Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fifteen adult patients undergoing allogeneic bone marrow transplantation (BMT) received cyclosporine (CSP) as prophylaxis of graft versus host disease (GVHD). In our patients eleven were hematologic malignancies, and four were severe aplastic anemia. Twelve patients were HLA-matched, and three were one locus mismatched. Three patients received CSP only, twelve received CSP and short term methotrexate. Seven patients had acute GVHD, but GVHD over Grade II were seen in only 3 patients who were transplanted from HLA-one locus mismatched donor. 7 patients had chronic GVHD. CSP were given intravenously at 3-5 mg/kg, starting 1 day before BMT. From about day 30, CSP was given orally. CSP concentrations when patients were given orally were lower in patients who had chronic GVHD. Although hypertension and water retention were seen in 8 patients, and renal dysfunction was seen in 3 patients, the side effects of CSP were mild and transient. There were no correlations between serum concentrations and the side effects of CSP. Three patients had the disturbance of hematopoiesis. Ten of fifteen patients are alive at median follow-up of 18.5 months (8-41 months) after BMT.
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PMID:[Cyclosporine as prophylaxis for graft versus host disease in adults undergoing allogeneic bone marrow transplantation]. 267 37

Intracranial saccular aneurysms have been a well-known clinical and pathological entity for over two centuries. The pathophysiological events that lead to aneurysm formation and rupture are, however, poorly understood. Besides an HLA-associated genetic factor, the most widely accepted risk factors are arterial hypertension, female gender, and increasing age. Some aneurysm patients have a deficient formation of Type III collagen. This seems to interfere with the mechanical integrity of the cerebral arterial wall encouraging aneurysm formation. While some of the risk factors may be involved in the process of aneurysm formation, others may be of importance in the actual aneurysm rupture. Medical and surgical developments have only had a slight impact on mortality rates from aneurysm rupture. The principal cause of death and disability is cerebral arterial spasm. Considerable effort has been expended in investigating the etiology of this phenomenon. Previous studies have failed to yield conclusive evidence of the causative agent(s) or the nature of cerebral artery narrowing. The time course of vasospasm after the onset of subarachnoid hemorrhage is consistent with an immune-mediated response, and more recent observations suggest that immunological processes including activation of the complement system may be involved. Missed minor bleeding episodes may thus be a risk factor for aneurysm patients in respect to the development of cerebral vasospasm.
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PMID:Risk factors in intracranial saccular aneurysms. Aspects on the formation and rupture of aneurysms, and development of cerebral vasospasm. 268 56

MDL-899 is new phthalazine derivative which has been developed as a substitute for hydralazine, which has several undesirable effects, in the treatment of hypertension. The effects of MDL-899 on human lymphocyte functions are analyzed. This drug inhibited in a dose-related fashion the blastogenesis of lymphocytes upon PHA and Con A activation and down-regulated the activation of allogeneic mixed lymphocyte reactions (MLR). On the contrary the drug enhanced the activation of autologous MLR of non T/T type. This effect was five times higher on cells which carried the HLA DR 4 phenotype. The above reported observations suggest that MDL-899, as well as hydralazine, affects the in vitro responsiveness of human lymphocytes mostly in subjects with HLA-DR 4 phenotype. Whether the impact of MDL-899 on immune function gives rise to a lupus like syndrome is not known. For this reason further studies are warranted to assess its long-term in vivo effects.
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PMID:The effects of a new phthalazine derivative (MDL 899) on human lymphocyte functions. 294 87

Diabetes secondary to pancreatic disease (PD) represents a useful model for the study of the effects of chronic hyperglycemia on microangiopathic complications in the absence of those genetic factors predisposing to Type I diabetes. Our aim was to evaluate the prevalence of nephropathy and retinopathy in a group of 86 patients with PD. The genetic pattern, assessed by the determination of HLA antigens, was different than in patients with Type I diabetes. A family history of diabetes was present in 53% of the patients. The prevalence of retinopathy was 37%. Eighteen percent of the patients with duration of diabetes less than 10 years showed an albumin excretion rate (AER) greater than 40 mg/24 hr. The prevalence of pathologic microalbuminuria (greater than 40 mg/24 hr) was found in 29% of the patients with duration of diabetes greater than 10 years. The prevalence of pathologic microalbuminuria is related to the duration of diabetes. Both diastolic and systolic blood pressure is positively correlated to albumin excretion rate (p less than 0.02), suggesting a possible role of hypertension in the evolution of nephropathy. Sixty-one percent of the patients with AER greater than 40 mg/24 h had retinopathy, thus confirming the close association between renal and ocular complications. Abnormal microalbuminuria and retinopathy were not influenced by a family history of diabetes. We conclude that the prevalence of microangiopathic complications is similar to that seen in Type I diabetes, and the metabolic abnormalities of diabetes can play a direct role in the development of diabetic microangiopathy.
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PMID:Prevalence of microangiopathic complications in hyperglycemia secondary to pancreatic disease. 296 58

