Publications by authors named "Gulsev Kale"

69 Publications

Diffuse Alveolar Damage Correlation with Clinical Diagnosis of Pediatric Acute Respiratory Distress Syndrome.

J Pediatr Intensive Care 2021 Mar 20;10(1):52-57. Epub 2020 Jul 20.

Department of Pediatric Pathology, Hacettepe University Ihsan Dogramaci Children's Hospital, Ankara, Turkey.

Diffuse alveolar damage (DAD) is one of the pathological hallmarks of acute respiratory distress syndrome (ARDS). We aimed to compare pathological findings of DAD with clinical ARDS criteria. We re-evaluated 20 patients whose clinical autopsy revealed DAD. Total 11/20 patients with DAD (55%) met the 1994 American-European Consensus Conference and 7/17 (41%) met the 2012 Berlin clinical criteria. DAD showed only moderate correlation with current clinical ARDS definition. Oxygenation index (OI), seems to be the most valuable tool in predicting pulmonary damage severity, though OI is not listed in either of the previous definitions. We support the recommended use of OI by 2015 consensus conference.
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http://dx.doi.org/10.1055/s-0040-1714127DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7870331PMC
March 2021

Inflammatory milieu of muscle biopsies in juvenile dermatomyositis.

Rheumatol Int 2021 Jan 26;41(1):77-85. Epub 2020 Oct 26.

Pediatric Pathology Unit, Department of Pediatrics, Hacettepe University, Ankara, Turkey.

Juvenile dermatomyositis (JDM) is an inflammatory myopathy which causes severe morbidity and high mortality if untreated. In this study, we aimed to define the T-helper cell profile in the muscle biopsies of JDM patients. Muscle biopsies of twenty-six patients (50% female) were included in the study. Immunohistochemical expression of CD3, CD20, CD138, CD68, IL-17, Foxp3, IFN-ɣ, IFN-alpha and IL-4 was studied and muscle biopsies were scored using the JDM muscle biopsy scoring tool. Inflammatory cells were in small clusters in perimysium and perivascular area or scattered throughout the endomysium in most biopsies; however in 2 biopsies, lymphoid follicle-like big clusters were observed, and in one, there was a very dense and diffuse inflammatory infiltration nearly destroying all the muscle architecture. Seventy-three per cent of the biopsies had T cells, 88% had B cells, 57% had plasma cells, and all had macrophages. As for T-helper cell subtypes, 80% of the biopsies were Th1 positive, 92% Th17 positive and 30% Treg positive. No IL-4 positive inflammatory cell was detected, and only 2 biopsies showed IFN-alpha positivity. The mean JDM biopsy score was 17.6, meaning moderate to severe muscular involvement. Visual analogue score of the pathologist was strongly correlated with histopathological features. B cells, macrophages, plasma cells and T cells constitute the inflammatory milieu of the JDM muscle biopsies. As for T cells, JDM is a disease mainly related with Th1 and Th17 T-helper cell subtypes and to some extend Treg. Th2 cells are not involved in the pathogenesis.
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http://dx.doi.org/10.1007/s00296-020-04735-wDOI Listing
January 2021

LARGE expression in different types of muscular dystrophies other than dystroglycanopathy.

BMC Neurol 2018 Dec 15;18(1):207. Epub 2018 Dec 15.

Department of Medical Biology, Hacettepe University Faculty of Medicine, 06100 Sihhiye, Ankara, Turkey.

Background: Alpha-dystroglycan (αDG) is an extracellular peripheral glycoprotein that acts as a receptor for both extracellular matrix proteins containing laminin globular domains and certain arenaviruses. An important enzyme, known as Like-acetylglucosaminyltransferase (LARGE), has been shown to transfer repeating units of -glucuronic acid-β1,3-xylose-α1,3- (matriglycan) to αDG that is required for functional receptor as an extracellular matrix protein scaffold. The reduction in the amount of LARGE-dependent matriglycan result in heterogeneous forms of dystroglycanopathy that is associated with hypoglycosylation of αDG and a consequent lack of ligand-binding activity. Our aim was to investigate whether LARGE expression showed correlation with glycosylation of αDG and histopathological parameters in different types of muscular dystrophies, except for dystroglycanopathies.

Methods: The expression level of LARGE and glycosylation status of αDG were examined in skeletal muscle biopsies from 26 patients with various forms of muscular dystrophy [Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), sarcoglycanopathy, dysferlinopathy, calpainopathy, and merosin and collagen VI deficient congenital muscular dystrophies (CMDs)] and correlation of results with different histopathological features was investigated.

Results: Despite the fact that these diseases are not caused by defects of glycosyltransferases, decreased expression of LARGE was detected in many patient samples, partly correlating with the type of muscular dystrophy. Although immunolabelling of fully glycosylated αDG with VIA4-1 was reduced in dystrophinopathy patients, no significant relationship between reduction of LARGE expression and αDG hypoglycosylation was detected. Also, Merosin deficient CMD patients showed normal immunostaining with αDG despite severe reduction of LARGE expression.

Conclusions: Our data shows that it is not always possible to correlate LARGE expression and αDG glycosylation in different types of muscular dystrophies and suggests that there might be differences in αDG processing by LARGE which could be regulated under different pathological conditions.
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http://dx.doi.org/10.1186/s12883-018-1207-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6295086PMC
December 2018

Home accident or physical abuse: Evaluation of younger children presenting with trauma, burn and poisoning in the Pediatric Emergency Department.

