Case 3. This is a 21-year-old case of a male with exercise limiting chest pain and no reported history of syncope. The patient reported intermittent chest pain since the age of 16 which had become worse since the beginning of the military training. The patient underwent coronary CT angiogram showing a left main coronary artery with an anomalous takeoff from the right coronary cusp. The left main coronary artery (LMCA) was seen to take a posterior course behind the ascending aorta before bifurcating into the left anterior descending and left circumflex arteries (). Exercise stress testing was completed with myocardial perfusion imaging during which the patient exercised for 14 minutes and 35 seconds (14.4 METS) with no ischemic EKG changes and no angina. Myocardial perfusion images showed no evidence for ischemia, normal left ventricular ejection fraction, and normal wall motion. The patient was started on no additional medical therapy and continues to experience some symptoms with heavy exertion.


A 76-year-old Austrian woman with recent bioprosthetic aortic valve replacement presented herself with recurring progressive dyspnoea (NYHA III).
She had been operated half a year ago because of a hemodynamically relevant stenosis of the tricuspid aortic valve. Access was enabled by mediane sternotomy and after application of the bypass (entire duration of an hour) and initiation of cardioplegia the calcificated valve was replaced by a mitroflow pericardial prothesis with a diameter of 21 mm. Operation was proceeded without any complications and the patient got extubated already the day after.
After initial improvement of symptoms within the first months she finally again suffered from a rapid drop of her performance level; however, she did not observe any other general symptoms or fever. The patient clinically showed signs of cardiac decompensation with minimal pretibial edema, but without any orthopnoea. An electrocardiogram showed an already known atrial fibrillation in normal frequency without pathological signs of repolarisation.
Auscultation, however, revealed a 3/6 holosystolic murmur at the left parasternal border with multidirectional conduction, so that again a two-dimensional transthoracic echocardiography was performed. Apart from normal chamber sizes and left ventricular (LV) ejection fraction, a huge dilatation especially of the right atrium with low tricuspid regurgitation due to coaptation dysfunction was shown with a systolic regurgitation velocity of 2.8 m/sec. which equates to a slight pulmonal hypertension.
Additionally, however, a high-velocity systolic jet just below the aortic valve was noticed entering the right atrium (RA), which was initially mistaken for tricuspid regurgitation (Figure ). Spectral Doppler distinguished a velocity of 5.6 m/sec, whereas a three-dimensional transesophageal echocardiography confirmed the jet taking a direct course from the left ventricle (LV) into the RA, consistent with a Gerbode defect (Figure ).
Due to the lack of typical symptoms of an endocarditis and the newly occurring pulmonary hypertension as well as the LV-RA-shunt, the recent aortic valve replacement was finally assumed to be the reason for this.
As a result diuretic therapy was increased and drug therapy optimized which led to an improvement of the patient’s condition (NYHA II). The Gerbode defect finally required no surgical intervention.


A 56-year-old woman was diagnosed with Durie-Salmon stage IIIA and International Staging System stage II MM. Four cycles of VAD (Vincristine 0.4 mg for 4 days, doxorubicine 9mg/m2 for 4 days, dexamethasone 40mg for 12 days) and zoledronic acid were given. Bone marrow aspiration and biopsy was normal. Esbach was 12 g/day at the beginning and it was negative after therapy. Induction with high dose melphalan (200 mg/m2) and mobilization was performed with cyclophosphamide (4 g/m2) plus G-CSF (5-10 µg/kg/d) and HSCT was performed. Hypothyroidism was detected 6 months after transplantation. Free T3 was 0.462 pg/mL (N:T3: 2.3-4.2 pg/mL), free T4 was 0.179 ng/mL (N: 0.61-1.12 ng/mL), and TSH was 771.6 mIU/L (N: 0.34-5.6 mIU/L). She had no known prior history of thyroid dysfunction. Antimicrosomal antibody was found to be high 600.1 U/mL (normal range: less than 50) and antityroglobuline was within normal limits (2.21 mg/dL). At the beginning hypothyroidism could not be controlled and dose was increased up to 200 mcg (). TSH alpha subunit and hypophysis MR were performed. Pathologic finding was not found. She has been free of disease in follow-up for 3 years and with normal thyroid function.


A 62-year-old Caucasian woman with a 20-year history of non-toxic multinodular goiter presented to our hospital for surgical treatment. She had no associated symptoms such as dysphagia, dyspnea, or dysphonia. Six years earlier, the patient had undergone a left nephrectomy for clear cell carcinoma. A physical examination revealed an indolent subhyoid swelling accompanied by a few round nodules. A preoperative neck ultrasound examination showed an enlarged thyroid gland, with a retrosternal left lobe. The whole left lobe was occupied by an isoechoic vascularized macronodule. No suspicious central or lateral neck lymph nodes were found. The indication for surgery was the result of the sonographic features of the lesion, the thyroid volume, and the retrosternal position of the left lobe; for these reasons fine needle aspiration cytology was considered unnecessary. A total thyroidectomy was performed in our department in April 2010. Her parathyroid glands and recurrent nerves were recognized and preserved. The postoperative course was uneventful, and she was discharged on the second postoperative day.
Upon macroscopic examination, her thyroid was enlarged and multinodular. The left lobe, which was larger than the right one, was occupied by a solid macronodule measuring 53mm; small yellow areas were observed within this nodule. A microscopic examination of the gland revealed thyroid hyperplasia. The left lobe nodule was characterized by microfollicular proliferation with edema and areas of sclerosis and was surrounded by an incomplete fibrous capsule. Inside the nodule, small yellow solid areas were present, and there was a proliferation of large cells with clear cytoplasm and small and hyperchromic nuclei. This second population of tumor cells was well-demarcated from the first (Figure
). Neoplastic cells were immunonegative for thyroid transcription factor-1 (TTF-1), Hector Battifora mesothelial cell monoclonal antibody-1, and galectin-3 (Figure
), whereas CD10 was positive (Figure
). Specimens of the previous renal cell carcinoma were examined, and histopathological features matched the present histology. With these findings, a diagnosis of metastasis of renal cell carcinoma in thyroid follicular adenoma was made.
Postoperative staging demonstrated no other foci of CCRC metastasis.
Three years after her thyroidectomy, no evidence of recurrent CCRC has been found.


A 55-year-old gentleman was admitted via the primary PCI pathway after presenting with severe, persistent central chest pain following exertion. He had no prior history of coronary artery disease, and his risk factors included tablet-controlled type 2 diabetes mellitus and a current smoking history. His ECG demonstrated significant ST-segment elevation in leads V1-3, though he was clinically stable on arrival with no evidence of decompensating left ventricular failure.
The patient also reported a history of haemophilia A (codon 2164 mutation) with no prior major intra-articular or intramuscular haemorrhage. At presentation, he was well controlled on tranexamic acid maintenance therapy. Haematological advice was that he required recombinant factor VIII or desmopressin only prior to major surgery.
Given his lack of significant bleeding episodes and his clinical presentation with chest pain and ST-segment elevation, he was transferred for primary percutaneous coronary intervention without further delay.
Catheterisation was performed via the right radial artery using a 6F JR4 and XB 3.0 guide catheter. The patient was loaded with 300 mg aspirin and 60 mg prasugrel prior to the procedure and was then administered with 5000 units of heparin. A flow-limiting 95% stenosis in the proximal left anterior descending artery was identified, with no significant disease in the left mainstem, circumflex, or right coronary arteries (). Two attempts at thrombus extraction using a Pronto LP device yielded very little, so the decision was made to direct stent the proximal LAD with a bare metal stent (3.5 × 23 mm Vision). The final result was excellent, with TIMI 3 flow down to the distal LAD (). Haemostasis at the puncture site was achieved using a TR band filled with 13 mLs air.
After PCI, the patient was transferred to the High Dependency Unit for observation. His blood revealed an APTT of 54.1, factor VIII level of 60%, and a peak troponin I level of 6.72. He was well after procedure and haemodynamically stable, with no further chest pain and normalisation of his ST-segment elevation. There was no bleeding or haematoma formation at the right radial puncture site. No further haematological intervention was needed.
The patient had an unremarkable recovery and after the requisite 72-hour monitoring period he was discharged on 75 mg/day aspirin and 75 mg/day clopidogrel for one month, followed by lifelong 75 mg aspirin thereafter with prophylactic tranexamic acid during clopidogrel therapy.


A 65-year-old woman was admitted because of progressive paraparesis (June 2012). Six months before admission, she had presented with fever, chills, abdominal pain and unusual headache for one week with spontaneous recovery. Abdomen and brain MRI were normal at that time. Two months before admission, she described a progressive onset of walking difficulties. At the same time, constipation and dysuria were noted. At her admission, neurological examination noted walking difficulties related to moderate paraparesis, moderate superficial and deep sensory dysfunction of the lower limbs and urinary retention. A very slight dysfunction of mental processing without speech disturbance was noted and confirmed by her family. No seizures, dyskinesia, movement disorders, psychiatric symptoms or autonomic dysfunction were observed.
Spinal cord MRI showed longitudinally extensive myelitis from C5 to T10 with gadolinium (Gd) enhancement (Figure
A,B). Brain MRI follow-up showed T2 hyperintensities within the insular regions, medial temporal lobes and thalamus (Figure
C), associated with gadolinium enhancement of the meninges and ventricles (Figure
D). Analysis of the cerebrospinal fluid (CSF) showed moderate lymphocytic pleocytosis (53 cells/mm3), mild increased protein concentration (0.91 g/L) and oligoclonal bands. PCR assays for herpesviridae in CSF were all negative. Ophthalmological examination showed delayed P100 latencies on the right side on visual evoked potentials and asymmetric global retinal nerve fibre layer (RNFL) thicknesses on optical coherence tomography (OCT) suggestive of asymptomatic right optic neuritis (ON). OCT analyses were both perfectly centred on the optic nerve head (Figure
E). Tests for anti-aquaporin 4 (AQP4) antibodies (Ab) in serum were negative. EEG showed slow and discontinuous activity in the left fronto-temporal regions. Extensive tests for auto-Ab (anti-onconeuronal, anti-DNA, anti-phospholipid, anti-voltage-gated potassium channel, anti-glutamate acid decarboxylase) were negative except for anti-NMDAR-Ab (IgG isotype) in the CSF and serum. No cancer was found (gynaecological examination, pelvic ultrasonography and MRI, mammography, total-body computed tomography scan and positron emission tomography scan were all normal).
Intravenous corticosteroids (1 g per day during 3 days plus 5 days) were given but the paraparesis worsened (bedridden patient). Brain and spinal cord MRIs follow-up (July 2012) showed an extension of T2 lesions without Gd enhancement (Figure
F) and a stable extensive T2 lesion without Gd enhancement, respectively. As soon as anti-NMDAR-Ab were detected, plasma exchanges (PLEX) were initiated and followed by intravenous rituximab (375 mg/m2 every week for 4 weeks) plus tapering oral corticosteroids (1 mg/kg). Brain and spinal cord MRIs follow-up (August 2012) showed the disappearance of the extensive spinal cord T2 lesion and stable cerebral T2 lesions. Biological follow-up of anti-NMDAR-Ab rates in CSF and serum was performed (Table
). CSF analysis was weakly positive just after corticosteroids/PLEX (M1) and was negative 2 months after rituximab (M3). Serum analysis was less correlated to clinical status. Four months after the inaugural myelitis, there was a significant improvement of the paraparesis (the patient was able to walk for 5 metres with bilateral support) and a total regression of the slight psychomotor retardation was observed. Unfortunately, the patient presented rapidly evolving pneumocystis pneumonia at this time and she died of respiratory failure. Autopsy was not performed.


