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Background MicroRNAs are small, endogenous, non-coding RNAs that regulate the expression of many genes. It has recently been shown that circulating microRNAs may be biomarkers of hypertrophy and fibrosis in patients with hypertrophic cardiomyopathy (HCM). Objective To determine whether circulating levels of microRNAs involved in HCM are associated with electrocardiographic and echocardiographic parameters. Methods This study enrolled 20 patients with familial HCM and 20 blood donors. Peripheral serum levels of miR-29a-3p, miR-199a-5p and miR-451a were assessed by quantitative real-time polymerase chain reaction and compared with levels in the control group. Whether circulating levels of miRNAs in HCM patients correlated with electrocardiographic and echocardiographic parameters was also assessed. Results Median circulating levels of miR-29a and miR-451a were significantly higher in HCM than the control group. Median miR-199a levels did not differ between groups. However, circulating levels of miR-199a negatively correlated with corrected QT duration (Bazett formula). Median miR-29a levels positively correlated with QRS duration. In addition, circulating levels of miR-29a correlated with maximal wall thickness, left ventricular mass index and left atrial volume index. Conclusions The data suggested that serum levels of miR-29a and miR-451a were significantly increased in HCM patients. As the circulating level of miR-29a correlated with QRS duration, left ventricular hypertrophy and left atrial dilatation, the serum miR-199a level negatively correlated with corrected QT duration. These miRNAs may be seen as potential biomarkers for further research in HCM pathophysiology.

Synthesis of 1,8-naphthalimide derivatives and biochemical studies using them as anti-cancer and cellular imaging agents have attracted significant attention in recent years. This study aimed to synthesize two new fluorescent lower rim-functionalized 4-sulfo-1,8-naphthalimide derivatives of calix[4]arenes [4 and 7] and examination of their cytotoxic properties for cancerous cells. For this purpose, p-tert-butylcalix[4]arene bearing dihydrazide [3] or diaminopropyl [6] units on its lower rim were reacted with 4-sulfo-1,8-naphthalic anhydrate. The structure of these synthesized compounds has been characterized using 1H-NMR, 13C-NMR, and FT-IR techniques. To investigate the anti-cancer properties, compounds 4, 7, and N,N-dimethylaminoethyl-1,8-naphthalimide [8] were tested on the human colorectal cancer cell line (DLD-1) as well as healthy colon epithelial cell line (CCD-18Co). The IC50 values of 4 and 7 were determined as 12.95 µM and 16.13 µM, respectively, on DLD-1 cells. Furthermore, following the treatment of CCD-18Co cells with 4 and 7, the IC50 values were determined as 508 µM and 269 µM, respectively. However, it was observed that compound 8 had no cytotoxic effect on both DLD-1 cells and CCD-18Co cells. Furthermore, the fluorescence imaging of human colorectal cancer cells treated with the synthesized compounds [4, 7, and 8] was performed on living cells.

Background: This study aims to investigate the effects of 2-aminoethoxydiphenyl borate (2-APB) on aortic clamping-induced lung and kidney tissue oxidation, tissue inflammation, and histological damage in a rat model. Methods: A total of 28 adult female Wistar albino rats were randomly allocated to four equal groups: Control group, ischemia-reperfusion group, dimethyl sulfoxide group, and 2-APB group. Animals in the control group underwent median laparotomy. In the remaining groups, supra-celiac aorta was clamped for 45 min and, then, reperfusion was constituted for 60 min. The 2-APB (2 mg/kg) was administered before clamping. The remaining groups received saline (ischemia-reperfusion group) or dimethyl sulfoxide (dimethyl sulfoxide group). Kidney and lung tissue samples were harvested at the end of reperfusion. Results: Aortic occlusion caused increased tissue total oxidant status and reduced total antioxidant status and glutathione levels in the ischemia-reperfusion and dimethyl sulfoxide groups. Tissue interleukin-1 beta and tumor necrosis factor-alpha levels, nuclear factor kappa beta activation, and histological damage severity scores were also higher in these groups. The 2-APB treatment eliminated the increase in total oxidant status and the decrease in total antioxidant status and glutathione levels. It also caused a decrease in the interleukin-1 beta levels, although it did not significantly alter the tumor necrosis factor-alpha levels, nuclear factor kappa beta immunoreactivity, and histological damage scores. Conclusion: Borate exerted a beneficial antioxidant effect as evidenced by reduced oxidative stress; however, it did not inhibit nuclear factor kappa beta activation and prevent histological damage in supra-celiac aortic clamping-induced kidney and lung injury in rats.

Objective Scabies is caused by an ectoparasite called Sarcoptes scabiei (S. scabiei), which penetrates the epidermis through skin folds and burrows in the stratum corneum, following the development of tunnels (sillion). The disease is specifically characterised by keratosis, allergy and itching that increases at night-time. This study aimed to investigate the frequency of S. scabiei in patients with a pro-diagnosis of scabies. Objective Between January 2012 and December 2019, a total of 746 [n=388 (52%), female; n=358 (48%) male] patients aged 0-80 years were admitted to Firat University Hospital Parasitology-mycology Laboratory. Skin scrapings were taken from suspected lesions on anatomic regions such as the hands (wrist, interdigital skin, fingertip and palm), abdomen, penis and legs (thigh and bottom foot). They were examined under a light microscope after adding 15% potassium hydroxide solution. Results S. scabiei was positive in 139 (18.63%) of 746 patients including a mother and her daughter and a married couple, where 68 (9.11%) were female and 71 (9.52%) were male. Conclusion To our best knowledge, this is the first comprehensive study of scabies in Elazig. Despite the recent socio-economic and cultural developments observed in our country, scabies and all other parasitic infestations still remain to be important problems. We believe that improvement of the public vigilance together with early diagnosis will improve sanitation and provide protection against scabies and parasitic infestations.

Objective: Ubiquitin C-terminal Hydrolase-L1 (UCH-L1) enzyme levels were investigated in patients with epilepsy, epileptic seizure, remission period, and healthy individuals. Methods: Three main groups were evaluated, including epileptic seizure, patients with epilepsy in the non-seizure period, and healthy volunteers. The patients having a seizure in the Emergency department or brought by a postictal confusion were included in the epileptic attack group. The patients having a seizure attack or presenting to the Neurology outpatient department for follow up were included in the non-seizure (remission period) group. Results: The UCH-L1 enzyme levels of 160 patients with epilepsy (80 patients with epileptic attack and 80 patients with epilepsy in the non-seizure period) and 100 healthy volunteers were compared. Whereas the UCH-L1 enzyme levels were 8.30 (IQR=6.57‒11.40) ng/mL in all patients with epilepsy, they were detected as 3.90 (IQR=3.31‒7.22) ng/mL in healthy volunteers, and significantly increased in numbers for those with epilepsy (p

Objective: Extracorporeal membrane oxygenation (ECMO) is a temporary life-support modality offered for stabilizing neonates with congenital diaphragmatic hernia (CDH) who are in a critical condition and unresponsive to optimal medical therapy. The aim of this study was to examine our institutional outcomes of early CDH repair on ECMO. Materials and Methods: A total of 17 ECMO-supported patients with CDH were evaluated and the demographic, diagnostic, laboratory, clinical data, complications and outcome of the patients were reported. Results: The study consisted of 7 females and 10 males. Mean birth weight (BW) was 3107 g (range, 2360–3840 g). Median age of ECMO initiation was 2.1 days. In total four patients received VV ECMO. Other patients received VA ECMO via aortic and right atrial cannulation. The mean duration of ECMO was 25 days (range: 1-140 days). Six patients (35.2%) could be weaned from ECMO. The most common ECMO related complications were hemorrhage, disseminated intravascular coagulation (DIC) and limb ischemia (64.7%, 41.1% and 29.4%, respectively). Diaphragmatic defect was repaired via a subcostal incision in 12 of 13 patients. In 88.2% of patients diaphragmatic repair was performed with polytetrafluoroethylene patch. In 77% of patients early CDH repair was performed concomitantly with ECMO insertion. The survival rate was 17.6%. Conclusion: Early repair of CDH in neonates on ECMO can be associated with high rates of morbidity and mortality. However, ECMO may be considered a life-saving measure for patients with CDH who would have otherwise not been salvageable.

