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Kinetics of the circulating levels of bone alkaline phosphatase in a case of hungry bone disease following total parathyroidectomy. 甲状旁腺全切除术后一例饥饿骨病患者骨碱性磷酸酶循环水平的动力学。
Luigi Francesco Morrone, Marilina Tampoia, Nicola Pansini, Loreto Gesualdo

We present the case of a 55-year-old patient who underwent total parathyroidectomy for severe hyperparathyroidism unresponsive to medical therapy, 4 years after having started hemodialysis treatment. It was decided to perform total parathyroidectomy because at macroscopic evaluation the parathyroid glands appeared completely compromised. After surgery, the patient developed hungry bone disease, characterized by severe hypocalcemia and hypophosphatemia. After parathyroidectomy, serial measurements were made for the long-term monitoring of the calcemia, phosphatemia and the serum levels of intact parathormone and bone alkaline phosphatase, a marker of bone turnover that mainly expresses bone formation. There was initially a slight decrease in the circulating levels of bone alkaline phosphatase as the calcemia dropped dramatically, then a new increase that anticipated the subsequent calcemia increase and finally, 6 months later, a decrease to very low values. We believe that the calcemia and blood bone alkaline phosphatase could be useful for the laboratory monitoring of the hungry bone state, providing information which may be useful to avoid excessive calcium administration and the dangerous consequences such as soft-tissue calcification.

我们报告了一例55岁的患者,在开始血液透析治疗4年后,因严重甲状旁腺功能亢进对药物治疗无反应而接受了全甲状旁腺切除术。我们决定进行全甲状旁腺切除术,因为在宏观评估中,甲状旁腺完全受损。术后患者出现饥饿性骨病,表现为严重的低钙血症和低磷血症。甲状旁腺切除术后,长期监测钙血症、磷血症及血清完整甲状旁激素和骨碱性磷酸酶水平,骨碱性磷酸酶是骨转换的标志,主要表达骨形成。随着血钙急剧下降,骨碱性磷酸酶的循环水平最初略有下降,然后又出现新的上升,预示着随后的血钙增加,最后,6个月后,下降到非常低的值。我们认为血钙和血骨碱性磷酸酶可用于饥饿骨状态的实验室监测,为避免过量钙的使用和软组织钙化等危险后果提供有用的信息。
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引用次数: 0
Alterations of the sympathovagal balance evaluated by heart rate variability in a rare case of adult Still's disease. 罕见成人斯蒂尔氏病的心率变异性对交感迷走神经平衡的影响。
Luigi Baratta, Massimo Delfino, Alessandra Fiorentini, Monica Martuscelli, Luigi Tubani

We present a rare case of adult Still's disease with cardiac involvement (myocarditis and coronary arteritis). The autonomic nervous system function was evaluated by heart rate variability (HRV) analysis performed by 24-hour electrocardiographic recording during the acute phase of the disease and the remission (after 1 month and 1 year). The HRV parameters were studied in the time (standard deviation of all NN intervals-total power - SDNN, square root of the mean of the sum of the squares of the differences between adjacent NN - RMS-SD and HRV index) and frequency domains (low frequency, high frequency, and low frequency/high frequency). The results of the analysis of the HRV highlight that in the acute phase of the disease with cardiac involvement the autonomic nervous system is globally altered, with modifications of the sympathovagal balance, due to impairment of the parasympathetic component. This trend tends to persist in the short period (1 month), but seems to resolve completely within 1 year. An altered sympathovagal balance should be considered as a possible marker of vasculitis-related ischemia.

我们报告一例罕见的成人斯蒂尔氏病累及心脏(心肌炎和冠状动脉炎)。在疾病急性期和缓解期(1个月和1年后),通过24小时心电图记录心率变异性(HRV)分析自主神经系统功能。在时间(所有NN区间的标准差-总功率- SDNN,相邻NN - RMS-SD与HRV指数之差平方和的平均值的平方根)和频域(低频、高频、低频/高频)上研究HRV参数。HRV分析结果强调,在心脏受累的疾病急性期,由于副交感神经成分受损,自主神经系统整体改变,交感迷走神经平衡改变。这种趋势倾向于在短期内(1个月)持续,但似乎在1年内完全解决。交感迷走神经平衡的改变应被认为是血管炎相关缺血的可能标志。
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引用次数: 0
[The health world's uneasiness: crisis of medicine or crisis of philosophy?]. 卫生界的不安:医学危机还是哲学危机?
Gian Carlo Secchi
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引用次数: 0
[Improving compliance in internal medicine: the motivational discussion]. [提高内科依从性:激励性讨论]。
Fabio Lugoboni, Gianluca Quaglio, Paolo Mezzelani, Benedetta Pajusco, Rebecca Casari, Alessandro Lechi

