Family-based group treatment versus individual treatment in the management of childhood obesity: randomized,prospective clinical trial

Introduction  This study assessed the short- and long-term effects of a 3-month family-based group treatment in the management of childhood obesity versus individual treatment. Materials and methods  Eighty obese children, aged between 6 and 14 years, and their parents were included in this prospective controlled clinical study. Forty participants were randomly assigned for group treatment and the other 40 for individual treatment. A 3-month intervention program was focused on implementing healthy eating behaviors. The weight and height of the children were measured initially and at each treatment session and at follow-up visits. Body mass index was calculated and expressed as standard deviation score. Results and discussion  At the end of 3-month treatment program, there was a significant decline in BMI SDS in both groups (p < 0.001). After 1 year of follow-up period, there was still a significant decrease in BMI SDS in the study group (p < 0.001), whereas the decrease in BMI SDS was not maintained over the follow-up period in the standard group. There was a significantly increased consumption of vegetable and fruit and reduced consumption of carbonated drinks and fruit juice in both groups (p < 0.001). Conclusion  These findings demonstrate that the group treatment is more successful than the individual treatment in the management of childhood obesity.     

Successful therapy with calcium channel blocker (nifedipine) in persistent neonatal hyperinsulinemic hypoglycemia of infancy

The appropriate management of persistent hyperinsulinemic hypoglycemia of infancy (PHHI) still remains controversial. Some patients show a response to treatment with diazoxide or somatostatin, but a number of children require total or near-total pancreatectomy to control hyperinsulinism. Recent studies suggest a dysfunction in the adenosine triphosphate-sensitive potassium channel present in the plasma membrane of pancreatic beta-cells in PHHI. The closure of these channels initiating the depolarization of the beta-cell membrane and opening of calcium channels results in an increase in intracellular calcium which triggers insulin secretion. A calcium channel blocking agent has been shown to block this process and decrease insulin secretion of the nesidioblastotic beta-cells in vitro and to control the hyperinsulinemic hypoglycemia of the patient in vivo. To examine the efficacy of calcium channel blocker therapy, three patients with PHHI were treated with nifedipine. PHHI was diagnosed by inappropriately high insulin levels for low blood glucose levels at 8-10 days of age. Normoglycemia was maintained by a high dose of glucose infusion at a rate of 14-16 mg/kg/min. Therapy using diazoxide and/or somatostatin analogue failed to restore euglycemia in these three patients. The first patient underwent near-total pancreatectomy; however, hyperinsulinism recurred 30 days after surgery. All patients were started on short acting nifedipine at a dose of 0.3 mg/kg/day per os in four doses. To maintain blood glucose levels in normal ranges, the dose of nifedipine was progressively increased to 0.7-0.8 mg/kg/day. Glucose infusion rate to restore euglycemia decreased and was discontinued on the 4th to 10th day of nifedipine treatment. The patients, who have now been followed on nifedipine therapy for over 12 months, are normoglycemic with normal insulin levels. The growth and neuromotor development of the patients are unremarkable except for mild developmental delay of the patient who underwent near-total pancreatectomy. No side effects were encountered at the doses used. In conclusion, calcium channel blocking agents can be used with efficacy and safety in PHHI to control the hyperinsulinemia.     

Epidural metastasis in Wilms' tumor: A case report and review of the literature

Spinal canal involvement is not a common pattern of metastasis in Wilms' tumor. Although early detection and treatment can achieve improvement of neurological deficit, mortality remains high. We present a 5-year-old girl who had an epidural metastasis while she was receiving chemotherapy for stage IV Wilms' tumor. Within 2 months following laminectomy, total removal of tumor, radiotherapy, and adjuvant chemotherapy some of the neurological signs improved. © 1994 Wiley-Liss, Inc.     

A previously unreported etiology of trigger toe

Trigger toe is a rare entity, with only a few cases reported in the literature. It is usually seen in ballet dancers as a result of compression of the flexor hallucis longus tendon in the tarsal tunnel beneath the medial malleolus. We report a case of trigger toe due to a constricting lesion on the extensor hallucis longus tendon.     

