The effects of growth hormone therapy on the somatic development of a group of Polish children with Silver-Russell syndrome.

Autor:	Sienko M; Department of Pediatrics, Endocrinology, Diabetology, Metabolic Diseases and Cardiology of the Developmental Age, Pomeranian Medical University, Szczecin, Poland., Petriczko E; Department of Pediatrics, Endocrinology, Diabetology, Metabolic Diseases and Cardiology of the Developmental Age, Pomeranian Medical University, Szczecin, Poland., Zajaczek S; Cytogenetics Unit, Department of Pathology, Pomeranian Medical University, Szczecin, Poland., Zygmunt-Gorska A; Department of Pediatric and Adolescent Endocrinology, Chair of Pediatrics, Polish-American Pediatric Institute, Jagiellonian University, Medical College, Cracow, Poland., Starzyk J; Department of Pediatric and Adolescent Endocrinology, Chair of Pediatrics, Polish-American Pediatric Institute, Jagiellonian University, Medical College, Cracow, Poland., Korpysz A; Department of Endocrinology and Diabetology, The Children's Memorial Health Institute, Warsaw, Poland., Petriczko J; Department of Plastic Endocrine and General Surgery, Pomeranian Medical University, Police, Poland., Walczak A; Department of Hygienae and Epidemiology, Pomeranian Medical University, Szczecin, Poland., Walczak M; Department of Pediatrics, Endocrinology, Diabetology, Metabolic Diseases and Cardiology of the Developmental Age, Pomeranian Medical University, Szczecin, Poland.
Jazyk:	angličtina
Zdroj:	Neuro endocrinology letters [Neuro Endocrinol Lett] 2017 Dec; Vol. 38 (6), pp. 415-421.
Abstrakt:	Objective: Silver-Russell Syndrome is both clinically and genetically a heterogeneous syndrome. Among the most important dysmorphic features of this condition are: a triangular shaped face with a small mandible, a prominent frontal eminence, a thin vermilion border with downward-pointing lip corners, clino- and brachydactyly of the 5th fingers as well as body asymmetry. The most well-known genetic mutations in this syndrome are: the 11p15 epimutation (20-60% patients) and the maternal uniparental chromosome 7 disomy present in 7% to 15% of patients. Children with SRS have severely impaired physical growth - intrauterine and after birth. This, together with the aforementioned dysmorphic features, forms the main diagnostic criteria. Material and Methods: The study group consisted of 12 children treated with growth hormone, aged 2 to 17 (8.9±4.0 years), therein, all of whom met the phenotype diagnostic criteria by Wollmann and Price. The effects of growth hormone therapy on somatic development of these children are also presented. Results: Height and weight improved as a result of growth hormone treatment, but the effects were significantly worse than in children with IUGR. Children from the study group presented also a smaller an improvement in growth velocity than children from the control group, but the difference was statistically insignificant. Conclusions: Growth hormone therapy accelerates the growth of children with SRS but to a smaller extent than the growth of children born with intrauterine growth retardation without dysmorphic features.
Databáze:	MEDLINE
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