Compliance to treatment is of crucial importance in medicine. High levels of noncompliance to treatment have been reported in the most relevant diseases such as hypertension, coronary artery disease, diabetes and asthma. The aim of this article was to fully evaluate the lack of adherence to treatments in internal medicine. The prevalence of noncompliance and methods to cope with it are presented. The theory of the stages of change is thoroughly examined. Motivational interviewing in low-compliant patients is illustrated dealing both with the theoretical principles and practical applications. Several communication blocks which interfere with the relationship between doctor and patient are finally presented through concrete examples.

对治疗的依从性在医学上至关重要。据报道,在高血压、冠状动脉疾病、糖尿病和哮喘等最相关的疾病中,不遵守治疗的情况很高。本文的目的是充分评估缺乏坚持治疗的内科。介绍了不合规的普遍性和应对方法。变化阶段的理论得到了彻底的检验。对低依从性患者进行动机性访谈的理论原理和实际应用进行了阐述。最后通过具体的实例,提出了影响医患关系的几种沟通障碍。
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引用次数: 0
[Arterial hypertension and cognitive deficit]. [动脉高血压和认知障碍]。
Cristina Paglieri, Daniela Bisbocci, Francesco Amenta, Franco Veglio

Cognitive impairment and dementia are more and more common in the elderly. The first begins, it advances silently and it leads to dementia in few years. Arterial hypertension represents the most important cerebrovascular risk factor after age. In numerous studies an inverse relationship between blood pressure values and cognitive performance emerges: it is possible that arterial hypertension plays a role in the pathogenesis of cognitive decline. Even in asymptomatic subjects the magnetic resonance signs of cerebral damage accompany cognitive impairment development. Antihypertensive therapy influence on cognitive function represents a subject of actual interest. The most studied drugs are calcium antagonists and ACE-inhibitors; they seem to have a protective effect on cognitive impairment, with regard to diuretics and beta-blockers. It would be important to study hypertensive patients, above all young asymptomatic hypertensives, even about cognitive functions, to prevent and consider cognitive decline and effective organ damage.

认知障碍和痴呆在老年人中越来越常见。第一种开始时,它悄无声息地发展,几年后就会导致痴呆。动脉高血压是老年后最重要的脑血管危险因素。在许多研究中,血压值与认知能力之间出现了反比关系:动脉高血压可能在认知能力下降的发病机制中起作用。即使在无症状的受试者中,脑损伤的磁共振迹象也伴随着认知障碍的发展。降压治疗对认知功能的影响是一个值得关注的课题。研究最多的药物是钙拮抗剂和ace抑制剂;就利尿剂和受体阻滞剂而言,它们似乎对认知障碍有保护作用。研究高血压患者,尤其是年轻无症状高血压患者的认知功能,以预防和考虑认知能力下降和有效器官损伤是很重要的。
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引用次数: 0
[Budd-Chiari syndrome]. (Budd-Chiari综合症)。
Antonino Picciotto
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引用次数: 0
[Budd-Chiari syndrome with fatal outcome in a patient with polycythemia vera and antiphospholipid antibody syndrome]. [真性红细胞增多症合并抗磷脂抗体综合征患者的Budd-Chiari综合征致死性结局]。
Gianpaolo Bragagni, Raffaele Brogna, Francesca Dazzani, Paola Franceschetti, Giorgio Zoli

We report the case of a 41-year-old woman, affected by Vaquez syndrome, admitted to our hospital for a severe pain in the right hypochondrium, suddenly followed by hepatomegaly and ascites. The clinical and laboratory data were suggestive of hepatic insufficiency and abdominal ultrasonography, integrated by color Doppler and computed tomography, revealed an interrupted hepatic venous outflow. In addition a spontaneous prolonged partial thromboplastin time was present and the patient was found to be positive for lupus anticoagulant. A transient clinical improvement, with a partial reperfusion of suprahepatic veins, was achieved with medical treatment by using anticoagulants, diuretics and paracentesis. However, the patient showed a subsequence of suprahepatic venous thrombosis, although two transjugular intrahepatic portosystemic shunts with stent placement and local thrombolysis were performed. The polycythemia vera is a disease mainly associated with Budd-Chiari syndrome but, in our patient, the thrombotic event occurred in spite of normal values of hematocrit and platelet count. Certainly in this case the lupus anticoagulant positivity represents an additional thrombogenic factor. Nowadays the antiphospholipid antibody syndrome is a recognized and not unusual cause of Budd-Chiari syndrome but, to our knowledge, this is the first case characterized by the presence of polycythemia vera and antiphospholipid antibody syndrome to be reported.

