A 15-year-old girl presented to the Neurology department of our hospital with behavioral problems, irritability, progressive academic decline and generalized dystonia also affecting speech. She had corneal Kaiser Fleschner (KF) ring. Serum copper was 54 μg/dl (normal – 70-140 μg/dl), serum ceruloplasmin was 0.002 OD/unit (normal – 0.2-0.5 OD/unit), 24 h urinary copper was 87 μg/day (normal – 20-50 μg/day) and MRI brain showed T2 hyperintensities in bilateral globus pallidus and substantia nigra. Liver function test was normal. She was diagnosed to have Wilson's disease and started on d-penicillamine, initially 250 mg/day, which was titrated over months to 3 gm/day along with zinc acetate. Dystonia improved partially and the patient was able to take orally. After two and half years of starting D-penicillamine, she developed tense vesico-bullous lesions filled with clear and hemorrhagic fluid over trauma- prone sites that ruptured in 7-10 days and healed with scarring in 1-2 months. She also complained of increased laxity of skin over these areas as well as all skin folds. On examination, the patient had severe generalized dystonia involving face, jaw, neck, trunk and limbs, with rigidity, and the deep tendon jerks were difficult to elicit. Dermatological examination revealed vesicobullous lesions and erosions symmetrically distributed over shoulders, elbows, hands, knee, and ankles. There was also evidence of scarring, hyperpigmentation and milia over these sites [Figure and ]. There was increased skin laxity in these areas and over body folds (neck, groin, cubital and popliteal fossae). Tzanck smear made from a blister was negative for acantholytic cells or inflammatory cells. Skin biopsy from a lesion over the hand revealed decreased, thickened and fragmented elastic fibers, highlighted on vVG staining (compared to control–normal skin sample) suggestive of elastolysis [Figure and ]. Patient was also evaluated for other side effects of D-penicillamine. Urine was normal without any proteinuria (0.02 gm/24 hour urine). CT chest incidentally revealed bilateral reticulonodular shadows suggesting early diffuse interstitial fibrosis. She had microcytic hypochromic anemia secondary to iron deficiency anemia with serum iron 11 ng/nl (normal – 50-150 ug/dl). Recent 24 hour urine copper was < 5 μg/day and serum copper was 31 μg/dl. Echocardiography was normal. Since the copper stores had significantly reduced, it was planned to decrease the dose of D-penicillamine, continue zinc acetate and keep the patient on follow up. After decreasing the dose to 1.5 gm her skin lesions improved dramatically (70-80%) over a period of 4-6 months.


A 29-year-old man, presented to neurology department with gradually progressive stiffness of all four limbs, action tremors in the upper limbs and history of frequent falls. He had history of jaundice at the age of 14 years which was conservatively managed. On examination, there was no evidence of anemia, jaundice or any sign of hepatic failure but he had inappropriate laughter. His Mini Mental status Examination was 17/24 and KF ring was present. There was generalized dystonia and rigidity of all the limbs and trunk and difficulty in writing because of severe action tremor. Investigations showed serum copper as 25 μg/dl, serum ceruloplasmin of 0.058 OD/unit, and 24 h urinary copper of 71 μg/day. He was diagnosed as Wilson's disease and started on zinc acetate and D-penicillamine, which was gradually increased from 1 to 3 gm/day. There was only partial improvement in the rigidity and dystonia. After 4 years of penicillamine therapy, he developed vesicobullous lesions over trauma-prone area that healed with milia formation and scarring []. Skin biopsy revealed fragmented elastic fibers in the reticular dermis. These features were consistent with penicillamine-induced skin fragility. Investigations toward systemic side-effects of D-penicillamine revealed no abnormality. The dose of penicillamine was decreased to 1 gm/day, skin lesions improved significantly after 6 months.
In both cases, this adverse-event to penicillamine yielded a score of 9 on the Naranjo adverse drug reaction (ADR) probability scale (definite ADR if score ≥ 9). It was a ‘certain ADR’ according to WHO-UMC causality category and a level 4 ADR on the Hardwig's Severity Assessment scale.[]


A 60 -year-old lady came to the department with a painful swelling and ulcer in the left groin of 1-year duration. Initially, she developed a painful papule followed by development of few more to form a plaque which subsequently ulcerated to discharge serous fluid. There after she continued to suffer from pain and discharge intermittently. There was no association with fever, cough or weight loss. Cutaneous examination revealed indurated plaque of size 2 cms × 1.5 cms in the left groin with multiple papules and erosion on the surface []. Two discrete lymph nodes were palpable in left inguinal region. Based on these findings she was provisionally diagnosed as a case of scrofuloderma. Actinomycosis, granuloma inguinale, hydradenitis suppurativa and botryomycosis were considered in the differential diagnosis. Routine laboratory investigations: Complete blood picture, blood sugar, blood urea, serum creatinine, X-ray chest, ultrasonography of abdomen were found to be normal. Mantoux-test was negative. Smear and culture for acid fast bacilli and for fungus were negative. Swab for bacterial culture did not yield any growth. Skin biopsy showed numerous tubular structures lined by cuboidal epithelium arranged in single and double rows in the dermis. Some of these cells showed clear cytoplasm [Figures and ]. Immunohistochemistry was strongly positive for AE1 and AE3 marker, [] which confirmed the diagnosis of clear-cell syringoma. As there is deep involvement in the reported case surgical excision is probably ideal. However, the case is lost to follow up.


The patient, CA, an 82-year-old female, suffering from type II diabetes mellitus for over 20 years complicated by peripheral neuropathy and arteriopathy, came to our attention presenting painful skin ulcers of the lower extremities (, day 0 on admission). She was under basal–bolus scheme insulin, and on admission, glycemic values were somewhat uncontrolled (HbA1c 55 mmol/mol, fasting glucose 180 mg/dL). Despite the ongoing analgesic treatment (acetaminophen 3,000 mg daily), pain control was poor and wound treatment was strongly limited by the intense pain. She had also been prescribed tramadol by her general practitioner, stopped early due to nausea and vomiting. On admission, OXN-PR 5/2.5 mg bid (twice a day) was started, and acetaminophen was stopped.
In order to evaluate the effectiveness and tolerability of the OXN-PR association, the following parameters were assessed during the time of observation: pain intensity (on a verbally administered 0–10-point Numeric Rating Scale [NRS], expressed as the mean of assessments at rest, on movement, daytime, and nocturnal of the last 24 hours), and the impact of pain on daily activities according to the Brief Pain Inventory (BPI) of both severity and interference domains, cognitive function using the Mini Mental State Examination (MMSE), and functional autonomy through the Barthel Index score. Bowel function was evaluated using the Bowel Function Index (BFI, a validated reproducible measure that detects clinically meaningful changes in constipation, with scores ranging from 0, free from symptoms, to 100, most severe symptoms; normal bowel function is defined as a BFI value of ≤29). Common opioid-related adverse events (nausea, vomiting, dizziness, drowsiness, dry mouth, and itching) were also monitored.
At baseline, pain was severe and had a markedly negative impact on daily activities. Functional autonomy was moderately impaired, with preserved cognitive status and bowel function ().
Subsequent pain assessment showed a clear reduction in the NRS score, which progressively decreased to 5 after 1 week in the absence of opioid-related adverse events. The dose of OXN-PR was increased to 10/5 mg bid. Residual pain was reported especially in the early hours of the morning and during ulcer dressing procedures. The evaluation after 2 weeks and 4 weeks showed a further reduction in NRS score, which decreased to 2 points during both assessments.
In concomitance with the striking subjective improvement, the lady became more compliant to dressing procedures, significantly contributing to the complete healing of the ulcers in the following 4 weeks ( and ). Throughout this period, no minor or major adverse effects were observed. No significant changes were also found regarding cognitive status and bowel function; at final functional assessment, optimal analgesic control was associated with a marked functional improvement ().


A 57-year old man was admitted to our department with a 10-year history of papular
dermatosis, involving the trunk. The lesions appeared at the same time and were
asymptomatic, except for occasional, mild pruritus during perspiration. He denied any
medical problems and using medications. No other family members had been affected by
similar skin lesions.
Physical examination revealed multiple, brownish erythematous, flat-topped papules 1-4mm
in diameter, distributed on the neck, shoulders, anterior chest, abdomen and axilla
(). The lesions were monomorphic,
bilateral and symmetrical. Darier sign was negative. The remainder of the physical
examination was unremarkable. Laboratory examinations did not show abnormal findings. He
was initially suspected of having cutaneous mastocytosis.
A skin biopsy was obtained from a lesion on the trunk. Histological examination of this
specimen revealed a normal epidermis and a dermal tumor, which was filled with multiple
small ducts embedded in a fibrous stroma. The ducts were lined by two rows of epithelial
cells and some had amorphous, keratinous material in their lumina ().
Taken together, the clinical and histological findings were compatible with the
diagnosis of eruptive syringoma. The patient was treated with low-dose isotretinoin (5
mg/day, alternate days), without any improvement.


A 64-year-old white woman was referred to our clinic for evaluation of symptomatic oral lesions that had been present for 3 days. The patient was complaining of a constant
burning sensation along with roughness and thickness on the oral mucosa. The intraoral examination revealed a detachable white-yellowish plaque on the hard palate and an erythematous patch partially covered by a thin, yellowish, fibrinous plaque on the left buccal mucosa (Figs. and ). Her medical history revealed an ongoing 3-year treatment with antidepressive drugs. She reported being a one-pack a day smoker for 45 years.
Differential diagnosis included mucous membrane pemphigoid, lichenoid contact reaction, erythema multiform and contact stomatitis. The first one was excluded since the onset of the lesions of the present case was abrupt, which is not the usual pattern of the development of oral lesion in mucous membrane pemphigoid. Lichenoid contact reaction was also considered but ruled out later on because the palatal mucosa (where lesions were also present) are never in contact with dental surfaces. Additionally, the patient had no amalgam filling on her upper-teeth. Erythema multiform and nonspecific contact stomatitis were very much possible, prompting additional investigation concerning any recent changes in the patient’s habits. Hence, she reported no particular changes in her habits, except that she had switched the flavor of her mints (brand, Tic-Tac – manufactured in Ireland)* to cinnamon shortly before the appearance of the lesions and considered it a possible cause. The patient was then advised to discontinue the use of cinnamon mints, which she did, obtaining great relief of symptoms, with complete healing of the lesions after 3 weeks (Figs. and ).
In order to confirm the hypersensitivity contact reaction, it was suggested (with the patient’s consent) that she go back to using the mints, what she did, and after a week the lesions recurred, (Figs. and ). Reinterruption of the use of mints resulted once again in resolution of the lesions (Figs. and ). Based on the clear relationship of the lesions and cinnamon flavoring, the diagnosis was contact stomatitis reaction.


