甲状旁腺功能减退性心肌病的相关进展

正甲状旁腺功能减退(简称"甲旁减")性心肌病是一种非常罕见的心脏疾病,是扩张型心脏病中为数不多的、可逆的一种,可引起射血分数降低型心力衰竭~([1-2])。甲旁减性心肌病由甲旁减导致的低钙血症引起,其治疗的关键在于低血钙的纠正。低钙血症初期如果是慢性和渐进的,则往往不会出现相关症状~([3]),严重的低钙血症患者常以急性心力衰竭入院,易被误诊为不明原因或难治性心力衰竭,此时,其心力衰竭的甲旁减原因常被忽视~([4]),导致错误的单一抗心力衰竭治疗,加重病情。     

扩张型心肌病以肺部表现首发误诊10例分析

扩张型心肌病（DCM）是一种以心脏扩大、心肌收缩功能障碍为主要特征的原因不明的心肌疾病,其临床表现以进行性心力衰竭、心律失常、血栓栓塞或猝死为基本特征。因其临床表现或辅助检查无特异性,容易误诊。现将我院被误诊为肺心病的10例患者总结如下。     

甲状旁腺功能减退症误诊6例分析

目的：探讨甲状旁腺功能减退症（甲旁减）长期误诊原因,提高对甲旁减的认识,减少漏诊、误诊。方法：分析6例甲旁减临床资料。结果：误诊为原发性癫痫最多,占一半以上,抑郁症及末梢神经炎各1例,经诊断后均给予钙剂和活性维生素D口服,临床症状消失。结论：血钙、磷的检测应作为癫痫、癔症样发作、末梢神经炎患者的常规检查,尤其是成年人癫痫发作患者除需行头颅CT检查外,一定要注意血钙测定,以免漏诊及误诊。     

甲亢性心脏病189例临床分析

对189例甲亢性心脏病患者临床表现进行综合分析。结果本组病例心脏扩大、心律失常、心力衰竭发生率分别为53. 4%、88. 4%和24. 9%,误诊率为11. 1%。甲亢性心脏病常易误诊,对不明原因的心脏扩大、心律失常、心力衰竭患者应注意甲状腺功能检查。     

小剂量硝普钠持续静点治疗扩张性心肌病难治性心力衰竭疗效观察

目的观察持续静点硝普钠对扩张性心肌病难治性心力衰竭的疗效。方法选择扩张性心肌病充血性心力衰竭患者48例,在基础治疗（包括休息,限制钠盐摄入,给于利尿剂ACEI类药物及硝酸甘油静点和洋地黄等）3～5d无效的情况下改硝酸甘油为硝普钠持续静点,起始以0.1μg/（kg.min）泵入据血压下降或升高程度调整给药速度,观察心率,测定B型钠尿肽（BNP）水平及以超声心动图检查评估心脏收缩功能。结果 44例患者在治疗后心率均减慢且心衰症状均有明显改善,BNP水平明显降低,射血分数有所升高。结论硝普钠能够有效改善扩张性心肌病难治性心力衰竭患者血流动力学异常。     

1例甲状旁腺功能减退致低血钙性心肌病的护理

低血钙性心肌病（hypocalcemic cardiomyopathy,HCM）是由于各种原因引起的长期低钙血症导致以心脏扩大和充血性心力衰竭为主要临床特点的一种可逆性心肌疾病。原因常见于甲状旁腺功能减退,其对心脏的影响主要是由于低钙血症引起的,长期低血钙可引起心肌病变导致心脏扩大、充血性心力衰竭、心脏传导阻滞,Q—T间期延长、室性心律失常,严重时威胁病人生命。2008年10月我科收治1例甲状旁腺功能减退致低血钙性心肌病病人,经过及时有效治疗及护理,病人病情好转出院。现将护理报告如下。     

原发性扩张性心肌病合并心力衰竭的临床特点及护理体会

原发性扩张性心肌病的主要特征是单侧或双侧心脏扩大，心肌收缩功能减退，伴或不伴有充血性心力衰竭，在我国发病率为13／10万。病因迄今不明，起病缓慢，多数患者在充血性心力衰竭临床症状明显时方就诊。本文对1999—2002年收治的38例原发性扩张性心肌病合并心力衰竭的患者临床综合扩张的情况进行分析，现报告如下。     

甲亢性心脏病临床分析

目的 分析甲亢性心脏病临床特征,探讨容易发生的误诊因素.方法 对43例甲亢性心脏病患者临床资料进行综合分析.结果 甲亢性心脏病误诊率为18.6%,多发生于无高代谢综合征的老年患者.心律失常37例(86.1%;心脏扩大者15例(34.9%);心袁13例(30.2%).房颤占甲亢性心脏病人的65.1%.结论 甲亢性心脏病误诊率高.对原因不明的心律失常,心脏扩大或心力衰竭的患者,应注意甲状腺功能检查.     

