Body fat distribution in childhood obesity: Association with metabolic risk factors

Objectives: To evaluate the clinical significance of body fat distribution in childhood obesity, we investigated the associations of subcutaneous and intraabdominal (preperitoneal and visceral) fat, estimated by ultrasonography, with metabolic risk factors. Subjects: Fifty-one obese (age 11.5±2.6 years) and 33 nonobese (age 12.2±2.7 years) children. Study Design: Case control study. Methods: Ultrasonographic measurements of fat thickness [maximum and minimum preperitoneal fat thicknesses (Pmax, Pmin), maximum and minimum subcutaneous fat thicknesses (Smax, Smin), visceral fat thickness (V), triceps (Tr) and subscapular (Ss) skin fold thicknesses] were documented. Blood pressures, lipid profiles, fasting insulin levels, glucose/insulin ratio and HOMAIR (homeostasis model assessment for insulin resistance) were evaluated in both groups and these parameters were correlated with body fat distribution. Results: In the obese group, fasting insulin level was correlated to Smin, Smax, and Pmin. HOMA, accordingly, was also correlated to Smin, Smax, and Pmin. Fasting insulin level and HOMA showed no correlation with either Pmax or visceral fat thickness. Analysis: Abdominal subcutaneous fat thickness measurements were the best predictors of hyperinsulinemia (R2: 0.32). Conclusion: We did not observe a significant correlation between blood pressure, lipid parameters and body fat distribution in obese group. Abdominal subcutaneous fat thickness might be a better predictor of the risk for hyperinsulinemia in childhood obesity

The retrospective evaluation of patientswith acute bronchiolitis

Amaç: Bronsiolit, küçük hava yollarının inflamatuar obstrüksiyonundan kaynaklanan ve özellikle 6 aydan küçük çocuklarda en sık hospitalizasyon nedeni olan bir hastalıktır. Bu çalısmada, klinigimize bronsiolit nedeniyle kabul edilen olguların demografik özellikleri, klinik bulguları, tedavisi ve prognozu analiz edildi. Bulgular: Olguların %59,7'si (n=40) erkek, %40,3'ü (n=37) kız olup, %40,3'ü kıs, %29,9'u ilkbahar, %23,9'u sonbahar, %6'sı yaz mevsiminde hastanemize kabul edildi. En büyük hasta gurubunu 3-6 aylık bebekler (%35,8) olusturmaktaydı. Ilk basvuru sikayetleri %85'inde öksürük, %53,7'sinde hırıltı ve %34,3'ünde atesti. Atopi öyküsü üç ve daha fazla atak geçirenlerde %41.6, bir ve iki atak geçirenlerde %20olarak saptandı. Sonuç: Ailede atopi öyküsü bulunmasının bronsiolitli olgularda atak sayısını ve steroid gereksinimini arttırdıgı görüldü.Aim: Bronchiolitis is an under respiratory tract disorder which is caused by the inflammatory obstruction of the small airways. It is also the most common reason for hospitalization of children younger than 6 months. In this study, demographic characteristics, clinical symptoms, treatment and prognoses of patients who had been admitted to our clinic due to bronchiolitis were investigated. Findings: 59.7% of patients were male, and 40.3% were female. 40.3% were admitted to our hospital in winter, 29.5% in spring, 23.6% in fall and 6% in summer. The largest group of patients consisted of infants between 3-6 months (35.9%). Initial complaints were coughing in 85%, wheezing in 53.7% and fever in 34.3%. Of the patients history of atopy was determined more frequently (41.6%) among those who suffered from three or more attacks compared with patients who experienced one to two attacks (20%). Result: A history of atopy in the patient's family increases the number of bronchiolitis attacks and the need for steroid

