1/86. Clinical spectrum of infantile free sialic acid storage disease.Infantile free sialic acid storage disease (ISSD) is a rare autosomal recessive metabolic disorder caused by a lysosomal membrane transport defect, resulting in accumulation of free sialic acid within lysosomes. Only a few cases have been described. We report on three new cases of ISSD with different modes of presentation: an infant with nephrotic syndrome, a case of fetal and neonatal ascites with heart failure, and a case of fetal ascites with esophageal atresia type III. From these patients and a review of the literature (27 cases total) we draw the following conclusions. 1) "Coarse facies," fair complexion, hepatosplenomegaly, and severe psychomotor retardation are constant findings in this disorder. 2) nephrotic syndrome occurred in most cases (four in seven) in which renal evaluation was performed. Therefore, ISSD is an important cause of nephrosis in infants with a storage disorder phenotype. 3) Fetal/neonatal ascites or hydrops was the mode of presentation in 13 (60%) of 21 cases. Thus, ISSD enters in the differential diagnosis of hydrops fetalis with a storage disease phenotype. 4) cardiomegaly was evident in nine cases. 5) Corneae were always clear, and albinoid fundi were reported in five cases. 6) Dysostosis multiplex was not prominent. 7) bone marrow aspiration could be negative. 8) death ensued in early infancy with a mean age of 13.1 months. All reported deaths were caused by respiratory infections.- - - - - - - - - - ranking = 1keywords = hydrops (Clic here for more details about this article) |
2/86. Treatment of protein-losing gastropathy with atropine.Protein loss from the gastric mucosa with hypertrophic gastric folds and hypoalbuminemia has been associated with low, normal and elevated gastric acid output. A case of protein-losing gastropathy with slightly elevated gastric acid output is described. Associated findings were hypertrophic gastric folds, hypoalbuminemia, hyperlipidemia, lymphadenopathy, edema, ascites and venous thrombosis. Oral administration of atropine resulted in a cessation of gastrointestinal protein loss and correction of hypoalbuminemia.- - - - - - - - - - ranking = 0.006766773636336keywords = edema (Clic here for more details about this article) |
3/86. Severe nonimmune hydrops fetalis and congenital corneal opacification secondary to human parvovirus B19 infection. A case report.BACKGROUND: In parvovirus infections in animals, congenital anomalies are seen, but the teratogenic potential in humans seems fairly low. CASE: A fetus with hydrops, ascites and pleural effusion was seen at a prenatal ultrasound examination. Fetal cordocentesis was performed, and fetal blood was positive for parvovirus antibodies. Intravascular fetal blood transfusion was given at 21 and 23 weeks of gestation. At 39 weeks labor started spontaneously, and a 2,960-g, female infant was delivered. The newborn had bilateral opacification of the cornea. CONCLUSION: In this case a combination of fetal parvovirus B19 infection and congenital corneal opacification was seen. This case also demonstrates that blood transfusions in hydropic fetuses may reverse the hydrops and prevent intrauterine death.- - - - - - - - - - ranking = 3keywords = hydrops (Clic here for more details about this article) |
4/86. Peripheral T-cell lymphoma presenting as ascites: a case report and review of the literature.Here we report an unusual case of T-cell lymphoma presenting as ascites. A 49-yr-old woman was admitted to the hospital for abdominal discomfort associated with increasing abdominal girth over the course of 3 mo. She also complained of nausea, vomiting, and diarrhea. On physical examination, a tense and distended abdomen and edema of the lower extremities were noted. Neither hepatosplenomegaly nor lymphadenopathy was found. A CT scan of the abdomen and pelvis showed a large abdominal/pelvic mass surrounding the small bowel and omentum and small nodes in the para-aortic and mesenteric regions. The cytospin prepared from the peritoneal fluid was hypercellular and composed of a population of monotonous, noncohesive cells with a high nuclear/cytoplasmic ratio and a single prominent central nucleolus. The cells were positive for leukocyte common antigen and Leu-22 (CD43) but negative for keratin, L26, UCHL-1, kappa, lambda, CD3, Ki-1 (CD30), S-100, and carcinoembryonic antigen. Morphologic and immunologic findings were suggestive of T-cell immunoblastic lymphoma. Peripheral T-cell lymphomas rarely present as ascites; this case demonstrates the value of effusion cytology in making this diagnosis.- - - - - - - - - - ranking = 0.006766773636336keywords = edema (Clic here for more details about this article) |
5/86. A rare cause of fetal ascites: A case report of Gunther's disease.Despite an arsenal of ever-improving diagnostic tools, determining the precise etiology of fetal ascites is not always possible. We report a case history where moderately-severe fetal ascites was retrospectively determined to be due to Gunther's disease (congenital erythropoietic porphyria). The infant was found to carry the mutation associated with the most severe disease phenotype in which fetal hydrops has been described.- - - - - - - - - - ranking = 0.5keywords = hydrops (Clic here for more details about this article) |