A 74-year-old woman was found to have a 2.2-cm liver mass with multiple satellite nodules (T2N0M0) on abdominal-pelvic CT (APCT) in January 2011 (). Protein induced by vitamin K absence or antagonist-II (PIVKA II) was within normal range, 28 mAU/mL, but alpha fetoprotein (AFP), 4040.05 ng/mL, was high, and anti-hepatitis C virus was positive. The patient initially received transarterial chemoembolization (TACE) in February 2011, but locally recurrent hepatocellular carcinoma with multiple pulmonary and mediastinal lymph node metastases were found on positron emission tomography-computed tomography (PET-CT) scan in July 2011 () and APCT in September 2011 (). She refused further treatment, including sorafenib tosylate (Nexavar, Bayer Health-Care AG, Leverkusen, Germany), and then visited our clinic with anorexia and general weakness in September 2011 requesting high-dose vitamin C to manage her symptoms. Twenty grams of vitamin C in 250 mL normal saline was initially administered via an ante-cubital vein twice a week in September 2011 after urine analysis and renal function were confirmed to be within normal range. To neutralize acidic pH (3.5-5.0) of vitamin C, it was mixed with NaHCO3, resulting in pH 6.2 (UniC®, 500 mg/mL from Unimed Pharmaceuticals, Seoul, Korea). In addition, normal saline was changed to distilled water as vitamin C dose increased to avoid too much intake of volume and sodium and too high concentration. Furthermore, magnesium sulfate 1 g was blended in the fluid to prevent vascular irritation. Notably, she did not have glucose-6-phosphate dehydrogenase deficiency. Prior to April 2012, no significant progression or regression of her multiple pulmonary metastases was found on serial chest X-ray, although she reported improved general wellbeing. In July 2012, multiple pulmonary nodules were found to have completely regressed on chest X-ray (), which was confirmed on PET-CT scan in September 2012 (). However, a 5.5-cm hepatic mass still remained on abdomen ultrasound and PET-CT scan (). Considering her good performance status, we recommended repeated TACE, but she declined. Therefore, high-dose vitamin C administration was continued for more than a year. In July 2013, she finally decided to undergo TACE in addition to the high-dose vitamin C treatment. Subsequently, three rounds of TACE were performed. After the fourth round of TACE, the hepatic mass was found to have entirely regressed (), and both PIVKA II and AFP levels had returned to normal range. The serial changes in AFP and PIVKA II levels are shown in . The patient denied any use of other anti-cancer medications or alternative therapies except pain medication for her intermittent abdominal pain. She was quite tolerant of high-dose vitamin C during the entire treatment period, but thirst was an occasional complaint, which was easily remedied by water intake.


A 47-year-old man visited the emergency room because of decreased mentality. He was diagnosed with diabetes mellitus (DM), but had not received regular treatment. His blood pressure was 60/30 mm Hg and body temperature was 38.5℃. Laboratory data showed white blood cell of 13.150×109/L, with a differential count of 90.7% neutrophils and 8.5% lymphocytes, hemoglobin of 14.3 g/dL, platelet count of 507×109/L, glucose levels of 443 mg/dL, blood urea nitrogen of 52.5 mg/dL, serum creatinine concentrations of 1.9 mg/dL, aspartate aminotransferase of 69 IU/L, alanine transaminase of 38 IU/L, sodium of 125.7 mmol/L, potassium of 5.4 mmol/L, C-reactive protein (CRP) levels of 19.42 mg/dL, ketone bodies in the blood 3 positive and HbA1c of 18.20%. Arterial blood gas analysis revealed a pH of 7.032, pCO2 of 21.8 mm Hg, pO2 of 83.3 mm Hg, and HCO3 of 5.8 mmol/L. Chest X-ray and computed tomography (CT) scans showed multiple consolidation and ground glass opacities in both lung fields (). The patient was initially diagnosed with diabetic ketoacidosis as a result of severe pneumonia. An immediate hydration and insulin therapy were started, and dose-adjusted piperacillin and tazobactam sodium were also administered for pneumonia. The patient was admitted to the intensive care unit (ICU) for mechanical ventilator therapy. The ketoacidosis had improved with hydration and insulin therapy, but on the second day of hospitalization, diuretic-resistant pulmonary edema developed and continuous renal replacement therapy was started. Klebsiella pneumonia was identified in sputum cultures. After 8 days of treatment, the lung lesions had regressed (). The patient was finally moved to the general ward. However, on the 5th day in the general ward, he started complaining of vague abdominal pain. His pain had aggravated with severe tenderness. An erect abdominal X-ray showed gaseous distention of small bowel loops with suspicious stepladder sign, suggesting mechanical obstruction (). Subsequent abdominal CT suggested multiple perforation of the transverse colon with panperitonitis ().
An emergency laparotomy was performed. Necrotic intestines were observed from the distal ascending colon to the proximal transverse colon. Necrotic portions were resected and the Periodic Acid Schiff and Grocott's methenamine silver staining of the resected specimen showed septated fungal hyphae with acute angle branching, suggesting aspergillus species (). The patient was finally diagnosed with colonic IA. Intravenous liposomal amphotericin-B (3 mg/kg/day) was additionally administered for 35 days. He was discharged with oral voriconazole to treat his reported remaining, abdominal discomfort and mildly elevated CRP levels. Voriconazole was discontinued at a follow-up visit to the outpatient department 2 weeks after discharge when his abdominal discomfort had relieved and CRP levels normalized.


A male patient aged four years and 10 months was brought in with a complaint of short stature. It was indicated that although his development was normal until 12 months of age, it halted afterwards. It was found out that the patient was born at term in breech position through cesarean section with a normal Apgar score and birth weight of 3,750 g, hospitalized postnatally for five days due to birth in breech position and was not noted to be hypothermic and hypoglycemic, was diagnosed with LCP at four years of age following detailed examination due to complaints of pain in the hips, and consequently underwent surgery (). Family history indicated that the patient's parents were related to each other within the second degree of consanguinity. His first sibling was being followed up for Tangier disease, and his second sibling was being followed up for hyperlipidemia by the Department of Pediatric Metabolism. Physical examination findings showed that the patient was prepubertal, and that he had a height of 96 cm (standard deviation score [SDS], -3.2), body weight of 14.6 kg (SDS, -1.9), 2 cm shorter left leg compared to right leg, a surgical scar in the left inguinal region, and stretched penile length of 4.5 cm. Other systemic examination findings were normal. While the patient's chronological age was four years and 10 months, his bone age was consistent with a 2-year-old's (according to Greulich and Pyle).
Laboratory tests yielded the following results: white blood cell count, 11,300/mm3; hemoglobin, 12.1 g/dL; thrombocyte, 320,000/mm3; urea, 7 mg/dL; creatinine, 0.9 ml/dL; serum sodium, 148 mmol/L; potassium, 4.3 mmol/L; chloride, 102 mmol/L; calcium, 9.7 mg/dL; phosphorus, 4.4 mg/dL; normal liver function; fasting blood glucose, 92 mg/dL; total cholesterol, 130 mg/dL; high-density lipoprotein, 41 mg/dL; low density lipoprotein cholesterol, 69 mg/dL; triglyceride, 50 mg/dL; and urine density, 1,022 g/cm3.
Detailed laboratory analyses showed thyroid function consistent with central hypothyroidism (sT4, 0.49 ng/dL [0.8-1.9 ng/dL]; thyroid-stimulating hormone, 2.57 mU/L [0.5-5 mU/L]). The patient was also determined to have hypocortisolemia (basal serum cortisol level of 2.75 g/dL, stimulated serum cortisol level of 5.71 g/dL [with low-dose adrenocorticotropic hormone]) and GHD (peak GH values in glucagon and levodopa tests while the patient was euthyroid were 0.257 and 0.46 ng/mL, respectively). Serum IGF-1 level was 15 ng/mL (SDS, -3) and Insulin-like growth factor binding protein-3 (IGFBP3) level was 900 ng/mL (SDS, -2.6). An etiology-oriented hypophyseal magnetic resonance imaging was performed due to high prolactin levels (37.4 ng/mL [normal range, 0-25 ng/mL]), which revealed in the sellar cavity center that the adenohypophyseal gland height was 3 mm, neurohypophyseal localization was abnormal, and that there was no signal intensity. In addition, T1A hyperintense nodular appearance with dimensions of 3 mm×2.5 mm showing diffused homogeneous contrast retention following injection of contrast agent was observed at the median eminence level (ectopic neurohypophysis). The pituitary stalk was not identified as a separate structure (). Considering the patient's medical history, it was deduced and evaluated as a pituitary stalk interruption syndrome due to difficult birth. The patient was sequentially started on hydrocortisone, L-thyroxin, and GH therapy.


A 57-year-old male, who was known to be suffering from psoriasis vulgaris for the past 10 years, presented to us with features of unstable psoriasis for a duration of two weeks. He had been previously treated for multiple exacerbations with methotrexate (cumulative dose - 2285 mg), cyclosporine, and topical steroids, on separate instances. There was no history of any adverse effects in the past. The patient had been on maintenance treatment with Tab. methotrexate 5 mg/week prior to this exacerbation. He was a known diabetic for the past 10 years, on regular treatment with tablet metformin 500 mg twice a day. His baseline complete blood count showed leukocytosis (leucocyte count - 18,300 cells/mm3; platelet count- 5 × 105/mm3) and the liver function tests were within normal limits.
In view of the suspicion of unstable psoriasis, Inj. Methotrexate 10 mg IM was given on admission. After 24 hours, he developed multiple pustules over the nape of the neck and multiple oral erosions, resulting in severe odynophagia, with the existing lesions becoming more erythematous []. The hemogram showed a fall in the leukocyte counts (7,700 cells/mm3). Methotrexate toxicity was diagnosed and the patient was started on intravenous folinic acid. He showed signs of improvement in three days. Improvement in his blood count was also noted, as they returned to normal in four days (leucocyte count - 9,700 cells/mm3; platelet count - 4.11 × 105/mm3). The causality assessment, done using the Naranjo algorithm, revealed a probable adverse drug reaction.
Following this, the patient was started on cyclosporine 100 mg, twice a day, to control the unstable psoriasis. One week after administration of cyclosporine, it was noticed that the platelet count increased three times from the baseline values (9.0 × 105/mm3) when tested, on multiple occasions. Cyclosporine was stopped due to suspicion of drug-induced thrombocytosis. The platelet count dropped to a high normal value (5 × 105/mm3) four days after withdrawing the drug, thus confirming our suspicion. The Naranjo scale scoring was 7, which again implied a probable adverse drug reaction. The patient was then started on acitretin 50 mg once a day and was discharged after resolution of the lesions []. The patient is currently in remission, while on acitretin.


