Nerve Growth Factor for the Treatment of Spinocerebellar Ataxia Type 3: An Open-label Study

Background:Spinocerebellar ataxia type 3 (SCA3) is the most common subtype of SCA worldwide,and runs a slowly progressive and unremitting disease course.There is currently no curable treatment availabl...     

Changes of QT Dispersion in Hemodialysis Patients after Administrating Zhigancao Decoction(炙甘草汤)

Objective: To observe the alteration of QT dispersion(QTd) and QTc dispersion(QTcd) in hemodialysis patients after oral administration of Zhigancao Decoction(炙甘草汤,Roasted Licorice Decoction,RLD).Methods: To investigate the alteration of QTd and QTcd in 68 routine hemodialysis patients before and after hemodialysis with 12-lead electrocardiogram(ECG) after orally administrated RLD for 4 weeks.Blood was also taken for measurement of plasma electrolytes,liver function,renal function,hemoglobin(Hgb) and hematocrit(HCT).Results: After hemodialysis,QTd and QTcd were prolonged evidently; the difference was significant between before and after hemodialysis(P0.05).After RLD orally administrated for 4 weeks,QTd and QTcd only slightly increased after dialysis compared with pre-dialysis(P0.05).The QTd and QTcd of the post-therapy-post-dialysis decreased significantly compared with the pre-therapy-post-dialysis(P0.05).There were no other significant changes in other variables(post-therapy-pre-dialysis vs.pre-therapy-pre-dialysis,or post-therapy-post-dialysis vs.pre-therapy-post-dialysis; P0.05).After therapy,the number of patients with supraventricular arrhythmia,occasional ventricular premature beat and multiple ventricular premature beat were decreased from 15 to 4,10 to 2 and 7 to 1,respectively.Conclusion: RLD therapy not only lowered the increased QTd and QTcd after hemodialysis,but also displayed a safety profile.     

Haematopoietic stem cell transplantation in Nigerian sickle cell anaemia children patients

Background:

Sickle cell anaemia (SCA) remains associated with high risks of morbidity and early death. Children with SCA are at high risk for ischaemic stroke and transient ischaemic attacks, secondary to intracranial arteriopathy involving carotid and cerebral arteries. Allogeneic haematopoietic stem cell transplantation (HSCT) is the only curative treatment for SCA. We report our experience with transplantation in a group of patients with the Black African variant of SCA.

Patients and Methods:

This study included 31 consecutive SCA patients who underwent bone marrow transplantation from human leukocyte antigen (HLA)-identical sibling donors between 2010 and 2014 following a myeloablative-conditioning regimen.

Results:

The median patient age was 10 years (range 2–17 years). Before transplantation, 14 patients had recurrent, painful, vaso-occlusive crisis; ten patients had recurrent painful crisis in association with acute chest syndrome; three patients experienced ischaemic stroke and recurrent vaso-occlusive crisis; two patients experienced ischaemic stroke; one patient exhibited leukocytosis; and one patient exhibited priapism. Of the 31 patients, 28 survived without sickle cell disease, with Lansky/Karnofsky scores of 100. All surviving patients remained free of any SCA-related events after transplantation.

Conclusion:

The protocols used for the preparation to the transplant in thalassaemia are very effective also in the other severe haemoglobinopathy as in the sickle cell anaemia with 90% disease free survival. Today, if a SCA patient has a HLA identical family member, the cellular gene therapy through the transplantation of the allogeneic haemopoietic cell should be performed. Tomorrow, hopefully, the autologous genetically corrected stem cell will break down the wall of the immunological incompatibility.     

Transcatheter Closure of Atrial Septal Defects Improves Cardiac Remodeling and Function of Adult Patients with Permanent Atrial Fibrillation

Background:

Permanent atrial fibrillation (AF) is the most common form of dysrhythmia associated with atrial septal defects (ASDs) in patients older than 40 years. However, little is known about cardiac remodeling after transcatheter closure in patients with permanent AF. This study was designed to compare cardiac events and remodeling effects after transcatheter closure in such patients.

