Long-term Outcome In Chinese Patients Research Articles

Clinical characteristics and long-term follow-up outcomes of myelin oligodendrocyte glycoprotein antibody-associated disease in Han Chinese participants.

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is an immune-mediated inflammatory demyelinating disease of the central nervous system. This study aimed to delineate the clinical manifestations, imaging features, and long-term outcomes in Chinese patients with MOGAD and analyze the recurrence-associated factors. The phenotypic and neuroimaging characteristics of 15 Han Chinese patients with MOGAD were retrospectively analyzed. Demyelinating attacks, MOG antibodies in the cerebrospinal fluid/serum, response to immunotherapy, follow-up outcomes, and recurrence-associated factors were recorded. The median age at disease onset was 34 years (range, 4-65 years). The most common initial presentations included vision loss (10/15, 66.7%) and seizures (5/15, 33.3%). Serum MOG-Ab titers in 14/15 cases were higher than those in the cerebrospinal fluid and were detected in 3/6 relapsed patients. Brain magnetic resonance imaging during acute attacks showed lesions in 10/15 patients (66.7%), mostly in the cortex/subcortical white matter (5/15, 33.3%). Recurrence occurred in 6/15 patients (40.0%); in 4 patients, recurrence occurred shortly after immunotherapy discontinuation. Residual neurological deficits were present in 5/15 patients (33.3%), including visual impairment, incapacitation, cognitive impairment, and speech reduction. Optic neuritis was the most common clinical manifestation of MOGAD. magnetic resonance imaging findings were heterogeneous and the cerebral cortex/subcortical white matter was the most susceptible brain region. Although patients in the acute phase responded well to methylprednisolone pulse therapy, the long-term recurrence rate was high. Consistently detected serum MOG antibodies and inappropriate maintenance immunotherapy may be associated with recurrence, and residual neurological deficits should not be ignored.

Late-onset cblC defect: clinical, biochemical and molecular analysis

BackgroundcblC defect is the most common type of methylmalonic acidemia in China. Patients with late-onset form (>1 year) are often misdiagnosed due to heterogeneous symptoms. This study aimed to describe clinical characteristics and evaluate long-term outcomes of Chinese patients with late-onset cblC defect.MethodsA total of 85 patients with late-onset cblC defect were enrolled. Clinical data, including manifestations, metabolites, molecular diagnosis, treatment and outcome, were summarized and analyzed.ResultsThe age of onset ranged from 2 to 32.8 years old (median age 8.6 years, mean age 9.4 years). The time between first symptoms and diagnosis ranged from a few days to 20 years (median time 2 months, mean time 20.7 months). Neuropsychiatric symptoms were presented as first symptoms in 68.2% of cases, which were observed frequently in schoolchildren or adolescents. Renal involvement and cardiovascular disease were observed in 20% and 8.2% of cases, respectively, which occurred with the highest prevalence in preschool children. Besides the initial symptoms, the disease progressed in most patients and cognitive decline became the most frequent symptom overall. The levels of propionylcarnitine, propionylcarnitine / acetylcarnitine ratio, methylmalonic acid, methylcitric acid and homocysteine, were decreased remarkably after treatment (P<0.001). Twenty-four different mutations of MMACHC were identified in 78 patients, two of which were novel. The c.482G>A variant was the most frequent mutated allele in this cohort (25%). Except for 16 patients who recovered completely, the remaining patients were still left with varying degrees of sequelae in a long-term follow-up. The available data from 76 cases were analyzed by univariate analysis and multivariate logistic regression analysis, and the results showed that the time from onset to diagnosis (OR = 1.025, P = 0. 024) was independent risk factors for poor outcomes.ConclusionsThe diagnosis of late-onset cblC defect is often delayed due to poor awareness of its various and nonspecific symptoms, thus having an adverse effect on the prognosis. It should be considered in patients with unexplained neuropsychiatric and other conditions such as renal involvement, cardiovascular diseases or even multiple organ damage. The c.482G>A variant shows the highest frequency in these patients. Prompt treatment appears to be beneficial.

The Characteristics, Long-Term Outcomes, Risk Factors, and Antithrombotic Therapy in Chinese Patients With Atrial Fibrillation and Bioprosthetic Valves.

