made this step easier []. Haematoma was then opened; right renal vein was clipped [] followed artery []. Nephrectomy was subsequently completed with specimen removal by morcellation. Patient had uneventful recovery with a post-operative creatinine of 0.7 mg/dL.


A 47-year-old male was brought to the Emergency department by ambulance after a high speed motor vehicle accident. On arrival, he had a Glasgow Coma Scale of 13, a patent airway with cervical spine collar in situ, spontaneous tachypnoeic breathing with an oxygen saturation of 97% on 15 l of oxygen. He had unequal breath sounds with decreased air entry on the left. He was tachycardic with a blood pressure of 110/70 mmHg. There were multiple bruises and abrasions to his torso and bilateral lower limbs. He had no previous medical history and was otherwise healthy. Initial chest x-ray was suspicious for a left-sided haemopneumothorax (Fig. ) and an intercostal chest tube (ICCT) was inserted to the left side with a repeat x-ray confirming its position. However, there was minimal drain output and little swing. Subsequent computed tomography (CT) scan of the chest detected a left diaphragm rupture with a large diaphragmatic hernia where most of the left thorax was replaced by intra-abdominal contents (Fig. ). A decision was made for emergency laparotomy to repair the diaphragmatic hernia. The patient was intubated in the emergency department prior to transfer to the operating theatre. While no ventilation problems were noted during transfer, the patient’s abdomen was seen to become progressively more distended to a point where it became tight and tympanic on percussion (Fig. ). An underlying lung injury was suspected and the cardiothoracic team was called. At laparotomy, a significant pneumoperitoneum was confirmed with sudden escape of air upon entering the peritoneal cavity. The large hernia containing spleen, stomach and small bowel was reduced and the posterolateral diaphragmatic defect was repaired with a non-absorbable suture. This was followed by a left thoracotomy that identified a superficial laceration in the left upper lobe of the lung which was repaired with Prolene sutures and re-enforced with tissue glue. The patient was then transferred to the intensive care unit in a stable condition and made a good recovery. It is likely that the lung injury was caused secondary to intercostal catheter insertion during resuscitation in the Emergency Department. However, it could also have been secondary to associated left rib fractures or blunt trauma during the accident.


A 60-year-old female with history of diabetes and hypertension presented to the hospital with nonmetastatic intraductal breast carcinoma. She was scheduled for elective right modified radical mastectomy 24 days after completing four cycles of cyclophosphamide and doxorubicin based chemotherapy.
On the day of surgery, the patient was afebrile, with heart rate 130/min and blood pressure 110/70 mmHg. Tachycardia was assumed to be due to anxiety, and patient underwent surgery under general anesthesia. Intraoperative course was uneventful except for persistent tachycardia ranging 110–130/min which did not respond to deepening plane of anesthesia, repeated doses of fentanyl for analgesia, and fluid bolus of 500 ml (10 ml/kg). Surgery lasted for two hours, and after extubation, patient was conscious, pain free, and afebrile.
Tachycardia (110–130/min) persisted in the postoperative period. Four hours after extubation, the patient developed significant hypotension with mean arterial pressure (MAP) staying in range of 55–60 mmHg despite fluid boluses. In the next 6 h, the patient received almost 5400 ml (100 ml/kg) of intravenous fluid and required noradrenaline infusion 0.07 μg/kg/min to maintain her MAP of 60–65 mmHg. Invasive hemodynamic monitoring was commenced. Arterial blood gas showed severe metabolic acidosis with high lactates (HCO3: 9.4 mmol/l, Base deficit 18.5, lactate: 8.8 mmol/l). Central venous pressure was 20 cm H2O. The patient was intubated and ventilated in view of severe hemodynamic instability. The patient remained oligoanuric (60 ml over 8 h) with dark-colored urine. Ten hours after surgery, patient was anemic (6 g/dL) and thrombocytopenic (26 × 109/L) requiring transfusion of packed red blood cells (PRBC) and platelets.
Electrocardiogram showed sinus tachycardia, and echocardiography revealed global hypokinesia with left ventricular ejection fraction of 35%–40%. Her blood sugar was in the range of 90–140 mg/dl (5–7.78 mmol/l).
Septic shock with multiorgan dysfunction was strongly suspected due to presence of circulatory shock, severe metabolic acidosis and coagulopathy. Antibiotics were empirically escalated to meropenem.
Over the next 24 h, patient continued to deteriorate requiring incremental doses of vasopressors and developed multiorgan failure. Hemolysis was suspected due to persistently low hemoglobin (<8 g %) in spite of three units of PRBCs transfusions, hemoglobinuria on dipstick, rising serum bilirubin (6.4 g%), high lactate dehydrogenase >1500 IU/L. The peripheral smear [] revealed fragmented RBCs and P. falciparum with high parasitic index (>10%). Intravenous artesunate was started. However, the patient succumbed within next 12 h. Patient's blood culture which was collected before antibiotic escalation did not grow any organism.


