Browsing by Author "Selcuk, Haldun"

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ACUTE NECROTIZING PANCREATITIS AFTER TRANSARTERIAL CHEMOEMBOLIZATION IN A PATIENT WITH HEPATOCELLULAR CANCER: CASE REPORT AND REVIEW OF THE LITERATURE
(2019) Ocal, Serkan; Suna, Nuretdin; Etik, Digdem Ozer; Bovyat, Fatih; Selcuk, Haldun; 31574073
Acute Pancreatitis Caused by Primary Pancreatic Lymphoma in A Geriatric Patient with Sarcoidosis: Report of A Complex Case
(2017) Ozer Etik, Digdem; Suna, Nuretdin; Tepeoglu, Merih; Coskun, Mehmet; Selcuk, Haldun; 0000-0002-4724-0728; 0000-0001-6234-7788; 0000-0002-9894-8005; 0000-0001-5630-022X; 0000-0002-8445-6413; AAJ-4707-2021; AAI-8822-2021; AAK-5222-2021; AAM-4120-2021; AAJ-6976-2021
The purpose of reporting this case is to raise the awareness of the rapid worsening clinical presentation of acute pancreatitis in an older patient, unexpected encounter with pancreatic lymphoma, and sarcoidosis-lymphoma syndrome. An 80-year-old woman was diagnosed with sarcoidosis 16 years ago. Following recent hospitalization because of acute pancreatitis, non-obstructive and non-invasive pancreatic mass was found on radiological images. Histopathology confirmed the mass to be diffuse large B-cell lymphoma. We report this case because of its complicated the etiology of acute pancreatitis in an older patient, presentation of pancreatic lymphoma, and immune pathogenesis of sarcoidosis-lymphoma syndrome.
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.
Clinical Course of Inflammatory Bowel Disease and Primary Sclerosing Cholangitis after Liver Transplantation
(2018) Dagli, Ulku; Etik, Digdem Ozer; Akdur, Aydincan; Selcuk, Haldun; Hilmioglu, Fatih; Boyacioglu, Sedat; Moray, Gokhan; Haberal, Mehmet; 0000-0003-0664-0976; 0000-0002-4724-0728; 0000-0002-8726-3369; 0000-0002-8445-6413; 0000-0002-6440-5686; 0000-0002-9370-1126; 0000-0003-2498-7287; 0000-0002-3462-7632; S-4068-2018; AAJ-4707-2021; AAA-3068-2021; AAJ-6976-2021; AAJ-4437-2021; AAE-7637-2021; AAE-1041-2021; AAJ-8097-2021
The Correlation Between Platelet Counts and Spleen Size After Successful Liver Transplantation
(2016) Ensaroglu, Fatih; Ocal, Serkan; Boyacioglu, Ahmet Sedat; Selcuk, Haldun; Hilmiogluu, Fatih; Moray, Gokhan; Haberal, Mehmet; https://orcid.org/0000-0003-3719-9482; https://orcid.org/0000-0002-9370-1126; https://orcid.org/0000-0002-8445-6413; https://orcid.org/0000-0003-2498-7287; https://orcid.org/0000-0002-3462-7632; ABH-4817-2020; AAE-7637-2021; AAJ-6976-2021; AAE-1041-2021; AAJ-8097-2021
Effect of HLA on Hepatitis C Virus Clearance and Persistence in Anti-HCV-positive End-stage Renal Disease Patients
(2014) Ocal, Serkan; Selcuk, Haldun; Korkmaz, Murat; Altun, Reskan; Yildirim, Abdullah E.; Akbas, Enver; 24976281
Background/Aims: The efficacy of immune response against hepatitis C virus (HCV) is determined by human leukocyte antigen (HLA) molecules of the host which present HCV antigens to CD4+ and CD8+ Tlymphocytes. In this study, we aimed to investigate the possible relationship between the frequencies of certain HLA class I-II alleles and the natural history of HCV in patients with end-stage renal disease (ESRD). Settings and Design: This is a retrospective cohort study conducted in a university hospital. Patients and Methods: The present study comprised 189 ESRD patients (candidates for renal transplantation) who had positive anti-HCV antibody test. The results concerning HCV and HLA status were gathered from patients files. The viral persistence was compared between the groups that were determined by HLA sub-typing. Statistical Analysis: Statistical evaluation was performed using Mann-Whitney U-test, Chi-square test, and Fisher's exact test. Level of error was set at 0.05 for all statistical evaluations, and P values < 0.05 were considered statistically significant. Results: We found possible association between the course of HCV infection and specific HLA alleles. HLA class I Cw*6 and HLA class II DRB*10 alleles were observed more frequently in the viral clearance group (P < 0.05). The HLA class I B*38 allele group was more prone to develop chronic hepatitis C (P < 0.01). Conclusions: These findings suggest that HLA class I Cw*6 and HLA class II DRB*10 alleles may be associated with immunological elimination of HCV in Turkish patients on hemodialysis. HLA sub-typing could help predict the prognosis of HCV infection.
