Browsing by Subject "GASTROSCHISIS"

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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.
  • 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.
  • Hukkinen, Maria; Kivisaari, Reetta; Merras-Salmio, Laura; Koivusalo, Antti; Pakarinen, Mikko P. (2017)
    Objective: To analyze risk factors and prognostic significance of small bowel (SB) dilatation in children with short bowel syndrome (SBS). Background: In SBS, the remaining SB may dilate as part of intestinal adaptation. The impact of dilatation on parenteral nutrition (PN) dependence and survival has not been studied systematically. Methods: SB diameter of SBS children (n = 61) was measured in contrast SB series (n = 169, median age 0.94, range 0.32-2.7 years) during 2002 to 2015, and expressed as millimeters (SB width) and as ratio to L5 vertebra height (SB diameter ratio). Linear regression was used to examine risk factors for dilatation. PN weaning and survival were analyzed with Cox proportional hazards regression. Results: Maximal SB diameter ratio during follow-up was predicted by PN dependence and SB atresia, while maximal absolute SB width by birth weight, age, PN duration, and remaining bowel length. Weaning off PN was 14-fold more likely in patients with maximal SB diameter ratio <2.00 compared with > 3.00 (P = 0.005), and 5.4-fold more likely when maximal SB width was <20mm compared with > 30mm (P = 0.023). After adjustment for age, remaining SB length, and the presence of ileocecal valve, both estimates of maximal SB dilatation remained significant independent predictors for weaning off PN. When all measurements were included, the cumulative survival was worse if SB diameter ratio exceeded 2.00 (P = 0.002-0.042). Conclusions: SB dilatation predicts prolonged PN duration and decreased survival in SBS children. Measurement of maximal SB diameter standardized to L5 vertebra height may be a valuable objective tool for patient follow-up and assessment of prognosis.