Objective To clarify the differential diagnosis between limb-girdle muscular dystrophy type 2B (LGMD2B) and polymyositis (PM). Methods Analyzed the character of clinical, histochemical and immunohistochemical pathologic with biopsied skeletal muscle in 5 patients with LGMD2B and the relationship with PM. Results Both LGMD2B and PM showed the muscle weakness in proximal limbs and elevated creatinkinase level and electromyogram showed myogenic damage. Histochemical stains: it was similar to PM, that was the muscle fibers degenerating, regenerating and necrotic and the infiltrating inflammatory cells in different extent in 5 patients. Immunohistoehemical stains: anfi-Dysferlin monoelonal antibody showed that Dysferlin was deficient on sarcolemma in LGMD2B, but it was normal in PM. Conclusions It is resemble on the clinical and histoehemieal pathologic characters in both LGMD2B and PM. It is key to antidiastole LGMD2B and PM by the immunohistochemieal stain of anti-Dysferlin monoelonal antibody.

Objective To investigate the clinical and pathological features of limb-girdle muscular dystrophy type 2B(LGMD2B).Methods The clinical data and the pathological result of skeletal muscle in five patients with LGMD2B were analyzed retrospectively.Results Five patients presented muscle atrophy and weakness(four limbs in three patients and both lower limbs in two patients),and the chronic onset,and the progressive deterioration.The pathological examination showed that the muscle fibers degenerating,necrotic and regenerating in different extents were observed,and the infiltration of inflammatory cells were appeared on all of cases.Immunohistochemical stains showed that the expression of anti-dysferlin monoclonal antibody was negative,anti-Dystrophy,Sarcoglycan and dystroglycan monoclonal antibodies were positive normaly,anti-CD8+T cell monoclonal antibody was negative,and anti-MHC-1 monoclonal antibody was up-regulated in five patients.Conclusions The clinical characters of LGMD2B are chronic onset,progressive muscle atrophy and weakness in four limbs or both lower limbs.The pathological characters of LGMD2B are similar to the changes of polymyositis.

12E7 Antigen ; Adult ; Antigens, CD ; metabolism ; Cell Adhesion Molecules ; metabolism ; Chondrosarcoma, Mesenchymal ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Hemangiopericytoma ; pathology ; Humans ; Lymphoma ; pathology ; Male ; Nasal Cavity ; Neuroectodermal Tumors, Primitive ; pathology ; Nose Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; Young Adult

?Optic neuritis ( ON) is one of the most common causes of vision loss by neural eye diseases in youth and middle-aged. In the past, the diagnosis simply according to the risk position, which did not distinguish from the pathogenesis and clinical characteristics, can not meet the current clinical diagnosis and treatment needs. Combining with the etiology, clinical characteristics and prognosis, the latest classification of the current international diagnosis of ON are typical and atypical ON. Typical ON relates to multiple sclerosis ( MS ) or demyelinating disease of the central nervous system, it has a relatively good therapeutic effect and prognosis. Rather than, atypical ON has complex etiology, clinical manifestation, and the treatment and prognosis are also different. At present there are many international ON treatment guidelines with level I evidence-based medical evidence, but with different genetic background, geographical environment and ethnic groups, they are not been determined. China lacks of such a multicenter large sample, a wide range of research evidence. In this paper, we will summarize the progress of the diagnosis and treatment about ON, especially about the atypical ON, in order to provide some suggestions to further improve the standardization and individualization for clinical diagnosis and treatment on ON.

