Rubinstein et al. (1986)1414 Rubinstein S, Moss R, Lewiston N. Constipation and meconium ileus equivalent in patients with cystic fibrosis. Pediatrics. 1986;78:473-9.
|
Retrospective study |
Determine the prevalence of constipation and meconium ileus |
Children's Hospital, Stanford, United States |
CF patients undergoing treatment |
0 to >30 years |
Clinical judgment |
168/54 |
32% |
Baker et al. (2005)1616 Baker SS, Borowitz D, Duffy L, Fitzpatrick L, Gyamfi J, Baker RD. Pancreatic enzyme therapy and clinical outcomes in patients with cystic fibrosis. J Pediatr. 2005;146:189-93.
|
Multicentric prospective study |
Evaluate the relationship between pancreatic enzymatic therapy and the clinical outcomes: growth, abdominal pain, constipation, flatulence, and bowel movements in patients with CF |
33 places credentialed by the Cystic Fibrosis Foundation |
CF patients |
>4 weeks to >14 years |
Patient's report |
1074/616 |
57% |
van der Doef et al. (2010)1515 van der Doef HP, Kokke FT, Beek FJ, Woestenenk JW, Froeling SP, Houwen RH. Constipation in pediatric cystic fibrosis patients: an underestimated medical condition. J Cyst Fibros. 2010;9:59-63.
|
Multicentric retrospective study |
Determine prevalence, risk factors, and treatment in constipated patients with CF, as well as the diagnostic value of abdominal radiography |
University Medical Center Utrecht, Holland |
Pediatric patients undergoing treatment |
≤18 years |
ESPGHAN, 2010 |
230/107 |
47% |
Ooi et al. (2016)1717 Ooi CY, Jeyaruban C, Lau J, Katz T, Matson A, Bell S, et al. High ambient temperature and risk of intestinal obstruction in cystic fibrosis. J Paediatr Child Health. 2016;52:430-5.
|
Retrospective study |
Determine if the prevalence of DIOS and constipation could be related to room temperature and volume of fluvial precipitation |
Sydney Children's Hospital, Australia |
Pediatric patients being treated at admission (outpatient not included) |
≤18 years |
ESPGHAN, 2010 |
241/30 |
13% |
Fraquelli et al. (2016)1818 Fraquelli M, Baccarin A, Corti F, Conti CB, Russo MC, Valle SD, et al. Bowel ultrasound imaging in patients with cystic fibrosis: relationship with clinical symptoms and CFTR genotype. Digest Liver Dis. 2016;48:271-6.
|
Prospective study |
Evaluate the frequency of ultrasound signals in the intestines of patients with CF regardless the presence of gastrointestinal symptoms and the co-relation of these signs with clinical symptoms and CFTR genotype |
Reference center of the Ospedale Maggiore Policlinico, Milan, Italy |
Outpatients |
10 to 22 years |
Patient's report |
70/11 |
16% |
Munck et al. (2016)1919 Munck A, Alberti C, Colombo C, Kashirskaya N, Ellemunter H, Fotoulaki M, et al. International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: associated factors and outcome. J Cyst Fibros. 2016;15:531-9.
|
Prospective longitudinal observational study |
Evaluate DIOS clinical manifestations (complete and incomplete) |
27 University Hospitals in 10 countries (9 European countries and Israel) |
Adult and pediatric patients admitted for DIOS |
6 to 24 years |
Patient's report |
109/44 |
40% |
Santos et al. (2018)2020 Santos AL, Santos HM, Nogueira MB, Távora HT, Cunha ML, Seixas RB, et al. Cystic fibrosis: clinical phenotypes in children and adolescents. Pediatr Gastroenterol Hepatol Nutr. 2018;21:306-14.
|
Analytical transversal |
Describe the clinical phenotype of children and adolescents with CF and evaluate the role of pancreatic insufficiency and neonatal screening in the diagnosis |
Pediatric Reference Center, Brasília, Brazil |
Pediatric patients undergoing treatment |
≤18 years |
Patient's report |
77/26 |
34% |
Mentessidou et al. (2018)2121 Mentessidou A, Loukou I, Kampouroglou G, Livani A, Georgopoulos I, Mirilas P. Long-term intestinal obstruction sequelae and growth in children with cystic fibrosis operated for meconium ileus: expectancies and surprises. J Pediatr Surg. 2018;53:1504-8.
|
Retrospective comparative study |
Investigated the incidence of long-term obstruction sequel (constipation, DIOS and growth) in patients with CF who have undergone surgery for meconium ileus in the neonatal period |
Aghia Sophia Children's Hospital, Greece |
CF patients operated for meconium ileus |
0 to 26 years |
Episodes of chronic gradual fecal impaction of the colon (according to CFF) |
49/5 |
10% |