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The earlyobservations made on this group of patients by Van Creveld,VonGieke andPompe pavedthewayfortheunderstandingofanew groupofchildhooddiseasescharacterizedby theinherentinability of “complete glycogen combustion”. The body’s lackof access to the energy released by the degradation of glyco-gen led to its accumulation in tissues, in some limited to theliver and kidneys, and in others to the heart and peripheralmuscles. Admirably, it was recognized at the time that thiswas in fact a heterogeneous group of anomalies with complexetiology and no foreseeable treatment. The brilliant conclu-sions made by these investigators in the early days from suchlimited information were later corroborated by the discoveryof the metabolic steps required in the synthesis and degrada-tion of glycogen.

Errores innatos del metabolismo y anestesia; tan lejos y sin embargo tan cerca