Budd-Chiari syndrome with hepatopulmonary syndrome: a case report and literature review / 中华儿科杂志

Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking "Budd-Chiari syndrome" and "hepatopulmonary syndrome" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023. Combined with this case, the clinical characteristics, diagnosis, treatment and prognosis of Budd-Chiari syndrome with HPS in children under the age of 18 were summarized. Results: A 13-year-old boy, presented with cyanosis and chest tightness after activities for 6 months, and yellow staining of the skin for 1 week. Physical examination at admission not only found mild yellow staining of the skin and sclera, but also found cyanosis of the lips, periocular skin, and extremities. Laboratory examination showed abnormal liver function with total bilirubin 53 μmol/L, direct bilirubin 14 μmol/L, and indirect bilirubin 39 μmol/L, and abnormal blood gas analysis with the partial pressure of oxygen of 54 mmHg (1 mmHg=0.133 kPa), the partial pressure of carbon dioxide of 31 mmHg, and the alveolar-arterial oxygen gradient of 57 mmHg. Hepatic vein-type Budd-Chiari syndrome, cirrhosis, and portal hypertension were indicated by abdominal CT venography. Contrast-enhanced transthoracic echocardiography (CE-TTE) was positive. After symptomatic and supportive treatment, this patient was discharged and received oxygen therapy outside the hospital. At follow-up until March 2023, there was no significant improvement in hypoxemia, accompanied by limited daily activities. Based on the literature, there were 3 reports in English while none in Chinese, 3 cases were reported. Among a total of 4 children, the chief complaints were dyspnea, cyanosis, or hypoxemia in 3 cases, and unknown in 1 case. There were 2 cases diagnosed with Budd-Chiari syndrome with HPS at the same time due to respiratory symptoms, and 2 cases developed HPS 1.5 years and 8.0 years after the diagnosis of Budd-Chiari syndrome respectively. CE-TTE was positive in 2 cases and pulmonary perfusion imaging was positive in 2 cases. Liver transplantation was performed in 2 cases and their respiratory function recovered well; 1 case received oxygen therapy, with no improvement in hypoxemia; 1 case was waiting for liver transplantation. Conclusions: The onset of Budd-Chiari syndrome with HPS is insidious. The most common clinical manifestations are dyspnea and cyanosis. It can reduce misdiagnosis to confirm intrapulmonary vascular dilatations with CE-TTE at an early stage. Liver transplantation is helpful in improving the prognosis.

Características clínicas, diagnóstico e tratamento de mulher com síndrome de Meigs: relato de caso e revisão narrativa / Clinical characteristics, diagnosis and treatment of a woman with Meigs syndrome: case report and narrative review

O objetivo deste estudo é descrever o caso de mulher com síndrome de Meigs e apresentar a revisão narrativa sobre o tema. Paciente do sexo feminino, 30 anos, nulípara, encaminhada ao hospital por massa anexial e história prévia de drenagem de derrame pleural. Evoluiu com instabilidade hemodinâmica por derrame pleural hipertensivo à direita, sendo submetida a drenagem torácica, com citologia do líquido negativa. Após, foi submetida a laparotomia: realizada salpingo-ooforectomia esquerda. A congelação e a análise histopatológica diagnosticaram fibroma ovariano. A citologia ascítica foi negativa. CA-125 elevado, presença de derrames cavitários e exame de imagem suspeito podem mimetizar um cenário de neoplasia maligna de ovário em estágio avançado. Entretanto, na síndrome de Meigs clássica, o tratamento é cirúrgico, sendo o diagnóstico obtido por meio da análise histopatológica do tumor ovariano. O manejo da síndrome de Meigs clássica é cirúrgico e, após a remoção do tumor, o derrame pleural e a ascite desaparecem.

