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Ileal exclusion for pruritus treatment in children with progressive familial intrahepatic cholestasis and other cholestatic diseases.
Van Vaisberg, Victor; Tannuri, Ana Cristina Aoun; Lima, Fabiana Roberto; Tannuri, Uenis.
Afiliação
  • Van Vaisberg V; Pediatric Surgery Division, Pediatric Liver Transplantation Unit and Laboratory of Research in Pediatric Surgery (LIM 30), University of Sao Paulo Medical School, Sao Paulo, Brazil.
  • Tannuri ACA; Pediatric Surgery Division, Pediatric Liver Transplantation Unit and Laboratory of Research in Pediatric Surgery (LIM 30), University of Sao Paulo Medical School, Sao Paulo, Brazil.
  • Lima FR; Pediatric Surgery Division, Pediatric Liver Transplantation Unit and Laboratory of Research in Pediatric Surgery (LIM 30), University of Sao Paulo Medical School, Sao Paulo, Brazil.
  • Tannuri U; Pediatric Surgery Division, Pediatric Liver Transplantation Unit and Laboratory of Research in Pediatric Surgery (LIM 30), University of Sao Paulo Medical School, Sao Paulo, Brazil. Electronic address: uenist@usp.br.
J Pediatr Surg ; 55(7): 1385-1391, 2020 Jul.
Article em En | MEDLINE | ID: mdl-31708211
BACKGROUND: Pruritus is a major health-related quality-of-life burden in progressive familial intrahepatic cholestasis (PFIC) and other childhood cholestatic liver diseases. Several nontransplant surgical techniques were developed in an attempt to ameliorate symptoms and slow disease progression. Very few case-series have been published on a particular intervention, ileal exclusion (IE), which has been considered to be inferior to the other approaches. METHODS: We conducted a single-center retrospective chart-review case-series of patients submitted to IE as the first-line surgical treatment at our institution from 1995 to 2018. The primary goal was pruritus relief, followed by survival with the native liver and improvement in biochemical parameters. RESULTS: Eleven patients were submitted to IE, with a mean follow-up of 60 months. Complete resolution or significant reduction of pruritus was obtained in 72.7% (n = 8) of patients. One patient (9.1%) had a major postoperative complication that required surgery. No other morbidities were reported. Two cases progressed to end-stage liver disease (ESLD) within the short-term and one year after surgery. CONCLUSIONS: This case series study shows that IE provided excellent results in pruritus control and permitted survival with the native liver. We believe IE is a safe procedure, with few associated morbidities, and should be considered more often as primary surgical treatment for PFIC and other cholestasis. LEVEL OF EVIDENCE: IV.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Prurido / Procedimentos Cirúrgicos do Sistema Biliar / Colestase Intra-Hepática / Íleo Tipo de estudo: Etiology_studies / Observational_studies / Risk_factors_studies Aspecto: Patient_preference Limite: Child / Humans Idioma: En Revista: J Pediatr Surg Ano de publicação: 2020 Tipo de documento: Article País de afiliação: Brasil País de publicação: Estados Unidos
Texto completo
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Prurido / Procedimentos Cirúrgicos do Sistema Biliar / Colestase Intra-Hepática / Íleo Tipo de estudo: Etiology_studies / Observational_studies / Risk_factors_studies Aspecto: Patient_preference Limite: Child / Humans Idioma: En Revista: J Pediatr Surg Ano de publicação: 2020 Tipo de documento: Article País de afiliação: Brasil País de publicação: Estados Unidos