Hereditary Coproporphyria Biochemistry usmle step 1 webinar lecture











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📌 𝐅𝐨𝐥𝐥𝐨𝐰 𝐨𝐧 𝐈𝐧𝐬𝐭𝐚𝐠𝐫𝐚𝐦:-   / drgbhanuprakash   • 📌𝗝𝗼𝗶𝗻 𝗢𝘂𝗿 𝗧𝗲𝗹𝗲𝗴𝗿𝗮𝗺 𝗖𝗵𝗮𝗻𝗻𝗲𝗹 𝗛𝗲𝗿𝗲:- https://t.me/bhanuprakashdr • 📌𝗦𝘂𝗯𝘀𝗰𝗿𝗶𝗯𝗲 𝗧𝗼 𝗠𝘆 𝗠𝗮𝗶𝗹𝗶𝗻𝗴 𝗟𝗶𝘀𝘁:- https://linktr.ee/DrGBhanuprakash • Hereditary coproporphyria is a rare autosomal dominant inherited form of acute hepatic porphyria , due to deficiency of coproporphyrinogen oxidase. The condition is characterized by neurovisceral attacks that last for 2 or more weeks. Photosensitivity cutaneous symptoms are present in 20%-30% of cases. Onset is mostly in female adolescents and young adults. • This disorder manifests as increasing abdominal pain with psychological and neurological symptoms including nausea, vomiting, constipation, depression, anxiety, irritability, myalgia, paresis, peripheral neuropathy, flaccid paralysis, and convulsions. Attacks commonly present with tachycardia and hyponatremia. Cutaneous symptoms of bullae and scars may appear on sun-exposed skin. • Risks include changes in female reproductive hormones, low caloric intake, and sudden drop in weight. • Respiratory paralysis and cardiac arrhythmia signal a medical emergency. • Management requires immediate cessation of the triggering agent medication, oral contraceptives and treatment of acute attacks with hematin infusion and carbohydrate. Dehydration, hyponatremia, and new-onset seizures need to be addressed. Patients unresponsive to therapies may be considered for liver transplantation.

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