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Home > West Medicine > Internal Medicine > Hematology
E80.203

Hereditary Coproporphyria

血液科

Common symptoms

肥厚瘢痕、疱疹、色素沉着与色素减退、光过敏

The pathology of disease

Hereditary coproporphyria is an autosomal-dominant coproporphyrinogen-oxidase deficiency causing acute neurovisceral attacks (abdominal pain, neuropathy, neuropsychiatric symptoms), with occasional mild cutaneous photosensitivity. Attacks are precipitated by drugs, alcohol, and fasting. Treatment: hemin and glucose for acute attacks; avoid triggers.

Common Symptoms

Acute abdominal pain; neuropathy; neuropsychiatric symptoms; occasional mild photosensitivity; dark urine

Causes and Pathogenesis

Coproporphyrinogen-oxidase deficiency causes overproduction of porphyrin precursors and coproporphyrin, causing neurovisceral toxicity.

Treatment

Hemin/glucose for acute attacks; remove triggers; sun protection if photosensitive

Preventive Measures

Avoid precipitant drugs/alcohol/fasting; genetic counseling.

Department to Visit

It is recommended to visit the Internal Medicine department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.