Dr Behcet Uz Children Hospital

İzmir, Turkey

Dr Behcet Uz Children Hospital

İzmir, Turkey
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Atas Berksoy E.,Izmir Tepecik Training and Research Hospital | Gunay I.,Dr Behcet Uz Children Hospital | Bekem Soylu O.,Dr Behcet Uz Children Hospital | Celik T.,Dr Behcet Uz Children Hospital
Turkiye Klinikleri Pediatri | Year: 2017

Objective: A pediatric observation unit may help to alleviate some of the stress caused by an increased number of patients. The aim of this study is to identify the intended functions as well as the clinical and operating characteristics of our observation unit, which is an alternative and important option to our inpatient and emergency services. Material and Methods: The hospital records of children aged between 0 months and 18 years who were admitted to our observation unit between January 2014 and December 2014 were reviewed. The patients' demographic and clinical characteristics, diagnosis at admission, the number of patients admitted to wards, the percentage of patients referred to other hospitals, and the length of observation were analyzed. Results: A total of 115.729 patients admitted to the emergency department in a year. Of these patients, 121 (0.10%) were admitted to a ward appropriate for their age and disease, while 7007 (6.05%) were admitted to the observation unit. Of the patients monitored in the observation unit, 2110 (30.11%) were eventually admitted to clinics appropriate for their age. 1559 (26.85%) of patients admitted to the observation unit had gastrointestinal system diseases; 1270 (18.12%) had nervous system diseases; 1265 (18.05%) had respiratory system diseases; and 1137 (16.24%) had infectious diseases. We found that 5970 of 7007 (85.20%) patients admitted to the observation unit stayed less than 24 hours, and 1037 (14.79%) patients stayed more than 24 hours. The mean length of stay for all patients admitted to the observation unit was 13 hours, 28 minutes (1,40 ± 34,4). Conclusion: Our observation unit has an important role in assessing and managing children with a variety of diseases. Also; our observation unit functions as a holding unit to provide short term care of overflow inpatients. Copyright © 2017 by Türkiye Klinikleri.

Genc B.,Sifa Hospital | Solak A.,Sifa Hospital | Doksoz O.,Dr Behcet Uz Children Hospital | Tavli V.,Izmir University
Turk Kardiyoloji Dernegi Arsivi | Year: 2013

Coronary artery fistulas are rare vascular anomalies characterized by abnormal communication, devoid of a capillary system between the coronary artery and the major vessels or cardiac chambers. In this report, we present a 14-year-old male patient with ischemic symptoms, a left coronary artery to right ventricle fistula and agenesis of the right coronary artery. The anatomy of the coronary arteriovenous fistula was determined in detail through a dual source CT coronary angiography. The patient underwent open cardiac surgery because of ischemic symptoms and a residual fistula was detected after the surgery. © 2013 Turkish Society of Cardiology.

PubMed | Marmara University, Inonu University, Karadeniz Technical University, Istanbul University and 19 more.
Type: Journal Article | Journal: American journal of medical genetics. Part A | Year: 2016

To evaluate the anthropometric features of girls with Turner syndrome (TS) at birth and presentation and the effect of karyotype on these parameters. Data were collected from 842 patients with TS from 35 different centers, who were followed-up between 1984 and 2014 and whose diagnosis age ranged from birth to 18 years. Of the 842 patients, 122 girls who received growth hormone, estrogen or oxandrolone were excluded, and 720 girls were included in the study. In this cohort, the frequency of small for gestational age (SGA) birth was 33%. The frequency of SGA birth was 4.2% (2/48) in preterm and 36% (174/483) in term neonates (P<0.001). The mean birth length was 1.3cm shorter and mean birth weight was 0.36kg lower than that of the normal population. The mean age at diagnosis was 10.14.4 years. Mean height, weight and body mass index standard deviation scores at presentation were -3.11.7, -1.41.5, and 0.41.7, respectively. Patients with isochromosome Xq were significantly heavier than those with other karyotype groups (P=0.007). Age at presentation was negatively correlated and mid-parental height was positively correlated with height at presentation. Mid-parental height and age at presentation were the only parameters that were associated with height of children with TS. The frequency of SGA birth was found higher in preterm than term neonates but the mechanism could not be clarified. We found no effect of karyotype on height of girls with TS, whereas weight was greater in 46,X,i(Xq) and 45,X/46,X,i(Xq) karyotype groups.

