Valproic acid-induced nocturnal enuresis in pediatric patients.

BACKGROUND: Valproic acid (VPA) is one of the commonly used antiepileptic drug. It has various side effects which may be fatal, such as fulminant hepatitis. Nocturnal enuresis (NE) has rarely been reported as side effect of VPA. The aim of this study is to evaluate the frequency of VPA-induced NE and discuss the possible reasons. MATERIALS AND METHODS: This retrospective cohort study was performed at the Department of Pediatric Neurology, Eskisehir Osmangazi University Hospital, in Eskisehir, Turkey, between April 2014 and April 2015. The patient population was generated from the epilepsy patients who were receiving VPA monotherapy. Control group population was generated from nonepileptic patients who visited our clinic for headache. Age range of the patients and the control group was determined to be 5-15 years. RESULTS: The patients group consisted of 189 (53.7%) boys and 163 (46.3%) girls and mean age of the patients was 9.1 ± 3.02 (5-15) years. The control group consisted of 92 (51.1%) girls and 88 (48.9%) boys and mean age of the patients was 8.75 ± 3.23 (5-15) years. We found the incidence of VPA-induced NE to be 5.7%. In the control group, incidence of NE was found to be 10.7%. CONCLUSION: This study is one of the largest series about VPA-induced NE. NE is a side effect of VPA that is generally overlooked by clinicians and slightly less well-known too. The literature on VPA-induced NE is very inadequate, and its etiology is not clear. In our study, we did not detect renal dysfunction in the patients with VPA-induced NE; therefore, we may speculate that the NE was caused by the increased sleep depth with VPA treatment. We believe that larger prospective studies including polysomnography may be helpful to shed light on the cause of VPA-induced NE.

Increasing challenges of inflammatory bowel disease in a Turkish cohort (2006-2016).

BACKGROUND: We aimed to describe the natural course of inflam-matory bowel disease (IBD) in adult Turkish patients during the past decade. METHODS: We performed a retrospective investigation in total 508 patients with IBD between 2006 and 2016. The severity of IBD was assessed by the need for hospitalization, biological therapy and surgery. The study consisted of 304 (59.8%) patients with UC, 180 (35.4%) with CD and 24 (4.7%) IBD unclassified patients. The ratio of severe disease for both CD and UC was as follows: hospitalization in 71 (39.4%) and 37 (12.2%), biological therapy received in 20 (11.1%) and 3 (1.0%), and surgery in 23 (12.8%) and 2 (0.7%) respectively. Patients were divided into two groups based on the date of disease onset. Earlier group includes the patients followed-up between 2006-2011 and later group, between 2011-2016. Our results revealed that the proportion of patients receiving biological therapies was significantly higher and the rate of surgery was significantly lower in the later group compared to the earlier group (14.5% to 0%, 9.4 to 23.8, respectively). The hospitalization rate was lower in the later group compared to earlier group but did not reach a significant difference (37.7% to 45.2% respectively). CONCLUSIONS: The course of IBD in Turkish patients appears to be between that of Europe and Asia. In patients with CD, proportion of patients receiving biologic therapy has increased while rate of surgery has decreased.

A Macro-Anatomical Investigation of the Some Skull Bones of Nehring's Blind Mole Rats (Spalacidae: Nannospalax nehringi).

This study was aimed to demonstrate the specific anatomical features of the skull bones of Nehring's blind mole rats. Eight skulls, belonging to animals of both sexes, were used. The occipital squama contributed to the formation of the caudal portion of the skull roof. The foramen magnum was quite large. The external occipital crest was present only in the males. The parietal bones formed the middle portion of the skull roof together with the well-developed inter-parietal bones. The zygomatic process of the temporal bone formed the zygomatic arch by extending to the temporal process of the zygomatic bone. The zygomatic process of the frontal bone and the frontal process of the zygomatic bone laterally bordered the orbit. There was a single septal process of the nasal bone. Each ramus of the mandibula had four processes. The mandibular ramus had an angular process on its caudal rim, which extended dorsolaterally. The dorsal free end of the mandibular ramus possessed a coronoid process. In the back, there were two other processes, situated medially and laterally. The medially situated process was referred to as a condylar process, and the laterally situated process was referred to as an alveolar process. The alveolar process detected on the mandibular ramus has not been reported in any rodent species, other than those of the family Spalacidae. Blind mole rats can be a real eye-opener for evolutionary science. The burrowing rodents are key to answering a controversial question about how new species arise.

