A Case of Hemophilia B with Abdominal and Pelvic Pseudotumor / 罕见病研究

Hemophilia B is a genetic disorder caused by coagulation factor Ⅸ(FⅨ) deficiency, mainly manifesting as joint, muscle and deep tissue bleeding. Hemophilia pseudotumor is a mass formed by soft tissue liquefaction and necrosis caused by repeated bleeding. Most pseudotumors occur in the bone and muscle. We report a case of hemophilia B with pseudotumor formation in the pelvis and abdomen, where lesion location is relatively rare. After active and effective hemostasis, the patient's hematuria symptom gradually improved. This case suggests that early and timely hemostatic treatment is crucial for patients with hemophilia.

Effects of Curcumin on Bone Metabolism Balance in Ovariectomized Osteoporosis Model Rats Based on OPG/ RANKL Signaling Pathway / 中国药房

OBJECTIVE：To investigate the effects of curcumin on bone metabolism balance in ovariectomized osteoporosis model rats ，and to investigate its potential mechanism. METHODS ：Totally female Wistar rats were randomly divided into blank group（group A ），model group （group B ），estradiol group [group C （positive control ），estradiol valerate 50 μg（/ kg·d）]， curcumin low-dose ，medium-dose and high-dose groups [group D-F ，55，110，165 μg（/ kg·d）]，with 15 rats in each group. Except for group A ，other rats were ovariectomized to establish osteoporosis model. After modeling ，group A and B were given normal saline intrgastrically ，and administration groups were given relevant medicine intrgastrically 30 mL/kg，once a day ，for consecutive 12 weeks. The contents of serum bone metabolism markers [BALP ，CBF-α1，CTX-Ⅰ，PINP and OC] were determined by ELISA. The bone mineral density （BMD）and trabecular structure indexes [relative bone volume fraction （BV/TV），trabecular number （Tb.N），trabecular thickness （Tb.Th），connectivity density （Conn. D ），trabecular separation （Tb.Sp）and structure model index （SMI）] were determined by micro CT imaging system. The mRNA and protein expression of OPG and RANKL in hypothalamus and femur were determined by RT-PCR and Western blotting assay. RESULTS ：Compared with group A ，the contents of serum bone metabolism markers ，BMD，BV/TV，Tb.N，Tb.Th，Conn.D，mRNA and protein expression of OPG were decreased significantly in group B ，while Tb.Sp ，SMI，mRNA and protein expression of RANKL were increased significantly @qq.com （P＜0.05）. Compared with group B ，the contents of serum bone metabolism markers ，BMD，BV/TV，Tb.N，Tb.Th，Conn.D，mRNA and protein expression of OPG were increased significantly in administration groups ；Tb.Sp，SMI，mRNA and protein expression of RANKL were decreased significantly ，in a dose-dependent manner among curcumin groups （P＜0.05 or P＜0.01）. CONCLUSIONS ：Curcumin can improve the level of bone metabolism，increase BMD ，improve the trabecular microstructure and inhibit bone absorption in ovariectomized osteoporosis model rats. Its mechanism may be related to the regulation of OPG/RANKL signaling pathway.

