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

Sudden permanent blindness of cerebral origin, in addition to severe abdominal pain, hypertension, convulsions, and peripheral neuropathy developed in a 21-year-old woman, a victim of acute intermittent porphyria. Findings of the pathological examination of the brain showed extensive infarction in both occipital lobes. The pathological changes were consistent with anoxia. We discuss and review the literature of the possibility of "vasospasm" of both posterior cerebral arteries. Follow-up studies with serial EEG showed either focal epileptogenic activity or diffuse slow waves. The most consistent epileptic discharges were found in the occipital regions. The favorable response to the treatment of seizures with carbamazepine in this patient might encourage further clinical trials.
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PMID:Blindness of cerebral origin in acute intermittent porphyria. Report of a case and postmortem examination. 19 74

The study of six patients with acute intermittent porphyria is reported. Clinical and biochemical studies have been performed during the long hospitalization of the patiets. The main disautonomic aspects were the arterial hypertension and the tachycardia in four patients, cardiac arrest in three patients and respiratory arrest in five patients. The cause of this problems is probably the involvment of the autonomic adrenergic system. The authors observed also three patients with hyponatremia probably resulting from inappropriate secretion of antidiuretic hormone. The prognosis of the acute intermittent porphyria is worse if the patients have disautonomic symptoms; three patients died and two had neurological sequalae (motor tetraparesis).
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PMID:[Dysautonomic aspects of acute intermittent porphyria. Apropos of 6 cases]. 49 3

Twenty cases of acute intermittent porphyria were studied during the acute phase. Cardiovascular manifestations were noted in all the cases, with tachycardia in 20 and with hypertension in 17. Propranolol in doses ranging from 20--200 mg was given to all the cases and produced adequate control of tachycardia and hypertension. At follow-up, smaller doses of propranolol were found to maintain the pulse rate and BP within normal limits and also to prevent acute attacks.
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PMID:Propranolol in acute intermittent porphyria. 51 31

The authors present a case of acute intermittent porphyria (AIP) in an almost fatal relapse with quadriplegia, bulbar paralysis and coma. Intravenous hematin produced an immediate arousal from coma and allowed a gradual resumption of bulbar and autonomic functions. Persistent tachycardia and hypertension necessitated huge doses of intravenous propranolol. Both hematin and propranolol administrations were followed by a remarkable decrease in urinary amino-levulinic acid and porphobilinogen excretion. Nevertheless, after the acute stage, the patient was left with a severe generalized muscle wasting. After 7 months of intensive physical therapy, complete recovery of all neuromuscular functions was achieved. The modern aspects of the management of AIP are presented; the efficacy and the limits of hematin and propranolol therapy are discussed.
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PMID:Hematin and propranolol in acute intermittent porphyria. Full recovery from quadriplegic coma and respiratory failure. 52 4

Three patients with acute intermittent porphyria were noted to have retinal branch vessel occlusion. Branch "vein" occlusion, segmental optic atrophy, and soft exudate were the most common ocular manifestation. Two patients had labile elevated hypertension. When patients present with retinal branch vessel occlusion and a constellation of bizarre symptoms that might include hypertension, abdominal pain, acute psychotic behavior and/or cutaneous photosensitivity, the diagnosis of acute intermittent porphyria should be considered.
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PMID:Retinal branch vessel occlusion in acute intermittent porphyria. 55 59

A patient who had hereditary tyrosinemia was observed during two illnesses to have characteristics of acute intermittent porphyria with associated hypertension. Metabolic studies revealed elevated levels of urinary aminolevulinic acid but normal levels of porphyrin metabolites associated with, and possibly explained by, decreased red blood cell activity of the zinc-dependent enzyme, aminolevulinic acid dehydratase. Zinc deficiency could not be directly associated with the diminished enzyme activity. The patient's hypertension appeared to be related to increased urinary excretion of catecholamines and to elevated renin activity in peripheral venous blood.
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PMID:Tyrosinemia with acute intermittent porphyria: aminolevulinic acid dehydratase deficiency related to elevated urinary aminolevulinic acid levels. 83 32