To describe a case of Meigs syndrome and present a narrative review of the condition. Female patient, 30 years old, nulliparous, referred to the hospital due to an adnexal mass and a previous drainage of pleural effusion. She developed hemodynamic instability due to a hypertensive right pleural effusion being submitted to chest drainage, with negative cytology of the fluid. She underwent laparotomy: Left salpingo-oophorectomy was performed and frozen section and histopathological analysis diagnosed an ovarian fibroma. Ascites cytology was negative. Elevated CA-125, presence of cavitary effusions, suspicious imaging exam can mimic a scenario of ovarian cancer at an advanced stage. However, in classical Meigs syndrome, treatment is surgical, and the diagnosis is obtained through histopathological analysis of the ovarian tumor. Classical Meigs syndrome' management is surgical. After tumor removal, pleural effusion and ascites resolve.

Afectación respiratoria en paciente con acrodisostosis: una asociación infrecuente de una enfermedad rara / Respiratory impairment in a patient with acrodysostosis: A rare association of an uncommon pathology

La acrodisostosis es una displasia esquelética rara, de herencia autosómica dominante, que se caracteriza por la presencia de disostosis facial y periférica, talla baja y diferentes grados de obesidad. La acrodisostosis de tipo 1, secundaria a la mutación heterocigota en el gen PRKAR1A (17q24.2), se caracteriza por la asociación de resistencia hormonal múltiple con anomalías esqueléticas. Su incidencia está infradiagnosticada debido a que comparte rasgos clínicos y de laboratorio con otras entidades como el seudohipoparatiroidismo. Presentamos el caso de una niña de 8 años, con acrodisostosis tipo 1, confirmada mediante estudio genético. Además del fenotipo característico descrito, la talla baja y la resistencia hormonal, la paciente presentó una afectación progresiva de la función pulmonar: un patrón pulmonar obstructivo no reversible. En la literatura revisada, no se han encontrado otros casos que describan esta asociación entre acrodisostosis y afectación respiratoria.

Acrodysostosis is a rare skeletal displasia, of autosomal dominant inheritance, characterized by the presence of facial and peripheral dysostosis, short stature and obesity. Type 1 acrodysostosis is secondary to a mutation in the PRKAR1A (17q24.2) gene, which results in multi hormonal resistance and skeletal anomalities. This syndrome is under-diagnosed as it shares analytical and clinical characteristics with other entities, such as pseudohypoparathyroidism. We report the case of an eight-year-old girl with genetically confirmed type 1 acrodysostosis. In addition to the characteristic phenotype described, the short stature and the hormonal resistance, the patient suffered a progressive lung function deterioration: an irreversible pulmonary obstructive pattern. We have not found in previous literature cases reporting an association between acrodysostosis and lung function impairement.

Embolismos sépticos pulmonares y sistémicos como forma de presentación de la endocarditis infecciosa / Septic pulmonary and systemic embolisms as form of presentation of infective endocarditis

Introducción: A partir de los años 50, la presentación clínica clásica de la endocarditis infecciosa ha variado. Debido al uso de antibióticos, drogas ilícitas, catéteres venosos, etc., las manifestaciones tradicionales no son frecuentes. Objetivo: Presentar un caso con endocarditis infecciosa y comentar las manifestaciones embolígenas, así como las medidas preventivas con las nuevas técnicas. Caso clínico: Enfermo con insuficiencia renal crónica, diabético, hipertenso, con catéter venoso central, que presentó, después de una sección de hemodiálisis; escalofríos intensos, fiebre de 39,5 0C, cefalea intensa, toma del estado general, dolor torácico intenso punzante, tos, expectoración con sangre roja rutilante, disnea, soplo regurgitante holosistólico. Se le realizó ecocardiograma dópler que muestra múltiples vegetaciones pequeñas, hemocultivos positivos a estafilococos dorado. Fue tratado según los resultados del antibiograma durante 6 semanas y resolvió su extrema gravedad. Conclusiones: La endocarditis infecciosa puede tener manifestaciones muy diferentes al de décadas anteriores; puede aparecer como cuadro agudo fulminante por manifestaciones embólicas y sépticas múltiples(AU)