Introduction: There were few data about the clinical profiles and long-term outcomes in Chinese patients with atrial fibrillation (AF) and bioprosthetic valves.Methods: The retrospective study enrolled 903 patients with bioprosthetic valve replacement at our hospital and discharged with a diagnosis of AF from January 2010 to December 2018.Results: The median age was 65.6 (61.9–69.1) years, and 548 (60.7%) patients were women. During a follow-up period of 3.84 (2.64–5.51) years, 68 (1.8 per 100 person-years) patients died, 81 (2.1 per 100 person-years) patients developed thromboembolism, and 23 (0.6 per 100 person-years) patients experienced major bleeding. The CHA2DS2-VASc score, as a categorical variable (low, moderate, or high risk), predicted the risk of thromboembolism with the C-statistic of 0.6 (95% CI: 0.511–0.689, p = 0.046). The incidence of the CHA2DS2-VASc score increment was 11.6 per 100 person-years, and the annual reclassification rate of stroke risk (from a low or moderate group to a higher group) was 12.7%. The current proportion of oral anticoagulants was 52.3, 59, and 63.2%, respectively, in the low, moderate, and high stroke risk groups. Age (OR: 1.04, 95% CI: 1.01–1.06, p = 0.01), left atrial size (OR: 1.05, 95% CI: 1.03–1.08, p < 0.001), and rheumatic heart disease (OR: 1.49, 95% CI: 1.05–2.10, p = 0.025) were positively associated with the use of oral anticoagulants. The history of chronic kidney disease (OR: 0.20, 95% CI: 0.05–0.76, p = 0.018), prior surgical ablation (OR: 0.33, 95% CI: 0.24–0.47, p < 0.001), and antiplatelet agent use (OR: 0.08, 95% CI: 0.05–0.13, p < 0.001) were inversely related to the use of oral anticoagulants. Higher admission estimated glomerular filtration rate (HR: 0.515, 95% CI: 0.311–0.853, p = 0.01), left ventricular ejection fraction (HR: 0.961, 95% CI: 0.931–0.992, p = 0.014), concomitant surgical ablation (HR: 0.348, 95% CI: 0.171–0.711, p = 0.004), and rheumatic heart disease history (HR: 0.515, 95% CI: 0.311–0.853, p = 0.01) were associated with a lower risk of death. Surgical ablation (HR: 0.263, 95% CI: 0.133–0.519, p < 0.001) and oral anticoagulants (HR: 0.587, 95% CI: 0.375–0.918, p = 0.019) were related to a lower risk of thromboembolism.Conclusion: Chinese patients with AF and bioprosthetic valve(s) were relatively young and had a high prevalence of rheumatic heart disease with few comorbidities. The percentage of mitral bioprosthetic valve replacement was high. The proportion of concomitant surgical ablation or surgical left atrial appendage occlusion or exclusion was relatively low. The thromboembolic events were the major long-term adverse events. The anticoagulation therapy was underused in patients at moderate or high stroke risk. The CHA2DS2-VASc score was verified to be used for predicting stroke risk in this population. The stroke risk dynamically changed; it needed to be reestimated once the risk factor changed.

Myopathy is a Risk Factor for Poor Prognosis of Patients with Systemic Sclerosis: A retrospective cohort study.

To compare clinical characteristics and identify long-term outcomes of Chinese patients with systemic sclerosis (SSc) with and without muscle involvement.We retrospectively investigated the medical records, laboratory results, and computed tomography images of 204 consecutive SSc patients. Kaplan–Meier analysis was performed to determine survival rates. Patients were allocated into groups with and without myopathy.The prevalence of myopathy was 21.6%. The myopathy group was more likely to develop diffuse cutaneous involvement (90.9% vs 56%, P = .006), interstitial lung disease (90% vs 56%, P < .001), digestive system involvement (56.7% vs 29.3%, P = .001), pulmonary hypertension (29.5% vs 10.5%, P = .004), and pericardial effusion (25% vs. 10%, P = .019). Patients with myopathy had lower single-breath diffusing capacity of the lung for carbon oxide (46.5 ± 11.1 vs 57.1 ± 13.4, P < .001).Further, the myopathy group has similar results in interstitial lung disease associated higher resolution computed tomography score (186.8 ± 64.5 vs 152.3 ± 45.5, P = .037), Valentini score for disease activity (3.4 ± 0.9 vs 2.0 ± 0.9, P < .001) and modified Rodnan total skin score (19.4 ± 6.1 vs 15.1 ± 7.7, P = .002), compared with non-myopathy group. Kaplan–Meier survival analysis revealed decreased overall survival rate of the myopathy group (P = .028).SSc Patients with myopathy had more severe clinical manifestations and higher disease activity compared with those without, which affected survival rates and indicated worse prognosis.

