Tıp Fakültesi / Faculty of Medicine
Permanent URI for this collectionhttps://hdl.handle.net/11727/1403
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Item Results of Pediatric Liver Transplant: A Single-Center Experience(2015) Moray, Gokhan; Tezcaner, Tugan; Akdur, Aydincan; Ozcay, Figen; Sezgin, Atilla; Kirnap, Mahir; Yildirim, Sedat; Arslan, Gulnaz; Haberal, Mehmet; 0000-0002-3641-8674; 0000-0002-8726-3369; 0000-0002-3462-7632; 0000-0002-5735-4315; 0000-0002-5214-516X; 0000-0003-2498-7287; 25894129; AAH-9198-2019; AAD-9865-2021; AAA-3068-2021; AAJ-8097-2021; AAF-4610-2019; ABG-5684-2020; AAE-1041-2021Objectives: Liver transplant is an established curative therapy for children with chronic end-stage liver disease or acute liver failure. In this study, we aimed to evaluate pediatric liver transplant in terms of outcomes, complications, and long-term follow-up results. Materials and Methods: Pediatric patients who had liver transplant in our institution were included. We retrospectively evaluated demographic features including body weight, Child-Pugh score, etiology of liver disease, graft source, perioperative outcomes, perioperative complications, postoperative complications, and long-term results. Outcomes of treatment of complications and revision transplant were evaluated. Results: Between September 2001 and December 2013, there were 188 pediatric liver transplants performed in our institution. Most grafts (90.9%) were obtained from living-related donors. There were 13 patients (6.9%) who had an intervention because of a hemorrhage postoperatively. Biliary leakage was observed in 33 patients (17.5%) and biliary stricture during follow-up was observed in 32 patients (17%). Thrombosis rates in the hepatic artery and portal vein were 12.3% and 0.5%. Revision transplant was performed in 11 patients (5.8%); reason for revision transplant was rejection in 50% patients. The remaining children were alive with good graft functioning after treatment of complications and revision transplant. The overall 5- and 10-year survival rates were 82.3% and 78.9%. Conclusions: The overall outcomes of pediatric liver transplant at our center are very promising. With improved care of younger children and the combined efforts of the parents and medical team, the number of the children receiving transplants will increase in the future.Item HPSE2 Mutations in Urofacial Syndrome, Non-Neurogenic Neurogenic Bladder and Lower Urinary Tract Dysfunction(2015) Bulum, Burcu; Ozcakar, Z. Birsin; Duman, Duygu; Cengiz, Filiz Basak; Kavaz, Asli; Burgu, Berk; Baskin, Esra; Cakar, Nilgun; Soygur, Tarkan; Ekim, Mesiha; Tekin, Mustafa; Yalcinkaya, Fatos; 0000-0003-4361-8508; 25924634; B-5785-2018Background: Urofacial syndrome (UFS) is characterised by congenital bladder dysfunction accompanied by a characteristic abnormal grimace upon smiling and crying. In recent years, biallelic mutations of HPSE2 and LRIG2 have been reported in UFS patients. Non-neurogenic neurogenic bladder (NNNB) has a bladder identical to UFS without typical facial features. The aim of this study was to analyse HPSE2 mutations in patients with UFS and NNNB or severe lower urinary tract dysfunction (LUTD) without abnormal facial expression. Methods: Patients with UFS, NNNB and severe LUTD were enrolled in the study. We examined a total of 35 patients from 33 families. There were seven UFS patients from five different families, 21 patients with NNNB and seven with LUTD. HPSE2 gene mutation analysis was performed using the polymerase chain reaction protocol followed by Sanger sequencing in these patients. Results: A twin pair with UFS was found to be homozygous for c.457C>T (p.Arg153*) mutation. No other pathogenetic variant was detected. Conclusion: HPSE2 mutations were found in one UFS family but not detected in patients with NNNB and severe LUTD. Considering the increasingly recognised cases of NNNB that were diagnosed in early childhood period, genetic factors appear to be responsible. Thus, further