定量组织速度成像对围产期心肌病左室功能的研究

围产期心肌病（PPCM）亦称产后心肌病，是发生在妊娠晚期或产后5个月内的一种不明原因的心肌疾病，临床发病较急，病程短，以左心扩大充血性心力衰竭为主要表现，死亡率18％～56％。作者应用定量组织速度成像（QTVI）技术对PPCM患者左心功能研究，结果报告如下。     

联用硝普钠、多巴胺和参麦注射液治疗扩张型心肌病30例

扩张型心肌病（DCM）系以不明原因心脏增大、心室扩张、收缩功能障碍及充血性心力衰竭（心衰）为特征的疾病，目前对终末期DCM尚无根治手段。我院采用中西医结合治疗DCM患者60例，取得了较好的效果，报告如下。     

Right Ventricular endomyocardial biopsy: clinicopathologic correlates in 100 consecutive patients

The first 100 consecutive patients to undergo right ventricular endomyocardial biopsy at the Mayo Clinic were divided retrospectively into five groups, depending on the prebiopsy clinical diagnosis, and the biopsies were review histologically in a single-blind format. Group 1 consisted of 34 patients with a diagnosis of unexplained congestive heart failure and a dilated heart; of these, 4 (12%) had active myocarditis by biopsy. Of the six patients in group 2 with a clinical diagnosis of myocarditis, only one (17%) had biopsy evidence of inflammation, but two (33%) had changes that, in the clinical setting, were suggestive of cardiomyopathy. Group 3 consisted of 27 patients with dysrhythmia, syncope, or cardiac arrest but without congestive heart failure; of these, 4 (15%) had active myocarditis by biopsy, and 8 (30%) had changes that, with the clinical history, were consistent with cardiomyopathy. Group 4 included 19 patients with unexplained congestive heart failure and a nondilated heart; 4 (21%) had cardiac amyloid on biopsy. Group 5 was a diverse group of 14 patients with possible cardiac involvement by a known systemic disease; myocardial disease was documented by biopsy in 7 (50%). On the basis of these findings, we recommend endomyocardial biopsy (1) in patients with dilated cardiomyopathy in whom myocarditis is suspected, (2) in patients with the clinical diagnosis of active myocarditis in whom tissue documentation is indicated before treatment with anti-inflammatory or immunosuppressive drugs, (3) in patients with clinically unexplained life-threatening dysrhythmias in whom myocarditis or cardiomyopathy may be present, and (4) in patients with apparent hypertrophic or restrictive cardiomyopathy in whom cardiac amyloid may be present.     

Hereditary juvenile haemochromatosis: a genetically heterogeneous life-threatening iron-storage disease

Juvenile haemochromatosis is a rare inborn error of iron metabolism with clinical manifestations before 30 years of age. Unlike adult haemochromatosis which principally affects men, juvenile haemochromatosis affects the sexes equally; it causes early endocrine failure, dilated cardiomyopathy and joint disease. We report four patients (two of each sex) from three pedigrees affected by juvenile haemochromatosis with a mean onset at 22 years (range 14-30). All had endocrine deficiency with postpubertal gonadal failure secondary to pituitary disease; two suffered near-fatal cardiomyopathy with heart failure. Mean time to diagnosis from the first clinical signs of disease was 9.8 years (range 0.5-20) but general health and parameters of iron storage responded favourably to iron-depletion therapy. A 24-year-old man listed for heart transplantation because of cardiomyopathy [left ventricular (LV) ejection fraction 16%] responded to intravenous iron chelation with desferrioxamine combined with phlebotomy (ejection fraction 31%). A 27-year-old woman with subacute biventricular heart failure refractory to medication required orthotopic cardiac transplantation before the diagnosis was established (LV ejection fraction 25%). Genetic studies showed that these two patients with cardiomyopathy from unrelated families were heterozygous for the HFE 845G-->A (C282Y) mutation and wild-type at the H63D locus: complete sequencing of the intron-exon boundaries and entire coding sequence of the HFE gene failed to identify additional lesions. Two siblings in a pedigree without cardiomyopathy were wild-type at the HFE C282Y locus; although the brother harboured a single copy of the 187C-->G (H63D) allele, segregation analysis showed that in neither sibling was the iron-storage disease linked to MHC Class I markers on chromosome 6p. Juvenile haemochromatosis is thus a genetically heterogenous disorder distinct from the common adult variant.     