Clinical features and long-term outcomes of Chinese patients with scleroderma renal crisis.

To investigate the clinical features, treatments, and long-term outcomes of Chinese patients with scleroderma renal crisis (SRC). We retrospectively reviewed the clinical and laboratory data of 538 patients with systemic sclerosis (SSc) at our center from January 2009 to December 2016, including 29 SRC and 509 SSc without SRC patients. The treatments and long-term outcomes of patients with SRC were also retrospectively analyzed. The prevalence of SRC was 5.4% in our cohort. Male gender (odds ratio [OR] =4.194 [95% CI 1.494-11.773]), glucocorticoid exposure (OR=3.666 [1.484-9.056]), pericardial effusion (OR=11.180 [4.515-27.681]), and myocardial involvement (OR=7.958 [1.664-38.064]) were associated with an increased risk of development of SRC. Despite the wide use of angiotensin-converting enzyme inhibitors, the permanent dialysis rate of patients with SRC was 48.3%. Sixteen patients died during follow-up, and the estimated 1- and 5-year survival rates of patients with SRC were 62.1% and 47.3%, respectively. Withdrawal of dialysis (5 patients) and myocardial complications (3 patients) were the main causes of death in patients with SRC. Patients with serum creatinine level >500µmol/L before treatment (log rank test 5.051, P=0.025) and/or those who needed dialysis at the onset of SRC (log rank test 12.870, P<0.001) showed poorer prognosis. SRC is a rare but severe complication in patients with SSc. Male gender, glucocorticoid exposure, pericardial effusion, and myocardial involvement were risk factors in the development of SRC. Withdrawal of dialysis and myocardial complications were the main causes of death in Chinese patients with SRC.

Long-term outcomes in Chinese patients with chronic hepatitis B receiving nucleoside/nucleotide analogue therapy in real-world clinical practice: 5-year results from the EVOLVE study.

In China, the optimal management of individuals living with chronic HBV infection (CHB) remains an unmet need. The EVOLVE Study was a 5-year prospective, longitudinal, observational study that compared the clinical outcomes in treatment-naive CHB patients receiving entecavir (ETV) or lamivudine (LAM)-based therapies. Males or females aged ≥18 years, diagnosed with CHB regardless of cirrhosis or hepatitis B e antigen (HBeAg) status were enrolled from tier 2 city hospitals (between 2012-2014). The choice of initial therapy and subsequent treatment modifications was at the discretion of treating physicians. Key outcomes included treatment modifications, virological response (HBV DNA <300 copies/ml) and HBV disease progression. Of the 3,408 patients enrolled, 1,807 and 628 received ETV and LAM-based therapy, respectively. The mean age was 39.5 years, 74% were male and 22.9% had cirrhosis. The rate of treatment modification was higher in the LAM-based versus ETV group (25.9% versus 13.7%); viral breakthrough was the most common reason in the LAM-based group versus financial reasons in the ETV group. At week 240, the virological response rate was 73% in both treatment groups. Compared with LAM-based therapy, ETV was associated with a significantly lower incidence of viral breakthrough (12.6% versus 2.1%) and genotypic resistance (10.1% versus 1.2%; P<0.0001 for both); significantly lower risk of HBV disease progression (14.0% versus 10.7%; P=0.0113); and lower rates of progression to decompensated cirrhosis (9.6% versus 6.4%) and hepatocellular carcinoma (1.9% versus 0.8%). This real-world, longitudinal study demonstrated a significantly lower risk of HBV-related disease progression, viral breakthrough and resistance with ETV versus LAM-based therapy. ClinicalTrials.gov NCT01726439.

