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    Assessment of epicardial adipose tissue thickness and the mean platelet volume in children with familial Mediterranean fever
    (Bmc, 2015) Uluca, Unal; Demir, Fikri; Ece, Aydin; Sen, Velat; Gunes, Ali; Aktar, Fesih; Tan, Ilhan
    Background: Familial Mediterranean fever (FMF) is an inflammatory disease, which is suggested to be associated with increased risk of atherosclerosis. Epicardial adipose tissue (EAT) thickness and the mean platelet volume (MPV) are parameters used in prediction of atherosclerotic risk in various conditions. These parameters were evaluated in children with FMF and compared with healthy controls. Methods: Forty-five patients with FMF and 54 age-and gender-matched healthy controls were assessed. Duration of symptoms, age at diagnosis, duration of delay in diagnosis, frequency and duration of FMF attacks, disease severity scores, response to colchicine therapy, MEditerraneanFeVer (MEFV) gene mutations, and MPV values were recorded. EAT thicknesses were measured by echocardiography. Results: Epicardial adipose tissue thicknesses of the children with FMF were found to be significantly greater than that of controls (5.1 +/- 1.4 vs. 4.5 +/- 0.9 mm, p = 0.036). FMF patients had significantly higher MPV values compared with the controls (7.8 +/- 1.1 vs. 7.3 +/- 1.4 fl, p = 0.044). Age at diagnosis, duration of delay in diagnosis, and MPV values were found to be correlated with EAT thickness in the patient group (r = 0.49, p = 0.001 for the former parameters and r = 0.32, p = 0.04 for MPV). Conclusion: Epicardial adipose tissue thickness and MPV values seem to be increased in children with FMF. These findings may indicate an increased risk of atherosclerosis in FMF patients.
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    Clinical and Laboratory Characteristics and Follow Up Results of 121 Children with Juvenile Idiopathic Arthritis
    (Modestum Ltd, 2013) Ece, Aydin; Sen, Velat; Yel, Servet; Gunes, Ali; Uluca, Unal; Tan, Ilhan; Karabel, Duran
    This study aimed to investigate the clinical and laboratory features of children with juvenile idiopathic arthritis (JIA) that followed up at Dicle University Hospital Department of Pediatrics. Totally, 121 (64 male, 57 female) children with the mean age of 10.0 +/- 4.1 (range, 1.5-1.8) years were included. The mean disease onset age was 7.9 +/- 3.8 (range, 0.8-15.4) years and the mean follow up period was 2.1 +/- 1.9 years. The percentages of JIA subtypes were as follows: Oligoarticular JIA 67 (55.4%), polyarticular 45 (37.2%), enthesitis related arthritis 5 (4.1%) and systemic JIA 4 (3.3%). The most common complaints were arthralgia (91.7%), fever (57.0%), fatigue (38.8%) and malaise (34.7%) and the most frequently involved joints were knee (74.4%), ankle (57.9) and wrist (48.8%). Complete remission were achieved in 28 (23.1%) and partial remission in 56 (46.3%), however 27 (21.3%) cases not responded to treatment satisfactorily. Significant risk factors for poor response to treatment with logistic regression were found as delay in treatment >= 6 months (Odds ratio, OR: 11.1; p=0.006), existence of thrombocytosis (OR: 7.5; p=0.009) and early disease onset (age<5 years) (OR: 18.1; p=0.004). In conclusion, JIA is a heterogeneous childhood disease with varied clinical manifestations. Early onset disease, delay in treatment and existence of thrombocytosis were the risk factors for an unfavorable outcome.
