Severe pulmonary hypertension due to combined pulmonary fibrosis and emphysema: another cause of death among smokers

In 2005, the combined pulmonary fibrosis and emphysema (CPFE) was first defined as a distinct entity, which comprised centrilobular or paraseptal emphysema in the upper pulmonary lobes, and fibrosis in the lower lobes accompanied by reduced diffused capacity of the lungs for carbon monoxide (DLCO). Recently, the fibrosis associated with the connective tissue disease was also included in the diagnosis of CPFE, although the exposure to tobacco, coal, welding, agrochemical compounds, and tire manufacturing are the most frequent causative agents. This entity characteristically presents reduced DLCO with preserved lung volumes and severe pulmonary hypertension, which is not observed in emphysema and fibrosis alone. We present the case of a 63-year-old woman with a history of heavy tobacco smoking abuse, who developed progressive dyspnea, severe pulmonary hypertension, and cor pulmonale over a 2-year period. She attended the emergency facility several times complaining of worsening dyspnea that was treated as decompensate chronic obstructive pulmonary disease (COPD). The imaging examination showed paraseptal emphysema in the upper pulmonary lobes and fibrosis in the middle and lower lobes. The echo Doppler cardiogram revealed the dilation of the right cardiac chambers and pulmonary hypertension, which was confirmed by pulmonary trunk artery pressure measurement by catheterization. During this period, she was progressively restricted to the minimal activities of daily life and dependent on caregivers. She was brought to the hospital neurologically obtunded, presenting anasarca, and respiratory failure, which led her to death. The autopsy showed signs of pulmonary hypertension and findings of fibrosis and emphysema in the histological examination of the lungs. The authors highlight the importance of the recognition of this entity in case of COPD associated with severe pulmonary hypertension of unknown cause.

Academic autopsies in Brazil - a national survey / Autópsias acadêmicas no Brasil - um inquérito nacional

Objective: To investigate the number and rate of academic autopsies, general organization, educational and research in Brazilian academic services. Methods: Standardized questionnaires were sent to Brazilian medical schools (n=177) and active pathology residency programs (n=53) from March to June 2009. Data were collected for years 2003 to 2008. Results: Thirty-two academic services in 11 Brazilian states answered the survey. Twenty-one (65.6%) perform less than a hundred autopsies for natural causes and less than fifty pediatric or fetal autopsies/year. Twenty-four (75%) perform less than a hundred adult autopsies/year. Many institutions (46.9%) reported a drop in the number of autopsies in a six-year period. The total autopsy count and autopsy rate in 2008 ranged 1-632 (median = 80), and 0-66% (mean = 10.6%), respectively. A steady decrease in the total count of autopsies in a pool of 19 institutions was observed (p<0.01). Median autopsy rates have fallen from 19.3%, in 2003, to 10.6%, in 2008 (p=0.07). Significant discrepancies at autopsies led to changes in institutional healthcare practice in 37.5% of the services. The low number of autopsies was a limiting factor in undergraduate education for 25% of respondents. A minimum number of autopsies is required to complete the pathology residency program in 34.6% of the services. Conclusion: The total number and the rate of academic autopsies have decreased in Brazil between 2003 and 2008. The number of autopsies and the general organization of academic services must be enhanced to improve medical education, research, and the quality control of patient care. .

Objetivo: Investigar o número e a taxa de autópsias acadêmicas, organização geral, ensino e pesquisa em serviços acadêmicos brasileiros. Métodos: Questionários padronizados enviados para escolas médicas brasileiras (n=177) e programas de residência em patologia ativos (n=53) de março a junho de 2009. Dados coletados referentes ao período de 2003 a 2008. Resultados: Trinta e dois serviços em 11 estados responderam à pesquisa. Vinte e um (65,6%) realizam menos de cem autópsias de causas naturais e menos de cinquenta autópsias fetais ou pediátricas/ano. Vinte e quatro (75%) realizam menos de cem autópsias de adultos/ano. Muitas instituições (46,9%) relataram queda no número de autópsias em seis anos. A contagem total e a taxa de autópsias em 2008 variaram, respectivamente, de 1 a 632 (mediana=80) e de 0 a 66% (média=10,6%). Foi observada uma redução contínua no total de autópsias em um grupo de 19 instituições (p<0,01). A mediana da taxa de autópsias caiu de 19,3%, em 2003, para 10,6%, em 2008 (p=0,07). Discrepâncias significativas observadas em autópsias levaram a mudanças na prática institucional de saúde em 37,5% dos serviços. O baixo número de autópsias foi limitante no ensino de graduação para 25% dos entrevistados. Um número mínimo de autópsias é necessário para completar o programa de residência em patologia em 34,6% dos serviços. Conclusão: O número total e a taxa de autópsias acadêmicas diminuíram no Brasil entre 2003 e 2008. O número de autópsias e organização geral dos serviços acadêmicos deve ser melhorado para fortalecer a educação médica, pesquisa e controle de qualidade prestado ao paciente. .