METABOLIC DISORDERS OF BONE TISSUE IN CHILDREN WITH CYSTIC FIBROSIS.

J. Konstantynowicz. A. Minarowska¹, M. Kaczmarski¹

Auxology Department and 3^rd Department Of Children's Diseases of Medical University, Bialystok, Poland

OBJECTW: Cystic fibrosis (CF) is a chronic, genetically determined disease which impairs physical development and nutrition. Due to its multisystemic character and a long therapy CF causes a disorder in bone tissue metabolism including bone mass loss by means of various mechanisms.

METHODS: In the study bone mass evaluation was performed in 8 children (5M, 3F) aged 5-19 years with Cystic fibrosis treated in the 3^rd Department of Children's Diseases. Anthropometry and radiologic evaluation of bone age and nutrition conditions were carried out. Densitometry was performed by DEXA method using DPX-L (Lunar system). Total bone mass (TOTAL BMD) density was determined.

RESULTS: Delayed bone age was found in 4 of 8 patiens. The body mass in 4 patiens ranged below 3^rd centile. Cole's coefficient was than 85%, indicating moderate malnutrition. In the total bone mass (TOTAL) density measurments decreased values of BMD were found in all standard (< 5^th centil basing on controls), indicating low bone mass.

CONCLUSIONS: Taking into consideration the risk of osteoporosis in patients with CF it is suggested to monitor their bone mass by DEXA method. Every year - or every other years - densitometry (at least at two locations of the bone system) should be included in the complete evaluation of the disease course.