The Effect of Pretransplant Chronic Hepatitis C Virus Infection Treatment on Graft and Patient Survival in Renal Transplant Recipients
(2015) Korkmaz, Murat; Faki, Sevgul; Ocal, Serkan; Harmanci, Ozgur; Ensaroglu, Fatih; Selcuk, Haldun; Haberal, Mehmet; 0000-0002-3462-7632; 0000-0002-8445-6413; 0000-0002-9333-782X; 0000-0003-3719-9482; 0000-0002-0643-4980; 25894152; AAJ-8097-2021; AAJ-6976-2021; AAM-1330-2020; ABH-4817-2020
Objectives: Studies have demonstrated worse graft and patient survival among hepatitis C virus-positive patients following kidney transplant. Eradication of hepatitis C virus infection before renal transplant with interferon should be considered in hepatitis C virus-infected patients undergoing dialysis who are on the waiting list for transplant. We investigated whether pretransplant hepatitis C virus infection treatment affected graft and patient survival, and we evaluated other contributing factors to these outcomes. Materials and Methods: We enrolled 83 antihepatitis C virus-positive patients who were diagnosed with chronic hepatitis C virus infection by serology or histopathology and had renal transplant at Baskent University Ankara Hospital from 1982 to 2013. Data were obtained from patient medical files retrospectively. Patients were divided into 2 groups that had or did not have interferon treatment. Results: In 83 renal transplant patients with chronic hepatitis C virus infection (57 male [69%] and 26 female [31%]), median age was 46 years (range, 26 - 69 y), and most patients were genotype 1-dominant (92%). Interferon monotherapy was received by 30 patients before renal transplant and 28 of 30 patients had long-term follow-up data. There were 14 of 28 patients (50%) who achieved sustained virologic response, and only 1 patient had relapse. Graft survival was significantly lower in patients who had treatment (6 y vs 9 y; P <= .003). However, patient survival rates were similar between groups. Patients who had interferon were younger and had longer hemodialysis duration before renal transplant than patients without treatment. Higher viral load was associated with higher mortality which was caused by sepsis. Conclusions: Pretransplant hepatitis C virus infection treatment, although recommended before renal transplant, does not always have good outcomes. Pretransplant dialysis treatment period, age of recipient, and posttransplant higher viral replication rates may be important contributing factors related to graft and patient survival.
Effect of Propranolol Treatment on the Incidence of Hepatocellular Carcinoma in Patients Waiting for Liver Transplant With Cirrhosis: A Retrospective, Surveillance Study in a Tertiary Center
(2019) Suna, Nuretdin; Etik, Digdem Ozer; Ocal, Serkan; Selcuk, Haldun; 31050621
Objectives: Hepatocellular carcinoma is the most frequent primary malignant tumor of the liver and the third most common cause of all cancer-related mortalities. There is a need to develop new strategies to prevent hepatocellular carcinoma, as the incidence of this cancer continues to increase despite all advancements. In this study, our aim was to determine the effects of propranolol treatment on the incidence of hepatocellular carcinoma in cirrhotic patients waiting for liver transplant. Materials and Methods: We retrospectively reviewed the data of patients waiting for liver transplant with cirrhosis due to various causes registered at the Hepatocellular Carcinoma Surveillance Program between June 2011 and December 2017 in our center. These data were compared between patients using propranolol and those not using propranolol. Results: Of the 231 patients, 135 (58.4%) were male and 96 (41.6%) were female. The mean age was 58.1 +/- 14 years. We noted that 153 of total patients (66.2%) were using propranolol. Three patients (2%) were using 20 mg propranolol, 125 (81.7%) were using 40 mg propranolol, 10 (6.5%) were using 60 mg propranolol, and 15 (9.8%) were using 80 mg propranolol. Of total patients, 36 (15.6%) developed hepatocellular carcinoma, including in 12 patients (7.8%) using propranolol and 24 patients (30.8%) who did not use this agent (P < .001).Thus, the hepatocellular carcinoma frequency was 5.22 times lower in patients receiving propranolol than in those not receiving propranolol. Conclusions: Although causes of cirrhosis and initial stages were similar in both groups using and not using propranolol, incidence of hepatocellular carcinoma was significantly lower in the propranolol group than in the group without propranolol. This result showed that propranolol treatment has a protective effect for hepatocellular carcinoma in patients waiting for liver transplant with cirrhosis.
