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      Bone and body composition analyzed by Dual-energy X-ray Absorptiometry (DXA) in clinical and nutritional evaluation of young patients with Cystic Fibrosis: a cross-sectional study

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          Abstract

          Background

          the improved general therapy has led to reduced morbidity and mortality from Cystic Fibrosis (CF), and bone status may have a potentially greater clinical impact.

          We investigated the correlation between the severity of the clinical condition, bone status and body composition parameters, in a group of children and young adults with CF.

          Methods

          we measured lumbar spine bone density and total body composition by dual energy x-ray absorptiometry (DXA) in 82 consecutive CF patients (42 males; median age: 13 years - range: 5-30). Eighty-two healthy subjects, matched for age, gender, height and pubertal stage were recruited as a control group.

          Results

          37 patients (45.1%) had a normal bone mineral density (BMD). A BMD reduction were observed in 45 (54.8%) patients. Lumbar spine Z score was positively related to Body Mass Index (BMI) and a higher Shwachman-Kulczycki score, and negatively related to Crispin-Norman score. A positive and significant correlation was also observed between lumbar spine Z score and total body composition.

          Conclusion

          a significant BMD reduction can be present early in CF children and adolescents. A careful follow up of bone status is required starting in childhood.

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          Most cited references34

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          Long-term effects of budesonide or nedocromil in children with asthma. The Childhood Asthma Management Program Research Group.

          Antiinflammatory therapies, such as inhaled corticosteroids or nedocromil, are recommended for children with asthma, although there is limited information on their long-term use. We randomly assigned 1041 children from 5 through 12 years of age with mild-to-moderate asthma to receive 200 microg of budesonide (311 children), 8 mg of nedocromil (312 children), or placebo (418 children) twice daily. We treated the participants for four to six years. All children used albuterol for asthma symptoms. There was no significant difference between either treatment and placebo in the primary outcome, the degree of change in the forced expiratory volume in one second (FEV1, expressed as a percentage of the predicted value) after the administration of a bronchodilator. As compared with the children assigned to placebo, the children assigned to receive budesonide had a significantly smaller decline in the ratio of FEV1 to forced vital capacity (FVC, expressed as a percentage) before the administration of a bronchodilator (decline in FEV1:FVC, 0.2 percent vs. 1.8 percent). The children given budesonide also had lower airway responsiveness to methacholine, fewer hospitalizations (2.5 vs. 4.4 per 100 person-years), fewer urgent visits to a caregiver (12 vs. 22 per 100 person-years), greater reduction in the need for albuterol for symptoms, fewer courses of prednisone, and a smaller percentage of days on which additional asthma medications were needed. As compared with placebo, nedocromil significantly reduced urgent care visits (16 vs. 22 per 100 person-years) and courses of prednisone. The mean increase in height in the budesonide group was 1.1 cm less than in the placebo group (22.7 vs. 23.8 cm, P=0.005); this difference was evident mostly within the first year. The height increase was similar in the nedocromil and placebo groups. In children with mild-to-moderate asthma, neither budesonide nor nedocromil is better than placebo in terms of lung function, but inhaled budesonide improves airway responsiveness and provides better control of asthma than placebo or nedocromil. The side effects of budesonide are limited to a small, transient reduction in growth velocity.
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            Osteoporosis in children and adolescents.

            In recent years, the issue of low bone density in children and adolescents has attracted much attention. The classical definition of osteoporosis should be valid at any age, yet its practical applicability to children and adolescents remains a matter of debate and there is no consensus on a diagnosis based solely on the BMD value. The clinical relevance of uncomplicated low bone density in the young and its long-term consequences remain difficult to evaluate and there is only preliminary evidence that the BMD value is a predictor of fracture risk in growing subjects. Moreover, the interpretation of densitometric data in the young is difficult because the "normal" BMD values to be used for comparison are continuously changing with age, and in addition, depend on several variables, such as gender, body size, pubertal stage, skeletal maturation and ethnicity. Although Z-score values below -2 are generally considered a serious warning, most bone specialists make a diagnosis of osteoporosis in children and adolescents only in the presence of low BMD and at least one fragility fracture. The scope of this review is limited to presenting a picture of the available knowledge. The literature on fractures will be presented in detail, since fractures are one of the key elements in the debate. There are countless papers on fractures in childhood and adolescence, but very few of them attempt to identify fragility fractures, and still fewer develop the concept of osteoporosis in the young in relation to fractures. The different forms of primary and secondary osteoporosis, the more technical aspects of bone densitometry in pediatrics, and the delicate issue of treatment will be discussed only briefly.
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              Liver disease in cystic fibrosis: A prospective study on incidence, risk factors, and outcome.

              Incidence of liver disease (LD) associated with cystic fibrosis (CF) and its clinical characterization still is unsettled. We have assessed prospectively the incidence and risk factors of this complication, and its impact on the clinical course of CF. Between 1980 and 1990, we enrolled 177 CF patients without LD in a systematic clinical, laboratory, ultrasonography screening program of at least a 10-year duration. During a 14-year median follow-up (2,432 patient-years), 48 patients developed LD, with cirrhosis already present in 5. Incidence rate (number of cases per 100 patient-years) was 1.8% (95% confidence interval: 1.3-2.4), with sharp decline after the age of 10 years and higher risk in patients with a history of meconium ileus (incidence rate ratio, 5.5; 2.7-11), male sex (2.5; 1.3-4.9), or severe mutations (2.4; 1.2-4.8) at multivariate analysis. Incidence of cirrhosis was 4.5% (2.3-7.8) during a median period of 5 years from diagnosis of liver disease. Among the 17 cirrhotic patients, 13 developed portal hypertension, 4 developed esophageal varices, 1 developed liver decompensation requiring liver transplantation. Development of LD did not condition different mortality (death rate ratio, 0.4; 0.1-1.5) or higher incidence of other clinically relevant outcomes. In conclusion, LD is a relatively frequent and early complication of CF, whose detection should be focused at the first life decade in patients with history of meconium ileus, male sex, or severe genotype. Although LD does not condition a different clinical course of CF, in some patients it may progress rapidly and require liver transplantation.
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                Author and article information

                Journal
                BMC Pediatr
                BMC Pediatrics
                BioMed Central
                1471-2431
                2009
                28 September 2009
                : 9
                : 61
                Affiliations
                [1 ]Unit of Cystic Fibrosis, Department of Pediatric Medicine - Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
                [2 ]Unit of Endocrinology, Department of Pediatric Medicine - Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
                Article
                1471-2431-9-61
                10.1186/1471-2431-9-61
                2760551
                19785733
                642d6c0f-0ac1-438e-8b8e-5bb6257a84f5
                Copyright © 2009 Lucidi et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 28 May 2009
                : 28 September 2009
                Categories
                Research Article

                Pediatrics
                Pediatrics

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