ClinicalfeaturesandCTanalysesofhematopathyGassociatedairGleaksyndromes / 实用放射学杂志

Objective ThispaperpresentedaninvestigationonthesimilaritiesanddifferencesintheclinicalfeaturesandCTfindings betweenallogeneichematopoieticstem celltransplantation (allo-HSCT)inducedandnon-transplantinducedair-leaksyndromes (ALS)inpatientssufferingfromhematopathy,toimprovetheunderstandingofALSinpatientswithhematopathy.Methods Retrospective analysesandcomparisonsofclinicaldataandCTimageswereconductedbetweenGroupA (12patientswithALSafterallo-HSCT) andGroupB (26patientswithnon-transplant-relatedALS).A M annG W hitney U testwasperformedtoevaluatethemeasurementdata, andthe χ 2testor Fisher exacttestwasconductedtoexaminetheenumerationdata.Differencethresholdsof P＜0.05from bothsides weretakentobethedeterminantforstatisticalsignificance.Results TheincidenceratesofALSinpatientswithhematopathyafter anallo-HSCTwerefoundtobesignificantlyhigherthanthoseinpatientsonwhomsuchtransplantshadnotbeenperformed(1.84%. vs.0.06%),P＜0.001.SymptomsofdyspneaweremuchmorefrequentlyobservedingroupAcomparedtogroupB (7/12vs1/26), P＜0.01;whereasthedifferencesforthesymptomsofchesttightness,chestpain,andpharyngalgia werenotadequateintermsofstatistical significance,P＞0.05.IngroupA,theoccurrencesofALSsecondarytolongonsetnon-infectionpulmonarycomplications(LONIPC) associatedwithchronicgraft-versus-hostdisease(cGVHD)werefoundin8/12patients,whereastheoccurrencesin15/26patients weresecondarytopulmonaryinfectioningroupB,P＜0.01.Therewerenostatisticallysignificantdifferencesinage,gender,BMI, backgroundblooddisease,basictreatmentcounts,CTtype,treatmentmethodsandCTdisappearancetimelengthbetweenthetwo groups,P＞0.05.Conclusion Thereweredifferencesintheincidencerates,basiclungdiseasesandclinicalsymptomsbetweenallo-HSCT inducedandnon-transplantinducedALSinpatientssufferingfromhematopathy.Hematopathy-associatedALSwascommoninyoung adultswithlankypostures,patientswithleukemiaasback-grounddisease,patientswithahistoryofchemotherapyandpatientswith pulmonarydiseases.Thecommonsymptomsofpatients with hematopathy-associated ALS were chesttightness and chest pain,andpatients’overallprognosisweregood,meanwhileCT manifestationsweremainlycharacterizedbymixedpulmonary interstitialemphysema(PIE)+pneumomediastinum (PM)andsimplepneumothorax (PT).

Comparison of the clinical efficacy of two surgical methods for tibial plateau fractures / 中国基层医药

Objective To compare the efficacy between arthroscopic assisted reduction and traditional opera-tion approach for the treatment of tibial plateau fractures.Methods 75 patients with tibial plateau fracture(SchatzkerⅠ -Ⅳ type)were recruited,which were randomly divided into the arthroscopic and traditional operation group according to different treatment methods.The clinical efficacy and value of arthroscopically assisted reduction was ana-lyzed and compared according to the incidence of postoperative complications,the operative time,incision length,blood loss,hospital stay,and clinical efficacy between the two groups.Results Compared with the data in the traditional operation group,the incidence of postoperative complications (2.70% vs.21.3%)was significantly lower(χ2 =5.980,P =0.014).The operative time[(88.1 ±15.2)min vs.(103.8 ±22.1)min]was significantly shorter(t =3.575,P =0.001).And the incision length[(6.7 ±2.3)cm vs.(10.8 ±2.7)cm)]declined greatly(t =5.745,P =0.000).The hospital stay time and blood loss were significantly lower than that of the traditional operation group[(4.5 ± 2.3)d vs.(6.5 ±3.1)d;(145.2 ±43.0)mL vs.(294.4 ±90.2)mL,respectively],and the differences were statisti-cally significant(t =3.166,P =0.003;t =7.829,P =0.000,respectively).The excellent rate of clinical features in arthroscopic group(86.49%)was higher than that of traditional operation group(71.05%),and the difference was statistically significant(χ2 =3.723,P =0.039).Conclusion Arthroscopic -assisted reduction has many features for the tibial plateau fractures (SchatzkerⅠ -Ⅳ type),such as lower incidence of postoperative complications,less trau-ma,shorter operative time,and better postoperative knee function and so on,so it is worthy of clinical application.