Evaluation and Treatment of Disorders of Phosphate Balance
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Pediatric Nephrology (BP Dixon and E Nehus, Section Editors)
Evaluation and Treatment of Disorders of Phosphate Balance Mark R. Hanudel Address Department of Pediatrics, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA Email: [email protected] 2 UCLA Department of Pediatrics, Division of Pediatric Nephrology, 10833 Le Conte Avenue, MDCC A2-383, Los Angeles, CA, USA
* Springer Nature Switzerland AG 2020
This article is part of the Topical Collection on Pediatric Nephrology Keywords Phosphate I Hypophosphatemia I Hyperphosphatemia I Rickets I Fibroblast growth factor 23 (FGF23) I Burosumab
Abstract Purpose of review The purpose of this article is to review (1) the molecular mechanisms and hormonal factors involved in phosphate handling and regulation, (2) how to appropriately interpret serum and urine phosphate measurements in pediatric patients, (3) the pathophysiology of hypophosphatemic and hyperphosphatemic conditions, and (4) current strategies for treatment of hypophosphatemia and hyperphosphatemia in pediatric patients. Recent findings For decades, treatment of some hypophosphatemic conditions like Xlinked hypophosphatemic rickets (XLH), characterized by a primary increase in fibroblast growth factor 23 (FGF23) activity, consisted of non-specific therapy with phosphate supplementation and calcitriol administration. However, in the past few years, burosumab, a targeted anti-FGF23 antibody, has been developed, representing a promising new medication for the treatment of pediatric XLH patients. The treatment of hyperphosphatemic conditions like chronic kidney disease (CKD) consists of dietary phosphate restriction and enteral phosphate binders; however, the development of new binders and inhibitors of cellular phosphate transporters may offer additional treatment options in the future. Summary The evaluation and treatment of disorders of phosphate balance in children is complex, as numerous interrelated mechanisms and hormones are involved in phosphate handling and regulation. Knowledge of the pathophysiology of hypophosphatemic and hyperphosphatemic conditions informs optimal diagnostic and treatment strategies.
Pediatric Nephrology (BP Dixon and E Nehus, Section Editors)
Introduction Phosphate is critically important in many aspects of human physiology, including skeletal development, bone mineralization, membrane composition, nucleotide structure, and cellular signaling [1, 2]. Appropriate handling of phosphate is essential in growing children, as they require a positive phosphate balance to achieve optimal skeletal growth and mineralization, with osteomalacia and rickets-like phenotypes resulting from negative phosphate balances.
Phosphate handling and regulation Phosphate homeostasis depends on the regulated partitioning of phosphate among multiple organ systems and fluid compartments, including the gastrointestinal system, kidneys, bone, extracellular fluid and serum, and intracellular fluid and soft tissue (Fig. 1). Phosphate regulation is a complex process involving many differ
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