Effects of Enteral Protein Supplements on Nutritional Status and Clinical Outcome in Unconscious Elderly Malnourished Patients
(2014) Gulsen Atalay, Betul; Yagmur, Cahide; Nursal, Tarik; Atalay, Hakan; Sezgin, Nurzen; Giray, Semih; Selcuk, Haldun; https://orcid.org/0000-0002-0722-3181; https://orcid.org/0000-0002-8445-6413; IQV-1169-2023; AAH-1091-2020; AAJ-6976-2021
Aim: Unconscious malnourished patients have reduced serum proteins. Enteral protein supplements may improve their nutritional status and clinical outcome. Our aim for the present study was to determine the effect on nutritional status and patient prognosis of enteral protein supplementation in tube-fed hospitalised unconscious elderly malnourished patients. Methods: In total, 20 patients were fed a standard enteral nutrition formula during the four-day adaptation period. The study group (n = 10) received an enteral protein supplement (2 g/kg/day protein) and the control group (n = 10) received only standard enteral nutrition (1 g/kg/day protein) during the 12-day study period. Nutritional status and clinical outcome were assessed. Results: No difference was observed between the groups with regard to biochemical, inflammatory parameters, anthropometric measurements or nitrogen balance (P > 0.05). Triceps and subscapular skinfold thickness mean levels were elevated in the study group at the end of the study compared with the beginning of the study (P < 0.05). No significant difference was observed between the groups with regard to complications or mortality rate (P > 0.05). Conclusions: In the present study, the use of enteral protein support (EPS) in unconscious malnourished patients provided some advantages in the early stages of medical treatment. Triceps skinfold thickness and subscapular skinfold thickness mean levels were increased significantly in our study group. Although there was no significant difference, serum insulin-growth factor, retinol-binding protein and prealbumin mean values showed a tendency to increase in the study group at the end of the study. We believe that further research is required to determine the effects of EPS on these values in malnourished elderly patients.
Efficacy and Tolerability of Direct-Acting Antiviral Agents for Hepatitis C Virus Infection in Kidney Transplant Recipients
(2018) Suna, Nuretdin; Etik, Digdem Ozer; Ocal, Serkan; Selcuk, Haldun; Dagli, Ulku; Hilmioglu, Fatih; Boyacioglu, Sedat; Haberal, Mehmet; https://orcid.org/0000-0001-6234-7788; https://orcid.org/0000-0003-3719-9482; https://orcid.org/0000-0002-8445-6413; https://orcid.org/0000-0002-6440-5686; https://orcid.org/0000-0002-9370-1126; https://orcid.org/0000-0002-3462-7632; AAI-8822-2021; ABH-4817-2020; AAJ-6976-2021; AAJ-4437-2021; AAE-7637-2021; AAJ-8097-2021
Expanded Criteria for Hcc in Liver Transplantation
(2017) Haberal, Mehmet; Akdur, Aydincan; Moray, Gokhan; Arslan, Gulnaz; Ozcay, Figen; Selcuk, Haldun; Ozdemir, Handan; 0000-0002-3462-7632; 0000-0002-8726-3369; 0000-0003-2498-7287; 0000-0002-5214-516X; 0000-0002-8445-6413; 0000-0002-7528-3557; AAJ-8097-2021; AAA-3068-2021; AAE-1041-2021; ABG-5684-2020; AAJ-6976-2021; X-8540-2019
Expanded Criteria for Hepatocellular Carcinoma in Liver Transplant
(2017) Haberal, Mehmet; Akdur, Aydincan; Moray, Gokhan; Arslan, Gulnaz; Ozcay, Figen; Selcuk, Haldun; Ozdemir, Handan; 0000-0002-7528-3557; 0000-0003-2498-7287; 0000-0002-8726-3369; 0000-0002-3462-7632; 0000-0002-8445-6413; 0000-0002-5214-516X; 28302000; X-8540-2019; AAE-1041-2021; AAA-3068-2021; AAJ-8097-2021; AAJ-6976-2021; ABG-5684-2020