Turk J Pediatr 2018 ;60(6):625-632

Departments of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Isıyel E, Tekşam Ö, Foto-Özdemir D, Özmert E, Tümer AR, Kale G. Home accident or physical abuse: Evaluation of younger children presenting with trauma, burn and poisoning in the Pediatric Emergency Department. Turk J Pediatr 2018; 60: 625-632. Most cases of physical abuse and neglect refer to pediatric emergency clinics, and these patients are diagnosed as only home accidents. Cases that cannot be diagnosed and managed correctly are again exposed to severe trauma and consequently, they may die. The aim of this study is to evaluate the physical abuse and neglect in children younger than three years of age, admitted to the pediatric emergency department with the complaints of trauma, burn, drug poisoning and/or caustic ingestion. This prospective study included 132 patients who were admitted to the pediatric emergency department. Children were classified into three groups as `no abuse`, `suspected abuse` and `abused` after being evaluated with a standard form. Additionally, suspected abuse and abused cases were evaluated once again by `The Team of Child Abuse and Neglect Evaluation, Research and Treatment` in Hacettepe University, İhsan Doğramacı Children's Hospital. The frequency of the suspected abuse or abused cases in all burn, trauma and poisoning cases was found to be 7.5%. It was noticed that 65 of the patients (49.2%) were physically neglected considering the mechanism of occurrence of events. Unplanned pregnancy, absence of prenatal follow-up, high number of siblings, previous physical abuse in the family, absence of witness during the event, and hospitalization were statistically significant differences between no abuse and suspected abuse or abused cases (p < 0.05). Moreover, age group and income were significant risk factors in the logistic regression model for the patients who were thought to be suspected abuse/abused in univariate analyses (age group: Odds ratio (OR) 0.279, 95% confidence interval (CI) 0.085-0.723, p=0.0049; income: OR 2.323, 95% CI 1.052-6.198, p=0.0345). In conclusion, most cases of physical abuse and neglect are referred to the pediatric emergency clinics, and these patients are misdiagnosed as home accidents. The physicians working in the emergency department should be informed and trained in recognizing the cases of abuse and neglect, making the differential diagnosis, identifying the high-risk families and appropriate physical and psychological treatment for the abused and neglected children.
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http://dx.doi.org/10.24953/turkjped.2018.06.002DOI Listing
January 2018

Clinical features and treatment results in children with anaplastic large cell lymphoma.

Turk J Pediatr 2015 Sep-Oct;57(5):458-66

Division of Pediatric Oncology, Hacettepe University Faculty of Medicine, Ankara Turkey.

Anaplastic large cell lymphoma (ALCL) tends to have frequent relapse and good response to salvage chemotherapy. The frequency of ALCL among 1486 Non-Hodgkin's lymphoma (NHL) cases followed-up since 1972 was 1.5%, however, the percentage was 9.3% in cases diagnosed after 2000. Event-free survival (EFS) and overall survival (OS) rates for 23 children were 32.2% and 72.8% at 3 years, respectively. Disseminated diseases, no response to first line treatment, anaplastic lymphoma kinase (ALK) negativity were found as significant predictors on survival of ALCL. The proper diagnosis and early referral is essential in these children for a better survival rate. The children with ALK negative status should be monitored carefully because of the poor prognostic factors, and treated differently. The survival rates in this study are need of further improvement since the survival rates with current protocols are achievable at a level more than 80%. This is mainly related with late referral of those children with advanced disease.
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September 2017

Final diagnosis of children and adolescents with musculoskeletal complaints.

Minerva Pediatr 2017 Feb 10;69(1):50-58. Epub 2015 Sep 10.

Division of Pediatric Nephrology and Rheumatology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Background: Musculoskeletal complaints (MSCs) are one of the leading causes of outpatient admissions. However, analytical and epidemiological data are limited. The aim of this study is to identify the etiology of MSCs (excluding acute traumatic conditions) in children and adolescents, and to identify clues for the differential diagnosis.

Methods: Children and adolescents presenting with musculoskeletal pain, swelling or limitation of movement were enrolled in a prospective design. Demographic, clinical and laboratory features were recorded.

Results: Four hundred and twenty-two children (48.2% female) with a mean age of 7.90±3.95 years were enrolled. Etiology was identified in 97.2% of the cases: non-inflammatory and mechanical conditions (NIMC; 42.2%), rheumatic diseases (RD; 31%), infection-related disorders (IRD; 21.6%) and malignancy (M; 2.4%). NIMC was characterized by longer duration of complaints, a higher rate of non-articular complaints, a lower rate of joint involvement and limping and lower levels of leukocytes, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). The prevalence of RD was higher in the age group of >12 years; younger age was associated with higher prevalence of IRD. Small-joint involvement was highest in the RD group. Median ESR in RD and M groups was higher; compared to the other groups; the frequency of patients with ESR ≥ 60 mm/hr was higher in the M group; compared to the RD group. In the RD group familial Mediterranean fever (9.7%), juvenile idiopathic arthritis (8.3%) and Henoch-Schönlein purpura (5.7%) were the leading causes of MSCs.

Conclusions: RD accounted for one-third of the etiologies for MSCs. Age, duration of complaints, pattern of joint involvement and acute phase reactants are practical tools that may guide the pediatrician for diagnosis.
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http://dx.doi.org/10.23736/S0026-4946.16.04216-XDOI Listing
February 2017

The efficacy of delayed surgery in children with high-risk neuroblastoma.

J Cancer Res Ther 2015 Apr-Jun;11(2):268-71

Department of Pediatric Oncology, Institute of Oncology, Ankara, Turkey.

Context: Surgery is an important part of treatment in children with neuroblastoma; however, exact timing is unclear. Both initial and delayed surgery was suggested as the best by numerous studies.

Aims: Thus, we aimed to investigate the role of delayed surgery on 31 children with high-risk neuroblastoma.

Materials And Methods: Thirty-one children with high-risk neuroblastoma were enrolled into the study.

Statistical Analysis Used: Statistical analysis was performed using Statistical Package for the Social Sciences (SPSS) for windows 10.0.