6/86. Ovarian capillary hemangioma presenting as an adnexal mass with massive ascites and elevated CA-125.OBJECTIVE: Ovarian hemangiomas are very rare with the majority being cavernous hemangiomas. We report a case of a capillary ovarian hemangioma. methods: A case report of a woman with a capillary ovarian hemangioma with massive ascites and an elevated CA-125 is presented. RESULTS: A 39-year-old woman presented with an enlarged ovary containing two ovarian cysts. Her CA-125 was elevated to 872 U/ml. On surgical exploration, she had 1500 cc of clear yellow ascitic fluid and a 7.9 x 6.5 x 4.5 cm left ovarian mass. Frozen section revealed marked stromal edema with luteinized cells and no evidence of malignancy. Histologically, the tumor was a cellular capillary hemangioma with an anastomosing vascular pattern. CONCLUSIONS: This is the first case, reported in the literature, of an ovarian capillary hemangioma presenting with an elevated CA-125 and massive ascites.- - - - - - - - - - ranking = 0.006766773636336keywords = edema (Clic here for more details about this article) |
7/86. Hepatic decompensation in patients with cirrhosis during infection with influenza A.BACKGROUND: patients with chronic liver disease can develop hepatic decompensation during systemic infections. Although gram-negative and gram-positive bacteria are well recognized as causes of decompensation, the effect of influenza virus infection on patients with chronic liver disease is poorly documented. methods: Retrospective analysis of patients with positive viral cultures who were seen at a liver transplantation clinic in a tertiary care referral center during the 1997-1998 influenza A (H3N2) epidemic in San Diego, Calif. RESULTS: Three patients with end-stage liver disease (1 with Wilson disease and 2 with alcoholic liver disease) developed hepatic decompensation and required hospitalization during infection with influenza A. Two patients had biochemical and clinical evidence of hepatic decompensation, including ascites, hepatic encephalopathy, and peripheral edema, and the third had acute hepatocellular damage, with elevated levels of aminotransferases. Viral hepatitis serologic test results, acetaminophen levels, drug and alcohol screening findings, and bacterial and fungal cultures were negative in all 3 patients. Hepatic decompensation resolved without the need for transplantation in the 2 patients with liver failure, and all patients recovered to their baseline liver function levels within 1 month of onset of acute illness. CONCLUSIONS: Influenza A infection can cause hepatic decompensation and hospitalization in patients having cirrhosis or who are awaiting liver transplantation. Effective prevention with vaccination and early recognition and treatment of influenza are strongly recommended in these individuals.- - - - - - - - - - ranking = 0.006766773636336keywords = edema (Clic here for more details about this article) |
8/86. Isolated fetal and neonatal ascites: report of two cases.Neonatal ascites is an uncommon problem that may be caused by a number of etiologies including diseases of genitourinary system and gastrointestinal system, cardiac disease, hepatic disease, systemic infection such as TORCH or parvovirus, chylous, ovarian cause, inborn error of metabolism and idiopathic. We reported two cases of neonatal ascites, one was caused by cytomegalovirus and no obvious causes could be detected in the second one. The ascites were diagnosed by prenatal ultrasound at the gestational age of 25 weeks and 37 weeks respectively and were resolved spontaneously after birth. One-year follow-up of both cases revealed normal growth and development. No recurrent ascites could be detected by abdominal sonography except for evidence of mild hepatomegaly that was noted in case 1. Thus, isolated fetal and neonatal ascites without other concomitant abnormalities were diagnosed, a separate entity from non-immune hydrops fetalis with excellent prognosis.- - - - - - - - - - ranking = 0.5keywords = hydrops (Clic here for more details about this article) |
9/86. ascites due to hypothyroidism in a patient with alcoholic cirrhosis.myxedema is the cause of ascites in less than 1% of new-onset ascites cases, where as only 4% of patients with hypothyroidism present ascites. When ascites is the first manifestation of thyroid insufficiency, there is usually a delay in diagnosis. We report here a case of myxedema ascites occurring in a patient with alcoholic cirrhosis, that was first thought to be the cause of the ascites, and review the features of 48 cases previously reported. Some clinic and analytical findings that have been commonly reported, are the long duration of the ascites before diagnosis, the prompt response (with resolution of ascites) to thyroid replacement treatment, a high total protein concentration in ascites fluid, white moderate white blood cell counts and a lymphocyte predominance. serum-ascites albumin gradient has been postulated to be high in myxedema ascites, but we believe this has been studied in too few cases thus far, to be conclusive.- - - - - - - - - - ranking = 0.020300320909008keywords = edema (Clic here for more details about this article) |
10/86. Recurrent ascites and pleural effusions after surgery for early-stage endometrial adenocarcinoma.A case of massive postoperative ascites in a woman treated for endometrial cancer is reported. A workup for typical causes of ascites yielded negative results, prompting a more detailed analysis of the patient's condition. hypothyroidism was discovered. After correction of the hypothyroidism, the ascites slowly resolved. Since myxedema is an uncommon cause of ascites, this is usually a diagnosis of exclusion. However, hypothyroidism must be ruled out to prevent unnecessary and possibly inappropriate treatments for ascites.- - - - - - - - - - ranking = 0.006766773636336keywords = edema (Clic here for more details about this article) |
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