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Featured researches published by Zeren Baris.


The American Journal of Gastroenterology | 2011

Prevalence of Celiac Disease in Healthy Turkish School Children

Buket Dalgic; Sinan Sari; Bilkay Basturk; Arzu Ensari; Odul Egritas; Aysegul Bukulmez; Zeren Baris

OBJECTIVES:Epidemiological studies of celiac disease (CD) in Turkey have been performed only within some regions of the country. The aim of this study was to determine the prevalence of CD in Turkish school children.METHODS:Between 2006 and 2008, serum samples were collected from 20,190 students (age range, 6–17 years) in 139 schools in 62 cities from different regions of Turkey. CD was screened using IgA antitissue transglutaminase (IgA-tTG) and total serum IgA. Subjects with selective IgA deficiency were further tested for IgG-tTG. Serum samples positive for IgA or IgG-tTG were further tested for IgA antiendomysial antibodies (IgA-EMAs) using an indirect immunofluorescence method. Small-intestinal biopsy was offered to all subjects with tTG antibody positivity.RESULTS:Of the 20,190 subjects, 489 were antibody positive (IgA-tTG only in 270, both IgA-tTG and IgA-EMA in 215, and IgG-tTG in 4). Selective IgA deficiency was detected in 108 patients, and 4 of them were positive for IgG-tTG. An intestinal biopsy was conducted in 215 subjects (IgA-tTG positive in 110, IgA-tTG and IgA-EMA positive in 104, and IgG-tTG positive in 1). The biopsy findings of 95 children were consistent with CD. Thus, the estimated biopsy-proven prevalence was 1:212 children. The positive predictive value (PPV) for IgA-tTG plus EMA was 75.9%. PPV was 44.3% when only IgA-tTG was used.CONCLUSIONS:We estimate that the prevalence of CD is at least 0.47% in healthy Turkish school children. Screening for IgA-tTG plus EMA provided better results for diagnosis when compared with testing for IgA-tTG alone.


Journal of Pediatric Gastroenterology and Nutrition | 2010

Primary Intestinal Lymphangiectasia in Children: Is Octreotide an Effective and Safe Option in the Treatment?

Sinan Sari; Zeren Baris; Buket Dalgic

Objective: Octreotide has been suggested as a medical treatment option in refractory cases of primary intestinal lymphangiectasia (IL). There are few data about the long-term effect and safety of octreotide for IL in the literature. In the present article we analyzed pediatric cases of primary IL with long-term octreotide treatment and discussed its safety profile. Methods: Between 1999 and 2008, 13 children were diagnosed in our clinic as having IL. Six patients with primary IL were followed up, receiving octreotide therapy. The clinical data of the patients and duration of therapy, dose, and side effects of octreotide were evaluated. Results: Octreotide, 15 to 20 μg per body weight 2 times daily subcutaneously, was given to all of the patients. Duration of the octreotide treatment changed between 3 and 37 months. Stool frequency decreased in all of the patients after starting octreotide treatment. Serum albumin could be maintained at normal levels in 3 patients. The requirement of albumin infusions decreased in all of the patients. Acute pancreatitis was observed as a side effect of octreotide in 1 patient. Conclusions: Octreotide may help to maintain serum albumin levels, improve clinical findings, and decrease the requirement of albumin infusions in refractory cases of primary IL.


The New England Journal of Medicine | 2017

CD55 Deficiency, Early-Onset Protein-Losing Enteropathy, and Thrombosis

Ahmet Ozen; William A. Comrie; Rico Chandra Ardy; Cecilia Domínguez Conde; Buket Dalgic; Ömer Faruk Beşer; Aaron Morawski; Elif Karakoc-Aydiner; Engin Tutar; Safa Barış; Figen Ozcay; Nina Kathrin Serwas; Yu Zhang; Helen F. Matthews; Stefania Pittaluga; Les R. Folio; Aysel Ünlüsoy Aksu; Joshua McElwee; Ana Krolo; Ayca Kiykim; Zeren Baris; Meltem Gulsan; İsmail Öğülür; Scott B. Snapper; R. H. J. Houwen; Helen L. Leavis; Deniz Ertem; Renate Kain; Sinan Sari; Tülay Erkan

Background Studies of monogenic gastrointestinal diseases have revealed molecular pathways critical to gut homeostasis and enabled the development of targeted therapies. Methods We studied 11 patients with abdominal pain and diarrhea caused by early‐onset protein‐losing enteropathy with primary intestinal lymphangiectasia, edema due to hypoproteinemia, malabsorption, and less frequently, bowel inflammation, recurrent infections, and angiopathic thromboembolic disease; the disorder followed an autosomal recessive pattern of inheritance. Whole‐exome sequencing was performed to identify gene variants. We evaluated the function of CD55 in patients’ cells, which we confirmed by means of exogenous induction of expression of CD55. Results We identified homozygous loss‐of‐function mutations in the gene encoding CD55 (decay‐accelerating factor), which lead to loss of protein expression. Patients’ T lymphocytes showed increased complement activation causing surface deposition of complement and the generation of soluble C5a. Costimulatory function and cytokine modulation by CD55 were defective. Genetic reconstitution of CD55 or treatment with a complement‐inhibitory therapeutic antibody reversed abnormal complement activation. Conclusions CD55 deficiency with hyperactivation of complement, angiopathic thrombosis, and protein‐losing enteropathy (the CHAPLE syndrome) is caused by abnormal complement activation due to biallelic loss‐of‐function mutations in CD55. (Funded by the National Institute of Allergy and Infectious Diseases and others.)