genetic studies are needed to discover novel associated gene variants in these bladder anomalies. (C) 2015 S. Karger AG, BaselItem Bloody Nipple Discharge As A Benign, Self-Limiting Disorder in Young Children: A Systematic Review Including Two Related Case Reports(2015) Acer, Tugba; Derbent, Murat; Hicsonmez, Akgun; 0000-0001-5391-9094; 26410727; E-4455-2019Background/purpose: Bloody nipple discharge (BND) is rare, distressing for parents, and presents a challenge for physicians. Methods: We used PubMed to search for cases of BND that were diagnosed before adolescence and added data from two of our cases. Results: The analyzed cohort comprised 46 patients (28 boys and 18 girls; mean [SD] age, 12.5 +/- 13.3 months; range, 20 days to 4 years). The mean time for spontaneous resolution was 2.8 +/- 2.4 months (range, 1 week to 8 months) after onset of BND without any intervention. The diagnosis was mammary ductal ectasia (MDE) in 15 patients, gynecomastia with MDE in two patients, hemorrhagic cysts in two patients, and gynecomastia alone in one patient. The majority (89.3%) of patients <1 year old were managed conservatively, but half of them aged >1 year (50.0%) underwent surgery. Surgery was performed more often in patients in whom a mass had been identified. Conclusions: Age and findings at physical examination affect selection of treatment, but not sex. We found no reported cases of malignancy. Symptoms in children who are managed conservatively resolve within 10 months. Children with BND should be conservatively managed to avoid the risk of developing breast deformities before adolescence. (c) 2015 Elsevier Inc. All rights reserved.Item The Sociodemographic and Clinical Features of Children with Obsessive Compulsive Disorder in A University Hospital In Turkey(2015) Kutuk, M.; Toros, F.; Sogut, F.; Erden, S.; Sanberk, S.; Yildirim, V.; 0000-0002-2918-7871; JMC-7945-2023; AAI-9626-2021; O-9225-2015Item Coexistence of Familial Mediterranean Fever and Hyperimmunoglobulinemia D Syndrome in a Child(2015) Yilmaz, Resul; Sezer, Taner; Esmeray, Haluk; 0000-0001-7672-8100; 0000-0002-2278-1827; A-2825-2012; AAJ-5931-2021Hereditary periodic fever syndromes are Mendelian inherited single gene diseases which are also known as hereditary autoinflammatory syndromes, are characterized by recurrent attacks of fever and inflammation. Familial Mediterranean Fever and Hyperimmunoglobulinemia D syndrome are prototypes and are inherited autosomal recessively. The diagnosis is based on clinical course, family history and is confirmed with genetic mutation analysis. We describe a 5-year-old boy who had recurrent attacks of fever, skin rash, and cervical lymphadenopathy since he was 2 years old. His genetic analysis revealed homozygous M694V and V377I for MEFV and MVK gene respectively. Due to our knowledge, this is the first report of a patient who has both HIDS and FMF clinical and genetic features.Item Report of 3 Patients With Urea Cycle Defects Treated With Related Living-Donor Liver Transplant(2015) Ozcay, Figen; Baris, Zeren; Moray, Gokhan; Haberal, Nihan; Torgay, Adnan; Haberal, Mehmet; 0000-0003-2498-7287; 0000-0001-9852-9911; 0000-0002-6829-3300; 0000-0002-5214-516X; 0000-0002-3462-7632; 26640932; AAE-1041-2021; AAB-4153-2020; AAK-4587-2021; AAJ-5221-2021; ABG-5684-2020; AAJ-8097-2021Urea cycle defects are a group of metabolic disorders caused by enzymatic disruption of the urea cycle pathway, transforming nitrogen to urea for excretion from the body. Severe cases present in early infancy with life-threatening metabolic decompensation, and these episodes of hyperammonemia can be fatal or result in permanent neurologic damage. Despite the progress in pharmacologic treatment, long-term survival is poor especially for severe cases. Liver trans plant is an alternative treatment option, providing sufficient enzymatic activity and decreasing the risk of metabolic decompensation. Three patients with urea cycle defects received related living-donor