%0 Journal Article
%T Lymphocyte respiration in children with Trisomy 21
%A Elhadi H Aburawi
%A Abdul-Kader Souid
%J BMC Pediatrics
%D 2012
%I BioMed Central
%R 10.1186/1471-2431-12-193
%X 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/¦Ó) 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 ¦ÌM 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.For control children (age = 8.8 ¡À 5.6 years, n = 26), the mean (¡À SD) value of kc (in ¦ÌM O2 per min per 107 cells) was 1.36 ¡À 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 ¡À 4.6 years, n = 26), the values of kc were 0.82 ¡À 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 kc values of 0.20 to 0.60 (i.e., <£¿2SD). The values of kc 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).Children with trisomy 21 in this study have reduced lymphocyte bioenergetics. The clinical importance of this finding requires further studies.Trisomy 21 is the most common chromosomal anomaly worldwide, affecting about 1 in 700 newborns [1]. These individuals typically have low resting metabolic rates [2] and are particularly susceptible to infections [3] and hypothyroidism [4]. Moreover, defects in the inner mitochondrial membrane potential [5] and mitochondrial respiratory chain enzymes are documented in th
%K Oxygen
%K Respiration
%K Mitochondria
%K Trisomy 21
%K Hypothyroidism
%U http://www.biomedcentral.com/1471-2431/12/193