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脂质沉积性肌病42例临床治疗和预后随访 总被引:3,自引:1,他引:2
目的探讨脂质沉积性肌病(LSM)的临床特点、药物治疗效果和预后情况。方法回顾性分析42例LSM患者临床资料并对其治疗效果和预后情况进行随访。结果全部患者均有治疗期间的短期随访资料,其中小剂量泼尼松治疗33例,单用核黄素(维生素B2)治疗9例,全部患者1个月内肌无力症状均获明显改善。随访1年以上者共32例,其中26例肌力完全恢复正常,另6例仍不能耐受重体力劳动,运动耐力较健康人差。13例患者症状有不同程度复发,其中7例有家族史,复发的13例中有12例是在冬季复发,均以劳累、感冒为诱因,5例患者复发2次以上。结论LSM是可治疗的肌肉疾病,对小剂量泼尼松治疗有效,部分患者有复发倾向,有家族史患者更容易复发。单用核黄素治疗反应良好的LSM可能为戊二酸尿症Ⅱ型所致,提示应根据LSM的不同病因采取不同的治疗方案。 相似文献
3.
R. Christen R. Morant J. Schneider R. Jenni J. Fehr 《Journal of molecular medicine (Berlin, Germany)》1989,67(6):358-365
Summary A female patient is described in whom the diagnosis of idiopathic hypereosinophilic syndrome (HES) with heart disease and peripheral neuropathy was made at the age of 32 years. Although prednisone induced a prompt and longstanding complete hematological remission, progressive and eventually intractable heart failure developed, and the patient died 6 years later. Endomyocardial biopsy at diagnosis showed infiltration with intact and disintegrated eosinophils and Charcot-Leyden crystals. Echocardiographic follow-up (including Doppler-Echocardiography) revealed mitral regurgitation with thickening and impaired motility of the posterior mitral leaflet, as well as progressive dilated cardiomyopathy. At autopsy, a diffuse interstitial fibrosis with patchy prominence in an eccentric hypertrophic and highly dilated heart was found. There were no significant endocardial thickening and no mural thrombi. In contrast to the findings of the initial endomyocardial biopsy, autopsy revealed no eosinophilic infiltrate.In this case, eosinophil-induced heart disease manifested as dilated cardiomyopathy, without endocardial fibrosis as originally described by Löffler. We speculate, that eosinophils have been deposited predominantly in the myocard at an early stage of disease, and — activated locally — secreted their granule proteins producing an initial damage to capillary endothelial cells and myocytes. After prednisone-induced clearance of eosinophils from blood and tissues, progressive, self-perpetuating interstitial fibrosis of the myocard and loss of myocytes eventually resulted in end-stage dilated cardiomyopathy.Abkürzungsverzeichnis HES
idiopathic hypereosinophilic syndrome
- UBBC
unsaturated vitamin B12 binding capacity
- ECP
eosinophil cationic protein
- MBC
major basic protein
- EDD
end-diastolic diameter of the left ventricle
- LA
left atrium diameter
- LVEDP
left ventricular end-diastolic pressure
- NIH
National Institutes of Health 相似文献
4.
为了解强的松对心肺肝脾肾影响的病理改变,应用正常Wistar大白鼠28只,分实验组及对照组各14只。实验组给予相当于人2mg/kg.d等效剂量的强的松片剂溶入当日饮水中,对照组饮自来水,共10周,之后解剖光镜见对照组心肺肝脾肾及实验组心肺均无异常。实验组肝肾轻度充血,脾充血明显,且见较多舍铁血黄素沉着,以红髓边缘明显。证实常规剂量的强的松对肝脾肾也有一定不良影响,应用激素时要注意及时调整剂量和给药方式与时间,尽量减少副作用。 相似文献
5.
雷公藤与糖皮质激素治疗重症甲亢疗效比较 总被引:3,自引:0,他引:3
目的:为探讨用雷公藤多甙(TⅡ)和泼尼松治疗重症甲状腺功能亢进(简称重症甲亢)的效果。 方法和结果:共选47例重症甲亢,分别用雷公藤多甙和泼尼松进行治疗,疗程12周。根据主要临床表现改善程度累计判断疗效,TⅡ治疗组显效率为59.8% ,明显高于泼尼松组(P< 0.01),总有效率为91.1% ,亦高于泼尼松组(P< 0.05),而无效率仅8.9% ,低于泼尼松组(P< 0.05),TⅡ剂量为每天30 m g 时不良反应少于泼尼松。 结论:小剂量TⅡ用于重症甲亢的免疫治疗具有疗效高、不良反应小的优点,有较好的应用前景。 相似文献
6.
