The Pyrophosphate Heart Scintigram in Children with Progressive Muscular Dystrophy

Summary

A pyrophosphate heart scintigram was obtained in 16 boys with progressive muscular dystrophy Duchenne. All of them showed pathological ECG findings and high plasma levels of CK, AST, ALT and LD. In 4 patients the scintigram was distinctly positive and in further 3 it reached borderline values. The remaining 9 boys had normal scintigraphic findings. Those with a positive heart scintigram had very high plasma levels of the enzymes under study which was suggestive of current progression of the disease. There was, however, no relation between heart scintigraphy and the affliction of the skeletal muscles expressed by means of an index.

Zusammenfassung

Die Pyrophosphat-Herzszintigraphie wurde bei 16 Knaben mit progressiver Muskeldystrophie vom Typ Duchenne durchgeführt. Alle zeigten pathologische EKG-Werte und hohe Plasmaspiegel von CK, AST, ALT und LDH. Bei vier Patienten war das Szintigramm deutlich positiv, bei weiteren drei wurden Grenzwerte erreicht. Die restlichen neun Knaben wiesen normale szintigraphische Befunde auf. Patienten mit positivem Herzszintigramm hatten sehr hohe Enzymplasmaspiegel, die eine noch stattfindende Progression der Erkrankung nahelegten. Andererseits fand sich keine Beziehung zwischen der Herzszintigraphie und der gesamten, durch einen Index ausgedrückten Affektion der Skelettmuskulatur.

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