A 40-year-old woman with undifferentiated connective tissue disease had been taking hydroxychloroquine for 7.7 years. She was originally on 400 mg/day but more recently was taking 300 mg/day. Her height was 162.6 cm, her ideal body weight was 63.5 kg, her actual body weight was 65.8 kg, and her cumulative dose was 1,124 g. Her adjusted daily dose was 6.29 mg/kg/d based on her ideal body weight, which is in a range recognized as nontoxic. She had no renal or liver disease and no preexisting maculopathy. shows four consecutive 10–2 VFs performed with a red target. The first VF (January 25, 2007) had a single scotoma point on the defect depth display (circled in red) that resolved on the second field (February 12, 2008), which in turn had four new scotoma points (circled in green). Three of the four green-circled scotoma points occurred in the low-risk zone outside the 2°–8° annulus encircling the fixation point. In the third field (February 17, 2009), all four scotoma points circled in green had resolved, and remained so in the fourth field (March 22, 2010). As the patient had a low pretest probability of retinopathy (nontoxic daily dosing, no renal disease, and no liver disease), no intensified testing was done. shows that in 2014, she had normal mfERG ancillary testing, corroborating the lack of evidence of retinopathy seen in all of the VFs.


A 69-year-old woman had been taking hydroxychloroquine for rheumatoid arthritis for 17 years. She had been taking 400 mg/day the entire time. She was 160.0 cm, had an ideal body weight of 61.2 kg, but an actual body weight of 64.9 kg. Therefore, her adjusted daily dose was in a toxic range of 6.52 mg/kg/d for the duration of therapy. Her cumulative dose was 2,450 g. She had no renal or liver disease, nor any preexisting maculopathy. shows three consecutive 10–2 VFs with a red target. The first field on January 28, 2009 shows an annular ring scotoma on the gray scale display. The defect depth display shows a single scotoma point (green circle). The red-circled point was not scotomatous in this display. The second VF on February 1, 2010 shows that the green-circled scotoma point persisted and the adjacent red-circled point had now become scotomatous (ie, the scotoma was reproducible and has enlarged). By the third VF of March 28, 2011, the red-circled scotoma point had deepened by 4 dB, the scotoma had enlarged to include the adjacent blue-circled scotoma point, and a separate scotoma (purple-circled) in the high-risk zone from 2° to 8° from fixation had developed. At this point, the patient’s ophthalmologist recognized retinopathy and her hydroxychloroquine was stopped. Scotoma points associated with hydroxychloroquine retinopathy do not resolve, unlike the situation with patients taking hydroxychloroquine who do not have retinopathy. Moreover, the scotomas tend to broaden over time. shows that on October 25, 2012, there was marked diminution of the mfERG amplitudes in rings R1–R2.


A 30-year-old female had a 2 years history of left frontal headache unresponsive to drug therapy with common analgesics. The intensity of this symptom increased over time. Her neurological examination was normal. The magnetic resonance imaging (MRI) showed a dural-based lesion in the left frontal region; the lesion size was 1.5 cm × 3.5 cm. It was hyperintense on T2-weighted images and isointense on T1-weighted images showing intense contrast enhancement. This small mass appeared to be adherent to the dura mater with a dural tail sign []. The appearance suggested a convexity meningioma.
Initially, the patient refused surgery.
After 1-year, brain control MRI showed no changes of the lesion.
Finally, the patient decided to undergo surgery because the episodes of headaches had become more and more frequent and intense.
A left frontal craniotomy was performed. After opening the dura, an encapsulated, red-colored tumor was exposed. The base of the implant was coagulated and the lesion was removed en-bloc without significant intraoperative bleeding.
The histopathological diagnosis deposed for a cavernous hemangioma of the dura mater [].
The postoperative MRI showed no residual hemangioma [].
At 1-year follow-up, the patient was asymptomatic without any neurologic deficit.


A 30-year-old female patient had the complaint of receding gum in lower front tooth region. On intra-oral examination, an isolated Miller class I gingival recession at labial surface of the lower right canine (no. 43) was present due to trauma from occlusion. The recession depth was 3 mm recorded using University of North Carolina-15 periodontal probe-measured from CEJ to the gingival margin []. Gingival height was 2.0 mm when measured from the apical portion of the gingival margin to mucogingival line and probing depth was 2.0 mm as when measured from the bottom of the sulcus to the most coronal end of the gingival margin.
The patient underwent basic periodontal treatment of phase I therapy including scaling, root planing and instructions for proper oral hygiene measures. Occlusal therapy including occlusal adjustments or coronoplasty was done for treatment of trauma from occlusion. A coronally directed “roll” technique was advised for tooth with gingival recession to minimize toothbrushing trauma of the gingival margin. Surgical treatment of gingival recession was not scheduled until the patient could able to maintain full mouth bleeding score[] of ≤20% and full mouth plaque score[] of ≤20% along with absence of plaque that is, “plaque-free” (area where plaque could not be removed with a manual probe) and bleeding on probing at the surgical tooth site.
Patient was instructed to do presurgical rinse by 0.2% chlorhexidine solution. The facial skin around the mouth was cleaned with spirit and scrubbed by 7.5% povidone iodine solution. Intra-oral surgical site was painted with 5% povidone iodine solution.[]
After proper part preparation, 2% lignocaine hydrochloride with 1:80,000 adrenaline was administered to anesthetize lingual, labial and inferior alveolar nerves. After local anesthesia, marginal incision was given all along the soft tissue margin of the recession defect. Two lateral vertical incisions along proximal side of recession defect were made, and a mucoperiosteal flap was reflected []. The exposed root surface was cleaned thoroughly by scaling and root planing to remove any root surface deposits. An attempt was made to flatten the root in the area of the root prominence. Root conditioning was done with 24% ethylenediaminetetra-acetic acid for 3 min [] and washed with a normal saline solution for 60 s []. A basal incision was given at the baseline to incise the periosteum, and then it was separated from the submucous connective tissue up to the borderline of the attached gingiva. The crestly pedicle periosteum was everted [] and transposed coronally where it was sutured with 5–0 absorbable suture []. After that, a coronal transposition of mucoperiosteal flap was done, and it was also sutured with 5–0 absorbable suture [].
Antibiotic (amoxicillin 500 mg, 1 tablet every 8 h, for 7 days) and analgesic (nimesulide 100 mg, 1 tablet every 12 h, for 3 days) were prescribed. Patient was instructed to be extremely cautious during mastication at meals and not to brush the teeth in the treated area for 2 weeks but to use 0.2% chlorhexidine mouthwash twice daily for 1 min. After this period, patient was advised to mechanical cleaning of treated tooth region using an extra soft toothbrush by coronally directed “roll” technique, together with 0.2% chlorhexidine mouthwash twice daily, 1 min for more 4 weeks. After this period, routine oral hygiene procedures could be reintroduced.
Clinical follow-up was performed once a week in the 1st postoperative month, every 2 weeks in the 2nd postoperative month and once a month after that up to 6 months. At each visit, recall programs including professional tooth cleaning and reinforcement of daily oral hygiene measures were done.
Healing was uneventful. Patient was satisfied with the treatment outcome. Complete epithelialization of the everted periosteum was seen 2 weeks after surgery. As the postoperative time increased, the progressive adaptation and morphologic resemblance were observed. After 6 months, 3.0 mm that is, 100% root coverage, 5.0 mm of gingival height and 1.0 mm of probing depth were obtained [].


A 10-year-old male child, a known case of congenital atrophy of left kidney presented with complaints of mild pain in the right lumbar area of 3 days. He did not have any urinary symptoms, vomiting, jaundice, bladder, or bowel disturbance. The patient was afebrile and hemodynamically stable. General physical and systemic examinations were unremarkable except for flank tenderness on the right side. Laboratory investigations revealed hemoglobin: 11.1 g/dl and a total leukocyte count: 16.51 × 103/µl. Renal function tests were normal with blood urea: 33 mg/dl and serum creatinine: 0.7 mg/dl. Ultrasound kidney, ureter, bladder regions on the right side showed enlarged kidney (11.7 × 5.6) with hydronephrosis of the mid and lower poles with hydroureter. A cystic lesion was seen in mid pole with sedimenting hyperechoic contents. An ill-defined hypoechoic area was seen in the upper pole measuring 4.2 cm × 3.1 cm, causing capsular bulge with upper pole hypertrophy, with a possible diagnosis of lobar nephronia. On the left side, it showed a small kidney (5.3 cm × 2.3 cm) with dilated ureter in its entire length. Computed tomography urography revealed enlarged right kidney (11.2 cm × 6 cm) with the irregular contour. There was decreased nephrographic density in the anterior-mid and lower parenchyma probably due to focal nephronia. Calyceal cut off and hydronephrosis were noted in the anterior-mid and lower pole calyces on the right side with no excretion of contrast even on delayed scans. Two of the cut off calyces in the anterior mid pole and one in the lower pole showed a high density contents and mild wall thickening on delayed scans, which was likely due to exudative fluid or pus. On the left side, it showed atrophic kidney []. No organism was isolated from urine culture. Based on these investigations, the patient was planned for ultrasound guided aspiration of the right renal abscess under local anesthesia. He underwent the procedure well, and the aspirate was sent to the microbiology lab for culture and sensitivity. Postprocedure, he was started empirically on cefoperazone-sulbactam 1g intravenous (IV) 3 times a day. Aspirate culture grew nonlactose fermenting Gram-negative Bacilli, which was identified as S. typhi by VITEK 2 compact. Serotyping was done to confirm the isolate. This isolate was found to be sensitive to ceftriaxone (MIC ≤ 1 µg/ml), cefoperazone-sulbactam (MIC ≤ 8 µg/ml), trimethoprim/sulfamethoxazole (MIC ≤ 20 µg/ml) but resistant to ciprofloxacin (MIC ≥ 1 µg/ml). As the patient was afebrile and clinically there was no suspicion of enteric fever at presentation, his blood culture was not sent. However, we managed to do the Widal test on his serum sample, which was stored in the lab following renal function tests. Widal came out to be positive for S. typhi (O and H antigens in the titer of 1:320). Treatment was continued with IV cefoperazone-sulbactam till the 4th day of his hospitalization as the isolate was sensitive to third generation cephalosporins. Pain and flank tenderness subsided, and the patient was discharged with an advice to take cefixime 200 mg twice daily for 7 days. The patient was asymptomatic on subsequent follow-up visits.


