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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The postoperative course in two children with extrahepatic biliary atresia and cardiovascular disease was reviewed and the correlation between biliary drainage and cardiac function was analyzed. Both patients obtained satisfactory biliary drainage after Kasai's hepatic portoenterostomy. One patient developed heart failure postoperatively due to severe viral myocarditis. This child's total serum bilirubin concentration remained elevated for eight months, despite adequate bilirubin excretion, until her cardiac function returned to normal. Another patient died of cardiac failure due to congenital heart disease 83 days after Kasai's operation, but his postoperative biliary drainage was satisfactory as long as cardiac function remained compensated. In both cases, fluid intake was restricted severely (30 to 70 ml/kg body weight/day), as titrated by echocardiographic assessment of cardiac function, but biliary excretion was satisfactory as long as the cardiac fractional shortening ratio was greater than 30% and the ejection fraction was greater than 55%. This suggests that cardiac decompensation affects postoperative biliary excretion in patients with biliary atresia; however, with careful medical management satisfactory biliary drainage can be achieved even in patients with severe heart diseases.
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PMID:Postoperative management of children with biliary atresia and heart failure. 161 Jul 49

Serious respiratory syncytial virus (RSV) disease requiring hospitalization occurs primarily in infants younger than 12 months. The incidence, risk factors, and clinical features in older children have not been studied extensively. Of 282 children hospitalized at our institution with severe RSV disease during a 3-year period, 62 (22%) were older than 12 months. These 62 older children were matched for sex, onset of illness, and hospital location with 62 hospitalized children younger than 12 months with proved RSV infection. Older children had underlying chronic disease more commonly than younger children (47 of 62 vs 24 of 62). Chronic illnesses in older children included bronchopulmonary dysplasia and/or reactive airway disease (34 of 47), congenital heart disease (9 of 47), gastrointestinal disease (7 of 47), and genetic disorders (7 of 47). Three of the four deaths from RSV infection occurred in older children; all four had underlying disease (three with congenital heart disease and one with biliary atresia). We conclude that children older than 12 months with underlying disease are at increased risk for serious or fatal RSV infection and are not always protected by previous RSV disease. Such older children should be considered candidates for passive or active immunoprophylaxis against RSV infection as such agents become available.
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PMID:Severe respiratory syncytial virus infection in older children. 230 43

Five patients were encountered in whom agenesis either of the proximal extrahepatic biliary ducts (four patients) or total absence of the extrahepatic bile ducts and gall bladder (one patient) were evaluated. Jaundice was diagnosed from at birth to 3 weeks of age (average, 1.2 weeks of age) in these five patients. The patients' ages ranged from 2 to 8 weeks at the time of surgical exploration. Findings at surgery showed either absence of the entire extrahepatic biliary ducts or proximal bile duct remnants, no evidence of an inflammatory process, and no fibrous mass present at the portahepatis. Liver biopsy specimens showed histological evidence of cholestasis, minimal bile duct proliferation and fibrosis, and nearly complete absence of inflammation. In three patients in whom a portocholecystostomy was performed, no bile flow was obtained. Two patients underwent surgical exploration and liver biopsy only. One patient died as a result of severe congenital heart disease at 3 months of age. Four patients have undergone successful hepatic transplantation. These patients are now 10 months to 6 years of age. In our review of the literature, we were unable to find any reports of bile duct agenesis despite the fact that it appears to be a known phenomenon. We conclude that patients with biliary agenesis have early onset of jaundice when compared with patients with biliary atresia, absence of inflammation at the portahepatis at the time of surgical exploration, as well as on biopsy of the liver. Portoenterostomy or portocholecystostomy are likely to fail. We believe that liver transplantation is the treatment of choice for this rare entity.
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PMID:Agenesis of the extrahepatic bile ducts: report of five cases. 238 Sep 2

