Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.1.3.1 (alkaline phosphatase)
 47,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three adolescents on regular haemodialysis were treated for more than one year with 1alphaOHD3 in doses of 0.25 - 10 mug/day orally. All the patients had hypocalcaemia, a high serum level of alkaline phosphatase and signs of uraemic osteodystrophy on X-ray. Clinical signs of bone disorder were present in two cases. During the treatment serum calcium and alkaline phosphatase normalised, and there was a marked improvement in the X-ray changes. Clinical improvement also took palce but not to the same extent as indicated by the laboratory data. It seems therefore important that treatment should start before severe bone lesions are present. Careful control of the treatment is necessary to avoid hypercalcaemia.
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PMID:Long-term treatment of uraemic osteodystrophy with 1-alpha-hydroxycholecalciferol. 93 14

The present clinical aspects of Paget's osteodystrophy are reviewed. The nosological definition, localiztion, natural course and signs are described and the recent description of "rheumatoid manifestations" in Paget's disease by FRANCK et al. is mentioned. The same authors revealed a positive correlation between the grade of extenstion of Paget's disease in the whole skeleton and the concentration values for alkaline phosphatase and uric acid in the serum. Among the complications of Paget's disease the orthopedic, neurological, haemodynamic, oncologic, hematological and dermatological are reviewed X-ray of the involved skeleton, which in most cases is diagnostic, may be supported by isotope scanning with 18F or 87mSr and bone biopsy for establishment of diagnosis. Current drug therapy is confined to diphosphonate and calcitonin. The antibiotic mithramycin, which is cytotoxic, reduces bone turnover and may improve the course in Paget's disease. However, toxic side effects on kidney, liver and hemopoiesis do not allow its further therapeutic use in this disease. A case is described which demonstrates that a spontaneous or traumatic fracture in the area of osteodystrophy exhibits almost the same potential for conso lidation as normal bone tissue following both conservative and osteosynthetic treatment of the fracture. In a further instance corrective osteotomy with osteosynthesis (plate) because of serious varisation and antecurvature of the femur due to Paget's disease were performed sucessfully without assisting drug therapy. A third patient displayed extensive osteodystrophy of the whole pelvic skeleton, which was discovered by X-ray as rehabilitation following CVA failed to progress due to severe bilateral reduction of hip joint function. In view of the age and general status of the patient and the absence of pain, no medication or surgical therapy was performed in this case.
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PMID:[Paget's deforming osteodysttophy]. 115 90

Four patients with advanced chronic renal failure and osteodystrophy were treated with 1-alphahydroxycholecalciferol, a synthetic vitamin D analogue, in a daily oral dose of 1.5 to 2.0 mug, for periods up to 1 year. They showed increased calcium absorption, positive calcium and phosphorus balances, moderate increases in serum calcium levels, marked reductions in serum alkaline phosphatase levels, a decrease in serum immunoreactive parathyroid hormone levels, and radiologic and histologic improvement in bone disease. One patient with proximal myopathy showed improvement in muscular strength. 1-Alphahydroxycholecalciferol appears to be effective therapy for renal osteodystrophy.
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PMID:1-alphahydroxycholecalciferol in chronic renal failure. Studies of the effect or oral doses. 125 63

Three adolescents with uraemic osteodystrophy were treated for 7 months with daily oral doses of 1alpha-hydroxycholecalciferol (0.25-10.0 mug). All the patients were hypocalcaemic and had high serum levels of alkaline phosphatase before the treatment. A rapid rise in serum calcium and a slow, but pronounced decline in serum alkaline phosphatase concentration were observed during the period of treatment. 1alpha-Hydroxycholecalciferol induced hypercalcaemia in two situations. In both cases the hypercalcaemia was transient, serum calcium being normalized in a few days by withdrawal of the drug. Withdrawal of the drug also in other situations resulted in a fall in serum calcium concentration in a couple of days. This suggests that 1alpha-hydroxycholecalciferol should be given daily or every other day. The response did not subside during 7 months of treatment. On the contrary, the maintenance dose necessary to keep serum calcium constant was smaller than the initial dose necessary to normalize serum calcium. Bone mineral content, estimated by photon absorptiometry, rose. The rachitic bone lesions seen radiologically were significantly ameliorated during the treatment.
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PMID:1alpha-hydroxycholecalciferol. Long-term treatment of patients with uraemic osteodystrophy. 126 9

