Elevated serum prohepcidin might indicate the role of inflammation in the etiology of anemia concurrent with HP.

The endocrinological complications in β-thalassemia major patients do affect the life quality to a large extend. In this study, the endocrinological complications of 47 β-thalassemia patients, who have been followed-up at our hospital's pediatric hematology department, were evaluated. Out of β-thalassemia major cases included to this study, the 55.3% was male and 44.7% was female. The patients' mean levels of ferritin, whose mean age was 10.0 ± 4.5 years (2-20 years), were 2497 ± 1469 ng/mL (472-8558 ng/mL). At least one endocrinological pathology in 27 out of 47 (57.4%) and more than one endocrinological pathology in 14 out of 47 (29.7%) thalassemia patients were observed. The most frequently observed complication in followed-up cases was vitamin D insufficiency and deficiency (78.2%). The other complications in decreasing order were pubertal failure (41.6%), growth retardation (25.5%), decreased bone-mineral density (22.2%), secondary hyperparathyroidism (11.5%), overt hypothyroidism (4.25%), subclinical hypothyroidism (2.12%), and impaired glucose tolerance (2.12%). There was no statistically significant difference between serum mean ferritin level and endocrin complications (P > .05). Four patients (8.5%) had decreased signal intensity in pituitary magnetic resonance imaging (MRI) but this finding was not associated with ferritin levels (P = .87). MRI parameters were similar between patients with and without gonadal dysfunction. Mean height of the pituitary gland was 4.98 ± 1.1 mm (3-9 mm) and this was similar to those normal values in the literature. Ferritin levels were not correlated with pituitary height (P > .05). Beta thalassemia major, having the potential of leading to multisystemic complications, is a chronic disease that should be treated and followed-up by a multidisciplinary approach. Due to frequently encountered endocrinological complications, beta thalassemic patients should be followed-up regularly by hematology and endocrinology departments in coordination.

Even the life span has prolonged for the last 40 years, increase in frequently seen complications with increasing age negatively affect the life quality of thalassemia patients. In our study, complications encountered in 67 ß-thalassemia patients who were followedup at our hospital between 1 January 2004 and 31 May 2009 were retrospectively analyzed. Fifty-six patients were followed up with diagnosis of thalassemia major and 11 with thalassemia intermedia. Totally, 56.7% of patients were male and 43.3% were female. Ages varied between 2-20 years with the mean age of 10.3±4.8 years. Mean ferritin level was 2212±1370 ng/mL (41-6263 ng/mL) for 4.5 years. Complications were increased with increasing age. Complication rates were significantly higher among thalassemia major patients compared to thalassemia intermedia patients. There was no statistically significant relationship between complications and mean ferritin levels. The most common complications were endocrine complications (38.8%). Cardiac complications developed in 22.4% of the patients; gastroenterological complications in 19.4%; allergic complications in 9%; infectious complications in 1.5%; and thrombosis was detected in 1.5%. The endocrine complications were osteoporosis, growth retardation, developmental delay, short stature, hypothyroidism, delayed puberty, hypogonadism, and diabetes mellitus. The cardiac complications were left ventricular wall hypertrophy, diastolic dysfunction, systolic dysfunction, heart failure, pericardial effusion, dilated cardiomyopathy, left ventricular dilatation, left atrial dilatation, and fatal arrhythmias. Mortality occurred in one (1.5%) out of 67 ß-thalassemia patient due to dilated cardiomyopathy and fatal arrhythmia. Keywords: Thalassemia major, Thalassemia intermedia, Complications ÖZET Beta Talasemili Hastalarda S›k Görülen KomplikasyonlarTalasemi hastalar›n›n son 40 y›l içerisinde yaflam sürelerinde uzama olmakla birlikte yafl artt›kça görülen komplikasyonlar hastalar›n yaflam kalitelerini olumsuz yönde etkilemektedir. Çal›flmam›zda 1 Ocak 2004-31 May›s 2009 tarihleri aras›nda hastanemizde takipli 67 ß-talasemi hastas›nda görülen komplikasyonlar retrospektif olarak incelenmifltir. Çal›flmaya dahil edilen hastalar›n 56's› talasemi majör, 11'i talasemi intermedia tan›s›yla takip edilmektedir. Olgular›n %56.7'si erkek, %43.3'ü k›z idi. Yafllar› 2-20 y›l aras›nda olup, yafl ortalamalar› 10.3±4.8 y›l olarak hesapland›. Hastalar›n 4.5 y›l boyunca bak›lan ortalama ferritin düzeyleri 2212±1370 ng/mL (41-6263 ng/mL) olarak bulundu. Hastalar›n komplikasyonlar›nda yaflla birlikte art›fl oldu¤u saptand›. Komplikasyonlar talasemi majörlü hastalarda talasemi intermedial› hastalara göre istatistiksel olarak anlaml› derecede daha s›k görüldü. Ortalama ferritin de¤eri ile komplikasyon geliflme s›kl›¤› aras›nda istatistiksel olarak anlaml› bir iliflki bulunmad›. Hastalar›m›zda en s›k rastlanan komplikasyonlar %38.8 oran› ile endokrin komplikasyonlard›. Olgular›n %22.4'ünde kardiyak, %19.4'ünde gastroenterolojik, %9'unda all...

