Intrahepatic cholestasis due to systemic mastocytosis: a case report and review of literature

A 35-yr-old female presented with symptoms of obstructive jaundice. Liver biopsy, bone marrow aspiration, and biopsy revealed systemic mastocytosis and acute myeloid leukemia. The liver biopsy specimen showed infiltration of mast cells within portal tracts with periductal and portal edema, irregularity of interlobular duct epithelium, and centrizonal cholestasis. Endoscopic retrograde cholangiography was normal. Following chemotherapy treatment with idarubicin and cytarabine for seven days for AML, the bilirubin levels continued to increase for two weeks and then decreased, reaching normal levels in two months. Infiltration of mast cells in the liver leads to hepatomegaly, liver function abnormality and rarely portal hypertension. Intrahepatic cholestasis due to systemic mastocytosis has never been reported. We report a rare case of systemic mastocytosis causing intrahepatic cholestasis that resolved with remission of AML following chemotherapy.     

Pleural fluid accumulation due to intra-abdominal endometriosis: a case report and review of the literature

A case is presented of massive ascites and right sided pleural effusion caused by endometriosis. The final diagnosis was not made for a considerable time. Massive ascites and a right sided pleural effusion caused by endometriosis is rare, with fewer than 10 reports in the literature worldwide. Physicians should be aware of this potentially tentially treatable cause, having excluded other possibilities such as malignancy and tuberculosis.     

Recurrent torticollis due to cervical osteochondroma. Case report and review of the literature]

A patient is presented who had bilateral abductor vocal fold paralysis pathologically proven to be due to multiple system atrophy (MSA) in the absence of other neurological features. MSA is a degenerative neurological condition that includes olivopontocerebellar atrophy, Shy-Drager syndrome and striatonigral degeneration. The usual predominant features of MSA are cerebellar ataxia, autonomic dysfunction and Parkinsonism. Stridor is present in over one third of patients and has been reported previously as a presenting symptom in MSA: however previously reported patients have always gone on to develop other neurological symptoms. The usual investigations of bilateral abductor vocal fold paralysis caused by MSA will not reveal the pathological process and we believe that magnetic resonance imaging (MRI) of the medulla and brain stem and autonomic function tests are probably the investigations of choice. It is a worthwhile exercise attempting to identify MSA as the cause of stridor as the prognosis is good in the medium term if appropriate support is offered.     

Septicemia due to Shigella. A case report and review of the literature

To develop a proper protocol for biological exposure monitoring of acetone, we evaluated whether exposure to acetone on the previous day affects the biological monitoring value at the end of a work day. One hundred and ten male workers exposed to acetone in three acetate fiber manufacturing plants were monitored using a liquid passive sampler on two consecutive working days after 2 days without exposure. Urine samples were collected at the start of the workshift and the end of the shift on both days for each subject. For ten exposed workers urine samples were collected approximately every 2 h during and after the first working day until the following morning. Acetone concentrations in urine (Cu) at the start of the first working day were 1.3 +/- 2.4 (range: ND-14.1) mg/l in nonexposed workers and 2.4 +/- 5.6 (range: ND-40.3) mg/l in exposed workers. The urinary acetone concentration at the beginning of the second working day indicated that urinary levels of acetone do not decline to background level by the following morning when exposure concentration exceeds 300 ppm. However, linear regression analysis demonstrated that the relationship between environmental exposure level and urine level was similar on the 1st day and the 2nd day. Thus, although urine acetone levels did not return completely to baseline after high exposures, under the present exposure levels the exposure on the previous day did not significantly affect urinary acetone at the end of the workshift of the next day.     

Left ventricular outflow tract obstruction due to a left ventricular myxoma: a case report and review of the literature

A 17-year-old-man who presented with syncope, had a left ventricular (LV) myxoma causing left ventricular outflow tract obstruction. The tumor was removed through left ventriculotomy using conventional cardiopulmonary bypass with good result. There have been 47 cases of LV myxoma reported in the world literature since 1957. Most of them are symptomatic (92.7%). Systemic embolism is the most common manifestation (50%) and often leads to death. The surgical removal should be performed urgently. Resection of the mass with limited normal tissue surrounding its pedicle is recommended. There are only three recurrences and five operative deaths.     

