Myoadenylate deaminase deficiency and forearm ischemic exercise testing.

Abstract

Myoadenylate deaminase (MADA) deficiency has been associated with symptoms of postexertional aches, cramps, weakness, and skeletal muscle dysfunction. Measurement of plasma lactate and ammonia concentrations after forearm ischemic exercise has been suggested as a screening test for this disorder. We performed forearm ischemic tests on 3 patients with histochemically defined MADA deficiency and 13 healthy control subjects, in a standardized fashion. Our results demonstrated that subject effort and/or performance during the exercise portion of testing is a critical variable. In addition to lactate and ammonia, plasma purine compounds (adenosine, inosine, and hypoxanthine) were measured. The finding of decreased purine release after exercise in MADA-deficient patients compared with that in normal individuals increases the specificity of the test and supports the hypothesis that disordered purine metabolism occurs in MADA deficiency.

• Authors: P A Valen, D A Nakayama, J Veum, A R Sulaiman, R L Wortmann
• Journal: Arthritis and rheumatism (Arthritis Rheum) Vol. 30 Issue 6 Pg. 661-8 (Jun 1987) ISSN: 0004-3591 [Print] United States
• PMID: 3606685 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Hypoxanthines
• Lactates
• Hypoxanthine
• Inosine
• Ammonia
• Nucleotide Deaminases
• AMP Deaminase
• Adenosine

Topics

• AMP Deaminase (deficiency)
• Adenosine (blood)
• Adult
• Ammonia (blood)
• Female
• Forearm (blood supply)
• Humans
• Hypoxanthine
• Hypoxanthines (blood)
• Inosine (blood)
• Ischemia (blood)
• Lactates (blood)
• Male
• Middle Aged
• Nucleotide Deaminases (deficiency)
• Physical Exertion