Methods:

Clinical data of 289 adult patients older than 40 years who underwent ASD closure at our center were analyzed retrospectively. Of them, 63 patients with permanent AF were assigned to the case group, and the other 226 patients without permanent AF were assigned to the control group. Cardiac events and changes in left and right cardiac cavity dimensions before the procedure and 6 months after the procedure were compared between the two groups.

Results:

Patients in the case group were significantly older than those in the control group. The right ventricular (RV) volume and right atrial (RA) volume were decreased significantly in both the groups during a median follow-up period of 6 months after closure (P < 0.001). The left atrial dimensions, left ventricular end-systolic dimensions, left ventricular end-diastolic dimensions and left ventricular ejection fraction showed no significant change before and after the procedure in both the groups. Changes of the RV volume and RA volume in the case group were significantly smaller than those in the control group (P = 0.005 and P < 0.001). The New York Heart Association cardiac function was improved in both the groups during the 6 months follow-up period.

Conclusions:

The transcatheter closure of ASD can improve the cardiac remodeling and cardiac function in patients with or without AF.     

The effect of preoperative intravenous paracetamol administration on postoperative fever in pediatrics cardiac surgery

Background:

Post-operative fever is a common complication of cardiac operations, which is known to be correlated with a greater degree of cognitive dysfunction 6 weeks after cardiac surgery. The aim of the present study was to examine efficacy and safety of single dose intravenous Paracetamol in treatment of post-operative fever in children undergoing cardiac surgery.

Materials and Methods:

In this randomised, double-blind, placebo-controlled clinical trial, 80 children, aged 1-12 years, presenting for open heart surgery were entered in the trial and randomly allocated into two groups: Placebo and Paracetamol. After induction of anaesthesia, 15 mg/kg intravenous Paracetamol solution was infused during 1 h in the Paracetamol group. Patients in placebo group received 15 mg/kg normal saline infusion during the same time. Since the end of operation until next 24 h in intensive care unit, axillary temperature of the two group patients was recorded in 4-h intervals. Any fever that occurred during this period had been treated with Paracetamol suppository (125 mg) and the amount of antipyretic drug consumption for each patient had been recorded. In order to examine the safety of Paracetamol, patients were evaluated for drug complication at the same time.

Results:

Mean axillary temperature during first 24 h after operation was significantly lower in Paracetamol group compared with placebo group (P = 0.001). Overall fever incidence during 24 h after operation was higher in placebo group compared with Paracetamol group (P = 0.012). Of Paracetamol group patients, 42.5% compared with 15% of placebo group participants had no consumption of antipyretic agent (Paracetamol suppository) during 24 h after operation (P = 0.001).

Conclusion:

This study suggests that single dose administration of intravenous Paracetamol before paediatric cardiac surgeries using cardiopulmonary bypass; reduce mean body temperature in the first 24 h after operation.     

QT间期离散度与心肌梗塞预后的关系

目的探讨ＣＴ间期离散度对急性心肌梗塞近期预后的预测价值．方法计算方法：每导联均测ＱＴ间期３～５个．取均值．则ＱＴ间期离散度（ＱＴｄ）＝ＱＴｍａｘ－ＱＴｍｉｎ，用心率校正后的ＱＴ间期（ＱＴｃ）的离散度（ＱＴｃｄ）＝ＱＴｃｍａｘ－ＱＴｃｍｉｎ．结果急性心肌梗塞组与对照组ＱＴｃ、ＱＴｄ．ＱＴｃｄ值比较有显著差异（Ｐ＜０．００１）．梗塞组有无恶性心律失常及猝死者ＱＴｃ、ＱＴｄ、ＱＴｅｄ对比有显著差异（Ｐ＜０．０１）．当ＱＴＣ≥４４０ｍｓ，ＱＴｄ、ＱＴＣｄ≥６０ｍｓ时发生恶性心律失常及猝死的诊断正确指数ＱＴｃｄ与ＱＴＣ、ＱＴｄ比较有明显差异（Ｐ＜０．０５）．但ＱＴｄ与ＱＴＣ比较无明显差异（Ｐ＞０．０５）．结论ＱＴ离散度能更好反映心室复极过程，尤其是用心率校正后的ＱＴ离散度，对判断急性心肌梗塞近期预后具有重要指导价值．     