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    Clinical, radiological and laboratory findings in 185 children with tuberculous meningitis at a single centre and relationship with the stage of the disease
    (Biomed Central Ltd, 2015) Gunes, Ali; Uluca, Unal; Aktar, Fesih; Konca, Capan; Sen, Velat; Ece, Aydin; Hosoglu, Salih
    Background: A delay in the diagnosis and treatment of tuberculosis meningitis (TBM) may lead to increased mortality and morbidity. The aim of this study was to describe the clinical, radiological and laboratory findings of TBM on a cohort of 185 pediatric patients at a single centre over a 10 year period and to investigate relationship between the stage of the disease. Methods: The hospital records of 185 TBM children that presented to the Pediatric Clinics of Dicle University Hospital were retrospectively evaluated. The age, gender, family history of tuberculosis, result of Mantoux skin test, status of BCG vaccination, stage of TBM at hospitalization, and clinical, laboratory and radiological features were recorded. Clinical staging of TBM was defined as follows: Stage I, no focal neurological findings and Glasgow Coma Scale (GCS) score 15; Stage II, GCS 15 presenting with focal neurological deficit or all the patients with GCS 10-14; Stage III, all the patients with GCS < 10. Relationships between results and stages of TBM were investigated. Results: The mean age of the patients was 53.5 +/- 44.9 months (4 months-18 years). 121 (65.4 %) of the patients were male and 64 (34.6 %) female. Family history of tuberculosis was defined in 62 (33.5 %) patients. Forty five (24.3 %) children had BCG vaccination scar. Mantoux skin test was interpreted as positive in 35 (18.9 %) patients. Sixty-eight (36.8 %) children were at stage I TBM, 57 (30.8 %) at stage II and 60 (32.4 %) were at stage III on admission. Mean duration of hospitalization was 23.9 +/- 14.1 days. Totally, 90 patients (48.6 %) had abnormal chest X-ray findings (parenchymal infiltration in 46 (24.9 %), mediastinal lymphadenopathy in 36 (19.5 %), miliary opacities in 25 (13.5 %), pleural effusion in 2 (1.1 %), and atelectasis in 2 (1.1 %) patients). One hundred sixty seven (90.3 %) patients had hydrocephalus in cranial computerized tomography. There were 24 (13.0 %) patients with positive culture for Mycobacterium tuberculosis and 3 (1.6 %) patients with positive acid-fast bacilli in cerebrospinal fluid. Overall mortality rate was 24 (13.0 %). Among the findings; patients at Stage III had less frequent positive chest X-ray abnormality, miliary opacities and BCG vaccination scar when compared with patients at Stage I and II (p = 0,005; p = 0,007, p = 0.020, respectively). Conclusions: Children with TBM and positive chest X-ray findings at hospital admission were more frequently diagnosed at Stage I, and BCG vaccination might be protective from the Stage III of the disease.
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    Congenital tuberculosis: presentation of a rare case
    (Soc Argentina Pediatria, 2015) Sen, Velat; Sen, Hadice Selimoglu; Aktar, Fesih; Uluca, Unal; Karabel, Musemma; Gurkan, Mehmet Fuat
    Congenital tuberculosis is a rare disease with a high mortality rate. Congenital tuberculosis is considered the result of mother-to-child transmission from the placenta to the fetus, through the ingestion of the amniotic fluid, or via transplacental transmission through the umbilical vein. Given the non-specific clinical signs of tuberculosis, it is usually difficult to diagnose it. The case of a 48-day-old male infant hospitalized due to weight loss, fever, cough, hemoptysis, and respiratory distress for the past 20 days, is presented. In this period, he had received broad spectrum antibiotics but with no improvement. A chest x-ray showed the presence of consolidation and a cavitary lesion in the upper and middle left lung fields. Mycobacterium tuberculosis was detected by polymerase chain reaction in a bronchoalveolar lavage specimen. Congenital tuberculosis was diagnosed based on this finding; hence, a tuberculostatic regimen was started accordingly. The patient died 13 days after treatment initiation. Congenital tuberculosis should be considered in infants with weight loss, fever, cough, hemoptysis and respiratory distress.
