Pub Date : 2021-04-30DOI: 10.32440/AR.2021.138.01.REV03
J. Gándara
HLH is a specific disease and always a syndrome characterized by excessive immune activation and clinical and analytical data of hyperinflammation that leads to a short survival if the appropriate treatment is delayed or not applied. It has been a poorly diagnosed and its incidence has apparently increased in recent times, perhaps due to its greater recognition. Genetic /familial-based cases occur in childhood from an inherited disorder of a poor cytotoxic function of T / NK cells. In adults, the majority of patients have predisposing causes, such as Epstein Barr virus infection, neoplasms most commonly due to non-Hodgkin lymphoma of T origin, autoimmune processes, and rarely are some patients classified as idiopathic. Therapy is based on the administration of the LHH-94 treatment protocol, promoted by the Histiocytic Society. All cases with a genetic base require the subsequent application of an allogeneic transplantation of hematopoietic progenitors, which is also a therapeutic option for adults with disease resistant, progressive or recurrent.
{"title":"Adult hemophagocytic lymphohistiocytosis. Syndrome and disease","authors":"J. Gándara","doi":"10.32440/AR.2021.138.01.REV03","DOIUrl":"https://doi.org/10.32440/AR.2021.138.01.REV03","url":null,"abstract":"HLH is a specific disease and always a syndrome characterized by excessive immune activation and clinical and analytical data of hyperinflammation that leads to a short survival if the appropriate treatment is delayed or not applied. It has been a poorly diagnosed and its incidence has apparently increased in recent times, perhaps due to its greater recognition. Genetic /familial-based cases occur in childhood from an inherited disorder of a poor cytotoxic function of T / NK cells. In adults, the majority of patients have predisposing causes, such as Epstein Barr virus infection, neoplasms most commonly due to non-Hodgkin lymphoma of T origin, autoimmune processes, and rarely are some patients classified as idiopathic. Therapy is based on the administration of the LHH-94 treatment protocol, promoted by the Histiocytic Society. All cases with a genetic base require the subsequent application of an allogeneic transplantation of hematopoietic progenitors, which is also a therapeutic option for adults with disease resistant, progressive or recurrent.","PeriodicalId":75487,"journal":{"name":"Anales de la Real Academia Nacional de Medicina","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46981732","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-04-30DOI: 10.32440/AR.2021.138.01.REV06
P. Calvillo-Batllés, C. Muñoz-Núñez, Enrique Zaldívar Olmeda, V. Belloch-Ripollés, L. Martí-Bonmatí
Knowledge of lung sequelae after coronavirus disease 2019 (COVID-19) is still limited given the short follow-up time. In this work, publications with a follow-up of radiological findings once the infection caused by other previously described viruses that have the lung as their target organ and that cause probably similar changes are reviewed, including the coronaviruses that cause Severe Acute Respiratory Syndrome (SARS-CoV) and Middle East respiratory syndrome (MERS-CoV), and influenza A-subtype H1N1 virus. Lung damage caused by these viruses leads to slow-resolution interstitial disease, with variable correlation with respiratory function tests. The greater extension of the sequelae has been associated with an older age and a greater severity of the infectious clinical picture. However, the pulmonary imaging findings and their long-term functional impact are still unknown.