Results: There 'were 15 male and 16 female patients with a median age of 3.0 ± 3.2 years. Primary tumor site was adrenal in 27, non-adrenal in two, pelvic in one, and mediastinal in one patient. MYCN gene was amplified in four and non-amplified in 11 children on totally 15 children with available data. Lactate dehydrogenase was elevated in 30 children. The tumor volumes at diagnosis and before surgery in the whole group were 154.3 and 12.5 mL, respectively. The decline in tumor volume was statistically significant (P < 0.0001). Initial surgery was performed in three and delayed in 20 children, and eight children were inoperable. Surgical complication rate was 66.6% (two out of three patients) in initial surgery group; however, the rate was 15% (3 out of 20 patients) in delayed surgery group. The 5-year event-free survival and overall survival rates in the whole group were 44.8% and 50.8%, respectively. Primary tumor area control rate was 95%

Conclusions: In conclusion, the delayed surgery with intensive chemotherapy and radiotherapy has been successful for primary control in high-risk neuroblastoma patients.
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http://dx.doi.org/10.4103/0973-1482.151852DOI Listing
April 2016

Placental findings of IUGR and non-IUGR.

Turk J Pediatr 2014 Jul-Aug;56(4):368-73

Neonatology Unit, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

This study aims to define the placental findings in intrauterine growth restriction (IUGR). The study group consisted of 40 neonates without IUGR and 24 neonates with IUGR, including their placentas. The cases were separated into three groups according to gestational age. Group A (n=21): 37 ≤ weeks, Group B (n=27): 32≤ - <37 weeks, Group C (n=16): < 32 weeks. Each group had two subgroups, IUGR and non-IUGR. The placentas from the non-IUGR subgroup were heavier than the IUGR subgroup placentas in the term group (p<0.05). Infarct (over 5%), increased syncytial knots and histiocytic intervillositis were more common in the IUGR cases (p<0.05). This study shows that chronic patterns of injury are significantly increased in placentas from pregnancies complicated by IUGR. If the injury in the placenta is acute or mild, fetal adaptation can compensate and prevent fetal growth restriction.
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April 2016

Burnout syndrome during residency in internal medicine and pediatrics in a country without working time directive.

Int J Health Care Qual Assur 2014 ;27(3):223-30

Department of Internal Medicine, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Purpose: The purpose of this paper is to demonstrate burnout syndrome among internal medicine and pediatrics residents in a country that does not have the working time directive (WTD) and also to determine the risk factors and consequent impact on efficient functioning in clinical areas.

Design/methodology/approach: A 57-item questionnaire was given to internal medicine and pediatrics residents. Responses from 22 pediatrics and 33 internal medicine residents were evaluated.

Findings: Demographic findings, burnout scores, having hobbies, social activities and reading books unrelated to medicine were similar between the two groups. Six pediatrics residents (27.3 per cent) and 11 (33.3 per cent) internal medicine residents met the criteria for clinically significant burnout. Personal accomplishment scores and reading books unrelated to medicine were found to be related to burnout.

Originality/value: Burnout is a syndrome characterized by depersonalization, emotional exhaustion and a low sense of personal accomplishment. It is important to document burnout in countries where WTDs are not implemented. Further studies might demonstrate burnout's effect on patient safety, service quality and physician's performance.
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http://dx.doi.org/10.1108/IJHCQA-12-2012-0127DOI Listing
October 2016

Studying cytokines of T helper cells in the kidney disease of IgA vasculitis (Henoch-Schönlein purpura).

Pediatr Nephrol 2015 Aug 4;30(8):1269-77. Epub 2015 Feb 4.

Department of Pediatric Nephrology, Faculty of Medicine, Hacettepe University, Sıhhiye, Ankara, 06100, Turkey,

Background: Immunoglobulin A vasculitis (IgA-V), formerly known as Henoch-Schönlein purpura (HSP), is the most common small vessel vasculitis in children. In recent years, the role of T cells in the pathogenesis of HSP/IgA-V has become a focus of research.

Methods: Renal biopsy specimens from 22 pediatric patients diagnosed with Henoch-Schönlein nephritis (patient group) were compared to normal renal tissue in nephrectomy specimens from 20 pediatric patients diagnosed with Wilms tumor (control group). All renal specimens were scored according to International Study of Kidney Disease in Children (ISKDC) and Oxford classification. Immunohistochemical analyses of interferon-gamma (IFN-γ), interleukin (IL)-4, IL-17 and FOXP3 expression were performed.

Results: All glomeruli and tubules of the HSP/IgA-V patients showed significantly higher IFN-γ and IL-17 expression than those of the control group. Glomerular IFN-γ and IL-17 staining grades correlated with the urinary protein/creatinine ratio (r = 0.62, p = 0.02 and r = 0.507, p = 0.016, respectively). IL-17 expression also correlated with the percentage of crescents (r = 0.518, p = 0.014). IL-4 staining was present in only nine of the 22 patient biopsies and did not correlate with any of the parameters studied. Interstitial areas of patient biopsies had more FOXP3+ cells/μm(2) than those of the control group (p < 0.001), but differences in glomerular and tubular FOXP3+ levels (cells/μm(2)) between the two groups were not statistically different. The ISKDC and Oxford scores did not correlate with any parameter studied. However, endocapillary hypercellularity did correlate with IFN-γ expression.

Conclusions: Based on these results, we conclude that IFN-γ and IL-17 contribute to HSP/IgA-V in children.
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http://dx.doi.org/10.1007/s00467-015-3051-4DOI Listing
August 2015

Clinical features and treatment results of children with diffuse large B-cell lymphoma.

Pediatr Hematol Oncol 2014 Sep 12;31(6):509-17. Epub 2014 Aug 12.

Department of Pediatric Oncology, Hacettepe University Faculty of Medicine , Ankara , Turkey.