In a review of pediatric autopsies from 1951 to 1985, we identified 40 cases in which pancreatitis was diagnosed pathologically. Twenty-six of these patients were under 4 years of age, and the male-to-female ratio was 1.5. Six groups of patients were identified: 10 with hepatobiliary disease, including 9 with biliary atresia; 7 with immunosuppressive therapy for tumors (n = 2), leukemia (n = 4) and aplastic anemia (n = 1); 6 with viral infections; 8 with congenital anomalies, including congenital heart disease (n = 3); and 9 with miscellaneous problems. Several patients had surgery and various intercurrent complications. Clinical features attributable to the pancreatitis included vomiting or excessive nasogastric drainage (60%), pleural effusions (40%), and abdominal pain (25%). However, the diagnosis was suspected clinically in only 5 of 40 patients. Our findings suggest several pathogenic mechanisms exist for childhood pancreatitis: biliary obstruction, infections, drug toxicity, immunosuppression (acting in synergy with drug toxicity, trauma, and low-flow states resulting from shock, heart failure, and vasculopathy.
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PMID:Clinicopathologic studies in childhood pancreatitis. 334 10

Between March 1981 and March 1986, 200 orthotopic heart transplantations were performed at the University of Pittsburgh. Fourteen of those procedures were carried out in children 2 to 16 years of age. Two children received combined liver and heart transplants; one because of familial hypercholesterolemia with associated ischemic heart disease, and the other because of dilated cardiomyopathy associated with intrahepatic biliary atresia. Eight patients had dilated cardiomyopathy, and two had myocarditis. Two had heart transplantations for congenital heart disease: one had multiple muscular ventricular septal defects repaired in infancy and had an associated cardiomyopathy, and the other developed a cardiomyopathic ventricle from a congenital right coronary artery to right atrial fistula. Chronic immune suppression consisted 0.2 to 0.5 mg/kg/d of prednisone and 5 to 50 mg/kg/d cyclosporine, with the addition of antithymocyte globulin for unresolved moderate or severe acute rejection. There were three early postoperative deaths: one from intracranial bleeding, one from Pseudomonas mediastinitis, and one from ischemic injury to transplanted organs. Early postoperative complications included reversible renal failure, hypertension, and seizures. Late problems were related to allograft rejection and side effects of cyclosporine and corticosteroids. Significant rejection episodes occurred in all patients surviving longer than 2 weeks, with seven requiring antithymocyte globulin. Two patients died 8 months following transplantation of severe acute and chronic rejection; another patient required retransplantation for ischemic cardiomyopathy resulting from chronic rejection but subsequently died of recurring rejection 3 months after the second transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Experience with heart transplantation in children. 354 Aug 34

Transfer of antibiotic (cefmetazole, CMZ) into the liver tissues of the infant with hepatic dysfunction, 6 cases of congenital biliary atresia, 4 cases of congenital bile duct dilatation, 1 case of congenital biliary hypoplasia, hepatic hemangioma and umbilical hernia with congenital heart disease is reported here. CMZ level in the liver tissues with hepatic dysfunction shows extremely low. Our study revealed that poor transfer of CMZ into the liver tissues might be a main cause of the poor excretion of CMZ into the bile in case of jaundiced infant.
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PMID:[Transfer of antibiotics into the liver tissue of infants with hepatic dysfunction]. 386 94

Situs inversus (SI) complicating neonatal bowel obstruction presents a challenging complex, and to facilitate rational decision making for treatment, we have reviewed 23 cases of abdominal SI seen in our hospital over the last 25 years. Preoperative roentgenographic studies most always predicted SI, the specific patient groups including: 12 abdominal SI with dextrocardia, 10 abdominal SI with levocardia, and 1 with partial heterotaxia. Major intraabdominal anomalies produced surgical emergencies in 7 neonates in the first year, 6 of these 7 being in the first month of life. In these 7 patients, multiple anomalies occurred including 1 child with a rotational anomaly with reversible ischemia secondary to midgut volvulus, and 4 with a rotational anomaly without volvulus, all being treated with a modified Ladd procedure. One of these children had an unrecognized intraluminal duodenal membrane, 1 an operatively diagnosed intraluminal membrane, 1 had annular pancreas, and 1 had a discontinuous jejunal atresia. A preduodenal portal vein was present in 4 of the 7 children, a branch being divided in 1 and the full vein bypassed in 2 of the other 3 patients. Two patients had biliary atresia, one of whom also had a diaphragmatic hernia. Five of the 7 neonates had associated major congenial heart disease accounting for 2 of the 3 deaths in this series. This review emphasizes the protean nature of abdominal SI, especially as it may cause or contribute to neonatal intestinal obstruction; and it is this understanding which is a prerequisite to optimal operative management.
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PMID:Situs inversus: the complex inducing neonatal intestinal obstruction. 666 1

