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Biomedical subjects

D Alagille

Publications and source records attributed to D Alagille.

At least 55 records · Page 3Linked to original sources
PubMed1986

[Portal hypertension in children. Therapeutic approach in cases of failure of a portosystemic shunt].

88 porto systemic shunts were performed between 1977-1985; 14 failures were observed. These failures occurred in ten children with extra-hepatic portal obstruction and in four with intra-hepatic obstruction. The treatment of these failures was different in these two groups: 7 reoperations in the extra-hepatic obstruction, none in the intra-hepatic. That reoperation is often not suitable in the intrahepatic obstruction because of the hepatic failure. The use of sclerotherapy or the beta receptor blocking agents is discussed in this group.

Adolescent↗
PubMed1986

[Serum DNA of hepatitis B virus in children with chronic infection].

Viral replication has been studied in 44 children with chronic infection induced by hepatitis B virus (HBV) investigating HBV-DNA by molecular hydridization technique, correlating its presence with immunological markers and activity of liver disease. HBV-DNA presence is correlated to HBe Ag detection, but this antigen was also found in absence of viral DNA (7 cases of 31). That can mean viral replication diminishes or disappears before the seroconversion. No differences were found in viral replication phases as for clinical manifestations in children. In presence of viral DNA, serum aminotransferases were higher (p less than 0,005) and active hepatic lesions more frequent. Therefore, more cytolysis an inflammation are found in presence of circulating virus. On the other, more developed lesions (inactive cirrhosis and hepatocellular carcinoma) have been found in viral replication absence.

Adolescent↗
PubMed1985

Management of paucity of interlobular bile ducts.

Two types of paucity of interlobular bile ducts (PIBD) may be observed in children with chronic cholestasis. The syndromatic type (80 cases) is the most frequent. The non-syndromatic type (32 cases) is the most severe and half of the patients in this group died from liver failure in the first years of life. The main therapeutic point is that patients with PIBD should not be operated on. The only treatment available is symptomatic medical treatment: the main helpful possibilities concern feeding of medium-chain triglycerides, large supplementation of liposoluble vitamins (A, D, E and K) given intramuscularly, phenobarbital and cholestyramine when there is severe pruritus. The place of the liver transplantation will be clarified in the near future.

Bile Ducts, Intrahepatic↗
PubMed1985

[Alpha 1-antitrypsin deficiency in childhood].

Alpha-1-antitrypsin is a blood glycoprotein synthesized in the liver. It is a protease inhibitor of the serpine group and has a specific action for elastase. Alpha-1-antitrypsin electrophoresis shows about 20 phenotypes, the normal one being PiM. The allele PiZ is usually responsible for liver or lung disease in children or adults, respectively. Eleven per cent of PiZZ infants present with prolonged neonatal cholestasis. Twenty-five of 45 PiZZ infants with prolonged neonatal cholestasis presented with later cirrhosis. Persistence of jaundice beyond the sixth month of age, early development of splenomegaly, persistence of hard hepatomegaly and liver function abnormalities, and early portal fibrosis have a poor prognostic significance. The most severe course occurs in infants with an early histologic pattern of paucity of interlobular bile ducts. Portal hypertension was present in 19 of 25 children presenting with cirrhosis; 8 of 25 PiZZ children with cirrhosis died during childhood.

Bilirubin↗
PubMed1985

[Results of portasystemic shunts in cirrhosis in children].

A surgical porto-systemic shunt was carried out in 37 children and adolescents (age: 2 yrs-18 yrs) who presented with cirrhosis and portal hypertension. All patients were classified "A" or "B" according to Child's criteria. Proof of the patency of the shunt was obtained by endoscopy and/or angiography in 31 children. Two children died in the early postoperative period. Thrombosis of the shunt occurred in 4 children. With a follow-up of 6 months to 11 years, none of the 31 children operated on successfully presented with gastro-intestinal bleeding due to portal hypertension. Nine presented with one or several episodes of porto-systemic encephalopathy. Five of these were transient allowing for a normal diet to be resumed later. These results indicate that porto-systemic shunts are effective to prevent gastro-intestinal bleeding, even in young children with cirrhosis. However, in addition to Child's criteria, indications for shunt surgery must take into account the degree of hemorrhage risk and the possible need for a liver transplantation later.

Brain Diseases↗
PubMed1985

Life-saving immunosuppressive treatment in severe autoimmune chronic active hepatitis.

A 13-year-old girl with a 1-year history of elevated serum alanine transferase was hospitalized because of liver failure. Low prothrombin time (12%) prevented needle liver biopsy. Because of a high titer of antismooth-muscle antibodies (1:500), a tentative diagnosis of "autoimmune" chronic active hepatitis was made and immunosuppressive therapy was started. Despite the severity of the liver disease, of her poor general condition, and of spontaneous bacterial peritonitis, she dramatically responded to treatment, prothrombin time returning to normal within 5 months. Diagnosis of chronic active hepatitis was later confirmed by liver biopsy. This report indicates that immuno-suppressive therapy can be life saving in children with severe chronic active hepatitis even when major signs of liver failure are present.

Adolescent↗
PubMed1984

Histological liver evaluation 5 years after surgery for extrahepatic biliary atresia: a study of 20 cases.

Clinical, biological, and histological data are described in 20 patients at least 5 years after surgery for extrahepatic biliary atresia. Seventeen had an " uncorrectable " type and underwent hepatoportoenterostomy or hepatoportocholecystostomy , 3 patients had a "correctable" type and underwent cystojejunostomy . Clearing of jaundice was observed in all patients. Portal hypertension developed in 16 out of the 20. Liver specimens were obtained by surgical biopsy in all patients. Definite biliary cirrhosis was seen in all but two. Particular patterns were observed in some cases: biliary structures without bile stasis were absent in 8, while 4 displayed marked cell infiltration in portal areas, and 7 had distended vascular channels. The appearance of liver cells was always normal. No relationship could be clearly established between the degree of fibrosis and the earlier presence of cholangitis.

Bile Ducts↗
PubMed1984

Treatment of autoimmune chronic active hepatitis in childhood.

Seventeen children with chronic active hepatitis and high serum titers of smooth-muscle or liver-kidney microsomal antibodies were given prednisone and azathioprine. Clinical and biochemical remission was obtained in all but two, who died of progressive liver failure. Evaluations in 14 children after a mean period of 22 months of treatment showed normal transaminase activity and gammaglobulin levels in 12, and serum autoantibody titers of less than 1: 100 in 10; liver histologic findings showed absence of inflammation in seven children, moderate portal or lobular inflammation in five, and minor features of aggressivity in two. Cessation of therapy was then attempted in nine children. Relapse occurred in all but one and could not be attributed to any previously recorded biologic or histologic feature. After follow-up of 18 months to 7 years, all but two patients are still receiving maintenance therapy with prednisone and azathioprine. Cirrhosis was present before treatment in 13 children and is now present in all but one. These results suggest that in most children with autoimmune chronic active hepatitis, immunosuppressive therapy can prevent further deterioration of liver function but must be pursued for several years before discontinuation is attempted.

Adolescent↗