A 59-year-old female was admitted to the hospital with sudden onset of acute chest pain. Coronary angiography showed focal significant stenosis in the distal segment of the right coronary artery (RCA), and a stent was then successfully placed at the site of narrowing. However, a newly developed vascular dissection was found in the proximal RCA at the end of the procedure, extending to the aortic arch and thoraco-abdominal aorta. Although new stents were implanted in the proximal and middle RCA to restore the true lumen, complete thrombotic occlusion developed in the entire RCA. The patient then underwent immediate aortic hemi-arch replacement and a bypass graft orgusing saphenous vein after the successful resuscitation of ventricular fibrillation. Nevertheless, she progressed to refractory cardiogenic shock and died 7 days after surgery.
Forensic examination was decided under the suspicion of acute myocardial infarction caused by thrombotic occlusion of the RCA associated with iatrogenic dissection during coronary intervention, and PMCTA was performed before standard autopsy dissection. Following the standardized protocol, cannulation of the right femoral vessels was performed using two cannulas with a diameter of 16-Fr for the artery and 18-Fr for the vein. PMCTA was then carried out on a 128-slice multidetector CT system (Somatom AS plus, Siemens Healthcare, Erlangen, Germany). Scanning was conducted from the vertex to the upper thigh with tube voltage of 120 kVp and tube current of 210 mAs using a 0.625 mm collimation. A pressure-controlled perfusion device (Virtangio, Fumedica AG, Maquet, Muri, Switzerland) was used to inject 1200 mL of a mixture of esters of polyiodinated fatty acid (Angiofil, Fumedica AG, Muri, Switzerland) and paraffin oil at the injection rate of 800 mL/min ().
Post-mortem CT angiography at the arterial phase (a retrograde injection of the contrast mixture into the arterial system through the femoral artery) demonstrated an aortic dissection with a contrast-filled true lumen and thrombosed false lumen (). Furthermore, multiplanar reconstructed (MPR) images of the coronary tree showed a normal contrast-filled lumen of the left anterior descending and circumflex artery with their smaller branches. However, MPR images of the RCA clearly demonstrated complete thrombosis of os to distal segment, and also depicted complete occlusion of both stents implanted in proximal and distal segment, respectively (). In addition, the short-axis view of the heart showed diffuse contrast enhancement of the left ventricular (LV) myocardium; interestingly, it also depicted a transmural contrast defect localized in the inferior and inferoseptal myocardium of the middle and basal LV that corresponds to the RCA territory ().
Gross pathological examination of the coronary arteries opened longitudinally revealed complete thrombotic occlusion of stents implanted in the false lumen of the proximal and middle segments, causing collapse of the true lumen of the involved segments and thrombosis of the remaining true lumen of the distal segment. On visual inspection of the heart, areas of damage were revealed as subtle mottling with a yellow-tan center. Using light microscopy of sections stained by routine hematoxylin and esoin stains, coagulation necrosis with the loss of nuclei and striations was identified at infarcted area ().


A previously well 43-year-old lady presented with bloody discharge from her right nipple. A bilateral mammogram and ultrasound demonstrated a solitary hypodense lesion within the inferior portion of her right breast. Fine-needle aspiration was suspicious for carcinoma. She underwent wide local excision and sentinel lymph node biopsy which revealed a 3.5-mm grade 1 invasive ductal carcinoma with associated intermediate-grade ductal carcinoma in situ. The tumour was hormone receptor (ER/PR) positive and HER2 negative. The closest margin was 1.7 mm posteriorly. Sentinel lymph node biopsy was negative, so she was staged pT1aN0M0.
The patient had no other significant medical co-morbidities. She was physically very fit and slim in keeping with her occupation as a dancer. The oral contraceptive pill which she was taking prior to diagnosis was ceased. Her mother had previously been treated for ductal carcinoma in situ at age 45 years.
Adjuvant whole-breast RT was recommended to reduce the risk of local recurrence. No hormone therapy was recommended due to the small primary lesion size, low-grade histology and node negativity. She received 50 Gy in 25 daily fractions over 5 weeks using standard tangential 6 MV photon fields to the whole breast, followed by an electron boost of 10 Gy in 5 fractions to the surgical cavity. Treatment was in accordance with International Commission on Radiation Units (ICRU) requirements with acceptable planning target volume coverage, homogeneity and dose to organs at risk. The maximum dose to her whole breast was <105%percnt; of the prescription dose. Daily monitor units were retrospectively reviewed to ensure correct treatment delivery.
A moderate (grade 2) radiation dermatitis developed during treatment, which resolved soon after completion of RT. In addition, a small (1.5-cm) area of cellulitis developed over the inferolateral portion of her right breast 1 month after treatment, which was treated with flucloxacillin and resolved completely.
Eleven months after RT, the patient developed severe right-sided lateral chest wall pain which was pleuritic in nature. Chest radiography was normal. A bone scan showed features consistent with costochondritis, but no evidence of metastatic disease. A computerised axial tomography (CT) pulmonary angiogram showed no evidence of pulmonary embolus, rib fracture or metastatic disease. Four months later, the patient developed intractable pain in the right chest wall upon minimal exertion. Repeat CT chest imaging demonstrated a healing fracture involving the right sixth rib (fig. ) and features consistent with a fracture of the right anterior second rib without evidence of bony destruction. Magnetic resonance imaging (MRI) was performed to ascertain aetiology, which confirmed fractures of the right fifth and sixth ribs. There was fatty marrow replacement involving the right second to sixth ribs consistent with radiation changes (fig. ). Repeat bone scan revealed mildly increased uptake in the right second, third, fifth and sixth ribs.
Investigations to rule out contributing factors including serum calcium, renal function, haematology, C-reactive protein, vitamin D, parathyroid hormone, thyroid function, bone mineral density and myeloma screen were within normal limits. Radiation ORN was determined as the most likely cause of the rib fractures. The patient commenced treatment with pentoxifylline, vitamin E and hyperbaric oxygen therapy. She declined clodronate treatment in view of the small risk of mandibular necrosis. There was no demonstrable improvement in symptomatology or radiological re-evaluation after 6 months of therapy.
Two years after her presentation with chest wall pain, a progress CT scan showed a non-united fracture involving the anterolateral aspect of the right fifth rib with localised sclerosis and a partially united fracture of the anterior aspect of the sixth rib at the costochondral junction. The previously demonstrated changes involving the right anterior second and third ribs had resolved. A repeat MRI scan performed 2.5 years after initial chest wall pain confirmed two areas of signal abnormality, which corresponded to the persistent non-united fractures on the CT scan 6 months prior, with a degree of sclerosis on T1 imaging and hyperintensity on T2 imaging (fig. ). There was no generalised bony abnormality or MRI features of widespread ORN.
Over the 2.5-year follow-up period, the patient's anterior second rib pain settled, and her right lateral rib pain improved without the need for narcotics or anaesthetic nerve blocks. Physical examination revealed progressive right breast shrinkage in keeping with RT-induced fibrosis. The patient's quality of life was negatively impacted, resulting in the modification of her active lifestyle with avoidance of all upper body exercises. As a result, she stopped professional dancing and became a dance teacher.


A 53-year-old male was referred to our center as acute on chronic liver failure with complaints of jaundice, abdominal distension, generalized weakness, and dehydration.
He was a known case of Hepatitis C related chronic liver disease since 2008. He probably acquired this infection due to the blood transfusions given during knee surgery in 1996. He also received multiple transfusions owing to variceal bleed 2-3 times a year in the past 4 years.
At the time of admission, the patient was pale, deeply icteric, and hypotensive (BP 90/60 mm Hg); he had bilateral pedal edema and ascites. Laboratory investigations were as follows: Hemoglobin 6.9 g/dl, hematocrit 24%, platelet count 81,000 cells/cu mm, total serum bilirubin 28.8 mg/dl, serum alanine transaminase 160 U/L, serum aspartate transaminase 53 U/L, serum alkaline phosphatase 131 U/L. Patient was on supportive treatment, and a request was received for two units of packed red cells.
The blood sample was screened for irregular antibodies as per our standard protocol using a commercial ID-Diacell I-II-III antibody screening Panel (Diamed, Switzerland) and ID-cards “LISS/Coombs” (Diamed, Switzerland).
The test was positive with Panel II (4+) and Panel III (4+) with negative autocontrol. Antibody was confirmed as anti-c Rh antibody using ID DiaPanel, antibody identification panel (Diamed, Switzerland). The titration was performed by conventional tube method using double dilution method and was found to be 512 in anti-human globulin phase.
The direct antiglobulin test (DAT) was performed using “LISS/Coombs” (Diamed, Switzerland) and DC-Screening I (Diamed, Switzerland) ID-Cards. Polyspecific DAT was 4+ positive, and Monospecific DAT showed the presence of IgG antibodies only (4+). Anti-c was identified in the elute obtained from patient's red blood cells (RBCs) by Diacidel acid elution Reagents (Diamed, Switzerland). The Rh phenotype and most probable genotype of the patient was R1R1 (DCe/DCe).
The positive antiglobulin tests (DAT and indirect antiglobulin test) and the presence of “anti-c” Rh antibody in eluate raised the suspicion of HTR. On reevaluation, the history revealed that on January 22, 2013, the patient received two units of whole blood transfusion in a local hospital on out-patient basis during which he felt uneasy. He further developed high-grade fever, chills, and severe abdominal pain 3 h after the transfusion. By the next morning, he noticed yellow discoloration of the skin and was passing red colored urine (Investigations not available). There was no rise in hemoglobin level after transfusion (Baseline Hb 6.7 g/dl). The serum bilirubin rose from baseline 1 mg/dl to 9 mg/dl by next day. The patient condition deteriorated during the next 7 days. Patient was referred to our hospital at this point of time on February, 2013.
A diagnosis of AHTR (i.e., within 24 h of transfusion) precipitating acute liver failure in a patient with hepatitis C virus-related chronic liver disease was made. Crossmatch compatible “c” antigen negative packed RBCs, and plasma components were issued and transfused.
Despite supportive care, the general condition of the patient deteriorated following an episode of variceal bleed leading to hepatorenal syndrome and patient succumbed to his illness on 18.2.2013.


A 16-year-old uncircumcised boy was admitted with chief complaints of passing a single stream of urine from the undersurface of the penis, forcing him to micturate in squatting position. There were no complaints of a narrow stream of urine or pain while passing urine. He was the only child born out of non-consanguinous marriage. The antenatal period was uneventful.
On examination, a wide congenital urethral fistula was noted at the scrotal level []. The urethral plate measured 4.5 cm in length and 2.6 cm in width. The urethra distal to the fistula was normal with normal prepuce and glans without any chordee []. The external urethral meatus was situated normally at the tip. The stretched penile length and diameter were normal for his age. No previous penile surgery had been done. Urinalysis was found normal.
The patient was posted under general anesthesia. The anterior urethrocutaneous fistula was identified at the level of scrotum []. It was calibrated with 12 number foley's catheter passing down to the urinary bladder without any obstruction. The distal urethra was calibrated with the gradually increasing size of urethral dilator []. Meatotomy was done at the lower end of the distal urethra. The exposed urethral plate was marked at its periphery with ink []. The fistula was circumscribed and repaired in 2 layers over 12 number foley's catheter. Dartos fascia and skin were closed over it []. The post-operative course of the patient was uneventful. The catheter was removed on 14th post-operative day []. No recurrent fistula occurred. After 4 months of follow-up, patient is voiding normally without any recurrence.


