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Biomedical subjects

M Gautier

Publications and source records attributed to M Gautier.

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

Glycine transport by cultured skin fibroblasts from a patient with isolated hyperglycinuria.

Glycine transport is studied in cultured skin fibroblasts from a patient with isolated hyperglycinuria and from five normal subjects. Fibroblasts from the patient take up glycine less well than do cell lines from controls. Kinetic studies are consistent with a single transport system in the patient's and controls' cell lines. Vmax value in the hyperglycinuric lines is normal, but the apparent affinity is always reduced as opposed to those of four different control lines separately tested. Statistical analysis shows significant difference between Km values.

Amino Acid Metabolism, Inborn Errors↗
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

The alternate program; an alternative for chronic care.

This paper describes a 2 1/2 year pilot project for long term schizophrenic patients. It describes the kind of patients involved, the program itself, the role of the staff, the results and finally the therapist's reflections working with chronic patients for long periods of time.

Chronic Disease↗
PubMed1979

Study of some enzymatic activities in human liver cell cultures.

Some enzymatic activities were studied in long ter cultures of human liver cells : glucose-6-phosphatase, U.D.P. glucuronosyltransferase, phenylalanine 4-hydroxylase and tyrosine aminotransferase. Only weak tyrosine aminotransferase activity has been found in 12 subcultures, and it has not been increased by addition of corticoids. This tyrosine aminotransferase activity has been measured at different passages of the culture. Our results are compared with those found in literature. The different reasons which could explain the absence of liver specific biochemical functions have been discussed.

Cell Survival↗
PubMed1979

[Abnormalities associated with extrahepatic bile duct atresia].

In 19 cases of extrahepatic biliary atresia there were other malformations, 9 of which could be included in the polysplenia syndrome. The importance of these associated with malformations for the early diagnosis of extrahepatic biliary atresia and for eventual surgery for secondary portal hypertension is emphasized. The histological findings of the fibrous remnants of the biliary system are similar in those with and without associated malformations. Thus, these abnormalities do not contribute of the understanding of the pathophysiology of extrahepatic biliary atresia but they do suggest that the initial insult may occur early in pregnancy.

Abnormalities, Multiple↗
PubMed1979

[Atresia of the extrahepatic bile ducts. Etiological hypothesis founded on a histological study of 130 fibrous remnants].

The hypothesis which might explain the congenital absence of extrahepatic bile ducts are reviewed from the study of samples obtained at surgery in 240 children with extrahepatic biliary atresia, and more especially from the pathologic study of 130 fibrous remnants taken as the first step of hepato-porto-enterostomy. This study allows the following pathophysiologic hypothesis: Epithelial damage occurring before birth and always at the same site in the biliary bud that has undergone normal migration and differentiation could be responsible for an atresia. The lesion could be due to abnormal vascularisation with the consequences similar to those observed in other hollow viscera. Below the lesion, bile retention would cause epithelial destruction and an inflammatory infiltrate as in auto-immune reactions. The portal fibrosis which progresses despite restoration of bile flow would be the result of stimulation of growth of the portal mesenchymal axis, which embryologically is only an extension of the fibrous remnant. This hypothesis could explain why it has never been possible to reproduce the condition exactly in animals because all experiments have been performed after birth.

Bile Ducts↗
PubMed1978

Effect of cycloleucine on amino acid accumulation by human diploid fibroblasts.

Cycloleucine is a synthetic amino acid which produces, in vivo, biochemical abnoramlities comparable to those seen in human cystinuria-lysinuria. The effect of cycloleucine on intracellular accumulation of amino acids overlapping separate transport systems was studied using human diploid fibroblasts subcultures on glass coverslips. The data indicated that alpha-alanine, serine and proline accumulation was inhibited significantly by cycloleucine. The percentage of inhibition was approximately the same. Lysine was less affected by cycloleucine, but this amino acid accumulation proceeded at a rate slower than for neutral amino acids. In vitro, this inhibitory effect seems to be a generalized phenomenon affecting substrates. These results confirm in human fibroblasts data reported for human and rat kidney slices.

