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

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 .

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 investigate the effects of artesunate( Art) on renal function and the expression of monocyte chemotactic protein-1(MCP-1)and tumor necrosis factor-α(TNF-α)in rats with type 2 diabetic nephropathy(DN). Methods Thirty six male SD rats were randomly divided into six groups, six rats in each, as the normal control, model control, low-dose Art, middle-dose Art, high-dose Art groups which were intragastrically administrated with Art at the dose of 10, 20 and 30 mg·kg-1 ·d-1 for consecutive 8 weeks, enalapril group, which were intragastrically administrated with enalapril 10 mg·kg-1 ·d-1 for consecutive 8 weeks. Rats were injected with low dosage streptozotocin and fed with high fat and high sugar diets to establish type 2 diabetic nephropathy rat model. After 8 weeks’ treatment, the plasma glucose, 24-hour urine protein, serum creatinine and blood urea nitrogen were measured to evaluate renal function. The pathological morphology of rats was performed. Immunohistochemistry and enzyme linked immunosorbent assay ( ELISA ) were performed to examine the expression levels of MCP-1 and TNF-α,respectively. Results Compared with the model control group, 24-hour urine protein, serum creatinine, blood urea nitrogen and blood glucose were significantly decreased in the treatment groups (P<0. 01). Art significantly decreased the serum expressions of MCP-1 and TNF-α in low-, middle-, high-dose Art group ( 157. 47 ± 38. 53, 138.65±18.03,105.09±12. 64 and 3. 14±0. 38,2. 58±0. 11,2. 25±0. 16) pg·mL-1, compared with model control group (181.71±23.06 and 3. 98±0. 24) pg·mL-1(P<0. 05). Conclusion Art has beneficial effects on type 2 diabetic nephropathy. The mechanism of which may be related to the inhibition of inflammatory factor MCP-1 and TNF-α,further relive the pathological injury of kidney and delay the progress of diabetic nephropathy to some extent.

Purpose With the progression of brain tissue aging, the transport and drainage characteristics of metabolites and secretory products for neurons in extracellular space occurs irreversible change. This paper aims to investigate and quantify MR tracer diffusion characteristics in cerebral interstitial fluid of elderly SD rats. Materials and Methods MR contrast agent Gd-DTPA was injected into the caudate nucleus of two groups of rats including 8 in experimental group (15-17 month old) and 15 in control group (7-10 month old). MR scan was performed at 0.25 h, 0.5 h, 1 h, 2 h, 3 h and 4 h to observe the dynamic distribution in the caudate and measure the diffusion and clearance rate. Results There was no statistically significant difference in diffusion rate and D* between control group with (3.32±0.70)×10-4 mm2/s and experimental group with (3.25±0.46)×10-4 mm2/s (t=1.739, P>0.05). The clearance rate k' was significantly different between control group (0.62±0.12)×10-4/s and experimental group (0.29±0.08)×10-4/s (t=11.602, P<0.05). Conclusion The degeneration of aging brain tissue changes the composition of extracellular space resulting in decreased speed of ISF clearance. This may cause accumulation of metabolites which eventually triggers a variety of age-related diseases.

