Objective: To evaluate the clinical effects of low- and intermediate-dose factor Ⅷ (F Ⅷ) prophylaxis in Chinese adult patients with severe hemophilia A. Methods: Thirty adult patients with severe hemophilia A who received low- (n=20) /intermediate-dose (n=10) F Ⅷ prophylaxis at Nanjing Drum Tower Hospital affiliated with Nanjing University Medical College were included in the study. The annual bleeding rate (ABR), annual joint bleeding rate (AJBR), number of target joints, functional independence score of hemophilia (FISH), quality of life score, and health status score (SF-36) before and after preventive treatment were retrospectively analyzed and compared. Results: The median follow-up was 48 months. Compared with on-demand treatment, low- and intermediate-dose prophylaxis significantly reduced ABR, AJBR, and the number of target joints (P<0.05) ; the improvement in the intermediate-dose prophylaxis group was better than that in the low-dose prophylaxis group (P<0.05). Compared with on-demand treatment, the FISH score, quality of life score, and SF-36 score significantly improved in both groups (P<0.05), but there was no significant difference between the two groups (P>0.05) . Conclusion: In Chinese adults with severe hemophilia A, low- and intermediate-dose prophylaxis can significantly reduce bleeding frequency, delay the progression of joint lesions, and improve the quality of life of patients as compared with on-demand treatment. The improvement in clinical bleeding was better with intermediate-dose prophylaxis than low-dose prophylaxis.
Humans ; Hemophilia A/drug therapy* ; Factor VIII/therapeutic use* ; Quality of Life ; Retrospective Studies ; Hemarthrosis/prevention & control* ; Hemorrhage/drug therapy*

OBJECTIVE: To explore the effect of lenalidomide on human fibroblast-like synovial cells (HFLS) and the therapeutic efficacy on hemophilic arthropathy in hemophilia A mice model. METHODS: In vitro, to remodel the inflammatory environment of synovial tissue after hemorrhage, ferric citrate and recombinant TNF-α were added into the cell culture medium of HFLS. Cell Counting Kit-8 (CCK-8), Enzyme-linked immunosorbent assay (ELISA), Quantitative Real-time PCR (RT-qPCR) and flow cytometry were employed for detection of the effects of lenalidomide on the proliferation ability, pro-inflammatory cytokines release and apoptosis of HFLS cells. In vivo, hemophilia arthropathy was remodeled in hemophilia A mice by induction of hemarthrosis. A series of doses of lenalidomide (0.1, 0.3 and 1.0 g/kg) was administrated intra-articularly. Tissues of knee joints were collected on the 14th day after administration, and the protective effect of lenalidomide on arthritis in hemophilia A mice were evaluated by RT-qPCR and histological grading. RESULTS: In vitro, compared with the untreated control group, lenalidomide could significantly inhibit the proliferation of HFLS cells (P<0.05), and the effect was the most significant when the concentration was 0.01 μmol/L (P<0.001). Compared with the control group, lenalidomide could significantly inhibit the expression levels of TNF-α, IL-1β, IL-6 and IFN-γ in HFLS cells (P<0.05). The flow cytometry results showed that lenalidomide could enhance the apoptotis of HFLS cells (P<0.05). The results of RT-qPCR showed that lenalidomide could significantly reduce the mRNA expression levels of TNF-α, IL-1β, IL-6,MCP-1 and VEGF in the joint tissues (P<0.05). Histological results showed that compared with the injured group, lenalidomide could significantly reduce the pathological sequela after hemarthrosis induction, e.g. synovial thickening and neo-angiogenesis in the synovium. The protection displayed a dose-response pattern roughly. CONCLUSION In vitro, lenalidomide can inhibit the proliferation of HFLS cells, promote their apoptosis, and inhibit the expression of pro-inflammatory cytokines. In vivo, lenalidomide can significantly decrease the expression of pro-inflammatory cytokines in the joints of mice, and prevent the development of inflammation and neo-angiogenesis. The results provide a theoretical and experimental basis for the clinical application of lenalidomide in the treatment of hemophilic arthropathy.
Animals ; Arthritis ; Cytokines/metabolism* ; Hemarthrosis/pathology* ; Hemophilia A/genetics* ; Humans ; Interleukin-6 ; Lenalidomide ; Mice ; Neovascularization, Pathologic ; RNA, Messenger ; Sincalide ; Tumor Necrosis Factor-alpha ; Vascular Endothelial Growth Factor A