Latex hypersensitivity/glove component reaction as a contributing factor to osteomyelitis of the hand: a case report and review of the literature

High-resolution computed tomography (HRCT) can be used to diagnose and quantify emphysema noninvasively, as significant correlations have been found between the histological grade on resected lung specimens and quantified (q) computed tomography (CT). In this study, we performed thin section qHRCT in patients with severe hereditary alpha-1-antitrypsin (AAT) deficiency. AAT deficiency is the most common genetic cause of emphysema in adults, and exercise intolerance is the most disabling, distressing consequence of emphysema for the majority of patients. qHRCT was used to quantify precisely the alterations in the lung parenchyma due to pulmonary emphysema. Up until now, the important relationship between the severity of emphysema and the reduced exercise capacity has received little attention. Therefore the purpose of the study was to investigate the relationship between emphysema as displayed by qHRCT and cardiopulmonary exercise testing (CPX) in patients with severe cardiopulmonary impairment. - qHRCT was performed in 21 patients with homozygous AAT deficiency. CT scans were obtained at three spirometrically standardized levels at the carina and (5 cm above and below the carina). The mean lung density at 50% of vital capacity and a quantitative histogram analysis of the frequencies of CT values were determined. All patients underwent symptom-limited CPX to analyse simultaneously cardiovascular and ventilatory systems responses. - In all patients, qualitative CT assessment demonstrated panlobular emphysema with large and extensive areas of uniform low attenuation, characteristically with a lower-lobe distribution. Mean CT density values of the patients (-845 +/- 6.9 (mean +/- SEM)) were significantly correlated with work capacity (r = 0.55, p <0.01), oxygen-pulse (r = 0.54, p <0.01) and functional dead space ventilation (r = -0.54, p <0.01). Moreover, severe emphysema index (CT values below a threshold value of 950 HU) correlated positively with functional dead space ventilation (r = 0.60, p <0.01) and alveolar-arterial oxygen difference (r = 0.70, p <0.001). - These results clearly demonstrate that CPX parameters, indicating a disturbed pulmonary gas exchange and a ventilation-perfusion-mismatch during exercise, are significantly related to the extent of lung emphysema.     

Duodenal phytobezoar: a case report and review of the literature

Phytobezoars are an unusual cause of small bowel obstruction. We report a case of small bowel obstruction due to phytobezoar in a 63 year-old female patient who had undergone gastric surgery (truncal vagotomy with pyloroplasty) for duodenal ulcer disease complicated by gastric outlet obstruction 10 years ago. We diagnosed this bezoar case by radiologic methods and these methods keep their importance for the diagnosis of small bowel obstruction with phytobezoars.     

Atelencephalic microcephaly: a case report and review of the literature

Rapid changes in the circulating blood volume or hemoglobin level during apheresis may pose a risk for healthy individuals donating allogeneic PBSC. In this study, a real-time noninvasive monitor CRIT-LINE was used for continuous monitoring of hematocrit values in a total of 16 aphereses performed in 4 adult (median age 30 years) and 4 pediatric donors (4 years). Donors received recombinant G-CSF (10 microg/kg s.c. for 5 days) for mobilization of PBSC. A CS3000 plus blood cell separator (Baxter) was used in two different procedures. Adults donors were subjected to modified program 1-120 using a combination of the granulocyte chamber and the small volume collection chamber (SVCC), and pediatric donors were subjected to specialized program 4 with a combination of the newly developed small volume separation chamber holder (SVSCH) and SVCC. In all of the procedures for children, the extracorporeal line was primed with 400 ml leukocyte-depleted allogeneic RBC or 200 ml autologous RBC after regular priming with normal saline, whereas none of the adult donors received this treatment. We found a marked contrast in the hematocrit kinetics during apheresis in the two cohorts/procedures. In adults, the initiation of apheresis was followed by an immediate decline in the hematocrit value over the initial 10 min until a stable plateau level was reached (7% decrease). In children, the values decreased slowly but progressively throughout the entire procedure to finally reach a 9% decrease at the completion of apheresis. These data may suggest that the use of SVSCH plus SVCC or priming with RBC can eliminate the abrupt decline in blood hemoglobin levels that occurs during apheresis.     

