Wilms tumor associated with polycythemia: case report and review of the literature

A 13 year review of patients diagnosed to have Ludwig's angina admitted to the Christian Medical College and Hospital, Vellore, India, between March 1982 and April 1995 is presented. The patients were either admitted to the ENT or paediatric surgical units. There were 41 patients, 24% being children and 76% adults. The clinical profile and outcome of these two groups were compared. In the paediatric group, none had dental caries while in the adult group, 52% had associated dental caries. In children, 70% were controlled with conservative medical management unlike the adults of whom 81% needed incision and drainage. Tracheostomy was necessary in 10% of the children and in 52% of the adults. The mortality was 10% in both groups.     

Veno-occlusive disease of the liver associated with oral contraceptives: case report and review of literature

This report concerns a young woman who, after taking a contraceptive preparation orally for three years, developed the Budd-Chiari syndrome as the result of a widespread chronic obliterative process involving the intrahepatic efferent venous system. Her prolonged course, which failed to respond to an end-to-side portacaval shunt procedure, mimicked chronic hepatitis evolving to cirrhosis. Additional noteworthy features were the presence of two small benign hepatic adenomas, observed both at biopsy and at necropsy, a lesion recently recognized as a complication of anovulatory drugs, and widespread hepatic calcifications found at autopsy.     

Acquired Glanzmann's thrombasthenia associated with Hodgkin's lymphoma: a case report and review of the literature

BACKGROUND: Acquired Glanzmann's thrombasthenia is a rare hemorrhagic diathesis resulting from impaired adhesive function of the platelet receptor GPIIb/IIIa (alpha(IIb)beta3). Typically, this disorder develops during adulthood, with patients manifesting fluctuating clinical and laboratory findings. To date, the underlying defect of most if not all cases of acquired Glanzmann's thrombasthenia results from an autoantibody or plasma protein inhibitor directed toward a demonstrably normal GPIIb/IIIa glycoprotein. METHODS: In this report, a patient with a history of treated Hodgkin's lymphoma presented with a severe hemorrhagic diathesis characterized by mild thrombocytopenia, a prolonged bleeding time, and defective platelet aggregation. RESULTS: Examination of the patient's platelet GPIIb/IIIa by Western blot analysis revealed no abnormality. Mixing studies demonstrated a non-immunoglobulin G plasma inhibitory factor, whereas flow cytometry analysis revealed elevated platelet-associated immunoglobulin (Ig) M. After an emergency colectomy for severe hemorrhage, the patient's qualitative and quantitative platelet parameters significantly improved. Pathology of the resected colonic segment demonstrated atypical lymphoid hyperplastic lesions. CONCLUSIONS: To the authors' knowledge, this is the first reported case of acquired Glanzmann's thrombasthenia associated with a putative IgM autoantibody. Furthermore, this report verifies the association of acquired thrombasthenia with lymphoproliferative disease. Although rare, awareness of this hemorrhagic diathesis as a possible sequelae of active or treated lymphoid disorders should encourage clinical vigilance of these patients.     

Pulmonary hemorrhage associated with balloon flotation catheters: report of a case and review of the literature

The development of the flow-directed balloon catheter has greatly facilitated the monitoring of seriously ill patients. As the use of this catheter has increased, so have the reports of complications, the most serious of which is fatal pulmonary hemorrhage. Eleven cases of pulmonary hemorrhage have been described in the literature, and we have reported an additional case. The presenting symptom in 10 patients was hemoptysis, and the course of eight of these patients was rapidly fatal. The most frequent significant finding at autopsy was a laceration of a small peripheral pulmonary artery, usually at a bifurcation. The pathogenesis, prophylaxis, and management of this complication are discussed.     

Septic abortion associated with Campylobacter fetus subspecies fetus infection: case report and review of the literature

In contrast to the situation in cattle, goat and sheep, Campylobacter fetus subspecies fetus only rarely causes disease in humans. While a major inducer of septic abortion in animals, only a minority of clinical infections in humans are found during pregnancy. Eleven cases have so far been described in pregnant women. Clinical symptomatology is usually mild during gestation but often leads to premature labor. Here we present a multigravida with positive cultures for C. fetus who went into septic shock. She completely recovered after delivery of a C. fetus-infected fetus at 18 weeks' gestation and treatment with a combination of cephazolin and gentamicin. C. fetus infections should be suspected in patients with intensive contact with (infected) cattle or after intake of unpasteurized dairy products.     

Chylothorax: case report and review of literature

C57BL/6J-Min/+ mice, which are heterozygous for a non-sense mutation in the Apc gene, provide a model for both familial adenomatous polyposis and sporadic colon cancers. In our study, gut tumors and small intestine lymphoid nodules were counted in Min mice fed fiber-enriched diets for 6 weeks. Neither starch-free wheat bran nor resistant starch modified the number of tumors. However, short-chain fructo-oligosaccharides dramatically reduced the incidence of colon tumors and concomitantly developed gut-associated lymphoid tissue. Our experiment shows that short-chain fructo-oligosaccharides counteract advanced stages of colon carcinogenesis, possibly via stimulation of antitumoral immunity by modulation of the colonic ecosystem.     

