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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.

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.

Immunosuppressive drugs predispose the kidney transplant recipient to reactivation of сytomegalovirus (CMV) infections. Prophylaxis given to these patients is very important for the prevention of opportunistic CMV infections. The objective of this study was to evaluate the short term and standard-dose valganciclovir prophylaxis for CMV infections in living donor kidney transplantation. Methods. This study is retrospective one. Between April 2014 and April 2019 100 patients after living donor kidney transplantation with results CMV PCR-DNA and prophylactic treatment were studied retrospectively at Medipol University Medical Faculty Hospital Organ Transplantation Department, Istanbul, Turkey. Results.The mean age was 38.3±15.6 years. 68 (68%) patients were males and 32 (32%) patients were females. All patients were treated with 900 mg daily and 90 days valganciclovir prophylaxis. Mean follow-up was 29.1±15 months. There were not detected CMV infections during the follow-up period. Conclusions: Short term and standard-dosevalganciclovir prophylaxis appears to be successful prevention CMV infections in living donor 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.

Objective: to determine the frequency of granulomatous inflammation within hepatocellular carcinoma (HCC) and its clinicopathologic associations. Material and method: fifty-eight HCCs (51 explants, 3 lobectomies, and 4 segmentectomies) were reviewed. Results: five (8.6%) cases (F/M=1/4, mean age: 63.6) were identified with granulomas.1/5 had history of neoadjuvant therapy. 4/5 patients presented with early stage (pT1/2). All were well-differentiated (Grade1-2/4). The mean number of tumor foci was 3.6, with a median size of 2.2 cm. All of them had advanced fibrosis. No difference was identified from cases without granulomas (n=53) in terms of prognosis and aforementioned parameters (p> 0.05). Granulomas were mainly concentrated in peripheral parts of the tumors. One case with nodule-in-nodule formation had granulomas lined along the border of the inner nodule. In 2 cases, granulomas were identified in steatohepatitic areas, while another had clear cell change. Only 1 had necrotizing granulomas, none with acid resistant bacilli. Two cases revealed concomitant granulomas in the adjacent liver parenchyma in addition to the tumor stroma. Except for one with a history of tuberculosis, none of the cases had a granulomatous disease.Conclusion: this is the largest case series of HCCs with granulomas by far. Our data revealed neither clinicopathologic and prognostic difference nor definite etiology related to granulomas. Yet, association with steatotic and clear tumor cells suggests the role of cytoplasmic content, while distribution of granulomas points to host immune response., NA

Background The aim of this study was to investigate the risk factors effective in perioperative morbidity and mortality in 161 living donor liver transplantations (LDLT). Results The most common indication for living donor transplantation was cryptogenic cirrhosis. The most common complication was biliary problems in 62.16% cases. Sepsis was the most common cause of in 52%. Patients in whom sepsis was observed, significantly prolonged stay under mechanical ventilation and prolonged ICU stay were detected. In patient group in whom mortality was observed, higher amounts of erythrocytes, fresh frozen plasma (FFP), and platelets were transfused, and patients remained longer under mechanical ventilation treatment, and in the ICU. Conclusion Perioperative morbidity and mortality was found to be significantly related with higher amounts of erythrocytes and FFP transfusions and longer operative and warm ischemia times. Sepsis was found to be the most common cause of mortality.

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.

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.