Management of Exocrine Pancreatic Insufficiency in Children

Sankararaman, Senthilkumar; Schindler, Teresa; Sferra, Thomas J.

doi:10.1002/ncp.10388

Cited by 26 publications

(36 citation statements)

References 112 publications

(234 reference statements)

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“…High doses of PERT can cause inflammation and fibrosing colonopathy. 18 Inspissated intestinal secretions, mucus accumulation, constipation, slow intestinal motility, the use of proton pump inhibitors and frequent courses of antibiotics are multiple risk factors for small bowel bacterial overgrowth in PwCF, which can cause inflammation, mucosal damage and aggravate maldigestion. 16 An unfavourable intestinal microbiome may also be a stimulus for inflammation.…”

Section: Discussionmentioning

confidence: 99%

Faecal calprotectin and rectal histological inflammatory markers in cystic fibrosis: a single-centre study

Roda

Maia

Almeida

et al. 2022

bmjpo

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ObjectiveTo analyse the association of faecal calprotectin with the genetic and clinical characteristics of paediatric patients with cystic fibrosis (PwCF). In a subset of these patients, we aimed to associate histological inflammatory features of rectal mucosa to faecal calprotectin levels.MethodsIn a prospective study, faecal calprotectin levels were collected in all 23 PwCF attending our paediatric centre, together with demographic and clinical data. Associations between faecal calprotectin and clinical features were determined. In 11 of these patients, endoscopic rectal biopsies were obtained and the association between faecal calprotectin and histological inflammatory markers was analysed. Statistical analyses included Spearman’s correlation coefficient, Mann-Whitney U test and Fisher’s exact test. Sensitivity and specificity was calculated.ResultsMedian age of PwCF was 12 years, 19 had pancreatic insufficiency (PI) (19/23). Seventeen (17/23) had elevated faecal calprotectin, and the median value was 88 µg/g (IQR=178 µg/g). Higher faecal calprotectin levels were observed in the PI group (101 vs 30 µg/g, p=0.027). No significant correlation between elevated faecal calprotectin level and body mass index z-score was found. Five patients (22%) reported abdominal pain, three (13%) complained of diarrhoea and three (13%) had constipation, but these symptoms were not associated with elevated faecal calprotectin.Unspecific focal rectal inflammation was found in four patients (4/11). An association between rectal mucosa inflammation and elevated faecal calprotectin was found (p=0.015). Sensitivity was 100% and specificity was 86%.ConclusionsIn our PwCF, elevated faecal calprotectin was frequent, particularly if PI, and it was not related to gastrointestinal symptoms or malnutrition. Elevated faecal calprotectin was present in patients with histological evidence of rectal inflammation. Faecal calprotectin may be an indicator of asymptomatic rectal inflammation in PwCF.

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Section: Discussionmentioning

confidence: 99%

Faecal calprotectin and rectal histological inflammatory markers in cystic fibrosis: a single-centre study

Roda

Maia

Almeida

et al. 2022

bmjpo

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“…The poor overall outcomes may be due to insufficient PERT dosages and/or poor recovery of pancreatic function. More specifically, in patients with persistent symptoms and signs of exocrine pancreatic insufficiency (EPI), patients prescribed appropriate dosages of PERT may still fail to respond to PERT treatment due to missed doses, improper storage of enzymes, expired enzymes, mistiming of PERT administration (taking PERT after meals), destruction of enteric coating and comorbidities (non-EPI-related conditions) [30]. Therefore, we advocate for inclusion of appropriate pancreatic enzyme replacement therapy within long-term treatment plans for these patients.…”

Section: Discussionmentioning

confidence: 99%

Prognosis and treatment of 46 Chinese pediatric cystic fibrosis patients

Chen

Shen

et al. 2021

BMC Pediatr

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Background Since public awareness of cystic fibrosis (CF) has increased, more children have been diagnosed with CF in China. This study aimed to investigate medical and other challenges faced by pediatric CF patients in China. Method Treatments and treatment outcomes were retrospectively analyzed for 46 pediatric CF patients diagnosed from August 2009 to June 2019. Pre- and post-treatment results were compared using independent samples t-test. Results Of 46 pediatric CF study patients, four died and five were lost to follow-up. Thirty-seven patients were monitored for 0.03 to 9.21 years; patients exhibited fewer attacks of respiratory tract infections after diagnosis (4.49 ± 2.13 episodes/year before diagnosis vs 1.97 ± 1.87 times/year after 1-year treatment, p < 0.05), significantly reduced sputum production and experienced 1.62 ± 1.71 exacerbations/year. Patient mean body mass index was 16.87 ± 3.53 and pancreatic malfunction persisted in 15 patients. For 17 children, no significant differences in lung function were found at follow-up as compared to lung function at diagnosis (FEV1: 82.45% ± 16.56% vs 75.26% ± 22.34%, FVC: 87.18% ± 13.64% vs 86.99% ± 19.95%, FEF75%: 46.51% ± 28.78% vs 36.63% ± 24.30%, P = 0.27, 0.97, 0.20, respectively). Pseudomonas aeruginosa (17/27) and bronchiectasis (22/22) were found during follow-up evaluation. Twenty-four patients (64.8%) maintained good adherence to therapies. Overall, azithromycin and tobramycin treatments were administered for 0.5–62 months and 0.5–48 months, respectively, and triggered no obvious adverse reactions. Conclusion No obvious declines in clinical presentation or lung function were found in Chinese pediatric CF patients after receiving standard therapeutic and active treatments, although malnutrition and low compliance were persistent challenges.

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“…El paciente con insuficiencia pancreática debe iniciar terapia de reemplazo enzimático, la dosis no debe sobrepasar las 10.000 unidades de lipasa por kg/día (ver capítulo nutrición). el objetivo del tratamiento es aliviar los síntomas, mejorar la digestión y promover un adecuado crecimiento y desarrollo 129 . la pancreatitis aguda se caracteriza por un dolor intenso epi o meso gástrico, a veces transfixiante e irradiado al dorso, asociado a vómitos, náuseas y compromiso del estado general con o sin alza febril.…”

Section: Páncreas 129unclassified

Consenso chileno para la atención integral de niños y adultos con fibrosis quística

Boza

Melo

Barja

et al. 2020

Rev. chil. enferm. respir.

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Chilean consensus for the integral care of children and adults with cystic fibrosisCystic Fibrosis (CF) is the most frequent hereditary disease in whites, with a reserved prognosis. Since 2003, Chile began a comprehensive National Cystic Fibrosis Program, directed by the Respiratory Health Unit of the Ministry of Health. To date, the main results of the Program record a significantly longer survival (average 27 years) and a significant reduction in the age of diagnosis of patients admitted from 2006 onwards. Access to Chilean Explicit Health Guarantees, the implementation of neonatal screening in some regions of the country, the organization and setting up of CF-trained teams of various specialties, has contributed to improving results. Although the main manifestations are of the respiratory and digestive system, the multisystemic nature of CF makes it necessary to know the different aspects involved in its management, in order to optimize the results of the treatment and the resources invested, both in the public and private sectors. This document is a review and an update on the main aspects of the diagnosis, monitoring and treatment of the respiratory and non-respiratory manifestations of CF.

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Management of Exocrine Pancreatic Insufficiency in Children

Cited by 26 publications

References 112 publications

Faecal calprotectin and rectal histological inflammatory markers in cystic fibrosis: a single-centre study

Faecal calprotectin and rectal histological inflammatory markers in cystic fibrosis: a single-centre study

Prognosis and treatment of 46 Chinese pediatric cystic fibrosis patients

Consenso chileno para la atención integral de niños y adultos con fibrosis quística

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