Cystic fibrosis
Conditions
Interventions
The interventions consist of following a test diet, the use of PERT (own capsules, test dose), the collection of feces that is marked by the intake of color capsules and keeping a nutritional diary. T
Sponsors
Erasmus Medical Center -Sophia Children's Hospital Rotterdam
Eligibility
Inclusion criteria
Inclusion criteria: 1. Diagnosis of CF as evidenced by one or more clinical feature consistent with the CF phenotype or positive CF newborn screen AND one or more of the following criteria: a) A documented sweat chloride ¡Ý 60 mEq/L by quantitative pilocarpine iontophoresis (QPIT) b) A documented genotype with two disease-causing mutations in the CFTR gene 2. Informed consent by parent or legal guardian; assent for children from age 12 years on 3. Having pancreatic insufficiency (stool elastase < 200 mcg/g stool) and using PERT 4. Age ¡Ý 12 months and < 18 years at Screening visit 5. Stable clinical status at least two weeks before signing the informed consent. 6. Patients¡¯ capacity and willingness to fulfill the meal test and the faeces collection during the weekend
Exclusion criteria
Exclusion criteria: 1. Acute infection associated with decreased appetite or fever at time of run-in visit 2. Acute abdominal pain necessitating an intervention at time of run-in visit 3. Severe cholestasis (direct bilirubin increase above 2 mg/dL with respect to the normal limit for age). 4. FEV1 <40% for age, gender, weight and height. 5. Severe hypoalbuminemia (albumin in blood <2.5g/mL). 6. Hospitalisation or intravenous antibiotics <2 weeks before signing the informed consent. 7. Changes in the usual treatment (prokinetics, antiacids, H2 blockers and antibiotics) < 2 weeks before signing the informed consent. 8. Presence of alterations that, according to the investigator consideration, could jeopardise the safety of the patient. 9. Hypersensibility or adverse reactions to the enzymatic supplements.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The main study parameter is to assess, how much the final fat in stools deviates from the normal concentration (6g/24h) after applying the individual correction factor (ICF). | — |
Contacts
Public ContactJM Hulst
Erasmus MC - Sophia Children's Hospital
Outcome results
None listed