A 61-year-old male hailing from Pondicherry, South India, presented with a history of intermittent high-grade fever since 1 month, swelling of right knee joint since 1 month which was associated with severe pain, and inability to weight bear. He gave a history of inability to walk due to knee pain since 1 week. Patient was treated for right knee pain, swelling, and for urinary tract infection in another health-care facility before being referred to our hospital. Details of previous treatment are not known. He was a known case of type II diabetes mellitus on treatment for few months. Patient gave a history of sudden onset breathlessness since the evening before admission. On arrival at the emergency department, he was conscious, febrile, tachycardic, tachypnoeic, and hypotensive with reduced saturation. He had extensive bilateral crepitations.
On examination, patient had right knee swelling which was warm and tender. A provisional diagnosis of septic arthritis right knee with severe septicemia in septic shock and type 2 diabetes mellitus was made. In view of severe respiratory distress and hypotension patient was intubated, fluid resuscitated and was started on inotropes and vasopressors. Patient was transferred to critical care unit for further management, intravenous meropenem and teicoplanin were administered immediately. This empiric cover was chosen because patient on presentation had septic shock with hemodynamic instability and had been treated for past few days in an outside hospital with broad-spectrum antibiotics. Blood gas analysis revealed severe metabolic acidosis with lactic acidemia. Patient had anuria, and his baseline investigations revealed severe leukopenia, deranged liver function, renal function, and coagulation profile []. These investigations were carried out during patient's admission to the Critical Care Unit. On the same day, patient developed ventricular tachycardia which was cardioverted. He subsequently developed bradycardia and was resuscitated with three cycles of cardiopulmonary resuscitation as per Advanced Cardiac Life Support protocol, but despite all efforts, he succumbed to the illness within 24 h of admission.
Blood culture was taken at the time of admission. Within 12 h of incubation in the BACTEC automated system, a Gram-negative organism with typical bipolar staining was observed on Gram stain. On blood agar, dry, wrinkled hemolytic colonies were seen. On MacConkey agar, dry, wrinkled colonies with metallic sheen were observed []. The organism was motile, oxidase positive, utilized citrate, and produced alkaline reaction on triple sugar iron agar. Based on the colony morphology and staining properties, upon suspicion of Burkholderia pseudomallei, antibiotic susceptibility testing was done for selective antibiotics according to CLSI 2016 M100S (26th edition) document. Polymyxin B minimum inhibitory concentration (MIC) was done by E-test and found to be resistant with no zone of inhibition. Antibiogram was also suggestive of B. pseudomallei with typical resistance to aminoglycosides and susceptibility to ceftazidime, co-trimoxazole, and carbapenems. The isolate was further confirmed to be B. pseudomallei by the VITEK 2 automated system from our multispecialty tertiary care hospital laboratory with NABL and Joint Commission International accreditation.