Introduction: Since the 1950s, the classical clinical presentation of infectious endocarditis (E.I) has varied. Due to the use of antibiotics, illicit drugs, venous catheters, traditional manifestations are not frequent. Objectives: To review the embolic presentation of endocarditis and pecify the preventive measures with the new techniques. Clinical case: A patient with chronic renal insufficiency, diabetic, hypertensive, with central venous catheter, intense chills, fever of 39.5 ° C, intense headache, general state, severe chest pain, cough, expectoration with bright red blood, dyspnea, holosystolic regurgitant murmur, after a section of hemodialysis. Doppler echocardiogram was performed, visualizing multiple small vegetation's, positive blood cultures to golden staphylococci, treatment according to antibiograms for 6 weeks, at the end of which the extreme severity was resolved. Comments: Infective endocarditis can have a very different behavior from previous decades; it can appear as an acute fulminating disease due to embolic, septic, multiple manifestations(AU)

Acute kidney injury and other factors associated with mortality in hiv-infected patients

SUMMARY OBJECTIVE: HIV-related mortality is still high, especially in developed countries. The aim of this study is to investigate factors associated to death in HIV-infected patients. METHODS: This is a cross-sectional study with all HIV adult patients admitted to a tertiary infectious diseases hospital in Fortaleza, Northeast Brazil, from January 2013 to December 2014. Patients were divided into two groups: survivors and non-survivors. Demo-graphical, clinical and laboratory data were compared and a logistic regression was performed in order to investigate risk factors for death. P values ≤0.05 were considered statistically significant. RESULTS: A total of 200 patients with mean age of 39 years were including in the study, 69.5% males. Fifteen patients (7.5%) died. Non-survivors presented a higher percentage of males (93.3 vs. 67.3%, p = 0.037). Non-survivors presented AKI (73.3 vs. 10.3%, p < 0.001), liver dysfunction (33.3 vs. 11.5, p = 0.031), dyspnea (73.3 vs. 33.0%, p = 0.002) and disorientation (33.3 vs. 12.4%, p = 0.025) more frequently. Non-survivors also had higher levels of urea (73.8 ± 52.7vs. 36.1 ± 29.1 mg/dL, p < 0.001), creatinine (1.98 ± 1.65 vs. 1.05 ± 1.07 mg/dL, p < 0.001), aspartate aminotransferase (130.8 vs. 84.8 U/L, p = 0.03), alanine aminotransferase (115.6 vs. 85.4 U/L, p = 0.045) and lactate dehydrogenase (LDH) (1208 vs. 608 U/L, p = 0.012), as well as lower levels of bicarbonate (18.0 ± 4.7 vs. 21.6 ± 4.6 mEq/L, p = 0.016) and PCO2 (27.8 ± 7.7 vs. 33.0 ± 9.3 mmHg, p = 0.05). In multivariate analysis, disorientation (p = 0.035, OR = 5.523, 95%CI = 1.130 - 26.998), dyspnoea (p = 0.046, OR = 4.064, 95%CI = 1.028 - 16.073), AKI (p < 0.001, OR = 18.045, 95%CI = 4.308 - 75.596) and disseminated histoplasmosis (p = 0.016, OR = 12.696, 95%CI = 1.618 - 99.646) and LDH > 1000 U/L (p = 0.038, OR = 4.854, 95%CI = 1.093 - 21.739) were risk factors for death.]CONCLUSION: AKI and disseminated histoplasmosis (DH) were the main risk factors for death in the studied population. Neurologic and respiratory impairment as well as higher levels of LDH also increased mortality in HIV-infected patients.

