Lymphocyte respiration in children with Trisomy 21

BMC Pediatrics

Abdul-Kader Souid¹  Elhadi H Aburawi¹ 

[1] Department of Pediatrics, UAE University, P.O. Box 17666, Al Ain, United Arab Emirates

关键词: Hypothyroidism;    Trisomy 21;    Mitochondria;    Respiration;    Oxygen;   

Others  :  1170593
DOI  :  10.1186/1471-2431-12-193

 received in 2012-05-10, accepted in 2012-12-12,  发布年份 2012

【 摘 要 】

Background

This study measured lymphocyte mitochondrial O₂ consumption (cellular respiration) in children with trisomy 21.

Methods

Peripheral blood mononuclear cells were isolated from whole blood of trisomy 21 and control children and these cells were immediately used to measure cellular respiration rate. [O₂] was determined as a function of time from the phosphorescence decay rates (1/τ) of Pd (II)-meso-tetra-(4-sulfonatophenyl)-tetrabenzoporphyrin. In sealed vials containing lymphocytes and glucose as a respiratory substrate, [O₂] declined linearly with time, confirming the zero-order kinetics of O₂ conversion to H₂O by cytochrome oxidase. The rate of respiration (k, in μM O₂ min^-1), thus, was the negative of the slope of [O₂] vs. time. Cyanide inhibited O₂ consumption, confirming that oxidation occurred in the mitochondrial respiratory chain.

Results

For control children (age = 8.8 ± 5.6 years, n = 26), the mean (± SD) value of k_c (in μM O₂ per min per 10⁷ cells) was 1.36 ± 0.79 (coefficient of variation, Cv = 58%; median = 1.17; range = 0.60 to 3.12; -2SD = 0.61). For children with trisomy 21 (age = 7.2 ± 4.6 years, n = 26), the values of k_c were 0.82 ± 0.62 (Cv = 76%; median = 0.60; range = 0.20 to 2.80), p<0.001. Similar results (p<0.000) were obtained after excluding the five trisomy 21 children with elevated serum TSH (values >6.1 mU/L). Fourteen of 26 (54%) children with trisomy 21 had k_c values of 0.20 to 0.60 (i.e., <−2SD). The values of k_c positively correlated with body-mass index (BMI, R >0.302), serum creatinine(R >0.507), blood urea nitrogen (BUN, R >0.535) and albumin (R >0.446).

Conclusions

Children with trisomy 21 in this study have reduced lymphocyte bioenergetics. The clinical importance of this finding requires further studies.

【 授权许可】

   
2012 Aburawi and Souid; licensee BioMed Central Ltd.

【 图 表 】

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