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Öğe Akut viral hepatit a ile plevral efüzyon birlikteliği:olgu sunumu(İnönü Üniversitesi Tıp Fakültesi Dergisi, 2007) Öncül, Mehmet; Celiloğlu, Can; Selimoğlu, Mukadder AyşeÖz: Hepatit A çocuklarda hafif semptomlarla veya asemptomatik seyreder. Buna karşın %15 vakada atipik bir seyir gösterebilir. Plevral efüzyon Hepatit A’nın nadir bir komplikasyonudur. Hepatit A enfeksiyonu ile plevral efüzyon birlikteliği bilindiği kadarıyla şimdiye kadar 10 vakada bildirilmiştir. Bu olgu sunumunda akut viral hepatit A seyri esnasında plevral efüzyon ve assit ile gelen 8 yaşında bir hastayı sunduk. Akut viral hepatite eşlik eden plevral efüzyonun patogenezi tam açıklanamamış olsa da kendiliğinden düzeldiği ve herhangi bir tedaviye gerek olmadığı bilinmektedir. Bu bağlamda transaminazlarda yükseklik ile birlikte hipoalbuminemi olsun olmasın plevral efüzyon kliniği ile gelen olgularda, olayın akut viral hepatite bağlı olabileceğinin akılda tutulması gerektiğini ve böyle olgularda klinik izlemde düzelme oluyorsa ileri araştırmalara girmenin gereksiz olduğunu vurguladık. Başlık (İngilizce): Association of acute viral hepatitis a and pleural effusion: Case report Öz (İngilizce): Although Hepatitis A usually manifests with mild symptoms or asymptomatic in children, in 15% of cases it may present with atypical symptoms and signs. Pleural effusion is a rare complication of hepatitis A. To the best of our knowledge, pleural effusion associated hepatitis A has been reported in only ten cases up to date. In this case report, we present an eight-year-old boy with pleural effusion and ascites associated with hepatitis A. Although the exact mechanism of pleural effusion in hepatitis A is unclear, it is known that it resolves spontaneously. Consequently, it is important to keep in mind that in the presence of pleural effusion with elevated transaminases, with or without hypoalbuminemia, hepatitis A should be considered, and if this is the case and clinical relief is observed during the follow-up, further diagnostic interventions are not warrented.Öğe Akut Viral Hepatit A ile Plevral Efüzyon Birlikteliği:Olgu Sunumu(İnönü Üniversitesi Tıp Fakültesi Dergisi, 2007) Öncül, Mehmet; Celiloğlu, Can; Selimoğlu, Mukadder AyşeHepatit A çocuklarda hafif semptomlarla veya asemptomatik seyreder. Buna karşın %15 vakada atipik bir seyir gösterebilir. Plevral efüzyon Hepatit A’nın nadir bir komplikasyonudur. Hepatit A enfeksiyonu ile plevral efüzyon birlikteliği bilindiği kadarıyla şimdiye kadar 10 vakada bildirilmiştir. Bu olgu sunumunda akut viral hepatit A seyri esnasında plevral efüzyon ve assit ile gelen 8 yaşında bir hastayı sunduk. Akut viral hepatite eşlik eden plevral efüzyonun patogenezi tam açıklanamamış olsa da kendiliğinden düzeldiği ve herhangi bir tedaviye gerek olmadığı bilinmektedir. Bu bağlamda transaminazlarda yükseklik ile birlikte hipoalbuminemi olsun olmasın plevral efüzyon kliniği ile gelen olgularda, olayın akut viral hepatite bağlı olabileceğinin akılda tutulması gerektiğini ve böyle olgularda klinik izlemde düzelme oluyorsa ileri araştırmalara girmenin gereksiz olduğunu vurguladık.Öğe Anne ve bebek sağlığı açısından emziren anne beslenmesinin önemi(İnönü Üniversitesi, Tıp Fakültesi, Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Malatya, Türkiye, 2013) Selimoğlu, Mukadder AyşeÖz: Emziren annenin dengeli beslenmesi, hem kendi fizyolojik gereksinimlerini karşılaması ve kendi besin yedeğini dengede tutması açısından, hem de ideal bir anne sütü miktarı ve içeriği yoluyla bebeğin sağlıklı büyüme ve gelişmesi için gereklidir. Anne sütünün besin profili bebeği korumayı ön plana alarak diyetten az etkilenir, özellikle protein, yağ, karbonhidrat ve folat anne diyetinde eksik bile olsa anne sütündeki miktar yeterli kalır. Ancak bebeği etkilemeyen bu durum, annenin depolarında yetersizliğe ve sonuçta annenin zararlanmasına neden olur. Anne sütünün A ve B vitamin düzeyleri annenin diyetine bağımlıdır, annede eksik ise bebekte de eksiklik gelişir. Türkiye’de yapılan çalışmalar annelerde mikrobesin eksikliğinin önemli bir sorun olduğunu, annelerde yüksek sıklıkta mikrobesin açlığı bulunduğunu göstermektedir. Bu bağlamda hem anne sağlığı için hem de ideal anne sütü içeriği için anne beslenmesi üzerinde durulması ve mikrobesin eksikliğinin sık olduğu ülkelerde tasarılar üretilmesi