Growth evaluation in Silver Russel Syndrome and Small Gestational Age patients : A Statistical Model

Author:

Migliaretti Giuseppe1,Stura I.1,Brondino F.1,Matarazzo P.1,Sanctis L.1

Affiliation:

1. University of Turin

Abstract

Abstract

Objective The study proposes a description and a mathematical model of growth of both Silver Russell Syndrome (SRS) and Small for Gestational Age (SGA) patients, with and without Growth Hormone (GH) treatment. Design and Methods Thirteen patients with molecular diagnosis of SRS were compared with 13 sex-paired SGA subjects. For each patient, neonatal and follow up auxological data were collected. A predictive growth model based on the Gompertzian function and optimization method, already tested and validated on a cohort of GH-deficient children, was used. Results Baselines anthropometric values do not significantly differ between the presented SRS and SGA populations. The applied mathematical model showed same natural growth hope for SGA and SRS subjects. In SRS patients under GH treatment a significant increase in growth hope seems to emerge from the prediction model, while an improvement in growth velocity was assessed in the counterpart of SGA subjects, which exhibit early response to the treatment. Conclusions The proposed mathematical prediction model indicates a benefit from GH treatment for both the SRS and SGA conditions; while SGA patients showed an earlier response, SRS patients have a longer term gain. These data suggest the need to anticipate the SRS diagnosis for starting with earlier therapy.

Publisher

Research Square Platform LLC

Reference22 articles.

1. Diagnosis and management of Silver-Russell syndrome: first international consensus statement;Wakeling EL;Nat Rev Endocrinol,2017

2. Epigenotype-phenotype correlations in Silver-Russell syndrome;Wakeling EL;J Med Genet,2010

3. Patients with Silver-Russell-Syndrome from Birth to Adulthood: Diagnosis, Development and Medical Care;Wollmann HA;Pediatr Endocrinol Rev PER,2017

4. Management of the child born small for gestational age through to adulthood: a consensus statement of the International Societies of Pediatric Endocrinology and the Growth Hormone Research Society;Clayton PE;J Clin Endocrinol Metab,2007

5. Small at Birth, but How Small? The Definition of SGA Revisited;Zeve D;Horm Res Paediatr,2016

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