The role of insulin like growth factor (IGF)–1 and IGF-binding protein-3 in diagnosis of growth hormone deficiency in short stature children.

Objective. To evaluate the role of IGF-1 and IGFBP-3 in diagnosis of short stature children and adolescents in whom Growth Hormone Deficiency (GHD) was found. Methods. In this cross sectional study the referred short stature children and adolescents to Namazi Hospital in Shiraz- Iran, in 2003-2005 were studied. The inclusion criteria were proved short stature based on the physical examination, weight, height, standard deviation score (SDS) of height < -2 , with considering stage of puberty and predicted height in children without any genetic or chronic disorders. The exclusion criteria were any positive physical or laboratory data suggesting hypothyroidism, rickets or liver disorders. For all patients a provocative growth hormone test was performed with propranolol and L-dopa and serum IGF-1 and IGFBP-3 were measured. GHD defined as peak(cutoff ) serum GH level under 10 ìg/L and low IGF-1 and IGFBP-3 considered as cutoff serum level under -2 standard deviation. Results. Eighty one short stature patients (39 boys and 42 girls) with mean age of 10.6 ± 3.5 years completed the study. Seventeen patients with GHD were found and in 18 patients IGF-1 level were low. Only in 6 patients both GH and IGF-1 were low and 2 of them had low IGFBP-3. There were no correlations between the levels of GH,IGF-1 and IGFBP-3 in children with short stature due to GHD. The sensitivity and specifity of IGF-1 and IGFBP-3 in assessment of GHD were 35% and 81% for IGF-1 and 12% and 94% for IGFBP-3, respectively. Conclusion. No correlations were found between GH level and serum levels of IGF-1 and IGFBP-3 in short patients and the sensitivity of these tests in assessment of GHD was poor.

Intravenous immune globulin versus intravenous anti-D immune globulin for the treatment of acute immune thrombocytopenic purpura.

OBJECTIVE: The purpose of this study was to compare the efficacy and side effects of intravenous immunoglobulin (IVIG) with intravenous anti-D immunoglobulin for treatment of newly diagnosed acute childhood Idiopathic thrombocytopenic purpura (ITP). METHODS: Children (6 months to 14 years) with newly diagnosed acute ITP and platelet count below 20,000/ microL were randomized to receive single dose intravenous 75 microg/kg anti-D or 1g/kg IVIG for two consecutive days (total dose 2 g/kg). Response rate defined as a platelet count over 20,000 / microL 72 hours after initial treatment. RESULTS: Eighty one patients (52 male and 29 female) with mean age of 5 years and 3 months randomly divided in anti-D group (n=42) and IVIG group (n=39). Mean baseline (pretreatment) platelet counts were 15406 / microL and 15230/ microL in anti-D and IVIG group, respectively. The response rate in IVIG group (98%) was more significant than anti-D group (76%); (P = 0.017). After 7 days the platelet counts of all patients in IVIG group were more than 20,000/ microL while in anti-D group 12% had platelet counts below 20,000/ microL. CONCLUSION: In acute childhood ITP, initial treatment with IVIG (2g/Kg in divided dose) increased platelet count more rapidly and more significant than intravenous anti-D (single dose of 75 microg/kg) within the first 72 hours.

Validation of initial serum creatinine as a predictive factor for development of end stage renal disease in posterior urethral valves.

OBJECTIVE: The aim of this study was to determine the value of initial serum creatinine in prediction of End-stage renal disease (ESRD) in children with posterior urethral valves PUV. METHODS: In this retrospective study, patients diagnosed with PUV admitted in Aliasghar Children's Hospital in Tehran, Iran from 1991 through 2001 were studied. Based on the development of ESRD (need for dialysis) they were classified in two groups and their demographics, initial presentations, laboratory data, imaging findings and outcomes were reviewed and compared. RESULTS: Thirty six male patients with PUV, treated with valve ablation (13), vesicostomy (13), or high ureterostomy (10) were followed for developing ESRD. Sixteen of the 36 patients developed ESRD at their last follow-up. There was no significant difference in age at initial presentation, presence of urinary tract infections, incidence of vesicoureteral reflux, renal dysplasia or type of primary surgical intervention between the patients with or without ESRD. Patients who progressed to ESRD had serum creatinine concentration > 1.0 mg/dl at diagnosis as compared to those without ESRD (P = 0.001). CONCLUSION: Initial serum creatinine is a valuable factor for prediction of renal outcome in patients with PUV.

Diagnostic utility of autopsy in a university hospital in Iran.

OBJECTIVE: The objective of this study were to evaluate the diagnostic utility of the autopsy in University hospital in Tehran, Iran. METHODS: In this retrospective descriptive-analytic survey during a six years period from 1998 to 2003, autopsies in the Bahrami Children Hospital, a teriary care hospital in Tehran, were studied. The clinical and autopsy diagnoses were compared and categorized as follows: 1. Change (clinical and autopsy diagnoses discordant), 2. Add (significant unexpected findings noted on the autopsy, although the clinical diagnosis was not altered), 3. Confirm (clinical and autopsy diagnosis concordant), 4. Autopsy inconclusive. RESULTS: Eighty four autopsies were studied. Out of 350 neonatal deaths in the period, autopsy was performed in 74 neonates (21%) and of 249 under 5 year deaths (except neonates) autopsy was performed in only 10 cases (4%). In 61 cases (73%) the autopsy diagnoses confirmed the clinical diagnosis, in 10 cases (12%) it changed the clinical diagnosis, in 11 cases (13%) it significantly added to the clinical diagnosis and in 2 cases (2%) it was inconclusive. CONCLUSION: This study demonstrated that neonatal and infantile autopsy continued to provide clinically useful data in 25% of cases and remains an invaluable tool in pediatric medicine.