In February 2015, a 2 months old male baby was admitted to the hospital with gross hematuria and generalized edema. He was born at term with an uncomplicated perinatal course and normal growth. The mother was a 27 years old Korean who had regular prenatal care. He had been healthy until the week before admission. Brown urine had developed 7 days prior to admission, and intermittent edema was detected after birth. At that time, his height was 56.5 cm (3–5th percentile), weight was 6.4 kg (25–50th percentile), and head circumference was 38.5 cm (10–25th percentile). A general physical examination revealed mild soft tissue edema and a distended abdomen without hepatosplenomegaly. There were no mucocutaneous symptoms such as skin eruption, desquamation, or snuffles and no limitations in the movement of extremities.
Complete blood cell counts demonstrated normocytic normochromic anemia (hemoglobin, 8.0 g/dL and hematocrit, 24.7%) with leukocytosis (white blood cells [WBC], 26,600/mm3). Serum electrolytes, liver, and renal function tests were within normal ranges. His C-reactive protein was elevated (8.4 mg/dL), and total protein and albumin levels were low (3.8 and 1.4 g/dL, respectively). Urinalysis revealed 4+ proteinuria, erythrocytes, and WBCs. The urinary protein/creatinine ratio was 62.5. Congenital nephrotic syndrome (CNS) was diagnosed and cause of CNS include congenital infection was evaluated at the first day of admission. Serologic tests for rubella, herpes simplex virus, and toxoplasmosis were all negative. He was positive for cytomegalovirus (CMV) immunoglobulin G (IgG) and immunoglobulin M (IgM) antibodies. However, CMV was not observed in culture and polymerase chain reaction analysis of urine and blood. Antinuclear antibody was positive with a cytoplasmic pattern, but anti-dsDNA and anti-Smith antibodies were negative and accompanied by normal complement levels. On the fourth day, a rapid plasma reaction (RPR) test and blood venereal disease research laboratory (VDRL) test were positive with titers of 3.4 RPR unit and 1:16, respectively. Despite no other symptoms of syphilis, a treponemal test was performed to confirm the diagnosis. The T. pallidum hemagglutination assay (TPHA) was reactive at a dilution of 1:1,280 and fluorescent-treponemal antibody-absorbed test was also reactive. Cerebrospinal fluid showed a normal cell count and protein level; however, the VDRL and TPHA were positive. Radiographs of both limbs disclosed symmetric single-layer periosteal reaction. Cranial ultrasonography was normal, and neurological examination including hearing test was normal. After diagnosing CS, his mother was tested for syphilis, and her RPR was positive at 24 RPR units. Nevertheless, the mother showed no specific symptoms. The results of her routine antenatal screening test were positive for syphilis; no treatment was done due to hospital error.
The baby was treated with penicillin G for 15 days. His anemia improved slowly during 2 weeks of hospitalization. In addition, his total protein and albumin levels increased slowly to 5.0 and 3.0 g/dL, respectively. Proteinuria and hematuria improved within several days. The VDRL titer decreased from 1:16 to 1:8 during treatment. No CMV IgG antibody was detected at the 2-week follow up, so it was considered transplacental transmission during pregnancy. Renal biopsy was not performed because of the remission of proteinuria.
Follow-up at 12 months after discharge, baby showed normal growth and development. His urinalysis result was normal with a normal level of albumin, and the RPR was non-reactive.


