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September 1985

Elevated Transaminase Levels and Neuromuscular Pathology

Author Affiliations

Division of Neurology Children's Hospital of Philadelphia Philadelphia, PA 19104

Am J Dis Child. 1985;139(9):860. doi:10.1001/archpedi.1985.02140110014010

Sir.—The recent report by Schwarz et al1 regarding prolonged elevation of presumed hepatic enzyme levels in children with unsuspected myopathy cannot be overemphasized. We recently had a similar experience.

Patient Report.—A 4-year-old, right-handed boy was referred to the Division of Neurology, Children's Hospital of Philadelphia, for evaluation of progressive muscle weakness. Neurologic examination disclosed mild proximal muscle weakness with preserved deep tendon reflexes. Gowers' sign was present. Pseudohypertrophy of calf muscles was noted. His creatinine phosphokinase (CPK) level was 15,000 IU/L. Muscle biopsy results demonstrated myopathic features consistent with Duchenne type muscular dystrophy (DMD).

On admission an extensive family history was taken. He had two 3-year-old siblings—monozygotic twin brothers who had "mild chronic hepatitis." Further history disclosed that they were born eight weeks premature. They did well until about 6 months of life when they were hospitalized with enteritis and dehydration. Screening chemistry tests had revealed elevations of