ISSN:0277-2116    

出版商:OVID    

年代:1989

数据来源: OVID

ISSN:0277-2116    

出版商:OVID    

年代:1989

数据来源: OVID

摘要:

Since the introduction of cyclosporine A, liver transplantation has become accepted as the therapy for end-stage liver disease. However, there are no definite criteria for liver replacement in biliary atresia. We investigated (a) the survival rate after hepatic portoenterostomy (n = 131), (b) liver function tests in fatal cases after an initially successful hepatic portoenterostomy (n = 9), and (c) liver function tests in the patients with episodes of cholangitis after a successful surgical treatment (n = 8). Patients with persisting jaundice after the surgery cannot be expected to survive long, and therefore they definitely should undergo liver transplantation. When total bilirubin concentration was above 10 mg/dl in patients with cholangitis after a successful operation, conservative therapy had almost no effect. Therefore, patients with total bilirubin levels above 10 mg/dl should be considered for liver transplantation. Of the liver function tests, only total bilirubin was reliable as a marker for hepatic failure in the end stage of biliary atresia. Prolongation of thrombo test and episodes of gastrointestinal bleeding also were used in selection of patients for liver replacement.

ISSN:0277-2116    

出版商:OVID    

年代:1989

数据来源: OVID

摘要:

Three children were observed to have extensive liver injury following protracted seizures. Two recovered with supportive care and one died from central nervous system complications. When first measured, the levels of aminotransferases were minimally elevated, but they increased to 250 to 8,000 times normal within 12 to 24 h after the seizure episode. They fell to near normal over the next 8 to 11 days in the survivors, and to one sixth of the peak level by 4 days in the patient who died. A percutaneous liver biopsy from one child demonstrated centrolobular necrosis consistent with severe ischemic injury. Common causes for liver dysfunction, including viral hepatitis, drug hepatitis, and Reye syndrome, were excluded on clinical, serologic, and histologic grounds. We reason that hepatic injury resulted from ischemia. We speculate that prior treatment with anticonvulsants, which are capable of inducing mixed-function oxidases in the liver, aggravated the ischemia-reperfusion injury by increasing the production of reactive oxygen intermediates and reducing cytoprotective mechanisms. Prevention of such injury should be directed toward control of seizures and early respiratory support when seizures occur, not restructuring medication regimens.

ISSN:0277-2116    

出版商:OVID    

年代:1989

数据来源: OVID

摘要:

A prospective study was undertaken on 49 infants with prolonged neonatal cholestasis to determine whether the specificity, sensitivity, and accuracy of99mTc-disofenin scintigraphy can be improved by performing simultaneous radioactivity measurements on duodenal and gastric aspirates along with routine imaging. Specificity of routine scintigraphy was improved by this modification from 45.7 to 76.4%. Accuracy of the test was also superior in improving from 55% with routine scintigraphy to 78.4% by counting additional radioactivity on both duodenal and gastric aspirates. We suggest that this modified technique be applied in the evaluation of infants with conjugated hyperbilirubinemia so that invasive procedures such as open liver biopsy and operative cholangiogram may be avoided.

ISSN:0277-2116    

出版商:OVID    

年代:1989

数据来源: OVID

摘要:

Twenty-eight consecutive children with either chronic liver disease or portal vein obstruction were investigated to study the presence of gastroesophageal collaterals due to portal hypertension by ultrasound and endoscopy. Sonographic measurements of the ratio of the lesser omentum thickness to aortic diameter (LO/Ad) and of portal vein diameter to body surface (Pv/m2) were significantly higher in children with esophageal varices than in those without varices. With a lower limit for the LO/Ad ratio of 1.3, no false negatives and only three false positives (91% predictive value of esophageal varices) could be detected. The combination of LO/Ad and Pv/m2would exclude the possibility of detecting esophageal varices if the LO/Ad ratio is <1.8 and the Pv/m2is <12 mm. All children with an LO/Ad ratio <1.9 had varices. Even in the presence of an overlap between children with small and large varices, it is possible to identify with high probability a child with large varices if the LO/Ad ratio is <2.5. This study confirms the value of ultrasound in the diagnosis and management of children with portal hypertension and suggests an algorithm to screen children with small and large varices.