In August 2012, a 64-year-old, male smoker presented with chest pain. A computed tomography (CT) scan showed a right lung nodule with rib metastasis (stage IV). His carcinoembryonic antigen (CEA) serum level was 12.5 ng/mL, while his neuron specific enolase (NSE) level was unremarkable. Bronchial biopsy revealed an adenocarcinoma. A multiplex analysis by MALDI-TOF with LungCarta Panel (Agena Bioscience, Mountain View, CA, USA) yielded no mutations in EGFR, K-RAS, BRAF and in the other tested genes (AKT1, ALK, BRAF, DDR2, EGFR, EPHA3, EPHA5, ERBB2, FGFR4, JAK2, KRAS, MAP2K1 , MET , NOTCH1 , NRAS, NRF2, NTRK1, NTRK2, NTRK3, PIK3CA, PTCH1, PTEN, PTPN11, PTPRD, STK11, TP53). ALK and ROS-1 rearrangement by immunohistochemistry (IHC) and fluorescence in-situ hybridization (FISH) were negative. The patient received cisplatin/pemetrexed chemotherapy for four cycles and achieved a partial response (PR). Afterward, the patient underwent an atypical lung resection with a concomitant biopsy of the rib lesion. The original histology was confirmed. Because the bone lesion was not radically resected, radiotherapy to the rib (45 Gy) was performed. Radiological follow-up showed no clear signs of disease progression during the subsequent three years. In February 2015, a CT scan showed a significant increase in the lung lesion with concomitant enlargement of mediastinal lymph nodes. While CEA levels ranged from 2.5 to 5.6 ng/mL, a rapid increase of NSE (25.6 μg/L) was observed. As a part of re-evaluation work-up, a secondary bronchial biopsy revealed small cell lung cancer (SCLC). Based on this data, the patient was treated with four cycles of carboplatin/etoposide and once again achieved PR (Fig ). The patient is still alive after 44 months of follow-up.


In May 2013, a 59-year-old, male smoker presented with cough and hemoptysis resulting from a mass located in the right upper lobe and involving hilar lymph nodes. NSE and CEA serum levels were 14 μg/L and 1.6 ng/mL, respectively. A bronchial biopsy revealed SCLC (stage T3N2M0). The patient achieved PR after four cycles of cisplatin/etoposide and sequential radiotherapy (57.2 Gy). Eight months later, ground-glass opacity with was detected during a CT scan of the right upper lobe. A slight increase in CEA (13.2 ng/mL) and a decrease in NSE (8 μg/L) levels were noted. The substantial modification of the radiological scenario combined with the fluctuation of serum markers led us to perform a secondary biopsy. A “wild-type” adenocarcinoma with lymphangitic spread was diagnosed. Molecular and immunohistochemical analyses were completely negative for targetable mutations. The patient commenced a second-line regimen with carboplatin/pemetrexed (six cycles) followed by maintenance with pemetrexed (three cycles, then halted as per the patient’s wishes), and achieved PR (Fig ). No peculiar toxicity was reported from the second-line treatment. The patient is alive and well after 36 months of follow-up.


A 68-year-old lady, para 3, presented with an episode of postmenopausal bleeding. A transvaginal ultrasound scan revealed an endometrial thickness of 5 mm. An outpatient hysteroscopy was done at 1450 h. The procedure was uneventful, cavity, and ostia appeared normal. Endometrial biopsy was not possible as the patient found it painful.
Within 15 h of the procedure, she presented to the emergency department (A and E) with abdominal pain, nausea, vomiting, fever, sweats, and rigors. On examination, she was tachycardic, hypotensive, and had an oxygen saturation of 92%. Examination findings were unremarkable. A high vaginal swab (HVS) was sent for microbiological assessment. Her bloods in the A and E were normal. An immediate computed tomography (CT) scan of the abdomen and pelvis was requested to rule out any perforation or collection in the pelvis.
While awaiting results, the patient started to desaturate. Her temperature spiked to 38.1°C, her blood pressure started falling to 84/48 mm Hg by mid-day. Arterial blood gases (ABGs) were done revealing a normal pH with a lactate of 3.6. She was admitted to the Intensive Treatment Unit (ITU) for septic shock and inotropic support. Regular ABG's revealed a worsening lactate. She was started on augmentin, metronidazole, and a stat dose of gentamicin. Procalcitonin, which is a sepsis marker, was more than 10.0 μg/L.[] With microbiology input, the metronidazole was replaced with gentamicin. The patient responded to the antibiotics. The fever started coming down, her lactate levels started decreasing after reaching a maximum of 4.25.
On day 3 of admission, she was stable enough to be stepped down to high dependency unit. Augmentin was replaced by 1.2 g benzylpenicillin QDS and clindamycin was added. Inotropes were stopped. She was then transferred to the gynecology ward. She was afebrile, vitals stable. Her antibiotics were switched to oral formulations. She was discharged home 8 days later.
Her investigations revealed a normal CT scan, ruling out a perforation, and any pelvic collection, which could have been a nidus for an infection. The HVS revealed a growth of beta-hemolytic Group A Streptococci (GAS). The blood cultures sent on the day of admission also revealed a growth of the same, thus confirming her diagnosis of having septic shock following GAS.