RESUMO INTRODUÇÃO: A mortalidade relacionada ao HIV ainda é alta, especialmente nos países em desenvolvimento. O objetivo deste estudo é investigar os fatores associados ao óbito em pacientes com HIV. MÉTODOS: Trata-se de um estudo transversal com todos os pacientes com HIV admitidos consecutivamente em um hospital terciário de doenças infecciosas em Fortaleza, Nordeste do Brasil, entre janeiro de 2013 e dezembro de 2014. Os pacientes foram divididos em dois grupos: sobreviventes e não sobreviventes. Dados demográficos, clínicos e laboratoriais foram comparados e análise de regressão logística foi feita para investigação dos fatores de risco para óbito. RESULTADOS: Um total de 200 pacientes, com média de idade de 39 anos, foi incluído no estudo, sendo 69,5% do sexo masculino. Óbito ocorreu em 15 pacientes (7,5%). Os não sobreviventes apresentaram maior percentual de homens (93,3 vs. 67,3%, p = 0,037) e um menor tempo de internação (8 ± 6 vs. 18 ± 15 dias, p = 0,005). Na análise multivariada, desorientação (p = 0,035, OR = 5,523), dispneia (p = 0,046, OR = 4,064), LRA (p < 0,001, OR = 18,045), histoplasmose disseminada (p = 0,016, OR = 12,696) e desidrogenase lática (LDH) > 1.000 U/L (p = 0,038, OR = 4,854) foram fatores de risco para óbito. CONCLUSÕES: LRA e histoplasmose disseminada foram os principais fatores de risco para óbito na população estudada. Distúrbios neurológicos e respiratórios, bem como níveis elevados de LDH, também estiveram associados com o aumento da mortalidade em pacientes com HIV.

Associação entre dispneia e o grau de disfunção diastólica ao ecocardiograma / Association among dyspnea and the degrees of diastolic dysfunction at echocardiography

ntrodução: A disfunção diastólica do ventrículo esquerdo (DDVE) pode levar a insuficiência cardíaca com fração de ejeção preservada. O ecocardiograma, em especial o Doppler tecidual, é o principal exame utilizado. A clínica geralmente cursa com dispneia, avaliada pela escala modified Medical Research Council (mMRC). Entretanto, existem poucos estudos que investiguem qual é a associação entre o sintoma e a disfunção. Objetivo: Avaliar se a queixa de dispneia se associa com a progressão da DDVE e se há relação entre mMRC e os graus de DDVE. Métodos: Estudo caso-controle e transversal, com 60 participantes, com avaliação clínica (mMRC) e ecocardiográfica (parâmetros bidimensionais, Doppler espectral e tecidual). Dentre os participantes avaliados, 49 configuraram o grupo caso (DDVE com dispneia) e 11 o grupo controle (DDVE sem dispneia). Foram excluídos participantes com comorbidades ou outras alterações ecocardiográficas relacionadas à dispneia. Resultados: A média de idade foi de 61,7 anos (± 7,9), sendo 72% mulheres. Do total, 82% dos participantes apresentaram dispneia. Destes, 82% apresentaram DDVE grau I. Todos apresentaram função ventricular sistólica preservada. A presença de dispneia se associou com o grau de DDVE (p = 0,04), relação que não se observou com a intensidade do sintoma (p = 0,72). Conclusão: Houve associação entre a presença de dispneia e grau de DDVE, porém não houve relação entre a progressão da dispneia e a evolução da DDVE. O aumento do átrio esquerdo e a presença de doença arterial coronariana foram associadas com graus mais avançados de DDVE

Introduction: The left ventricle diastolic dysfuntion (LVDD) can lead to heart failure with preserved ejection fraction. Echocardiography, especially the tissue Doppler, is the main exam. The clinic has dyspnea as a typical symptom, which is evaluated by modified Medical Research Council (mMRC). However, there are few studies that investigate what is the association between the symptom and LVDD. Objective: Evaluate if dyspnea is associated with the advancement of LVDD and if there is a linkage between mMRC and the degrees of LVDD. Method: Case-control transversal study, with 60 participants, with clinical (mMRC) and echocardiographic (bidimensional parameters, spectral and tissue Doppler) evaluation. Among the participants, 49 constituted the case group (LVDD with dyspnea) and 11 the control group (LVDD without dyspnea). Participants with co-morbidity or other echocardiographic abnormalities related to dyspnea were excluded. Results: The average age was 61,7 (± 7,9) years and 72% were women. In overwall, 82% of the participants had dyspnea. Among them, 82% had LVDD degree I. All of the study population had preserved ventricular systolic function. The presence of dyspnea was associated with the degree of LVDD (p = 0,04), however, the symptom severity was not (p = 0,72). Conclusion: Dyspnea was associated with the degree of LVDD, but there was no association between the symptom severity and the evolution of LVDD. The aging, the increase of left atrium and coronary artery disease were associated with the advanced grades of LVDD