甲状旁腺功能减退性心肌病1例报告并文献复习

目的:探讨甲状旁腺功能减退性心肌病临床特征,并提高其诊断及治疗水平。方法:对1例甲状旁腺功能减退性心肌病患者的综合治疗进行分析,并文献复习。结果:1例甲状旁腺功能减退性心肌病诊断明确后通过补充钙剂治疗使本病的心功能得以部分恢复。结论:甲状旁腺功能减退性心肌病为少见疾病,易漏诊,结合临床表现、实验室检查可以减少误诊率。     

Thymoma-associated hypocalcemic crisis

A previously healthy male was diagnosed with a malignant thymoma. During the workup, he had syncope, which was due to severe unrecognized hypocalcemia. Additional workup was suggestive of parathyroid failure. In particular, there was no evidence of autoimmune parathyroid failure due to antibodies against the calcium-sensing receptor. Literature review reveals one additional thymoma case with these clinical features of chronic hypoparathyroidism of unknown cause.     

Cinacalcet-associated cardiogenic shock in a patient with cardiomyopathy

INTRODUCTION: Cinacalcet is a calcimimtic agent used to treat secondary hyperparathyroidism in patients with end-stage renal disease on dialysis or hypercalcemia related to parathyroid carcinoma. This report describes recurring circulatory collapse in a patient treated with cinacalcet for unrelated refractory primary hyperparathyroidism. CASE SUMMARY: A white man, aged 54 years and weighing 68 kg, was admitted to the hospital with lethargy, dyspnea, and twitching in the extremities. He was diagnosed previously with primary hyperparathyroidism and nonischemic dilated cardiomyopathy. Surgical parathyroidectomy had been unsuccessful. His serum calcium concentration was 3.15 micromol/L and was refractory to bisphosphonate therapy. Therapy with cinacalcet 30 mg/d was reinitiated, resulting in a reduction in serum calcium concentration and greatly increased heart failure requiring inotropic drug therapy and hemofiltration. After 13 days of treatment, cinacalcet was withdrawn, and the patient's condition unproved. On reintroduction of cinacalcet 30 mg/d, the patient decompensated and required emergency circulatory support. Decompensation resolved 5 days after discontinuation of cinacalcet. DISCUSSION: Based on a score of 7 on the Naranjo adverse drug reaction probability scale, cinacalcet was the probable cause of cardiogenic shock in this patient. Calcium-channel antagonist poisoning is associated with hypotension and bradycardia, whereas the calcium sensitizer levosimendan has been reported to improve low-output heart failure, suggesting that calcium may have inotropic properties. CONCLUSIONS: It appears that cardiac function in this patient had adapted to hypercalcemia and became destabilized after introduction of cinacalcet. Caution should be exercised when considering treatment with cinacalcet in patients with heart failure.     

透析患者甲状旁腺切除术后低钙血症的发生及处理

目的 分析血液透析患者因继发性甲状旁腺机能亢进症行甲状旁腺切除术(parathyroidectomy,PTX)后低钙血症的发生及相关影响因素,总结处理经验.方法 回顾近10年行PTX的117例透析患者,观察术后血清钙的变化,分析术后1月内最低的血钙水平与术前血清全段甲状旁腺激素(intact parathyroid hormone,iPTH)、碱性磷酸酶(alkaline phosphatase,ALP)、钙及切除的甲状旁腺数量和病理类型等因素的关系,收集低钙血症的临床表现,统计补充元素钙和骨化三醇的用量.结果 在117例患者中,低钙血症的发生率为61.5%(72/117),一般发生在术后1天,少数在术后1～4周.主要表现为烦躁、多汗、四肢末梢发麻;少数表现为心悸、低血压、肌肉痉挛、肺水肿甚或腹泻.术后1天的血钙为(1.92±0.38)mmol/L.术后血钙与术前血iPTH负相关(r=-0.416,P<0.01),与术前血钙负相关(r=-0.756,P<0.01),与术前血ALP及甲状旁腺切除的数量和病理类型(甲状旁腺腺瘤)无关.大部分患者在术后1周口服元素钙(2.55±1.68)g/d和骨化三醇(1.42±0.75)μg/d即能维持血钙在(2.08±0.53)mmol/L.结论 成功的PTX后都有血钙下降,术前更高的血iPTH和钙是导致术后低钙血症发生的危险因素.低钙血症的临床表现各异,足量补充钙制剂和骨化三醇,可以避免和迅速缓解其发生.     