European Journal of Pediatrics | 2010

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE): case report with a new mutation

Zeren Baris; Tuba F. Eminoglu; Buket Dalgic; Leyla Tümer; Alev Hasanoglu

IntroductionMitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare autosomal recessive multisystem disorder characterized by severe gastrointestinal dysmotility and leads to cachexia, ptosis, external ophthalmoplegia, peripheral neuropathy, and leukoencephalopathy.Results and discussionIt is often misdiagnosed as anorexia nervosa or intestinal pseudoobstuctions and are unnecessarily treated with surgery. It has been established that MNGIE is caused by mutations in the gene encoding thymidine phosphorylase, which lead to absolute or nearly complete loss of its catalytic activity, producing systemic accumulations of its substrates, thymidine and deoxyuridine.ConclusionWe present herein the clinical, neuroimaging, and molecular findings of a patient with MNGIE caused by a novel homozygous TYMP gene mutation (c.112G>T which convert codon 38 from glutamate to a stop codon [p.38E>X]).


Transplantation | 2018

Clinical Findings and Explant Liver Histology in Crigler Najjar Disease

Zeren Baris; Gonca Özgün; Oya Balci Sezer; Figen Ozcay; Mehmet Haberal

Introduction We evaluated pediatric patients with Crigler Najjar disease who received LT at our center. Materials and Methods We reviewed the clinical and laboratory data of patients with Crigler Najjar disease who received LT at Başkent University from 2001-2017. Histopathologic examinations of explanted livers were also recorded. Results There was total of 7 patients (5 female 71.4%). The mean age at the time of LT was 5±7.1 years (range: 0.2-17.5 y). The liver function tests before LT were: total bilirubin: 31.28±7.3 mg/dL (range: 20.7-43.3 mg/dL), direct bilirubin: 1.39±1.19 mg/dL (range: 0.46-3.93 mg/dL), AST: 174±330 mg/dL (range: 24-922 IU/L), ALT: 79±88 mg/dL (range: 19-276 IU/L), GGT: 67±53 mg/dL (range: 27-184 U/L), ALP: 569±327 mg/dL (range: 115-1055 IU/L). Patients were given 11±3 hours (range: 8-16 hours) phototherapy (6/7 patients), phenobarbital (5/7 patients), cholestyramine (3/7 patients) before LT. Plasmapheresis was used in 1 patient to decrease bilirubin levels before LT. The mean body weight of the patients at the time of transplant was 20.9±19.6 kg (range: 6.7-58 kg). The donor was father in 5 and mother in 2 LT. Left lateral lobe was used in 5, left lobe was used in 1 and right lobe was used in 1 patient. Patients were followed up for 5.83±4.3 years (range: 1-11.5 y). Postoperative complications included hepatic artery thrombosis in 2 patients, hepatic vein thrombosis in 1, bile leak in 2, acute cellular rejection in 2, pneumothorax in 1, primary graft dysfunction and cholestasis in 1. One patient had neurologic sequela and died after vomiting and aspiration during his sleep at 1 year posttransplant. One patient has mild mental retardation. Other patients are alive with normal developmental milestones. One patient had cirrhosis and homozygous mutation in UGT1A1 gene consistent with Crigler Najjar type 1. She was only given ursodeoxycholic acid and cholestyramine as treatment before liver transplant. She is doing fine without any neurologic deficit after LT. Explanted livers showed normal structure in all patients except one. 5 patients had mild hepatocanalicular cholestasis, cirrhotic patient additionally had dilated bile ducts filled with cholestatic plugs and prominent ductular and vascular proliferation. 1 patient without cirrhosis had mild portoportal fibrosis, as well as mild bile ductular proliferation. 5 patients had lymphomononuclear inflammation in the portal areas. 3 patients showed ballooning degeneration in the hepatocytes.3 patients had neutrophilic infiltration in the paranchyme and subcapsular areas. 1 patient had diffuse microvesicular steatosis. Conclusions Severe form of Crigler Najjar disease can be successfully cured by LT. Mild increase in liver function tests other than bilirubin may be recorded before surgery. Histopathological explants may show cholestatic changes and portal/paranchymal inflammation. This finding may be secondary to co-existing liver diseases or Crigler Najjar disease itself.


