'Picture frame' fibres in a carrier of the trait for malignant hyperpyrexia

S Afr Med J. 1975 Nov 1;49(46):1923-6.

Abstract

A member of a family which was known to be susceptible to malignant hyperpyrexia, who was identified as a carrier by the presence of an elevated serum creatine-phosphokinase, has been investigated further. Muscle was examined biochemically, and the study included the sarcoplasmic ATPase-activity, actinomycin, Mg2+ ATPase activity, ATP, phosphocreatine and glucose-6-phosphate. In addition, the calcium uptake by the sarcoplasmic reticulum was studied. The histochemical analysis of the muscle revealed the presence of a new fibre type characterised by a dense rim of ATPase activity, which gives the impression of a 'picture-frame'. Ultramicroscopic study revealed changes in the mitochondria and areas of myofibrillar disruption with swelling of the sarcoplasmic reticulum.

MeSH terms

  • Adenosine Triphosphatases / analysis*
  • Adult
  • Calcium / metabolism
  • Halothane / pharmacology
  • Heterozygote*
  • Histocytochemistry
  • Humans
  • Hydrogen-Ion Concentration
  • Male
  • Malignant Hyperthermia / enzymology*
  • Malignant Hyperthermia / pathology
  • Malignant Hyperthermia / physiopathology
  • Microscopy, Electron
  • Mitochondria
  • Muscle Contraction / drug effects
  • Muscles / enzymology*
  • Muscles / pathology
  • Sarcoplasmic Reticulum / metabolism

Substances

  • Adenosine Triphosphatases
  • Calcium
  • Halothane