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    Correlation of mean platelet volume, neutrophil-to-lymphocyte ratio, and disease activity in children with juvenile idiopathic arthritis
    (E-Century Publishing Corp, 2015) Gunes, Ali; Ece, Aydin; Sen, Velat; Uluca, Unal; Aktar, Fesih; Tan, Ilhan; Yel, Servet
    Juvenile idiopathic arthritis (JIA) is a chronic inflammatory arthritis characterized by periods of remission and relapse. Mean platelet volume (MPV) is an indicator of systemic inflammation. In the present study, we aimed to determine the association between mean platelet volume (MPV), neutrophil/lymphocyte ratio (NLR), platelet distribution width (PDW) and clinical measures of diseases activity in children with JIA. The study included 115 patients with JIA (64 with active disease and 51 with inactive disease) and 64 age-gender matched healthy control subjects. Routine laboratory methods were used to measure white blood cell count (WBC), platelet count (PLT), neutrophil count, lymphocyte count, hemoglobin (Hb), MPV, PDW, NLR, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) in all subjects of both the patient and control groups. Active disease was associated with significantly increased MPV (8.23 +/- 1.16 fl) compared with inactive disease (7.00 +/- 1. 08 fl) and control subjects (6.77 +/- 1.08 fl) P<0.001, P<0.001, P=NS, respectively). NLR was significantly higher in patients with active (2.11 +/- 1.19) and inactive (2.03 +/- 1.51) disease relative to the control subjects (1.33 +/- 0.66) (P<0.001, P=0.017, respectively). Mean PDW was significantly higher in patients with active disease (17.84 +/- 1.06) compared with the control group (17.19 +/- 0.93) (P=0.01). Our results suggest that MPV may be a useful marker of disease activity in patients with JIA. Regular treatment may decrease platelet activation in JIA patients. However, NLR was not a predictive marker of disease activity in patients with JIA.
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    Effects of Gluten-Free Diet on Quality of Life and Depression in Children With Celiac Disease
    (Lippincott Williams & Wilkins, 2015) Simsek, Seref; Baysoy, Gokhan; Gencoglan, Salih; Uluca, Unal
    Objectives: The aim of this study was to investigate the level of depression and quality of life in children with celiac disease (CD). In addition, it aimed to examine the relations of depression level and life quality with adherence to a gluten-free diet (GFD). Methods: Twenty-five children with CD and 25 healthy controls were included. The Depression Scale for Children and the General Purpose Health-Related Quality of Life Scale for Children were performed on patients before and after receiving recommendations to follow a GFD. Results: No significant differences were found in the depression scores between the patients and the control subjects (P>0.05). In contrast, total scores and scores of the emotional well-being subscale of the measure of Quality of Life Scale for Children were significantly lower in patients with CD compared with the control group (P<0.05). No significant improvements were observed in depression or life quality scores of the total subsample of celiac patients, all of whom received a recommendation to follow a GFD (P>0.05). Significant decrease was observed in the depression scores, however, of celiac patients who were able to actually adhere to the GFD compared with nonadherent patients. Conclusions: CD negatively affected quality of life in children. Adherence to GFD was associated with reduction in depression symptoms. Improving the adherence of celiac patients to a GFD may have a favorable effect on their depression symptoms.
