Response of recombinant human growth hormone in isolated growth hormone deficiency

To observe the response of growth hormone in isolated growth hormone deficiency. Moreover the response was compared when treatment was initiated earlier or later in childhood. Quasi experimental study. The study was conducted in the department of paediatrics, Military Hospital Rawalpindi, from January 2001 to January 2006. Thirty children [19 boys, 11 girls] with isolated growth hormone deficiency, aged 3-17 years [mean 7.2 +/- 3.4] were identified. Their mean growth velocity was 1.8 cm/year [SD 0.6]. They were treated with intramuscular injections of recombinant human growth hormone [rhGHT] [0.1 U/ kg] 06 times a week for first three years of treatment. After three years of rhGHT the mean height gain was 33.5 cms [SD 8]. Mean height gain in 1st year was 15.5 cms [SD 4], in second year 10.4 cms [SD 2.8], in third year 7.6 cms [SD 2.0]. Mean total height gain was 40.6 cms [SD 4.2] when treatment was started before the age 06 years was 28.8 cms [SD 7.2] when treatment was started after the age 06 years [p < 0.05]. This study clearly demonstrates that children with isolated growth hormone deficiency treated with intramuscular rhGH resulted in increase in growth velocity but was more pronounced when started early [before age six years]

Wilms tumour a comparison of surgical aspects in patients with or without pre-operative chemotherapy

To compare the technical aspects of Wilms' tumour [WT] surgery in patients with and without pre-operative chemotherapy. Quasi-experimental. Military Hospital [MH] and Combined Military Hospital [CMH], Rawalpindi, from January 1999 to December 2004. Patients of WT, presenting between January 1999 and December 2001, were treated, using the NWTSG protocol, with primary surgery [group I]. Between January 2001 and December 2004, WT patients were treated according to SIOP protocol, with pre-operative chemotherapy followed by surgery [group II]. Volume reduction with chemotherapy, duration of surgery, rupture of tumour, extent of excision, adherence and damage to surrounding structures, blood loss, complications, stay in hospital and event-free survival [EFS] were compared in the two groups. Out of 22 patients in group I, 19 [86.4%] underwent primary surgery. Of the 23 patients in group II, 21 [91.3%] received pre-operative chemotherapy followed by surgery. Average volume reduction in this group was 54% with chemotherapy. Difference in duration of surgery and blood loss was significantly low in group II [p=0.003 and p<0.001, respectively]. In group I, rupture [6 vs 2], adherence [14 vs 10] and damage to surrounding structures [5 vs 2] were more. Complete macroscopic excision was possible in 90.5% of WT in group II as compared to 73.7% in group I. Immediate postoperative complications and length of hospital stay were similar in both groups. There was no difference in EFS. Pre-operative chemotherapy makes it technically easier and safer to operate, without jeopardizing the final outcome

Severe, recurrent hiatal hernia in Schwartz syndrome

An 18 months old baby boy was brought by parents with recurrent vomiting since birth, which had increased over the past four months, failure to gain weight, and altered facial features with delayed development. The baby was reported perfectly normal looking till the age of six months. The parents were first cousins, and married for four years. This was their second child and their first baby had died within a week of birth with an encephalocele. The patient had been variably treated for pneumonia, tuberculosis and malnutrition with no improvement. On examination the baby was emaciated and stunted weighing just 6.9 kg with a length of 68 cms, but alert and apprehensive of strangers. There was blepharophimosis with twitching of the upper eyelids, a puckered mouth and abnormal posturing of the limbs. The knees and elbows were flexed, hands fisted and feet were planter flexed. All joints could be passively extended with ease. There were no dysmorphic features. At 18 months of age the baby could not sit independently, though he could cruise along furniture on tip toes, could eat with his own hand, could speak with a vocabulary of 5-6 meaningful words and had appropriate fine motor movements so that he could draw a single match stick out of its box. Though there was generalized hypertonia, the tendon jerks were not exaggerated. Gastroesophageal reflux with tetany was the initial diagnosis. Urine R/E, Serum calcium, parathormone, bicarbonate and serum albumin, serum CPK, aldolase were within normal limits. Barium meal showed a sliding hiatal hernia with marked gastro esophageal reflux and a dilated esophagus. EMG and NCS showed no evidence of myopathy or neuropathy. Ultrasonography of abdomen and CT scan of brain were normnal. Radiograph of the clavicles was normal but the lumbosacral spine showed shortening of the vertebrae. Baseline ECG was also normal. Having excluded the other conditions, a diagnosis of Schwartz syndrome with hiatal hernia was made. After initial supportive management surgical correction with fundoplication was carried out. Carbamazepine helped to revert the tone to near normal. Three months later the patient weighed 9 kg but the tone was still more than normal. Ternaline was added to the treatment and the tone remarkably improved. A repeat barium meal at that time showed normal position of the stomach and a slightly dilated esophagus