BMD and body composition in children and young patients affected by cystic fibrosis.

UNLABELLED: Longer survival in cystic fibrosis has led to more bone complications. One hundred thirty-six young patients were studied for 12-24 months. Low BMD was found in 66%. Fat mass and lean mass were also reduced. Impaired pulmonary function and total steroid dose had the greatest negative influence on bone. INTRODUCTION: Low BMD is reported as a frequent complication in adult patients affected by cystic fibrosis (CF), but the available data are less consistent for younger patients. MATERIALS AND METHODS: This study was designed to evaluate BMD longitudinally over 12-24 months in a sample of 136 young patients (3-24 years of age) and to investigate its major determinants. BMC and body composition were also evaluated. RESULTS: BMD (expressed as Z score) of spine and of total body was reduced in 66% of patients. The prevalence of low BMD was the same in children, adolescents, and young adults. The main determinants of BMD were forced expiratory volume in 1 s (FEV1; as an index of pulmonary function), puberty, platelet count (as an index of portal hypertension), and cumulative steroid dose. Changes of FEV1 over time influenced BMD changes. Bone mass, fat mass (FM) and fat-free (lean) mass (FFM) were reduced in CF patients at both total body and subregions (trunk, limbs). Lean mass influenced BMD of total body and lower limbs, whereas fat mass (and BMI) influenced spine BMD. FEV1 also influenced FFM. CONCLUSIONS: Low BMD was present in a significant proportion of CF patients, independent of sex and age. BMD depended on pulmonary function, steroid dose, and presence of advanced liver disease. Pulmonary function and puberty were the main stimuli for the increase of BMD over time. CF also altered body composition, and FFM was influenced by pulmonary function.

Quality of life in post-menopausal osteoporosis.

BACKGROUND: To evaluate the impact of osteoporosis on the patients' quality of life, particularly in the absence of fractures. METHODS: 100 post-menopausal women (age 50-85)--62 with uncomplicated primary osteoporosis and 38 with primary osteoporosis complicated by vertebral fractures; all already treated--were studied using two validated questionnaires: Qualeffo-41 for quality of life in osteoporosis, and Zung for depression. Data were compared to those of 35 controls of comparable age, affected by a different chronic disease (hypothyroidism). RESULTS: Family history of osteoporosis and T-score of spine were similar in the two subgroups of osteoporotic women. Body mass index, age at menopause and education level were similar in the two subgroups of osteoporotic women and in the control group. The patients affected by osteoporosis perceived it as a disease affecting their personal life with undesirable consequences: chronic pain (66% of women with fractures and 40% of women without fractures), impaired physical ability, reduced social activity, poor well-being (21% of women without fractures) and depressed mood (42% of women irrespective of fractures). Overall, 41% of the women showed a reduced quality of life. On the contrary, in the control group only 11% reported a reduced quality of life. CONCLUSION: The quality of life of osteoporotic patients should be investigated even before fractures, in order to develop appropriate counselling, support and care interventions to help patients develop efficient strategies for accepting the disease and coping with it.

Imbalance of osteoclastogenesis-regulating factors in patients with celiac disease.

UNLABELLED: Celiac disease is an autoimmune disorder characterized by atrophy of the intestine villi triggered by ingestion of gluten in genetically susceptible individuals. The association between celiac disease and low BMD has been recognized, but the mechanisms of disturbance are poorly understood. We show imbalance of cytokines relevant to bone metabolism in celiac patients' sera and the direct effect of these sera on in vitro bone cell activity. INTRODUCTION: Celiac disease is associated with mineral metabolism derangement and low BMD. We investigated whether imbalance of serum factors in celiac patients could affect human bone cell activity in vitro. MATERIALS AND METHODS: We studied two groups of celiac patients--one on a gluten-free diet and another before the diet--both with decreased bone mass. Patients were investigated for bone turnover markers, and their sera were used for culturing bone cells from healthy donors and evaluate changes in cell activity. RESULTS: The N-terminal telopeptide of procollagen type I and interleukin (IL)-6 were higher than normal in patients not on the gluten-free diet. IL-1beta and TNF-alpha/beta were normal in all patients. IL-12 was reduced in all patients, whereas IL-18 was reduced only in patients on the diet. The RANKL/osteoprotegerin (OPG) ratio was increased in patients not on the gluten-free diet. Persistently increased osteoclast numbers were obtained from peripheral blood mononuclear cells of healthy donors on incubation with sera of patients not on the gluten-free diet versus control sera and sera from patients on the diet. In human osteoblasts from healthy individuals, IL-18 was reduced on incubation with sera from all patients, whereas OPG expression was lower when sera from patients not on the diet were used. Proliferation, alkaline phosphatase, and nodule mineralization were increased in osteoblast cultures containing sera from all celiac patients, either on or not on the gluten-free diet. CONCLUSIONS: We conclude that bone loss in celiac disease might also be caused by a cytokine imbalance directly affecting osteoclastogenesis and osteoblast activity.