Bone Disease and Serum Fibroblast Growth Factor-23 Levels in Renal Transplant Recipients.

BACKGROUND: Posttransplantation bone disease develops commonly and results in important complications. In this study, we aimed to investigate the relationship between bone diseases and serum fibroblast growth factor-23 (FGF-23) in renal transplant recipients. METHODS: This study was conducted in 106 kidney transplant recipients (KTrs; group G1) and 30 patients with chronic kidney disease (group G2). Patients with fever, heart failure, angina pectoris, acute renal failure, malignant disease, or any gastrointestinal disease were excluded. KTrs were treated with triple immunosuppressive drugs including glucocorticoids. Complete blood count (CBC), blood urea nitrogen (BUN), creatinine, glomerular filtration rate (GFR, Modification of Diet in Renal Disease [MDRD] formula), lipid profile, calcium (Ca), phosphorous (P), parathormone (PTH), 25OHD3, serum levels of tacrolimus/cyclosporine, and intact FGF-23 were measured. Bone mineral density (BMD) was measured with dual energy X-ray absorptiometry. RESULTS: The mean patient age was 40.1 ± 11.1 years and 39.2 ± 11.3 years in G1 and G2, respectively (P > .05). In G1 and G2, 76 and 15 patients were male, respectively. Compared with the G2 patients, G1 patients had lower body mass index (BMI), serum glucose levels, P, Mg, and Ca·P (P < .05 for all). T scores of the lumbar vertebrae/femur were -1.82 ± 0.99/-1.34 ± 0.89 and -1.13 ± 1.34/-0.51 ± 1.18 in G1 and G2 patients, respectively (P < .05 for all). The incidences of osteopenia/osteoporosis in the lumbar spine and femur were 50.9%/27.4% and 57.5%/10.4% in G1 and 16.6%/23.3%, and 40%/3.3% in G2. There were positive correlations between BMD and BMI, the time elapsed after renal transplantation, and GFR. In our study, a statistically significant relationship was found between lipid parameters and BMD, PTH, and 250HD3 levels, as well as use of corticosteroid and calcineurin inhibitors (P < .05 for all). In G1 and G2, BMD of the lumbar spine in patients with serum creatinine >1.5 mg/dL was lower than that in patients with serum creatinine <1.5 mg/dL. CONCLUSION: The association between age and BMD was found only in the femur of KTrs. No relationship was observed between serum FGF-23 levels and BMD values. In both groups, the BMD T score of the lumbar spine was lower compared to the BMD T score of the femur and in patients with serum creatinine >1.5 mg/dL. In long-term follow-up of renal transplantation by as much as 58 months, the incidence of bone disease such as osteoporosis/osteopenia was as high as 67% and was also higher than that of nontransplant patients with similar GFR. In addition to decreased renal function, dyslipidemia, inflammation, and continuing hypophosphatemia were also accompanied by decreased BMD as in cardiovascular disease in KTrs.

The efficacy of single-high dose inhaled corticosteroid versus oral prednisone treatment on exhaled leukotriene and 8-isoprostane levels in mild to moderate asthmatic children with asthma exacerbation