önemli bir öncelik olmalıdır. (Türk Ped Arfl 2013; 48: 183-7)Öğe Aortic aneurysm a rare complication of ulcerative colitis(Anadolu Kardiyoloji Dergisi/The Anatolian Journal of Cardiology, 2007) Karakurt, Cemşit; Koçak, Gülendam; Selimoğlu, Mukadder Ayşe; Özen, MetehanUlcerative colitis is an idiopathic chronic inflammatory disorder localized in the colon and rectum. Systemic and extraintestinal complications of ulcerative colitis are arthritis, delayed growth and sexual maturation, nutritional deficiency secondary to malabsorbtion, mucocutaneous lesions, renal disease, hepatobiliar disease and ocular complications (1). Cardiac complications including myocarditis, pericarditis and increased risk of infective endocarditis may be rarely seen during the clinical course but aortic aneurysm is an extremely rare complication of ulcerative colitis.Öğe Asetaminofen hepatotoksisitesinin ve nefrotoksisitesinin yenidoğan ve erişkin sıçanlarda karşılaştırmalı olarak incelenmesi(İnönü Üniversitesi Tıp Fakültesi Dergisi, 1995) Eşrefoğlu, Mukaddes; Selimoğlu, Mukadder Ayşe; Çiftçioğlu, M. Akif; Gepdiremen, Akçahan; Parlak, MehmetBu çalışma asetaminofen hepatotoksistesini ve nefrotoksistesini yenidoğan ve erişkin sıçanlarda karşılaştırmalı olarak incelemek amacı ile planlandı. Çalışmada 10 erişkin ve 10 yenidoğan Wistar albino türü sıçan kullanıldı, her grupta hayvanların dördüne 300mg/kg ve dördüne de 500mg/kg asetaminofen intraperitoneal olarak uygulandı. Spontan ölüm gözlenmedi, 8 saat sonra hayvanlar öldürüldüler. Karaciğer ve böbrek kesitleri hematoksilen-eozinle boyandı. 300mg/kg dozda yeni-doğan sıçanların karaciğerlerinde az sayıda nekrotik hücre izlenirken; 500mg/kg dozda bütün yeni-doğanlarda hafif sentrilobüler hepatik nekroz gözlendi. Erişkin sıçanlarda ise 300mg/kg dozda orta derecede, 500mg/kg dozda ciddi hepatik nekroz izlendi.Öğe Assessment of behavioral feeding difficulties in young children(2019) Kıvılcım, Meltem; Doğan, Derya Gümüş; Kortay Canaloğlu, Sinem; Varol, Fatma İlknur; Selimoğlu, Mukadder AyşeAbstract: Aim: Feeding difficulties are common in typically developing children and the prevalence ranges from 25% to 45%. Although using an assessment tool is the best approach when planning an intervention, assessment of feeding disorders is mostly accomplished by informal parent interviews in clinical practice. This study was designed to assess the mealtime behaviours of young children with ‘Feeding Difficulties’ (FD) by an instrument and to compare the results with ‘Typically Developing’ (TD) children. Material and Methods: A total of 61 children with FDs and 63 TD children aged 6-42 months were included. The Behavioral Pediatric Feeding Assessment Scale (BPFAS) was completed to describe the child’s feeding behaviors and the parents’ mealtime strategies. Results: FD group had higher scores than TD group in all BPFAS subtests’ scores. The mean ‘Total Frequency Score’ was 104.6 in FD group whereas 72.9 in TD group (p=0.0001). The FD group had more problematic feeding behaviours in comparison to TD group. The mean ‘Total Problem Score’ of TD group was 6.1 whereas 20.1 in children with FD (p=0.0001). Conclusion: This study supports the BPFAS to be a useful and practical feeding assessment tool and also has the advantage of incorporating parents’ feelings about child’s feeding behaviors.Öğe Eight different viral agents in childhood acute gastroenteritis Original(2015) Bozkurt, Derya; Selimoğlu, Mukadder Ayşe; Otlu, Barış; Sandıkkaya, AyşeAbstract: Bozkurt D, Selimoğlu MA, Otlu B, Sandıkkaya A. Eight different viral agents in childhood acute gastroenteritis. Turk J Pediatr 2015; 57: 68-73.Viral gastroenteritis is the most frequent cause of acute gastroenteritis (AGE) of childhood. The aim of this study was to determine the prevalence of viral agents including astrovirus, rotavirus, adenovirus, enterovirus, norovirus, parechovirus, Aichivirus and sapovirus in children with AGE in a pediatric Turkish population. Fecal specimens of 240 children with AGE were investigated by polymerase chain reaction, and viral agents were identified in 131 (54.6%) samples. The distribution of viral agents was as follows: 56 (42.8%) norovirus, 44 (33.6%) rotavirus, 29 (22.1%) enterovirus, 21 (16.0%) adenovirus, 21 (16.0%) parechovirus, 5 (3.8%) sapovirus and 1 (0.8%) Aichivirus. Single and multiple viral agents were detected