Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: 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)

A case is reported of a patient, aged 20, with idiopathic hypoparathyroidism, manifested with the picture of epilepsy with a long treatment with antiepileptic remedies. Two years after the initiation of the antiepilertic treatment, severe skeleton disturbances occurred with manifested osteoporosis and multiple symmetric zones of bone reconstruction, hypocalcemia, hyperphosphatemia and elevated serum alkaline phosphatase. The disturbances of calcium and phosphorus level in blood are favourably affected by the application of relatively high vitamin D doses. The role of the long-term anticonvulsive treatment in the development of vitamin D resistant rickets and osteomalacia is discussed.
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PMID:[Bone metabolic disorders after prolonged treatment with antiepileptic agents in a patient with idiopathic hypoparathyroidism]. 101 20

The heights and symptoms of 52 patients, aged at least 18 years, with X-linked hypophosphataemic rickets were analysed retrospectively; 47 had been seen as children and 5 were adult at their first examination. 2 patients were lost to follow-up. 3 patients had died but their adult heights were known. There was no evidence that any form of treatment (ie, vitamin D in high doses, vitamin D plus phosphate supplements, or calcitriol plus phosphate) had any effect on adult height, symptoms, or alkaline phosphatase levels. There was a negative relation between adult height and the number of osteotomies undergone. The complications of treatment, such as renal failure, which occurred secondary to vitamin D intoxication in 3 patients in their twenties, may outweight any possible benefits. Until a treatment is established as effective in controlled trials, it may be better that these patients remain untreated.
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PMID:Hypophosphataemic rickets: final height and clinical symptoms in adults. 196 43

A single case of hypophosphatemic rickets with hypercalciuria and an elevated level of serum 1,25 dihydroxyvitamin D is reported. The characteristic features (genu valgum, rickets, short stature, increased renal phosphate excretion, decreased serum phosphorus level, elevated serum alkaline phosphatase level, and normal serum calcium level) were comparable to those in hypophosphatemic vitamin D resistant rickets. Massive doses of 1 alpha-hydroxyvitamin D were not effective for the rickets and the biochemical defect in this patient. Long-term phosphate supplementation on its own resulted in the reversal of all clinical and biochemical abnormalities except for the decreased ratio between the maximum tubular reabsorption rate for phosphorus and the glomerular filtration rate. In this patient, the concentration of serum 1,25 dihydroxyvitamin D seemed to be controlled by the concentration of serum phosphorus rather than by the serum parathyroid hormone level. It is noted that this is the first case of a single hypophosphatemic rickets with hypercalciuria.
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PMID:A single case of hypophosphatemic rickets with hypercalciuria. 376 Nov 17

Vitamin D-resistant rickets is characterized by short stature, lower extremity deformities, and defective mineralization of bone. While basic biochemical defects vary among involved individuals, all patients show a failure of the proximal tubule of the kidney to resorb inorganic phosphate. Laboratory findings consist of low serum phosphorus, elevated alkaline phosphatase, and abnormal serum calcium. Previously recommended treatment programs of high doses of vitamin D have effected some roentgenographic improvement in the rachitic lesions, but no related increase in height or severity of deformities has been associated with significant complications resulting from vitamin D toxicity. Daily administration of low doses of vitamin D and oral phosphates has more recently been suggested to be beneficial in promoting growth and preventing deformities. Thirteen children with documented vitamin D-resistant rickets were treated with oral phosphate and low doses of vitamin D for a mean of five years, and followed for a mean of ten years. Partial roentgenographic resolution of rachitic lesions was similar to those who received vitamin D alone. While the majority had consistently lowered alkaline phosphatases, no patient had consistently normal phosphate levels. No patient exceeded the third percentile in height. One half required osteotomies of the lower extremities. All osteotomies (eight) performed before maturity required revision, whereas those done after maturity (12) did not. Since no apparent clinical roentgenographic benefit can be documented by the addition of oral phosphate to low doses of vitamin D, we would not recommend continually doing so at this time.
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PMID:The effect of treatment on growth and deformity in hypophosphatemic vitamin D-resistant rickets. 627 46