BACKGROUND: The anti-inflammatory effect of high-dose inhaled corticosteroids (ICS) in children with asthma exacerbation is unknown. We aimed to investigate the efficacy of single-high dose ICS versus oral prednisone treatment followed by a course of six day high-dose ICS or oral prednisone (P) treatment on the concentrations of Cys-LTs and 8-isoprostane levels in the exhaled breath condensate (EBC) of children with asthma exacerbation. METHODS: Ninety-four children with moderate-severe asthma exacerbation were evaluated with asthma scores, peak expiratory flow rate (PEF), forced expiratory volume in first second (FEV1) and exhaled Cys-LT and 8-isoprostane levels before and after treatment. EBC was collected from 52 patients before and four hours after treatment with inhaled fluticasone propionate (FP) (4000 μg) or P and after six days of treatment with FP-1000 μg/day or P. Cys-LTs and 8-isoprostane concentrations were determined using a specific immunoassay kit. RESULTS: Both single high-dose FP (n = 59) and p (n = 35) treatment resulted in a significant improvement in asthma score (p < 0.0001), PEF (p < 0.0001), and FEV1 (p < 0.0001). Cys-LT concentration in the EBC decreased significantly both after the initial treatment (p = 0.001), and at the end of the six-day period in the FP group (p < 0.0001). 8-Isoprostane concentration was lower only after six days of treatment with FP-1000 μg/day in the FP group (p = 0.023). There was a significant decrease in exhaled Cys-LTs after four hours (p = 0.012) and six days of P treatment (p = 0.018) in children with asthma exacerbation. CONCLUSIONS: High-dose ICS treatment may be useful in the treatment of children with asthma exacerbation. The effects start as early as after four hours. The suppression of Cys-LTs production contributes to the early effects. Suppression of both Cys-LTs and oxidants may favourably contribute to the effects observed later

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The efficacy of single-high dose inhaled corticosteroid versus oral prednisone treatment on exhaled leukotriene and 8-isoprostane levels in mild to moderate asthmatic children with asthma exacerbation.

BACKGROUND: The anti-inflammatory effect of high-dose inhaled corticosteroids (ICS) in children with asthma exacerbation is unknown. We aimed to investigate the efficacy of single-high dose ICS versus oral prednisone treatment followed by a course of six day high-dose ICS or oral prednisone (P) treatment on the concentrations of Cys-LTs and 8-isoprostane levels in the exhaled breath condensate (EBC) of children with asthma exacerbation. METHODS: Ninety-four children with moderate-severe asthma exacerbation were evaluated with asthma scores, peak expiratory flow rate (PEF), forced expiratory volume in first second (FEV1) and exhaled Cys-LT and 8-isoprostane levels before and after treatment. EBC was collected from 52 patients before and four hours after treatment with inhaled fluticasone propionate (FP) (4000 µg) or P and after six days of treatment with FP-1000 µg/day or P. Cys-LTs and 8-isoprostane concentrations were determined using a specific immunoassay kit. RESULTS: Both single high-dose FP (n=59) and p (n=35) treatment resulted in a significant improvement in asthma score (p<0.0001), PEF (p<0.0001), and FEV1 (p<0.0001). Cys-LT concentration in the EBC decreased significantly both after the initial treatment (p=0.001), and at the end of the six-day period in the FP group (p<0.0001). 8-Isoprostane concentration was lower only after six days of treatment with FP-1000 µg/day in the FP group (p=0.023). There was a significant decrease in exhaled Cys-LTs after four hours (p=0.012) and six days of P treatment (p=0.018) in children with asthma exacerbation. CONCLUSIONS: High-dose ICS treatment may be useful in the treatment of children with asthma exacerbation. The effects start as early as after four hours. The suppression of Cys-LTs production contributes to the early effects. Suppression of both Cys-LTs and oxidants may favourably contribute to the effects observed later.

Relationship between exhaled leukotriene and 8-isoprostane levels and asthma severity, asthma control level, and asthma control test score