{"title":"Imaging of pulmonary sequelae described in viral pandemics prior to SARS-CoV-2 as a prediction model","authors":"P. Calvillo-Batllés, C. Muñoz-Núñez, Enrique Zaldívar Olmeda, V. Belloch-Ripollés, L. Martí-Bonmatí","doi":"10.32440/AR.2021.138.01.REV06","DOIUrl":"https://doi.org/10.32440/AR.2021.138.01.REV06","url":null,"abstract":"Knowledge of lung sequelae after coronavirus disease 2019 (COVID-19) is still limited given the short follow-up time. In this work, publications with a follow-up of radiological findings once the infection caused by other previously described viruses that have the lung as their target organ and that cause probably similar changes are reviewed, including the coronaviruses that cause Severe Acute Respiratory Syndrome (SARS-CoV) and Middle East respiratory syndrome (MERS-CoV), and influenza A-subtype H1N1 virus. Lung damage caused by these viruses leads to slow-resolution interstitial disease, with variable correlation with respiratory function tests. The greater extension of the sequelae has been associated with an older age and a greater severity of the infectious clinical picture. However, the pulmonary imaging findings and their long-term functional impact are still unknown.","PeriodicalId":75487,"journal":{"name":"Anales de la Real Academia Nacional de Medicina","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44866927","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-01-01DOI: 10.32440/ar.2021.138.01.supl01.art02
C. Martínez de Irujo
{"title":"Medalla de Honor de la RANME: Cayetano Martínez de Irujo","authors":"C. Martínez de Irujo","doi":"10.32440/ar.2021.138.01.supl01.art02","DOIUrl":"https://doi.org/10.32440/ar.2021.138.01.supl01.art02","url":null,"abstract":"","PeriodicalId":75487,"journal":{"name":"Anales de la Real Academia Nacional de Medicina","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"69671739","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-01-01DOI: 10.32440/ar.2021.138.03.org04
J. Tuells, C. Magdalena Egoavil, E. Hoz Tuells, O. Lebrero Catalá, C. Lebrero Catalá, E. Montagud
The objective of the study was to explore the knowledge and attitudes of Spanish medical students about vaccines, their sources of information, the training received during their studies and their future expectations about postgraduate training activities. A descriptive cross-sectional study was carried out in three Spanish universities between February 1 and May 31, 2019, through a self-administered survey of 55 questions: baseline sociodemographic questionnaire, knowledge about vaccination, necessary vaccines in health personnel, sources of information and opinion on the teaching received in vaccinology. The scale had a Cronbach’s alpha level of 0.767. There was a 67.2% (1821/2709) contact rate, a 52.2% (951/1821) response rate, and an eligibility of 93.2% (887/951). The mean age was 21.7 ± 3.6 years, of which 67.9% (646/1821) were women. The mean score for knowledge was 44.6 (SD 4.3), with a good proportion of correct answers and with significant differences between first and second cycle students, in addition to differences by gender in two statements: vaccines are effective and vaccination must be mandatory to achieve universal coverage. 34.98% (332/949) did not know the vaccination schedule. The mean attitude score was 36.7 (SD 4.1). The main sources of information on vaccines came from the academic environment and the family / internet and 87.7% (828/951) declared only ≤3 courses related to vaccination. 80.1% (461/951) believed that doctors should be the best trained professionals in vaccination. 51.2% believed that universities should be the central institution for training in vaccines during postgraduate studies, compared to 25.2% professional associations, 16.4% scientific societies and only 5.4% the pharmaceutical industry. The deficient training in vaccinology of Spanish medical students must be improved from academic institutions and professional associations, both undergraduate and graduate.
{"title":"Training in vaccinology of Spanish medical students","authors":"J. Tuells, C. Magdalena Egoavil, E. Hoz Tuells, O. Lebrero Catalá, C. Lebrero Catalá, E. Montagud","doi":"10.32440/ar.2021.138.03.org04","DOIUrl":"https://doi.org/10.32440/ar.2021.138.03.org04","url":null,"abstract":"The objective of the study was to explore the knowledge and attitudes of Spanish medical students about vaccines, their sources of information, the training received during their studies and their future expectations about postgraduate training activities. A descriptive cross-sectional study was carried out in three Spanish universities between February 1 and May 31, 2019, through a self-administered survey of 55 questions: baseline sociodemographic questionnaire, knowledge about vaccination, necessary vaccines in health personnel, sources of information and opinion on the teaching received in vaccinology. The scale had a Cronbach’s alpha level of 0.767. There was a 67.2% (1821/2709) contact rate, a 52.2% (951/1821) response rate, and an eligibility of 93.2% (887/951). The mean age was 21.7 ± 3.6 years, of which 67.9% (646/1821) were women. The mean score for knowledge was 44.6 (SD 4.3), with a good proportion of correct answers and with significant differences between first and second cycle students, in addition to differences by gender in two statements: vaccines are effective and vaccination must be mandatory to achieve universal coverage. 34.98% (332/949) did not know the vaccination schedule. The mean attitude score was 36.7 (SD 4.1). The main sources of information on vaccines came from the academic environment and the family / internet and 87.7% (828/951) declared only ≤3 courses related to vaccination. 80.1% (461/951) believed that doctors should be the best trained professionals in vaccination. 51.2% believed that universities should be the central institution for training in vaccines during postgraduate studies, compared to 25.2% professional associations, 16.4% scientific societies and only 5.4% the pharmaceutical industry. The deficient training in vaccinology of Spanish medical students must be improved from academic institutions and professional associations, both undergraduate and graduate.","PeriodicalId":75487,"journal":{"name":"Anales de la Real Academia Nacional de Medicina","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"69672240","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-01-01DOI: 10.32440/ar.2021.138.03.cc01
A. Utrera-Costero, A. M. Yepes-Agudelo, E. Moreno-Moreno, D. Veiga-Canuto, P. Bello-Arqués, L. Martí-Bonmatí
Castleman’s disease is a rare lymphoproliferative disorder. The localized form is the most frequent and it is usually asymptomatic. Although the definitive diagnosis is histologic, it is necessary to know how it manifests in imaging studies. The most frequent location in cases of unicentric Castleman’s disease is the thorax. We present the case of a patient from our center to improve the interpretation of imaging proof in this pathology.
