Intestinal Failure‐Associated Liver Disease

Beath, S. V.; Woodward, Jeremy

doi:10.1002/9781405195805.ch21

Cited by 7 publications

(5 citation statements)

References 67 publications

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“…Liver function tests were performed, in addition to imaging and biopsy procedures, classifying the hepatic function in mild, moderate, and severe, according to the IFALD. 13 IFALD was defined as the persistent elevation of serum transaminases 1.5 times above the normal upper limit in the presence of short bowel. 8 Different changes in patient treatment protocol were seen over the years, thus making comparisons difficult.…”

Section: Methodsmentioning

confidence: 99%

Ultrashort Bowel Syndrome Outcome in Children Treated in a Multidisciplinary Intestinal Rehabilitation Unit

et al. 2017

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Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in the pediatric population. Our aim was to review long-term outcome of ultrashort bowel syndrome (USBS) in an Intestinal Rehabilitation Unit (IRU). Retrospective study of patients with USBS (defined as< 10 cm of remnant small bowel) treated between 2000 and 2015. Demographic data, clinical, and treatment variables including parenteral nutrition (PN), surgical techniques, and intestinal transplantation (IT) were analyzed. Out of 250 children, 30 referred to the IRU met inclusion criteria. Upon first assessment, patients had a median age of 3 (1-217) months and had undergone 3 (1-6) previous laparotomies that left 5 (0-9) cm of remnant small bowel. The main cause of USBS was neonatal midgut volvulus (50%). Follow-up was 28 (4-175) months. Advanced IF-associated liver disease (IFALD) was documented in 63%. None of the patients achieved digestive autonomy and was consequently considered for IT. One patient was excluded, five died before IT, and three are still on the waiting list. Six patients received an isolated IT, 6 a combined liver IT, and 18 a multivisceral graft. Digestive autonomy was achieved in 71% after 31 (14-715) days after IT and currently 62% are alive and off total PN. A significant drop in IFALD progression prior to IT was observed with the introduction of new lipid emulsions in 2010 (SMOF or Soy oil MCT (mid-chain triglycerides) Olive oil Fish oil). A multidisciplinary IRU including an IT program offers a comprehensive approach for patients with IF and is crucial to improve survival rate of USBS. New PN lipid emulsions had an impact on IFALD progression and may eventually reduce overall mortality.

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Section: Methodsmentioning

confidence: 99%

Ultrashort Bowel Syndrome Outcome in Children Treated in a Multidisciplinary Intestinal Rehabilitation Unit

et al. 2017

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“…Liver function tests were performed, in addition to imaging and biopsy procedures, classifying the hepatic function in mild, moderate, and severe, according to the IFALD criteria. 9 From a surgical standpoint, data of the remaining bowel length before and after the procedure were noted; the elongation rate was calculated as the difference between both lengths. The elongation percentage was the result of the division of the elongation by the remaining bowel length before the procedure.…”

Section: Methodsmentioning

confidence: 99%

Surgical Rehabilitation Techniques in Children with Poor Prognosis Short Bowel Syndrome

et al. 2015

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Intestinal failure (IF) requires a multidisciplinary management based on nutritional support, surgical and medical rehabilitation, and transplantation. The aim of this study is to review our experience with surgical rehabilitation techniques (SRTs: enteroplasty, Bianchi, Serial Transverse Enteroplasty Procedure [STEP]) in patients with short bowel syndrome (SBS) and poor prognosis due to complex abdominal pathology. We performed a single-center retrospective study of patients with IF evaluated for intestinal transplantation in the Intestinal Rehabilitation Unit who underwent an SRT. Nonparametric tests were used for statistical analysis.A total of 205 patients (107 males/98 females) with mean age of 25 ± 7 months were assessed for IF. A total of 433 laparotomies were performed on 130 patients including intestinal resection, enteroplasties, adhesiolysis, and transit reconstruction. SRT were performed in 22 patients: 12 enteroplasties, 8 STEPs, and 4 Bianchi procedures. All patients were parenteral nutrition (PN) dependent with different stages of liver disease: mild (13), moderate (5), and severe (4). The adaptation rate for patients who underwent enteroplasty, STEP, and Bianchi were 70, 63, and 25%, respectively, although the techniques are not comparable. Overall, intestinal adaptation was achieved in nine (41%) patients, and four (18%) patients showed significant reduction of PN needs. One child did not respond to SRT and did not meet transplantation criteria. The remaining eight (36%) patients were included on the waiting list for transplant: four were transplanted, two are still on the waiting list, and two died. Better outcomes were observed in milder cases of liver disease (mild 77%, moderate 40%, severe 25%) (p < 0.05). Conversely, a trend toward a poorer outcome was observed in cases with ultrashort bowel (p > 0.05). One patient required reoperation after a Bianchi procedure due to intestinal ischemia and six needed further re-STEP or adhesiolysis procedure several months later. The median follow-up was 62 (3-135) months. Overall mortality was 19%, and was due to end-stage liver disease and/or central venous catheter-related sepsis. SRT led to intestinal adaptation in a significant number of patients with poor prognosis SBS referred for intestinal transplantation. However, SRT requires a multidisciplinary evaluation and should be attempted only in suitable cases. Careful assessment and optimal surgical timing is crucial to obtain a favorable outcome.

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“…3. Алгоритм диагностики и лечения ПП-ассоциированного заболевания печени [18] ПЕДИАТРИЧЕСКАЯ ФАРМАКОЛОГИЯ /2013/ ТОМ 10/ № 4 вается, вытесняя токсичные гидрофобные желчные кисло-ты и холестерин. В гепатоцитах она конъюгирует с таурином и глицином, выделяется с желчью в кишечник, где рас-щепляется до литохолевой кислоты.…”

Section: консультация гепатологаunclassified

“…2 суммированы рекомендации по ведению пациентов с ПП-ассоциированным заболеванием печени [18], на рис. 3 отражен алгоритм диагностики и лечения ПП-ассоциированного заболевания печени [18].…”

Section: консультация гепатологаunclassified