A 64-year-old white woman presenting cholestatic jaundice and weight loss. A solid lesion (3.5 × 2.2 cm2) in contact to the SMV posterior wall in the inferior border of the pancreas head was identified through multislice computed tomography. The 3D CT vascular reconstruction showed that SMV was formed by two ileal trunks, and that was a tumor in contact to one of them (Fig. ). There was no tumor in contact with the superior mesentery artery. An upper digestive endoscopy and serum Ca 19-9 (2740 U/ml) were performed. The patient was submitted to preoperative enteral nutrition for 22 days. Tumor infiltration into the pancreatic head and into one of the SMV ileal tributaries was confirmed during surgery. The vascular clamp occlusion test showed immediate venous congestion in two-thirds of the distal small intestine (Fig. ). No reconstruction attempt was performed, as well as the resection of the small intestine segment, due to the small size of the vessel and the reduced amount of viable residual intestine. A pancreatic-duodenal lymph node biopsy (frozen section examination: adenocarcinoma) and a biliodigestive anastomosis were carried out. In the postoperative period, the patient presented persistent vomiting, and a gastric bypass was performed. The patient was discharged 8 days later and referred for chemotherapy.


A 28-year-old Indian male was riding a bike when a horizontal metallic rod of a ladder projecting from the rear of a vehicle in front of his bike hit against the anterior part of his neck due to sudden brake by a front vehicle. He lost his control and sustained blunt trauma to the neck. He was brought to the emergency department with breathing difficulty and inability to speak. On examination, he had surgical emphysema over neck and chest and a lacerated wound over the left parotid region. There was no sign of external trauma over larynx in the form of swelling, laceration, or bruising. The patient was unable to speak and had stridor in lying down position. Since a laryngeal injury was suspected on the basis of mode of injury, tracheostomy was done. X-ray chest revealed pneumomediastinum. The wound over parotid developed salivary collection which was drained and pressure dressing applied. Computed tomography (CT) scan of the neck was obtained after 3 days after general condition of the patient stabilized. It revealed a comminuted fracture of the left lamina of the thyroid cartilage with two fracture lines, one vertical in midline displacing the left lamina and other horizontal in the left thyroid lamina along with chip fracture of cricoid displacing the arytenoid anteromedially [Figures and ]. Preoperative videolaryngoscopy showed narrowed glottic chink even on deep inspiration and gap between the anterior commissure and vocal cords fallen back [Figures and ]. Based on endoscopic and CT findings, the laryngeal injury was graded as SF Grade IV []. He was taken to the operating room and underwent an open reduction and internal fixation (ORIF) of the thyroid cartilage with 4–0 prolene sutures [Figures and ]. Plate fixation was not possible as the cartilage was soft and tight screw fixation was not possible []. Mucosal tears were sutured with 6–0 vicryl. Vocal cords had fallen back and were resuspended with 4–0 vicryl to external perichondrium of thyroid cartilage. Endolaryngeal stenting was done with 3.5 mm long polyvinylchloride (PVC) endotracheal tube of 6 mm external diameter and closing the top end by sutures to prevent aspiration []. The portion of the tube approximating the true and false cords was narrowed with a clamp. The stent was positioned to extend up through the true and false vocal cords and inferiorly to first tracheal ring and secured with 4–0 prolene sutures that traversed the stent superiorly through the laryngeal ventricle and inferiorly through the cricothyroid membrane. The sutures were passed through the strap muscles laterally and then through a button, secured to the skin to be removed later at the time of stent removal. Injectable antibiotics – piperacillin and tazobactam, gentamicin, and clindamycin along with dexamethasone – were given along with nebulization with acetylcysteine and adrenalin to decrease secretions and edema. After 11 days, stent removal was done by direct laryngoscopy. Videostroboscopy performed 2 weeks after the event showed reduced left vocal cord mobility with normal anterior commissure and reasonably patent glottic chink []. He had slight aspiration problems initially which he overcame by swallowing maneuvers. There is no significant swallowing or breathing problem after 6 months with persistent mild hoarseness of voice, for which he is receiving speech therapy.