Renal bone disease is an important cause of morbidity in patients on dialysis. The prevalence of renal bone disease, especially aluminium related bone disease, has not been studied in the Singapore dialysis population. As such, we studied 45 haemodialysis patients for renal bone disease using biochemical and radiological parameters. Selected patients underwent a renal biopsy. There were 29 males and 16 females, mean (+/- SEM) age, 44.6 +/- 13.4 years. The duration of haemodialysis ranged from two months to ten years, mean 18.5 months. 75.4% of patients had hyperphosphatasemia, 24.4% had hypocalcemia and two patients had hypercalcemia. There was a wide range in the serum parathyroid hormone levels and 55.4% of patients had serum parathyroid hormone levels > 1000 pmol/L. Patients with symptoms and radiological abnormalities had significantly higher serum parathyroid hormone and alkaline phosphatase levels than those without (P < 0.005). The desferrioxamine infusion test was positive, with an increment in serum aluminium (DL) > 100 mg/L in five patients. Skeletal survey was positive for renal bone disease in 24.4% of patients. There was a significant correlation between the serum parathyroid hormone level, DA1 and the duration of dialysis (r = 0.752, p < 0.001 and r = 0.837, p < 0.001 respectively). There was no correlation between serum parathyroid hormone, calcium, phosphate levels and DA1. The serum haemoglobin concentration and ferritin levels did not show a correlation with DA1. Bone biopsy revealed hyperparathyroid bone disease in two patients, aluminium-related bone disease in one patient and mixed uraemic osteodystrophy in one patient.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Renal bone disease in patients on haemodialysis: biochemical and radiological assessment. 129 14

The investigation on several forms of uremic osteodystrophy by means of bone mineral content (BMC) measurement led to contradictory conclusions. BMC in 27 patients on periodical hemodialysis treatment was measured correlating it to the seric levels of Ca, P, Mg, alkaline phosphatase (AP), calcitonin (Ct), osteocalcin (BGP), intact parathormone (PTHi), c-terminal and mean molecule PTH. Patients on dialysis treatment from a long period of time showed high AP and low BMC levels. This correlation proved significant just for the values recorded at a third distal site of radius. Patients with BMC under the normal range showed higher BGP levels and a longer period of dialytic treatment than those presenting normal BMC. The former showed a Ct inverse correlation as to age and mineralization indexes. Higher values of Ct and BMC have been reported in males rather than in females. Hence BMC is not suited to investigate different kinds of uremic osteodystrophy. Seric PTH dosage is certainly best fitted to discriminate patients affected with hyperparathyroidism from those with low turnover osteodystrophy. BMC determination is a valid support to evaluate the bone mineral loss in patients on haemodialysis treatment. It significatively correlates to the duration of the dialytic treatment; it is higher in female than in male population; it mainly affects cortical components rather than trabecular ones and is related to a seric Ct decrease.
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PMID:[Determination of bone mineral content and correlations with calciotropic hormones in periodic hemodialysis patients]. 129 8

Chronic renal failure (CRF) in the young is complicated by, among other conditions, growth retardation, hyperparathyroidism and uremic osteodystrophy. Many children with CRF are now being treated with growth hormone (GH). Since GH has a direct mitogenic effect on osteoblasts in culture, we studied the effects of GH therapy on osteoblastic activity, such as serum alkaline phosphatase (AP), bone GLA-protein (BGP) and bone mass density (BMD) in poorly growing children with and without CRF. Fifteen (4 girls, 11 boys) healthy children with short stature (SS) and 10 (3 girls, 7 boys) children with end-stage renal failure (CRF) 4.5-12.4 years of age were treated with daily subcutaneous injections of GH in a dose of 0.1-0.125 IU/kg/day for 1 year. IGF-I, BGP and BMD of the spine were determined before and after the year of treatment. During GH therapy, a similar increase in height velocity and IGF-I were noted in SS and CRF groups: 3.8 +/- 0.77 to 8.38 +/- 1.25 (p < 0.001) vs. 4.0 +/- 0.6 to 7.14 +/- 1.3 cm/year (p < 0.001) and 7.8 +/- 2.6 to 21.8 +/- 7.5 (p < 0.01) vs. 7.9 +/- 1.3 to 21.5 +/- 5.6 nmol/l (p < 0.01), respectively. AP increased from 205 +/- 27 to 274 +/- 50 IU/l (p < 0.01) in the SS group but not in CRF patients (223 +/- 58 pre- 218 +/- 51 IU/l post-GH therapy).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Effect of growth hormone therapy on IGF-I, bone GLA-protein and bone mineral content in short children with and without chronic renal failure. 130 46