Familial Mediterranean Fever (FMF) is the most common hereditary autoinflammatory disorder characterized by recurrent fever and serositis episodes. Identification of low penetrant or heterozygous MEFV mutations in clinically diagnosed FMF patients did raise a concern on whether epigenetic or environmental factors play an additional role in FMF pathogenesis. We aimed to investigate the expression profile of apoptosis-related miRNAs in FMF and their influence on clinical manifestations in the present study. 191 pediatric FMF patients and 31 healthy children included in the study. Expressions of 33 apoptosis-related, circulating cell-free miRNAs were evaluated by a quantitative polymerase chain reaction, statistically calculated within ΔΔCt values and fold changes were evaluated by Welch T test, in which p

Juvenile dermatomyositis is a multisystemic autoimmune disease with uncertain etiology. Both innate immunity and adaptive immunity play a role on the pathogenesis of the disease. Selective immunoglobulin A deficiency is the most common primary immunodeficiency. Association between immunoglobulin A deficiency and autoimmune diseases including few juvenile dermatomyositis patients have been reported. A previously healthy 15-year old girl was diagnosed with juvenile dermatomyositis according to Bohan and Peter criteria and selective immunoglobulin A deficiency due to the low level of immunoglobulin A (

PubMedID: 31662011 Objectives: Henoch Schönlein Purpura (HSP) is the most common systemic vasculitis in childhood. We aimed to evaluate the clinical features, seasonal variation, treatment outcomes and the possible predicting factors related to outcome among a large cohort of pediatric HSP patients. Methods: We conducted a medical record review study between July 2016 and January 2019 and evaluated the clinical manifestations and potential risk factors for severe gastrointestinal (GI) involvement, biopsy-proven nephritis and relapses. Results: The study included 420 HSP patients, of which the mean age at diagnosis was 7.68 ± 3.15 years. Clinical manifestations were arthralgia and/or arthritis (n = 244, 58.1%), abdominal pain (n = 235, 56%), subcutaneous edema (n = 163, 38.8%), and renal involvement (n = 125, 29.8%). Disease recurred for at least once, in 69 (16.4%) patients and colchicine treatment yielded a favorable response in 11 of 12 relapsing patients, who did not respond to ibuprofen or steroids. Frequencies of renal involvement and biopsy-proven nephritis were higher in patients with severe GI involvement. Besides, patients with biopsy-proven nephritis had higher rates of abdominal pain, intussusception, severe GI involvement, and systemic steroid administration. Conclusion: We speculate that renal involvement, biopsy-proven nephritis and severe GI involvement can be related to each other. Colchicine may be effective in patients with relapsing disease. © 2019, © 2019 Japan College of Rheumatology.

Takayasu arteritis (TA) is classified as a large vessel vasculitis of predominantly aorta and its main branches, resulting in fibrosis and stenosis. Only a minority of TA patients are diagnosed in pre-stenosis phase when constitutional symptoms including fever, arthralgia, weight loss, headache, abdominal pain, and elevated acute phase reactants are dominant insidious characteristics. In this article, we present a 12-year-old female patient, who was referred to our department with a one-year history of low-grade fever, fatigue, and myalgia. Physical examination did not reveal pulse and blood pressure discrepancies between any extremities. Acute phase reactants were markedly elevated, and autoantibodies were negative. Magnetic resonance angiography (MRA) findings have confirmed TA diagnosis with prominent vessel wall thickening in the ascendant and abdominal aorta, focal ectasias and a thoracoabdominal fusiform aneurysm. As methotrexate and methylprednisolone treatment during three months was unsuccessful, infliximab was induced. During the next 12 months, patient had clinical improvement, but worsening of MRA findings and new onset of carotidynia forced us to switch methotrexate to mycophenolate mofetil. Six months later, laboratory and radiological remission were achieved. In conclusion, we report a challenge to diagnose pre-pulseless childhood-TA (c-TA) in the state of prolonged fever with no signs of vascular stenosis, systemic hypertension, pulses and blood pressure discrepancies, bruits and claudication. Therefore, we wish to discourse the importance of early diagnosis of TA since, to our knowledge, there are no studies investigating treatment success only in the early phases of c-TA.

Introduction and Objectives Vitamin D deficiency is associated with increased susceptibility to infections and wheezing. We aimed to evaluate the relation between vitamin D levels, viral infections and severity of attacks in children with recurrent wheezing. Materials and methods A total of 52 patients who applied with wheezing, at the ages of 12–60 months with a history of three or more wheezing attacks in the last year and 54 healthy children were included. Sociodemographic data, risk factors for recurrent wheezing, and the severity of the wheezing attacks were recorded. 25(OH)D3, calcium, phosphor, alkaline phosphatase and parathormone levels of all children were measured. Nasopharyngeal samples of the patients for viruses were studied by multiplex polymerase chain reaction. Results For the patient group, being breastfed for six months or less, history of cesarean section, cigarette exposure, humid home environment, and family history of allergic disease were significantly higher compared with the control group. Serum vitamin D levels in the patient group were significantly lower compared to the control group. There was no significant relationship between vitamin D levels and hospitalization, oxygen or steroid therapy. Virus was detected in 38 patients (73%). Rhinovirus (63.2%) was the most frequently detected virus. Coinfection was found in 14 (36.8%) patients. There was no statistically significant difference between detection of virus and vitamin D levels. Conclusions Cigarette exposure, being breastfed six months or less, humid home environment, history of cesarean section, family history of allergic disease and vitamin D deficiency might be risk factors for recurrent wheezing.

