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Biomedical subjects

D Alagille

Publications and source records attributed to D Alagille.

At least 73 records · Page 4Linked to original sources
PubMed1984

Plasma vitamin E levels in children with cholestasis.

Plasma vitamin levels were assayed in 58 children presenting with chronic cholestasis. In the infants who developed cholestasis during the first weeks of life, vitamin E levels dropped below normal values after the age of 4 months. In the older children, vitamin E levels were not correlated with the etiology of cholestasis but with the degree of cholestasis, as expressed by serum bilirubin, serum bile acids, and fat absorption coefficient. We did not find any relationship between vitamin E levels and other biological parameters such as alkaline phosphatases, triglycerides, phospholipids, and cholesterol. These results further support the importance of vitamin E deficiency in chronic cholestasis of infants and children.

Absorption↗
PubMed1983

Early assessment of evolution of liver disease associated with alpha 1-antitrypsin deficiency in childhood.

We observed 45 children with a history of neonatal cholestasis associated with alpha 1-antitrypsin deficiency (phenotype PiZ). Twenty-five developed cirrhosis (group 1), and in the other 20, without cirrhosis (group 2), the outcome was considered to be good. Certain clinical, biochemical, and histologic features of each group were studied to permit early assessment of hepatic evolution. Liver biopsy showed that fibrosis was more frequent and severe in group 1 during neonatal cholestasis. Later this group was characterized by possible persistence of jaundice, early development of splenomegaly, and persistence of hard hepatomegaly and liver function abnormalities. Of the latter, sustained elevation of SGPT and direct bilirubin values were the most striking findings. The characteristics of group 2 were harder to identify: clinical recovery and return to normal biochemical values were always signs of a good outcome, as confirmed by the histologic findings; on the other hand, although some of the children in this group without cirrhosis had only minimal abnormalities, histologic evidence of significant portal fibrosis in some patients made long-term prognosis less certain.

Cholestasis↗
PubMed1983

Portal obstruction in children. I. Clinical investigation and hemorrhage risk.

We examined 108 children with obstruction of the portal vein. Symptoms included splenomegaly and gastrointestinal tract hemorrhage. Obstruction was secondary to portal vein injury in 44 children and was combined with congenital malformations in 17 others. Ultrasonography provided the correct diagnosis in 36 of the 37 children in whom it was performed. Angiography, performed in 101 children, showed that the obstruction extended to the superior mesenteric vein in 14 children and to the entire portal venous system in seven; intrahepatic branches were involved in half the cases. Natural splenorenal shunts were visible in 19 children but were not clearly associated with a lower risk of gastrointestinal tract bleeding; in five of 30 children, cavography displayed abnormalities of the inferior vena cava. Spontaneous gastrointestinal tract hemorrhage occurred in 78 children. Fiberoptic endoscopy showed esophageal varices in 79 of the 81 children studied. The presence of tense varices and congestion of esophageal mucosa clearly augmented the risk of bleeding. These results suggest a simple method of investigation based on ultrasonography for diagnosis and on endoscopy for prognosis. Angiography should be limited to children with a history of gastrointestinal tract bleeding for whom a surgical portosystemic shunt is being considered.

Adolescent↗
PubMed1983

Portal obstruction in children. II. Results of surgical portosystemic shunts.

Seventy-six children with portal vein obstruction underwent surgical portosystemic shunt, for severe gastrointestinal tract bleeding in 64 and for prophylactic purposes in 12. Endoscopy and angiography or both showed shunt patency in 70 children; thrombosis occurred in the remaining six. The mean age at successful shunt surgery was 6 years 10 months. Early postoperative assessment of shunt patency was judged from regression of splenomegaly and thrombocytopenia when splenectomy was not performed; when done, early postoperative ultrasonography correctly indicated the result. Significant regression of endoscopy was most often delayed postoperatively for up to six months. Children with a proved patent shunt did not have any further episodes of gastrointestinal tract bleeding, displayed no clinical signs of encephalopathy, and often exhibited a striking increase in growth velocity. These results strongly support the contention that a portosystemic shunt is the best treatment for portal vein obstruction after the first spontaneous bleeding episode, even in young children.

Adolescent↗
PubMed1982

[HB virus antigens in the liver during chronic hepatitis in children].

An immunoperoxidase study of HBsAg and HBcAg was carried out on liver biopsies of 30 children with chronic hepatitis or idiopathic cirrhosis. Presence of HBsAg and HBcAg occurred only in children who were HBsAg positive in their serum. The number of cytoplasmic HBsAg positive hepatocytes appears higher in children whose liver disease is histologically less severe. The number of hepatocytes with nuclear HBcAg seems directly related to the presence or absence of HBeAg in the serum. Those results are in agreement with previously published observations suggesting that the host's immune response is an important factor of the severity of the liver disease in hepatitis B infections.

Autoimmune Diseases↗
PubMed1982

Prolonged neonatal jaundice: a manifestation of heterozygote state for Crigler--Najjar syndrome?

A prolonged but transient neonatal jaundice due to unconjugated hyperbilirubinemia was observed in two unrelated babies who were siblings of patients with Crigler--Najjar disease (Arias type I). The bilirubin-UDP-glucuronyl transferase activity measured in the liver of both patients, at the age of 6 and 7 months, respectively, was found to be low, suggesting a permanent deficiency in bilirubin glucuronidation, which might be the expression of a heterozygote state.

