Incidentally detected cholangiocarcinoma in an explanted liver with biliary atresia after Kasai operation.

This report presents the case of a 30-yr-old woman with BA who developed incidental cholangiocarcinoma following the Kasai operation. She showed progressive liver dysfunction and cirrhosis at the age of 30 yr and underwent LDLT. A 4-cm-diameter liver tumor in the anastomotic site of portoenterostomy was incidentally found as a result of a pathological examination of the explanted native liver.

Long survival of congenital alveolar capillary dysplasia patient with NO inhalation and epoprostenol: effect of sildenafil, beraprost and bosentan.

The case is described herein of a patient with alveolar capillary dysplasia with double-outlet right ventricle and duodenal atresia who survived for a remarkably long time. The newborn girl was born at a gestational age of 36 weeks and weighed 1926 g. One min after delivery the Apgar score was 4.

Living donor liver transplantation with renoportal anastomosis for a patient with congenital absence of the portal vein.

A congenital absence of the portal vein (CAPV) is a rare disorder that may lead to an intrapulmonary shunt. A 14-year-old male with CAPV underwent living donor liver transplantation with a left lobe graft from his father. The portal vein reconstruction was achieved with a renoportal anastomosis using an interpositional graft from the native collateral vein, because portal venous system directly drains to the left renal vein without constructing the confluence of superior mesenteric vein and splenic vein.

Living donor liver transplantation using grafts with hepatic cysts.

Cystic lesions in the liver are often found through the evaluation of liver donors. Multiple cysts are worrisome, and donor candidates with multiple cysts may be unacceptable as liver donors, especially when their recipients have fibrocystic disease (FCD), which is an inherited disorder. This study reviewed 183 cases of living donor liver transplantation.

ZNF385B is characteristically expressed in germinal center B cells and involved in B-cell apoptosis.

We previously identified zinc finger (ZF) protein ZNF385B as a molecule specifically expressed in Burkitt's lymphoma (BL) among hematologic malignancies. Here, we investigated ZNF385B expression in healthy B cells in a variety of hematological tissues by RT-PCR and immunohistochemistry. ZNF385B expression was found to be limited to a subset of GC B cells, the healthy counterpart to BL B cells.

Association between activities of daily living and mortality among institutionalized elderly adults in Japan.

Background: We assessed the association between activities of daily living (ADL) and mortality among nursing home residents in Japan.

Methods: This 1-year prospective cohort study investigated 8902 elderly adults in 140 nursing homes. Baseline measurements included age, sex, height, weight, body mass index (BMI), ADL, and dementia level.

Amidic and acetonic cryoprotectants improve cryopreservation of volvocine green algae.

A number of volvocalean green algae species were subjected to a two-step cryopreservation protocol with various cryoprotectants. Potential cryoprotectants were methanol (DMSO), N,N-dimethylformamide (DMF), N,N-dimethylacetamide, N-methylformamide, and hydroxyacetone (HA). We confirmed prior reports that MeOH was effective for cryopreserving Chlamydomonas, but did not work well for larger volvocaleans such as Volvox.

Devastating outflow obstruction after pediatric split liver transplantation.

HVOO is a rare complication after pediatric LT, which may lead to graft failure. There are various causes of HVOO, such as mechanical anastomotic obstruction and SOS. A 10-month-old female underwent split LT from a deceased donor for ALF.

ALK-positive anaplastic large cell lymphoma limited to the skin: clinical, histopathological and molecular analysis of 6 pediatric cases. A report from the ALCL99 study.

Anaplastic large cell lymphomas are peripheral T-cell lymphomas that are characterized by a proliferation of large anaplastic blasts expressing CD30. In children, systemic anaplastic large cell lymphomas often present at advanced clinical stage and harbor translocations involving the anaplastic lymphoma kinase (ALK) gene leading to the expression of chimeric anaplastic lymphoma kinase (ALK)-fusion proteins. Primary cutaneous anaplastic large cell lymphoma is regarded as an ALK-negative variant confined to the skin and is part of the spectrum of primary cutaneous CD30-positive T-cell lymphoproliferative disorders.

Isolation of Hepatic Progenitor Cells From Human Liver With Cirrhosis Secondary to Biliary Atresia Using EpCAM or Thy-1 Markers.

We sought to determine whether hepatic progenitor cells can be isolated from cirrhotic liver using epithelial cell adhesion molecule (EpCAM) or Thy-1 markers. Liver tissue with cirrhosis secondary to biliary atresia (BA) was collagenase digested, and nonparenchymal cells (NPCs) were cultivated for 24 h. Noncirrhotic NPCs derived from patients with carbamyl phosphate synthetase and ornithine transcarbamylase deficiencies were used as controls.

[Retrospective analysis of 157 patients with pediatric Hodgkin lymphoma in Japan: investigation by four pediatric cancer study groups].

Hodgkin lymphoma is an easily curable malignancy in the pediatric age group and is less frequently observed in Japan. No study with a large sample size of Japanese patients has been conducted. From 1985 to 2000, 157 Japanese patients with Hodgkin lymphoma were retrospectively analyzed based on their clinical characteristics, treatment regimen, and treatment outcome by 4 pediatiric cancer study groups.