in 38.8% and 15.8% of patients, respectively. The duration of hospitalization was longer in children with multiple viral agents than in those infected with a single viral agent (p<0.001). While the highest rate of rotavirus infection was detected in winter, the highest rate of norovirus was found in the summer. In conclusion, norovirus and rotavirus are the most frequent causes of childhood AGE in our countryÖğe Evaluation of growth after liver transplantation in a group of Turkish pediatric patients(2021) varol, Fatma İlknur; Selimoğlu, Mukadder Ayşe; Gungor, Sukru; Yılmaz, SezaiAbstract: Aim: Growth failure is considered as an important predictor of negative outcomes after liver transplantation (LT). In our study we aimed to evaluate the growth of liver transplanted children both at the time of LT and on follow-up and to determine factors which are effective on growth. Materials and Methods: Seventy nine children were included in the study. Evaluation of growth just before the LT and post-LT 6th month,1st,2nd and 3rd years was done by using weight for age Z (WAZ) and height for age Z scores (HAZ). Results: Sixteen (20.3%) patients had HAZ score<-2 SD (standard deviation) and 13 (16.5%) had WAZ score <-2 SD. Stunting was detected in 17.8% and 23.5% of children with acute liver failure and chronic liver disease, underweight was present in 8.9% and26.5%of them, respectively (p=0.52 and p=0.037, respectively). Both HAZ and WAZ scores increased after LT, especially in the first year. Not mean pre-LT WAZ but mean HAZ score was lower in children who died on follow-up (p=0.023).Conclusion: Malnutrition before LT is a common problem in children. As stunting is a factor that reduces the chance of survival after LT, prevention and correction is very important.Öğe The hematologic manifestations of pediatric celiac disease at the time ofdiagnosis and efficiency of gluten-free diet(Turkish Journal of Medical Sciences, 2015) Çatal, Ferat; Topal, Erdem; Erimiştekin, Halime; Yıldırım, Nurdan Acar; Sinanoğlu, Muhammed Selçuk; Karbiber, Hamza; Selimoğlu, Mukadder AyşeÖz (İngilizce): Background/aim: To determine the hematologic manifestations at the time of diagnosis of celiac disease in children and the effects of a gluten-free diet on hematologic signs upon follow-up. Materials and methods: The records of patients with celiac disease who received a follow up examination at the Pediatric Gastroenterology Clinic between June 2006 and June 2013 were retrospectively examined. Results: Ninety-one patients were included in the study. The mean age at diagnosis was 8.1 ± 4.21 years and 59 patients (64.8%) were female. Thirty-two patients (35.2%) had hematologic signs at the time of diagnosis. Anemia (24.2%) was the most common hematologic sign, followed by thrombocytosis (16.5%) and leukopenia (4.4%). The tTG IgA titers were screened in 80 of the 91 patients during diagnosis. Follow-up examinations found that remission for anemia (P = 0.017), thrombocytosis (P = 0.039), and decreases in tTG IgA titers (P = 0.034) were more prominent in patients who had followed a strict gluten-free diet. Conclusion: Approximately one-third of the celiac disease patients had hematologic manifestations at the time of diagnosis. Remission in hematologic signs and decrease in tTG IgA titers were more prominent in patients who had adhered to a gluten-free diet.Öğe An intrahepatic portal vein aneurysm presenting with esophageal variceal bleeding in a pediatricpatient: a rare clinical entity(GALENOS YAYINCILIK, ERKAN MOR, MOLLA GURANI CAD 21-1, FINDIKZADE, ISTANBUL 34093, TURKEY, 2018) Güngör, Şükrü; Varol, Fatma ilknur; Kutlu, Ramazan; Yılmaz, Sezai; Selimoğlu, Mukadder AyşeÖğe Is celiac disease common in patients with vitiligo?(Turkish Journal of Gastroenterology, 2011) Seyhan, Muammer; Kandı, Başak; Akbulut, Handan; Selimoğlu, Mukadder Ayşe; Karıncaoğlu, MelihBaşlık ( ): Çölyak hastalığı vitiligolu hastalarda sık mıdır?