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Smith-Lemli-Opitz syndrome is an autosomal recessive metabolic disease characterized by mental retardation and multiple congenital anomalies. The main pathology is the lack of the enzyme 3β-hydroxysterol Δ7-reductase, which is the last enzymatic step in cholesterol synthesis, ending with a low cholesterol level. Cholesterol is vitally important in cell membranes and myelination of the nervous system. The cholesterol level affects many systems of the body, especially the nervous system. The cause of liver involvement in Smith-Lemli-Opitz syndrome is unclear, and many hypotheses have been suggested. Here, we present the early results of a patient with Smith-Lemli-Opitz syndrome who underwent living-donor liver transplant due to cirrhosis. As a result of liver transplant, normal cholesterol levels were shown, as well as improvements in the patient's neurodevelopment and behavior. Early liver transplant may be considered for patients with a defect of cholesterol biosynthesis, even in the absence of cirrhosis, and may be a future treatment option to prevent risks of neurologic deterioration.

Background: While early relaparotomy following living donor liver transplantation (LDLT) is an important event and its outcomes are still unclear, the aim of this retrospective study is to investigate the causes and outcomes of early relaparotomy in recipients of adult living donor liver transplantations. Materials and methods: Between April 2015 and April 2020 at Medipol University Medical Faculty Hospital Organ Transplantation Department, Istanbul, Turkey, 15 patients (9.3%) who underwent early relaparotomy were studied retrospectively. Results: Eight patients (53.3%) had a history of upper abdominal surgery in the early laparotomy group. The most common cause of early relaparotomy had postoperative bleeding in 9 patients (60%). The overall survival rate was 66.7%. Mortality was higher in the group over 60 years old and with a history of upper abdominal surgery. Conclusions: The history of upper abdominal surgery appears to be a risk factor for early relaparotomy. Also, previous upper abdominal surgery group and elderly patients group present with more mortality. We should be very careful in these groups.

Introduction: Liver transplantation (LT) is the most effective treatment modality in patients with end-stage liver disease (ESLD). With the increased prevalence of diabetes and non-alcoholic fatty liver disease (NAFLD), coupled with a steady increase in the age of transplant population, liver transplant candidates are at increased risk for coronary artery disease (CAD). Improvements in anesthesia, surgical experiences, and increased effectiveness of drugs have facilitated concurrent surgical interventions in transplantation, allowing for orthotopic LT to be performed in elderly patients with a high incidence of coexisting diseases, especially those of the cardiovascular system. In this study, we present two cases of combined off-pump coronary artery bypass surgery and living donor liver transplantation. Case presentation: A 68-year-old male patient with ESLD due to chronic hepatitis C infection and multi-vessel CAD, and a 65-year-old female patient with NAFLD cirrhosis and multi-vessel CAD were referred to our institution for an LT evaluation. We performed combined living donor LT and off-pump coronary artery bypass surgery in both patients. Both the first and second patients were alive with good liver and cardiac function at the 25 and 15 months after surgery, respectively. Conclusion: Combined off-pump coronary artery bypass surgery and living donor LT are an alternative treatment for patients with severe CAD and ESLD. We consider that combined coronary artery bypass graft surgery and LT can be safely performed by experienced anesthesia and surgical teams in selected patients.

Akut karaciğer yetmezliği, ilerleyici karaciğer disfonksiyonudur. Karaciğer nakli, etkin bir tedavi seçeneğidir. Bu çalışmanın amacı akut karaciğer yetmezliği nedeniyle yapılan canlı vericili karaciğer nakillerini değerlendirmektir. Nisan 2014 ile Nisan 2019 tarihleri arasında, Medipol Üniversitesi Tıp Fakültesi Hastanesi Organ Nakli Bölümü’nde akut karaciğer yetmezliği nedeniyle canlı vericili karaciğer nakli yapılan 13 hasta retrospektif olarak değerlendirildi. Hastaların yaş ortalaması 21,1±20,9 yıldı. Hastaların dokuzu (%69,2) kadın ve yedisi (%53,8) çocukluk (< 18 yıl) yaş grubunda idi. En sık toksik hepatit nedeniyle (%38,5) karaciğer nakli yapıldı. Hastaların ortalama takip süresi 35,1±12,2 aydı. Hastaların beşinde (%38,5) komplikasyon gelişti. Hastalardan dördünde (%30,7) mortalite görüldü. Genel sağkalım oranı 1 ve 5 yıllık sırasıyla % 84,7 ve % 69,3 idi. Akut yetmezlikte yapılan canlı vericili karaciğer nakillerinin başarılı olduğu görünmektedir.

