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In this experiment, we studied the effect of valproate (VPA) on weight gain, and serum leptin levels in prepubertal epileptic children receiving VPA. Our purpose was to determine whether or not long-term use of VPA causes weight gain in childhood, and to evaluate serum leptin levels in a group of prepubertal children receiving VPA. Our study included 15 patients (9 males, 6 females) with new diagnosed epilepsy and 16 healthy age-matched controls (9 males, 7 females). The subjects' ages ranged from 9 months to 12 years. Weight gain was noted in 9 (60%) of 15 patients in the study group, and 8 (50%) of 16 subjects in the control group (p > .05). There was no difference between the groups for body mass index (BMI) and serum leptin levels. Although higher serum leptin levels were found in the patients treated with VPA weight gaining (5.65 +/- 3.06 ng/ml vs. 3.28 +/- 1.69 ng/ml), we did not find a difference between the patients weight gaining and nonweight gaining (p > .05). While a significant correlation between BMI and serum leptin levels was found in the study group (r = .704; p = .003), it was not significant in the control group (r = .330; p = .211). In conclusion, our findings showed that long-term use of VPA did not cause weight gain in a group of prepubertal children receiving VPA and, parallel to this, serum leptin levels were similar in both the control and study group.

Intraabdominal adhesions represent a significant problem because of the morbidity associated with adhesive disease, including small bowel obstruction, difficulties in reoperative surgery, and possibly chronic pain. Coating solution of sodium hyaluronate (Sepracoat; Genzyme Production-Surgical Products, Cambridge, MA) was studied in New Zealand white rabbits to determine its potential role for prevention of postoperative adhesions following laparoscopic intraabdominal mesh insertion. A 2-cm polypropylene mesh was inserted laparoscopically to the left iliac fossa and fixed to anterior abdominal wall using a single prolen suture. Group 1 (n = 10) acted as the control group. Mesh was coated using 4% sodium hyaluronate in phosphate buffered saline (Sepracoat) in Group 2 (n = 10). Fourteen days later, all animals underwent diagnostic laparoscopy, and findings were recorded. All animals then were killed, the abdominal cavities were inspected, and adhesions were graded from 0 to 4. All meshes were removed and sent for histologic examination. The degrees of inflammation, fibrosis, and congestion were scored. No adhesions were seen on trocar sites on both groups. Eight of 10 animals in the control group and 5 of 10 animals in the study group had intraabdominal adhesions. The scoring of adhesions revealed that study group had only one (10%) significant adhesion, whereas the control group had eight (80%; < 0.001). Our study suggests that the Sepracoat reduces the incidence and severity of abdominal adhesions following laparoscopic mesh insertion and should be considered as a prophylactic agent, especially in those undergoing laparoscopic transabdominal mesh repair for hernia.

Unlabelled: This study intends to describe growth and endocrine disorders secondary to chemotherapy among long-term survivors of pediatric acute leukemia. Sixteen patients including 14 ALL and 2 AML entered the study. Four were females and 12 were males with the mean age of 17.38 ± 3.81 years. Following the completion of their therapy, the mean follow up period of the patients was 62.43 ± 41.11 months. Somatic growth, sexual maturation, hypothalamic- pituitary-thyroid axis and hypothalamic-pituitary-gonadal axis were evaluated in all the patients. Two out of 16 had pathologic short stature (12.5%) and 3 patients had enuchoid status (18.75%) with antrophometric measurements. Eleven patients had normal thyroid gland dimensions and homogeneous thyroid tissue on ultrasonographic examination. One patient had Ia and another four patients had Ib diffuse goitre according to WHO criteria. Two out of 16 patients were diagnosed as subclinic primary hypothyroidism (12.5%), and three of them were diagnosed as subclinic central hypothyroidism (18.75%) according to TRH testing. Three patients were subclinic subtle central hypothyroidism. Thyroid auto-antibodies were in normal range in all patients. Eight patients (66.6%) out of 12 male subjects revealed impaired HHG axis. Hypergonadotropic hypogonadism (Leydig and Sertoli dysfunction) were observed in five of them and isolated Sertoli dysfunction was detected in three of them. Azoospermia was encountered in all patients with isolated Sertoli dysfunction. Testicular biopsy was obtained from only one of them and atrophic testicular tissue was detected. Female patients show normal pubertal development and gonadal functions., Conclusion: Subclinic subtle primary and subclinic central hypothyroidism were found in 31.2%, impaired Sertoli and Leydig with Sertoli cell function in 66.66% of long-term survivors pediatric acute leukemia and testicular tissue was more sensitive to adverse effects of chemotherapy than ovarian tissue.