OBJECTIVE: Exhaled breath condensate (EBC) is a completely non-invasive method for the collection of airway secretions to measure intense inflammation in the airways of asthmatics. It has been shown that the childhood asthma control test (c-ACT) is a good tool for use in the evaluation of asthmatics. Whether the c-ACT score and asthma control level correlate with the airway inflammation is not well known. We aimed to evaluate the relationship between exhaled cysteinyl leukotrienes (Cys-LTs) and 8-isoprostane levels and asthma severity, asthma control level and c-ACT score in asthmatic children. METHODS: Thirty asthmatic children were evaluated with c-ACT score and pulmonary function tests. Asthma severity and asthma control level were assessed according to GINA. EBC was collected and Cys-LTs and 8-isoprostane concentrations were determined using a specific immunoassay kit. RESULTS: Exhaled 8-isoprostane level in patients with moderate persistent asthma [114 (55-146) pg/ml] was higher than in the mild persistent group [52 (21-91) pg/ml] (p = 0.05, Mann-Whitney U [MWU]). EBC 8-isoprostane in children with 1-4 asthma exacerbations/year [52 (16-80) pg/ml] was significantly lower than in children with > 4 asthma exacerbations/year [114 (57-129) pg/ml] (p < 0.05, MWU). No significant relation was determined between exhaled 8-isoprostane and Cys-LTs levels and c-ACT score and asthma control level. Exhaled 8-isoprostane correlated negatively with bronchodilator response (p = 0.015, r = −0.45). CONCLUSIONS: Exhaled 8-isoprostane, as an oxidative stress specifier, was found to be increased in relation with asthma exacerbation frequency and oxidative stress increases with the severity of asthma. In contrast to asthma severity level, c-ACT score and asthma control level may not reflect airway inflammation

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Relationship between exhaled leukotriene and 8-isoprostane levels and asthma severity, asthma control level, and asthma control test score.

OBJECTIVE: Exhaled breath condensate (EBC) is a completely non-invasive method for the collection of airway secretions to measure intense inflammation in the airways of asthmatics. It has been shown that the childhood asthma control test (c-ACT) is a good tool for use in the evaluation of asthmatics. Whether the c-ACT score and asthma control level correlate with the airway inflammation is not well known. We aimed to evaluate the relationship between exhaled cysteinyl leukotrienes (Cys-LTs) and 8-isoprostane levels and asthma severity, asthma control level and c-ACT score in asthmatic children. METHODS: Thirty asthmatic children were evaluated with c-ACT score and pulmonary function tests. Asthma severity and asthma control level were assessed according to GINA. EBC was collected and Cys-LTs and 8-isoprostane concentrations were determined using a specific immunoassay kit. RESULTS: Exhaled 8-isoprostane level in patients with moderate persistent asthma [114 (55-146)pg/ml] was higher than in the mild persistent group [52 (21-91)pg/ml] (p=0.05, Mann-Whitney U [MWU]). EBC 8-isoprostane in children with 1-4 asthma exacerbations/year [52 (16-80)pg/ml] was significantly lower than in children with >4 asthma exacerbations/year [114 (57-129)pg/ml] (p<0.05, MWU). No significant relation was determined between exhaled 8-isoprostane and Cys-LTs levels and c-ACT score and asthma control level. Exhaled 8-isoprostane correlated negatively with bronchodilator response (p=0.015, r=-0.45). CONCLUSIONS: Exhaled 8-isoprostane, as an oxidative stress specifier, was found to be increased in relation with asthma exacerbation frequency and oxidative stress increases with the severity of asthma. In contrast to asthma severity level, c-ACT score and asthma control level may not reflect airway inflammation.

Prevalance of latex sensitization and associated risk factors in Turkish children with spina bifida.

PURPOSE: Among children with spina bifida there is a high prevalence of latex allergy in reports from Europe and USA. The aim of this study was to investigate the incidence of latex sensitization and associated risk factors in children with spina bifida in Middle-Eastern region (Istanbul, Turkey). MATERIALS AND METHODS: Eighty consecutive children affected with spina bifida were included in the study. Data were collected about age, sex, family and personal history of atopy, and the number and type of operations classified as neurosurgical, urological, and orthopedic. All patients underwent skin prick test using latex solutions and cross-reacting foods. Total and latex-specific IgE levels were also measured. RESULTS: Latex sensitization was shown in 16% (13/80) of the patients with specific IgE > 0.7 kU/L. The number of operation, using intermittent bladder catheterization and ventricular-peritoneal shunt were significantly associated with latex sensitization. CONCLUSION: The incidence of latex sensitization in this study was lower than reported in the literature. Environmental factors such as the type and number of operations and geographical location may play a role in the development of latex sensitization.

