Objectives To investigate the clinical profile and prognosis of hemophagocytic syndrome (HPS). Methods A retrospective study was carried out to analyze the clinical features and laboratory findings in 28 children with HPS. Fisher's exact probability method and Logistic multivariate regression were used to explore the prognostic risk factors.. Results HPS was clinically characterized by prolonged fever (100%), hepatomegaly (64.29%),and other minor features including respiratory symptoms (53.57%), splenomegaly (50%), hydrops of multiple serous cavity (42.86%), lymphadenectasis (32.14%), jaundice (17.85%), skin rash (14.29%), central nervous system involvement (14.29%), and alimentary tract hemorrhage (10.71%). Labo-ratory data showed that 1iver dysfunction, pancytopenia, coagulation abnormalities, disseminated intravascular coagulation, hy-pertriglyceridemia, decreased number of natural killer cells and hyponatremia were prominent. The etiological analysis indicated that infection associated hemophagocytic syndrome was most common (60.71%), in which EB virus associated HPS was pre-dominant, accounting for 64.71%. Significant difference was observed in the difference of albumin,blood urea nitrogen and acti-vated partial thromboplastin time between death and survival cases (P<0.05). The Logistic regression multivariate analysis showed that hypoalbuminemia was an independent prognostic factor. Conclusions There are various underlying diseases and clinical manifestations for HPS. The lower level of serum albumin is an independent prognostic factor. A prompt diagnosis and treatment is very important for HPS prognosis due to the rapid progression and high mortality.

Objective To explore the rehabilitation for chorea after bilateral thalamus hemorrhage. Methods A case was reviewed. Re-sults The score of Fugl-Meyer Assessment of balance increased from 0 to 7, the modified Barthel Index increased from 25 to 50, and the co-ordination of movement improved significantly after rehabilitation treatment. Conclusion Early comprehensive rehabilitation may reduce the chorea movement, improve the activities of daily living and balance for chorea after bilateral thalamus hemorrhage.

Objective To optimize the extraction process of Radix Paeoniae Alba in Baijin Capsule by orthogonal experiment.Methods The study employed the extraction rate of paeoniflorin and total glucosides of paeony as evaluation indexes. The orthogonal design was used to investigate effects of solvent volume, extraction time and extraction frequency on extraction results.Results The optimal extracting condition was extracted 3 times, with 14 fold 70% alcohol, 1.5 h for each time. Conclusion The method is simple and steady, which will provide instruction and reference to the production of Baijin Capsule.

Objective:To investigate the labial(buccal)bone thickness at alveolar crest zone and alveolar crest height of the maxil-lary anterior teeth and premolars of young adults with normal occlusion.Methods:The alveolar bone of the anterior teeth and premo-lars of 67 eligible Han national young volunteers was scanned by CBCT.Then the facial bone thickness and the distance between the facial alveolar crest and Cemento-enamel Junction(CEJ)of the anterior teeth and premolars were measured and analyzed after recon-struction.Results:The distance between labial (buccal)crest and labial (buccal)CEJ of the maxillary first premolars was the lar-gest(P <0.05);there was a negative correlation between the labial(buccal)crest height and the facial alveolar bone thickness at 2 mm from CEJ toward root derection[(P <0.05),0.6 <|r|<0.8].Conclusion:The labial (buccal)crest of the first premolars was higher than that of other teeth in maxillary aesthetic zone.At alveolar crest zone,when the labial (buccal)bone was thinner,the dis-tance between labial (buccal)crest and labial (buccal)CEJ was larger,and the implant aesthetic risk is higher.