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    The Effects of Pomegranate and Carvacrol on Methotrexate-Induced Bone Marrow Toxicity in Rats
    (Canadian Soc Clinical Investigation, 2014) Sen, Velat; Bozkuri, Mehtap; Soker, Sevda; Ece, Aydin; Gunes, Ali; Uluca, Unal; Soker, Murat
    Purpose: The aim of this study was to evaluate the e ff ects of pomegranate (PMG) extract and carvacrol (CARV) on methotrexate (MTX)- induced oxidative stress and bone marrow toxicity. Methods: Wistar albino rats (32 rats) were divided into four groups (n=8): Group 1 was control; Group 2 was given a single intraperitoneal injection of methotrexate (20 mg/kg); Group 3 was treated with carvacrol (73 mg/kg i.p.) one day before MTX (20 mg/kg i.p.) injection; and, Group 4 received a single dose of MTX (20 mg/kg i.p) while PMG was administered orally for seven days at 225 mg/kg. After animals were euthanized, blood samples were taken to evaluate hematological parameters and oxidative stress. In addition, the femur was cropped and bone marrow was extracted for examination. Results: White blood cell count, hemoglobin, hematocrit and platelet count were found to be decreased in the MTX group, but these changes were prevented in the groups that received CARV and PMG. Furthermore, decreased bone marrow cellularity was found in the groups treated with MTX, whereas the PMG and CARV groups had cellularity similar to controls. Strikingly, oxidative stress increased in the MTX group, but was ultimately decreased in the rats that received the antioxidants PMG and CARV. Conclusion: Carvacrol and PMG were found to be protective against methotrexateinduced oxidative bone marrow damage. Use of these antioxidants, in combination with chemotherapeutics, may help to reduce some adverse effects of methotrexate.
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    Evaluation of the Mean Platelet Volume in Children with Juvenile Idiopathic Arthritis
    (Modestum Ltd, 2014) Sen, Velat; Ece, Aydin; Uluca, Unal; Gunes, Ali; Tan, Ilhan; Tuncel, Tuba; Aktar, Fesih
    Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory arthritis in children. Mean platelet volume (MPV) is an indicator of platelet size and has been investigated as an inflammation marker in several diseases. This study was designed to investigate the MPV values in patients with JIA and healthy subjects, and determine the correlation between MPV, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR). The hospital records of a total of 40 children with JIA, diagnosed using the International League of Associations for Rheumatology (ILAR) criteria, and 40 healthy controls were enrolled into the study. White blood cell count (WBC), platelet count, CRP, ESR, and MPV levels were retrospectively recorded. Children with JIA had significantly higher MPV values (8.28 +/- 1.12 fL) compared to the control group (7.53 +/- 1.07 fL) (p=0.003). Significant correlations were found between MPV, ESR, and CRP in the JIA group (r=0.676, p<0.001 and r=0.430, p<0.006). In addition, a negative correlation was found between platelet count and MPV (r=-0.818, p<0.001) in JIA patients. There was no significant difference in WBC values between the patient and the control groups (p>0.05). Our results suggest that MPV levels may be a useful marker of inflammation and prognostic factor for atherosclerosis risk in pediatric JIA patients.
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    Fasciola hepatica infestation in Turkish children
    (Edizioni Minerva Medica, 2017) Tokgoz, Yavuz; Karabel, Musemma; Baysoy, Gokhan; Goya, Cemil; Cicek, Muttalip; Pirinccioglu, Ayfer G.; Uluca, Unal
    BACKGROUND: The data on childhood fascioliasis are limited. We aimed to heighten physicians' awareness of Fasciola Hepatica by presenting 14 cases in this paper. METHODS: The medical records of pediatric patients treated for F. Hepatica infection within the last 2 years were reviewed. ELISA method with a cut-off level of 10 was used as the serological test, in which an absorbance level of >11 DU/mL was considered positive. RESULTS: Ten (71.4%) patients were female. Complaints at presentation were abdominal pain (N.=14; 100%) and loss of appetite (N.=12; 85.7%). Twelve (85.7%) patients were from rural areas. All patients had an elevated eosinophil count (mean: 3885.6/mm(3), minimum: 566/mm(3),maximum: 18687/mm(3)). Six patients had a leucocyte count >10.000 cells/mm(3) and 8 (57%) patients had an ESR>20 mm/h. Gamma-glutamyt transferase (GGT) was normal in all but 1 patient presenting with cholangitis. In hepatobiliary ultrasonography (USG) the most common finding was a heterogeneous solid lesion found in 11 patients. Advanced imaging methods revealed multiple cystic lesions in 10 patients and magnetic resonance imaging (MRI) showed hypo-hyperintense hepatic parenchymal changes in 9 patients. The patients were started on Triclabendazole 10 mg/kg/dose. Clinical, laboratory, and radiological findings of the patients were improved after the therapy. CONCLUSIONS: Fascioliasis should be suspected in children from endemic regions presenting with abdominal pain, weight loss, and fever, which are accompanied by eosinophilia, elevation in transaminases, and cystic/solid lesions in liver, particularly in bile ducts or parenchyma. Triclabendazole is very effective for treatment of this disease.