Background: BK virus is the most common infectious causes of nephropathy and graft loss after kidney transplantation. In our study, we investigated the factors that may affect positive BK virus in the blood in patients with kidney transplantation.

Amaç: Bu çalışma ile merkezimiz de yapılan böbrek nakli olgularımızı retrospektif olarak değerlendirmeyi amaçladık.Gereç ve Yöntem: Hastaların demografik özellikleri, kadavra ve canlı donör kullanım sayıları, akrabalık durumları, hastanede yatış süreleri, postoperatif morbidite, greft kaybı ve mortalite oranları belirlendi.Bulgular: Hastaların yaş ortalaması 37.6 ± 16.2 (3 - 67) idi, % 61.7’ si erkek ve % 38.3’ü kadındı. Hastalarımızın % 91.7’ sine canlı vericiden böbrek nakli yapıldı. Ortalama yatıs süresi 11 gündü. Hastaların % 8.3’ünde rejeksiyon görüldü ve greft kaybı oranı % 3.3 idi. Mortalite oranı % 4.2 idi. Sonuç: Son dönem böbrek yetmezliği nedeniyle takip edilen hastalar için en etkin tedavi yöntemi böbrek naklidir. Hasta sağlığı ve konforu açısından esas amaç böbrek nakli sayılarının arttırılması olmalıdır.

The last two decades have seen a paradigm shift in the selection of patients with hepatocellular carcinoma (HCC) for liver transplantation. Microvascular invasion and differentiation have been the most significant factors affecting post-transplant recurrence; however, because of inherent disadvantages of pre-transplant biopsy, histological criteria never gained popularity. Recently, the selection criteria evolved from morphological to biological criteria, such as biomarkers and response to loco-regional therapy. With the introduction of multimodality imaging, combination of computed tomography with nuclear medicine imaging, particularly, 18F-fluorodeoxyglucose positron emission tomography fulfilled an unmet need and rapidly became a critical component of HCC management. This review article will focus on the use of 18F-fluorodeoxyglucose positron emission tomography combined with computed tomography in the pre-transplant evaluation of HCC patients with special discussion on its ability to predict HCC recurrence after liver transplantation.

Aim: The only definitive treatment of chronic liver disease (cholestatic, metabolic, autoimmune), acute liver failure and liver tumors are liver transplantation in pediatric patients. The aim of this study was to present the experience of our center on pediatric living donor liver transplantation and review of the literature.Methods: This is retrospective cohort study. Pediatric patients who receive living donor liver transplantation between December 2014 and December 2017 included in the study. Demographic features, complications after transplantation, and mortality rates were recorded.Results: A total 29 patients were included in the study. Mean age of cases were 3.1 (1-13) years, 18 (62.1%) of the patients were male. Mean Pediatric End-Stage Liver Disease (PELD) scores were 15.6 (-6-37). Mean follow-up period was 60 months. Complication was detected in 11 patients (37.9%) and 5 patients died (mortality rate: 17.9%). In our study, the causes of death were disseminated intravascular coagulation in three patients and sepsis due to biliary leakage in two patients. Conclusion: Complications and mortality rates related to pediatric patients with donor liver transplantation in our center are consistent with the literature., Amaç: Kronik karaciğer hastalığının (kolestatik, metabolik, otoimmün), akut karaciğer yetmezliği ve karaciğer tümörlerinin pediatrik hastalarda ekin tek tedavisi karaciğer naklidir Bu çalışmanın amacı merkezimizde yapılan pediatrik canlı vericili karaciğer nakillerini değerlendirmek ve literatürü tartışmaktır.Yöntemler: Bu çalışma retrospektif kohort çalışmadır. Çalışmaya Aralık 2014 ile Aralık 2017 tarihleri arasında canlı vericili karaciğer nakli yapılan pediatrik hastalar alındı. Demografik özellikler, nakil sonrası komplikasyonlar ve mortalite oranları kaydedildi.Bulgular: Çalışmaya toplam 29 hasta dahil edildi. Hastaların yaş ortalaması 3,1 (1-13) yıl, 18'i (%62,1) erkekti. Hastaların ortalama Pediatrik Son Dönem Karaciğer Hastalığı (PELD) skorları 15,6 (-6-37) idi. Ortalama takip süresi 60 aydı. Komplikasyon oranı %37,9 (11 hasta) ve mortalite oranı %17,9 (5 hasta) olarak saptandı. Çalışmamızda hastalarımızın ölüm nedeni dissemine intravasküler koagülasyon ve safra kaçağına bağlı sepsis idi.Sonuç: Merkezimizde pediatrik hastalara yapılan canlı vericili karaciğer nakillerinin komplikasyon ve mortalite oranları literatür ile uyumludur.

