After successful kidney transplantation, accumulated waste products and electrolytes are excreted and regulatory hormones return to normal levels. Despite the improvement in mineral metabolites and mineral regulating ...After successful kidney transplantation, accumulated waste products and electrolytes are excreted and regulatory hormones return to normal levels. Despite the improvement in mineral metabolites and mineral regulating hormones after kidney transplantation,abnormal bone and mineral metabolism continues to present in most patients. During the first 3 mo, fibroblast growth factor-23(FGF-23) and parathyroid hormone levels decrease rapidly in association with an increase in 1,25-dihydroxyvitamin D production. Renal phosphate excretion resumes and serum calcium, if elevated before, returns toward normal levels. FGF-23 excess during the first 3-12 mo results in exaggerated renal phosphate loss and hypophosphatemia occurs in some patients. After 1 year, FGF-23 and serum phosphate return to normal levels but persistent hyperparathyroidism remains in some patients. The progression of vascular calcification also attenuates. High dose corticosteroid and persistent hyperparathyroidism are the most important factors influencing abnormal bone and mineral metabolism in long-term kidney transplant(KT) recipients. Bone loss occurs at a highest rate during the first 6-12 mo after transplantation. Measurement of bone mineral density is recommended in patients with estimated glomerular filtration rate > 30 mL/min. The use of active vitamin D with or without bisphosphonate is effective in preventing early post-transplant bone loss. Steroid withdrawal regimen is also beneficial in preservation of bone mass in long-term. Calcimimetic is an alternative therapy to parathyroidectomy in KT recipients with persistent hyperparathyroidism. If parathyroidectomy is required, subtotal to near total parathyroidectomy is recommended. Performing parathyroidectomy during the waiting period prior to transplantation is also preferred in patients with severe hyperparathyroidism associated with hypercalcemia.展开更多
Oncogenic osteomalacia (OOM) is an uncommon metabolic and bone disease caused by fibroblast growth factor 23 (FGF23), a phosphaturic factor produced by phosphaturic mesenchymal tumors (mixed connective tissue variant,...Oncogenic osteomalacia (OOM) is an uncommon metabolic and bone disease caused by fibroblast growth factor 23 (FGF23), a phosphaturic factor produced by phosphaturic mesenchymal tumors (mixed connective tissue variant, PMTMCTV) characterized by phosphate leakage from kidneys and subsequent hypophosphatemia. In this paper, we present the case of a patient, 42-year-old woman affected by left side limp and pain involving lumbar spine, pelvis and hip joints, referred to the Rheumatology Department of our Hospital for the treatment of a suspected sero-negative spondilo-arthritis. During hospitalization patient began an immuno-suppressive therapy with TNF-alpha inhibitors associated with Pamidornate, Indometacin, Esomeprazole and vitamin D3. Nevertheless pain did not decrease and a new examination found a worst hypophosphatemia (1 mg/dl) with normal Ca and PTH’s plasma values. During the same check-up a painful bulge on the anterior part of the right knee was observed and the Magnetic Resonance Imaging scan revealed an ovular solid lesion in the soft tissue closed to the upper part of the patella. Histological analysis identified the lesion as a PMTMCTV. After surgical removal patient got complete recovery. We will discuss about diagnostic evaluation, differential diagnosis and treatment.展开更多
Objective: The aim of this paper was to assess the relationships among chemical, phase and structural composition and?etiopathogenic factors of non-infectious phosphate calculi formed in patients with low and high uri...Objective: The aim of this paper was to assess the relationships among chemical, phase and structural composition and?etiopathogenic factors of non-infectious phosphate calculi formed in patients with low and high urinary phosphate concentrations, and to characterize the mechanism of their formation related on biochemical results. Material and Methods: Twelve samples of phosphate renal calculi were obtained, 4 from patients with low phosphaturia and 6 from patients with high urinary phosphate concentrations. Their chemical composition was determined qualitatively by energy dispersive X-ray analysis and quantitatively by spectrophotometric and thermal analysis;and their phase composition was determined by Fourier transform infrared transmission spectroscopy and X-ray diffraction. The structure of the calculi was assessed by scanning electron microscopy. Results: Non-infectious phosphate renal calculi of patients with low phosphaturia consist of poorly crystalline carbonate hydroxyapatite, whereas those of patients with high urinary phosphate concentrations consist of poorly crystalline hydroxyapatite with some amount of calcium oxalate crystals. Calculi of patients with high urinary phosphate concentrations are formed at urinary supersaturation with respect to hydroxyapatite and calcium oxalate about 4 times higher than in patients with low phosphaturia. Conclusion: In patients with low phosphaturia, the non-infectious phosphate renal calculi are formed in urine near pH 7 and contain only poorly crystalline carbonate hydroxyapatite. In patients with high urinary phosphate concentrations and hypercalciuria, the calculi are formed in urine near pH 6 and consist of both poorly crystalline hydroxyapatite and some amount of calcium oxalate crystals.展开更多