A 78-year-old man presented with acute onset of severe retrosternal chest pain associated with shortness of breath. He had a history of non-obstructive coronary artery disease and severe aortic stenosis (aortic valve area = 0.9 cm2, peak velocity = 4.2 m/s, mean gradient = 38 mm Hg) status-post successful transfemoral TAVR with 25-mm direct flow valve 6 months prior, which was complicated by heart block requiring permanent pacemaker implantation. Non-selective coronary angiography at that time showed non-obstructive coronary artery disease with TIMI flow grade 3 (). Before the procedure, CT angiogram noted a coronary ostia height of 11.6 mm, a sinus of Valsalva (SOV) diameter of 28.7 mm and a sinotubular junction diameter of 20.3 mm.
At the time of current presentation, on arrival to the emergency department, the patient was found to have a blood pressure of 80/50 mm Hg; the remainder of his vital signs and physical examination findings were within normal limits. Electrocardiogram revealed sequential atrioventricular-paced rhythm with 7 mm ST elevations in inferolateral leads (). The patient was taken for emergent cardiac catheterization and prior to the procedure he had cardiac arrest due to ventricular tachycardia. Cardiopulmonary resuscitation was initiated and direct-current cardioversion restored normal rhythm. Coronary angiography demonstrated ostial flush occlusion of the right coronary artery (RCA) (). The patient’s blood pressure deteriorated rapidly during the procedure and an intra-aortic balloon pump was placed. The patient had another prolonged cardiac arrest due to ventricular fibrillation and cardiopulmonary resuscitation was resumed, unfortunately his rhythm could not be restored and the patient passed away.


Patient 1: The patient 1 is a 21-year-old man who complained of episodic stiffness of extremities and trunk when he attempts to start sudden exercise. He reported that his symptoms began at age 12 and none of his family members had the same symptoms. He denied episodic muscle weakness or cold sensitivity. His muscle strength was normal and no muscle hypertrophy was observed. Laboratory tests were unremarkable except for the mildly elevated serum CK levels (463 IU). Needle EMG showed myotonic discharges. Mexiletine were prescribed and the symptoms were moderately alleviated.


Patient 2: This 20-year-old man first recognized trouble in exercising at high school, when he was 16 years old, and since then exercising was accompanied by discomfort all along. There was no family history of the same symptoms he suffered. He denied a transient muscle weakness, but stated that his symptom tends to get worse when exposed to the cold temperature. Muscle hypertrophy or muscle weakness was not observed, and needle EMG showed myotonic discharges.


Patient 3: A 22-year-old man suffering intermittent muscle stiffness for two years visited our neuromuscular clinic for evaluation of his symptom. He had no affected family member, and denied episodic muscle weakness or cold sensitivity. Serum CK level was slightly elevated (203 IU), and needle EMG showed myotonic discharges in his muscles. Muscle strength was normal and muscle hypertrophy was not observed. Muscle biopsy did not show any specific abnormality. Although mexiletine and carbamazepine were tried to alleviate his symptom, the response was poor and medications were discontinued.


Patient 4: The patient 4 was a 42-year-old lady who complained occasional muscle stiffness following sudden exercise since 20 years old. Her family history was compatible with autosomal dominant inheritance. She had a mild hypertrophy in her thigh and calf muscles. Serum CK level was normal (111 IU), and needle EMG showed myotonic discharges. Muscle biopsy did not show any significant abnormality. Carbamazepine showed a dramatic alleviation of her symptom.