TeleCondutas: doença pulmonar obstrutiva crônica / TeleGuides: chronic obstructive pulmonary disease

A Doença Pulmonar Obstrutiva Crônica (DPOC) é caracterizada por sintomas respiratórios (dispneia, tosse e expectoração) e pela limitação persistente ao fluxo aéreo, que não é completamente reversível. É uma doença progressiva decorrente a resposta inflamatória anormal das vias aéreas e dos pulmões a partículas nocivas e gases inalados. A doença afeta 5% da população e está associada a alta morbidade e mortalidade. O tabagismo é o principal fator de risco, porém outros poluentes (produtos químicos, poeira, pó de carvão, combustíveis e fumaças) devem ser considerados na avaliação do paciente. Estabelecer o diagnóstico corretamente é importante, pois o manejo adequado reduz sintomas, frequência e gravidade das exacerbações, melhor qualidade de vida e aumenta a sobrevida do paciente. Esta guia apresenta informação que orienta a conduta para casos de doença pulmonar obstrutiva crônica no contexto da Atenção Primária à Saúde, incluindo: Sinais e sintomas, Diagnóstico, Avaliação, Abordagem Integral, Tratamento Oxigenoterapia Domiciliar Prolongada, Exacerbação, Técnica Inalatória, Encaminhamento para serviço especializado.

Abordagem clínica da congestão pulmonar aguda no cardiopata / Clinical approach to acute pulmonary congestion in heart disease

A congestão pulmonar aguda no paciente com doença cardíaca é uma manifestação clínica de extrema gravidade, ocorrendo em aproximadamente 25% dos casosde insuficiência cardíaca aguda. O diagnóstico é essencialmente clínico, baseado na anamnese e exame físico. Os exames complementares não devem retardar o início do tratamento na sala de emergência. Descontrole pressórico, progressão da doença valvar, infarto do miocárdio e arritmias são fatores desencadeantes frequentes paraedema agudo de pulmão. O tratamento inicial fundamenta-se na suplementação de oxigênio e suporte ventilatório, administração de opioides, diuréticos e vasodilatadores endovenosos. Inotrópicos estão indicados na presença de instabilidade hemodinâmicacom disfunção orgânica...

Acute pulmonary congestion in patients with cardiac disease is a clinical manifestation of extreme severity, occurring in approximately 25% of cases of acute heart failure. Diagnosis is essentially clinical, based on history and physical examination. Complementarytests should not delay the start of treatment in the emergency room. Uncontrolled blood pressure, progression of valvular disease, myocardial infarction, and arrhythmias are common triggers for acute pulmonary edema. Initial treatment is based on supplemental oxygen and ventilatory support, administration of opioids, intravenous diuretics, andvasodilators. Inotropic agents are indicated in the presence of hemodynamic instability with organ dysfunction...

Characteristics of Patients with Chronic Obstructive Pulmonary Disease at the First Visit to a Pulmonary Medical Center in Korea: The KOrea COpd Subgroup Study Team Cohort

The Korea Chronic Obstructive Pulmonary Disorders Subgroup Study Team (Korea COPD Subgroup Study team, KOCOSS) is a multicenter observational study that includes 956 patients (mean age 69.9 ± 7.8 years) who were enrolled from 45 tertiary and university-affiliated hospitals from December 2011 to October 2014. The initial evaluation for all patients included pulmonary function tests (PFT), 6-minute walk distance (6MWD), COPD Assessment Test (CAT), modified Medical Research Council (mMRC) dyspnea scale, and the COPD-specific version of St. George's Respiratory Questionnaire (SGRQ-C). Here, we report the comparison of baseline characteristics between patients with early- (Global Initiative for Chronic Obstructive Lung Disease [GOLD] stage I and II/groups A and B) and late-stage COPD (GOLD stage III and IV/groups C and D). Among all patients, the mean post-bronchodilator FEV1 was 55.8% ± 16.7% of the predicted value, and most of the patients were in GOLD stage II (520, 56.9%) and group B (399, 42.0%). The number of exacerbations during one year prior to the first visit was significantly lower in patients with early COPD (0.4 vs. 0.9/0.1 vs. 1.2), as were the CAT score (13.9 vs. 18.3/13.5 vs. 18.1), mMRC (1.4 vs. 2.0/1.3 vs.1.9), and SGRQ-C total score (30.4 vs. 42.9/29.1 vs. 42.6) compared to late-stage COPD (all P < 0.001). Common comorbidities among all patients were hypertension (323, 37.7%), diabetes mellitus (139, 14.8%), and depression (207, 23.6%). The data from patients with early COPD will provide important information towards early detection, proper initial management, and design of future studies.