Defective lipid metabolism in sickle cell anaemia subjects in vaso-occlusive crisis

Background:

Abnormal lipid homeostasis has been reported in sickle cell anaemia (SCA) as well as in other haematological disorders. However, there is little information on the lipid profile of SCA subjects in vaso-occlusive crisis (VOC). This study determined the lipid profile of adult SCA subjects in VOC and in steady state (SSCA).

Materials and Methods:

Fifty-eight (58) adults with HbSS (30 in steady state and 28 in vaso-occlusive crisis) and 24 age-matched healthy individuals with HbAA genotype were recruited into this study. Standard methods were used for the determination of blood pressure (BP), packed cell volume (PCV), total white blood cell count (WBC) and haemoglobin phenotype. After an overnight fast, 5 ml of venous blood was obtained from each SSCA and the controls while samples were collected upon admission in the VOC group. Plasma lipid profile was determined using enzymatic method. Differences between two groups were determined using independent Student''s t-test or Man-Whitney U as appropriate. P-values less than 0.05 were considered significant.

Results:

Plasma total cholesterol (TC) and high density lipoprotein (HDL) were significantly lower while the ratio of triglyceride (TG) to HDL (TG/HDL) was significantly higher in SSCA compared with the controls. Low density lipoprotein (LDL) and TC were significantly lower in SCA subjects in VOC compared with controls. However, TC, TG, LDL and TG/HDL were significantly lower while HDL was significantly higher in VOC compared with SSCA.

Conclusion:

Sickle cell anaemia subjects have defective fasting lipid metabolism which becomes pronounced with VOC.     

Very Long-term Outcomes and Predictors of Percutaneous Coronary Intervention with Drug-eluting Stents Versus Coronary Artery Bypass Grafting for Patients with Unprotected Left Main Coronary Artery Disease

Background:

There are limited data on longer-term outcomes (>5 years) for patients with unprotected left main coronary artery (ULMCA) disease who underwent percutaneous coronary intervention (PCI) in the drug-eluting stents (DES) era. This study aimed at comparing the long-term (>5 years) outcomes of patients with ULMCA disease underwent PCI with DES and coronary artery bypass grafting (CABG) and the predictors of adverse events.

Methods:

All consecutive patients with ULMCA disease treated with DES implantation versus CABG in our center, between January 2003 and July 2009, were screened for analyzing. A propensity score analysis was carried out to adjust for potential confounding between the two groups.

Results:

Nine hundred and twenty-two patients with ULMCA disease were enrolled for the analyses (DES = 465 vs. CABG = 457). During the median follow-up of 7.1 years (interquartile range 5.3–8.2 years), no difference was found between PCI and CABG in the occurrence of death (P = 0.282) and the composite endpoint of cardiac death, myocardial infarction (MI) and stroke (P = 0.294). Rates of major adverse cardiac and cerebrovascular events were significantly higher in the PCI group (P = 0.014) in large part because of the significantly higher rate of repeat revascularization (P < 0.001). PCI was correlated with the lower occurrence of stroke (P = 0.004). Multivariate analysis showed ejection fraction (EF) (P = 0.012), creatinine (P = 0.016), and prior stroke (P = 0.031) were independent predictors of the composite endpoint of cardiac death, MI, and stroke in the DES group, while age (P = 0.026) and EF (P = 0.002) were independent predictors in the CABG group.

Conclusions:

During a median follow-up of 7.1 years, there was no difference in the rate of death between PCI with DES implantation and CABG in ULMCA lesions in the patient cohort. CABG group was observed to have significantly lower rates of repeat revascularization but higher stroke rates compared with PCI. EF, creatinine, and prior stroke were independent predictors of the composite endpoint of cardiac death, MI, and stroke in the DES group, while age and EF were independent predictors in the CABG group.     

