A Rare Case of Childhood Malformations, Abernethy Syndrome: Literature Review and Presentation of Two Cases.

Abernethy syndrome is a rare congenital vascular anomaly. In this condition, blood from the portal system is diverted to the caval system through a portocaval shunt, entirely or partially bypassing the liver. Prevalence figures in the literature range from 1 per 30000 population up to 1 per 50000 population.

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.

Overview of the Etiology, Pathology, and Prognosis of Giant Cell Hepatitis.

Objectives: Giant cell hepatitis is an important diagnostic consideration in early childhood, especially for patients who present with jaundice. Different diseases may play a role in their etiology. In this study, we presented pediatric patients in our center diagnosed with giant cell hepatitis.

A rare cause and an even rarer treatment of hypertension in a 5-year-old boy: Mid-aortic syndrome.

Renal artery stenosis is one of the common vascular diseases that cause hypertension in children. However, renal artery aneurysms and abdominal aortic aneurysms, which may be components of mid-aortic syndrome, are rarely associated with renal artery stenosis. Despite its rarity, early diagnosis and treatment are critical to prevent fatal complications.

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.

Electrocardiographic measurements in children with pre-dialysis chronic kidney disease and undergoing kidney replacement therapy.

Cardiovascular diseases are the main causes of morbidity in children with chronic kidney disease (CKD). Electrocardiography (ECG) can provide important information about cardiac functions and parameters associated with sudden cardiac death. This study aims to evaluate the potentially dangerous changes in CKD and kidney replacement therapies by ECG and to determine the value of ECG in predicting cardiovascular outcome compared with echocardiography.

Value of Neutrophil-Lymphocyte and Platelet-Lymphocyte Ratios in the Evaluation of Acute Rejection and Chronic Allograft Nephropathy in Children With Kidney Transplantation.

Objectives: Neutrophil-to-lymphocyte ratio and platelet (thrombocyte)-to-lymphocyte ratio have become accepted markers of inflammation in recent years and are used to assess disease activity in some diseases. In this study, we investigated the relationship between these values and acute rejection attacks, as well as their role in determining chronic allograft nephropathy, in follow-up of pediatric kidney transplant recipients.

Materials And Methods: Our study included 58 kidney transplant recipients (age 5-18 years) with at least 5-year follow-up at our center.

Two Cases With Neonatal Cholestasis and Renal Disorders Due to DCDC2 Mutation.

Ciliopathies are a heterogeneous group of diseases that are observed after deterioration of the ciliary structures on the cell surface that facilitate communication with the environment. Both liver and kidney involvement are frequently observed in this disease. Recently, a doublecortin domain containing protein 2 (DCDC2) mutation in a ciliopathy disease group was identified.

Encapsulated Peritoneal Sclerosis in an Adolescent With Kidney Transplant After Long-Term Peritoneal Dialysis.

Encapsulated peritoneal sclerosis is a rare complication of long-term peritoneal dialysis that has a high rate of morbidity and mortality. We present an 18-year-old female patient who was first diagnosed with renal failure at 8 years of age and who had 7 years of peritoneal dialysis and then hemodialysis before kidney transplant from a deceased donor. Before transplant, the patient developed encapsulated peritoneal sclerosis and was treated with tamoxifen and steroids.

Partial External Biliary Diversion for Severe Diarrhea After Liver Transplant in Patients with Progressive Familial Intrahepatic Cholestasis Type 1.

Progressive familial intrahepatic cholestasis is a heterogeneous group of autosomal recessive disorders, and liver transplant is the only curative treatment. A biliary diversion operation for disruption of enterohepatic circulation in patients with progressive familial intrahepatic cholestasis type 1 without cirrhosis is another option. We present a pediatric patient with progressive familial intrahepatic cholestasis type 1 who underwent liver transplant due to end-stage liver disease.

Long-Term Outcomes of Patients With Progressive Familial Intrahepatic Cholestasis After Biliary Diversion.

Objectives: Progressive familial intrahepatic cholestasis is a heterogeneous group of genetic disorders characterized by disrupted bile homeostasis. Patients with this disease typically present with cholestasis and pruritus early in life and often progress to end-stage liver disease. The clinical symptoms that patients with progressive familial intrahepatic cholestasis encounter are usually refractory to medical treatment.

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.

Obstructive jaundice and severe pancreatitis due to the foramen of Winslow hernia with multiple anomalies.

Internal hernia through the foramen of Winslow is a very rare condition, especially in children. Here we report a 16-month-old girl who presented with obstructive jaundice and elevation of pancreatic enzymes and was ultimately diagnosed with internal hernia and malrotation by radiologic investigation and open approach surgery. To the best of our knowledge, obstructive jaundice with pancreatitis and other congenital abnormalities in children with the foramen of Winslow hernia have not been reported previously in the literature.

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.

Portal Hypertensive Biliopathy as a Cause of Severe Cholestasis in Children With Congenital Hepatic Fibrosis.

Portal hypertensive biliopathy may occur in patients with noncirrhotic hepatic fibrosis. Portal hypertensive biliopathy treatment should be focused on management of portal hypertension and relief of biliary obstruction. In patients with noncirrhotic portal fibrosis and symptomatic portal hypertensive biliopathy, portal decompression surgery by proximal splenorenal shunt is one successful treatment option.

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.

Surgical Complications After Pediatric Renal Transplant.

Objectives: Renal transplant is the most appropriate treatment for both adult and pediatric patients with end-stage renal failure. Here, we analyzed surgical complications after pediatric renal transplant at our center.

Materials And Methods: We retrospectively analyzed data from patient files and hospital charts of pediatric patients who had renal transplant at our center (Gazi University, Ankara, Turkey).

Micronuclei and other nuclear anomalies in buccal epithelial cells of children with chronic kidney disease.

The objective of this study was to reveal the likely genomic instability in children with chronic kidney disease (CKD) using micronucleus (MN) assay on buccal epithelial cells (BEC). We investigated the frequencies of micronuclei and other nuclear anomalies, such as nuclear buds, binucleated cells, condensed chromatin, and karyorrhectic and pyknotic cells in BEC. Children with CKD were grouped as follows: children in the pre-dialysis (PreD) stage (N=17), children on regular haemodialysis (HD) (N=14), and children who have undergone transplantation (Tx) (N=17).

Nonsurgically Treated Sigmoid Volvulus in a Young Patient 3 Days After Renal Transplant.

Sigmoid volvulus is a rare clinical condition in young individuals. It should be accurately diagnosed and treated in a rapid manner. Surgical and nonsurgical conservative methods are used for the treatment of sigmoid volvulus.

Role of ATP-dependent K channels in the effects of erythropoietin in renal ischaemia injury.

Background & Objectives: Erythropoietin (EPO) has cytoprotective and anti-apoptotic effects in pathological conditions, including hypoxia and ischaemia-reperfusion injury. One of the targets to protect against injury is ATP-dependent potassium (KATP ) channels. These channels could be involved in EPO induced ischaemic preconditoning like a protective effect.

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.