Cardiac calcification in acute intermittent porphyria

Our plain-English summary of Cardiac calcification in acute intermittent porphyria, by T. Ghatak, A. Azim, A.K. Baronia, et al (Annals of Pediatric Cardiology, 2011).

The study

• Authors: T. Ghatak, A. Azim, A.K. Baronia, B. Poddar
• Journal: Annals of Pediatric Cardiology
• Published: 2011;4(2):186-8
• Type of article: Case report
• Cited by: 1 article, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Aetiology of pericardial calcifications can be multifactorial. Tuberculosis has been reported as the most common cause. Other known causes include uraemia, asbestosis, post-traumatic or postoperative. We report a rare case of pericardial calcification seen in a patient with established acute intermittent porphyria. A direct causal relationship cannot be established between porphyria and pericardial calcification, but it may be due to deposition of the porphyrin in the pericardium.

In plain English

This article was a case report about a single patient with acute intermittent porphyria (AIP), an inherited disorder of heme production. It did not study asbestos. Asbestosis was mentioned only in the abstract and introduction, as one of several previously known causes of pericardial calcification (calcium deposits in the sac around the heart). The patient was a 17-year-old female seen at a hospital in Lucknow, India, with abdominal pain, seizures and rapidly worsening limb weakness. A urine test was positive for porphobilinogen, and she was treated for AIP. On the 60th day of her intensive care stay, an ECG showed ST segment elevation. A heart ultrasound then showed calcification of the back of the pericardium, the anterior mitral leaflet and the papillary muscle, with signs suggesting early pericardial constriction. Her blood calcium and phosphorus were normal. Fluid taken from her breathing tube tested negative for acid-fast bacilli on three consecutive days. The authors concluded that this case showed cardiac calcification can occur as a complication of AIP. They stated that a direct causal relationship could not be established, and that the mechanism was unclear.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: case report (one patient)
• Who was studied: one 17-year-old female patient with acute intermittent porphyria, treated in the intensive care unit of the Department of Critical Care Medicine, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
• Exposure: No asbestos exposure was studied or reported. The condition studied was acute intermittent porphyria. Asbestosis appears only in the abstract and the introduction, in lists of known causes of pericardial calcification.
• Main results:
  • The patient's urine Watson–Schwartz test was positive for porphobilinogen, and she was treated as a case of AIP.
  • On the 60th day of her ICU stay, ST segment elevation was seen. The ECG showed it in lead II, and cardiac enzyme markers were not elevated.
  • Echocardiography showed posterior pericardial calcification with anterior mitral leaflet and papillary muscle calcification. It also showed a relaxation abnormality suggestive of early pericardial constriction.
  • Serum calcium (10.8 mg/dl) and phosphorus (5 mg/dl) were within normal limits.
  • Tracheal tube aspirate was negative for acid-fast bacilli for 3 days in a row.
  • Because the patient had no symptoms, no anti-heart-failure treatment was started.
• The authors' conclusion: The authors concluded that cardiac calcification and constrictive pericarditis can occur as a complication of AIP. They suggested that deposition of porphyrin precursors in pericardial tissue may be responsible for the calcification.
• Limitations the authors state:
  • The authors stated that a direct causal relationship between porphyria and pericardial calcification cannot be established.
  • The authors stated that they could not comment on the exact mechanism, and that vasospastic mechanisms causing pericardial injury could not be excluded.

Topics

Medical terms in this study, explained in our Lexicon: Pericardial, Deposition, Pericardium.

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