ObjectivesTo investigate the composition of clinical classiifcation and pathological patterns and their rela-tionships and change in children with renal disease undergoing biopsy.MethodsA retrospective analysis of pathological and clinical data obtained from children (≤14 year) with renal disease undergoing biopsy from 1984-1997 and from 1998-2011 was performed.ResultsOne thousand four hundred and sixty-two children underwent renal biopsy in 28 years, and 1313 patients were recruited in this study, 824 males (62.8%) and 489 females (37.2%). The mean age was 9 years and 4 months at renal biopsy. There were 921 children (70.1%) with primary glomerular disease (PGD) and 312 children (23.8%) with secondary glomerular disease (SDG). The main clinical classiifcations of PGD were nephrotic syndrome (NS, 31.2%), isolated hematuria (IH, 16.1%), and acute glomerulonephritis (AGN, 11.0%). The main pathological patterns of PGD were IgA nephrop-athy (IgAN, 27.6%), minimal change disease (MCD, 24.0%), and mesangial proliferative glomerulonephritis (MsPGN, 16.9%). The main causes of SGD were lupus nephritis (LN, 40.7%), Henoch-Sch?nlein purpura nephritis (HSPN, 34.3%), and hepatitis B virus related glomerulonephritis (HBV-GN, 19.6%). In this 28 years, the composition of PGD was decreased, however, the compositions of SGD and other renal diseases were increased. Compared with 1984-1997, the pathological manifestations of IgAN, MCD and focal segmental glomeralosclerosis were increased, MsPGN, IgMN, and crescentic glomerulonephritis were decreased in 1998-2011. The difference was statistically significant (P<0.05). In SGD patients, HBV-GN was significantly decreased (P<0.05).ConclusionsPGD is the main disease in children undergoing renal biopsy. IgAN is the most common pathological pattern. NS is the most common clinical classiifcation. In this 28 years, the composition of PGD is decreased, SGD and other renal diseases are increased in children undergoing renal biopsy.

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We reported a case of preeclampsia complicated by acute stress cardiomyopathy following cesarean section at 37 weeks of gestation. The patient presented with chest tightness and suffocation two days after the operation and was considered as acute stress cardiomyopathy. The patient recovered and was discharged nine days after the surgery after a series of management, including non-invasive ventilation, vasodilation, lowing blood pressure, maintaining a negative fluid balance, anti-infection, and anticoagulation. Stress-induced cardiomyopathy during pregnancy and postpartum is rare, and cesarean section and preeclampsia may be the risk factors.

Objective: The prevalence of HIV/AIDS in people aged ≥50 years is increasing in China, resulting in another focus for AIDS prevention and control. A Meta-analysis was conducted to evaluate the HIV infection, its area and time distributions in people aged ≥50 years in China to provide evidence for the prevention and control of HIV/AIDS. Methods: Information on HIV infection rate in people aged ≥50 years in China were collected from Wanfang digital database, China National Knowledge Infrastructure (CNKI), PubMed and Embase. The R Studio1.1.456 software was used to calculate the pooled infection rate, and assessed the publication bias. Results: A total of 30 studies which covered 13 provinces were selected. From 2010 to 2018, the pooled HIV infection rate in people aged ≥50 years was 1.68% (95%CI:1.00%-2.79%). The HIV infection rate (2.60%) was higher in eastern China than in central and western China (0.16%, 2.13%). Conclusion The overall HIV infection rate in people aged ≥50 years is generally high in China. It is important to control the HIV infection in this population to facilitate the AIDS prevention and control in China.

OBJECTIVE: To explore the molecular pathogenesis for a pedigree affected with hereditary coagulation factor XII (FXII) deficiency. METHODS: Potential variant of the F12 gene was analyzed by PCR and Sanger sequencing. Expression plasmids were constructed by site-directed mutagenesis based on the wild-type and transiently transfected into 293T cells. FXII:C and FXII:Ag of the expression products were determined in the supernatant and cell lysate. Western blotting was used to verify the identify of the protein. RESULTS: Gene sequencing revealed that the proband has carried 46TT genetype and heterozygous p.Glu502Lys variants in exon 13, and a heterozygous p.Gly542Ser variant in exon 14 of the F12 gene. Transfection experiment suggested that the FXII:C and FXII:Ag of p.Glu502Lys variant in the supernatant were 28% and 24%, compared with the wild-type (100%) and FXII:Ag of cell lysates was 39% compared to the wild-type (100%). The FXII:C and FXII:Ag of p. Gly542Ser variant in the supernatant were 32% and 17% and the FXII:Ag of cell lysates was 59%. CONCLUSION The 46TT genetype, p.Glu502Lys and p.Gly542Ser variants of the F12 gene probably underlie the low FXII level in the proband. As shown by in vitro experiment, the p.Glu502Lys and p.Gly542Ser variants can both inhibit the synthesis and secrection of the FXII protein.
Exons ; Factor XII ; genetics ; Factor XII Deficiency ; genetics ; Heterozygote ; Humans ; Pedigree