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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An outbreak of congenital biliary atresia and jaundice is described, in which approximately 300 crossbred lambs and 9 crossbred calves died. The affected animals failed to thrive, developed jaundice and white scours and died within 4 weeks of birth. A common feature of this outbreak and a similar occurrence 24 years previously was the grazing of plants growing on the exposed silt foreshores of Burrinjuck Dam by ewes and cows in the early stages of pregnancy. Epidemiological and pathological findings suggested that a toxic insult to the foetus in early gestation caused choledysgenesis and biliary atresia, leading to diffuse, subacute to chronic cholangiohepatopathy and cirrhosis.
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PMID:Congenital biliary atresia and jaundice in lambs and calves. 233 68

Anatomical abnormalities of the small bowel that cause intestinal stagnation result in bacterial overgrowth and a blind loop syndrome (BLS). Bacterial breakdown of bile salts and deamination of protein lead to malabsorption, steatorrhea, and fat-soluble vitamin deficiencies. Four children developed BLS as a complication of necrotizing enterocolitis, jejunal atresia, gastroschisis, and biliary atresia. BLS was suggested by abdominal pain, feculent vomiting, steatorrhea, and hypoalbuminemia. Dilated, stagnant bowel loops were demonstrated in each instance by upper gastrointestinal contrast study. Positive intestinal bacterial aspirates were confirmatory. Antibiotic treatment in two patients improved symptomatology but all children ultimately required surgery. Surgical procedures consisted of blind loop resection, intestinal plication, and catheterization of the bilioenteric conduit. All patients are now asymptomatic but one child suffers from parenteral nutrition-related cirrhosis and another requires chronic antibiotic therapy.
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PMID:The blind loop syndrome in children. 240 46

From December 1986 to April 1989, 38 patients with biliary atresia (eight newly diagnosed) were evaluated with doppler ultrasound of the portal venous system. Peak and mean velocities were computer derived from the spectral waveform. Good velocity was greater than 15 cm/s, intermediate velocity was 8 to 14 cm/s, abnormal velocity was less than 7 cm/s or hepatofugal. Patients were grouped according to clinical status: group 1 (n = 14), normal liver function; group 2 (n = 15), recurrent cholangitis; group 3 (n = 2), established bile drainage but complicated cirrhosis; group 4 (n = 7), failed portoenterostomy. All patients with normal liver function (group 1) had good or intermediate velocities. Thirteen of 15 patients with recurrent cholangitis (group 2) had good or intermediate velocities. Both patients in this group with abnormal velocities required transplantation. In group 3 the patient with abnormal velocity is on the transplant waiting list. In group 4, abnormal velocities preceded or coincided with deterioration of liver function in five of seven patients. Doppler ultrasound provides useful anatomic information, determines direction of flow, quantitates velocity of flow, and, when performed serially, provides adjunctive information on liver status in children with biliary atresia. These preliminary results suggest that patients with abnormal or significantly decreasing velocity are destined for transplantation. Patients with good portal venous velocity warrant ongoing, aggressive surgical management.
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PMID:Portal venous velocity in biliary atresia. 240 46

Between 1-1-1982 and 1-1-1988 52 children with an end-stage liver disease were evaluated to determine whether orthotopic liver transplantation (OLT) would be appropriate. 24 children were accepted as candidates in the long term. Twelve were not accepted as potential recipients. The parents of 3 decided not to accept OLT as treatment for their children. Two children died before a suitable donor liver was available, so that OLT was carried out in 11 children. Two of these children needed a second transplant. In 3 children only a part of a donor liver was transplanted. Shortage of donor livers of small size is partly alleviated by using a part of a larger liver. The underlying diseases of the transplanted children were cryptogenic cirrhosis (1x), biliary atresia with a hepatoportoenterostomy (8x) and cirrhosis following neonatal hepatitis (2x). Ten children with OLT are clinically and physically well. Postoperatively a primary graft dysfunction occurred in one child. He was retransplanted. The median waiting time for a donor liver was 5 months. The timing for OLT has to take this in account. In treating children with end-stage liver disease (partial) OLT should be considered.
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PMID:[Orthotopic liver transplantation in children]. 250 30