Inappropriate antimicrobial use in Turkish pediatric hospitals: a multicenter point prevalence survey.

OBJECTIVES: Although well-defined principles of rational antimicrobial use are available, inappropriate prescribing patterns are reported worldwide. Accurate information on the usage of antimicrobials, including factors associated with and influencing their use, is valuable for improving the quality of prescription practices. METHODS: In this cross-sectional point prevalence survey, data on patients hospitalized in 12 different children's hospitals were collected on a single day. Appropriateness of prescription was compared between the types of antimicrobials prescribed, indications, wards, and presence of/consultation with an infectious disease physician (IDP). RESULTS: A total 711 of 1302 (54.6%) patients evaluated were receiving one or more antimicrobial drugs. The antimicrobial prescription rate was highest in pediatric intensive care (75.7%) and lowest in the surgery wards (37.0%). Of the 711 patients receiving antimicrobials, 332 patients (46.7%) were found to be receiving at least one inappropriately prescribed drug. Inappropriate use was most frequent in surgery wards (80.2%), while it was less common in oncology wards (31.8%; p<0.001). Respiratory tract infection was the most common indication for antimicrobial use (29.4%). Inappropriate use was more common in deep-seated infections (54.7%) and respiratory infections (56.5%). Fluoroquinolones were used inappropriately more than any other drugs (81.8%, p=0.021). Consultation with an IDP appears to increase appropriate antimicrobial use (p=0.008). CONCLUSIONS: Inappropriate antimicrobial use remains a common problem in Turkish pediatric hospitals. Consultation with an IDP and prescribing antimicrobial drugs according to microbiological test results could decrease the inappropriate use of antimicrobials.

The effect of rapid diagnostic testing for influenza on the reduction of antibiotic use in paediatric emergency department.

AIM: To determine the influence of rapid diagnosis of influenza on antibiotic prescribing to children presenting with influenza-like illness in the emergency department in a inner city hospital in Istanbul, Turkey. METHODS: Patients aged 3 to 14 years presenting to an urban children's teaching hospital emergency department were screened for fever and cough, coryza, myalgias and/or malaise. After obtaining informed consent, patients were allocated into two groups. Group 1: patients were prescribed antibiotics after only physical examination; or Group 2: patients were prescribed antibiotics after rapid influenza testing. Nasopharyngeal swabs obtained from all patients were immediately tested in a single-blind manner with Influenza A/B Rapid Test(R) for influenza A and B. RESULTS: A total of 97 patients were enrolled, and 33 (34%) of these tested positive for influenza. Although frequency of positive results for influenza between the groups was similar (36% vs 32%, respectively), patients in Group 2 were less likely to be prescribed antibiotics when compared to those in Group 1 (32% vs 100%, respectively, p < 0.0001). CONCLUSION: Rapid diagnosis of influenza in the paediatric emergency department may allow a significant reduction in the over-prescription of antibiotics.

Wolfram syndrome in a family with variable expression.

Wolfram syndrome is a rare neurodegenerative disorder with autosomal recessive inheritance. The main characteristic features of this disorder are diabetes mellitus and optic atrophy. However, diabetes insipidus, sensorineural deafness, renal tract and neurologic abnormalities are seen in majority of patients. In this study, we describe a family in which two members had the main features of the syndrome while a third sibling had only sensorineural deafness. DNA analysis revealed that the fully affected siblings were homozygote for a pointmutation on chromosome 4p whereas the third sibling with deafness was a heterozygote carrier for the same mutation. The characteristics of disease and phenotypic variations that possibly related to heterozygote carrier state were discussed.

Mediators of inflammation in children with type I diabetes mellitus: cytokines in type I diabetic children.