liver transplants at our hospital. Patients presented with late-onset ornithine transcarbamylase deficiency, argininosuccinate lyase deficiency, and citrullinemia. Maximum pretransplant ammonia levels were between 232 and 400 mu mol/L (normal range is 18-72 mu mol/L), and maximum posttransplant values were 52 to 94 mu mol/L. All patients stopped medical treatment and dietary protein restriction for urea cycle defects after transplant. The patient with late-onset ornithine transcarbamylase deficiency already had motor deficits related to recurrent hyperammonemia attacks pretransplant. A major improvement could not be achieved, and he is wheelchair dependent at the age of 6 years. The other 2 patients had normal motor and mental skills before transplant, which have continued 12 and 14 months after transplant. Hepatic artery thrombosis in the patient with the ornithine transcarbamylase deficiency, intra-abdominal infection in the patient with argininosuccinate lyase deficiency, and posterior reversible encephalopathy syndrome in the patient with citrullinemia were early postoperative complications. Histopathologic changes in livers explanted from patients with ornithine transcarbamylase deficiency and citrullinemia were nonspecific. The argininosuccinate lyase-deficient patient had portoportal fibrosis and cirrhotic nodule formation. In conclusion, liver transplant was a lifesaving procedure for our patients. Proper timing for transplant is important because high ammonia levels may result in permanent neurologic damage; however, transplant at younger ages also may increase morbidity.Item Overview of Therapeutic Plasma Exchange in Pediatric Neurology: A Single-Center Experience(2018) Ozkale, Murat; Erol, Ilknur; Ozkale, Yasemin; Kozanoglu, Ilknur; https://orcid.org/0000-0003-0625-1057; https://orcid.org/0000-0002-3530-0463; https://orcid.org/0000-0003-3009-336X; https://orcid.org/0000-0002-5268-1210; 29882008; A-7806-2016; AAK-4825-2021; AAL-6136-2021; AAE-1241-2021Therapeutic plasma exchange (TPE) is used in the treatment of neurological, hematological, renal and autoimmune diseases with known or suspected immune pathogenesis. In comparison with neurological diseases of adults, knowledge about the use of TPE in children is incomplete. We report our experience on TPE in children with neurological diseases in a single institution and describe the underlying etiology, clinical course, treatment and outcome. We retrospectively evaluated 22 consecutive children (12 girls, 10 boys, aged 2-16 years) who underwent TPE in the pediatric intensive care unit between January 2010 and January 2017. There were 135 TPE procedures with median 6 TPE sessions per patient. Fresh frozen plasma was used as a replacement fluid in all cases. Most common indications were inflammatory polyneuropathy followed by acquired demyelinating diseases of the central nervous system. Other indications were autoimmune encephalitis and paraneoplastic limbic encephalitis. No mortality was recorded during TPE. The complication rate was 2.2% and consisted of transient events like hypotension and allergic reactions. Therapetic plasma exchange is one of the safe methods of treatment for neuroimmunological disorders in children, with Guillain-Barr, syndrome as the most common indication.Item Rituximab Therapy for Rejection in Pediatric Heart Transplant(2018) Erdogan, Ilkay; Varan, Birgul; Sezgin, Atilla; Pirat, Arash; Zeyneloglu, Pinar; 0000-0002-6719-8563; 0000-0003-2312-9942; 0000-0001-6887-3033; 27210774; ABB-1767-2021; C-3736-2018; ABB-2220-2021Objectives: Humoral rejection is the B-cell-mediated production of immunoglobulin G antibody against the transplanted heart. Antibody-mediated rejection may be resistant to standard immunosuppressive therapy and is associated with high mortality and graft loss. Rituximab can be used to treat antibodymediated rejection in heart transplant recipients. This retrospective study describes our experience with rituximab treatment in children with