Presence of Fragmented QRS Complexes in Patients with Obstructive Sleep Apnea Syndrome

Background:

Obstructive sleep apnea syndrome (OSAS) is a disease with increasing prevalence, which is mainly characterized by increased cardiopulmonary mortality and morbidity. It is well-known that OSAS patients have increased prevalence of cardiovascular diseases including coronary heart disease, heart failure, and arrhythmias. The aim of this study was to evaluate the presence of prolonged and fragmented QRS complexes, which have previously been associated with cardiovascular mortality, in OSAS patients.

Methods:

Our study included 51 patients (mean age 41.6 ± 10.1 years) who were recently diagnosed with OSAS (apnea-hypopnea index [AHI] ≥5 events/h) and never received therapy. The control group consisted of 34 volunteers (mean age 43.1 ± 11.6 years) in whom OSAS was excluded (AHI <5 events/h). The longest QRS complexes was measured in the 12-lead electrocardiogram (ECG) and the presence of fragmentation in QRS complexes was investigated.

Results:

Fragmented QRS frequency was significantly higher in patients with OSAS (n = 31 [61%] vs. n = 12 [35%], P = 0.021). QRS and QTc durations were also significantly longer in OSAS patients than controls (99.8 ± 13.9 ms vs. 84.7 ± 14.3 ms, P < 0.001; 411.4 ± 26.9 ms vs. 390.1 ± 32.2 ms, P = 0.001, respectively). Analysis of the patient and controls groups combined revealed a weak-moderate correlation between AHI and QRS duration (r = 0.292, P = 0.070). OSAS group had no correlation between AHI and QRS duration (r = −0.231, P = 0.203).

Conclusions:

In our study fragmented QRS frequency and QRS duration were found to increase in OSAS patients. Both parameters are related with increased cardiovascular mortality. Considering the prognostic importance of ECG parameters, it may be reasonable to recommend more detailed evaluation of OSAS patients with fragmented or prolonged QRS complexes with respect to presence of cardiovascular diseases.     

Serum Bilirubin and 6-min Walk Distance as Prognostic Predictors for Inoperable Chronic Thromboembolic Pulmonary Hypertension: A Prospective Cohort Study

Background:

Inoperable chronic thromboembolic pulmonary hypertension (CTEPH) is a severe clinical syndrome characterized by right cardiac failure and possibly subsequent liver dysfunction. However, whether serum markers of liver dysfunction can predict prognosis in inoperable CTEPH patients has not been determined. Our study aimed to evaluate the potential role of liver function markers (such as serum levels of transaminase, bilirubin, and gamma-glutamyl transpeptidase [GGT]) combined with 6-min walk test in the prediction of prognosis in patients with inoperable CTEPH.

Methods:

From June 2005 to May 2013, 77 consecutive patients with inoperable CTEPH without confounding co-morbidities were recruited for this prospective cohort study. Baseline clinical characteristics and 6-min walk distance (6MWD) results were collected. Serum biomarkers of liver function, including levels of aspartate aminotransferase, alanine aminotransferase, GGT, uric acid, and serum bilirubin, were also determined at enrollment. All-cause mortality was recorded during the follow-up period.

Results:

During the follow-up, 22 patients (29%) died. Cox regression analyses demonstrated that increased serum concentration of total bilirubin (hazard ratio [HR] = 7.755, P < 0.001), elevated N-terminal of the prohormone brain natriuretic peptide (HR = 1.001, P = 0.001), decreased 6MWD (HR = 0.990, P < 0.001), increased central venous pressure (HR = 1.074, P = 0.040), and higher pulmonary vascular resistance (HR = 1.001, P = 0.018) were associated with an increased risk of mortality. Serum concentrations of total bilirubin (HR = 4.755, P = 0.007) and 6MWD (HR = 0.994, P = 0.017) were independent prognostic predictors for CTEPH patients. Patients with hyperbilirubinemia (≥23.7 μmol/L) had markedly worse survival than those with normobilirubinemia.