Quality of Life on Arterial Hypertension: Validity of Known Groups of MINICHAL / Qualidade de Vida na Hipertensão Arterial: Validade de Grupos Conhecidos do MINICHAL

Introductions: In the care of hypertension, it is important that health professionals possess available tools that allow evaluating the impairment of the health-related quality of life, according to the severity of hypertension and the risk for cardiovascular events. Among the instruments developed for the assessment of health-related quality of life, there is the Mini-Cuestionario of Calidad de Vida en la Hipertensión Arterial (MINICHAL) recently adapted to the Brazilian culture. Objective: To estimate the validity of known groups of the Brazilian version of the MINICHAL regarding the classification of risk for cardiovascular events, symptoms, severity of dyspnea and target-organ damage. Methods: Data of 200 hypertensive outpatients concerning sociodemographic and clinical information and health-related quality of life were gathered by consulting the medical charts and the application of the Brazilian version of MINICHAL. The Mann-Whitney test was used to compare health-related quality of life in relation to symptoms and target-organ damage. The Kruskal-Wallis test and ANOVA with ranks transformation were used to compare health-related quality of life in relation to the classification of risk for cardiovascular events and intensity of dyspnea, respectively. Results: The MINICHAL was able to discriminate health-related quality of life in relation to symptoms and kidney damage, but did not discriminate health-related quality of life in relation to the classification of risk for cardiovascular events. Conclusion: The Brazilian version of the MINICHAL is a questionnaire capable of discriminating differences on the health‑related quality of life regarding dyspnea, chest pain, palpitation, lipothymy, cephalea and renal damage. .

Fundamento: No cuidado ao hipertenso, é importante que o profissional de saúde disponha de ferramentas que possibilitem avaliar o comprometimento da qualidade de vida relacionada à saúde, de acordo com a gravidade da hipertensão e o risco para eventos cardiovasculares. Dentre os instrumentos criados para avaliação da qualidade de vida relacionada à saúde, destaca-se o Mini-Cuestionario de Calidad de Vida en la Hipertensión Arterial (MINICHAL), recentemente adaptado para a cultura brasileira. Objetivo: Estimar a validade de grupos conhecidos da versão brasileira do MINICHAL em relação à classificação de risco para eventos cardiovasculares, sintomas, intensidade da dispneia e lesões de órgãos-alvo. Métodos: Foram investigados 200 hipertensos em seguimento ambulatorial, cujos dados sociodemográficos, clínicos e de qualidade de vida relacionada à saúde foram obtidos por meio de consulta ao prontuário e da aplicação da versão brasileira do MINICHAL. O teste de Mann-Whitney foi utilizado para comparar qualidade de vida relacionada à saúde em relação aos sintomas e às lesões de órgãos-alvo. Teste de Kruskal-Wallis e ANOVA com transformação nos ranks foram empregados para comparar qualidade de vida relacionada à saúde em relação à classificação de risco para eventos cardiovasculares e intensidade da dispneia, respectivamente. Resultados: O MINICHAL discriminou qualidade de vida relacionada à saúde em relação aos sintomas e dano renal (lesões de órgãos-alvo), porém não discriminou qualidade de vida relacionada à saúde em relação à classificação de risco para eventos cardiovasculares. Conclusão: A versão brasileira do MINICHAL é um instrumento capaz de discriminar diferenças na qualidade de vida relacionada à saúde em relação aos sintomas de dispneia, precordialgia, palpitação, lipotímia, cefaleia e presença de dano renal. .

