A5047047293- Cystic Fibrosis Research Advances
- Liver Disease and Transplantation
- Organ Transplantation Techniques and Outcomes
- Pediatric Hepatobiliary Diseases and Treatments
- Genetic and Kidney Cyst Diseases
- Liver Disease Diagnosis and Treatment
- Drug Transport and Resistance Mechanisms
- Infant Nutrition and Health
- Renal Transplantation Outcomes and Treatments
- Neonatal Respiratory Health Research
- Transplantation: Methods and Outcomes
- Child Nutrition and Feeding Issues
- Dialysis and Renal Disease Management
- Metabolism and Genetic Disorders
- Iron Metabolism and Disorders
- Trace Elements in Health
- Esophageal and GI Pathology
- Palliative Care and End-of-Life Issues
- Amino Acid Enzymes and Metabolism
- Gastroesophageal reflux and treatments
- Clinical Nutrition and Gastroenterology
- Gastrointestinal disorders and treatments
- Diabetes and associated disorders
- Congenital gastrointestinal and neural anomalies
- Tracheal and airway disorders
University Medical Center Groningen
2015-2024
University of Groningen
2015-2024
Dialyse Centrum Groningen
2009-2015
Boston Children's Hospital
2015
Introduction In the past decades, short-term results after solid organ transplantation have markedly improved. Disappointingly, this has not been accompanied by parallel improvements in long-term outcomes transplantation. To improve graft and recipient outcomes, identification of potentially modifiable risk factors development biomarkers are required. We provide rationale design a large prospective cohort study transplant recipients (TransplantLines). Methods analysis TransplantLines is...
ABSTRACT Objectives: Hepatobiliary complications are a leading cause of morbidity and mortality in cystic fibrosis (CF) patients. Knowledge the underlying pathological aspects optimal clinical management is, however, sorely lacking. Methods: We provide summary lectures given by international speakers at European Society for Paediatric Gastroenterology, Hepatology, Nutrition (ESPGHAN) monothematic conference on fibrosis–related liver disease (CFLD) held Paris January 2016, to discuss status...
Abstract The development, homeostasis, and repair of intrahepatic extrahepatic bile ducts are thought to involve distinct mechanisms including proliferation maturation cholangiocyte progenitor cells. This study aimed characterize human organoids (ECO) using canonical Wnt-stimulated culture medium previously developed for (ICO). Paired ECO ICO were derived from common duct liver tissue, respectively. Characterization showed both organoid types highly similar, though some differences in size...
Cystic fibrosis (CF) may cause a spectrum of hepatobiliary complications, including portal hypertension, multilobular cirrhosis, and liver failure. Current guidelines on the detection monitoring complications in CF were published 1999. The Foundation assembled committee to evaluate research advances formulate revised for CF-associated disease. A hepatologists, gastroenterologists, pulmonologists, pharmacists, nurses, dietitians, individuals with CF, parents child devised "population,...
Abstract The broad spectrum of hepatobiliary involvement in cystic fibrosis (CF) has been commonly referred to as liver disease (CFLD). However, differences the definitions CFLD have led variations reported prevalence, incidence rates, and standardized recommendations for diagnosis therapies. Harmonizing description all people with CF (pwCF) is deemed essential providing a reliable account natural history, which turn supports development meaningful clinical outcomes patient care research....
Pancreatic enzyme replacement therapy (PERT) improves nutritional status and growth in patients with cystic fibrosis (CF) pancreatic insufficiency (PI). The current recommendation for infants young children, who are not able to swallow the whole capsule, is open capsule mix beads a spoon some applesauce; however, efficacy safety data of this approach currently lacking. aim study was assess efficacy, palatability (ease swallowing), 4 dose levels pancrelipase microtablets (Pancrease MT)...
Identification of patients at risk for developing cirrhotic cystic fibrosis liver disease (CCFLD) is essential targeting potentially preventive treatment. We studied the evolution serum enzymes and thrombocyte counts as predictors CCFLD development.For this study, we defined diagnosis combination splenomegaly (on either physical examination or ultrasound scan) macronodularity on scan. reviewed medical records 277 pediatric with CF CCFLD. In each patient CCFLD, in 2-year period preceding...
ABSTRACT Objectives: Treatment targets in inflammatory bowel disease (IBD) move away from controlling symptoms towards complete recovery of the intestinal mucosa. Currently, most frequently used noninvasive surrogate marker mucosal healing is a faecal calprotectin concentration target range. This study tested if there was relation between time‐to‐reach and first flare. Methods: We prospectively included new‐onset IBD patients ages 17 younger cloud‐based registry (FastForwardCare) followed...
Fatigue and impaired health-related quality of life (HRQoL) are common among kidney transplant recipients (KTR). We hypothesized that both may partially be attributable to poor sleep.Cross-sectional longitudinal data KTR enrolled in the TransplantLines Biobank Cohort Study were used. Sleep was assessed using Pittsburgh Quality Index questionnaire. Individual strength (i.e. a composite fatigue, concentration, motivation physical activity), societal participation HRQoL validated...
Undiagnosed and untreated tyrosinemia type 1 (TT1) individuals carry a significant risk for developing liver fibrosis, cirrhosis hepatocellular carcinoma (HCC). Elevated succinylacetone (SA) is pathognomonic TT1 therefore often used as marker newborn screening (NBS). While SA was long considered to be elevated in every patient, here we present recent false-negative screen. A nine-year-old boy presented with HCC cirrhotic liver. Additional tests the underlying cause unexpectedly revealed TT1....
Kidney transplant recipients (KTRs) experience more fatigue, anxiety, and depressive symptoms lower concentration health-related quality of life (HRQoL) compared with the general population. Anemia is a potential cause that well-recognized treated. Iron deficiency, however, often unrecognized, despite its detrimental effects related to unrelated anemia. We investigated interplay anemia, iron patient-reported outcomes in 814 outpatient KTRs (62% male, age 56 ± 13 years) enrolled...
Gut dysbiosis has been associated with impaired outcomes in liver and kidney transplant recipients, but the gut microbiome of lung recipients not extensively explored. We assessed 64 fecal samples from end-stage disease patients before transplantation 219 after using metagenomic sequencing. To identify dysbiotic microbial signatures, we analyzed 243 age-, sex-, BMI-matched healthy controls. By unsupervised clustering, identified five groups different combinations immunosuppressants...