BSPED2018 Oral Communications Oral Communications 5 (9 abstracts)
Growth outcomes in adolescents and adults with Silver-Russell syndrome and the effects of childhood growth hormone treatment
Oluwakemi Lokulo-Sodipe 1, , Ana P M Canton 3, , Eloise Giabicani 5, , Nawfel Ferrand 8 , Jenny Child 9 , Emma L Wakeling 10 , Gerhard Binder 11 , Irène Netchine 5, , Deborah J G Mackay 12 , Hazel M Inskip 13, , Christopher D Byrne 14, , Justin H Davies 16 & I Karen Temple 12,
1Human Development and Health, Faculty of Medicine, University of Southampton, Southampton, UK; 2Wessex Clinical Genetics Service, Princess Anne Hospital, University Hospital Southampton NHS Foundation Trust, Southampton, UK; 3Sorbonne Université, INSERM, UMR_S 938 Centre de Recherche Saint Antoine, APHP, Hôpital Armand Trousseau, Explorations Fonctionnelles Endocriniennes, F-75012, Paris, France; 4Unidade de Endocrinologia do Desenvolvimento, Laboratorio de Hormonios e Genetica Molecular LIM/42 do Hospital das Clinicas, Disciplina de Endocrinologia da Faculdade de Medicina da Universidade de Sao Paulo, 05403-900,, Sao Paulo, Brazil; 5Sorbonne Universities, UPMC UNIV Paris 06, 4 place Jussieu, 75005, Paris, France; 6AP-HP, Hôpitaux Universitaires Paris Est (AP-HP) Hôpital des Enfants Armand Trousseau, Service d’Explorations Fonctionnelles Endocriniennes, 26 avenue du Dr Arnold Netter, 75012, Paris, France; 7Centre de Recherche Saint Antoine, INSERM UMR S938, 34 rue Crozatier, 75012, Paris, France; 8University Children’s Hospital, Pediatric Endocrinology, Hoppe-Seyler-Strasse 1, 72072, Tuebingen, Germany; 9Child Growth Foundation, Edgware, HA8 7RA, UK; 10North West Thames Regional Genetics Service, London North West Healthcare NHS Trust, Watford Road, Harrow, HA1 3UJ, UK; 11University Children’s Hospital, Pediatric Endocrinology, Hoppe-Seyler-Strasse 1, 72070, Tuebingen, Germany; 12Human Development and Health, Faculty of Medicine, University of Southampton, Southampton, SO16 6YD, UK; 13MRC Lifecourse Epidemiology Unit, University of Southampton, Southampton, SO16 6YD, UK; 14NIHR Southampton Biomedical Research Centre, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton, SO16 6YD, UK; 15Nutrition and Metabolism, Human Development and Health Academic Unit, Faculty of Medicine, University of Southampton, Southampton, SO16 6YD, UK; 16Department of Endocrinology, Southampton Children’s Hospital, University Hospital Southampton NHS Foundation Trust, Southampton, SO16 6YD, UK; 17Wessex Clinical Genetics Service, Princess Anne Hospital, University Hospital Southampton NHS Foundation Trust, Coxford Road, Southampton, SO16 5YA, UK.
Childhood short stature in Silver-Russell syndrome (SRS) is frequently treated with growth hormone (GH), however final height and long-term body mass index (BMI) data are limited.
Objective: To assess height and BMI in older individuals with molecularly confirmed SRS and compare those previously treated with GH to those untreated.
Methods: Growth data on individuals aged ≥13 years with SRS were evaluated from UK, French and German cohorts. Height and BMI standard deviation scores (SDS) were calculated using country-specific reference data.
Results: 71 individuals (40 females) aged 13.17–69.71 years (median 22.03) were recruited. Molecular diagnoses: loss of methylation at H19/IGF2 (80.3%), maternal uniparental disomy for chromosome 7 (16.9%), IGF2 mutation (2.8%). 77.5% received GH for a median of 7.10 years (IQR 3.96 to 11.00). The median time since GH cessation was 9.97 years (IQR 2.68–15.94). Median early height SDS in GH-untreated and GH-treated individuals were −2.91 (IQR −3.62 to −2.40) and −3.46 (IQR −5.15 to −2.76) respectively (P=0.055). Median height gain from early to final height SDS in GH-untreated and GH-treated individuals were 0.53 (IQR −0.13 to 1.37) and 1.53 (IQR 0.80 to 2.52) respectively (P=0.006). The median final height SDS of GH-untreated and GH-treated individuals were −2.74 (IQR −3.36 to −1.13) and −2.22 (IQR −3.66 to −1.16) respectively (P=0.720). Median change in BMI from early to final BMI SDS in GH-untreated and GH-treated individuals were 3.58 (IQR 1.85 to 5.18) and 1.95 (IQR 0.76 to 2.69) respectively (P=0.005). The median BMI SDS of GH-untreated versus GH-treated individuals were 1.66 (IQR −0.73 to 2.03) and −1.10 (IQR −1.80 to 0.00) respectively (P=0.002).
Conclusions: Height gain was significantly greater in GH-treated individuals who (were shorter at treatment initiation but) reached similar final heights to GH-untreated individuals. Historical GH treatment was associated with reduced BMI and reduced gain in BMI, indicating long-term effects.
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