Alveolar echinococcosis is an infectious disease caused by Echinococcus multilocularis and it is frequently diagnosed as a space-occupying lesion in the liver. The growth pattern may be similar to that of a malignant tumour with extensive liver infiltration, spreading into neighbouring organs and forming metastasis-like masses in distant organs. Thus, it is one of the differential diagnoses of liver cancer. We report a case that presented as a klatskin tumour clinically and radiologically, but was revealed by pathologic and serologic work-up. Since the courses of these two diseases, a malignancy and an infectious disease, are far beyond comparison, echinococcosis should always be considered in differential diagnosis of obstructive jaundice, especially in the endemic regions. Alveolar Ekinokokkosis, Echinococcus multilocularis’in sebep olduğu bir enfeksiyon hastalığı olup, sıklıkla karaciğerde yer kaplayan lezyon ile kendini gösterir. Karaciğerdeki lokal infiltratif paterni yanısıra, çevre organlara yayılımı ve uzak organlarda metastazile karışan kitle oluşturması nedeniyle, malign bir tümörü taklit eder. Bu nedenle karaciğer kanserlerinin önemli bir ayırıcı tanısını oluşturur. Burada, klinik ve radyolojik olarak klatskin tümörünü düşündüren ancak patolojik ve serolojik çalışmalarla Alveoler Ekinokokkosis tanısı alan bir olgu sunulmuştur. Malign tümörlerin ve enfeksiyon hastalıklarının tedavi ve prognozlarının birbirinden çok farklı olması nedeniyle, özellikle Ekinokkozisin endemik olduğu bölgelerde, tıkanma sarılığının ayırıcı tanısında bu etken mutlaka akla gelmelidir.

Aim: Hepatocellular carcinoma (HCC) is the most common primary solid tumor of the liver. Hepatitis B virus (HBV) infections, Hepatitis C virus (HCV) infections and at alcoholism can be seen. The aim of this study was to evaluate living donor liver transplantation in hepatocellular carcinoma.Methods: This is a retrospective cohort study. Between April 2014 and December 2017 at Medipol University Medical Faculty Hospital Organ Transplantation Department, 38 patients in living donor liver transplantation for HCC were evaluated.Results: The mean patient age was 58.0 (9.9) (20–74) years; 31 (81.6%) of the 38 were male. The mean MELD score was 14.2 (8–32). Outside Milan criteria (P=0.003), poorly differentiated and cholangiocarcinoma tumor component (P=0.003) appears to be worse in living donor liver transplantation in hepatocellular carcinoma.Conclusions: In this study, outside Milan criteria, poorly differentiated and cholangiocarcinoma tumor component appears to be worse in living donor liver transplantation in hepatocellular carcinoma., Amaç: Hepatosellür karsinom ( HCC ) karaciğerin en sık görülen primer solid tümörüdür. Hepatit B virus (HBV) enfeksiyonları, hepatit C virus ( HCV ) enfeksiyonları veya alkolizm zemininde de görülebilir. Bu çalışmanın amacı, HCC nedeniyle canlı vericili karaciğer nakli yapılan hastaların değerlendirilmesidir.Yöntemler: Retrospektif kohort çalışma planlandı. Nisan 2014 – Aralık 2017 tarihleri arasında Medipol Üniversitesi Tıp Fakültesi, Organ Nakli Merkezi'nde HCC nedeniyle canlı vericili karaciğer nakli yapılan 38 hasta değerlendirildi. Bulgular: Ortalama yaş 58,0 (9,9) (20-74) yıldı; 38 kişiden 31'i (%81,6) erkekti. Ortalama MELD skoru 14,2 (8–32) idi. Milan kriterleri dışında (P=0,003), kötü farklılaşmış ve kolanjiokarsinom tümör bileşeni (P=0,003), olan hepatosellüler karsinomda canlı vericili karaciğer naklinde daha kötü görünmektedir.Sonuçlar: Bu çalışmada Milan dışı, az differansiye ve kolanjiokarsinom komponenti olan tümörlerin daha kötü olduğu görünmektedir.

