Endogenous fecal losses of calcium compromise calcium balance in pancreatic-insufficient girls with cystic fibrosis (original) (raw)
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The American journal of clinical nutrition, 2003
Reduced bone mass is common in both children and adults with cystic fibrosis (CF) and may be a consequence of inadequate calcium absorption. The effect of CF on intestinal calcium absorption and retention has not been described in children. Calcium absorption and urinary losses were characterized in clinically stable girls with CF consuming self-selected diets and following usual pancreatic enzyme regimens. The percentage of calcium absorption was assessed in 23 girls (aged 7-18 y) with CF by using oral ((44)Ca) and intravenous ((42)Ca) stable isotopes. Girls were grouped according to Tanner stage of breast development. True calcium absorption (V(a)) was determined as the product of percentage calcium absorption and average 4-d daily calcium intake. Calcium balance was estimated by subtracting urinary calcium and estimated endogenous fecal losses from the measure of V(a). Analysis of variance was used to compare outcomes among pubertal groups, and regression analysis was used to des...
Calcium kinetics are altered in clinically stable girls with cystic fibrosis
The Journal of clinical endocrinology and metabolism, 2004
Reduced bone mass in individuals with cystic fibrosis (CF) may result from alterations in calcium metabolism. Bone calcium deposition and resorption rates, calcium balance, and markers of bone turnover were assessed using stable isotopes of calcium in 22 prepubertal and pubertal girls with CF. Bone calcium deposition was associated with the availability of dietary calcium, total serum osteocalcin, and leptin concentrations. Reduced bone mass in individuals with CF may result from inadequate bone calcium (Ca) deposition, and excessive resorption, although these parameters have not been directly assessed in children with CF. We used stable Ca isotopes to measure rates of bone Ca deposition (Vo+), resorption, and retention in 22 clinically stable girls with CF (aged 7-18 yr). Rates of bone Ca deposition were determined by mathematically modeling the disappearance of iv Ca stable isotope ((42)Ca) for 6 d post dosing. Indirect markers of bone turnover and hormones associated with puberta...
Altered Calcium Homeostasis in Adults with Cystic Fibrosis
Osteoporosis International, 1999
Bone mineral density (BMD) in cystic fibrosis (CF) patients falls progressively below normal with advancing age, in part due to steroid administration, low levels of sex hormones, chronic inflammatory disease, physical inactivity, and chronic malabsorption of calcium and/or vitamin D. The purpose of this study was to compare the fractional absorption of 45 Ca and urinary excretion of calcium in CF subjects and normal controls following a high-calcium breakfast containing 45 Ca. Seven young men and 5 young women with CF with pancreatic insufficiency were studied on two separate occasions, with and without administration of pancreatic enzymes. Eleven healthy young adults with normal BMD measurements served as controls. Mean Tscores at the lumbar spine and femur were significantly lower in the CF subjects (p50.002). Following baseline, fasting collections, timed serum and urine samples were obtained for 5 h after the meal. Fractional absorption (FA) of 45 Ca was estimated by the method of Marshall and Nordin. At baseline, CF subjects had lower mean serum 25-hydroxyvitamin D, calcium and albumin values (p50.03 for each), slightly, but not significantly (p = 0.12), lower albumin-corrected calcium values, equivalent serum 1,25-dihydroxyvitamin D values and a trend toward a higher mean serum parathyroid hormone (PTH) value (p = 0.10). Without pancreatic enzymes, CF subjects showed significantly impaired calcium absorption (5 h FA: 11.8 ± 0.5 for controls vs 8.9 ± 0.2 for CF subjects, p = 0.02) and excretion (4 h excretion: 0.20 ± 0.08 mg Ca/mg creatinine for controls vs 0.16 ± 0.09 mg Ca/mg for CF subjects, p = 0.025). Addition of pancreatic enzymes did not fully compensate for this deficiency. In addition, CF patients had higher serum PTH values after a high-calcium meal (p = 0.03), suggesting mild secondary hyperparathyroidism. Altered calcium homeostasis is likely to be a factor in the development of bone disease in CF patients.
Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA, 2006
Few longitudinal data are available characterizing bone development in adolescents with cystic fibrosis (CF) although this is a critical time for bone mineralization. Dual energy X-ray absorptiometry (DXA) scans were obtained at 1- to 4-year intervals in 18 prepubertal and pubertal girls (age 7-18 years) with CF to determine calcium (Ca) accretion rates and changes (Delta) in total body bone mineral content (TBBMC) and lumbar spine bone mineral density (LS BMD) Z-scores. Daily Ca acquisition rates were calculated assuming TBBMC was composed of 32.2% Ca. Bone Ca accretion averaged 82 mg/day (2.05 mmol/day) [(range:-38 to +197 mg/day (-0.95 to 4.9 mmol/day)] on approximately 1,200 mg/day (30 mmol/day) Ca intakes. Estimated mean peak Ca accretion was 160 mg/day (4 mmol/day) at age 13 years; losses of bone Ca occurred in late puberty. Gains in insulin-like growth factor 1 (IGF-1) predicted Ca accretion (p<0.06). Body mass index (BMI) Z-score predicted LS BMD and TBBMC Z-score cross-s...
Bone mineral and body composition alterations in paediatric cystic fibrosis patients
Pediatric Radiology, 2010
Background With the increased life span of cystic fibrosis (CF) patients, CF-related bone diseases could have an increased prevalence and morbidity in this group. In children, previous retrospective and prospective studies have yielded conflicting results on bone mineralization. Objective To monitor body composition and bone mineral status of children with CF. Materials and methods We reviewed the dual-energy X-ray absorptiometry (DXA) data of 161 children with CF (age 10±4.8 years). Total body bone mineral content (BMCt), total lean tissue mass (LTMt) and total fat mass (FMt) were measured and compared to expected data calculated from ideal weight for height (Wi; e.g. BMCti, LTMti, FMti). The bt (BMCt/BMCti), lt (LTMt/LTMti) and ft (FMt/FMti) ratios were used as quantitative variables. Results Low bt ratio was found at all ages (mean bt ratio 0.94±0.10; P<0.001), even in children <6 years of age. However, the children's BMCt was satisfactorily adapted to their weight. lt and ft ratios were not constant across age groups. Children <10 years had 8% reduction of their lt ratio, maintaining normal levels thereafter. The opposite trend was found for ft ratio. Poor clinical, nutritional status and vitamin A levels were correlated with bt and lt ratios. Conclusion Our results indicate that children with CF could have early alterations in their bone status and that lt and ft ratios did not have constant values across ages. Interpreting DXA data using this approach is suitable in children with CF.
Bone mineral status in 134 patients with cystic fibrosis
Archives of Disease in Childhood, 1999
Patients and methods-Whole body bone mineral content (BMC), projected bone area, and bone mineral density (BMD) were determined by dual energy x ray absorptiometry in 134 patients with CF and compared with 396 healthy controls. Results-In patients < 19 years of age, BMD for age was normal in boys and marginally reduced in girls, whereas BMC for age was significantly reduced in both sexes. Height for age and bone area for height were significantly reduced, indicating "short" and "narrow" bones, whereas BMC for bone area was increased, indicating increased size corrected BMC. In patients > 19 years of age, BMD and BMC for age were significantly reduced. Conclusion-Short and narrow bones were the main reasons for reduced BMC for age in patients < 19 years of age, indicating that treatment to prevent osteoporosis in younger patients should be directed at increasing bone size, whereas conventional treatment with calcium and vitamin D supplementation alone might not be as eVective. Because of the significant decrease in BMD and BMC in adult patients, we fear that these patients may develop osteoporotic fractures prematurely. (Arch Dis Child 1999;81:235-240) Keywords: bone mineral content; bone mineral density; cystic fibrosis Dis Child 1999;81:235-240 235 Department of Growth and Reproduction GR,
Journal of Cystic Fibrosis, 2008
Background: Low bone mineral density is common in adults with cystic fibrosis. Children with good lung function compared to controls matched for body size have normal bone mineralisation. There are few data in large unselected populations of children. Methods: All children between five and 16 years were invited to take part. Disease severity was assessed. Bone mineral measurements using a GE-Lunar Prodigy densitometer were expressed as age and gender matched Z-scores. Bone mineral apparent density for L2-L4 was estimated and data from UK Caucasian children used to create age and gender specific reference ranges for predicted values. Z-scores were calculated. Total body analysis utilised the Molgaard method. Blood was sampled for measurement of 25-hydroxyvitamin D, and parathyroid hormone levels. Results: 107 children entered the study. 18 and 10 children had low areal and apparent bone mineral density respectively. Short, narrow bones were common. Fifteen children reported 22 fractures, 20 with associated trauma. The best predictors of bone status were ZBMI and percent predicted FEV 1. Conclusions: Bone mineral density corrected for body size was normal in over 90% of children. These results are similar to previously reported results in small studies of children with well preserved respiratory function.
