title: Physical growth, puberty and hormones in adolescents with Nodding Syndrome; a pilot study.
abstract: BACKGROUND
  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.
authors.forename: Theresa
authors.initials: T
authors.lastname: Piloya-Were
authors.forename: Beatrice
authors.initials: B
authors.lastname: Odongkara-Mpora
authors.forename: Hanifa
authors.initials: H
authors.lastname: Namusoke
authors.affiliation: Department of Paediatrics and Child Health, Mulago Hospital/Makerere University College of Health Sciences, P,O Box 7072, Kampala, Uganda. ridro1@gmail.com.
authors.forename: Richard
authors.initials: R
authors.lastname: Idro
mesh_terms: D000293:Adolescent; D001827:Body Height; D001842:Bone and Bones / Q000378:metabolism / Q000503:physiopathology*; D004967:Estrogens / Q000097:blood; D005260:Female; D005640:Follicle Stimulating Hormone / Q000097:blood; D006066:Gonads / Q000378:metabolism* / Q000503:physiopathology; D013006:Growth Hormone / Q000097:blood; D006801:Humans; D018972:Insulin-Like Growth Factor Binding Protein 3 / Q000097:blood; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D007986:Luteinizing Hormone / Q000097:blood; D008297:Male; D064128:Nodding Syndrome / Q000097:blood / Q000503:physiopathology*; D010281:Parathyroid Hormone / Q000097:blood; D010865:Pilot Projects; D010913:Pituitary-Adrenal System / Q000378:metabolism* / Q000503:physiopathology; D011388:Prolactin / Q000097:blood; D011627:Puberty / Q000097:blood*; D013739:Testosterone / Q000097:blood; D013961:Thyroid Gland / Q000378:metabolism
chemical_list: D004967:Estrogens; C583557:IGF1 protein, human; C515497:IGFBP3 protein, human; D018972:Insulin-Like Growth Factor Binding Protein 3; D010281:Parathyroid Hormone; D013739:Testosterone; D007334:Insulin-Like Growth Factor I; D011388:Prolactin; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone; D013006:Growth Hormone