ABSTRACT
Ten children, five boys and five girls with true precocious puberty at an early age were found to have hypothalamic hamartomas on brain imaging. Very early onset of puberty, varying from a few weeks to three years of age, and rapid progression were characteristic. Accelerated growth velocity and markedly advanced bone age were evident in all. Gonadotropin and gonadal hormone levels were elevated above the prepubertal range. Six children had associated developmental delay or hyperactivity.
Subject(s)
Child, Preschool , Female , Gonadal Steroid Hormones/blood , Hamartoma/blood , Humans , Hypothalamic Neoplasms/blood , Hypothalamus/pathology , Infant , Magnetic Resonance Imaging , Male , Puberty, Precocious/bloodABSTRACT
Eighty children (58 girls and 22 boys) with isosexual precocity seen in the past eight years were evaluated clinically and investigated to identify the underlying cause. Of these, 50% (29 girls and 11 boys) had centrally mediated true precocious puberty (TPP). The girls could be classified into five major groups (I) Central precocious puberty 29-subclassified into idiopathic (ITPP, 15) and organic or neurogenic (NTTP, 14), (II) Premature thelarche (PT, 20), (III) Premature menarche (PM, 2), (IV) Premature adrenarche (PA, 5), and, (V) Others: hypothyroid (n = 1), and McCune Albright Syndrome (n = 1). ITPP as a cause of precocity in girls was seen less often (52%) and NTPP more often (48%) compared to most Western series, with tubercular meningitis as the cause in 31% and hypothalamic hamartomas in 10%. Though the LH and estradiol levels were significantly higher (p < 0.05) in TPP, compared to PT, these were not helpful in differentiating because of considerable overlap. LH-predominant-response (LH/FSH ratio > 1) to LHRH testing was seen in TPP. Amongst the 22 boys, 11 (50%) had TPP, ITPP in 27% and NTPP in 73%. Hamartomas (n = 4) and TBM (n = 3) contributed equally to NTPP; pineal tumor was seen in one. The adrenal (n = 7) and testicular (n = 2) causes together involved 41% of the boys with precocity, congenital adrenal hyperplasia (CAH) CAH, 11-beta hydroxylase being the commonest cause. Of the 6 boys witdeficiency was found in four and nonsalt losing form of 21-hydroxylase deficiency in 2. Testicular and adrenal tumors and testotoxicosis were noted in one case each. The etiologic factors were more varied in boys.
Subject(s)
Adrenal Gland Diseases/complications , Age Determination by Skeleton , Breast/growth & development , Central Nervous System Diseases/complications , Child , Child, Preschool , Female , Humans , Infant , Male , Menarche/physiology , Penis/growth & development , Puberty, Precocious/classification , Retrospective Studies , Testicular Diseases/complicationsABSTRACT
Of the 430 children referred for the evaluation of short stature 100 (23%) were confirmed to have growth hormone deficiency. The male to female ratio was 1.94:1. Less than 10% belonged to the lower socio-economic group. Most of the cases (73%) presented between the ages of 6-15 years though growth failure was usually recognised earlier. Minimum of two stimulation tests were performed in each case. Seventy five GH deficient children had idiopathic GHD (IGHD) and 31% of these were familial. Fourteen had organic causes and 11 had GH resistance. Of 75 with IGHD, 18 had abnormal deliveries, breech or birth asphyxia. Multitropic pituitary hormone deficiency (MPHD) was found in 9/75 cases of idiopathic GHD and in three of the organic group. The height age was much more retarded than chronologic age in the GH resistant group (p less than 0.05) and the HA/BA ratio was also lowest in this group (p less than 0.001). Growth velocity was less than 4 cm/year in all the GHD children but was lowest in those with MPHD. The interesting feature of this study is the marked predominance of the familial cases 31% and a high incidence of growth hormone resistant cases (11%).