TY  - JOUR
T1  - HYperglycemic acidosis with mortality in kearns-sayre syndrome
AU  - WELCH E, GELBAND H
Y1  - 1989/10/01
N1  - 10.1001/archpedi.1989.02150220023010
JO  - American Journal of Diseases of Children
SP  - 1135
EP  - 1136
VL  - 143
IS  - 10
N2  - Sir.—Kearns-Sayre syndrome (KSS) is a rare multisystemic disorder, usually described as the clinical triad of chronic progressive ophthalmoplegia, retinitis pigmentosa, and cardiomyopathy.1-3 There is a pathologic degeneration of the central nervous system (ie, spongiform degeneration of the brain) and skeletal and pancreatic abnormalities.4,5 Sudden death in patients with KSS has been primarily attributed to the cardiac conduction defects and cardiomyopathy.3Recently, the association of chemical diabetes and hyperglycemic acidotic coma has been documented in the ophthalmology literature.6 We describe three patients with KSS who we believed died secondary to complications related to KSS and hyperglycemic coma. We would like to bring this potentially devastating problem to the attention of the pediatrician.Patient Reports.—Patient 1.—A 14-year-old boy was first seen at 10 years of age with bilateral ptosis, proximal skeletal myopathy, and external ophthalmoplegia. At that time, the results of his cardiac examination were
SN  - 0002-922X
M3  - doi: 10.1001/archpedi.1989.02150220023010
UR  - http://dx.doi.org/10.1001/archpedi.1989.02150220023010
ER  -