P3634Impact of gender difference in clinical presentation on long-term outcomes in chinese patients undergoing percutaneous coronary intervention

P3634Impact of gender difference in clinical presentation on long-term outcomes in chinese patients undergoing percutaneous coronary intervention

Impact of high-sensitivity C-reactive protein on outcomes in patients with acute coronary syndrome undergoing drug-eluting stent implantation

Objective: To investigate the association between high-sensitivity C-reactive protein (hs-CRP) and long-term outcomes in Chinese patients with non-ST-segment elevation acute coronary syndrome (NSTE-ACS) after drug-eluting stent (DES) implantation. Methods: A total of 4 815 consecutive NSTE-ACS patients who treated with DESs were included.Patients were divided into three groups: <1.00 mg/L, 1.00 to 2.99 mg/L and ≥3.00 mg/L, based on the level of hs-CRP on admission.Major adverse cardiovascular and cerebrovascular events (MACCE, including all-cause death, myocardial infarction, revascularization, in-stent thrombosis and stroke) were compared among groups during 2-year follow-up. Results: Patients with higher hs-CRP had more risk factors of cardiovascular events such as concomitant morbidities and multi-vessel lesions(68.5% vs 73.6% vs 76.2%, P<0.001). Higher hs-CRP value was associated with increased rates of MACCE (8.8% vs 11.2% vs 12.6%, P=0.003) and revascularization (6.5% vs 8.5% vs 9.8%, P=0.003). However, the rates of all-cause death, myocardial infarction, stroke, and stent thrombosis were comparable among groups(all P>0.05). Ongoing divergences in MACCE and revascularization among three groups were significant on Kaplan-Meier curves (both Log-rank P=0.003). Multivariable Cox regression analysis indicated that compared to hs-CRP<1.00 mg/L group, MACCE in the >3.00 mg/L group was increased by 42% [HR 1.42 (1.13-1.78), P=0.002]. Meanwhile, multivessel leisions, ejection fraction<50%, elevated white blood cell counts were also independent risk factors.CRP≥3.00 mg/L(HR 1.56, 95%CI 1.16-2.08, P=0.003, compared to <1.00 mg/L) and multivessel leisions were independent predictors of revascularization. Conclusions: (1)Patients with higher hs-CRP on admission have more risk factors of cardiovascular events.(2)Higher hs-CRP value is associated with increased rates of MACCE and revascularization.(3)Pre-procedural hs-CRP is an independent predictor of 2-year outcomes for Chinese NSTE-ACS patients treated with DESs.

TIMI and GRACE Risk Scores Predict Both Short-Term and Long-Term Outcomes in Chinese Patients with Acute Myocardial Infarction.

Little is known about the long-term prognostic values of both thrombolysis in myocardial infarction (TIMI) and Global Registry of Acute Cardiac Events (GRACE) risk scores (RSs) to the Asian ethnicity. The purpose of this study is to compare the usefulness of these two scores in risk stratification and prediction of long-term (up to 3 years) outcomes for Chinese patients with myocardial infarction (MI). We calculated the TIMI and GRACE RSs for 726 consecutive patients with MI [55.6% with ST-segment elevation (STEMI) and 44.4% with non-ST-segment elevation (NSTEMI)]. Although the risk profile of our population (median TIMI score = 5 for STEMI, 4 for NSTEMI, and median GRACE score = 164) was higher, the in-hospital mortality (7.1% for NSTEMI and 6.7% for STEMI) was comparable to that predicted by GRACE RS. The GRACE RS worked well in predicting short-term and long-term death (C-statistics range 0.710 to 0.789) and triple (death, MI, and stroke) endpoints (C-statistics range 0.695 to 0.764) in both subsets of MI. GRACE RS performed significantly better than the TIMIRS in predicting 3-year mortality in NSTEMI (p = 0.035) and 1-year and 3-year mortality in STEMI (p = 0.028 and 0.009, respectively). Stratification by tertiles of GRACERS furnished greater prognostic information versus risk assessment by the TIMI RS. The use of RSs revealed a fair to good discriminatory accuracy in predicting both short-term and long-term major adverse cardiac events in Asian patients with MI. Compared with the simpler TIMI RS, the GRACERS was more accurate in predicting long-term mortality.