Studies in three families (A, B, and C) revealed five patients with congenital adrenal hyperplasia (CAH) due to partial and combined 21- and 11 beta-hydroxylase deficiency. One patient (A-11 1), a 23-yr-old severely virilized chromosomal female, was reared as a male, and two females (B-11 2 and C-1) complained only of hirsutism, acne, and menstrual abnormalities. Patients A-11 2 and B-11 8 (17 1/2 and 10 yr old) were asymptomatic and detected by finding an HLA genotype identical to that of their respectively affected brother and sister. Three patients (A-11 1, A-11 2, and C-1) had moderate hypertension. In spite of the wide range of clinical manifestations, all individuals had elevated androgen levels, while cortisol secretion was severely impaired only in A-11 2. 21-Hydroxylase deficiency was diagnosed on the basis of markedly increased plasma and urinary levels of 17-hydroxyprogesterone (17-OHP) and 21-deoxycortisol and their respective urinary metabolites pregnanetriol and pregnanetriolone. PRA was elevated in three patients, while urinary aldosterone was normal or increased. 11 beta-Hydroxylase deficiency was diagnosed on the basis of increased 11-deoxycortisol and deoxycorticosterone in plasma and tetrahydro-11-deoxycortisol and deoxycorticosterone in urine, particularly after ACTH administration. In contrast to classical 11 beta-hydroxylase deficiency CAH, urinary 18-hydroxycorticosterone and 18-hydroxy-11-deoxycorticosterone were normal or elevated. The nature and mechanism of a combined enzymatic defect are unknown. The coincidental presence in a single individual of the mutant genes for both 21- and 11 beta-hydroxylase deficiency CAH is very unlikely to occur. Two alternative hypotheses may explain our findings. One is the existence of a genetically inherited abnormal (or aberrant) 11 beta-hydroxylase, whose affinity for its normal substrate is changed for an abnormal one (17-OHP). As a result, 11 beta-hydroxylation of 11-deoxycortisol is deficient while 17-OHP 11 beta-hydroxylation is markedly enhanced. Thus, both 11-deoxycortisol and 21-deoxycortisol as well as their urinary metabolites accumulate. The ability for 18-hydroxylation, however, remains normal. In this case, 21-hydroxylase is not deficient, yet 21-deoxycortisol cannot be further hydroxylated to cortisol, since this steroid is not a suitable substrate for the enzyme. Such a disorder may represent a new allelic variant of 11 beta-hydroxylase deficiency CAH, which, similar to 21-hydroxylase deficiency, is completely linked to the HLA complex.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Combined 21- and 11 beta-hydroxylase deficiency in familial congenital adrenal hyperplasia. 298 4

A profile of dexamethasone-suppressible hyperaldosteronism (DSH), a variant of primary aldosteronism, is drawn by reviewing its pathophysiological and clinical aspects. Genetic studies show no HLA linkage and point to an autosomal dominant mode of inheritance, suggesting that the prevalence of this disease has been underestimated in the past. Hypertension, hypokalemia, suppressed renin, and high aldosterone values characterize DSH in the basal state, similar to the other forms of primary aldosteronism, i.e., aldosterone-producing adenoma (APA) or bilateral idiopathic adrenal hyperplasia (IAH). Biochemically DSH and APA can be differentiated from IAH since in both aldosterone does not respond to upright posture, to angiotensin II infusion, and to angiotensin-converting enzyme (ACE) captopril. In contrast, morphologically DSH is similar to IAH, since neither macroscopic nor histologic examinations of the adrenals give evidence of any unilateral abnormality. However, DSH is differentiated from APA and IAH by the hyperresponsiveness of aldosterone to acute ACTH administration as well as by the failure of aldosterone to escape from prolonged ACTH stimulation. The final diagnosis of DSH rests upon the prompt reversal of the features of mineralocorticoid excess by glucocorticoid therapy. In some cases hypertension is unresponsive to dexamethasone and needs alternative treatment. The main pathogenetic hypotheses point to a pituitary and/or an adrenal abnormality, but the intrinsic nature of the disease remains to be elucidated.
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PMID:Dexamethasone-suppressible hyperaldosteronism: pathophysiology, clinical aspects, and new insights into the pathogenesis. 303 79