Öğe Kistik fibroz ve alfa-1 antitripsin eksikliğinde pankreatik tutulum(Türk Pediatri Arşivi, 2009) Karabiber, Hamza; Selimoğlu, Mukadder AyşeÖz: Kistik fibroz ve alfa-1 antitripsin eksikliği birçok sistemi tutan genetik kökenli, otozomal çekinik geçişli iki önemli hastalıktır. Her iki hastalığın ortak olarak etkileyebildiği organlardan birinin de pankreas olduğu bilinmektedir. Kistik fibroz’da pankreatik tutulum, normale yakın pankreas işlevinden endokrin ve ekzokrin işlevlerinin tamamen kaybına kadar değişen klinik tablolara yol açar. Hastalığın genotipi ile fenotipi arasında yakın bir ilişki vardır. Pankreas tutulumu yağ ve protein emilim bozukluğu ve büyüme geriliği ile belirgindir. Yağ emilim bozukluğu sonucu yağda eriyen vitamin eksikliklerine bağlı belirtiler oluşur. Emilim bozukluğu tedavisi enzimin yerine konulan ve vitamin eksikliklerinin düzeltilmesi şeklinde yapılmalıdır. Gen tedavisi hastalığın tümüyle tedavisi için bir umut ışığıdır. Alfa-1 antitripsin eksikliği ve kronik pankreatit birlikteliği ile ilgili birçok olgu sunumu yayınlanmış olmakla birlikte pankreatitli hastaların, sağlıklı kontrollerle karşılaştırıldığı çalışmalarda alfa-1 antitripsin eksikliğinin kronik pankreatit etiolojisinde rolü olmadığı sonucuna varılmıştır. Ancak alfa-1 antitripsinin pankreasın kendi kendini sindirmesini önleyen etkisi göz önüne alındığında etiolojik bir etmen olmasa da eksikliğinin pankreas hastalığını artırıcı bir rolü olduğu düşünülebilir. Kistik fibroz ve alfa-1 antitripsin eksikliği olan hastalarda pankreas tutulumunun erken saptanması ve tedavisi bu hastalıklardan ölüm oranının azaltılmasına önemli katkıda bulunur. Başlık (İngilizce): Pancretic disease in cystic fibrosis and alpha-1 antitrypsin deficiency Öz (İngilizce): Cystic fibrosis and alpha-1 antitrypsin deficiency are two important autosomal recessive multi-organ diseases. They are complex and severe diseases that involve various organs including pancreas. Pancreatic disease in cystic fibrosis varies from complete loss of exocrine and endocrine functions to nearly normal pancreatic function. A strong correlation between genotype and phenotype is recognized. Pancreatic involvement is characterized by malabsorption of fat and protein, and growth failure. Fat malabsorption leads to special problems with the fat-soluble vitamins. Enzyme and fat-soluble vitamin replacements are the milestones of the malabsorption treatment. Gene therapy offers the best hope for a cure of cystic fibrosis. Although an association between alpha1-antitrypsin deficiency and chronic pancreatitis has been reported in several case reports, in prospective studies it has been shown that pancreatitis prevalence in patients with alpha1-antitrypsin deficiency was not different from that of healthy controls. Thus it was concluded that alpha-1 antitrypsin deficiency was not related to the pathogenesis of idiopathic or hereditary chronic pancreatitis. However, protective role of alpha-1 antitrypsin against pancreatic auto digestion suggests that the deficiency of that protein may worsen the pancreatic disease rather than causing it. Early detection and management of pancreatic insufficiency is essential to optimize health and outcomes in cystic fibrosis and alpha-1 antitrypsin deficiency patients.Öğe Living donor liver transplantation for acute liver failure in pediatric patients caused by the ingestion of fireworks containing yellow phosphorus(Liver Transplantation, 2011) Ateş, Mustafa; Dirican, Abuzer; Özgör, Dinçer; Aydın, Cemalettin; Işık, Burak; Ara, Cengiz; Yılmaz, Mehmet; Selimoğlu, Mukadder Ayşe; Kayaalp, Cüneyt; Yılmaz, SezaiYellow phosphorus is a protoplasmic toxicant that targets the liver. The ingestion of fireworks containing yellow phosphorus, either by children who accidentally consume them or by adults who are attempting suicide, often results in death due to acute liver failure (ALF). We present the outcomes of 10 children who ingested fireworks containing yellow phosphorus. There were 6 boys and 4 girls, and their ages ranged from 21 to 60 months. One patient remained stable without liver complications and was discharged. Three patients died of hepatorenal failure and cardiovascular collapse, and living donor liver transplantation (LDLT) was performed for 6 patients. The patients had grade II or III encephalopathy, a mean alanine aminotransferase level of 1148.2 IU/L, a mean aspartate aminotransferase level of 1437.5 IU/L, a mean total bilirubin level of 6.9 mg/dL, a mean international normalized ratio of 6.6, a mean Pediatric End-Stage Liver Disease score of 33.7, and a mean Child-Pugh score of 11.3. Postoperatively, 2 patients had persistent encephalopathy and died on the second or third postoperative day, and 1 patient died of cardiac arrest on the first postoperative day despite a well-functioning graft. The other 3 patients were still alive at a mean of 204 days. In conclusion, the ingestion of fireworks containing yellow phosphorus causes ALF with a high mortality rate. When signs of irreversible ALF are detected, emergency LDLT should be considered as a lifesaving procedure; however, if yellow phosphorus toxicity affects both the brain and the heart in addition to the liver, the mortality rate remains very high despite liver transplantation.