Bone and Mineral Disorders Clinic, Section of Pediatric Nephrology, Children's Mercy Hospitals and Clinics, University of Missouri at Kansas City School of Medicine, 2401 Gillham Road, Kansas City, MO 64108, USA.
Until a decade ago, two main hormones were recognized as directly affecting phosphate homeostasis and, with that, bone metabolism: parathyroid hormone and 1,25(OH)(2) vitamin D (calcitriol). It was only a decade ago that the third major player hormone was found, linking gut, bone, and kidney. The physiologic role of fibrinogen growth factor (FGF)23 is to maintain serum phosphate concentration within a narrow range. Secreted from osteocytes, it modulates kidney handling of phosphate reabsorption and calcitriol production. Genetic and acquired abnormalities in FGF23 structure and metabolism cause conditions of either hyper-FGF23-manifested by hypophosphatemia, low serum calcitriol, and rickets/osteomalacia-or hypo-FGF23, expressed by hyperphosphatemia, high serum calcitriol, and extra-skeletal calcifications. In patients with chronic renal failure, FGF23 levels increase as kidney functions deteriorate and are under investigation to learn if the hormone actually participates in the pathophysiology of the deranged bone and mineral metabolism typical for these patients and, if so, whether it might serve as a therapeutic target. This review addresses the physiology and pathophysiology of FGF23 and its clinical applications.
"Moreover, the urinary calcium excretion is normal, whereas X-ray changes may demonstrate rickets or osteomalacia. FGF23 levels are typically high, either due to overproduction or under-catabolism, and in children with rickets, the combined evaluation of FGF23 and PTH leads differential diagnosis in the direction of impaired phosphate homeostasis (high FGF23 and normal PTH) or altered metabolism of vitamin D, calcium or magnesium (low FGF23 and high PTH) (Alon 2010). In the case of hyperphosphatemia, there is usually high plasma phosphate, an inappropriate normal % TRP and TmP/GFR, a low or normal PTH and normal renal function. "
[Show abstract][Hide abstract] ABSTRACT: Rickets remains a common problem among infants and children in many countries worldwide. Although the classical presentation associated with bone abnormalities is well known, paediatricians need to be aware of atypical presentations, especially in the first 6 months of life. Furthermore, although vitamin D deficiency rickets remains the commonest form of rickets in most countries, health care providers need to be aware of other possible causes and their typical clinical and biochemical presentations. This article discusses these and highlights the characteristic features of various forms of rickets and possible pitfalls clinicians should be aware of when confronted with a patient with suspected rickets. In conclusion, the recent advances made in understanding the underlying pathogeneses of the various forms of rickets has helped to delineate the diagnostic tests that assist in the diagnosis and management of the disease in children.
European Journal of Pediatrics 09/2011; 170(9):1089-96. DOI:10.1007/s00431-011-1529-z · 1.89 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: To compare the pattern of serum FGF23 levels in pediatric renal transplant recipients and GFR-matched controls. We performed a cross-sectional matched pair study in 19 stable pediatric renal transplant recipients and 19 GFR-matched controls with native CKD. After assessment for normal distribution, demographic and bone metabolism parameters were compared with Student's t-test, Wilcoxon's matched pairs (for non-normal distribution) test, and correlation analysis. The groups were comparable for anthropometric parameters, cystatin C eGFR (71.10 ± 37.28 vs. 76.11 ± 26.80 mL/min/1.73 m(2) ), cystatin C, urea, creatinine, intact PTH, pH, CRP, alkaline phosphatase, phosphate, calcium, ionized calcium, FGF-23 (63.44 [IQR 38.42, 76.29], 49.92 [IQR 42.48, 76.97]), albumin, and urinary calcium/creatinine ratio. The renal transplant patients had significantly lower 25-(OH) vitamin D levels (66.63 ± 17.54 vs. 91.42 ± 29.16 ng/mL), and higher 1,25-(OH)(2) vitamin D levels (95.78 ± 34.54 vs. 67.11 ± 35.90 pm). FGF-23 levels correlated negatively with cystatin C eGFR (r = -0.3571, p = 0.02770) and positively with PTH (r = 0.5063, p = 0.0026), but not with serum phosphate (r = 0.2651, p = 0.1077). We conclude that the increase in FGF23 levels with GFR decline in pediatric renal transplant patients remains similar to that in the patients with CKD. The relationship between FGF23 and serum vitamin D needs further evaluation.
Nazi Chen, Jishen Ma, Yang Zhao, Meiyu Wu, Huanhuan Yang, Weiyue Gong, Jiang Chao, Xiaokun Li
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