Henoch-Schonlein purpuralı hastaların analizi

Amaç: HSP'li hastalarımızın epidemiyolojik ve klinik özellikleri, laboratuar bulguları ve hastalıgın seyrinin incelenmesi amaçlandı. Yöntem: 2000-2006 yılları arasında, hastanemiz Çocuk Saglıgı ve Hastalıkları Klinigi'nde Henoch-Schonlein Purpurası tanısı alan ve izlenen 45 hasta retrospektif olarak incelendi. Bulgular: Hastaların 27 (%60,0)'si kız, 18 (%40,0)'i erkek, kız / erkek oranı 1.5 idi.Yas ortalaması 6 yas 9 ay (8 ay-14 yas) olarak saptandı. Basvuru sikayetleri sıklık sırasına göre döküntü (%100), yürüyememe ve/veya eklem agrısı (%71,1) ve karın agrısı (%40,0) idi. Hastaların % 57,7'inde ortalama 12 gün önce geçirilmis enfeksiyon öyküsü tespit edildi. Fizik muayenede purpurik döküntü (alt ekstremite ve gluteal bölgede %100, yaygın %11,1), artrit (32 hastada, %71,1, en sık ayak bilegi ve diz eklemlerinde), ates (9 hastada, %20,0) bulundu. Laboratuar bulguları anemi (8/45 hasta, %17,7), lökositoz (6/45 hasta, %13,3), trombositoz (16/45 hasta, %35,5), sedimentasyon yüksekligi (16/25 hasta, %64,0), CRP pozitifligi (33/34 hasta, %97,0), hematüri (4/45 hasta, %8,8), proteinüri (2/45 hasta, %,4,4), gaitada gizli kan pozitifligi (14/42 hasta, %33,3) bulundu. Hematüri ve proteinüri devam etmedigi için hiçbir hastada böbrek biyopsisine gerek duyulmadı. Gastrointestinal sistem tutulumu oldugu düsünülen 17 hastaya steroid tedavisi baslandı ve ortalama 9.1 gün kullanıldı. Perforasyon, invaginasyon gözlenmedi. Ortalama 15. günde 9 hastada relaps görüldü. Santral sinir sistemi tutulumu hiçbir hastada yoktu. Sonuç: Henoch-Schonlein Purpurası çocukluk çagında sık görülen benign karakterli bir hastalık olup komplikasyon ve sekel oranı oldukça düsüktür.Aim: The aim of this study was to investigate the clinical and epidemiologic features, laboratory finding and outcome of disease in our patients with Henoch-Schönlein purpura. Methods: Fourty-five patients with Henoch-Schönlein purpura who were diagnosed and observed in our department of pediatrics between 2000-2006 were retrospectively evaluated. Results: Twentyseven (60.0%) of the patients were female and 18 (40.0%) were male, female to male ratio was1.5. Mean age of the patients was 6 year 9 months (8 months-14 years). İnitial symptoms and findings in the time of admission with decreasing frequency were purpura (100%), disability to walk and/or arthralgia (71.1%) ve stomachache (40.0%). An infection history was present in 57.7% of the patients in the last 12 days. In physical examination, purpura (lower extremity and hips 100%, diffuse 11.1%), arhtritis (32 patients, 71.1%, mostly diffused on ankle and knee), fever (9 patients, 20,0%) were discovered. Anaemia (8/45 patients, 17.7%), leukocytosis (6/45 patients, 13.3%), trombocytosis (16/45 patients, 35.5%), increased erythrocyte sedimentation rate (16/25 patients, 64.0%), positive CRP (33/34 patients, 97.0%), hematuria (4/45 patients, 8.8%), proteinuria (2/45 patients, 4.4%), positive fecal blood tests (14/42 patients, 33.3%) were determined. Renal biopsy was not needed as there was no persistant hematuria and proteinuria. 17 patients who had gastrointestinal involvement were treated with steroid therapy for a mean period of 9.1 days. Perforation and invagination was not observed. Relapse was seen in 9 patients. (mean duration 15th day). There was no nervous system involvement in any patients. Conclusion: Henoch-Schonlein Purpura is a frequent benign disease of childhood. Complicatios and sequeles are infrequent

