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Biomedical subjects

M Gautier

Publications and source records attributed to M Gautier.

At least 109 records · Page 6Linked to original sources

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↗

Histologic study of biliary fibrous remnants in 48 cases of extrahepatic biliary atresia: correlation with postoperative bile flow restoration.

The fibrous remnants of bile ducts that were removed at surgery for extrahepatic biliary atresia were studied by serial section. Forty-eight specimens were classified into three types on the basis of histopathologic changes in the proximal portions of the speciments from the vicinity of the porta hepatis: (1) connective tissue without glands or other epithelial structures (15 cases); (2) connective tissue containing glands lined with cuboidal epithelium (15 cases); (3) connective tissue containing central biliary ducts and peripheral glands (18 cases). Histopathologic changes in sections from distal portions of the specimens did not conform to this classification. A precise correlation of prognostic significance could not be established between these histologic data and postoperative restoration of bile flow, although failure to restore flow was encountered most frequently in cases without demonstrable ducts or glands (TYPE 1).

Bile↗

[Results of Mustard's operation for transposition of the great vessels. Apropos of 81 cases of children who survived the intervention with a follow-up of from 1 to 6 years].

In a series of 81 children who survived to Mustard's operation, with a follow-up varying from 6 months to 6 years, there were 19% of secondary deaths, 19% of bad results, 29% of questionable results and only 33% of good results. The most frequent complications were: disturbances of cardiac rhythm and obstructions to pulmonary or caval venous return.

Adolescent↗

Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur.

Among 30 children with hepatic ductular hypoplasia and normal extrahepatic bile ducts, 15 formed a hemogeneous, readily recognizable group. In addition to chronic cholestasis, they have characteristic facies, a mesosystolic murmur, vertebral arch defects, growth retardation, mental retardation, and hypogonadism. Typical biochemical and histologic features aid in differentiation of this group from patients with other varieties of biliary disease.

Abnormalities, Multiple↗