A5032411876- Organ Transplantation Techniques and Outcomes
- Liver Disease and Transplantation
- Pediatric Hepatobiliary Diseases and Treatments
- Renal Transplantation Outcomes and Treatments
- Drug Transport and Resistance Mechanisms
- Liver Disease Diagnosis and Treatment
- Liver Diseases and Immunity
- Neonatal Health and Biochemistry
- Childhood Cancer Survivors' Quality of Life
- Metabolism and Genetic Disorders
- Immunodeficiency and Autoimmune Disorders
- Congenital Anomalies and Fetal Surgery
- Adolescent and Pediatric Healthcare
- Gallbladder and Bile Duct Disorders
- Biomedical Research and Pathophysiology
- Genetic and Kidney Cyst Diseases
- Organ Donation and Transplantation
- Hemoglobinopathies and Related Disorders
- Kidney Stones and Urolithiasis Treatments
- Family Support in Illness
- Acute Lymphoblastic Leukemia research
- Hepatitis B Virus Studies
- Transplantation: Methods and Outcomes
- Neuroblastoma Research and Treatments
- Parvovirus B19 Infection Studies
Universität Hamburg
2013-2023
University Medical Center Hamburg-Eppendorf
2013-2023
Klinik und Poliklinik für Kinder- und Jugendmedizin
2002-2023
Hospital Universitario La Paz
2022
University Medical Center Groningen
2022
University of Groningen
2022
Children's Hospital of Eastern Ontario
2022
University of Ottawa
2022
Eppendorf (Germany)
2017
Pediatrics and Genetics
2008-2016
Organ fibrosis is a shared endpoint of many diseases, yet underlying mechanisms are not well understood. Several pathways governed by the primary cilium, sensory antenna present on most vertebrate cells, have been linked with fibrosis. Ciliopathies usually start early in life and represent considerable disease burden. We performed massively parallel sequencing using cohorts genetically unsolved individuals unexplained liver kidney failure correlated this clinical, imaging, histopathological...
Progressive familial intrahepatic cholestasis (PFIC) is caused by mutations of the bile salt export pump or multidrug resistance P-glycoprotein, resulting in chronic hepatic failure. Partial external diversion ileal bypass effective some cases and, others, liver transplantation (OLT) necessary. Forty-two children were included this study. Twenty-six suffered from PFIC type 2 and 16 3. Symptoms pruritus, cholestasis, cirrhosis, growth retardation. Seventeen patients received biliary...
Anti-HLA antibodies and especially donor-specific (DSA) play a significant role in graft survival after solid organ transplantation. Their impact on long-term adult liver transplantation (LT) is controversial, but they may be risk factor. The effects of DSA pediatric LT are still unclear.We performed retrospective evaluation sera from 43 children who had received transplants at our tertiary center. Twenty-four patients good clinical laboratory function (group 1), whereas 19 recipients...
Progressive familial intrahepatic cholestasis type 2 (PFIC‐2) is caused by mutations in ABCB11 , encoding the bile salt export pump (BSEP). In 2009, we described a child with PFIC‐2 who developed PFIC‐like symptoms after orthotopic liver transplantation (OLT). BSEP‐reactive antibodies were demonstrated to account for disease recurrence. Here, characterize nature of this antibody response 7 more patients antibody‐induced BSEP deficiency (AIBD). Gene sequencing and immunostaining native...
OBJECTIVE. Neonatal hemochromatosis is a severe, often fatal multiorgan disorder of iron metabolism. Liver transplantation can be curative; the benefit antioxidant treatment discussed controversially. We summarize our experience with neonatal over past 13 years. METHODS. A retrospective study was performed 16 patients acute liver failure attributable to between 1992 and 2004. RESULTS. Median age at onset 2 days (range: 0–21 days). weight time diagnosis 2900 g 1520–4200 g). All had elevated...
Despite major surgical and medical advances, it is still a challenge to perform transplantation in small infants. This study, focusing on short- long-term outcomes, summarizes our 10-year experience with liver (LTx) infants aged less than 6 months.We analyzed 43 patients months or (range: 12-184 days, median: 136 days) whose median weight at the time of LTx was 5.8 kg 2.8-8.0 kg). The reasons for were biliary atresia (n=27; 62.8%), neonatal hepatitis (n=6; 14%), cholestasis (n=4; 9.3%),...