Myopathy and cardiomyopathy associated with selenium deficiency: case report, literature review, and hypothesis

A case of selenium-deficiency myopathy, secondary to total parenteral nutrition, is presented. The literature on selenium-deficiency myopathy and cardiomyopathy is reviewed in the context of the literature concerning selenium status in numerous diseases. An hypothesis of a wider role for selenium deficiency in the pathogenesis of myopathy and cardiomyopathy is presented.     

Episodic pain associated with a tumor in the parietal operculum: a case report and literature review

We report a young woman suffering brief painful episodes in her right arm which sometimes spread to the whole right side of her body. The episodes were initially rare, but over 13 months they became increasingly frequent until occurring every 20 min. Neurological examination was normal. Magnetic resonance imaging revealed a lesion in the white matter of the parietal operculum. Lesions in the parietal operculum associated with persisting thalamic pain or loss of pain sensation have been reported, but rarely with episodic pain. Since episodic painful attacks have been described in association with various suprathalamic lesions, we conclude that paroxysmal pain attacks may be another consequence of the disturbance of the normal pattern of thalamocortical connections to the second sensory cortical area by a lesion, in our case, of the subcortical area of the parietal operculum.     

Mesenteric panniculitis: case report and literature review

Mesenteric panniculitis is an extremely rare inflammatory condition of the adipose tissue of unknown etiology in which the mesentery is replaced with fibrosis. Knowledge of this rare syndrome should prevent any unwarranted aggressive therapy and help to use the clinical, radiological, and surgical sources to obtain the diagnosis. This paper is a review of symptomatology, pathology, treatment, and outcome of this disorder. A case report is described that presented with obstruction of the sigmoid colon.     

Wegener''s granulomatosis: case report and literature review

The columnar-cell variant of papillary carcinoma is a rare tumor of the thyroid, associated with aggressive behavior, early visceral metastasis, and a rapidly fatal course. In this report we present the fine-needle aspiration cytologic findings of two examples of this variant of papillary carcinoma with cytohistologic correlation. In the smears, clusters, monolayered sheets, and scattered papillary fronds of tumor cells were present. The tumor cells were columnar and exhibited overlapping and stratification of the nuclei. In the first case the tumoral cells showed round nuclei with finely granular chromatin pattern, small nucleoli and vacuolated-appearing cytoplasm. The malignant cells in the second case presented oval to elongated nuclei with stippled chromatin, inconspicuous nucleoli and indistinct cytoplasmic borders. It is important to distinguish this tumor from the common thyroid papillary carcinoma because of its much more aggressive behavior.     

Kenny syndrome: case report and literature review

A 34-month-old girl presented with a clinical picture of Kenny syndrome. The clinical manisfestations included growth retardation, persistent open anterior fontanelle, prominent forehead, mid-facial dysplasia, hypocalcemic tetany and characteristic radiologic skeletal abnormalities. Serum levels of immunoreactive parathyroid hormone (PTH) remained inappropriately low during hypocalcemic episodes in the neonatal period; indicating that hypocalcemia was a consequence of the hypoparathyroid state. This is the first reported case of Kenny syndrome in Taiwan. The literature on the pathogenesis, etiology and genetic basis of this disorder is reviewed in this paper.     