Vitamin D-resistant rickets (VDRR) in adults is characterized by low serum phosphorus and osteomalacia. Despite the disappearance of rickets after the closure of epiphyses, some adults with VDRR present with symptomatic bone disease while other are asymptomatic. In order to test the presumption that asymptomatic adults no longer have active bone disease, we have compared bone histology in 10 symptom-free adults to 6 age-comparable symptomatic adults presenting with bone pain and persistent deformities. Both groups had similar low serum phosphorus and increased serum alkaline phosphatase values. Serum calcium, parathyroid hormone, and vitamin D metabolite concentrations were not different in the two groups. Histomorphometric study of bone formation and resorption was made on undecalcified sections of iliac crest bone biopsies obtained after in vivo single or dual tetracycline labeling. Bone histology revealed that both groups of patients had comparable osteomalacia, as evidenced by increased amount of osteoid tissue, prolonged mineralization lag time, and reduced bone formation rate. Despite the presence of osteomalacia, the trabecular calcified bone volume was within or above normal values in the two groups, implying a remodeling imbalance between the rates of bone resorption and formation. The data show that despite the absence of symptoms and the disappearance of rickets, adults with VDRR still have active bone disease characterized by moderate to severe osteomalacia. The normal to increased trabecular bone mass implies that the occurrence of painful symptoms results from factors other than trabecular osteopenia. These observations thus lead one to question the utility of active medical treatment with vitamin D and/or phosphate in asymptomatic adults with VDRR.
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PMID:Bone histomorphometry in asymptomatic adults with hereditary hypophosphatemic vitamin D-resistant osteomalacia. 630 50

Female rats were given 1,25-dihydroxyvitamin D3 (1,25(OH)2D3), 0.25 microgram per 100 g body weight (bw), 25-hydroxyvitamin D3 (25(OH)D3), 1.7 micrograms/100 g bw or 24,25-dihydroxyvitamin D3 (24,25(OH)2D3) 1.7 micrograms/100 g bw, subcutaneously three times a week for 12 weeks. Traditional variables pertaining to calcium homeostasis and growth, i.e. blood and urine calcium (Ca) and phosphate (P), serum levels of vitamin D3 metabolites parathyroid hormone, (PTH), calcitonin (CT), prolactin (PRL) and growth hormone (GH) were measured every four weeks. This data pool was correlated with bone matrix turnover parameters, i.e. serum levels of alkaline phosphatase (ALP) and urinary hydroxyproline (u-HYP) excretion. After 12 weeks of treatment, 1,25(OH)2D3 significantly enhanced serum total and ionized Ca, urine Ca and urine P, and also diminished urine cAMP due to reduced renal function (creatinine clearance). However, 25(OH)D3 administration had no such impact. 24,25(OH)2D3 opposed the effect of 1,25(OH)2D3 after 12 weeks by significantly augmenting serum P and diminishing serum levels of total Ca and ionized Ca. Cross sectional group analyses showed that circulating levels of ALP were directly related with serum 1,25(OH)2D3 and inversely related to serum 24,25(OH)2D3 and CT. Total u-HYP and per cent non-dialysable HYP (ndHYP) were reciprocally and positively correlated with serum PRL, respectively. However, no such relations were observed with serum GH. It appears that rats with elevated circulating levels of 1,25(OH)2D3 exhibit increased bone resorption, while augmented 24,25(OH)2D3 is associated with the opposite. Apparently, high bone turnover (i.e. reduced total urinary HYP and enhanced ndHYP) is associated with high serum PRL.
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PMID:Bone turnover in rats treated with 1,25-dihydroxyvitamin D3, 25-hydroxyvitamin D3 or 24,25-dihydroxyvitamin D3. 839 94

To evaluate bone change in severely disabled patients, bone density was assessed on X-ray pictures by the microdensitometry (MD) method, and urinary hydroxyproline (U-HYP), urinary glycosaminoglycans (U-GAG) serum calcium (S-Ca) and serum alkaline phosphatase (S-AP) were assessed in 43 patients (mean age 16 years) at the National Sanatrium Ehime Hospital. On division of the severity of bone change into grades 0 to 3 using the MD method. U-HYP was found to be significantly higher in grades 2 and 3 than in controls. Tendencies were similar for U-GAG and S-AP. U-HYP in immobile patients was significantly higher than in mobile patients. Immobilisation was one of the most important contributors to the development of bone change and results obtained using the MD method, U-HYP and U-GAG were valuable-indices of the bone change in severely disabled patients.
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PMID:Estimating bone change in patients with severe disability. 856 66

