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)

During a 23 year period at Memorial Hospital, the diagnosis of liver cell carcinoma was made in 42 patients who were 11 to 40 years old. Ninety per cent were Caucasian, mostly born in the United states. No occupational hazard was detected. Serum hepatitis antigen was demonstrated in only one patient. Alpha fetoprotein was found in the serum of 55 per cent of nine patients tested. Eight-three per cent were Rh positive, 43 per cent were ABO groups, A or O, respectively. Twenty-three per cent of 13 patients with sufficient material for study had an associated cirrhosis. Of these, active hepatitis with cirrhosis was present in one patient; postnecrotic cirrhosis was present in another. Approximately 7 per cent had a history of previous liver disease. One patient had infectious mononucleosis, and nearly 13 per cent gave a family history of cancer. Weight loss or pain in the right upper abdominal quadrant was present in 65 per cent, and hepatomegaly was found in 88 per cent. Only one patient presented with hemoperitoneum simulating an acute condition within abdomen. The liver profile examinations characteristically revealed an elevation in serum alkaline phosphatase, 5 nucleotidase, and Bromsulphalein retention with normal bilirubin level. The most common finding, upon roentgenographic examination, was an elevated right hemidiaphragm. Selective celiac and superior mesenteric angiography and 99mTc sulfur colloid liver scans were both done in 13 patients. There was a 75 per cent accuracy rate in localization of the tumor. At laparotomy, the tumor was found to be confined to one lobe in seven patients and involved both lobes in ten. Twenty-seven patients were thought to have multicentric tumors and 15 unicentric lesions. Only ten were found to be candidates for hepatic lobectomy. Five and ten years survival rates were 20 per cent; the operative mortality rate was 40 per cent. Twenty per cent died within a year, ten per cent, one patient, is alive with disease at 28 months and another is free of disease at 31-months. Paraneoplastic syndromes were erythrocytosis in two patients, terminal stage of hypoglycemia in one patient, and hypocholesterolemia with associated excess beta globulin in one patient.
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PMID:Liver cell carcinoma during the prime of life. 17 34

A 22-year-old black male presented with erythrocytosis and proteinuria. The erythrocytosis was characterized by increased red cell mass, normal arterial oxygen saturation, and normal hemoglobin electrophoresis and oxygen affinity. There was no splenomegaly, and the white blood cell count, platelet count, serum uric acid concentration, serum B12 levels and leukocyte alkaline phosphatase activity were normal. Tumors of the liver, lung, kidney and cerebellum, which have been associated with erythrocytosis, were not found. The only associated disease was biopsy proven focal glomerulosclerosis. Renal vein thrombosis was excluded by renal venography and arteriography. This case illustrates the rarely reported association of the nephrotic syndrome and erythrocytosis. Other nephrogenic causes of erythrocytosis are mentioned, including renal cysts, tumors, renal artery stenosis and transplantation. The role of the kidney in erythropoietin production and possible mechanisms of nephrogenic erythrocytosis are discussed.
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PMID:Focal glomerulosclerosis and erythrocytosis. 50 18

In a study of 30 patients with hypernephromas, 23 patients manifested systemic effects of the tumor, and in 5 of these, the systemic effects were the presenting feature that led to the diagnosis. In contrast to this, only 17 patients had urologic complaints, and no single patient in this study had the classic triad of hematurial, loin pain, and mass. Weight loss (52 per cent), pyrexia, and elevated sedimentation rate (36 per cent) were seen most frequently. Anemia was seen in 25 per cent of patients. Other features seen in this group wer abnormalities in liver function, elevated alkaline phosphatase, hypertension, erythrocytosis, and hypercalcemia. In the majority of instances, removal of tumor was associated with remission of these effects. The effects were classified as those of a general toxic nature, those due to normal or abnormal production of hormones, and those due to production of abnormal substances by tumor cells. The evaluation of these effects was useful in making an early diagnosis and in follow-up care.
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PMID:Systemic effects of hypernephroma. 89 63

A case of blastic transformation of essential thrombocythemia (ET) is reported. A 69-year-old male was first admitted to hospital because of fever in February, 1982. He was diagnosed as having ET because of marked thrombocytosis (205.5 x 10(10)/1), absence of erythrocytosis, absence of splenomegaly, normal karyotype and no increment of blasts in the bone marrow, and normal levels of neutrophil alkaline phosphatase, vitamin B12 and folate. He was treated with busulfan, and subsequently his platelet count was well controlled for about five years. At the second admission, blasts were present in the peripheral blood, and later accounted for 49% of the total leukocyte count. Histological examination of a bone biopsy specimen showed homogeneous proliferation of blastic cells and slight reticulin fibrosis. At autopsy, the degree of bone marrow fibrosis had increased. This was considered to be a very rare case of ET with blastic transformation in the terminal phase.
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PMID:Blastic transformation of essential thrombocythemia. A case report. 258 80

Symptomatic erythrocytosis developed in a 59-year-old man with polycystic kidney disease (PKD) while he was receiving maintenance hemodialysis. Major clinical and laboratory data suggested a diagnosis of polycythemia vera (PV), despite a normal serum alkaline phosphatase level and leukocyte count. Secondary erythrocytosis, related to chronic hypoxemia and increased erythropoietin production, was excluded by appropriate laboratory studies. Despite previous documentation of secondary erythrocytosis in patients receiving hemodialysis, to my knowledge, PV has not been described in this population.
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PMID:Polycystic kidney disease and polycythemia vera. Occurrence in a patient receiving hemodialysis. 661 11

