Infantile spasms is a type of refractory epilepsy syndrome.This epilepsy syndrome is characterized by special tonic spasms,a peculiar set of electroencephalographic findings termed hypsarrhythmia,and arrest of psychomotor development in most patients.The etiology is not clearly understood.Recently,mutations of the arista less related homeobox gene(ARX),cyclin-dependent kinase-like 5(CDKL5)/se-rine/threonine kinase 9 gene(STK9),membrane associated guanylate kinase 2 gene(MAGI2),et al,and abnormal chromosome had been found to be responsible for infantile spasms.In this review,progress of infantile spasms in molecular genetics are discussed.

Child ; Encephalitis, Viral ; pathology ; Enterovirus A, Human ; Enterovirus Infections ; complications ; Heart Failure ; etiology ; Humans ; Respiratory Insufficiency ; etiology

Objective To investigate the treatment effect of the proximal humeral internal locking system (PHIL()S) on the elderly patients with displaced proximal humeral fractures.MethodsFrom Feb 2004 to Mar 2007, 36 patients (mean age: 72.2 years) with proximal humeral fractures were treated with PHILOS plate fixation which included 14 cases with 2-part, 17 cases with three-part and 5 cases with four-part fractures according to Neer classification.Operation time,intraoperative blood loss, blood transfusion, perioperative complications and function evaluation of the operated shoulder joint were calculated with 14.5 months follow-up at average.Results The average operation time and blood loss were (61.5± 11.6) min and ( 165.2±91.2) ml, respectively. 1 case with accidents of blood vessel and 1 case with pneumonia were found without neurovascular injuries. All fractures were radiographically healed in an average of 3-5 months.No necrosis of humeral head appeared and 30 (83.3％) cases were excellent or good according to Neer scoring system. Two part fracture and early operation ( within 3 days after operation) might improve the postoperative function of shoulder joint, but the patient's age, gender and ASA score were not statistically with Neer score.Conclusions PHILOS plate fixation is a suitable procedure for displaced proximal humeral fractures via stable fixation and early rehabilitation, especially for elderly patients combined with osteoporosis.

A BBB co-culture cell model consisting of rat brain microvascular endothelial cells (BMEC) and astrocytes (AS) was established to study the effect of Angelica dahurica coumarins on the transport behavior of puerarin across blood-brain barrier (BBB) in vitro and in vivo. The barrier function of this model was evaluated by measuring the transendothelial resistance, phenol red permeability and BBB related protein expression. The permeability assay and western blot methods were performed to study the effects of Angelica dahurica coumarins on the BBB permeability and the expression of BBB related protein. The animal experiment protocols in this study were approved by the Animal Ethics Committee of Xi'an Jiaotong University (Animal Ethics No.: 2021-1329). The results showed that the established BMEC/AS co-culture model could be used to evaluate drug transport across BBB in vitro. After combined with Angelica dahurica coumarins, the transport capacity of puerarin was significantly increased in vitro and in vivo. Additionally, Angelica dahurica coumarins enhanced BBB permeability and inhibited the protein expression of P-glycoprotein (P-gp), zonula occludens-1 (ZO-1) and occludin. Angelica dahurica coumarins might increase BBB permeability by inhibiting the expression of P-gp and tight junction protein, thereby increasing the content of puerarin in brain tissue.

This paper reported a new type of amylase (neoamylase) secreted by a Bacillus strain ZX99. The enzyme was a kind of ectoenzyme that could catalyze starch into isomalto-oligosaccharide effectively, but could not act on pullulan as substrate. The strain Bacillus ZX99 was mutated by ultraviolet ray and a mutant strain BS3.232 was screened. The activity of the neoamylase produced from BS3.232 increased by 60% over that from ZX99 under the same conditions. The results of thin-layer chromatography of products from starch and pullulan catalyzed by the enzyme demonstrated that the enzyme was different from neopullulanase and can be used to produce isomaltooligosaccharide from starch, including isomaltose, panose, isomaltotriose, isomaltotetose.

Animals ; China ; Hantavirus Infections ; veterinary ; Rodent Diseases ; virology

Adenoma, Sweat Gland ; pathology ; Adult ; Female ; Humans ; Immunohistochemistry ; Leg ; pathology ; Skin Neoplasms ; pathology

Objective BK virus-associated nephropathy ( BKVAN) after kidney transplantation is a key factor that influence the prognosis of transplant kidney .To our knowledge , it is believed to be associated with immune suppression .We observed the cura-tive effect and influencing factorsof anti-rejection scheme that Leflunomide was administered instead of Mycophenolate Mofetil ( MMF) on transplant kidney BKVAN .. Methods This study included 15 kidney transplant recipients with pathologically confirmed BKVAN in Nanjing General Hospital of Nanjing Military Region form March 2007 to March 2013 .Leflunomide was administered instead of Myco-phenolate Mofetil ( MMF) .Serum creatinine level , renal allograft loss rate and side effects of leflunomide were monitored after medica-tion switch.The patients were divided into two groups , which were renal allograft loss group and renal allograft survival group , for fur-ther analyses . The differences between each groups in clinical characteristics as well as histochemical features of the transplanted kidneys were analyzed to determine the cause of renal allograft loss in patients with BKVAN . Results Six patients experienced renal al-lograft loss after switching to leflunomide and needed hemodialysis , and 9 patients had stable renal allograft function , renal allograft loss rate was 40.0%.Hyperuricemia occurred in 8 patients in the period before the medication switch and in 5 patients after the switch;a decrease in blood white cell orplateletcount was found in 2 patients during both periods;an increase in Alanine aminotransferase ( ALT) level occurred in one patient after the medication switch .There were no statistically significant differences in any of the above parame-ters before and after the medication switch.Compared to allograft survival group, serum creatinine level[(1.80 ±0.53)mg/dL vs (2.74 ±0.58)mg/dL, P=0.007], the number of B lymphocytes [(206.44 ±144.96) vs (439.67 ±267.77), P=0.047] and CD68[(588.44 ±271.80) vs (944.67 ±259.32), P=0.025] in renal allograft tissue were significantly higherin the allograft loss group. ConclusionLeflunomide is a safe and effective medication for BKVAN .Patients with significantly increased serum creatinine level might have a poorer prognosis .Significantly increased B lymphocytes and CD 68 cells in renal allograft tissue might indicate a poor prognosis.