Öğe Pariyetal hücrelerde sirkadiyan fonksiyonel aktivitelerine bağlı olarak izlenen sirkadiyan ultrastrüktürel değişiklikler(2003) Eşrefoğlu, Mukaddes; Selimoğlu, Mukadder Ayşe; Vardı, NigarAmaç: Pariyetal hücrelerde iki membran sistemi bulunur. Biri mikrovilluslu intraselüler kanalikül sistemi, diğeri tubuloveziküler sistemdir. Bu hücrelerin elektron mikroskopik özelliklerinin fonksiyonel aktivitelerine bağlı olarak değiştiği gösterilmiştir. Çalışmamızda günün saatinin ve tokluk derecesinin pariyetal hücrelerin elektron mikroskopik özelliklerinde yol açtığı değişikliklerin incelenmesi amaçlandı. Materyal ve Metod: Çalışmamızda 16 adet erkek Wistar albino fare kullanıldı. Hayvanlar dört eşit gruba ayrıldı. Dört fare sabah, dördü akşam saatlerinde, diğer dört hayvan açken, dördü tokken dekapite edildi. Mide fundusundan alınan örnekler %3'lük gluteraldehid ve %2'lik Os04 ile fıkse edildikten sonra araldid CY212'ye gömüldü. İnce kesitler uranil asetat ve kurşun sitrat ile boyanarak Jeol 100 SX transmisyon elektron mikroskopta (TEM) incelendi. Bulgular: Sabah saatlerinde pariyetal hücrelerin sitoplazmalarında çok sayıda tubul ve vezikül izlendi. Gece alınan örneklerde tubuloveziküler sistem belirsizleşmişti. Kanaliküller yer yer genişlemişti. Kanalikülleri döşeyen mikrovilluslar genellikle dolgunlaşmıştı. Tok hayvanlarda tubuloveziküler sistem belirgindi. Kanaliküllerin yüzeylerinde çok miktarda mikrovillus bulunmaktaydı. Bazı hücrelerin kanalikül lümeni belirgindi, mikrovilluslar ince seyrediyordu. Aç hayvanlarda tubuloveziküler sistem belirsizleşmişti. Apikal yüzeyi ve kanalikülleri döşeyen mikrovilluslar kısalmıştı. Sonuç: Çalışmamızda pariyetal hücrelerin sekretuvar aktivite-lerinde sirkadiyan ritm gösterdikleri, bununla bağlantılı olarak farklı fizyolojik koşullarda farklı elektron mikroskopik özellikler sergiledikleri sonucuna varıldı.Öğe Report of seven children with hepatopulmonary syndrome(Turk J Gastroenterol, 2014) Erge, Duygu; Selimoğlu, Mukadder Ayşe; Karakurt, Cemşit; Karabiber, Hamza; Elkıran, Özlem; Çatal, Ferhat; Köroğlu, ReyhanHepatopulmonary syndrome is an important pulmonary vascular complication of liver disease. Its diagnosis is based on the presence of hypoxaemia and the demonstration of intrapulmonary shunting by contrast-enhanced echocardiography or perfusion lung scanning. Awareness of this condition is critical to improve the outcomes of patients with chronic liver disease and/or portal hypertension because hepatopulmonary syndrome receives additional priority on the waiting list for transplantation. A non-invasive measurement of the blood oxygen saturation with pulse oximetry is recommended as a screening tool for this syndrome. The aim of this report was to present clinical and laboratory findings and follow-up of seven paediatric patients who were diagnosed with HPS at our centre.Öğe Single center analysis of the first 304 living donor liver transplantations in 3 years(Hepatogastroenterology, 2013) Yılmaz, Sezai; Kayaalp, Cüneyt; Ara, Cengiz; Yılmaz, Mehmet; Işık, Burak; Aydın, Cemalettin; Özgör, Dinçer; Dirican, Abuzer; Barut, Bora; Ünal, Bülent; Pişkin, Turgut; Ateş, Mustafa; Kutlu, Ramazan; Toprak, Hüseyin İlksen; Kırımlıoğlu, Saime Hale; Aladağ, Murat; Harputoğlu, Muhsin Murat Muhip; Selimoğlu, Mukadder Ayşe; Karabiber, Hamza; Yalçın, Kendal; Bayındır, Yaşar; Kırımlıoğlu, VedatBackground/Aims: Living donor liver transplantations (LDLT) has been established as an excellent treatment method for patients with end-stage liver disease and has achieved exponential growth, especially in the countries that have the donation problem. Between April 2007 and April 2010, we performed LDLT in 289 patients. Fifteen of the cases required re-transplantations. In the present study, these 304 consecutive LDLTs were evaluated to determine both donor and recipient outcomes. Methodology: Complication rates and survival data of the