{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,7]],"date-time":"2026-01-07T18:35:40Z","timestamp":1767810940408,"version":"3.49.0"},"reference-count":9,"publisher":"BMJ","issue":"1","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2025,12,22]]},"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:p>CHAPLE syndrome is a recently described genetic disorder characterised by dysregulation of the complement pathway, with the acronym standing for \u2018CD55 deficiency with complement hyperactivation, angiopathic thrombosis and protein-losing enteropathy\u2019. Some patients develop thrombotic complications such as Budd-Chiari syndrome (BCS). Due to its rarity, management guidelines are lacking. We attempted endovascular angioplasty and stenting of the thrombosed right hepatic vein in a male child with BCS secondary to CHAPLE, diagnosed by symptoms and genetic testing. This case is potentially the first documented instance of CHAPLE syndrome associated with BCS in which endovascular therapy was used. The child was on enoxaparin post procedure, and although ascites and effusions initially improved, the stent occluded, necessitating repeat angioplasty and stenting 5 days later. Re-thrombosis occurred despite good intraoperative flow, leading to supportive care to manage symptoms. Our case shows limited benefits of endovascular therapy in such cases.<\/jats:p>","DOI":"10.1136\/bcr-2025-270739","type":"journal-article","created":{"date-parts":[[2026,1,7]],"date-time":"2026-01-07T12:45:46Z","timestamp":1767789946000},"page":"e270739","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Hepatic vein angioplasty and stenting in a paediatric patient with Budd-Chiari syndrome secondary to CHAPLE syndrome: is endovascular therapy the answer?"],"prefix":"10.1136","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3317-8353","authenticated-orcid":false,"given":"Biswajit","family":"Sahoo","sequence":"first","affiliation":[{"name":"Radiodiagnosis, AIIMS Bhubaneswar, Bhubaneswar, Odisha, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Harsh","family":"Agarwal","sequence":"additional","affiliation":[{"name":"Radiodiagnosis, AIIMS Bhubaneswar, Bhubaneswar, Odisha, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Krishna Mohan","family":"Gulla","sequence":"additional","affiliation":[{"name":"Paediatrics, AIIMS Bhubaneswar, Bhubaneswar, Odisha, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1146-2302","authenticated-orcid":false,"given":"Manoj Kumar","family":"Nayak","sequence":"additional","affiliation":[{"name":"Radiodiagnosis, AIIMS Bhubaneswar, Bhubaneswar, Odisha, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Manas Kumar","family":"Panigrahi","sequence":"additional","affiliation":[{"name":"Gastroenterology, AIIMS Bhubaneswar, Bhubaneswar, Odisha, India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2026,1,7]]},"reference":[{"key":"2026010704454196000_19.1.e270739.1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1615887"},{"key":"2026010704454196000_19.1.e270739.2","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1111\/imr.12715","article-title":"CHAPLE syndrome uncovers the primary role of complement in a familial form of Waldmann\u2019s disease","volume":"287","author":"Ozen","year":"2019","journal-title":"Immunol Rev"},{"key":"2026010704454196000_19.1.e270739.3","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1186\/s13023-024-03277-9","article-title":"Pozelimab for CHAPLE disease: results from in-trial interviews and clinical outcome assessments","volume":"19","author":"Litcher-Kelly","year":"2024","journal-title":"Orphanet J Rare Dis"},{"key":"2026010704454196000_19.1.e270739.4","doi-asserted-by":"crossref","DOI":"10.1542\/peds.2022-059335","article-title":"CD55 Deficiency With Budd-Chiari Syndrome Treated by Liver Transplantation and Eculizumab","volume":"152","author":"Ohlsson","year":"2023","journal-title":"Pediatrics"},{"key":"2026010704454196000_19.1.e270739.5","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1056\/NEJMc1707173","article-title":"Loss of CD55 in Eculizumab-Responsive Protein-Losing Enteropathy","volume":"377","author":"Kurolap","year":"2017","journal-title":"N Engl J Med"},{"key":"2026010704454196000_19.1.e270739.6","doi-asserted-by":"crossref","first-page":"1421","DOI":"10.1080\/14656566.2024.2388267","article-title":"Pharmacotherapy for CD55 deficiency with CHAPLE disease: how close are we to a cure?","volume":"25","author":"Can","year":"2024","journal-title":"Expert Opin Pharmacother"},{"key":"2026010704454196000_19.1.e270739.7","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1097\/MPG.0000000000002198","article-title":"Eculizumab Is Safe and Effective as a Long-term Treatment for Protein-losing Enteropathy Due to CD55 Deficiency","volume":"68","author":"Kurolap","year":"2019","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"2026010704454196000_19.1.e270739.8","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1016\/S0140-6736(23)02358-9","article-title":"Evaluating the efficacy and safety of pozelimab in patients with CD55 deficiency with hyperactivation of complement, angiopathic thrombosis, and protein-losing enteropathy disease: an open-label phase 2 and 3 study","volume":"403","author":"Ozen","year":"2024","journal-title":"Lancet"},{"key":"2026010704454196000_19.1.e270739.9","doi-asserted-by":"crossref","first-page":"2587","DOI":"10.3748\/wjg.v9.i11.2587","article-title":"Long-term effect of stent placement in 115 patients with Budd-Chiari syndrome","volume":"9","author":"Zhang","year":"2003","journal-title":"World J Gastroenterol"}],"container-title":["BMJ Case 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