Aim: 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).
Methods: 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.
Results: 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 < 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.
Conclusions: 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.
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http://dx.doi.org/10.1111/j.1440-1754.2008.01406.x | DOI Listing |
Diagnostics (Basel)
March 2025
Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul 06591, Republic of Korea.
: With increasing cases of osteoporosis in children and adolescents, the need for timely diagnosis, management, and follow-up has become important. This study aimed to determine whether bone turnover markers (BTMs), particularly serum bone-specific alkaline phosphatase (BsALP) and serum C-telopeptide of collagen type 1 (CTx), accurately reflect BMD. : In this retrospective study, 280 post-puberty males and females who were previously diagnosed with hemato-oncologic, rheumatic, gastrointestinal, and endocrinologic diseases at a single tertiary care center were reviewed.
View Article and Find Full Text PDFJ Pediatr Orthop
March 2025
Department of Orthopaedics and Traumatology.
Background: Although cystic fibrosis (CF) mainly affects the respiratory and gastrointestinal systems, it may frequently present with musculoskeletal manifestations including bone fractures, low bone mineral density, and spinal pathologies. Assessment of spinal pathologies in CF patients is of vital importance because the effects on lung capacity and spinal posture are clearly defined.
Questions/purposes: The frequency of vertebral pathologies in CF patients has yet to be determined.
Nutrients
February 2025
Department of Pediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, 04-730 Warsaw, Poland.
Background: This cross-sectional study aimed to assess growth, body weight, and bone mineralization and to identify predictors of metabolic bone disease (MBD) in children with chronic intestinal failure (CIF) on long-term parenteral nutrition (LPN).
Methods: Twenty-six children with CIF were evaluated on total parenteral nutrition (PN) for at least three years, and 60 healthy controls were assessed. Measurements included body weight, height, BMI, serum levels of 25-hydroxyvitamin D3 (25-OHD3), calcium (Ca), phosphorus (P), magnesium (Mg), and aluminum (Al), as well as urinary excretion of these elements.
J Clin Endocrinol Metab
February 2025
Department of Pediatrics, Endocrine Unit, Endo-ERN Center for Rare Endocrine Diseases, IRCCS San Raffaele Hospital, Milan, Italy.
Context: Patients affected by the classic form of congenital adrenal hyperplasia (CAH) need lifelong glucocorticoid therapy (GC). GC represents one of the primary causes of secondary osteoporosis, however the effect of steroid therapy on bone mineral density (BMD) in patients with CAH is still controversial.
Objective: To evaluate and compare the BMD of a group of prepubertal patients and a subgroup of young adult patients with CAH receiving chronic GC therapy, with healthy controls.
Osteoporos Int
February 2025
Department of Endocrinology, Key Laboratory of Endocrinology of National Ministry of Health, National Health and Family Planning Commission, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Shuaifuyuan No. 1, Dongcheng District, Beijing, 100730, China.
Unlabelled: This study evaluated trabecular bone score (TBS) for assessing vertebral fractures and spinal deformity in children and adolescents with osteogenesis imperfecta (OI). TBS showed superior performance in identifying vertebral fractures compared to areal bone mineral density (aBMD), especially in patients without densitometric osteoporosis, suggesting its potential for monitoring vertebral fractures and spinal deformity risk.
Background: TBS, derived from a textural greyscale analysis of lumbar spine dual-energy X-ray absorptiometry (DXA) images, offers a non-invasive and indirect evaluation of bone microarchitecture.
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