heart transplants. Materials and Methods: We present 7 pediatric patients with antibody-mediated rejection who were treated with plasma exchange and rituximab therapy. Rituximab was given at a dose of 375 mg/m2 by slow infusion in the intensive care unit after 5 days of plasmapheresis, in addition to a conventional regimen consisting of steroids, mycophenolate mofetil, and tacrolimus. The peripheral blood count and sodium, potassium, serum urea nitrogen, creatinine, aspartate aminotransferase, and alanine aminotransferase levels were measured in all patients before and after treatment. Results: Seven patients were treated with plasma exchange and rituximab. We repeated this therapy in 5 patients because of refractoriness or recurrent rejection. After diagnoses of antibody-mediated rejection, 4 patients died within 6 months (mortality rate of 57.1%). We did not observe any adverse effects or complications related to rituximab. Conclusions: Rituximab can be used in humoral rejection after pediatric heart transplant. However, the success of the treatment is controversial, and further study is needed to find an effective treatment for antibody-mediated rejection and steroid-resistant cellular rejection in children.Item Pediatric Liver Transplantation Experience(2016) Haberal, Mehmet; https://orcid.org/0000-0002-3462-7632; AAJ-8097-2021Background In this study, we aimed to evaluate pediatric liver transplantation in terms of outcomes, complications, and long-term follow-up results. Materials and methods Between September 2001 and August 2015, 215 pediatric liver transplants were performed in our institution. Results Posttransplant biliary complications were biliary leakage (16.2 %) and biliary stricture (14.8 %). Complication rates in hepatic artery and portal vein were 16.7 % and 4.6 %. Overall mortality rate was 17.8 %. The overall 5aEuroyear survival rate was 82.3 % and 10-year survival rate was 78.9 %. Conclusion With improved care of younger children and the combined efforts of the parents and medical team, the number of the children receiving transplants with good outcomes will continue to increase.Item Meckel Diverticulum in Children: Evaluation of Macroscopic Appearance for Guidance in Subsequent Surgery(2016) Gezer, Hasan Ozkan; Temiz, Abdulkerim; Ince, Emine; Ezer, Semire Serin; Hasbay, Bermal; Hicsonmez, Akgun; 0000-0002-4635-2613; 0000-0001-8789-6003; 0000-0002-9597-3264; 26435520; J-3197-2013; A-4719-2018; AAJ-9529-2021Background: The treatment of incidentally encountered asymptomatic Meckel diverticulum(MD) is controversial. We evaluated whether the macroscopic appearance correlates with clinical features, histopathological findings, future complications, and management decisions. Methods: Patients who underwent MD resection at a single institution from 2000 to 2012 were retrospectively analyzed in terms of age, sex, clinical features, laboratory data, perioperative findings (diverticulum length, diameter, depth, thickening, and height-to diameter ratio [HDR]), pathology, and postoperative follow-up. Results: Fifty children were enrolled. Sixteen percent of the resected MDs were found incidentally. Of 42 complicated MDs, 17 (40%) were long (HDR >= 2), 14 (33%) were thickened, and 29 (70%) exhibited base widening. Histopathologically, ectopic mucosa was found in 32 (64%) of all MDs and in 5 (62%) of incidentally removed MDs. There was no statistical difference between the macroscopic appearance and clinical signs, sex, or presence of ectopic tissue based on palpation. Conclusion: The macroscopic appearance of MD does not indicate the presence or absence of HGM and cannot be used to guide subsequent surgery. Additionally, 40% of symptomatic patients in our study had life-threatening complications requiring prompt fluid resuscitation. We consider that incidentally detected MD should be removed regardless of its macroscopic appearance. (C) 2016 Elsevier Inc. All rights reserved.
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