FGF-23 is a
phosphaturic hormone secreted by osteoblasts and osteocytes in response to elevated serum P.
FGF23 elevation in early CKD is an adaptive response to maintain phosphate metabolism by its
phosphaturic action.
Ernst, "Calciotropic and
phosphaturic hormones in heart failure," Nutrition, Metabolism and Cardiovascular Diseases, vol.
The term "
phosphaturic mesenchymal tumor" (PMT) was introduced by Weidner and Santa Cruz [8], and PMT was eventually added to the 2013 WHO classification of tumors of the soft tissue and bone [9].
With increasing renal function impairment, phosphate retention induces compensating elevation of the
phosphaturic hormones FGF23 and PTH.
Serum level of the
phosphaturic factor FGF23 is associated with abdominal aortic calcification in men : The STRAMBO study.
Hence a diagnosis of
phosphaturic mesenchymal tumour-mixed connective tissue variant was made.
In a physiological setting, FGF23 acts as a
phosphaturic hormone and decreases serum phosphate concentration, which is expected to prevent vascular calcification.
In this type, gene mutation does not exist rather it has regulation defect of PTH receptor as well as decreased nephrogenic c AMP response without other hormonal resistance and somatic features of AHO.6,7 Type II PHP is characterized by increased PTH, hypocalcemia, hyperphosphatemia and absolute lack of physical somatic features of AHO, further unique differentiating features is a normal cAMP response to PTH infusion but a deficient
phosphaturic response indicating a defect distal to c AMP generation in renal cells and normal GNAS1 activity.8-11 Our case at presentation was diagnosed as PHP on the basis of common features of PHP which are increased PTH, hyperphosphatemia and hypocalcemia.
Phosphaturic effect of dopamine in dogs: possible role of intrarenally produced dopamine in phosphate regulation.
These and other disorders leading to HRO such as fibrous dysplasia (FD) are associated with increased secretion of
phosphaturic substances called 'phosphatonins' which cause proximal tubular leak of phosphate resulting in hypophosphataemia.
Based on their histologic features, they have been subdivided into 4 types: 1)
phosphaturic mesenchymal tumor, mixed connective tissue type (PMTMCT); 2) osteoblastoma-like tumors; 3) ossifying fibroma-like tumors; and 4) nonossifying fibroma-like tumors.