Search PubMed⌕ Search

Biomedical subjects

M Odievre

Publications and source records attributed to M Odievre.

At least 55 records · Page 3Linked to original sources
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↗
PubMed1981

Liver glycogenosis caused by a defective phosphorylase system: hemolysate analysis.

Investigated were 24 cases of glycogenosis caused by a reduction in liver phosphorylase activity. The intravenous glucagon tolerance test could not discriminate between phosphorylase kinase deficiency [glycogen storage disease (GSD) IX] and phosphorylase deficiency (GSD VI). These two subgroups were distinguished by hemolysate enzyme assays: (1) GSD IX was characterized by a residual phosphorylase kinase activity, a low activation curve for endogenous phosphorylase b and increased amylo-1,6-glucosidase activity. (2) GSD VI was characterized by a normal or increased phosphorylase kinase activity, a slight activation of endogenous phosphorylase b and a normal amylo-1,6-glucosidase activity.

Child↗
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↗
PubMed1980

"Uncorrectable" extrahepatic biliary atresia: relationship between intrahepatic bile duct pattern and surgery.

Postmortem examination of intrahepatic bile ducts in the vicinity of the porta hepatis was performed in 26 patients. Fifteen of them (group I) had resection of fibrous remnants with or without biliary-intestinal anastomosis, and the remaining 11 (group II) had had exploratory laparotomy only or were not operated at all. The mean age at death was 10 and 7 1/2 mo, respectively, for each group. In group I, the diameter ranged from 1.5 to 15.0 mm (mean:5.23); the duct was dilated and contained bile concretions. In group II, the diameter of bile duct ranged from 0.2 to 1.2 mm (mean:0.63); the duct appeared flattened and its lumen was empty. Because a similar difference was observed in the patients of either group who died at 4 to 8 mo of age, previous surgical section of fibrous remnants may be responsible for enlargement of the intrahepatic bile duct. Whether section of nerves plays a role in this process remains to be determined.

Bile Ducts↗
PubMed1980

[Oculo-cutaneous tyrosinosis (Richner-Hanhart syndrome). Histo-pathological study of a case].

The Richner-Hanhart syndrome corresponds to a tyrosine elevation in serum due to deficit in soluble tyrosine aminotransferase in liver cells. This new enzymopathy which is transmitted in an autosomal recessive mode is called oculocutaneous tyrosinosis. It is curable by a poor diet in tyrosine and its precursors. The diagnosis has been invoked in a 18 months old girl, on the association of punctuate palmar and plantar keratosis, dentritic ulcerated keratitis, and mental retardation. The diagnosis is confirmed by elevation of tyrosinemia to 52 mgs/100 mls associated with a high urinary elimination of tyrosine and plenylcetonic acid. Absences of anomaly in the metabolism of methionin and hepatorenal absence of disturbance of hepatorenal system is characteristic. The keratosis accompany orthokeratotic hyperkeratosis. The keratinocytes show 2 types of anomaly ranged in strates in the epiderm. Intracytoplasmic vacuoles which include or lead to pseudomyelinic formations extend progressively from the mitochondrial alterations in the epidemial basal layers. Bulky polyhedral electron dense particles are found in the cytoplasm of the superficial keratinocytes. Most of these aspects have been demonstrated anteriorly in the keratinocytes and the cornea; on the other hand, signs of mitochondrial sulferance had not been observed. The genesis of these cellular alterations based on the liberation of lysosomial enzymes by the action of crystals of tyrosine has been suggested by Goldsmith from experimental facts. However, it seems the mitochondrial defect occurs outside this mechanism.

Female↗
PubMed1978

Lipolytic activity in milk from mothers of unjaundiced infants.

Lipoprotein lipase activity and free fatty acid concentrations are measured in samples of milk collected from 50 mothers of infants without prolonged neonatal jaundice; in many samples the values are in the same high range as observed in all samples collected from nine mothers of jaundiced infants. These findings are discussed with relevance to the problems of breast-milk jaundice and factors controlling the milk secretion.

Fatty Acids, Nonesterified↗
PubMed1976

[Bilirubin metabolism in the newborn. Recent progress].

The main stages of bilirubin metabolism and the present state of neonatal hyperbilirubinemia are reviewed. The heterogeneity of substrates for bilirubin synthesis and the regulation of the heme-oxygenase activity are stressed. The importance of albumin for the bilirubin transport and the factors modifying this transport are studied. The detection of free bilirubin and reserve bilirubin binding capacity in neonatal jaundice is discussed as a method to estimate the risk of kernicterus. The induction of bilirubin glucuronyl transferase activity with some drugs and the phototherapy are effective in the treatment of hyperbilirubinemia; their values are discussed from the most recent reports.

Bilirubin↗
PubMed1976

[Hereditary fructose intolerance with early onset].

Four cases of hereditary fructose intolerance with an early onset are reported. The features of acute liver failure in the neonatal period include a haemorrhagic syndrome, collapse, neurological features, hypoglycaemia, disturbed bleeding and clotting studies and abnormal liver function tests. Investigations into the aetiology include a search for bacterial or viral infection but particularly for a metabolic cause: especially for hereditary fructose intolerance which may be difficult to distinguish from tyrosinosis. Finally, methods of treatment are discussed: continuous glucose infusion, exchange transfusion, assisted ventilation, and dietary measures beginning with protein exclusion. The importance of careful observation is stressed (particularly sequential studies of bloodclotting factors).

Alanine Transaminase↗
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↗