A study of specific and non-specific immune functions was performed in 14 children presenting with severe malnutrition, before and after parenteral hyperalimentation by central catheter. Anomalies of cellular functions (reduction of the percentage of E rosettes and deficient proliferation with mitogens) were rarely found. Measurements for serum immunoglobulins did not show any anomaly; however reduced percentages of EAC rosettes and important increase in "null" cells were found in about one third of the cases. The main finding was a decreased serum chemotactic activity following activation of the classic complement pathway. This was found in 12 children and returned to normal values in all cases after hyperalimentation.

Chemotactic Factors↗

PubMedDate not supplied

[In vitro intestinal cytotoxicity of various fractions from gliadin or cows's milk proteins in children: quantitative assessment by a morphometric method].

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Adolescent↗

PubMedDate not supplied

[Chronic neutropenia and Crohn's disease in childhood. Report of 2 cases ].

Two children in whom Crohn's disease complicated the course of chronic neutropenia are reported. These two cases had other features in common: consanguinity of the parents, relatively good clinical tolerance of neutropenia and almost complete lack of blood and marrow eosinophils. The possible relationships between the 2 diseases are discussed.

Adolescent↗

PubMedDate not supplied

[Hyperphenylalaninaemia with normal phenylalanine-hydroxylase activity and a deficiency of tetrahydrobiopterin and dihydropteridine reductase].

Two cases of hyperphenylalaninemia with a normal activity of phenylalanine hydroxylase are described. No activity of DHP reductase was found in the first case, having very high biopterin levels in basal conditions and after intravenous perfusion of phenylalanine. In the other case, the DHP reductase activity is normal but plasma and urinary levels of the reduced forms of biopterin are largely lowered and do not increase during the phenylalanine load. Early substitutive treatment with L-dopa and 5-HTP in one of the cases avoided the development of the "progressive neurological illness unresponsive to dietary treatment" characterizing two variants. This raises the question of a liver biopsy in order to assay the hydroxylation enzyme activities when screening hyperphenylalaninemia whatever the type.

Biopterins↗