Pseudohypoparathyroidism and growth

Pseudohypoparathyroidism (PHP) is a disorder characterized by hypocalcemia and hyperphosphatemia due to resistance to parathyroid hormone (PTH). There are two main subtypes of PHP, named as PHP types Ia and Ib and caused by genetic alterations within upstream of the GNAS locus. Heterozygous inactivating mutations within alpha subunit of the stimulatory G protein (Gs?) encoding GNAS exons are found in patients with PHP-Ia. These patients often present with additional hormonal resistance and show characteristic physical features that are totally called as Albright's hereditary osteodystrophy (AHO). AHO is characterized by short stature, brachydactyly, obesity, skull facial deformities (round face, short neck, flat wide nose, and teeth hypoplasia), short limbs due to brachydactyly or short metacarpal bones, subcutaneous calsinosis, and, in some cases, mental or developmental abnormalities. These features are also present in pseudopseudohypoparathyroidism (PPHP); however, patients affected by this disorder show the same features except hormone resistance. Maternal inheritance of a mutation leads to PHP-Ia with AHO plus hormone resistance, while paternal inheritance of the same mutation leads to PPHP with AHO only. PHP-Ib patients present predominantly with renal PTH resistance and do not show any features of AHO. The proposed mechanism of both the familial autosomal dominant form of PHP-Ib and the sporadic form of PHP-Ib is the distribution of long-range imprinting control elements of GNAS locus, with consequent decreased Gs? transcription in the proximal renal tubules and PTH resistance. It is widely known that patients with PHP-Ia and PPHP have short stature. The effects of Gs? -inactivating mutations on skeletal growth are likely to involve a number of factors. One effect will be intrinsic to the growth plate, because Gs? couples to PTH/PTH-related polypeptide (PTHrP) receptor to adenylate cyclase and cyclic adenosine monophosphate production, controlling the proliferation and differentiation of chondrocytes. Another explanation is that many patients with PHP-Ia have insufficient growth hormone (GH) and exhibit a partial resistance to growth hormone-releasing hormone (GHRH). This condition could have an additive effect on the growth defects, which are mediated by blunted signaling from the PTH/PTHrP receptor owing to Gs? deficiency. In contrast with what is observed in PHP-Ia, patients with PHP-Ib display normal responsiveness to GHRH and normal IGF-I levels. These data provide new information on this rare disease and emphasize the clinical heterogeneity of genetic defects within the GNAS locus. © Springer Science+Business Media, LLC 2012. All rights reserved

Pseudohypoparathyroidism and growth

Pseudohypoparathyroidism (PHP) is a disorder characterized by hypocalcemia and hyperphosphatemia due to resistance to parathyroid hormone (PTH). There are two main subtypes of PHP, named as PHP types Ia and Ib and caused by genetic alterations within upstream of the GNAS locus. Heterozygous inactivating mutations within alpha subunit of the stimulatory G protein (Gsα) encoding GNAS exons are found in patients with PHP-Ia. These patients often present with additional hormonal resistance and show characteristic physical features that are totally called as Albright's hereditary osteodystrophy (AHO). AHO is characterized by short stature, brachydactyly, obesity, skull facial deformities (round face, short neck, flat wide nose, and teeth hypoplasia), short limbs due to brachydactyly or short metacarpal bones, subcutaneous calsinosis, and, in some cases, mental or developmental abnormalities. These features are also present in pseudopseudohypoparathyroidism (PPHP); however, patients affected by this disorder show the same features except hormone resistance. Maternal inheritance of a mutation leads to PHP-Ia with AHO plus hormone resistance, while paternal inheritance of the same mutation leads to PPHP with AHO only. PHP-Ib patients present predominantly with renal PTH resistance and do not show any features of AHO. The proposed mechanism of both the familial autosomal dominant form of PHP-Ib and the sporadic form of PHP-Ib is the distribution of long-range imprinting control elements of GNAS locus, with consequent decreased Gsα transcription in the proximal renal tubules and PTH resistance. It is widely known that patients with PHP-Ia and PPHP have short stature. The effects of Gsα -inactivating mutations on skeletal growth are likely to involve a number of factors. One effect will be intrinsic to the growth plate, because Gsα couples to PTH/PTH-related polypeptide (PTHrP) receptor to adenylate cyclase and cyclic adenosine monophosphate production, controlling the proliferation and differentiation of chondrocytes. Another explanation is that many patients with PHP-Ia have insufficient growth hormone (GH) and exhibit a partial resistance to growth hormone-releasing hormone (GHRH). This condition could have an additive effect on the growth defects, which are mediated by blunted signaling from the PTH/PTHrP receptor owing to Gsα deficiency. In contrast with what is observed in PHP-Ia, patients with PHP-Ib display normal responsiveness to GHRH and normal IGF-I levels. These data provide new information on this rare disease and emphasize the clinical heterogeneity of genetic defects within the GNAS locus. © Springer Science+Business Media, LLC 2012. All rights reserved

