Cases reported "Hypertension, Malignant"

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1/5. Infantile polyarteritis nodosa presenting as hyponatraemic hypertensive syndrome.

    The association of arterial hypertension with hyponatraemic dehydration, known as hyponatraemic hypertensive syndrome (HHS), is a rare and serious hypertensive complication. Here, we describe a 17-mo-old girl who presented with severe hyponatraemic dehydration, hypokalaemia, polyuria, and nephrotic-range proteinuria associated with malignant arterial hypertension and systemic inflammatory disease. diagnosis of classic polyarteritis nodosa (c-PAN) was made on the basis of renal arteriography demonstrating small arterial aneurysms in association with non-aneurismal changes such as arterial cut-off, arterial tapering stenosis and nephrogram perfusion defect. A decrease of blood pressure by antihypertensive treatment resulted in the normalization of HHS abnormalities. However, c-PAN became well controlled only after 4 mo of immunosuppressive therapy. CONCLUSION: The main interest of this case was the uncommon presentation of systemic polyarteritis nodosa in a very young child. Renal ischaemia from intrarenal vessel disease may have been the trigger event for HHS in our case. Management of PAN-associated severe arterial hypertension is based on immunosuppressive and antihypertensive treatment.
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2/5. Cerebral complications in the treatment of accelerated hypertension.

    The malignant phase of hypertension is invariably fatal unless treated, and rapid reduction of arterial pressure is thought to be the treatment of choice. Ten patients with accelerated hypertension are described in whom abnormal neurological signs developed following the rapid reduction of arterial pressure. Three patients died without recovering from the neurological damage. A fourth died of an unrelated cause a month later. Areas of ischaemic damage were found in the brains of three of these cases. Of the six survivors, four were left with some permanent neurological disability. It is likely that these changes resulted from the inability of the cerebral circulation in patients with severe hypertension to autoregulate blood flow to the brain, so that a rapid reduction in arterial pressure led to ischaemia, especially of the watershed areas of the brain. Cerebrovascular autoregulation is likely to be compromized in patients with cerebral oedema, stenosis of major cranial vessels or in those patients with long-standing severe hypertension. It is suggested that the blood pressure in patients with accelerated hypertension should be lowered gently over a period of several hours or even days in order to allow time for the cerebrovascular autoregulatory mechanisms to recover.
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3/5. Gelfoam embolization of the kidneys for treatment of malignant hypertension.

    Some chronic renal failure patients maintained on dialysis have uncontrollable hypertension. Those with elevated renin levels require bilateral nephrectomies prior to kidney transplant to avoid nephrosclerosis. The morbidity and mortality from surgical nephrectomies are high. In 2 such patients we embolized the renal arteries with gelfoam and successfully occluded all the major vessels. One patient became normotensive. The second remained hypertensive and had increased renin levels, probably on the basis of ischemia. Subsequent surgical nephrectomies demonstrated completely occluded segmental branches but only focal areas of infarction. Collateral blood supply determines the success of the procedure.
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4/5. californium-252 neutron curietherapy for advanced cervical cancer.

    Low dose rate neutron radiotherapy using fast neutrons emitted by the radionuclide 252Cf was combined with fractionated X- or gamma rays for the radiotherapy of advanced cervicovaginal tumors. Two different implant schedules were tested to determine the response of the tumors to the scheduling of therapy with the 252CF either early or delayed in the fractionated radiotherapy course. A 90% frequency of complete local clearance of pelvic tumors was observed by the early application of 252Cf. Neutron curietherapy applied as a boost treatment at the end of fractionated radiotherapy, resulted in only 40% local control. Improved results were observed by early implantation therapy and is postulated to be the result of more effective hypoxic tumor therapy and reoxygenation of the hypoxic pelvic tumor. The two groups of patients were compared as to their general status and medical condition and were found similar in age, frequency of associated disease, body weight and tumor stage. The failures of local control by early 252Cf neutron therapy, occurred in 2 patients with high-stage tumor and severe vascular disease and suggests that tumor response was partly dependent upon the integrity and elasticity of the small vessel system.
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5/5. Malignant hypertension in antiphospholipid syndrome without overt lupus nephritis.

    The antiphospholipid syndrome is usually defined by the association of a clinical manifestation (recurrent venous and/or arterial thrombosis, recurrent spontaneous miscarriages) and a biological abnormality (anticardiolipin antibody, lupus anticoagulant). We retrospectively analyzed the records of 5 patients (4 females, 1 male, aged 30 /- 12 years) with antiphospholipid syndrome, primary (n = 1) or secondary to systemic lupus erythematosus (n = 4), who developed malignant systemic hypertension with renal insufficiency, in the absence of lupus nephritis. Before the episode of malignant hypertension, all patients had normal systemic blood pressure and renal function. During malignant hypertension the systolic pressure was 206 /- 39 mmHg and the diastolic pressure 130 /- 25 mmHg, peak serum creatinine was 204 /- 95 mumol/l, daily proteinuria was 1.1 /- 0.8 gr, and complement serum levels were normal in all patients. Renal angiography found normal proximal renal arteries. Renal biopsy showed ischaemic glomeruli without proliferative lesions (n = 5), focal intimal fibrosis either isolated (n = 3) or associated with thrombosis (n = 2) of the intrarenal vessels, and the absence of vasculitis. Immunofluorescence study did not reveal typical lupus deposits. patients were treated with antihypertensive agents, increasing doses of prednisone (n = 3), and anticoagulant (n = 2) or anti-aggregant therapy (n = 1). After a mean follow-up of 6.8 /- 5.2 years, 4 patients were still alive with normal blood pressure and renal function, whereas 1 patient died of a probable catastrophic antiphospholipid syndrome. patients with antiphospholipid syndrome, primary or secondary to systemic lupus erythematosus, may develop malignant hypertension with renal insufficiency and intrarenal vascular lesions, in the absence of lupus nephritis.
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