Effect of Cardiac Surgery-Associated Acute Kidney Injury on Long-Term Outcomes of Chinese Patients: A Historical Cohort Study

Background/Aims: To evaluate the long-term outcomes of Chinese patients with cardiac surgery-associated acute kidney injury (CSA-AKI). Methods: Patients who underwent cardiac surgery with a median 3-year follow-up were enrolled. The long-term survival rate and the incidence of chronic kidney disease (CKD) were recorded, and related risk factors were analyzed. Results: Of all 1,363 patients, 457 (33.5%) developed CSA-AKI. The AKI patients had a lower 3-year survival rate (88.8 vs. 97.2%, respectively, p < 0.001) and a higher incidence of CKD stages 3-5 (9.9 vs. 2.3%, respectively, p < 0.001) than the non-AKI patients. Cox regression analysis showed that AKI, atrial fibrillation, chronic cardiac insufficiency, longer surgical duration, respiratory failure after surgery, and longer mechanical ventilation time were associated with long-term mortality, while AKI, older age, and lower baseline kidney function were associated with incident CKD stages 3-5. Conclusion: CSA-AKI increased the risk of 3-year mortality and incident CKD stages 3-5.

Clinical Profiles and Prognosis of Patients with Distinct Antisynthetase Autoantibodies.

To compare the clinical characteristics and identify the longterm outcomes of Chinese patients with different antisynthetase antibodies. We investigated retrospectively 124 consecutive patients with antisynthetase syndrome. Medical records, laboratory results, and computed tomography images were obtained. The antisynthetase antibodies we investigated were anti-Jo1 (n = 62), anti-PL7 (n = 31), anti-PL12 (n = 12), and anti-EJ (n = 19). The overall prevalence of interstitial lung disease (ILD) reached 94.4% among study patients. Eleven patients (8.9%) developed rapidly progressive ILD (RP-ILD). Eight patients (6.5%) experienced malignancy. RP-ILD was statistically more prevalent in patients with antisynthetase syndrome with anti-PL7 than those without anti-PL7 (p = 0.028). Anti-Ro52-positive patients with antisynthetase syndrome experienced higher frequency of RP-ILD than those without anti-Ro52 (p = 0.001). Further, anti-PL7-positive patients coexisting with anti-Ro52 exhibited more RP-ILD than those without anti-Ro52 (p = 0.001). Patients with antisynthetase syndrome with RP-ILD had a higher proportion of neutrophils in bronchoalveolar lavage fluid and serum ferritin than those without RP-ILD (p = 0.006 and p = 0.013, respectively). Although no differences were observed between the Kaplan-Meier curves of the 4 antisynthetase antibodies subgroups (p = 0.349), the survival rate of patients with anti-PL7 decreased more rapidly in the early stage of longterm followup compared with those with other antisynthetase antibodies. The presence of RP-ILD, malignancy, and elevated serum ferritin was identified to be associated with poor prognosis in patients with antisynthetase syndrome. Our study investigates the clinical phenotypes and outcomes of patients with antisynthetase syndrome with distinct antisynthetase antibodies and highlights the link between the anti-PL7 antibody and RP-ILD.

Impact of type 2 diabetes mellitus on recurrent myocardial infarction in China.

To evaluate the influence of type 2 diabetes mellitus on the long-term outcomes of Chinese patients with previous myocardial infarction, we studied 864 patients with previous myocardial infarction, including 251 with type 2 diabetes mellitus and 613 without type 2 diabetes mellitus, over a median follow-up time of 2.9 years. The type 2 diabetes mellitus patients were subdivided into 95 insulin-treated diabetes mellitus and 156 non-insulin-treated diabetes mellitus subjects. The crude incidences (per 1000 patient-years) in the type 2 diabetes mellitus subjects versus the non-type 2 diabetes mellitus subjects were 43.7 versus 25.1 for recurrent myocardial infarction, 68.7 versus 28.3 for all-cause death and 99.8 versus 49.9 for the composite end point (i.e. recurrent myocardial infarction or all-cause death). Cox regression analysis showed that the adjusted hazard ratios for recurrent myocardial infarction, all-cause death and their combination were 1.67 (95% confidence interval: 1.06-2.74), 1.90 (1.25-2.90) and 1.72 (1.23-2.40), respectively. Significant associations were also observed between insulin treatment and all-cause death. Our findings suggested that type 2 diabetes mellitus is an independent risk factor for recurrent myocardial infarction, all-cause death and the composite end point among previous myocardial infarction patients.

