Search

Infant small-bowel intussusceptions, most of the time idiopathic, may exceptionally reveal a severe digestive disease. We report the case of a 4-month-old infant who presented multiple and simultaneous ileal intussusceptions associated with severe acute gastroenteritis. Initially, the infant showed a protein-losing enteropathy with a clear alteration of the general state of health and undocumented fever, resistant to broad-spectrum antibiotic therapy. Skin and splenic nodules associated with hepatosplenomegaly and pancytopenia set in progressively. The etiologic evaluation led to the diagnosis of a Mycobacterium bovis BCG infection related to severe combined immune deficiency. The treatment consisted in anti-tuberculosis quadruple therapy in association with immunoglobin supplementation. Secondarily, the patient underwent gene therapy in a clinical trial. An early BCG vaccine in the first weeks of life, before the outbreak of infection revealing the immune deficiency, is a risk factor in triggering a disseminated BCG infection in immunodepressed infants. This clinical case is the first reported of severe combined immune deficiency revealed by small-bowel intussusceptions related to a disseminated BCG infection., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Objective: To study nutritional status in children with chronic bronchitis (CB) in relation with lung function., Methods: In this cohort of study, 46 patients aged 6.0 to 17.5 years (mean: 11.9 years) with chronic bronchitis were recruited. None had cystic fibrosis. Body weight, height, skinfold thicknesses, percentage of ideal body weight-for-height (percentage of IBW), body mass index (BMI), BMI Z-score, fat mass and fat-free mass were used to evaluate nutritional status. Arterial blood gases, vital capacity (VC), forced expiratory volume in one s (FEV1), functional residual capacity (FRC) and maximum inspiratory (Pi(max)) and expiratory (Pe(max)) pressures at the mouth were used to evaluate respiratory function., Results: Thirteen children (28%) had malnutrition defined as percentage of IBW lower than 90%, with a predominant fat mass depletion. VC (65+/-13% versus 79+/-15%; p=0.006) and FEV1 (59+/-16% versus 69+/-14%; p=0.03) were significantly lower in children with malnutrition than in children without malnutrition, but no significant differences were observed with regard to the FEV1/VC ratio and blood gases. Pi(max) (56+/-11% versus 88+/-37%, p=0,02) and Pe(max) (46+/-12% versus 58+/-19%, p=0,3) were also lower in children with malnutrition as compared to than without malnutrition., Conclusion: Malnutrition can be observed in children with CB and is associated with significant lower lung function parameters. This could be explained by decrease in respiratory muscle strength.

Unlabelled: Achalasia of the cardia is rare in children. We report our experience in the management of 20 children with achalasia., Patients: Twenty children (seven girls including two sisters) with achalasia were studied, seven of them had a morbid association. Age at diagnosis ranged from eight months to 18 years (med: 6.4 yrs). Duration of symptoms prior to diagnosis ranged from one to 62 months (med: 8 months). Regurgitations, weight loss and recurrent pneumonias were the most common presenting symptoms. Diagnosis was established using esophageal manometry, which showed aperistalsis throughout the esophageal body with impaired relaxation of lower esophageal sphincter in all children, and chest x-ray and barium esophagram, which were abnormal in 11 and 18 children respectively., Outcome: Nifedipine used in nine children was ineffective. Two balloon dilatations were performed in one child with a poor result. Surgery with Heller's myotomy combined with an antireflux procedure was performed in 19 children. One child died six months later due to an hypoglycemic coma. During the follow-up (6 months-21 years) symptoms reappeared in five children. Seventy-two percent of the children had no symptoms one year after the surgery and 45%, five years after. Esophageal manometry performed after surgery in seven children showed a normal lower esophageal sphincter pressure but with impaired relaxation, and an aperistalsis throughout the esophageal body. These results justify prolonged follow-up of children with achalasia of the esophagus.