Prevalence of Left Ventricular Diastolic Dysfunction in Patients with Normal Systolic Function and its Relation with Other Risk Factors from Eastern India.

Impaired diastolic filling of left ventricle may be the important cause of symptoms ranging from mild to severe form of dyspnea with overt features of pulmonary edema which may be wrongly imparted to systolic failure. To study prevalence and severity of diastolic dysfunction and its relation with various risk factors. Methods: Patients from in and out patients department were subjected to detail history, estimation of BMI, blood sugar levels, BP measurement, echocardiographic assessment of cardiac function. Patients of isolated diastolic dysfunction were identified and the degree of diastolic dysfunction was correlated with functional class (NYHA grade) of the patients as well as various risk factors. Results: Out of 400 patients 16 (12.5%) asymptomatic patients had diastolic dysfunction (DD). Severity of diastolic dysfunction increased as the presenting NYHA grade was higher. In NYHA grades III and IV, 59% and 57% of the patients respectively had diastolic dysfunction .DD was significantly associated with diabetes (p<.0001), BMI (p =0.0002), dyslipidemia (p =0.0491), hypertension (p=0.0489). Conclusion: DD is a significant cardiac morbidity. Hypertension, diabetes, dyslipidemia and obesity have significant association with diastolic dysfunction. There is no randomized control trials for treatment for DD. Whether asymptomatic DD needs treatment is not known.

Bronchiolitis Obliterans Associated with Stevens-Johnson Syndrome: A Case Report

We report a case of bronchiolitis obliterans associated with Stevens-Johnson syndrome. A 59-year-old man presented with respiratory distress that gradually worsened over 3 months. He had been diagnosed with Stevens-Johnson syndrome 3 months before admission. He had no history of previous airway disease. On physical examination, expiratory breathing sounds were not audible, and a chest X-ray revealed a hyperinflated lung. A pulmonary function test indicated a severe obstructive pattern. Computed tomography scans of inspiratory and expiratory phases of respiration showed oligemia and air trapping, and both were more prominent on expiration view than on inspiration view. The pathogenesis of bronchiolitis obliterans associated with Stevens-Johnson syndrome is largely unknown.

Mortality and Embolic Potential of Cardiac Tumors / Mortalidade e Potencial Embólico dos Tumores Cardíacos

Background: Cardiac tumors are rare, mostly benign with high embolic potential. Objectives: To correlate the histological type of cardiac masses with their embolic potential, implantation site and long term follow up in patients undergoing surgery. Methods: Between January 1986 and December 2011, we retrospectively analyzed 185 consecutive patients who underwent excision of intracardiac mass (119 females, mean age 48±20 years). In 145 patients, the left atrium was the origin site. 72% were asymptomatic and prior embolization was often observed (19.8%). The diagnosis was established by echocardiography, magnetic resonance and histological examination. Results: Most tumors were located in the left side of the heart. Myxoma was the most common (72.6%), followed by fibromas (6.9%), thrombi (6.4%) and sarcomas (6.4%). Ranging from 0.6cm to 15cm (mean 4.6 ± 2.5cm) 37 (19.8%) patients had prior embolization, stroke 10.2%, coronary 4.8%, peripheral 4.3% 5.4% of hospital death, with a predominance of malignant tumors (40% p < 0.0001). The histological type was a predictor of mortality (rhabdomyomas and sarcomas p = 0.002) and embolic event (sarcoma, lipoma and fibroelastoma p = 0.006), but not recurrence. Tumor size, atrial fibrillation, cavity and valve impairment were not associated with the embolic event. During follow-up (mean 80±63 months), there were 2 deaths (1.1%) and two recurrences 1 and 11 years after the operation, to the same cavity. Conclusion: Most tumors were located in the left side of the heart. The histological type was predictor of death and preoperative embolic event, while the implantation site carries no relation with mortality or to embolic event. .

