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Background:  Cochlear implants (CIs) improve spoken language skills as well as social and academic performance in children with severe and profound sensorineural hearing loss., Purpose:  We compare the social skills, academic competence, and problem behaviors in children with CIs and their typical-hearing peers and investigate the relationship between language skills and social skills, academic competence, and problem behaviors in children with CIs., Research Design:  Thirty-two children with CIs were divided into two groups according to implantation age: early ( n  = 17) and late-implanted group ( n  = 15). The control group was formed by the inclusion of five same-sex classmates of each implanted child selected by the teachers of the children with CIs. Thus, 160 typical-hearing children were included in the control group., Study Sample:  Thirty-two children with CIs and 160 typical-hearing children participated in this study., Data Collection and Analysis:  Primary school teachers rated the children's social skills, academic competence, and problem behaviors using the Social Skills Rating System (SSRS). The language skills of the children with CIs were assessed with the Turkish Expressive and Receptive Language Test (TIFALDI)., Results:  A statistically significant difference was found between the SSRS scores of the study and control groups, which was mostly due to lower social skills, and academic competence, and higher problem behavior ratings in the late-implanted group. In the study group, there was a statistically significant correlation between TIFALDI and SSRS scores., Conclusion:  Early cochlear implantation provides advantages in social skills and vocabulary for children with profound congenital hearing loss. Late-implanted children need special support in different developmental areas despite attending mainstream education., Competing Interests: None declared., (American Academy of Audiology. This article is published by Thieme.)

Background: Left ventricular systolic dysfunction (LVSD) may develop without coronary artery disease, hypertension (HT), or valvular pathologies in patients with diabetes mellitus (DM), which is defined as diabetic cardiomyopathy (DCM) and its pathophysiology is still unclear. Diabetic retinopathy (DR) is a microvascular complication of DM, and patients with DR have increased risk for the development of heart failure (HF). Two-dimensional speckle tracking echocardiography (2D-STE) evaluates longitudinal deformation in left atrium (LA) myocardium and previous studies utilizing 2D-STE have revealed the detrimental effects of DM on LA functions. Although some studies have shown the association between DR and left ventricle (LV) systolic functions, as far as the researchers of this study investigated, there is no study evaluating the relationship between LA deformation parameters and DR. Hence, we aimed to investigate the relationship between the presence and the degree of DR and LA deformation parameters., Results: LA deformation parameters were analyzed in terms of LA reservoir, conduit, and contractile functions according to the degree of DR. LA reservoir strain value was 14.2 ± 3.6 in normal retina group, 12.2 ± 4.1 in non-proliferative diabetic retinopathy (NPDR) group, and 13 ± 3.7 in proliferative diabetic retinopathy (PDR) group (P = 0.04). LA contractile strain was 15.9 ± 6.8 in normal retina group, 13.1 ± 47.4 in NPDR group, and 9.9 ± 4.7 in PDR group (P < 0.001). LA conduit strain was 30.1 ± 6.6 in normal retina group, 25.3 ± 6.5 in NPDR group, and 22.9 ± 4.9 in PDR group (P < 0.001). Proportional odds regression for association between clinical data, echocardiographic parameters, and LA contractile strain function showed that increasing creatinine (from 0.7 to 1.0; OR 0.71; 95% CI 0.51-0.99; P = 0.04), DR presence (OR 0.24; 95% CI 0.11-0.50; P = 0.001), and increasing left atrial volume index (LAVI) (from 33.5 to 52.6; OR 0.62; 95% CI 0.43-0.89; P = 0.01) were associated with decreasing LA function; however, other variables indicated no association., Conclusions: Our results showed the relationship between LA deformation parameters and DR, although microvascular involvement is not a certainly defined cardiovascular risk factor. Further prospective studies are needed to determine the clinical importance of DR presence and its degree for deformation parameters., (© 2022. The Author(s).)

Objectives: This study aims to evaluate the relationship between phoneme recognition skills and language development skills in pediatric auditory brainstem implant (ABI) users. It further intends to identify the delays and problems that may occur in the phoneme recognition skills of children with ABI and shed light on rehabilitation programs., Methods: Our study included 20 children using ABI and another 20 using cochlear implants (CI). They were aged between 6 and 8 years 11 months. The participants exhibited homogenous demographic and audiological characteristics. The Turkish version of the Test of Language Development-Primary: Fourth Edition (TOLDP:4) was used to evaluate the language development skills, and the Turkish version of the Phoneme Recognition Test (PRT) was applied to assess the phoneme recognition skills., Results: There was a statistically significant difference (p < 0.05) in the PRT values as well as in the language development skills between the children with ABI and those with CI. It was observed that the values of the children with CI were significantly higher than those of children with ABI., Conclusion: Although children with ABI were not able to match the skills of their peers with CI, their language development and communication skills improved. It is believed that this study will contribute to the literature by demonstrating that the use of ABI improves phoneme recognition skills in children who are not eligible for CI or who do not adequately benefit from CI., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Macrolides are often accepted as safe antibiotics due to their low allergenicity. However, studies on macrolides, particularly studies evaluating cross-reactivity in macrolides, are highly rare in children. This study aimed to evaluate the clinical manifestations, confirmation rate, and frequency of cross-reactivity in children admitted with suspicious clarithromycin or azithromycin allergy., Materials and Methods: A total of 61 children suspected of macrolide antibiotic allergy (clarithromycin, n = 39 and azithromycin, n = 22) were evaluated. Allergy work-up including drug provocation tests were performed in all patients to confirm drug allergy., Results: Macrolide allergy was confirmed in 9.8% (n = 6) of patients (azithromycin, 18.2% [n = 4] and clarithromycin, 5.1% [n = 2]). There was no significant difference between the confirmation rate of clarithromycin and azithromycin (P = .117). Cross-reaction with clarithromycin was confirmed in 2 (33.3%) patients with azithromycin allergy., Conclusion: Drug skin tests are not capable of confirming or ruling out macrolide allergy, and oral provocation tests are essential for a definitive diagnosis. Cross-reactivity, albeit rare, can occur between clarithromycin and azithromycin, which are the most frequently used macrolides in children.

The relationship between insulin resistance and thyroid nodules is not clearly understood. The purpose of this study is to investigate the relationship between insülin resistance and thyroid nodules in non-diabetic patients. 410 patients who applied to the Internal Medicine outpatient clinic from June 2018 to July 2019 were reviewed retrospectively. A total of 216 non-diabetic patients were divided into two groups. The relationship of 105 patients with thyroid nodules and 110 patients without thyroid nodules with insulin resistance was investigated. Homeostasis model assessment of insulin resistance value, serum triglycerides and total cholesterol level, and the ratio of women were higher in the group with thyroid nodules (p < 0.05). There was a positive and significant relationship between homeostasis model assessment of insulin resistance value and thyroid nodules (r + 0.29, p < 0.05). In non-diabetic thyroid nodule patients, a significant relationship was detected between nodule formation and insulin resistance. However, these data must be confirmed by other studies in the future., (© 2021. Società Italiana di Medicina Interna (SIMI).)