静息心率对慢性心力衰竭合并高血压患者心功能及预后的影响

目的 探讨静息心率(resting heart rate, RHR)与慢性心力衰竭合并高血压患者心功能及预后的关系。方法 2016年9月至2017年8月于保定市第一中心医院心内科因慢性心力衰竭住院的高血压患者250例,按入院RHR水平分为3组:RHR1组,RHR＜70次/min;RHR2组,70次/min≤RHR＜90次/min;RHR3组,RHR≥90次/ min。测量患者超声心动图参数,包括左心室舒张末期内径(left ventricular end diastolic diameter, LVEDD)和左心室射血分数 (left ventricular ejection fraction, LVEF),按照纽约心脏病协会(NYHA)分级情况对患者入院、出院及随访时的心功能进行评估并记录,对比各组之间各项指标及疗效的差异,并分析其相关性。结果 随着入院RHR的升高,LVEF呈现逐渐降低的趋势（P＜0.05）, 而LVEDD、B型脑钠肽(BNP)均逐渐升高,以RHR3 组最高（P＜0.05）;RHR1组、RHR2组出院疗效及随访疗效均优于RHR3组（P＜0.05）;RHR3组入院RHR水平与出院疗效呈负相关（r=-0.251, P均＜0.05）;RHR2组、RHR3组（入院-出院）RHR差值与出院疗效、随访疗效均呈正相关（其与出院疗效相关性r值分别为0.197、0.279, 其与随访疗效相关性的r值分别为0.214、0.321, P均＜0.05）。结论 对于慢性心力衰竭合并高血压的患者,静息心率水平越高,左心功能越差,出院疗效及随访疗效也越差,积极合理的控制RHR有助于改善预后。     

Successful Radiofrequency Catheter Ablation of Automatic Atrial Tachycardia with Regression of the Cardiomyopathy Picture

Ectopic atrial tachycardia (EAT) is often refractory to pharmacological suppression, and if uncontrolled, it can lead to cardiomyopathy. Although RF current catheter ablation therapy has been effective in eliminating the arrhythmia, there is limited information. particularly in adult patients with regard to the reversal of the tachycardia induced cardiomyopathy. Four adult patients, 20–56 years of age, and a 6-year-old boy, were referred with refractory EAT. Four patients had heart failure and three had depressed LV function by echocardiographic criteria. AH patients underwent electrophysiological study, and RF ablation was successful in abolishing the arrhythmogenic foci. Of these, four were located in the right atrium and one in the left atrium, and were identified by recording of the earliest atrial activation. No complications occurred. Termination of the EAT resulted in symptomatic improvement. Serial echocardiographic assessment of LV function indicated a significant reversal of the cardiomyopathy picture with reduction in chamber size and recovery in systolic function; indices of diastolic dysfunction persisted in one patient. Chronic, uncontrolled EAT can cause tachycardia induced cardiomyopathy. The picture of the cardiomyopathy resolves after elimination of the focus. RF ablation is both effective and safe, and may be considered as early therapy, particularly in patients with incessant EAT and ventricular dysfunction.     

维持性血液透析患者钙磷代谢及全段甲状旁腺激素水平调查分析

目的通过调查维持性血液透析患者的钙磷代谢情况及全段甲状旁腺激素(iPTH)水平,了解其达标情况和治疗状况。方法回顾性分析90例终末期肾病患者矫正的血钙、血磷、钙磷乘积及iPTH的水平及临床资料。结果 90例患者矫正的血钙、血磷、钙磷乘积和iPTH的达标率分别为35.56%(32例)、33.33%(30例)、61.11%(55例)、27.78%(25例)。90例患者四项指标中,1项达标占31.11%(28例)、2项同时达标占30.00%(27例)、3项同时达标占17.78%(16例)、4项同时达标占3.33%(3例)。29例低钙血症患者中8例(27.59%)患者在服用钙剂,56例高磷血症患者中有20例(35.71%)患者在服用磷结合剂碳酸钙,55例iPTH>300 pg/ml的患者中有11例(20%)患者在服用骨化三醇。透析时间>3年的40例患者iPTH水平较透析时间≤3年的50例患者明显增高(t=3.99,P<0.05)。透析充分组的10例患者与透析不充分组的11例患者血钙、血磷、钙磷乘积、iPTH水平比较,差异均无统计学意义(t分别=0.09、0.29、0.12、0.16,P均>0.05)。结论本次研究多数维持性血液透析患者钙磷代谢及iPTH的控制仍未达到K/DOQI指南推存的应保持的目标范围,以血磷及iPTH增高更为明显。有相当一部分患者没有得到及时有效的治疗。