Objectives: This study aims to analyze the growth parameters in children with familial Mediterranean fever (FMF) according to disease characteristics including genotype and disease severity by a recently validated tool in relatively more patients. Patients and methods: This retrospective study included 126 patients with FMF (70 males, 56 females; mean age 7.3±3.6 years; range, 4.1 to 18 years). MEditerranean FeVer (MEFV) gene analysis was performed with a molecular diagnostics tool by using a next-generation sequencing platform. Disease severity was determined for the first visit by the validated tool in children, international severity scoring system for FMF. Growth parameters including weight and height were investigated after standard deviation (SD) scores were calculated by anthropometric references in Turkish children. Results: Median follow-up duration was 74.7 months (range, 7.5 to 169 months). Ninety-three patients (73.8%) had at least one M694V mutation in MEFV gene. Six patients (4.8%) had severe disease, 58 (46%) had intermediate severity, and 62 (49.2%) had mild disease. Mean height SD score was significantly lower at last visit than before colchicine treatment. Initial and last height and weight SD scores were lower in patients with at least one M694V mutation than those without. However, the difference was statistically significant for only initial height SD score. We also found statistically significant lower initial height, final height, and weight SD scores in patients with intermediate severity-severe disease activity than mild disease. Conclusion: We advise physicians to score disease severity prospectively and pay attention to patients with intermediate severity-severe disease to avoid growth disturbances. © 2019 Turkish League Against Rheumatism.

maç: Bu çalışmanın amacı kardiyoloji polikliniğine çarpıntı veya göğüs ağrısı yakınmasıyla başvuran 18-25 yaş aralığındaki bireylerde kalp hastalığı varlığı ve yokluğuna göre anksiyete ve depresyon düzeylerini karşılaştırmaktır.Gereç ve Yöntem: Başkent Üniversitesi Adana Dr. Turgut Noyan Araştırma ve Uygulama Merkezi Kardiyoloji Polikliniğine başvuran 18-25 yaş aralığındaki bireyler değerlendirilmiştir. Ellidört erkek ve 60 kadın olmak üzere toplam 114 kişi çalışmaya alınmıştır. Hastaneye başvuranların yanında refakatçi olarak gelen 18-25 yaş aralığındaki sağlıklı 44 kişi kontrol grubunu oluşturmuştur. Veri toplama aracı olarak Beck Depresyon Ölçeği (BDÖ) ve Beck Anksiyete Ölçeği (BAÖ) kullanılmıştır. Bulgular: Vaka grubunda 77 kişide herhangi bir kalp hastalığı saptanmamıştır. Otuz yedi kişiye bir kalp hastalığı tanısı konmuştur. Gruplar BDÖ ve BAÖ puanları açısından karşılaştırılmıştır. Göğüs ağrısı veya çarpıntı yakınması ile başvuran kalp hastalığı olan ve kalp hastalığı olmayan gruplardaki BDÖ ve BAÖ puanları yakınması olmayan kontrol grubuna göre anlamlı derecede daha yüksek saptanmıştır. Sonuç: Kardiyoloji polikliniğine göğüs ağrısı veya çarpıntı yakınmasıyla başvuran 18-25 yaş aralığındaki bireylerde kalp hastalığı varlığından bağımsız olarak depresyon ve anksiyete düzeyi puanları kontrol grubundan anlamlı olarak yüksek bulunmuştur., Purpose: The aim of this study is to compare the levels of anxiety and depression according to the presence and absence of heart disease in individuals aged 18-25 years who apply to the Cardiology Outpatient Clinic for palpitations or chest pain.Materials and Methods: Persons who 18-25 years old applied to Başkent University Adana Dr. Turgut Noyan Research and Application Center Cardiology Department for palpitaion or chest pain were taken into the study. A total of 114 people, including 55 males and 60 females, were included in the study. Fourty-four people those in the same ages who came as a companion were taken to the control group. Participants were asked to fill in the Beck Anxiety Inventory (BAI) and Beck Depression Inventory (BDI) by themselves. Results: In the case group, 77 patients did not have any heart disease. Thirty-seven people were diagnosed with a heart disease. The BAI and BDI scores of the groups were compared. The BDI and BAI scores of the groups who presented with chest pain and palpitation were significantly higher than the control group. Conclusion: The anxiety and depression symptom levels of the patients who were diagnosed and not diagnosed with cardiac disease in 18-25 year olds who applied to the cardiology policlinic were more than the control group.

PubMedID: 31522599 Deficiency of Adenosine Deaminase 2 (DADA2) is a monogenic autoinflammatory disorder characterized by livedo reticularis, skin ulcers, subcutaneous rash, aphthous ulcers, and leukocytoclastic vasculitis, neurological signs such as early onset stroke and polyneuropathy. A minority of DADA2 patients suffer from severe cytopenia and lymphoproliferation. Herein, we report an adolescent patient, followed up as having a hematological disorder for many years, eventually diagnosed as having DADA2. In view of the presence of elevated acute phase reactants, hepatosplenomegaly, low IgM level, lymphopenia, anemia, and neutropenia, and a subtle neurological involvement we considered DADA2 diagnosis. The diagnosis was confirmed by identification of a novel L451W mutation in CECR1 gene. The patient has been successfully treated with etanercept, monthly intravenous immunoglobulin replacement, and low-dose methylprednisolone. In conclusion, although the absence of skin and neurological findings, low IgM levels, and persistent lymphopenia should lead the physicians to consider DADA2 in patients with particularly complicated hematological abnormalities. © 2019, © 2019 Taylor & Francis Group, LLC.

Introduction:The endothelial damage, changes in vascular permeability and plaque formation are caused by the effects of cytokines and chemokines that plays role in chronic inflammation in rheumatoid arthritis (RA) and ankylosing spondylitis (AS). It has been reported that arterial stiffness and the level of cell adhesion molecules are affected by inflammation. In this study, we aimed to determine the relationship between vascular endothelial growth factor (VEGF), E-selectin, arterial stiffness and disease activity in patients with rheumatic diseases.Methods:Thirteen patients diagnosed with AS, 28 patients diagnosed with RA and 30 healthy controls were included in the study. Arterial stiffness, VEGF, E-selectin, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were evaluated in all patients and healthy controls. Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and Disease Activity Score-28 (DAS-28) were calculated in patients with rheumatic diseases. The level of serum VEGF and E-selectin were determined by ELISA, and arterial stiffness was measured by oscillometric method. The data were statistically evaluated by using Student t-test, Mann-Whitney U Test and Wilcoxon test.Results:DAS-28, BASDAI, ESR and CRP levels were significantly decreased on the 3rd month of treatment in all patients (p

Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by malar rash, oral ulcers, arthralgia, photosensitivity and nephritis. Herein, we report a rare comorbidity, multiple avascular necrosis (AVN), in an adolescent SLE patient and also highlight the importance of risk factors for this comorbidity with a brief literature review. A 13-year-old female patient was admitted with severe headache, visual plus auditory hallucinations, polyarthritis and a history of recurrent oral ulcers. Acneiform malar rash, arthritis, cytopenia, low complement levels and autoantibody positivity yielded SLE diagnosis. We diagnosed her as having multifocal AVN after the 4th dose of cyclophosphamide, with bilateral knee pain and swelling and typical geographical lesions on magnetic resonance imaging. Avascular necrosis is a rare comorbidity of SLE and neuropsychiatric involvement, cyclophosphamide administration and severe disease may be the possible risk factors in addition to corticosteroid use. Further multicenter studies investigating the possible risk factors of AVN with a large number of patients are needed. © 2019 Termedia Publishing House Ltd.. All rights reserved.

Objective: Hypericum perforatum is widely used for depression and distress treatment as an over-the-counter plant at any age. This study investigated the safety of H. perforatum on ovarian function and infertility. Material and Methods: H. perforatum was given to rats in two different dosages (100 and 300 mg/kg/day) with drinking water for four weeks. Half of the treatment groups were sacrificed at the end of the four-week intervention, the remainder was sacrificed after an additional four-week waiting period to see if there was reversibility. At the end of the experiment, blood samples and both ovarian tissues were obtained under anesthesia with ketamine and xylazine (50 mg/kg and 5 mg/kg, respectively). Results: Although primordial follicle numbers were not affected with a dose of 100 mg/kg, they were significantly decreased (28.6%) when the dose was tripled. Primary follicle numbers stayed the same, but secondary and tertiary follicles numbers were significantly dose-dependently decreased, and remained significantly low four weeks after the intervention. Anti-mullerian hormone (AMH) levels were not significantly different between the groups. Conclusion: H. perforatum treatment did not change serum levels of AMH because the primary follicle number did not decrease. However, the other follicle counts decreased in a dose-dependent manner and full recovery was not regained after four weeks. The detrimental effect of H. perforatum on primordial follicles should be taken into consideration because any woman using H. perforatum could also experience ovarian failure.