Riedel's thyroiditis: a case report and review of the literature

Riedel's thyroiditis is a very rare disease of unknown aetiology, occasionally associated with retroperitoneal and mediastinal fibrosis. It is a benign condition, but may be confused with an anaplastic carcinoma of the thyroid. The differential diagnosis with anaplastic carcinoma is assured only by intraoperative biopsy. The Authors report a clinical case: symptoms were a progressive enlargement of the thyroid gland, left recurrential palsy, dyspnoea and dysphagia. The surgical treatment was total thyroidectomy, performed with bilateral neurolysis of recurrent nerves. The patient was also under adjuvant corticosteroid treatment.     

Cryptococcal myositis: a case report and review of the literature

Only rare cases of cryptococcal myositis have been previously reported in the literature. All of these cases have occurred in the setting of human immunodeficiency virus (HIV) infection. We report a case of cryptococcal myositis diagnosed premortem on a needle biopsy in a heart transplant patient undergoing immunosuppressive therapy.     

Gallbladder diverticulum: a case report and review of the literature

BACKGROUND: The clinical significance of the 14 and 6 positive spikes (PS) electroencephalographic (EEG) phenomenon is not well established. This study was performed to provide further data regarding the clinical correlates of the PS, particularly attention-deficit/hyperactivity and somatic symptoms. METHODS: Diagnostic information gathered through structured interviews was compared among four groups of psychiatric inpatients aged 4-17 years who underwent an EEG examination over a 2-year period. Groups consisted of patients with: 1) 14 and 6 PS (n = 25); 2) epileptic discharges (n = 29); 3) slow-wave abnormalities (n = 23); and 4) a normal EEG group age and sex matched to the 14 and 6 PS group (n = 25). RESULTS: Attention-deficit hyperactivity disorder (ADHD) symptoms were significantly more frequent in the PS group (chi 2 = 2.96, p < .05) compared to the other three groups combined. Although somatic symptoms were not increased in the 14 and 6 PS group, anxiety symptoms tended to occur more in this group (chi 2 = 3.50, p < .06). CONCLUSIONS: The PS profile appears associated with ADHD symptoms. Possible treatment implications of this finding (e.g., use of anticonvulsants in ADHD patients with PS) need to be explored.     

Bilateral elastofibroma: a case report and review of the literature

Elastofibroma, or elastofibroma dorsi as it was first described, is an uncommon tumor-like process that characteristically appears as an ill-defined mass in the infrascapular region of elderly patients. This lesion can occur bilaterally and may appear asynchronously. The occurrence of elastofibroma in other anatomic sites has been reported, although with much less frequency. Elastofibromas display typical diagnostic histologic, cytologic, and electron microscopic features. The use of magnetic resonance imaging can lead to a presumptive diagnosis in elderly individuals with suprascapular lesions. It is important to differentiate this lesion from other soft-tissue lesions, such as sarcomas and desmoid tumors. This study presents a case of bilateral elastofibromas in a 72-year-old man and a review of the literature.     

Carcinoid to cecal bowel localization: a case report and literature review

Data are submitted from long-term follow-ups over 78 patients with Gilbert-Meylengraht's syndrome (71 men and 7 women). The above syndrome was identifiable predominantly in those persons aged 15-30. A major proportion of the patients (75%) displayed different dyspeptic phenomena and asthenovegetative disturbances, 25% had no complaints. The principle diagnostic criterion for the syndrome is predominant or solitary elevation of blood serum levels of free (unconjugated, indirect) bilirubin without signs of affection liver, obturation of the biliary tract, and enhanced hemolysis. This form hyper bilirubinemia runs benign course. All above patients remain able bodied having enough capacity for work. In exacerbations, it is helpful to prescribe small doses of phenobarbital (0.05-0.15 gr daily), under the influence of which drug the serum level of bilirubin returns to normal, this effect lasting only for a period of the drug administration.     