Objectives In this paper, the results of liver transplant due to hepatoblastoma in 10 pediatric patients at Istanbul Sisli Memorial Hospital Transplantation Center are presented. Materials and methods We retrospectively evaluated medical records of pediatric patients diagnosed with hepatoblastoma and who underwent liver transplant at our clinic between January 2009 and March 2014. We examined age, weight, chemotherapy regimen, graft type for liver transplant, duration of hospital stay, complications, follow-up duration, and survival information. Results The median age of the 10 patients included in our study was 13.5 months (range, 8-120 mo), and the median weight was 10 kg (range, 6.5-30 kg). Two of the patients were twins. Five patients had pretreatment extent of disease III (centrally placed cases), and five had pretreatment extent of disease IV hepatoblastoma. Preoperative chemotherapy was given to 7 patients as cisplatin plus doxorubicin and to 3 patients per the International Childhood Liver Tumors Strategy Group 3 High-Risk Protocol at external centers. These protocols were administered according to treatment center preference. Nine patients received transplants from living donors. Two grafts were right lobes, and 7 were left lateral segments. In the remaining patient, a whole liver was received from a deceased donor. The histopathologic subgroups were epithelial in 5 patients, with others being of mixed type. Postoperative complications occurred in 3 patients as infection, intra-abdominal fluid collection, and acute rejection. The median follow-up was 32 months. One patient died because of lung metastasis within 9 months after transplant. Conclusions Centers should offer liver transplant to patients with centrally located tumors. For centers that have an insufficient number of deceased donors, living-donor liver transplant with optimal planning and early treatment can be performed.

Purpose To report the measurements of central corneal thickness (CCT) in uveitic eyes with Behcet disease (BD) and compare it with age- and sex-matched healthy controls. Materials and methods This study included 69 eyes with ocular BD with no history of corneal disease, glaucoma, or ocular surgery and 50 eyes of healthy controls. Eyes with ocular BD were subdivided into active and inactive groups. Active group was defined as the presence of anterior uveitis or panuveitis, whereas inactive group was defined as having had at least 1 previous attack and absence of any active inflammation in the eye within the last 3 months. CCT was measured with ultrasonic pachymeter. Statistical analyses were performed, and P Results Active group had 24 patients and inactive group had 45 patients. Demographic characteristics of patients with ocular BD and control subjects were similar (P > 0.05). There was no significant difference in respect to the disease duration between active and inactive groups (P = 0.160). The mean CCT was significantly greater in active group (584.75 ± 20.94 μm) than in inactive group (540.55 ± 36.16 μm) and control group (543.04 ± 25.35 μm) (P = 0.0001). Conclusions We found that eyes with active BD had increased CCT because of active inflammation when compared with inactive and control groups, and mean CCT of inactive BD was normal. Therefore, we assume that CCT is in normal range in the inactive phase, and recurrent uveitis does not lead to a permanent change in CCT in BD.