Objective To observe the effects and mechanism of Ganoderma Lucidum Polysaccharides (GLP) on the activity of superoxide dismutase(SOD), the content of malondialdehyde(MDA) of hippocampus tissue and the learning and memory ability. Methods AD rats induced by A? 25-35 were treated with GLP for 7 days and then observing the Morris water maze to study the learning and memory ability, the content of MDA, and the activity of SOD was measured in the hippocampus tissue with spectrophotometer. Ultrastructural changes of neuron were viewed under transmission electron microscope. Results GLP can distinctly improve the learning and memory ability of A?-injected rats(P

Objective:To study the detection value of platelet activation indexes and platelet parameters in patients with hepatitis B-induced cirrhosis.Methods:40 patients with hepatitis B-induced cirrhosis in our hospital from November 2015 to August 2016 were selected as the observation group.40 healthy subjects went through physical examination during the same period were chosen as the control group.The platelet activation indexes and platelet parameters of subjects with same age in two groups were detected and compared.The detection results of the two items in patients with different stages of hepatitis B-induced cirrhosis were compared,too.Results:The PLT of observation group was lower than that of control group,while the other platelet parameters and platelet activation indexes were all higher than those of control group.The detection results of two items in patients with different stages of hepatitis B-induced cirrhosis were with significant differences,all P＜0.05.Conclusion:The platelet activation indexes and platelet parameters in patients with hepatitis B-induced cirrhosis are obviously abnormally expressed.There are significant differences among the detection results in patients with different stages.Therefore,surveillance of those indexes in patients with hepatitis B-induced cirrhosis should be strengthened.

Objective To explore the CT findings of pediatric hepatoblastoma and its correlation with pathological subtypes.Methods CT findings and pathological characteristics of 110 patients with hepatoblastoma confirmed by pathology were retrospectively analyzed.Results The locations of the tumor were right lobe (67/110,60.90％),right and left lobe (25/110,22.72％),left lobe (15/110,13.63％) and caudate lobe (3/110,2.72％).The average tumor size was (287.14±272.36) cm3.Most cases were solitary (94/110,85.45％) and round shape (96/110,87.27％).Most cases were well defined (86/110,78.18 ％).Necrosis were irregular in 102 cases,calcification were in 60 cases.Lung metastases were common (23/110,20.90 ％) and vessel involvement were common (56/110,50.90 ％).Lymph node metastasis was in 5 cases.Calcification and homogeneous enhancement in different pathological types had statistically significance (both P ＜0.001).Conclusion CT is helpful for pre-operation and pathological diagnose.

Objective To determine Haemophilus influenzae type b strains in molecular level using PCR,and to study the immunogenicity of capsular polysaccharide conjugates in mice.Methods Extracting genomes using bacterial DNA extract kit from Hoemophilus influenzae type b strains,and PCR for determining the strains through serotyping-specific and capsular genotyping primers respectively.Various capsular polysaccharides conjugated TT respectively,and the conjugates were administered subcutaneously to mice through dilution.After vaccination with two doses,blood samples were collected for the detection of antibody levels to polyribosylribitol phosphate ( PRP),the capsular polysaccharide of Hib.Results All five Haemophilus influenzae type b strains contain type-specific(482 bp) and capsular type (343 bp)DNA fragment through PCR detecting.The DNA fragments were sequenced.BLAST show that these sequences are 100％ homology comparing the above strains respectively,and are 99％ and 100％ homology comparing the GenBank X78559.1 and M19995.1 respectively.The immunogenicity of mice from various capsular polysaccharide conjugates (PRP-TT) was not significantly different by ELISA detecting.Conclusion Through PCR,Haemophilus influenzae type b strain can be determined in molecular level.The immunogenicity of mice from purified capsular polysaccharide conjugates was not different.The study provides a detection means for the features and heredity stability of Haemophilus influenzae type b strain and reference data for the immunogenicity of different polysaccharide conjugates in vaccine research and development and production.

Aged ; Alzheimer Disease ; drug therapy ; physiopathology ; Brain ; physiopathology ; Chitosan ; pharmacology ; therapeutic use ; Electroencephalography ; Female ; Humans ; Male ; Phospholipids ; pharmacology ; therapeutic use

Humans ; Infant, Newborn ; Male ; Poland Syndrome