Pediatric asthma quality of life questionnaire (PAQLQ): Validation among asthmatic children in Thailand

Quality of life (QoL) is an important consideration among asthma sufferers. The Pediatric Asthma Quality of Life Questionnaire (PAQLQ) is one of the most widely used instruments for measuring health-related QoL in children with asthma. The standardized version of PAQLQ contains 23 questions in three domains, i.e., activity limitation, symptoms and emotional function. The objective of this study was to validate the Thai-translated version of the PAQLQ. The study design consisted of a five-week single cohort study. Patients recorded symptoms, and peak expiratory flow rate (PEFR) each morning and evening during the first and fifth week of the study in asthma diary. At each clinic visit, a trained-interviewer administered the PAQLQ and performed spirometric measurements. Fifty-one children, ages between 7 and 17 yr participated in the study. Scores from the asthma diary were used to classify patients into stable vs. unstable groups. The construct validity of the questionnaire was confirmed in both cross-sectional and longitudinal studies by demonstrating correlations between various PAQLQ domains with clinical asthma parameters (asthma diary, beta-agonist use and PEFR). There was high internal consistency for scores of the three domains (Cronbach's alpha-coefficient = 0.83-0.95). For those with stable asthma, the reliability of PAQLQ was good for the rating scale (intra-class correlation coefficient--ICC = 0.84) and for total score (alpha = 0.97) indicating high reproducibility of the PAQLQ. The significant difference of changes QoL scores between stable and unstable groups was observed in all domains. We conclude that the Thai version of PAQLQ is valid and reliable for implementing in Thai children with asthma.     

直肠癌切除前后腹腔冲洗液脱落细胞学对比研究

目的研究直肠癌术中腹腔多次冲洗，对于降低患者腹腔内脱落癌细胞阳性率的临床意义。方法对63例直肠癌患者术中腹腔冲洗分6次进行，肿瘤切除前3次，切除后3次；将6次腹腔冲洗液行常规病理学涂片细胞学检查结果进行对比。结果肿瘤切除前，全组患者腹腔冲洗液脱落癌细胞均呈阳性表达。肿瘤切除后第1次腹腔冲洗液脱落癌细胞阳性40例，第2次阳性33例，第3次阳性13例。肿瘤切除后第1次冲洗液的脱落癌细胞阳性结果与切除后第2次冲洗液阳性结果比较，P〉0.05，差异无统计学意义；但第3次冲洗液的脱落癌细胞阳性结果与第2次比较，P〈0.01，差异有统计学意义。结论对直肠癌患者进行术中多次腹腔冲洗可降低腹腔内脱落癌细胞的阳性率。     

Inherited focal, episodic neuropathies

Hereditary neuropathy with liability to pressure palsies (HNPP; also called tomaculous neuropathy) is an autosomal-dominant disorder that produces a painless episodic, recurrent, focal demyelinating neuropathy. HNPP generally develops during adolescence, and may cause attacks of numbness, muscular weakness, and atrophy. Peroneal palsies, carpal tunnel syndrome, and other entrapment neuropathies may be frequent manifestations of HNPP. Motor and sensory nerve conduction velocities may be reduced in clinically affected patients, as well as in asymptomatic gene carriers. The histopathological changes observed in peripheral nerves of HNPP patients include segmental demyelination and tomaculous or “sausage-like” formations. Mild overlap of clinical features with Charcot-Marie-Tooth (CMT) disease type 1 (CMT1) may lead patients with HNPP to be misdiagnosed as having CMT1. HNPP and CMT1 are both demyelinating neuropathies, however, their clinical, pathological, and electrophysiological features are quite distinct. HNPP is most frequently associated with a 1.4-Mb pair deletion on chromosome 17p12. A duplication of the identical region leads to CMT1A. Both HNPP and CMT1A result from a dosage effect of the PMP22 gene, which is contained within the deleted/duplicated region. This is reflected in reduced mRNA and protein levels in sural nerve biopsy samples from HNPP patients. Treatment for HNPP consists of preventative and symptom-easing measures. Hereditary neuralgic amyotrophy (HNA; also called familial brachial plexus neuropathy) is an autosomal-dominant disorder causing episodes of paralysis and muscle weakness initiated by severe pain. Individuals with HNA may suffer repeated episodes of intense pain, paralysis, and sensory disturbances in an affected limb. The onset of HNA is at birth or later in childhood with prognosis for recovery usually favorable; however, persons with HNA may have permanent residual neurological dysfunction following attack(s). Episodes are often triggered by infections, immunizations, the puerperium, and stress. Electrophysiological studies show normal or mildly prolonged motor nerve conduction velocities distal to the affected brachial plexus. Pathological studies have found axonal degeneration in nerves examined distal to the plexus abnormality. In some HNA pedigrees there are characteristic facial features, including hypotelorism. The prognosis for recovery of normal function of affected limbs in HNA is good, although recurrent episodes may cause residual deficits. HNA is genetically linked to chromosome 17q25, where mutations in the septin-9 (SEPT9) gene have been found.     

