Browsing by Subject "Short bowel syndrome"

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  • Hukkinen, Maria; Merras-Salmio, Laura; Pakarinen, Mikko P. (2018)
    Treatment results of pediatric intestinal failure have improved markedly during the last decades. With improved survival the attention is turning to other essential outcomes including quality of life and neurodevelopment. So far, relatively few studies with limited number of patients and variable methodology have addressed these issues. Based on these studies using generic health related quality of life tools, children with intestinal failure demonstrate decreased physical health, while PN-dependence is also associated with compromised emotional functioning. Impairments of social functioning are frequently observed among older children and parents. Few recent studies on neurodevelopment imply significant impairments in motor and mental skills among children with intestinal failure despite small sample sizes and limited follow-up times. Development of a disease-specific survey designed for the pediatric intestinal failure population could better reveal the health issues with greatest impact on quality of life. Robust studies with appropriate methodology on neurodevelopment in pediatric intestinal failure with extended follow-up times are urgently needed. Quality of life and neurodevelopment requires greater attention from medical professionals managing children with intestinal failure. (C) 2018 Elsevier Inc. All rights reserved.
  • Ylinen, Elisa; Merras-Salmio, Laura; Gunnar, Riikka; Jahnukainen, Timo; Pakarinen, Mikko P. (2018)
    Objective: Although impaired renal function has been a frequent finding among adults with intestinal failure (IF), the data on children is scarce. The aim of this study was to assess renal function in pediatric-onset IF. Methods: Medical records of 70 patients (38 boys) with pediatric-onset IF due to either short bowel syndrome (n = 59) or primary motility disorder (n = 11) and a history of parenteral nutrition (PN) dependency for >= 1 mo were evaluated. Renal function at the most recent follow-up was studied using plasma creatinine, cystatin C, and urea concentrations and estimated glomerular filtration rate (eGFR). Results: At a median age of 5.7 y and after PN duration of 3.2 y, 20 patients (29%) had decreased eGFR and higher cystatin C and urea concentrations. Patients with decreased renal function had significantly longer duration of PN (3.2 versus 0.9 y; P = 0.030) and shorter percentage of age adjusted small bowel length remaining (22 versus 32%; P = 0.041) compared with patients with preserved renal function. No other predisposing factors for decreased eGFR were identified. Conclusions: Patients with pediatric-onset IF are at significant risk for impaired renal function, which is associated with the duration of PN and the length of the remaining small bowel. In the present study, no other predisposing factors for decreased eGFR were found. Further studies using measured GFR are needed. (C) 2017 Elsevier Inc. All rights reserved.
  • Mutanen, Annika; Lohi, Jouko; Merras-Salmio, Laura; Koivusalo, Antti; Pakarinen, Mikko P. (2021)
    Background & Aims: Diagnostic criteria, progression risk and optimal monitoring for intestinal failure (IF)-associated liver disease (IFALD) remain undefined. We assessed predictors, noninvasive markers and progression of histopathological liver disease in patients with IF. Methods: In total, 77 children with IF and median age of 1.7 years underwent diagnostic liver biopsy, which was repeated in 48 patients after 2.9 years with simultaneous evaluation of liver biochemistry, liver stiffness, serum citrulline (a surrogate for viable enterocyte mass), spleen size, esophageal varices and clinical data. Patients were staged according to histopathological liver disease activity: active IFALD (cholestasis and/or inflammation), chronic IFALD (significant fibrosis and/or steatosis), or no IFALD (none of these features). Results: Diagnostic liver biopsy revealed active, chronic or no IFALD in 48%, 21% and 31% of patients. Active IFALD was segregated by low serum citrulline, parenteral nutrition (PN) dependency and young age, while weaning off PN and older age predicted chronic IFALD. Although the liver histopathology in most patients either normalized (52%) or transformed to a less reactive (chronic) disease stage (23%), 19% of patients retained and 6.3% progressed to an active cholestatic/inflammatory IFALD phenotype. Decreased serum citrulline and PN-dependency also predicted active IFALD in follow-up biopsies. Increased median liver biochemistry values and liver stiffness only associated with active IFALD, which was accurately identified by gammaglutamyltransferase (GGT), citrulline and liver stiffness, their combinations reaching diagnostic and follow-up AUROC values above 0.90. Conclusions: Active IFALD, essentially predicted by intestinal disruption and PN-dependency, was accurately detected by GGT, liver stiffness and citrulline, which together with recent advances in clinical management options, provides new avenues for monitoring and targeted liver protection in patients with IF. Lay summary: Liver disease is a common and critical complication in patients with intestinal failure, who require intravenous nutrition for survival due to severe intestinal dysfunction. We showed that both intravenous nutrition dependency and intestinal disruption essentially predicted development of active histopathological liver disease, which persisted in 25% of patients during long-term follow-up and could be accurately detected without the need for liver biopsy. Identification of the active and potentially progressive histopathology offers new possibilities for monitoring and targeted liver protection in patients with intestinal failure. (C) 2020 European Association for the Study of the Liver. Published by Elsevier B.V.
