Progressive Familial Intrahepatic Cholestasis Associated With Ubiquitin-Specific Peptidase 53 Gene Variant Presented with Acute-on-Chronic Liver Failure in Turkish Siblings.

Progressive familial intrahepatic cholestasis represents a group of disorders characterized by defective bile excretion, which causes a multitude of clinical symptoms of variable severity and usually begins in childhood. During the past few decades, a number of gene sequence variants have been shown to be associated with progressive familial intrahepatic cholestasis, and new subtypes continue to be discovered. Sequence variants of the ubiquitinspecific peptidase 53 gene have previously been associated with a novel autosomal recessive form of cholestasis with coincident normal or low γ-glutamyl transferase, with mild phenotypes.

Portal Hypertension in Children: A Tertiary Center Experience in Turkey.

Purpose: Portal hypertension (PH) and its complications have a significant impact on morbidity and mortality. This study aimed to evaluate the etiology; clinical, laboratory, and endoscopic findings; treatment approaches; long-term outcomes; and prognosis of pediatric PH.

Methods: This retrospective study included 222 pediatric patients diagnosed with PH between 1998 and 2016, and data encompassing clinical, laboratory, and radiological features; treatments; and complications were analyzed.

Surgical Complications After Deceased Donor Renal Transplant.

Objectives: Deceased donor renal transplant is an accepted treatment for patients with end-stage renal disease. We retrospectively analyzed urological and surgical complications and outcomes in our series.

Materials And Methods: Since 2016, we have performed 263 renal transplants at the Gazi University Transplantation Center, Ankara, and 92 of these were from deceased donors.

Predictable and Unusual Adverse Effects of Immunosuppression in Pediatric Liver Transplant Patients.

Objectives: Our aim was to determine potentially adverse effects of immunosuppressive protocols after liver transplantation in children.

Materials And Methods: The medical records of 60 children who underwent liver transplant retrospectively analyzed. Corticosteroid, tacrolimus, and mycophenolate mofetil were the primary immunosuppressive agents used in our center.

Outcome of the Double-J Stent Placement in Pediatric Kidney Transplant: A Single Center Experience.

Objectives: Double J stent placement at kidney transplant may reduce stenosis or leakage complication rates. However, placement may also increase risk for early urinary tract infection (ie, < 3 mo after transplant). In children, the usefulness of double J stent placement is not well defined.

Urologic Complications After Renal Transplant: A Single-Center Experience.

Objectives: Urologic complications after kidney transplant are associated with significant morbidity, mortality, and prolonged hospital stay. An intervention or second surgical procedure is frequently required. Here, we report urologic complications in adult kidney recipients.

Chylous ascites after a living-donor liver graft, effectively treated in a child with octreotide.

Chylous ascites after a liver transplant is a rare complication of surgery. We report a 11-month-old girl with biliary atresia who was presented with chylous ascites after a liver transplant. On the seventh day after surgery, while being fed, chylous ascites was observed.

Resistance to the induction of mixed chimerism in spontaneously diabetic NOD mice depends on the CD40/CD154 pathway and donor MHC disparity.

Blockade of CD40/CD154 pathway has proven effective in promoting the induction of allogeneic mixed chimerism. Using NOD mouse model of human type 1 diabetes, we investigated whether allogeneic mixed chimerism can be induced in prediabetic NOD mice and in spontaneously diabetic NOD mice under nonmyeloablative and irradiation-free conditioning therapy and anti-CD154 mAb as a short-term posttransplant treatment. We found that spontaneously diabetic NOD mice are more resistant to the induction of allogeneic mixed chimerism than prediabetic NOD mice under our nonmyeloablative and irradiation-free conditioning therapy.

Analysis of postsurgical complications in 75 living liver transplantation donors.

Seventy-five living donor liver hepatectomies were performed at our transplantation center between April 1990 and December 2004. We collected the data from patient charts, files, and the Baskent University Liver Registry. There were 39 male and 36 female donors (mean age, 35.

Plasma homocysteine levels in living kidney donors before and after uninephrectomy.

An increased prevalence of hyperhomocysteinemia has been observed among patients with end-stage renal disease, and numerous studies have demonstrated that kidney function is one of the most important determinants of plasma total homocysteine (tHcy) concentration. In an effort to understand the mechanism of hyperhomocysteinemia in renal disease, we chose, as our model, living kidney donors who had undergone uninephrectomy. We studied 10 living kidney donors and measured fasting plasma tHcy, plasma creatinine, folate, vitamins B(12) and B(6), and high-sensitivity C-reactive protein (hsCRP) 24 hours before nephrectomy and 2 days, 6 weeks, and 6 months after nephrectomy compared to the values 24 hours before nephrectomy.

A substantial level of donor hematopoietic chimerism is required to protect donor-specific islet grafts in diabetic NOD mice.

Background: Mixed chimerism can induce tolerance to alloantigens and restore self-tolerance to autoantigens, thereby permitting islet transplantation. However, the minimal level of donor chimerism that is required to prevent islet allograft rejection and recurrence of autoimmune diabetes has not been established.

Methods: We investigated whether allogeneic Balb/c donor chimerism can be induced in C57BL/6 mice, in prediabetic NOD mice, and in diabetic NOD mice after transplantation of a modest dose of bone marrow by using purine nucleoside analogue, fludarabine phosphate and cyclophosphamide conditioning therapy, followed by short-term anti-CD40 ligand monoclonal antibody and rapamycin posttransplant treatment.

Inducing tolerance to MHC-matched allogeneic islet grafts in diabetic NOD mice by simultaneous islet and bone marrow transplantation under nonirradiative and nonmyeloablative conditioning therapy.

Background: Human type 1 diabetes is associated with defects in the hematopoietic stem cells. Simultaneous donor islet and bone marrow transplantation may be an ideal therapeutic approach for inducing tolerance to islet allogeneic antigens and restoring self-tolerance to islet autoimmune antigens.

Methods: Using a nonobese diabetic (NOD) mouse model of human type 1 diabetes, we investigated whether tolerance to MHC-matched allogeneic islet grafts from male nonobese diabetes-resistant (NOR) donors can be induced in female NOD recipients by simultaneous islet and bone marrow transplantation under fludarabine phosphate-based nonmyeloablative and irradiation-free conditioning therapy.