Tıp Fakültesi / Faculty of Medicine
Permanent URI for this collectionhttps://hdl.handle.net/11727/1403
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Item Literature Review and 2 Cases of Isolated Chylothorax After Pediatric Living-Donor Liver Transplant(2023) Tirnova, Ismail; Alim, Altan; Vehbi, Sezan; Demir, Baris; Akbulut, Akin; Karatas, Cihan; Kanmaz, Turan; 0000-0003-4488-1607; 37503801; ISB-9235-2023Objectives: Chylothorax without chylous ascites after liver transplant is rare. We present 2 cases of isolated chylothorax after liver transplant and a literature review. Materials and Methods: We compiled a literature review of chylothorax cases after abdominal surgery and analyzed the cases related to liver transplant. The demographic information, follow-up results, and treatment details of our 2 cases of chylothorax after living-donor pediatric liver transplant were discussed. Results: An 8-month-old child and a 15-month-old child with cholestatic liver disease and urea cycle defect, respectively, underwent living-donor left lateral segment liver transplant. Patients who presented with chylothorax after discharge were treated conservatively. Conclusions: Isolated chylothorax is rare complication after abdominal surgery, which is mostly possible to treat with conservative methods. Interventional procedures and a surgical approach should only be performed in resistant cases when conservative treatment has failed.Item The Middle Ear Functions and Vestibular-Evoked Potentials in Springboard-Platform Diving Children(2023) Borisova, Natalia; Ozer, Fulya; Kuntman, Berna Deniz; Erbek, Seyra H.; 0000-0002-8453-6069; 0000-0003-0038-8167; AAJ-2445-2021; JNE-6747-2023Objective: To examine the middle ear and Eustachian tube functions of children who perform springboard and platform diving sports professionally and to evaluate the vestibulospinal and vestibuloocular reflexes of these athletes with evoked myogenic potentials.Methods: Two groups consist of athletes and a control group. Pure tone audiometry and speech audiometry, Eustachian tube function test and resonance frequency in multifrequency tympanometry, and ocular/cervical vestibular-evoked myogenic potentials were performed in all participants.Results: Forty-five ears in 25 athletes were evaluated as non-patent Eustachian tube. In athlete girls, resonance frequency mean value was measured lower than the control group in both ears. Cervical vestibular-evoked myogenic potentials amplitude mean values were statistically lower in athlete girls in the left ear (P = .031), and ocular vestibular-evoked myogenic potentials amplitude mean values were statistically higher in athlete boys in the left ear (P = .024). Conclusions: Repeated diving from very high meters platform did not cause significant difference on resonance frequency of the middle ear, but cause frequently common Eustachian tube dysfunction. Acrobatic movements on the air before the diving caused changes in ocular/cervical vestibular-evoked myogenic potentials amplitude values of athletes. Eustachian tube function should be followed at different times of the year to see any effect on the performance of this sport. The importance of the vestibular system and the medial vestibulospinal tract in spring-board and platform diving athletes was emphasized first in this study. To monitor health of vestibular system and middle ear with different and more specific test materials may be important for their longer professional careers. However, this issue should be proven with future studies.Item An Infrequent Case of Sudden Flushing: Infantile Cutaneous Mastocytosis(2023) Gultekingil, A.; Olcay, L.; Togral, A. Karatas; Ayva, E. S.; 0000-0001-7955-5735; AAR-9945-2020Introduction: Sudden flushing is a common symptom in infants but it can be a manifestation of lifethreatening disease, therefore differential diagnosis is crucial for an infant with flushing. Case Description: Here, we describe a two-month-old boy who presented to the Paediatric Emergency Department with four sudden attacks of flushing with accompanying fatigue, especially after feedings. Upon physical examination, a 5x7 cm brown lesion on his left hypochondra was noted. His laboratory tests were unremarkable. Pathological examination of a biopsy of the lesion revealed diffuse mast cell infiltration. The patient was diagnosed with cutaneous mastocytosis. Conclusion: This case underscores the importance of the detailed examination of children with sudden attacks of flushing to make a correct diagnosis and to prevent future life-threatening complications of infrequent clinical entities.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 COVID-19 Infections in Pediatric Renal Transplant Recipients(2022) Yilmaz, Aysun Caltik; Baskin, Esra; Gulleroglu, Kaan; Karakaya, Deniz; Akdur, Aydincan; Moray, Gokhan; Haberal, Mehmet; https://orcid.org/0000-0003-0774-4419; https://orcid.org/0000-0003-1434-3824; https://orcid.org/0000-0002-3462-7632; 35384829; AAD-1877-2021; AAJ-8833-2021; AAJ-8097-2021Objectives: The new coronavirus SARS-CoV-2 (COVID-19) first appeared in Turkey in March 2020, spread rapidly, and caused many deaths. Although COVID-19 is mostly a respiratory disease, it can cause kidney and multiorgan failure in some cases. We believe that by sharing information about the course and effects of COVID-19 infection in kidney transplant recipients receiving long-term immunosuppressive therapy our understanding will improve. Materials and Methods: Between March 2020 and October 2021, COVID-19 was researched in kidney transplant recipients under the age of 20 years who were followed at the Baskent University Transplantation Center. We documented the clinical characteristics and prognosis of pediatric kidney transplant recipients with COVID-19 disease. Results: Our study group included 23 patients with COVID-19 infection from 215 pediatric kidney transplant recipients. The mean age of the patients was 14.6 +/- 4.7 years; there were 9 female patients. The mean follow-up time posttransplant was 62.3 +/- 43.2 months. In 13 patients (56.5%), fever was the most frequent symptom. Most patients (n = 18, 78%) had minor symptoms and recovered completely after receiving supportive treatment. Four patients (17%) required hospitalization. One was diagnosed with COVID-19 infection 1 week after being treated with rituximab for acute antibody-mediated rejection. That patient died because of significant lung disease and multiorgan failure. Conclusions: Despite the fact that most of our pediatric transplant recipients had mild symptoms of COVID-19, we believe that particular caution should be observed in patients who have recently received intensive immunosuppressive medications. As a result of potential new vaccines, national immunization programs, and the emergence of novel virus strains, the clinical picture may change in the future. We believe that, as information sharing increases, we will learn more about COVID-19 in renal transplant recipients.