OBJECTIVES: Recent evidence favors primary role of cellular autoimmunity and its humoral mediators in pathogenesis and following Type I diabetes mellitus (DM). The present study was carried out to investigate serum concentrations of C-reactive protein (CRP), interleukin (IL)-6, IL-8 and tumor necrosis factor (TNF)-alpha in children with type I DM. Potential role of lipid metabolism, glycemic control, body mass index (BMI) and disease duration were evaluated. DESIGN AND METHODS: Thirty-five children with type I DM and 30 age and gender matched nondiabetic controls were recruited for this study. RESULTS: Circulating IL-8 levels were elevated in children with type I DM (12.7 +/- 1.7 pg/mL) compared with nondiabetic controls (5.5 +/- 0.3 pg/mL) and the difference remained significant after adjustment for cofactors and covariates (p: 0.033). Although statistically insignificant serum CRP concentrations were slightly higher in diabetic children (p: 0.075). Serum TNF-alpha and IL-6 levels were comparable in diabetic and nondiabetic groups. However newly diagnosed (<1 yr) cases had higher TNF-alpha and IL-6 levels compared to cases with longer standing DM. In diabetic children BMI was independently associated with an increase in serum IL-8 levels. Serum CRP, lipids, apolipoproteins and glycemic control were not significant predictors of cytokine concentrations in children with type I DM. CONCLUSION: Circulating levels of IL-8 were elevated and were correlated with BMI in children with type I DM, hinting perhaps at adipose tissue as a site of production. Elevated systemic IL-6 and TNF-alpha were limited to newly diagnosed cases suggesting activation of the inflammatory immune response system at early stages of the disease.

Immunoglobulin isotypes and IgG subclasses in recurrent infections.

The frequency of major immunoglobulin isotype and IgG subclass deficiencies among 74 children aged six months to 14 years with recurrent infections was studied. All children had at least five to six episodes of respiratory tract infections, while recurrent diarrhea had occurred in eight. Two selective and six partial IgA deficiencies were detected. IgG4, IgG3 and IgG2 deficiencies, either isolated or combined, were found in 13, nine and one patient respectively. Among these there were two combined deficiencies of IgA + IgG4, three of IgA + IgG3, one of IgA + IgG2 + IgG4, one of IgG1 + IgG3 and two of IgG1 + IgG4. There was one patient with panhypogammaglobulinemia. Our results, similar to those of other studies, showed that the occurrence of the Ig isotype, particularly subclass deficiencies, is not uncommon in children with frequent infections.

Unusual malformations in occult spinal dysraphism.

Two cases of occult spinal dysraphism with different clinical symptoms, signs and congenital pathologies are presented. One had malformations including scoliosis, dermoid tumor, hydromyelia, diastematomyelia, dermal sinus, low conus, vertebrae anomalies and dextrocardia. The occurrence of dextrocardia in association with occult spinal dysraphism was found to be extremely unusual. The second case is presented in relation to the rarity of teratoma with dermal sinus and tethered cord in the lumbar area. Myelography, computed tomography, (CT), Myelo CT and magnetic resonance were used in making a diagnosis.

Kawasaki disease associated with gallbladder hydrops.

Kawasaki disease is an acute, febrile, systemic syndrome of unknown etiology which principally affects young children. We present a five-year-old boy, who developed an unusual finding, acute hydrops of the gallbladder, in addition to the characteristic signs and symptoms of Kawasaki disease. Clinical features of the disease and treatment regimens, including early administration of intravenous gamma globulin (IVGG), which prevents cardiac complications, are discussed.

Whistling face (Freeman-Sheldon) syndrome in two siblings.

Two siblings with typical manifestations of whistling face (Freeman-Sheldon) syndrome (WFS) born to unaffected parents are presented. In Case 1, deep-set eyes, epicanthus, blepharophimosis, right lid ptosis, strabismus, anti mongoloid slant, small mouth, mask-like face, high-arched palate, nasal speech, dysphagia, kyphosis and minimal scoliosis were noted, while Case 2 displayed blepharophimosis, mask-like face, long philtrum, high-arched palate, scoliosis, bilateral post-axial polydactyly of the feet and pes varus. We corrected the blepharophimosis in Case 1 by bilateral canthotomy and canthoplasty. This syndrome is usually inherited as an autosomal dominant trait; however, some authors have reported an autosomal an autosomal recessive form of this syndrome similar to our cases. Nevertheless, this could be explained by genetic expression of the mutant gene.