{"title":"Imaging studies in Castleman disease. A case report","authors":"A. Utrera-Costero, A. M. Yepes-Agudelo, E. Moreno-Moreno, D. Veiga-Canuto, P. Bello-Arqués, L. Martí-Bonmatí","doi":"10.32440/ar.2021.138.03.cc01","DOIUrl":"https://doi.org/10.32440/ar.2021.138.03.cc01","url":null,"abstract":"Castleman’s disease is a rare lymphoproliferative disorder. The localized form is the most frequent and it is usually asymptomatic. Although the definitive diagnosis is histologic, it is necessary to know how it manifests in imaging studies. The most frequent location in cases of unicentric Castleman’s disease is the thorax. We present the case of a patient from our center to improve the interpretation of imaging proof in this pathology.","PeriodicalId":75487,"journal":{"name":"Anales de la Real Academia Nacional de Medicina","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"69671875","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-01-01DOI: 10.32440/ar.2021.138.03.org02
X. Pérez-Aizpurua, J. I. Monzó-Gardiner, J. Maqueda-Arellano, E. Buendía-Gonzalez, L. Cuello-Sánchez, J. Tufet i Jaumot, J. Alonso-Román, B. Gómez-Jordana Mañas, C. González-Enguita
Objectives: During 2019 there was a worldwide shortage of BCG strains for intravesical instillation, limiting the availability of full dose schemes for maintenance courses. The main objective of the present study is to analyze whether patients affected during the shortage by reduced dose courses, are at a higher risk of tumor recurrence than those receiving full dose schemes. Methods: Retrospective cohort study with a total sample of 154 subjects (60 treated during 2019 and 94 during 2017) with high-risk non-muscle invasive bladder cancer and treated with a combination of Transurethral bladder resection (TURB) followed by adjuvant intravesical instillation with BCG. Basal characteristics of both groups were analyzed. Times to event of interest (relapse; including recurrence and/or progression) were estimated by survival analysis using the Kaplan-Meier method. Relapse-free survival rates were analyzed using a multivariable Cox regression model of proportional hazards. Results: Median follow-up in the 2019 sample was 503 days and 1197 days in the 2017 group, with a median number of instillations of 8 and 12 respectively. There was a 37.7% proportion of tumoral relapse observed in the 2019 group and 29.03% during 2017. Survival analysis revealed mean relapse-free follow-up of 683 days (95%CI 601.3-765.5) in the 2019 group and of 1359 days (95%CI 1244.3-1474) in 2017. Further analysis using LogRank test revealed observed differences to be statistically significant (p=0.02) Multivariable analysis revealed a proportional Hazard ratio (HR) for relapse-free survival rate of 2.2 (95% CI: 1.13-4.25; p=0.019) regarding the 2019 sample treated with a reduced-dose course. Conclusions: BCG shortage and the subsequent reduced-dose schemes used for intravesical instillation, due to limited availability, had a significant impact on tumor relapse rates during follow-up. These findings are consistent with other published studies and show the need for full-dose BCG courses in order to prevent higher relapse rates after TURB.