Metabolic syndrome in childhood obesity

Objectives: We determined the frequency of metabolic risk factors and the prevalence of Metabolic Syndrome in childhood obesity. Subjects: 186 obese children (97 females and 89 males), aged 11.2 ± 2.8 (6-16) years and 98 healthy children (46 females and 52 males), aged 10.9 ± 3.2 (6-16) years were recruited for the study, as study and control groups, respectively. Methods: Subjects were evaluated for anthropometry, blood pressure (BP) and biochemical cardiovascular risk factors. Metabolic syndrome was defined in presence of ≥ 3 of the following: (i) fasting triglyceride ≥ 100 mg/dL; (ii) high density lipoprotein - cholesterol 75th percentile for age and gender and (v) systolic BP > 90th percentile. Results: We found that 144 (77.4%) children in the obese group had one, two or more cardiovascular risk factors. Using a pediatric definition, the prevalence of metabolic syndrome was 2.1%. In the control group, the clustering of one, two and three risk factors was very rare. Conclusion: Childhood obesity is associated with increased frequency of cardiovascular risk factors and metabolic syndrome

Pseudohypoaldosteronism: Case report

Giriş: Psödohipoaldosteronizm, aldosterona periferik direnç sonucu gelişen ve tuz kaybı ile karakterize bir hastalıktır. Olgu Sunumu: Emmede azalma, emerken uyuklama şikayetiyle getirilen hastada hiponatremi, hiperkalemi, metabolik asidoz, yüksek renin ve aldosteron düzeyi saptandı. Olguya sistemik form psödohipoaldosteronizm tanısı konuldu. Oral tuz ile tedavisine devam edildi. Tartışma: Psödohipoaldosteronizm primer, sekonder ve Gordon sendromu olarak üç tiptir. Primer form epitelyal sodyum kanalı ve mineralokortikoid reseptör genindeki mutasyondan, sekonder form sıklıkla üriner malformasyon ve idrar yolu enfeksiyonlarından kaynaklanır. Gordon sendromunda ise plazma aldosteron düzeyi genellikle normal olup, mineralokortikoidlere yeterli cevap vardır ve plazma renin aktivitesi baskılanmıştır. Olgumuzda üriner enfeksiyon saptanması nedeniyle aynı zamanda sekonder psödohipoaldosteronizm olasılı¤ı araştırıldı. Ter testinin pozitif olması ve tuz ihtiyacının uzun süre devam etmesi nedeniyle hasta sistemik form primer psödohipoaldosteronizm olarak kabul edildi.Introduction: Pseudohypoaldosteronism is a disease which occurs as a result of peripheral resistance to aldosterone and is characterised by salt wasting. Case Report: Hyponatremia, hyperkalemia, metabolic acidosis, high renin and aldosterone levels were determined in the patient admitted with decrease in sucking and getting sleepy during breast-feeding. The case was diagnosed as systemic form of pseudohypoaldosteronism. His treatment was continued with oral salt. Conclusion: Pseudohypoaldosteronism has three types as primary, secondary and Gordon syndrome. Primary form is due to epithelial sodium channel and mineralocorticoid receptor gene mutation, whilst secondary form is frequently caused by urinary malformation and urinary tract infections. In Gordon syndrome, plasma aldosterone level is usuallly normal, and plasma renin activity is depressed, there is an adequate response to mineralocorticoids. Probability of secondary pseudohypoaldosteronism was investigated at the same time because of the urinary infection present in our subject. The patient was accepted as systemic form of primary pseudohypoaldosteronism because of positive sweat test result and prolonged salt necessity

Psödohipoaldosteronizm: Olgu sunumu

Giriş: Psödohipoaldosteronizm, aldosterona periferik direnç sonucu gelişen ve tuz kaybı ile karakterize bir hastalıktır. Olgu Sunumu: Emmede azalma, emerken uyuklama şikayetiyle getirilen hastada hiponatremi, hiperkalemi, metabolik asidoz, yüksek renin ve aldosteron düzeyi saptandı. Olguya sistemik form psödohipoaldosteronizm tanısı konuldu. Oral tuz ile tedavisine devam edildi. Tartışma: Psödohipoaldosteronizm primer, sekonder ve Gordon sendromu olarak üç tiptir. Primer form epitelyal sodyum kanalı ve mineralokortikoid reseptör genindeki mutasyondan, sekonder form sıklıkla üriner malformasyon ve idrar yolu enfeksiyonlarından kaynaklanır. Gordon sendromunda ise plazma aldosteron düzeyi genellikle normal olup, mineralokortikoidlere yeterli cevap vardır ve plazma renin aktivitesi baskılanmıştır. Olgumuzda üriner enfeksiyon saptanması nedeniyle aynı zamanda sekonder psödohipoaldosteronizm olasılığı araştırıldı. Ter testinin pozitif olması ve tuz ihtiyacının uzun süre devam etmesi nedeniyle hasta sistemik form primer psödohipoaldosteronizm olarak kabul edildi