Prevalence and genotype distribution of rotaviruses in children with gastroenteritis in Rize province.

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.

Endocrinopathies in Turkish children with Beta thalassemia major: results from a single center study.

The endocrinological complications in ß-thalassemia major patients do affect the life quality to a large extend. In this study, the endocrinological complications of 47 ß-thalassemia patients, who have been followed-up at our hospital's pediatric hematology department, were evaluated. Out of ß-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.

Kidney growth and renal functions under the growth hormone replacement therapy in children.

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 (p<0.05). Significant positive correlations were found between anthropometric measurements and kidney length and kidney volume (p<0.05). Height was the most significant predictor of kidney volume in rhGH received children (p<0.001). After 3-years of follow-up significantly increases were found in kidney length and volume compared with the first measurements (p<0.05). 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, p>0.05). 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 follow-up (p<0.05). 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.

Assessment of peripapillary retinal nerve fiber layer thickness in children with vitamin B12 deficiency.

PURPOSE: Vitamin B(12) deficiency is a worldwide problem. It affects all ages, including children. It is one of the most common nutritional disorders and can cause harmful effects on the nervous system. In this study, we compared the peripapillary retinal nerve fiber layer thickness (RNFLT) in a healthy control group with children with vitamin B(12) deficiency. In our study, we aimed to evaluate the effect of vitamin B(12) deficiency on the RNFLT in children with the optical coherence tomography (OCT) method. METHODS: Sixty-six children with a diagnosis of vitamin B(12) deficiency (patient group) and 66 age- and sex-matched healthy children (control group) were enrolled in this prospectively designed study. Blood counts, vitamin B(12) levels, folate levels, and full biochemical parameters were obtained for all the subjects in each group. Peripapillary RNFLT measurements were performed with Cirrus HD spectral domain OCT. RESULTS: The thickness of the superior retinal nerve fiber layer (RNFL) in the vitamin B(12) deficiency group was significantly lower than that of the control group (p = 0.037). Although the average thickness of the RNFL was lower in the patient group, there was no statistically significant differences (p = 0.216). In the vitamin B(12) deficiency group, the average RNFL thickness and the superior RNFL thickness were significantly correlated with vitamin B(12) levels ((r1) = 0.353, (p1) < 0.004 and (r2) = 0.416, (p2) = 0.001, respectively). CONCLUSION: Our study showed that a deficiency in vitamin B(12), elsewhere it is important for the development of the central nervous system, is associated with a reduction in the thickness of the superior RNFL.

The role of prohepcidin in anemia due to Helicobacter pylori infection.

BACKGROUND: Hepcidin, a key regulator of iron homeostasis, increases when inflammation and some infections occur. It plays a critical role in macrophage iron retention, which underlies inflammation/infection caused anemia. It is known that Helicobacter pylori (HP) may lead to iron deficiency (ID) due to occult blood loss or reduced iron absorption. This study investigates the role of prohepcidin, hepcidin's precursor, in ID and ID anemia (IDA) with a concurrent HP infection. METHODS: In this prospectively designed study, 15 patients with IDA and a concurrent HP infection (group 1), 11 patients with an ID and a concurrent HP infection (group 2), and 18 patients with HP infection (group 3) were observed. All groups received only HP eradication therapy. Twenty-five age- and sex-matched children without ID/IDA and HP infection were included in the study as the control group. In all groups and control group, measurements were taken for pre- and posttreatment hemoglobin, serum prohepcidin, serum ferritin, serum iron (SI), transferrin saturation, erythrocyte sedimentation rate, fibrinogen, and C-reactive protein levels. RESULTS: The pretreatment prohepcidin levels were significantly higher only in group 1 compared to the control group (P < .05). In group 1, a significant increase in hemoglobin and SI levels and a significant reduction in prohepcidin levels were additionally observed following HP eradication treatment (P < .05). However, in groups 2 and 3, significant differences in hemoglobin, iron, and prohepcidin levels between pre- and posttreatment were not observed. CONCLUSION: Elevated serum prohepcidin might indicate the role of inflammation in the etiology of anemia concurrent with HP.

Peripapillary retinal nerve fiber layer thickness in children with iron deficiency anemia.