  • Hukkinen, Maria; Kivisaari, Reetta; Koivusalo, Antti; Pakarinen, Mikko P. (2017)
    Background: In remains unclear why in some short bowel syndrome (SBS) patients, the remaining small bowel (SB) dilates excessively leading to requirement of tapering surgery. Methods: Among SBS children, we retrospectively analyzed risk factors for tapering surgery with logistic regression and compared the outcome of operated patients (n = 16) to those managed conservatively (n = 44) with Cox proportional hazards regression. Results: SBS was caused by necrotizing enterocolitis (NEC) (n = 31), SB atresia (SBA) (n = 13), midgut volvulus (n = 12), or gastroschisis (n = 4). Patients with spontaneous symptomatic SB dilatation unable to wean parenteral nutrition (PN) underwent tapering surgery at median age of 1.04 (interquartile range 0.70-3.27) years. Missing ICV was related to an 8-fold (p = 0.003) increased risk while SBA diagnosis was related to a 13-fold risk of tapering surgery (p <0.001). Increasing SB length and NEC diagnosis were protective of tapering (p = 0.027-0.004). Of operated patients, 75% reached enteral autonomy during follow-up and their postoperative adjusted PN weaning rate was similar to nonoperated children (p = 0.842). Conclusion: SBS children with short remaining SB, missing ICV, and SBA etiology are more likely while NEC patients are less likely than others to necessitate tapering surgery. Postoperative PN weaning rates were comparable to patients who initially had more favorable intestinal anatomy and adapted without surgery. (C) 2017 Elsevier Inc. All rights reserved.
  • Mutanen, Annika; Pakarinen, Mikko P. (2017)
    Aim: Glukagon-like-peptide-1 (GLP-1) and -2 (GLP-2), produced by intestinal L-cells, are key hormones regulating intestinal transit, secretion, absorption, and mucosal growth. We evaluated nai ve fasting serum GLP-1 and GLP-2 levels in pediatric intestinal failure (IF). Methods: Fifty-five IF patients with median age 4.2 (IQR 1.3-12) years and 47 matched healthy controls underwent measurement of fasting serum GLP-1 and GLP-2. Results: Serum GLP-2 [19.9 (13.8-27.9) vs 11.6 (7.0-18.6) ng/mL, P <0.001], but not GLP-1 [6.1 (4.0-15.7) vs 6.4 (3.9-10.7) ng/mL, P = 0.976], levels were increased in IF patients. Serum GLP-2 concentrations were higher in patients with small bowel -colic continuity [21.1 (15.0-30.7) ng/mL] compared to patients with an endostomy [10.4 (6.6-17.9) ng/mL, P = 0.028], whereas no association with preservation of ileum or ileocecal valve was observed. During PN delivery, GLP-2 inversely associated with remaining small bowel length (r = -0.500, P = 0.041) and frequency of PN infusions (r = -0.549, P = 0.042). Serum GLP-1 levels were lower in patients receiving PN currently [4.1 (2.8-5.1)] compared to patients, who had weaned off PN [6.5 (5.1-21.1), P = 0.005],. and correlated positively with duration of PN (r = -0.763, P = 0.002) and negatively with percentage parenteral energy requirement (r = -0.716, P = 0.006). Conclusions: In pediatric IF, serum GLP-2 levels increase in patients with small bowel -colic continuity proportionally to the length of resected small intestine. Increase in serum GLP-1 and GLP-2 levels paralleled reducing requirement for parenteral support. These findings support regulation of intestinal adaption by GLP-2 and GLP-1 in children with IF. (C) 2016 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.