Conclusion:

Elevated serum bilirubin and decreased 6MWD are potential predictors for poor prognosis in inoperable CTEPH.     

Dipstick urinalysis findings in children with Plasmodium falciparum in the South Tongu District: A case-control study

Background:

Malaria ranks among the major health and developmental challenges facing some of the poorest countries in tropical and sub-tropical regions across the globe. We determined urinary abnormalities and its relationship with parasite density in children ≤12 years with Plasmodium falciparum infection.

Materials and Methods:

From December 2013 to March 2014, we randomly recruited 116 participants comprising 58 malaria patients (cases) and 58 healthy controls from the Comboni Mission and the Sogakope District Hospitals both in the South Tongu district. Blood was collected for the estimation of hemoglobin and total white blood cells; thick and thin blood films were used for the determination of malaria parasite density. Urine was collected for the measurement of the various biochemical components using the automated urine analyzer. A pretested questionnaire was used to obtain demographic and clinical data.

Results:

Urine protein (P < 0.001), blood (P < 0.001), bilirubin (P < 0.001), urobilinogen (P < 0.001), and ketones (P = 0.001) were significantly higher in individuals with P. falciparum infection than in healthy controls. Proteinuria (P = 0.247; r = 0.155), hematuria (P = 0.142; r = 0.195), bilirubinuria (P = 0.001; r = 0.438), urobilinogenuria (P = 0.876; r = 0.021), and ketonuria (P = 0.136; r = 0.198) were positively correlated with malaria parasite density; however, only bilirubinuria was significantly higher at higher parasitemia.

Conclusion:

Malaria has a significant effect on the chemical composition of urine with bilirubin positively correlated with parasite density. Dipstick urinalysis can be used together with light microscopy in resource-limited malaria-endemic areas to accurately diagnose falciparum malaria infection.     

F Wave Study in Amyotrophic Lateral Sclerosis: Assessment of Segmental Motoneuronal Dysfunction

Background:

Dysfunctional spinal circuit may play a role in the pathophysiology of amyotrophic lateral sclerosis (ALS). The purpose of this study was to use F waves for assessment of segmental motoneuronal excitability following upper motor neuron (UMN) dysfunctions in ALS.

Methods:

We studied the F waves of 152 ulnar nerves recorded from abductor digiti minimi in 82 patients with ALS. Two groups of hands were defined based on the presence or absence of pyramidal signs in the same upper limb. The group with pyramidal signs in the upper limbs was designated as the P group, and the group without pyramidal signs in the upper limbs was designated as the NP group.

Results:

The mean (P < 0.001), median (P < 0.001) and maximum (P = 0.035) F wave amplitudes, mean (P < 0.001), median (P < 0.001) and maximum (P = 0.003) F/M amplitude ratio, index repeating neuron (P < 0.001) and index repeater F waves (P < 0.001) of the P group were significantly increased compared with the NP group. No significant differences were identified for F wave chronodispersion (P = 0.628), mean F wave latency (P = 0.151), minimum F wave latency (P = 0.211), maximum F wave latency (P = 0.199), F wave persistence (P = 0.738), F wave duration (P = 0.152), F wave conduction velocity (P = 0.813) and number of giant F waves (P = 0.072) between the two groups.

Conclusions:

In this study, increased F wave amplitude, F/M amplitude ratio and number of repeater F waves reflected enhanced segmental motoneuronal excitability following UMN dysfunctions in ALS.     

Pattern Differences of Small Hand Muscle Atrophy in Amyotrophic Lateral Sclerosis and Mimic Disorders

Background:

Amyotrophic lateral sclerosis (ALS) and some mimic disorders, such as distal-type cervical spondylotic amyotrophy (CSA), Hirayama disease (HD), and spinobulbar muscular atrophy (SBMA) may present with intrinsic hand muscle atrophy. This study aimed to investigate different patterns of small hand muscle involvement in ALS and some mimic disorders.

