porphyrias/horúčka

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Acute intermittent porphyria presenting as acute muscle pain, fever, and weakness.

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Chloroquine in the treatment of porphyria cutanea tarda.

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Porphyria cutanea tarda (PCT) is common in Ethiopia and invariably affects the liver. Treatment by abstension from alcohol and avoidance of direct sunlight often failed to achieve lasting improvement. Phlebotomy is unacceptable to most of our patients and impractical as a routine therapy.

Juvenile acute intermittent porphyria with hypercholesterolemia and epilepsy: a case report and review of the literature.

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A case of acute intermittent porphyria in a 10-year-old boy with seizures and hypercholesterolemia is presented. The problems of management when seizures and porphyria coincide and discussion of hypercholesterolemia are included. A comprehensive review of the world literature reveals that

[Acute intermittent porphyria at the Hospital Arzobispo Loayza of Lima (1983-1994). A report of 14 cases].

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In order to determine the incidence and clinical characteristics of acute intermittent porphyria (AIP) a retrospective study was done in Hospital Arzobispo Loayza of Lima for the period 1983-1994. Of 16 patients with that diagnosis, 14 ones (13 female and one male) entered to the study because of

Anesthetic considerations in hepatic porphyrias.

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Porphyrias are inherited defects of heme synthesis with manifestations that can mimic surgical diseases; they can be provoked by administration of certain drugs. Manifestations such as abdominal pain, vomiting, tachycardia, hypertension, neuropathy, fever, confusion, and seizures have been

delta-Aminolevulinic acid dehydratase deficiency porphyria (ADP) with syndrome of inappropriate secretion of antidiuretic hormone (SIADH) in a 69-year-old woman.

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delta-Aminolevulinic acid dehydratase deficiency porphyria (ALAD porphyria, ADP) with syndrome of inappropriate secretion of antidiuretic hormone (SIADH) in a 69-year-old woman is reported. The patient was admitted to our hospital complaining of slight cough with low-grade fever, and treated with

Polychemotherapy of acute myelogenous leukemia in a patient with acute intermittent porphyria.

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The safety of drugs in hepatic porphyrias has largely been established by clinical experience, which is very limited in the case of antineoplastic agents. We administered three cycles of polychemotherapy consisting of daunorubicin, cytarabine and 6-thioguanine, and modified supportive care to a

[Cholelithiasis in a previously not described case of congenital erythropoietic porphyria (author's transl)].

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The signs of congenital erythropoietic porphyria (Günther) had their onset at the age of two in a male patient born in 1934: there were blisters and scars after exposure to sunlight, red urine, marked cutaneous pigmentation, acral mutilation, lanugo and erythrodontia. Porphyrin levels in red blood

[Autonomic dysfunctions in acute intermittent porphyria].

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A young woman with acute intermittent porphyria is described. She was admitted in a prolonged attack and had developed a flaccid quadriplegia. During the course she showed various manifestations of the autonomic nervous system, including pupils, gastrointestinal tract, cardiovascular system and

Report of a novel Indian case of congenital erythropoietic porphyria and overview of therapeutic options.

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Congenital erythropoietic porphyria is a rare disorder of heme biosynthesis, resulting from decreased enzymatic activity of uroporphyrinogen III synthase. Clinical manifestations are heterogenous, of variable severity, and with occasional phenotypic-genotypic correlation. A 14-month-old boy

Lead poisoning mimicking acute porphyria!

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We are presenting a case of a 13-year-old autistic boy whose urine porphyrin test came positive on three separate occasions. The child was brought to emergency department of Kasturba Medical College Hospital, Attavar, Mangalore, India, with fever and acute abdominal pain, with no previous history of

[Sacroiliitis in familial Mediterranean fever].

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A 15-year-old girl of Turkish descent had for one year complained of severe recurrent fever-associated deep back pains. Since she was three years of age she had suffered from repeated attacks of fever and severe abdominal pain which ceased spontaneously in 1-3 days. On physical examination the

Systemic lupus erythematosus and hydroxychloroquine-related acute intermittent porphyria.

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Porphyrias, particularly acute intermittent porphyria (AIP), are rare disorders which could be associated with systemic lupus erythematosus (SLE). Although the association with AIP has been known since 1952, only 11 cases have been published to date. It is widely known that precipitating causes such

Acute Intermittent Porphyria: A rare cause of hyponatraemia.

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Hyponatraemia is one of the most frequent laboratory findings in hospitalised patients. We present an unusual case of hyponatraemia in a 23-year-old female secondary to acute intermittent porphyria (AIP), a rare inborn error of metabolism.The patient

ED presentation of acute porphyria.

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Abdominal pain is a common complaint for visits to ED. Among the causes of abdominal pain, the acute porphyria may confuse emergency physicians. With wide range of unspecific symptoms and signs, acute porphyria is rarely considered as a differential diagnosis of acute abdomen in ED. Some patients

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