Bone mass density and associated factors in cystic fibrosis patients of young age
Journal of Paediatrics and Child Health, 2008
To investigate bone mineral status in young cystic fibrosis (CF) patients and determine risk factors related with the development of low bone mineral density (BMD). We determined, in 81 patients with CF, 4 to 23-years-old, BMD as well as factors, which are thought to play a role in the development of reduced BMD. BMD Z-score was between -1 and -2.5 in 27 (33%) and lower than -2.5 in 9 (11%). Means of BMD Z-score were lower than the expected value of 0 in the three groups of children, adolescents and young adults (P = 0.004; P &amp;amp;amp;amp;amp;lt; 0.001; P = 0.048, respectively), but they did not differ among them (P = 0.114). Analysis showed that Shwachman-Kulczycki (SK) score, gender and levels of 25-hydroxy-vitamin D were significant predictors of BMD Z-score. Significant also was the interaction between gender and SK score. Our study supports that BMD may be reduced from a young age in CF patients though this needs to be confirmed using true volumetric measures of BMD. This defect is related to disease severity with males being more vulnerable. Inefficient levels of vitamin D are very common and contribute significantly to impaired bone health. The latter finding underlines the need for higher supplementation doses.
Some biochemical indices of nutrition in treated cystic fibrosis patients
The American journal of clinical nutrition, 1981
Postprandial levels of copper, ceruloplasmin, iron, total iron binding capacity, cholesterol, vitamin A, carotene, folic acid, vitamin C, albumin, and total globulins in plasma, of 25-OH-vitamin D in serum, and of glutathione reductase activity, an index of riboflavin status, in erythrocytes were determined in a group of 18 juvenile cystic fibrosis patients receiving specialized outpatient care with attention to diet, vitamin supplementation, and pancreatic enzyme replacement. Bone mineralization was assessed by radiographic and photon beam technique. In the plasma of cystic fibrosis patients, levels were elevated for copper, ceruloplasmin, total globins, and total proteins and were depressed for iron, vitamin D, vitamin A, carotene, and albumin. Cortical thickness was diminished in the patients, but bone density was not. For patients with cystic fibrosis, a relation was established between forced vital capacity and certain biochemical indices in plasma. As forced vital capacity dec...
Undercarboxylated osteocalcin and bone mass in 8–12 year old children with cystic fibrosis
Journal of Cystic Fibrosis, 2008
Young adults with cystic fibrosis (CF) frequently develop bone disease. One suggested aetiological factor is suboptimal vitamin K status with impaired carboxylation of osteocalcin and abnormal bone formation. Methods: We measured bone mineralization and turnover in thirty-two 8-12 year old CF patients (14 boys) using Dual Energy X-ray absorptiometry (whole body (WB) and lumbar spine (LS)), 25-OH Vitamin D, PTH and markers of bone formation (plasma osteocalcin, N-terminal pro-peptide of type 1 collagen (P1NP)), plus an indirect measure of vitamin K status, undercarboxylated osteocalcin (uc-OC). Results: LS bone mineral density (BMD) standard deviation (SD) scores were b −1.0 in 20% of subjects. Size-adjusted LS and WB bone mass was normal. Compared to reference data, % uc-OC was high and P1NP low. LS bone mass was predicted by % uc-OC but not other markers (0.4% decrease in size-adjusted LSBMC (p = 0.05); 0.04 SD decrease in LSBMAD (p = 0.04) per 1% increase in uc-OC). Conclusion: Markers suggestive of sub-optimal vitamin K status and low bone formation were present despite normal size-adjusted bone mass. The association between LSBMC and % uc-OC is consistent with the hypothesis that sub-optimal vitamin K status is a risk factor for CF bone disease. This should ideally be investigated in an intervention trial.