Eight years of growth hormone treatment in children with Prader-Willi syndrome: maintaining the positive effects.

BACKGROUND: The most important reason for treating children with Prader-Willi syndrome (PWS) with GH is to optimize their body composition. OBJECTIVES: The aim of this ongoing study was to determine whether long-term GH treatment can counteract the clinical course of increasing obesity in PWS by maintaining the improved body composition brought during early treatment. SETTING: This was a multicenter prospective cohort study. METHODS: We have been following 60 prepubertal children for 8 years of continuous GH treatment (1 mg/m(2)/d ≈ 0.035 mg/kg/d) and used the same dual-energy x-ray absorptiometry machine for annual measurements of lean body mass and percent fat. RESULTS: After a significant increase during the first year of GH treatment (P < .0001), lean body mass remained stable for 7 years at a level above baseline (P < .0001). After a significant decrease in the first year, percent fat SD score (SDS) and body mass index SDS remained stable at a level not significantly higher than at baseline (P = .06, P = .14, resp.). However, body mass index SDSPWS was significantly lower after 8 years of GH treatment than at baseline (P < .0001). After 8 years of treatment, height SDS and head circumference SDS had completely normalized. IGF-1 SDS increased to +2.36 SDS during the first year of treatment (P < .0001) and remained stable since then. GH treatment did not adversely affect glucose homeostasis, serum lipids, blood pressure, and bone maturation. CONCLUSION: This 8-year study demonstrates that GH treatment is a potent force for counteracting the clinical course of obesity in children with PWS.

Comparison of handling and acceptability of two spacer devices in young children with asthma.

UNLABELLED: This study compared parents' preference for two spacer devices, NebuChamber and Babyhaler for the treatment of young children with asthma. In this open, cross-over study 141 patients (aged 5-57 mo) who used inhaled steroids via a spacer device were randomized to budesonide via NebuChamber or beclomethasone dipropionate via Babyhaler. Both treatments were given by the parents twice daily for 2 wk. At the final visit parents completed a questionnaire on preference in general and for a given set of features. Acceptability and handling were scored in a diary. Diary scores on acceptability by the child and handling of both spacer devices were comparable. In the preference questionnaire, 68% of parents preferred NebuChamber [95% confidence interval (CI) 60-76] and 25% Babyhaler (95% CI 18-33). The preference was independent of the type of spacer used before the study and was also apparent in the different features: acceptability by child, carrying around, cleaning, close fitting of face mask, assembling and disassembling, damage resistance and size. These differences were statistically significant for all features, except for acceptability by the child. CONCLUSION: Two-thirds of parents prefer NebuChamber over Babyhaler for the treatment of their young asthmatic children.

The effective case management of childhood diarrhoea with oral rehydration therapy in the Kingdom of Lesotho.

In Lesotho prior to 1986, diarrhoea was the leading cause of hospital mortality in children less than 5 years of age. At the Queen Elizabeth II Hospital, diarrhoea-related admissions as a proportion of all admissions in children less than 5 years of age declined from 23% in the year prior to the opening of the Oral Rehydration Therapy Unit (ORTU) to 13% in the first nine months of 1987 (p less than 0.05). In addition, the case-fatality ratio of children treated in the ORTU declined from 1.4% in the first quarter of 1986 to zero in the second and third quarters of 1987 (p less than 0.05). In a case-control study conducted to identify reasons for children failing ORTU treatment, factors associated with an increased risk of hospitalization included male gender (odds ratio [OR] = 4.9; 95% confidence limits [CL] = 2.0, 11.9), fever greater than or equal to 38.5 degrees C (OR = 2.0; CL = 1.2, 3.3), undernutrition (OR = 3.2; CL = 1.1, 9.4), and moderate dehydration (OR = 2.3; CL = 1.2, 4.4) or severe dehydration. (OR = 12.1; CL = 3.8, 38.5). Breastfed children less than 2 years of age were at decreased risk of hospitalization (OR = 0.4; CL = 0.2, 0.7). At this major hospital in Lesotho, the standardization of outpatient treatment for diarrhoea with oral rehydration salts (ORS) in the context of an ORTU resulted in a marked decrease in diarrhoea-associated hospitalization and deaths in children less than 5 years of age.