Background: Several factors that increase the risk of severe food-induced anaphylaxis have been identified. Objective: We aimed to determine the demographic, etiologic, and clinical features of food-induced anaphylaxis in early childhood and also any other factors associated with severe anaphylaxis. Methods: We carried out a medical chart review of anaphylaxis cases from 16 pediatric allergy and immunology centers in Turkey. Results: The data of 227 patients with 266 food-induced anaphylaxis episodes were included in the study. The median (interquartile range) age of the first anaphylaxis episode was 9 months (6-18 months); 160 of these patients were boys (70.5%). The anaphylaxis episodes were mild in 75 cases (28.2%), moderate in 154 cases (57.9%), and severe in 37 cases (13.9%). The most frequent food allergens involved were cow's milk (47.4%), nuts (16.7%), and hen's egg (15.8%). Epinephrine was administered in only 98 (36.8%) of these anaphylaxis episodes. A logistic regression analysis revealed two statistically significant factors that were independently associated with severe anaphylaxis: the presence of angioedema and hoarseness during the anaphylactic episode. Urticaria was observed less frequently in patients who developed hypotension. In addition, confusion and syncope were associated with 25.9- and 44.6-fold increases, respectively, in the risk of concomitant hypotension. Conclusion: Cow's milk, nuts, and hen's egg caused the majority of mild and moderate-to-severe anaphylaxis episodes. The presence of angioedema and hoarseness in any patient who presents with a history of food-induced anaphylaxis should alert clinicians that the reaction may be severe. In addition, the presence of confusion, syncope, or stridor probably indicates concomitant hypotension.

Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease of the central nervous system that is caused by John Cunningham virus (JCV). It occurs almost exclusively in immunosuppressed individuals, for example, patients with AIDS and hematological and lymphoreticular malignancies. In this article, we present a review of the literature and 2 case reports with PML. The first report examines a 15-year-old male (who presented with dedicator of cytokinesis 8 deficiency) who was diagnosed as having PML based on characteristic magnetic resonance imaging (MRI) lesions and a positive PCR for JCV in cerebrospinal fluid. He was transferred for bone marrow transplantation after stabilization with therapy of maraviroc and cidofovir. The second report examines a 6-year-old male who presented with encephalitis and was also diagnosed with AIDS. He was diagnosed with PML and started treatment with cidofovir. His clinical status and MRI findings deteriorated rapidly. In immunosuppressive patients who developed encephalopathy, JCV ought to be considered.

Concern about the negative effects of logging residue extraction on the sustainability of forest ecosystems has been rising recently. Tree residues, including leaves, branches, bark and roots, left in the forest after logging may supply most of the nutrients for tree growth. The aim of this study was to (i) determine the carbon and nutrient stocks in different components and (ii) model the carbon and nutrient stocks in tree biomass of a mature Scots pine forest. The study site was located on the Turkmen mountain range in the Central Anatolia Region of Turkey. In sample plots, stand measurements were made, and samples collected from trees, soil and the forest floor for analysis of carbon and nutrients and the stock of each nutrient per unit area were calculated. Data were analysed using analysis of variance and regression analysis. Significant differences were found in carbon and nutrient concentrations and stocks between ecosystem components. C, Ca, Mg, Na, Fe, Cu and Mn stocks were higher in wood; the N stock was higher in needles, and P, K, S and Zn stocks were higher in roots. In the ecosystem, trees had the highest C stock; the soil had the highest N, P, K, Ca, Mg, Na, Cu, Zn and Mn stocks, and the forest floor had the highest Fe and S stocks. Therefore, it is critical that the forest floor is protected as it is an important element of the ecosystem nutrient cycle and source of Fe and S stocks. Maximum attention should be paid to leaving behind needles, bark, roots and thin branches with low economic value to minimise carbon and nutrient loss in the nutrient-limited forests. Equations predicting carbon and nutrient stocks through stem volume can be used for estimation of nutrient loss due to biomass removed from the system through interventions, contributing to sustainable forest management., (© 2021. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Friedreich's ataxia (FRDA) is a rare neurodegenerative disorder which is caused by triplet repeat expansion (GAA) in the first intron of FXN gene. In this present study, we generated induced pluripotent stem cells (iPSC) lines from fibroblasts of three unrelated FRDA patients using integration-free episomal vectors. All iPSC lines express the pluripotency markers such as OCT4 and SSEA4, display normal karyotypes and can differentiate into all three germ layers via in vivo teratoma formation assay., (Copyright © 2021 The Authors. Published by Elsevier B.V. All rights reserved.)

Purposes: The main purpose of this study is to investigate whether there is a difference in phoneme recognition and school-age language skills in children with bilateral and unilateral cochlear implants (CI). The second aim of the study is to examine language-based skills in bilateral cochlear implanted children with the first implant, second implant and in the bilateral listening situations., Method: 60 to 108-month-old children with similar demographic and audiological features were included. Of the 64 participants in total, 30 are bilateral cochlear implant users and 34 of them use unilateral cochlear implants. Turkish version of "Test of Language Development-Primary: Fourth edition (TOLD-P:4)" and "Phoneme Recognition Test (PRT)" were implemented for the evaluation of the language sub-components skills and auditory perception. In addition, the PRT test audio file was presented directly to the implant with connection cables via the fitting program methodologically., Results: Children with bilateral cochlear implants were more successful in all language-based skills than children with unilateral cochlear implants (p < 0.05). In the PRT test, the most successful scores were obtained in the bilateral listening conditions, the second with the experienced implant side, and the most unsuccessful scores in the listening conditions with second implant., Conclusion: Bilateral cochlear implants are very useful in terms of language-based skills in children with severe/profound hearing loss. This can positively affect even the future academic and social skills of children.

Objective: Irritant contact dermatitis and Allergic contact dermatitis are two distinct forms of contact dermatitis. Allergic contact dermatitis is a Type 4 (delayed-type) hypersensitivity reaction that occurs during subsequent contact with an allergen to a previously sensitized person. The number of allergens that cause allergic contact dermatitis is increasing day by day. Although it is not the gold standard for the detection of these allergens, skin patch testing is a very helpful method. This study aimed to determine the most common contact allergens in the pediatric age group., Material and Methods: All patients with the diagnosis of contact dermatitis who underwent a skin patch test (TRUE TEST) in the department of Pediatric Allergy and Immunology between March 2017-February 2018 were enrolled in this study. The patch test was evaluated 72 hours later by the same physician and interpreted as recommended by the American Academy of Dermatology. In addition to the patient files, demographic and clinical characteristics, localization of lesions, and itch score according to visual analog scale were recorded., Results: A total of 80 children enrolled in the study; 45 (56.3%) were girls and 35 (43.7%) were boys. The mean age of the children was 7.37±3.84 years and 57.5% of the patients who underwent skin patch testing had a positive response to at least one or more allergens. The most common allergens were Nickel sulfate, CI + Me-Isothiazolinone, Thiuram Mix, Formaldehyde, and P-tert-butylphenol formaldehyde resin (14.8%, 10%, 6.3%). There was no difference in terms of age, sex, duration of complaints, and pruritus score according to nickel sensitization., Conclusion: In the presence of chronic dermatitis in children, allergic contact dermatitis should be considered in the differential diagnosis. The culprit allergen should be determined. Also, the most common contact allergen is Nickel Sulphate in the world and the increased sensitization to other allergens is due to the increased contact of children with cosmetics and different contact allergens., Competing Interests: Conflict of Interest: The authors have no conflicts of interest to declare., (Copyright © 2021 Turkish Pediatric Association.)