我们报告一例41岁的女性,患有Vaquez综合征,因右侧胁肋剧烈疼痛,突然出现肝肿大和腹水而入院。临床和实验室资料提示肝功能不全,腹部超声,结合彩色多普勒和计算机断层扫描,显示肝静脉流出中断。此外,自发性延长部分凝血活酶时间存在,患者被发现为狼疮抗凝剂阳性。通过使用抗凝剂、利尿剂和穿刺等药物治疗,出现了短暂的临床改善,肝上静脉部分再灌注。然而,患者随后出现肝上静脉血栓形成,尽管进行了两次经颈静脉肝内门体分流术并放置支架和局部溶栓。真性红细胞增多症是一种主要与Budd-Chiari综合征相关的疾病,但在本例患者中,尽管红细胞比容和血小板计数正常,但仍发生血栓形成事件。当然,在这种情况下,狼疮抗凝血阳性代表一个额外的血栓形成因素。目前,抗磷脂抗体综合征是一种公认的且并不罕见的Budd-Chiari综合征的病因,但据我们所知,这是第一例以真性红细胞增多症和抗磷脂抗体综合征为特征的病例报道。
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引用次数: 0
[Bone mass, bone metabolism, and prevalence of spinal fractures in recipients of allogenic bone marrow transplantation for leukemia]. [骨量、骨代谢和白血病患者接受同种异体骨髓移植后脊柱骨折的发生率]。
Claudio Letizia, Massimiliano Celi, Anna Paola Iori, Cristiana Verrelli, Chiara Caliumi, Daniele Diacinti, William Arcese, Emilio D'Erasmo

The aim of this study was to evaluate in 65 patients, who had previously undergone allogenic bone marrow transplantation (ABMT), the bone mineral density (BMD), the skeletal turnover and the prevalence of vertebral fractures. At the moment of recruiting, 10 of 65 transplanted subjects (15.3%) presented with signs of rejection of the transplanted tissue, thus they were excluded. The remaining 55 patients (21 males, 34 females, mean age 30.8 +/- 6.4 years), with a follow-up of 60 +/- 9 months after the transplant and without any treatment inducing osteopenia, underwent ABMT respectively for: chronic myeloid leukemia (n = 24); acute myeloid leukemia (n = 18); acute lymphatic leukemia (n = 13). One hundred and ten healthy control subjects (42 males and 68 females, mean age 31.0 +/- 3.7 years) matching with the patients for age, weight and height, were successively enrolled. All the participants were submitted to a densitometric evaluation (DEXA) of lumbar spine (L1-L4), of femoral neck and total femur; besides some skeletal metabolism parameters were dosed, such as: total alkaline phosphatase, bone alkaline phosphatase and urinary excretion of C-terminal telopeptide fragments normalized to creatinine. On the contrary, the morphometric evaluation, performed through a lateral dorsolumbar radiography, was actually carried out only in patients who had already undergone ABMT. The L1-L4 BMD study showed: 10/55 osteoporotic (18.1%), 19/55 osteopenic (34.5%) and 26/55 normal patients (47.4%). In transplanted patients BMD values, obtained at the three considered sites, resulted significantly reduced (p < 0.01) in comparison to controls. Moreover, in patients who underwent ABMT, a statistically significant increase was observed, in comparison to healthy subjects, in total alkaline phosphatase (p < 0.01), in bone alkaline phosphatase (p < 0.01) and in urinary excretion of C-terminal telopeptide fragments normalized to creatinine levels (p < 0.001). Seven of the 55 transplanted patients (12.7%) presented at the moment of Rx morphometric evaluation at least one vertebral fracture: 6 of whom were affected by osteoporosis and 1 by osteopenia. In conclusion, the subjects who had previously undergone ABMT maintain, even at a certain time after the transplant and without any rejection, an increased skeletal turnover and BMD values meanly lower than normal, leading to an increased risk for vertebral fracture.