Aim: Severe biotin deficiency associated with biotinidase enzyme deficiency in newborns is seen as severe neurological problems and hearing loss. However, the effect on the infant of deficiencies i...

Background/aims Most important cause of mortality in chronic obstructive pulmonary disease (COPD) patients is known to be cardiovascular disease (CVD). The objective of the present study was to evaluate the echocardiographic parameters in COPD patients with or without pre-diagnosed CVD and to investigate the relationship between echocardiographic parameters and systemic inflammation markers. Methods A total of 60 stable COPD patients (23 patients with CVD, group 1; 37 patients without CVD, group 2) and 21 healthy controls (group 3) were included in the study. Six-minute walking test (6MWT), COPD assessment test (CAT), and Body mass index, airflow Obstruction, Dyspnea, and Exercise (BODE) index results were recorded. High-sensitivity C-reactive protein (HsCRP), interleukin 8 (IL-8), fetuin-A, Clara cell protein (CCL-16), N-terminal pro-brain natriuretic peptide levels were studied in serum. Parameters of left and right ventricular systolic and diastolic function were measured by echocardiography. Results Patients with COPD had higher levels of systemic inflammation markers and lower level of inflammation inhibitor fetuin-A. When three groups were compared, group 1 had lower 6MWT result. HsCRP was highest in group 2 while other inflammatory markers were similar in groups 1 and 2. Regarding echocardiographic parameters, left ventricular ejection fraction (LVEF) was lower and left ventricle end-diastolic diameter (LVED), left ventricle end-systolic diameter (LVES) diameters were higher in group 1. The aortic diameter was higher in COPD patients. Fetuin-A was correlated with diameter of aorta and LVES. LVEF, LVED, and LVES were found to be correlated with functional parameters of COPD cases. Conclusion In COPD, left ventricular functions are affected as well as right ventricle before prominent clinical findings of cardiac disease and these echocardiographic parameters correlate with functional parameters of COPD patients.

Inactivating autosomal recessive mutations in fibroblast growth factor 23 (FGF23), klotho (KL) and polypeptide N-acetylgalactosaminotransferase 3 (GALNT3) genes lead to a rare disorder, hyperphosphatemic familial tumoral calcinosis (HFTC). Patients with HFTC present with hyperphosphatemia and tumor like soft tissue calcifications. Although 78% of patients develop their first symptoms between the ages of 2-13 years, diagnosis is usually delayed until adulthood. Some individuals with the same genetic defect develop a condition named hyperphosphatemic hyperostosis syndrome. Herein we report two siblings suffering from periarticular, warm, hard and tender subcutaneous masses. Subcutaneous calcifications were present on X-ray and biopsy results were consistent with calcinosis in both patients. Laboratory results showed marked hyperphosphatemia and elevated renal tubular phosphate reabsorption rates, normal renal function tests and normal serum 25-hydroxyvitamin D levels. Thus, we suspected HFTC and performed next generation sequencing for the GALNT3 gene, reported as the most frequent cause. A novel homozygote P85Rfs*6 (c.254_255delCT) mutation in GALNT3 was identified in both siblings. Our report adds two new patients to the literature about this rare genetic disease and suggests that small deletions in the GALNT3 gene may be related with HFTC phenotype.

WOS: 000484452900003 PubMed ID: 30872923 Aim: To evaluate the effect of umblical cord clamping time on lymphocyte subgroups in term and late preterm infants. Material and Methods: Seventy-four infants between 34 and 41 weeks of gestation were included in the study. Of these, 37 were umbilical cord clamped immediately after birth and the remaining 37 were clamped after waiting one minute. Babies were divided into two groups as term and preterm. The prenatal, natal, postnatal characteristics of the infants were recorded. Hematologic and lymphocyte subgroups were investigated in cord blood and venous blood at day 7. Lymphocyte subgroups were evaluated using flow cytometry. Results: With the delay of cord clamping, the leucocytes count and the percentage of CD3+T lymphocytes in cord blood of preterm infants decreased and this decrease continued at day 7. On the contrary, CD19+B lymphocyte levels in the cord blood of preterm infants increased, and this increase continued at day 7. Also, the percentage of CD4+T lymphocytes of preterm infants decreased with the delay of cord clamping at day 7. There was no difference between groups for the rate of sepsis development. Conclusion: With the delay of cord clamping, the leucocytes count, the percentage of CD3+T, and CD4+T lymphocytes decreased, and the percentage of CD19+B lymphocytes increased in preterm infants. The delay in cord clamping time in term and preterm infants seems to have no impact on the rate of sepsis development. Larger series of studies are needed to assess the effect of these findings on the development of infection in late preterm infants who have delayed cord clamping. Cukurova University Scientific Research Project Development Fund The authors received financial support from Cukurova University Scientific Research Project Development Fund.

Introduction The progression of periodontitis depends on the changes in bone and connective tissue homeostasis and the imbalance of the biofilm and the host immunoinflammatory response, particularly matrix metalloproteinases (MMP). Aim of the study To assess the probable relation between subgingival anaerobic flora and the expression of MMP-3 in patients with generalized aggressive periodontitis (AgP), chronic periodontitis (CP) and healthy subjects, and to evaluate these levels according to varied tissue loss severity. Material and methods The plaque index (PI), gingival index (GI), probing depth (PD) and clinical attachment levels (CAL) were evaluated. MMP levels obtained from gingival sulcus fluid (GCF) were measured with Enzyme Linked Immuno Assay (ELISA). The bacterial counts were determined with Parocheck®. Results Higher levels of MMP-3 in patients with AgP compared to subjects with CP and healthy individuals were observed. The microorganisms responsible of possible tissue destruction in both AgP and CP are red complex bacteria. T. denticola, T. forsythia, P. intermedia and F. nucleatum show positive correlation with MMP-3 levels. Conclusions MMP-3 is a biomarker associated with AgP, and red complex bacteria levels are correlated with increasing periodontal tissue loss in both periodontitis forms. The diagnosis of aggressive periodontitis, or site-specific treatment strategies can be orchestrated based on the evaluation of MMP-3 and the bacterial counts in patients with periodontitis.

Background/aim: Cardiopulmonary bypass (CPB)-associated acute kidney injury (AKI) is a common situation and rapid diagnosis and risk classification are important in the prevention and management of AKI. Changes in serum creatinine (SCr) levels in the current consensus criteria do not allow clinicians to diagnose CPB-associated AKI until 48 h after surgery. Materials and methods: We conducted a prospective single center study involving 57 patients who underwent cardiac surgery with CBP to compare serum beta-trace protein (BTP), neutrophil gelatinase-associated lipocalin-2 (NGAL), and cystatin C (CysC) levels with SCr for early diagnosis of CPB associated AKI. We defined AKI according to KDIGO criteria. Results: AKI was diagnosed in 24 (42.1%) patients. Mean duration of postoperative intensive care unit stay was 4.79 (± 6.12) days for the AKI+ group and 2.15 (± 0.56) days for the AKI- group. The average length of hospital stay was 8.13 (± 5.75) days for the AKI+ group and 7.21 (± 3.68) days for the AKI- group, which was significantly longer in the AKI+ group (P < 0.001, P = 0.011). Unlike other biomarkers, a significant increase in postoperative CysC levels was always found in patients with AKI during follow-up when compared to patients in whom AKI did not develop (P < 0.001). Conclusion: Increase in serum CysC levels showed a significant positive correlation with increase in SCr levels. We have not seen this correlation between other biomarkers and SCr. According to our study, serum CysC was a reliable biomarker that may aid in the early detection and follow-up of AKI after cardiac surgery.