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    High frequency of E148Q sequence variation in children with familial Mediterranean fever in southeast Turkey
    (Soc Argentina Pediatria, 2015) Uluca, Unal; Ece, Aydin; Sen, Velat; Coskun, Salih; Gunes, Ali; Yel, Servet; Tan, Ilhan
    Objective: The aim of this study was to investigate the spectrum of Mediterranean fever (MEFV) gene mutations and genotype-phenotype correlation in children with familial Mediterranean fever (FMF) in southeast Turkey. Methods: A total of 507 children (274 females) with FMF and MEFV gene mutation(s) were included. A 15-year retrospective evaluation was conducted; parameters analyzed were: age, sex, age at symptoms onset, age at FMF diagnosis, delay between symptoms onset and diagnosis, FMF attack symptoms, and response to colchicine. Disease severity scores were calculated and MEFV mutation analysis was performed via real-time PCR for the 6 most frequent mutations. Children with comorbid diseases or tested negative for MEFV gene mutations were excluded to provide homogeneity. Results: A family history of FMF was found in 60.2% (n=305) of patients. The most common symptoms reported for FMF attacks were abdominal pain (98.0%), fever (93.9%) and arthralgia (47.3%); 75.0% of patients (n= 380) were heterozygous, 14.2% were homozygous (n= 72) and 10.8% were compound heterozygous (n= 55). The following MEFV gene mutation alleles were identified: E148Q (40.1%), M694V (25.9%), V726A (15.8%), R761H (7.4%), M680I (6.8%), and P369S (4.1%). The M694V subgroup had the lowest mean age of disease onset and the highest mean disease severity score, whereas the E148Q group had later mean disease onset and the lowest mean disease severity score (p<0.05). Conclusion: The highest E148Q mutation frequency and milder disease in the course of FMF in our study population may be due to geographic and ethnic background dissimilarities of southeast Turkey.
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    Indications of a Major Cause of Hospitalization in Intensive Care: Child and Adolescent Acute Poisoning
    (Duzce Univ, 2016) Uluca, Unal; Sen, Velat; Karabel, Duran; Gunes, Ali; Bozkurt, Fatma; Sahin, Cahit; Sabaz, Muhammed Nurullah
    Aim: Acute poisoning is an important health problem in pediatric patients. The aim of this study is to evaluate the epidemiological and clinical signs and treatment results of the children with acute poisoning. Methods: All of the cases that admitted to the children hospital between September 2012 and June 2014 enrolled to the study. File records of cases was retrospectively evaluated. Age, gender, admission season, intended purpose of agent, feature of agent, ingested route, symptoms, physical examination findings, mental state, applied treatments and intensive care needs were recorded. Results: 65 males, 63 girls were included to the study. 57.8% of patients were under the age of 5. The most admission seasons were autumn (50.8%) and summer (26.6%). Ten cases, mostly girls, had suicidal ingestion. In all cases, except one, oral poisoning had occurred. Three patients were mentally handicapped. The most frequent symptoms and signs during admission were nausea and vomiting (10.9%) and lethargy (7.8%). Before admission, the majority of patients (68.0%) had undergone gastric lavage and activated charcoal. Intensive care was required in 21 (16.4%) patients. Pharmacological or non-pharmacological intoxication were not related with intensive care need. Hospitalization duration was significantly longer in patient hospitalized in intensive care unit. The age of the patients who need intensive care were lower than those without. Nine (7.0%) patients died during follow-up in intensive care. Death mostly had occurred as a result of non-pharmacological intoxication. Conclusion: Acute poisoning cases among the important reasons for emergency visits, and can result in significant morbidity and mortality. Therefore, this issue of parents and health professionals to increase their level of knowledge in daily practice is important to consider acute intoxication.