Objective To observe the effects of different anesthetic solutions on postoperative cognitive function and serum S100β protein levels.Methods A prospective randomized controlled trial was conducted. Ninety patients necessary to perform off-pump coronary artery bypass grafting (op-CABG) in Tianjin Chest Hospital from November 2013 to July 2014 were enrolled. They were divided into three groups by random number table: P1, P2 and P3 groups, 30 cases in each group. The anesthesia was maintained with propofol by target-controlled infusion (TCI) in all the patients in the three groups, and the respective dosages were < 2.0μg/mL, 2.0 - 3.0μg/mL and > 3.0 - 4.0μg/mL. The operation time, anesthesia time, dosages of propofol and the incidence of postoperative cognitive dysfunction (POCD) were compared among the three groups. At the following times: before induction of anesthesia (T0), completion of anesthesia induction (T1), after tracheal intubation (T2), skin incision (T3), 1 hour after operation (T4), 2 hours after operation (T5) and the end of operation (T6), the narcotrend index (NTI) and hemodynamic levels were observed; the serum S100β protein levels were measured at the following times: before induction of anesthesia (Ta), 2 hours after operation (Tb), the end of operation (Tc), postoperative 6 hours (Td) and postoperative 24 hours (Te).Results There were no significant differences in operation times and anesthesia times among three groups (allP > 0.05); dosages of propofol in P2 and P3 groups were obviously higher than those of P1 group (mg: 1 746.3±43.9, 2 332.7±42.8 vs. 968.5±35.6, bothP < 0.05), and the incidences of POCD in P2 and P3 groups were lower than that in P1 group (10.00%, 6.67% vs. 33.33%, bothP < 0.05). With the extension of anesthesia time, the level of NTI was gradually declined in each group, in P3 group, it was occasionally increased at T6, and beginning from time point T1 afterwards, the NTI levels were lower than those of P1 and P2 groups at all the time points (allP < 0.05); the mean arterial pressure (MAP) in the three groups had a tendency of firstly going down and then increasing, and the inflection point being at T2, in P1 group, the elevation of MAP level persisted to T4, and it began to decline at T5, while in P2 and P3 groups, the levels started to decline at T4; the heart rate (HR) in three groups showed a tendency of firstly going up and then declining, and the inflection point being at T3; until T6, in P3 group, MAP and HR were all lower than those of P1 and P2 groups, the differences being statistically significant [MAP (mmHg, 1 mmHg = 0.133 kPa): 74.9±8.3 vs. 85.3±11.2, 84.2±10.1;HR (bpm): 74.1±4.2 vs. 80.9±8.1, 78.7±7.9, allP < 0.05]. The serum S100β protein levels of three groups at Tb began to be obviously higher than those at Ta, and reached the peak points at Tc, then the levels started to decline until Td, and the levels at Te was approximately close to those of Ta, but the serum S100β protein levels in P2 and P3 groups were lower than that in P1 group, the differences being statistically significant (mg/L: 1.05±0.22, 1.04±0.21 vs. 1.33±0.22, bothP < 0.05).Conclusion Application of propofol by TCI 2.0 - 3.0μg/mL for maintenance of anesthesia can achieve the satisfactory depth of anesthesia, and it not only can reduce the effects on hemodynamics, but also can decrease the serum S100β protein level and the incidence of POCD.

Objective To investigate the effect of metabolic syndrome and its components on imaging of brain impairment in subcortical ischemic vascular disease.Methods 120 patients with subcortical ischemic vascular disease (SIVD) including 25 cases without metabolic syndrome,25 cases with the risk of metabolic syndrome,70 cases with metabolic syndrome.The diagnostic criteria of metabolic syndrome was the National Cholesterol Education Program Adult Treatment Panel Ⅲ (NCEP-ATP Ⅲ).Brain MRI was assessed by the Scheltens scale,and divided into 3 regions:periventricular hyper intensities (PVH),leukoaraiosis (LA),basal ganglia hyper-intensities (BG).Results The assessment scores of PVH,LA,BG and Scheltens scores were higher in patients with the risk of metabolic syndrome and patient with metabolic syndrome than in patient without metabolic syndrome [(3.75±1.60),(4.21±1.09) us.(2.76±1.62),(10.67±5.26),(13.79±5.25) us.(6.36±3.93),(3.21±2.62),(6.90±4.25) us.(1.52±1.50),(17.62±8.32),(24.90±9.25)vs.(10.58± 5.89),respectively,all P＜0.05].Waist circumference had positive correlations with LA and Scheltens scores (r=0.185,P=0.046; r=0.488,P＜0.001).Positive correlation was found between triglyceride (TG) and LA,BG scores (r=0.188,P=0.042; r=0.311,P =0.001).The positive correlations of impaired glucose tolerance (IGT) with LA,BG and Scheltens scores were found (r=0.235,P=0.011; r=0.229,P=0.013; r=0.206,P=0.027).High density lipoproteincholesterol (HDL-C) was correlated negatively with LA,BG and Scheltens scores (r=-0.238,P=0.010;r=-0.189,P=0.042; r=-0.335,P＜0.001).The further multivariate linear regression analysis showed that IGT,HDL-C had significant correlations with LA assessment score (both P＜0.05),TG had significant correlation with BG assessment score (P＜0.05),and waist circumfernce,IGT,HDL-C had significant correlations with Scheltens scores (all P＜ 0.05).Conclusions Metabolic syndrome and its components correlate with the imagings of cerebral damage in SIVD.Abdominal obesity,TG,IGT,HDL-C are the important risk factors for SIVD.

