Effectiveness of current interventions in obese New Zealand children and adolescents.

Aims: To determine the effectiveness of current interventions in New Zealand in obese children and adolescents accessing either a standard model of care (medical input alone or with the addition of dietitian and physical activity input), or one of the country's long-standing multi-disciplinary intervention programmes.

Methods: Data were recorded over approximately 2.1 years of intervention from 290 patients across four centres in New Zealand, who manage obese and overweight children and adolescents aged 3-16 years in paediatric clinics.

Skeletal phenotype of mandibuloacral dysplasia associated with mutations in ZMPSTE24.

Mandibuloacral dysplasia (MAD) is a rare recessively inherited premature aging disease characterized by skeletal and metabolic anomalies. It is part of the spectrum of diseases called laminopathies and results from mutations in genes regulating the synthesis of the nuclear laminar protein, lamin A. Homozygous or compound heterozygous mutations in the LMNA gene, which encodes both the precursor protein prelamin A and lamin C, are the commonest cause of MAD type A.