The patient is a 46-year-old woman who presented with abdominal pain and new onset of ascites. There was no known prior diagnosis of MCC. Computerized tomography of the abdomen showed a cirrhotic liver with diffuse heterogeneous enhancing lesion at the porta hepatis measuring 4 cm × 2.7 cm and a 3.6 cm × 6.7 cm arterially enhancing lesion in hepatic segment IVa. The portal, superior mesenteric, and splenic veins were patent and normal in caliber. No gastroesophageal varices were present. A moderate amount of ascites was present. There were enlarged periportal, gastrohepatic, peripancreatic, retroperitoneal, and mesenteric lymph nodes seen. The largest node in the periportal region measured 2.8 cm x 2.7 cm. The radiologic impression was cirrhosis with multifocal arterially enhancing hepatic lesions and heterogeneous appearance of the liver concerning for diffusely infiltrating hepatocellular carcinoma with upper abdominal and retroperitoneal lymphadenopathy likely representing metastatic disease. The tumor markers were within normal limits (AFP 9.2 ng/ml and CEA 1.3 ng/ml). The patient underwent paracentesis.
The thin prep smears stained with Papanicolaou showed malignant cells arranged singly [], in clusters [] and occasional single-file pattern []. The individual cells showed round to oval nuclei, irregular nuclear borders, stippled chromatin pattern, inconspicuous nucleoli, and scant cytoplasm []. Mitotic figures were identified []. Occasional nuclear molding was present.
The cell block preparation stained with hematoxylin and eosin showed loosely dispersed malignant cells with high nucleus to cytoplasmic ratio, fine to clumped chromatin pattern, occasional small inconspicuous nucleoli, irregular nuclear borders, and scant eosinophilic cytoplasm []. Occasional mitotic figures were identified. Immunohistochemical stains showed the tumor cells were immunoreactive for AE1/AE3, CK20 [], chromogranin, synaptophysin, and Merkel cell polyomavirus (MCPyV) monoclonal antibody (CM2B4) []. The tumor cells were negative for CK7, CEA, B72.3, CD45, CD138, CD56, TTF-1, BerEp4, S-100, Hep Par1, CK5/6, and calretinin.


A 44-year-old woman presented with an alleged intra-abdominal mass on routine health screening. Her past medical history consisted of a Caesarean section, 10 years previously. When she visited our hospital, she had no abnormal physical findings, except for the palpable mass. On a blood tumor markers test, the carcinoembryonic antigen (CEA, reference value ≤5.0 ng/ml) and carbohydrate antigen 19-9 (CA19-9, reference values ≤37 U/ml) were 0.58 ng/ml and 1,690 U/ml, respectively.
The patient underwent an abdomen computerized tomography (CT) scan initially, and 2-18F-fluoro-2-deoxy-d-glucose (FDG) positron emission tomography (PET)-CT were requested to investigate the pancreatic abnormality. These studies demonstrated that the abdomen CT scan showed a large cystic mass with internal septa at the tail of the pancreas, and the FDG PET-CT image showed no abnormal FDG tracer uptake at the pancreatic cystic mass (, ). The patient was suspected to have a pancreatic malignancy.
She underwent a laparotomy of the distal pancreatectomy and a splenectomy, with the intention of both diagnostic and therapeutic purposes. Operative findings revealed that a lesion with a diameter of 6.5 cm was located at the pancreatic tail, and its boundary was clear with adhesion to the spleen. In the fluid analysis of the pancreas cystic mass, the CEA and CA19-9 were 618 ng/ml and 3.9 U/ml, respectively. The resected pancreas specimen showed a 6.5 cm-sized cyst of the pancreas tail. The cyst was well circumscribed and multilocular ().
During the postoperative recovery period, the follow-up CA19-9 level was down to 5.8 U/ml. The final pathology report of the resected pancreas specimen noted that the cyst was multilocular, and the cyst lining showed stratified squamous epithelium covering lymphoid tissue containing lymphoid follicles, which was consistent with a lymphoepithelial cyst (, ). The patient recovered uneventfully from surgery and has been doing well for the past three months.


A 72-year-old female presented to the Emergency Department with symptomatic anaemia. Past medical history includes atrial fibrillation and a stroke 6 months prior with no residual deficit. She was on apixaban (factor 10 inhibitor). She had noticed 3 months of progressive shortness of breath and tiredness. Her haemoglobin was 66 g/L in emergency. Iron studies showed Fe 3.2 mmol/L, transferrin 5.1 mg/dL, ferritin 159 pmol/L and iron saturation 27%, indicating iron-deficiency anaemia. She was admitted, transfused 2 units of red cells and had a diagnostic colonoscopy on Day 4 of her admission.
Shown in Fig. is a colonoscopy view of the caecum showing the submucosal mass protruding into the lumen with a central ulceration and some slight ooze bleeding on biopsy.
On colonoscopy, she was found to have a caecal submucosal mass that bled when biopsy was attempted. It was 2–3 cm and appeared likely to be a gastrointestinal stromal tumor (GIST). Otherwise, the colonoscopy was normal.
CT abdomen was performed showing a 3 cm caecal mass likely submucosal; the histopathology of the biopsy taken on colonoscopy showed only normal colonic mucosa.
Shown in Fig. is a CT scan showing the caecal mass near to the ileocaecal valve.
Over the next few days, her haemoglobin slowly decreased and she had ongoing malaena requiring one more unit of transfusion. Surgical opinion was obtained for management of a likely bleeding GIST in her caecum. The decision was made for surgical resection rather than repeat colonoscopy or further imaging given the ongoing bleeding and likely diagnosis. She underwent a laparoscopic converted to open limited right hemicolectomy as the mass was close to the ileocaecal valve, so cecectomy was unable to be performed. Her recovery was complicated by some previously unknown heart failure and fluid overload, but this was settled on low-dose frusemide. She was discharged home on Day 7 postoperatively. Formal histopathology showed an appendiceal faecolith protruding back into the caecum underneath the submucosa resembling a mass
Shown in Fig. is a macroscopic view of the resected specimen having been opened coronally, showing the faecolith in the obliterated appendiceal lumen and protruding back into the caecum.


A 31-year-old patient of type GSD-1a (von Gierke's disease) with known multiple HCAs presented for MR imaging. The contrast-enhanced multiphasic liver magnetic resonance imaging (MRI) was performed with gadopentetate dimeglumine in an outside facility in May 2014 and demonstrated multiple HCAs ranging in size from less than 1 cm up to 5 cm, some of which were hemorrhagic. The lesions showed variable heterogeneity and arterial enhancement with subtle washout []. Given the higher frequency of β-catenin–activated HCA associated with GSD-1a, regular surveillance imaging was recommended.
Follow-up MRI with gadoxetate disodium (gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid/Gd-EOB-DTPA) in our institution was performed after 6 months in Nov 2014. It showed a mild interval increase in the size of one lesion from 4.0 × 3.9 cm to 4.9 × 4.2 cm. The lesion demonstrated predominantly isointense to slightly increased intensity compared with the rest of the liver parenchyma on the T2-weighted (T2W) image, with an eccentric, ill-defined, T2-hyperintense possible scar, and did not reveal intralesional lipid. There was no T1 hyperintensity to suggest interval hemorrhage []. It was arterial enhanced with washout, with an enhancing rim on the venous phase, and did not show uptake of hepatocyte-specific contrast on the delayed phase images []. The size of the remaining adenomas either marginally decreased or was stable []. In view of this isolated increase in size, the lesion was presumed to be suspicious.
Accordingly, a short-interval follow-up scan or a biopsy was recommended to rule out malignant transformation. An ultrasound (US)-guided biopsy was performed as per patient preference. The biopsy revealed absent normal portal tracts in the tissue, increased unpaired arteries, and moderate steatosis, with many ballooned cells, some containing Mallory–Denk bodies. A reticulin stain showed focal loss of the reticulin framework []. The hepatocytes did not show increased Nuclear: Cytoplasmic (N: C) ratio, and there were no overtly prominent nucleoli or mitotic figures. Although there was no pseudoglandular formation and β-catenin was negative for nuclear staining, focal Glypican-3 staining was present. Taken together, while the differential diagnosis included hepatocellular adenoma and well-differentiated carcinoma, the focal Glypican -3 stain and focal loss of reticulin stain led to the final diagnosis of a well-differentiated hepatocellular carcinoma (HCC). The patient continues to be asymptomatic and has been put on the hepatic transplant list, with close monitoring of the HCC on imaging pending the transplantation.


A 41-year-old man, worked as a diamond polisher for more than 15 years, presented to our department with a 3-year history of dry cough and progressive breathlessness on exertion. Examination revealed grade 3 clubbing, exercise desaturation and bilateral bibasilar fine end inspiratory crackles. Routine hematological and biochemical values were normal including ACE level as 33U/l. An arterial blood gas analysis was normal but showed an increased alveolar-arterial gradient. Chest radiograph [] showed bilateral reticulonodular opacities. Spirometry was suggestive of severe restrictive abnormality with forced vital capacity (FVC) of 1.38 liters (31%), forced expiratory volume in first second (FEV1) of 1.24 liters (35%), FEV1/FVC ratio of 89, and diffusion of lung for carbon monoxide (DLCO) of 10.08 ml/min/mmHg (35% predicted). High-resolution computed tomography (HRCT) of thorax revealed ill-defined patchy areas of confluent centrilobular nodules with a central and peripheral distribution pattern and fissural nodules with mildly enlarged mediastinal lymph nodes []. Whole body positron emission tomography – computed tomography (WB PET-CT) was done to see extent of underlying inflammation. It showed diffuse low-grade fluorodeoxy glucose (FDG) uptake in bilateral lungs with sparing of peripheral right middle lobe laterally, and low to moderate FDG uptake in right paratracheal, subcarinal, right tracheobronchial, bilateral hilar, bilateral inguinal lymph nodes and aortopulmonary window []. An analysis of the powder used for polishing diamonds at his workplace was performed. It revealed a high quantity of Cobalt -24.65 parts per million. The patient refused lung biopsy. However, in view of significant occupational exposure to cobalt, HRCT findings and normal ACE levels, diagnosis of hard metal disease due to cobalt exposure was made. He was advised to change his job. Steroids i.e., tablet prednisolone 40 mg was given as diagnosis could not be proven with biopsy and FDG-PET scan showed increased uptake suggesting underlying inflammation. One year later his lung functions were stable with FVC-1.38, FEV1-1.29 and FEV1/FVC ratio-93. Follow-up HRCT after 1 year showed similar findings as baseline.


A 30-year-old woman with good general health was referred to the Oral and Maxillofacial Surgery Department of the University General Hospital of the University of Cuiaba for surgical correction of transverse maxillary atresia. On examination, it was observed that in addition to the transverse maxillary atresia, an osseointegrated implant was placed to replace superior left canine (-). Prior to the SARPE, pre-operative panoramic radiography was evaluated () and the patient was explained over the possibility of having the implant malpositioned.
The SARPE was performed under local anesthesia and oral sedation in an outpatient setting. The Hyrax distractor activation was started on the 3rd post-operative day, planned to go as two activations daily (¼ turn each time) until 20% of overcorrection to the inferior dental arch was achieved. During activations, it was observed that the left hemi-maxilla had greater expansion, causing an asymmetry in the frontal view of the maxilla (-), but the decision was to continue the activations as planned. After approximately 18 months of post-surgical orthodontic treatment, an acceptable dental occlusion was noticed except for the malpositioned implant (-).
After clinical and dental casts evaluation, surgical relocation of the malpositioned implant was simulated on a semi-adjustable articulator through a segmental osteotomy. As the prosthesis over the implant was satisfactory, it was planned to use the bracket as a reference to the new position. After a trapezoidal vestibular mucoperisoteal flap (), a 699 drill and a chisel were used to perform two vertical alveolar osteotomies adjacent to the implant, including buccal and palatal bony walls, connected to a buccal horizontal apical osteotomy (). Subsequently, a “greenstick” fracture was done, followed by repositioning of the implant. The bracket on the implant was then connected to the orthodontic arch and stabilized with chemically cured resin surrounding the implant and two adjacent teeth on each side (). The bone gap formed by the movement of the block was filled with halogen bone graft (Geistlich Bio-Oss®, Wolhusen, Switzerland) and the crown of the implant was adjusted to be out of occlusion (). The acrylic resin was removed with 45 days. Radiographic control included the immediate post-operative period, 30, 90 days and 16 months. The patient is currently on a 16 months post-operative follow-up, the bone-implant block shows no clinical or radiographic changes, obtaining the clinical success and patient satisfaction for the treatment performed (-).