A 59-year-old female first presented at the Hino Municipal Hospital (Tokyo, Japan) following a relapse in spells of vertigo 15 years following EDS (). The patient’s vertigo attacks usually lasted several hours, with nausea and a worsening of tinnitus, and occurred a few times a week. Careful analysis of the patient’s history revealed marked anxiety regarding the vertigo attacks, although the patient’s Hospital Anxiety and Depression Scale (HADS) score revealed no significant anxiety. The patient had no history of migraine or a present migraine. Sertraline (50 mg/day) therapy was commenced. The patient’s vertigo spells as well as anxiety improved for two weeks and stabilized. The symptoms of Ménière’s disease did not recur. Sertraline treatment ceased after six months. The patient’s functional level scale (FL) score () improved from 4 to 1, and the patient remained symptom-free a year later.


A 45-year-old male experienced a relapse in vertigo spells seven years after intratympanic gentamicin treatment. The patient had no history of anxiety disorders or migraines, past or present. The patient feared a relapse of Ménière’s disease and wanted to be a candidate for EDS. Although the patient’s HADS score was within the normal range, the patient’s anxiety regarding the attacks was marked. The vertigo attacks occurred several times a week for ~1 h. The patient was persuaded to undergo treatment with sertraline (50 mg/day) for two weeks prior to surgery. The vertigo spells were completely controlled. Sertraline treatment was continued for six months and, a year later, the symptoms remained well controlled.


A 72-year-old female first presented at the Hino Municipal Hospital following a relapse in vertigo spells 18 years after EDS (). The patient had no history of anxiety disorders but did have a migraine headache. The vertigo attacks had occurred three times a week for a month and were usually accompanied by headache. The patient was afraid of a relapse of Ménière’s disease. However, from the patient’s clinical history, migraine-related vertigo was suspected and sertraline (50 mg/day) therapy was initiated. Following two weeks of treatment, the vertigo and headaches were controlled. Sertraline treatment was continued for six months, and the patient remained vertigo-free a year later.
In all three patients, vertigo attacks were completely controlled by the administration of sertraline; however, the hearing thresholds of the patients did not alter.


A 32-year-old female patient reported for the filling of her lower 1st molar. On clinical examination tooth number 36 was root canal treated one month back (). It was asymptomatic and the occlusogingival height of the remaining crown structure was approximately 4 mm. The radiographic findings revealed well obturated canals with no periapical changes.
A conservative approach of restoring the tooth with an endocrown was decided as the treatment option, as more than half the residual tooth structure was remaining and there were no occlusal wear facets. On additional request by the patient for an advanced and a prompt restoration, CAD/CAM ceramic was chosen.
After removal of the provisional restoration, preparation for endocrown was initiated. Resin modified glass ionomer cement (Fuji II LC GC Corporation, Tokyo, Japan) was used to achieve a flat pulpal floor and to block the undercuts. The preparation consisted of a circular equigingival butt-joint margin and central retention cavity into the entire pulp chamber constructing both the crown and the core as a single unit. The appropriate reduction of the buccal and lingual walls was done ().
Interocclusal space was carefully evaluated and occlusal reduction done to achieve a clearance of 2 mm. Shade-B1 was selected (VITAPAN Zahnfabrik, Germany). Retraction cord was placed and impressions made with polyvinyl siloxane impression material (Aquasil LV, Putty/Light Body, Dentsply DeTrey, Germany) using putty wash technique. Die stone model was fabricated.
CAD/CAM Processing. The three-dimensional reconstruction of the preparation was done using the Yenadent D40 milling machine (Yenadent, Istanbul, Turkey) and DWOS scanner (Dental Wings Inc., Montreal, Canada). The 3D scanning of the individual die and the antagonist arch for occlusal function (virtual articulation) were done. The milling was then initiated on a monolithic solid zirconia block (Metoxit AG, Thayngen, Switzerland) (Figures and ).
The finished endocrown was checked for shade, fit, and occlusion in the patient's mouth and then cemented using dual cure resin luting agent (Variolink, Ivoclar/Vivadent, Schaan/Liechtenstein).
Clinical and radiographic evaluation was done and follow up after 28 months showed no secondary caries, fracture, discoloration or loosening/decementation of the crown (Figures and ).


A 26-year-old female patient reported with a chief complaint of pain since 2 days. On radiographic examination radiolucency involving pulp of tooth 36 was seen. Based on the clinical and radiographic examination tooth 36 was diagnosed with acute irreversible pulpitis. Root canal treatment was performed. Based on the remaining tooth structure, that is, approximately 4-5 mm, occlusal evaluation, and patients esthetic demands, IPS E.max Press endocrown was decided as the treatment option. The endocrown preparation and the impression technique were performed as described in the previous case. IPS E.max Press HO Lithium-disilicate glass ceramic ingots (Ivoclar/Vivadent, Schaan/Liechtenstein) were used for the press technology. The restoration was fabricated according to the lost wax technique of investing and wax pattern burnout followed by pressing of the ceramic ingot in the pressable furnace at a press temperature of 915–920°C. It was then finished and polished with Proxyt pink polishing paste (Ivoclar/Vivadent, Schaan/Liechtenstein). The endocrown was cemented using a dual cure resin luting agent (Variolink, Ivoclar/Vivadent, Schaan/Liechtenstein). Clinical and radiographic evaluation was done and a 28-month followup showed no secondary caries, fracture, discoloration or loosening/decementation of the crown (Figures , , , , , and ).


A 52-year-old woman was admitted to the cardiology department ICU with generalized edema and orthopnea in July 2008. She had no significant medical history and was not taking any medications. An electrocardiogram revealed atrial fibrillation with a heart rate of 178 beats per minute. A chest X-ray detected pulmonary edema and cardiomegaly. A two-dimensional echocardiogram showed an ejection fraction of 57%, an enlarged left atrium without thrombi and grade 3 diastolic dysfunction. Diltiazem was used for heart rate control, but amiodarone or dopamine was not used during the hospitalization. Thyroid function tests were ordered as part of the atrial fibrillation evaluation. Serum free T4 level was 0.282 ng/dL (normal, 0.8–1.71 ng/dL), T3 was 0.307 ng/mL (normal, 0.6–1.6 ng/mL), and TSH was < 0.005 μIU/mL (normal, 0.4–4.8 μIU/mL). A physical examination revealed no apparent thyroid associated orbitopathy, but a large asymmetrical goiter, which was easily visible and palpable, was observed. A thyroid ultrasonogram demonstrated a 4.6 × 5.1 × 2.3 cm cystic dominant nodule in the lower portion of the right thyroid lobe and atrophy of the contralateral lobe (Figure ). Five milliliters of serous, thin fluid was aspirated from the cyst. Aspiration cytopathology of the solid portion of the nodule was consistent with adenomatous goiter. An endocrine consultation was sought to evaluate the possibility of thyroid disease. She was diagnosed with NTI accompanying heart failure and a decision was made not to replace thyroid hormones but to follow-up on the thyroid function testing. Her symptoms resolved after 2 weeks, and she was subsequently discharged with a calcium channel blocker, an angiotensin receptor antagonist, a diuretic and aspirin. Thyroid function tests demonstrated a free T4 level of 0.860 ng/dL, and a TSH level of 0.017 μIU/mL at discharge.
The thyroid function tests were not followed up until February 2012 (42 months) when she consulted the endocrinology department again for follow-up of the thyroid nodule. Her free T4 and T3 levels had increased to 5.30 ng/dL and 4.80 ng/mL, respectively, and TSH was < 0.005 μIU/mL. Anti-thyroid antibodies including anti-TPO antibody, anti-thyroglobulin antibody and TSH-binding inhibitory immunoglobulin were all negative. A follow-up thyroid ultrasonogram revealed an increase in the size of the previously observed nodule in the right lobe to 6.4 × 7.8 × 3.2 cm with contralateral lobe atrophy. A 99m-Technetium-pertechnetate thyroid scan demonstrated heterogeneous uptake in the large nodule of the right thyroid gland with no visibility in the remaining gland, suggesting a functioning toxic nodule (Figure ). She refused an operation or radioactive iodine therapy; therefore, 10 mg methimazole twice per day was prescribed. Her free T4 and T3 levels normalised (0.873 ng/dL and 0.941 ng/mL, respectively) 2 weeks later, and TSH was 0.009 μIU/mL.


A 54-year-old man presented with right flank pain for eight months. The physical examination detected a palpable mass in the right upper abdominal quadrant. The computerized tomographic scan revealed an enlarged right kidney up to 20 × 15 cm with hydronephrosis and nodular thickening of the pelvic lining, accompanied with enlargement of the hilar lymph nodes. The findings were suggestive of urothelial carcinoma with lymph node metastasis. Multiple renal stones were also present. No clinical evidence or a previous history of any other neoplastic lesion was identified. There was no previous history of any hormonal treatment. The patient was referred to our institution for further surgical management.
Intraoperatively, nephroureterectomy was performed, but there were residual matted lymph nodes extending from the renal hilum with aortic encasement that could not be removed. On gross examination, the cystically dilated kidney showed multiple papillary and solid nodules of pale tan tissue, up to 2.5 cm in diameter, involving almost the entire pelvicalyceal surface with thin residual renal parenchyma. The ureter was free of involvement.
Histologically, the tumor was composed of complex papillary structures lined with neoplastic cells with frequent hobnail appearance, clear cytoplasm, and large round nuclei containing distinct nucleoli (). The papillary cores contained a variable amount of stroma with occasional deposits of hyaline basement membrane-like material. Concentric and laminated calcifications or psammoma bodies were focally observed. The tumor invaded into the atrophic renal parenchyma and the peripelvic fat tissue. The pelvicalyceal surface was completely lined with clear cells and hobnail cells. No invasive or in situ component of conventional urothelial carcinoma or adenocarcinoma of non-CCA type was identified after a total submission of the renal pelvis for histologic examination in 32 sections. No benign Müllerian-type epithelium was seen. The neoplastic cells showed positive immunoreaction for cytokeratin (CK) 7, hepatocyte nuclear factor-1 beta (HNF), and PAX8, with focal expression of CA-125 (). The immunohistochemical stains for RCC marker antigen, CD10, uroplakin III, p63, and CK20 were negative.
Postoperatively, adjuvant chemotherapy was given, composed of vinblastine, Adriamycin, and cisplatin. Multiple pulmonary metastases were detected after three cycles of chemotherapy. He died of the disease six months after surgery.


