Original ArticlePredicting Liver Failure in Parenteral Nutrition-Dependent Short Bowel Syndrome of Infancy
Section snippets
Methods
A total of 84 patients with SBS, defined as a minimum PN requirement of 6 weeks after resection, and cholestasis2 were examined between August 2003 and December 2007. Of these 84 patients, 61 in whom designated demographic and laboratory data could be collected were included in the investigation. Frequent multiple caregivers at multiple institutions before referral was the usual explanation for inadequate information leading to exclusion of patients, most of whom were in advanced LF at the time
Demographic and Anatomical Comparison of the LR and LF Groups
Of the 61 subjects, LF developed in 47; the remaining 14 patients later experienced hepatic recovery. As noted in Table I, the average age at initial examination of the 47 patients in whom LF developed was approximately 6 months; 41 of these patients (87%) were referred by other institutions. In contrast, 8 of 14 patients (57%) who experienced LR were referred from other institutions (P < .01, χ2 test) and were on average approximately half the age of the other patients. No patient referred
Discussion
Reports from several intestinal transplant centers have described the frequently advanced character of PNALD in pediatric patients at presentation, resulting in high mortality rates before transplantation can be attempted.13, 14, 15 One factor contributing to late referral is a lack of detailed information on the natural history of PNALD. In this study, we sought to ascertain whether delineation of trends in laboratory abnormalities, particularly at an early stage of the disease, might be more
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Transplantation
Organ allocation for liver-intestine candidates
Liver Transpl
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2022, Clinical Nutrition ESPENCitation Excerpt :Different studies with adult PNALD patients have shown elevated alkaline phosphatase being the most common abnormality [7]. Kaufman et al. in a cohort of infants proved that an increased platelet count and an increased albumin concentration predicted liver failure [8]. Since PNALD-related hepatitis may lead to progressive fibrosis and cirrhosis, and the available data on effective treatment are insufficient, it is reasonable to actively search for patients at high risk of liver injury.
Management of pediatric intestinal failure related to short bowel syndrome
2022, Seminars in Pediatric SurgeryThrombin dynamics in children with liver disease or extrahepatic portal vein obstruction or shunt
2020, Thrombosis ResearchCitation Excerpt :In particular, the international normalised ratio (INR) is used to predict adverse haematological outcomes and is also used for prognosis through its incorporation into the paediatric end stage liver disease (PELD) and model for end stage liver disease (MELD) scores [7]. Despite its use to date to predict mortality, there is growing evidence that the INR is not reliable for the purpose of predicting adverse haematological outcomes in the setting of paediatric liver disease [8–17]. The thrombin generation assay (TGA) is increasingly being used to assess the coagulation profile in the setting of chronic liver disease [6,13,18].
Short Bowel Syndrome
2020, Pediatric Gastrointestinal and Liver Disease, Sixth EditionVisceral transplantation in patients with intestinal-failure associated liver disease: Evolving indications, graft selection, and outcomes
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