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Biomedical subjects

J Sarles

Publications and source records attributed to J Sarles.

18 recordsLinked to original sources
PubMed1992

[A rare cause of neonatal hyperphenylalaninemia: tyrosinemia type I].

Phenylalanine, the direct precursor of tyrosine, is rarely increased in tyrosinemia I. We report on a case of tyrosinemia type I diagnosed through neonatal hyperphenylalaninemia. This case report points out the need for a biological evaluation of the hepatic function in cases of neonatal hyperphenylalaninemia.

Amino Acid Metabolism, Inborn Errors↗
PubMed1992

[Current techniques for investigating exocrine pancreatic function in children].

The exocrine pancreas produces many of the enzymes responsible for the digestion of food. Severe alterations in exocrine pancreas function cause malabsorption which predominantly affects fats. Unfortunately, because it is a deep organ the pancreas is a difficult target for investigations. A large number of diagnostic tests have been developed to gather information on pancreatic function with minimal invasiveness. Although helpful in everyday practice, each of these methods investigates only one of the multiple components of pancreatic secretory function and all are relatively insensitive, i.e., detect only severe secretion deficiencies. Furthermore, none of these tests can evaluate water and electrolyte secretion. Consequently, invasive duodenal juice studies with stimulation remain the "gold standard" for evaluating exocrine pancreas function.

4-Aminobenzoic Acid↗
PubMed1990

Hepatic hypervitaminosis A: a familial observation.

Four siblings with hepatic fibrosis are described. The liver damage in these patients was secondary to chronic ingestion of massive doses of vitamin A for congenital ichthyosis. Although the extrahepatic manifestations were helpful in the diagnosis of hypervitaminosis A, the distinctive features of hepatic histopathology were confirmatory. The plasma concentrations of vitamin A and retinol-binding protein were misleading. The recovery from the liver damage in these patients was slow despite a complete withdrawal of the vitamin A intake. These cases show the importance of hepatic vitamin A assessment in the diagnosis of hepatic fibrosis.

Adult↗
PubMed1990

[Exocrine pancreas diseases in children].

Except cystic fibrosis, disorders of the exocrine pancreas are uncommon in the child; however they are numerous and varied in terms of their pathogenesis and clinical manifestations. The author surveys the various diseases of this organ in pediatrics. More recent theories concerning pancreatic involvement in cystic fibrosis are discussed.

Child↗
PubMed1989

[Chronic diarrhea with hypergastrinemia and achlorhydria without gastritis in a 5-year-old child].

We report a case of chronic diarrhea due to hypergastrinemia with achlorhydria but without gastritis in a five-year-old boy. Symptoms responded promptly to oral administration of hydrochloric acid and resolved completely after one year of treatment. The pathophysiologic situation in this patient closely resembled that normally seen in neonates, suggesting prolonged but nevertheless transient immaturity of gastric secretions.

Achlorhydria↗
PubMed1988

The effect of cholecystokinin-octapeptide, insulin, glucagon, triiodothyronine, and epidermal growth factor on amylase activity in fetal pancreas in vitro.

To evaluate the possible role of various hormones on fetal pancreas development, late gestational fetal rat pancreata (20 days) were cultured in a serum-free medium for 6 days in the presence of cholecystokinin-octapeptide (CCK-8), epidermal growth factor, triiodothyronine, or glucagon with or without dexamethasone. In the absence of any added hormone, the tissue amylase activity declined very rapidly. Epidermal growth factor alone (4.10(-7) M) could not preserve the amylase activity, whereas triiodothyronine (0.1 microM) and glucagon (4 micrograms/ml) had a deleterious effect that was prevented by the addition of DXM (3.10(-6) M). In the presence of CCK-8 (2.10(-11) M) 50 and 30% of the amylase activity was maintained on the 2nd and the 4th day of culture, respectively. The CCK-8 effect was dose dependent and was inhibited by asperlicin (10 microM). The combination of CCK-8 and dexamethasone maintained more than 80% of the amylase activity in the fetal pancreas explants through 4 days of culture. Fetal pancreas cultured in this optimal medium and treated with streptozotocin (10(-7) M) during the 1st day of culture showed a significantly lower tissue amylase activity on the 4th and 6th days than those not treated with streptozotocin. The streptozotocin effect was attenuated when insulin (0.1 U/ml) was added. These data suggest that, in addition to the well-known effect of glucocorticoid on enzyme activities in the fetal pancreas, two additional hormones, CCK and insulin, could play a role in the modulation of pancreatic amylase activity in the fetal rat.

Amylases↗
PubMed1985

Aminopeptidase N- and human blood group A-antigenicity along the digestive tract and associated glands in the rabbit.

The cellular localization of an aminopeptidase N homologous to the brush-border intestinal enzyme and that of human blood group A-substances were investigated using the immunofluorescence technique on thin frozen sections (200 nm) of the digestive tract and associated glands of A+ and A- rabbits. Aminopeptidase N was found to be a common specific marker of both the apical region of plasma membrane of acinar cells in submaxillary and parotid glands and pancreas and the brush border of jejunum and colon absorbing cells. In hepatocytes, the enzyme was localized in the sinusoidal domains. Soluble A-substances were present in mucus secretory granules of intestinal goblet cells and those of stomach and gall bladder mucous cells. In contrast, the mucous acini of sublingual and submaxillary glands were devoid of A-antigenicity. The columnar cells of striated ducts of these glands exhibited A-antigenicity. Soluble A-substances were also found in zymogen granules of parotid and pancreas acinar cells and those of stomach chief cells. Moreover, in all cells secreting A-substances, and in the non-secreting absorbing intestinal cells, the glycoproteins of the plasma membrane bore A-determinants. Aminopeptidase N was one of the membrane-bound glycoproteins that bore A-determinants in cells that expressed A-antigenicity.

ABO Blood-Group System↗
PubMedDate not supplied

Subcellular localization of class I (A,B,C) and class II (DR and DQ) MHC antigens in jejunal epithelium of children with coeliac disease.

Thin frozen sections of 11 jejunal biopsies from 10 children at different stages of coeliac disease were stained by immunofluorescence technique using a panel of anti-HLA class I (A, B, C) and anti-HLA class II (DR and DQ) monoclonal antibodies. On the epithelium of flattened mucosa, in contrast with control sections, the intensity of the labeling on the basolateral membranes with both anti-class I and class II DR antibodies decreased strongly from the bottom to the upper part of the crypts, and no bright patchy staining was observed on the apical part of enterocytes with anti-HLA DR antibodies. Numerous cells with large granules expressing class I and class II DR antigens were found in the epithelium of the small intestine. Children with a fully recovered mucosa expressed MHC antigens identical to those previously observed in normal epithelium. On the other hand, children with intermediate mucosal lesions showed the presence of MHC antigens in varying degrees. The results of this report indicate that immunological mechanisms may play a prominent role in coeliac disease.

Celiac Disease↗
PubMedDate not supplied

Human gastric lipase: ontogeny and variations in children.

The aim of this study was to investigate the precise origin of the acid pre-duodenal lipase during human development and to evaluate its possible changes, at the tissue level, in children with gastritis or pancreatic insufficiency. Human gastric lipase appears around the 11th week of gestation and increases slowly during pre- and postnatal development. It is localized in the fundus of the stomach without any lingual localization. Human gastric lipase reaches its adult level in the third month of life, and does not vary in relation to pancreatic insufficiency. It is only rarely impaired during gastritis.

Adolescent↗