Objectives: Hepatocellular carcinoma is the sixth most common cancer worldwide and is the third highest cause of malignancy-related death. Because of its typically late diagnosis, median survival is ap proximately 6 to 20 months, with 5-year survival of < 12%. Hepatocellular carcinoma typically arises in the background of cirrhosis, with liver transplant regarded as the optimal therapy for selected patients. Initially, orthotopic liver transplant was limited to patients with extensive unresectable tumors, resulting in uniformly dismal outcomes due to high tumor recurrence rates. Here, we evaluated our long-term results with expanded-criteria liver transplant. Materials and Methods: From December 1988 to January 2017, we performed 552 liver transplants at Baskent University. In candidates with hepatocellular carcinoma, our expanded criteria for liver transplant is applied regardless of tumor size and number, includes those without major vascular invasion and without distant metastasis, and those with negative cytology (if the patient has ascites). Since 1994, of 61 liver transplants for hepatocellular carcinoma, 36 patients received transplants according to our expanded criteria. Results: Of 36 expanded-criteria patients, 11 were children and 25 were adults. Sixteen patients (4 pediatric, 12 adult) were within our expanded criteria both radiologically and pathologically before trans plant. The other 20 patients (7 pediatric, 13 adult) were within Milan criteria radiologically before transplant; however, after liver transplant, when pathologic specimens were evaluated, patients were found to be within our center's expanded criteria. During follow-up, 9/36 patients (25%) had hepatocellular carcinoma recurrence. In pediatric patients, 5-year and 10-year survival rates were 90%; in adults, 5-year survival was 58.7% and 10-year survival was 49.7%. Overall 5-year and 10-year survival rates were 71.7% and 62.7%. Conclusions: Liver transplant is safe and effective in patients with hepatocellular carcinoma in com bination with interventional radiology procedures, regardless of tumor size and number, without major vascular invasion and distant metastasis.
Extensive Indications for HCC in Living Donor Liver Transplantation
(2016) Haberal, Mehmet; Akdur, Aydincan; Moray, Gokhan; Arslan, Gulnaz; Ozcay, Figen; Selcuk, Haldun; Ozdemir, Handan; https://orcid.org/0000-0002-3462-7632; https://orcid.org/0000-0002-8726-3369; https://orcid.org/0000-0003-2498-7287; https://orcid.org/0000-0002-5214-516X; https://orcid.org/0000-0002-8445-6413; AAJ-8097-2021; AAA-3068-2021; AAE-1041-2021; ABG-5684-2020; AAJ-6976-2021
Extraordinary biliary variant
(2017) Suna, Nuretdin; Etik, Digdem Ozer; Ocal, Serkan; Selcuk, Haldun; Hilmioglu, Fatih; Boyacioglu, Sedat; 0000-0003-3719-9482; 0000-0002-9370-1126; 0000-0002-6440-5686; 0000-0002-4724-0728; 0000-0002-8445-6413; 0000-0001-6234-7788; 28336501; ABH-4817-2020; AAE-7637-2021; AAJ-4437-2021; AAJ-4707-2021; AAJ-6976-2021; AAI-8822-2021
Fractalkine receptor polymorphism may not be associated with the development and clinical course of ulcerative colitis
(2015) Gokcan, Hale; Yurtcu, Erkan; Selcuk, Haldun; Sahin, Feride I.; 26042517
Fractalkine (CX3C), a chemokine expressed by epithelial cells within normal and inflamed colorectal mucosa, induces leukocyte adhesion and migration via fractalkine receptor. The aim of this study was to investigate two single nucleotide polymorphisms of the fractalkine receptor gene as a risk factor both for the development and clinical findings of ulcerative colitis. In this study, si patients with ulcerative colitis (UC) and 8o controls were recruited. Genotypes of fractalkine receptorc.743G>A (V249I) and c.839C>T (T280M) polymorphisms were identified by restriction fragment length polymorphism analyses after polymerase chain reaction.Genotype distribution and allele frequencies of V249I and T280M were not statistically significantly different between UC and control groups (p>0.05). No statistically significant relationship was found between fractalkine receptor polymorphisms and clinical findings of UC. We observed no significant difference in fractalldne receptor polymorphism between patients and control group and no genotype-phenotype relation. Therefore, we concluded that fractalkine receptor polymorphisms may not contribute to the molecular pathogenesis of UC.