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    Mean platelet volume in retinopathy of prematurity
    (Springer, 2015) Sahin, Alparslan; Uluca, Unal; Turkcu, Fatih Mehmet
    [Abstract Not Available]
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    The MEFV mutations and their clinical correlations in children with familial Mediterranean fever in southeast Turkey
    (Springer Heidelberg, 2014) Ece, Aydin; Cakmak, Erdal; Uluca, Unal; Kelekci, Selvi; Yolbas, Ilyas; Gunes, Ali; Yel, Servet
    The aim of this study was to determine the Mediterranean fever (MEFV) gene mutations and their clinical correlations in children with familial Mediterranean fever (FMF) in southeast Turkey. Clinical and laboratory characteristics of 147 (65 males, 82 females) consecutive children with FMF having a positive MEFV gene mutation were prospectively investigated. Patients with negative MEFV gene mutations or atypical FMF presentations and those from other regions of the country were excluded. Clinical manifestations and disease severity scores were recorded. The six most frequent MEFV mutations including M694V, V726A, R726H, P369S, E148Q and P369S were investigated by a reverse hybridization test method. The median age of study group was 9.0 years, median age at diagnosis was 7.8 years, median age at disease onset was 5.0 years, and median follow-up duration was 4.0 years. A positive family history of FMF and parent-to-offspring transmission was found in 58.5 and 42.2 % of families, respectively. The frequencies of independent alleles, with decreasing order, were E148Q (30.7 %), M694V (26.0 %), R761H (13.5 %), V726A (13.0 %), P369S (10.5 %) and M680I (6.3 %) in FMF patients. The M694V subgroup had higher mean disease severity score and longer attack duration compared with E148Q and other mutations subgroups (p < 0.05). Two patients with amyloidosis had the M694V homozygote genotype. In conclusion contrast to other regions and many other ethnicities of the world, the most frequent MEFV gene mutation was E148Q in southeast Turkey. The M694V mutation frequency was lower, and disease severity was relatively mild in FMF children of this region.
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    Meningitis in Children: Evaluation of 197 Patients
    (Derman Medical Publ, 2015) Gunes, Ali; Konca, Capan; Aktar, Fesih; Sen, Velat; Uluca, Unal; Yel, Servet; Tas, M. Ali
    Aim: The aim of our study was to evaluate the epidemiologic, clinic and laboratory results and the answers to treatment of meningitis cases. Material and Method: In this study, the epidemiologic, clinic and laboratory results of 197 patients hospitalized with central nervous system infection diagnosis in the Department of Pediatric Health and Diseases of the Faculty of Medicine of Dicle University between 1st of January 2003 and 1st of January 2006 have been studied retrospectively. The files have been studied in details for age, sex, complaints, and results of physical examination, laboratory results, radiological results and treatments applied. Results: 118 of the patients were male, 79 were women and the mean age calculated was 62,2 +/- 47,3 months. 137, 27 and 33 patients have been respectively considered as ABM, AM and TM. The most frequent complaints of application to hospital were fever (95,4%), vomiting (82,7%), headache (45,6%) and change of consciousness (21,3%). The presence of many risks about meningitis has been observed. The most frequent risk factors were head trauma history, parenchymal lung tuberculosis, military tuberculosis, presence of V-P shunt, meningocele, varicella history, having mumps, and the presence of purulent ear discharge. BOS has developed in 7 patients and for five patients, reproduction occurred in blood culture. The most important central nervous system sequels or complications were in order of frequency hydrocephalies requiring the installation of V-P shunt, brain edema, epilepsies, subdural effusions, tuberculoma, retention of head pair, and brain apses. The rate of mortality was (% 13.1). Discussion: During the period of execution of the study, the mortality and morbidity of central nervous system diseases were still at high risk. But this may be associated to the absence of vaccination programs for frequent meningitis factors such as pneumococcus and H. influenza were not in routine vaccination program in our country; thus we consider that further studies about this issue after vaccination are required.