Background: Urticaria can be the only sign of a food allergy or can be seen together with other signs and symptoms of a food allergy., Objective: To determine the demographic, etiologic, and clinical features of food-induced acute urticaria in childhood., Methods: Patients suspected of food-induced acute urticaria were included in this prospective cross-sectional multicenter study., Results: Two hundred twenty-nine urticaria cases were included in this study. Seventeen patients who did not meet the inclusion criteria of the study were excluded. Of the 212 included cases, 179 (84.4%) were diagnosed with definitive food-induced acute urticaria. The most common foods causing acute urticaria were cow's milk, hen's eggs, and nuts in 56.4, 35.2, and 19% of cases, respectively. The positive predictive value of a history of milk-induced acute urticaria together with a milk-specific IgE >5 kU/L for cow's milk-induced acute urticaria was 92% (95% CI: 81-96%). A history of cow's milk-induced and/or hen's egg-induced acute urticaria was consistent with a definitive diagnosis of food-induced urticaria (Chen's kappa: 0.664 and 0.627 for milk and eggs, respectively). Urticaria activity scores were higher in patients with food-induced acute urticaria (p = 0.002)., Conclusion: Cow's milk, hen's eggs, and nuts were the most common allergens in the etiology of childhood food-induced acute urticaria. Although the urticaria activity score provides guidance for diagnosis, an oral food challenge is often essential for the definitive diagnosis of a patient with a history of food-induced acute urticaria., (© 2021 S. Karger AG, Basel.)

Although it is known that auditory training is essential for hearing-impaired individuals, patients do not willingly participate in auditory training sessions, because individual training is a time-consuming and costly process. Computer-based auditory training programs are under development for reducing the cost and time. The aim of this study is to develop a computer-based auditory training program and to evaluate the usability of the program by applying it to adults with normal hearing indifferent age groups and professions. The developed auditory training program consists of nine modules: identification, discrimination, recognition, auditory closure, comprehension, auditory sequencing, phonological awareness, auditory memory, and attention. Forty adults (age ranges of 25-34, 35-44, 45-54, and 55-65 years), nine audiologists, and one software engineer were included in this study. The computer-based auditory training program was applied to all individuals. An evaluation form was filled out by the participants to evaluate the usability of and satisfaction with the program, and the average performances of the individuals were calculated for each module. The form measures five usability subscales: ease of use, comprehensibility, design, satisfaction, and motivation. The professionals evaluated the program and found it to be quite positive in terms of design, ease of use, motivation, and comprehensibility, and positive in terms of the satisfaction subscale. The participants evaluated all factors and found them to be quite positive. There were no statistically significant differences between the gender, age, and computer use groups in terms of the average performance value of the modules (p > 0.05). However, a significant difference was found in the average performance for the auditory sequencing module considering the educational status of individuals (p = 0.019). Furthermore, it was determined that computer experience (> 5 years) and having a personal computer have a significant effect on auditory memory (p = 0.049) and identification (p = 0.027). These results show that the instructions and information used in the program are clear and understandable, the colors and texts used in the program are readable, the program is easy to use, and the individuals are not disturbed by the sounds used in the program. However, it would be valuable to apply it to individuals with hearing losses to evaluate the efficacy of the program.

Objective: Intracranial pressure increase is known to affect inner ear pressure through the cochlear and vestibular aqueducts. This finding forms a good model for inner ear pressure studies. Standard techniques used to detect this pressure increase are neither reliable nor easily repeatable or cheap. Studies with immitancemetry and otoacoustic emissions have been giving hopeful results. This study aims to confirm the results in the literature with wideband tympanometry and add a new parameter of otoacoustic emissions to inner ear pressure testing., Methods: Wideband tympanometry (WBT) and distortion product otoacoustic emissions (DPOAE) tests were applied to 40 healthy participants in sitting, supine, and Trendelenburg positions. DPOAE were measured under ambient or peak pressure. Resonance frequency, tympanic peak pressure, 1000, 1500, 2000, 3000, 4000, and 6000 Hz frequencies in DPOAE were measured., Results: The increase in the tympanic peak pressure and the decrease in resonance frequency (RF) due to position change were found statistically significant (p<0.01). Signal noise ratio (SNR) decrease at 1 kHz frequency and SNR increase at 2, 3, 6 kHz in the normal protocol, SNR decrease at 1 kHz in the pressurized protocol were found statistically significant (p<0.01)., Conclusion: RF in WBT and 1 kHz DPOAE SNR parameters were found useful in supporting the diagnosis in pathologies that increase intracranial pressure and inner ear pressure. Future research may ease their widespread use in clinical practice as they are non-invasive and rapidly applicable., Competing Interests: Conflict of Interest: The authors have no conflicts of interest to declare., (© Copyright 2020 by Official Journal of the Turkish Society of Otorhinolaryngology and Head and Neck Surgery.)

Neuromuscular diseases (NMDs) encompass a variety of ailments from muscular dystrophies to ataxias, in the course of which the functioning of the muscles is eventually either directly or indirectly impaired. The clinical diagnosis of a particular NMD is not always straightforward due to the clinical and genetic heterogeneity of the disorders under investigation. Traditional diagnostic tools such as electrophysiological tests and muscle biopsies are both invasive and painful methods, causing the patients to be reluctant. Next-generation sequencing, on the other hand, emerged as an alternative method for the diagnosis of NMDs, both with its minimally invasive nature and fast processing period. In this study, clinical exome sequencing (CES) was applied to a cohort of 70 probands in Turkey, 44 of whom received a final diagnosis, representing a diagnostic rate of 62.9%. Out of the 50 mutations identified to be causal, 26 were novel in the known 27 NMD genes. Two probands had complex/blended phenotypes. Molecular confirmation of clinical diagnosis of NMDs has a major prognostic impact and is crucial for the management and the possibility of alternative reproductive options. CES, which has been increasingly adopted to diagnose single-gene disorders, is also a powerful tool for revealing the etiopathogenesis in complex/blended phenotypes, as observed in two probands of the cohort.

Purpose: The purpose of this study is to compare the temporal processing performance of children with cochlear implant (CI) according to the age of implantation and to determine their relation with auditory perception scores., Methods: In this study, 30 cochlear implant users and ten normal hearing children at 9 and 10 years were included. Children with cochlear implants are divided into two groups according to the age of implantation: group I includes participants whose implantation age is between 13 and 35 months (20 children), group II includes participants whose implantation age is between 36 and 45 months (10 children). Individuals were evaluated with random gap detection test (RGDT), duration pattern test (DPT), frequency pattern test (FPT), the Mr. Potato Head task, word recognition, and sentence recognition test., Results: A significant difference was found between the control and CI groups in temporal processing performance. The temporal processing ability of CI groups was significantly worse than those of normal hearing. Although there was no significant difference among the groups with cochlear implant in terms of temporal processing performance, children who started to use CI at an earlier age showed a tendency of better performance on temporal processing tasks. There was a significant relationship between Daily Sentence Test and FPT, and the Mr. Potato Head task and FPT rev (the score calculated by accepting the reverse patterns correctly). There was a significant relationship between duration of implant use and temporal ordering performance CONCLUSION: In this study, children with CI cannot perform as well as normal-hearing peers on temporal processing tasks, even if they had started to use their CIs at an early age. It is important to evaluate temporal processing in implanted individuals and to guide auditory training considering the evaluation results.

Objective: To report the initial surgical and audiological outcomes of three pediatric patients with severe inner ear malformations who were simultaneously implanted with cochlear and brainstem implants in the same surgical session., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Three pediatric patients with severe inner ear malformations between ages of 1.9 to 2.5 years, who were simultaneously implanted with cochlear implant in one ear and auditory brainstem implant in the other ear., Intervention(s): Simultaneous application of cochlar implant in one ear, auditory brainstem implant in the other ear., Main Outcome Measures: Free field thresholds with cochlear and brainstem implants. Surgical issues are also discussed., Results: The study is descriptive in nature. Free field thresholds with each device alone and together showed good progress. One of the patients had slower progress possibly due to comorbid CHARGE syndrome., Conclusions: The results showed good progress in terms of audition with both devices. Simultaneous cochlear and brainstem application serves as a remedy for pediatric patients who are candidates for cochlear implant on one side and brainstem implant on the other side. With this simultaneous application precious time for auditory development is not lost.

