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Biomedical subjects

D Alagille

Publications and source records attributed to D Alagille.

At least 91 records · Page 5Linked to original sources
PubMed1980

[Ultrasound and portal hypertension in children. Demonstration of gastro-oesophageal collaterals. Preliminary report (author's transl)].

The authors studied the value of ultrasonography in demonstrating oesophageal collaterals in portal hypertension in childhood. 22 children with portal hypertension underwent successively an ultrasound examination and an endoscopy looking for submucosal oesophageal varices. Oesophageal collaterals were said to be present when ultrasound showed vascular structures in the gastro-hepatic ligament above the coeliac trunk origin. Among those 22 patient 18 had a satisfactory ultrasound examination. 12 true positive, 3 true negative, 3 false negative ultrasound examinations were obtained. 4 ultrasound studies were inconclusive. The value of ultrasound in the pre and post surgical check-up of portal hypertension in children is emphasized.

Adolescent↗
PubMed1978

Hereditary fructose intolerance in childhood. Diagnosis, management, and course in 55 patients.

The early manifestations of hereditary fructose intolerance are described in a series of 55 patients. Management of this metabolic disorder depends on the severity of liver impairment. When the patients are given a fructose-free diet, the improvement is a dramatic but liver enlargement and fatty vacuolization of liver cells often persist. These hepatic findings were also observed in the five homozygous infants who were given a fructose-free diet from birth; this outcome may support the hypothesis that minimal amounts of fructose are esential for human beings.

Carbohydrate Metabolism, Inborn Errors↗
PubMed1978

Hepatitis B in children. I. Analysis of 80 cases of acute and chronic hepatitis B.

From 1971 to 1975, HBV-induced hepatitis was observed in 80 children. The diagnosis was based upon the detection in serum of HBsAg and/or the secondary occurrence of anti-HBs. Thirty-one patients presented with acute viral hepatitis, 16 with severe or fulminant hepatitis, 17 with chronic persistent hepatitis, 12 with chronic active hepatitis, and 4 were asymptomatic chronic carriers of HBsAg. Twenty-nine of 80 children were under one year of age (36%), the peak of frequency occurring from 2 to 5 months. The source of infection, determined in 27 of 29 infants, was administration of blood derivatives in 15 cases and contact with an HBsAg carrier mother in nine instances. In the latter type, the incubation time (103 days) was compartible with an oral route of infection, Persistent antigenemia occurred in only 3 of 29 patients. The overt type of disease developed by most infants, as well as the small number of patients who became HBsAg carriers, suggest that the carrier state, often encountered in neonatally infected infants in other countries, may be related to environmental or genetic factors rather than to immaturity of theimmune system.

Acute Disease↗
PubMed1978

Case of congenital nonobstructive, nonhaemolytic jaundice. Successful long-term phototherapy at home.

Use of cholestyramine made it possible to shorten the daily duration of phototherapy in a case of congenital nonobstructive, nonhaemolytic jaundice. Treatment at home was therefore possible, allowing normal parental care. Development and neurological examinations were normal at the age of 27 months. Frequent determinations of reserve bilirubin binding capacity may be useful in controlling such management.

Bilirubin↗
PubMed1978

[Severe viral hepatitis in childhood: course and prognosis].

In 22 children with severe acute viral hepatitis, the course of the disease followed 3 patterns: 8 children completely yielded (regenerative hepatitis); 8 died during the first three weeks of evolution (aregenerative hepatitis); 8 had a prolonged evolution (hyporegenerative hepatitis). In the latter group, 6 patients died after an average survival time of 55 days and 2 patients rapidly developped a cirrhosis. This type of evolution was characterized by persistence of liver failure manifestations, in spite of liver regeneration, as indicated by increased levels of alpha-foetoprotein and presence of pseudo-acini, giantcells and nodules at histological examination. During the second week of evolution, the size of liver, levels of clotting factors VII +X and alpha-foetoprotein concentrations seem to constitute important prognostic factors.

Child↗
PubMed1977

Congenital abnormalities associated with extrahepatic portal hypertension.

Congenital abnormalities were present in 12 out of 30 (40%) children with extrahepatic portal hypertension of unknown cause, but in only 2 out of 17 (12%) children with extnahepatic portal hypertension secondary to umbilical vein catheterization or omphalitis. The most frequent abnormalities in this series and in published reports were atrial septal defect, malformation of the biliary tract, and anomalous inferior vena cava. These findings are consistent with the view that some cases with extrahepatic portal hypertension are congenital in origin.

Adolescent↗
PubMed1976

Hepatic porto-enterostomy or cholecystostomy in the treatment of extrahepatic biliary atresia. A study of 49 cases.

