The effects of 3-year growth hormone treatment and body composition in Polish patients with Silver-Russell syndrome

Introduction: Silver-Russell syndrome (SRS) is characterized by clinical and genetic heterogeneity. SRS is the only disease entity associated with (epi)genetic abnormalities of 2 different chromosomes: 7 and 11. In SRS, the 2 most frequent molecular abnormalities are hypomethylation (loss of methylation) of region H19/IGF2:IG-DMR on chromosome 11p15.5 (11p15 LOM) and maternal uniparental disomy of chromosome 7 (upd(7)mat). Therapy with recombinant human growth hormone (rhGH) is implemented to increase body height in children with SRS. The effect of the administered rhGH on height, weight, body mass index (BMI), body composition, and height velocity in patients with SRS during 3 years of rhGH therapy was analysed. Material and methods: 31 SRS patients (23 with 11p15 LOM, 8 with upd(7)mat) and 16 patients small for gestational age (SGA) as a control group were diagnosed and followed up in The Children’s Memorial Health Institute. Patients were eligible for the 2 Polish rhGH treatment programmes [for patients with SGA or with growth hormone deficiency (GHD)]. Anthropometric parameters were collected in all patients. Body composition using bioelectrical impedance was measured in 13 SRS and 14 SGA patients. Results: Height, weight, and weight for height (SDS) at baseline of rhGH therapy were lower in SRS patients than in the SGA control group: –3.3 ± 1.2 vs. -2.6 ± 06 (p = 0.012), –2.5 vs. -1.9 (p = 0.037), –1.7 vs. –1.1 (p = 0.038), respectively. Height SDS was increased from –3.3 ± 1.2 to –1.8 ± 1.0 and from –2.6 ± 0.6 to –1.3 ± 0.7 in the SRS and SGA groups, respectively. Patients with 11p15 LOM and upd(7)mat achieved similar height, 127.0 ± 15.7 vs. 128.9 ± 21.6 cm, and –2.0 ± 1.3 vs. –1.7 ± 1.0 SDS, respectively. Fat mass percentage decreased in SRS patients from 4.2% to 3.0% (p < 0.05) and in SGA patients from 7.6% to 6.6% (p < 0.05). Conclusions: Growth hormone therapy has a positive influence on the growth of SRS patients. Regardless of molecular abnormality type (11p15 LOM vs. upd(7)mat), height velocity was similar in SRS patients during 3 years of rhGH therapy

Prospective BMI changes in preschool children are associated with parental characteristics and body weight perceptions : the ToyBox-study

Objective: To examine the effect of the intervention implemented in the ToyBox-study on changes observed in age- and sex-specific BMI percentile and investigate the role of perinatal factors, parental perceptions and characteristics on this change. Design: A multicomponent, kindergarten-based, family-involved intervention with a cluster-randomised design. A standardised protocol was used to measure children’s body weight and height. Information was also collected from parents/caregivers via the use of validated questionnaires. Linear mixed effect models with random intercept for country, socio-economic status and school were used. Setting: Selected preschools within the provinces of Oost-Flanders and West-Flanders (Belgium), Varna (Bulgaria), Bavaria (Germany), Attica (Greece), Mazowieckie (Poland) and Zaragoza (Spain). Participants: A sample of 6268 preschoolers aged 3·5–5·5 years (51·9 % boys). Results: There was no intervention effect on the change in children’s BMI percentile. However, parents’ underestimation of their children’s actual weight status, parental overweight and mothers’ pre-pregnancy overweight/obesity were found to be significantly and independently associated with increases in children’s BMI percentile in multivariate modelling. Conclusions: As part of a wide public health initiative or as part of a counseling intervention programme, it is important to assist parents/caregivers to correctly perceive their own and their children’s weight status. Recognition of excessive weight by parents/caregivers can increase their readiness to change and as such facilitate higher adherence to favourable behavioural changes within the family