Wos Açık Erişimli Yayınlar

Permanent URI for this collectionhttps://hdl.handle.net/11727/10754

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    Three-Country Snapshot of Ornithine Transcarbamylase Deficiency
    (2022) Aydin, Halil Ibrahim; https://orcid.org/0000-0001-7994-4394; 36362876
    X-linked ornithine transcarbamylase deficiency (OTCD) is the most common urea cycle defect. The disease severity ranges from asymptomatic carrier state to severe neonatal presentation with hyperammonaemic encephalopathy. We audited the diagnosis and management of OTCD, using an online 12-question-survey that was sent to 75 metabolic centres in Turkey, France and the UK. Thirty-nine centres responded and 495 patients were reported in total. A total of 208 French patients were reported, including 71 (34%) males, 86 (41%) symptomatic and 51 (25%) asymptomatic females. Eighty-five Turkish patients included 32 (38%) males, 39 (46%) symptomatic and 14 (16%) asymptomatic females. Out of the 202 UK patients, 66 (33%) were male, 83 (41%) asymptomatic and 53 (26%) symptomatic females. A total of 19%, 12% and 7% of the patients presented with a neonatal-onset phenotype in France, Turkey and the UK, respectively. Vomiting, altered mental status and encephalopathy were the most common initial symptoms in all three countries. While 69% in France and 79% in Turkey were receiving protein restriction, 42% were on a protein-restricted diet in the UK. A total of 76%, 47% and 33% of patients were treated with ammonia scavengers in Turkey, France and the UK, respectively. The findings of our audit emphasize the differences and similarities in manifestations and management practices in three countries.
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    Disease Burden And Management Of Crigler-Najjar Syndrome: Report Of A World Registry
    (2022) Aronson, Sem J.; Junge, Norman; Trabelsi, Mediha; Kelmemi, Wided; Hubert, Aurelie; Brigatti, Karlla W.; Fox, Michael D.; de Knegt, Robert J.; Escher, Johanna C.; Ginocchio, Virginia M.; Iorio, Raffaele; Zhu, Yan; Ozcay, Figen; Rahim, Fakher; El-Shabrawi, Mortada H. F.; Shteyer, Eyal; Di Giorgio, Angelo; D'Antiga, Lorenzo; Mingozzi, Federico; Brunetti-Pierri, Nicola; Strauss, Kevin A.; Labrune, Philippe; Mrad, Ridha; Baumann, Ulrich; Beuers, Ulrich; Bosma, Piter J.; 35274801
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    Analysis of Quality of Life, Depression, and Sexual Function in Patients on the Liver Transplant List
    (2021) Fidan, Cihan; Akdur, Aydincan; Kirnap, Mahir; Selcuk, Haldun; Yildirim, Sedat; Moray, Gokhan; Haberal, Mehmet; 0000-0002-3462-7632; 0000-0002-8726-3369; 34609310; AAJ-8097-2021; AAA-3068-2021
    Background: I he only treatment in patients developing liver failure is liver transplantation. According to the Ministry of Health, the number of patients waiting for a liver transplantation is 2141, the average waiting period for liver transplantation is approximately 5 years, and 15-18% of these patients lose their life while waiting for transplantation. In these patients, limitations in daily activities and depression-anxiety are commonly found. The aim of this study was to analyze life quality, depression symptoms, and existence of sexual functional disorders of patients waiting for liver transplantation. Methods: A total of 74 patients, who were registered in Baskent University Hospital between 2015 and 2018, were included into the study. Short Form-36, Beck Depression Inventory, and Arizona Sexual Experiences Scale were applied to 56 patients who approved study. Results: Most of the patients were male (64.3%), and the mean age was 46 (18-64). Short Form-36 scores were low in all patients. The mean Beck Depression Inventory score of patients was found as 18.4 +/- 11.3, and they were suffering from moderate depressive symptoms. According to Arizona Sexual Experiences Scale, total mean scores of males was found as 16.3 +/- 5.5, and for females, it was 19.5 +/- 5.3 with a statistically significant difference (P < .05). It was found that sexual dysfunction mostly had moderate to mild. Conclusion: Depression and sexual dysfunction are common in patients with chronic liver diseases, and their life qualities deteriorate significantly. It is anticipated that evaluation of these patients in terms of psychological issues and sexual dysfunction will increase their quality of life during the organ waiting period and affect their well-being post-transplant.
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    Portopulmonary hypertension in liver transplant candidates
    (2016) Bozbas, Serife Savas; Bozbas, Huseyin; 26877607
    Pulmonary vascular disorders including portopulmonary hypertension (PoPHT) are among the common complications of liver disease and are prognostically significant. Survival is very low without medical treatment and liver transplantation. With advances in medical therapy for elevated pulmonary artery pressure (PAP) and liver transplant surgery, survival of patients with PoPHT and advanced liver disease is significantly improved. Because of the prognostic significance of PoPHT and the limited donor pool, a comprehensive preoperative cardio-pulmonary assessment is of great importance in cirrhotic patients prior to transplant surgery. Therefore, a detailed transthoracic Doppler echocardiographic examination must be an essential component of this evaluation. Patients with mild PoPHT can safely undergo liver transplant surgery. In cases of moderate to severe PoPHT, right heart catheterization (RHC) should be performed. In patients with moderate to severe PoPHT on RHC (mean PAP 35-45 mmHg), vasodilator therapy should be attempted. Liver transplantation should be encouraged in cases that demonstrate a positive response. Bridging therapy with specific pulmonary arterial hypertension treatment agents should be considered until the transplant surgery and should be continued during the peri-and post-operative periods as needed.