Methods:

We compared the abductor digiti minimi/abductor pollicis brevis (ADM/APB) compound muscle action potential (CMAP) ratios between 200 ALS patients, 95 patients with distal-type CSA, 88 HD patients, 43 SBMA patients, and 150 normal controls.

Results:

The ADM/APB CMAP amplitude ratio was significantly higher in the ALS patients (P < 0.001) than that in the normal controls. The ADM/APB CMAP amplitude ratio was significantly reduced in the patients with distal-type CSA (P < 0.001) and the HD patients (P < 0.001) compared with that in the normal controls. The patients with distal-type CSA had significantly lower APB CMAP amplitude than the HD patients (P = 0.004). The ADM/APB CMAP amplitude ratio was significantly lower in the HD patients (P < 0.001) than that in the patients with distal-type CSA. The ADM/APB CMAP amplitude ratio of the SBMA patients was similar to that of the normal controls (P = 0.862). An absent APB CMAP and an abnormally high ADM/APB CMAP amplitude ratio (≥4.5) were observed exclusively in the ALS patients.

Conclusions:

The different patterns of small hand muscle atrophy between the ALS patients and the patients with mimic disorders presumably reflect distinct pathophysiological mechanisms underlying different disorders, and may aid in distinguishing between ALS and mimic disorders.     

Body mass index,pain and function in individuals with knee osteoarthritis

Background:

Obesity is a risk factor for progression of knee osteoarthritis (OA), and high body mass index (BMI) may interfere with treatment effectiveness on pain and function in individuals with knee OA. This study investigated the effects of BMI on pain and function during a four-week exercise programme in patients with knee OA.

Materials and Methods:

Forty-six (31 women and 15 men) participants with knee OA of different BMI categories (15 normal weight participants, 13 over weight participants and 18 obese participants), received standardised exercise therapy programme twice a week for 4 weeks. Outcome included a 10-point pain rating scale for pain-intensity and the western Ontario and McMaster university osteoarthritis index (WOMAC) for physical function.

Results:

Two-way repeated measure analysis of variance (ANOVA) on pain assessment score revealed a significant effect of time (F = 1049.401, P < 0.001) and group (F = 9.393, P < 0.001) on pain. Similar significant effect of time (F = 595.744, P < 0.001) and group (F = 5.431, P = 0.008) was obtained for WOMAC score on function. Post hoc analysis revealed significant difference between the normal weight and overweight group (t = 2.472, P = 0.016) and between normal weight and obese group (t = 3.893, P = 0.005) on pain outcome at the 4^th week post treatment. No significant difference was found at 4^th week post treatment on WOMAC scores (F = 2.010, P = 0.146).

Conclusion:

Exercise improved pain and function scores in OA patients across the BMI groups. Overweight independent of obesity may interfere with effectiveness of pain control during the symptomatic treatment of knee OA patients.     

Lupus Myocarditis: A Case–Control Study from China

Background:

Myocarditis is an uncommon but serious manifestation of systemic lupus erythematosus (SLE). This study aimed to investigate clinical characteristics and outcomes of lupus myocarditis (LM) and to determine risk factors of LM in hospitalized Chinese patients with SLE.

Methods:

We conducted a retrospective case–control study. A total of 25 patients with LM from 2001 to 2012 were enrolled as the study group, and 100 patients with SLE but without LM were randomly pooled as the control group. Univariable analysis was performed using Chi-square tests for categorical variables, and the Student''s t-test or Mann–Whitney U-test was performed for continuous variables according to the normality.

Results:

LM presented as the initial manifestation of SLE in 7 patients (28%) and occurred mostly at earlier stages compared to the controls (20.88 ± 35.73 vs. 44.08 ± 61.56 months, P = 0.008). Twenty-one patients (84%) experienced episodes of symptomatic heart failure. Echocardiography showed that 23 patients (92%) had decreased left ventricular ejection fraction (<50%) and all patients had wall motion abnormalities. A high SLE Disease Activity Index was the independent risk factor in the development of LM (odds ratio = 1.322, P < 0.001). With aggressive immunosuppressive therapies, most patients achieved satisfactory outcome. The in-hospital mortality was not significantly higher in the LM group than in the controls (4% vs. 2%,P = 0.491).