The demographic, clinical characteristics, and treatment groups of 33 children with diffuse large B-cell lymphoma (DLBCL) were recorded and analyzed among 1486 non-Hodgkin lymphoma (NHL) cases since 1972. The median age was 9.7 years (range 1.4-16.9) and male/female ratio was 24/9 = 2.6. Kaplan-Meier methods and logrank tests were used in treatment analysis. The frequency of DLBCL among 1486 NHL cases was 2.2%, however, the percentage was 9.3% in cases diagnosed after 2000. The event-free survival (EFS) and overall survival (OS) rates for 33 children were 61% and 65.1% at 5 years, respectively. The EFS and OS rates of low stage (stages I and II) disease decreased to lower level in advanced stage (stages III and IV) disease. Associated conditions and ages older than 14 years were found as poor prognostic factors in multivariate analysis. The survival rates in children with DLBCL need further improvement. This is mainly related with late referral of those children with advanced disease. The proper diagnosis and early referral is essential in these children for a better survival rate. The children with associated conditions and older children must be handled with care since these are found as poor prognostic factors.
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http://dx.doi.org/10.3109/08880018.2014.940434DOI Listing
September 2014

Primary liver tumors in children: Hacettepe experience.

Turk J Pediatr 2014 Jan-Feb;56(1):1-10

Divisions of Pediatric Oncology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

We aimed to review our experience with the clinical characteristics and outcome in childhood liver tumors. We investigated the clinical, laboratory and pathological characteristics, treatments and outcome in hepatoblastomas (HBL) and hepatocellular carcinomas (HCC). We identified 91 HBL and 42 HCC cases. Distant metastases were detected in 16% of HBLs and 22% of HCCs. PRETEXT stages were I/II in 34% and III/IV in 66% of HBLs and I/II in 16% and III/IV in 84% of HCCs. Most cases received cisplatin + doxorubicin chemotherapy. At a median of 58 months, 90 cases had died, 28 were alive, and 15 were lost to follow-up. Five-year survival rates were 32.4% for all HBLs and 15.6% for HCCs. Five-year survival rates were 47% in HBLs and 22.8% in HCCs diagnosed after 1990. In HBLs, distant metastases and absence of chemotherapy response indicated poor prognosis. Prognosis for childhood liver tumors has improved over the last two decades with preoperative chemotherapy with cisplatin + doxorubicin. Surgical resectability is important for cure. For HCC, more effective chemotherapy approaches are essential.
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September 2015

Studying IFN-gamma, IL-17 and FOXP3 in pediatric lupus nephritis.

Pediatr Nephrol 2014 May 31;29(5):853-62. Epub 2014 Jan 31.

Department of Pediatrics, Hacettepe University Children's Hospital, Ankara, Turkey.

Background: We studied the cytokines secreted by the inflammatory T cell subgroups (IFN-γ and IL-17) and FOXP3 expression in lupus nephritis (LN) and analyzed associations with clinical and histopathological parameters.

Methods: Renal tissue samples of 39 LN patients were studied. Immunohistochemical staining was carried out with antibodies against IFN-γ, IL-17, and FOXP3.

Results: Both IFN-γ (+) and IL-17+ cells were statistically higher in LN tissues when compared with controls (p < 0.01). The cells in the tubulointerstitium were CD3 + CD4+ displaying a Th1 and Th17 phenotype, whereas the less intense population in the glomeruli was CD3-CD4-. Interstitial CD3 + CD4+ FOXP3+ cells were also significantly higher in LN biopsies than in control tissues (p < 0.01). IFN-γ (+) and IL-17+ cells were more intense among class IV LN as compared to class II, III LN (p < 0.01 and p = 0.001, respectively). Subsequently, when IL-17 and IFN-γ staining was compared between the proliferative LN classes, class III and IV patients had more intense staining compared to class II (all p < 0.05). IFN-γ immunostaining correlated positively with serum creatinine and negatively with albumin levels and glomerular filtration rate (GFR). IL-17 immunostaining correlated with proteinuria, requirement for pulse steroids, and SLEDAI renal score, and negatively with GFR. Furthermore, glomerular and interstitial IL-17 and IFN-γ stainings were significantly associated with various parameters of histological activity (p < 0.05).

Conclusion: We suggest that IFN-gamma and IL-17 could have a role in the pathogenesis and progression of LN. The Th1 and Th17 cells may be imperative in the severity of LN. Recognizing the complexity of the immune pathways involved in lupus reminds us that targeting B cells only may not suffice to control the progression of the inflammation.
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http://dx.doi.org/10.1007/s00467-013-2695-1DOI Listing
May 2014

Munchausen syndrome by proxy presented as recurrent respiratory arrest and thigh abscess: a case study and overview.

Turk J Pediatr 2013 May-Jun;55(3):337-43

Department of Child and Adolescent Psychiatry, Hacettepe University Faculty of Medicine, Ankara, Turkey.

While many physicians are familiar with the sexual or physical abuse of children, there is little awareness about Munchausen syndrome by proxy (MSBP). As case reports of MSBP increase, awareness among physicians is thought to increase as well. We thus present herein a 16-month-old girl who admitted to Hacettepe University İhsan Doğramacı Children's Hospital with the complaint of seizure, recurrent apnea and thigh abscess, who was later diagnosed as MSBP. The case was being followed by the Child Protection Team of the hospital (Haceteppe University Child Protection Unit [HU-CPU]). HU-CPU contributed to the early detection of this case and protected the child from a possible fatal outcome. The mother was confronted for MSBP and refused to take responsibility for her child's symptoms. As seen in this case, when MSBP is suspected, psychiatric evaluation of the mother, evaluation of the mother-child interaction and collection of a detailed family and social history can have a positive impact on the prognosis in these cases. This case report underlines the importance of multidisciplinary team work to share the responsibility and reduce the burden during the treatment process of these difficult and complicated cases.
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July 2014

Serum alpha-fetoprotein levels in neonatal cholestasis.

Turk J Pediatr 2013 Mar-Apr;55(2):152-7

Division of Pediatric Gastroenterology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Alpha-fetoprotein (AFP) is used as a tumor marker for hepatocellular carcinoma, hepatoblastoma and germ cell tumors. It may also be elevated in infants with some hepatobiliary disorders. The mechanism of AFP elevation in neonatal cholestasis is not known. We retrospectively evaluated serum AFP levels in 53 infants with neonatal cholestasis. Thirty patients (56.6%) had elevated AFP, and the ratio of patients with elevated AFP was significantly high in both the metabolic diseases and idiopathic neonatal hepatitis groups (p=0.021). Serum aspartate aminotransferase (AST) levels increased significantly in patients with elevated AFP (p=0.004). Steatosis was the distinctive histopathological finding of the patients with high AFP. The patients with steatosis had significantly higher standard deviation (SD) score of AFP than the patients without steatosis (p=0.001). We have shown AFP elevation in neonatal cholestasis due to metabolic disorders and idiopathic neonatal hepatitis and its association with steatosis and AST elevation.
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December 2013

Clinicopathological and immunohistological features in childhood IgA nephropathy: a single-centre experience.