Our case is a 48-year-old lady who was referred to our department with a long-standing history of unilateral left-sided pulsatile tinnitus for one year. There were no associated complaints such as trigeminal neuralgia, facial weakness, vertigo, or hearing deficits. Clinical and otoscopic examinations were normal. An arteriovenous vascular malformation was suspected, so she underwent a 3-tesla magnetic resonance (MR) imaging of brain and cerebellopontine angle along with a 3D time of flight (TOF) angiography in our hospital.
MR and MRA base images show a serpiginous vessel (black arrows in Figures and ) arising from the cavernous part of the left internal carotid artery (ICA), coursing posterior, and laterally around the dorsum sellae up to the basilar artery (BA) (white arrows in Figures and ) in the prepontine cistern. This vessel is the primitive persistent trigeminal artery (PPTA) forming an anastomosis between the carotid and the basilar systems. The proximal basilar artery is hypoplastic (white arrow in Figures and ) but increases in calibre beyond the site of anastomosis (white arrow in ) and divides into its terminal posterior cerebral artery branches. On TOF angiographic projection images (Figures and ), the PPTA forms a “Tau” sign (block arrow) with the ICA (∗) as it courses from ICA to basilar artery (white arrow).


A 74-year-old Japanese man was transferred to our department from a local hospital under a diagnosis of pneumonia. He had been in good health five days before transfer to our hospital, but with pyrexia and dyspnea upon effort. He had been treated with oral antihyperglycemic drugs to control type 2 diabetes mellitus five years previously. He was a carpenter who did not consume alcohol, but had a smoking history of 44 pack-years. Although he could walk unaided, he seemed very ill. His consciousness level was E4V5M6 (Glasgow coma scale), and his vital signs were: blood pressure, 90/50 mmHg; temperature, 37.3°C; heart rate, 86 beats per min with sinus rhythm; respiratory rate, 24 breaths per minute; and oxygen saturation, 78% with an oxygen supply of 10 L/min delivered via a reservoir mask. He was immediately admitted to the intensive care unit (ICU). Physical findings were normal except for coarse crackles in the bilateral middle to lower lung fields. Upon admission to our hospital (day 1), a chest X-ray revealed infiltration throughout all lung fields except the left upper lung. These results together with positive findings of pneumococcal urinary antigens indicated a diagnosis of pneumococcal pneumonia (CURB 65 score was 3, which is defined as Confusion, Urea nitrogen, Respiratory rate, Blood pressure, and 65 years of age and older; pneumonia severity index was class IV). He was immediately intubated and treated with intravenous meropenem (1.5 g/day) plus ciprofloxacin (600 mg/day), which gradually improved his respiratory status over a period of two weeks. However, acute watery diarrhea amounting to 4 L/day (determined by inserting a rectal tube) together with pyrexia (38°C) was evident on day 19 (Figure
). Although stool specimens were negative for Clostridium difficile toxins A and B, pseudomembranous colitis was considered and metronidazole (1 g/day) was started via a nasogastric tube (Figure
). However, the watery diarrhea was refractory to metronidazole, and Gram staining stool samples revealed abundant fecal leukocytes (Figure
), suggesting colonic membranous damage. Although colonic fiberscopy on day 22 provided limited information only as far as the sigmoid portion because the health status of the patient was unstable, definitive evidence of pseudomembranous formation other than membranous edema or hyperemia was not found. Furthermore, methicillin-resistant Staphylococcus aureus (MRSA; 104 colony forming unit/mL) was predominantly isolated from stool cultures on day 19 whereas other enteric bacterial pathogens and parasites or ova were negative, indicating MRSA enterocolitis. Vancomycin (VCM; 1 g/day) delivered via a nasogastric tube completely resolved the watery diarrhea within a few days. However, at day 30, high fever recurred with moderate watery diarrhea (~500 mL/day), and one of two sets of blood cultures was positive for MRSA. Thereafter, intravenous VCM (0.5 g/day) with dose adjustment for renal dysfunction was initiated, and repeated blood cultures were negative for MRSA. The total amount of watery diarrhea excreted between days 19 and 34 was 22.43 L and the maximum volume per 24 h reached 3.50 L on day 25.
The general status of the patient gradually improved except for persistent low-grade fever, and he was able to walk unaided by day 64. Intermittent pain in the right hip joint started on day 71 after he started rehabilitation for walking (Figure
). A physical examination revealed the obturator or psoas sign, and he was positive for the Gaenslen maneuver, suggesting inflammation at the retroperitoneal or lumbar vertebrae and/or sacroiliac joint. We explored the source of the fever and right hip joint pain using magnetic resonance imaging (MRI) (Figure
A and B) and 67gallium scintigraphy of the hip joint. T2-weighted MRI (T2WI) revealed an area of high intensity around the head of the right femur indicating fluid collection (Figure
A). Fat-saturated T2-weighted images (T2WI) revealed another area of high intensity on the right obturator externus or adducent muscles (Figure
B), suggesting right hip arthritis that 67gallium scintigraphy confirmed as a hotspot (Figure
C). Joint drainage fluid collected on day 100 showed abundant leukocytes enclosed by phagocytic gram-positive cocci (Figure
D) that were later identified as MRSA. Pulsed-field gel electrophoresis (PFGE) showed that the MRSA strains in the samples isolated from stool (day 19), blood (day 30) and synovial fluid (day 100) were identical (Figure
), suggesting a hematological spread of MRSA originating from the colonic membrane damaged by MRSA enterocolitis. The PFGE profiles of two strains isolated from sputum on days 10 and 44 and that of a strain isolated from a catheter tip on day 20 were similar, but all three strains differed by one band from strains isolated from stool, blood and synovial fluid specimens. These strains were considered to be derived from the same clone with different subtypes according to Tenover's criteria
[], and they had the same antibiogram (VCM ≤ 1, teicoplanin ≤ 1, linezolide 2). These findings suggested that persistent colonized MRSA could co-evolve even in a host environment.
None of the findings were consistent with the criteria for confirmed toxic shock syndrome (TSS; fever > 38.9°C, hypotension ≤ 90 mmHg), diffuse erythroderma, desquamation (unless death occurs before desquamation) and the involvement of at least three organ systems) or probable TSS (missing one characteristic for confirmed TSS)
[] or pseudomembranous colitis. Thus, he was finally diagnosed with MRSA enterocolitis that subsequently caused septic arthritis in the right hip.
Indeed, multilocus sequence typing confirmed that the allelic profiles of the MRSA strains obtained from blood, sputum and synovial fluid on days 30, 45 and 100, respectively, were identical (Table
), belonged to sequence type (ST) 764, clonal complex (CC) 5
[] and carried SCCmec type II. These strains carried enterotoxins G and I, but no other SEs or TSST-1. Our patient was discharged uneventfully on day 156 after completing a two-month course of oral linezolide (1200 mg/day) with intravenous clindamycin (CLDM; 1200 mg/day) followed by teicoplanin (400 mg/day) with intravenous CLDM (1200 mg/day) infusion.


Our case is that of a 32 year old male, who had a small dermal nodule on the dorsal aspect of middle finger for three years. There was no associated pain, joint swelling, redness or restriction of joint mobility. The patient did not recall any specific history of trauma. Later on the lesion becomes ulcerated due to which he seek medical attention. The patient was laborer by occupation and belonged to the rural part of the country. The initial clinical suspicion was leishmaniasis, due to which a punch biopsy was done. The biopsy revealed a dermal fibro-osseous lesion and therefore excision was advised. The lesion measured 1.7 × 1.5 × 1.2 cm and no connection with underlying bone was demonstrated. Post-operatively, the wound healed quickly. The patient was followed for one year, however no recurrence was noted.
Grossly the specimen consisted of a skin covered fragment with a firm nodular lesion in dermis measuring 1.5 cm in maximum dimention. Microscopic sections examined revealed biphasic fibro-osseous lesion present in the dermis, composed of stromal and osseous components. Overlying skin appeared midly hyperplastic and borders of the lesion were well circumscribed. Stromal component of the tumor is composed of bland spindle shaped cells with interspersed blood vessels. Stromal cells appear fibroblastic with elongated nuclei, eiosinophillic cytoplasm and indistinct cell borders. They show minimal nuclear atypia and pleomorphism. Osseous component was formed by mature bony trabeculae with prominent osteoblastic rimming. Zonation pattern was not evident in the lesion. No mitotic activity was noted. The lesion appeared completely excised (Figure
).
Immunohistochemical stains were performed on the section by DAKO envision method using antibodies against pancytokeratin (CKAE1/AE3), ASMA, S100 and CD 34. Stromal cells were negative with cytokeratin, however focal positivity was noted with ASMA, CD 34 and S100 stains (Figure
).


A 36-year-old Caucasian male patient was admitted to the emergency department with acute onset of left thoracic chest pain. The pain suddenly appeared without physical activity, cough or trauma. Medical history of this patient revealed a chronic low back pain, surgery for left epicondylitis lateralis humeri and no specific cardiovascular risk factors except for active smoking.
Physical examination showed a man with diminished breath sounds on the left apex over the left anterior chest without palpable subcutaneous emphysema and with normal oxygen saturation. There were no other abnormal clinical findings.
A routine chest X-ray was performed and suggested an apical pneumothorax on the left side, though HRCT showed a massive bulla of the left lung, with a 10 cm diameter, occupying the whole upper left hemithorax, with signs of centrilobular emphysema also on the right side [Figure
A-C].
The initial arterial blood gas analysis without oxygen showed pH 7.38, pO2 94 mmHg, and pCO2 43 mmHg. Carboxyhemoglobine status was 6.4% and other blood results were normal.
The patient underwent VATS with bullectomy. Surgery was performed under general anaesthesia with double lumen endotracheal intubation and discontinuing ventilation on the left side in half lateral position. Two 12 mm trocars and one 5 mm trocar were used. The giant bulla was located at the apex of the lower lobe with total compression of the left upper lobe and pleural irritation [Figure
A]. Bullectomy was done with two 60 mm Endo-GIA (Gastro-Intestinal Anastomosis) linear endoscopic stapling devices (Covidien®, Norwalk, CT, USA) without extra suture reinforcement [Figure
B and C]. Subsequently an extensive stripping of the parietal pleura was carried out [Figure
D].
Anatomopathologic examination of the pleural wall of the giant bulla showed signs of chronic inflammatory infiltrates without any malignancy.
The lung expanded well and the pleural cavity was drained with two chest tubes (thoracic drainage systems Pleur-evac) connected to a water seal under 10 cm H20 suction. No air leak was noted, tubes were removed on second post-operative day.
Patient developed a bronchopneumonia of the right lower lobe, which was treated with intravenous antibiotics and quickly resolved. After five days the patient could leave the hospital in a good general condition.
Three months later the patient was free of complaints. Auscultation and spirometry were perfectly normal. No restrictive syndrome was observed with FEV1 of 78% (reversible), FVC of 82% and normal lung volumes.
Chest radiography showed good expansion of the left lung without pneumothorax or residual pleural effusion [Figure
]. The patient ceased tobacco consumption since surgery, supported by Vareniciline (Champix®, Pfizer, New York, USA). One year follow-up revealed no recurrence, no intercostal pain syndrome and preserved pulmonary function.