Conclusions:

LM could result in cardiac dysfunction and even sudden death. High SLE disease activity might potentially predict the occurrence of LM at the early stage of SLE. Characteristic echocardiographic findings could confirm the diagnosis of LM. Early aggressive immunosuppressive therapy could improve the cardiac outcome of LM.     

Fractional Flow Reserve Guided Percutaneous Coronary Intervention Improves Clinical Outcome with Reduced Cost in Contemporary Clinical Practice

Background:Fractional flow reserve (FFR) is currently considered as the gold standard for evaluating the functional significance of coronary stenosis.However,its potential benefits in real-world practi...     

Comparing of Light Transmittance Aggregometry and Modified Thrombelastograph in Predicting Clinical Outcomes in Chinese Patients Undergoing Coronary Stenting with Clopidogrel

Background:

Several platelet function tests are currently used to measure responsiveness to antiplatelet therapy. This study was to compare two tests, light transmittance aggregometry (LTA) and modified thrombelastography (mTEG), for predicting clinical outcomes in Chinese patients after percutaneous coronary intervention (PCI).

Methods:

Prospective, observational, single-center study of 789 Chinese patients undergoing PCI was enrolled. This study was investigated the correlations between the two tests and performed receiver operating characteristic curve (ROC) analysis for major adverse cardiovascular events (MACEs) at 1-year follow-up.

Results:

MACEs occurred in 32 patients (4.1%). Correlations were well between the two tests in the adenosine diphosphate induced platelet reactivity (Spearman r = 0.733, P < 0.001). ROC-curve analysis demonstrated that LTA (area under the curve [AUC]: 0.677; 95% confidence interval [CI]: 0.643–0.710; P = 0.0009), and mTEG (AUC: 0.684; 95% CI: 0.650–0.716; P = 0.0001) had moderate ability to discriminate between patients with and without MACE. MACE occurred more frequently in patients with high on-treatment platelet reactivity (HPR) when assessed by LTA (7.4% vs. 2.7%; P < 0.001), and by TEG (6.7% vs. 2.6%; P < 0.001). Kaplan–Meier analysis demonstrated that HPR based on the LTA and mTEG was associated with almost 3-fold increased risk of MACE at 1-year follow-up.

Conclusions:

The correlation between LTA and mTEG is relatively high in Chinese patients. HPR measured by LTA and mTEG were significantly associated with MACE in Chinese patients undergoing PCI.     

Incidence and Clinical Characteristics of Pulmonary Hypertension in Patients with Idiopathic Pulmonary Fibrosis

Background:

Pulmonary hypertension (PH) frequently complicates the course of idiopathic pulmonary fibrosis (IPF) patients and is associated with significantly worse outcomes. The aim of the present study was to investigate the incidence of PH in IPF patients and evaluate the correlation between clinical parameters and systolic pulmonary artery pressure (sPAP).

Methods:

Hospitalized patients with IPF, who were evaluated for sPAP by Doppler echocardiography from January 2004 to December 2011, were enrolled in our study. Patients were defined as PH by an estimated sPAP > 50 mmHg and graded as PH likely, PH possible and PH unlikely, based on the 2009 European Society of Cardiology/European Respiratory Society PH Guidelines. The correlations between clinical parameters and sPAP were analyzed by multiple linear regression.

Results:

Totally, 119 IPF patients were enrolled in our study and 28 (23.5%), 20 (16.8%) and 71 (59.7%) patients were PH likely, PH possible and PH unlikely, respectively. Borg dyspnea score was positively correlated with sPAP, r = 0.467, P < 0.001. Oxygen saturation was negatively correlated with sPAP, r = −0.416, P < 0.001. Diffusing capacity of the lung for carbon monoxide percentage predicted was negatively correlated with sPAP, r = −0.424, P = 0.003. N-terminal fragment of pro-brain natriuretic peptide and pulmonary artery width was positively correlated with sPAP, r = 0.452, P = 0.011 and r = 0.513, P < 0.001, respectively.

