Gastrostomy Tube Insertion in Pediatric Patients With Autosomal Recessive Polycystic Kidney Disease (ARPKD): Current Practice
Introduction: Autosomal recessive polycystic kidney disease (ARPKD) is a severe hepatorenal disorder of childhood. Early renal disease in ARPKD may require renal replacement therapy and is associated with failure to thrive resulting in a need for nasogastric tube feeding or gastrostomy. In ARPKD pat...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2018-06-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fped.2018.00164/full |
| _version_ | 1818943833595969536 |
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| author | Kathrin Burgmaier Joy Brandt Rukshana Shroff Peter Witters Lutz T. Weber Jörg Dötsch Franz Schaefer Djalila Mekahli Djalila Mekahli Max C. Liebau Max C. Liebau |
| author_facet | Kathrin Burgmaier Joy Brandt Rukshana Shroff Peter Witters Lutz T. Weber Jörg Dötsch Franz Schaefer Djalila Mekahli Djalila Mekahli Max C. Liebau Max C. Liebau |
| author_sort | Kathrin Burgmaier |
| collection | DOAJ |
| description | Introduction: Autosomal recessive polycystic kidney disease (ARPKD) is a severe hepatorenal disorder of childhood. Early renal disease in ARPKD may require renal replacement therapy and is associated with failure to thrive resulting in a need for nasogastric tube feeding or gastrostomy. In ARPKD patients, the benefit of a gastrostomy in nutrition and growth needs to be weighed against the potential risk of complications of congenital hepatic fibrosis (CHF) and portal hypertension like variceal bleeding. CHF in ARPKD has thus been considered as a relative contraindication for gastrostomy insertion. Yet, data on gastrostomies in pediatric patients with ARPKD is lacking.Methods: We conducted a web-based survey study among pediatric nephrologists, pediatric hepatologists and pediatric gastroenterologists on their opinions on and experiences with gastrostomy insertion in ARPKD patients.Results: 196 participants from 39 countries shared their opinion. 45% of participants support gastrostomy insertion in all ARPKD patients, but portal hypertension is considered to be a contraindication by a subgroup of participants. Patient-specific data was provided for 38 patients indicating complications of gastrostomy that were in principal comparable to non-ARPKD patients. Bleeding episodes were reported in 3/38 patients (7.9%). Two patients developed additional severe complications. Gastrostomy was retrospectively considered as the right decision for the patient in 35/38 (92.1%) of the cases.Conclusions: This report on the results of an online survey gives first insights into the clinical practice of gastrostomy insertion in ARPKD patients. For the majority of participating physicians benefits of gastrostomy insertion retrospectively outweigh complications and risks. More data will be required to lay the foundation for clinical recommendations. |
| first_indexed | 2024-12-20T07:33:37Z |
| format | Article |
| id | doaj.art-e4b3c9633b084ef7abf42c9fc8544d26 |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-12-20T07:33:37Z |
| publishDate | 2018-06-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-e4b3c9633b084ef7abf42c9fc8544d262022-12-21T19:48:21ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602018-06-01610.3389/fped.2018.00164358268Gastrostomy Tube Insertion in Pediatric Patients With Autosomal Recessive Polycystic Kidney Disease (ARPKD): Current PracticeKathrin Burgmaier0Joy Brandt1Rukshana Shroff2Peter Witters3Lutz T. Weber4Jörg Dötsch5Franz Schaefer6Djalila Mekahli7Djalila Mekahli8Max C. Liebau9Max C. Liebau10Department of Pediatrics, University Hospital of Cologne, Cologne, GermanyDepartment of Pediatrics, University Hospital of Cologne, Cologne, GermanyGreat Ormond Street Hospital for Children NHS Foundation Trust, London, United KingdomDepartment of Pediatric Gastroenterology and Hepatology, University Hospitals Leuven, Leuven, BelgiumDepartment of Pediatrics, University Hospital of Cologne, Cologne, GermanyDepartment of Pediatrics, University Hospital of