recipients and donors of 304 LDLT cases were analyzed. Results: All donors were alive and well. Overall complication rate was 27% (83 donors). These complications included bile leakage in 2%, intraabdominal bleeding in 2%, chylous peritonitis in 0.6%, hepatic venous obstruction due to not performing falsiformepexia in 0.3%, wound infection in 11%, incisional henia in 2%, and pulmonar complications (atelectasia, pneumonia) in 8%. The recipient complication rate was 51% in early postoperative period. The most frequent complication was infections. Five patients died due to aggressive infections. In the long term there were 57 biliary stricture cases. Five patients had chronic bile fistula. Hepaticojejunostomies were performed in 13 patients. Endoscopic stents were placed in 20 cases. Twenty-four patients were managed by percutaneous biliary catheter. Chronic and acute rejection attacks developed in 7 and 103 patients respectively. Hepatic artery thrombosis developed in 25 patients (8%). The mean follow-up was 19 months. One, two and three year survival rates were 82%, 79% and 75% respectively. In hospital mortality rate was 16%. There were a total of 74 (25%) recipient mortalities along follow up period due to 15 vascular complications, 39 septic complications, 9 liver dysfunctions, 6 chronic rejections and 5 different causes. Conclusions: More than 150 liver tranplantations per year in a single center is a challenge in Turkey, where there is a shortage of deceased donor grafts. LDLT is a safe procedure for the donors and an effective therapy for the patients with end-stage liver diseases. We believe that with accumulation of experience in surgery and clinical management, better outcomes of LDLT can be expected.Öğe Strain and strain rate echocardiography in children with Wilson s disease(Cardiovascular Journal of Africa, 2016) Karakurt, Cemşit; Çelik, Serkan Fazlı; Selimoğlu, Mukadder Ayşe; Varol, İlknur; Karabiber, Hamza; Yoloğlu, SaimObjective: This study aimed to evaluate strain and strain rate echocardiography in children with Wilson’s disease to detect early cardiac dysfunction. Methods: In this study, 21 patients with Wilson’s disease and a control group of 20 age- and gender-matched healthy children were included. All the patients and the control group were evaluated with two-dimensional (2D) and colour-coded conventional transthoracic echocardiography by the same paediatric cardiologist using the same echocardiography machine (Vivid E9, GE Healthcare, Norway) in standard precordial positions, according to the American Society of Echocardiography recommendations. 2D strain and strain rate echocardiography were performed after the ECG probes of the echocardiography machine were adjusted for ECG monitoring. Longitudinal, transverse and radial strain, and strain rate were assessed from six basal and six mid-ventricular segments of the left ventricle, as recommended by the American Society of Echocardiography. Results: Left ventricular wall thickness, systolic and diastolic diameters, left ventricular diameters normalised to body surface area, end-systolic and end-diastolic volumes, cardiac output and cardiac index values were within normal limits and statistically similar in the patient and control groups (p > 0.05). Global strain and strain rate: the patient group had a statistically significant lower peak A longitudinal velocity of the left basal point and peak E longitudinal velocity of the left basal (VAbasR) point, and higher global peak A longitudinal/ circumferential strain rate (GSRa) compared to the corresponding values of the control group (p < 0.05). Radial strain and strain rate: end-systolic rotation [ROT (ES)] was statistically significantly lower in the patient group (p < 0.05). Longitudinal strain and strain rate: end-systolic longitudinal strain [SLSC (ES)] and positive peak transverse strain (STSR peak P) were statistically significantly lower in the patient group (p < 0.05). Segmental analysis showed that rotational strain measurement of the anterior and lateral segments of the patient group were statistically significantly lower than the corresponding values of the control group (p < 0.05). Segmental analysis showed statistically significantly lower values of end-systolic longitudinal strain [STSR (ES)] of the basal lateral (p < 0.05) and end-systolic longitudinal strain [SLSC (ES)] of