Effects of creatine supplementation in cystic fibrosis: results of a pilot study.
BACKGROUND
The CF transmembrane conductance regulator (CFTR), whose mutations cause cystic fibrosis (CF), depends on ATP for activation and transport function. Availability of ATP in the cell and even more in specific cellular microcompartments often depends on a functional creatine kinase system, which provides the 'energy buffer' phosphocreatine. Creatine supplementation has been shown to increase phosphocreatine levels, thus promoting muscle growth and strength in athletes and having protective effects in neuromuscular disorders.


OBJECTIVE
To test clinically, if creatine supplementation improves maximal isometric muscle strength (MIMS), lung function and CFTR channel activity in patients with CF, and to determine enzymatic activity of creatine kinase in respiratory epithelial cells.


METHODS
In an open-label pilot study 18 CF patients (8-18-year-old) with pancreatic insufficiency and mild to moderate lung disease received daily creatine supplementation during 12 weeks. Patients were monitored during 24-36 weeks. Enzymatic activity of creatine kinase was measured in primary epithelial cell cultures.


RESULTS
After creatine supplementation, there was no change in lung function and sweat electrolyte concentrations, possibly due to the very low creatine kinase activities detected in respiratory epithelia. However, the patients consistently showed significantly increased MIMS (18.4%; P < 0.0001), as well as improved general well-being, as assessed by a standardized questionnaire. Except for one patient with transient muscle pain, no side effects were reported.


CONCLUSIONS
Our pilot study suggests, that creatine supplementation should be further evaluated as a possible clinically beneficial adjuvant therapy for patients with CF to increase muscle strength, body-weight and well-being.
Braegger
Christian P
CP
Divisions of Gastroenterology and Nutrition and Clinical Chemistry and Biochemistry, University Children's Hospital, Steinwiesstrasse 75, Zürich CH-8032, Switzerland. christian.braegger@kispi.unizh.ch
Schlattner
Uwe
U
Wallimann
Theo
T
Utiger
Anna
A
Frank
Friederike
F
Schaefer
Beat
B
Heizmann
Claus W
CW
Sennhauser
Felix H
FH
D000293:Adolescent; D002648:Child; D003401:Creatine / Q000627:therapeutic use*; D003550:Cystic Fibrosis / Q000150:complications* / Q000175:diagnosis; D019587:Dietary Supplements; D010188:Exocrine Pancreatic Insufficiency / Q000150:complications* / Q000175:diagnosis / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D010186:Pancreatic Function Tests; D010865:Pilot Projects; D011336:Probability; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome
D003401:Creatine