An intrinsic phosphate (Pi) transport defect in the proximal tubule (PT) presumably underlies X-linked hypophosphatemic rickets. We recently reported normal Pi transport in the S1 segment of the Hyp mouse PT. Whether Pi wasting results from an abnormality in the S2 or S3 segment remains unknown. Thus, we compared Pi transport in S2 and S3 immortalized cells from transgenic (simian virus 40) normal and Hyp mice. These cells display biochemical features of PT cells, including alkaline phosphatase- and hormone- stimulated cAMP activity as well as gluconeogenesis. Moreover, kinetic studies in S2 cells reveal a similar Km[0.26 +/- 0.03 (+/-SEM) vs. 0.22 +/- 0.03 mM] and maximum velocity (Vmax; 5.5 +/- 0.66 vs. 5.9 +/- 0.72 nmol/mg x 5 min) in normal and Hyp mice, respectively. Km and Vmax were also similar in cells from the S3 segment; however, the Vmax values in S3 cells in normal and Hyp mice (2.8 +/- 0.45 and 3.0 +/- 0.56 nmol/mg x 5 min) were reduced in both animal models compared to those in S2 cells (P < 0.001), whereas the Km values in S3 cells from normal and Hyp mice (0.10 +/- 0.02 and 0.11 +/- 0.04 mM) were increased relative to those in S2 cells (P < 0.001). These data indicate that Pi transport throughout the PT of Hyp mice is intrinsically normal. Such observations exclude the presence of a nascent defect in renal Pi transport in the kidneys of Hyp mice and support the hypothesis that a humoral abnormality underlies X-linked hypophosphatemic rickets.
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PMID:Normal phosphate transport in cells from the S2 and S3 segments of Hyp-mouse proximal renal tubules. 860 7

We compared serum levels of total alkaline phosphatase (TAP) and bone-specific alkaline phosphatase (BAP) as determined by three different assays (lectin affinity electrophoresis, immunoradiometric assay, enzyme-linked immunosorbent assay) in subjects aged 5-20 years suffering from X-linked hypophosphatemic rickets (n = 14), chronic renal failure (n = 10) and chronic cholestatic liver disease (n = 16). Results were compared to controls of the same age and were expressed as standard deviation scores (SDS). TAP correlated significantly with BAP (r > 0.9 for each assay; p < 0.001) in controls. In children with cholestatic diseases, TAP (median SDS + 2.0) was elevated, but BAP, as measured by the electrophoretic assay, was within the reference range for most patients (median SDS: -0.4; p = 0.003 for the difference between the median SDS of TAP and BAP). In contrast, results for BAP as determined by the two immunoassays were not significantly different from TAP in any of the three patient groups (p > 0.05 in each group for both assays). In this study, the two immunoassays did not have a detectable advantage over lectin affinity electrophoresis in the determination of BAP.
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PMID:Comparison of total alkaline phosphatase and three assays for bone-specific alkaline phosphatase in childhood and adolescence. 920 91

Silymarin (SIL), a standardized plant extract containing about 60% polyphenole silibinin, is used as a hepatoprotective agent. Its antifibrotic potential in chronic liver diseases has not been explored. Therefore, we applied SIL to adult Wistar rats that were subjected to complete bile duct occlusion (BDO) by injection of sodium amidotrizoate (Ethibloc). This treatment induces progressive portal fibrosis without significant inflammation. Rats with sham-operation that received SIL at 50 mg/kg/d (n = 10) and rats with BDO alone (n = 20) served as controls, whereas groups of 20 animals were fed SIL at a dose of 25 and 50 mg/kg/d during weeks 1 through 6 or doses of 50 mg/kg/d during weeks 4 through 6 of BDO. Animals were sacrificed after 6 weeks for determination of blood chemistries, total and relative liver collagen (as hydroxyproline [HYP]), and the serum aminoterminal propeptide of procollagen type III (PIIINP). BDO in untreated rats caused an almost ninefold increase in total liver collagen (16.1 +/- 3.1 vs. 1.8 +/- 0.4 mg HYP, P < .001). SIL at 50 mg/kg/d reduced total HYP by 30% to 35%, either when given from week 1 through 6 or from week 4 through 6 after BDO (10.6 +/- 2.7 and 10.2 +/- 3.9 mg HYP, both P < .01 vs. BDO alone), whereas 25 mg/kg/d were ineffective. Because SIL at 50 mg/kg/d also reduced the collagen content per gram of liver tissue, it acted as a true antifibrotic agent. The single value of PIIINP at killing paralleled the antifibrotic activity of SIL with 11.6 +/- 3.8 and 9.9 +/- 3.7 vs. 15.3 +/- 5.2 microg/L in both high-dose groups (P < .05 and P < .01, respectively, vs. rats with BDO alone). Except for a decreased alkaline phosphatase and a lower histological fibrosis score in the groups that received SIL, clinical-chemical parameters were not different among all groups with BDO. We therefore conclude that 1) BDO with Ethibloc is a suitable model to test for pure antifibrotic drugs because it induces progressive rat secondary biliary fibrosis without major inflammation; 2) oral SIL can ameliorate hepatic collagen accumulation even in advanced (biliary) fibrosis; and 3) PIIINP appears to be a suitable serum marker to monitor the inhibition of hepatic fibrogenesis in this model of biliary fibrosis.
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PMID:Silymarin retards collagen accumulation in early and advanced biliary fibrosis secondary to complete bile duct obliteration in rats. 1117 60


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