A5032235551- Organ Transplantation Techniques and Outcomes
- Liver Disease and Transplantation
- Pediatric Hepatobiliary Diseases and Treatments
- Congenital Anomalies and Fetal Surgery
- Gallbladder and Bile Duct Disorders
- Renal Transplantation Outcomes and Treatments
- Transplantation: Methods and Outcomes
- Metabolism and Genetic Disorders
- Clinical Nutrition and Gastroenterology
- Congenital gastrointestinal and neural anomalies
- Liver Disease Diagnosis and Treatment
- Congenital Diaphragmatic Hernia Studies
- Intestinal Malrotation and Obstruction Disorders
- Organ Donation and Transplantation
- Liver physiology and pathology
- Lysosomal Storage Disorders Research
- 3D Printing in Biomedical Research
- Tissue Engineering and Regenerative Medicine
- Neonatal Respiratory Health Research
- Genetic and Kidney Cyst Diseases
- Glycogen Storage Diseases and Myoclonus
- Mesenchymal stem cell research
- Esophageal and GI Pathology
- Viral-associated cancers and disorders
- Gastrointestinal disorders and treatments
National Center For Child Health and Development
2019-2025
Kyushu University
2015-2024
Meiji Pharmaceutical University
2017
Ministero della Salute
2015
Cell-based therapy has been proposed as an alternative to orthotopic liver transplantation. The novel transplantation of in vitro-generated bud might have therapeutic potential. In vivo and ex methods for growing a are essential paving the way clinical translation We herein report method buds that grown involving on transected parenchyma liver, which showed long engraftment marked growth comparison heterotopic Furthermore, this study demonstrates rapidly fabricating scalable liver-like...
Abstract Introduction Liver transplantation is a gold standard treatment for intractable liver diseases. Because of the shortage donor organs, alternative therapies have been required. Due to their potential differentiate into variety mature cells, stem cells are considered feasible cell sources regeneration. Stem from human exfoliated deciduous teeth (SHED) exhibit hepatogenic capability in vitro . In this study, we investigated vivo capabilities homing and hepatocyte differentiation...
Neonates with congenital diaphragmatic hernia often require surgical defect closure a patch. Alternatives to native tissue are critically needed for this paediatric surgery. The clinical efficacy of mesh patches is limited by complications associated residual foreign material and recurrence. In study, we used novel bio-3D printer method generate large scaffold-free composed human cells. resulting constructs had high elasticity strength. Cellular were transplanted into rats surgically created...
Liver transplantation (LT) is a well-accepted treatment for primary sclerosing cholangitis (PSC) with generally good outcomes, although recurrent PSC (rPSC) poses significant challenges. This study aimed to describe patient characteristics and identify potential risk factors of rPSC in pediatric LT recipients. retrospective analyzed 13 patients who underwent at single center. Patient characteristics, factors, outcomes were compared between those without rPSC. The median age diagnosis was 5.2...
While sarcopenia is an important predictor of LT outcomes in adults, few studies have examined the association with pediatric patients. We investigated clinical influence on post-transplant infants BA. To define infants, cross-sectional area tPMA 93 healthy control was measured by computed tomography. Sarcopenia defined as a lower than two standard deviations below mean infants. Eighty-nine BA median age at 7.6 months old were enrolled. The characteristics and verified group (n = 21)...
Abstract Background Methylmalonic acidemia (MMA) is an autosomal recessive disorder caused by defects in propionyl‐CoA (P‐CoA) catabolism; of note, liver neoplasms rarely occur as a long‐term complication the disorder. Herein, we report case patient with MMA and hepatocellular carcinoma (HCC) who was successfully treated living‐donor transplant (LDLT) following prior kidney transplantation. Case Report A 25‐year‐old male underwent LDLT left lobe graft because metabolic instability neoplasms....
Abstract Sandhoff disease (SD) is caused by the loss of β-hexosaminidase (Hex) enzymatic activity in lysosomes resulting from Hexb mutations. In SD patients, Hex substrate GM2 ganglioside accumulates abnormally neuronal cells, loss, microglial activation, and astrogliosis. −/− mice, which manifest a phenotype similar to SD, serve as animal models for examining pathophysiology SD. mice reach ~8 weeks without obvious neurological defects; however, trembling begins at 12 accompanied startle...
