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)

The authors describe a group of patients where the surgical operation involved among others filling of bone defects with glass-ceramic material--dense (as granules) or porous (as a block). Glass-ceramics BAS O were developed in the laboratories of LASAK Co. in Prague. Two defects were of traumatic origin and osteosynthesis was part of three operations. The remaining defects were juvenile bone cysts, fibrous dysplasia and benign bone tumours. The follow-up period after operation varied in the first 11 patients between 6 months and 2 years. In patients of the mentioned group no problems of healing of the surgical wound were recorded nor allergic and side-reactions. The incorporation of glass-ceramic material was followed up by X-ray after three-months intervals. In no instance lighter areas were found on the X-ray pictures suggesting a fibrous outer layer. On the contrary, the trabeculae reached gradually its close vicinity. Based on experience from experimental work and investigation of X-ray signs of healing, the patients were allowed to burden the operated extremity after three months. The basic laboratory examinations made in these patients were within the normal range. In particular calcium throughout the investigation period in the normal range, the phosphorus levels varied near the upper borderline, alkaline phosphatase levels were in many young patients elevated and acid phosphatases varied. In eight patients during the postoperative period eosinophilia was revealed in the haemogram.
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PMID:[Initial clinical experience with BAS O, a bioactive glass-ceramic material]. 828 13

Endoscopic diagnosis completed by biopsy achieved remarkable accuracy. New trends--endoscopy with the use of ultrasound orange red porphyrin fluorescence elicited by blue light e. g. krypton laser and strip biopsy not only greater accuracy of endoscopic methods but also open up new therapeutic possibilities. Histochemical and histoenzymatic methods allow to classify histological findings of the norm (presence of pepsinogen and neutral glycoprotein) of atrophic gastritis with intestinal metaplasia (acid glycoprotein, activity of sucrose, alkaline phosphatase-AP, leucin-aminopeptidase-LAP) and of dysplasia (presence of sulfomucin, decrease of AF, LAP, sucrose and trehalase activity) and to divide them into well defined groups. We are able to distinguish which mucosal changes are suspect, we know that malignant transformation can after a certain period of time be expected approximately 2-14% yet we still do not know whether the period from dysplastic change to malignant transformation is not longer than malignant change in resected stomach. Epidemiologic and experimental data gives us enough reasons for dietary and chemopreventive measures (apart of other treatment) especially in risk groups patients.
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PMID:[Early diagnosis of gastric cancer]. 850 56

The homozygous form of beta-thalassemia, the most common single gene disorder, is treated by red cell transfusion therapy. Following transfusion, the chelator, deferoximine, is administered to patients to remove excess iron. However, when this drug is given to young children, metaphyseal dysplasia and abnormalities of linear growth are frequently observed. To explore the notion that deferoximine interferes with endochondral growth by chelating zinc, we examined the effect of the drug on chondrocytes maintained in long-term culture. We found that deferoximine caused a dose-dependent inhibition of a wide range of functions including cell proliferation, protein synthesis (and possibly under-hydroxylation of type X collagen), and mineral deposition. Directly relevant to the mineralization process was the observation that the drug dramatically lowered the activity of alkaline phosphatase, a zinc-requiring enzyme. To test the hypothesis that enzyme inhibition was due to chelation of zinc by deferoximine, the cell culture medium was supplemented with excess zinc. However, this treatment did not overcome the deferoximine-dependent change in enzyme activity. We next examined the possibility that deferoximine, in the presence of ascorbate, could form a free radical system that would serve to inactivate the enzyme. Using alkaline phosphatase extracted from chick cartilage, we noted that the activity of the phosphatase was markedly reduced in the presence of deferoximine and ascorbate. These effects were consistant with the notion that deferoximine and ascorbate can act as a prooxidant couple. This conclusion was confirmed when we measured the oxidative activities of the system using nitrobule tetrazolium and cytochrome c. Indeed, we noted that deferoximine markedly activates the autocatalytic oxidation of ascorbate.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Effects of deferoximine on chondrocyte alkaline phosphatase activity: proxidant role of deferoximine in thalassemia. 857 42