Objective: To reveal the related factors of inhibitors and differences ofhemorrhage and joint disease before and after the production of inhibitors in children with hemophilia A (HA) . Methods: Retrospective analyses of the clinical data of 381 children with HA under the age of 16 registered in the Registration Management Center of Hemophilia in Henan Provincial from January 2015 to August 2018. Results: A total of the 381 children were enrolled with 116 (30.4%) mild, 196 (51.4%) moderate, and 69 (18.1%) severe cases; 54 patients (14.2%) had inhibitors, including 22 high and 32 low titer inhibitors. Positive family history was positively associated with inhibitors[P<0.001, OR=3.299 (95%CI 1.743-5.983) ], and high-intensity exposure was associated with inhibitors[P=0.002, OR=2.587 (95%CI 1.414-4.731) ]. High-intensity exposure was associated with high titer inhibitor production[P=0.001, OR=8.689 (95%CI 2.464-30.638) ], and high-intensity exposure increased the risk of high titer inhibitors in HA patients. After inhibitors occurred in 54 patients with HA, the rates of overall joint annual bleeding (z=-3.440, P=0.001) and traumatic annual bleeding (z=-2.232, P=0.026) increased, but the rates of the annual joint bleeding (z=-1.342, P=0.180) and spontaneous annual bleeding (z=-1.414, P=0.157) remained to be not statistically significant. The joint ultrasound score did not change significantly after the inhibitor information (z=-0.632, P=0.527) . Conclusions: Positive family history and high-intensity exposure could increase the risk of F Ⅷ inhibitors in HA patients, and high-intensity exposure increased the risk of high titer inhibitors. The rates of the overall joint annual bleeding and traumatic annual bleeding increased after the inhibitor information.
Child ; Factor VIII/therapeutic use* ; Hemarthrosis ; Hemophilia A/drug therapy* ; Hemorrhage ; Humans ; Retrospective Studies

Hemarthrosis occurring after arthroscopic surgery for lesions of the shoulder joint is a very rare complication that can develop due to an injury to the blood vessels when an anterior portal is formed. This is a complication that rarely develops in patients who are taking antithrombotic drugs or who do not have associated diseases, such as thrombocytopenia. We report a case of hemarthrosis that occurred after performing arthroscopic surgery to repair a rotator cuff tear in a patient with a stenosis in an arteriovenous fistula for hemodialysis in the ipsilateral upper arm.
Arm ; Arteriovenous Fistula ; Arthroscopy ; Blood Vessels ; Constriction, Pathologic ; Hemarthrosis ; Humans ; Kidney Failure, Chronic ; Renal Dialysis ; Rotator Cuff ; Shoulder ; Shoulder Joint ; Tears ; Thrombocytopenia

OBJECTIVE: To assess the clinical outcome of chemical synovectomy with rifampicin in hemophilic arthropathy by using the World Federation of Hemophilia (WFH) scoring system and plain radiograph. METHODS: We performed rifampicin synovectomy (RS) on 30 joints of 28 hemophilic patients diagnosed as hemophilic arthropathy stage I–III (based on Fernandez-Palazzi clinical classification). Clinical status (bleeding frequency, pain, joint physical status) and radiological staging were evaluated as parts of the WFH scoring system before and 1 year after RS. The patients were divided into two groups by the Arnold-Hilgartner scale of the initial X-ray as stage 3 or less for the low-stage group (n=17) and over 3 for the high-stage group (n=13). RESULTS: Total WFH joint physical scores were reduced after injection, and the number of bleeding episodes and pain showed especially significant improvement. For other subscores of the WFH joint physical score, only swelling, range of motion, and crepitus showed statistically significant improvement. According to the severity of the radiologic finding, the WFH joint physical score of both the low-stage and high-stage groups showed significant improvement. In the radiological aspect, the low-stage group, without joint space narrowing at the initial plain radiograph, showed no further aggravation after injection. However, in the high-stage group, radiology found aggravation regardless of the procedure. CONCLUSION: It is suggested that chemical synovectomy with rifampicin may prevent hemarthrosis and improve clinical symptoms. Especially in the early stage of arthropathy without joint-space narrowing, it seems to have an additional benefit that delays radiological aggravation and preserves joint status.
Arthralgia ; Hemarthrosis ; Hemophilia A ; Hemorrhage ; Humans ; Joints ; Range of Motion, Articular ; Rifampin*