Clin Kidney J 2013 Apr 24;6(2):169-175. Epub 2013 Feb 24.

Department of Pediatric Nephrology and Rheumatology , Faculty of Medicine, Hacettepe University , Ankara , Turkey.

Background: IgA nephropathy is a glomerular disease diagnosed by renal biopsy and is characterized by a highly variable course ranging from a completely benign condition to rapidly progressive renal failure. We aimed to evaluate the clinical, histopathological and inflammatory characteristics of children with IgA nephropathy.

Methods: Data of 37 patients with IgA nephropathy diagnosed between the years 1980 and 2008 were retrospectively reviewed. Immunohistochemistry was performed in 24 patients. Expression of CD3, CD4, CD8, CD20, CD68, IL-1β, IL-10, IL-17, TGF-β, TNF-α and the newly proposed tubulointerstitial fibrosis marker nestin were evaluated.

Results: The median age at diagnosis was 10 years. Recurrent macroscopic haematuria (66%) was the most common clinical manifestation, and 35% of the patients had synpharyngitic presentation. A significant correlation was found between proteinuria and increase in mesangial matrix ( = 0.406, P = 0.013). The presence of CD4+ T lymphocytes and CD68+ macrophages were also significantly associated with proteinuria >1 g/day. While cytokines IL-1β, IL-10 and TNF-α were mainly expressed in tubular epithelial cells, TGF-β was evident in glomeruli but they had no correlation to clinical features and severity of the disease. Nestin was detected at the tubules in almost half of the patients with no correlation to proteinuria and tubulointersititial fibrosis.

Conclusions: We found a correlation between proteinuria and mesangial matrix expansion. The presence of CD4+ T-lymphocytes and CD68+ macrophages were also significantly associated with proteinuria >1 g/day. Although there are many evidences, for immunological basis of IgA nephropathy, the immunological markers were not fully expressed in children to evaluate glomerular and tubulointerstitial inflammation, and progression of the disease. Further studies with the extended number of children are needed to shed light on the immunological basis of the disease.
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http://dx.doi.org/10.1093/ckj/sft004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3811980PMC
April 2013

Mutations in KLHL40 are a frequent cause of severe autosomal-recessive nemaline myopathy.

Am J Hum Genet 2013 Jul 6;93(1):6-18. Epub 2013 Jun 6.

Western Australian Institute for Medical Research and the Centre for Medical Research, University of Western Australia, Nedlands, Western Australia 6009, Australia.

Nemaline myopathy (NEM) is a common congenital myopathy. At the very severe end of the NEM clinical spectrum are genetically unresolved cases of autosomal-recessive fetal akinesia sequence. We studied a multinational cohort of 143 severe-NEM-affected families lacking genetic diagnosis. We performed whole-exome sequencing of six families and targeted gene sequencing of additional families. We identified 19 mutations in KLHL40 (kelch-like family member 40) in 28 apparently unrelated NEM kindreds of various ethnicities. Accounting for up to 28% of the tested individuals in the Japanese cohort, KLHL40 mutations were found to be the most common cause of this severe form of NEM. Clinical features of affected individuals were severe and distinctive and included fetal akinesia or hypokinesia and contractures, fractures, respiratory failure, and swallowing difficulties at birth. Molecular modeling suggested that the missense substitutions would destabilize the protein. Protein studies showed that KLHL40 is a striated-muscle-specific protein that is absent in KLHL40-associated NEM skeletal muscle. In zebrafish, klhl40a and klhl40b expression is largely confined to the myotome and skeletal muscle, and knockdown of these isoforms results in disruption of muscle structure and loss of movement. We identified KLHL40 mutations as a frequent cause of severe autosomal-recessive NEM and showed that it plays a key role in muscle development and function. Screening of KLHL40 should be a priority in individuals who are affected by autosomal-recessive NEM and who present with prenatal symptoms and/or contractures and in all Japanese individuals with severe NEM.
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http://dx.doi.org/10.1016/j.ajhg.2013.05.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3710748PMC
July 2013

The analysis of child abuse and neglect cases assessed by a multidisciplinary study group between 2005-2008.

Turk J Pediatr 2012 Jul-Aug;54(4):333-43

Department of Child and Adolescent Psychiatry, Hacettepe University Faculty of Medicine, Ankara, Turkey.

This study aimed to evaluate the diagnosis, treatment and follow-up of child abuse and neglect (CAN) cases. Subjects were 102 cases that were clinically assessed by the multidisciplinary hospital team. Early and late childhood cases (according to the age at first abuse) were compared by means of characteristics of abuse. Among the 102 subjects, 64 were female and 38 were male (mean age: 8.68 years). Being abused by someone within the family was found to be significantly higher in preschool children compared to children in late childhood. 27.5% (n: 28) of the cases concomitantly had low socioeconomic and educational level and high parental psychological problems and 64.8% had psychiatric problems. A limited number of studies have compared characteristics of early and late childhood abuse considering the age of onset of maltreatment. Consistent with the literature, our study showed that early age of onset of maltreatment is a poor prognostic factor in many ways.
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August 2013

Malignant sacrococcygeal germ cell tumors in children: a 30-year experience from a single institution.

Tumori 2013 Jan-Feb;99(1):51-6

Institute of Oncology, Department of Pediatric Oncology, Hacettepe University, Ankara, Turkey.

Background: Our aim was to analyze treatment results and survival characteristics of our patients with malignant sacrococcygeal germ cell tumors.

