Unexpected high frequency of skeletal dysplasia in idiopathic short stature and small for gestational age patients

ObjectiveTo assess the prevalence of skeletal dysplasias (SDs) in patients with idiopathic short stature (ISS) or small for gestational age (SGA) status.SettingRare Endocrine/Growth Diseases Center in Paris, France.DesignA prospective study on consecutive patients with ISS and SGA enrolled from 2004 to 2009.MethodWe used a standardized workup to classify patients into well-established diagnostic categories. Of 713 patients with ISS (n=417) or SGA status (n=296), 50.9% underwent a skeletal survey. We chose patients labeled normal or with a prepubertal slowdown of growth as a comparison group.ResultsDiagnoses were ISS (16.9%), SGA (13.5%), normal growth (24.5%), transient growth rate slowing (17.3%), endocrine dysfunction (12%), genetic syndrome (8.9%), chronic disease (5.1%), and known SD (1.8%). SD was found in 20.9% of SGA and 21.8% ISS patients and in only 13.2% in our comparison group. SD prevalence was significantly higher in the ISS group than in the comparison group, especially (50%) for patients having at least one parent whose height was <−2 SDS. Dyschondrosteosis and hypochondroplasia were the most frequently identified SD, and genetic anomaly was found in 61.5 and 30% respectively. Subtle SD was found equally in the three groups and require long-term growth follow-up to evaluate the impact on final height.ConclusionSD may explain more than 20% of cases of growth retardation ascribed to ISS or SGA, and this proportion is higher when parental height is <−2 SDS. A skeletal survey should be obtained in patients with delayed growth in a context of ISS or SGA.

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The Impact of Long-Term Growth Hormone Treatment on Metabolic Parameters in Japanese Patients with Short Stature Born Small for Gestational Age

Hormone Research in Paediatrics ◽

10.1159/000358196 ◽

2014 ◽

Vol 81 (4) ◽

pp. 272-279 ◽

Cited By ~ 15

Author(s):

Anne-Marie Kappelgaard ◽

Fumiaki Kiyomi ◽

Reiko Horikawa ◽

Susumu Yokoya ◽

Toshiaki Tanaka

Keyword(s):

Growth Hormone ◽

Short Stature ◽

Gestational Age ◽

Small For Gestational Age ◽

Growth Hormone Treatment ◽

Japanese Patients ◽

Hormone Treatment ◽

Metabolic Parameters ◽

The Impact

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Delayed Bone Age Might Accelerate the Response to Human Growth Hormone Treatment in Small for Gestational Age Children with Short Stature

International Journal of Endocrinology ◽

10.1155/2019/8454303 ◽

2019 ◽

Vol 2019 ◽

pp. 1-6

Author(s):

Jung-Eun Moon ◽

Cheol Woo Ko

Keyword(s):

Growth Hormone ◽

Short Stature ◽

Gestational Age ◽

Small For Gestational Age ◽

Human Growth ◽

Bone Age ◽

Pediatric Endocrinology ◽

Gh Therapy ◽

Gh Treatment ◽

The Impact

Purpose. Growth hormone (GH) treatment is recommended to improve growth and psychosocial problems in short stature children born small for gestational age (SGA). Although GH therapy in these patients has been extensively studied, the impact of therapy according to delays in bone age (BA) is not known well. Objective. To investigate the effects of GH therapy in SGA patients with short stature according to BA delay. Methods. We retrospectively analyzed changes in height SD score (SDS) and BA/chronological age (CA) after 6 and 12 months of GH therapy in patients grouped according to BA delay. We studied 27 SGA children with short stature in the pediatric endocrinology clinic of Kyungpook National University Children’s Hospital. Results. Of the 27 patients, 9 had <2 years of BA delay, while 18 had >2 years of delay. There were no significant differences between the two groups in terms of gestational age and weight at birth, height SDS, IGF-1 SDS, and growth hormone dosage at the beginning of therapy. However, height SDS increased significantly in the group with >2 years of BA delay after 6 months of GH therapy (−2.50 ± 0.61 vs −1.87 ± 0.82; p=0.037) and 12 months (−2.27 ± 0.70 vs −1.63 ± 0.65; p=0.002). When height SDS was compared between with and without GHD, there were no significant differences. Conclusions. Delayed BA (>2 years) may impact the response to GH treatment in SGA children with short stature.