Objective To detect the levels of serum bilirubin and uric acid as well as their relationship with cognitive function in patients with subcortical ischemic vascular disease (SIVD).Methods Serum direct bilirubin (DBIL),indirect bilirubin (IBIL),total bilirubin (TBIL),uric acid (UA) and vascular risk factors were analyzed in 238 individuals comprising 161 patients with SIVD and 77 controls with normal cognitive function.SIVD patients were divided into two subgroups:those with cognitive impairment (SVMCI) and those with dementia (SVaD).All of them were subject to the cognitive assessment including Mini-mental State Examination(MMSE),the Cambridge Cognitive Examination-Chinese Version (CAMCOGC) and Clinical Demential Rating (CDR).Leukoaraiosis was graded according to the severity by their MRI scan appearances.Results MMSE and CAMCOG scores were significant lower in SVMCI and SVaD groups (17.9 ± 5.01,59.87 ± 12.89 ; 24.84 ± 1.57,83.66 ± 4.79) when compared to those in the controls (28.19 ± 1.03,91.66-± 4.93 ; Z =197.63,P =0.000 ; Z =186.54,P =0.000).In comparison with the controls,serum levels of DBIL,IBIL,and TBIL in SVaD group were significant lower ((2.85 ± 1.09) μmol/L vs (3.24 ± 1.30) iμmol/L; (7.50 ±3.27) μmol/L vs (9.06 ±3.52) μmol/L; (10.37 ±4.10) μ mol/L vs (12.31 ±4.64) μmol/L; P =0.035,P =0.005,P =0.006).Also,serum IBIL level was significant lower in SVMCI group compared to the controls ((7.86 ± 3.28) μmol/L vs (9.06 ± 3.52) μmol/L,P =0.034).While serum level of UA was significantly higher in SVMCI ((341.47 ± 92.80) μmol/L) and SVaD ((356.34 ±80.89) μmol/L) groups as compared to those in controls((310.52 ±79.85) μmol/L;P =0.025 ;P =0.001).The UA level was negatively correlated with MMSE scores and CAMCOG-C scores (r =-0.180,P =0.005; r =-0.203,P =0.002),while the bilirubin level was positively correlated with language (r =0.130,P =0.045) and recent memory (r =0.160,P =0.014) scores in CAMCOG-C.The UA level remained associated with MMSE after controlling for age,sex,education,hypertension,diabetes,smoking,and BMI (β =-0.121,P =0.044).While,after adjusting grading of a leukoaraiosis,UA has no significant association with MMSE.Conclusions The bilirubin level is decreased while the UA level is elevated in SIVD patients.The serum TBIL is negatively correlated with cognition dysfunction,while UA is strongly related to cognition dysfunction in SIVD patients.Cognitive dysfunction resulting from UA might have close association with leukoaraiosis.

Objective:To investigate the relationship between pemphigus vulgaris(PV) and HLA-DR,DQ haplotypes in Han nations of northeast China.Methods:Polymerase chain reaction-sequence specific primers(PCR-SSP) method was used to detect the HLA-DRB1 and DQB1 alleles of 27 PV patients of Han nation of northeast China, analysed haplotyes and compared with 99 healthy controls.Results:Compared with control group, the frequencies of the haplotypes of HLA-DRB1*140x-DQB1*0503,DRB1*140x-DQB1*0201,DRB1*120x-DQB1*0503 and DRB1*140x-DQB1*0302 increased significantly in PV group. After statistical test, the difference between the two groups was significant.Conclusion:The special haplotypes may contribute to genetic susceptibility to PV in northeast Chinese.