Procedure: Patient files of children with malignant sacrococcygeal germ cell tumors, treated at our institution between 1979 and 2009, were searched. Patient characteristics, histopathological subtypes, extension of disease, alpha-fetoprotein (AFP) level at the time of diagnosis and relapse, extent of surgical resection, chemotherapy protocols, details of radiotherapy and survival characteristics were recorded.

Results: A total of 58 patients (M/F = 20/38) with malignant sacrococcygeal germ cell tumor was included in analysis. With a mean follow-up of 156 months (range, 26 days to 288.8 months) overall and event-free survival rates of the 58 patients were 50.9% and 43.8%, respectively. AFP status of the patients (37% in patients with <10,000 ng/ml, 68.9% in patients with ≥ 10,000 ng/ml), type of resection (total vs others), coccygeal resection, chemotherapy protocol (PEB vs others) and number of chemotherapy courses had an impact on event-free survival in univariate analysis. In multivariate analysis, AFP status had the greatest effect on prognosis.

Conclusions: Our treatment results are worse than those reported in the literature. Elevated AFP level at the time of diagnosis had a beneficial effect on prognosis, but year of diagnosis, tumor stage, presence of metastasis, tumor size and histopathological subtype had no impact on survival in patients with malignant sacrococcygeal germ cell tumors.
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http://dx.doi.org/10.1700/1248.13788DOI Listing
May 2013

Role of CXCR1 (CKR-1) in inflammation of experimental mesangioproliferative glomerulonephritis.

Ren Fail 2013 22;35(3):380-5. Epub 2013 Jan 22.

Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

CXCR1 (CKR-1), a receptor of IL-8, is expressed in various cells including neutrophils and monocytes, both of which play a major role in proliferating glomerular diseases. We investigated time-dependent expression of CXCR1 and the effect of single-dose cyclosporine A (CsA) treatment on this expression in experimental mesangioproliferative glomerulonephritis induced by anti-thymocyte serum (ATS). Wistar rats were divided into three groups. Group 1 (control, n = 24) received non-immune serum. Group 2 (nephritis, n = 24) received ATS. Group 3 (nephritis + CsA, n = 24) received ATS and CsA concomitantly. Kidneys from six rats in each group were removed at sixth hour, 3 days, 5 days, and 7 days. ATS induced proteinuria compared to controls (p < 0.001) and CsA precluded the development of proteinuria. Glomerular inflammation and mesangial proliferation were significantly higher in ATS group than control and CsA-treated rats (p < 0.001). ATS injection caused marked interstitial inflammation that was precluded by CsA (p < 0.001). CXCR1 was not expressed in control kidneys. However, ATS induced expression of CXCR1 in both glomeruli and tubulointerstitium. CsA treatment precluded CXCR1 expression in both glomeruli and tubulointerstitium only in the first 6 h. CXCR1 may contribute to inflammation in experimental mesangioproliferative glomerulonephritis. CsA may be beneficial by inhibiting CXCR1 expression and corresponding inflammation.
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http://dx.doi.org/10.3109/0886022X.2012.760410DOI Listing
December 2013

Thirty-three-year experience on childhood poisoning.

Turk J Pediatr 2012 May-Jun;54(3):251-9

Department of Pediatrics, Ihsan Doğramaci Children's Hospital, Hacettepe University Faculty of Medicine, Ankara, Turkey.

By comparing our data for the period 1985-2008 with findings from a previous report covering the period 1975-1984, we aimed to share our experience with poisoning cases in order to contribute toward its prevention, diagnosis and treatment. The records of patients admitted to the Pediatric Intensive Care Unit with acute poisoning between November 1985 and October 2008 were evaluated retrospectively. The records of 2251 patients with acute poisoning could be retrieved. Poisoning mostly occurred in the home (92%), via the oral route (92.5%) and by a single intoxicant (81.3%). Two distinct peaks were observed: in boys between 1-5 years of age and in girls between 13-16 years of age. It was noted that 67.4% of poisoning cases were accidental, whereas 25.9% were suicidal and 6.7% were a result of a therapeutic error. Nearly two-thirds (64%) of cases were drug-related, while 36% were non-drug-related. Analgesics-antipyretics ranked first among the drug-related cases, whereas ingestion of a corrosive substance was most common among cases with non-drug poisoning. Colchicine was associated with the highest fatality, while among the causes of non-drug poisoning, carbon monoxide was the deadliest. The overall mortality rate in this study was 1.9%. Mortality from non-drug poisoning was higher than from drug-related causes (3.9% vs. 1.3%). Almost all cases of poisoning below the age of 6 years are potentially preventable. The results of this study highlight the need for reforms in industrial and health policies, with the aim of increasing awareness regarding potential toxins, appropriate storage of potential toxins, and general precautions to promote safety in the home.
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December 2012

Different features of lung involvement in Niemann-Pick disease and Gaucher disease.

Respir Med 2012 Sep 10;106(9):1278-85. Epub 2012 Jul 10.

Hacettepe University, Department of Pediatric Pulmonology, Ankara, Turkey.

Background: Niemann-Pick disease (NPD) and Gaucher disease (GD) are well-known lysosomal storage diseases. Respiratory system involvement is an important cause of morbidity and mortality in patients with NPD and GD.

Objectives: We tried to assess the clinical, radiological, and histological features of GD and NPD patients with lung involvement.

Methods: We reviewed medical history, physical examination, radiological, and histological data of 10 NPD and 7 GD patients.

Results: The most common respiratory symptoms were recurrent lung infection and dyspnea. Although lung examination results in 6 NPD patients were normal, they had lung involvement; 3 patients were diagnosed as NPD directly via lung biopsy during investigation of recurrent lung infection or interstitial lung disease. All GD patients but 1 had respiratory system symptoms at the time of diagnosis. Hepatopulmonary syndrome was present in 4 GD patients. A ground-glass pattern and atelectasis were 2 important high-resolution computed tomography features in the NPD and GD patients. Flexible bronchoscopy and bronchoalveolar lavage were used for emergency extraction of bronchial casts in 1 NPD patient.

