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

The acute or inducible hepatic porphyrias comprise four inherited disorders of heme biosynthesis. They usually remain asymptomatic for most of the lifespan of individuals who inherit the specific enzyme deficiencies but may cause life-threatening attacks of neurovisceral symptoms. Failure to consider the diagnosis frequently delays effective treatment, and inappropriate diagnostic tests and/or mistaken interpretation of results may lead to misdiagnosis and inappropriate treatment. The four disorders are ALA dehydratase deficiency porphyria, acute intermittent porphyria, hereditary coproporphyria, and variegate porphyria. Other conditions that clinically and biochemically may mimic acute porphyria include lead poisoning and hereditary tyrosinemia type I. The diagnosis of one of these acute porphyric syndromes should be considered in many patients with otherwise unexplained abdominal pain, severe constipation, systemic arterial hypertension, or other characteristic symptoms. Critical to the rapid diagnosis of the three most common of these disorders is demonstration of markedly increased urinary porphobilinogen (PBG) in a single-void urine specimen. The treatment of choice for all but mild attacks of the acute porphyrias is intravenous hemin therapy, which should be started as soon as possible. Intravenous glucose alone is recommended only for mild attacks (no weakness or hyponatremia) or until hemin is available.
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PMID:Neurovisceral porphyrias: what a hematologist needs to know. 1630 55

The environmental contaminant 2,3,7,8-tetrachlordibenzo-p-dioxin (TCDD) belongs to the category of highly toxic, persistent organic pollutants that accumulate in animal fat and plant tissues. Today, background TCDD levels in human fat are showing a decreasing trend. The food chain is the main source of exposure in the human population. TCDD regulates the expression of a wide range of drug-metabolizing enzymes and has an impact on a large number of biological systems. The most pronounced effects have occurred in occupational settings following the uncontrolled formation of TCDD after industrial accidents, as well as in rare intentional intoxications. Although the acute effects of TCDD exposure are well described in the literature, the long-term consequences have been underevaluated. The most well-known symptoms of severe acute intoxication are chloracne, porphyria, transient hepatotoxicity, and peripheral and central neurotoxicity. Because of the long-term persistence of TCDD in the human body, atherosclerosis, hypertension, diabetes, vascular ocular changes, and signs of neural system damage, including neuropsychological impairment, can be present several decades after massive exposure. Such chronic effects are nonspecific, multifactorial, and may be causally linked to TCDD only in heavily intoxicated subjects. This opinion is supported by the dose-dependent effect of TCDD found in exposed workers and by experimental animal studies.
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PMID:Adverse health effects in humans exposed to 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD). 1689 75

The discovery of propranolol in 1964 and its introducing to the clinical practice has been essential for the progress in the diagnostics and therapy of cardiovascular diseases. Indications for the use ofpropranolol are numerous. Propranolol has shown clinical usefulness in the treatment of angina pectoris, hypertension, cardiac arrhythmias, hyperthrophic obstructive cardiomyopathy, mitral stenosis, and pheochromocytoma. It has proved efficacy in the treatment ofhyperthyroidism, porphyria, cirrhosis, migraine and in the therapy of many neuropsychiatric disorders. The article presents a review of the actual clinical applications of propranolol.
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PMID:[Propranolol--a place in the modern therapy]. 1701 88

Poor glycaemic control and the duration of diabetes mellitus are known to accelerate development and progression of neuropathy. Diabetic co-morbidities: hypertension and hyperlipidaemia, have been postulated to associate with development of neuropathy. A diabetic foot with low temperature and frequent exposure to low temperature environment has recently been hypothesized to be at higher risk to develop early neuropathy. This cross-sectional study is undertaken to identify risk factors for diabetic neuropathy and the association between foot temperature and development of diabetic neuropathy by using simple clinical examination in the outpatient setting. From April 18, to April 30, 2005, universal sampling method was used to select 134 diabetic patients (type 1 or type 2 for >1 year) with peripheral neuropathy. Excluded are those with chronic alcoholism, drug-induced neuropathy, dietary history of vitamin B deficiency and family history of porphyria and hereditary sensorimotor neuropathy. The patient's duration of diabetes, glycaemic control status and the presence of co-morbids: hypertension and hyperlipidemia, were recorded. The temperature of the foot was measured by using thermo buddy. Of 134 patients representing Malaysian ethnic distribution with an equal number of males and females, 20.1% were in the age group of 61 to 65 years and, 85.1% and 67.9% belonged to lower socioeconomic and educational groups respectively. Associations between diabetic neuropathy and glycaemic control (p = 0.018) and duration of diabetes (p < 0.05) were significant. However, hypertension, hyperlipidaemia and low foot temperature were not significantly associated with development of diabetic neuropathy. Poor glycaemic control is significantly associated with diabetic neuropathy. Foot temperature alteration is merely an effect of autonomic neuropathy with a cold foot is attributed to co-existing peripheral arterial disease.
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PMID:Alteration of foot temperature in diabetic neuropathy: is it another piece of puzzle? 1704 21