PURPOSE: To evaluate peripapillary retinal nerve fiber layer (RNFL) thickness in children with iron deficiency anemia (IDA) in comparison with healthy controls and to investigate the correlation between peripapillary RNFL thicknesses and the hematologic parameters in these subjects. METHODS: Forty eyes of 40 children with a diagnosis of IDA (anemic group) and 40 eyes of 40 age- and sex-matched healthy children (control group) were enrolled in this study. Peripapillary RNFL thickness measurements were performed using Cirrus HD optical coherence tomography (OCT). RESULTS: 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.

Deferasirox therapy in children with Fanconi aplastic anemia.

Thirty-nine children with Fanconi aplastic anemia (FAA) have been followed up in our center between January 2008 and November 2010. Eight of these children (20%) with a transfusional iron overload had been undergoing deferasirox treatment during the study period. In the English literature, transfusional iron overload and the use of an iron chelator in children with FAA has not yet been evaluated. Here, we have presented the effectivity and tolerability of deferasirox in children with FAA and a transfusional iron overload. Before the deferasirox treatment, the mean serum ferritin level was 3377 ± 2200 ng/mL. After a mean 13.6-month treatment duration, the mean ferritin level decreased to 2274 ± 1300 ng/mL (P<0.05). In our series, 3 patients had renal and 3 had hepatic toxicity during the treatment. Two patients had peliosis hepatis and 2 had congenital renal abnormalities before the treatment. There may be differences in the side-effect profiles of deferasirox treatment in patients with FAA. In our series, despite the low number of cases, nephrotoxicity and hepatotoxicity were common side effects instead of gastrointestinal disturbances reported in other studies. Deferasirox is an oral, easily applicable, and effective iron chelator; baseline hepatotoxicity and nephrotoxicity may increase the development of toxic side effects in children with FAA. Patients with FAA receiving deferasirox treatment should be followed up closely for these side effects.

Leptin promoter G-2548A genotypes and associated serum leptin levels in childhood acute leukemia at diagnosis and under high-dose steroid therapy.

Genotype/allele distributions of leptin promoter G-2548A polymorphism, serum leptin and insulin levels and body weight were not significantly different between 72 children (39 male/33 female; age range 1.08-16, median 6 years) with acute leukemia (56 acute lymphoblastic leukemia [ALL]/16 acute non-lymphoblastic leukemia [ANLL]) at diagnosis and 70 age- and sex-matched controls (p > 0.05). The - 2548GG genotype was associated with the highest leptin levels in controls and patients with acute leukemia after 7-day high-dose methylprednisolone (HDMP) therapy (p < 0.05), while no significant association of genotype with leptin levels was detected in patients at diagnosis (p > 0.05). One-week HDMP therapy in patients carrying the - 2548G allele caused a significant increase in leptin levels and body weight (p < 0.001), whereas increases in those carrying the - 2548AA genotype were insignificant (p > 0.05). Decreases in white blood cell counts of patients after therapy were insignificant in - 2548GG (p > 0.05) yet significant in - 2548GA and - 2548AA (p < 0.05) genotypes. These results revealed no association of leptin genotype with the etiology of childhood acute leukemia but a possible association with leptin levels and effects of HDMP therapy.

Kidney growth in children with congenital hypothyroidism.

The effect of hypothyroidism on kidney size has not been studied in children. The aim of this study was to examine the role of congenital hypothyroidism and levothyroxine (L-thyroxine) treatment on renal growth. Forty children with congenital hypothyroidism and 37 healthy controls were prospectively included. The mean age of patients was 8.2+/-4.7 years. Patients had lower height and weight standard deviation scores compared with controls. The mean L-thyroxine initial age and treatment duration were 37.0 and 60.5 months, respectively. In 62.5% of patients, L-thyroxine was initiated after 6 months of age, and 60.0% of patients had severe hypothyroidism. Patients had lower kidney length and total kidney volume compared with controls (P < 0.05). No significant differences were found in kidney volume/body weight and kidney volume/ body height ratios between patients and controls (P > 0.05). Multiple regression analysis showed significant relationship between relative kidney volume and average free thyroxine level (P < 0.05). No significant differences in kidney sizes were found between patients who had L-thyroxine initiated before and after 6 months of age or between mild/moderate and severe hypothyroidism at diagnosis (P > 0.05). In conclusion, normal renal growth can be accomplished with L-thyroxine replacement despite considerable delay in treatment initiation and/or severe hypothyroidism.

Acute renal failure during ATRA treatment.

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.

A rare condition associated with celiac disease: Evans syndrome.

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.