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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thrombotic microangiopathy most likely represents a spectrum of diseases consisting of multiple etiologies that has a final common pathway of multiorgan microvascular thrombosis. The variable responses to several different modes of therapy would suggest that more than one pathogenetic mechanism is involved. Untreated, it has been associated with very high morbidity and mortality rates. A poor understanding of the basic disease process has prevented specific treatment modalities, although early diagnosis and availability of dialysis and blood product transfusion services remain crucial. Several modes of therapy have been used to date, with plasma exchange being the most effective method studied and shown to improve survival. On the basis of current knowledge, this form of treatment should be instituted promptly in severe cases. Anecdotal reports of recovery with vincristine or IgG alone or with the use of IgG after the apparent failure of plasma therapy appear promising and deserve further investigation as initial therapeutic measures used in thrombotic microangiopathy. Although the majority of patients recover with normal renal function, those with severe thrombotic microangiopathy may heal through sclerosis with residual hypertension and chronic renal impairment requiring continual medical therapy.
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PMID:Thrombotic microangiopathy: a case report and review of the literature. 139 6

The frequency of renal vascular lesions (RVL) and their relevance in the progression of renal damage were evaluated by the Pathology Group of the "Gruppo Italiano per lo Studio della Nefrite Lupica" (GISNEL). Of 285 patients with lupus nephritis collected from 20 nephrology centers in Italy and classified according to World Health Organization (WHO) criteria, 79 cases (27.7%) with RVL were identified and classified as follows: (1) lupus vasculopathy (n = 27); (2) hemolytic-uremic syndrome/thrombotic thrombocytopenic purpura (HUS/TTP) malignant hypertension-like lesions (n = 24); (3) vasculitis (n = 8); (4) arterio-arteriosclerosis (n = 20). At the time of renal biopsy, patients with RVL had mean serum creatinine levels significantly higher than patients without RVL (201.8 +/- 195.9 mumol/L [2.2 +/- 2.2 mg/dL] v 108.1 +/- 108.0 mumol/L [1.2 +/- 1.2 mg/dL]; P less than 0.01). Hypertension was more frequent in patients with RVL than in those without (68.4% v 30.5%; P less than 0.01). The probability of kidney survival assessed according to the Kaplan-Meier method at 5 and 10 years was, respectively, 74.3% +/- 5.9% and 58.0% +/- 8.9% in patients with RVL, compared with 89.6% +/- 2.7% and 85.9% +/- 3.7% in patients without RVL. However, the two groups did not differ significantly as regards overall survival, the probability of survival at 5 and 10 years being 86.5% +/- 4.5% and 78.8% +/- 6.6% in patients with RVL and 92.2% +/- 2.2% and 83.3% +/- 4.4% in patients without RVL.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Renal vascular lesions as a marker of poor prognosis in patients with lupus nephritis. Gruppo Italiano per lo Studio della Nefrite Lupica (GISNEL). 186 81

Thrombotic thrombocytopenic purpura (TTP) is a syndrome that occurs mainly in adults with multiorgan microvascular thrombosis consisting of thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, renal involvement, and fever. The female to male ratio is 3:2, and peak incidence occurs in the 3rd decade of life. Clinical signs are the consequence of hyaline thrombosis and occlusion of capillaries and arterioles. Renal ailment manifests itself in hematuria and proteinuria with azotemia and even overt renal failure. In severe disease, azotemia is typical of hemolytic uremic syndrome (HUS). TTP was first described in 1925 by Moschcowitz. The clinical picture of TTP consists of a prodromal phase, a viruslike disease occurring in up to 40% of patients. 60% have neurologic disturbances, 90% have purpura initially, and fever occurs in all. Anemia is often severe with hemoglobin values of 7-9 gm/dl, renal involvement in 90%, and renal failure in 40-80% of patients. Clinical variants include the acute and fulminant variety mortality, the chronic form, and the relapsing form. Predisposing factors and triggering agents are autosomal recessive inherited traits in acute idiopathic TTP, systemic diseases, tumor antigens, pregnancy and puerperium, viruses (endotoxins for HUS), and possibly oral contraceptives and hypertension. Therapy includes corticosteroids (prednisone 100-400 mg/day); heparin for postpartum HUS; and antiplatelet agents (Dextran 70, aspirin, and dipyridamole in high doses). The infusion of PGI2 is controversial; splenectomy is also questionable; and vincristine, azathioprine, and cyclophosphamide have unproven efficacy. Fresh-frozen plasma exchange is the method of choice as it produces survival in 90%. Others are iv immunoglobulins, vitamin E, and dialysis and renal transplant. Platelet transfusions are contraindicated because of sudden death and decreased survival.
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PMID:Thrombotic thrombocytopenic purpura and related disorders. 210 74

CSA toxicity includes renal impairment, microangiopathic hemolytic anemia (MAHA), thrombocytopenia (T), and consumptive coagulopathy (CC). We report five BMT patients who developed CSA-associated hematological toxicity. All were conditioned with Ara-C, Cyclophosphamide, Methylprednisolone, TBI, and in two cases busulfan. IV CSA was started the day after marrow infusion and, when practicable, changed to the enteral route. Five patients developed MAHA and T resulting in significantly increased transfusion requirements. All patients had renal impairment and red cell fragmentation. In all patients fragmentation was noted before renal impairment. All developed disproportionate increases in BUN relative to serum creatinine consistent with decreased renal perfusion. Hypertension followed renal impairment in four cases and occurred at the same time as the renal impairment in one case. Two developed CC, prolongation in APTT, and marked decreases in plasma fibrinogen. All patients improved on reduction of the CSA dose. BMT recipients receiving CSA at variable doses may develop evidence of a TTP-like syndrome and/or CC.
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PMID:Coagulation defects in cyclosporine A treated allogeneic bone marrow transplant patients. 304 63

