Liver Failure From Ultra-Short Bowel Syndrome on the Intestinal Transplant Waiting List: A Retrospective Study

Noguchi, Yuki; Ueno, Takayoshi; Matsuura, Ryohei; Kodama, Tatsuhiko; Deguchi, Kentaro; Umeda, Satoshi; Yamamichi, Taku; Nakahata, Kengo; Zenitani, Masahiro; Takama, Yuichi; Yamanaka, Hiroaki; Tazuke, Yuko; Okuyama, Hiroomi

doi:10.1016/j.transproceed.2016.10.019

Cited by 9 publications

(3 citation statements)

References 11 publications

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“…Patients with short bowel syndrome (SBS) are potential candidates for small‐intestine transplantation. Surgery is particularly indicated if they have ultra‐short bowel syndrome (USBS), which is the most severe form of the syndrome and normally indicates that they have a residual small bowel length that is less than 20 cm , or they would need long‐term parenteral nutrition (PN) . For the purposes of this study, we have defined USBS as a residual small bowel length of below 10 cm.…”

Section: Introductionmentioning

confidence: 99%

Nutritional therapy complications in children with ultra‐short bowel syndrome include growth deficiency but not cholestasis

et al. 2018

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AimChildren with ultra‐short bowel syndrome (USBS) have not been extensively studied to date because the condition is rare. The aim of the study was to assess the nutritional status of children with USBS receiving home parenteral nutrition, using citrulline serum concentration and cholestasis.MethodsWe studied 17 patients with USBS, with a median age of 6.6 years and median duration of parenteral nutrition of 6.6 years. The study was carried out at The Children's Memorial Health Institute, Warsaw, from January 2014 to January 2015.ResultsThe median standard deviation score (SDS) was −1.2 for body mass according to chronological age, −1.72 according to height and −0.59 according to height for age. Patients requiring seven days per week parenteral nutrition had a citrulline concentration below 10 μmol/L. Decreased bone‐mineral density was observed in 87% of the patients. Low values of 25‐hydroxyvitamin D were found in 53% of the children. None of the patients had elevated conjugated bilirubin levels above 34.2 μmol/L.ConclusionChildren with USBS were growth deficient according to their chronological age, with frequent abnormal bone mineralisation and vitamin D deficiency. Children requiring parenteral nutrition seven days a week had citrulline concentrations below 10 μmol/L. Cholestasis was not seen.

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Section: Introductionmentioning

confidence: 99%

Nutritional therapy complications in children with ultra‐short bowel syndrome include growth deficiency but not cholestasis

et al. 2018

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“…Preliminary unpublished analyses show that 1‐year survival of ITx was 66.6% and the 2‐year survival was 44.5% in our program. Although this point of view has recently been challenged as some believe that early referral of patients for ITx will decrease rates of IFALD and liver survival, decrease costs attributed to long‐term PN and HPN and will eventually improve quality of life among patients with SBS 26–28 . In a study by Abu‐Elmagd, they concluded that early ITx within the first 2 years, similar to the situation with kidney transplantation and dialysis, is more cost‐effective compared with long‐term PN 26 .…”

Section: Discussionmentioning

confidence: 99%

“…Although this point of view has recently been challenged as some believe that early referral of patients for ITx will decrease rates of IFALD and liver survival, decrease costs attributed to long-term PN and HPN and will eventually improve quality of life among patients with SBS. [26][27][28] In a study by Abu-Elmagd, they concluded that early ITx within the first 2 years, similar to the situation with kidney transplantation and dialysis, is more cost-effective compared with long-term PN. 26 The same conclusion was seen in the study by Sudan et al, 29 as they documented that patients with IF often have psychiatric problems because of the chronic nature of their disease, which is more pronounced in regions where facilities for HPN are either missing or limited and patients are required to be hospitalized for long durations to receive PN.…”

Section: Variables Statisticsmentioning

confidence: 99%

Experiences with intestinal failure from an intestinal rehabilitation unit in a country without home parenteral nutrition

