Pediatric Intestinal Failure: The Key Outcomes for the First 100 Patients Treated in a National Tertiary Referral Center During 1984–2017

Merras‐Salmio, Laura; Mutanen, Annika; Ylinen, Elisa; Rintala, Risto J.; Koivusalo, Antti; Pakarinen, Mikko P.

doi:10.1002/jpen.1164

Cited by 39 publications

(48 citation statements)

References 41 publications

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“…We defined IF as >50% resection of the small bowel and/or duration of PN over 60 days (17). Since 2009, a standardized multidisciplinary IF management and follow-up program including modern fish oil containing PN lipid emulsions, autologous reconstructive surgical J o u r n a l P r e -p r o o f options and intestinal transplantation has been running in our center as described in detail previously (17,24) (Supplementary methods). Diagnostic liver biopsies are routinely obtained, while follow-up biopsies were performed to patients considered to be in the greatest risk for progression of liver injury (6,17).…”

Section: Patients and Study Designmentioning

confidence: 99%

“…While cholestasis occurs in as many as over two thirds of PN-dependent newborns (9), 20-30% of children with IF requiring long-term PN will develop clinically evident IFALD (9,(15)(16)(17), and 60% of patients display advanced liver fibrosis when evaluated for transplantation (18). As outlined in a recent review, histopathology of the acute IFALD phase is predominated by cholestasis and inflammation, which are largely replaced by fibrosis and steatosis in the later chronic phase of the disease (8).…”

Section: Introductionmentioning

confidence: 99%

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Prediction, identification and progression of histopathological liver disease activity in children with intestinal failure

Mutanen

Merras‐Salmio

Koivusalo

et al. 2021

Journal of Hepatology

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Section: Patients and Study Designmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Prediction, identification and progression of histopathological liver disease activity in children with intestinal failure

Mutanen

Merras‐Salmio

Koivusalo

et al. 2021

Journal of Hepatology

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“…Parenteral nutrition (PN) is required to ensure adequate nutrition, growth, and survival . Although most children with SBS are able to gradually wean off PN because of intestinal adaptation within several years after bowel resection, a significant proportion of patients remains PN‐dependent for years or permanently …”

Section: Introductionmentioning

confidence: 99%

Parenteral Nutrition–Dependent Children With Short‐Bowel Syndrome Lack Duodenal‐Adaptive Hyperplasia but Show Molecular Signs of Altered Mucosal Function

Sanaksenaho

Mutanen

Godbole

et al. 2020

J Parenter Enteral Nutr

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Background Although adaptive mucosal growth of the remaining small intestine is an essential compensatory mechanism to bowel resection in experimental short‐bowel syndrome (SBS), only scarce clinical data are available. We studied structural and molecular mechanisms of intestinal adaptation in children with SBS. Methods : Fourteen patients, who had been dependent on parenteral nutrition (PN) since neonatal period for a median (interquartile range)1.4 (0.7–6.5) years, were studied at the age of 1.5 (1.0–6.5) years. Median length of remaining small bowel was 33 (12–60) cm, and 6 patients had their ileocecal valve preserved. Six children without gastrointestinal disorders served as age‐matched and gender‐matched controls. All patients underwent duodenal biopsies. Mucosal microarchitecture, proliferation, apoptosis, inflammation, and epithelial‐barrier function were addressed using histology, immunohistochemistry, and quantitative real‐time polymerase chain reaction. Results : Villus height, crypt depth, enterocyte proliferation, and apoptosis were similar in patients and matched controls. Messenger RNA (mRNA) expression of numerous genes regulating gut epithelial‐barrier function (TGFB2, CAV1, CLDN1, MUC2, and NLRC4) was significantly altered. Of various nutrient transporters studied, only expression of SLC2A1 encoding facilitative glucose transporter GLUT1 was increased among patients, whereas RNA expression of genes encoding sodium‐dependent glucose, sterol, fatty‐acid, and peptide transport remained unchanged. Conclusion : Duodenal mucosal hyperplasia has a limited role in mediating physiological adaptation following intestinal resection among PN‐dependent children with SBS. Further clinical studies addressing functional significance of the observed alterations in mucosal RNA expression are warranted.

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“…tions of NIs, has been commonly reported in hospitalized pediatric patients with IF and in adults requiring longterm home PN (HPN). [12][13][14][15][16][17] However, the prevalence and associated risk factors for NIs in hospitalized adult IF patients have never been reported.…”

Section: Introductionmentioning

confidence: 99%

Prolonged Parenteral Nutrition Is One of the Most Significant Risk Factors for Nosocomial Infections in Adult Patients With Intestinal Failure

et al. 2020

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Background Intestinal failure (IF) and its management are associated with an increased likelihood of infectious complications. This study aimed to evaluate the prevalence and potential risk factors for nosocomial infections (NIs) in hospitalized adult patients with IF. Methods In total, 259 eligible patients with IF admitted to a single clinical nutrition center in a tertiary referral hospital from January 1, 2012, to January 1, 2019, were retrospectively identified. NIs were defined according to the 2008 Centers for Disease Control and Prevention criteria. Univariate and multivariate analyses were performed to identify independent risk factors for NIs. Results The mean age of the study population was 47.0 ± 17.7 years, and 158 (61.0%) were men. The mean body mass index was 16.2 ± 2.9 kg/m2, and 219 (84.6%) were diagnosed with malnutrition. The prevalence of NIs was 25.5% (113 NIs in 66 patients). The most common NIs were pneumonia (14.3%), bacteremia of unknown origin (13.5%), catheter‐related bloodstream infection (5.0%), lower respiratory tract infection (5.0%), surgical site infection (3.9%), and urinary tract infection (1.9%). Multivariate analysis revealed that decreased serum albumin level (odds ratio [OR], 0.884; 95% CI, 0.883–0.978, P < .05), presence of gallbladder stones or cholestasis (OR, 3.144; 95% CI, 1.044–9.464; P < .05), and prolonged parenteral nutrition (PN) use (OR, 1.072; 95% CI, 1.039–1.105; P < .001) were independent predictors for NIs. Conclusions NIs remain prevalent in hospitalized adult patients with IF. Prolonged PN use was one of the most significant predictors for NIs.

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Pediatric Intestinal Failure: The Key Outcomes for the First 100 Patients Treated in a National Tertiary Referral Center During 1984–2017

Abstract: Despite reassuring rates of survival and weaning off PN, long-term PN failed in 14% of patients solely because of catheter complications in the recent era. Achievement of enteral autonomy in those with the shortest remaining small bowel and functional cause of IF remains challenging.

Cited by 39 publications

References 41 publications

Prediction, identification and progression of histopathological liver disease activity in children with intestinal failure

Prediction, identification and progression of histopathological liver disease activity in children with intestinal failure

Parenteral Nutrition–Dependent Children With Short‐Bowel Syndrome Lack Duodenal‐Adaptive Hyperplasia but Show Molecular Signs of Altered Mucosal Function

Prolonged Parenteral Nutrition Is One of the Most Significant Risk Factors for Nosocomial Infections in Adult Patients With Intestinal Failure

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