Hepatitis B- and Hepatitis D-Virus Related Liver Transplant: Single-Center Data
(2015) Ocal, Serkan; Korkmaz, Murat; Harmanci, Ozgur; Ensaroglu, Fatih; Akdur, Aydincan; Selcuk, Haldun; Moray, Gokhan; Haberal, Mehmet; 0000-0002-3462-7632; 0000-0002-8726-3369; 0000-0003-3719-9482; 0000-0002-9333-782X; 0000-0003-2498-7287; 0000-0002-8445-6413; 0000-0002-0643-4980; 25894142; AAJ-8097-2021; AAA-3068-2021; ABH-4817-2020; AAM-1330-2020; AAE-1041-2021; AAJ-6976-2021
Objectives: Hepatitis B and D virus coinfection or superinfection lead to chronic liver disease and have poor treatment results and poor prognosis. After transplant, these patients have difficult problems. We aimed to report long-term data of liver transplant recipients who had hepatitis B and D virus-related chronic liver disease. Materials and Methods: This retrospective, longitudinal study included 25 consecutive hepatitis B surface antigen-positive patients with anti-hepatitis D virus antibodies. Patient data (age, sex, antiviral treatment, posttransplant use of hepatitis B hyperimmunoglobulin and/or nucleoside/nucleotide analogues, the presence of hepatocellular carcinoma, age at transplant, follow-up) were extracted from patient records. Results: Females comprised 32% patients. The median age was 44 years (range, 23-63 y). The serum Hepatitis B envelope antigen level was negative in all patients. At the time of transplant, 4 patients were positive for hepatitis B virus DNA and 11 patients also had hepatocellular carcinoma. Posttransplant follow-up was 59 months (range, 3-120 mo). During follow-up, 4 patients died, 4 patients were lost to follow-up, and 17 patients were alive. Posttransplant survival of patients with hepatocellular carcinoma was 50.45 months (range, 3-84 mo) and without hepatocellular carcinoma was 65.8 months (range, 4-120 mo). There were 3 patients who had acute rejection and were treated successfully with pulse doses of prednisolone. Hyperimmunoglobulin therapy was used in conjunction with oral nucleotide/nucleoside analogues for 12 months (range, 3-24 mo) and then stopped. After transplant, 4 patients had antiviral medicine changed to adefovir or entecavir because of drug resistance, and otherwise all patients remained negative for hepatitis B virus DNA during follow-up. Conclusions: Patients transplanted for hepatitis B and D virus cirrhosis, even with hepatocellular carcinoma, had favorable prognosis and good long-term results. Close follow-up of patients and effective viral suppression with suitable drugs were key factors for efficient patient care.
Impaired Coronary Microvascular and Left Ventricular Diastolic Function in Patients with Inflammatory Bowel Disease
(2015) Caliskan, Zuhal; Gokturk, Huseyin Savas; Caliskan, Mustafa; Gullu, Hakan; Ciftci, Ozgur; Ozgur, Gulsum Teke; Guven, Aytekin; Selcuk, Haldun; 0000-0003-2579-9755; 0000-0002-8445-6413; 0000-0002-6463-6070; 25128749; AAJ-8546-2021; JYO-9455-2024; IXD-5147-2023; AAJ-6976-2021; A-7318-2017
Background and aim: Increased incidence of coronary vascular events in patients with inflammatory bowel disease (IBD) is known. However, the association between coronary microvascular function and IBD has not been fully defined. We aimed to investigate whether coronary flow reserve (CFR) and left ventricular diastolic function were impaired in IBD patients. Methods: Seventy-two patients with IBD (36 patients with ulcerative colitis [UC] and 36 Crohn's disease [CD]) were registered. Each subject was evaluated after a minimum 15-day attack-free period. For the control group, 36 age- and sex-matched healthy volunteers were included into the study. IBD clinical disease activity in UC was assessed by the Truelove-Witts Index (TWAS) and in CD by the Crohn's Disease Activity Index (CDAI). In each subject, CFR was measured through transthoracic Doppler echocardiography. Results: Compared to the controls, the CD group and UC group had significantly higher high-sensitivity C-reactive protein (hs-CRP) and erythrocyte sedimentation rate. Baseline diastolic peak flow velocity (DPFV) of the left anterior descending artery (LAD) was significantly higher in the IBD group (24.1 +/- 3.9 vs. 22.4 +/- 2.9, p < 0.05), and hyperemic DPFV (56.1 +/- 12.5 vs. 70.6 +/- 15.3, p < 0.05) and CFR (2.34 +/- 0.44 vs. 3.14 +/- 0.54, p < 0.05) were significantly lower in the IBD group than in the control group. In stepwise linear regression analysis, hs-CRP and lateral Em/Am ratio were independently correlated with CFR. Conclusion: CFR, reflecting coronary microvascular function, is impaired in patients with IBD. CFR and left ventricular diastolic function parameters are well correlated with hs-CRP. (C) 2014 Published by Elsevier Inc.
