Paroxysmal nocturnal haemoglobinuria ( PNH) is a clonal hematopoietic stem cell disease characterized by intravascular hemolytic anemia , pancytopenia and thrombosis .Although PNH is an non-malignant disease , its complications have very negative impacts on patient's quality of life .The most common serious complication is thrombosis formation .

Congenital Hypothyroidism ; diagnosis ; Diagnostic Errors ; Humans ; Infant, Newborn ; Male ; Water-Electrolyte Imbalance ; complications

ObjectiveTo analyze the relative influential factors of hearing rehabilitation of the deafened children with multichannel cochlear implant. Methods42 prelingually deafened children who accepted multichannel cochlear implant were evaluated with their hearing ability, hearing thresholds and talent level, while their family and usage of the multichannel cochlear were investigated. Results and ConclusionThe result shows that the factors influencing the hearing rehabilitation are the occupations of the parents, income of the family, the time between diagnosing deaf and the operation, the time after the cochlear implant operation and the talent level. The results of the logistic regression show that the deafened children can gain their hearing developing rapidly if they living in a family with high income, their mothers have accepted more education, and they accepted longer time of continuing hearing-aid, etc.

ObjectiveTo analyze the relative influential factors of hearing rehabilitation of the deafened children with multichannel cochlear implant. Methods42 prelingually deafened children who accepted multichannel cochlear implant were evaluated with their hearing ability, hearing thresholds and talent level, while their family and usage of the multichannel cochlear were investigated. Results and ConclusionThe result shows that the factors influencing the hearing rehabilitation are the occupations of the parents, income of the family, the time between diagnosing deaf and the operation, the time after the cochlear implant operation and the talent level. The results of the logistic regression show that the deafened children can gain their hearing developing rapidly if they living in a family with high income, their mothers have accepted more education, and they accepted longer time of continuing hearing-aid, etc.

Objective To study the microsurgical and anatomic structures of brachial plexus roots and vertebral canal to find the best approaches for reimplantation of avulsed brachial plexus ventral roots into the spinal cord.Methods On nineteen cervicothoracic spine specimens,the brachial plexus nerves were exposed along to intervertebral foramen,and the spinal cord and brachial plexus roots were exposed by excising the vertebral arch and sectioning the spinal dura mater.The anatomy of brachial plexus roots and vertebrae,and the relative positions of spinal cord segments to vertebral discs were measured and observed.Results The relative positions of spinal cord segments to vertebral discs are:C5-7 spinal cord segments face C3,4,C4,5 and C5,6 vertebral discs;and C8 and T1 spinal cord segments face C6 and C7 vertebrae.Based on the anatomic finding,four approaches were found out: the lateroventral approach,the lateral approach by enlarging intervertebral foramen,the laterodorsal approach and the lateral and dorsal combined approach.Conclusions The brachial plexus ventral roots can be best reimplanted into the spinal cord by the lateroventral approach and the lateral approach.Although the laterodorsal approach and the lateral and dorsal combined approach are not the best,they are less difficuh and dangerous.

Objective To study the effect of three kinds of disinfection liquid,namely chlorine dioxide,silver ion and BIOCIDE,on sterilization of Legionella pneumophila.Methods The suspended quantified disinfection test was conducted by using 7 type of Legionella p neumophila,0.5 ml of bacterial suspension and 4.5 ml disinfection liquid were mixed for certain period, the normal saline was used as the control.1.0 ml of the above liquid or its dilution was incubated with BCYE for 72 h in CO_2 and 35 ℃,the colony count and sterilization rate were calculated.Results The sterilization rate of chlorine dioxide was 6.04%,7.87% and 18.18% at the sterilization time of 0.5,1 and 2.5 h respectively.The sterilization rate of silver ion was 52.61%,99.46% and 99.80% at the time of 0.5,1,1.5 and 2.0 h respectively.As for BIOCIDE,the sterilization rate was 25.28%,93.62%,96.60% and 99.81% at the time of 0.5,1,1.5 and 2 h respectively.Conclusion Compared with chlorine dioxide,silver ion and biological disinfectant has a better sterilization effect on Legionella pneumophila.

Objective To evaluate the efficacy and safety of methotrexate(MTX)on the hip involve ment in patients with ankylosing spondylitis(AS).Methods Among the AS patients with hip joint involvement admitted to the department from 1999—2002,50 patients were treated with sulfasalazine(SSZ)(control group, CG)and 48 MTX(observation group,OG).One kind of NSAIDs was taken by the patients in both groups as the basic therapy.The treatment was maintained in both groups after being discharged.Patients were followed-up for three years.The observation parameters included symptoms,signs,Bath AS disease activity index(BASDAI), Bath AS functional index(BASFI),hip joint function score,the CT staging of hip joint involvement,serum in- flammation markers,laboratory tests and side effects.The data were analyzed with SPSS10.0 statistics software. Results At the 1st,2nd and 3rd year,44,38 and 32 cases in the OG and 45,38 and 31 cases in the CG were followed up respectively.During the three-year follow-up period,the hip joint function score of the OG was significantly higher than that of the CG(P