Prevalence, Prognostic Factors, and Survival Analysis of Systemic Lupus Erythematosus-Related Serositis

Objective: To investigate the risk factors associated with systemic lupus erythematosus (SLE)-related serositis and its long-term outcome in Chinese patients. Method: Medical records of 609 hospitalized SLE patients who were followed up in Chang Gung Medical Center in Taiwan from 2005 to 2011 were retrospectively reviewed. Results: All 609 patients (89% women) who were fulfilled at least 4 of the ACR criteria for SLE and were included in the study. The mean ages at onset and at admission were 35.8 ± 16.5 years and 40.3 ± 16.0 years, respectively. Two hundred and thirty four patients (38%) had SLE-related serositis. 111 (18%) had pericarditis, 202 (33%) had pleuritis. 79 (13%) had concomitant pleuritis and pericarditis. Multivariate analysis indicated that the admission ages, proteinuria, thrombocytopenia, and lymphopenia, were independent predictors of serositis. Of the 609 SLE patients, 94 patients (15.4%) died during the study period. Of the 94 mortality cases, 63 patients had serositis. Multivariate Cox regression analysis indicated that the independent predictors of mortality were age ＞ 50 years at admission (Hazard ratio(HR) 3.10, p ＜ 0.001), thrombocytopenia (HR 3.23, p ＜ 0.001), serositis (HR 3.03, p ＜ 0.001), seizure (HR 2.13, p = 0.007) and leukopenia (HR 1.78, p = 0.041). Conclusion: This study underlines the prevalence and outcome of serositis in SLE, which is significant related to survival rates of patients. Survival was significantly lower in patients with serositis than in patients without serositis. Close follow up of SLE patients with serositis may be necessary.

Retinal artery lesions and long-term outcome in Chinese patients with acute coronary syndrome.

ObjectiveTo investigate the relationship between staging of retinal artery lesions and the prognosis of acute coronary syndrome (ACS) in a Chinese population.MethodsA total of 436 Chinese patients with ACS underwent coronary angiography and the eyes fundus examinations. All the patients were divided into three groups: group 1, no retinal artery lesions (n=111); group 2, retinal artery lesions of <Stage 2 (Stage 1—a broadening of the light reflex from the artery can be seen, with minimal or no arteriovenous compression; n=135); and group 3, retinal artery lesions of ≥Stage 2 (Stage 2—the changes similar to those in Stage 1, but more prominent, Stage 3—the arteries have a ‘copper wire' appearance and this is much more arteriovenous compression, and Stage 4—the arteries have a ‘silver wire' appearance and the arteriovenous crossing changes are more severe; n=190). The endpoints were main adverse cardiovascular and cerebrovascular events (MACCE), including all-cause death, myocardial infarction (MI), and stroke after 3–6 years of follow-up.ResultsThere was no significant differences of the baseline data among the three groups. After 3–6 years of follow-up, we found that patients of group 3 had more MACCE and death than those of the group 1 or group 2. Cox regression analysis found that factors related to the prognosis of ACS was staging of retinal artery lesions (P<0.05) in addition to traditional risk factors such as age, gender, diabetes, hypertension, and hypercholesterolemia.ConclusionStaging of retinal artery lesions plays an important role in the long-term outcome of patients with ACS.

New Attempts to Modify Periodontal Risk Assessment for Generalized Aggressive Periodontitis: A Retrospective Study