A 13-year-old Caucasian male presented to clinic with an insidious onset of nondominant, left-sided, mid-clavicular pain for six weeks of duration. The mother described the texture of this area as “sand-like.” There was no history of trauma, fever, chills, night sweats, weight loss, joint pain, or swelling. On physical examination, the left clavicle demonstrated an isolated, tender, firm, nonerythematous, nonfluctuating, soft tissue swelling directly over the mid-clavicle without a noticeable deformity. Skin changes were absent. Laboratory examination showed total blood count of 8.9 K/UL (4.5–13.5) with normal differential. Erythrocyte sedimentation rate (ESR) was 14 mm/hour (0–10) and C-reactive protein (CRP) was 1.3 mg/dL (<1.0).
Conventional radiograph of the left clavicle and a Tc-99m-MDP bone scan were performed. Subsequently CT scan and MRI were performed. An incisional biopsy of left mid-clavicle was performed in the operating room and the fragments of soft red-tan tissue were sent for histopathologic examination. It showed a histiocytic neoplasm admixed with eosinophils, confirming the diagnosis of Langerhans Cell Histiocytosis (LCH). Cultures were negative. The lesion was treated by curettage, bone grafting, and internal fixation with a 3.5 mm reconstruction plate due to the presence of pathologic fracture. At 2 years of followup, patient was asymptomatic and the lesion showed complete resolution without recurrence.


A previously healthy, 31-year-old Caucasian male presented with complaints of abdominal pain radiating to the flanks, dark colored urine, and fatigue for the last 2 months. On physical examination, patient was noted to be afebrile, and his blood pressure was 130/84 mmHg. He had pale conjunctivae. The lungs were clear on auscultation, and no focal neurological deficit was found. Examination of the extremities revealed no pedal edema. Laboratory analysis revealed a normal white blood cell count, hemoglobin of 8.5 gm/dL, serum blood urea nitrogen of 79 mg/dL, creatinine of 22.5 mg/dL, and total serum calcium of 11.2 mg/dL. LDH was elevated at 1178 U/L. Urinalysis was significant for 1+ proteinuria, no cast or crystals was visualized by urine microscopy. The spot urine protein/creatinine ratio was estimated at 2.4 grams per day. The computed tomography of abdomen and pelvis for evaluation of abdominal pain demonstrated diffuse lytic bone lesions, which prompted work-up for multiple myeloma. Serum protein electrophoresis did not show any M-spike. Urine immunoelectrophoresis revealed kappa light chains. Serum light chain assay revealed high kappa light chain at 931 mg/dL (normal 0.33–1.94 mg/dL). Lambda light chains were suppressed at 0.25 mg/dL (normal 0.57–2.63 mg/dL). Bone marrow biopsy showed 60%–70% of bone marrow area was replaced by plasma cells. A kidney biopsy revealed cast nephropathy with strong staining of kappa light chain in tubular basement membrane on immunofluorescence. No electron dense deposits were seen on electron microscopy.
The patient was initiated on hemodialysis with standard high flux membrane (not high cut off membrane dialyzer) and received his first of 6 cycles of chemotherapy (bortezomib, doxorubicin, and dexamethasone). After the second cycle of chemotherapy, patient's renal function improved significantly, and he did not require further dialysis (). He received a total 4 weeks of hemodialysis on a three times per week schedule. During his outpatient follow-up visits, the serum creatinine continued to improve, and by the last chemotherapy session, patient's serum creatinine was 1.4 mg/dL. Patient's most recent creatinine is less than 1 mg/dL.


A 72-year-old Japanese man had a 12-year history of ALS. He was bedridden and had been on mechanical ventilation and percutaneous endoscopic gastrostomy (PEG) tube feeding at home for the previous 10 years. He was fed 1000mL (1000kcal) of a commercial product (Ensure liquid®; Abbott Japan, Tokyo, Japan) containing <1nmol/mL of carnitine per day. He was also sometimes given homemade products such as miso soup and coffee with milk, all via the PEG tube. Over one year, his ability to communicate using facial expressions gradually deteriorated. He was still able to flex his left toe to control the communication device, but his energy appeared to diminish when attempting to focus on communication. He did not have muscle cramps, and his diabetes mellitus was well controlled with 25mg of alogliptin without renal insufficiency. His medical history included a colonic polyp, which was resected via endoscopy four years previously, and pneumothorax, which was treated conservatively one year previously. He had no history of rhabdomyolysis.
Laboratory evaluations performed to determine the cause of his altered mental state showed that his electrolytes, hematological parameters, including HbA1c, and liver and renal function parameters were within normal ranges (Table ). However, his serum total carnitine was abnormally low, at 26.7μmol/L (normal range, 45 to 91μmol/L), as were his free carnitine (20.6μmol/L; normal range, 36 to 74μmol/L) and acylcarnitine ester (6.1μmol/L; normal range, 6 to 23μmol/L) levels. However, his ammonium level was normal (48μg/dL; normal range, 12 to 68μg/dL).
Within two weeks of starting carnitine supplementation (600mg twice daily via the PEG tube), our patient’s mental state improved to the level noted the previous year. His serum total carnitine recovered to 172.3μmol/L (normal range, 45 to 91μmol/L) one year after starting carnitine supplementation. His free carnitine (140.6μmol/L; normal range, 36 to 74μmol/L) and acylcarnitine ester (31.7μmol/L; normal range, 6 to 23μmol/L) levels had also recovered at this time. Our patient’s mental state has been maintained since starting carnitine supplementation.


A 74-year-old right-handed Asian woman was referred to our hospital with complaints of limited motion of her right little and ring fingers. Our patient had been experiencing an inability to extend her right little finger for two months, and subsequently, her ring finger for two weeks. Our patient denied any associated pain and warning symptoms such as tendon irritation.
On physical examination, our patient was unable to actively extend her right little and ring fingers at the metacarpophalangeal joint (Figure ). A palpable mass with swelling at the wrist dorsum and severe instability of the DRUJ on the load and shift test was observed. Laboratory examinations revealed that her blood cell count, erythrocyte sedimentation rate, and C-reactive protein were within normal range. Her rheumatoid factor was also negative. Plain radiographs showed osteoarthritic change at the DRUJ. Deepening and widening of the sigmoid notch and radial shift of the ulnar head were also revealed (Figure ).
Intraoperatively, extensor digiti minimi (EDM), along with the fourth and fifth extensor digitorum communis (EDC) tendons, were ruptured with fraying of the tissue in the ruptured margin. Their distal ends were matted together in the scar and the fraying tissue and their proximal ends lay just proximal to the DRUJ. The ulnar head was directly exposed and the dorsally overlaying capsule was worn out (Figure ). The ulnar head had arthritic change, but a smoothened surface without osteophyte formation. Although mild synovitis was present, there was no macroscopic evidence of rheumatoid arthritis.
Because end-to-end repair was impossible, the extensor indicis proprius tendon was transferred to the EDM tendon, and the distal stumps of the fourth and fifth EDC tendon were sutured to the intact EDC tendon, in the end-to-side fashion. The ulnar head was resected. At 15 months after surgery, she can extend her ring and little fingers without difficulty for daily activities. She denied any pain around her wrist area.


Having made a bet with his friend on whether yew or juniper is more poisonous, a 39-year-old Caucasian healthy man consumed a decoction from needles of the common yew (Taxus baccata) at approximately midnight. Gradually, weakness, nausea and vomiting began. Subsequently, clonic spasms developed in his extremities, followed by heart failure and blood circulation arrest. Urgent resuscitation, assisted via telephone, was started according to the instructions given by the dispatch center and lasted for five minutes. The emergency call was received at 7.30 a.m., and an ambulance arrived at 7.35 a.m. He arrived in the emergency department at 8.15 a.m., where bradyarrhythmia of 25 to 30 beats/min shifted to ventricular tachycardia (fibrillation) with repeated defibrillation. Indirect heart massage by means of an AutoPulse® (Zoll Medical Corporation, Chelmsford, MA, USA) device and extended cardiopulmonary resuscitation (CPR) were performed the entire time. Blood circulation was supported with noradrenaline 0.1mg/min intravenously. Adrenaline was repeatedly administered at a total dose of 6mg intravenously, plus sodium bicarbonate 300ml 8.4%, calcium gluconicum 10ml, calcium resonium 6 in graduated measures into a nasogastric tube, amiodarone 450mg intravenously and potassium and magnesium hydrogen aspartate 20ml intravenously. External and internal stimulation of the heart was ineffective. Taking into account the known cause of the blood circulation arrest and the probable absence of a delay in CPR, the introduction of venoarterial extracorporeal membrane oxygenation (VA-ECMO) was indicated as a rescue support therapy until the blood circulation was stabilized. DigiFab® (digoxin-reactive Fab protein, Protherics Inc., Brentwood, TN, USA) 120mg intravenously as an antidote was administered. Gradually, his blood circulation stabilized, and his heart rate was restored. Concurrently, clinical and laboratory signs of adult respiratory distress syndrome were observed. Corticotherapy with methylprednisolone 125mg intravenously once daily was started. VA-ECMO was successfully disconnected the next day; his oxygenation improved, and his Glasgow Coma Scale score was 3. An attempt to discontinue the use of sedation led to the development of generalized spastic activity. Our patient was evaluated for serious post-hypoxic encephalopathy with generalized myoclonic seizures. After a protracted, intensive rehabilitation period and symptomatic therapy, our patient was bedridden and breathing spontaneously, with minimal cortical reactivity and a persisting serious neurological deficit. It is not clear if our patient’s improvement was due to the therapy with DigiFab® or the result of supportive therapy. A native computed tomography (CT) scan of the brain showed slight hypodensity in the basal ganglia and post-ischemic changes after prolonged hypoxemia (Figure ).


