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Biomedical subjects

M Hadchouel

Publications and source records attributed to M Hadchouel.

At least 109 records · Page 6Linked to original sources
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

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↗
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

Hepatitis B virus DNA in children's liver diseases: detection by blot hybridisation in liver and serum.

Molecular hybridisation using cloned hepatitis B virus DNA (HBV DNA) was applied to liver and serum samples from 46 children (39 with liver diseases and seven controls) for detection of HBV DNA sequences, free and integrated into the liver cell genome. HBV DNA integration was observed in 10 children. The young age of some of these cases indicates that such integration can occur early in liver disease and is not related to the duration of viral infection. Thirteen children exhibited serological evidence of active viral multiplication. All but one had free HBV DNA in liver tissue and integrated HBV DNA sequences were found in four cases. Integrated HBV DNA sequences alone were also detected in three children with neither HBV-antigens nor HBV DNA in serum. One had inactive cirrhosis, and the two others, chronic active hepatitis. Consequently DNA hybridisation may be useful for diagnosis, in the absence of serological signs of HBV infection; its specificity was enhanced in the present investigation by negative results in six children with autoimmune chronic active hepatitis. Taken together, the above results imply that HBV-DNA integration can occur in both active and inactive liver disease. Integrated HBV DNA was also observed in the liver of three children with fatal hepatic failure who presented with antibodies to HBsAg and/or to hepatitis B core antigen in the serum. This finding raises the question of the relationship between the host immune factors and the state of HBV DNA.

Adolescent↗
PubMed1982

Latent chronic cholangitis in congenital hepatic fibrosis.

In a five-year-old girl with congenital hepatic fibrosis raised erythrocyte sedimentation rate and serum gammaglobulins were noted for 3 years. Needle liver biopsy showed histological features of cholangitis and Klebsiella oxytoca was cultured from a liver biopsy specimen. Antibiotic treatment resulted in normalization of erythrocyte sedimentation rate and serum gammaglobulins. This suggest that cholangitis can be clinically latent in congenital hepatic fibrosis and should be investigated and treated when unexplained inflammatory signs are present.

Child, Preschool↗
PubMed1982

Infantile phytanic acid storage disease, a possible variant of Refsum's disease: three cases, including ultrastructural studies of the liver.

Three cases of phytanic acid storage disease with symptoms during the first months of life are reported. Hepatomegaly, facial dysmorphia, growth and/or mental retardation and osteopenia were observed in addition to retinitis pigmentosa and neurosensory deafness. The presence of phytanic acid in serum (160-320 mumol/1 (50-100 micrograms/ml)) was accompanied by hypocholesterolaemia. Electron microscopy showed that a storage material had accumulated in mesenchymal and parenchymal liver cells. Lamellar structures were seen in hepatocytes and other storing cells. These inclusions resembled the structures found in plant chloroplasts containing phytol. Some of the clinical and biological data obtained were consistent with Refsum's disease. However, other characteristics such as mental retardation, hepatomegaly, osteopenia, hypocholesterolaemia and hypoalphalipoproteinaemia, as well as the ultrastructural findings in the liver, suggested that our patients' illness was either a phytanic acid storage disease different from the classical form of Refsum's disease, or a more severe early symptomatic form of Refsum's disease. Early diagnosis by phytanic acid assay and electron microscopic liver examination calls for prescription of a low phytanate diet in the hope of improving the child's condition.

Child↗
PubMed1981

Detection of hepatitis B virus DNA in liver and serum: a direct appraisal of the chronic carrier state.

The detection of hepatitis B virus (HBV) DNA in the liver and the serum permits direct study of the interaction between the virus and the liver cell. 40 HBV chronic carriers were studied by the blot technique of Southern to detect HBV DNA, and the results were compared with the serological status and histological status of the patients. It was possible to define two different chronic carrier states. The first is characterised by free viral DNA in the liver, with viral DNA and hepatitis B e antigen (HBeAG) in the serum; integrated HBV DNA is also present, at least in some patients. The second carrier state is characterised by the presence of only integrated HBV DNA sequences in the liver: viral DNA and HBeAg are not present in the serum. In one HBeAg-negative patient, however, free HBV DNA was detected in the liver and HBV DNA was present in the serum. The hybridisation technique appears to be a very sensitive test which could reflect viral multiplication better than HBeAg radioimmunoassay. Since a needle biopsy sample provides sufficient tissue for the Southern blot technique, it should be useful in understanding chronic hepatitis, the selection of patients for antiviral therapy, and the estimation of its efficiency.

Autoradiography↗
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

State of hepatitis B virus DNA in hepatocytes of patients with hepatitis B surface antigen-positive and -negative liver diseases.

Using the Southern blot technique and cloned hepatitis B virus (HBV) DNA as a probe, we studied the state of HBV DNA in the liver of 13 patients with hepatocellular carcinoma, 17 patients with chronic hepatitis, and 2 patients with acute hepatitis. The hybridization results were compared with the serological and immunohistological data. Integration of HBV DNA in cellular DNA of the liver from patients with hepatocellular carcinoma was demonstrated. In two patients from which tumorous and nontumorous liver tissue samples were available the integration patterns were different. In one patient with hepatitis B e antigen (HBeAg)-positive early hepatocellular carcinoma, free viral DNA was present in the liver. In some patients with HBeAg-negative chronic hepatitis, without tumor, integration of HBV DNA in cellular DNA was also demonstrated. This suggests that HBV is not the only factor involved in the development of a tumor. In patients with HBeAg-positive chronic hepatitis, free viral DNA was detected in the liver. In the two acute hepatitis patients analyzed, the restriction endonuclease patterns strongly suggested HBV DNA integration. Therefore, viral DNA integration seems to occur early in infection. Whatever the form of the disease, discrete bands were observed, suggesting the existence of limited and specific integration sites in host cellular DNA. The presence of integrated or free DNA sequences has implications for antiviral therapy. In addition, detection of HBV DNA in the liver is another sensitive viral marker that could be useful for diagnostic purposes.

Carcinoma, Hepatocellular↗
PubMed1981

Immunoglobulin deposits in the biliary remnants of extrahepatic biliary atresia: a study by immunoperoxidase staining in 128 infants.

Bile duct remnants taken from 128 infants during surgery for extrahepatic biliary atresia were paraffin embedded prior to immunoperoxidase staining. Immunoglobulin deposits were found in 44 remnants. They were made up of IgM alone in 25 cases and of IgM and IgG in 19. Deposits were observed along the basement membranes of glandular structures. These findings suggest that extrahepatic biliary atresia might be an acquired and evolving disease.

Bile Ducts↗