Pituitary enlargement on magnetic resonance imaging in congenital hypothyroidism.

OBJECTIVE: To assess pretreatment and posttreatment pituitary gland size by magnetic resonance imaging in children with subtle or overt signs of long-standing hypothyroidism. DESIGN: Etiologic diagnosis of hypothyroidism was confirmed by thyrotropin, triiodothyronine, and thyroxine assays; thyroid antibody tests; and radionuclide thyroid scan. Repeated magnetic resonance imaging was obtained after 6 to 12 months of therapy with levothyroxine sodium to restore a euthyroid state. SETTING: Endocrine service at a hospital for children in Bombay, India. PATIENTS: Ten children whose mean (+/-SD) chronologic age, bone age, and duration of symptoms were 11.39 +/- 1.81, 3.78 +/- 2.05, and 6.95 +/- 2.91 years, respectively. One patient was seen for acute neurologic symptoms suggesting a suprasellar lesion. RESULTS: Magnetic resonance imaging showed homogeneous diffuse enlargement of the pituitary gland in all patients. The superior margin of the gland was flat in five patients and convex in the rest, with suprasellar extension and partial or complete obliteration of the infundibulum in three and mild compression of optic chiasma in two, thus mimicking a sellar or suprasellar tumor. Pretreatment pituitary mean (+/-SD) vertical height in the coronal plane was 10.02 +/- 2.24 mm, with a posttreatment regression to 4.93 +/- 1.11 mm (P < .001, Student's t test) and restoration of clinical and hormonal euthyroid status. CONCLUSION: Awareness of pituitary enlargement and the rare occurrence of neurologic symptoms and chiasmal syndrome are important in children with longstanding congenital hypothyroidism.

Hypothalamic hamartomas and precocious puberty.

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.

Isosexual precocity: the clinical and etiologic profile.

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.

Profile of growth hormone deficiency in Bombay.

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%).

Short stature in Indian children: the extent of the problem.

PIP: A study of child growth included 2500 consecutive admissions to Bai Jerbai Wadia Hospital for Children in Bombay, India. 140 (5.6%) were considered to be of short stature (less than the 5th percentile of an Indian standard). The causes of growth retardation were in order of frequency: protein energy malnutrition (42), chronic systemic disease (23), chronic anemia (19), skeletal disorders (16), constitutional short stature (15), endocrine disorders (15), intrauterine growth retardation (5), chromosomal disorders (2), and miscellaneous (3). All 10.7% of cases with endocrine problems had congenital hypothyroidism. A study of short stature among 430 children referred to the same hospital's endocrine clinic showed endocrine disorders were responsible for most short stature cases (143 or 33.3%). 97 of these cases (67.8%) had a deficiency of growth hormone, while just 6.3% suffered from hypothyroidism. Malnutrition and chronic disease caused short stature in just 8.4%. Another survey conducted in the outpatient clinic showed that the height of 50 of 500 (10%) children was below the 5th percentile of the Indian standard. 32% were below the 3rd percentile of a Western standard. A study of 200 children referred to the hospital for their short stature demonstrated that 132 (66%) were below the 5th percentile of the Indian standard. 67.8% of the children between the Indian standard's 5th percentile and the Western standard's 3rd percentile were of normal variant short stature. In fact, 90.4% of their heights compared to their parents' heights. 56.8% of those below the 5th percentile had endocrine problems and just 19.6% correlated with their parents' height. 52% of these children were deficient in growth hormone. Therefore, growth hormone deficiency accounted for 20% of all short stature cases.^ieng