Porphyria
DEFINITION
It is an inherited disease linking abnormalities the construction of heme pigments i.e. hemoglobin and an additional group of material called cytochromes.
SYMPTOMS
The symptoms of Porphyria is red urine, sensitivity to sunlight ,Blister forms on the exposure to sunlight, Sometimes it swells on the exposure of sunlight,Photodermatitis,Constipation,Vomiting,Pain in the limbs and muscles and paralysis.
CAUSES
Porphyrias is inherited as autosomal dominant or autosomal recessive traits, It depends on which type of porphyria is involved. Some types of porphyria starts at the early age, some at teenage years and some begin during maturity. A classical attack of this disease starts by severe pain in abdominal part and followed by vomiting and constipation. Acute attacks can be life-threating, low B.P and shock. Urine may become red or brown.
TREATMENT
When there is attack, these treatment can be given to the patient:
Electrolyte balance should be maintained
Carbohydrate intake should be increased
Pain medication should be given
Propranolol for Blood Pressure.
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