Congenital mesenchymal chondrosarcoma of the orbit: case report and review of the literature

PURPOSE: To report the first case of congenital extraskeletal mesenchymal chondrosarcoma arising in the orbit. DESIGN: Interventional case report and review of the literature. METHODS: Ophthalmologic examination and computed tomography scan of the orbit were performed. Histologic and histochemical examination and immunohistochemistry of the biopsy specimen were evaluated. MAIN OUTCOME MEASURES: Ocular and systemic disease control. RESULTS: A 5-month-old girl was seen with a papillomatous lesion in the right lower tarsal conjunctiva present from birth. Histologic examination demonstrated an admixture of undifferentiated mesenchymal cells and islands of mature hyaline cartilage, and immunohistochemistry studies revealing positivity for vimentin and S-100 were consistent with the diagnosis of mesenchymal chondrosarcoma. The patient was treated with combined chemotherapy (ifosfamide, epirubicin, and cisplatin), radiotherapy, and surgery (exenteration). She is alive with no evidence of disease after 4 years of follow-up. Facial asymmetry and dental malformations have developed as late effects. Further reconstructive surgery is planned for the malformations. CONCLUSIONS: Extraskeletal mesenchymal chondrosarcoma in the orbit is extremely rare. This study reports the first case of congenital extraskeletal mesenchymal chondrosarcoma arising in the orbit (also the youngest patient) both in skeletal and extraskeletal sites. Multimodality treatment (surgery, chemotherapy, and radiotherapy) may lead to long-term survival.     

Ewing's sarcoma of the axial system in patients older than 15 years: dismal prognosis despite intensive multiagent chemotherapy and aggressive local treatment

BACKGROUND: Older age and axial location of Ewing's sarcoma have been reported as unfavorable prognostic factors. METHODS: The records of patients older than 15 years with the Ewing's family of tumors were reviewed retrospectively. After the induction chemotherapy consisting of alternating vincristine, adriablastin, cyclophosphamide (VAC) and etoposide, ifosfamide with mesna protection (IE), a local treatment modality was chosen based on tumor and patient characteristics. RESULTS: Twenty-five patients with a median age of 19 years were evaluated. Median follow-up was 26 months (range 4-58). Seventeen patients (68%) had died. In univariate analysis, factors predictive of shorter survival were the patients presenting with metastatic disease, with the primary tumor located at the pelvis, those who never achieved complete response to chemotherapy and those who had chemotherapy for <12 months. Only a negative link with pelvic location was observed in multivariate analysis [risk ratio 7.5; 95% confidence interval (CI) 1.52-37.06; P = 0.0134]. Median progression-free survival (PFS) and overall survival (OS) were 10 months (95% CI 6.2-13.8) and 14 months (95% CI 9.3-18.7), respectively. Cumulative 2-year PFS and OS were 19.0% (95% CI, SD +/-8.4) and 32.7% (95% CI, SD +/-9.8), respectively. CONCLUSIONS: The prognosis of patients with axial Ewing's sarcoma is dismal despite an intensive, multimodality approach including multiagent, alternating chemotherapy, surgery and/or radiotherapy. A more aggressive approach should be considered for this group of Ewing's sarcoma patients.     

Growth hormone treatment in Aarskog syndrome: analysis of the KIGS (Pharmacia International Growth Database) data

Aarskog syndrome is an X-linked disorder characterized by faciogenital dysplasia and short stature. The present study set out to determine the effect of growth hormone (GH) therapy in patients with Aarskog syndrome enrolled in KIGS--the Pharmacia International Growth Database. Twenty-one patients (20 males) were evaluated. Median age at start of treatment was 8.3 years (10-90th percentiles, 5.1-14.1 years) and median height SDS was -2.8 (10-90th percentiles, -2.1 to -3.7). The median dose of GH was 0.22 mg/kg/week (10-90th percentiles, 0.15-0.30 mg/kg/week) given at a median frequency of six (4-7) times per week. Prepubertal patients were followed longitudinally for 1 year (n = 13) or 3 years (n = 7). After 1 year, the median height SDS had improved from -2.8 to -2.3 in 13 patients. After 3 years, height SDS had improved significantly (p <0.05) to -1.8 (10-90th percentiles, -2.1 to -1.1) in the seven patients. No adverse events were noted. Although final height data for these patients are still awaited, the present results support the use of GH to promote growth in children with Aarskog syndrome.     