Severe neonatal cholestasis is a clinical problem requiring a differential diagnosis of intra- (hepatitis) and extrahepatic (biliary atresia) causes, prognosis and therapy being different in the two cases. Eighteen patients of pediatric age underwent US and hepatobiliary scintigraphy. US findings were aspecific in both hepatitis and biliary atresia. In the 11 patients with hepatitis, hepatobiliary scintigraphy after phenobarbital revealed labeled bile in the bowel. Only in 1 patient with cytomegalovirus hepatitis was a scintigraphic pattern similar to that of biliary atresia. On the contrary, no intestinal radioactivity within 24 hours was seen in 6 patients with biliary atresia. A portoenterostomy (Kasai's operation) was performed on 4/6 cases with biliary atresia. These patients were followed with hepatobiliary scintigraphy in order to evaluate anastomotic functionality. In a case of biliary cirrhosis secondary to occlusion, orthotopic liver transplantation was performed whose success was scintigraphically monitored. Our results point to hepatobiliary scintigraphy after phenobarbital as the best noninvasive procedure for both diagnosis and postoperative follow-up of biliary atresia. Labeled bile excretion within 24 hours was rarely found in both atresia and neonatal hepatitis.
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PMID:[Hepatobiliary scintigraphy in the study of neonatal hepatic cholestasis]. 251 35

The Hospital for Sick Children's initial 2-year experience with pediatric liver transplantation is reviewed. Patients are divided into high- and low-risk groups according to certain criteria. The high-risk group includes patients under 10 kg in weight, those with extrahepatic biliary atresia (EHBA), those with portal vein atresia or thrombosis, and those in hepatic coma. All others were considered low risk. Twenty-nine patients were assessed for transplantation: 18 were transplanted and 6 (21% of total referred) died while on the waiting list. Eighteen patients received 23 transplants. Of the 18 recipients, nine had EHBA, four had fulminant hepatic failure, two had tyrosinemia, one had glycogen storage disease, one had Indian childhood cirrhosis, and one had idiopathic cirrhosis. Seven of the 13 patients in the high-risk group survived (55% survival) with 1 to 23 month follow-up. Survival was significantly higher (80%) in the low-risk group (P less than 0.05). Four patients were retransplanted and two survived. Early deaths occurred from prolonged warm ischemia, recurrent portal vein thrombosis, and brain death in a patient who had been transplanted in hepatic coma. Late deaths occurred from cytomegalovirus (CMV) disease (2 patients), acute rejection (1 patient), and myocardial infarction (1 patient). The incidence of primary nonfunction was 4.3% (1 of 23) and of arterial thrombosis was 13% (3 of 23). Survival in patients transplanted for EHBA (67%) was slightly higher than it was for the rest of the group, although not as good as it was in the low-risk group.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Liver transplantation in children: the initial Toronto experience. 255 8

To determine whether portal lymphadenopathy in primary biliary cirrhosis is caused by deposition of lipofuscin pigment in sinus histiocytes and to compare primary biliary cirrhosis with other liver diseases a retrospective study on a consecutive series of 169 livers obtained at transplantation was carried out. There were grouped into eight diagnostic categories: primary biliary cirrhosis (n = 51), primary sclerosing cholangitis (n = 10), extrahepatic biliary atresia (n = 6), chronic rejection (n = 9), cirrhosis (other causes) (n = 38), primary liver neoplasia (n = 21), acute liver disease (n = 20), and retransplantation (other) (n = 14). Lymph nodes were present in 66 specimens. Fifty of these contained granules of lipofuscin pigment. The highest incidence of lymph node enlargement and the largest amounts of pigment were present in cases of primary biliary cirrhosis. A similar pattern of lymph node enlargement was also commonly observed in other chronic cholestatic conditions (primary sclerosing cholangitis, biliary atresia, chronic rejection). Much less pigment was seen in nodes draining livers with non-cholestatic cirrhosis or primary tumours. Nodes were not found in acute liver disease. It is concluded that portal lymphadenopathy associated with lipofuscin is a common finding in various chronic cholestatic liver diseases. The pathogenesis of this lesion is uncertain. Most cases are asymptomatic with enlarged nodes which may be detected only at laperotomy or necropsy and may be wrongly attributed to neoplastic disease. Diagnostically, the finding of large amounts of lipofuscin in enlarged portal lymph nodes is a good indicator of underlying chronic cholestatic liver disease.
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PMID:Portal lymphadenopathy associated with lipofuscin in chronic cholestatic liver disease. 258 29

