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Query: UMLS:C0018801 (heart failure)
 72,216 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

Bile duct patterns in the hilar region of the liver were demonstrated in two autopsy cases of biliary atresia by three-dimensional graphic reconstructions. In one case, jaundice had completely disappeared after successful hepatic portojejunostomy. However, at the age of 4 months, this patient died of cardiac failure caused by endocardial cushion defect. The histological reconstruction of this case showed that there were large patent bile ducts, 150 microns to 400 microns in diameter, and plenty of bile ductules surrounding the large ducts to form networks. The other patient died of hepatic failure at the age of 9 months. In this case, only a few large ducts ended in bile lakes that did not connect with the portojejunostomy, and only a few ductules were found around the large ducts. These results indicate that the existence of networks of bile ductules and the patency of large bile ducts at the porta hepatis contribute to good postoperative bile excretion.
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PMID:Bile duct patterns in the hilar region of the liver in two cases of biliary atresia. 231 98

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

The liver in an infant or child is as liable to the same pathologies afflicting the adult liver but with certain differences in prevalence and causes. Genetic disorders are more likely to present in the paediatric age group where many involve metabolic processes such as galactosemia, phenylketonuria, glycogen storage disease and others. Many of these present in the newborn period. However, neoplasms and hamartomas also present in the newborn period, such as congenital neuroblastoma with an enormously enlarged liver, hepatoblastoma and haemangioma. The latter may present with intractable cardiac failure as a result of considerable shunting of blood. Acquired liver lesions often present in the newborn period or early infancy and this includes hepatitis and biliary atresia. The difficulties in the differentiation of the two lesions will be discussed together with the management of biliary atresia. As the child grows older, Reyes encephalopathy with microvesicular fat in the liver is not uncommon. The pathophysiology of Reyes encephalopathy as seen locally will be described. The choledochal cyst with direct (Caroli's disease) or indirect effect on the liver will be described. Problems of childhood portal hypertension as well as congenital hepatic fibrosis will be described. Hemosiderosis of the liver is chiefly seen in homozygous beta-thalassaemia patients who have been kept alive with repeated blood transfusions. Amoebic and pyogenic hepatitis, fatty liver due to protein malnutrition, biliary ascariasis, etc, which are common in tropical and subtropical countries are rarely seen now in Singapore children.
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PMID:Paediatric liver disorders in Singapore. 346 38

Cardiovascular anomalies such as absent inferior vena cava and preduodenal portal vein are reported in cases of biliary atresia and make hepatic portoenterostomy a technical challenge. The authors present the case of a severe cardiac anomaly that significantly altered the functional outcome of a Kasai procedure. Baby M., an 8-week-old boy born with total anomalous pulmonary venous return (TAPVR), underwent hepatic portoenterostomy for biliary atresia. Over the next 3 months he remained icteric and febrile, and failed to gain weight. After multiple antibiotic treatments for suspected cholangitis, he underwent reexploration of the portoenterostomy, with no improvement in his overall condition. His prognosis was considered dismal because correction of the cardiac anomaly is associated with a high mortality rate (> 90%). The cardiac surgeon agreed to attempt a cure of the TAPVR, provided liver transplantation is contemplated if the patient survived. Within 48 hours postoperatively, his hepatic function had improved drastically. He became afebrile, had an improved appetite and weight gain, and was finally discharged 203 days after admission. One year later, he is thriving and remains anicteric. The exact reason for this drastic improvement is not well understood, but the right-sided cardiac failure caused by the TAPVR had a significant effect on the functional outcome of the portoenterostomy.
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PMID:Total anomalous pulmonary venous return complicating portoenterostomy for biliary atresia. 780 57

Long-term survival of biliary atresia patients after Kasai hepatoportoenterostomy is being increasingly reported. Favorable prognostic factors are: 1) Young age at operation (max. 60 days). 2) Few incidents of postoperative cholangitis. Jaundice-free long-term survival is achieved in one-fourth to one-third of patients undergoing Kasai hepatoportoenterostomy. Without treatment the average life span is 19 months. Twenty infants with biliary atresia were treated with the Kasai operation between 1976 and 1992. Nine children are alive 11 months to 16 years postoperatively, two after a liver replacement, one at ten months and one at two and a half years old. A tenth child had a well functioning anastomosis but died of heart failure. Eight children with a well functioning anastomosis were all younger than 60 days at the operation. Only one child older than 60 days survived without a liver transplantation. Our results confirm that early diagnosis and treatment of biliary atresia, before 60 days of age, is necessary.
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PMID:[Biliary tract atresia. Hepatoportoenteric anastomosis and/or liver transplantation]. 832 49