Deficiency of carbonic anhydrase II (CA II) isoenzyme produces metabolic disorders of bone, kidney and brain. In this report we describe the clinical, radiological, pathological and genetic findings in three brothers who were affected with the autosomal recessive syndrome of osteopetrosis, renal tubular acidosis (RTA) and cerebral calcification. The RTA was hybrid type, but urinary concentration ability was intact. Additional features were severe mental retardation, stunted growth, microcephaly, dental malocclusion, high-arched palate, and broad thumbs. Previous reported patients with this syndrome were predominantly from the Middle East and Mediterranean region. This is the first report with CA II deficiency from the Turkish population. The presence of mental retardation and relative infrequency of skeletal fractures in our patients resembles the clinical course of patients with the Arabic mutation of the CA II gene, but this mutation was not found in our patients.

Familial glucocorticoid deficiency (FGD) or unresponsiveness to ACTH at the receptor level is a rare autosomal recessive hereditary syndrome characterized by a low cortisol level despite high serum ACTH concentration. Aldosterone levels are normal. The clinical entity generally presents in the first year of life with skin hyperpigmentation and hypoglycemic convulsions. Cortisol response to exogenous ACTH is also absent. Unresponsiveness to ACTH may be due to a mutation in the ACTH receptor; sometimes no mutation is found. We discuss the clinical and laboratory findings and genetic studies in six patients with a diagnosis of FGD. A homozygous V142L mutation was detected in three of the patients and a homozygous D103N mutation was detected in two patients.

Objective: Obesity and malnutrition are the main nutritional problems of childhood. Tumor necrosis factor-alpha (TNFalpha) is a well-known cytokine with effects on adipose tissue. In this study relative body mass index (RBMI) and serum TNFalpha levels were compared in obese and malnourished children, and hyperinsulinism was evaluated in the obese children., Subjects and Methods: Thirty-four children of whom 14 (9 M, 5 F) were obese, 10 (5 M, 5 F) were malnourished and 10 (4 M, 6 F) constituted the control group were evaluated. RBMI was used to define the groups according to the formula: patient's BMI/predicted BMI x 100. Mean RBMI values were 148.7 +/- 26, 77.6 +/- 4.63, and 100 +/- 8.2 in the obese, malnourished and control group, respectively. Mean ages of the obese, malnourished and control children were 9.75 +/- 2.59, 12.3 +/- 1.86 and 12.4 +/- 1.3 years, respectively., Results: Higher serum TNFalpha levels were found in the obese children (20.94 +/- 0.38 pg/ml) in comparison with the control group (2.97 +/- 0.05 pg/ml) (p <0.001). Mean TNFalpha level in the malnourished group was 2.77 +/- 0.04 pg/ml, not statistically different from the control group (p >0.05). TNFalpha concentrations of the hyperinsulinemic and normoinsulinemic obese patients were not significantly different (p >0.05)., Conclusion: We showed higher levels of serum TNFalpha in obese children. Increased TNFalpha levels do not always reflect hyperinsulinism. There was no difference in TNFalpha levels between the controls and the malnourished group.

The concurrence of ambiguous genitalia, nephropathy and predisposition to Wilms' tumor are characteristics of Denys-Drash syndrome. Some of the reported patients do not express the full spectrum of the syndrome, while the occurrence of nephropathy has become a generally accepted common feature of this syndrome. We report an infant with male pseudohermaphroditism due to partial gonadal dysgenesis and nephropathy without Wilms' tumor but with a Wilms' tumor suppressor gene (WT1) mutation. The high risk of Wilms' tumor mandates regular surveillance and the use of prophylactic bilateral nephrectomy as a treatment is not yet clear.