Objective Sirolimus ( SRL) is a potent immunosuppressive drug used to prevent acute allograft rejection after re-nal transplantation.Nevertheless, the occurrence of proteinuria has recently been recognized among patients treated with SRL-based therapy.The aim of this study was to investigate the therapeutic effect of tripterygium wilfordii hook F ( T II) on proteinuria caused by SRL in renal transplant recipients who were treated by trilogy immunosuppressive therapy of sirolimus combined with mycophenolate and hormone. Methods 52 recipients were divided into 2 groups randomly:TⅡgroup (n=27) and valsartan group (n=25).The TⅡgroup was administered 1 mg/kg/d, and the valsartan group 80-160 mg/d for consecutive 12 months.Based on primary trilogy immu-nosuppressive therapy of sirolimus combined with mycophenolate and hormone, the dosage of sirolimus was adjusted according to the target concentration 6-10 ng/ml( ELASA approach) and mycophenolate was administered 750 mg twice per day, adjusting dosage ac-cording to the mycophenolate AUC 0-12 level(35-45 mg· h/L).The evaluation of therapeutic effect includes: complete remission, proteinuria decreased by>50%; partial remission, proteinuria decreased by 20% to 50%; ineffective, proteinuria decreased by<20%. Results During the 12 month follow-up, the total effective rates in the TⅡgroup and the valsartan group were 95.2%and 86.7%respectively, in which the TⅡ group decreased more significantly (P<0.01).The total cholesterol level and triglyceride level in TⅡgroup were obviously lower than those in valsartan group(P<0.01). The total cholesterol level and triglyceride level in valsartan group increased ([6.60±0.2]mmol/L vs [7.11±1.13]mmol/L, [2.47± 1.48]mmol/L vs [2.49±0.32] mmol/L).The serum protein level in TⅡ group was obviously higher than that in valsartan group ([41.1±1.2]g/L vs [37.9±4.2]g/L, P<0.05).At 3 month, 6 month and 12 month follow-up, the average serum creatinine levels in TⅡgroup were obviously lower than those in valsartan group ([1.5±0.4]mg/dl vs [1.6±0.3]mg/dl, P<0.05), ([1.3±0.3]mg/dl vs [1.8±0.5]mg/dl, P<0.05), ([1.1±0.4]mg/dl vs [2.1±0.5]mg/dl, P<0.05).The incidence rate of adverse reaction in valsartan group was obviously greater compared with TⅡgroup( P<0.05) . Conclusion Both tripterygium wilfordii multiglycosides and valsar-tan can reduce proteinuria caused by SRL in renal transplant patients,while the application of TⅡhas more remarkable effect.

Objective To compare of the effect of intravenous zoledronic acid and calcitonin on the elderly with osteoporotic intertrochanteric fractures.Methods From June 2009 to November 2012,610 patients with osteoporotic intertrochanteric fractures were treated with closed reduction and internal fixation.543 consecutive patients were grouped sequentially according to the admission time.From June 2009 to April 2011,control group (n=325)received calcitonin for osteoporosis (male 107,female 218,mean age 75.02±5.65 years).The number of Evans-Jensen type Ⅰ,Ⅱ,and Ⅲ type patients were 87,136,and 102,respectively.Average lumbar bone mineral density (BMD) and hip BMD were 0.737±0.08 g/cm2 and 0.725±0.05 g/cm2,respectively.From May 2011 to November 2012,218 patients (male 82,female 136,mean age 74.71±5.32 years) received zoledronic acid for osteoporosis.The number of Evans-Jensen type Ⅰ,Ⅱ,and Ⅲ type patient were 62,91 and 65,respectively.Average lumbar BMD and hip BMD were 0.738±0.05 g/cm2 and 0.722±0.06 g/cm2,respectively.All patients received once-yearly intravenous zoledronic acid in one week after operation.The BMD during hospitalization and one year after operation were compared between the two groups.Harris score and VAS score were applied to evaluate the function and pain degree of the operated hip joint.Results The average follow-up time were 12.8 months (range,5 to 22 months) in control group and 12.5 months (range,4 to 19 months) in treatment group.The average fracture union time,Harris score,VAS score were 14.25±1.38 weeks,68.88±5.71 points,and 0.36±0.55 points respectively in treatment group and 14.39± 1.12 weeks,69.47±4.60 points,and 0.33±0.48 points respectively in control group.There were no statistical differences between two groups in fracture union time,Harris score and VAS score.The average lumbar BMD and hip BMD were 0.76±0.06 g/cm2 and 0.75±0.04 g/cm2 in treatment group,which were 0.75±0.07 g/cm2 and 0.74± 0.07 g/cm2 in control group one year after operation,respectively.Compared with the baseline,the BMD of lumbar spine and hip one year after operation slightly increased without statistically significant difference in control group.The BMD increased significantly in treatment group.Conclusion Early administration of intravenous zoledronic acid does not influence bone healing and it could increase BMD one year after closed reduction and internal fixation.