Challenges in the diagnosis and treatment of a case of acute intermittent porphyria in India

The patient improved by the 2 nd day of treatment with hemin (3 mg/kg body weight by vein daily for 4 days), and with avoidance of exacerbating factors recovered sufficiently to restart school 6 months later. [2] Because the porphyrias that elevate PBG (AIP, hereditary coproporphyria and variegate p...

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Bibliographic Details
Published inJournal of postgraduate medicine Vol. 59; no. 3; pp. 241 - 242
Main Authors Dosi, R, Ambaliya, A, Patell, R, Sonune, N
Format Journal Article
LanguageEnglish
Published India Medknow Publications 01.07.2013
Medknow Publications and Media Pvt. Ltd
Medknow Publications & Media Pvt. Ltd
Subjects
Abdomen
Adolescent
Arginine - therapeutic use
Care and treatment
Developing Countries
Diagnosis
Female
Heme - therapeutic use
Humans
India
Medical research
Metabolic disorders
Porphobilinogen - urine
Porphyria
Porphyria, Acute Intermittent - diagnosis
Porphyria, Acute Intermittent - drug therapy
Porphyria, Acute Intermittent - urine
India
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Summary:The patient improved by the 2 nd day of treatment with hemin (3 mg/kg body weight by vein daily for 4 days), and with avoidance of exacerbating factors recovered sufficiently to restart school 6 months later. [2] Because the porphyrias that elevate PBG (AIP, hereditary coproporphyria and variegate porphyria) are treated identically during attacks, treatment can be started once a substantial increase in urinary or plasma PBG is documented while further biochemical testing, including measurement of porphyrins in plasma, urine, and feces, and erythrocyte PBG deaminase activity is in progress.
ISSN:0022-3859
0972-2823
DOI:10.4103/0022-3859.118056