the basal septal segment (p < 0.05) in the patient group. End-systolic longitudinal strain [SLSC (ES)] and positive peak transverse strain (STSR peak P) were statistically significantly lower in the patient group (p < 0.05). Segmental analysis showed statistically significantly lower values of endsystolic longitudinal strain [SLSC (ES)] of the mid-anterior and basal anterior segments (p < 0.05), end-systolic longitudinal strain [STSR (ES)] measurements of the posterior and mid-posterior segments, end-systolic longitudinal displacement [DLDC (ES)] of the basal posterior, mid-posterior and mid-antero-septal segments in the patient group. Conclusion: Cardiac arrhythmias, cardiomyopathy and sudden cardiac death are rare complications but may be seen in children with Wilson’s disease due to copper accumulation in the heart tissue. Strain and strain rate echocardiography is a relatively new and useful echocardiographic technique to evaluate cardiac function and cardiac deformation abnormalities. Our study showed that despite normal systolic function, patients with Wilson’s disease showed diastolic dysfunction and regional deformation abnormalities, especially rotational strain and strain rate abnormalities.Öğe Subclinical diastolic dysfunction in children with Wilson s disease assessed by tissue Doppler echocardiography a possible early predictor of cardiac involvement(Acta cardiologica, 2013) Elkıran, .Özlem; Karakurt, Cemşit; Selimoğlu, Mukadder Ayşe; Karabiber, Hamza; Koçak, Gülendam; Çelik, Serkan Fazlı; Çolak, CemilBackground The aim of this study was to evaluate the left ventricular systolic and diastolic functions and cardiac rhythm problems for the early detection of myocardial dysfunction in children with Wilson’s disease. Methods We compared patients who had Wilson’s disease (n = 22) with age- and sex-matched healthy control subjects (n = 21). The diagnosis of Wilson’s disease was based on clinical symptoms and laboratory tests (serum ceruloplasmin, urinary and hepatic copper concentrations). Standard echocardiographic examination, as well as pulsed-wave Doppler, tissue Doppler echocardiography examinations of all patients were performed. 24-hour ECG Holter monitoring was also performed in all subjects. Results All patients were asymptomatic on cardiological examination and had sinus rhythm on electrocardiography. Left ventricular ejection fraction, fractional shortening, wall thickness and left ventricular mass were similar in both groups. In comparison to healthy subjects, children with Wilson’s disease had signifi cantly lower mitral E velocity, mitral E/A ratio (P = 0.046, P= 0.021, respectively) and longer isovolumetric relaxation time values (P = 0.015) as estimated by pulsed wave Doppler echocardiography. Wilson patients had longer isovolumetric relaxation time (IVRT) values estimated by tissue Doppler echocardiography (P = 0.006) compared to the controls. On 24-hour ECG Holter monitoring, none of the Wilson patients showed life-threatening cardiac arrhythmia. Conclusion Our study showed results that might be consistent with subclinical diastolic dysfunction in cardiologically asymptomatic Wilson’s disease children which probably represents an early stage of cardiac involvement. Therefore it may be useful to monitor these signs in the follow-up of the patients.Öğe Wilson Hastalıklı Çocuklarda Böbrek Fonksiyonlarının Değerlendirilmesi(Güncel Pediatri, 2017) Tabel, Yılmaz; Selimoğlu, Mukadder Ayşe; Varol, Fatma İlknur; Elmas, Ahmet Taner; Karabiber, Hamza; Güngör, ŞükrüÖz: Giriş: Bu çalışmada Wilson hastalığında (WH) böbrek fonksiyonlarının üriner N-Asetil-?-D-glukozaminidaz (NAG) ve NAG/kreatinin aktivite indeksi kullanılarak değerlendirilmesi amaçlanmıştır. Gereç ve Yöntem: WH tanısı alan 20 hasta çalışma grubu, benzer yaş ve cinsiyette olan 37 sağlıklı çocuk kontrol grubu olarak alındı. NAG düzeyleri mlU/L olarak hesaplanırken, NAG aktivite indeksi mlU/mg kreatinin olarak belirlendi. Bulgular: Toplam dört hasta (%20) tanı anında asemptomatik iken; sırasıyla kronik hepatit, nörolojik WH, fulminan hepatit ve akut hepatit ise altı (%30), beş (%25), üç (%15) ve iki (%10) hastada saptandı. Çalışma anında toplam 13 hasta (%65) trientin ve çinko tedavisi alırken, yedi (%35) hasta ise D-penisilamin ve çinko tedavileri almaktaydı. Olguların 10’unda (%50) proteinüri, birinde (%5) glikozüri ve bir