[Congenital hyperinsulinism in 15 infants, 1981-1999; experiences and new insights]. / Congenitaal hyperinsulinisme bij 15 kinderen, 1981-1999; ervaringen en nieuwe inzichten.

OBJECTIVE: To report on a retrospective study into the diagnostics and treatment of infants with congenital hyperinsulinism (CHI; persistent hyperinsulinemic hypoglycaemia). DESIGN: Retrospective and descriptive. METHOD: The study included all 15 patients diagnosed with CHI at the St Radboud University Medical Centre, the Netherlands, from 1981 until 1999. Data gathered by systematically searching case-notes included: presentation, clinical admission, laboratory results, treatment and follow-up. RESULTS: Four of the 15 infants were macrosomatic; 12 (80%) were presented within 4 days of birth, and the rest after the age of 5 months. Their symptoms were partially aspecific (feeding poorly, lethargy) and partially clear, corresponding to neuroglycopaenia (jitteryness, hypotonia). Nine infants experienced convulsions. The amount of glucose that had to be administered to achieve normoglycaemia (average: 16.9 mg/kg/min) was far above the basal requirement of 4-8 mg/kg/min. Ketone serum and free fatty acid values were lowered during a hypoglycaemic episode, hyperinsulinism was detected after repeated measurements. Five infants responded well to treatment with diazoxide. Ten children underwent subtotal pancreatectomy after which 4 remained normoglycaemic. Three of the 10 children who underwent surgery developed an exocrine pancreas dysfunction. We did not systematically examine neuropsychological development, but in 5 of the 15 children this was clearly disturbed.

The CD11b/CD18-integrin in the pathogenesis of psoriasis.

The aim of the present investigation was to study the distribution of CD11b, CD18, and ICAM-1 over the various cell populations present in the margin zone of the actively spreading psoriatic lesion and distant uninvolved psoriatic skin. This study was performed in order to obtain more insight in the relevance of these receptor molecules in the early phase of psoriatic plaque development. Skin biopsies were taken and inflammation markers were assessed using immunohistochemical techniques. All dermal changes in the margin zone preceded those in the epidermis. In the margin zone the most peripheral change was a decrease in dermal ICAM-1-expression, followed by an increase in dermal CD11b-expression. CD11b-positive cells showed a different tissue distribution from polymorphonuclear leukocytes and were more abundantly present, suggesting significant amounts of CD11b-bearing cell populations other than polymorphonuclear leukocytes in the early phase of psoriasis. Decreased numbers of CD18-positive cells in distant uninvolved skin suggest the existence of a selective modulating mechanism altering the trafficking of myeloid subpopulations. Polymorphonuclear leukocytes appeared to invade the skin well after the appearance of CD11b- and CD18-positive cells.