{"title":"Oncological outcomes of high risk non-muscle invasive bladder tumours affected by bcg shortage for intravesical instillation","authors":"X. Pérez-Aizpurua, J. I. Monzó-Gardiner, J. Maqueda-Arellano, E. Buendía-Gonzalez, L. Cuello-Sánchez, J. Tufet i Jaumot, J. Alonso-Román, B. Gómez-Jordana Mañas, C. González-Enguita","doi":"10.32440/ar.2021.138.03.org02","DOIUrl":"https://doi.org/10.32440/ar.2021.138.03.org02","url":null,"abstract":"Objectives: During 2019 there was a worldwide shortage of BCG strains for intravesical instillation, limiting the availability of full dose schemes for maintenance courses. The main objective of the present study is to analyze whether patients affected during the shortage by reduced dose courses, are at a higher risk of tumor recurrence than those receiving full dose schemes. Methods: Retrospective cohort study with a total sample of 154 subjects (60 treated during 2019 and 94 during 2017) with high-risk non-muscle invasive bladder cancer and treated with a combination of Transurethral bladder resection (TURB) followed by adjuvant intravesical instillation with BCG. Basal characteristics of both groups were analyzed. Times to event of interest (relapse; including recurrence and/or progression) were estimated by survival analysis using the Kaplan-Meier method. Relapse-free survival rates were analyzed using a multivariable Cox regression model of proportional hazards. Results: Median follow-up in the 2019 sample was 503 days and 1197 days in the 2017 group, with a median number of instillations of 8 and 12 respectively. There was a 37.7% proportion of tumoral relapse observed in the 2019 group and 29.03% during 2017. Survival analysis revealed mean relapse-free follow-up of 683 days (95%CI 601.3-765.5) in the 2019 group and of 1359 days (95%CI 1244.3-1474) in 2017. Further analysis using LogRank test revealed observed differences to be statistically significant (p=0.02) Multivariable analysis revealed a proportional Hazard ratio (HR) for relapse-free survival rate of 2.2 (95% CI: 1.13-4.25; p=0.019) regarding the 2019 sample treated with a reduced-dose course. Conclusions: BCG shortage and the subsequent reduced-dose schemes used for intravesical instillation, due to limited availability, had a significant impact on tumor relapse rates during follow-up. These findings are consistent with other published studies and show the need for full-dose BCG courses in order to prevent higher relapse rates after TURB.","PeriodicalId":75487,"journal":{"name":"Anales de la Real Academia Nacional de Medicina","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"69672165","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-01-01DOI: 10.32440/ar.2021.138.03.rev03
G. Martos-Moreno, J. Argente
Obesity is currently one of the most common consultations in pediatrics due to its high prevalence rate in our environment. Even though an impaired balance between caloric intake and energy expenditure underlies the excessive accumulation of adipose tissue in most cases of childhood and adolescent obesity, there is a high interindividual variability in the susceptibility to develop obesity, which is based largely on each person’s singular genetic background. Advances in our understanding of the latter arise from the progressive unravelling of the pathophysiological bases of the mechanisms involved in the control of appetite, satiety, and energy expenditure. An important body of evidence in this field has derived from the study of an increasing number of cases of obesity with proven genetic (either syndromic or not), endocrinological or secondary etiology. Consequently, we now need to speak in term of «childhood obesities» when referring to the common phenotypic trait of excessive adipose tissue accumulation as the underlying pathophysiological bases are widely different between subjects; thus, individualized diagnostic and management approaches both, for obesity itself as for its associated comorbidities are required. Furthermore, the advances made in identifying patients with monogenic diseases cosegregating with the phenotype of obesity have considerably increased the modern pathophysiological bases of obesity. Therefore, it is imperative to individualize the diagnostic strategy, thus leading to a relevant change in the therapeutic approach. In fact, the results obtained by using the analog of melanocortin, setmelanotide, in patients with POMC (proopiomelanocortin), LEPR (leptin receptor) and PCSK1 (proprotein concertase subtilisin-kexin type 1) deficiency, and more recently studies of the structure and architecture of the MC4R (melanocortin receptor number 4), may allow a more precise analysis of the effects of the loss-of-function mutations in MC4R associated to obesity, as well as structural prediction of their responsiveness to setmelanotide, leading to a personalized therapy based on precision medicine.