Clinical Enzyme Tests↗
PubMed1981

Congenital hepatic fibrosis in children.

Twenty-seven children with congenital hepatic fibrosis were followed for three months to 12 years. Hepatosplenomegaly, normal liver function tests, and kidney abnormalities were present in most patients, indicating that a correct diagnosis of congenital hepatic fibrosis could be made using simple clinical, biologic, and radiologic criteria. Esophageal endoscopy showed varices in 21 patients. Sixteen children underwent portal-systemic shunt surgery. Follow-up examinations did not show any impairment of liver function or any sign of hepatic encephalopathy. Cholangitis was present in only three children.

Adolescent↗
PubMed1981

Severe giant cell hepatitis with autoimmune hemolytic anemia in early childhood.

Four children, aged 6 1/2 months to 2 years, presented with liver disease and autoimmune hemolytic anemia. Clinical signs included fever, jaundice, firm or hard hepatomegaly, and splenomegaly. Direct Coombs test results were of the mixed (IgG + C) type. Liver function tests showed high direct bilirubin transaminase, and serum gamma globulin values, and a prolonged prothrombin time. The liver histology was characterized by marked lobular fibrosis and giant cell transformation. The course of the disease was severe, resulting in the death of three patients from liver failure. However, the liver disease seemed responsive to corticosteroid treatment, which in one patient was clearly beneficial.

Anemia, Hemolytic, Autoimmune↗
PubMed1981

Long-term prognosis for infants with intrahepatic cholestasis and patent extrahepatic biliary tract.

One hundred and three infants with prolonged cholestasis beginning before 3 months were classified as having alpha-1-antitrypsin deficiency (17 patients), scanty interlobular bile ducts (16 patients), or "neonatal hepatitis" (70 patients). Twenty-two gradually developed chronic liver disease and the remaining 81 recovered within a few months. Prognosis was found to be poor for infants with alpha-1-antitrypsin deficiency, scanty interlobular bile ducts, and familial "idiopathic" hepatitis. Patients who developed cirrhosis often presented with severe and persistent neonatal cholestasis, mimicking extrahepatic biliary atresia and leading to laparotomy. Thus, a high-risk group of infants-defined by aetiology, family history, and degree of cholestasis-can be recognised in the first months of life.

Bile Ducts, Intrahepatic↗
PubMed1981

Obstructive jaundice in children with histiocytosis X.

Prolonged cholestasis was observed in 6 children with histiocytosis X. Operative cholangiograms confirmed the patency of the extrahepatic biliary tree and showed marked distortion of intrahepatic bile ducts resembling that observed in sclerosing cholangitis. Histologic examination showed portal fibrosis in all patients; only one was found to have portal histiocytic infiltration. The subsequent course confirmed the ominous significance of cholestasis in histiocytosis X, a rare finding in this disease: One patient died of progressive liver failure and three others from sepsis after unsuccessful attempts to improve the condition of the liver with chemotherapy.

Adolescent↗
PubMed1981

[Length of hospitalization and quality of care in a department of pediatrics. Results of a prospective study (author's transl)].

The records of 150 patients hospitalized in a department of specialized pediatrics were analysed immediately after discharge, in order to detect the factors involved in the length of stay. Unexpected course of the disease and familial factors most often increase the length of an hospitalization programed according to technical factors. The interactions between quality of care and length of stay are discussed.

Child↗
PubMed1981

[Length of hospitalization and quality of care in a department of pediatrics. An approach to the parents' point of view (author's transl)].

159 sets of questions answered immediately after discharge by parents of children hospitalized in a department of specialized pediatrics were analysed in order to appreciate how families experience the multiple factors involved in the relation between quality of care and length of hospitalization. The minor importance of the "housekeeping" parameters seems to be related to the fact that families consider them as evident. On the contrary, the relation between hospital staff and parents is privileged by the families : therefore, the quality of hospitalization goes far beyond technical efficiency or financial cost and must include material and psychological factors that are considered as essential by hospitalized children and their families.

Child↗
PubMed1980

Portal diversion for portal hypertension in children. The first ninety patients.

Ninety children with portal hypertension were treated by portal diversion. Fifty-two had cavernous transformation of the portal vein and 38 had an intrahepatic block from various causes. There were 59 central splenorenal shunts, 19 mesocaval, 11 portacaval and one distal splenorenal. In 61 peripheral shunts the veins used for the anastomosis were less than 10 mm in diameter. There was no operative mortality in children with extrahepatic block. One child with cystic fibrosis died postoperatively. Thrombosis of the shunt occurred in five children (5.6 per cent) and was responsible for recurrent bleeding in two. Four children with a thrombosed shunt underwent succesful reoperation and one is awaiting another anastomosis. No late complications occurred in the 52 children with extrahepatic block, while encephalopathy developed in four children with intrahepatic block. These figures confirm our earlier results in the management of portal hypertension in childhood and suggest that portal diversion is the treatment of choice. Several precautions have permitted lowering of the rate of thrombosis whichever shunt is performed. Portal diversion should be indicated following the first episode of hemorrhage in children with extrahepatic block. In patients with intrahepatic block, congenital hepatic fibrosis and cystic fibrosis are good indications as are in general the hepatic diseases with no or mild activity.

Adolescent↗