Atherosclerosis, aorto-arteritis and fibromuscular dysplasia are the most common causes of vasorenal hypertension. Determination of plasma renin activity is a valuable diagnostic test at early stages of vasorenal hypertension. HLA studies demonstrated significantly elevated antigens B8 and B12 in patients with essential hypertension, and antigen A9 in patients with affected renal arteries. These findings may expand the possibilities of differential diagnosis for the selection of patients, eligible for angiographic investigation. A less than three-years duration of the disease in the presence of high plasma renin activity is a favorable prognostic criterion.
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PMID:[Ways of improving the diagnosis and prognosis in vasorenal hypertension]. 307 42

CsA-Pred therapy yields equivalently good patient survival for LRD and 2 degrees CAD versus 1 degree CAD transplants. There is a long-term graft survival advantage for LRD versus 1 degree CAD transplants (5 years; 83% vs 58%). 2 degrees CAD transplants have inferior graft survival when compared with 1 degree CAD grafts (one year; 78% vs 67%). Multiple donor factors adversely affecting graft outcome include increased warm and cold ischemia times, pulsatile perfusion, use of pressors or diuretics in the donor, donor age less than 10 years, donor blood transfusions, and kidneys shipped from other centers. Recipient factors adversely affecting graft outcome include retransplantation and CMV infection as well as noncompliance with therapy. HLA-matching and pretransplant blood transfusions have not contributed in a statistically significant way to graft outcome although they may affect the quality of graft function at this center. Immunosuppressive therapy with CsA-Pred must be tailored to the individual patient. Continuous IV CsA infusion in the preoperative period and slow steroid taper impact favorably on graft outcome. The complications of CsA therapy include neuroectodermal toxicity, hepatotoxicity, and most importantly, nephrotoxicity. Other problems unique to CsA-Pred therapy include hypertension, delayed graft thrombosis, and de novo hemolytic uremic syndrome. Hepatotoxicity may eventuate in biliary and pancreatic complications necessitating surgical therapy. The overall incidence of infection and neoplasm remains low with CsA-Pred therapy. The use of therapeutic trough CsA level monitoring, as well as pharmacokinetic and pharmacodynamic analyses may assist in clinical decision making regarding administered doses, dosing interval, and discrimination between rejection and nephrotoxicity.
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PMID:Factors determining renal transplant outcome at the University of Texas at Houston. 315 93

Patients with haematological malignancies with HLA-identical marrow donors were randomized to treatment with cyclosporin (CSA) or methotrexate (MTX). Two of the 29 patients randomized to MTX died before engraftment compared with none of the 30 treated with CSA. Engraftment by leucocytes (P less than 0.0001), granulocytes (P less than 0.02), and reticulocytes (P less than 0.01) was faster among the CSA patients. There were no significant differences between the two groups regarding transfusions, hospitalization and incidence of early septicaemia. Granulocyte transfusions were required in seven of 29 MTX and two of 30 CSA patients (not significant: NS). Overall (grade I-IV) acute graft-versus-host disease (GVHD) was more common (P = 0.001) in the CSA patients. Grade II-IV acute GVHD was seen in 40% of the CSA patients compared with 22% in the MTX patients (NS). In the adult patients grade II-IV GVHD was slightly more common (P less than 0.05) in those treated with CSA compared with MTX. Chronic GVHD appeared in 30 and 39% in the two groups respectively. Actuarial 3-year survival was 58% for the CSA patients and 69% for the MTX patients. There were no significant differences regarding the incidence of interstitial pneumonitis or relapses between the two groups. The side-effects of CSA treatment includes nephrotoxicity (83%), hepatotoxicity (20%), hirsutism (43%), hypertension (23%), tremor (27%) and gingival hyperplasia (27%). Serum creatinine values were increased at 3 and 6 months in the CSA group but were within the normal range after 6 months. A blind study on oral side-effects revealed that CSA patients more often had a normal mucosa (P = 0.025) and less frequently had mucositis (P = 0.01) compared with the MTX group.
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PMID:A randomized trial comparing use of cyclosporin and methotrexate for graft-versus-host disease prophylaxis in bone marrow transplant recipients with haematological malignancies. 333 19

A case of double bilateral renal vessels in both twins of a MZ pair is reported for the first time. Even in non-twins, the anomalies reported so far involved the inferior polar arteries in agreement with the embryological development. The two male twins, examined at the age of 14 years, had simultaneously developed a marked hypertension at the age of 7 years. Zygosity was determined by blood group and HLA analysis and various clinical tests were carried out to diagnose the condition. It is suggested that the anomaly is the result of a genetically induced early block in embryonic development.
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PMID:Unusual vascular malformation of the kidney in both twins of a monozygotic pair: short case report. 345 22


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