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Strana 1 od 48 výsledky
Grand mal seizures and acute intermittent porphyria. The problem of differential diagnosis and treatment.
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A 36-year-old white man had both acute intermittent porphyria and long-standing idiopathic grand mal seizures. Diphenylhydantoin apparently adversely affected both the clinical and biochemical parameters of the acute intermittent porphyria. Comparison of urinary levels of the porphyrin precursors,
The effects of superoxide dismutase mimetic MnTMPyP on the altered blood-brain barrier integrity in experimental preeclampsia with or without seizures in rats.
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We investigated the effects of a cell-permeable superoxide dismutase mimetic, manganese(III) tetrakis(1-methyl-4-pyridyl)porphyrin (MnTMPyP) on blood-brain barrier (BBB) integrity following pentylenetetrazole (PTZ)-induced seizures in experimental preeclampsia symptoms induced by
Effects of sodium valproate on haem biosynthesis in man: implications for seizure management in the porphyric patient.
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The short-term effects of sodium valproate (VPA) on haem biosynthesis were assessed in a placebo-controlled crossover trial in eight healthy male subjects who ingested VPA 500 mg t.i.d. and matched placebo for 5 days. All showed augmented activity of leucocyte 5-aminolaevulinate synthase (ALA-S)
Effects of new anticonvulsant medications on porphyrin synthesis in cultured liver cells: potential implications for patients with acute porphyria.
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Some patients with acute hereditary porphyrias have seizures and require anticonvulsant therapy, but many anticonvulsants induce exacerbations of the hepatic porphyrias. Recently, several new anticonvulsants have become available. Among these are gabapentin, vigabatrin, felbamate, lamotrigine, and
Porphyrin-Induced Protein Oxidation and Aggregation as a Mechanism of Porphyria-Associated Cell Injury.
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Genetic porphyrias comprise eight diseases caused by defects in the heme biosynthetic pathway that lead to accumulation of heme precursors. Consequences of porphyria include photosensitivity, liver damage and increased risk of hepatocellular carcinoma, and neurovisceral involvement, including
Porphyrin and heme metabolism and the porphyrias.
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Porphyrins and metalloporphyrins are the key pigments of life on earth as we know it, because they include chlorophyll (a magnesium-containing metalloporphyrin) and heme (iron protoporphyrin). In eukaryotes, porphyrins and heme are synthesized by a multistep pathway that involves eight enzymes. The
Abnormal systemic metabolism of iron, porphyrin, and calcium in Fahr's syndrome.
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Striopallidodentate calcinosis (Fahr's disease) is characterized clinically by seizures, rigidity, and dementia and pathologically by mineral deposition in the basal ganglia, dentate nucleus, and cerebral cortex. Disorders of iron and calcium-phosphate metabolism are thought to play a role in its
Epilepsy and porphyria: new perspectives.
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Porphyria is a group of disorders caused by alterations in the enzymatic pathway involved in haem biosynthesis. The clinical picture consists of extraneurological and neurological manifestations, seizures being an important feature. The pathogenesis of seizures is probably related to metabolic
A case of acute intermittent porphyria.
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Acute intermittent porphyria (AIP) is a rare autosomal dominant hereditary metabolic disorder having protean manifestations. The clinical presentations of abdominal pain, peripheral neuropathy and changes in mental status are the classic triad of an acute attack. A 15-year old girl was admitted in
[A 25-year-old patient with colonic pseudo-obstruction, hyponatremia, hypertension, and diffuse pain].
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METHODS
A 25-year-old hypertensive patient presented to the Emergency Department with constipation and diffuse pain which had been increasing for 10 days. She had consulted several doctors before, but neither various analgesics nor metoclopramide had been beneficial. Blood analysis showed
Acute intermittent porphyria caused by novel mutation in HMBS gene, misdiagnosed as cholecystitis.
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BACKGROUND
Acute intermittent porphyria (AIP) is an autosomal dominant neurovisceral inherited disorder due to a defect in the heme biosynthesis pathway. Misdiagnosis of the porphyrias is not uncommon.
METHODS
We present a case of a 26-year-old female with suspected acute cholecystitis, mental
Porphyria turcica. Twenty years after hexachlorobenzene intoxication.
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Porphyria involving more than 4,000 persons occurred in Eastern Turkey from 1956 to 1961 due to ingestion of hexachlorobenzene (HCB), a fungicide added to wheat seedlings. During a site visit in 1977, 32 porphyric Turks (mean age, 29 years) were examined. Porphyrin concentrations were determined
Challenges in the successful management of a case of acute intermittent porphyria in India.
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Acute intermittent porphyria (AIP) is a rare metabolic disease involving a defect in haem biosynthesis resulting in the accumulation and excessive secretion of porphyrins and its precursors. Acute attacks present with episodes of severe abdominal pain, nausea, confusion and severe life-threatening
Observation on bovine congenital erythrocytic protoporphyria in the blonde d'Aquitaine breed.
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Three blonde d'Aquitaine calves (one male and two females) about four months old, exhibited skin lesions just after birth, the site and nature of which suggested photosensitisation. Their porphyrin metabolism indicated a marked decrease in the activity of lymphocytic ferrochelatase, leading to a
Porphyria: reexamination of psychiatric implications.
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Acute intermittent porphyria mimics a variety of commonly occurring disorders and thus poses a diagnostic quagmire. Psychiatric manifestations include hysteria, anxiety, depression, phobias, psychosis, organic disorders, agitation, delirium, and altered consciousness ranging from somnolence to coma.
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