Background : Acute rheumatic fever (ARF) continues to be a public health problem in low- and middle-income countries. Because there is no specific laboratory test for the exact diagnosis of ARF, the diagnosis is made by the modified Jones criteria. Periostin is an extracellular matrix protein which has been shown to be expressed during remodelling, mechanical stress and tissue repair. There are no data on the relationship between periostin and ARF. Aim : To evaluate serum periostin levels in patients with ARF and investigate its usefulness as a biomarker for diagnosing and monitoring the efficacy of treatment. Methods : Serum periostin levels were estimated in 31 patients with ARF and compared with a control group of 25 healthy patients. The control group comprised patients referred to the outpatient clinic for further evaluation of cardiac murmur, palpitations or chest pain. Patients who were diagnosed with any other cardiac or systemic illness after detailed systemic and cardiac examination were excluded. Results : The mean (SD) age at diagnosis was 12.1 (3.3) years in the patient group, 48.4% of whom were male. There were no significant differences in age or gender between patients and controls. All the study patients had various degrees of carditis, 58.1% had arthritis and 9.6% had Sydenham chorea. Mean (SD) serum periostin levels in patients with ARF on admission [58.4 (13.9) ng/mL] were significantly higher than in the control group [35.1 (10.7) ng/mL, p < 0.01] and were also significantly decreased in the patient group after treatment [35.1 (13.1) ng/mL, p < 0.01]. There was no significant difference in serum periostin levels regarding the severity of carditis in the patient group while patients with any severity of carditis had significantly higher periostin levels than the controls ( p < 0.01). Periostin levels were significantly correlated with ESR and CRP values before treatment, but this correlation was detected for only ESR after treatment. For the initial diagnosis of ARF, a serum periostin level of 53.45 ng/mL was found to be the cut-off point with 80.6% sensitivity and 100% specificity. Conclusion : There was a significant increase in serum periostin levels in patients with ARF and a reduction after adequate treatment which was independent of the severity of carditis. Periostin may be a biomarker which acts as an acute phase reactant in ARF.

Objective: To study the effect of age at auditory brainstem implant (ABI) surgery on auditory perception, language, and speech intelligibility., Study Design: Retrospective single cohort design., Setting: Tertiary referral center., Patients: In this study, 30 pediatric ABI users with no significant developmental issues were included. Participants were divided into two groups, according to age at surgery (Early Group: < 3 yr old [n = 15], Late Group: ≥ 3 yr old [n = 15]). Groups were matched by duration of ABI use and participants were evaluated after 5 years (±1 yr) experience with their device. The mean age at ABI surgery was 22.27 (ranged ± 6.5) months in the early group, 45.53 (ranged ± 7.9) months in the late group., Intervention(s): Retrosigmoid craniotomy and ABI placement., Main Outcome Measure(s): Auditory perception skills were evaluated using the Meaningful Auditory Integration Scale and Categories of Auditory Performance from the Children's Auditory Perception Test Battery. We used a closed-set pattern perception subtest, a closed-set word identification subtest, and an open-set sentence recognition subtest. Language performance was assessed with the Test of Early Language Development and Speech Intelligibility Rating, which was administered in a quiet room., Results: In this study, the results demonstrated that the Early Group's auditory perception performance was better than the Late Group after 5 years of ABI use, when children had no additional needs (U = 12, p < 0.001). Speech intelligibility was the most challenging skill to develop, in both groups. Due to multiple regression analysis, we found that auditory perception categories can be estimated with speech intelligibility scores, pattern perception scores, receptive language scores, and age at ABI surgery variables in ABI users with no additional handicaps., Conclusions: ABI is a viable option to provide auditory sensations for children with cochlear anomalies. ABI surgery under age 3 is associated with improved auditory perception and language development compared with older users.

The redox state of the neural progenitors regulates physiological processes such as neuronal differentiation and dendritic and axonal growth. The relevance of endoplasmic reticulum (ER)-associated oxidoreductases in these processes is largely unexplored. We describe a severe neurological disorder caused by bi-allelic loss-of-function variants in thioredoxin (TRX)-related transmembrane-2 (TMX2); these variants were detected by exome sequencing in 14 affected individuals from ten unrelated families presenting with congenital microcephaly, cortical polymicrogyria, and other migration disorders. TMX2 encodes one of the five TMX proteins of the protein disulfide isomerase family, hitherto not linked to human developmental brain disease. Our mechanistic studies on protein function show that TMX2 localizes to the ER mitochondria-associated membranes (MAMs), is involved in posttranslational modification and protein folding, and undergoes physical interaction with the MAM-associated and ER folding chaperone calnexin and ER calcium pump SERCA2. These interactions are functionally relevant because TMX2-deficient fibroblasts show decreased mitochondrial respiratory reserve capacity and compensatory increased glycolytic activity. Intriguingly, under basal conditions TMX2 occurs in both reduced and oxidized monomeric form, while it forms a stable dimer under treatment with hydrogen peroxide, recently recognized as a signaling molecule in neural morphogenesis and axonal pathfinding. Exogenous expression of the pathogenic TMX2 variants or of variants with an in vitro mutagenized TRX domain induces a constitutive TMX2 polymerization, mimicking an increased oxidative state. Altogether these data uncover TMX2 as a sensor in the MAM-regulated redox signaling pathway and identify it as a key adaptive regulator of neuronal proliferation, migration, and organization in the developing brain., (Copyright © 2019 American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.)

Objectives: This study compared the auditory reasoning skills of school-going children with early and late cochlear implantation and assessed the relationship between auditory reasoning skills, language development, vocabulary knowledge, and communication skills., Materials and Methods: In this case series study, 90 pre-lingually deaf children aged 7-10 years were assessed. Children were divided into two groups: early-implanted group with children who received cochlear implants before 3 years of age (mean, 23.45; 12-35 months) and late-implanted group with children implanted after 3 years of age (mean, 50.54; 36-84 months). Tests were performed in the auditory-visual condition. Correlational analyses were used to assess the relationships between daily communication skills, language development performances, vocabulary knowledge, and auditory reasoning skills of both the groups., Results: Auditory reasoning skills were better in the early-implanted group than in the late-implanted group (Mann-Whitney U test=518, p<0.05). Language performances of the early-implanted group were significantly better than those of the late-implanted group (receptive language performances: Mann-Whitney U=522, p<0.05; expressive language performances: Mann-Whitney U=552, p<0.05). Stepwise regression analysis showed that expressive language performances, vocabulary knowledge, and chronological age could predict 82% of the variance., Conclusion: Reasoning skills of children with cochlear implants should be supported during the language-learning process.

Objectives: The purpose of this study was to compare performance in the areas of verbal working memory (VWM), vocabulary skills, and speech intelligibility between children with cochlear implants (CIs) and children with typical development (TD). The correlations between participant variables and the scores of children with CIs in VWM and the measures of language were examined. Also, it was important to identify which variables predict VWM in children with CIs., Methods: A total of 59 children participated in this study with the study group being comprised of 31 children who had received a CI and the control group being comprised of 28 children with TD. The assessment techniques utilized in this study were the backward digit span (BDS), non-word repetition, speech intelligibility, and vocabulary skills., Results: The study results revealed significant differences in the non-word repetition, speech intelligibility, and vocabulary tasks. The results all favored the typically developing children while the findings for the BDS were equal for both groups., Discussion: In children with CIs the results for VWM observed in this study are believed to be related to the modality of assessment presentation, prior vocabulary knowledge, and familiarity with the presented material. The results from this study also revealed that the variables which predicted VWM in children with CIs were speech perception, duration of CI use, and vocabulary knowledge.