Three patients with extrahepatic biliary atresia associated with cyanotic congenital heart disease are presented and their clinical courses reviewed. In these patients, the coincidence of cyanosis and jaundice caused an atypical green-appearing cutaneous discoloration which masked the early clinical detection of biliary atresia in one infant and cyanotic heart disease in the other two. In all infants, either partial or complete correction of the congenital cardiac lesion was possible. Two infants died from hepatic disease despite attempted surgical revision and one has satisfactory bile drainage one month following portoenterostomy. Awareness of the rare concurrence of cyanotic congenital heart disease and extrahepatic biliary atresia is helpful to the clinician when evaluating infants with atypical cutaneous discoloration. Early oversight of either underlying disease process can be avoided, and early evaluation and management of both entities can be possible.
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PMID:Extrahepatic biliary atresia associated with cyanotic congenital heart disease. Three case reports and a review. 744 48

Hypertrophic osteoarthropathy (HOA), well known in adults, is rarely encountered in children. The clinical features include clubbing of the fingers and toes, arthritis, and a sometimes painful ossifying periostitis of the tubular bones. Apart from a hereditary form (primary HOA), most of the cases encountered in children are secondary and associated with conditions such as chronic suppurative lung processes (e.g., cystic fibrosis), congenital heart disease, biliary atresia, and polyposis coli. The association with malignant disorders, which is relatively common in adults, is very rare in children. In 1986 the authors published a case report of a patient with carcinoma of the nasopharynx who developed HOA. Another similar patient has been encountered. In both, the appearance of HOA was associated with a very poor prognosis. A meticulous research of the literature from 1890 to 1990 revealed only 24 children (19 boys, 5 girls) under the age of 18, with malignancy and associated HOA. Among them were 10 patients with a carcinoma of the nasopharynx, 8 with osteosarcoma, 3 with Hodgkin's lymphoma, 1 with a periosteal sarcoma, 1 with mesothelioma of the pleura, and 1 with carcinoma of the thymus. In five patients with HOA, there were no abnormalities of the lungs, mediastinum, or pleura, and none developed during the course of the disease. Many authors mention the predictive value of HOA, especially in association with malignant tumors. In contrast to suppurative processes in the lungs, in those with neoplastic disease involving the chest, HOA may precede pulmonary symptoms by 1-18 months. A striking feature of HOA in these instances is the reversibility of the complaints after successful treatment of the disorder of the chest, both in benign and malignant conditions. The present case is the second reported by the authors and the first description of a girl with carcinoma of the nasopharynx developing HOA.
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PMID:Hypertrophic osteoarthropathy in childhood malignancy. 841 3

The features of Goldenhar complex have been well-described and classically include branchial arch abnormalities, epibulbar dermoid and vertebral abnormalities. We have identified an infant with these features in association with complex congenital heart disease and intrahepatic biliary atresia. Although Lambert described an autosomal recessive disorder with an association of biliary atresia and branchial arch abnormalities, none of those cases had epibulbar dermoid. Diagnostic considerations in this case include inclusion of biliary atresia as a new feature in the expanding spectrum of the Goldenhar complex, versus Lambert syndrome with epibulbar dermoid.
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PMID:Branchial cleft anomaly, congenital heart disease, and biliary atresia: Goldenhar complex or Lambert syndrome? 1089 66


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