WOS: 000457336800001 PubMed ID: 30513227 Objectives: Henoch-Schonlein purpura (HSP) is characterized by non-thrombocytopenic palpable purpura, abdominal pain, and arthralgia/arthritis. We aimed to describe the clinical presentations of children with HSP in a single center and compare the prevalence of each manifestations between patients with MEFV variants, particularly in exon 10 and those without. Methods: This cohort retrospectively included 144 HSP (59 females, 85 males) patients without Familial Mediterranean Fever (FMF) symptoms and followed for at least 6 months. We utilized the MEFV gene sequencing by using next-generation sequencing platform (MiSeq System, Illumina). Results: At least one MEFV variant was detected in 73 (50.7%) of 144 HSP patients and 5 (3.5%) patients were homozygote for M694V mutation. Although severe gastrointestinal involvement and nephritis rates were similar, we found that serum IgA, leukocyte, and platelet count at diagnosis were higher and hemoglobin was lower in HSP patients with MEFV gene variants in exon 10 than those without. Additionally, HSP patients with MEFV variants in exon 10 more often present with abdominal pain and intussusception. Conclusion: MEFV variants in exon 10 may affect clinical presentation of HSP in populations where FMF is common. While HSP may be an initial symptom of FMF, we speculate that physicians should be aware of FMF possibility in children with intussusception and lower hemoglobin, higher serum IgA, leukocyte, and platelet count. Cukurova University Scientific Research UnitCukurova University [TAY-2016-4020] This study was supported by the Cukurova University Scientific Research Unit under TAY-2016-4020.

Next Generation Sequencing (NGS) has uncovered hundreds of common and rare genetic variants involved in complex and rare diseases including immune deficiencies in both an autosomal recessive and autosomal dominant pattern. These rare variants however, cannot be classified clinically, and common variants only marginally contribute to disease susceptibility. In this study, we evaluated the multi-gene panel results of Common Variable Immunodeficiency (CVID) patients and argue that rare variants located in different genes play a more prominent role in disease susceptibility and/or etiology. We performed NGS on DNA extracted from the peripheral blood leukocytes from 103 patients using a panel of 19 CVID-related genes: CARD11, CD19, CD81, ICOS, CTLA4, CXCR4, GATA2, CR2, IRF2BP2, MOGS, MS4A1, NFKB1, NFKB2, PLCG2, TNFRSF13B, TNFRSF13C, TNFSF12, TRNT1 and TTC37. Detected variants were evaluated and classified based on their impact, pathogenicity classification and population frequency as well as the frequency within our study group. NGS revealed 112 different (a total of 227) variants with under 10% population frequency in 103 patients of which 22(19.6%) were classified as benign, 29(25.9%) were classified as likely benign, 4(3.6%) were classified as likely pathogenic and 2(1.8%) were classified as pathogenic. Moreover, 55(49.1%) of the variants were classified as variants of uncertain significance. We also observed different variant frequencies when compared to population frequency databases. Case–control data is not sufficient to unravel the genetic etiology of immune deficiencies. Thus, it is important to understand the incidence of co-occurrence of two or more rare variants to aid in illuminating their potential roles in the pathogenesis of immune deficiencies.

INTRODUCTION Cardiac sympathetic denervation (CSD) is an effective procedure for the treatment of inherited channelopathies. Left CSD has traditionally been recommended as neuromodulation to prevent arrhythmia episodes; however, it is thought that bilateral sympathectomy in combination with Kuntz ablation may have additional effects effective due to the anatomical variability of preganglionic sympathetic fibers. The aim of the study was to share our single-center clinical experience with bilateral thoracoscopic sympathectomy for cardiac denervation in different groups of pediatric patients with malignant arrhythmias. METHODS Fourteen patients (seven with CPVT, five with LQTS, one with resistant ventricular tachycardia as a sequela of myocarditis, and one with cardiomyopathy and atrial tachycardia) underwent bilateral thoracoscopic sympathectomy for cardiac denervation. RESULTS In all patients, arrhythmia episodes persisted despite medical therapy, and patients with implantable cardioverter-defibrillator received appropriate therapies. The rate of appropriate therapies was 3.25 per year (1-5 per year) in the year before sympathectomy. No major complications related to the procedure were observed in any of the patients. The QTc interval of LQTS patients decreased from 506.2 ± 16.9 ms before the procedure to 476 ± 28.8 ms after the procedure. The mean duration of follow-up after the procedure was 23.3 months (11-47 months). Only two patients received single episodes of therapy 12 and 22 months after CSD, and one patient had arrhythmic events due to noncompliance to medical therapy. CONCLUSION Due to the anatomical variability of preganglionic fibers bilateral CSD with Kuntz nerve ablation effective treatment and is a safe option.

Objective: To determine the cumulative effects of Mediterranean fever gene polymorphisms and mutations in patients with ?nflammatory bowel diseases. Methods: The case-control study was conducted from January, 2012, to January, 2016,at Cukurova University, Turkey, and comprised patients diagnosed with inflammatory bowel diseases and followed up at the Children Gastroenterology Department. By using molecular methods, 12 Mediterranean fevergene variants most frequently observed in the country were examined in all the diagnosed cases. The results were compared with age-matched healthy population data from the Genetic Diseases Diagnosis and Treatment Centre. Data was analysed using Graph Pad Prism. Results: Of the 151 subjects, 46(30.4%) were cases and 105(69.5%) were controls. Among the cases, there were 23(50%) subjects with a mean age of 14.8±3 years who had ulcerative colitis, and 23(50%) with mean age 14.5±3.2 years who had Crohn’s disease. The mean age of the controls was 16.4±3.2 years (p=0.716). Patients with ulcerative colitishad high frequencies of C allele in D102D T>C variant, G allele in G138G A>G variant, A allele in A165A C>A variant and A allele in R202Q G>A variant. Those with Crohn’s disease frequently had wild type of R202Q G>A variant. Also, D102D T>C / R314R C>T haplotype was common at a certain level in the UC group. Conclusions: Mediterranean fever gene variant was more frequently found in cases with ulcerative colitis compared to the controls. Key Words: MEFV, IBD, FMF, Polymorphism, Children. Continuous....

