Research Progress on Genetic Factors and Hemophilia A Clotting Factor Inhibitor-Review / 中国实验血液学杂志

Hemophilia A (Hemophilia A, HA) is an X-linked recessive hereditary coagulation function disorder, the deficiency and dysfunction of blood coagulation were caused by the mutations of gene encoding clotting factor VIII. The treatment of hemophilia A still depends on the replacement therapy with blood coagulation factor. However, the repeated infusion of clotting factor will produce the neutralizing antibody against FVIII, then resulting in one of the serious complications. The reports on the incidence of inhibitor are different at home and abroad. Due to diverse factors, the inhibitors of hemophilia A clotting factor mainly can be divided into genetic and environmental factors, In this review, the inhibitors of hemophilia A clotting factor and their risk factors are briefly summarized.

Relationship between polymorphisms of FCGR2B and susceptibility of children idiopathic thrombocytopenic purpura / 中国实验血液学杂志

The aim of study was to investigate the relationship between polymorphisms of FCGR2B232 1/T oligonucleotide and the susceptibility of children with idiopathic thrombocytopenic purpura (ITP). DNA from 76 patients with ITP and 37 controls was extracted. The SNPs of FCGR2B-232 was detected by polymerase chain reaction (PCR) combined with direct sequencing. The genotype distribution and allele frequency among different groups were compared. The results showed that the genotype (I/I, I/T, T/T) of FCGR2B-232 were 55.3%, 42.1%, and 2.6% in 76 patients with ITP, while 81.1%, 18.9%, 0% in 37 controls. The allele frequencies of FCGR2B-232 in patients with ITP were 76.3% (I232) and 23.7% (T232), but 90.5% and 9.5% in controls. There were significant differences in genotype distributions between the ITP patients and controls (chi(2) = 7.45, = 0.024). The enrichment in Thr232 allele carrier was also significant among the ITP patients as compared with the controls (chi(2) = 7.18, p = 0.007, odds ratio 3.47). There were also significant differences in allele frequencies between the ITP patients and controls [chi(2) = 6.54, p = 0.011, odds ratio 2.97, 95% CI (1.25 - 7.05)]. It is concluded that the polymorphisms of FCGR2B-232 significantly correlates with the susceptibility of children suffering from ITP. The minor Thr232 allele may be a risk genetic factor to ITP children.

Effects of Helicobater Pylori Infection on Henoch-Schonlein Purpura with Renal Impairment in Children / 实用儿科临床杂志

Objective To evaluate the relationship between Henoch-Schonlein purpura (HSP) accompanying renal impairment and helicobater pylori(Hp) infection.Methods This study consisted of 304 patients with HSP.The patients were divided into 2 groups(group A and group B) based on Hp infection or not(91 cases in group A and 213 cases in group B).Compared with the rates of accompanying renal impairment in 2 groups.And observed the recovery from renal impairment between the patients who were turned into negative(group C)and patients still were positive after the anti-Hp therapy(group D).Numeration data were analyzed by ?2 test.Results Group A which was with Hp infected,the accompanying renal impairment ratio was 65.9%.Group B which was without Hp infected,the ratio was 35.2%.There was significant difference between 2 groups(?2=24.378 P