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Biomedical subjects

M Hadchouel

Publications and source records attributed to M Hadchouel.

At least 127 records · Page 7Linked to original sources
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

[A study of the abnormal polysaccharide in a child with type IV glycogen storage disease (author's transl)].

In type IV glycogen storage disease, the abnormal storage material is a partially amylase-resistant, PAS-positive polysaccharide and a deficiency of the branching enzyme is present in virtually all cases studied so far. Electron microscopic, biochemical and enzymatic studies were carried out in a child presenting with the clinical and histological features of this disease. Electron microscopic study showed the amylase-resistant material to be fibrillar and poorly soluble in buffers. Iodine spectrum analysis indicated that the lambda max of the liver polysaccharide was between that of normal glycogen and that of typical type IV glycogen. Branching enzyme activity was not detectable in the patient's leucocytes but was close to normal in the liver and clearly detectable in cultured fibroblasts. These results suggest that the absolute value of the deficiency of the branching enzyme in the liver of patients with type IV glycogen storage disease could be questioned. Alternatively this patient as well as another one reported in the literature could be considered as subtypes of the disease in whom liver and fibroblast branching enzyme activity remains detectable in vitro.

1,4-alpha-Glucan Branching Enzyme↗
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↗
PubMed1978

Reduced ratio of portal tracts to paucity of intrahepatic bile ducts.

The syndrome of "paucity" of intrahepatic bile ducts is characterized by a reduction of the ratio of interlobular bile ducts to portal areas. The present unidirectional study of liver biopsy specimens in cases of so-called intrahepatic biliary atresia, controlled essentially by age-matched autopsy control, showed that there is also a reduced number of portal areas in the livers of these subjects. This fact suggests an injury to the vascular anlage associated with biliary injury.

Bile Ducts, Intrahepatic↗
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↗
PubMed1976

An animal model for the study of human alpha-1-antitrypsin deficiency.

Turkeys with "round heart disease" often have a deficiency in alpha1 globulin. Within their liver cells, PAS +, diastase resistant granules are present, and are remarkably similar to those of persons with inherited AAT deficiency. The electrophoretic patterns made according Fagerhol's method showed differences between the serum of healthy birds and the turkeys suffering from "round heart disease".

Animals↗
PubMed1976

Histopathologic study of the liver in the early cholestatic phase of alpha-1-antitrypsin deficiency.

Liver biopsies obtained during the first six months of life were studied in 15 children who had prolonged neonatal cholestasis and alpha-1-antitrypsin deficiency (Pi ZZ). Intracellular PAS-positive globules were always observed, even during the first months of life. At this early stage of the disease, three morphologic patterns of hepatic alteration were distinguished. Group 1: six cases with cholestasis, hepatocellular damage, and slight portal fibrosis; Group 2: five cases with important portal fibrosis and bile duct proliferation; and Group 3: four cases in which ductular hypoplasia was the main feature. A correlation between histologic patterns and clinical course may be established: improvement of liver injury in Group 1, early cirrhosis in Group 2, and prolonged cholestasis in Group 3. The natural evolution of the disease appears to be different in each of the three groups.

Adolescent↗
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↗
PubMedDate not supplied

Detection of hepatitis B virus DNA in serum by a simple spot hybridization technique: comparison with results for other viral markers.

A simplified spot method for determination in serum of hepatitis B virus DNA (HBV DNA) by molecular hybridization is proposed. For simultaneous testing of 30 serum samples, it reduced to about 1 hr the duration of the steps preceding hybridization proper. The method also greatly reduced the loss of DNA during these steps and allowed more sensitive detection in samples of only 25 or 50 microliters. HBV DNA was determined in 181 serum samples by this method, and the results were pooled with 67 previous determinations by the Southern blot technique. Results for the pool were then compared to those obtained with radioimmunoassay for serological HBV markers. Ninety-six of the 248 samples were HBV DNA positive. Eleven others gave variable or inconclusive results, probably due to low viral particle titers. Seventy-two HBsAg- and HBeAg-positive sera contained HBV DNA, confirming that HBeAg is a marker of active viral replication. Fourteen other HBsAg- and HBeAg-positive sera, obtained from eight patients, were either HBV DNA negative or oscillated between negative and positive, or, again, were weakly positive; serological follow-up in 7 patients showed seroconversion to anti-HBe in 5, 3 of which became HBsAg negative. Eight of the HBsAg-positive sera were negative or borderline for HBeAg but contained HBV DNA and may, therefore, have been infective; seven of these sera had anti-HBe. Six HBsAg-negative sera contained HBV DNA and may also have been infective; five of these exhibited HBV antibodies. These results indicate that molecular hybridization not only provides a more sensitive and direct method for detecting hepatitis B virus in serum but also defines additional serological patterns with predictive or epidemiological value.