SUMMARY Tuberculous aneurysm of the aorta is a very rare disorder. There have been only 50 reported cases of tuberculous aneurysm since 1895 till now, of which 88% were false aneurysms with majority being single in number. And only four were found to have multiple tuberculous aneurysms. The mean ages of the patients were 50?16 years. Treatment of tuberculous aortic aneurysm must be a combined medical and surgical approach. Once tuberculous aneurysm is identified, surgery must be performed promptly. The size of the aneurysm does not influence the need for surgery. A 70-year-old male with intermittent episodes of abdominal pain after receiving an inappropriate antitubercular therapy for pulmonary tuberculosis was diagnosed as multiple false tubercular aneurysm of abdominal aorta. An aneurysm of base 3.3 cm and greatest diameter 5 cm?6 cm was seen just above the opening of Coeliac trunk. Also at the dorsal aspect of abdominal aorta and superior mesenteric artery junction and ventral aspect of renal artery false aneurysms were seen, each with the size of 1 cm in diameter. Apart from antitubercular and antimicrobial treatment, first line surgery Endovascular Exclusion of Abdominal Aortic Aneurysm was performed for ruptured false abdominal aortic aneurysm. Even before the second line surgery, smaller abdominal aortic aneurysms got ruptured and the patient ’s party gave up further treatment. Finally the patient died of gastrointestinal haemorrhage.

Objective To provide an optimal working process for crossmatching by full-automatic blood typing instrument.Methods Two workflows were applied to crossmatch test by full-automatic blood typing instrument.One was diluting red blood cells of donor samples to concentrate of 1%,the other was detecting directly the donor′s packed red blood cells.Compared consistency and processing time of the two different workflows.Results Cross match results of two workflows were consistent well(U=0,P>0.05).The average processing times before testing and undergoing testing were not significantly different(t=0.692,t=0.562,P>0.05),whereas the average processing times after testing and throughout testing were significantly different(t=146.485,t=67.053,P<0.05).Conclusion The workflow of diluting donor′s sample before testing saved processing time and better suits hospital having a large quantity of specimens.

Objective To investigate effect of artesunate on expressions of Toll-like receotor-4 (TLR4) and interleu?kin-8 (IL-8) in renal tissue of diabetic nephropathy rats. Methods Male SD rats of over 200 g in body weight (n=45) were injected with low dosage streptozotocin and fed with high fat and sucrose diets to establish diabetes nephropathy (DN) model. Among all rats of successful model, thirty-two were randomly selected and divided into four groups (n=8 in each group), Mod?el group (Group B), Artesunate at high dose treatment group [30 mg/(kg · d)] (Group C), Artesunate at low dose treatment group [10 mg/(kg·d)] (Group D), Enalapril treatment group [10 mg/(kg·d)] (Group E). Another eight rats without STZ injec?tion whose body weight is under 200 g were assigned as Normal group (Group A). Then, rats in Group A and B were given the same volume of normal saline [10 mg/(kg·d)] while rats in Group C, D and E were given 30 mg/(kg·d) Artesunate, 10 mg/(kg·d) Artesunate and 10 mg/(kg · d) Enalapril respectively intragastrically for consecutive 12 weeks. Fasting blood glucose, body weight, kidney index, 24-hours proteinuria, serum creatinine (Cr) and blood urea nitrogen (BUN) were measured. Expression level of TLR4 in renal tissue of rats were examined by Western blot;ELISA was used to quantify the concentration of IL-8 in serum and renal tissue of rats. Results Compared with rats in Group A, the levels of Fasting blood glucose, kidney index , 24-hours proteinuria, Cr and BUN as well as the level of TLR4 in kidney, levels of IL-8 in serum and kidney all significant?ly increased in rats in Group B, C, D and E(P<0.05). On the other hand, body weight were decreased in rats of Group B, C, D and E compared to rats in Group A(P<0.05). The level of TLR4 in kidney, levels of IL-8 in serum and kidney, 24-hours proteinuria, Cr and BUN in rats of Group C, D and E were significantly lower than those in rats of Group B(P<0.05). Furthermore, compared with rats in Group D, the levels of TLR4 in kidney, IL-8 in serum and kidney, 24-hours proteinuria, Cr and BUN were decreased in rats in Group C and E(P<0.05). Pearson correlation analysis showed that the expression lev?el of TLR4 positively correlated with IL-8 level in serum (r=0.969, P<0.05), IL-8 level in kidney (r=0.972, P<0.05), 24-hours proteinuria(r=0.965, P<0.05), Cr(r=0.944, P<0.05)and BUN(r=0.903, P<0.05). IL-8 level in kidney positively correlated with 24-hours proteinuria(r=0.962, P<0.05), Cr(r=0.929, P<0.05)and BUN(r=0.940, P<0.05). Conclu?sion Artesunate decreases expression of TLR4 and IL-8, reduce 24-hours proteinuria, inhibits the inflammation of the DN,relives the pathological lesions of nephron rats with diabetic nephropathy.