Telocytes在大鼠心脏不同部位的分布*********◆

背景：心脏Telocytes可能是心肌细胞及心脏内源性干细胞的联系及支持细胞,在心肌损伤后的修复与再生和心室重构中起重要作用,但目前对Telocytes在心脏中的分布与相应的功能所知甚少。 目的：观察Telocytes在大鼠心脏不同部位的分布情况。 方法：取成年SD雌性大鼠心脏,行横断面连续冰冻切片,分别取心脏心底部、心脏中间部、心房-心耳部分的代表性切面进行抗c-kit免疫荧光染色,观察Telocytes在心脏的分布情况。 结果与结论：结果发现心底部(26个/mm2)和心房-心耳部(20个/mm2)的Telocytes密度明显多于中间部(9个/mm2)。上述三个代表性横断面,其心肌外层区域Telocytes密度(心底部：18个/mm2;中间部：5个/mm2;心房-心耳部：13个/mm2)显著多于心肌内层区域(心底部：7个/mm2;中间部：4个/mm2;心房-心耳部：7个/mm2)。结果提示心脏Telocytes在心脏不同部分的分布不同,可能与心肌细胞及其内源性干细胞在上述区域分布不同有关。     

Cardiac involvement in Becker's muscular dystrophy, necessitating heart transplantation, 6 years before apparent skeletal muscle involvement

In Becker's muscular dystrophy cardiac abnormalities usually occur after onset of neuromuscular symptoms. We describe a Becker muscular dystrophy patient in whom chronic heart failure, necessitating cardiac transplantation, was the initial manifestation. Neuromuscular symptoms occurred not earlier than 6 years after the initial cardiac symptoms and 5 years after heart transplantation. In conclusion, severe heart failure due to dilated cardiomyopathy may be the initial manifestation of Becker's muscular dystrophy and may predate neuromuscular symptoms for years.     

Cardiac involvement in myasthenia gravis

Non-specific ECG changes and histological changes in the myocardium in patients with myasthenia gravis (MG) have been described. In this study, the left ventricular function using systolic time intervals (STI) was assessed in patients with MG. 4 patients out of the 10 studied showed abnormalities of the STI which reverted towards normal after injection of neostigmine. This finding suggests that myocardial involvement in MG may be more common than clinically suspected.     

Involving parents in structured therapy groups for adolescents

Initial and on-going techniques for feedback to parents about progress in adolescent groups are described including: therapy contracts, monthly group generated, feedback letter to parents, and a “Suggestions to Parents From the Group Booklet” developed and “published” by group members. Structured techniques for actual parental participation in “Parent Hot Seat” exercises are described which increase opportunities for development of curative factors and allow practice in negotiation, increased positive shared perceptions of child and parent, and an increased acceptance of parental limit setting. who was formerly the Chief Psychologist for the Wake County Juvenile Court.     

Nemaline myopathy: two autopsy reports

Nemaline myopathy belongs to the group of congenital non-progressive myopathies; however, in rare cases death occurs in early infancy. We report two cases of rapidly fatal nemaline myopathy. The first patient, who died at the age of 26 months, showed atrophy of type 1 fibers containing numerous rods in biopsy sections. Biopsy of the second patient, who had died at the age of 5 months, revealed severe maturational arrest and myopathy, but rods were so rare that diagnosis could only be made at the ultrastructural level. Autopsy of both patients showed that atrophy of type 1 fibers and maturational arrest had disappeared in the very same muscles; rods had moved to a central position in the first and significantly increased in number in the second case. Diaphragma muscles contained abundant amounts of rods in both cases. The cardiac musculature showed a few rods only in the first patient, who had developed heart insufficiency 11 months prior to death. Immunohistochemical analysis showed that rods did not contain desmin or ubiquitin.     