Using hormone replacement therapy (HRT), absolute and relative contradictions have to be considered, which are primarily classified according to a "worst case" scenario on the assumption of group effects, in order to satisfy forensic demands. However, in patients with severe complaints it make sense to apply HRT even at increased risk. To minimize the risk, a differentiated choice of the preparation especially in terms of progestin component and application mode is feasible apart from a general dose reduction. For internal risk patients, transdermal estradiol in a patch or gel and neutral progestins like progesterone and dydrogesterone or combination patches for a completely transdermal HRT are to be preferred. In the Women's Health Initiative, a study investigating a population strongly burdened with cardiovascular risks, the most important risks were venous thromboses and strokes, in old age also myocardial infarctions. In this context, the risk groups with diabetes, hypertension and dyslipoproteinemia as well as smokers in general are of particular importance. Other common internal risk groups comprise women with thyroid and hepatobiliary diseases. Rare but prognostically important diseases such as porphyria and lupus erythematosus are considered as relative contraindications. The available data on these risk groups are described and practical recommendations are given.
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PMID:[Hormone replacement therapy for internal risk patients]. 1706 2

Despite the little known association between renal damage and the acute porphyrias, limited information is available on the characteristics and pathogenesis of renal disease in this patient group. Previous reports have focused on hypertension as the principal etiological factor. We have studied a series of 9 patients with acute intermittent porphyria (AIP) attending the Porphyria Clinic at King's College Hospital, London, UK, who were referred to the Renal Unit for investigation and treatment of their renal disease. No evidence of a glomerular lesion was found in any of the patients. In contrast, renal histology showed features of a tubulointerstitial disease, and there was evidence of impaired erythropoietin production. Hypertension and nonsteroidal antiinflammatory drug use were present in about a half of the patients. It is postulated that the nephrotoxic effects of porphyrin precursors may contribute to the etiology of this clinical syndrome.
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PMID:Acute intermittent porphyria and chronic renal failure. 1853 96

A 47-year-old man presented with abdominal pain, neck stiffness, severe transient hypertension and unusually dark urine. Cerebrospinal fluid investigations and angiography confirmed the diagnosis of a subarachnoid haemorrhage. Porphyrin studies on the patient and his family demonstrated that the family has acute intermittent porphyria. This is the second case report of an acute hepatic porphyria presenting with a subarachnoid haemorrhage. Acute transient hypertension during the attack of porphyria caused the rupture of an intracranial arterial aneurysm.
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PMID:Acute intermittent porphyria presenting with a subarachnoid haemorrhage. 1956 64

Selected health hazards of outdoor work are reported. The hazards are attributable to physical agents (cold and hot microclimate, UV radiation), chemical agents (e.g., pesticides and herbicides, exhaust fumes), fine particulate dust, biological agents (insect bites, organic dusts, bacteria, poisonous vegetables), excessive physical (static and dynamic) loads. Exposures to those agents may cause circulatory diseases (arterial hypertension, ischemic heart disease), symptoms of lower and upper spine or renal calculosis. Particular attention was paid to dermal diseases caused by exposure to solar radiation, such as sunburns, idiopathic dermatoses, chronic lesions, exacerbation of other skin diseases (lupus erythematosus, porphyria), phototoxic and photoallergic reactions, melanoma and nonmelanoma skin cancer. Besides, solar radiation causes premature skin ageing and premalignant lesions (lentigo maligna, solar keatosis).
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PMID:[Outdoor work-related health hazards]. 1938 63

We describe a case of acute intermittent porphyria in a woman who presented repeatedly with abdominal pain. Porphyrias are caused by decreased enzyme activity in the heme biosynthetic pathway leading to overproduction of heme precursors if demand increases. This can cause symptoms such as abdominal pain, nausea and vomiting, constipation, tachycardia and hypertension. Treatment includes removal of causative factors, administration of carbohydrates or hemin to reduce the production of heme precursors as well as symptomatic treatment.
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PMID:[Acute abdominal pain caused by acute intermittent porphyria - case report and review of the literature]. 2051 71

This review critically appraises the data emerging from small retrospective and prospective cohort studies suggesting that patients with the autosomal dominant acute porphyrias may be at increased risk of hepatocellular cancer (HCC), hypertension (HT) and renal impairment. The most striking finding is a marked excess risk of HCC in Swedish patients with acute intermittent porphyria (AIP). As Sweden has a relatively high prevalence of AIP due to a founder effect, it is uncertain to what extent the finding is generalisable to other populations or other acute porphyrias and whether early intervention through screening can improve outcomes. As yet there is no evidence for the cost-effectiveness of systematic surveillance for HCC in acute porphyria outside Sweden. Data from several populations also suggest a high prevalence of chronic sustained HT and renal impairment in AIP, but it is uncertain if this represents a true excess risk, in particular for asymptomatic patients. As these long-term complications are important and potentially treatable, a pragmatic recommendation is that symptomatic patients with acute porphyria should be offered specialist long-term follow-up and, for those aged >50 years, annual liver ultrasound may be considered following discussion of the likely risks and benefits. Opportunistic cardiovascular risk assessment can readily be incorporated into a structured annual review so that appropriate drugs safe for use in acute porphyria are prescribed promptly. As these diseases are rare, collaborative international epidemiological studies such as those being coordinated through the European Porphyria Network are essential to inform best clinical practice.
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PMID:Review of hepatocellular cancer, hypertension and renal impairment as late complications of acute porphyria and recommendations for patient follow-up. 2285 9


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