Physical growth, puberty and hormones in adolescents with Nodding Syndrome; a pilot study
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Date
2014
Journal Title
Journal ISSN
Volume Title
Publisher
BMC Research Notes
Abstract
Nodding syndrome is an epidemic symptomatic generalized epilepsy syndrome of unknown cause in
Eastern Africa. Some patients have extreme short stature. We hypothesized that growth failure in nodding
syndrome is associated with specific endocrine dysfunctions. In this pilot study, we examined the relationship
between serum hormone levels and stature, bone age and sexual development.
Results: We recruited ten consecutive children, 13 years or older, with World Health Organization defined nodding
syndrome and assessed physical growth, bone age, development of secondary sexual characteristics and serum
hormone levels. Two children with incomplete results were excluded. Of the eight remaining, two had severe
stunting (height for age Z [HAZ] score < -3) and three had moderate stunting (HAZ score between-3 and -2). The
bone age was delayed by a median 3(range 0-4) years. Serum growth hormone levels were normal in all eight but
the two patients with severe stunting and one with moderate stunting had low levels of Somatomedin C (Insulin
like Growth Factor [IGF1]) and/or IGF binding protein 3 (IGFBP3), mediators of growth hormone function. A linear
relationship was observed between serum IGF1 level and HAZ score. With the exception of one child, all were either
pre-pubertal or in early puberty (Tanner stages 1 and 2) and in the seven, levels of the gonadotrophins (luteinising
and follicle stimulating hormone) and the sex hormones (testosterone/oestrogen) were all within pre-pubertal
ranges or ranges of early puberty. Thyroid function, prolactin, adrenal, and parathyroid hormone levels were all
normal.
Conclusions: Patients with nodding syndrome may have dysfunctions in the pituitary growth hormone and
pituitary gonadal axes that manifest as stunted growth, delayed bone age and puberty. Studies are required to
determine if such endocrine dysfunction is a primary manifestation of the disease or a secondary consequence
of chronic ill health and malnutrition and if so, whether targeted interventions can improve outcome.
Description
Keywords
Nodding syndrome, Epilepsy, Growth, Puberty, Hormones
Citation
Piloya-Were, T., Odongkara-Mpora, B., Namusoke, H., & Idro, R. (2014). Physical growth, puberty and hormones in adolescents with Nodding Syndrome; a pilot study. BMC Research Notes, 7, 1-5. doi:10.1186/1756-0500-7-858