Objective: To report the clinical manifestations and laboratory features of five patients with congenital thrombotic thrombocytopenic purpura (cTTP) and explore its standardized clinical diagnosis and treatment along with a review of literature. Methods: Clinical data of patients, such as age of onset, disease manifestation, personal history, family history, and misdiagnosed disease, were collected. Treatment outcomes, therapeutic effects of plasma infusion, and organ function evaluation were observed. The relationship among the clinical manifestations, treatment outcomes, and ADAMTS13 gene mutation of patients with cTTP was analyzed. Additionally, detection of ADAMTS13 activity and analysis of ADAMTS13 gene mutation were explored. Results: The age of onset of cTTP was either in childhood or adulthood except in one case, which was at the age of 1. The primary manifestations were obvious thrombocytopenia, anemia, and different degrees of nervous system involvement. Most of the patients were initially suspected of having immune thrombocytopenia. Acute cTTP was induced by pregnancy and infection in two and one case, respectively. ADAMTS13 gene mutation was detected in all cases, and there was an inherent relationship between the mutation site, clinical manifestations, and degree of organ injury. Therapeutic or prophylactic plasma transfusion was effective for treating cTTP. Conclusions: The clinical manifestations of cTTP vary among individuals, resulting in frequent misdiagnosis that delays treatment. ADAMTS13 activity detection in plasma and ADAMTS13 gene mutation analysis are important bases to diagnose cTTP. Prophylactic plasma transfusion is vital to prevent the onset of the disease.
Female ; Pregnancy ; Humans ; Adult ; Blood Component Transfusion ; Plasma ; Purpura, Thrombotic Thrombocytopenic/therapy* ; Mutation ; Purpura, Thrombocytopenic, Idiopathic ; ADAMTS13 Protein/therapeutic use*

Background: The development of auditory and speech perception ability of children with hearing loss is affected by many factors after they undergo cochlear implantation (CI). Age at CI (CI age) appears to play an important role among these factors. This study aimed to evaluate the development of auditory and speech perception ability and explore the impact of CI age on children with prelingual deafness present before 3 years of age. Methods: Two hundred and seventy-eight children with pre-lingual deafness (176 boys and 102 girls) were included in this study, and the CI age ranged from 6 to 36 months (mean age, 19 months). Categorical auditory performance (CAP) was assessed to evaluate auditory ability, and the speech intelligibility rating was used to evaluate speech intelligibility. The evaluations were performed before CI and 1, 3, 6, 12, 18, 24, 36, 48, and 60 months after CI. Results: The auditory ability of the pre-lingually hearing-impaired children showed the fastest development within 6 months after CI (k = 0.524, t = 30.992, P < 0.05); then, the progress started to decelerate (k = 0.14, t = 3.704, P < 0.05) and entered a plateau at the 24th month (k = 0.03, t = 1.908, P < 0.05). Speech intelligibility showed the fastest improvement between the 12th and 24th months after CI (k = 0.138, t = 5.365, P < 0.05); then, the progress started to decelerate (k = 0.026, t = 1.465, P < 0.05) and entered a plateau at the 48th month (k = 0.012, t = 1.542, P < 0.05). The CI age had no statistical significant effect on the auditory and speech abilities starting at 2 years after CI (P < 0.05). The optimal cutoff age for CI was 15 months. Conclusions Within 5 years after CI, the auditory and speech ability of young hearing-impaired children continuously improved, although speech development lagged behind that of hearing. An earlier CI age is recommended; the optimal cutoff age for CI is at 15 months.

Adult ; China ; Databases, Factual ; Female ; Humans ; Male ; Middle Aged ; Pituitary Diseases ; classification ; epidemiology ; Registries ; Software

Objective To establish an analysis method for quantitatively determining phenolic character-istic components and correlation analyzing their quality representation of specific chromatograms in Shaji (Seabuckthorn Fruit,Hippophae rhamnoides),and to review the quality of Shaji effectively and accurate-ly by applying association analysis-reviewing mode.Methods HPLC-PDA method was used to quantita-tively determine the content of phenolic characteristic components in 11 batches of Shaji(protocatechuic acid,ellagic acid,narcissin,quercetin,isorhamnetin),and to establish phenolic specific chromatograms of Shaji.The quality of 11 batches of Shaji was characterized based on teasing characteristic peaks and chemical types.The quantity of 11 batches of Shaji was characterized based on quantity and peak areas of protocatechuic acid, ellagic acid, narcissin, quercetin, isorhamnetin, phenolic acids(represented by protocatechuic acid)and flavonoids(represented by narcissin)in the specific chromatograms.The characterized results of quality and quantity of 11 batches of Shaji were given association analysis based on baseline material of Shaji.Results The characteristic components of protocatechuic acid, ellagic acid,narcissin,quercetin,isorhamnetin all had good linear correlation,and the results of methodological investigation were in accordance with the quantitative determination requirements.Taken batch 7 of Shaji as baseline material, there were totally 18 characteristic peaks in phenolic specific chromatograms of Shaji,including 3 peaks of phenolic acids and 15 peaks of flavonoids, and all 18 characteristic peaks appeared in the chromatograms of 11 batches of Shaji.The quantity of characteristic components were higher in batch 8, 7, 10, 4, 5, 11 and 1 after analyzed and reviewed by using association analysis-reviewing mode.The relevance of batch 6, 2, 3, 1 and 11 was the highest with baseline material of Shaji.Comprehensive reviewing showed that the excellent extents of batch 1,11,8,6 and 7 were prior. Conclusion The quantitative determination method of phenolic characteristic components in Shaji estab-lished in this study is easy and accurate.The association analysis-reviewing mode for quality characteriza-tion of phenolic specific chromatograms can be used for analyzing the quality and application validity of Shaji and reviewing quality of Shaji effectively and accurately.