文摘After successful kidney transplantation, accumulated waste products and electrolytes are excreted and regulatory hormones return to normal levels. Despite the improvement in mineral metabolites and mineral regulating hormones after kidney transplantation,abnormal bone and mineral metabolism continues to present in most patients. During the first 3 mo, fibroblast growth factor-23(FGF-23) and parathyroid hormone levels decrease rapidly in association with an increase in 1,25-dihydroxyvitamin D production. Renal phosphate excretion resumes and serum calcium, if elevated before, returns toward normal levels. FGF-23 excess during the first 3-12 mo results in exaggerated renal phosphate loss and hypophosphatemia occurs in some patients. After 1 year, FGF-23 and serum phosphate return to normal levels but persistent hyperparathyroidism remains in some patients. The progression of vascular calcification also attenuates. High dose corticosteroid and persistent hyperparathyroidism are the most important factors influencing abnormal bone and mineral metabolism in long-term kidney transplant(KT) recipients. Bone loss occurs at a highest rate during the first 6-12 mo after transplantation. Measurement of bone mineral density is recommended in patients with estimated glomerular filtration rate > 30 mL/min. The use of active vitamin D with or without bisphosphonate is effective in preventing early post-transplant bone loss. Steroid withdrawal regimen is also beneficial in preservation of bone mass in long-term. Calcimimetic is an alternative therapy to parathyroidectomy in KT recipients with persistent hyperparathyroidism. If parathyroidectomy is required, subtotal to near total parathyroidectomy is recommended. Performing parathyroidectomy during the waiting period prior to transplantation is also preferred in patients with severe hyperparathyroidism associated with hypercalcemia.
文摘Oncogenic osteomalacia (OOM) is an uncommon metabolic and bone disease caused by fibroblast growth factor 23 (FGF23), a phosphaturic factor produced by phosphaturic mesenchymal tumors (mixed connective tissue variant, PMTMCTV) characterized by phosphate leakage from kidneys and subsequent hypophosphatemia. In this paper, we present the case of a patient, 42-year-old woman affected by left side limp and pain involving lumbar spine, pelvis and hip joints, referred to the Rheumatology Department of our Hospital for the treatment of a suspected sero-negative spondilo-arthritis. During hospitalization patient began an immuno-suppressive therapy with TNF-alpha inhibitors associated with Pamidornate, Indometacin, Esomeprazole and vitamin D3. Nevertheless pain did not decrease and a new examination found a worst hypophosphatemia (1 mg/dl) with normal Ca and PTH’s plasma values. During the same check-up a painful bulge on the anterior part of the right knee was observed and the Magnetic Resonance Imaging scan revealed an ovular solid lesion in the soft tissue closed to the upper part of the patella. Histological analysis identified the lesion as a PMTMCTV. After surgical removal patient got complete recovery. We will discuss about diagnostic evaluation, differential diagnosis and treatment.
基金FEDER founds (European Union) the Conselleria d’Educació, Cultura i Universitats (Govern de les Illes Balears).
文摘Objective: The aim of this paper was to assess the relationships among chemical, phase and structural composition and?etiopathogenic factors of non-infectious phosphate calculi formed in patients with low and high urinary phosphate concentrations, and to characterize the mechanism of their formation related on biochemical results. Material and Methods: Twelve samples of phosphate renal calculi were obtained, 4 from patients with low phosphaturia and 6 from patients with high urinary phosphate concentrations. Their chemical composition was determined qualitatively by energy dispersive X-ray analysis and quantitatively by spectrophotometric and thermal analysis;and their phase composition was determined by Fourier transform infrared transmission spectroscopy and X-ray diffraction. The structure of the calculi was assessed by scanning electron microscopy. Results: Non-infectious phosphate renal calculi of patients with low phosphaturia consist of poorly crystalline carbonate hydroxyapatite, whereas those of patients with high urinary phosphate concentrations consist of poorly crystalline hydroxyapatite with some amount of calcium oxalate crystals. Calculi of patients with high urinary phosphate concentrations are formed at urinary supersaturation with respect to hydroxyapatite and calcium oxalate about 4 times higher than in patients with low phosphaturia. Conclusion: In patients with low phosphaturia, the non-infectious phosphate renal calculi are formed in urine near pH 7 and contain only poorly crystalline carbonate hydroxyapatite. In patients with high urinary phosphate concentrations and hypercalciuria, the calculi are formed in urine near pH 6 and consist of both poorly crystalline hydroxyapatite and some amount of calcium oxalate crystals.