Attenuated 24-h heart rate variability in apparently healthy subjects,subsequently suffering sudden cardiac death

Attenuated cardiac parasympathetic activity appear to be an important risk factor contributing to sudden cardiac death in subjects with overt coronary disease but its predictive value in otherwise healthy normal subjects is not known. We have for 8 years followed 260 apparently healthy adult subjects who underwent Hotler monitoring. Twelve died, 14 developed ischaemic heart disease and four suffered sudden cardiac death. A healthy control subject was matched, along with other risk factors, for each case. In each subject 24-h heart rate variability was calculated as the deviation of all normal R—R intervals from mean R—R (SD) and the percentage of successive R—R interval differences exceeding 6% (%DIF6%)—this was used as an index of cardiac parasympathetic activity. There were no significant differences in heart rate variability between the cases developing problems and controls. In the sudden cardiac death victims, however, there was a clear trend towards lower heart rate variability. In them waketime mean SD was 73 ms versus 85 ms for cases and controls respectively (p = 0.08), and for sleeptime 61 ms versus 76 ms (p = 0.07). Compared to normal limits for heart rate variability obtained in 140 subjects that remained healthy for 8 years, figures for both SD and %DIF6% in sudden cardiac death subjects were at or below 95% confidence limits. The results indicate that altered autonomic balance may contribute to sudden cardiac death even in apparently healthy subjects. Subjects with a low 24-h heart rate variability on Holter monitoring may be predicted at an early stage of being at greater risk. This has considerable implications not only for predicting subjects at risk but for assessing physiological (such as exercise) and pharmacological interventions which may reduce such risk.     

Non-invasive study of airways inflammation in sleep apnea patients

The current view foresees that airway inflammation and oxidative stress are both important in the pathophysiology of obstructive sleep apnea syndrome (OSAS). Notwithstanding the fact that these events play a key role in OSAS, their monitoring is not included in the current management of this disease.The direct sampling of airways is made possible today thanks to what can be defined as quite invasive techniques, such as bronchoscopy with broncho-lavage and biopsy. Recently there has been increasing interest in the non-invasive methods that allow the study of airways via the induced sputum (IS), the exhaled breath volatile mediators and the exhaled breath condensate (EBC). The non-invasiveness of these techniques makes them suitable for the evaluation and serial monitoring of OSAS patients. The aim of this review is to spread current knowledge on the non-invasive airway markers and on their potential clinical applications in OSAS.     

Patient polyhandicapé et épisode d’insuffisance respiratoire aigu : prise en charge en milieu hospitalier

Acute respiratory failure in severe disabled people is a frequent and serious complication with a high morbidity and mortality rate. Common management relies on antibiotic therapy combined with clearance of airway secretions. Several manual and instrumental airway clearance techniques can be used to improve patients and tend to avoid mechanical ventilation, which is a controversial ethical issue. Postural changes should be associated with these techniques to decrease muscular contraction and enhance alveolar recruitment. This article is an overview of the most useful airway clearance techniques in disabled patients.     

多发性肌炎合并心脏损害的临床特点

目的　探讨多发性肌炎合并心脏损害的临床特点、诊断、治疗与预后。方法　回顾性分析41例多发性肌炎合并心脏损害患者的临床资料。结果　合并心脏损害者占同期全部多发性肌炎患者的38 3%,其中无症状者25例(60. 1% );平均年龄较无心脏损害者高(P<0 .05);出现发热、合并间质性肺病的比率高(P<0. 05);心电图异常主要表现为窦性心动过速(31.7% )、窦性心律不齐(26. 8% )。超声心动图异常者中以心包积液居多。血清抗核抗体(ANA)阳性率及C 反应蛋白含量明显增高(均P<0 .05)。对心脏损害采用病因及对症治疗,治愈14例,症状改善23例,恶化2例,死亡2例。结论　心脏损害是多发性肌炎最常见的并发症,大多数患者无自觉症状,心电图是诊断心脏损害的主要方法。严重的心脏损害是导致患者死亡的重要因素,及时发现心脏损害并采取有效治疗对于改善患者的预后有重要意义。     

Low-symptomatic skeletal muscle disease in patients with a cardiac disease – Diagnostic approach in skeletal muscle laminopathies

Mild skeletal muscle symptoms might be accompanied with severe cardiac disease, sometimes indicating a serious inherited disorder. Very often it is a cardiologist who refers a patient with cardiomyopathy and/or cardiac arrhythmia and discrete muscle disease for neurological consultation, which helps to establish a proper diagnosis. Here we present three families in which a diagnosis of skeletal muscle laminopathy was made after careful examination of the members, who presented with cardiac arrhythmia and/or heart failure and a mild skeletal muscle disease, which together with positive family history allowed to direct the molecular diagnostics and then provide appropriate treatment and counseling.