A 2-year-old girl was resuscitated at Washington Regional Medical Center, Fayetteville, AR, USA, from a Glasgow Coma Scale (GCS) of 3, fixed dilated pupils, and body temperature of 85.1°F (28.9°C) after a 15-minute submersion in 41°F (5°C) water. After 100 minutes of cardio-pulmonary resuscitation, the arterial pH was 6.53. Following hypothermia, vasopressors, ventilator support (10 days), and critical care at Arkansas Children’s Hospital, Little Rock, AR, USA, the patient was discharged home 35 days post drowning unresponsive to all stimuli, immobile with legs drawn to chest, and with constant squirming and head shaking. MRIs at 3 () and 31 () days post drowning showed thalamic injury then generalized atrophy with evolving gray and white matter injury.
Author Paul G. Harch was consulted and commenced 2 L/minute nasal cannula 100% oxygen for 45 minutes twice/day bridging normobaric oxygen therapy at 55 days post-drowning. Within hours the patient was more alert, awake, and stopped squirming (see the movie at . Entire movie is pre-, and post-drowning up to pre- and post-normobaric oxygen therapy, shown at 7 minutes 40 seconds mark and then 9 minutes mark). Neurological improvement rate increased over the ensuing 23 days with laughing, increased movement of arms, hands, grasp with the left hand, partial oral feeding, eye tracking, and short sentence speech (pre-drowning speech level, but with diminished vocabulary). Physical (PT), occupational (OT), and speech therapy (ST) were added on the 10th day for 2 weeks.
Seventy-eight days post-drowning, hyperbaric oxygen treatment (HBOT) commenced with compressed air at 1.3 atmosphere absolute (1 ATA = 131.7 kPa) for 45 minutes total treatment time, once daily. Within hours the patient experienced decreased tone, increased gross motor activity, vocabulary, and alertness. After 10 sessions of HBOT, the patient’s mother reported that the patient was “near normal, except for gross motor function;” PT was then reinstituted. After 39 sessions of HBOT, the patient exhibited: assisted gait, speech level greater than pre-drowning, near normal motor function, normal cognition, improvement on nearly all neurological exam abnormalities, discontinuance of all medications (buspar, propranolol, baclofen), and residual emotional, gait, and temperament deficits (). Gait improvement was documented immediately upon return home at . MRI at 27 days after 40-session HBOT and 162 days post-drowning demonstrated mild residual injury and near-complete reversal of cortical and white matter atrophy (Figures –), while further improved gait was documented at: .