The patient under consideration was a 12-year-old boy who had been under the supervision of different ophthalmologists for 4 years. During this period, the patient had undergone various ophthalmologic and neurological examines. However, no noticeable organic reason was put forth for the disorder. Finally, with the possibility of a psychological reason, the patient was referred to a clinical psychologist. After the different psychological evaluations, he was considered to be a patient afflicted with functional neurological symptom disorder-conversion disorder by a clinical psychologist and a psychiatrist.
The examinee was the first child of the family. The parents of the child had divorced when he was three and half years old. The father had married again and during this period, the child had lived with a father. It seems that the problems of child vision problems had intensified after the divorce and, in particular, when the second wife of his father gave birth to a new child.
The patient underwent CAT treatment plan. Single-case design (A/B) was used to assess the effects of an intervention. Before and after intervention set of tools were done. The intervention includes twelve sessions of CAT and four sessions of follow-up. And during the treatment, intensity and weakness of conversion symptoms were observed. Also, the long-term effects of interventions during the treatment period were evaluated.
Zung's self-rating anxiety scale (SAS), beck depression inventory, second edition (BDI-II) and visual acuity test (VA) have been used for evaluation before and after the intervention. BDI-II used for measuring symptoms and intensity of depression which includes 21 items. Each item has a score between 0 and 3 and it is one of the valid depression measurement scales in a clinical trial.[] SAS, introduced by Zung, has been widely used in research and in clinical practice for the detection of anxiety.[] In this study, in order to evaluate the vision power of the examinee, the VA test was used by an ophthalmologist.
The cognitive-analytical intervention in psychogenic myopia had a significant effect on the increase of VA. Although, had a significant effect on the decrease of depression and anxiety. A raw score of depression based on BDI-II was 44 in the pretest and after 5-month follow-up it become 15. However row score of anxiety based on SAS was 47 in pretest and after 5-month follow-up it become 23, and so on VA increased as it comes in below table in both eyes [ and ]. Moreover, based on continues clinical interview and reports of family there was a significant improvement in complaints of the case.


Peter M; male, 36 years old, married. Father of a 5-year-old boy.
School: degree in Business and administration. Job: Director of a municipal office.
Diagnoses: Major depression form 2 months, without previous depressive episodes present in anamnesis.
Stress events: mother dead; a preferment in his job role.
Motivation to the treatment program: high and fully compliant.
Social resources: good as well personal abilities.
Psychotherapeutic treatment plan: 15 weekly single cognitive behavioral sessions. The cognitive behavioral therapy was based on a self-help manual.
Pharmacological treatment: started a month before the psychological sessions. It was suspended at the end of psychological treatment.
Outcome: no more depressive symptoms and full recovery of the social life habits and job satisfaction.
The table presents the five CBA-OE scales scores used for monitoring at six steps during psychotherapeutic treatment and at the conclusive session. The Z score refers to normative data obtained by the clinical sample before treatment (N=130). shows that, for each scale, the scores variations in Cohen’s d were assessed from T0, appointment request, to the final session T6. shows the raw and Z scores obtained in the five scales from the appointment request to the last session.


A 62-year-old man was admitted to our orthopedic hospital presenting severe lower back pain and a sciatic disorder following the L5 dermatome. The patient described the pain starting 6 days ago with a sudden onset. Initially, the patient took several non-steroidal anti-inflammatory drugs, such as diclofenac 150 mg and metamizol 1000 mg per day, without having any pain relief. After 3 days the patient consulted his general practitioner, where he received a local pain relief injection with a corticosteroid. Having no pain relief again and with a progressive symptomatology in the right leg accompanied by numbness, the patient was admitted to our hospital.
At admission the patient showed severe lumbar spine pain with radiation alongside the dermatome L5 on the right side till the first toe persisting for 6 days and suggesting a nucleus pulposus prolapse. Since 2 days the patient had a hypoesthesia in the same dermatome with a maximum in the lower leg and foot. The femoral nerve stretch test was positive on the right side and the straight leg raise test was early positive at 20° elevation of the right leg while these tests were negative on the left side. The Achilles tendon reflex and the patella tendon reflex could be provoked on both sides similarly. There was no motoric deficit in both legs, no bladder or bowel dysfunction, and no saddle anesthesia. Importantly, the skin had no abnormal findings. The medical history was unremarkable except of arterial hypertension and a nucleotomy of a cervical disc. For ambulant non-controllable pain the patient was admitted to our hospital. Under intravenous pain therapy the medical condition ameliorated slightly. For differential diagnosis the patient underwent a MRI scan of the lower back. This showed no herniated disc but a slight spinal stenosis between L4 and L5, as well as, an unspecific swelling of the spinal nerve root L5 on the right side (). Based on these findings a conservative treatment including oral analgesia, physiotherapy and a series of lumbar epidural corticosteroid infiltrations was initiated. After the first lumbar epidural infiltration the patient developed a stain on the skin of his lower back. A closer examination of this mark showed an erythema with a group of small red blisters which had been partially dried out (). There was no sign for a bacterial skin infection induced by the infiltration therapy. Also, the laboratory infection parameters including the leucocyte count and the C-reactive protein showed normal values.
The epidural infiltration therapy was stopped immediately and the patient was sent to a dermatological specialist who diagnosed a herpes zoster infection with neuralgic pain and hypoesthesia in the dermatome L5. After a pain therapy with ibuprofen 600 mg 3 times a day combined with a systemic antiviral treatment with acyclovir 800 mg oral 5 times a day for a period of 7 days the patient could be discharged soon from our hospital in a considerable ameliorated condition.


A 65-year-old lady referred to our hospital with a complaint of dyspnea. She was not a smoker and her past medical history consisted of hypertension and hyperlipidemia. On admission, physical examinations revealed an elevated jugular vein pressure accompanied by pulmonary rales up to two-thirds from the base of the lung. The patient was tachypnic (respiratory rate = 28) and blood pressure was 180/100 with a normal oral temperature (36.9 °C). Her electrocardiogram showed slight T-wave inversions in the leads V2 through V6 as well as leads I and aVL. Occasional ventricular premature complexes were also present (). The cardiac troponin level was normal. Echocardiogram of the patient showed a LV ejection fraction (LVEF) of 23% without regional wall motion abnormalities with dilated LV and atrium. Presence of moderate amounts of mitral regurgitation and moderate concentric LV hypertrophy was also noticeable.
Since there was no past medical history of heart failure, we proceed with coronary angiography to exclude ischemic causes as the basis of her disease. Cannulation of the left main coronary artery (LMCA) displayed normal courses of the LMCA, left circumflex (LCX), and LAD. An anomalous RCA as a separate large branch arose from the proximal area of LAD. The abnormally originated RCA had three significant stenoses in the proximal and distal portions (A-C). Attempts to cannulate the RCA with the right Judkins catheter were unsuccessful. Aortography did not show the presence of another origin for a supplementary RCA from another site (D). This was concluded to be a benign anomaly with an atherosclerotic disease. As the amount of LVEF impairment was not concordant with the epicardial coronary abnormalities, the patient was considered as a case of dilated cardiomyopathy and the patient was given long-term medical therapy for her heart failure as well as her atherosclerotic disease. At follow-up, it was observed that the patient had an asymptomatic clinical status.


A 30-year-old woman working as a clerical staff, requiring daily commuting by crowded trains, was suffering from left knee pain for last 6 years. Her difficulty in walking had worsened since last 6 months and did not respond to nonsurgical management. She was bedridden for 2 months before she presented to us. She also had a history of intermittent rectal bleeding.
On examination, there were cutaneous and soft tissue hemangiomas alongwith varicose veins throughout the affected left lower extremity from hip to toes, mainly on the gluteal region, posterior thigh, popliteal fossa, calf and foot [Figure -]. There were no visible salmon patches or portwine stains. Left lower extremity was not significantly enlarged compared to right lower extremity. There were no lesions over the anterior aspect of left knee. There was a 10° valgus alignment of the knee []. She had a flexion deformity of 20° with further flexion up to 40°. The movements were very painful in the available range of motion (ROM). The knee society pain score was 29 and her functional score was 0. There was no true leg length discrepancy. Dorsalis pedis and posterior tibial pulsations were palpable. The distal neurological status was normal. The right knee had full range of movements with no pain or deformity. There were no vascular lesions in right lower extremity. Bilateral hip examination was normal.
Radiographs [] showed severe degenerative tricompartmental arthritis in the left knee joint with bone loss from the lateral femoral condyle and the lateral tibial plateau. There was subchondral sclerosis and osteophyte formation. Phleboliths in the vascular malformations were noticed on the X-rays.
Magnetic resonance imaging and magnetic resonance venogram [] of the left lower limb with pelvis showed vascular malformation; mostly multifocal hemangiomas involving superficial and deep soft tissues of the entire left lower limb, pelvic region, rectum and left femur. There were no communications between the superficial and the deep lesions. Dual phase angiogram did not show any feeder artery or draining vein. As there were no feeder vessels, preoperative embolization was not done. Her coagulation profile was normal (bleeding time -5 s, clotting time -3 min, prothrombin time-13 s, international normalization ratio-1.1, activated partial thromboplastin time-28 s, Factor VIII-110% and Factor IX-100%). She did not have any systemic involvement. In the absence of limb hypertrophy, we could not label it as classical Klippel–Trenaunay syndrome, but it can be called as a variant of Klippel–Trenaunay syndrome.
Surgery was done under spinal anesthesia with tourniquet and exsanguination of limb using Eshmarch bandage. Anterior midline incision with medial parapatellar approach was used. Computer-assisted navigated surgery was done due to intra medullary involvement of the lesions []. Ci navigation system (DePuy, Brainlab, Germany) was used. Two thin threaded pins with bicortical holds were used to fix the clamps carrying the tracker ball arrays. The pin hold was good and remained well till the end of the surgery as the bone quality was good on both femoral as well as tibial sides. The synovium was grey-black with an orange tinge of the remaining articular cartilage. Femoral condyles were squared. Femoral bone cuts also revealed vascular malformations in the bone which were curetted out [].
Cemented TKA using posterior cruciate substituting femoral prosthesis (PFC Sigma, Depuy, Johnson and Johnson) with all-poly tibial component [] was done. Bone wax was used to control bony bleeding from metaphyseal lesions at the femoral cuts. Complete hemostasis was achieved after release of tourniquet. Closure was done under re-inflated tourniquet as there was generalized ooze but no active bleeding. Total tourniquet time was 80 min. Intravenous (I.V) tranexamic acid was used (900 mg I.V. infusion, first dose 30 min before tourniquet inflation, second dose 3 h later, and additional third dose 6 h after second dose).
Postoperatively, her hemoglobin dropped from 12.5 g% to 8.6 g% and she was transfused three units of packed cell volume. Intra-operative blood loss was 300 ml. Postoperative drain was kept for 48 h. The total collection in drain was 650 ml. Prophylactically oral rivoroxaban 10 mg (Xeralto - Bayer Zydus Pharma) was given 2 weeks postsurgery, because such patients have increased risk of thromboembolism. Subsequently, she was kept on oral aspirin 75 mg daily for a period of 4 weeks. Patient was kept on intermittent calf pumps for 3 days postsurgery. She was given DVT above knee stocking and encouraged to do active ankle-foot exercises. Aggressive mobilization was used under supervision of a physiotherapist. Continuous passive motion and full weight bearing walking with a walker were started from next day of surgery. She was walking with walker support and had 0–90° ROM by 7th day, when she was discharged. Her wound healed well, and sutures were removed on the 15th day postsurgery.
At 1-year followup, her knee society pain score improved to 97 and functional score improved to 100. She was walking normally without pain and had joined her job. She was also able to commute by crowded public trains without any support.
Histopathological examination of the removed bone and synovial tissue showed vascular proliferation with lymphoplasmacytic exudates. Conglomerate of vessels with thick walls and red blood cells in slit like lumina with hemosiderophages were seen. These findings were consistent with the histological examination of hemangiomas of Klippel–Trenaunay Syndrome.