Periodontal risk assessment (PRA) model was designed for risk evaluation of treated patients with periodontal disease. However, its use on generalized aggressive periodontitis (GAgP) had been scarcely reported. This study aims to investigate the association of original PRA/modified PRA (MPRA) and compliance of periodontal maintenance with long-term treatment outcomes of Chinese patients with GAgP. Eighty-eight patients from a GAgP cohort, who completed active periodontal treatment (APT) and accepted reevaluation 3 to 11 years (mean of 5.5 years) afterward, were enrolled. PRA was modified (three strategies involving replacement of bleeding on probing with bleeding index >2, counting sites with probing depth ≥6 mm and changing method of bone loss [BL] calculation) to classify patients into different risk groups based on data at the first recall after APT. PRA and three MPRA models were investigated regarding long-term association with tooth loss (TL) and alteration of bone level (∆BL). Based on original PRA, 87 patients (98.8%) had a high-risk profile. According to three MPRA models, annual TL per patient values were greater in high-risk groups than in low-to-moderate risk groups (MPRA-1, 0.20 ± 0.33 versus 0.04 ± 0.14; MPRA-2, 0.18 ± 0.32 versus 0.05 ± 0.14; MPRA-3, 0.17 ± 0.32 versus 0.05 ± 0.15; P <0.05). By MPRA-1, irregular compliers with low-to-moderate risk profile had greater ∆BL (0.027 ± 0.031, indicating bone increment) than those with high risk (-0.012 ± 0.064, tendency for BL). For regular compliers, no significant differences of annual TL or ∆BL were found between risk groups. MPRA models could be used for evaluating the long-term outcomes of Chinese patients with severe GAgP, especially irregular compliers. High-risk patients of MPRAs exhibited more TL and less bone fill than low-to-moderate risk ones.

China Interventional Stroke Registry: Rationale and Study Design

Background: Angioplasty and stenting have recently become attractive options for cerebral large artery stenosis in China. However, there are limited data on safety and long-term outcomes in Chinese patients having undergone cerebral interventional procedures. To address this need, we set up a common database - the China Interventional Stroke Registry (CISR) - to describe patient characteristics, interventional images, periprocedural complications and long-term clinical outcomes and to determine how adherence to guideline-based treatments in the ‘real world' works in China. Methods: Six medical centers have participated since 2004. They entered into an Internet-based database data on demographics, clinical history and angiography of patients undergoing angioplasty with/without stenting in extracranial arteries (carotid, vertebral and subclavian arteries) and/or intracranial arteries (middle cerebral artery, vertebral artery and basilar artery) in China. CISR is a research-funded project. Results: As of October 2012, we have already collected detailed clinical information on 1,356 patients (age: 63.6 ± 10.2 years, male: 1,084, 79.9%). The overall data quality is good. Site data quality control is supported via detailed monthly feedback reports and quarterly data monitoring meetings. Conclusions: The CISR is the first and the largest registry for angioplasty with/without stents in China. The database will provide the characteristics and outcomes of patients and the situation of adherence to guideline-based treatments under ‘real-world' conditions in China.

Imatinib as preoperative therapy in Chinese patients with recurrent or metastatic GISTs.

Imatinib has dramatically altered the options for management of patients with gastrointestinal stromal tumours. However, it has become clear that secondary resistance to the drug develops during long-term therapy. The purpose of our study was to retrospectively analyze safety and long-term outcomes in Chinese patients with recurrent or metastatic GISTs treated with imatinib preoperatively. Between June 2003 and June 2011, 22 patients underwent surgery for recurrent or metastatic GISTs after preoperative treatment with imatinib. Complete resection was accomplished in 8 of the 10 responsive disease (RD) patients (80%), and in 3 of the 12 patients (25%) who had progression disease (PD). The amount of blood loss during the operation in PD patients was higher than in RD patients. There was 1 hospital death in PD group related to surgery, while the other patients recovered with conservative therapy because complications were mild. The difference in median PFS between patients with RD and those with PD was significant (24.8 vs. 2.81 months, P<0.001). The difference in 2-year OS rate between patients with RD and those with PD was not significant (100% vs. 87.5%, P>0.05). Our study indicates that surgical intervention can improve the PFS of Chinese patients with recurrent or metastatic GISTs responsive to imatinib, but does not prolong OS as well as in patients who develop imatinib resistance. Surgical resection following imatinib treatment is feasible and can be considered for patients with advanced GISTs responsive to imatinib.