A 77-year-old man visited an emergency department for chest pain that developed 1 hour before the hospital visit. The pain began when he washed his face after getting out of bed in the morning. The patient felt a tightening pain in the chest, through the back, and in the area between both the scapulae. The patient was taking aspirin 100 mg, clopidogrel 75 mg, and metformin 500 mg for hypertension and diabetes. He visited the hospital one year prior for a similar pain and underwent a stent insertion in the left anterior descending coronary artery for ST elevation myocardial infarction. When the patient visited the hospital this time, the pain was rated as an 8 out of 10 and continued even after sublingual administration of the patients own nitroglycerin. Vital signs were as follows: blood pressure, 166/88 mmHg; pulse rate, 72 beats/min; respiratory rate, 15 breaths/min; and body temperature, 36.7°C. Sinus rhythm and first-degree atrioventricular block were observed on electrocardiography. Bedside echocardiography showed similar results with the previous one, except for a decrease in ejection fraction to 38%. Laboratory data including hemoglobin, platelet count, white blood cell, blood chemistry, and cardiac enzyme test as well as urinalysis were normal. Thirty minutes after the hospital admission, the chest pain was relieved; however, the pain through the scapula remained. Therefore, aortic dissection was suspected and an aortic dissection computed tomography was obtained. The computed tomography showed multiple dissections at an infrarenal level. Since the patient had slipped and fallen 3 days prior, spinal radiography was performed and revealed an acute compression fracture at T8. Four hours later, he complained of numbness in the entire left leg below the knee. The numbness alternately occurred in the left and right leg during a neurological examination but the motor activity was measured as grade 5. Spinal magnetic resonance imaging (MRI) was obtained to check if a spinal cord injury was caused by the compression fracture, but there was no definite signal change in the spinal cord (). Six hours after the visit, the upper back pain increased in severity to the point that the patient was unable to lift his knee up, and the motor activity of the lower limb decreased to grade 3. A spinal cord infarction was suspected and the patient was admitted to the department of neurology. Nine hours after the hospital visit, a sudden sensory change–hypoesthesia (3/10)–occurred, loss of sensation progressed up to the T4 dermatome, the strength in both lower extremities deteriorated to grade 0, and a decrease in anal tone and deep tendon reflex was observed. The patient was numb to pain and temperature stimuli, but vibratory sensation was intact. Steroid pulse therapy (methylprednisolone 1 g/day) was initiated. Seventy-two hours after the hospital admission, no change in the neurological symptoms was observed. A follow-up spinal MRI showed a signal change with a diffuse pattern within the spinal cord at T2-T6 (T2 hyperintensity, T1 hypointensity without definite enhancement), and spinal cord swelling was observed (). Steroid pulse therapy was continued for 5 days. After a month of hospitalization, touch sensation in his right big toe had improved slightly, but the patient was still undergoing rehabilitation treatment while showing no improvement in the other neurological symptoms.


A 52-year-old man presented with lower limb edema and hypertension. His past medical history was notable for appendicectomy in 1995 and hypertension since 1 year treated with ramipril 5 mg daily. On admission to hospital, he was hypertensive (blood pressure 160/95 mmHg) and had peripheral edema. There was no rash and no peripheral lymphadenopathy.
Laboratory results were as follows: serum albumin 17 g/L, serum protein 51 g/L, 7 g proteinuria from a 24 hr collection, and hematuria 2 × 105/ml, normal electrolytes, serum creatinine: 88 μmol/L, C-reactive protein: 1 mg/L, hemoglobin: 11.3 g/dl, white cell count: 3.7 × 109/L, platelet count: 162 × 109/L, neutrophil count: 1 × 109/L, and lymphocytes count 2 × 109/L. Tests for autoantibodies including antinuclear antibody, anti-DNA were negative. Serum C3 complement was 0.88 g/L and C4 was 0.22 g/L. Serum protein electrophoresis showed polyclonal hypergammaglobulinemia. Kidney ultrasound revealed normal sized kidneys (right 11.5 cm and left 11 cm) without structural abnormalities.
A kidney biopsy was performed. Light microscopy revealed 18 glomeruli showing diffuse and global endocapillary proliferation, mesangial matrix expansion, massive subendothelial deposits, and double contours of glomerular capillary wall on silver stain. There was moderate interstitial inflammation. Immunofluorescence microscopy disclosed IgG, C3, and C1q reaction along the mesangium and the peripheral segments of the glomeruli. Type 1 MPGN's diagnosis was rendered. Hepatitis B surface antigen, hepatitis C antibodies, and cryoglobulins were negative. HIV 1, 2 antibodies screening by ELISA was twice positive. Western blot essay was positive for GP120, GP41, GP160, P17, P24, P31, P55, and P66. Viral load by polymerase chain reaction was 200 copies/ml.
The patient was treated with furosemide 40 mg daily, ramipril 5 mg daily, simvastatin 20 mg daily, and antiretroviral therapy including lamivudine 150 mg twice daily, zidovudine 300 mg twice daily, and efavirenz 600 mg daily. After 8 weeks, the edema resolved, viral load decreased to 46 copies/ml, 24-h protein decreased to 0.5 g/24 h, and serum albumin increased to 35 g/L.