Rotavirus gastroenteritis among children under five years of age in Izmir, Turkey

Little is known about the epidemiology of rotavirus infection in Turkey. The aim of the study was to determine the incidence and clinical significance of rotavirus gastroenteritis, in view of the potentially available prevention by rotavirus vaccination. The study also sought to determine possible risk factors for rotavirus gastroenteritis. Therefore, 920 children under five years of age with acute gastroenteritis admitted to three pediatric hospitals in Izmir were studied. Rotavirus was identified in 39.8% of the children. Most children with rotavirus gastroenteritis (80.7%) were younger than two years of age. Marked seasonality of rotavirus gastroenteritis was observed, with a peak incidence from January to March. A total of 91% of rotavirus strains that were typed were of serotypes G 1-4. There was no significant difference among rotavirus-positive and rotavirus-negative patients with regard to family income. Compared with children who were exclusively breast-fed, those who were not exclusively breast-fed were at a two-fold greater risk of rotavirus diarrhea. Rotavirus gastroenteritis was significantly more severe than non-rotavirus gastroenteritis; 69% of children with rotavirus infection had severe gastroenteritis (score > or = 11). In conclusion, rotavirus is the most common cause of severe gastroenteritis among children under five years of age in Izmir. A new potent rotavirus vaccine, when available, will provide effective protection against severe rotavirus infection. Promotion of breast-feeding would augment the impact of rotavirus vaccines in preventing severe childhood diarrhea.     

HLA-DR and -DQ associations with insulin-dependent diabetes mellitus in a population of Turkey

Genetic susceptibility to insulin-dependent diabetes mellitus (IDDM) has been shown to be associated with MHC in many studies. To extend this data with a population with relatively low IDDM incidence, MHC DRB, DQA, and DQB have been investigated by polymerase chain reaction and sequence specific oligonucleotide probe hybridization (PCR/SSO) in 178 IDDM patients from Turkey and compared to 248 healthy controls. Significant differences are detected between IDDM and control groups in the frequencies of DRB1*0402 DQA1*03 DQB1*0302 (28.1% vs. 5.2%, p < 0.0001, OR: 7.1) and DRB1*0301 DQA1*0501 DQB1*02 (57% vs. 18.1%, p < 0.0001, OR: 6.1). Among the negative associations, the most strong ones are with DRB1*1401 DQA1*0101 DQB1*0503 (0.6% vs. 8.9%, p < 0.0001, OR: 0.1), DRB1*1502 DQA1*0103 DQB1*0601 (1.1% vs. 7.7%, p = 0.0023, OR: 0.1), DRB1*1301 DQA1*0103 DQB1*0603 (0.6% vs. 6.9%, p = 0.0039, OR: 0.2) and DRB1*1101 DQA1*0501 DQB1*0301 (3.9% vs. 12.1%, p < 0.0001, OR: 0.2). When the DRB, DQA or DQB genotypes of the susceptible alleles are compared, the most strong susceptibility marker of the disease is found to be DRB1*0301/*04 (31.4% vs. 2.8%, p < 0.0001, OR: 15.8) and among these, heterozygote genotype DRB1*0301/*0401 (4.5% vs. 0, p = 0.0008, OR: 24.8).These results confirm the positive associations with IDDM previously observed in other Caucasian populations and reveal many negative and strong associations which maybe underlining several characteristics that distinguish Turkish diabetics form other Caucasians.