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    MRI-based evaluation of the factors leading to pituitary iron overload in patients with thalassemia major
    (Masson Editeur, 2016) Cetincakmak, Mehmet Guli; Hattapoglu, Salih; Menzilcioglu, Sait; Alan, Bircan; Uluca, Unal; Ucar, Ahmet; Soker, Murat
    Aim: Given the lack of studies evaluating pituitary iron overload in patients with thalassemia major, we used magnetic resonance imaging (MRI) to evaluate these patients and the factors affecting the disease process. Materials and methods: The 84 patients with beta-thalassemia major who were included in this study were referred to our clinic for cardiac and hepatic T2* MRI. T2*-weighted images of the pituitary gland, heart, and liver were obtained using a 1.5-tesla MRI unit and a multi-echo gradient-echo sequence. Associations between pituitary T2*, cardiac T2*, hepatic T2*, pituitary height, serum ferritin (SF) level, patient age, and other demographic findings were assessed. Results: Pituitary T2* values correlated with hepatic TZ values, cardiac T2* values, SF level, and patient age (P <= 0.001, 0.001, 0.001, 0.01, respectively) but not with pituitary height (P=0.76). Pituitary and cardiac T2* values were lower in the subset of patients who underwent splenectomy (P=0.046 and P= 0.002, respectively). Conclusion: Pituitary iron overload rapidly increases during puberty and in this study correlated with cardiac and hepatic T2* values, patient age, SF level, and liver size, but not with the height of the pituitary. Pituitary iron overload also increases following splenectomy. Together, these findings indicate that numerous factors contribute to pituitary iron overload. (C) 2016 Elsevier Masson SAS. All rights reserved.
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    Preventive Effects of Dexmedetomidine on the Liver in a Rat Model of Acid-Induced Acute Lung Injury
    (Hindawi Ltd, 2014) Sen, Velat; Guzel, Abdulmenap; Sen, Hadice Selimoglu; Ece, Aydin; Uluca, Unal; Soker, Sevda; Dogan, Erdal
    The aim of this study was to examine whether dexmedetomidine improves acute liver injury in a rat model. Twenty-eight male Wistar albino rats weighing 300-350 g were allocated randomly to four groups. In group 1, normal saline (NS) was injected into the lungs and rats were allowed to breathe spontaneously. In group 2, rats received standard ventilation (SV) in addition to NS. In group 3, hydrochloric acid was injected into the lungs and rats received SV. In group 4, rats received SV and 100 mu g/kg intraperitoneal dexmedetomidine before intratracheal HCl instillation. Blood samples and liver tissue specimens were examined by biochemical, histopathological, and immunohistochemical methods. Acute lung injury (ALI) was found to be associated with increased malondialdehyde (MDA), total oxidant activity (TOA), oxidative stress index (OSI), and decreased total antioxidant capacity (TAC). Significantly decreased MDA, TOA, and OSI levels and significantly increased TAC levels were found with dexmedetomidine injection in group 4 (P < 0.05). The highest histologic injury scores were detected in group 3. Enhanced hepatic vascular endothelial growth factor (VEGF) expression and reduced CD68 expression were found in dexmedetomidine group compared with the group 3. In conclusion, the presented data provide the first evidence that dexmedetomidine has a protective effect on experimental liver injury induced by ALI.