Analysis of prognostic factors in 333 Chinese patients with high‐grade osteosarcoma treated by multidisciplinary combined therapy

To investigate prognostic factors for long-term outcomes in Chinese patients with high-grade osteosarcoma of the extremities or trunk treated by multidisciplinary combined therapy. In total, 333 consecutive patients with non-metastatic or primary metastatic high-grade osteosarcoma of the extremities or trunk were retrospectively reviewed to analyze the correlation between survival and sex, age, site, histological type, clinical stage, alkaline phosphatase (ALP) level, preoperative chemotherapy or not, response to preoperative chemotherapy, postoperative chemotherapy cycles and manner of surgery. The combined therapy included preoperative and postoperative chemotherapy as well as limb salvage surgery or amputation. The median survival time was 52 months for all 333 patients. Univariate analysis revealed that sex, ALP level, preoperative chemotherapy and cycle numbers of postoperative chemotherapy may influence the prognosis of high-grade osteosarcoma of the extremities or trunk. Multivariate analysis revealed that the female, a normal ALP level, preoperative chemotherapy with good response and ≥4 cycles of postoperative chemotherapy correlated with a better outcome. Preoperative chemotherapy is important in high-grade osteosarcoma treatment and a good response to it is an important marker of prognosis. It should be given with ≥ cycles of postoperative chemotherapy after surgery.

Clinicopathologic Features and Long-Term Outcomes of Chinese Patients with Hepatocellular Carcinoma in Non-Cirrhotic Liver

Background/Aims: Most hepatocellular carcinomas (HCC) are associated with cirrhosis. The clinicopathologic characteristics and outcomes of HCC present in non-cirrhotic livers are not well known in Chinese patients. This study was performed to explore the features of these patients and their outcomes after hepatectomy. Methods: 96 patients with histologically confirmed HCC in non-cirrhotic liver who underwent partial hepatectomy between 1995 and 2001 in our cancer center were reviewed. A retrospective analysis of the clinicopathologic features was performed, and survival of patients was analyzed by the Kaplan-Meier method and Cox regression model. Results: Operative mortality and morbidity were none and 8.3% (8/96), respectively. Postoperative overall survival (OS) rates at 1, 3, 5 and 10 years were 84.4, 62.5, 47.9 and 38.2%, respectively, with a median OS of 57 months. Disease-free survival (DFS) rates at 1, 3, and 5 years were 56.3, 39.6, and 33.3%, respectively, with a median DFS of 18 months. TNM stage was an independent prognostic factor for both OS and DFS of non-cirrhotic HCC. Operative blood loss was an independent prognostic factor for OS and DFS of patients who received curative resection. Conclusion: Curative partial hepatectomy was an effective and safe treatment for non-cirrhotic HCC. Aggressive local therapies were recommended for patients with intrahepatic recurrence.

Clinical characteristics of and long-term outcome in chinese patients with hypertrophic cardiomyopathy

Background Data on the phenotypical pattern and natural history of hypertrophic cardiomyopathy in Chinese patients are very limited. The purpose of this study was to describe the clinical characteristics of and long-term outcome in Chinese patients with hypertrophic cardiomyopathy. Methods We evaluated 118 Chinese patients (62 male) who were diagnosed with hypertrophic cardiomyopathy at Queen Mary Hospital from 1973 to 2002. Diagnosis was based on the demonstration of left ventricular hypertrophy (wall thickness ≥15 mm during diastole), either in a specific region or with diffuse distribution, using echocardiography or magnetic resonance imaging. Clinical predictors of major cardiovascular events related to hypertrophic cardiomyopathy (cardiovascular death, potentially fatal cardiac arrhythmia, and refractory heart failure requiring cardiac transplantation or percutaneous alcohol septal ablation) were evaluated with univariate and multivariate Cox proportional hazards regression models. Results The mean (± SD) age at presentation was 54 ± 18 years. During a mean follow-up of 5.8 ± 4.3 years (range, 1 to 29 years) from presentation, major cardiovascular events related to hypertrophic cardiomyopathy occurred in 19 patients (16%), including 9 deaths. Annual cardiovascular mortality was 1.6%. Fifty-five patients (47%) had one or more cardiovascular complications related to hypertrophic cardiomyopathy, of which atrial fibrillation was the most common (35%, n = 41). The most common type of hypertrophic cardiomyopathy was the apical variant (41%, n = 49). In multivariate analysis, female sex was the only independent predictor of major cardiovascular events related to hypertrophic cardiomyopathy (hazard ratio = 5.86; 95% confidence interval: 1.77 to 7.21; P = 0.007). Conclusion Hypertrophic cardiomyopathy in Chinese patients is characterized by late onset of presentation, a high incidence of the apical form of the condition, and adverse clinical outcome in female patients, which suggest a different phenotypical pattern than in white patients.