OBJECTIVETo treat patients with vascular mild cognitive impairment (VMCI) using traditional Chinese medicine (TCM), it is necessary to classify the patients into TCM syndrome types and to apply different treatments to different types. In this paper, we investigate how to properly carry out the classification for patients with VMCI aged 50 or above using a novel data-driven method known as latent tree analysis (LTA).

METHODA cross-sectional survey on VMCI was carried out in several regions in Northern China between February 2008 and February 2012 which resulted in a data set that involves 803 patients and 93 symptoms. LTA was performed on the data to reveal symptom co-occurrence patterns, and the patients were partitioned into clusters in multiple ways based on the patterns. The patient clusters were matched up with syndrome types, and population statistics of the clusters are used to quantify the syndrome types and to establish classification rules.

RESULTSEight syndrome types are identified: Qi deficiency, Qi stagnation, Blood deficiency, Blood stasis, Phlegm-dampness, Fire-heat, Yang deficiency, and Yin deficiency. The prevalence and symptom occurrence characteristics of each syndrome type are determined. Quantitative classification rules are established for determining whether a patient belongs to each of the syndrome types.

CONCLUSIONA solution for the TCM syndrome classification problem for patients with VMCI and aged 50 or above is established based on the LTA of unlabeled symptom survey data. The results can be used as a reference in clinic practice to improve the quality of syndrome differentiation and to reduce diagnosis variances across physicians. They can also be used for patient selection in research projects aimed at finding biomarkers for the syndrome types and in randomized control trials aimed at determining the efficacy of TCM treatments of VMCI.

Objective To observe the effect of complement fragment C3f on synthesis of α-smooth muscle actin (α-SMA) in human embryonic lung fibroblasts (MRC-5).Methods MRC-5 was cultured with C3f of different concentrations (15.6,62.5,250 ng · mL-1) in low,moderate and high dosage experimental groups.And a positive control of 10 ng· mL-1 transforming growth factor-β1 (TGF-β1) and a negative control of 0 ng · mL-1 C3f were also set up for comparative study.Then α-SMA was detected by immunohistochemistry.Results The moderate dose experimental group and positive control group stimulates the synthesis of α-MRC-5 cell SMA with no statistical difference (7.46 ±0.66 vs 7.54 ±0.51,P ＞0.05).It demonstrated that C3f can stimulate the function of MRC-5 on synthesis of α-SMA.With the increase of C3f concentration,the expression of intracellular α-SMA in three doses experimental groups were increased for 4.95 ± 0.78,7.46 ±0.66 and 9.23 ±0.21,respectively,significant differences in three doses experimental groups (all P ＜ 0.05).Moreover,the concentration of C3f was positively correlated with the expression of α-SMA (P ＜ 0.05).C3f has the function of promoting the expression of α-SMA in MRC-5.Conclusion Complement fragment C3f can stimulate the synthesis of α-SMA in MRC-5 cells.

Objective: To explore effect of high glucose on expression of osteoprotegerin (OPG) and receptor activator of NF- κ B ligand (RANKE) in rat aortic vascular smooth muscle cells. Methods: SD rats were intraperitoneally injected with streptozotocin, OPG and RANKL expression in rat thoracic aortas were detected by immunohistochemical staining. In cultured vascular smooth muscle cells (VSMCs) (A7r5), qRT-PCR and Western blot analysis were used to examine the mRNA and protein levels of OPG and RANKL. Results: Our results demonstrated that OPG expression was increased in hyperglycemic rat aortic VSMCs, while RANKL expression was decreased. Besides, in vitro experiments high glucose induced OPG expression, but depressed RANKL expression by dose- and time-dependent manner in cultured A7r5. Conclusions: Our findings suggested that high glucose could promote the expression of OPG, and inhibit the expression of RANKL in VSMCs, which may be partly be the molecular mechanism of diabetic vascular calcification.