hastada (%5) mikroskopik hematüri saptandı. Hasta grubunun idrar sodyumu ve kreatinini kontrol grubundan anlamlı olarak düşük iken (p=0,048 ve p=0,001), NAG ve NAG indeks anlamlı olarak daha yüksek saptandı (p=0,049 ve p=0,03). Klinik başvuru şeklinin bu parametreler üzerinde etkili olmadığı gözlendi (p>0,05). İdrar kreatinini trientin alanlarda anlamlı olarak yüksek bulundu (p=0,004). Child-Pugh skoru ile bu parametreler arasında herhangi bir ilişki saptanmadı (p>0,05). Sonuç: Bu çalışmada; WH’li çocuklarda WH’nin kendisinin ve/veya kullanılan ilaçların böbrek fonksiyonları üzerine olumsuz etkisinin olduğu, karaciğer hasarının ciddiyeti ve klinik özelliklerin etkisinin olmadığı görülmüştür. Başlık (İngilizce): Evaluation of Renal Function in Children with Wilson’s Disease Öz (İngilizce): Introduction: In this study it was aimed to evaluate the renal functions in Wilson’s disease (WD) using urinary N-acetyl-?-D-glucosaminidase (NAG) and NAG/ creatinine activity index. Materials and Methods: Twenty children of similar age and gender with WD were determined to be the patient group and 37 healthy children were determined to be the control group for the study. NAG levels were calculated as mlU/L and NAG activity index was determined as mlU/mg. Results: While four (20%) patients were asymptomatic at diagnosis, chronic hepatitis, neurologic WD, fulminant hepatitis, and acute hepatitis were observed in six (30%), five (25%), three (15%), and two (10%) patients, respectively. Of children, 13 (65%) were on trientine and zinc treatment and seven (35%) were on d-penicillamine and zinc. Ten (50%) children had proteinuria, one (5%) had glycosuria, and one (5%) had microscopic hematuria. While mean urine sodium and creatinine levels were significantly lower compared to controls (p=0.048 and p=0.001, respectively), NAG and NAG index were significantly higher (p=0.049 and p=0.03). Clinical presentation was observed to be not effective on those parameters (p>0.05). No relationship was found between Child-Pugh score and these parameters (p>0.05). Conclusions: It was concluded that while WD itself and/or the drugs that are used for the treatment have negative effects on renal functions for children with WD, however it does not have any effect on the liver damage severity and clinical presentation of the disease.Öğe Wilson hastalıklı çocukların nütrisyonel değerlendirmesi: tek merkez deneyimi(2019) Güngör, Şükrü; Selimoğlu, Mukadder Ayşe; Varol, Fatma İlknurÖz: Amaç: Beslenme durumu, kronik karaciğer hastalığı olan çocuklarda prognostik bir belirteç olarak kabul edilmiştir. Dizinde, Wilson hastalarında yetersiz beslenme ve mikro besin eksikliğinin sıklığı ve prognostik etkilerini araştıran çalışmaların eksikliği nedeniyle merkezimizde izlenen 94 Wilson hastasını geriye dönük olarak incelemeyi amaçladık. Gereç ve Yöntemler: Çalışmamıza 2006–2017 yılları arasında İnönü Üniversitesi Tıp Fakültesi Çocuk Gastroenteroloji, Hepatoloji ve Beslenme bölümünde 94 Wilson hastalıklı çocuk alındı. Bu hastaların nutrisyonel durumunu değerlendirebilmek için, başvuru şekilleri, antropometrik ölçümleri, laboratuvar bulguları, prognostik etmenleri geriye dönük olarak incelendi. Bulgular: Hastalar demografik özelliklerine göre değerlendirildiğinde yaş ortalaması: 9,11±3,2 (3,5–17) ve kız/erkek oranı 40/54 idi. Yaş ortalaması asemptomatik hastalarda semptomatik olanlardan anlamlı olarak düşük bulundu (p<0,001). Hastalarda tüm değişkenler birlikte değerlendirildiğinde 43 hastada (%45,7) malnütrisyon saptandı. Fulminan Wilson hastalarında nörowilson hastalarına göre boy ve kilo Z skorlarında istatiksel olarak anlamlı yükseklik vardı. (sırasıyla, p=0,045, p=0,019) Hipokalsemi, hipofosfatemi, hipoürisemi, hipoalbuminemi ve anemi kolestazlı hastalarda kolestazı olmayanlara oranla istatiksel olarak daha sık görüldü (p=<0,001). Vitamin A, E kolestazı olanlarda olmayanlara kıyasla daha düşüktü (p<0,05). Hipokalsemi, hipofosfatemi ve hipoürisemi fulminan grupta daha yüksek oranda saptandı (p<0,001). Mortalite skorlarına göre bakıldığında (Dhawan, model for end-stage liver disease ve Child-Pugh); Mortalitesi yüksek olan hastalarda, boy Z skoru yüksek bulundu (p<0.05). Çıkarımlar: Wilson hastalığında büyümenin değerlendirilmesinde ayrıntılı antropometrik ölçümlerin yanında vitamin, eser elementler ve elektrolitlerin de yakından izlenmesi gerekmektedir..