Transplantation | 2018

Experience of Post-Transplant Lymphoproliferative Disorder (PTLD) After Pediatric Liver Transplant: Incidence, Outcomes and Association with Food Allergy

Zeren Baris; Figen Ozcay; Ozlem Yilmaz Ozbek; Nihan Haberal; Faik Sarialioglu; Mehmet Haberal

Introduction We evaluated our 16 years of experience of pediatric PTLD cases who were liver transplanted in Başkent University Hospital between 2001-2017 years. Materials and Methods We reviewed the clinical and laboratory data of 8 patients who were diagnosed as PTLD out of 236 pediatric patients who were liver transplanted in Başkent University Hospital between 2001-2017 years. Pretransplant EBV statuses of 172 patients were also recorded. Results The total PTLD incidence was 3.4%. The incidence of PTLD was 10% in pretransplant EBV IgG negative patients, while it was 0.8% in pretransplant EBV seropositive patients. Mean age of the patients at liver transplant was 2.71±3.21 years; four patients were under 1 year of age at the time of transplant. PTLD was diagnosed 21.81±18.1 months after transplant. The primary site of involvement was variable among patients: peripheral and mediastinal lymph nodes, stomach-intestinal, transplanted graft, bone marrow, and nasopharynx. Eosinophil count varied greatly among patients, with mean values of 524.62±679. Food allergy prevalence was higher than the non-PTLD patients (23/236; 10% versus 3/8; 37.5%) The lymphoproliferative disease was of B cell origin in 6 of the patients. One patient died due to neutropenic sepsis during chemotherapy, while seven patients are being followed up in full remission for 7.75±4 years. Conclusion PTLD is a life-threatening complication of solid organ transplantation with a heterogenous clinic. Food allergy had a close association with PTLD. Close follow-up of patients who had risk factors and early diagnosis with appropriate treatment may lead to good outcome.


The Turkish journal of gastroenterology | 2018

A single-center experience of post-transplant lymphoproliferative disorder (PTLD) cases after pediatric liver transplantation: Incidence, outcomes, and association with food allergy

Zeren Baris; Figen Ozcay; Ozlem Yilmaz Ozbek; Nihan Haberal; Faik Sarialioglu; Mehmet Haberal

BACKGROUND/AIMS We evaluated our 16-year single-center experience of pediatric post-transplant lymphoproliferative disorder (PTLD) cases who underwent liver transplantation between 2001 and 2017. MATERIALS AND METHODS Of the 236 pediatric patients who underwent liver transplantation between 2001 and 2017, the clinical and laboratory data of eight patients diagnosed with PTLD were reviewed. The pre-transplant Epstein-Barr virus (EBV) status of 172 patients was also recorded. RESULTS The total incidence of PTLD was 3.4%. The incidence of PTLD was 10% in pre-transplant EBV immunoglobulin G (IgG)-seronegative patients and 0.8% in pre-transplant EBV IgG-seropositive patients. The mean age of the patients at liver transplantation was 2.71±3.21 years, and four patients were aged below 1 year at the time of transplantation. PTLD was diagnosed at 21.81±18.1 months after transplantation. The primary site of involvement was variable among patients: peripheral and mediastinal lymph nodes, stomach and intestine, transplanted graft, bone marrow, and nasopharynx. The eosinophil count varied greatly among patients, with a mean value of 524.62±679/mm3. Three patients had a food allergy and were administered an elimination diet at the time of PTLD diagnosis. Six patients had PTLD of B-cell origin. One patient died due to neutropenic sepsis during chemotherapy, whereas seven patients were followed up in full remission for 7.75±4 years. CONCLUSION PTLD is a life-threatening complication of solid-organ transplantation with a heterogeneous clinical spectrum. Food allergy had a close association with PTLD. A close follow-up of patients with risk factors and an early diagnosis with appropriate treatment may lead to a better outcome.


Journal of Pediatric Hematology Oncology | 2017

Solid Liver Lesions in an Infant With Neonatal Cholestasis: Is it Always Malignant?

Zeren Baris; Pelin Börcek; Kemal Murat Haberal; Figen Ozcay

In this report we describe a patient with neonatal cholestasis who was found to have a liver lesion with suspicious imaging features, although ultimately it was histologically proved to be a pseudotumor. We discuss the characteristic features and imaging findings of macroregenerative nodules of the liver.


Transplantation | 2018

Clinical Features, Laboratory Findings and Prognosis in Fulminant Wilsonʼs Disease

Figen Ozcay; Zeren Baris; Oya Balci Sezer; Mehmet Haberal


Transplantation | 2018

Incidence, Clinical Features and Prognosis of Food Allergy in Children who Underwent Liver Transplantation

Burcu Tahire Köksal; Zeren Baris; Figen Ozcay; Ozlem Yilmaz Ozbek; Mehmet Haberal

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