Herbert L. Bonkovsky, Author at Pulmonology Advisor

Herbert L. Bonkovsky

All articles by Herbert L. Bonkovsky

Chronic cutaneous porphyrias

How can I be sure that the patient has chronic cutaneous porphyria? Unlike acute porphyrias, which may be clinically indistinguishable, cutaneous porphyrias may be more easily identified from each other in the clinical setting due to some of their unique properties. Together with the occasional photosensitivity of HCP (hereditary coproporphyria) and VP (variegate porphyria), cutaneous…

Acute porphyrias

How can I be sure that the patient has acute porphyrias? Porphyrias are a group of nine metabolic disorders associated with enzymatic defects of heme biosynthesis, which may lead to overexcretion of porphyrins or their precursors (ALA and PBG) in urine or feces (Table I). All are in-born errors of metabolism, except for porphyria cutanea…

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