Nikoupour

Arasteh

Shamsaeefar

et al. 2021

J Parenter Enteral Nutr

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Objective: For the first time, we reported experiences with an intestinal rehabilitation unit (IRU) from a country without home parenteral nutrition (HPN). Methods:We included patients with a diagnosis of intestinal failure (IF) since the establishment of our IRU from February 2018 to February 2020. We further report on our protocols for management of enterocutaneous fistulas (ECFs), short-bowel syndrome (SBS), chronic intestinal pseudo-obstruction and motility disorders.Results: Among a total of 349 patients who have been admitted, 100 patients had IF and were included . Mean (SD) age of patients was 46.3 ± 16.1 years. Most common cause of IF was ECFs (32%), SBS (24%), and SBS + fistula (22%). Most common causes of SBS were mesenteric ischemia (63.3%) and repeated surgery (22.4%). Median (interquartile range [IQR]) duration of parenteral nutrition (PN) for patients was 32 (18-60) days. The most common reconstructive surgery performed was resection and anastomosis (75.4%), followed by serial transverse enteroplasty procedure (10.5%) and closure of ostoma (7%). Patients were hospitalized for a median (IQR) of 33 (17.5-61) days. Most common complications were sepsis (45%), catheter infections (43%), and catheter thrombosis (20%), respectively. At the final follow-up, 61% stopped receiving PN, 23% became candidates for transplantation, and 16% died. Conclusion:Considering that most countries lack facilities for HPN, by establishing IRUs using specific treatment protocols and autologous gastrointestinal reconstruction techniques will provide a means to manage patients with IF, thus decreasing death rates and number of patients who require intestinal transplantations due to IF.

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Early detection of liver fibrosis with serum Mac-2 binding protein glycosylation-modified isomer (M2BPGi) during follow-up intestinal failure patients without intestinal failure-associated liver disease (IFALD)

et al. 2022

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Mac-2 binding protein glycosylation-modi ed isomer (M2BPGi) is a new marker for hepatic brosis progression. We examined the relationship between serum M2BPGi levels and liver histological ndings in intestinal failure (IF) patients without IF-associated liver disease (IFALD). MethodsThis study included IF patients without IFALD followed at our hospital. All patients underwent routine liver biopsies per protocol every 1-2 years. We examined M2BPGi levels and histological ndings in relation to aspartate aminotransferase (AST) to platelet ratio index, brosis-4 index, and AST/ALT ratio. Liver brosis was evaluated based on the METAVIR score. ResultsTotal 18 liver biopsies out of 8 patients were included. The median age was 11.5 years. Mean M2BPGi was 0.44 cutoff index (COI) in patients with F0 brosis; 0.78 COI in patients with F1 brosis; and 1.63 COI in patients with F2 brosis. Mean M2BPGi was signi cantly higher in patients with F2 versus F1 or F0 brosis (P < 0.016 and P < 0.028, respectively). M2BPGi levels were more strongly correlated with brosis stage than with other conventional brosis markers. ConclusionSerum M2BPGi is a novel marker of liver brosis in patients with IF. It is useful for follow-up prior to IFALD. Serum M2BPGi levels can support the interpretation of liver status.

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Liver Failure From Ultra-Short Bowel Syndrome on the Intestinal Transplant Waiting List: A Retrospective Study

Cited by 9 publications

References 11 publications

Nutritional therapy complications in children with ultra‐short bowel syndrome include growth deficiency but not cholestasis

Nutritional therapy complications in children with ultra‐short bowel syndrome include growth deficiency but not cholestasis

Experiences with intestinal failure from an intestinal rehabilitation unit in a country without home parenteral nutrition

Early detection of liver fibrosis with serum Mac-2 binding protein glycosylation-modified isomer (M2BPGi) during follow-up intestinal failure patients without intestinal failure-associated liver disease (IFALD)

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