The late complications in 25 patients more than 3 years after successful Kasai's operation for biliary atresia were presented. Nine (36%) of these 25 patients had episodes of variceal hemorrhage with portal hypertension, and biliary reobstruction in 3 (12%), bleeding from duodenal ulcer in 2, bleeding from gastric erosion in one, and diffuse pulmonary arterio-venous shunt in one were observed. Recently esophageal varices have been well controlled by endoscopic sclerotherapy, and the surgical treatment may be indicated in older children complicated with severe hypersplenism and marked splenomegaly, which are resistant for sclerotherapy. As a surgical procedure, splenectomy with periesophago-gastric devascularization (Hassab's operation) seems to be very useful for portal hypertension by biliary cirrhosis. The reoperation, hepaticoenterostomy, was performed in 3 patients with biliary reobstruction. All three patients are alive and well, and two of them are at 19 years (university student) and 24 years (married life) of age. Hepatic Rehepaticoenterostomy is well indicated rather than liver transplantation, if biliary reobstruction is incurable with conservative therapy. Many of the patients with complications were able to carry on an almost normal life, if suitable treatments for complications were taken.
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PMID:[Late complications after successful Kasai's operation for biliary atresia]. 258 18

There are four major clinical methods for determination of serum total bile acids (TBA) levels. The first is fasting TBA (FTBA), which increases specifically in liver disorders. It increased more sensitively and normalized faster than transaminases in acute hepatic injury. Secondly, the bile acid loading test is useful to examine the degree of liver cell damage. This test improved the detection rate of FTBA for the liver dysfunction and reflects the severity of the diseases. The third is the profiles and dynamic changes of individual bile acids (IBA). In liver cirrhosis synthesis of cholic acid (CA) was lowered and the ratio of CA + deoxycholic acid (DCA)/chenodeoxycholic acid (CDCA) + lithocholic acid (LCA) was less than 1. On the other hand in cholestasis, secondary bile acids were reduced and CA increased markedly due to the regulation of bile acid metabolism, so that the ratio of CA + DCA/CDCA + LCA was more than 1. This phenomenon can be used for the differential diagnosis of liver cell damage and cholestasis. The last is the detection of unusual bile acids, such as 3 beta-hydroxy-5-cholenoic acid is useful for the marker of hepatic cell carcinoma and the screening of congenital biliary atresia.
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PMID:[Clinical examination of serum bile acids for the diagnosis of hepatobiliary diseases]. 260 Oct 71

The Doppler spectrum of the normal inferior vena cava (IVC) shows a majority of flow toward the right atrium, with wide variations in flow velocity and direction owing to effects of the cardiac and respiratory cycles. Seventeen subjects underwent duplex sonography: nine children after liver transplantation, two children with right upper quadrant neuroblastoma, and six healthy control subjects. In six children the spectrum obtained from the IVC bore a strong resemblance to the unidirectional, low-velocity, continuous-flow pattern associated with the portal vein; these children included two children with biliary atresia and cirrhosis, two liver transplant recipients, and two children with IVC compression by neoplasm. In healthy children and adults, a pseudoportal Doppler signal in the IVC can be generated by subcostal right upper quadrant compression. The pseudoportal IVC appears to be a sign of partial IVC obstruction. Knowledge of this potential pitfall and meticulous morphologic imaging can help prevent mistaking the IVC for a patent portal vein.
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PMID:Pseudoportal Doppler signal in the partially obstructed inferior vena cava. 264 43


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