A 31-year-old, ASA II, superobese male patient (190 kg, 180 cm, BMI: 58 kg/m2) was admitted to the emergency department with a segmental Gustilo-Anderson type IIIA open tibial fracture and ankle dislocation following an in-vehicle traffic accident. The initial proper management of open fracture and joint dislocation was performed by orthopaedic and traumatology surgeons in the emergency department. The patient had no history of additional diseases. The preoperative airway examination revealed a class 3 Mallampati airway and, in light of the difficulty of intubation, an intrathecal block with a 150 mm needle was planned. After the written consent was obtained, 2 mg IV midazolam and 50 μg fentanyl were administered for sedation. The vertebral anatomical structures were barely distinguishable despite ultrasound guidance. After two unsuccessful attempts, we decided not to use spinal anesthesia and planned to perform a combined sciatic and femoral nerve block. As a tourniquet was not required above the knee, the blockade of these two nerves would be adequate for surgical anesthesia.
Before the femoral nerve block, the pannus was taped cephalad as the fatty tissue hanging from the abdomen made it difficult to access the inguinal region. The femoral nerve was identified at a depth of 5 cm with a linear US probe (Esaote, 10–18 MHz, Florence, Italy). Fifteen mL (8 mL of 0.5% levobupivacaine and 7 mL of 2% lidocaine) of local anesthetic mixture was administered around the femoral nerve using the in-plane approach. Due to the difficulties in situating the patient in the prone position, the distal extremities of the patient were elevated with folded blankets in the supine position (). The sciatic nerve could not be visualized despite several attempts using both linear and convex probes; therefore separate blocks of the tibial and common peroneal nerves were planned. The tibial nerve was identified in the popliteal fold, posterior to the popliteal artery at a depth of 3 cm (). Visualization of the tibial nerve was highly difficult in areas more proximal than the point 2 cm above the popliteal fossa. The common peroneal nerve (CPN) and the bifurcation of the sciatic nerve could not be visualized despite several attempts using both linear and convex probes. Therefore, 10 mL (7 mL of 0.5% levobupivacaine and 3 mL of 2% lidocaine) of local anesthetic mixture was administered around the tibial nerve 2 cm above the popliteal fold by using a linear probe. Screening was performed in the distal direction over the lateral side of the patient's leg with the linear probe placed transversely on the edge of the patella to identify the CPN (). The nerve was visualized posteriorly and laterally to the fibular head, 4 cm distally to the edge of the patella at a depth of 2 cm (). At this level, 10 mL (7 mL of 0.5% levobupivacaine and 3 mL of 2% lidocaine) of local anesthetic mixture was administered around the nerve with the in-plane technique using a linear probe. A 100 mm 21-G echogenic needle (Pajunk, Geisingen, Germany) was used for all blocks. A nerve stimulator was not used for any blocks since the patient had extremity pain associated with trauma. Surgical anesthesia was established within 30 minutes after anesthetic administration and no complications occurred in association with the blocks. Emergent reduction and fixation with an external fixator were performed for the ankle dislocation and fracture of the tibia. Additional local anesthetic injection or additional sedation was not required during the operation. The operation lasted for approximately 1.5 hours and was completed without problems. The patient's consent was obtained for the publication of this case report.


A 71-year-old female was admitted under the surgical service for elective chemoport placement. She was known diabetic and hypertensive and well controlled on medications. She was also diagnosed with stage II gastric cancer. She underwent distal gastrectomy and Bilroth II reconstruction several months ago and was scheduled for chemotherapy as part of her management.
Just prior to the procedure the patient developed symptomatic bradycardia manifested as near syncope with her heart rate dropping to 40 beats per minute. Consequently, the procedure was cancelled and the patient was admitted to the medical service for further workup. Cardiology was consulted and she was started on Plavix in addition to the Lovenox she was having for DVT prophylaxis. Workup including transthoracic echocardiography (TTE) was within normal and she was cleared for the procedure. Later Plavix was stopped at the time of the procedure.
The patient was taken to operating room the following day for the placement of a chemoport. Her chest X-ray () was normal and she was asymptomatic. General anesthesia was induced with no complications. The patient was draped and positioned in Trendelenberg. Ultrasound guided insertion of a right internal jugular venous (IJV) access was attempted with a total of 4 trials using Seldinger's technique. Resistance was met while threading the guide wire. Ultrasound examination showed a chronic clot burden in the Right IJV. The vascular surgery team was consulted intra operatively. A right IJV access using a microset access under fluoroscopic guidance was obtained. The procedure was then completed without any further issues. Following extubation, and upon transfer of the patient to the stretcher, she started complaining of right-sided chest pain radiating to the back. She became tachycardic with a heart rate ranging between 105 and 110 beats per minute. CXR was immediately obtained and it revealed a contained white out in the right upper lung field ().
Soon after, the patient decompensated with the systolic blood pressure dropping to 60 mm Hg. Volume resuscitation with crystalloids was started and the patient was cross-matched for transfusion of blood products. A third CXR was obtained during this time revealing the progression of a hemothorax in the right side of the chest ().
A median sternotomy was performed. Upon exploration of the thorax, a posterior mediastinal hematoma tracking into right pleural cavity was detected. There was active bleeding from the puncture site at IJV-subclavian (SCV) junction. By the end of the surgery, the patient received a total of four units of (PRBC), two units of fresh frozen plasma (FFP), and one unit of platelets. The patient was transferred to the surgical intensive care unit (SICU) postoperatively intubated and hemodynamically stable. A right pleural chest tube was kept in place for drainage. Postoperative CXR was done showing marked improvement of the right-sided hemothorax (). The next morning, the patient was extubated and the mediastinal chest tube was removed. The patient had an unremarkable recovery course and was transferred out of the SICU after removal of the right pleural chest tube.


A 23-year-old male with prior multiple gunshot wounds to the abdomen requiring complete small bowel resection and chronic TPN support via a Hickman catheter since the age of 17 presented with anasarca and low grade fevers in June 1996. Basic urine evaluations revealed 2+ blood without evidence of casts and 2.0 g proteinuria from a 24-hour collection. A serum chemistry panel revealed creatinine of 1.9 mg/dL (estimated glomerular filtration rate of 50 mL/min/1.73 m2), blood urea nitrogen (BUN) of 37 mg/dL, and albumin of 2 gm/dL. His baseline creatinine levels were unknown. Routine serology evaluation including human immunodeficiency virus (HIV), rapid plasma reagin (RPR), antinuclear antibody (ANA), and hepatitis B and C screen were all negative. Complement studies revealed C3 of 68 mg/dL (reference range, 90–180 mg/dL), fourth component of complement (C4) of 19 mg/dL (reference range 16–47 mg/dL), and total complement levels (CH50) of <28 mg/dL (reference range 60–90 mg/dL). Echocardiogram showed no vegetations. Blood cultures were positive for coagulase negative staphylococcus. Kidney ultrasound revealed right kidney measuring 10.6 cm and left kidney measuring 10.8 cm without structural abnormalities or evidence of obstruction. A kidney biopsy was performed.
Light microscopy revealed 15 glomeruli showing a lobular pattern with mesangial hypercellularity, a moderate number of capillary wall double contours, and leukocytes within capillary lumina. Three glomeruli had segmental crescents. There were interstitial inflammation and edema, associated with acute tubular cell injury. Immunofluorescence microscopy disclosed five glomeruli staining for IgM (3+), C3 (3+), and kappa (trace to 1+) and lambda (trace) light chains along capillary walls in a granular pattern and peripheral distribution. Mesangial regions were stained for IgM (1 to 2+), C1q (trace), C3 (1+), and kappa (trace) and lambda (trace) light chains in a granular pattern. Electron microscopy of three glomeruli revealed small subendothelial and few mesangial electron dense deposits. There were no tubuloreticular structures in the cytoplasm of any cells ().
Diagnoses of membranoproliferative glomerulonephritis type I and acute tubulointerstitial nephritis were rendered.
The patient received vancomycin and underwent Hickman catheter replacement with subsequent rapid and complete resolution of his acute kidney injury (serum creatinine improved to 1.3 mg/dL), proteinuria, and anasarca. Due to the temporal association of treatment and renal disease resolution, the MPGN was presumed to be secondary to staphylococcal bacteremia.
He was lost to follow-up for several years until February 2010 when he presented with upper extremity edema and chills. On admission he had anemia, reduced kidney function, and hypoalbuminemia. Again, he was found to be infected with coagulase negative staphylococcus bacteremia (S. epidermidis). His spot urine protein to creatinine ratio at presentation was 2.4 g/g creatinine but increased to 5.8 g/g over several days without associated blood pressure changes. Routine laboratory investigations revealed creatinine of 2.2 mg/dL, BUN 19 mg/dL, WBC 4,400/mm3, and hemoglobin 5.9 g/dL. Urinalysis revealed 300 mg/dL protein, large blood, large leukocyte esterase, 196 WBC/high power field (HPF), 224 RBC/HPF, 33 hyaline casts, few WBC clumps, 14 granular casts, and 24 cellular casts. He was treated with vancomycin pending repeat evaluation of the underlying nephrotic syndrome. Of interest, the patient commented that “every time I swell up, they give me antibiotics and the swelling goes away.” Further evaluation of his renal disease was again pursued as all his previous medical records were lost in a hospital fire. Serum protein electrophoresis (SPEP), urine protein electrophoresis (UPEP), serum protein immunofixation (SPIF), urine protein immunofixation (UPIF), antineutrophil cytoplasmic antibody (ANCA), RPR, ANA, and HIV were all negative. C3 was low at 71 mg/dL with normal C4 of 23 mg/dL. A kidney ultrasound revealed normal sized kidneys (right 11.0 cm and left 11.7 cm) without structural abnormalities. Evaluation for subacute bacterial endocarditis was negative. His Hickman catheter was replaced and subsequent blood cultures confirmed resolution of his bacteremia. His proteinuria improved markedly (greater than 50% reduction) within a few days of antibiotic therapy initiation. Serial creatinine measurements documented improvement in his creatinine to 1.75 mg/dL within 20 days of presentation. A repeat kidney biopsy performed in June 2010 confirmed MPGN type I (), with acute tubulointerstitial nephritis and mild-to-moderate chronic renal parenchymal injury.
In July 2010, he presented for the third time with anasarca, fevers, and acute kidney injury (creatinine of 3.29 mg/dL, elevated from baseline level 1.7–2.0 mg/dL). Initial urinalysis showed 100 mg/dL protein, large blood, 215 WBC/HPF, 252 RBC/HPF, and 42 hyaline casts. Complement levels revealed C3 of 45 mg/dL, C4 of 18 mg/dL, and CH50 < 13 mg/dL. Blood cultures revealed coagulase negative staphylococcus bacteremia (S. epidermidis) and spot urine protein-creatinine ratios rapidly rose from 5.1 g/g to a peak of 9.5 g/g within 2 days without accompanying blood pressure changes. Treatment with vancomycin and Hickman line replacement led to rapid reduction in proteinuria and anasarca with creatinine improving to his recent baseline of 1.7 mg/dL by October 2010. As the two previous kidney biopsies demonstrated MPGN type I, this third episode of acute kidney injury accompanied by hematuria and nephrotic range proteinuria was attributed to a recurrence of MPGN secondary to recurrent Hickman catheter infection.