Anti-high mobility group box 1 and box 2 non-histone chromosomal proteins (HMGB1/HMGB2) antibodies and anti-Saccharomyces cerevisiae antibodies (ASCA): accuracy in differentially diagnosing UC and CD and correlation with inflammatory bowel disease phenotype.

Background: The development of a supportive diagnostic method has long been required to differentially diagnose ulcerative colitis (UC) and Crohn's disease (CD). Several antibodies circulate in the sera of patients with inflammatory bowel disease. We previously identified the high mobility group box 1 and box 2 non-histone chromosomal proteins (HMGB1 and HMGB2) as novel antigens of perinuclear type anti-neutrophil cytoplasmic antibodies (pANCA) and discovered anti-HMGB1/HMGB2 antibodies in sera from patients with UC.

The role of liver transplantation for congenital extrahepatic portosystemic shunt.

Background: Congenital extrahepatic portosystemic shunt (CEPS) is reported more frequently because of advances in imaging techniques. Liver transplantation (LT) is a therapeutic option, although the indications for LT are still controversial.

Methods: This study reviewed 34 cases of LT for CEPS, including 30 cases reported in the English medical literature and the patients treated in our department, to collect the clinical data associated with LT.

Consideration of a Safe Protocol for Hepatocyte Transplantation Using Infantile Pigs.

Hepatocyte transplantation is hoped to be an alternative treatment to certain cases of liver transplantation. The most promising indication is for congenital metabolic diseases, especially in infants. To establish a safe protocol for hepatocyte transplantation into infants, we examined physiological changes during treatment using infantile pigs.

Glycemic management in living donor liver transplantation for patients with glycogen storage disease type 1b.

GSD type 1b is an autosomal recessive inborn error of carbohydrate metabolism caused by defects of the G6Pase translocase (G6PT). Patients with GSD1b have severe hypoglycemia with several clinical manifestations of hepatomegaly, obesity, a doll-like face, and neutropenia. LT has been indicated for severe glucose intolerance.

Portal vein reconstruction in pediatric living donor liver transplantation for patients younger than 1 year with biliary atresia.

Background/purpose: Infants with biliary atresia undergoing living donor liver transplantation (LDLT) are at increased risk of portal vein (PV) complications because of their smaller vascular caliber and sclerosis because of previous Kasai portoenterostomy and recurrent cholangitis.

Method: Of 154 children who underwent transplantation between November 2005 and January 2011, 34 with biliary atresia received a transplant while younger than 1 year. Six patients underwent PV reconstruction with an interposition vein graft, and the others underwent the branch patch technique.

Squamous metaplasia in the cyst epithelium of type 1 congenital pulmonary airway malformation after thoracoamniotic shunt placement.

Thoracoamniotic shunting is the treatment of choice for management of the fetus with type 1 congenital pulmonary airway malformation. Thoracoamniotic shunting has been performed to reduce life-threatening risks such as fetal hydrops. However, caution is needed because of possible complications.

Analysis of chromosome 19q13.42 amplification in embryonal brain tumors with ependymoblastic multilayered rosettes.

Recently, it was reported that ependymoblastoma and embryonal tumor with abundant neuropil and true rosettes (ETANTR) show 19q13.42 amplification at a high frequency, suggesting that these tumors may constitute a single entity. As ependymoblastic rosettes are the most prominent features in both subtypes, embryonal tumor with multilayered rosettes (ETMR) was proposed, for which 19q13.

[A case of hepatic artery aneurysm that had formed asymptomatically and penetrated into the duodenum].

A 41-year-old man was admitted to our hospital with gastrointestinal bleeding. Esophagogastroduodenoscopy revealed a submucosal protrusion with erosion in the duodenal bulb which was thought to be the bleeding source. Dynamic CT scan, ultrasonography and angiography of the abdomen revealed a hepatic artery aneurysm and a dilated celiac artery that dissected from its origin.

Vitamin D receptor activators inhibit vascular smooth muscle cell mineralization induced by phosphate and TNF-α.

Background: Vascular calcification is a highly regulated process. Tumor necrosis factor-α (TNF-α) has been shown to accelerate the highly regulated osteogenic process in vascular smooth muscle cells (VSMCs). Vitamin D receptor activators (VDRAs) have been associated with beneficial cardiovascular outcomes in patients with chronic kidney disease.

Critical infantile hepatic hemangioma: results of a nationwide survey by the Japanese Infantile Hepatic Hemangioma Study Group.

Background: The current survey aimed to describe the clinical features of critical infantile hepatic hemangioma (IHH) and the implications of recent treatments.

Materials And Methods: A nationwide survey of critical IHH patients treated between 2005 and 2010 was performed in all 117 registered pediatric surgical hospitals in Japan. As a result, 19 patients were identified and reviewed using a statistical analysis.

Pediatric liver-kidney transplantation for hepatorenal fibrocystic disease from a living donor.

The indications for and the timing of LT and/or KT for the patients with HRFCD are based on the severity of liver and kidney involvement. Most organs come from living donors, because the number of deceased donors is extremely low in Japan. Therefore, patients with HRFCD may need two organs from living donors.