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Abnormal growth in mitochondrial disease
Author(s) -
Wolny S,
McFarland R,
Chinnery P,
Cheetham T
Publication year - 2009
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.2008.01148.x
Subject(s) - medicine , confidence interval , disease , body mass index , mitochondrial disease , pediatrics , short stature , el niño , mitochondrial dna , biochemistry , chemistry , gene
Aim: To review the height and weight of children with mitochondrial disease attending our supra‐regional service. Methods: This was a retrospective audit of cross‐sectional data. Height and weight measurements were available in 24 children and adolescents (median 7.86 years; range 1.76–20.5 years) who all had a confirmed diagnosis of mitochondrial disease. Values were converted to SD scores using UK reference data. Results: Patients with mitochondrial disease were short with an overall SD score of −1.97 (95% confidence intervals −2.72 to –1.23 SD). Patients were also thin with a relatively low BMI SDS of −1.07 (95% confidence intervals −1.69 to ‐ 0.07 SD), which fell with advancing years (r =−0.7; p < 0.000). Conclusion: Short stature and a progressive reduction in body mass index (BMI) are features of mitochondrial disease in childhood. The mechanisms underlying the abnormal growth in these patients need to be studied in more detail.