Importance of Malnutrition in Patients with Cirrhosis
(2015) Gokturk, Huseyin Savas; Selcuk, Haldun; 0000-0002-8445-6413; 26038997; AAJ-6976-2021
Malnutrition is common in patients with chronic liver disease and is associated with poor outcomes. Inadequate intake, poor quality diet, maldigestion, malabsorption, altered macronutrient metabolism, and hypermetabolism all contribute to the development of malnutrition in this patient population. Although it is generally easy to detect, clinicians often overlook malnutrition and its measurement is complicated by the lack of a simple, standardized diagnostic method. Early detection of malnutrition and multidisciplinary treatment approaches greatly increase the probability for successful outcomes.
Lansoprazole-induced Acute Pancreatitis
(2014) Ocal, Serkan; Korkmaz, Murat; Yildirim, Abdullah Emre; Altun, Reskan; Akbas, Enver; Selcuk, Haldun; https://orcid.org/0000-0003-3719-9482; https://orcid.org/0000-0002-9333-782X; https://orcid.org/0000-0002-4386-9297; https://orcid.org/0000-0002-8445-6413; 25417626; ABH-4817-2020; AAM-1330-2020; AAG-6561-2020; F-3628-2015; AAJ-6976-2021
Late-Onset Drug-Induced Cholestasis in a Living-Related Liver Transplant Donor With Progressive Familial Intrahepatic Cholestasis
(2015) Harmanci, Ozgur; Ensaroglu, Fatih; Ozcay, Figen; Ocal, Serkan; Korkmaz, Murat; Ozdemir, B. Handan; Selcuk, Haldun; Moray, Gokhan; Haberal, Mehmet; 0000-0002-7528-3557; 0000-0003-3719-9482; 0000-0002-9333-782X; 0000-0002-3462-7632; 0000-0002-8445-6413; 0000-0003-2498-7287; 0000-0002-5214-516X; 0000-0002-0643-4980; 26640927; X-8540-2019; ABH-4817-2020; AAM-1330-2020; AAJ-8097-2021; AAJ-6976-2021; AAE-1041-2021; ABG-5684-2020
We present a rare case of progressive familial intrahepatic cholestasis within a family. A 34-year-old female became a living-related liver transplant donor for her son, who had the disease. Nine years after the transplant, the mother developed severe intrahepatic cholestasis, for which she was evaluated after using an oral contraceptive drug. She presented with jaundice, pruritus, and increased bilirubin levels, together with elevated gamma glutamyl transferase and alkaline phosphatase levels. A liver biopsy revealed findings consistent with intrahepatic cholestasis. However, despite follow-up management and cessation of the insulting drug, her total bilirubin count continuously increased to 20 mg/dL and was accompanied by intractable pruritus. A total of 9 plasmapheresis sessions were performed, and she was started on a regimen of ursodeoxycholic acid (13 mg/kg/d) and cholestyramine (4 g, 3 times daily). The clinical and laboratory picture dramatically improved following cessation of the oral contraceptive, plasmapheresis sessions, and drug treatment. The patient's cholestasis normalized within 3 months, and she recovered uneventfully. A genetic analysis of the whole family revealed that both parents were heterozygous for the mutation c.124G>A in ABCB11, and the son was homozygous for this mutation. These findings supported varying degrees of bile salt export pump deficiency in the family members. Defective bile salt excretory system function can result in a wide spectrum of clinical presentations, ranging from progressive familial intrahepatic cholestasis requiring liver transplant to late-onset drug-induced cholestasis. Our findings suggest that, in a heterozygous carrier of a progressive familial intrahepatic cholestasis mutation, drug-induced cholestasis is responsive to treatment, after which the clinical picture can normalize within 3 months.