TAILIEUCHUNG - Lymphocyte respiration in children with Trisomy 21

This study measured lymphocyte mitochondrial O2 consumption (cellular respiration) in children with trisomy 21. Children with trisomy 21 in this study have reduced lymphocyte bioenergetics. The clinical importance of this finding requires further studies. | Aburawi and Souid BMC Pediatrics 2012 12 193 http 1471-2431 12 193 BMC Pediatrics RESEARCH ARTICLE Open Access Lymphocyte respiration in children with Trisomy 21 Elhadi H Aburawi and Abdul-Kader Souid Abstract 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 t 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 pM 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 years n 26 the mean SD value of kc in pM O2 per min per 107 cells was coefficient of variation Cv 58 median range to -2SD . For children with trisomy 21 age years n 26 the values of kc were Cv 76 median range to p . Similar results p were obtained after excluding the five trisomy 21 children with elevated serum TSH values mU L . Fourteen of 26 54 children with trisomy 21 had kc values of to . -2SD . The values of kc positively correlated with body-mass index BMI R serum creatinine R blood urea nitrogen bun R and albumin R . Conclusions Children with trisomy 21 in this study have reduced lymphocyte bioenergetics. The clinical importance of this finding requires further studies. Keywords Oxygen Respiration Mitochondria Trisomy 21 Hypothyroidism Background Trisomy

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