BACKGROUND We aimed to determine the diagnostic and prognostic value of serum irisin level in patients with acute pulmonary embolism (PE) admitted to the emergency department. METHODS Ninety patients who underwent computed tomography pulmonary angiography (CTPA) due to suspected PE were included in the study. Demographic data, PE risk factors, and associated diseases, vital signs, Wells score, Revised Geneva score, pulmonary embolism severity index (PESI), and simplified PESI (sPESI) were recorded. Irisin levels were measured by enzyme linked-immunosorbent assay. RESULTS Serum irisin level in patients with confirmed PE (n = 45) was significantly lower than that in patients (n = 45) without PE (p = 0.001). On receiver operating characteristic curve analysis, use of optimal irisin cutoff level of 8.6 µg/mL for diagnosis of PE was associated with 82.2% sensitivity, 60% specificity, 67.3% positive predictive value (PPV), and 77.1% negative predictive value (NPV) [area under the curve (AUC): 0.744, 95% confidence in-terval (CI): 0.641 - 0.830, p < 0.001)]. Use of optimal D-dimer cutoff level of 1,720 µg/L was associated with 86.7% sensitivity, 62.2% specificity, 69.6% PPV, and 82.4% NPV (AUC: 0.801, 95% CI: 0.704 - 0.878, p < 0.001). Irisin level showed no significant correlation with Wells score or revised Geneva score; however, irisin level showed a significant negative correlation with PESI and sPESI. CONCLUSIONS Patients with acute PE showed significantly lower serum levels of irisin. The sensitivity, specificity, NPV, and PPV of irisin level for diagnosis of PE were lower than those of D-dimer.

Background Probiotic ingestion is associated with an increase in intestinal flora of useful bacteria, which contributes to the known protective effects it has on the intestinal barrier and thereby reducing infection. The present study aims to investigate the protective effect of Lactobacillus rhamnosus gg (LGG) as an important probiotic with gastrointestinal barrier strengthening effect in sepsis. Methods Our study was conducted in the Animal Experiments Laboratory after obtaining ethicalapproval to conduct this study.Twenty-four rats were randomly divided into threegroups and group 1 (control group n=8), group 2 (sepsis group, n=8), group 3 (sepsis + probiotic group, n=8) were planned as double-blind. LGG was used as a probiotic. For the sepsis model, E. coli (0111: B4) was injected intraperitoneally, and the rats were sacrificed 48 hours after treatment. Blood samples were collected from all animals before sacrification and sent to the biochemistry laboratory to evaluate oxidant and antioxidant parameters. Results CRP values of Group 1 were significantly lower than Group 2, and CRP values of Group 3 were significantly lower. While total thiol levels of Group 2 were significantly lower than Group 1, total thiol levels of Group 3 were significantly higher than Group 2. There was no statistically significant difference between the groups for eNOS, GPX, PON1 and MDA levels. Conclusion Prophylactic use of probiotics, such as LGG to reduce bacterial translocation and strengthen the immune system, is an inexpensive and effective method of treatment, and we recommend the repetition of studies supported by prospective clinical trials.

This study aimed to determine oxidative stress in the tissue after testicular torsion biochemically and histopathologically and to examine the effects of Nesfatin-1 treatment on this injury. Thirty-two rats were randomly divided into four groups: sham, torsion + detorsion (4 hr torsion followed by 1 hr detorsion), ischaemia/reperfusion + saline (I/R + S) and I/R + nesfatin-1. I/R + S group a single-dose saline treatment was administered intraperitoneally at the two-hundred-tenth minute of torsion (ischaemia; 10 cc/kg). Similarly, I/R + nesfatin-1 group a single dose of nesfatin-1 treatment was administered intraperitoneally at the two-hundred-tenth minute of ischaemia (10 µg/kg). Myeloperoxidase, total oxidant status and oxidative stress index values were significantly increased in the I/R and I/R + S group compared to the sham group. Superoxide dismutase was significantly decreased in the I/R + S group compared to the sham group. No significant difference was found between the I/R + nesfatin-1 group and the other I/R groups (I/R and I/R + S) in terms of biochemical parameters. The mean diameter of the seminiferous tubule decreased in the I/R groups. However, the mean diameter of the seminiferous tubules was not significantly different between the I/R + S group and the I/R + nesfatin-1 group. Thus, the administration of nesfatin-1 after ischaemia did not reduce testicular-oxidative stress.

Objective To evaluate the effect of canakinumab on growth parameters of patients with autoinflammatory diseases. Methods This retrospective study included Colchicine resistant familial Mediterranean fever (FMF), Mevalonate kinase deficiency (MKD), Tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS), Deficiency of adenosine deaminase 2 (DADA2) patients treated with canakinumab for at least six consecutive months. Results Eleven patients with FMF, 9 with MKD, 3 with TRAPS, and 1 with DADA2 were included. The median age (range) at diagnosis and drug initiation was 6.06 (1.45-16.06) years and 9.72 (1.82-19.11) years, respectively. The mean weight, height, and BMI SD scores significantly increased after canakinumab. There were significant improvements in laboratory parameters and disease activities. However, growth parameters after the drug did not differ according to gender, the duration of diagnostic delay, and age at the diagnosis. Conclusions Canakinumab seems to have a positive effect on growth in patients with autoinflammatory diseases by controlling disease activity and inflammation.

Inflammatory myofibroblastic tumour usually has a benign course and is very rarely associated with the heart. It can have life-threatening consequences, depending on its position or the presence of aggressive and metastatic complications. A 3-month-old boy presented with pericardial tamponade and was diagnosed with intrapericardial inflammatory myofibroblastic tumour associated with Coronavirus OC43. A large tumour attached to the left ventricle was completely removed by surgical resection and he made a full recovery.ALK: anaplastic lymphoma kinase; CMV: cytomegalovirus; CRP: C-reactive protein; EB: Epstein-Barr virus; ESR: erythrocyte sedimentation rate; IM: inflammatory myofibroblastic tumour; NSAI: non-steroidal anti-inflammatory drugs; PTFE: polytetrafluoroethylene; SMA: smooth muscle actin.

Neutralizing antibodies targeting the receptor-binding domain (RBD) of the SARS-CoV-2 spike (S) block severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) entry into cells using surface-expressed angiotensin-converting enzyme 2 (ACE2). We developed a surrogate neutralization test (sVNT) to assess at what degree serum antibodies interfere with the binding of SARS-CoV-2-S-RBD to ACE2. The sVNT revealed neutralizing anti-SARS-CoV-2-S antibodies in the sera of 90% of mildly and 100% of severely affected coronavirus-disease-2019 (COVID-19) convalescent patients. Importantly, sVNT results correlated strongly to the results from pseudotyped-vesicular stomatitis virus-vector-based neutralization assay and to levels of anti-SARS-CoV-2-S1 IgG and IgA antibodies. Moreover, levels of neutralizing antibodies also correlated to duration and severity of clinical symptoms, but not patient age or gender. These findings together with the sVNT will not only be important for evaluating the prevalence of neutralizing antibodies in a population but also for identifying promising plasma donors for successful passive antibody therapy.

CD55 and CD59 are complement regulatory proteins suggested to be related with progression of diabetes and its complications. The stromal cell-derived factor 1 (SDF-1) and C-X-C chemokine receptor type 4 (CXCR4) are chemokine proteins. We aimed to investigate the relation of CD55 and CD59 expression levels and polymorphisms of SDF-1 and CXCR-4 with type 2 diabetes mellitus (T2DM) and its complications. Seventy-five T2DM patients and 73 controls were enrolled. Expression levels of CD55 and CD59 were measured by FACS Calibur; qRT-PCR was used to determine SDF-1 and CXCR-4 gene polymorphisms. CD55 and CD59 expressions in patients with nephropathy, retinopathy and cardiovascular disease were significantly lower than controls. Frequency of CXCR-4 T allele carrying was high in patients and created 1.6 fold risk for the disease (p = .07). CXCR-4 a allele carriers had decreased nephropathy; although there was no statistical significance in carrying CXCR-4 T allele, presence of nephropathy was approximately 2 times higher (p = .254). The nephropathy risk increased 10-fold in CXCR-4 TT genotype carriers (p = .02). All SDF-1 CC genotype carriers had retinopathy, so, it was considered that the CC genotype was effective in retinopathy development (p = .031). For the presence of cardiovascular disease, significant difference was observed for SDF-1 genotypes. Increased cardiovascular risk of 5- and 1.9-fold in SDF-1 T (p = .007) and CXCR-4 T (p = .216) allele carriers, respectively, was observed. We suggest that CD55 and CD59 protein levels and SDF-1 and CXCR-4 have predictive importance in process, complications and tendency of T2DM.