An 18-year old female patient presented to our office for an evaluation of bilateral Achilles and posterior compartment pain ongoing for approximately 2 years. The pain was dull aching and made worse with exercising- mainly running. She was evaluated by another physician who recommended physical therapy, NSAIDS and night splints. While she responded modestly to physical therapy, she did not experience any relief from the splints or medication.
On physical examination, the patient had bilateral tenderness to the superficial posterior compartment roughly at the musculotendinous junction and muscle belly. She also had some swelling noted. On strength testing, the patient had 4/5 muscle strength with dorsiflexion. She had no loss of motion. Radiographs were obtained which showed a mild Haglund's deformity of the bilateral ankles. The patient's magnetic resonance imaging (MRI) revealed some subtalar synovitis and an os trigonum. These later pathologies were only mildly painful and did not contribute or replicate her main posterior leg pain. After reviewing the MRI, we offered the patient a pre and post exertional compartment pressure check, however she refused. Due to her lack of responsiveness to conservative therapy, we elected to perform a superficial and a deep compartment fasciotomy of her right leg and a right subtalar joint arthroscopy with extensive debridement, os trigonum resection, Haglund calcaneal exostectomy, as well as a left posterior superficial and a deep compartment release staged 6 months apart. Since, the right leg was more symptomatic, surgical treatment was first directed to this extremity. Intraoperatively, neither the gastrocnemius nor the soleus muscle showed any signs of ischemia or necrosis.
Immediately postoperatively, a Bulky Jones dressing was applied. She was allowed to weight bear in a Controlled Ankle Motion (CAM) walker 2 weeks postoperatively. Six months postoperatively, the patient had a full resolution of her symptoms on the right. She was continued to have symptoms on the left and underwent a similar compartment release of the superficial and deep compartments. She was allowed to weight bear immediately in a CAM boot. At 4 weeks after the surgery, she discontinued the boot and was progressed in her activity level. At 6 months after the second surgery, she had 5/5 plantar flexion strength bilaterally. She is currently running with minimal pain and is able to play soccer for her collegiate intramural team.


A 38 year-old male military officer presented with a history of left calf pain for the past 6 months. There was no history of trauma. The patient stated that he had surgical intervention on the right calf twice in form of fascial release and was now having similar symptoms on the left. He reported that his compartment pressure testing had been normal as per his military surgeon. The patient reported pain while running and could not run more than one mile without pain. The pain was sharp with some residual achiness and throbbing. Anti-inflammatories failed to relieve his pain.
On examination, the patient had pain with deep palpation of the gastrocsoleus complex []. He had normal strength testing, sensation and range of motion. The patient was offered a diagnostic compartment pressure check with a needle device; he refused since his symptoms were so similar to the other leg and his prior studies were negative. Due to his lack of responsiveness to conservative therapy, we elected to perform a superficial and a deep compartment fasciotomy of his left leg. Intraoperatively, the muscle showed no signs of ischemia or necrosis.
Immediately postoperatively, the patient was placed in a Bulky Jones dressing. He was allowed to weight bear 2 weeks postoperatively in a CAM boot. At 6 months postoperatively, the patient reported significant symptom improvement. He rated his pain as 0/10. He returned to aggressive physical activity, his normal military duties and running as desired without difficulty.


An 11-year-old male child presented with asymptomatic, dark-brown, spiny, hyperkeratotic, verrucous scales and ridges all over body since birth. The patient gave history that scales shed after scrubbing and reappear in 5-7 days. The patient was born of a full-term, normal vaginal delivery. There was no history of parental consanguinity, collodion presentation at birth, blistering or erythroderma. None of the family members were affected.
On examination, the affected skin showed symmetrically distributed, generalized, thickened, verrucous, dark-brown cobblestone-like lesions []. There was sparing of scalp and central part of face with involvement of ear []. Axillary region, dorsum of hand, popliteal fossa and antecubital fossa were less markedly affected [Figures and ]. Severe palmoplantar keratoderma and fissuring was seen []. Hair, nails, teeth, mucosa and other systemic examination was normal.
Skin biopsy (H and E staining) showed hyperkeratosis, acanthosis and papillomatosis with thick granular layer []. Perinuclear vacuolization and coarse keratohyaline granules were seen in upper spinous layers []. Electron microscopy could not be performed due to lack of facilities.


A 50 year old male labourer, presented with complaints of multiple nodules and papules associated with itching distributed over generalized body area and bony prominences of elbow and knee joint since 3 months duration [Figure and ]. There was history of fever associated with similar nodular eruption 1 year back which subsided on its own leaving behind hyperpigmentation at few areas. He also had complaints of tingling numbness with joint pain over lower limb and generalized weakness on and off since 1 year. No history of epistaxis and pedal edema.
No family history and contact history with known case of leprosy. General physical and systemic examinations were within normal limits. On dermatological examination, there were multiple grouped skin-colored, well-demarcated, shiny, non-tender nodules, firm to soft in consistency measuring from 0.5 to 1cm over lower back, upper back, elbow and knee joint []. Bilateral ulnar nerves and right common peroneal nerve were thickened, non-tender.
No lymphadenopathy, muscle weakness/wasting and trophic ulcer was present. Investigation revealed high blood sugar levels of 453 mg% and slight leukocytosis in blood picture. ELISA for HIV and VDRL was negative. Slit skin smear done by Ziehl-Neelsen stain revealed plenty of acid-fast bacilli occuring singly or in clusters, they appeared as uniform solid stained, long rods with tapering ends, with a bacterial index of 6 + and morphological index of >70% []. Histopathology of lower back nodule shows thinned out epidermis, presence of grenz zone and plenty of vacuolated macrophages, lymphocytes and histiocytes which are spindle shape arranged in storiform pattern in the dermis [Figure and ]. The patient has been put on multibacillary multidrug therapy (MBMDT) and is on regular follow-up.


A 55-year-old man was admitted to our intensive care unit on May 23, 2011 after a suicide attempt. He had ingested about 100 mL of 28% sodium chlorite solution, 0.75 L of whiskey, and ibuprofen (12 tablets of 400 mg), after which he had attempted to hang himself.
His relatives found him lying on the floor, cyanotic, and with lowered consciousness, as judged by his inadequate response to verbal stimulus. Upon arrival of the ambulance, he vomited and was immediately transported to the hospital’s emergency department and subsequently admitted to our department.
On arrival, his Glasgow Coma Scale score was 13 (E4M5V4), body temperature 33.6°C, heart rate 110/min, blood pressure 150/80 mm Hg, respiratory rate 28/min, pulse oximetry (SpaO2) 65%. Physical examination revealed generalized skin cyanosis, neck markings after strangulation, a silent abdomen, and incontinence for urine and feces. Blood samples from the arterial line rendered chocolate-brown serum. Further physical examination showed no abnormalities.
The patient’s clinical history disclosed depression, a previous suicide attempt, and alcohol abuse. He did not take any regular medication. The patient was sedated, intubated, and mechanically ventilated (ventilator Savina, Dräger Medical GmbH, Lübeck, Germany; ventilation mode was bilevel positive airway pressure).
The initial laboratory evaluation showed the following: hemoglobin 179 g/L, erythrocytes 5.45 × 1012/L, hematocrit 0.54, leukocytes 28.5 × 109/L, platelet count 279 × 109/L, body urea nitrogen (BUN) 5.5 mg/dL (Figure ), creatinine 142 μmol/L (Figure ), potassium 7.5 mM, osmolality 351 mosm/L, arterial lactate 6.5 mM, and myoglobin 433 ng/mL. Bilirubin was elevated to 28 mM whereas liver enzymes and coagulation factors were normal.
Arterial blood gas analysis was performed while ventilated with 100% oxygen. The pH was 7.25, PaCO2 4.45 kPa, PaO2 52.45 kPa, BE −12.2 mmol/L, HCO3 14.1 mmol/L, SaO2 99%, and lactate 6, 48 mmol/L. The laboratory tests also revealed 40.1% of MetHb (Figure ).
Toxicology revealed 2 mg/g of alcohol in blood, ibuprofen was found both in gastric content and urine. Gastric fibroscopy was performed to check for burns in the gastrointestinal tract caused by sodium chlorite. Multiple superficial necrotic spots were found in the antrum, the body of the stomach, and the duodenum, whereas edema of the pylorus was observed. After 3 days, ulcerations were found in the upper part of esophagus, stomach, and duodenum.
On admission, the electrocardiogram showed peaked T waves. A chest x-ray and a CT scan of the brain were normal.
Antidote therapy with MB was instituted 2 hours after admission by an intravenous bolus of 34 mg MB (approximately 0.5 mg/kg, Bleu Patenté V sodique Guerbet 2.5%, Guerbet, Roissy CdG Cedex, France). Four and half hours after admission, because of acute renal failure (anuria, hyperkalemia, metabolic acidosis), continuous venovenous hemodiafiltration (CVVHDF) was initiated on the multiFiltrate (Fresenius Medical Care, Bad Homburg, Germany) with hemofilter (Ultraflux AV600S, Fresenius Polysulfone, Fresenius Medical Care AG & Co. KGaA, Bad Homburg, Germany), with a blood flow of 250 mL/min, a hemodialysis flow of 1800 mL/h, and a substitution hemofiltration solution flow of 35 mL/kg/h for a total duration of 10.5 hours. Later that day, another 4 hours of hemodialysis was performed because of hyperkalemia (Fresenius Medical Care 4008B; dialysis filter, FX80, Fresenius Helixone, High-flux [Fresenius Medical Care AG & Co. KGaA, Bad Homburg, Germany]).
After 14 hours, the lactate level dropped to 2.55 mmol/L whereas the level of MethHb decreased to 15.2%, further declining to 10.2% after 29 hours (Figure ). Within 24 hours, the patient developed hemolytic anemia and disseminated intravascular coagulation (DIC, hemoglobin levels dropped from 179 to 116 g/L, thrombocytes from 279 to 164, D-dimer 29.1 mg/mL, international normalized ratio: 1.8, activated partial thromboplastin time ratio: 1.4). Over the course of the next 4 days, the hemolytic anemia deteriorated. Hemoglobin levels on the fifth day dropped to 59 g/L and platelet count to 77 × 109/L. In response, 4 units of packed red blood cells were transfused in 3 hours, amounting in total to 1074 mL).
Twenty hours after admission, a norepinephrine drip was instituted (0.16 μg/kg/min) because of hypotension (80/60 mm Hg) during the next 37 hours.
On day 8, the patient developed bronchopneumonia and sepsis (body temperature 39°C, antibiotics were initiated).
On day 10, the gastric and esophageal ulceration appeared improved, and on day 13 the patient was weaned from mechanical ventilation. Day 17 onward, intermittent hemodialysis (IHD) was performed every second day. By day 22, the patient started to urinate (2500 mL of urine/24 h, with blood urea 12 mg/dL (Figure ), creatinine 428 μmol/L (Figure ), and an estimated GFR of 15.6 mL/min). He was subsequently transferred to a medium care facility without further need of hemodialysis. In total, hemodialysis had been performed 16 times during hospitalization.
By day 43, the patient was transferred to a psychiatric ward and discharged from hospital on day 64. Patient gave written informed consent.


