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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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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Abstract 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.
AbstractList 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.
Audience General
Author Patell, R
Ambaliya, A
Sonune, N
Dosi, R
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  organization: Department of Medicine, Government Medical College and S.S.G. Hospital, Vadodara, Gujarat
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crossref_primary_10_5005_jp_journals_10071_24133
crossref_primary_10_1016_j_amjms_2021_09_009
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References 24346403 - J Postgrad Med. 2013 Oct-Dec;59(4):338-9
24490311 - J Postgrad Med. 2013 Oct-Dec;59(4):339
Harper (key-10.4103/0022-3859.118056-3) 2007
Puy (key-10.4103/0022-3859.118056-1) 2010
Anderson (key-10.4103/0022-3859.118056-2) 2005
References_xml – start-page: 439
  volume-title: Recommendations for the diagnosis and treatment of the acute porphyrias
  year: 2005
  ident: key-10.4103/0022-3859.118056-2
  publication-title: Ann Intern Med
  contributor:
    fullname: Anderson
– start-page: 924
  volume-title: Porphyrias
  year: 2010
  ident: key-10.4103/0022-3859.118056-1
  publication-title: Lancet
  contributor:
    fullname: Puy
– start-page: 444
  volume-title: Treatment options in acute porphyria, porphyria cutanea tarda, and erythropoietic protoporphyria
  year: 2007
  ident: key-10.4103/0022-3859.118056-3
  publication-title: Curr Treat Options Gastroenterol
  contributor:
    fullname: Harper
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SubjectTerms 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
Title Challenges in the diagnosis and treatment of a case of acute intermittent porphyria in India
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