Fundamento: Os tumores do coração são infrequentes, em sua maioria benignos e com alto potencial embólico. Objetivo: Correlacionar o tipo histológico do tumor cardíaco com seu potencial embólico, com o sítio de implantação e analisar a evolução tardia destes pacientes submetidos à cirurgia. Métodos: No período de dezembro de 1986 a setembro de 2011 foram retrospectivamente analisados 186 pacientes operados (119 do sexo feminino e idade média de 48 ± 20 anos). Foram 145 tumores de átrio esquerdo (77%), 72% dos pacientes assintomáticos e 19,8% com embolização prévia. O diagnóstico foi confirmado por ecocardiograma, ressonância magnética e exame histológico. Resultados: A maioria dos tumores situava-se nas câmaras esquerdas. O mixoma foi o mais frequente (72,6%), seguido dos fibromas (6,9%), trombos (6,4%) e sarcomas (6,4%). Seus tamanhos variaram de 0,6cm a 15 cm (média de 4,6 ± 2,5cm). Houve 37 embolizações prévias à operação (10,2% AVC, 4,8% IAM e 4,3% periférica). Foram 5,4% de óbito hospitalar, com predomínio nos tumores malignos (40% p < 0,0001). O tipo histológico foi preditor de mortalidade (rabdomioma e sarcomas p = 0,002) e de evento embólico (sarcomas, fibroelastoma e lipoma p = 0,006), porém não de recidiva. O tamanho tumoral, a fibrilação atrial, a cavidade e valva acometida não apresentaram relação com o evento embólico. Durante o seguimento (média de 80 ± 63 meses), houve 2 óbitos (1,1%) e duas recidivas tumorais 1 e 11 anos após a operação, ambas para a mesma cavidade. Conclusão: O tipo histológico foi preditor de óbito e de evento embólico pré-operatório, enquanto o sítio de implantação não. .

Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia

Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.

Risk Factors for Death during Pulmonary Tuberculosis Treatment in Korea: A Multicenter Retrospective Cohort Study

The data regarding risk factors for death during tuberculosis (TB) treatment are inconsistent, and few studies examined this issue in Korea. The purpose of this study was to evaluate baseline prognostic factors for death during treatment of adult patients with pulmonary TB in Korea. A multicenter retrospective cohort study of 2,481 patients who received TB treatment at eight hospitals from January 2009 to December 2010 was performed. Successful treatment included cure (1,129, 45.5%) and treatment completion (1,204, 48.5%) in 2,333 patients (94.0%). Unsuccessful treatment included death (85, 3.4%) and treatment failure (63, 2.5%) occurred in 148 patients (6.0%). In multivariate analysis, male sex, anemia, dyspnea, chronic heart disease, malignancy, and intensive care unit (ICU) admission were significant risk factors for death during TB treatment. Therefore, male sex, anemia, dyspnea, chronic heart disease, malignancy, and ICU admission could be baseline prognostic factors for death during treatment of adult patients with pulmonary TB in Korea.

Consenso Intersociedades para el manejo de infecciones respiratorias: bronquitis aguda y enfermedad pulmonar obstructiva crónica / [Inter-society consensus for the management of respiratory infections: acute bronchitis and chronic obstructive pulmonary disease].

The Argentine Society for Infectious Diseases and other national societies issued updated practical guidelines for the management of acute bronchitis (AB) and reactivations of chronic obstructive pulmonary disease (COPD) with the aim of promoting rational use of diagnostic and therapeutic resources. AB is a condition characterized by inflammation of the bronchial airways which affects adults and children without underlying pulmonary disease. It is usually caused by a virus. The diagnosis is based on clinical findings after community acquired pneumonia has been ruled out. Treatment of AB is mainly symptomatic. Antibiotics should be used in immune-compromised hosts, patients with chronic respiratory or cardiac diseases and in the elderly with co-morbidities. Reactivation of COPD is defined as an acute change in the patient’s baseline clinical situation beyond normal day to day variations, with an increase in dyspnea, sputum production and/or sputum purulence, warranting a change in medication. An increase in one symptom is considered a mild exacerbation, two as moderate, and the presence of three symptoms is considered a severe exacerbation. An infectious agent can be isolated in sputum in 50 to 75

of COPD reactivations. Moderate and severe episodes must be treated with antibiotics, amoxicillin/ beta-lactamase inhibitor, macrolides and fluoroquinolones are first choice drugs.