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Villous atrophy in an infant immediately suggests food intolerance. We report a case with an unusual cause. This female infant was first examined at 5 months for anorexia and failure to thrive. Intestinal biopsy showed total villous atrophy. A diet excluding gluten and cow milk proteins failed to improve her condition and the infant was hospitalized at 7 months for further investigations. The infant was hypotonic with a head lag. No other clinical sign was noted. Serum transaminases were increased 5- to 10-fold and CSF proteins concentration was increased. Metabolic investigations revealed hyperlactacidaemia and an increased lactate/pyruvate ratio during fasting and feeding, suggesting a mitochondrial cytopathy. Respiratory chain enzymatic activity measurements confirmed the diagnosis and showed severely decreased activities of complexes I, III and IV in both the liver and muscle. Molecular analysis demonstrated depletion of mitochondrial DNA in the liver (75%) and in muscle (97%). The infant was discharged under continuous enteral nutrition. Improvement was of short duration and the infant died at 1 year of age of massive hepatic failure. This is the first report of a mitochondrial DNA depletion with total villous atrophy and malabsorption as early clinical onset. A mitochondrial cytopathy should be considered in such conditions when food exclusion diets fail.

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INTRODUCTION: Fibreoptic bronchoscopy (FB) is an important diagnostic examination in paediatric pulmonology. In 2002 the Paediatric Pulmonology and Allergy Club undertook a retrospective study to establish the current status of fibreoptic bronchoscopy among its members. METHODS: In 2001 sixty five paediatric pulmonologists carried out an average of 116 examinations (+/- 111) in 35 paediatric centres. FB was performed either in an operating theatre (15 centres), a dedicated bronchoscopy suite (6 centres) or an endoscopy suite shared with gastro-enterologists (7 centres). Other examinations were performed in areas dedicated to, or associated with intensive care. General anaesthesia was routinely used in 18 centres. The others used sedation including an equimolar mixture of oxygen and nitrous oxide in 14 centres. Ten centres performed less than 50 examinations, 12 between 51 and 100, 4 between 101 and 200 and 8 centres more than 200 in the year. Seventy two per cent of the children were less than 6 years old. The washing and disinfection procedures were manual in 20 centres and automatic in 15. RESULTS: Three principal indications were reported: persistent wheezing, suspicion of a foreign body and ventilatory difficulties. Cough, desaturation and fever were the most frequently reported side effects. CONCLUSIONS: This is the first survey in paediatric pulmonology in France. It shows a wide variation in the practice of fibreoptic bronchoscopy in children.

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UNLABELLED: The incidence of nosocomial rotavirus infections was evaluated by a study made in the pediatric ward in Cholet during the winter of 1993-1994. A second study was performed three years later at the same place and in similar conditions in order to evaluate the efficacy of the prevention measures taken in between. PATIENTS AND METHODS: All children below three years of age and admitted to hospital between 1 December 1996 and 15 March 1997 were included in the prospective study (348 children). Fecal specimens were collected for each patient at admission in order to search for rotavirus. Then, a second stool analysis was performed if diarrhea occurred during hospitalization or within 48 hours of discharge. These last cases were detected by a phone call. RESULTS: Thirty percent of the children had diarrhea at admission to hospital (19.3% in 1993-1994). Rotavirus was present in 11.8% of the first stool analyses (8.6% in 1993-1994). The rotavirus nosocomial infection rate has decreased from 3.7% (13 cases) in 1993-1994 to 2.9% (ten cases). The mean length of hospitalization has also decreased from 2.7 to 1.6 days. CONCLUSION: The decrease in the rotavirus nosocomial infection rate leads to enforcing the prevention measures, among which the most important seems to us to be the short length of stay. However, the study also shows the limits of prevention that are linked to the virus characteristics and the conditions of hospitalization.

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A case of hypothalamic dysfunction in a girl with a twelve-year follow-up is reported. Onset occurred at the age of three with severe obesity, hypothermia, hypersomnia, and lethargy. Somatotropic, gonadotropic, and thyrotropic hormones were low, whereas prolactin was increased. Imaging techniques failed to disclose any lesion of the hypothalamus or pituitary. Clomipramine improved the vegetative disorders. The literature on clinical and hormonal disorders of hypothalamic dysfunction is reviewed.

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BACKGROUND: Conventional insulin therapy for diabetes mellitus is sometimes inadequate for controlling metabolic disturbances in young children; the risk of hypoglycemia is particularly high at this age. METHODS: Ten newly-diagnosed insulin-dependent diabetic children aged 1 to 7 years were treated by continuous subcutaneous insulin infusion using a portable pump. The mean duration of treatment was 17 months (4 to 42 months). RESULTS: The mean individual capillary blood glucose calculated over periods of 6 months ranged from 84 +/- 23 to 206 +/- 97 mg/dl. The glycosylated hemoglobin values were 6.5 to 8.9% during the same period. There was no hypoglycemic coma; 7 children experienced a total of 16 episodes of ketonuria. The average daily dose of insulin used by these 10 patients was 0.72 +/- 0.24 units/kg. CONCLUSION: Continuous subcutaneous insulin infusion is a feasible therapy and it was well tolerated even in the youngest children. External insulin infusion devices appear to be an alternative to conventional insulin therapy and a way of overcoming the therapeutic difficulties encountered with these young patients.

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Insulin-like growth factor I (IGF I) is like prealbumin and transferrin a marker of nutritional status. Its level increases with gestational age. The levels of IGF I (96 times), transferrin (86 times) and prealbumin (69 times) were measured in blood samples from 26 premature infants aged 8 to 78 days (gestational age: 28 to 34 weeks, birth weight: 840 to 1,800 g). At the time of sampling, all the infants were on total parenteral nutrition (360 +/- 42 kJ/kg/day and 2.5 +/- 0.3 g of proteins/kg/day). The results were analysed with reference to anthropometric parameters (weight, height, head circumference, skinfolds and arm circumference). There was no correlation between plasma IGF I and anthropometric measurements. There were significant correlations between IGF I and transferrin (p less than 0.01), prealbumin (p less than 0.05), protein intake (p less than 0.01) an energy intake (p less than 0.05). Plasma IGF I increased at the end of the first week of parenteral nutrition in all the 5 infants having initial low values. The plasma IGF I was not correlated with the duration of parenteral nutrition in the 26 infants after the second week of nutrition. IGF I measurement is useful for evaluating the protein nutritional status of premature infants on total parenteral nutrition.

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