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Endocrine studies in children with myelomeningocele
L Perrone1, D Del Gaizo, E D'Angelo
1Dipartimento di Pediatria, Università di Napoli, Italy.
Insights
This study found abnormal hypothalamic-pituitary function in individuals with myelomeningocele (MMC), highlighting the need for hormonal evaluation and monitoring of pubertal development in these patients.
Area of Science:
- Neuroendocrinology
- Pediatric Endocrinology
- Developmental Biology
Background:
- Pituitary-hypothalamic dysfunction is a known complication of impaired cerebrospinal fluid circulation.
- Myelomeningocele (MMC) can affect endocrine function due to its impact on the central nervous system.
Purpose of the Study:
- To evaluate pituitary, thyroid, adrenal, and gonadal function in prepubertal and pubertal subjects with myelomeningocele.
- To investigate hormonal abnormalities associated with myelomeningocele and its treatments, such as shunts.
Main Methods:
- Assessed basal serum levels of various hormones (FT3, FT4, TSH, PRL, LH, FSH, T/E2, cortisol, 17-OH-P, DHEA-S) using radioimmunoassay.
- Conducted Thyrotropin-releasing hormone (TRH) and Gonadotropin-releasing hormone (GnRH) stimulation tests in a subset of patients.
Main Results:
- Abnormalities in thyroid-stimulating hormone (TSH) and prolactin (PRL) responses to TRH were observed.
- Elevated follicle-stimulating hormone (FSH) levels and responses to GnRH were noted in some patients, particularly females with shunts.
- Altered basal and stimulated PRL levels were associated with the presence of shunts.
Conclusions:
- Myelomeningocele is associated with significant hypothalamic-pituitary dysfunction.
- Regular hormonal evaluation and monitoring of pubertal development are crucial for individuals with myelomeningocele.
Abstract:
Pituitary-hypothalamic abnormalities due to impaired cerebrospinal fluid circulation have long been recognized. The aim of this study was to assess pituitary, thyroid, adrenal, and gonadal function in 46 prepubertal (22 M and 24 F) and 10 pubertal (4 M and 6 F) subjects with myelomeningocele (MMC). Basal serum levels of FT3, FT4, TSH, PRL, LH, FSH, T or E2, cortisol, 17-OH-P and DHEA-S were measured by routine radio-immunoassay methods. Twenty-two prepubertal patients had a TRH test for TSH and PRL evaluation, and eight underwent a GnRH test. Three patients presented with precocious puberty. Six subjects had modest elevations of serum TSH together with normal free thyroid hormone levels. In three cases, TSH responses to TRH were significantly exaggerated and prolonged: in two patients, TSH responses were delayed. The mean basal plasma FSH level in females with ventriculo-peritoneal shunt was significantly higher than in controls. In six cases FSH responses to GnRH were significantly higher than in controls. Both basal and stimulated PRL levels were elevated in patients with shunts; in patients without shunts, basal PRL was normal, but peak PRL levels following TRH stimulation were elevated. Our data show an abnormal hypothalamic-pituitary function in MMC subjects. These findings reinforce the importance of physical examination, hormonal evaluation and follow-up of pubertal development in patients with myelomeningocele.