Background: To evaluate the growth hormone reserve and the growth hormone response to recombinant human growth hormone (GH) in prepubertal thalassemic children with growth retardation., Methods: Twenty thalassemic patients with short stature and delayed bone age were studied. Patients were randomized into GH-treated (n = 10) and non-GH treated (control; n = 10) groups. The GH-treated group received recombinant human (rh)-GH (Genotropin) at the dose of 0.7 IU/kg per week for 12 months., Results: There was a significant discordance between GH response to pharmacologic stimuli and physiological secretion of GH/GHRH testing. Following the administration of rhGH, growth velocity increased from 2.47 +/- 0.48 cm/year to 6.27 +/- 0.76 cm/year (P = 0.005), whereas there was not a similar change in the non-GH-treated group. The height velocities of the two groups during the 1 year follow-up period were significantly different (6.27 +/- 0.76 vs 3.99 +/- 0.34 cm/year; P = 0.025). There were significant differences between the height velocity improvements and height velocity standard deviation scores of the two groups as well., Conclusion: The present study has demonstrated that rhGH is a safe and efficacious mode of treatment in thalassemic children.

Introduction: The purpose of this study was to determine the pancreas reserve in siblings of diabetic patients by screening islet cell antibodies (ICA), insulin auto antibodies (IAA), reduced C-peptide levels, first-phase insulin release and the derangement of cellular immunity (reduction of natural killer cells, abnormality of the T cell subpopulations)., Methods and Results: Twelve siblings (aged 9.3 +/- 2.8 years) of diabetic children were evaluated and results were compared with the control group (12.1 +/- 3.5 years). For siblings of the diabetic children, fasting, post-prandial and glucagon response C-peptide mean values were 2.2 +/- 1.2, 7.2 +/- 7.1 and 5.3 +/- 3.6 ng/mL, respectively, while in the control group they were 1.5 +/- 0.8, 3.6 +/- 2.0 and 5.1 +/- 2.9 ng/mL, respectively. There were no differences between the two groups. In 33%, postprandial C-peptide, and in 11% of the siblings, glucagon response C-peptide values were exaggerated. In siblings the first phase insulin release (FPIR) during an intravenous glucose tolerance test was 128.5 +/- 96.6 (above the 50th percentile) and stimulated insulin release (SIR) was 103.8 +/- 92.5 (above 25th percentile). Sibling values were significantly lower than the control group (FPIR 152.4 +/- 42.5, P = 0.01; SIR 134.9 +/- 38.2, P = 0.01). Values for FPIR (in two children) and SIR (three cases) were below the 5th percentile. In one, FPIR and SIR levels were both below the 1st percentile. Islet cell antibodies and IAA were also present in this subject. Treatment with nicotinamide was started in the cases with FPIR and SIR below the 5th percentile. We did not observe overt diabetic symptoms during the follow-up period of more than 3 years., Conclusion: We recommend that borderline insulin secretion be tested annually in siblings who show insufficient FPIR.

Shoulder pain (SP) is frequently mentioned in recent literature following laparoscopic operations. In the literature, many causes have been declared to explain shoulder pain after CO2 insufflation, such as direct peritoneal irritation of the CO2 gas, excessive traction of the triangular ligament, and overstretching of the diaphragmatic muscle fibers due to the high rate of insufflation. This study was planned as multicentric, and 76 patients, aged between 35 to 45, were entered into the study. They were all selected by a randomized sampling method, with equal numbers of men and women, to achieve true evaluation. The low flow-rate (LFR) group was insufflated with 2.5 L/min and the high flow-rate (HFR) group with 7.5 L/min. All cases were evaluated by subjective pain classification on postoperative day 3. According to the subjective pain scale method, shoulder pain average was 23.9+/-3.1 in the LFR group and 55.4+/-6.5 in the HFR group. The difference between these groups was significant (p > 0.01). There is no significant difference for the operation time (LFR%: 64+/-15 minutes, HFR: 61+/-20 minutes, p > 0.05). Our results suggest that there is a significant statistical relation between the postoperative shoulder pain levels and increased insufflation rates. For this reason, low insufflation rate significantly reduces the shoulder pain but does not increase the operation time. Therefore, a low insufflation rate should be applied in all cases for patients' comfort and safety.