A 39-year-old woman, diagnosed as RA in 1997, had been treated with MTX, bucillamine, and prednisolone, but unfortunately was resistant to these medications. In May 2005, her disease activity of RA elevated as high as Disease Activity Score including a 28-joint count (DAS28 [])-erythrocyte sedimentation rate (ESR) of 5.60. Moreover, the progression of joint destruction and functional status was recognized as stage III and class III by Steinbrocker's classification [, ], respectively. Then IFX, a biologic agent inhibiting tumor necrosis factor (TNF)-α, was introduced with a dose of 3 mg/kg. After three times of IFX administration at 0, 2, and 6 weeks, DAS28-ESR improved significantly to 3.96.
Ten days after the third infusion of IFX, she felt sore throat, dysphagia, and left otalgia, with development of vesicles on the left pinna. Then three days after these initial symptoms, she felt motor paralysis on the left side of the face. She visited otolaryngologist in a private clinic who immediately consulted the otolaryngology department in our hospital at the same day; thereafter, she was diagnosed with Ramsay Hunt syndrome. Physical examination presented multiple vesicles on the left pinna, as well as on the left side of soft palate, tongue, and epiglottic vallecula (Figures and ). Facial nerve score was 4 points with Yanagihara' grading scale ([]; total, 40 points). Neurootological examination presented sensorineural hearing loss (36.3 dB; normal value, ≤30 dB), nystagmus, and diminished lacrimation (Schirmer test, 7 mm; normal value, ≥10 mm) on the left side, with the disappearance of the left stapedial reflex and the reduced sense of taste (electrogustometry, 32 dB; normal value, ≤8 dB). Since such symptoms as sternocleidomastoid muscle atrophy, trapezius muscle paralysis, neck stiffness, and Kernig's sign were not recognized, either accessory nerve paralysis or meningitis was not suggested. Serological study by enzyme immunoassay (EIA) for specific antibodies against VZV presented IgM 0.45 (normal value, <0.80 antibody index) and IgG 29.3 (normal value, <2.0 EIA titer). At 12 days after the onset, increased levels of VZV antibodies were recognized: IgM 2.02 and IgG 83.9 ().
Treatment was started immediately after her visit at our hospital with valaciclovir 3000 mg for 7 days and hydrocortisone sodium succinate for 9 days (500 mg for 3 days, 300 mg for 3 days, and 100 mg for 3 days), while all medications for RA, IFX, MTX, bucillamine, and prednisolone were discontinued. At 6 days after the onset, left recurrent laryngeal nerve paralysis and left glossopharyngeal nerve paralysis, the symptom of which is characterized as “curtain sign”, appeared. At 7 days after the onset, electroneurography (ENoG) and nerve excitability test (NET) were performed for prognostic diagnosis [], and ENoG was 24.5% (excellent, ENoG ≥ 40%; good, 20% ≤ ENoG < 40%; fair, 10% ≤ ENoG < 20%; poor, ENoG < 10%), while NET was 4.2 mA (good, NET ≤ 3.5 mA; fair, 3.5 mA < NET ≤ 20 mA; poor, NET > 20 mA), both indicating unfavorable prognosis of paralysis. Then another course of hydrocortisone sodium succinate with the same dose for 9 days was conducted (). After these treatments were introduced, facial nerve paralysis, as graded by Yanagihara's scale, improved successfully to 36 points at 3 months after the onset, and finally to full marks at 6 months (). Other cranial nerve symptoms recovered at 2 months after the onset. However, facial synkinesis appeared at 3 months after the onset and remained even at 6 months, though it improved gradually to low grade. As for serological study, levels of VZV antibodies demonstrated a tendency to decrease after the treatment was introduced, and IgM level declined to normal value, 0.55, at 2 months after the onset whereas IgG level still remained high, 47.6 ().
With regard to treatment of RA, prednisolone 10 mg/day and bucillamine 300 mg/day were readministered at 26 days after the onset of Ramsay Hunt syndrome. Then MTX 4 mg/week was readministered at 5 months after the onset and was increased to 8 mg/week at 6 months. Disease activity of RA elevated as high as DAS28-ESR of 4.98 at 3 months after the onset but finally improved to DAS28-ESR of 2.68 after readministration of medications, especially of MTX. Recurrence of Ramsay Hunt syndrome was not recognized, and the patient transferred to another hospital for her own reasons at 9 months after the onset ().


The patient was a 4-week-old male infant born at an estimated gestational age of 30 weeks with a birth weight of 1665 grams and APGAR scores of 8 and 9 at one and five minutes, respectively. Gross congenital anomalies were not identified. The mother was a gravida 3, para 2, abortion 1, 21-year-old African American with an adequate prenatal care. Her pregnancy was complicated by group b streptococcus urinary tract infection and placental abruption requiring pregnancy termination. In addition, there was a history of hand-foot-mouth syndrome in his sibling two weeks prior to delivery. Immediately after birth, the neonate developed respiratory distress and cyanosis requiring intubation and admission to the neonatal intensive care unit. The initial physical exam revealed a grade III-IV holosystolic murmur and generalized edema. The initial X-rays and echocardiographs demonstrated a moderate to large patent ductus arteriosus (PDA), moderate to severe pulmonary hypertension, and biventricular changes suggestive of hypertrophic cardiomyopathy. Bedside PDA ligation was performed. Subsequent studies revealed Coxsackie B viremia (confirmed by polymerase chain reaction (PCR)); however, TORCH serology were all negative. Viral induced hypertrophic cardiomyopathy was the presumptive diagnosis. However, daily chest X-rays demonstrated progressive nonspecific calcification of the chest soft tissue and the vessels of both arms (). Despite multiple intravenous immunoglobulin treatments, loop diuretics, phosphodiesterase 3 inhibitor, and dopamine for the presumed viral induced cardiomyopathy, his symptoms progressed and the patient expired at 4 weeks of age secondary to intraventricular brain hemorrhage and multiorgan failure. At autopsy, there was severe anasarca, but gross congenital anomalies were not identified. Cardiovascular dissection revealed increased biventricular wall thickness () with evidence of gross arterial calcification along the large vessels. Microscopic sections of the heart demonstrated cardiac myocyte nuclei enlargement consistent with hypertrophy and focal calcium deposition and fibrosis within the myocardium. However, inflammation suggestive of myocarditis could not be identified (Figures and ). The coronary arteries demonstrated focal calcification of the internal elastic lamina (), while sections from the ascending and descending aorta exhibited extensive calcification with disruption of the elastic lamina and intimal fibrosis (). Other organs including lungs, brain, pancreas, kidneys, skin, muscles, and testicles also displayed extensive calcium deposition involving the internal elastic lamina with ingrowth of loose fibromyxoid tissue resulting in luminal narrowing and obstruction. The renal hilar vessels were extensively involved (). The gross and microscopic features were consistent with the diagnosis of IIAC. In this case, Coxsackie B viremia and a history of hand-foot-mouth syndrome in the sibling as the etiology of the cardiopulmonary failure were a red herring. However, the single nucleotide polymorphism microarray analysis indicated no clinically significant abnormality and was consistent with a normal male chromosome complement, but the gene sequencing to evaluate the presence or absence of ENPP1 gene mutation was not performed. This case report aims to emphasize the importance of taking into consideration this rare but curable disease in infants presenting with refractory cardiopulmonary failure.


In May 2009, a 5.5 years old girl was refereed to the child psychiatric clinic for further assessments. She was born in Afghanistan and until the time of gathering data she was living in Iran. She had two siblings and she was the youngest one. They had no physical and/or mental abnormalities. Their parents were consanguineous and they had no abnormalities either. The patient’s uncle was diagnosed with BD several years ago. The socio economic level of the family was low. The patient didn't have any past psychiatric or medical problems. Her symptoms started about 6 months ago by some rapid cycles of mood swings and she showed marked mood liability, distractibility and rages and explosive temper tantrums (lasting up to several hours). At each episode she was starting to laugh most of the time even whole night for about two weeks. She didn't show talkativeness and impairment in perception or thought. After passing this phase, she was crying with the same pattern for another two weeks. Her behavior was clingy and her appetite has decreased during the depressive phases. Meanwhile she has started to scare while sleeping and also using the bathroom or toilet, this situation got severe since 2 months ago. Then after a while she also scared from other people and children; she wanted to be hugged and being protected by her parents all the times. Since then she always repeated the sentence “I want to be clean”, but she showed no compulsive symptoms. Based on these symptoms she had a separation anxiety problem but the parents didn't seek any help for her problem. Her attention span had decreased. She was also very aggressive and had marked irritability and she bit her nails. She had distractibility, hyperactivity and labile emotion and also restlessness and fidgetiness. Her sleep was decreased as well. Her social interaction was poor. She had no oppositional behavior, racing thoughts or grandiosity. She also had no difficulty getting up in the morning, bed wetting or night terrors. There were no signs or symptoms of compulsive behavior, tics, paranoia, hallucinations and delusions in her. The parents didn't mention any cruelty to animals in her. She didn't have any suicidal ideation. The child's developmental history was normal but her emotional development was impaired. About 6 months ago she had visited by a general psychiatrist and risperidon was prescribed for her but she refused to get it. There is no report about the diagnosis on that time. At our clinic, a child psychiatrist and a general psychiatrist visited her. She had a typical full-blown BD that rarely is seen in the children. The diagnosis was based on DSM-IV-TR criteria for BD. There were no comorbidities. Based on our diagnosis, we strongly suggested treating her at in-patient psychiatric setting, but her family refused to admit her at the hospital.
During our observation she had severe mood labiality, behavioral disturbances and decreased need to sleep. She didn't accept to eat tablet, therefore the only mood stabilizer that is accessible in syrup form (valprovate 200mg/day) was prescribed. Her biochemistry and thyroid function tests were normal. No impairment was reported in EEG and brain imaging. In June 2009, we followed her up by speaking to her mother via telephone. The patient had a very poor compliance and didn't take her medication so she didn't have any changes in her behavioral problems but her mood labiality got milder without any medication. In fact it is accounted as a form of child neglect which parents ignore the right of child to receive the treatment.


The patient was a 21-year-old African-American female who presented with a single nodule on the flank area. Grossly, the subcutaneous nodule was red-tan, oval, and well-demarcated, measuring approximately 0.5 cm in diameter. Microscopically, the mass was composed of spindle cells arranged in a swirling and intersecting pattern inside the lumens of two blood vessels (Figure A). It extended through the vascular walls into the surrounding fibroadipose tissue; in some sections, the spindle cells were intermixed with the perivascular fibrous tissue (Figure B). Elastin stain revealed remnants of elastic lamina partially surrounding the lesion (Figure C). The nuclei of the spindle cells were relatively uniform with tapered ends and prominent nucleoli (Figure D). No significant mitotic activity was observed. Multinucleated giant cells were scattered among the spindle cells (Figure E), along with infiltrating lymphocytes and extravasated red blood cells (Figure F). Immunohistochemical stains showed the spindle cells were positive for smooth muscle actin (SMA; Figure G), focally positive for muscle specific actin (Figure H), and negative for S-100 (Figure I), confirming their myofibroblastic differentiation. The overall morphological and immunohistochemical features are consistent with intravascular fasciitis.