Hypohidrotic or anhidrotic ectodermal dysplasia (HED/EDA) is characterized by impaired development of the hair, teeth, or sweat glands. HED/EDA is inherited in an X-linked, autosomal dominant, or autosomal recessive pattern and caused by the pathogenic variants in 4 genes: EDA, EDAR, EDARADD, and WNT10A. The aim of the present study was to perform molecular screening of these 4 genes in a cohort of Turkish individuals diagnosed with HED/EDA. We screened for pathogenic variants of WNT10A, EDA, EDAR, and EDARADD through Sanger sequencing. We further assessed the clinical profiles of the affected individuals in order to establish phenotype-genotype correlation. In 17 (63%) out of 27 families, 17 pathogenic variants, 8 being novel, were detected in the 4 well-known ectodermal dysplasia genes. EDAR and EDA variants were identified in 6 families each, WNT10A variants in 4, and an EDARADD variant in 1, accounting for 35.3, 35.3, 23.5, and 5.9% of mutation-positive families, respectively. The low mutation detection rate of the cohort and the number of the EDAR pathogenic variants being as high as the EDA ones were the most noteworthy findings which could be attributed to the high consanguinity rate., (© 2019 S. Karger AG, Basel.)

Objective: Corticosteroids have been applied via transtympanic route for a long time to treat the inner ear disorders. A few animal models were used to answer the questions, "How much drug goes into the inner ear?" and "How far does the drug reach through the scala tympani and/or scala vestibuli?" However, the cerebrospinal fluid contamination poses a major problem. The aims of this study were to create a new sampling model showing the dexamethasone distribution in the inner ear and to provide more reliable data about drug concentrations., Methods: Ten Hartley strain albino guinea pigs that weighted between 400 and 600 g were used. After dexamethasone application to the left ear, they were sacrificed at two time points: after 0.5 hours (Exp 1) and after 2 hours (Exp 2). The temporal bones were immediately dissected and put into liquid nitrogen for freezing. The apex, second turn, and basal turns of the cochlea and vestibule were separated, while the bone was in the frozen state. The samples were prepared and measured with ultraviolet (UV) spectroscopy., Results: The total amount of dexamethasone was statistically higher in the left ear than the control ear. Although the basal turn and vestibule were the most prominent parts, there was no statistical difference between the different parts of the inner ear at 0.5 hours. The vestibule and the apex showed the highest level of dexamethasone at 2 hours., Conclusion: Although the model has some limitations, it can measure dexamethasone concentrations and show the time variability in the inner ear., Competing Interests: Conflict of Interest: The authors have no conflicts of interest to declare.

Pharyngitis in most cases is due to viral microorganisms however drug therapy without the detection of etiological agent leads to unnecessary use of antibiotics. On the other hand, when the etiologic agent is group A beta-hemolytic streptococci (GAS) it is important to identify the etiologic agent rapidly which will guide the treatment with appropriate antibiotics. The use of highly sensitive rapid tests will contribute significantly to early diagnosis and appropriate therapy. The aim of this study is to evaluate the efficacy of Mascia Brunelli rapid antigen test for the detection of GAS in throat swab samples. A total of 833 throat swab samples submitted to our laboratory with pre-diagnosis of pharyngitis were assessed between June 2016 and August 2016. The samples were simultaneously cultured and tested by rapid Mascia Brunelli Strep-A Card (Mascia Brunelli S.p.a, Italy). For identification, bacitracin sensitivity, PYR test and latex agglutination test in addition to Bruker MALDI-TOF MS (Daltonics, Germany) system were used. The density of GAS growth in the culture was noted. The samples that were false negative with Mascia Brunelli test were re-tested with QuickVue + Strep A Test (Quidel Corporation, San Diego, USA) rapid antigen test. A total of 833 patients, 376 (45.2%) female and 457 (54.8%) male were included in the study. The age range was between 0-94 years with a mean value of 7.86 ± 6.72. 125 (15%) and 94 (11.28%) of the samples were positive with culture and rapid antigen test, respectively. Mascia Brunelli antigen test gave negative results for 31 culture positive samples. Of these 31 samples, 28 were found positive by QuickVue + Strep A antigen test. As a result, the sensitivity of the test was found to be independent of the inoculum effect. The culture positivity rate in patients between 5-15 years was 18.4%. The sensitivity, specificity, positive predictive value, negative predictive value and the accuracy of Mascia Brunelli antigen test, with respect to culture, were 75.2%, 100%, 100%, 95.81% and 96.28%, respectively. In conclusion, the selection of rapid antigen tests with high sensitivity in the diagnosis of GAS pharyngitis will contribute to the prevention of resistance development by appropriate use of antibiotics as well as early diagnosis and appropriate treatment. However, confirmation of negative rapid antigen test results by culture is very important in terms of false diagnosis and prevention of incomplete treatment.

Objective: The aim of this study was to validate the Turkish version of the 'Medication Regimen Complexity Index' (MRCI)., Methods: This validation study has been conducted in prescriptions of the first 100 elderly patients who had visited the pharmacy for their prescription refill to evaluate convergent and divergent validity of the Turkish version. The reliability of the Turkish version was assessed with inter-rater and test-retest analysis after its translation and cultural adaptation., Results: The mean age of the 100 patients (53 women) was 74.9 years (SD = 7.58, 65-95). The scale showed high inter-rater reliability and test-retest reliability for the total and subscale scores (p < 0.05). A strong and positive correlation between the number of medications in a prescription and the total Medication Regimen Complexity Index scores (r = 0.930, p < 0.001) was determined. There were no statistically significant differences between age, gender and MRCI scores (p > 0.05)., Conclusion: These results show that the Turkish version of MRCI is a reliable and valid tool in elderly patients., (© 2016 John Wiley & Sons, Ltd.)

The objective of the this study was to evaluate the effects of cellular phone radiation on oxidative stress parameters and oxide levels in mouse brain during pentylenetetrazole (PTZ) induced epileptic seizure. Eight weeks old mice were used in the study. Animals were distributed in the following groups: Group I: Control group treated with PTZ, Group II: 15min cellular phone radiation+PTZ treatment+30min cellular phone radiation, Group III: 30min cellular phone radiation+PTZ treatment+30min cellular phone radiation. The RF radiation was produced by a 900MHz cellular phone. Lipid peroxidation, which is the indicator of oxidative stress was quantified by measuring the formation of thiobarbituric acid reactive substances (TBARS). The glutathione (GSH) levels were determined by the Ellman method. Tissue total nitric oxide (NOx) levels were obtained using the Griess assay. Lipid peroxidation and NOx levels of brain tissue increased significantly in group II and III compared to group I. On the contrary, GSH levels were significantly lower in group II and III than group I. However, no statistically significant alterations in any of the endpoints were noted between group II and Group III. Overall, the experimental findings demonstrated that cellular phone radiation may increase the oxidative damage and NOx level during epileptic activity in mouse brain., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Introduction: To evaluate long-term outcome of patients with granulomatous polyangitis (GPA) followed up in a tertiary university hospital., Patients and Methods: We reviewed medical records of 22 patients with GPA diagnosis confirmed by tissue biopsies between 2004 and 2014., Result: The mean time from the onset of symptoms to diagnosis was 7.8 ± 12.3 months [interquartile range (IR)= 4.0]. The most commonly involved organs were the upper respiratory tract (URT) (72.7%), lower respiratory tract (81.8%) and kidneys (72.7%). URT involvement indicated good prognosis (p= 0.046). Survival in the patients with and without URT involvement was 124.6 ± 6.9 months and 59.7 ± 22.9 months, respectively. End-stage renal failure (ESRF) requiring dilaysis and cardiac involvement were associated with mortality (p= 0.022 and p= 0.026, respectively). Of the 12 dialysis-dependent patients at diagnosis, 11 survived > 3 months and seven regained renal function permanently. Dialysis dependency was significantly lower in patients who received plasmapheresis (p= 0.047). Overall mortality rate was 18% (4/22). Mean survival was 55.9 ± 42.8 months (IR= 84.0)., Conclusions: Diagnosis of GPA may be delayed by the nonspecific nature of its symptoms. URT involvement was associated with good prognosis, whereas cardiac involvement and ESRF requiring dialysis were associated with poor outcome. Plasmapheresis may increase the rate of renal recovery in the patients with ESRF requiring dialysis.