We studied the osteoblastic abnormalities resulting from activating mutation of the Gs alpha gene in two patients with McCune-Albright syndrome and one patient with monostotic fibrous dysplasia. Histomorphometric analysis of dysplastic lesions showed a low number of differentiated osteoblasts along the bone surface and numerous immature alkaline phosphatase-positive mesenchymal cells actively depositing a woven bone matrix. Osteoblastic cells isolated from dysplastic bone lesions expressed a missense mutation in the Gs alpha gene in position 201 and showed increased intracellular basal cyclic adenosine 3',5'-monophosphate levels compared with normal cells isolated from a noninvolved area in the same patient. Cell proliferation evaluated by DNA synthesis was two-fold to threefold greater in osteoblastic cells expressing the mutation compared with normal cells from the same patient and was greater in cells isolated from more severe than less severe fibrotic lesions. In contrast, the synthesis of osteocalcin, a marker of mature osteoblasts, was lower in osteoblastic cells expressing the Gs alpha mutation compared with normal cells from the same patient and was lower in cells isolated from severe compared with less severe fibrotic lesions, indicating that the increased growth in mutated osteoblastic cells was associated with reduced cell differentiation. The results show that activating mutation of Gs alpha in osteoblastic cells leads to constitutive activation of adenylate cyclase, increased cell proliferation, and inappropriate cell differentiation, resulting in overproduction of a disorganized fibrotic bone matrix in polyostotic and monostotic fibrous dysplasia.
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PMID:Increased proliferation of osteoblastic cells expressing the activating Gs alpha mutation in monostotic and polyostotic fibrous dysplasia. 906 Aug 42

Morquio syndrome (mucopolysaccharidosis IV) presents with multiple bone dysplasia and is characterized by the inability to degrade keratan sulfate due to deficient N-acetylgalactosamine-6-sulfate sulfatase in Morquio A syndrome and deficient beta-D-galactosidase in Morquio B syndrome. The aim of our study was to investigate into the pathogenetic mechanism as it is not clear whether the accumulation of keratan sulfate is toxic for osteoblasts or inhibits osteoblast activity as e.g. bone resorption. The glycosaminoglycans keratan sulfate, heparan sulfate, dermatan sulfate, chondroitin-4,6-sulfate and hyaluronic acid were tested in rat neonatal calvarian cultures for their effects on bone resorption, osteoblast activity and toxicity. Bone resorption was evaluated by calcium release into the medium, osteoblast activity by the determination of alkaline phosphatase and toxicity by measuring lactate dehydrogenase in the culture media. Keratan sulfate had no effect on bone resorption but inhibited osteoblast activity at the low, nontoxic concentration of 10 ng per ml organ culture supernatant significantly (p<0.05). At a concentration of 100 ng per ml keratan sulfate revealed toxic effects as reflected by significantly (p<0.05) elevated lactate dehydrogenase activity. None of the other glycosaminoglycans inhibited osteoblast activities. Heparan sulfate showed at toxic levels (10 microg per ml supernatant) significantly increased bone resorption (p<0.05) accompanied by increased alkaline phosphatase activity. The specific keratan sulfate effects of inhibiting osteoblast activity and toxicity towards bone, which were never tested before, suggest a role for this glycosaminoglycan in the pathogenesis of bone dysplasia in Morquio syndrome.
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PMID:The effects of acid glycosaminoglycans on neonatal calvarian cultures--a role of keratan sulfate in Morquio syndrome? 927 6

One hundred seventy two consecutive cases of female breast pathology have been intraoperatively processed by frozen sections (FS) in combination with aspecific alkaline phosphatase (AAF) histochemical evaluation. The corresponding histology on permanent embedded tissues (ET) was compared with both these techniques. The related theoretical and practical methodological aspects are presented and discussed. The consultations concerned the following histopathological types (WHO terminology): 136 malignant epithelial tumours(intraductal carcinoma: 11; invasive ductal carcinoma: 100; invasive lobular carcinoma: 8; medullary carcinoma: 3; mucinous carcinoma: 8; papillary carcinoma: 3; tubular carcinoma: 2); one cystosarcoma phyllodes; 19 benign tumours; 15 mammary dysplasias (fibrocystic disease); one tumour-like lesion(hamartoma); one fibromatous lesion. In terms of concordance/discordance between FS and ET diagnoses no false positive cases and only one false negative resulted. The diagnosis was deferred in 18 circumstances (10,5%). The AAF reaction in 64/153 (41,8%) cases (correctly interpreted on FS) displayed more histological evidence with special reference to the detection of the myoepithelial cells. The morphological implementation was particularly relevant as far as the differentiation between intraductal and microinvasive carcinoma, carcinomatous and papillomatous papillary proliferation, carcinomatous and non carcinomatous tubular structures, pseudocarcinomatous (florid and/or sclerosing) dysplasia and carcinoma is concerned. Analogously in 12/17 (70,6%) comparable deferred diagnoses as well as in the single false negative case the interpretation could have been correctly stated intraoperatively. The normal and pathological features as disclosed by the AAF reaction have been analytically examined. The AAF procedure seems to be a possible relevant tool in order to improve the classical FS performance also in respect to the increasingly systematic, precocious and conservative approach of the modern breast surgery.
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PMID:[Measurement of aspecific alkaline phosphatase in the intraoperative histological examination of the breast]. 931 32