A 31-yr-old female with pregnancy-induced hypertension (PIH) underwent caesarean section (CS) at 35 weeks of pregnancy (due to fetal growth retardation). Spinal anesthesia was used for the procedure. On the second day post-partum, she noticed diffuse occipital headache and neck pain when she tried to get off the bed, which improved on lying down. This postural headache was persistent over next 8 days following which it changed character and became continuous. A diagnosis of PL headache was made and she was discharged on Ibuprofen 400mg thrice daily. On 12
th post-partum day she had a generalized tonic clonic seizure and had persistent drowsiness. She was taken to local hospital for seizure; there she received lorazepam 4mg slow IV bolus followed by phenytoin 900mg IV infusion over 60 minutes. After stabilization of vitals she was transferred to our comprehensive stroke care center. Prior to this episode she had been detected to have PIH in two earlier pregnancies and the first pregnancy had resulted in intrauterine death. During the present pregnancy she received dalteparin 5000 IU/day subcutaneous injection from 6 weeks pregnancy from local maternity centre with suspicion of anti-phospholipid syndrome; it was continued until the 8
th post-partum day. There was no past history of abortions or deep vein thrombosis.
On examination in the emergency room after 12 hours of seizure she was conscious and oriented. She had no papilledema and her visual acuity and visual field were normal. She had weakness and appendicular ataxia of the left upper limb and mild dysarthria. Her National Institute of Health Stroke Scale on admission was 3.
She was investigated with a CT scan of the head (
) after 8 hours of ictus at a local hospital; this showed a right high parietal hemorrhagic infarct. MR imaging of the brain (
) and spine (
) (sequences: T1 Weighted, T2 Weighted, fluid attenuated inversion recovery (FLAIR), susceptibility weighted imaging (SWI), diffusion weighted imaging (DWI), apparent diffusion coefficient (ADC) and MR venography (MRV) was done after 14 hours of seizure ictus. This showed a right posterior high parietal lobe hematoma with mass effect and right side superficial cortical veins and partial sagittal sinus thrombosis. In addition there was evidence of intracranial hypotension with CSF leakage at the lumbar puncture site, as evidenced by corpus callosum sagging, pachymeningeal enhancement, and diffuse prominence of the cortical vein and rim of CSF seen in the epidural space from the D10 to the L3 vertebra. Her routine blood investigations, including a haemogram, liver function test and renal function test, were within normal limits. Erythrocyte sedimentation rate was elevated with 40 mm/hour. Her coagulation parameters including prothrombin time and antithromboplastin time were normal. Prothrombotic (etiological) work-up sent prior to starting of heparin therapy revealed reduced Protein C and Protein S activity of 31% (normal range 67–195%, plasma-clotting time based assay) and 26% (normal range 55–123%, plasma-clotting time based assay), respectively. Antithrombin III antigen level was normal at 209 mg/l (normal range: 170–300 mg/l, chromogenic assay). Factor V Leiden mutation (real time PCR method) was not detected. The vasculitic work up (antineutrophil antibody, ANA, double stranded DNA, dsDNA, antiphospholipid antibody, APLA, IgG and IgM and antineutrophil cytoplasmic antibody, ANCA) was negative.
Unfractionated heparin was started (16 hours after time of onset) at 800 units/hour to maintain plasma thromboplastin time between 75–90 seconds along with bed rest and caffeine immediately after the MRI venography. She responded early and was discharged after 6 days on warfarin 4mg once daily and optimized international normalized ratio (INR) of 2.1. On discharge she did not have any headache and neurological deficit had subsided, with NIHSS of 0 and modified Rankin Scale (mRS) of 0. On follow-up after 3 months, she had no further recurrences or new symptoms.


A 42-year-old Indian female was admitted with clinical picture of acute pyelonephritis. She had a 7 day history of fever, left loin pain and vomiting. She had no previous significant history. Physical examination revealed a sick patient with high fever and tender left loin. Basic investigations on admission reported high blood sugar (36 mmol/L) with HbA1c of 16.7%. Urine routine and microscopy showed excess WBC but negative results for ketones. She had acute renal failure (s.urea: 11.9 mmol/L, Creatinine 243 umol/L). Arterial blood gas revealed normal pH with normal oxygen saturation. CBC (complete blood count) showed leukocytosis (17 × 109/L) with dominant polymorphs, normal hemoglobin, and thrombocytopenia (92 × 109/L).
She had slightly impaired liver function tests with normal bilirubin, high alkaline phosphatase of 331 IU/L, and normal coagulation profile. Chest X-ray showed minimal left pleural effusion. Plain KUB revealed no significant abnormality. Urgent ultrasound reported normal right kidney but left kidney was not visualized because of a lot of gases.
She was diagnosed as acute pyelonephritis with sepsis and uncontrolled hyperglycemia. She was started empirically on cefotaxime and amikacin injection, placed on intravenous fluids and insulin infusion pump. After 24 hours blood and urine cultures reported growth of resistant E coli (ESBL positive). Antibiotics were changed to meropenem injection.
Despite full supportive measures, she continued to be ill, highly febrile, with further deterioration of renal functions, and persistent leukocytosis with thrombocytopenia.
Plain CT-abdomen was performed after 72 hours. It reported mild left pleural effusion with enlarged left kidney heterogeneous with multiple areas of air density and thickened Gerotas fascia with perinephric fat stranding. A picture of left–sided emphysematous nephritis. The right kidney had normal findings ( and ).
Based on the clinical picture and the radiological findings the urology team was involved and she was scheduled for urgent nephrectomy which was performed after 96 hours of admission. She had severely edematous perinephric space. The removed kidney was gangrenous, and a drain was left which was removed after 12 days. The histopathology of the removed kidney showed features of acute pyelonephritis with extensive patchy areas of suppurative inflammation and necrosis (), also glomeruli showed diffuse increase in mesangial matrix with thickened capillary walls, and a nodule (Kimmelstiel-Wilson lesion) of diabetic glomerulosclerosis ().
She had an uneventful recovery, required 5 days in ICU, recovering gradually. She received meropenem for 21 days. Renal and liver function tests and CBC normalized. Blood sugar became normal with small dose of metformin. She was discharged with an excellent prognosis.


A 60-year-old woman presented to our hospital with a four- month history of increasing pain in the right knee. No difference was noted in pain character with motion and at rest. Pain was localized in medial tibial metaphysis. Local tenderness and painful arch of knee motion were observed without any tumoral tissue clinical inflammatory findings. She had no history of fatigue, cough, weight loss, diabetes, night sweats or of a malignancy. The blood count and CRP were within normal range. Sedimentation rate was elevated. A plain radiogram revealed a lytic lesion in proximal metaphysic of tibia. Computed tomography showed a lytic and exocentric lesion starting intramedullary and destructing the cortex with soft tissue extension, whereas MR delineated a 3x2 cm hypointense mass in T1 sequences and heterogenic hyperintense lytic mass with contrast enhancement in STIR sequences (). Radiographically, the preliminary diagnosis was chondrosarcoma. The patient was referred to nuclear medicine department for confirmation of the preliminary diagnosis as well as for the investigation whether metastasis was present.
FDG-PET imaging with I.V. administration of 370 MBq FDG was performed following six hours of fasting while the patient had a serum glucose level of 92 mg/dl. We waited one hour for distribution of FDG in the body then the patient was imaged using PHILIPS Gemini Dual GS (Philips medical Systems, Cleveland UK) integrated PET/CT scanner with 2 slice CT. Whole body PET/CT showed increased FDG uptake at the left side of the anterior arch of the first cervical vertebra and at the interarticular region and at the proximal metaphysis of right tibia (). Also in mediastinal slices, hypermetabolic conglomerated nodules measuring 3.5x3.0x4.0 cm were observed at right upper-lower paratracheal lymph nodes (). The maximum standard uptake values (SUVmax) for vertebra, tibia and mediastinal lymph nodes were 8.4, 14.4 and 12.0, respectively. Neither FDG uptake nor fibrotic lesion was found in parenchyma of both the lungs. Normal physiological distribution of FDG was observed at the rest of the body. FDG-PET/CT results were initially evaluated as malignant lymphadenopathies at right lower paratracheal lymph nodes in the mediastinum, because there was increased FDG uptake in both lytic lesions located at the first cervical vertebra and proximal tibia.
Based on the results of imaging studies, oncology and chest diseases consultations were requestedt. The clinical manifestations, physical examination and blood tests were not in line with the imaging results. The patient underwent thoracic lymph node biopsy with mediastinoscopy and percutaneous bone biopsy from tibia under fluoroscopy. The pathologic examination confirmed the diagnosis of bone tuberculosis and lymph node involvement. The patient received chemotherapy with ethambutol, rifampicin and isoniacid for 6 months and recovered totally without needing any surgery.