Purpose To investigate the effects of adalimumab pretreatment on the lipopolysaccharide-mediated myocardial injury. Methods Twenty-eight Wistar rats were randomized into four groups (n=7). Control (C) group animals were injected once a day with intraperitoneal (i.p) 0.9 % saline for two days. In the Adalimumab (Ada) group, adalimumab was injected at a dose of 10 mg/kg/ day (i.p) for two days. Lipopolysaccharide (Lps) group rats were injected with a dose of 5 mg/kg (i.p) lipopolysaccharide. Lipopolysaccharide + Adalimumab (Lps+Ada) group rats received adalimumab before the administration of lipopolysaccharide. The animals were sacrificed 24 h after the last injection and blood samples were obtained for determination of biochemical cardiac injury markers and circulating levels of TNF-α and interleukin-6 (IL-6). Hearts were harvested for histological examination. Results Endotoxin exposure resulted in significant increases in serum cardiac injury markers, serum cytokines and histological myocardial injury scores in the Lps group. The levels of circulating cytokines, cardiac injury markers and histological injury scores for myocardial necrosis, perivascular cell infiltration, and inflammation were significantly reduced in Lps+Ada as compared to Lps group (p

Dogruel D, Gundeslioglu OO, Yilmaz M, Alabaz D, Altintas DU, Kocabas E. Clinical findings and genetic analysis of the patients with IL- 12Rβ1 deficiency from southeast Turkey. Turk J Pediatr 2019; 61: 174-179. IL-12Rβ1 deficiency is an autosomal recessive disorder characterized by predisposition to poorly pathogenic mycobacteria, salmonella and candida species. We aimed to analyze the clinical manifestations, immunological and genetic features of IL-12Rβ1 deficiency in 10 Turkish patients from a single center. We retrospectively studied the clinical manifestations and genetic analysis of the IL-12Rβ1 deficiency patients from 2008 to 2016. Ten patients were diagnosed and followed for eight years. The mean age at onset and diagnosis were 24.1±42.5 (med:10.5) and 52.3±6.83 (med:20) months, respectively. Parental consanguinity rate was 81.8%. All patients were BCG vaccinated. Abscess and axillary lymphadenopathy in the vaccinated area was the most common initial presentation following the BCG vaccination, six patients had recurring oral candidiasis. Active infections were treated appropriately, in addition to prophylactic therapy with IFNɣ. We identified 6 different mutations in the IL12RB1 gene in 10 patients including 5 splice-site mutations, 3 missense, 1 frameshift, 1 premature stop codon. One of these mutations was novel. The most common mutation was IVS8+1G > A(c.783+1G > A) followed by p.R175W(c.523C > T). This study emphasizes that patients presented with abscess and axillary lymphadenopathy associated with BCG vaccination should be evaluated for IL-12Rβ1 deficiency.

PubMed: 30339503, Muscle-flap transferring is a routine approach utilized in reconstructive operations; however, flap morbidity is often a source of post-operative difficulty. Ischemia-Reperfusion Injury (IRI) is an important contributor to the viability of flaps after transferring. The goal of this research was for assess the probable useful impacts of ozone on flap survival in a rat muscle-flap design. Materials and Methods: We examined the effects of postconditioning ozone administration on viability of pedicled composite flaps. Twenty-eight Wistar rats were randomized into four groups: sham-operated (S), ischemia-reperfusion (IR), sham-operated + ozone (O), IR + ozone (IR + O), respectively. The animals were sacrificed on the eighth day. In a general histological evaluation, flap tissues were examined with a light microscope, and apoptotic cells were counted. The Apoptotic Index (AI) was then calculated. Flap-tissue samples were sent for analyses of malondialdehyde (MDA), catalase (CAT), glutathione peroxidase (GSH-Px), superoxide dismutase (SOD), and protein carbonyl (PCO), and blood samples were sent for analyses of Total Oxidant Score (TOS), and Total Antioxidant Capacity (TAC). Data were evaluated statistically using the Kruskal–Wallis test. Results: The histomorphometric score was remarkably greater in O (p =.002). The AI was greater in IR (p =.002). The antioxidant parameters values as regards SOD, GSH-Px, CAT, and TAC were found to be greater in O (p

To evaluate prestin as a biomarker for the identification of early ototoxicity.Rats (n = 47) were randomly assigned to five groups: low-dose (LAG) or high-dose (HAG) amikacin (200 and 600 mg/kg/day, respectively, for 10 days), low-dose (LCIS)or high-dose (HCIS) cisplatin (single doses of 5 and 15 mg/kg, respectively, for 3 days), and control (n = 8). At the end of the experiment, measurement of distortion product-evoked otoacoustic emissions (DPOAE) were performed to evaluate hearing, then blood samples and both ear tissues were collected under anesthesia. Prestin levels were determined by ELISA. Cochlear damage was evaluated histologically using a 4-point scoring system.The mean serum prestin levels were 377.0 ± 135.3, 411.3 ± 73.1, 512.6 ± 106.0, 455.0 ± 74.2 and 555.3 ± 47.9 pg/ml for control, LCIS, HCIS, LAG and HAG groups, respectively. There was significant difference between prestin levels of Control-LCIS-HCIS groups (p = 0.031) and prestin levels of Control-LAG-HAG groups (p = 0.003). There were also significant differences in prestin levels between the low- and high-dose cisplatin and amikacin groups (p = 0.028 and p = 0.011, respectively). Each group had significantly lower DPOAE results at 4, 6 and 8 kHz than control groups (p 0.001). The LAG, HAG, LCIS and HCIS groups had significantly higher cochlear damage scores than the control group (p 0.05).Higher doses of cisplatin and amikacin were associated with the greatest increases in serum prestin level and cochlear damage score. The results of this study suggest that prestin is a promising early indicator of cochlear damage.

PubMedID: 30101368 Henoch–Schönlein purpura (HSP), the most common childhood vasculitis is characterized by non-thrombocytopenic palpable purpura, arthritis/arthralgia, abdominal pain and renal involvement. Functional gastrointestinal disorders (FGIDs) are heterogeneous disease spectrum with unclear etiology and include the most common subtypes: functional dyspepsia, irritable bowel syndrome (IBS), functional abdominal pain and functional constipation. Formerly, FGIDs were known as non-organic disorders; however, recent advances revealed that low-grade inflammation may also play a role. We aimed to clarify whether HSP predisposes to FGIDs in pediatric population. Seventy-four children with HSP, diagnosed at least 6 months before the study and 78 healthy controls were enrolled to the study. Patients with red flag signs for organic GI disorders were excluded. Rome IV criteria were utilized for FGIDs diagnosis. We compared the frequencies of FGIDs between HSP patients and healthy subjects. We also examined the parameters including age, abdominal pain, arthralgia, bloody stool, renal involvement and treatment with corticosteroids and laboratory results at HSP diagnosis such as erythrocyte sedimentation rate, C-reactive protein, hemoglobin, leukocytes and platelet counts among patients with and without FGIDs. Overall FGIDs and IBS frequency were 35.1% (n = 26) and 10.8% (n = 8) in HSP patients, 19.2% (n = 15) and 2.6% (n = 2) in healthy controls, respectively. Disease characteristics and laboratory parameters at disease onset were similar between HSP patients with and without FGIDs. Overall FGIDs rate, particularly IBS were statistically higher in HSP patients. We speculate that children with preceding HSP may be predisposed to FGIDs. Since the FGIDs pathogenesis is still remains unclear, further studies are needed to confirm this hypothesis and clarify the etiology. Physicians also should pay attention to FGIDs in HSP patients with ongoing abdominal pain and thus prevent this comorbidity with dietary and psychologic measures. © 2018, Springer-Verlag GmbH Germany, part of Springer Nature.