本研究的目的是评估65例先前接受过同种异体骨髓移植(ABMT)的患者的骨矿物质密度(BMD)、骨骼更替和椎体骨折的发生率。在招募时,65例移植患者中有10例(15.3%)出现移植组织排斥反应,因此被排除。其余55例患者(男性21例,女性34例,平均年龄30.8 +/- 6.4岁),移植后随访60 +/- 9个月,未接受任何骨质减少治疗,分别为:慢性髓性白血病(n = 24);急性髓系白血病(18例);急性淋巴性白血病(n = 13)。健康对照110例,男性42例,女性68例,平均年龄31.0±3.7岁,与患者年龄、体重、身高相匹配。所有参与者均接受腰椎(L1-L4)、股骨颈和全股骨的密度测量评估(DEXA);此外,还测定了一些骨骼代谢参数,如:总碱性磷酸酶、骨碱性磷酸酶和尿中c端端肽片段的排泄量。相反,通过侧背腰椎x线摄影进行的形态计量学评估实际上只在已经接受过ABMT的患者中进行。L1-L4骨密度研究显示:10/55骨质疏松(18.1%),19/55骨质减少(34.5%),26/55正常(47.4%)。在移植患者中,在三个考虑的部位获得的BMD值与对照组相比显著降低(p < 0.01)。此外,在接受ABMT的患者中,与健康受试者相比,总碱性磷酸酶(p < 0.01)、骨碱性磷酸酶(p < 0.01)和尿c端端肽片段正常化至肌酐水平(p < 0.001)的排泄量均有统计学意义的增加。55例移植患者中有7例(12.7%)在Rx形态学评估时出现至少一处椎体骨折,其中6例骨质疏松,1例骨质减少。综上所述,先前接受过ABMT的受试者,即使在移植后的一定时间内,没有任何排斥反应,骨骼周转率和骨密度值的增加均低于正常水平,导致椎体骨折的风险增加。
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引用次数: 0
Hemodynamic derangement and cardiac dysfunction in cirrhosis. 肝硬化患者血流动力学紊乱与心功能障碍。
Paolo Gentilini, Giorgio La Villa, Giacomo Laffi, Massimo Pinzani

Liver cirrhosis is characterized by a long course that lasts between 15 and 20 years. The natural history of this disease depends mainly on the occurrence and progression of single complications which are today more fully understood and therefore more treatable. More specifically, those complications involving hemodynamic mechanisms have been extensively studied in recent years. Indeed, the mechanisms involved in the occurrence of sodium positive balance and ascites, with or without renal dysfunction, have been clarified. It is now possible to distinguish between two different stages in the presence of hemodynamic modifications. In the first stage, an increasing accumulation of water and sodium may occur, leading to an increase in total plasma flow. Subsequently, there is a period of vascular instability and finally, the progressive appearance of typical signs of hyperdynamic circulation. During the second stage, cardiac function may be modified and consequently profoundly altered. The early administration of diuretics (antialdosteronics) seems to be capable of modifying cardiac dysfunction, leading to a return towards a physiological status through a rapid increase in diuresis and natriuresis and a decrease in plasma volume.

肝硬化的特点是病程长,持续15至20年。本病的自然史主要取决于单一并发症的发生和进展,这些并发症目前已得到更充分的了解,因此也更容易治疗。更具体地说,这些涉及血流动力学机制的并发症近年来得到了广泛的研究。事实上,钠正平衡和腹水发生的机制,伴或不伴肾功能不全,已被澄清。在血流动力学改变的情况下,现在可以区分两个不同的阶段。在第一阶段,可能会发生水和钠的积累增加,导致总等离子体流量增加。随后,有一段时间的血管不稳定,最后,逐渐出现典型的高动力循环迹象。在第二阶段,心功能可能发生改变,并因此发生深刻改变。早期使用利尿剂(抗醛固酮)似乎能够改善心功能障碍,通过利尿和钠尿的快速增加和血浆容量的减少,导致恢复到生理状态。
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引用次数: 0
[Gastrointestinal angiodysplasias: Is Rendu-Osler-Weber disease or hereditary hemorrhagic telangiectasis the tip of an iceberg?]. [胃肠道血管发育不全:Rendu-Osler-Weber病或遗传性出血性毛细血管扩张是冰山一角吗?]。
Carlo Sabbà, Giovanni Gasbarrini
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引用次数: 0
期刊
Annali italiani di medicina interna : organo ufficiale della Societa italiana di medicina interna
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