肥胖是目前儿科最常见的咨询之一，因为它的高患病率在我们的环境。尽管在大多数儿童和青少年肥胖病例中，热量摄入和能量消耗之间的平衡受损是脂肪组织过度积累的基础，但肥胖的易感性在个体间存在很大差异，这在很大程度上取决于每个人的单一遗传背景。我们对后者的理解的进步源于对食欲、饱腹感和能量消耗控制机制的病理生理基础的逐步揭示。这一领域的重要证据来自于对越来越多的肥胖病例的研究，这些病例已被证实是遗传(无论是否综合征)、内分泌或继发病因。因此，当涉及到脂肪组织过度积累的共同表型特征时，我们现在需要谈论“儿童肥胖”，因为潜在的病理生理基础在受试者之间存在很大差异;因此，对于肥胖本身及其相关的合并症，都需要个性化的诊断和管理方法。此外，在识别与肥胖表型共分离的单基因疾病患者方面取得的进展大大增加了肥胖的现代病理生理基础。因此，必须个性化诊断策略，从而导致治疗方法的相关变化。事实上，通过在POMC (proopiomelocortin)、LEPR (leptin受体)和PCSK1 (proprotein concertase subtilisin-kexin type 1)缺乏症患者中使用黑素皮质素类似物setmelanotide获得的结果，以及最近对MC4R (melanocortin receptor number 4)的结构和结构的研究，可能允许更精确地分析与肥胖相关的MC4R功能丧失突变的影响。以及他们对setmelanotide反应的结构预测，导致基于精准医学的个性化治疗。
{"title":"Precision medicine in childhood obesities","authors":"G. Martos-Moreno, J. Argente","doi":"10.32440/ar.2021.138.03.rev03","DOIUrl":"https://doi.org/10.32440/ar.2021.138.03.rev03","url":null,"abstract":"Obesity is currently one of the most common consultations in pediatrics due to its high prevalence rate in our environment. Even though an impaired balance between caloric intake and energy expenditure underlies the excessive accumulation of adipose tissue in most cases of childhood and adolescent obesity, there is a high interindividual variability in the susceptibility to develop obesity, which is based largely on each person’s singular genetic background. Advances in our understanding of the latter arise from the progressive unravelling of the pathophysiological bases of the mechanisms involved in the control of appetite, satiety, and energy expenditure. An important body of evidence in this field has derived from the study of an increasing number of cases of obesity with proven genetic (either syndromic or not), endocrinological or secondary etiology. Consequently, we now need to speak in term of «childhood obesities» when referring to the common phenotypic trait of excessive adipose tissue accumulation as the underlying pathophysiological bases are widely different between subjects; thus, individualized diagnostic and management approaches both, for obesity itself as for its associated comorbidities are required. Furthermore, the advances made in identifying patients with monogenic diseases cosegregating with the phenotype of obesity have considerably increased the modern pathophysiological bases of obesity. Therefore, it is imperative to individualize the diagnostic strategy, thus leading to a relevant change in the therapeutic approach. In fact, the results obtained by using the analog of melanocortin, setmelanotide, in patients with POMC (proopiomelanocortin), LEPR (leptin receptor) and PCSK1 (proprotein concertase subtilisin-kexin type 1) deficiency, and more recently studies of the structure and architecture of the MC4R (melanocortin receptor number 4), may allow a more precise analysis of the effects of the loss-of-function mutations in MC4R associated to obesity, as well as structural prediction of their responsiveness to setmelanotide, leading to a personalized therapy based on precision medicine.","PeriodicalId":75487,"journal":{"name":"Anales de la Real Academia Nacional de Medicina","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"69672308","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-01-01DOI: 10.32440/ar.2021.138.01.supl01.art08
F. González de Posada
{"title":"Elogio del Prof. Juan Jiménez Collado","authors":"F. González de Posada","doi":"10.32440/ar.2021.138.01.supl01.art08","DOIUrl":"https://doi.org/10.32440/ar.2021.138.01.supl01.art08","url":null,"abstract":"","PeriodicalId":75487,"journal":{"name":"Anales de la Real Academia Nacional de Medicina","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"69671794","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-01-01DOI: 10.32440/ar.2021.138.03.doc01
J. R. de Berrazueta Fernández
Pulmonary Hypertension is characterized by the increase of the pulmonary systolic pressure above 30 mm Hg or 25 of mean pressure. Different diseases generate changes in the physiology of the vessel wall. The balance between vasoconstrictor and vasodilator molecules, mainly released by the endothelium, is lost in favor of vasoconstrictors, which in addition to generating an inadequate vasoconstrictor response, induce proliferative changes of different cell lines that modify the structure of the vessel wall, from the hypertrophy of the media until progressive occlusion of the arteriolar beds, formation of angiomatous lesions, micro aneurysms, plexiform lesions, thrombotic occlusions and necrotizing arteritis corresponding to the different degrees of pulmonary hypertension. The symptoms of right ventricular failure, the radiographic and electrocardiographic data of right ventricular growth or dilatation, allow us to suspect the diagnosis that can be confirmed by echocardiography, measuring the peak velocity of the tricuspid regurgitation that corresponds to the systolic pressure of the pulmonary artery. The progressive severity of the lesions allows the treatment to be staggered, including measures to compensate for heart failure with loop diuretics, permanent anticoagulation, and vasodilators such as Calcium Channel Blockers and to greater severity, endothelin antagonists, phosphodiesterase 5 inhibitors, prostacyclin and derivatives and combinations of several of these drugs. The creation of a right to left shunt decompresses the lung bed, reducing pressure, with desaturation and an increase in systemic output, but improving exercise tolerance and survival.