Amino Acids↗
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

[The myocardiopathies of glycogenosis].

Thirty-three patients with glycogen abnormalities and myocardial disease were studied. 27 of them has type II glycogen disorders (Pompe's disease, with an intralysozymal deficit of acid maltase) and 6 with type III glycogen disorders Forbes disease, with a deficit in amylo-1-6-glucosidase). The picture of a type II abnormality in the infant is very standard: early onset, often neonatally; the association with asystole and muscular hypotonia and a characteristics clinical picture; invariable cardiomegaly and typical ECG findings (short PR interval, high voltage complexes). Death occurs before one year of age, treatment has limited effect, and attention is centred on the early discovery of heterozygotes and of diagnosis antenatally. The possibility of an obstructive type (4 out of 24) and a type with endocardial fibroelastosis (3 out of 24) must be emphasised. In the late onset myopathic form of type II disorder (3 cases), involvement of the myocardium is always found, but is of secondary importance in determining the clinical picture and natural history. The same can be said of type III disorders in which, despite the infrequency of asystole or significant cardiomegaly, a hypertrophic cardiomyopathy which may be obstructive can lead to sudden death in infancy (2 cases out of 6).

Angiocardiography↗
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

[Cholesterol ester storage disease in children. Comparative biochemistry of hepatocyte and fibroblast cultures].

The results of biochemical studies in three children with cholesterol ester storage disease are reported. This rare disease (13 published cases) and the related Wolman's disease are characterised by a deficiency of acid lipase. Affected children mostly present with isolated hepatomegaly. Hepatic cells (one patient) and fibroblasts (two patients) were cultured and cholesterol accumulation measured. Hepatic cells contained more cholesterol than fibroblasts but the enzyme deficiency, assessed by the abnormal degree of esterification was the same in both cell types.

Child↗
PubMed1977

Children sea-blue histiocytosis (2 cases) compared with phospholipidosis induced by 4-4' DET (4-4' p (diethylamino-2-ethoxy phenyl) 3-4 hexane) in rat.

Two cases of children with liver and spleen enlargement are reported. Sea-blue histiocytes and Pick cells were found in both cases in liver, spleen, bone marrow and blood. Further more, lysobisphodphatidic acids were identified in phospholipid analysis of liver biopsies and cultived liver cells. Absence of neurologic involvement at 14 and 18 years fo age suggest a Crocker type C of Niemann-Pick disease, i.e. a not yet well defined entity. Resemblance of these morphological and biochemical abnormalities with certain cases of drug poisoning (especially the well-known intoxication by 4-4' DET) is discussed on the basis of results from experimental studies with this drug in the rat.

Adolescent↗
PubMed1977

Effects of serum-free culture media on human liver and fibroblastic cells.

The morphological aspects and amino acid variations of human fibroblast and liver cell monolayer cultures were studied in serum free media. Under these conditions, the behaviour of the two cell types differed greatly. The morphological changes for the liver cells, as compared with the fibroblasts, appear more quickly and some of these changes are particular. Furthermore, extracellular amino acid variations are of lesser importance for the liver cells, during the 10 days following the suppression of serum, except for serine.

Amino Acids↗
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

Comparative study of extracellular amino acids in culture of human liver and fibroblastic cells.

Amino acid concentration are studied in the extracellular media of ten series of human fibroblast and liver cell monolayer cultures. These two cell types consume and produce ostensively the same amino acids. Among the nonessential amino acids, the most significant variations involve serine and aspartate which are decreased; alpha-alanine, glutamate, ornithine and proline are, on the contrary, increased. Among the essential amino acids, leucine, isoleucine and glutamine are preferentially decreased. The variations of some amino acids are correlated with the cell density. The interrelations which may exist between the variations of these different amino acids are discussed. Furthermore, the glycolytic acitivity of the cells studied is very high: 85% of glucose consumed is found in the form of lactate.

Alanine↗