Gastric leiomyoblastoma: literature review and report of a case

The extended V-Y flap, a modified V-Y advancement flap, is very useful in closing relatively large defects on the face. Its extension limb is hinged down as a transposition flap on the end of the V-Y advancement flap to close the most distal portion of the defect. We applied this flap in closing a defect following excision of skin tumors on the face with excellent cosmetic results in 11 patients. However, this flap tended to make a distortion at the base of the flap in the primary closure site. By drawing figures, we concluded that the distortion was due to the characteristic of this technique as a V-Y advancement-rotation flap or V-Y advancement flap with rotation.     

Infectious aneurysm due to Listeria monocytogenes: a new case and review of the literature

INTRODUCTION: Infections due to Listeria monocytogenes usually occur in pregnant women, in the elderly and in immunocompromised patients. Arterial aneurysms due to this germ are rare. Only 16 cases have been previously described in the literature. EXEGESIS: We report the case of a patient who had been hospitalized for recurrent fever over the past 3 months. Aortic mycotic aneurysm was diagnosed; blood and aneurysm cultures revealed Gram-positive bacilli consistent with the presence of Listeria monocytogenes. We also review previous reports focusing on infections due to Listeria monocytogenes. Mycotic aneurysms due to this germ are mainly observed in elderly male patients and occur on large arteries. In the present study, only one patient was immunocompromised. Furthermore, all patients who were not operated on died. CONCLUSION: Arterial aneurysm due to Listeria moncytogenes is best managed via surgical resection in combination with antimicrobial therapy. Immunosuppression is not necessary for the development of arterial aneurysm due to Listeria. Bacteriological and histological examinations should be done systematically when surgical resection of an aneurysm is required.     

Congenital microgastria: a case report and review of literature

Congenital microgastria is an uncommon result of impairment of normal foregut development. To date, only 39 cases have been described in the literature. We report a boy born with microgastria and bilateral hypoplastic kidneys who had feeding problems, resulting in failure to thrive and growth retardation. After a short period of conservative management, he was operated upon at the age of 11 months. A Hunt-Lawrence pouch was created, leading to toleration of increasing amounts of oral feeding. Although his feeding problems have decreased, his height and weight are below normal (<10th percentile). The embryology, clinical presentation, diagnostic procedures, associated anomalies, and management are discussed.     

Odontogenic keratocysts: review of the literature and report of a case

The beta 3-adrenergic receptor (beta 3AR) is expressed in visceral fat and is a regulator of resting metabolic rate, thermogenesis, and lipolysis. We genotyped 61 unrelated Mexican Americans for a variant in the beta 3AR gene (codon 64 TGGTrp-->CGGArg; TRP64ARG). The allele frequency was 0.13. The TRP64ARG variant was significantly associated with an earlier age of onset of non-insulin-dependent diabetes mellitus (41.3 +/- 4.6 years vs 55.6 +/- 2.6 years; P < 0.02) and in non-diabetics, with elevated 2-h insulin levels during an oral glucose tolerance test (810 +/- 120 pmol/l vs 384 +/- 6 pmol/l; P < 0.005). Non-diabetic subjects with the variant allele tended to have higher body mass indices (BMI), waist-to-hip ratios, and diastolic blood pressures. The study group was expanded to include 421 related subjects from 31 families in the San Antonio Family Diabetes Study. Using a measured genotype analysis approach to estimate genotype-specific means for each trait, those who were homozygous for the TRP64ARG variant had significantly higher 2-h insulin levels (P = 0.036) and trends towards higher BMI compared to the other two genotypes. We detected no associations of these traits in the TRP64ARG heterozygotes in the larger group. We conclude that the TRP64ARG beta 3AR variant is a susceptibility gene for several features of the insulin resistance syndrome in Mexican Americans. Since its effects are modest, study design (e.g., subject selection, genetic background, and statistical analyses) may influence which traits are associated with this variant and whether or not the effect is detectable in heterozygotes.