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Observed and Predicted Total Pubertal Growth during Treatment with Growth Hormone in Adolescents with Idiopathic Growth Hormone Deficiency, Turner Syndrome, Short Stature, Born Small for Gestational Age and Idiopathic Short Stature: KIGS Analysis and Review

Hormone Research in Paediatrics ◽

10.1159/000324117 ◽

2011 ◽

Vol 75 (6) ◽

pp. 423-432 ◽

Cited By ~ 20

Author(s):

Michael B. Ranke ◽

Anders Lindberg ◽

Keyword(s):

Growth Hormone ◽

Growth Hormone Deficiency ◽

Short Stature ◽

Turner Syndrome ◽

Gestational Age ◽

Small For Gestational Age ◽

Idiopathic Short Stature ◽

Hormone Deficiency ◽

Pubertal Growth ◽

Idiopathic Growth Hormone Deficiency

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Bone age in prepubertal children with nonfamilial or familial idiopathic short stature and prepubertal short-stature children born small for gestational age: a longitudinal data analysis

Clinical Pediatric Endocrinology ◽

10.1297/cpe.30.171 ◽

2021 ◽

Vol 30 (4) ◽

pp. 171-177

Author(s):

Mari Satoh

Keyword(s):

Data Analysis ◽

Longitudinal Data ◽

Short Stature ◽

Gestational Age ◽

Small For Gestational Age ◽

Longitudinal Data Analysis ◽

Bone Age ◽

Idiopathic Short Stature ◽

Prepubertal Children

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Psychosocial Functioning of Adolescents with Idiopathic Short Stature or Persistent Short Stature Born Small for Gestational Age during Three Years of Combined Growth Hormone and Gonadotropin-Releasing Hormone Agonist Treatment

Hormone Research in Paediatrics ◽

10.1159/000087700 ◽

2005 ◽

Vol 64 (2) ◽

pp. 77-87 ◽

Cited By ~ 6

Author(s):

Hanneke Visser-van Balen ◽

Rinie Geenen ◽

Mirjam Moerbeek ◽

Rutger Stroop ◽

Gerdine A. Kamp ◽

...

Keyword(s):

Growth Hormone ◽

Short Stature ◽

Gestational Age ◽

Small For Gestational Age ◽

Psychosocial Functioning ◽

Gonadotropin Releasing Hormone ◽

Idiopathic Short Stature ◽

Releasing Hormone ◽

Gonadotropin Releasing Hormone Agonist

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Three years of growth hormone therapy in children born small for gestational age: results from the ANSWER Program

Endocrine Connections ◽

10.1530/ec-18-0286 ◽

2018 ◽

Vol 7 (10) ◽

pp. 1096-1104 ◽

Cited By ~ 2

Author(s):

Robert Rapaport ◽

Peter A Lee ◽

Judith L Ross ◽

Paul Saenger ◽

Vlady Ostrow ◽

...

Keyword(s):

Growth Hormone ◽

Short Stature ◽

Gestational Age ◽

Small For Gestational Age ◽

Growth Failure ◽

Hormone Deficiency ◽

Birth Size ◽

Growth Disorders ◽

Gh Therapy ◽

The Impact

Growth hormone (GH) is used to treat short stature and growth failure associated with growth disorders. Birth size and GH status variably modulate response to GH therapy. The aim of this study was to determine the effect of birth size on response to GH therapy, and to determine the impact of GH status in patients born small for gestational age (SGA) on response to GH therapy. Data from the prospective, non-interventional American Norditropin Studies: Web-Enabled Research (ANSWER) Program was analyzed for several growth outcomes in response to GH therapy over 3 years. GH-naïve children from the ANSWER Program were included in this analysis: SGA with peak GH ≥10 ng/mL (20 mIU/L), SGA with peak GH <10 ng/mL (20 mIU/L), isolated growth hormone deficiency (IGHD) born SGA, IGHD not born SGA and idiopathic short stature. For patients with IGHD, those who did not meet criteria for SGA at birth showed greater improvements in height SDS and BMI SDS than patients with IGHD who met criteria for SGA at birth. For patients born SGA, response to GH therapy varied with GH status. Therefore, unlike previous guidelines, we recommend that GH status be established in patients born SGA to optimize GH therapy.

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