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PubMed1992

Endocrine dysfunction in Kearns-Sayre syndrome.

Kearns-Sayre syndrome (KSS) is a form of mitochondrial myopathy in which specific clinical features, namely progressive external ophthalmoplegia, pigmentary retinal degeneration and cardiac conduction defects, occur. KSS has also been associated with a variety of endocrine and metabolic disorders, in particular short stature, gonadal failure, diabetes mellitus, thyroid disease, hyperaldosteronism, hypomagnesaemia, and bone, tooth and calcification abnormalities. A case is described exhibiting all of these features. A survey of the literature was conducted to determine the prevalence of these conditions among reported cases. Cases with hypoparathyroidism were considered separately to see if they constituted a distinct subgroup with multiple endocrine dysfunction. Short stature was common, being documented in 38% of cases. Gonadal dysfunction before or after puberty was also common (20% of cases) and affected both sexes equally. Diabetes mellitus was recorded in 13% of cases, half of which required insulin. Thyroid disease, hyperaldosteronism and hypomagnesaemia were uncommon but were probably not looked for in many cases. Bone or tooth abnormalities and calcification of the basal ganglia were found both in those with and without hypoparathyroidism. While endocrine and metabolic dysfunction was found more commonly in those with hypoparathyroidism this is likely to be due to increased recognition rather than increased prevalence. No evidence of an autoimmune polyendocrine syndrome including hypoparathyroidism was found.

Child↗
PubMedDate not supplied

[Stages of formation and calcification of the third molar (radiographic study of 500 Mexican children and adolescents)].

Five hundred orthopantomograph radiographs of mexican children and adolescent from 7 to 18 tears old were reviewed in order to establish the third molar formation and calcification stages and the most frequent anomalies related to these teeth. We find that tooth bud presence is between 9 and 11 years; Third molar is absent in 32.4% of the population; 7.4% of the molars presented early formation stage malposition; Therefore oligodontia diagnosis and third molar malposition can not be done before 13 years of age.

Adolescent↗