Hepatic porto-enterostomy or cholecystostomy (Kasai's procedure) was successful in restoring bile flow in 31 of 49 patients with "noncorrectable" extrahepatic biliary atresia. However, all but one of the 31 developed acute or chronic complications such as cholangitis, bile peritonitis, or portal hypertension. During a five-year follow-up period, 26 (53%) died while 9 of the 23 survivors continue to manifest chronic or recurrent cholangitis. Thirteen of the 19 survivors who are more than one year of age have developed portal hypertension. These complications limit the prognosis of infants with "noncorrectable" biliary malformations.

Bile Ducts↗
PubMed1976

Alpha1-antitrypsin deficiency and liver disease in children: phenotypes, manifestations, and prognosis.

Among 424 children with liver disease, 20 had alpha1-antitrypsin deficiency associated with protease inhibitor ZZ phenotype. This disorder manifested itself as cholestasis in early infancy in 19 children. Jaundice and pruritus cleared in 16 of these by 7 months of age, but hepatomegaly and laboratory evidence of mild hepatic dysfunction persisted in all. Biliary cirrhosis and portal hypertension eventually developed or was suspected in eight, and hypoplasia of intraheptic bile ducts was demonstrated in another four. Routine screening revealed intermediate alpha1-antitrypsin deficiency in 16 other children with various types of liver disease. The phenotype in these patients was MZ, MS, or SZ. PAS-positive granules were present in liver of all patients with the ZZ phenotype and in none with other phenotypes. The findings indicate that manifestations and prognosis of this inherited liver disease are extremely variable.

Adolescent↗
PubMed1976

[Intravascular coagulation and severe hepatic failure in infants].

Diagnostic criteria for disseminated intravascular coagulation occurring in severe liver failure were reviewed by analyzing 11 pediatric cases. In this series, intravascular coagulation was often latent, but became overt following inconsiderate administration of procoagulant concentrates. Exchange-transfusion of infusion of fresh-frozen plasma, with or without addition of heparin, appeared to be the best mode for correcting potential bleeding tendencies.

Blood Cell Count↗
PubMed1975

[Alpha-1-antitrypsin deficiency in children: liver ultrastructure and speculations (author's transl)].

Fourteen liver biopsies from twelve young patients with liver diseases associated with homozygous, PiZZ phenotype, alpha-1-antitrypsin deficiency in their sera were examined by electron microscopy. In all these biopsies characteristic homogeneous material was found in some hepatocytes and corresponded, when observed on adjacent semithin sections by light microscopy, to the deposit stained by periodic acid Schiff reaction. The accumulation in perinuclear spaces resulted in intranuclear invaginations, but the major deposit was located in lumens of the endoplasmic reticulum. The limiting membranes were rough and smooth but the extent of the latter was so large that only this type of reticulum seemed peculiarly involved in the accumulating process. On the contrary, Golgi complexes did not seen obligatorily involved by this process because, when observed, they appeared almost normal even in heavily overloaded liver cells. At least for the PiZZ phenotype, the abnormal substance would be an asialo form of normal alpha-1-antitrypsin. Thus the subject of this study is the morphologic translation of an impairment in the synthesis of a glycoprotein. In the light of data concerning the synthesis of such proteins our findings lead us to suggest: The ultrastructural patterns observed in alpha-1-antitrypsin deficiency cannot give the expected morphologic evidence of the biochemical data which locate the first binding steps of monosaccharide residues in the rough endoplasmic reticulum. The absence of sialic acid could not result from an enzymatic defect primarily located in Golgi complexes but could be secondary to an impairment in the binding of one monosaccharide residue which improves subsequent fixation of sialic acid, in the smooth endoplasmic reticulum. Finally it seems necessary to emphasize that the relationship between the abnormal substance and various important non specific lesions is largely unknown and that we don't know the significance of polymorphous dense bodies observed in ductular cells during the cholestatic period.

Cell Nucleus↗
PubMed1975

Severe viral hepatitis type B in infancy;.

Fourteen infants aged from 2 to 5 months were admitted to hospital with acute viral hepatitis. Their clinical presentation ranged from severe disease to fulminant hepatitis. In all patients the prothrombin-time was 10% or less of normal and serum glutamic pyruvic transaminase and bilirubin were increased. In eight cases liver-biopsy specimens were obtained during liver failure and showed a widespread necrosis without inflammatory cells. Hepatitis-B-surface antigen (HBSAg) and antibody (HBSAb) were sought by several techniques, including passive haemagglutination and radioimmunoassay. Hepatitis was associated with hepatitis-B virus in eleven out of fourteen patients as judged by the detection of HBSAg and/or a secondary rise in HBSAb. In eight cases, the infants had received blood-derivatives in the neonatal period. The mothers of five of the remaining cases were found to be chronic carriers of HBSAg. Despite intensive supportive therapy, including repeated exchange transfusions and administration of anti-HBS gamma-globulins (six cases), eight patients died. These cases demonstrate that severe or fulminant type-B hepatitis can develop in infants, who are capable of completely eliminating the hepatitis-B virus. They also suggest that severe hepatitis can result from maternal contamination.

Alanine Transaminase↗