Objective: To report the long-term outcomes of children who received auditory brainstem implant (ABI) because of severe inner ear malformations., Study Design: Retrospective chart review., Setting: Tertiary referral otolaryngology clinic., Subjects and Methods: Between July 2006 and October 2014, 60 children received ABI at Hacettepe University. Preoperative work up included otolaryngologic examination, audiological assessment, radiological evaluation together with assessment of language development and psychological status. The surgeries were performed via retrosigmoid approach with a pediatric neurosurgeon. Intraoperatively, electrical auditory brainstem response was utilized. Initial stimulation was done 4 to 5 weeks postoperatively. Outcomes were evaluated with Categories of Auditory Performance (CAP), speech intelligibility rate (SIR), functional auditory performance of cochlear implant (FAPCI) and Manchester Spoken Language Development Scale scores; receptive and expressive language ages were determined., Results: Sixty children who received ABI were between ages of 12 and 64 months. Thirty-five patients with follow up period of at least 1 year, were reported in means of long-term audiological and language results. The most prevelant inner ear malformation was cochlear hypoplasia (n = 19). No major complication was encountered. Majority of the patients were in CAP 5 category, which implies that they can understand common phrases without lip reading. SIR was found out to be better with improving hearing thresholds. Children with ABI were performing worse than average cochlear implantation (CI) users when FAPCI scores were compared. Patients with the best hearing thresholds have expressive vocabulary of 50 to 200 words when evaluated with Manchester Spoken Language Development Scale. There was no relationship between the number of active electrodes and hearing thresholds. The type of inner ear anomaly with the best and the worst hearing thresholds were common cavity and cochlear aperture aplasia, respectively. Patients with additional handicaps had worse outcomes. Among 35 children, 29 had closed set discrimination and 12 developed open set discrimination above 50%. It was determined that, progress of the patients is faster in the initial 2 years when compared with further use of ABI., Conclusion: ABI is an acceptable and effective treatment modality for pediatric population with severe inner ear malformations. Bilateral stimulation together with CI and contralateral ABI should be utilized in suitable cases.

The aims of the study are to demonstrate the effect of probiotic use on the healing of radiation proctitis (RP) and evaluate the efficiency of fecal biomarkers at follow-up of the treatment. Thirty-two male/female rats were randomly separated into four groups of eight rats. The first group (control) was not radiated. RP was created by 17.5 Gy single dose rectal irradiation. The second group (RP) was subjected to RP, but not treated. The third group (RP+ASA) was treated with 5-aminosalicylic acid (5-ASA) 250 mg/kg daily by gastric lavage for 14 days after the irradiation, and the forth group (RP+LGG) was treated with Lactobacillus GG (LGG) 25 × 100 million CFU daily. Feces samples were taken at the 7th and 14th day of the treatment for fecal biomarkers. Rectums of the rats were resected at the 14th day by laparotomy. Samples were evaluated both macroscopically and microscopically. RP was achieved both macroscopically and microscopically. Weight loss of RP group is statistically significant (p < 0.005) than other groups. The healing ratio of RP+ASA and RP+LGG groups was significantly better than the RP group (p < 0.005) both macroscopically and microscopically. But there was no significant difference between ASA and LGG groups. Biochemically, fecal calprotectin was found to be more effective than fecal myeloperoxidase and fecal lactoferrin to show the efficacy of treatment of radiation proctitis. The results of our study demonstrate that probiotic is as effective as 5-aminosalicylic in the treatment of radiation proctitis, and fecal calprotectin is a useful biomarker in determining the response to the treatment.

The aim of this study was to investigate the amount of binaural squelch effect (BSE) and head shadow effect (HSE) in children who use unilateral cochlear implants (CI) and contralateral hearing aids (HA). The study group consisted of 19 CI recipient children who consistently wore a contralateral HA. Speech sounds were used to evaluate speech perception performance in noise. Testing was performed in three listening conditions: (1) bimodal listening with noise source on HA side; (2) CI only with noise source contralaterally (HA off); (3) CI only with noise source on the CI side. Statistical analysis revealed a significant difference between the three listening conditions and post hoc tests indicated significant differences for all pairwise comparisons (p < 0.001). The average BSE and HSE were 11.8% and 17.1% respectively. The majority of bimodal CI users showed BSE and HSE with significant speech perception improvement in the presence of noise.

Objective: The aim of this study is to describe the rehabilitative outcomes of pediatric auditory brainstem implant (ABI) users in the Department of Otolaryngology in the Hacettepe University. It was a retrospective study, and all patients' files were reviewed., Materials and Methods: The data was collected from 41 children who were fitted with ABI between 2005 and 2013. Inclusion criteria for children in our study are profound, congenital bilateral sensory-neural hearing loss with anomalies (such as cochlear, labyrinthine, and cochlear nerve aplasia) and more than one year of auditory experience with ABI. Post-meningitis patients and neurofibromatosis type 2 (NF2) patients were excluded. Auditory perception was evaluated using the Meaningful Auditory Integration Scale (MAIS), Functioning after Pediatric Cochlear Implantation (FAPCI) instrument, Categories of Auditory Performance (CAP), and Children's Auditory Perception Skills Test in Turkish (CIAT). Speech intelligibility was categorized with speech intelligibility rating (SIR), and language development was assessed using the Test of Early Language Development-Third Edition (TELD-3) and Manchester Spoken Language Development Scale (MSLD)., Results: All patients gained basic audiological functions and were able to recognize and discriminate sounds by the third month of ABI surgery. According to the duration of ABI use and learning skills, patients revealed development from word identification to sentence recognition level in a wide spectrum., Conclusion: Preliminary results indicate that all children have gained basic auditory perception skills. On the other hand, language and speech development data were varying among children. Additional handicaps seemed to slow down progression. Secondary improvement was seen at psychosocial areas with respect to behavioral and social adjustment as well as eagerness to start communication.

Sarcoidosis is a multisystem disease characterized by noncaseating granuloma development. Scar sarcoidosis is a rare cutaneous form of sarcoidosis developing on previous cutaneous scar areas. The lesions may be solitary or occur along with systemic disease. We present the case of a female patient that developed cutaneous sarcoidosis in an old scar area on the forehead that was acquired 30 years ago due to injuries from a fall. Histopathological examinations of the excisional scar biopsy revealed non-necrotizing, noncaseating granulomatous inflammatory structures comprised of epithelioid cells and Langhans giant cells with lymphocytic infiltration within the reticular dermis consistent with sarcoidosis. High-resolution CT revealed bilateral mediastinal lymphadenopathy. Patients with inflammatory skin lesions at the sites of preexisting scars should be investigated for sarcoidosis. Histopathological examination of skin biopsy specimens usually provides the correct and final diagnosis.