PURPOSE: The purpose of this study is to evaluate the incidence and treatment of recurrent hemarthrosis after total knee replacement (TKR). MATERIALS AND METHODS: Among a total of 5,510 patients who underwent TKR from March 2000 to October 2016, patients who had two or more bleeding 2 weeks after surgery were studied. Conservative treatments were performed for all cases with symptoms. In patients who did not respond to conservative treatment several times, embolization was performed. We retrospectively evaluated the postoperative bleeding time, bleeding frequency, treatment method, and outcome. RESULTS: Seventeen (0.3%) of the 5,510 patients developed recurrent hemarthrosis. Bleeding occurred at an average of 2 years 3 months after the operation. Joint aspiration was performed 3.5 times (range, 2 to 10 times) on average, and 14 cases (82.3%) were treated with conservative treatment. In 3 patients with severe bleeding and hemorrhage, embolization was performed. CONCLUSIONS: Recurrent hemarthrosis after TKR is a rare disease with a low incidence of 0.3% and usually could be treated by conservative treatment. If recurrences occur repeatedly, embolization through angiography or surgical treatment may be considered, but the results are not satisfactory and careful selection of treatment modalities is warranted.
Angiography ; Arthroplasty ; Arthroplasty, Replacement, Knee ; Bleeding Time ; Hemarthrosis ; Hemorrhage ; Humans ; Incidence ; Joints ; Knee ; Methods ; Rare Diseases ; Recurrence ; Retrospective Studies

Objective: To assess the feasibility of HEAD-US scale in the clinical application of hemophilic arthropathy (HA) and propose an optimized ultrasound scoring system. Methods: From July 2015 to August 2017, 1 035 joints ultrasonographic examinations were performed in 91 patients. Melchiorre, HEAD-US (Hemophilic Early Arthropathy Detection with UltraSound) and HEAD-US-C (HEAD-US in China) scale scores were used respectively to analyze the results. The correlations between three ultrasound scales and Hemophilia Joint Health Scores (HJHS) were evaluated. The sensitivity differences of the above Ultrasonic scoring systems in evaluation of HA were compared. Results: All the 91 patients were male, with median age of 16 (4-55) years old, including 86 cases of hemophilia A and 5 cases hemophilia B. The median (P₂₅, P₇₅) of Melchiorre, HEAD-US and HEAD-US-C scores of 1 035 joints were 2(0,6), 1(0,5) and 2(0,6), respectively, and the correlation coefficients compared with HJHS was 0.747, 0.762 and 0.765 respectively, with statistical significance (P<0.001). The positive rates of Melchiorre, HEAD-US-C and HEAD-US scale score were 63.0% (95%CI 59.7%-65.9%), 59.5% (95%CI 56.5%-62.4%) and 56.6% (95%CI 53.6%-59.6%) respectively, and the difference was statistically significant (P<0.001). Even for 336 cases of asymptomatic joints, the positive rates of Melchiorre, HEAD-US-C and HEAD-US scale score were 25.0% (95%CI 20.6%-29.6%), 17.0% (95%CI 12.6%-21.1%) and 11.9% (95%CI 8.4%-15.7%) respectively, and the difference was statistically significant (P<0.001). There were significant changes (P<0.05) in the ultrasonographic score of HA before and after onset of hemorrhage in 107 joints of 40 patients. The difference in variation amplitude of HEAD-US-C scores and HEAD-US scores before and after joint bleeding was statistically significant (P<0.001). Conclusion: Compared with Melchiorre, there were similar good correlations between HEAD-US, HEAD-US-C and HJHS. HEAD-US ultrasound scoring system is quick, convenient and simple to use. The optimized HEAD-US-C scale score is more sensitive than HEAD-US, especially for patients with HA who have subclinical state, which make up for insufficiency of sensitivity in HEAD-US scoring system.
Adolescent ; Adult ; Child ; Child, Preschool ; China ; Hemarthrosis ; Hemophilia A ; Hemophilia B ; Humans ; Male ; Middle Aged ; Ultrasonography ; Young Adult