DNA, Viral↗
PubMedDate not supplied

Seroimmunologic classification of chronic hepatitis in 57 children.

A seroimmunologic evaluation of 57 children with chronic hepatitis is presented. Twenty-one patients had chronic persistent hepatitis and 36 had chronic active hepatitis. Serum samples obtained before treatment were tested for HBsAg, anti-HBs, anti-HBc, smooth muscle antibody, and antibody to endoplasmic reticulum. A persistently positive HBsAg was observed in the serum of 18 of the 21 patients with chronic persistent hepatitis. The chronic active hepatitis group was divided into three subgroups according to the presence of hepatitis B-virus markers (7 patients), smooth muscle antibody (10 patients), and endoplasmic reticulum antibody (9 patients). Determination of these markers could be useful for classifying children with chronic hepatitis.

Adolescent↗
PubMedDate not supplied

Pigment gallstones of the common bile duct in infancy.

Ten infants of less than 6 months of age presented with cholestatic jaundice and gallstones. Jaundice occurred after a lag period, and sepsis was present in three children. Ultrasound examination showed dilatation of intrahepatic and extrahepatic bile ducts in eight patients and detected cholelithiasis in three. Percutaneous transhepatic cholangiography and/or surgery allowed separation of the patients into two groups: (i) six children with lithiasis in the distal common bile duct, and (ii) four children with lithiasis associated with bile duct perforation at the junction between the cystic and common bile ducts with gallstones probably secondary to bile stasis and infection. Surgical treatment was confined to removal of calculi and drainage in eight children; biliary reconstructive surgery was necessary in the other two who had serious biliary duct lesions. No recurrence was observed after 1 to 7 years. The pigmentary nature of cholelithiasis was established by stone morphology in all cases, and by bile and stone analysis in several cases.

Bile↗
PubMedDate not supplied

A retrospective study of the role of delta agent infection in children with HBsAg-positive chronic hepatitis.

The prevalence of intrahepatic delta antigen and/or anti-delta antibody was retrospectively investigated in 102 children with chronic HBsAg-positive hepatitis who were seen consecutively in three medical institutions between 1974 and 1982. Delta infection markers were found in 13 patients (12.7%) who exhibited high serum titers of anti-delta antibody; intrahepatic delta antigen was detected in ten. Eleven of the 13 children had severe progressive liver disease associated in all but one with absence of hepatitis B virus replication as evaluated by analysis of serum hepatitis B virus DNA. The factors which seem to increase the risk of delta infection in children who are hepatitis B virus carriers are geographic origin, a history of exposure to blood derivatives and age. A further 37 of 102 children had chronic active hepatitis (20 patients) or cirrhosis (17 patients) without evidence of delta infection. These results indicate that delta infection occurs in children with chronic hepatitis. This possibility should be considered in investigation of children with HBsAg-positive chronic liver disease. Although the delta agent is an important cause of progressive liver disease in children who are chronic HBsAg carriers, severe liver injury and especially cirrhosis can occur without evidence of delta infection.

Adolescent↗
PubMedDate not supplied

Biochemical indicators of vitamin A depletion in children with cholestasis.

Biochemical indicators of vitamin A status were measured in 24 children (1 month to 6 years old) with severe cholestasis starting early in life and in 21 children (3 months to 13 years old) with liver disease but without cholestasis. Liver vitamin A concentrations, expressed as micrograms of retinol per gram of liver (mean +/- S.D.), were 6.3 +/- 7.1 (range: 0.14 to 28) and 143 +/- 108 (range: 18 to 424), respectively, in cholestatic and non-cholestatic children. In infants less than 6 months of age, liver vitamin A values less than 10 micrograms per gm were found in 14 of 17 cholestatic children but in none of 3 non-cholestatic subjects. Plasma vitamin A values, expressed as micrograms of retinol per deciliter (mean +/- S.D.), were 23 +/- 18 (range: 3 to 62) and 46 +/- 33 (range: 14 to 125), respectively, for the two groups. Plasma retinol values less than 10 micrograms per dl were always associated with liver concentrations less than 10 micrograms per gm. Plasma retinol-binding protein was only reduced to 71% of control values in cholestatic children. The fatty acid composition of liver retinyl esters was unaffected by any condition studied. Infants with chronic cholestasis are in a precarious nutritional status very early in life relative to liver reserves of vitamin A. Plasma vitamin A values, unless less than 10 micrograms retinol per dl, are poor indicators of inadequate vitamin A status.

Adolescent↗