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Open Journal of Respiratory Diseases 2012年第2期2卷 17-24页

作者： Manuel Lopez-Meseguer Río Aguilar Carles Bravo Víctor Monforte Laura Dos Carmen P. Simeon enric domingo Antonio Roman Cardiology Department Hospital Universitari de Bellvitge Hospitalet de Llobregat Spain Cardiology Department Hospital Universitari Vall d’Hebron Institut de Recerca del Hospital Universitari Vall d’Hebron Universitat Autònoma de Barcelona Barcelona Spain Department of Physiology Universitat Autonòma de Barcelona Barcelona Spain Internal Medicine Department Hospital Universitari Vall d’Hebron Institut de Recerca del Hospital Universitari Vall d’Hebron Universitat Autònoma de Barcelona Barcelona Spain Respiratory Department Hospital Universitari Vall d’Hebron Institut de Recerca del Hospital Universitari Vall d’Hebron Universitat Autònoma de Barcelona Barcelona Spain

Background: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are serious diseases with similar pathophysiologic aspects. The prognosis of patients with these conditions is highly uncertain, particularly incident cases. Methods: A Cox proportional hazards model was applied to a group of 85 patients (81% women, mean age 52 (18 - 82) years) with PAH (80%) and non-surgical CTEPH (20%) to evaluate risk factors for mortality. The following variables were included in the model: age, etiology, baseline 6-minute walk test (6 mWT), cardiac index, and improvement in the 6 mWT following initiation of first medical treatment. Results: In the multivariate analysis, the response to treatment, assessed by an improvement on the 6 mWT, was the most relevant prognostic factor in these patients (RR, 4.832 (95% CI, 1.888 - 12.364);p = 0.001). The remaining variables studied in this model had less influence on the prognosis: age > 50 years (RR, 0.744 (95% CI, 0.26 - 2.133);p = 0.582);etiology of connective tissue disease-associated PAH (RR, 3.145 (95% CI, 0.995-9.946);p = 0.051) or CTEPH (RR, 0.654 (95% CI, 0.179 - 2.387);p = 0.521) with respect to idiopathic PAH;baseline 6 mWT (RR, 1.173 (95% CI, 0.599 - 4.895);p = 0.315);or cardiac index (RR, 2.295 (95% CI, 0.793 - 6.642);p = 0.125). Conclusions: There is a high degree of uncer-tainty regarding the prognosis of PAH and CTEPH at the start of appropriate treatment. Our results support the idea that the initial treatment response is of paramount importance as prognostic factor in these patients.

关键词： Pulmonary Arterial Hypertension Chronic Thromboembolic Pulmonary Hypertension Survival Risk Factors

Assessment of right ventricular afterload by pressure waveform analysis in acute pulmonary hypertension

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World Journal of Cardiology 2011年第10期3卷 322-328页

作者： Juan C Grignola enric domingo Lucía Devera Fernando Ginés Department of Pathophysiology. Hospital de Clínicas Facultad de Medicina. Universidad de la República Area del Cor. Hospital Universitari Vall d'Hebron. Passeig Vall d'Hebron 119-129 Department of Physiology Universitat Autono- ma Barcelona 08193 Bellaterra (Cerdanyola del Vallès)

AIM: To characterize hydraulic right ventricle (RV) af- terload by pulmonary arterial pressure waveform analy- sis in an acute pulmonary hypertension (PH) model. METHODS: Pulmonary artery (PA) flow and pressure were recorded in six anesthetized sheep. Acute iso- baric PH was induced by phenylephrine (active) and PA mechanical constriction (passive). We estimated the amplitude of the forward and reflected pressure waves according to the inflection point. In most cases the in- flection pressure was smooth, thus the inflection point was defined as the time at which the first derivative ofpulmonary arterial pressure reached its first minimum. We calculated the input and characteristic (Z C , time- domain Li method) impedances, the capacitance index (stroke volume/pulse pressure), the augmentation index (AI) (reflected pressure/pulse pressure), the frac- tional pulse pressure (pulse pressure/mean pressure) and the wasted energy generated by the RV due to wave reflection during ejection (E W ). RESULTS: Pulse pressure, fractional pulse pressure, AI and Z C increased and capacitance index decreased during passive PH with respect to control (P 0.6, P < 0.05). CONCLUSION: PA pressure waveform analysis al- lows the quantification of the dynamic RV afterload. Prospective clinical studies will be necessary to validate this time-domain approach to evaluate the dynamic RV afterload in chronic PH.

关键词： Pulmonary hypertension Time-domain analysis Augmentation index Characteristic imped- ance Reflected wave

Hepatic glycogenosis: An underdiagnosed complication of diabetes mellitus?

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World Journal of Diabetes 2015年第2期6卷 321-325页

作者： María Teresa Julián Núria Alonso Isabel Ojanguren Eduarda Pizarro enric Ballestar Manel Puig-domingo Department of Medicine Universitat Autònoma de Barcelona Department of Endocrinology Hospital de Mataró Department of Pathology Hospital Germans Trias i Pujol

Hepatic glycogenosis(HG) is characterized by excessive glycogen accumulation in hepatocytes and represents a hepatic complication of diabetes that particularly occurs in patients with longstanding poorly controlled type 1 diabetes(T1D). HG has been reported to be a very rare disease, although it is believed to be extremely underdiagnosed because it is not possible to distinguish it from non-alcoholic fatty liver disease(NAFLD) unless a liver biopsy is performed. In contrast to HG, NAFLD is characterized by liver fat accumulation and is the more likely diagnosis for patients with type 2 diabetes and metabolic syndrome. The pathogenesis of HG involves the concomitant presence of insulin and excess glucose, which increases glycogen storage in the liver. HG is characterized by a transient elevation in liver transaminases and hepatomegaly. Differentiating between these two conditions is of the utmost importance because HG is a benign disease that is potentially reversible by improving glycemic control, whereas NAFLD can progress to cirrhosis. Therefore, HG should be suspected when liver dysfunction occurs in patients with poorly controlled T1 D. The aim of this article is to review the epidemiology, clinical characteristics, pathogenesis and histology of HG.

关键词： Hepatic complications Diabetes mellitus Type 1 diabetes Hepatic glycogenosis Non-alcoholicfatty liver disease

免疫抑制是断奶后多系统消耗性综合征的一个特征

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国外畜牧学（猪与禽） 2006年第5期26卷 45-46页

作者：吴昌新 Joaquim Segales enric Mateu 西班牙自治大学兽医学院动物卫生和解剖系动物卫生研究中心

研究表明,Ⅱ型猪圆环病毒(PCV2)与许多病症有关,它们统称为猪圆环病毒病(PCVD).在这些疾病中,在经济上最重要的是断奶后多系统消耗性综合征(PMWS),由于该病能直接引起死亡以及显著提高不可上市猪的比例,所以可造成严重的经济损失(Segale... 详细信息

研究表明,Ⅱ型猪圆环病毒(PCV2)与许多病症有关,它们统称为猪圆环病毒病(PCVD).在这些疾病中,在经济上最重要的是断奶后多系统消耗性综合征(PMWS),由于该病能直接引起死亡以及显著提高不可上市猪的比例,所以可造成严重的经济损失(Segales和 domingo, 2002).

关键词：断奶后多系统消耗性综合征免疫抑制 Ⅱ型猪圆环病毒特征猪圆环病毒病经济损失病症死亡