Conclusions:

The incidence of PH in IPF patients was 23.5% in a single center of China. PH may worsen the dyspnea, right heart dysfunction and decrease the life quality of the patients with IPF.     

Importance of Sample Size for the Estimation of Repeater F Waves in Amyotrophic Lateral Sclerosis

Background:

In amyotrophic lateral sclerosis (ALS), repeater F waves are increased. Accurate assessment of repeater F waves requires an adequate sample size.

Methods:

We studied the F waves of left ulnar nerves in ALS patients. Based on the presence or absence of pyramidal signs in the left upper limb, the ALS patients were divided into two groups: One group with pyramidal signs designated as P group and the other without pyramidal signs designated as NP group. The Index repeating neurons (RN) and Index repeater F waves (Freps) were compared among the P, NP and control groups following 20 and 100 stimuli respectively. For each group, the Index RN and Index Freps obtained from 20 and 100 stimuli were compared.

Results:

In the P group, the Index RN (P = 0.004) and Index Freps (P = 0.001) obtained from 100 stimuli were significantly higher than from 20 stimuli. For F waves obtained from 20 stimuli, no significant differences were identified between the P and NP groups for Index RN (P = 0.052) and Index Freps (P = 0.079); The Index RN (P < 0.001) and Index Freps (P < 0.001) of the P group were significantly higher than the control group; The Index RN (P = 0.002) of the NP group was significantly higher than the control group. For F waves obtained from 100 stimuli, the Index RN (P < 0.001) and Index Freps (P < 0.001) of the P group were significantly higher than the NP group; The Index RN (P < 0.001) and Index Freps (P < 0.001) of the P and NP groups were significantly higher than the control group.

Conclusions:

Increased repeater F waves reflect increased excitability of motor neuron pool and indicate upper motor neuron dysfunction in ALS. For an accurate evaluation of repeater F waves in ALS patients especially those with moderate to severe muscle atrophy, 100 stimuli would be required.     

Whole Lung Lavage Treatment of Chinese Patients with Autoimmune Pulmonary Alveolar Proteinosis: A Retrospective Long-term Follow-up Study

Background:

Pulmonary alveolar proteinosis (PAP) is a rare lung disease, the most common type of which is autoimmune PAP. The gold standard therapy for PAP is whole lung lavage (WLL). Few studies have reported the optimal technique with which to evaluate the response to WLL. In this study, we aimed to identify parameters with which to assess the need for repeat WLL during a long-term 8-year follow-up.

Methods:

We conducted a retrospective analysis of 120 patients with autoimmune PAP with 80 of whom underwent WLL. Physiologic, serologic, and radiologic features of the patients were analyzed during an 8-year follow-up after the first WLL treatment.

Results:

Of the 40 patients without any intervention, 39 patients either achieved remission or remained stable and only one died of pulmonary infection. Of the 56 patients who underwent WLL for 1 time, 55 remained free from a second WLL and 1 patient died of cancer. Twenty-four required additional treatments after their first WLL. The baseline PaO₂ (P = 0.000), PA-aO₂ (P = 0.000), shunt fraction rate (P = 0.001), percent of predicted normal diffusing capacity of the lung for carbon monoxide (DLCO%Pred) (P = 0.016), 6-min walk test (P = 0.013), carcinoembryonic antigen (CEA) (P = 0.007), and neuron-specific enolase (NSE) (P = 0.003) showed significant differences among the three groups. The need for a second WLL was significantly associated with PaO₂ (P = 0.000), CEA (P = 0.050), the 6-minute walk test (P = 0.026), and DLCO%Pred (P = 0.041). The DLCO%Pred on admission with a cut-off value of 42.1% (P = 0.001) may help to distinguish whether patients with PAP require a second WLL.

Conclusions:

WLL is the optimal treatment method for PAP and provides remarkable improvements for affected patients. The DLCO%Pred on admission with a cut-off value of 42.1% may distinguish whether patients with PAP require a second WLL.