ABSTRACT
We herein report two cases of Fanconi syndrome with refractory hypophosphatemic osteomalacia that was difficult to correct by phosphorus replacement therapy. The pathological result was a bony giant cell tumor and osteosarcoma, respectively. Interestingly, after resection of the tumors, the patient with osteosarcoma recovered completely but the patient with the bony giant cell tumor had a relapse. Although she underwent nine operations, her symptoms and laboratory tests did not improve. These findings indicate that Fanconi syndrome can result from a bone tumor.
Subject(s)
Bone Neoplasms/complications , Fanconi Syndrome/etiology , Giant Cell Tumor of Bone/complications , Osteosarcoma/complications , Adult , Child , Fanconi Syndrome/drug therapy , Female , Humans , Hypophosphatemia/drug therapy , Hypophosphatemia/etiology , Osteomalacia/drug therapy , Osteomalacia/etiology , Phosphorus Compounds/administration & dosageABSTRACT
Serum level of phosphate is regulated by the kidney, especially proximal tubule. The transcellular transport of phosphate in the proximal tubule is mediated via Na dependent transporters, i.e., NPT2a and NPT2b at the luminal membrane, and unknown channel at the basolateral side. The transport of phosphate via NPT2a and NPT2b is further regulated by factors, such as PTH, FGF23, and 1,25(OH)(2)D. Several hereditary diseases that cause hypophoshatemia specically are known. In addition, dysfunction of proximal tubule may develop Fanconi syndrome, which also causes hypherphosphaturia. In this section, I describe the renal mechanisms of phosphate handling and the causes of hypophosphatemia along with its treatment.
Subject(s)
Hypophosphatemia/etiology , Hypophosphatemia/metabolism , Kidney Tubules, Proximal/metabolism , Phosphates/metabolism , Administration, Oral , Calcitriol/physiology , Chloride Channels , Dent Disease/etiology , Dent Disease/genetics , Dent Disease/metabolism , Fanconi Syndrome/etiology , Fanconi Syndrome/metabolism , Fibroblast Growth Factor-23 , Fibroblast Growth Factors/physiology , Humans , Hypophosphatemia/therapy , Mitochondrial Diseases , Oculocerebrorenal Syndrome , Parathyroid Hormone/physiology , Phosphoric Monoester Hydrolases , Phosphorus Compounds/administration & dosage , Phosphorus Compounds/therapeutic use , Sodium-Phosphate Cotransporter Proteins, Type IIa/physiology , Sodium-Phosphate Cotransporter Proteins, Type IIc/physiology , Vitamin D/administration & dosageABSTRACT
The aim of this study was to evaluate the physical properties, chemical structure and bioactivity of sol-gel processed oxide (SiO(2)-CaO-P(2)O(5)) composites used as controlled release materials for Metronidazole-drug applied in periodontal disease treatment. The obtained composite materials were characterized by Fourier transform infrared spectroscopy (FTIR), X-ray powder diffraction (XRD), the Brunauer-Emmet-Teller (BET) technique and further monitoring in the ultraviolet and visible light regions (UV-Vis) of the in vitro release of the drug over time. Using tetramethoxysilane (TMOS) as a precursor of silica matrix and calcium nitrate tetrahydrate (Ca(NO(3))(2) 4H(2)O), triethyl phosphite (P(OC(2)H(5))(3)) as precursors of CaO and P(2)O(5) respectively, xerogels with different morphology and physical properties were obtained. The applied modifications improved also the bioactivity and changed the profile of the drug release. Based on the presented results of this study, it may be concluded that applied xerogel matrices could be promising candidates for the formulation in local delivery systems.
Subject(s)
Calcium Compounds/chemistry , Gels/chemistry , Metronidazole/chemistry , Oxides/chemistry , Phosphorus Compounds/chemistry , Silicon Dioxide/chemistry , Calcium Compounds/administration & dosage , Delayed-Action Preparations , Drug Delivery Systems/methods , Drug Stability , Gels/administration & dosage , Metronidazole/administration & dosage , Nitrates/chemistry , Oxides/administration & dosage , Phase Transition , Phosphites/chemistry , Phosphorus Compounds/administration & dosage , Silanes/chemistry , Silicon Dioxide/administration & dosageABSTRACT
Phosphate plays a vital role forming the high-energy band within ATP. The pathophysiological results of phosphate deficiency are inadequate supplies of energy-rich phosphates and, in particular, inhibition of glyceraldehyde-3- phosphate dehydrogenase, which occupies a key position in glycolysis. The effect of this on the central nervous system, muscle and erythrocyte energy metabolism is to reduce ATP and 2,3-diphosphoglycerate levels, leading to left-hand displacement of the oxygen-hemoglobin dissociation curve with decreased peripheral oxygen uptake and transport. Therefore, detection and treatment of acute hypophosphatemia is important in many hospitalized patients particularly in ICU patients. Severe hypophosphatemia is also associated with a number of neuromuscular and cardiovascular sequelae, in which phosphate supplementation leads to improved symptoms and clinical parameters. In clinical practice it is common on administering 0.4 mmol (12 mg) phosphate/kg per day, and to adjust this on the basis of the serum phosphate analysis.
Subject(s)
Hypophosphatemia/drug therapy , Phosphorus Compounds/administration & dosage , Adenosine Triphosphate/metabolism , Energy Metabolism , Glyceraldehyde-3-Phosphate Dehydrogenases/physiology , Humans , Hypophosphatemia/etiology , Monitoring, Physiologic , Parenteral Nutrition, Total/adverse effects , Phosphorus/blood , Phosphorus/deficiency , Phosphorus/physiologyABSTRACT
Patch testing was performed with phosphorus sesquisulfide P4S3 in 2 groups containing equal numbers of patients using different concentrations (0.5% P4S3 in pet. and 1% P4S3 in pet., the usual suggested test concentration as recommended by the International Contact Dermatitis Research Group). We found that there was a statistically significant increase in the number of clinically irrelevant irritant reactions in the group tested with the concentration (chi 2 = 16, p < 0.0004). We recommend that patch testing with phosphorous sesquisulfide should be at a reduced concentration of 0.5% pet.