The change in serum IGF-1 z-scores in response to GHT in Indian children may not be a good indicator for monitoring growth hormone responsiveness.

McCune-Albright syndrome (MAS) is defined by the clinical triad of fibrous dysplasia of bone (FD), café-au-lait spots and precocious puberty (PP). It is a rare disease with estimated prevalence between 1/100,000 and 1/1,000,000. The authors report a case of MAS in an Indian boy who had history of unilateral macroorchidism (but no other signs of MAS) since birth, then presented with PP, FD and café-au-lait spots at 6 years of age. On examination he had asymmetry of the face, café-au-lait spots and signs of sexual precocity with a right testicular volume of 25 ml (left 8 ml). Investigations revealed suppressed gonadotropins with elevated testosterone levels. Skeletal survey showed dysplastic changes involving multiple bones and advanced bone age. Reports of MAS in a boy with unilateral macroorchidism are scarce. Thus, unilateral macroorchidism at birth in a baby may be a feature of MAS and should be followed up with care.

The present results suggest that the WHO 2006 growth standards classify children with growth disorders appropriately and the classification is in concordance with the clinical assessment. They provide health practitioners in a clinical setting with an effective tool to assess growth of children.

A 15-year-old male child, born of a third degree consanguineous marriage presented with an acute onset progressive weakness of both lower and upper limbs without any sensory, bowel or bladder involvement. The patient had been admitted with a similar episode at the age of 5 years. Examination at the time of fi rst hospitalisation had revealed that the child was 120 cm (2.2 SD) tall, weighed 21 kg (1.0 SD) and his blood pressure was 240/120 ( > 95th percentile). He had hyperpigmentation (as compared with the other family members), signs of virilisation, his voice had broken and he was in pseudoprecocious puberty with Tanner staging axillary hair 2, pubic hair 2, genital 5 and testicular volume 2 mL each. Motor system examination revealed proximal muscle power grade 2/5 (Medical Research Council grading) in both the lower and the upper limbs with intact sensations and grade 1 deep tendon refl exes. Blood tests revealed a serum potassium concentration of 1 mEq/L (3 -5 mEq/L), serum sodium of 158 mEq/L (133 -145 mEq/L), serum adrenocorticotrophic hormone (ACTH) was > 1000 pg/mL ( < 50 pg/mL), serum testosterone of 12 nmol/L (adult range), 17-hydroxy progesterone (17-OHP) was > 12 µ g/L ( < 5 µ g/L), 11-de-oxycorticosterone was 12 nmol/L (0.06 -1.03 nmol/L) and his bone age (Tanner Whitehouse 2 method) was 11 years (chronological age 5 years). In view of his pseudoprecocious puberty (testicular volume 2 mL), hypertension, hyperpigmentation, raised 17-OHP, ACTH, androgens and 11-de-oxycorticosterone, a fi nal diagnosis of congenital adrenal hyperplasia due to 11 β -hydroxylase defi ciency was made, and child was treated with intravenous hydrocortisone and potassium (1, 2). His weakness completely recovered in the next 48 h and he was discharged on oral hydrocortisone (10 mg/m 2 /day) (3).After 14 years of age, he was non-compliant, had become very rebellious and had stopped all medication. The patient had another attack of weakness in all four extremities very similar to that which had occurred at 5 years of age. On this admission patient was 161 cm (0.1 SD) tall and weighed 44 kg ( -0.5 SD). Patient also had bradycardia (pulse rate 54/min) with an irregular bounding pulse, respiratory rate of 22/min, blood pressure in the supine position of 180/110 mm Hg ( > 95th percentile). Generalised hyperpigmentation was noticed which was more pronounced on the knuckles, nipples, lips, axilla and groin (Figure 1 A, B). On performing Tanner staging he was found to have adult Tanner stage 5.The proximal muscle power was grade 2/5 in both the lower and the upper limbs with weakness of the neck Figure 1 (A, B) Generalised hyperpigmentation which was more pronounced on the knuckles, nipples, lips and axilla. (C) Electrocardiogram showing bradycardia, presence of U waves, a prolonged QT interval (0.64 s) and left ventricular hypertrophy.Brought to you by |

Indian growth hormone deficient children with hypoplastic pituitary respond better to therapy with GH in short term.