In 17 patients (15 women, 2 men) with acute intermittent porphyria in the incidence of 23 clinical symptoms during 49 attacks was calculated. The most frequent symptoms in percentage of attacks were: Red colour of the urine 100%, abdominal pain 92%, tachycardia 88%, hypertension 75%, vomiting 54%, peripheral neuropathy 50%. In 35% of acute attacks a transient normochromic, normocytic anemia developed which is probably due to a disturbance of heme synthesis. Oliguria was found in 25%, azotemia in 12.5% of attacks. 4 patients with an average of 5 preceding acute attacks showed a persistent reduction of renal function during the symptom-free interval, in contrast to 12 patients with an average of 1.7 previous attacks and normal renal function. During the observation period from 1960-1974 3 (= 18%) of the 17 patients died.
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PMID:[Acute intermittent porphyria: report on 17 patients with 49 attacks (author's transl)]. 99 30

14 women, aged 30-51 years, were studied concerning the course of acute intermittent porphyria. 7 were treated 2-7 years with ovulation inhibtors and had no further acute exacerbations. In 2 other cases a bilateral surgical oophorectomy and a radiotherapeutic castration were performed, and these 2 patients died following multiple acute exacerbations. Of 5 untreated women in a control group, 2 continued to suffer from acute intermittent exacerbations. 4 of the 5 women treated with oral contraceptives developed severe and persistent arterial hypertension in contrast to the 5 untreated women whose blood pressure remained normal. Thus the benefits and dangers should be carefully weighed before using oral contraceptives as a prophylaxis for acute intermittent porphyria. Studies of similar cases are urgently needed to verify the findings of this research.
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PMID:[Oral contraceptives in prophylaxis of acute exacerbations of intermittent porphyria (author's transl)]. 100 Dec 15

We evaluated the prognosis of acute porphyria among 206 adult Finnish patients with acute intermittent porphyria (AIP) or variegate porphyria (VP). The series represents all known patients with these porphyrias in Finland. Of the 47 patients who had a total of 117 acute attacks during the period 1967-1989, 6 died during an attack and 21 attacks were associated with paresis; the frequency of severe attacks was significantly smaller than before 1967 (p = 0.00002). Most pareses and deaths occurred because of a delay in diagnosis and inappropriate treatment of porphyria. For those patients who were symptom-free at the time of diagnosis (1365 follow-up years), the risk of the first subsequent attack was significantly smaller than for those who had had an acute attack before the diagnosis of porphyria (1047 follow-up years, p = 0.005). In addition, milder symptoms of porphyria were more common among those who had had previous attacks than among those who had not (p less than 0.00001). In AIP the risk of attacks correlated with the excretion of porphobilinogen in the urine during remission among adults (p = 0.03); a low rate of excretion predicted freedom from acute attacks. A regular use of many precipitating drugs was never associated with symptoms of porphyria. Two percent of the surgical operations and 4% of the pregnancies were associated with acute attacks. Nearly one-third of the women had symptoms of porphyria associated with the menstrual cycle, but these seldom proceeded to an acute attack. Forty-six percent of the women had used sex-hormone preparations regularly; 2 of them (4.5%) experienced associated acute attacks. Patients with AIP or VP showed increased incidences of hepatocellular carcinoma, and probably also chronic renal failure and hypertension.
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PMID:Prognosis of acute porphyria: occurrence of acute attacks, precipitating factors, and associated diseases. 154 56

Among many metabolic disorders, porphyrias and Fabry disease are known to affect autonomic nervous system. In patients with acute intermittent porphyria, hereditary coproporphyria, and variegate porphyria, autonomic symptoms such as abdominal pain, vomiting, hypertension and tachycardia are among the most prominent clinical manifestations. Fabry disease is clinically characterized by severe limb pain, hypohidrosis, angiokeratomas and various autonomic symptoms. In both porphyrias and Fabry disease, pathological changes in the central and peripheral autonomic nervous system have been documented. In porphyrias, a loss of myelinated fibers, axonal degeneration, and segmental demyelination in peripheral autonomic nerves as well as chromatolysis of several brain stem nuclei have been found. In Fabry disease, abnormal amount of the substrates of alpha-galactosidase, i.e. ceramide di- and trihexoside, are found to be accumulated in the central and peripheral autonomic nerves.
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PMID:[Autonomic dysfunction in metabolic diseases]. 161 65


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