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/m2/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.
Despite the widespread use of bacillus Calmette-Guérin vaccination, Mycobacterium tuberculosis infection remains globally the leading cause of death from a single infectious disease. The complicated ...and often protracted dynamics of infection and disease make clinical trials to test new tuberculosis vaccines extremely complex. Preclinical selection of only the most promising candidates is therefore essential. Because macaque monkeys develop a disease very similar to humans, they have potential to provide important information in addition to small animal models. To assess the relative merits of rhesus and cynomolgus monkeys as screens for tuberculosis vaccines, we compared the efficacy of bacillus Calmette-Guérin vaccination and the course of infection in both species. Unvaccinated rhesus and cynomolgus monkeys both developed progressive disease with high levels of C-reactive protein, M. tuberculosis-specific IgG, and extensive pathology including cavitation and caseous necrosis. Bacillus Calmette-Guérin vaccination protected cynomolgus almost completely toward the development of pathology, reflected in a striking 2-log reduction in viable bacteria in the lungs compared with nonvaccinated animals. Rhesus, on the other hand, were not protected efficiently by the bacillus Calmette-Guérin. The vaccinated animals developed substantial pathology and had negligible reductions of colony-forming units in the lungs. Comparative studies in these closely related species are likely to provide insight into mechanisms involved in protection against tuberculosis.
Background:
Knowledge about the effects of GH treatment on cognitive functioning in children with Prader-Willi syndrome (PWS) is limited.
Methods:
Fifty prepubertal children aged 3.5 to 14 yr were ...studied in a randomized controlled GH trial during 2 yr, followed by a longitudinal study during 4 yr of GH treatment. Cognitive functioning was measured biennially by short forms of the WPPSI-R or WISC-R, depending on age. Total IQ (TIQ) score was estimated based on two subtest scores.
Results:
During the randomized controlled trial, mean sd scores of all subtests and mean TIQ score remained similar compared to baseline in GH-treated children with PWS, whereas in untreated controls mean subtest sd scores and mean TIQ score decreased and became lower compared to baseline. This decline was significant for the Similarities (P = 0.04) and Vocabulary (P = 0.03) subtests. After 4 yr of GH treatment, mean sd scores on the Similarities and Block design subtests were significantly higher than at baseline (P = 0.01 and P = 0.03, respectively), and scores on Vocabulary and TIQ remained similar compared to baseline. At baseline, children with a maternal uniparental disomy had a significantly lower score on the Block design subtest (P = 0.01) but a larger increment on this subtest during 4 yr of GH treatment than children with a deletion. Lower baseline scores correlated significantly with higher increases in Similarities (P = 0.04) and Block design (P < 0.0001) sd scores.
Conclusions:
Our study shows that GH treatment prevents deterioration of certain cognitive skills in children with PWS on the short term and significantly improves abstract reasoning and visuospatial skills during 4 yr of GH treatment. Furthermore, children with a greater deficit had more benefit from GH treatment.
Observations of the tropical atmosphere are fundamental to the understanding of global changes in air quality, atmospheric oxidation capacity and climate, yet the tropics are under-populated with ...long-term measurements. The first three years (October 2006–September 2009) of meteorological, trace gas and particulate data from the global WMO/Global Atmospheric Watch (GAW) Cape Verde Atmospheric Observatory Humberto Duarte Fonseca (CVAO; 16° 51′ N, 24° 52′ W) are presented, along with a characterisation of the origin and pathways of air masses arriving at the station using the NAME dispersion model and simulations of dust deposition using the COSMO-MUSCAT dust model. The observations show a strong influence from Saharan dust in winter with a maximum in super-micron aerosol and particulate iron and aluminium. The dust model results match the magnitude and daily variations of dust events, but in the region of the CVAO underestimate the measured aerosol optical thickness (AOT) because of contributions from other aerosol. The NAME model also captured the dust events, giving confidence in its ability to correctly identify air mass origins and pathways in this region. Dissolution experiments on collected dust samples showed a strong correlation between soluble Fe and Al and measured solubilities were lower at high atmospheric dust concentrations. Fine mode aerosol at the CVAO contains a significant fraction of non-sea salt components including dicarboxylic acids, methanesulfonic acid and aliphatic amines, all believed to be of oceanic origin. A marine influence is also apparent in the year-round presence of iodine and bromine monoxide (IO and BrO), with IO suggested to be confined mainly to the surface few hundred metres but BrO well mixed in the boundary layer. Enhanced CO
2
and CH
4
and depleted oxygen concentrations are markers for air-sea exchange over the nearby northwest African coastal upwelling area. Long-range transport results in generally higher levels of O
3
and anthropogenic non-methane hydrocarbons (NMHC) in air originating from North America. Ozone/CO ratios were highest (up to 0.42) in relatively fresh European air masses. In air heavily influenced by Saharan dust the O
3
/CO ratio was as low as 0.13, possibly indicating O
3
uptake to dust. Nitrogen oxides (NO
x
and NO
y
) show generally higher concentrations in winter when air mass origins are predominantly from Africa. High photochemical activity at the site is shown by maximum spring/summer concentrations of OH and HO
2
of 9 × 10
6
molecule cm
−3
and 6 × 10
8
molecule cm
−3
, respectively. After the primary photolysis source, the most important controls on the HO
x
budget in this region are IO and BrO chemistry, the abundance of HCHO, and uptake of HO
x
to aerosol.
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