The patient was a 72-year-old woman with a history of hypercholesterolemia, treated with atorvastatin 10 mg per day, and toxic multinodule goiters, treated with Iodine131 2 years earlier. She had hypertension detected at 26 years of age, which had been well controlled with hydrochlorothiazide 25 mg per day, atenolol 50 mg per day, and enalapril 20 mg per day until 1 year earlier, when her blood pressure became persistently higher than 160/95 mm Hg, despite the successive addition of amlodipine 5 mg per day, spironolactone 25 mg per day, and doxazosin 4 mg per day. The patient was on a low-sodium diet and reported no consumption of nonsteroidal anti-inflammatory drugs. She had no dyspnea, chest pain, or claudication or lower limb weakness. Her son had undergone repair of aortic coarctation at 23 years of age.
Physical examination showed a blood pressure of 165/98 mm Hg, with no differences between arms. Her body mass index was 28.5 kg/m2. Funduscopy showed grade 1 hypertensive retinopathy. Cardiac auscultation revealed a 2/6 aortic systolic ejection murmur. Her femoral pulses were weak and delayed compared to the radial pulses. The lower limb blood pressure was 145/88 mm Hg. There was no clinical evidence of heart failure.
Analytical tests showed blood glucose 98 mg/dL, creatinine 0.8 mg/dL, creatinine clearance 72 mL/min, LDL cholesterol 95 mg/dL, HDL cholesterol 54 mg/dL, negative microalbuminuria, and a normal thyroid profile. Holter blood pressure monitoring showed a mean blood pressure of 158/92 mm Hg, with a nondipper pattern. Chest radiography showed rib punch, slight cardiomegaly, and a posterior mediastinal calcification suggestive of a thoracic aneurysm. The electrocardiogram showed bifascicular block (right bundle branch block with left anterior hemiblock). The transthoracic echocardiogram showed signs of left ventricular concentric hypertrophy predominantly septal (interventricular septum 13 mm), altered diastolic function with a normal ejection fraction, and a trans-coarctation peak systolic gradient of 24 mm Hg.
Thoracoabdominal computed tomography angiography (CTA) confirmed the initial clinical suspicion of aortic coarctation (Figure A), distal to the origin of the left subclavian artery, with a partially thrombosed post-stenotic saccular aneurysm of the descending thoracic aorta, measuring 40 × 36 mm. Magnetic resonance angiography (MRA) of the circle of Willis ruled out the presence of aneurysms and abdominal ultrasound showed no pathological findings.
After matching clinical recommendations about the convenience of an interventional repair with the patient's preferences, balloon angioplasty with placement of a covered stent was undertaken (Figure B). The peak-to-peak coarctation gradient fell from 26 to 8 mm Hg after repair.
Following the intervention, the patient's antihypertensive medication could be reduced and her blood pressure has been well controlled (mean: 135/80 mm Hg) with olmesartan 40 mg per day, hydrochlorothiazide 12.5 mg per day, and atenolol 25 mg per day. Three years after the repair the patient remains asymptomatic and with good blood pressure control.
This case report was approved by the Ethics and Research Committees of the Carlos Haya Hospital, and informed consent was obtained from the patient.


A 23-month-old female was admitted to the neurosurgery service with a 3-month history of a progressively enlarging neck mass. There was associated redness, swelling, and tenderness to palpation, but no neurological deficits on examination. A noncontrast computed tomography (CT) scan of the neck and magnetic resonance imaging (MRI) with contrast showed an osteolytic contrast-enhancing lesion primarily involving the C2 posterior elements, with a compressive circumferential epidural component extending from C2 to C5 []. A skeletal survey was negative for any other osseous lesions. She underwent C2 to C5 laminectomy with partial resection of the lesion without complication. Pathology was consistent with Langerhans cell (LC) histiocytosis (LCH). She was discharged home several days after her operation and subsequently started outpatient chemotherapy with cytarabine.


A 17-year-old male was admitted to the neurosurgery service with a 6-week history of a progressively enlarging scalp mass. There were tenderness and intermittent bleeding from the ulcerated lesion, with no focal neurologic deficits on examination. A noncontrast CT scan of the head and MRI brain, with and without contrast, showed a large transosseous contrast-enhancing lesion of the right frontal bone []. CT venography showed compression of the adjacent superior sagittal sinus. Surgery with gross total resection of the lesion was performed. Pathology was consistent with LCH. The patient was discharged home the day after his operation. He subsequently started outpatient chemotherapy with cytarabine.


A 65-year-old female patient presented with a swelling in the right ramus region for past 3 months. On intraoral examination, generalized destructive periodontitis and few carious teeth were noticed. She did not have any other relevant medical history or habits. Orthopantomograph (OPG) of the mandible showed a moth-eaten type of radiolucency.
An incisional biopsy was performed, and the specimen was sent for histopathological examination. The hematoxylin and eosin stained sections of the specimen under the microscope showed a highly cellular connective tissue stroma. The lesional tissue comprised diffuse, monotonous sheets of neoplastic, plasmacytoid cells with perinuclear halo [Figures and ]. Uniform background of eosinophilic area suggestive of amyloid deposits were also observed showing positivity for Congo red stain []. The lesion was diagnosed provisionally as plasma cell neoplasm, but to know whether it was unifocal or multicentric to give diagnosis of plasmacytoma or MM, respectively, we sent the patient for skull radiograph and other relevant blood investigations. It was observed that the patient was anemic with Hb count of 7 g/dl and the serum calcium level was also raised. Urine test showed elevated Bence-Jones protein of 0.10 g. Multiple punched out radiolucencies were seen in the skull radiograph [Figures and ]. On account of these features, the case was finally diagnosed as MM.
The swelling in the mandible and the radiolucency in OPG made us to give a clinical diagnosis of the odontogenic tumor. A differential diagnosis of eosinophilic granuloma, plasmacytoma, MM, lymphoma or Langerhans cell histiocytosis was made considering the mononuclear infiltrate in the histopathological section.


An 83-year-old man was brought to the emergency facility because of the dislodgment of his gastrostomy feeding tube the day before. He had undergone the gastrostomy 9 months before (Stamm’s technique) because of a swallowing disorder acquired after an acute ischemic stroke. As the stoma was stenotic, it was dilated and a new tube was inserted through the original orifice. The patient was discharged afterwards. He experienced abdominal pain and vomiting soon after the diet administration through the replaced tube. He returned to the hospital within 36 hours. Physical examination showed an ill-looking patient, dehydrated, with hypotension, and tachycardia. The abdomen was distended and diffusely tender, predominantly in the left hypochondrium and flank, close to the stoma. The abdominal computed tomography (CT), after the infusion of 40 mL of iodine contrast medium through the gastrostomy tube, showed a cavity formed by peritoneal blockade filled by gas and fluid ().
Therefore, the patient was submitted to an exploratory laparotomy. Surgical findings included a significant amount of a lumpy liquid in the peritoneal cavity, and peritoneal adhesions between the loops and the abdominal wall. At the site of the gastrostomy, a false path was found linking the abdominal orifice with a blockade purulent collection, adjacent to the stomach. Another gastrostomy was performed and the stomach was sutured to the abdominal wall. A thorough lavage of the peritoneal cavity with saline was undertaken and the abdomen was drained. The patient was referred to the intensive care unit, but the outcome was accompanied with evisceration requiring re-operation. Multiple organ failure ensued and he died 43 days later.


Parents of this 2-month old male infant were complaining of bilateral identical movements of the hands of the child. He was born to healthy and non-consanguineous parents by spontaneous delivery at 37th gestational week with 2,950 gr birth weight, 49 cm birth length. There was no family history. He was breast feeding. The physical examination was normal. A complete physical and neurological examination was performed and mirror movements were assessed according to the Woods and Teuber grading scale (). Except for bilateral involuntary synkinetic imitative movements in hands, neurological examination was normal. Amplitude of the intentional movements were greater than involuntary movements of the contralateral hand. At first, transfontanel ultrasonograhy was performed to evaluate brain which showed corpus callosum hypogenesis and then cranial MRI was performed to assess brain exactly. Corpus callosum dysgenesis, hypogenesis and dilation of bilateral lateral ventricular posterior horns (colpocephaly) were detected. The patient was asymptomatic except for mirror movement of the hands. On follow-up, he started to walk at 13 months and started to say one word nearly at 15 months of age. He was cooperating well. Motor-mental developmental steps were appropriate for his age.
At the age of 7 years, he was referred to child psychiatrist because of hyperactivity and inability to concentrate in the school. After psychiatric evaluation he was started to use metylphenydate due to attention deficit and hyperactivity syndrome. He was free of symptoms after 1 year of metylphenydate treatment. No other serious physical or psychological disease occured. The mirror movements were decreasing in amplitude by years and were not so serious to affect normal life activities.