We describe two patients who had similar features of erythrocytosis and marked leukocytosis but who were subsequently classified as having chronic myelogenous leukemia and polycythemia vera, respectively, using leukocyte alkaline phosphatase scores and cytogenetic studies to detect the Philadelphia chromosome. The patient diagnosed as having polycythemia vera had a leukocyte count of 164,000/cu mm, which remained at similar levels when the patient was not in remission. We believe this is the first well documented case of PV with a leukocyte count above 150,000/cu mm.
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PMID:Erythrocytosis and marked leukocytosis in overlapping myeloproliferative diseases. 695 6

There is no single diagnostic marker for the only known type of primary acquired erythrocytosis, polycythemia vera (PV). The Polycythemia Vera Study Group (PVSG) used a combination of major and minor diagnostic criteria. However, these guidelines have some limitations and in the presence of newer diagnostic tools, have been re-evaluated. The recommendations of the Radionuclide Panel of the International Council for Standardization Hematology based on surface area are recommended over red blood cell mass (RCM) mL/kg expressions. Absolute erythrocytosis can be assumed in males and females with packed cell volume (PCV) values greater than 0.60 and greater than 0.56, respectively. A satisfactory strategy of investigation for a secondary erythrocytosis must be used. Hypoxemia, as well as renal and hepatic pathology, must be excluded. In unexplained absolute erythrocytoses, pO(2)(50) values and serum erythropoietin (EPO) levels should be examined. The latter can be disappointing in the confirmation of a secondary erythrocytosis, but elevated values contraindicate a diagnosis of a primary erythrocytosis. Establishment of a clonal marrow population supports a diagnosis of PV. Thus an acquired karyotypic abnormality is a major criterion. Palpable splenomegaly remains an important diagnostic marker. Scanning techniques to demonstrate splenic enlargement should be used with caution. Allowance must be made for interobserver and intraobserver differences and variation in normal spleen size with age and size of the subject. Splenomegaly demonstrated in this way should be taken as a minor criterion. An increased neutrophil count (>10 x 10(9)/L and >12.5 x 10(9)/L in smokers) is readily measurable and should replace total white blood cell count. The error in measurement of neutrophil alkaline phosphatase (NAP) score is large, making it an unsuitable diagnostic criterion. Neutrophil and platelet counts (>400 x 10(9)/L) should be taken as separate minor criteria. Endogenous erythroid colonies (EEC) grown from the peripheral blood have been used as a marker of PV, but it is an expensive technique that is not standardized and not totally specific for PV. Low serum EPO values found in the majority of patients with PV should hold a linked minor criterion position with EEC. Expert opinions should be obtained if bone marrow histology is to be used in the diagnosis of PV, but histology holds an important role in confirming the diagnosis. Semin Hematol 38(suppl 2):21-24.
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PMID:Evaluation of diagnostic criteria in polycythemia vera. 1124 98

IgA nephropathy (IgAN) is one of the most frequent forms of glomerulonephritis (GN). However, its association with polycythemia vera (PV) has rarely been described. We report a case of IgAN combined with PV. The patient was a 46-year-old male with chronic renal failure, heavy proteinuria and erythrocytosis. He also presented hypertension and hematuria as well as splenomegaly, high arterial oxygen saturation and elevated leukocyte alkaline phosphatase activity. Possible causes of secondary erythrocytosis were ruled out. The renal biopsy revealed mesangial proliferative GN with predominant IgA deposition in mesangium. He was diagnosed as having IgAN and PV concomitantly. After administration of hydroxyurea, enalapril and felodipine, blood cell count and blood pressure normalized, while azotemia persisted. There was also a partial remission of the heavy proteinuria. We describe a case of IgAN associated with PV, and possible pathophysiologic relationships between two diseases are discussed with review of the literature.
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PMID:IgA nephropathy in a patient with polycythemia vera. Clinical manifestation of chronic renal failure and heavy proteinuria. 1216 77

A 39-yr-old man presented to our hospital with unexplained erythrocytosis and hypertension. His follow-up for erythrocytosis had begun 2 yr earlier in another hospital and he had been diagnosed with polycythemia rubra vera. On admission to our hospital he was hypertensive (165/95 mmHg) and, except for the presence of moon-like face and facial plethora, his physical examination was normal. His hemoglobin concentration was 19.2 g/dl, and hematocrit was 58.9% with an increased red blood cell mass of 58 ml/kg as measured by radioisotope (Cr51). Blood film, other hematological indices except for elevated leukocyte alkaline phosphatase score, arterial gas analysis, and examination of aspirated bone marrow were all normal. An abdominal ultrasonography showed no evidence of splenomegaly. A diagnosis of probable secondary erythrocytosis was made. Early-morning serum cortisol and 24-h urinary free cortisol concentration as well as serum ACTH were high. Serum cortisol was not suppressed by low-dose dexamethasone, but suppressed by high-dose dexamethasone. Pituitary magnetic resonance imaging showed no lesion. After inferior petrosal sinus sampling suggesting right-central ACTH secretion, the patient underwent transnasal-transsphenoidal pituitary adenomectomy. Both hypercortisolemia and erythrocytosis regressed completely after the adenomectomy. After the operation, the patient's hemoglobin concentration and hematocrit decreased steadily, and 1 month post-adenomectomy his hemoglobin is 14.9 g/dl and hematocrit 44.8%. Thus, Cushing's syndrome should be a routine part of evaluation of unexplained polycythemia.
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PMID:Polycythemia as the first manifestation of Cushing's disease. 1909 3

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