{"title":"Pulmonary hypertension. From vasomotor molecules to treatment","authors":"J. R. de Berrazueta Fernández","doi":"10.32440/ar.2021.138.03.doc01","DOIUrl":"https://doi.org/10.32440/ar.2021.138.03.doc01","url":null,"abstract":"Pulmonary Hypertension is characterized by the increase of the pulmonary systolic pressure above 30 mm Hg or 25 of mean pressure. Different diseases generate changes in the physiology of the vessel wall. The balance between vasoconstrictor and vasodilator molecules, mainly released by the endothelium, is lost in favor of vasoconstrictors, which in addition to generating an inadequate vasoconstrictor response, induce proliferative changes of different cell lines that modify the structure of the vessel wall, from the hypertrophy of the media until progressive occlusion of the arteriolar beds, formation of angiomatous lesions, micro aneurysms, plexiform lesions, thrombotic occlusions and necrotizing arteritis corresponding to the different degrees of pulmonary hypertension. The symptoms of right ventricular failure, the radiographic and electrocardiographic data of right ventricular growth or dilatation, allow us to suspect the diagnosis that can be confirmed by echocardiography, measuring the peak velocity of the tricuspid regurgitation that corresponds to the systolic pressure of the pulmonary artery. The progressive severity of the lesions allows the treatment to be staggered, including measures to compensate for heart failure with loop diuretics, permanent anticoagulation, and vasodilators such as Calcium Channel Blockers and to greater severity, endothelin antagonists, phosphodiesterase 5 inhibitors, prostacyclin and derivatives and combinations of several of these drugs. The creation of a right to left shunt decompresses the lung bed, reducing pressure, with desaturation and an increase in systemic output, but improving exercise tolerance and survival.","PeriodicalId":75487,"journal":{"name":"Anales de la Real Academia Nacional de Medicina","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"69671891","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-01-01DOI: 10.32440/ar.2021.138.03.rev04
E. Armas, E. Sanz, J. J. Jover, M.F. Alarcón, S. Martin, L. Cristóbal, A. Maldonado
Brachial plexus and peripheral nerve injuries are complex and suppose a great functional deficit. An early diagnostic and surgical exploration and reconstruction is essential in some cases. Muscular atrophy generated by the chronic denervation limits surgical treatments that can not be performed once overcoming a specific time frame. Radiologic and neurophysiological tests are essential to perform a correct diagnostic and follow-up of these injuries, and rehabilitation is crucial to achieve a good postsurgical outcome. Surgical treatments include primary nerve repair, repair with nerve grafts, nerve transfers, free functional muscle transfers and tendon transfers. Each technique should be adequate to the clinic and evolution time of the injury. Once muscle atrophy is established, nerve repair and nerve transfers do not achieve a good functional result. Due to the high complexity of these injuries and the need for different medical specialists, these patients must be treated in multidisciplinary units to achieve the best possible result.
{"title":"Current treatment of traumatic brachial plexus and peripheral nerve injuries","authors":"E. Armas, E. Sanz, J. J. Jover, M.F. Alarcón, S. Martin, L. Cristóbal, A. Maldonado","doi":"10.32440/ar.2021.138.03.rev04","DOIUrl":"https://doi.org/10.32440/ar.2021.138.03.rev04","url":null,"abstract":"Brachial plexus and peripheral nerve injuries are complex and suppose a great functional deficit. An early diagnostic and surgical exploration and reconstruction is essential in some cases. Muscular atrophy generated by the chronic denervation limits surgical treatments that can not be performed once overcoming a specific time frame. Radiologic and neurophysiological tests are essential to perform a correct diagnostic and follow-up of these injuries, and rehabilitation is crucial to achieve a good postsurgical outcome. Surgical treatments include primary nerve repair, repair with nerve grafts, nerve transfers, free functional muscle transfers and tendon transfers. Each technique should be adequate to the clinic and evolution time of the injury. Once muscle atrophy is established, nerve repair and nerve transfers do not achieve a good functional result. Due to the high complexity of these injuries and the need for different medical specialists, these patients must be treated in multidisciplinary units to achieve the best possible result.","PeriodicalId":75487,"journal":{"name":"Anales de la Real Academia Nacional de Medicina","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"69672350","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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