The authors reviewed experience gained over a 20-year period of asplenia or polysplenia syndrome, focusing on patients with associated digestive tract disorders (DTDs). Eleven of 27 patients (40%) with asplenia/polysplenia had associated DTDs. The DTDs comprised malrotation of the intestine in nine, both preduodenal portal vein and gastric volvulus in three, esophageal hiatal hernia in two, and biliary atresia in one. Laparotomy was carried out on four patients with symptoms of acute bowel obstruction and on one patient with biliary atresia. One patient with both malrotation and gastric volvulus, and another with only associated malrotation survived. Nine patients died, eight of cardiac insufficiency and one because of hepatic insufficiency. When infants are diagnosed with heterotaxia, they should be examined for other combined DTDs, because they may have a chance for survival if they undergo surgery when their condition is still stable.
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PMID:Digestive tract disorders associated with asplenia/polysplenia syndrome. 902 79

The diagnosis and detection of some forms of liver and bile duct disease have become possible since the adoption of routine fetal ultrasound scanning in the UK, and the remarkable advances in molecular biological techniques. Nevertheless, although antenatal detection may well have improved, it remains difficult to offer a specific diagnosis, much less a prognosis, in most cases. Cystic dilatation of the biliary tree is probably the most common finding. This may be due to cystic choledochal malformations or, occasionally but crucially, biliary atresia. Postnatal investigations (such as ultrasonography, magnetic resonance cholangiography and liver histology) must take the latter possibility into account, as early surgery is the key to a successful outcome following reconstructive biliary surgery. Antenatally diagnosed liver tumours are usually of vascular origin and have an unpredictable natural history. Some may cause fetal cardiac failure and are candidates for in-utero intervention, but in all likelihood, a large proportion will resolve without specific therapy.
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PMID:Prenatal diagnosis of liver and biliary tract disease. 1500 Nov 23

A new case of severe clinical phenotype of the cat-eye syndrome: We report on a female infant with severe clinical phenotype of Cat-Eye Syndrome (CES). At birth, she had respiratory distress and marked hypotonia. Physical examination showed major craniofacial anomalies including microcephaly, bilateral total absence of the external ears, hypertelorism, bilateral ocular coloboma of iris and micrognathia. In addition, she had anal stenosis, a patent ductus arteriosus and intra- and extra- hepatic biliary atresia. She deteriorated with the development of bradycardia. She died at age one month of cardiac failure. Cytogenetic analysis of the proband showed an extra de novo small bisatelllited marker chromosome in all cells examined. Molecular cytogenetic analysis with fluorescence in situ hybridization (FISH) identified the marker as a CES chromosome. Thus, the patient's karyotype was: 47, XX, +idic(22)(pter-->q11.2 ::q11.2-->pter). The duplication breakpoints giving rise to the CES chromosome were distal to the DiGeorge Syndrome (DGS) locus 22q11.2. The marker could be classed as a type 11 symmetrical (10). According to a recent review of CES literature (1) only 41 % of the CES patients have the combination of iris coloboma, anal anomalies and preauricular anomalies. Almost 60% are hard to recognize by their phenotype alone. Only twelve patients showed a severe clinical phenotype leading to the death of the child. This phenotypic variability increases the difficulties of genetic counseling.
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PMID:A new case of a severe clinical phenotype of the cat-eye syndrome. 1565 20

Little is known about the safety of LRLTx in children with end stage liver disease associated with congenital cardiac anomalies. We report the successful LRLTx in a case with extrahepatic biliary atresia associated with cTGA, VSD, and PS. Preoperative cardiac function was evaluated by cardiac echogram and cardiac catheterization. The recipient's cardiac function was preserved (EF; 54%); however, because of the left to right shunt disease, oxygen saturation was 91%. At operation, carbon dioxide insufflation into the abdominal cavity was attempted to prevent sudden air embolism. Hemodynamic variables were stabilized during partial clamping of the inferior vena cava, and at reperfusion of the portal vein. However, a sudden decrease in blood oxygenation was observed during hepatico-jejunostomy, which was easily normalized by graft mobilization. Post-operatively, neither heart failure nor cerebral infarction because of air embolism was observed. In conclusion, together with preserved cardiac function and carbon dioxide insufflation, LRLTx was successful. Further studies are required to establish the algorithm for the strategy of treating both congenital cardiac anomalies and liver failure.
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PMID:Successful living related liver transplantation in a case with biliary atresia associated with corrected transposition of the great arteries. 1763 Oct 11


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