Ondine-Hirschsprung syndrome (Haddad syndrome) - Further delineation in two cases and review of the literature

Two unrelated children with congenital central hypoventilation syndrome (CCHS-Ondine syndrome) and long segment Hirschsprung disease are reported. Patient 1, a girl, is still alive at 3 years. Patient 2, a boy, died of viral pneumonia at 5.5 years. Continuous mechanical ventilation was necessary for months and those children could never be weaned from the respirator during sleep. Seventeen cases of this complex neurocristopathy are reviewed. Only six children (including our cases) survived beyond 2 years of age. Hypotonia, delay in developmental milestones or epilepsy were frequently observed. Ventilator dependency does not improve with time. Multifocal congenital neuroblastoma occurred in two children. Aetiology is unknown. © 1993 Springer-Verlag.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Pulmonary outcome and its correlates in school-aged children born with a gestational age ≤ 32 weeks

SummaryBackgroundThere is limited data regarding factors influencing the respiratory outcome at school age of ex-preterms born since the introduction of antenatal steroids, surfactant replacement together with less aggressive ventilation.ObjectivesTo establish the main antenatal, neonatal and early childhood respiratory correlates of respiratory status in school-aged children born at ≤32weeks of gestation.MethodsEx-preterm children born at ≤32weeks of gestation between 1997 and 2001 at Bordeaux University Hospital were evaluated at school age, using a respiratory questionnaire and lung function tests (spirometry, plethysmography, exercise challenge test and CO lung diffusing capacity DLCO measurements). Factors associated with lung function were investigated using polynomial regression analyses.ResultsOf the 151 included children [mean age: 8.6 ± 0.8 years; mean gestational age, 30.1 ± 1.7 weeks; mean birth weight = 1310 ± 380 g; 68.2% ventilated at birth; 46.4% treated with surfactant; 36.4% with prior bronchopulmonary dysplasia (BPD)], 47% presented obstructive lung abnormalities, 11% restrictive or mixed lung abnormalities, 41% exercise-induced bronchoconstriction, and 15.5% reduced DLCO. Surfactant therapy was independently associated with a lower risk of lung abnormalities (p < 0.05). The association between BPD and lung abnormalities at school age was not significant, but prior BPD increased the risk of restrictive or mixed abnormalities (odds ratio: 6.11, confidence interval [1.1; 33.99]). Early childhood respiratory events were not associated with the occurrence of lung abnormalities.ConclusionChildren born at ≤32 weeks of gestation remain at risk for impaired lung function at school age in particular when they did not receive surfactant. Restrictive or mixed lung defects are mainly associated with prior BPD

A map of African humid tropical forest aboveground biomass derived from management inventories

AbstractForest biomass is key in Earth carbon cycle and climate system, and thus under intense scrutiny in the context of international climate change mitigation initiatives (e.g. REDD+). In tropical forests, the spatial distribution of aboveground biomass (AGB) remains, however, highly uncertain. There is increasing recognition that progress is strongly limited by the lack of field observations over large and remote areas. Here, we introduce the Congo basin Forests AGB (CoFor-AGB) dataset that contains AGB estimations and associated uncertainty for 59,857 1-km pixels aggregated from nearly 100,000 ha of in situ forest management inventories for the 2000 – early 2010s period in five central African countries. A comprehensive error propagation scheme suggests that the uncertainty on AGB estimations derived from c. 0.5-ha inventory plots (8.6–15.0%) is only moderately higher than the error obtained from scientific sampling plots (8.3%). CoFor-AGB provides the first large scale view of forest AGB spatial variation from field data in central Africa, the second largest continuous tropical forest domain of the world.</jats:p

A map of African humid tropical forest aboveground biomass derived from management inventories

International audienceForest biomass is key in Earth carbon cycle and climate system, and thus under intense scrutiny in the context of international climate change mitigation initiatives (e.g. REDD+). In tropical forests, the spatial distribution of aboveground biomass (AGB) remains, however, highly uncertain. There is increasing recognition that progress is strongly limited by the lack of field observations over large and remote areas. Here, we introduce the Congo basin Forests AGB (CoFor-AGB) dataset that contains AGB estimations and associated uncertainty for 59,857 1-km pixels aggregated from nearly 100,000 ha of in situ forest management inventories for the 2000 – early 2010s period in five central African countries. A comprehensive error propagation scheme suggests that the uncertainty on AGB estimations derived from c. 0.5-ha inventory plots (8.6–15.0%) is only moderately higher than the error obtained from scientific sampling plots (8.3%). CoFor-AGB provides the first large scale view of forest AGB spatial variation from field data in central Africa, the second largest continuous tropical forest domain of the world