Background: Isolated deficiency of glucocorticoids is characterized by elevated levels of adrenocorticotropin (ACTH) and normal aldosterone production. It is rare for isolated deficiency of glucocorticoids to be associated with liver involvement. A case of an infant with isolated deficiency of glucocorticoids presenting with cholestasis is presented in this article. A male infant on his 39th postnatal day was referred to our hospital for evaluation of prolonged jaundice and convulsion. He had two episodes of hypoglycemic convulsion on postnatal 8th and 39th day, after which he was admitted to our hospital., Results: Physical examination revealed systemic jaundice, hyperpigmentation of the skin, hepatomegaly and splenomegaly on admission. He had normal male genitalia with 3.5 cm of penis and bilateral scrotal testes. Laboratory values were as follows: glucose 45 mg/dL, total biluribin 18.14 mg/dL, direct biluribin 6.54 mg/dL, aspartate aminotransferase 378 IU/L, alanine aminotransferase 46 IU/L, and alkaline phosphatase (ALP) 1302 IU/L. In abdominal ultrasound and biliary tract scanning, extra- and intrahepatic biliary tracts were shown to be normal. Finally, biopsy of the liver revealed cholestasis. An endocrinological evaluation showed high levels of adrenocorticotropin (ACTH, 1000 pg/mL), low levels of cortisol (1 microgram/dL) and normal aldosterone levels., Conclusions: The diagnosis of cholestasis secondary to isolated glucocorticoid deficiency was suspected with clinical and laboratory findings. Hydrocortisone treatment (30 mg/m2 per day) was initiated after which hyperpigmentation and jaundice decreased and ACTH and ALP levels reduced to 39 pg/mL and 440 IU/l, respectively. We emphasize that cholestasis in infants may be a component of isolated deficiency of glucocorticoids.

The aim of this study is to describe a new technique using laparoscopic instruments for percutaneous bladder stone management and determine the efficacy of this procedure. The technique is based on percutaneous removal of the bladder stone(s) by using laparoscopic instruments under video-cystoscopic visualization. Laparoscopic large grasper or butterfly net like sac were used to extract bladder stones in six patients who were candidates of open cystolithotomy. Satisfactory results have been obtained in patients with solitary bladder stones that have diameters up to 3 cm or multiple stones. In selected cases, operation time, urethral and mucosal damage, hospital stay, and cost can be decreased to a minimum with this simple technique. It may be an alternative method to previously reported percutaneous bladder calculi management modality, in which percutaneous nephrolithotomy devices have been utilized.

Empty sella syndrome (ESS) is a multicausal entity. The incidence of primary empty sella syndrome (PESS) in children with neuroendocrine dysfunction is not known. In the pediatric age group, frequency seems to have been underestimated. A total of 117 cases of neuroendocrine disorders, including complete growth hormone deficiency, primary hypothyroidism with pituitary resistance to thyroid hormone, obesity, central precocious puberty, hypothalamic hypogonadism and central diabetes insipidus, have been studied with computed tomography and/or magnetic resonance imaging of sellar region for etiologic evaluation. Twenty-one patients were found to have PESS. We noted a high incidence of PESS in children with neuroendocrine dysfunction (17.9%). Children with neuroendocrine dysfunction should be investigated with respect to PESS, and children with PESS recognized coincidentally should be studied with the particular consideration of subclinical neuroendocrine dysfunction.

To assess the growth-promoting effect of different doses of growth hormone-releasing hormone(1-29)-NH2 (GHRH(1-29)-NH2) in GH deficiency (GHD) of hypothalamic origin, 43 prepubertal children aged between 4.3 and 18.9 years (mean 10.4 +/- 2.9 years) were randomly assigned to three treatment regimens: low-dose GHRH(1-29)-NH2 (LD group; n = 15), high-dose GHRH(1-29)-NH2 (HD group; n = 12) and GH (GH group; n = 16). The LD group received GHRH(1-29)-NH2 at 30 micrograms/kg/day s.c. in three daily doses, the HD group received 60 micrograms/kg/day s.c. in three daily doses and the GH group received GH, 0.1 IU/kg/day s.c. once daily. All children were treated for a period of 6 months. Evaluation included anthropometry, bone age, intravenous and subcutaneous GHRH(1-29)-NH2 tests and determination of insulin-like growth factor I (IGF-I) levels. An increase in height velocity of 2 cm/year or more was observed in all except two children. Height velocity during treatment was lowest in the LD group, but comparable in the HD and GH groups. An increase in height SDS for bone age occurred only in the GH-treated group. GH responses to intravenous GHRH(1-29)-NH2 showed a priming effect of the LD GHRH(1-29)-NH2 treatment, while a decrease in response occurred in the GH-treated group. Following a subcutaneous test dose of one-third of the daily dose of GHRH(1-29)-NH2, GH levels remained unchanged in both the LD and HD groups. There was accumulation of GHRH immunoreactivity over time in the HD group, but there was no correlation between measured GHRH and GH levels.(ABSTRACT TRUNCATED AT 250 WORDS)