Ebarasi L.,Uppsala University | Ebarasi L.,Karolinska Institutet | Ashraf S.,Harvard University | Bierzynska A.,University of Bristol | And 16 more authors.
American Journal of Human Genetics | Year: 2015

Nephrotic syndrome (NS), the association of gross proteinuria, hypoalbuminaemia, edema, and hyperlipidemia, can be clinically divided into steroid-sensitive (SSNS) and steroid-resistant (SRNS) forms. SRNS regularly progresses to end-stage renal failure. By homozygosity mapping and whole exome sequencing, we here identify recessive mutations in Crumbs homolog 2 (CRB2) in four different families affected by SRNS. Previously, we established a requirement for zebrafish crb2b, a conserved regulator of epithelial polarity, in podocyte morphogenesis. By characterization of a loss-of-function mutation in zebrafish crb2b, we now show that zebrafish crb2b is required for podocyte foot process arborization, slit diaphragm formation, and proper nephrin trafficking. Furthermore, by complementation experiments in zebrafish, we demonstrate that CRB2 mutations result in loss of function and therefore constitute causative mutations leading to NS in humans. These results implicate defects in podocyte apico-basal polarity in the pathogenesis of NS. © 2015 The American Society of Human Genetics.

PubMed | Dr Behcet Uz Children Hospital, Izmir University and Tepecik Research Hospital
Type: | Journal: Open journal of cardiovascular surgery | Year: 2014

Patent ductus arteriosus (PDA) is commonly seen in premature infants with low birth weights (LBW). It is a condition that has high mortality and morbidity rates. Early closure of the ductus arteriosus may require surgery or medical treatment. However, the decision of first medical approach for symptomatic PDA closure is still debated. In this study, we compared the surgical and medical treatments for the closure of PDA in premature LBW infants.This study included 27 premature infants whose birth weights were lower than 1500 g, who were born in the period between 2011 and 2013 and had symptomatic PDA. Patients were separated into two groups: groups A and B. Group A included patients whose PDAs were closed with medical treatment (n = 16), and group B included patients who had undergone surgical operations for PDA closure (n = 11).There were no statistically significant differences between groups A and B when the groups were compared in terms of birth weight, gestational age, respiratory distress syndrome (RDS), necrotizing enterocolitis (NEC), sepsis, intraventricular hemorrhage (IVH), retinopathy of prematurity (ROP), and pneumothorax. Although the mortality rate was determined to be lower in group B (2 out of 11, 18.1%) than in group A (7 out of 16, 43.7%), no statistically significant difference was found between the two groups. A statistically significant increase was determined in the incidence of kidney function loss in patient group that received Ibuprofen, a medical treatment, in comparison to the patients who had surgery.In conclusion, surgery is a safe method to repair PDA in premature LBW infants. Although there is no remarkable difference between surgery and medical treatment, we suggest that a surgical approach may be used as a first choice to repair PDA considering the lower rate of mortality and morbidity and higher rate of closure compared to medical treatment.

PubMed | Hacettepe University, University of Hamburg, University of Duisburg - Essen, Dr Behcet Uz Children Hospital and 6 more.
Type: Journal Article | Journal: Nature genetics | Year: 2016

Nucleoporins are essential components of the nuclear pore complex (NPC). Only a few diseases have been attributed to NPC dysfunction. Steroid-resistant nephrotic syndrome (SRNS), a frequent cause of chronic kidney disease, is caused by dysfunction of glomerular podocytes. Here we identify in eight families with SRNS mutations in NUP93, its interaction partner NUP205 or XPO5 (encoding exportin 5) as hitherto unrecognized monogenic causes of SRNS. NUP93 mutations caused disrupted NPC assembly. NUP93 knockdown reduced the presence of NUP205 in the NPC, and, reciprocally, a NUP205 alteration abrogated NUP93 interaction. We demonstrate that NUP93 and exportin 5 interact with the signaling protein SMAD4 and that NUP93 mutations abrogated interaction with SMAD4. Notably, NUP93 mutations interfered with BMP7-induced SMAD transcriptional reporter activity. We hereby demonstrate that mutations of NUP genes cause a distinct renal disease and identify aberrant SMAD signaling as a new disease mechanism of SRNS, opening a potential new avenue for treatment.

Buyukyavuz B.I.,Suleyman Demirel University of Turkey | Duman L.,Suleyman Demirel University of Turkey | Karaaslan T.,Suleyman Demirel University of Turkey | Turedi A.,Dr Behcet Uz Children Hospital
Turkish Neurosurgery | Year: 2012

Placement of ventriculoperitoneal(VP) shunt is a worldwide accepted procedure for treatment of hydrocephalus. This procedure have various intra-abdominal complications, of which pseudocyst formation is a rare one. Common presentations of this complication are abdominal mass, abdominal pain, intestinal obstruction, and shunt dysfunction. In this paper, we report a case of 3 year-old boy with cerebrospinal fluid (CSF) pseudocyst of the VP shunt presenting with hyponatremic seizure. To the best of our knowledge, hyponatremic seizure has not been previously reported as a presentation of abdominal CSF pseudocyst in the literature. Our case has also the largest CSF pseudocyst with respect to body surface area of the child in the literature.

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