Aim: We aimed to determine the rate of primary immune deficiency (PID) among children presenting to our immunology outpatient clinic with a history of frequent infections and with warning signs of primary immune deficiency., Material and Methods: The files of 232 children aged between 1 and 18 years with warning signs of primary immune deficiency who were referred to our pediatric immunology outpatient clinic with a complaint of frequent infections were selected and evaluated retrospectively., Results: Thirty-six percent of the subjects were female (n=84) and 64% were male (n=148). PID was found in 72.4% (n=164). The most common diagnosis was selective IgA deficiency (26.3%, n=61). The most common diseases other than primary immune deficiency included reactive airway disease and/or atopy (34.4%, n=22), adenoid vegetation (12.3%, n=8), chronic disease (6.3%, n=4) and periodic fever, aphtous stomatitis and adenopathy (4.6%, n=3). The majortiy of the subjects (90.5%, n=210) presented with a complaint of recurrent upper respiratory tract infection. PID was found in all subjects who had bronchiectasis. The rates of the diagnoses of variable immune deficiency and Bruton agammaglubulinemia (XLA) were found to be significantly higher in the subjects who had lower respiratory tract infection, who were hospitalized because of infection and who had a history of severe infection compared to the subjects who did not have these properties (p<0.05 and p<0.01, respectively). Growth and developmental failure was found with a significantly higher rate in the patients who had a diagnosis of severe combined immune deficiency or hyper IgM compared to the other subjects (p<0.01). No difference was found in the rates of PID between the age groups, but the diagnosis of XLA increased as the age of presentation increased and this was considered an indicator which showed that patients with XLA were being diagnosed in a late period., Conclusions: It was found that the rate of diagnosis was considerably high (72.4%), when the subjects who had frequent infections were selected by the warning signs of PID.

Ankylosis of temporomandibular joint is a condition in which partial or complete immobilization of mandible occurs because of fusion between mandibular condyle and skull base. This condition can be treated orthodontically, surgically, or therapeutically or by prosthodontic rehabilitation. A 10-year-old female patient presented to the Department of Oral and Maxillofacial Surgery, Gazi University Faculty of Dentistry, with limited mouth opening. She got injury in the face when she was 5 years old. Extraoral and intraoral examination findings were facial asymmetry on the left side, micrognathic mandible, and 19-mm mouth opening. After radiographic examination, ankylosis (Shawney type I) on the left side was revealed, and the patient was referred to Department of Orthodontics. After orthodontic clinical examination, we create following multidisciplinary treatment approach: (1) acrylic posterior bite block with spring application, (2) interpositional arthroplasty operation, and (3) physiotherapy (passive mouth-opening exercises). After the follow-up of 9 months, significant improvement (5 mm) was noticed in the opening of the mouth, and we decided to remove appliance and operate on the patient. Surgical procedure was performed under general anesthesia via blinded nasotracheal intubation. To prevent postoperative relapse, temporal fascia was interpositioned and sutured. Passive mouth-opening exercises were started 10 days after the surgery. Thirty-one-millimeter mouth opening was reached after the surgery and passive mouth-opening exercises. Patient's routine controls have been continued for 2 years.

Appendiceal mucocele is a rare clinical condition that results from distension of the appendix lumen with mucus. It is only found in 0.1-0.3% of all appendectomy specimens. Twenty-five per cent of these cases are asymptomatic and are incidentally discovered either during surgery or upon radiological examination. The treatment of appendiceal mucocele is surgical, and while appendectomy is usually sufficient in some cases right hemicolectomy may be considered as a treatment option. Mucoceles are histologically subdivided into four types: retention cysts, mucosal hyperplasia, cystadenomas and cystadenocarcinomas. Herein, we present a case of appendiceal mucocele clinically mimicking urolithiasis in a 62-year-old female patient with a complaint of colicky right flank pain and a single episode of macroscopic hematuria. The patient's abdominal ultrasonography and abdominal CT scan showed a mass consistent with mucocele in the right lower quadrant of the abdomen. Colonoscopy gave the impression of a mass lesion exerting pressure from the outside. The patient was electively operated. Histopathological diagnosis was reported as mucinous cystadenoma. Appendiceal mucocele or mucinous cystadenomas are usually seen in patients undergoing surgery with a diagnosis of appendicitis but, as in our case, these lesions may rarely occur with a clinical picture that mimics urological disease.

Behçet's disease (BD) is a chronic multi-system disease presenting with recurrent oral and genital ulceration, and relapsing uveitis. Heart rate recovery (HRR) after exercise is a marker of parasympathetic activity. A delayed recovery of systolic blood pressure (SBP) after exercise might reflect sympathetic hyperactivity. The analysis of variations in heart rate has also been used to determine the balance between sympathetic and vagal nerve activities in the heart. Our objective was to determine HRR, the SBP response to exercise and heart rate variability (HRV) in patients with BD in the absence of neurological involvement. The study population consisted of 32 patients with BD and 30 healthy controls who were matched with respect to age, sex, and physical activity. Heart rate recovery was calculated as the difference between heart rate at peak exercise and heart rate at 1, 2, and 3 min of recovery. Blood pressure recovery indexes were determined by dividing the systolic blood pressure at 2 and 3 min in recovery to the systolic blood pressure at peak exercise. In patients with BD, mean HRR at 1 min (HRR1) were not significantly different than that of controls (21 ± 7 vs 20 ± 7 bpm, p = 0.50). Although, resting mean SBP of patients with BD was higher than controls (121 ± 13 vs 115 ± 12 mmHg, p = 0.039), the SBP recovery indices of the patients with BD at 2 and 3 min were similar to those of controls (0.84 ± 0.07 vs 0.84 ± 0.09, p = 0.89 and 0.78 ± 0.09 vs 0.78 ± 0.08, p = 0.93, respectively). Both time domain and frequency domain parameters of patients with BD were similar to that of controls. This study shows that the patients with BD have normal HRR1 and normal SBP response to exercise and normal HRV. These findings might suggest unaltered autonomic neural control of the cardiovascular system in this disorder in the absence of neurological involvement.

Laparoscopic splenectomy became the gold standard recently for the removal of spleen. One of its rare indication is wandering spleen (WS). WS is characterized by incomplete fixation of the spleen. Patients usually have an asymptomatic abdominal mass. When they are symptomatic, abdominal pain with a mobile mass is the major finding. Laboratory data are nonspecific, but the diagnosis can be confirmed by imaging studies; computed tomography, magnetic resonance imaging, and ultrasonography are preferred modalities. It can predispose one to life-threatening complications such as splenic infarction, portal hypertension, pancretitis, and hemorrhage, due to torsion of its vascular pedicle. Herein, we present a female patient, a 24-year-old, 18-week primigravida. She was admitted with an intra-abdominal mass and lower abdominal pain. Ultrasonography revealed a mass behind the uterus. Magnetic resonance imaging was planned, and the spleen was seen at this area. We performed laparoscopic splenectomy for the treatment of a WS, which would be dangerous for pregnancy. The patient was discharged without any postoperative complication on the third day postoperatively. A healthy baby was delivered at term. Minimally invasive procedures should also be the primary choice in the treatment of such pregnant women. This is the first case of laparoscopic splenectomy in a pregnant woman for WS in the English literature.