Conclusions: Lung involvement in NPD and GD patients should be included in the differential diagnosis of interstitial lung disease. Besides interstitial appearance on HRCT, atelectasis related to bronchial cast and bronchiectasis are other radiological findings in these group of patients. Analysis of bronchoalveolar fluid and lung biopsy provide very important clues for diagnosis. Hepatopulmonary syndrome is an important vascular complication observed in GD patients.
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http://dx.doi.org/10.1016/j.rmed.2012.06.014DOI Listing
September 2012

Varying clinical features of Turkish Kawasaki disease patients.

Turk J Pediatr 2012 Jan-Feb;54(1):1-6

Division of 1Pediatric Nephrology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Kawasaki disease (KD) is an acute, systemic and self-limited vasculitis that is complicated with the development of coronary artery (CA) aneurysms. We present the clinical features of Turkish KD patients from a tertiary referral center. When 33 KD patients were assessed, a number of features stood out as differing from the expected, for example, periungual peeling 7.5 +/- 7.5 days after fever onset--42.4% of patients had periungual peeling within 14 days after fever onset. CA involvement was detected at an average of 12.3 +/- 7.9 days after fever onset. Fifty percent of the patients had been diagnosed to have CA involvement within eight days after the onset of fever. The performance of criteria suggested by American Heart Association was satisfactory, with 19 of 29 patients (65.5%) having three or more of the required laboratory features (sensitivity 65.5%). We believe Turkish patients may present differences in the course of KD.
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April 2012

Solid tumors in Turkish children: a multicenter study.

World J Pediatr 2013 Feb 21;9(1):25-31. Epub 2011 Nov 21.

Pathology Department, Ankara Child Diseases Hematology Oncology Research and Training Hospital, Ankara, Turkey.

Background: This paper presents a detailed incidence study on childhood solid tumors comprising a histopathology-based documentation of benign and malignant lesions.

Methods: The Ankara Pediatric Pathology Working Group collected databases of pediatric solid tumors from six pediatric reference centers in order to analyze the incidence, distribution and some epidemiologic characteristics of the tumors and to establish a multicenter database for further studies. A five-year retrospective archive search was carried out. Excluding epithelial tumors of the skin, leukemia, lymphoreticular system neoplasias, metastatic tumors, and hamartomas, 1362 solid tumors in 1358 patients were classified according to age, sex, localization, histopathology and clinical behavior.

Results: The male/female ratio was 0.9; 14.8% (201) of the patients belonged to 0-1 year age group, 20.7% (281) to 2-4 years, 25.9% (352) to 5-10 years, 22.2% (301) to 11-14 years, and 16.4% (223) to 15-18 years. Among all tumors, 708 (52.0%) were malignant, 645 (47.4%) benign tumors, 2 (0.1%) borderline tumors, and 2 (0.1%) unknown behavioral tumors. Malignant tumors were found in 50.2% (357) of female patients and in 54.0% (349) of male patients. A balanced distribution between benign and malignant entities among children under 18 years was observed. Comparison between the age groups revealed malignant cases outnumbered benign cases under 4 years of age while benign tumor numbers increased after 10 years of age. The most common entities in the malignant group were of sympathetic nervous system origin, while soft tissue tumors far outnumbered the others in the benign group.

Conclusions: We conclude that the cancer patterns of children in the Ankara region mostly resemble with those of the western population. This study provides useful information on the diagnosis of solid tumors in children and highlights variations in cancer incidence in different age groups.
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http://dx.doi.org/10.1007/s12519-011-0323-3DOI Listing
February 2013

A congenital muscular dystrophy with mitochondrial structural abnormalities caused by defective de novo phosphatidylcholine biosynthesis.

Am J Hum Genet 2011 Jun;88(6):845-851

National Institute of Neuroscience, Department of Neuromuscular Research, National Center of Neurology and Psychiatry, Tokyo 1878502, Japan. Electronic address:

Congenital muscular dystrophy is a heterogeneous group of inherited muscle diseases characterized clinically by muscle weakness and hypotonia in early infancy. A number of genes harboring causative mutations have been identified, but several cases of congenital muscular dystrophy remain molecularly unresolved. We examined 15 individuals with a congenital muscular dystrophy characterized by early-onset muscle wasting, mental retardation, and peculiar enlarged mitochondria that are prevalent toward the periphery of the fibers but are sparse in the center on muscle biopsy, and we have identified homozygous or compound heterozygous mutations in the gene encoding choline kinase beta (CHKB). This is the first enzymatic step in a biosynthetic pathway for phosphatidylcholine, the most abundant phospholipid in eukaryotes. In muscle of three affected individuals with nonsense mutations, choline kinase activities were undetectable, and phosphatidylcholine levels were decreased. We identified the human disease caused by disruption of a phospholipid de novo biosynthetic pathway, demonstrating the pivotal role of phosphatidylcholine in muscle and brain.
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http://dx.doi.org/10.1016/j.ajhg.2011.05.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3113344PMC
June 2011

Papillary thyroid carcinoma does not have standard course in children.

Pediatr Surg Int 2011 Sep 24;27(9):931-6. Epub 2011 May 24.

Department of Pediatric Surgery, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Background And Purpose: Papillary thyroid carcinoma has a favorable outcome in children. Recent experience with young children cases with early recurrences, after the total thyroidectomy and excision of palpable lymph nodes, in contrast to usual course of disease in adolescent cases, prompted us to review our experience on papillary carcinoma surgery.

Materials And Methods: Sixteen children who underwent surgery for papillary carcinoma between 1997 and 2010 were included. The charts and surgery notes were evaluated retrospectively and age, sex, complaints and physical findings at presentation, past medical history, results of laboratory tests, imaging findings, aspiration biopsy, extent of disease, details of surgery, complications and postoperative course were noted.