BACKGROUND: Persistent pain after total knee arthroplasty (TKA) is dissatisfying to the patient and frustrating to the surgeon. The purpose of this study is to evaluate the aseptic causes and clinical course of intractable pain following TKA. METHODS: Of the total 2,534 cases of primary TKA reviewed, 178 cases were classified as having aseptic persistent pain that was not resolved within 1 year after surgery. Except for the cases with periprosthetic fracture (56 knees), 122 cases of aseptic painful TKA were divided into two groups: intra-articular group (83 knees) and extra-articular group (39 knees). RESULTS: In the intra-articular group, the main reasons for pain were aseptic loosening (n = 40), polyethylene wear (n = 16), instability (n = 10), recurrent hemarthrosis (n = 5), patellar maltracking (n = 4), tendon ruptures (n = 4), and stiffness (n = 2). In the extraarticular group, 10 knees (25.6%) were found to have nerve entrapment in the spine, 6 knees (15.4%) were found to have hip osteoarthritis or femoral head avascular necrosis. The reasons for persistent knee pain in the remaining 23 knees (59.0%) still remain elusive. CONCLUSIONS: Persistent pain after TKA originated from pathology of extra-articular origin in a considerable number of cases in this study. Therefore, it is important to perform thorough preoperative evaluations to reduce pain resulting from extra-articular causes. Furthermore, meticulous surgical procedures and optimal alignment are required to reduce pain of intra-articular origin related to implant wear, instability, and patellar maltracking.
Arthroplasty, Replacement, Knee* ; Chronic Pain ; Head ; Hemarthrosis ; Humans ; Knee ; Necrosis ; Nerve Compression Syndromes ; Osteoarthritis, Hip ; Pain, Intractable ; Pathology ; Periprosthetic Fractures ; Polyethylene ; Rupture ; Spine ; Tendons

Traumatic or spontaneous hemarthroses are an important cause of joint effusions, and can complicate innate or acquired coagulopathies. The elbow is an unusual location for a spontaneous hemarthrosis; we describe a previously unreported case of warfarin-induced spontaneous elbow hemarthrosis, diagnosed by point-of-care ultrasound. On the basis of clinical and ultrasound findings arthrocentesis was deferred, and the patient was successfully treated with warfarin reversal and conservative care. Physical examination is unreliable for the detection of a joint effusion, and misdiagnosis and can lead to unnecessary investigation or resource use. Point-of-care ultrasound allows accurate, prompt, direct visualization of a joint effusion, and non-invasive confirmation of a hemarthrosis. Ultrasound can facilitate accurate diagnosis and characterization of joint effusions to improve the care of patients with coagulopathy.
Arthrocentesis ; Diagnosis ; Diagnostic Errors ; Elbow* ; Hemarthrosis* ; Humans ; Joints ; Physical Examination ; Point-of-Care Systems* ; Ultrasonography* ; Warfarin

Synovial hemangioma is a rare benign intraarticular tumor. Synovial hemangioma of the knee joint has unspecific symptoms, which are pain, limitation of motion and hemarthrosis, often seen as an internal derangement of the knee. A 24-year-old woman presented with intermittent joint effusion and pain of the left knee joint and physical examination revealed slight atrophy of the quadriceps and tenderness around lateral joint line of knee. The patient was performed arthroscopic surgery due to synovial hemangioma about 17-years ago. Magnetic resonance imaging (MRI) showed the synovial hemangioma from Hoffa fat pad to anterior aspect of lateral meniscus, and thus surgical treatment was planned. Arthroscopic excision was performed and additional lesion was observed in lateral gutter, suprapatellar pouch of the knee. The biopsy confirmed the diagnosis of synovial hemangiomas. After 3 year, the patient was completely asymptomatic and showed no signs of recurrence.
Adipose Tissue ; Arthroscopy ; Atrophy ; Biopsy ; Diagnosis ; Female ; Hemangioma* ; Hemarthrosis ; Humans ; Joints ; Knee Joint ; Knee* ; Magnetic Resonance Imaging ; Menisci, Tibial ; Physical Examination ; Recurrence ; Young Adult