Seventy patients (55 infants and 15 children) with the hemolytic uremic syndrome (HUS) were reviewed to evaluate the incidence of hypertension in HUS. Refractory hypertension was only observed in 5 infants (3 at onset and 2 after several months), whereas it was present in 9 children (4 at onset and 5 in the course of the disease). Controllable hypertension was present in 10 patients (8 infants and 2 children). Histopathological examination of renal specimens in 52 patients showed 3 patterns: cortical necrosis in 10, Thrombotic microangiopathy (TMA) with predominant glomerular involvement in 29 and TMA with predominant arterial involvement in 13. Clinicopathologic correlations showed that severe hypertension was almost exclusively observed in the patients with "vascular TMA". Bilateral nephrectomy performed in 8 patients led to the rapid disappearance of hypertension, thus demonstrating the role of hyperreninaemia in the development of hypertension in the HUS.
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PMID:[Hemolytic-uremic syndrome in children and arterial hypertension]. 679 28

On June 29, 1995, a 49-year-old man was admitted with acute onset of fever, petechiae on his legs, and mental confusion He had suffered hypertension since 6 months previously and was on nicardipine (60 mg/day), ifenprodil (60 mg/day) and ticlopidine (300 mg/day). He had been on ticlopidine for 4 weeks and on the other drugs for 6 months. Soon after admission he had frequent grand mal seizures and needed mechanical ventilation. A diagnosis of TTP was made. He was treated with plasmapheresis (50 ml/kg per day), aspirin 81 mg/day and dipyridamole 300 mg/day. On the sixth day his mental status returned to normal. He recovered gradually from microangiopathic hemolytic anemia, thrombocytopenia and elevated serum creatinine. We reviewed the literature and discussed the possible mechanism of TTP related to the use of ticlopidine.
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PMID:[Thrombotic thrombocytopenic purpura after administration of ticlopidine]. 896 Jun 74

Two patients with CsA-associated neurotoxicity developed severe cerebellar swelling and thrombotic thrombocytopenic purpura after switching to FK506 and high-dose corticosteroids. The prodrome of CsA-associated neurotoxicity, TTP and hypertension while receiving FK506, and high-dose corticosteroids could all be implicated in the development of this syndrome. Close monitoring of patients receiving FK506 and high-dose corticosteroids, for the development of TTP is warranted. Early radiological examination should also be considered in such patients to allow early surgical intervention.
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PMID:Severe cerebellar swelling and thrombotic thrombocytopenic purpura associated with FK506. 948 5

The complication of thrombotic thrombocytopenic purpura or hemolytic uremic syndrome (TTP/HUS) can occur in cancer patients. It is characterized by a microangiopathic hemolytic anemia, severe thrombocytopenia, and renal failure. Pulmonary manifestations, especially pulmonary edema, are a common observation. Neurologic changes are also frequently seen. The etiology is unknown at this time. It has been observed in many different types of cancer and is most commonly seen in gastric adenocarcinoma followed by carcinoma of the breast, colon, and small cell lung carcinoma. The hemolysis can be massive and is due to red cell fragmentation, as schistocytes are present in all the cases. Though immune complexes are present in the plasma, the antiglobulin (Coomb's) test is negative. Chemotherapeutic agents, especially mitomycin C, have been implicated as causative factors. There is a correlation of this complication with the cumulative dose. However, chemotherapy cannot account for all the cases as the syndrome can occur in untreated patients. It can be differentiated from disseminated intravascular coagulation by the absence of a coagulopathy. Management should consist of plasma exchange, use of a Staphylococcus aureus column (Prosorba), and control of hypertension. Because of the susceptibility to pulmonary edema, blood volume overloading should be avoided.
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PMID:Thrombotic microangiopathy manifesting as thrombotic thrombocytopenic purpura/hemolytic uremic syndrome in the cancer patient. 1035 89

We report an unusual case of renovascular hypertension in a 16-year-old boy with primary antiphospholipid syndrome (PAPS), admitted to our clinic for severe drug-resistant hypertension and hypokalemia. Hormonal investigation revealed secondary aldosteronism and positive captopril test for renovascular disease. Aortography confirmed the occlusion of the left renal artery. After nephrectomy, normalization of blood pressure and secondary aldosteronism occurred. Presently the patient remains in good health, receiving warfarin anticoagulant therapy. PAPS is defined by the presence of antiphospholipid antibodies and recurrent thrombosis. Arterial thrombosis (29%) appears to be less prevalent than venous thrombosis. Thrombotic microangiopathy of the kidney is frequently observed but renal artery occlusion, as seen in our patient, is unusual.
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PMID:Hypertension due to renal artery occlusion in a patient with antiphospholipid syndrome. 1120 81

The antiphospholipid or Hughes syndrome is the association between antiphospholipid antibodies (aPL), venous and arterial thromboses and pregnancy morbidity. Antiphospholipid syndrome (APS) commonly coexists with autoimmune diseases usually systemic lupus erythematosus (SLE), when it is known as secondary APS. When present in isolation it is known as primary APS (PAPS). Although the kidney may be affected in APS, its involvement is perhaps not as well described as that of other organs. Thrombotic microangiopathy (TMA) affecting the kidney has been reported as a manifestation in both primary and secondary APS. This report describes hypertension related to underlying renal TMA as a presenting symptom of APS.
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PMID:Hypertension as the presenting feature of the antiphospholipid syndrome. 1204 90


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