Anti-HBB polyclonal antibody

We report four cases of compound heterozygotes for Hb S (HBB: c.20A>T) and a rare beta(0)-thalassemia (beta(0)-thal) mutation, Hb Westdale (HBB: c.380_396delTGCAGGCTGCCTATCAG), characterized by a 17bp deletion between codons 126 to 131 in exon 3 of the beta-globin gene of human hemoglobin (Hb) confirmed by direct beta-globin gene sequencing. All four cases were from four unrelated families belonging to the Agharia caste, an endogamous ethnic community of the Sundargarh and Jharsuguda districts of Odisha State, India. Detailed observations indicated that all four cases of Hb S/Hb Westdale were clinically severe. On family screening, six family members were found to be heterozygous for Hb Westdale and were asymptomatic. Deletional alpha-thalassemia (alpha-thal) and XmnI polymorphism were studied for all the Hb Westdale cases. The Hb S/Hb Westdale cases had an early median age at onset of symptoms and presentation, more requirement of blood transfusions, splenomegaly and hepatomegaly and were found to be clinically more severe when compared with the Hb S-beta-thal with IVS-I-5 (G>C) (HBB: c.92+5G>C) cases. Overall, the findings indicate that this rare and hitherto unreported compound heterozygosity of Hb S/Hb Westdale is a clinically significant hemoglobinopathy and its finding in a large endogamous community of Odisha State, India will have important implication in the epidemiology and understanding of the clinical spectrum of sickle cell disease in Indian context and prenatal diagnosis.

Brominated and phosphorus-based flame retardants (PFRs) were measured in foam and fabric samples from 18 newly marketed children's car seats. The concentrations of two cyclic phosphonates {PMMMPs, 5-ethyl-2-methyl-2-oxido-1,3,2-dioxaphosphinan-5-yl)methyl methyl methylphosphonate and bis[(5-ethyl-2-methyl-1,3,2-dioxaphosphorinan-5-yl)methyl] methyl phosphonate p,p'-dioxide} were quantitatively measured for the first time in the North American environment and were much higher than those of other flame retardants. Median PMMMP concentrations were 73.6 mu g/g, accounting on average for 52% of the total FR concentrations, indicating an intentional addition of PMMMPs during the manufacturing process of these car seats. Two other emerging PFRs [tris(2,4-di-Hi Katie,t-butylphenyl) phosphate (TDTBPP) and resorcinol bis(diphenyl phosphate) (RDP)] were detected for the first time in baby products at median levels of 1.11 and 6.15 mu g/g, respectively. Other frequently detected PFRs included triethyl phosphate (TEP), triphenyl phosphate (TPHP), and tris(2-butoxyethyl) phosphate (TBOEP). Among the brominated flame retardants monitored, decabromodiphenyl ethane (DBDPE), with a median concentration of 128 mu g/g, was the only halogenated FR measured at levels suggesting intentional use. Other brominated FRs such as hexabromobenzene (HBB) and 2,3-dibromo 2,4,6-tribromophenyl ether (DPTE) were sporadically detected with median concentrations of 0.23 and 0.18 mu g/g, respectively. Despite being phased out in the United States starting in 2013, polybrominated diphenyl ethers (PBDEs) were still observed in 75% of our samples, although at modest levels (median total PBDE levels of 0.24 mu g/g). Trace PBDE levels suggest background contamination rather than intentional use. The high levels of FRs measured in these children's car seats together with the negative health effects associated with some of these compounds are a cause for concern for children's health.