Portal vein thrombosis can occur as a result of primary anomalies, after liver transplantation, and for other reasons. It may in severe complications secondary to portal hypertension, such bleeding from esophageal or gastric varices, hypersplenism, impaired somatic growth. In this retrospective study, we analyzed the outcome 25 children who underwent Rex shunt procedure. The following venous grafts were used shunt: autologous internal external jugular (n = 17) cryopreserved graft 5); three...
Abstract: Background: The presence of autoantibodies and development autoimmune hepatitis after liver transplantation has recently been reported as one the causes for chronic graft dysfunction. pathogenesis clinical significance this disease still remains unclear. Methods: We evaluate 96 patients prevalence pediatric review their follow‐up including virus serologies, ultrasound examination biopsies. Results: Positive were detected in 74% OLT. Graft dysfunction was observed 46% these...
Liver transplantation (LT) has been shown to be a feasible treatment in patients with severe forms of maple syrup urine disease (MSUD). Because sufficient extrahepatic enzyme activity non ‐ MSUD individuals, the organ can used as domino graft. We performed retrospective data collection all LTs for carried out at University Medical Center Hamburg‐Eppendorf (2016‐2018). Moreover, from consecutive grafts either transplanted our institution or allocated other transplant centers were analyzed....
Abstract: An AGS is a dominant inherited multisystem disorder caused by mutations in the Notch signaling pathway ( JAG1 ). In our center, 5.3% of liver transplantations (OLT) are performed children with AGS. Some affected fulfilled criteria for OLT, despite absence cirrhosis. The aim present study was to evaluate indications and outcome OLT this complex as clear difficult establish clinical practice. A total 37 patients were included retrospective analysis. Twenty‐four underwent chronic...
Abstract: It has been shown that an induction therapy with the monoclonal anti‐interleukin‐2 receptor antibody basiliximab (Simulect TM ) is capable to reduce incidence of acute graft rejection in adult and pediatric liver transplantation (Ltx). However, data on long‐term results using children post‐Ltx are still pending. Therefore, objective our study was report transplant recipients. A total 54 received two single doses addition cyclosporine prednisolone following Ltx. We analyzed chronic...
Abstract Liver transplantation offers excellent results for children with end‐stage liver disease, and efforts should be directed toward maintaining long‐term graft health. We evaluate pathology in healthy pediatric transplant recipients low‐maintenance immunosuppressive medications to assess whether protocol biopsies are helpful adapting immunosuppression protecting function. were performed on 60 recipients, histological findings correlated laboratory, serological, radiological results....
Introduction Combined or sequential liver and kidney transplantation (CLKT/SLKT) restores function corrects the underlying metabolic defect in children with end-stage disease primary hyperoxaluria type 1 (PH1). However, data on long-term outcome, especially infantile PH1, are rare. Methods All pediatric PH1-patients who underwent CLKT/SLKT at our center were analyzed retrospectively. Results Eighteen patients (infantile PH1 n = 10, juvenile 8) (CLKT 17, SLKT 1) a median age of 5.4 years...
Several factors may contribute to post-transplant cholestatic complications after liver transplantation. These include ischemic reperfusion injury, hypoperfusion, bile duct strictures, and hepatotoxic drugs. Up now, there have been no publications on tacrolimus toxicity in clinical transplantation when the drug was used therapeutic doses. We describe six pediatric graft recipients whom developed under a tacrolimus-based immunosuppression following all of them suffered from previous...
The aim of the study was to investigate occurrence fatigue in 100 pediatric liver transplant recipients aged 2-18 years and its impact on their health-related quality life (HRQL). HRQL were measured using PedsQL 4.0 Inventory Multidimensional Fatigue Scale, which encompasses three subscales: general fatigue, sleep/rest cognitive fatigue. different domains clinical sociodemographic factors identified with stepwise multiple regression analyses. Parent proxy-reports available for all...
Background. As long-term survival of pediatric liver transplant recipients increases, the assessment physical, psychological, and social well-being becomes more important. Methods. In this retrospective analysis, 120 young adult patients (age ≥18 y) who underwent transplantation in childhood were studied. Patients with ideal outcome defined as perfect graft function, no complications from immunosuppressive medication, late retransplantation, steroid treatment. Also, patients’ drug adherence...
Acute neonatal liver failure is a rare condition that often fatal. Liver transplantation (LTx) in affected neonates may be life saving, but there are only few data on the long-term outcome of LTx.We conducted retrospective study 11 LTx performed 10 pediatric patients with acute first month life. Median age at was 15 days (range: 7-31 days) and median weight 3.25 kg 2-4 kg). The reasons for were hemochromatosis (n=5), hemangioendothelioma (n=2), infection caused by echovirus type (n=1),...