We evaluated the outcomes of liver transplantation (LT) in pediatric and adult patients with biliary atresia (BA). focused on bowel perforation after LT (BPLT) as most common surgical complication analyzed its risk factors.This was a retrospective analysis 70 BA who underwent LT. The were divided into three groups according to timing LT: within first year age (Group A), between 1 12 years B), C). clinical presentations BPLT compared. variables without assess factors.The did not affect...
Glycogen storage disease (GSD) type 1b (Online Mendelian Inheritance in Man [OMIM] 232220) is an autosomal recessive inborn error of carbohydrate metabolism caused by defects glucose‐6‐phosphate translocase. GSD1b patients have severe hypoglycemia with several clinical manifestations hepatomegaly, obesity, a doll‐like face, and neutropenia. Liver transplantation (LT) has been indicated for glucose intolerance, poor metabolic control (PMC), growth (PG). We retrospectively reviewed 11 children...
Abstract Living donor liver transplantation (LDLT) has become a major life‐saving procedure for children with end‐stage disease in Japan, whereas deceased (DDLT) achieved only limited success. The annual number of pediatric transplantations is approximately 100‐120, patient 20‐year survival rate 81.0%. In 2005, the program at National Center Child Health and Development Tokyo, was initiated, an overall 560 patients to date. July 2010, our center qualified as DDLT center; total 132 were...
In Sandhoff disease (SD), the activity of lysosomal hydrolytic enzyme, β-hexosaminidase (Hex), is lost due to a Hexb gene defect, which results in abnormal accumulation substrate, GM2 ganglioside (GM2), neuronal cells, causing loss, microglial activation, and astrogliosis. We established induced pluripotent stem cells from SD mice (SD-iPSCs). present study, we investigated occurrence differentiation development neural lineage asymptomatic phase vitro using mouse fetus-derived (NSCs)...
ABSTRACT Objectives: Acetylcholinesterase (AChE) staining has become the gold standard for definitively diagnosing Hirschsprung disease (HD), although some pitfalls have been reported. We reevaluated a large series at our institute in order to validate accuracy of AChE detecting HD. Methods: A retrospective study rectal mucosal specimens all children with suspected HD during 13‐year period was performed. The were stained according modified Karnovsky‐Roots method staining. final diagnosis,...
Determining the cause of bowel obstruction without a history laparotomy (BO HL) is difficult and can result in delay treatment development potentially life-threatening situation. We herein investigated clinical characteristics pediatric patients who underwent due to BO HL.All surgical aged <16 age were diagnosed with HL between January 2004 September 2014 included. Etiology BO, intraoperative findings postoperative outcome retrospectively.Seventy this period. In these 70 patients,...
To review the current institutional practice to treat patients with congenital extrahepatic portosystemic shunt (CEPS) and determine optimal strategy.We retrospectively reviewed records of 55 diagnosed CEPS at our center between December 2008 March 2022.Among these patients, 44 (80.0%) received treatment for a median age 4.7 years. The most common indication was cardiopulmonary complications (45.5%). Therapeutic intervention included closure by endovascular techniques (50.0%) or surgery...
In the last few decades, collaboration between international pediatric oncology groups has resulted in significant improvement survival after liver transplantation (LT) for tumors, and LT become accepted standard of care unresectable tumors-either living donor or deceased transplantation. Hepatoblastoma HCC are common malignancies treated by LT, is now treatment modality nonmetastatic cases. The long-term rate more than 80% hepatoblastoma transplants. Furthermore, with advent...
Liver transplantation has been indicated for propionic acidemia (PA) patients with frequent metabolic decompensation and performed as an enzyme replacement therapy. We retrospectively evaluated the outcomes of PA analyze appropriate timing living donor liver (LDLT).
The prophylactic oral administration of vitamin K to newborns has markedly reduced the incidence deficiency (VKD); however, intracranial hemorrhage (ICH) is still one complications found in biliary atresia (BA) patients and associated with VKD bleeding. Therefore, we aimed investigate long-term outcome ICH BA who previously received during neonatal period.Eighty-eight consecutive infants were treated followed up at Kyushu University Hospital from 1979 2009. clinical records imaging study...