Parathyroid hormone (PTH), osteocalcin and alkaline phosphatase (AP) were investigated before and after parathyroidectomy in 12 patients receiving hemodialysis. Early post-parathyroidectomy, PTH decreased (p less than 0.001), AP increased (p less than 0.05), but osteocalcin remained unchanged. At 3 months, osteocalcin and AP declined. A negative correlation was observed between aluminum staining and post-parathyroidectomy osteocalcin. In conclusion, early post-parathyroidectomy, osteocalcin and AP reflect persistent osteoblastic activity, which declined after 3 months. In patients receiving hemodialysis both variables may represent different aspects of osteoblastic activity and osteocalcin allows mixed uremic osteodystrophy after parathyroidectomy.
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PMID:Evolution of osteocalcin, alkaline phosphatase and parathyroid hormone after parathyroidectomy in patients receiving chronic maintenance hemodialysis. 157 55

Fifty-nine chronic hemodialysis patients who had been on dialysis for an average of 77 months underwent bone biopsies and the pathologic findings were correlated with biochemical and demographic data. All but two had evidence of renal osteodystrophy, 23 with osteitis fibrosa (OF), 19 with osteomalacia and/or adynamic disease (OM/AD), and 15 with mixed osteodystrophy (MOD). Patients in each group were similar with regard to age, sex distribution, duration of dialysis, unstimulated serum aluminum, calcium and phosphorus. Patients with osteitis fibrosa (OF) had statistically higher DFO stimulated aluminum, alkaline phosphatase and PTHC levels than the other two groups although there was marked individual variation. The bone biopsies were also evaluated for the amount of aluminum deposited in the osteoid seam. All 23 of the patients with OF and 11 of the 15 patients with MOD had no, mild, or minimal aluminum deposition but 12 of the 19 patients with OM/AD had moderate to marked aluminum deposition. Patients with minimal to mild aluminum deposition were similar in age, duration of dialysis, sex distribution, unstimulated and DFO stimulated aluminum levels, calcium, phosphorus, alkaline phosphatase to those with moderate to marked deposition but had significantly higher parathormone levels. All patients had been treated in a similar fashion regarding diet, oral phosphate binders and vitamin D; therefore, the observed differences in bone pathology were not readily explicable. However, patients who were found to have osteitis fibrosa and those with minimal to mild aluminum deposition had significantly higher parathormone levels when compared with patients in the other groups at the inception of dialysis.
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PMID:The clinical spectrum of renal osteodystrophy in 57 chronic hemodialysis patients: a correlation between biochemical parameters and bone pathology findings. 201 18

Congenital erythropoietic porphyria (CEP) is a rare disorder of heme biosynthesis that results in the production of large quantities of photoactive porphyrins. The clinical syndrome is dominated by extreme photosensitivity with mutilation of light exposed extremities and hemolytic anemia. Bone disease has been occasionally noted, but is not well characterised. We describe a man with CEP who developed bone pain and spinal crush fractures at the age of 22. Skeletal radiographs revealed features typical of other severe hemolytic anemias, but in addition there was loss of the terminal phalanges of the hand as a result of photomutilation. Spinal bone density (assessed by DPA) was reduced and at the hip bone density was at the lower limit of normal. The metacarpal cortical bone density was 2.9 standard deviations below normal. Biochemical and histological studies accelerated bone turnover. Although the serum 250H vitamin D concentration was very low (because of light avoidance) there was no evidence that the bone disease was a consequence of this. Treatment for one year with clodronate and a high transfusion regime was associated with small reductions in serum alkaline phosphatase and urine hydroxyproline excretion, but there was no improvement in bone mineral density. We conclude that CEP has a distinctive osteodystrophy comprising osteolysis of light-exposed extremities and a high turnover type of osteoporosis. Privational vitamin D deficiency may also occur. The effect upon bone of the new therapies for CEP should be considered.
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PMID:The osteodystrophy of congenital erythropoietic porphyria. 206 45


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