Objective To investigate the effects of dexmedetomidine on patients undergoing combined hysteroscopy and laparoscopy . Methods Ninety patients undergoing combined hysteroscopy and lapa-roscopy were randomly divided into control group ( C ) , dexmedetomidine group1 ( D1 ) and dexmedetomidine group 2 ( D2 ) , with thirty patients each.Dexmedetomidine 0.4 μg · kg -1 was injected in each patient in group D1 and D2 intravenously in 10 min by infusing pump after induc-tion of anesthesia , while an equal volume of 0.9%NaCl was administered to patients in group C.Peri-operative mean arterial pressure (MAP), heart rate ( HR) , bispectral index ( BIS ) , operation time , and extuba-tion time were recorded.The intensity of pain was valued using visual analogue scale ( VAS) at 2, 4, 12, 24 h after operation.The adverse effects and the usage of analgesic drugs were recorded.Results Hemo-dynamic parameters were more stable during operation in group D 1 and D2 than those in group C , and BIS of group D1 and D2 were lower than that of group C.Extubation time of group D 1 was longer than that in group C , while there was no significant difference in extubation time be-tween group D2 and C.Compared with group C ,the data of VAS in group D1 and D2 were decreased , with less adverse reaction and usage of anal-gesic drugs.Conclusion Dexmedetomidine 0.4 μg · kg -1 injected intravenously can reduce the doses of drugs for anesthesia maintenance in patients undergoing combined hysteroscopy and laparoscopy , without a prolongation of extubation time , but can release postoperative pain , decrease the incidence of nausea , vomiting and shivering , and will save postoperative analgesics.

BACKGROUNDAccording to data from the China Hospital Invasive Fungal Surveillance Net (CHIF-NET) 2010, Candida tropicalis (C. tropicalis) is the third most common pathogen causing invasive candidiasis. Moreover, the majority of fluconazole-resistant C. tropicalis isolates were from a single hospital. Therefore, a molecular epidemiological survey is necessary to investigate the genetic relatedness of C. tropicalis isolates in China.

METHODSIn this study, 48 C. tropicalis isolates causing invasive fungal infections from four tertiary hospitals in China were studied. All the isolates were identified by sequencing the internal transcribed spacer region. Antifungal susceptibility to triazoles, amphotericin B, and caspofungin was determined by the Clinical and Laboratory Standards Institute standard broth microdilution method. Multilocus sequence typing (MLST) was performed, and phylogenetic analysis was further performed by the eBURST and maximum parsimony (MP) methods to characterize the genetic relatedness of isolates.

RESULTSMLST discriminated 40 diploid sequence types (DSTs) among 48 isolates, including 36 novel DSTs, and the XYR1 gene showed the highest discriminatory power. The DSTs obtained from this study were compared with those of previously reported C. tropicalis isolates, and there was poor type alignment with regional strains. Nine groups and 11 singletons were identified by eBURST, whereas two groups and 10 subgroups were clustered by MP analysis. Generally, there were no obvious correlations between clonal clusters generated and the specimen source or hospital origin. Seven fluconazole-resistant isolates were confirmed and assigned to three distinguishable branches.

CONCLUSIONSThe results suggested diverse origins of invasive C. tropicalis isolates in China. Although most invasive C. tropicalis strains in the mainland of China were clustered with previously characterized Asian isolates, major C. tropicalis clusters identified in this study were genetically distinct from those of other geographic regions.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Antifungal Agents ; therapeutic use ; Candida tropicalis ; drug effects ; pathogenicity ; Candidiasis ; drug therapy ; China ; Drug Resistance, Fungal ; genetics ; Female ; Humans ; Male ; Middle Aged ; Multilocus Sequence Typing ; methods ; Phylogeny ; Young Adult

Background According to data from the China Hospital Invasive Fungal Surveillance Net (CHIF-NET) 2010,Candida tropicalis (C.tropicalis) is the third most common pathogen causing invasive candidiasis.Moreover,the majority of fluconazole-resistant C.tropicalis isolates were from a single hospital.Therefore,a molecular epidemiological survey is necessary to investigate the genetic relatedness of C.tropicalis isolates in China.Methods In this study,48 C.tropicalis isolates causing invasive fungal infections from four tertiary hospitals in China were studied.All the isolates were identified by sequencing the internal transcribed spacer region.Antifungal susceptibility to triazoles,amphotericin B,and caspofungin was determined by the Clinical and Laboratory Standards Institute standard broth microdilution method.Multilocus sequence typing (MLST) was performed,and phylogenetic analysis was further performed by the eBURST and maximum parsimony (MP) methods to characterize the genetic relatedness of isolates.Results MLST discriminated 40 diploid sequence types (DSTs) among 48 isolates,including 36 novel DSTs,and the XYR1 gene showed the highest discriminatory power.The DSTs obtained from this study were compared with those of previously reported C.tropicalis isolates,and there was poor type alignment with regional strains.Nine groups and 11 singletons were identified by eBURST,whereas two groups and 10 subgroups were clustered by MP analysis.Generally,there were no obvious correlations between clonal clusters generated and the specimen source or hospital origin.Seven fluconazole-resistant isolates were confirmed and assigned to three distinguishable branches.Conclusions The results suggested diverse origins of invasive C.tropicalis isolates in China.Although most invasive C.tropicalis strains in the mainland of China were clustered with previously characterized Asian isolates,major C.tropicalis clusters identified in this study were genetically distinct from those of other geographic regions.