Results: Male to female ratio was 3:1. The mean age was 10.9 years (range, 5-16). The presenting symptom was palpable swelling in the neck (n = 12) or asymptomatic nodule was detected incidentally (n = 4). Physical examination findings were palpable nodule (n = 7), lymphadenopathy (n = 6) or both (n = 4). Thyroid ultrasound (US) revealed nodule (n = 9), heterogenisity of the parenchyma and enlargement of thyroid (n = 9), and microcalcifications (n = 5). Fine-needle aspiration biopsy was performed in 12 patients and was suggestive for malignancy in most cases (91%). Pulmonary metastasis was detected at presentation in two patients and appeared after thyroidectomy in one patient. Complementary thyroidectomy was performed in nine patients because the total thyroidectomy with or without cervical lymph node dissection was the treatment of choice (n = 15). Iodine scan was performed 4 weeks later following thyroidectomy. Radioactive iodine ablation (RIA) therapy was given to 11 patients who had residual or recurrent disease. Postoperative complications were permanent hypoparathyroidism (n = 4), recurrent laryngeal nerve injury (n = 3), lymphorea (n = 1) and Horner's syndrome (n = 1).

Conclusion: Total thyroidectomy and excision of affected lymph nodes is the current mode of surgical management for thyroid papillary carcinoma in children. However, especially in young children (<10 years), modified lymph node dissection should be added to total thyroidectomy in order to avoid leaving the residual tumor foci which can impair the efficacy of the ablation therapy.
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http://dx.doi.org/10.1007/s00383-011-2932-2DOI Listing
September 2011

Prenatal echocardiographic diagnosis of cardiac right/left axis and malpositions according to standardized Cordes technique.

Anadolu Kardiyol Derg 2011 Mar 8;11(2):131-6. Epub 2011 Feb 8.

Department of Pediatric Cardiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Objective: The aim of this study was to evaluate distinguishing the right/left side of the fetus, cardiac axis and position according to the standardized Cordes technique in 20 cases with cardiac malposition.

Methods: We studied retrospectively 1536 cases whose fetal echocardiographic examinations were performed between 1999 and 2006 in prenatal cardiology unit. Among these, cardiac malpositions were determined in 20 cases. The cardiac axis and position were determined according to the Cordes technique. All cases were followed-up by serial fetal echocardiograms until birth or intrauterine death occurred. In cases of intrauterine death, an autopsy was performed. After birth, physical and echocardiographic examinations were done and prenatal and postnatal diagnoses were compared.

Results: Of 1536 fetal echocardiograms performed, 144 revealed congenital heart diseases (9.4%), among these cases 20 were diagnosed with cardiac malposition. Of cases with cardiac malposition, 16 had congenital heart disease, and four had extracardiac malformation. There were six cases of isolated dextrocardia, three cases of situs inversus totalis, six cases of situs ambiguous, and one case of situs inversus with isolated levocardia. Of four cases with extracardiac malformations, two cases had mesoposition, one had dextroposition, and one had extreme levoposition. In six cases the autopsy findings were the same as that their prenatal echocardiographic findings. When postnatal echocardiographic results of the remaining cases with cardiac malposition due to congenital heart disease were compared with prenatal diagnoses, the same echocardiographic findings were verified.

Conclusion: The fetal right/left axis must be determined correctly for the accurate diagnosis of cardiac malpositions. Therefore, we recommend that Cordes technique provides a simple and reliable determination of the fetal right/left axis and fetal situs.
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http://dx.doi.org/10.5152/akd.2011.033DOI Listing
March 2011

Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants.

Turk J Haematol 2010 Dec;27(4):308-13

Hacettepe University, Division of Pediatric Hematology 06100, Ankara, Turkey, Phone: +90 312 305 11 70 E-mail:

Giant cell hepatitis associated with direct Coombs' test-positive hemolytic anemia is a rare condition of childhood and the pathogenesis remains unclear. An autoimmune activation and loss of self-tolerance in these patients may be the underlying pathology related to the response of some of the patients to immunosuppressive treatment. Herein, we report the clinical presentation and course of three consecutive patients with this rare condition. We conclude that serum ferritin at diagnosis may be used for prediction of the outcome.
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http://dx.doi.org/10.5152/tjh.2010.55DOI Listing
December 2010

Effects of enteral insulin on hypoxic changes in a rat model of necrotizing enterocolitis.

Indian Pediatr 2010 Oct;47(10):887-8

The aim of this study was to determine if oral administration of insulin would protect intestinal cell damage in a hypoxia-induced experimental NEC model in rats. Rats were subjected to hypoxia-reoxygenation and then were returned to standard conditions, other were treated with insulin. According to our results, oral insulin does not prevent mild intestinal mucosal changes during hypoxic injury in rats.
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http://dx.doi.org/10.1007/s13312-010-0143-xDOI Listing
October 2010

Treatment of Wilms tumor: a report from the Turkish Pediatric Oncology Group (TPOG).

Pediatr Hematol Oncol 2010 Apr;27(3):161-78

Hacettepe University Faculty of Medicine, Dept. of Pediatric Oncology, Ankara, Turkey.

Aim: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey.

Methods And Patients: Between 1998 and 2006, WT patients were registered from 19 centers. Patients <16 years with unilateral WT whose treatment started in first postoperative 3 weeks were included. Treatments were stage I favorable (FH) and unfavorable histology (UH) patients, VCR + Act-D; stage IIA FH, VCR + Act-D; stage IIB FH, VCR + Act-D + radiotherapy (RT); stage III-IV FH, VCR + Act-D + adriamycin (ADR) + RT; stages II-IV UH tumors, VCR + Act-D + ADR + etoposide + RT.

Results: 165/254 registered cases were eligible (bilateral, 5.9%) [median age 3.0 years; M/F: 0.99; 50/165 cases < or =2 years]. 9.7% cases had UH tumors. Disease stages were stage I 23.6%; IIA 36.4%; IIB 5.5%; III 22.4%; IV 12.1%. Cases >2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV.

Conclusions: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.
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http://dx.doi.org/10.3109/08880010903447375DOI Listing
April 2010