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Growth hormone treatment in children with sporadic primary microcephaly
G L Spadoni1, S Cianfarani, S Bernardini
1Department of Pediatrics, Second University of Rome, Italy.
Insights
Growth hormone therapy effectively increased growth rates in children with primary microcephaly, short stature, and delayed bone age. This treatment showed positive results despite varying spontaneous growth hormone secretion levels.
Area of Science:
- Pediatric Endocrinology
- Growth Disorders
- Genetics
Background:
- Primary microcephaly is a rare condition characterized by a small head circumference.
- Associated features can include short stature, delayed bone age, and reduced growth velocity.
- The role of growth hormone in managing these specific growth disturbances requires further investigation.
Purpose of the Study:
- To evaluate the efficacy of exogenous growth hormone (GH) treatment in children with sporadic primary microcephaly.
- To assess the impact of GH therapy on growth rate in this specific pediatric population.
- To correlate treatment outcomes with baseline growth hormone secretion patterns.
Main Methods:
- Case series describing four children with sporadic primary microcephaly.
- Assessment of growth parameters including height, bone age, and growth velocity.
- Evaluation of spontaneous and pharmacologically stimulated growth hormone secretion.
- Administration of exogenous growth hormone therapy and monitoring of growth response.
Main Results:
- All four patients exhibited increased growth rates following GH administration.
- One patient demonstrated reduced spontaneous nocturnal GH secretion.
- Normal GH responses were observed in standard pharmacological tests for all participants.
- Exogenous GH treatment proved beneficial irrespective of baseline GH secretion levels.
Conclusions:
- Exogenous growth hormone therapy can effectively enhance growth velocity in children with sporadic primary microcephaly.
- GH treatment may be beneficial even in cases with normal stimulated GH response or reduced spontaneous secretion.
- Further research is warranted to elucidate the precise mechanisms and long-term outcomes of GH therapy in this condition.
Abstract:
Four children with sporadic primary microcephaly associated with short stature, delayed bone age, and low growth velocity are described. All of the children showed a normal growth hormone response to standard pharmacological tests but one of the patients had a reduced spontaneous growth hormone nocturnal secretion. Regardless of the results of their somatotropic function evaluation, the patients were treated with exogenous growth hormone and all of them showed an increase in growth rate.