Gestational psittacosis: case report and literature review

OBJECTIVE: To evaluate the safety, efficacy, and biocompatibility of icodextrin- and glucose-containing dialysis fluid during continuous cycling peritoneal dialysis (CCPD), patients were treated for 2 years with either icodextrin- or glucose-containing dialysis fluid for their daytime dwell (14-15 hours). Prior to entry into the study, all patients used a standard glucose solution (Dianeal 1.36%, 2.27%, or 3.86%, Baxter, Utrecht, The Netherlands). DESIGN: Open, randomized, prospective, two-center study. SETTING: University hospital and teaching hospital. PATIENTS: Both established and patients new to CCPD were included. A life expectancy of more than 2 years, a stable clinical condition, and written informed consent were necessary before entry. Patients aged under 18, those with peritonitis in the previous month, and women of childbearing potential, unless taking adequate contraceptive precautions, were excluded. Thirty-eight patients entered the study, and 25 (13 glucose, 12 icodextrin) had a follow-up period of 12 months or longer in December 1996. MAIN OUTCOME MEASURES: Serum icodextrin metabolites: one to five glucose units (G1-G5), a high molecular weight fraction (G > 10), and total carbohydrate level, as well as a biochemical profile were determined every 3 months in combination with all other study variables. RESULTS: In icodextrin-treated patients, serum disaccharide (maltose) concentrations increased from 0.05 +/- 0.01 (mean +/- SEM) at baseline, to an average concentration in the follow-up visits of 1.14 +/- 0.13 mg/mL (p < 0.001). All icodextrin metabolites increased significantly from baseline, as illustrated by the serum total carbohydrate minus glucose levels: from 0.42 +/- 0.05 mg/mL to an average concentration in the follow-up visits of 5.04 +/- 0.49 mg/mL (p < 0.001). At the same time, serum sodium levels decreased from 138.1 +/- 0.7 mmol/L to an average concentration in the follow-up visits of 135.4 +/- 0.8 mmol/L (p < 0.05). However, after 12 months the serum sodium concentration increased nonsignificantly (NS) from baseline to 136.6 +/- 0.9 mmol/L, after an initial decrease. Serum osmolality increased significantly from baseline in icodextrin users at 9 and 12 months, but did not differ significantly from glucose users in any visit. In icodextrin-treated patients, the calculated serum osmolal gap increased significantly from 4.1 +/- 1.4 mOsm/kg to an average of 11.8 +/- 1.7 mOsm/kg (p < 0.01). The sum of the serum icodextrin metabolites in millimoles/liter equaled the increase in osmolal gap. Body weight increased in icodextrin users (71.9 +/- 2.8 kg to 77.8 +/- 3.0 kg; NS). Clinical adverse effects did not accompany these findings. Residual renal function remained stable during follow-up. CONCLUSIONS: The serum icodextrin metabolite levels in the present study increased markedly and were the same as those found previously in continuous ambulatory peritoneal dialysis patients treated with icodextrin, despite the longer dwell time for CCPD patients (14-16 hr versus 8-12 hr). The initial decrease in serum sodium concentration was followed by an increase to a concentration not different from baseline at 12 months. The pathophysiology of this finding is speculated. Calculated osmolal gap in icodextrin patients increased significantly (p < 0.01) at every follow-up visit, and could be explained by the serum icodextrin metabolite increase. We encountered no clinical side effects of the observed levels of icodextrin metabolites.     

Adult-onset Still's disease associated with G6PD deficiency: a case report and literature review

A 37-year-old man presented with symptoms consistent with adult-onset Still's disease. Fever and leukocytosis were prominent, and the patient was started on high-dose aspirin for possible acute rheumatic fever. He developed severe anemia as a result of glucose-6-phosphate dehydrogenase deficiency. His treatment was changed to naproxen, and he recovered with restoration of his hematologic parameters. Although Still's disease is frequently accompanied by mild-to-moderate anemia, the development of severe anemia should raise the possibilities of hemolysis secondary to glucose-6-phosphate dehydrogenase deficiency.     

Epidural abscess: case report and review of the literature

Spinal epidural abscess is an uncommon site of infection, resulting in back pain, fever, weakness and loss of sensibility. These signs should suggest the diagnosis, and quick confirmation by MRI should be performed. Immediate surgical decompression and antibiotherapy is necessary, because this is the base of a possible successful functional recovery. Empiric therapy consisting of high dose of penicillinase-resistant antibiotics is advised because most often an epidural abscess is caused by Staphylococcus aureus. However, because other bacteria can be involved, an aminoglycoside or a cephalosporin should be added to the empiric treatment, until the results of the cultures are known. When diagnosis and therapy are delayed, permanent paralysis and death are common.     

Gangrenous cystitis: case report and review of the literature

A case of gangrenous cystitis in a 67-year-old woman is reported. Associated etiological factors include invasive recurrent carcinoma of the cervix, radiotherapy and atherosclerosis. Primary infection was not a feature. The patient was treated with antibiotics, bladder drainage and total cystectomy with complete recovery. The etiology and management of this unusual condition are discussed.     

Serotonin syndrome: case report and review of the literature

We present the case of a 35-year-old woman who developed serotonin syndrome after receiving a single dose of the cyclic antidepressant imipramine (Tofranil). She was already being treated for depression with paroxetine (Paxil), a selective serotonin reuptake inhibitor. Two hours after receiving imipramine, the patient developed tachycardia, delirium, bizarre movements, and myoclonus, all classic findings of serotonin syndrome. Her antidepressants were discontinued and she was treated with intravenous fluids, sedation, and a short course of cyproheptadine, a serotonin receptor antagonist. All symptoms resolved completely within 24 hours. In this case report, we review the drug interactions that can precipitate serotonin syndrome, and give recommendations for the diagnosis and treatment of this potentially fatal disorder.     

Intrasellar meningioma: case report and review of the literature

BACKGROUND: Intrasellar meningioma is a rare clinical entity, and surgical resection may be difficult when it is hypervascularized. METHODS: A case of subdiaphragmatic hypervascular intrasellar meningioma with attachment to the dura of the anterior wall of the sella turcica is described. Literature review of 18 cases with operatively confirmed intrasellar meningioma discloses unexpected intraoperative bleeding and relatively low resectability of the tumor are also described. RESULTS: We used preoperative endovascular embolization of feeding arteries, and resected a hypervascular intrasellar meningioma by a combined transsphenoidal-transcranial approach safely and without massive bleeding. CONCLUSIONS: Preoperative endovascular embolization of feeding arteries and combined transsphenoidal-transcranial approach are useful for the surgical resection of hypervascular intrasellar meningiomas.