An eleven-year-old male child presented with complaints of headache for the past 6 months. Patient also had recurrent vomiting of 1 month duration. He also complained of numbness over the right side of face. On examination, patient was conscious and oriented. Sensory loss was present over right V2, V3 distribution of trigeminal nerve. His fundus examination revealed papilloedema. Patient did not have any motor deficits.
Noncontrast CT scan head revealed an isodense right middle cranial fossa lesion. On MRI scan, the lesion was hypointense on T1 weighted images and hyperintense on T2 weighted images. The lesion was homogenously enhancing. It was extra-axial in location, involved right middle cranial fossa and extended extracranially into the infratemporal fossa. The lesion was creating a significant mass effect with midline shift (). Routine haematological investigations and blood chemistry were within normal limits.
Patient underwent right frontotemporal craniotomy with zygomatic osteotomy. Intraoperatively, the tumor was present extradurally in right middle cranial fossa which was also extending through the dura into intradural compartment in temporal region as well as the posterior fossa into cerebellopontine angle cistern. The tumor was destroying temporal base and was extending extracranially into subtemporal region. The tumor was highly vascular, firm with good plane of cleavage with brain. The tumor was stuck to the lateral wall of cavernous sinus from which it was separable. The tumor extending into the posterior fossa was excised by drilling the petrous apex. Complete excision of intracranial part of tumor was done. As there was significant bleeding during the surgery, complete excision of infratemporal extension of the tumor was not attempted and only partial decompression was done. The dural defect at middle fossa floor was repaired with temporalis fascia. Postoperative course was uneventful.
Tumor tissue in 10% neutral buffered formalin was routinely processed and paraffin embedded. Five microns sections were cut for routine haematoxylin and eosin staining and immunohistochemistry. Immunohistochemical studies were performed using antibodies directed against vimentin (Novocastra, dil, 1 : 100), S-100 (Dako Denmark, dil, 1 : 1500), myogenin (Novocastra, dil 1 : 100), desmin (Dako, dil 1 : 100), and MIC-2 (Novocastra, dil 1 : 200). Labelled streptavidin biotin kit (Universal) was used as a detection system (Dako, Denmark). Antigen retrieval, when required, was performed in a microwave oven. The MIB-1 labelling index (LI) was calculated in the highest proliferating area as the percentage of labelled nuclei per 1000 cell.
Microscopic examination showed small round cells arranged in linear fashion (Indian file pattern). At places there were spindling of cells whereas in other areas cells were arranged in microalveolar pattern. The cellular outlines were indistinct with scant amount of cytoplasm. Mitotic activity was brisk. The background stroma was hyalinising and myxoid, at places giving chondroid appearance (). No rhabdomyoblasts or strap cells were seen. The tumour cells were immunopositive for desmin (diffuse), vimentin (diffuse), and smooth muscle antigen and focal for myogenin and were immunonegative for s-100, MIC2(CD 99) (). MIB 1 LI was 25% in the highest proliferating areas.
Based on above histomorphological features differential diagnoses of alveolar rhabdomyosarcoma and sclerosing rhabdomyosarcoma were kept. The former was excluded in the absence of multinucleated cells with wreath-like arrangement of nuclei, vacuolated or cells with clear cytoplasm and fibrous septae. Moreover myogenin and desmin immunopositivity was focal in comparison to alveolar rhabdomyosarcoma, where it is strong and diffuse. Absence of strap cells, rhabdomyoblasts, and spindle cells and presence of focal microalveolar pattern excluded the possibility of embryonal rhabdomyosarcoma.
Finally based on histomorphological and immunohistochemical features the possibility of sclerosing rhabdomyosarcoma was entertained.
Metastatic work-up (including CECT chest and abdomen, bone scan, and CSF cytology) was negative. Patient was given adjuvant radiotherapy and chemotherapy. He received 45 Gy/25# external beam radiotherapy followed by 10 Gy/5# boost to the tumor bed. Patient received 12 courses of vincristine (1.5 mg/m2), Actinomycin D (1.35 mg/m2), and Cyclophosphamide (2.2 gm/m2) (VAC) chemotherapy at 3-week intervals. Postadjuvant therapy CECT head revealed significant reduction in the residual infratemporal tumor. Patient remained asymptomatic till 14 months after initial surgery when he developed rapidly progressive weakness of both lower limbs. Patient was paraplegic and incontinent when he reported again in the clinic. MRI of brain and whole spine revealed disseminated meningeal spread of the tumor. Multiple enhancing intracranial meningeal nodules and metastatic tumor deposits were seen compressing the cervical spinal cord (). Patient was subjected to craniospinal radiation (30 Gy/20#). Patient was also given 2 courses of palliative chemotherapy at an interval of 3 weeks (Ifosfamide 2 gm/m2 on days 1–3, Carboplatin 500 mg/m2 on day 3, Etoposide 100 mg/m2 on days 1–3). Further chemotherapy was not continued in view of patient's poor general condition.


An 18-year-old male complained of a sequential decrease in binocular vision over two years. IOPs were 16 mmHg oculus dexter (OD) and 14 mmHg oculus sinister (OS). The patient was diagnosed with LHON that had been caused by a 11778A mtDNA mutation. Bilateral optic disc appearance was characterized via diffuse excavation of the optic cup and rim pallor (). A central scotoma was the predominant visual field defect observed.


A 62-year-old female with CLRAO and CRVO of the right eye exhibited increasing excavation of the cup with superior and inferior rim loss. Temporal rim pallor was identified in the follow-up examinations (). IOP was 15 mmHg OD.


A 31-year-old female complained of a visual field defect of the right eye over a six-month period. IOPs were 14 mmHg OD and 14 mmHg OS. A magnetic resonance imaging scan revealed a hemorrhage in the left basal ganglia, which also involved the left cerebral peduncle and basal ganglia. Left temporal and right nasal disc pallor and rim loss were observed with the corresponding visual field defect of right homonymous hemianopia ().


A 47-year-old male experienced decreased vision in both eyes for ten months. The patient had been diabetic for three years. IOPs were 17 mmHg OD and 16 mmHg OS. Optic disc excavation and disc pallor were identified in both eyes, but was more serious in the left eye (). The patient exhibited congenital physiological ODC with disc pallor as a result of optic atrophy following optic neuritis.


A 21-year-old unmarried female patient presented to our gynaecology department with complaints of increased blood loss during menstruation with passage of blood clots for the past 4 months. She had swelling in hands and feet with facial puffiness. She also had easy fatigability, shortness of breath on moderate exertion and lower abdominal pain for the past 2 months. The patient also complained of excessive dryness of skin, intolerance to cold, weight gain, heaviness of voice and increased hair fall. On physical examination, she was found to have facial puffiness with the presence of mild swelling in the feet. Her weight was 72 kg and her skin was dry. Deep tendon reflexes were prolonged on examination. A clinical diagnosis of hypothyroidism was made, and the patient was investigated further.


A 55-year-old woman was referred to our Reproductive Endocrinology Clinic to further evaluate an undetectable morning serum cortisol level. Initial concern for adrenal insufficiency had been raised 5 years earlier during evaluation for low bone density when a serum DHEA-S level was measured and found to be ‘low’ by the patient's history. Earlier this year, the patient's primary gynecologist observed ‘worsening’ osteopenia and referred her to an endocrinologist. Metabolic bone evaluation was normal. However, the patient mentioned her history of low DHEA-S level, prompting an evaluation of morning cortisol level, which was found to be undetectably low. She subsequently underwent two cosyntropin stimulation tests and both tests revealed baseline morning serum cortisol and adrenocorticotropic hormone (ACTH) levels below the assay detection limits (1.5 μg/dl for cortisol and 5 pg/ml for ACTH) consistent with central hypoadrenalism. Cortisol levels remained undetectably low after cosyntropin stimulation. Serum thyroxine (6.1 μg/dl), thyroid-stimulating hormone (TSH; 2.03 μU/ml), and insulin-like growth factor 1 (101 ng/ml) measurements were normal as was a chemistry panel including tests for renal and hepatic functions and fasting glucose (79 mg/dl). Follicle-stimulating hormone (FSH) levels were in the menopausal range (66.9 IU/l). Dual energy X-ray absorptiometry (DEXA) bone density measurements demonstrated T-scores of the spine, femoral neck, and total hip of −2.4, −1.9, and −2.1. Spinal Z-score was −1.3. Spinal BMD was stable between 2009 and 2012 (0.794 and 0.785 g/cm2). She was placed on hydrocortisone supplementation, but she did not tolerate it due to malaise. At this time, she presented to our clinic for further evaluation. She reported only mild fatigue with mild weight gain and denied having nausea, increased skin pigmentation, or postural symptoms. Symptoms of hypoadrenalism were notably absent, despite the very low cortisol levels. She also reported no symptoms of Cushing's syndrome other than mild weight gain and fatigue and had no symptoms suggestive of cortisol excess on physical examination. Past medical history was relevant for asthma controlled with 220 μg of inhaled fluticasone twice daily (a dose of 110 twice daily initiated in May 2001 and increased to 220 μg in February 2009), ipratropium bromide, and levalbuterol. She denied having any other health problems or using steroids with no history of autoimmune or pituitary disease, hepatic or renal disease, or pulmonary disease other than asthma.


A 42-year-old man was referred to our clinic with a history of feet pain, gait strain, and dementia for three years. The patient had progressive neurological complaints containing ataxia and amnesia. He had continuous pain in both ankles and memory disturbances. The neuroradiologic findings commonly encountered are mild to moderate cortical atrophy, bilateral calcifications in the basal ganglia, and white matter signal changes on MRI ().
There were no significant findings except pain in physical examination of extremity and laboratory tests. Anteroposterior and lateral radiographs and MRI studies of bilateral ankles, together with brain MRI, were performed. Patient has been showing some demential symptoms for the last 3 years.
Lateral radiographs of bilateral ankles showed multiple lytic lesions with sclerotic rims in both tali (Figures and ). On MRI studies fat containing cystic lesions surrounded by smooth, thick rims with septations in both talus have been shown ().
The other skeleton survey radiograms were normal. Because of patient's pain, the procedure containing curettage and autologous grafting has been done to both talus (Figures and ). No postoperative complication has been seen and the pathology report was concordant with polycystic lipomembranous osteodysplasia. At the early postoperative stage, touch-down weight bearing was performed. After the 3 months, patient has begun walking with full weight bearing. Radiological treatment was observed at the 6th month. Three years of followup there has been no problem at the surgery site but demantia and CNS symptoms progressively got worse.


A 64-year-old African American female, gravida (G) 0 with menopause at age 52, presented with a four-week history of vaginal bleeding in July 2013. The bleeding occurred daily, ranging in severity from spotting to moderate bleeding. Her review of systems was otherwise negative, and she had no documented past medical history. She reported that her last Pap smear was performed several years ago and was normal. The vital signs were unremarkable. On pelvic examination, cervical ulceration of 2-3 cm with friability was found. The physical examination was otherwise unremarkable.
Laboratory investigations showed only microcytic anemia with a hemoglobin level of 11.3 g/dL (normal range: 14.0–18.0 gm/dL) and CA-125 of 343 (normal range: 0–35 U/mL). On transabdominal ultrasound, a small amount of fluid within the endometrial cavity was demonstrated, with no significant endometrial thickening. A cervical biopsy was performed, which revealed papillary serous carcinoma with mitotic activity at 4 mitotic figures per 10 high-power fields and occasional psammoma bodies (please see ). The immunostaining was positive for Ki-67 and p53 and negative for estrogen (ER) and progesterone (PR) receptors. A computed tomography (CT) scan of the abdomen and pelvis showed a fluid-filled and distended uterine endometrial cavity with free fluid in the posterior cul-de-sac (please see ). Positron emission tomography (PET) scan revealed extensive lymphadenopathy throughout the abdomen, pelvis, and bilateral hilar lung regions, along with multiple diffuse noncalcified nodules in both lung fields consistent with metastases identified on CT scan of the chest (please see Figures and , resp.). Based on imaging and Ki-67 immunopositivity, the main tumor was determined to be located in the uterine cervix with no extension into the vaginal or uterine walls. It was staged as IVB, based on the revised FIGO staging for cervical cancer (please see ). Given the tumor's advanced stage at presentation, she underwent debulking surgery with total abdominal hysterectomy and bilateral salpingo-oophorectomy in September 2013 (please see ), followed by combination chemotherapy with carboplatin and paclitaxel. The patient received four cycles of chemotherapy so far and tolerated it well without any major side effects.