三磷酸腺苷结合盒运转体A1与颈动脉粥样硬化斑块的关系

目的 探讨三磷酸腺苷结合盒运转体A1(ATP binding cassette transporter A1,ABCA1)在人颈动脉粥样硬化斑块中的表达变化及作用机制.方法 收集24例人颈动脉粥样硬化斑块标本和10例肠系膜动脉标本(对照组),采用RT-PCR测定ABCA1 mRNA和视黄酸X受体α(RXRα)mRNA表达水平,并采用Western Blot检测ABCA1及RXRα的蛋白表达水平.24例人颈动脉粥样硬化斑块标本按病理分级,比较病理组织为Ⅲ级和Ⅰ级动脉粥样硬化组织间ABCA1 mRNA、RXRαmRNA表达水平及蛋白表达水平.结果 颈动脉粥样硬化斑块组的ABCA1 mRNA(0.79±0.04)和RXRα mRNA(0.73±0.04)表达与对照组相比上调,差异有统计学意义(P＜0.05);ABCA1 mRNA与RXRα mRNA增加水平相关(P＜0.05);颈动脉粥样硬化斑块的ABCA1蛋白表达(0.22±0.03)下调水平与对照组(0.53±0.03)相比差异有统计学意义(P＜0.05);Ⅲ级和Ⅰ级动脉硬化斑块ABCA1mRNA、RXRα mRNA及蛋白表达水平差异有统计学意义(P＜0.05).结论 ABCA1及RXRα蛋白表达水平下调可能是进展性动脉粥样硬化损害的关键因素.     

大鼠静脉窦高压致硬膜血管变化的研究

目的探讨大鼠静脉窦高压后硬膜血管变化及其静脉窦高压在硬脑膜动静脉瘘形成中的作用。方法体重200～250g的SD雄性大鼠110只,随机分为静脉窦高压组(85只)和假手术组(25只),将静脉窦高压组85只大鼠闭塞左侧横窦和上矢状窦前1／3,并吻合右侧颈总动脉和颈外静脉,制成静脉窦高压模型。假手术组大鼠单纯解剖相应的颈部血管和硬脑膜窦,但不行吻合或闭塞。术后90d,随机取7只静脉窦高压组大鼠和5只假手术组大鼠,行硬脑膜血管明胶墨汁灌注,观察硬膜血管的状况。结果术后90d静脉窦高压组和假手术组大鼠的硬膜血管数分别为(10.7±1.5)条／mm,(10.3±0.6)条／mm,差异无显著性。在静脉窦高压组中1只大鼠有硬脑膜动静脉瘘,其形态和结构与生理性动静脉短路类似。结论大鼠静脉窦高压一段时间后,颅内硬膜血管无明显增生。颅内硬脑膜动静脉瘘的形成很可能是由动静脉间的短路发展而来。     

Therapeutic Consequences of Variation in Intraarterial Pressure Measurements After Iliac Angioplasty

Purpose: To assess the accuracy of intraarterial measurement of transstenotic pressure gradients for the detection of hemodynamically suboptimal iliac angioplasty. Methods: In 14 patients, referred for diagnostic angiography, mean pressure gradients in the aorta and iliac artery were obtained twice, using a double-sensor pressure catheter. Additional iliac measurements were performed during pharmacologically induced flow augmentation. Repeatability was assessed by calculation of the mean difference plus standard deviation (MD ± SD) and repeatability coefficient (2 × SD). These results were extrapolated to 137 iliac angioplasty procedures with secondary stenting where there was a residual pressure gradient > 10 mmHg. Results: MD ± SD for repeated measurements at rest and during flow augmentation were 0 ± 2 mmHg and 1 ± 3 mmHg, respectively. Repeatability coefficients were 3 and 6 mmHg. Mean pressure gradients after hemodynamically insufficient angioplasty were 8 ± 7 mmHg at rest and 17 ± 5 mmHg following vasodilatation. Inaccurate pressure recordings may have led to inappropriate stent placement in less than 2.5%, and inappropriate denial of stent placement in less than 5% of the lesions. Conclusion: Variability of intraarterial pressure measurements has little consequence in the detection of hemodynamically significant stenosis after angioplasty. Received: 0/00/00/Accepted: 0/00/00     

Effect of a patellar realignment brace on patellofemoral relationships: Evaluation with kinematic MR imaging

The effect of a newly developed patellar realignment brace was evaluated in 21 patellofemoral joints (19 patients) with patellar subluxation (13 joints with lateral subluxation and eight with medial subluxation) by using active-movement, loaded kinematic magnetic resonance (MR) imaging. Sixteen patellofemoral joints (76%) demonstrated a qualitative correction of or improvement in patellar subluxation (ie, centralization of the patella or a decrease in the displacement of the patella) after application of the brace. Four of the five “failures” occurred in patellofemoral joints that had patella alta and/or dysplastic bone anatomy. These results indicate that the patellar realignment brace was able to counteract patellar subluxation in the majority of patellofemoral joints studied, as shown by active-movement, loaded kinematic MR imaging. This brace appears to be useful for conservative treatment of patients with patellofemoral joint pain secondary to patellar malalignment and maltracking.