Doppler-guided hemorrhoidal artery ligation (DGHAL) is a nonexcisional surgical technique for the treatment of hemorrhoidal disease, consisting of the ligation of the distal branches of the superior rectal artery, resulting in a reduction of blood flow and decongestion of hemorrhoidal plexus resulting in fibrosis. The aim of the study was to assess the efficacy and safety of DGHAL, define its indications, and identify its possible advantages and limitations for the treatment of second- and third-degree hemorrhoids. The procedure was performed using a specially designed proctoscope. The Doppler probe was used to locate all the terminal branches of hemorrhoidal arteries, which were then sutured. Patients were followed up for 2 years. From November 2006 to May 2009, 50 patients (29 female, mean age 38.2 years) underwent this procedure. The procedure was performed under local anesthesia. An average of five ligatures was placed. Average length of hospital stay was 2 hours and return to work was 2.5 days. The mean postoperative pain score was 1.72. There were no intra- or immediate postoperative major complications. In 44 patients (88%), surgery resolved the symptoms completely in a 2-year follow-up period. DGHAL is a safe and effective procedure. DGHAL can be the choice for second- and third-degree hemorrhoids with minimal postoperative pain and quick recovery.

Objectives: We aimed to assess the sedative effects of midazolam and dexmedetomidine and their effects on hemodynamics and the cardiovascular system under epidural anesthesia., Methods: This study included 50 patients. Approval of the ethics committee and written consent from patients were obtained. The patients were separated into two groups to receive dexmedetomidine (D group) or midazolam (M group). Perifix no. 18 was placed in the epidural space from the L(3-4) interspace. After lidocaine 60 mg/3 ml was applied, isobaric bupivacaine 0.5% was given as 1 ml per segment. After block reached the T10 level, midazolam was given to the M group as a bolus of 0.015 mg kg(-1) in 10 minutes until beginning the operation, followed by continuous infusion as 0.1-0.2 mg kg(-1)h(-1) dosage. Dexmedetomidine 1 mcq kg(-1) was given to the D group as a bolus dosage in 10 minutes until beginning the operation, followed by continuous infusion as 0.4-0.7 mcq kg(-1) h(-1) dosage. Systolic arterial pressure (SAP), diastolic arterial pressure (DAP), mean arterial pressure (MAP), heart rate (HR), SpO(2), Ramsay Sedation Score (RSS), bispectral index (BIS), and respiratory rate (RR) were recorded. Sedative infusion was stopped when skin suture was closed. Adverse effects were also recorded., Results: In group D, MAP was significantly higher and HR was significantly lower than in group M (p<0.05)., Conclusion: We concluded that both drugs provided good sedation with no respiratory depression, stable hemodynamics and alertness with good cooperation. However, we consider midazolam as the first option due to its cost benefit.

Although airbags reduce the overall risk of injury and death from motor vehicle accidents, the airbag may cause injuries during deployment. We present a case of apparently isolated sternal fracture caused by airbag deployment during a motor vehicle crash and we discuss the ultrasonographic diagnosis. We also examine the mechanism of injury caused by the airbag.

The nature of a tube thoracostomy -a blind maneuver- renders it subject to complications. Nevertheless, it is very uncommon to create a diaphragmatic hernia with this procedure. Herein, we present the occurrence of this complication after six months under emergency conditions that was treated by thoracotomy.

Aim: Head trauma is associated with an acute phase response which is characterized by leucocytosis due to increased levels of catecholamine and cortisol. Early edema formation following severe head injury may also be associated with microglia activation. Therefore, increased white blood cell (WBC) count after head trauma may be a predictive parameter of the severity of craniocerebral trauma., Material and Methods: We retrospectively studied 59 patients with severe, moderate and minor injury between February 2007 and March 2009. WBC counts of all patients were obtained within the first day of their admission. All patients were divided into separate groups according to their Glasgow Coma Scale (GCS) scores and hospital stay durations. Their CT progressions and Glasgow Outcome Scale (GOS) scores were also assessed. The relationship between these parameters and WBC counts were evaluated by statistical methods., Results: There was a favorable correlation between WBC counts of the patients and GCS scores (pI0.01), hospital stay (p=0.006), CT progression (p < 0.01) and GOS scores (p < 0.01)., Conclusion: AWBC count exceeding 17.5 x 106/l has a predictive value for poor GCS score, and long hospital stay. CT progression tends to be seen in patients with moderate and severe head injury.

Surgical treatment is extremely difficult with the combined defects of skin, cartilage, and nasal mucosa. Besides efforts geared toward ascertaining the best aesthetic outcome, an important concern is restoring normal nasal function. This can be achieved only by providing sufficiently and anatomically adapted cartilage and bone support, followed by covering the inner part using tissue closely resembling mucosa and the outer part using skin compatible with the surrounding skin. The surgical technique for three-dimensional nasal reconstruction in the first session of this study involved placing a silicon sheet between the skin and galea, which allowed two separate flaps to be obtained for the next session without vascular damage. For the epithelialization of the defect on the nasal surface, the lower surface of the galea was prefabricated with a thin skin graft obtained from the thigh. In this way, nasal mucosa cover was ensured. The expander placed under all these structures thinned them down to a thickness close to that of nasal skin and mucosa and also enabled primary closure of the donor area. Thus, the defect that emerged during the second session in cartilage framework was repaired by cartilage grafts taken from the nasal septum. The mucosal surface and skin part then could be closed with two separate flaps. The forehead flap used in this technique enabled production of an aesthetically and functionally satisfactory outcome by providing an anatomically sufficient amount of nasal skin and nasal mucosa for whole-layer wide nasal defects in only three sessions without necessitating an additional flap.

Teratomas, the most common extragonadal germ cell tumor of childhood, involve at least two of the ectodermal, mesodermal, and endodermal layers. Of the teratomas seen in the first 2 months of life, 82% are sacrococcygeal. The head and neck region is the second most common location for teratomas in early infancy, accounting for five (14%) of those cases. We describe a female neonate with a teratoma of the nasopharyngeal area, bilateral cleft palate/lip, and columellar sinus pathologies. The mass, which was 8 x 5 x 7 cm and soft in consistency, blocked the airway and prevented oral feeding. On macroscopic examination of the excised mass, there was a notable typical cilia arrangement and lower eyelid appearance. The patient, who was diagnosed with a well-differentiated teratoma after the pathologic examination, did not have any complications in the postoperative period.

Warfarin sodium is a commonly used oral anticoagulant agent. It has been well documented that, when effective anticoagulant therapy is employed in treating thromboembolic disease, hemorrhage is a possible complication that can be spontaneous without a history of trauma. The numerous sites of bleeding are the genitourinary and gastrointestinal tracts, the central nervous system, the nose (epistaxis), the penis (priapism), the retroperitoneum, wounds (surgical or traumatic), and subcutaneous tissues during warfarin therapy, but the hemorrhage rarely causes bleeding compromising a patient's airway. We report a case of a spontaneous lingual hematoma that developed during oral anticoagulation therapy. This life-threatening complication of warfarin therapy and its successful management without surgery indicates that observation, close monitoring and reversal of anticoagulation can be a reasonable management option.

Transverse myelitis is a group of disorders characterized by focal inflammation of the spinal cord and results in loss of motor and sensory function below the level of injury. Occurrence of this condition during or following varicella infection is uncommon. This report describes two cases of transverse myelitis caused by varicella zoster.