Cologne, Cologne, GermanyDivision of Pediatric Nephrology, Center for Pediatric and Adolescent Medicine, University of Heidelberg, Heidelberg, GermanyDepartment of Pediatric Nephrology, University Hospitals Leuven, Leuven, BelgiumPKD Research Group, Department of Development and Regeneration, KU Leuven, University of Leuven, Leuven, BelgiumDepartment of Pediatrics, University Hospital of Cologne, Cologne, GermanyCenter for Molecular Medicine, University Hospital of Cologne, Cologne, GermanyIntroduction: Autosomal recessive polycystic kidney disease (ARPKD) is a severe hepatorenal disorder of childhood. Early renal disease in ARPKD may require renal replacement therapy and is associated with failure to thrive resulting in a need for nasogastric tube feeding or gastrostomy. In ARPKD patients, the benefit of a gastrostomy in nutrition and growth needs to be weighed against the potential risk of complications of congenital hepatic fibrosis (CHF) and portal hypertension like variceal bleeding. CHF in ARPKD has thus been considered as a relative contraindication for gastrostomy insertion. Yet, data on gastrostomies in pediatric patients with ARPKD is lacking.Methods: We conducted a web-based survey study among pediatric nephrologists, pediatric hepatologists and pediatric gastroenterologists on their opinions on and experiences with gastrostomy insertion in ARPKD patients.Results: 196 participants from 39 countries shared their opinion. 45% of participants support gastrostomy insertion in all ARPKD patients, but portal hypertension is considered to be a contraindication by a subgroup of participants. Patient-specific data was provided for 38 patients indicating complications of gastrostomy that were in principal comparable to non-ARPKD patients. Bleeding episodes were reported in 3/38 patients (7.9%). Two patients developed additional severe complications. Gastrostomy was retrospectively considered as the right decision for the patient in 35/38 (92.1%) of the cases.Conclusions: This report on the results of an online survey gives first insights into the clinical practice of gastrostomy insertion in ARPKD patients. For the majority of participating physicians benefits of gastrostomy insertion retrospectively outweigh complications and risks. More data will be required to lay the foundation for clinical recommendations.https://www.frontiersin.org/article/10.3389/fped.2018.00164/fullARPKDcongenital hepatic fibrosisportal hypertensionperitoneal dialysisPKHD1pediatric polycystic kidney disease |
| spellingShingle | Kathrin Burgmaier Joy Brandt Rukshana Shroff Peter Witters Lutz T. Weber Jörg Dötsch Franz Schaefer Djalila Mekahli Djalila Mekahli Max C. Liebau Max C. Liebau Gastrostomy Tube Insertion in Pediatric Patients With Autosomal Recessive Polycystic Kidney Disease (ARPKD): Current Practice Frontiers in Pediatrics ARPKD congenital hepatic fibrosis portal hypertension peritoneal dialysis PKHD1 pediatric polycystic kidney disease |
| title | Gastrostomy Tube Insertion in Pediatric Patients With Autosomal Recessive Polycystic Kidney Disease (ARPKD): Current Practice |
| title_full | Gastrostomy Tube Insertion in Pediatric Patients With Autosomal Recessive Polycystic Kidney Disease (ARPKD): Current Practice |
| title_fullStr | Gastrostomy Tube Insertion in Pediatric Patients With Autosomal Recessive Polycystic Kidney Disease (ARPKD): Current Practice |
| title_full_unstemmed | Gastrostomy Tube Insertion in Pediatric Patients With Autosomal Recessive Polycystic Kidney Disease (ARPKD): Current Practice |
| title_short | Gastrostomy Tube Insertion in Pediatric Patients With Autosomal Recessive Polycystic Kidney Disease (ARPKD): Current Practice |
| title_sort | gastrostomy tube insertion in pediatric patients with autosomal recessive polycystic kidney disease arpkd current practice |
| topic | ARPKD congenital hepatic fibrosis portal hypertension peritoneal dialysis PKHD1 pediatric polycystic kidney disease |
| url | https://www.frontiersin.org/article/10.3389/fped.2018.00164/full |
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