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Diabetes Mellitus in Kearns-Sayre Syndrome

Diabetes Mellitus in Kearns-Sayre Syndrome Abstract Sir.—In their report in the June 1977 issue of the Journal of a patient with Kearns-Sayre syndrome, Seigel et al1 mention diabetes mellitus as a previously undescribed accompaniment of this condition. We have seen three typical cases of Kearns-Sayre syndrome with progressive external ophthalmoplegia, retinal pigmentary degeneration, cardiac conduction defects, small stature, elevated CSF protein, and "ragged-red" fibers in a muscle biopsy specimen. In addition, two of these patients had ataxia, neurosensory hearing loss, and hypogonadism. In a girl, now 17 years old, diabetes mellitus was diagnosed at the age of 13 years. She was treated with diet and insulin. Fasting blood glucose values were also repeatedly elevated (135 to 150 mg/100 ml) in our second case, a young woman aged 21 years. We are aware of six patients2-7 with Kearns-Sayre syndrome and chemical diabetes mellitus (elevated fasting blood glucose or abnormal glucose tolerance test). In addition References 1. Seigel AM, Shaywitz BA, Ciesielski T: Kearns-Sayre syndrome: The importance of early recognition . Am J Dis Child 131:711-712, 1977. 2. Shastri SD, Tulgan H, Colker JL: Progressive ophthalmoplegia, retinitis pigmentosa, and complete heart block . NY State J Med 71:587-590, 1971. 3. Schneck L, Adachi M, Briet P, et al: Ophthalmoplegia plus with morphological and chemical studies of cerebellar and muscle tissue . J Neural Sci 19:37-44, 1973.Crossref 4. Lowes M: Chronic progressive external ophthalmoplegia, pigmentary retinopathy, and heart block (Kearns-Sayre syndrome) . Acta Ophthal 53:610-619, 1975.Crossref 5. Reske-Nielsen E, Lou HC, Lowes H: Progressive external ophthalmoplegia . Acta Opthal 54:553-573, 1976.Crossref 6. Haas J, Haller P, Patzold U: Das Kearns-Syndrom . Dtsch Med Wochenschr 101:1523-1528, 1976.Crossref 7. Toppet M, Telerman-Toppet N, Sliwowski HB, et al: Oculocraniosomatic neuromuscular disease with hypoparathyroidism . Am J Dis Child 131:437-441, 1977. 8. Cullen RF, Daroff RB, Popoff N: Early onset external ophthalmoplegia and retinopathy: A distinct clinical and neuropathologic syndrome . Neurology 23:406, 1973.Crossref 9. Walsh FB: Progressive external opthalmoplegia (PEO): A brief review . Trans Pa Acad Ophthalmol Otolaryngol 22:88-96, 1969. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Diabetes Mellitus in Kearns-Sayre Syndrome

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References (12)

Publisher
American Medical Association
Copyright
Copyright © 1978 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1978.02120280105028
Publisher site
See Article on Publisher Site

Abstract

Abstract Sir.—In their report in the June 1977 issue of the Journal of a patient with Kearns-Sayre syndrome, Seigel et al1 mention diabetes mellitus as a previously undescribed accompaniment of this condition. We have seen three typical cases of Kearns-Sayre syndrome with progressive external ophthalmoplegia, retinal pigmentary degeneration, cardiac conduction defects, small stature, elevated CSF protein, and "ragged-red" fibers in a muscle biopsy specimen. In addition, two of these patients had ataxia, neurosensory hearing loss, and hypogonadism. In a girl, now 17 years old, diabetes mellitus was diagnosed at the age of 13 years. She was treated with diet and insulin. Fasting blood glucose values were also repeatedly elevated (135 to 150 mg/100 ml) in our second case, a young woman aged 21 years. We are aware of six patients2-7 with Kearns-Sayre syndrome and chemical diabetes mellitus (elevated fasting blood glucose or abnormal glucose tolerance test). In addition References 1. Seigel AM, Shaywitz BA, Ciesielski T: Kearns-Sayre syndrome: The importance of early recognition . Am J Dis Child 131:711-712, 1977. 2. Shastri SD, Tulgan H, Colker JL: Progressive ophthalmoplegia, retinitis pigmentosa, and complete heart block . NY State J Med 71:587-590, 1971. 3. Schneck L, Adachi M, Briet P, et al: Ophthalmoplegia plus with morphological and chemical studies of cerebellar and muscle tissue . J Neural Sci 19:37-44, 1973.Crossref 4. Lowes M: Chronic progressive external ophthalmoplegia, pigmentary retinopathy, and heart block (Kearns-Sayre syndrome) . Acta Ophthal 53:610-619, 1975.Crossref 5. Reske-Nielsen E, Lou HC, Lowes H: Progressive external ophthalmoplegia . Acta Opthal 54:553-573, 1976.Crossref 6. Haas J, Haller P, Patzold U: Das Kearns-Syndrom . Dtsch Med Wochenschr 101:1523-1528, 1976.Crossref 7. Toppet M, Telerman-Toppet N, Sliwowski HB, et al: Oculocraniosomatic neuromuscular disease with hypoparathyroidism . Am J Dis Child 131:437-441, 1977. 8. Cullen RF, Daroff RB, Popoff N: Early onset external ophthalmoplegia and retinopathy: A distinct clinical and neuropathologic syndrome . Neurology 23:406, 1973.Crossref 9. Walsh FB: Progressive external opthalmoplegia (PEO): A brief review . Trans Pa Acad Ophthalmol Otolaryngol 22:88-96, 1969.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Mar 1, 1978

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