Objectives This study aims to investigate the effectiveness and reliability of ozone (O3) in Freund's complete adjuvant (FCA)-induced arthritis, an animal model for rheumatoid arthritis. Patients and methods Thirty-six four- to five-month-old male Wistar rats weighing between 274-420 gr were used in this study. Saline was injected into the hind paws of half of these rats, and FCA was injected into the other half. At the end of two weeks, 40 μg of O3 was administered to nine rats from each group twice a week for seven total doses. The rats were followed-up in terms of clinical findings. At the sixth week, the rats were sacrificed and serum malondialdehyde, glutathione peroxidase, and superoxide dismutase levels were measured. In addition, ankle joints were separated for histopathological examination. Results Significant improvement was observed in terms of hind-paw diameter, severity of arthritis, and histopathological findings of inflammation after O3 treatment in the group with FCA-induced arthritis. Although it was not quite significant, an upward trend was detected in oxidative stress markers with O3 treatment. Conclusion This study, the first to investigate the effects of systemic O3 on the clinical and histopathological outcomes of rheumatoid arthritis, indicates that O3 is a highly effective and reliable treatment method in FCA-induced arthritis in animal models.

Aim: The aim of the study was to evaluate the clinical, endoscopic, histopathological, laboratory, and imaging findings of patients with familial Mediterranean fever (FMF) presenting with dyspeptic symptoms. Material and methods: Endoscopy was performed in 28 patients with FMF undergoing colchicine therapy, who presented to our hospital between 2012 and 2014 with abdominal pain, diarrhoea, and dyspeptic complaints. Upper gastrointestinal system endoscopy was performed in all patients; 16 of these underwent colonoscopy. Demographic characteristics, clinical and laboratory data, genetic analysis, imaging data, and endoscopy and colonoscopy findings were evaluated in all patients. Results: Histopathological analysis of biopsy specimens revealed gastrointestinal pathology in 21 (75%) patients. Upper endoscopy analysis revealed that 14 (50%) patients had antral gastritis (8 Helicobacter pylori (-) antral gastritis and 6 H. pylori (+) chronic active gastritis) and 2 (7%) cases had giardiasis. Four antral gastritis cases had concomitant esophagitis, and 2 patients had widespread ulcers in the terminal ileum. The rest had a normal colonic appearance; however, three patients with normal colonoscopic findings were found to have chronic active colitis on histopathological examination. Conclusions: In this study, most patients with FMF presenting with dyspeptic complaints had gastrointestinal pathology. Furthermore, there was higher inflammatory bowel disease incidence in these patients. © 2018 Termedia Publishing House Ltd. All rights reserved.

Purpose: Nitric oxide is an important mediator that plays a major role in platelet adhesion/aggregation. Asymmetric dimethylarginine (ADMA) is a competitive inhibitor of endothelial nitric oxide synthase. In this study, we aimed to examine the ADMA levels in diagnosis and follow-up of acute pulmonary thromboembolism.Materials and Methods: The study was designed as a prospective study. Thirty consecutive patients with acute PTE and 20 healthy volunteers were enrolled into the study. Patients were followed 1 month. Plasma ADMA levels were measured on admission and following the first month.Results: Ona dmision there was statistically significant difference between two groups. When we compared serum ADMA levels difference between patients group with resolution and without resolution, there was not any statistically significant difference. There was a significant difference between serum ADMA levels of massive group and the other 2 groups.Conclusion: Plasma ADMA levels elevated in patients with pulmonary thromboembolism. This finding supports the idea ADMA levels correlated with thrombus burden.

Introduction Migraine, the most widespread cause of headache in young adults, is a frequent reason for presentation to emergency departments. The aim of this study was to determine the diagnostic value of serum galectin-3 and high-sensitivity C-reactive protein (hsCRP) levels in migraine patients. Material and methods Serum galectin-3 and hsCRP levels were measured using enzyme-linked immunosorbent assay in 70 migraine patients and 70 healthy control subjects. Results The mean values of hsCRP and galectin-3 levels were significantly higher in the migraine group than those in the control group (1.67 ±0.49, 1.12 ±0.38 mg/l, p < 0.001; and 3.76 ±1.45, 2.05 ±0.29 ng/ml, p < 0.001, respectively). When the diagnostic values of the biomarkers were assessed in the migraine attack and control groups, the sensitivity and specificity of the test for hsCRP at the cut-off point of 1.32 mg/l were 70% and 73% (AUC = 0.805, 95% CI: 0.74–0.88, p < 0.001), and for galectin-3 at the cut-off point of 2.4 ng/ml, 89% and 90% (AUC = 0.97, 95% CI: 0.95–0.99, p < 0.001), respectively. Conclusions This study showed the presence of a relation between high serum levels of hsCRP and galectin-3 in migraine patients. However, well-controlled, carefully executed longitudinal studies are required to confirm these findings.

We report the successful surgical treatment of a case of double drainage of the right upper pulmonary vein into the superior vena cava and left atrium in a 9-year-old girl.

Anomalous single coronary artery from pulmonary artery is a very rare congenital heart anomaly. Anomalous single coronary artery from pulmonary artery has high mortality rates and poor surgical outcome despite advanced surgical techniques. We report a 4-month-old infant presented by congestive heart failure findings and diagnosed with anomalous single trunk coronary arteries arising from right pulmonary artery.

PubMedID: 29143127 Familial Mediterranean fever is an autoinflammatory disorder characterized by recurrent fever and serositis. We aimed to describe serum vitamin B 12 and vitamin D levels and their correlation with self-reported sleep quality of pediatric FMF patients. Sixty-three children with FMF were enrolled to the study. Information on sleep quality was obtained using self-administration of Pittsburg Sleep Quality Index (PSQI). The patients were divided into subgroups depending on vitamin D serum concentrations: ? 20 and < 20 ng/ml. We also grouped patients according to their serum B 12 concentration: ? 200, < 200 pg/ml. B 12 levels were not correlated with PSQI scores, whereas significant correlation was found between vitamin D and total PSQI scores and daytime sleepiness. Total PSQI score, sleep disorders and daytime sleepiness sub-scores were statistically high in patients with serum D vitamin levels below 20 ng/ml. Sleep efficiency was found lower in patients with serum B 12 levels below 200 pg/ml. B 12 may have a positive role on effective sleep. More importantly, we suggest that vitamin D is protective against sleep disorders and poor sleep, it may also improve daytime activities. © 2017, Springer-Verlag GmbH Germany, part of Springer Nature.