ISSN:0277-2116    

出版商:OVID    

年代:1989

数据来源: OVID

摘要:

A group of children with autoimmune hepatitis is characterized by the presence in their sera of anti-liver-kidney microsome antibody (LKMA) as defined by immunofluorescence. Immunoblot analysis of the sera of 21 such children using rat-liver microsome total proteins as antigen allowed separation into three groups—group 1, whose sera recognized a 50 kDa protein; group 2, whose sera recognized a 66 kDa protein; and group 3, whose sera recognized both proteins. Patients with the anti-66-kDa reactivity more often displayed an acute onset of the disease, less signs of portal hypertension, better sensitivity to immunosuppressive therapy, and less tendency to relapse. They also displayed a lower titer of anti-rat-liver microsome antibody in enzyme-linked immunosorbent assay (ELISA) and a serum reactivity with a rat-liver cytosolic protein. These results (a) indicate that the LKMA-positive autoimmune hepatitis of children is heterogeneous from both clinical and immunological view points, (b) suggest that children with anti-66-kDa reactivity could have a less severe disease than children with the 50-kDa reactivity, and (c) indicate that immunoblot analysis should be added to ELISA and immunofluorescence studies to achieve better characterization of these patients.

ISSN:0277-2116    

出版商:OVID    

年代:1989

数据来源: OVID

摘要:

Lipid profile analyses were performed on serum samples from six children (ages 4–17 years) who had previously been subjected to orthotopic liver transplantation. All six patients were receiving prednisone and cyclosporine and four were additionally receiving azathioprine. Marked elevations in serum triglycerides (>95%) were seen in 3/6 patients, while a notable decrease (<5%) in HDL-cholesterol was noted in 4/6 patients. Total serum cholesterol was normal in all patients. Postheparin lipase activity determined in the serum of one patient revealed a significant decrease compared with control values. These observations suggest the need for studying large numbers of liver transplant recipients to determine the prevalence of lipid abnormalities and to consider their potential contribution to the development of premature atherosclerotic disease.

ISSN:0277-2116    

出版商:OVID    

年代:1989

数据来源: OVID

摘要:

Severe failure to thrive is an important feature in children with both syndromic and nonsyndromic paucity of interlobular bile ducts (PILBD). Thirteen children age 7 months-11 years with PILBD had pancreatic secretions in duodenal aspirate measured for 40 min after secretin-pancreozymin stimulation, six of these children had chronic diarrhoea. Studies were also performed in seven children age 2–12 years who presented with failure to thrive or nonspecific diarrhoea that subsequently resolved. The results of the control and the PILBD children were similar except in the six with chronic diarrhoea. These children had significant reductions in total volume of duodenal aspirate (p < 0.05), bicarbonate concentration (p < 0.02) and output (p < 0.05), and in lipase concentrations (p < 0.005). Five of these children have had a reduction in stool frequency and more rapid weight gain since receiving pancreatic supplementation. In children with PILBD and diarrhoea, pancreatic insufficiency may be a contributory factor to poor weight gain. These children may benefit from pancreatic extract supplementation.

ISSN:0277-2116    

出版商:OVID    

年代:1989

数据来源: OVID

摘要:

Five adolescents, 13–18 years of age, underwent esophageal manometric studies because of chronic symptoms suggestive of esophageal dysfunction. Four of five patients had episodic nonexertional midchest pain; two patients experienced intermittent dysphagia. The manometric findings for these adolescents were consistent with a primary motility disorder known as diffuse esophageal spasm, a condition not previously reported in this age group. This represents approximately 1% of all pediatric patients undergoing esophageal manometry at our institution for the past 5 years. They have been followed for at least 2 years and three have experienced gradual resolution of their symptoms with normalization of manometric findings. Our report emphasizes two main points: (a) Diffuse esophageal spasm may cause chest pain and dysphagia in adolescents; and (b) the clinical history and esophageal manometric findings establish the diagnosis of diffuse esophageal spasm.

ISSN:0277-2116    

出版商:OVID    

年代:1989

数据来源: OVID