Mean age of each group was 11.3±2.7 years. Average RNFL and RNFLs of superior and inferior quadrants were significantly thinner in the anemic group than in the control group (p=0.006, p=0.005, and p=0.005, respectively). In addition, average peripapillary RNFL thickness and RNFL thicknesses of superior, inferior, and temporal quadrants were correlated with hemoglobin levels (r1=0.734, p1<0.001, r2=0.456, p2=0.005, r3=0.598, p3<0.001, r4=0.349, p4=0.037, respectively) in anemic group. CONCLUSIONS. We found that children with IDA had different peripapillary RNFL profile measured by Cirrus HD spectral-domain OCT. We caution ophthalmologists when they measure RNFL thickness in children to diagnose glaucoma or other neuro-ophthalmic disorders.

All-trans-retinoic acid (ATRA), which is used in acute promyelocytic leukemia, is usually well tolerated, but some side effects can be observed. Retinoic acid syndrome is the most severe side effect. Triazole derivatives such as fluconazole inhibit the NADPH-dependent cytochrome P-450-mediated catabolism of ATRA and are increased plasma levels of ATRA. Here, the authors report a case of APL who developed acute renal failure during ATRA and concurrent use of fluconazole.

Objective: The aim of this study was to investigate the kidney growth and renal functions in children receiving recombinant human growth hormone (rhGH) treatment. Materials and methods: A total of 37 children who received rhGH for 1.5 years before the study was started and 48 healthy controls were included at first evaluation. Hormone levels were determined and kidney sizes were measured by ultrasound. Kidney functions were assessed by serum creatinine and estimated glomerular filtration rate (eGFR). After 3 years of first evaluation, 23 patients were re-assessed. Results: Kidney sizes were found to be lower in rhGH received children compared with controls at first evaluation (p50.05). Significant positive correlations were found between anthropometric measurements and kidney length and kidney volume (p50.05). Height was the most significant predictor of kidney volume in rhGH received children (p50.001). After 3-years of follow-up significantly increases were found in kidney length and volume compared with the first measurements (p50.05). Increase percentage of body height was similar to increasing percent of kidney length and liver long axis (14.2%, 11.7.1% and 7.7%, respectively, p40.05). Although no abnormal renal function test results were found at first and second evaluations; rhGH received children had significantly lower eGFR, at first evaluation, compared with controls; however, renal functions significantly increased after 3 years of followup (p50.05). Conclusions: In conclusion, effect rhGH treatment on kidney growth is parallel to growth in body height and other visceral organs. A 3-years rhGH treatment resulted in significant increases in renal functions.

Determination of the distribution of rotavirus genotypes is essential for understanding the epidemiology of this virus responsible for nearly half a million of deaths in patients with gastroenteritis worldwide. In the present study, we aimed to genotype the rotavirus strains isolated from diarrheal stool samples in children under 5 years old. A total of 1297 fecal samples were collected, and rotavirus antigen was detected in 73 of these samples. Antigen-positive samples were transferred to the Public Health Agency of Turkey, Molecular Microbiology Research Laboratory, and were tested for determination of genotypes G and P using semi-nested multiplex polymerase chain reaction method performed with consensus- and genotype-specific primers. Twelve specimens were found to be negative for rotavirus in genotyping method. All the positive-strains were in G1-4, G8-9, P(4), P(8), and P(9) genotypes. The most frequent GP genotype combinations were found to be G9P(8) in 21 strains (34.4%), G2P(4) in 14 strains (23.0%), and G1P(8) in 12 strains (19.7%). We found 10 distinct genotypes amongst a total of 61 strains. Among the strains isolated and genotyped in our study, 90.2% (55/61) and 67.2% (41/61) have already been included in the two existing commercial vaccines. In conclusion, these findings implicate the necessity of development of region-specific vaccines after evaluation of the local genotype distribution. Further studies on the large number of rotavirus strains would contribute to this process.

Celiac disease (CD) is one of the most common chronic disorders in childhood. Autoimmune and nonautoimmune disorders including dermatitis herpetiformis, type 1 diabetes mellitus, and autoimmune thyroiditis can be encountered associated with CD. Common hematologic manifestations of CD include anemia owing to iron, folate, or vitamin B12 deficiency. We report a case with CD associated with Evans syndrome of whom to our knowledge, is the first child to be reported in the literature.