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    Rapid and Easy Diagnosis of Netherton Syndrome with Dermoscopy
    (B C Decker Inc, 2014) Akkurt, Zeynep Meltem; Tuncel, Tuba; Ayhan, Erhan; Ucmak, Derya; Uluca, Unal; Ucak, Haydar
    Background: Netherton syndrome is a rare autosomal recessive disease demonstrating ichthyosis linearis circumflexa, atopic findings, and hair shaft anomalies. Trichorrhexis invaginata is the pathognomonic hair shaft anomaly seen in this syndrome. Objective: In recent years, hair shaft anomalies have been described as matchstick and golf tee signs. We present a patient with Netherton syndrome diagnosed by the presence of matchstick and golf tee hairs in addition to trichorrhexis invaginata.
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    A Rare Cause of Neonatal Liver Failure: Neonatal Hemochromatosis
    (Duzce Univ, 2013) Uluca, Unal; Sivash, Ercan; Erkan, Melih Engin; Kervancioglu, Selim; Karakok, Metin; Tan, Ilhan
    Neonatal hemochromatosis (NH) is a severe rare liver disease in neonatal period associated with ekstrahepatic siderosis. This disease is characterized by hepatocellular insufficiency that presented with jaundice, hypoglycemia, hypoalbuminemia, low fibrinogen levels, thrombocytopenia, anemia, direct and indirect hyperbilirubinemia from the first days of life. Herein we reported a case with Rh incompatibility whose jaundice was noted at the first day of life and referred to our hospital for exchange transfusion, but thereafter diagnosed as NH and reviewed the literature in the view point of the latest developments related to the topic.
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    A Rare Vasculitis of Childhood, Takayasu Arteritis
    (Modestum Ltd, 2013) Ece, Aydin; Sen, Velat; Yavuz, Sevgi; Gunes, Ali; Uluca, Unal; Sahin, Cahit
    Takayasu arteritis is a potentially life threatening vasculitis that characterized by granulomatous inflammation of large vessels including aorta, its major branches and pulmonary arteries. True incidence of TA is not known in children, since TA is very rare; however, incidence of TA has been estimated as 0.8-2.6 per million children. Making a diagnosis is more difficult in childhood compared with adults due to non-specific clinical findings. The diagnosis of TA is done based on clinical criteria, laboratory investigations support and radiologic images confirm the diagnosis. Corticosteroids with other immunosuppressive drugs are used for treatment and biological agents are given to resistant cases. In this report, it was aimed to draw attention to a potentially fatal disease that frequently diagnostic delays and therapeutic difficulties occur. We, herein reported signs, symptoms, imaging methods and treatment modalities of two children, a 12 year-old male and a 13 year-old female patients, that presented with non-specific initial symptoms and diagnosed as TA with clinical findings and radiological images
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    Rhizomelic Chondrodysplasia Punctata Type 1 Caused by a Novel Mutation in the PEX7 Gene
    (Galenos Yayincilik, 2015) Cim, Abdullah; Coskun, Salih; Gorukmez, Orhan; Yuksel, Hatice; Uluca, Unal; Di Pietro, Erminia; Plourde, Francois
    Peroxisomes are involved in various metabolic reactions. Rhizomelic chondrodysplasia punctata (RCDP) type 1 is one of the peroxisomal biogenesis disorders caused by mutations in the PEX7 gene and is inherited in an autosomal recessive manner. We present a nine-year-old boy with skeletal abnormalities and dysmorphic facial appearance. The patient was born to parents who were first cousins. Very-long-chain fatty acids and pristanic acid levels were in the normal range, but an elevated phytanic acid level was detected by gas chromatography/mass spectrometry. The PEX7 gene was sequenced in the patient and his parents. A novel homozygous mutation, c.192delT (p.F64Lfs*10), was identified in the patient and was present in heterozygosity in both parents. In conclusion, the clinical presentation and peroxisome profile of the patient suggest that this novel mutation leads to RCDP type 1.