Prednisone can protect against exercise-induced muscle damage 总被引:7,自引:0,他引:7
S. C. J. M. Jacobs A. L. Bootsma P. W. A. Willems P. R. Bär J. H. J. Wokke 《Journal of neurology》1996,243(5):410-416
In an experimental animal exercise model we tested whether daily administration of prednisone prevents the development of mechanically induced muscle fibre damage. Six-week-old rats were treated with different doses of prednisone ranging from 1 to 50 mg/kg body weight per day or with placebo, for 8 days. On day 6 of treatment the rats were forced to run for 2 h on a level treadmill. Two days after exercise morphological damage in the soleus muscles was quantified using light microscopy and a semi-automatic image analysis system. Creatine kinase (CK) activity was measured before exercise (day 5) and directly after exercise (day 6). The expression of dystrophin in a placebo group and in a group that received 5 mg prednisone/kg body weight per day with and without performing exercise was studied with Western blotting. The effect of prednisone on fibre type distribution was determined with an antibody against fast myosin and the effect of prednisone on the proliferative activity of muscle satellite cells was studied using bromodeoxyuridine (BrdU) immunohistochemistry. Exercise-induced muscle fibre damage varied in a dose-dependent way. In the placebo group the mean (SEM) damaged muscle fibre area was 4% (1%). The groups that received low doses of prednisone, 1 or 2.5 mg/kg per day, showed a similar level of muscle damage. However, with 5 mg prednisone/kg per day the amount of muscle fibre damage [mean (SEM)] was significantly reduced to 1.4% (0.5%) (P 0.05, Student'st-test). High doses of prednisone had no protective effect. Directly after exercise the CK activity was increased two-fold, except in the group that received 50 mg prednisone/kg body weight per day. No changes in the amount of dystrophin were found after densitometric analysis of the Western blots. Prednisone did not affect the fibre distribution or the labelling index of satellite cells. We conclude that prednisone, given in an appropriate dose, protects muscle fibres against the development of mechanically induced damage, possibly by stabilizing the muscle fibre membranes. This action may explain the beneficial effect of prednisone observed in Duchenne muscular dystrophy patients. 相似文献
7.
Penina Tarshish Jay Bernstein Jonathan N. Tobin Chester M. Edelmann Jr 《Pediatric nephrology (Berlin, Germany)》1992,6(2):123-130
It has been claimed that long-term prednisone treatment ameliorates the course of children with mesangiocapillary glomerulonephritis (MCGN). The International Study of Kidney Disease in Children conducted a randomized, double-blinded, placebo-controlled clinical trial in 80 children with idiopathic MCGN, including 42 patients with type I disease, 14 with type II disease, 17 with type III disease, and 7 with nontypable disease. Criteria for admission included heavy proteinuria and a glomerular filtration rate of greater than or equal to 70 ml/min per 1.73 m2. Prednisone or lactose, 40 mg/m2, was given every other day as a single morning dose. The mean duration of treatment was 41 months, renal failure being the most common reason for termination of therapy. Treatment failure was defined as an increase from baseline of 30% or more in serum creatinine, or more than 35 mol/l. Overall, treatment failure occurred in 55% of patients treated with lactose, compared with 40% in the prednisone group. Life-table analysis showed a renal survival rate (i.e., stable renal function) at 130 months of 61% among patients receiving prednisone and 12% among patients receiving lactose (P=0.07). Of patients with type I or III MCGN, 33% treated with prednisone were treatment failures, compared with 58% in the lactose group. Long-term treatment with prednisone appears to improve the outcome of children with MCGN. 相似文献
8.
C. Scoppetta P. Tonali A. Evoli P. David F. Crucitti M. L. Vaccario 《Journal of neurology》1979,222(1):11-21
Summary In the treatment of myasthenia gravis (MG) considerable progress has recently been achieved. Our experience is based on the observation of 139 patients with an average follow-up of 3 years and 4 months.A treatment plan and results are presented.Indications for thymectomy: all cases of MG in adult life, apart from ocular myasthenia without radiological thymoma and without electrophysiological and pharmacological signs of generalization; before puberty only cases with radiological thymoma and severely incapacitating or life-threatening signs.Median sternotomy is preferable for thymoma, the transcervical approach with a sternal split for non-neoplastic thymus. Mediastinal radiotherapy is indicated after removal of an invasive or adhesive thymoma.Indications for corticosteroids: 1) before thymectomy: respiratory weakness; 2) soon after thymectomy: life-threatening signs; 3) later after thymectomy: incapacitating or life-threatening signs; 4) as an alternative to thymectomy: when surgery cannot be performed or it is not indicated. Oral Prednisone was nearly always preferred: alternate-day high single dose (75 to 115 mg) has given good results in most cases even if in some cases a small dose was required in the off day; inversely a lower alternate-day or daily dose was often sufficient.Long-term results: following this schedule for adult patients good results were scored in 67% of thymomas, in 94% of hyperplasias, and in 62% of unthymectomized patients: in prepuberal life the few cases of severe MG have all shown a favorable evolution.