Recent advances in molecular and cellular biology have contributed significantly to the elucidation of the pathogenesis of many kinds of skeletal dysplasia. The number of skeletal dysplastic diseases that are identified to have associated abnormalities in genes has increased. Some diseases such as achondroplasia, thanatophoric dysplasia and hypochondroplasia are shown to be allelic. In addition to those diseases associated with mutations of the fibroblast growth factor receptor 3 gene, the abnormalities in collagen, Gs alpha, vitamin D receptor and tissue nonspecific alkaline phosphatase genes are briefly reviewed in this article.
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PMID:Recent advances in molecular analysis of skeletal dysplasia. 931 99

Sclerosing bone dysplasias are diagnosed on the basis of a characteristic pattern of osteosclerosis and clinical manifestations; in many of them, cause and pathogenesis are still unknown. A 33-year-old man had five fractures of the humerus, tibiae, and femur as a result of mild traumatic incidents that occurred between the ages of 18 and 33 years as well as a remnant of rib fractures without apparent trauma on radiographs. His height was 158 cm (-2.2 SD). Radiographic evaluation showed cranial sclerosis, longitudinal striations in the metaphyses of the femur and tibia, fan-like striation in the ilium, metaphyseal widening in the femur and tibia, and sclerosis of the ribs. The blood chemistry findings, including serum calcium, phosphorus, and alkaline phosphatase, were normal. Biopsy from the ilium showed thick trabeculae composed of woven bone. The coexistence of osteopathia striata, cranial sclerosis, metaphyseal undermodeling, and bone fragility has not been recognized previously. Our case appears to represent a new form of sclerosing bone dysplasia.
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PMID:Unclassified sclerosing bone dysplasia with osteopathia striata, cranial sclerosis, metaphyseal undermodeling, and bone fragility. 955 97

Cammurati-Engelmann's Disease or Progressive Diaphyseal Dysplasia (PDD), is a rare autosomal dominant disorder, sometimes non hereditable, which begins in childhood, and is characterized by symmetrical excess of osseous apposition in diaphysis and metaphysis of long bones. In severe cases skull and vertebrae could be involved. Clinically, patients refer limb pain, muscular weakness and atrophy, easy fatigability and waddling gait. Later on S. Ribbing described an illness that he thought was a separate entity with sclerosis and enlargement of diaphysis of femora and tibiae, which begins after puberty, is less extensive, not always symmetric and without gait or neurological involvement. Some authors think it may be an adult form of the PDD. As no specific treatments are available we report one case of each entity, treated with the bisphosphonate pamidronate, by the oral route. A white female, 69 years old, with clinic and radiology of Ribbing's Disease, had positive scintigraphy in the affected areas and elevated bone biochemical markers: Serum alkaline phosphatase (SAP): 57 UKA. Total urinary hydroxyproline (THP): 60 mg/24 h. Bone Gla protein (BGP): 40 ng/ml. Considering the high bone turnover treatment with oral pamidronate, 400 mg/day plus Calcium 1g/day was started, dose was then progressively reduced. After two months pain almost disappeared, and THP became normal: 14 mg/24 h; with normalization of BGP values: 8 ng/ml, and a decrease of SAP: 21 UKA, 99mTc MDP uptake by affected bones decreased after 1 year of treatment. Because of these results we decided to begin treatment in a white female 17 years old, 32 kg weight, 1.47 m height with PDD characteristics and also a high bone turnover (THP: 95 mg/24 h. SAP: 32 UKA). After six months of Calcium 1 g/day, given with meals, and oral pamidronate 100 mg/day, she became painless with normal strength and gait, almost normalization of THP (48 mg/24 h). Although a small decrease of SAP, and no charges in scintigraphy. These results obtained with pamidronate suggest that it may be useful to treat dysplasias with high bone turnover.
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PMID:[Clinical, humoral and scintigraphic assessment of a bisphosphonate as potential treatment of diaphyseal dysplasia: Ribbing and Cammurati-Engelmann diseases]. 956 56

Hereditary hemorrhagic teleangiectasia, or M. Osler (Osler-Weber-Rendu disease), is an autosomal dominant, systemic fibrovascular dysplasia. This may lead to increased liver blood flow from arteriovenous fistulas. A 45-year-old woman with a known M. Osler was admitted for liver transplantation. On admission, exertional dyspnea was the predominant symptom. Radiological investigations revealed multiple intrahepatic arteriovenous fistulas and consecutive high-output heart failure. Laboratory findings revealed remarkably elevated bilirubin and alkaline phosphatase. To alleviate the high-output cardiac failure, the hepatic artery was ligated. Fourteen months later, the patient presented again with increased levels of bilirubin and recurrent bleeding episodes from esophageal varices grade IV. The patient underwent liver transplantation and post-transplant recovery was excellent. A hyperdynamic circulatory state due to a hepatic M. Osler has been treated in several cases by ligation or embolization of the hepatic artery. This procedure, however, is recommended only for patients with normal liver function and carries a considerably risk of bile duct necrosis.
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PMID:Effective therapy for hepatic M. Osler with systemic hypercirculation by ligation of the hepatic artery and subsequent liver transplantation. 970 2


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