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Two general types are known: the erythropoietic porphyrias, which are concerned with the formation of erythrocytes in the bone marrow; and the hepatic porphyrias, which are responsible for liver dysfunction. Manifestations of porphyria include gastrointestinal, neurologic, and psychologic symptoms, cutaneous photosensitivity, pigmentation of the face (and later of the bones), and anemia with enlargement of the spleen. Large amounts of porphyrins are excreted in the urine and feces.
Treatment of this condition has been primarily symptomatic and varies in its effectiveness. Emphasis is on prevention of attacks by avoiding fasting and drugs that precipitate the symptoms. Photosensitivity may be controlled by avoiding exposure to light. Removal of the spleen is useful in some cases of the erythropoietic type of porphyria. Drug therapy includes the use of phenothiazines, chlorpromazine and promazine in particular. These drugs allay pain and nervousness and apparently allow a period of remission from symptoms. Meperidine hydrochloride (Demerol) may be given for pain and hydroxypheme (Hemetin) is given intravenously to compensate for genetic impairment of heme synthesis.
Patients with porphyria must not be given barbiturates, sulfonamides, alcohol, or chloroquine as these chemicals may precipitate or intensify attacks. It is recommended that persons with this disease carry with them at all times identification saying that they have porphyria so that in an emergency they will not be given medication that could precipitate an attack or even death.
hormonechemical substance that acts locally or is carried via blood to another body part, to cause a regulatory effect on functional activity or structure
adrenocorticotrophic hormone; ACTH; corticotrophin; adrenocorticotrophin anterior pituitary hormone stimulating release of glucocorticoid and mineralocorticoid and sex hormones (androgens) from cortex of adrenal gland; plasma ACTH levels are reduced in Cushing's syndrome (due to excess production of adrenal cortex hormones) and raised in Addison's disease (due to reduced adrenal cortex function)
antidiuretic hormone; ADH; vasopressin anterior pituitary hormone; causes urine concentration by increasing resorption of solute at the distal convoluted tubule of nephron; absence of ADH causes diabetes insipidus
follicle-stimulating hormone; FSH anterior pituitary hormone; acts on ovaries/testes to influence the release of the sex hormones (oestrogen/testosterone), and induce sperm growth/ovulation
growth hormone; GH; somatotropin anterior pituitary hormone; acts on many tissues, inducing normal growth
luteinizing hormone; LH anterior pituitary hormone; acts on ovaries/testes to influence sex hormone release (oestrogen/testosterone) and induce sperm growth/ovulation