Zusammenfassung Bei der Behandlung der Myasthenie wurden in letzter Zeit beachtliche Fortschritte erzielt. Wir teilen hier unsere Erfahrungen anhand von 139 Patienten mit einer durchschnittlichen Katamnese von 3 Jahren und 4 Monaten mit.Wir betrachten als Indikationen für eine Thymektomie: alle Fälle von Myasthenie beim Erwachsenen mit Ausnahme der rein okulären Formen ohne radiologisch nachweisbares Thymom und ohne elektrophysiologische oder pharmakologische Zeichen einer Generalisierung; bei Kindern vor der Pubertät empfehlen wir die Thymektomie nur in Fällen mit radiologisch nachweisbarem Thymom und mit schwerer Beeinträchtigung oder gar Lebensgefährdung durch die Symptome.Die mediane Sternotomie ist beim Thymom vorzuziehen, der transzervikale Zugang mit Spaltung des Sternums für die nicht neoplastischen Thymusvergrößerungen. Mediastinale Strahlentherapie ist nach Exstirpation eines invasiven Thymoms oder eines Thymoms mit Adhäsionen angezeigt.Als Indikation für die Corticosteroidtherapie betrachten wir: 1. wenn vor der Thymektomie Atemstörungen bestehen; 2. wenn bald nach der Thymektomie lebensbedrohliche Symptome auftreten; 3. wenn später nach Thymektomie nennenswert behindernde oder lebensbedrohliche Symptome in Erscheinung treten; 4. als Alternative zur Thymektomie, wenn diese nicht durchgeführt werden kann oder nicht indiziert ist. Die orale Prednisontherapie wurde fast immer vorgezogen: wir gaben an alternierenden Tagen jeweils hohe Einzeldosen (75–115 mg) mit gutem Erfolg in den meisten Fällen. In gewissen Fällen war eine kleine Dosis an den Tagen zwischen der Hauptdose aber genügend, ebenso in Einzelfällen eine allgemein niedrigere Dosierung. Die Langzeiterfolge mit diesem therapeutischen Vorgehen betrugen 67% gute Ergebnisse bei Thymomen und 94% bei Thymushyperplasie. Unter den nicht thymektomierten Patienten wiesen 62% ein gutes Ergebnis auf. Vor der Pubertät zeigten die allerdings wenigen Fälle schwerer Myasthenie alle ein gutes Ansprechen auf die Therapie.相似文献
9.
糖皮质激素诱导试验与儿童急性淋巴细胞性白血病预后的关系 总被引:6,自引:0,他引:6
目的 通过糖皮质激素早期诱导试验评估儿童急性淋巴细胞性白血病的预后。方法 诱导试验是根据初发病时外周血幼稚细胞计数及服用糖皮质激素 (GC) 7d后外周血中幼稚细胞的动态变化。此后 ,所有病例都接受同样强烈的化疗方案。结果 6 0例患儿中对泼尼松诱导试验敏感 (PGR)者为 4 9例 ,占 81 7% ,其中高危患儿 6例 ,中危患儿 8例 ,低危患儿 35例。在 4 9例PGR患儿中 38例 (77 6 % )处于持续缓解状态 (CCR) ,中位缓解期为 2 2 5个月 ;复发 3例 ,占 6 1%。 6 0例患儿中对泼尼松诱导试验不敏感 (PPR)者为 11例 ,占 18 3%。在PPR 11例中 ,5例早期复发 ,占 4 5 5 % ;2例达CCR ,占 18 2 %。从治疗第 19天、第 30天的骨髓检查 ,PPR组 19dM1、M2 、M3分别为 72 7%、18 1%、9 2 % ;PGR组 19dM1、M2 分别为 96 %、4 % ,无一例为M3 。达CR时间 ,PPR组明显迟于PGR组。结论 GC在用于治疗恶性淋巴细胞增生性疾病中 ,起到了重要作用。 相似文献
10.
以泼尼松为主联合治疗多发性肌炎和皮肌炎32例 总被引:3,自引:0,他引:3
目的:比较泼尼松联合疗法和单用治疗多发性肌炎 (PM) 和皮肌炎 (DM)的疗效。方法:PM和DM患者67例分为对照组(单用糖皮质激素)35例,给予泼尼松1 mg·kg 1·d 1,qd。治疗组 32例,给予泼尼松1 mg·kg 1·d 1, qd,加甲氨蝶呤片 7.5~10.0 mg,po,每周1次;加火把花根片3片,tid。疗程4周。主要观察指标有肌力变化,皮疹,血清 ALT、AST、LDH、CK的水平及药物副作用发生率。结果∶治疗后两组患者的肌力均有不同程度改善,肌力改善幅度≥2级患者在对照组中占54.3%,在治疗组中占62.5%,二者差异无显著性(P>0.05)。两组患者在治疗后血清 ALT、AST、LDH、CK水平均比治疗前有明显下降,差异有极显著性(P<0.01)。治疗组患者的血清ALT、AST、LDH、CK下降的幅度明显大于对照组,差异有显著性(P<0.05)。结论:与单用糖皮质激素的疗法相比,联合治疗方法能够使血清肌酶更快地下降,对PM和DM有更好的近期疗效。 相似文献