Historical role of alpha-1-antitrypsin deficiency in respiratory and hepatic complications.

Alpha-1-antitrypsin (AAT) deficiency is a heritable disease that is commonly associated with complications in the respiratory and hepatic systems. AAT acts as a regulatory enzyme that primarily inhibits neutrophil elastase activity thus protecting tissues from proteolytic damage after inflammation. This paper provides a historical review of the discovery, classification, phenotypic expression, and treatment of AAT deficiency. While its pattern of inheritance has been long understood, the underlying mechanism between AAT deficiency and related diseases remains to be elucidated. Most commonly, AAT deficiency is associated with the development of emphysema in the lungs as well as various liver injuries. Cigarette smoke has been shown to be particularly detrimental in AAT deficient individuals during the development of lung disease. Therefore, understanding familial history may be beneficial when educating patients regarding lifestyle choices. While numerous AAT deficient phenotypes exist in the human populations, only specific variants have been proven to markedly predispose individuals to lung and liver disorders. The exact relationship between AAT levels and the aforementioned diseases is an essential area of further research. It is imperative that clinicians and researchers alike strive to standardize diagnostic criteria and develop safe and effective therapies for this genetic disease.

Rudhe syndrome: reversible right middle lobe emphysema in infants with left-to-right shunts--an historical review.

In 1971, the Swedish radiologist Ulf Rudhe wrote a provocative paper on right middle lobe emphysema in infants with left-to-right shunts in which he suggested cardiac surgery rather than lung resection. At the time, this was counter to accepted medical practice. Earlier diagnosis and better medical management of ventricular septal defect in infants has proved Rudhe correct. However, two current cases of large left-to-right shunts in infants with emphysema of the right middle lobe prompt this historical review of what seemed a closed-episode in pediatric cardiac surgery.

The history of COPD.

The evolution of knowledge concerning COPD and its components--emphysema, chronic bronchitis, and asthmatic bronchitis--covers 200 years. The stethoscope and spirometer became important early tools in diagnosis and assessment. Spirometry remains the most effective means of identification and assessment of the course of COPD and responses to therapy, and is grossly underused for this purpose. Knowledge of the pathogenesis, course and prognosis, and new approaches to therapy have dramatically improved our understanding of this important clinical entity. Smoking cessation improves the early course of disease. Long-term oxygen improves the length and quality of life in selected patients with hypoxemia. Surgery benefits a select few. Today, COPD is a steadily growing global healthcare problem, with increasing morbidity and mortality. Early identification and prevention, and treatment of emerging stages of disease through smoking cessation and a growing number of bronchoactive drugs promises to change the outcome.

Historical perspectives.

The history of surgery for emphysema is filled with numerous surgical attempts to improve the disorder. Although many of these procedures have proved to be futile, lung volume reduction surgery has now arrived. This article outlines the progression of procedures and the logic for which they were thought to be of benefit.

Revisiting "respiratory function in emphysema in relation to prognosis".

BACKGROUND: The 1956 paper by DV Bates, JMS Knott and RV Christie, "Respiratory function in emphysema in relation to prognosis" Quart J Med 1956;97:137-157 is largely reprinted with a commentary by the first author, Dr David Bates. Although the pathology of emphysema was well recognized at the time, the clinical diagnosis and assessment of its severity were known to be imprecise; physiological measurements assessing and following the clinical course had not been established. The study aimed to follow systematically a group of patients, selected by clinical criteria using standardized clinical and physiological techniques, over four years and correlate physiological and clinical changes in relation to prognosis and eventually to postmortem findings. Fifty-nine patients were recruited to an emphysema clinic at St Bartholomew's Hospital, London, England. Inclusion criteria were dyspnea without other causes and no cor pulmonale present. Patients' symptoms were assessed by a standardized questionnaire, and measurements were taken of lung volumes, maximal ventilatory volume, carbon monoxide diffusing capacity at rest, exercise and oxygen saturation by oximetry. During the four years of the study, 17 patients died (actuarial expected - four) and 13 presented with signs of pulmonary heart failure. All postmortem examinations (n=9) showed advanced emphysema. A seasonal variation in dyspnea was established (the period included the infamous 1952 London smog). Four patients improved, and the remainder were unchanged or deteriorated. Close relationships were shown between dyspnea and function results, particularly for the diffusing capacity of lungs for carbon monoxide (DLCO). A comparison among a group of patients with chronic bronchitis without dyspnea showed that the DLCO discriminated between them. A loss of the normal increase in DLCO during exercise was shown in emphysema. IMPORTANCE: The study showed the value of standardized clinical and physiological techniques in following chronic obstructive pulmonary disease patients, and of separating the effects of airflow obstruction from impaired gas exchange function. Impaired gas exchange was shown to be important in influencing prognosis.

History of emphysema surgery.

Ever since the advent of modern chest surgery, surgical pioneers have attempted operations to alleviate, if not cure, patients with emphysema. From the physiologic standpoint illogical procedures such as costochondrectomy, phrenisectomy, or thoracoplasty lead to disastrous results, whereas the effect of operations on the autonomous nervous system was unpredictable. The only worthwhile procedure was bullectomy for localized bullous emphysema. The concept of volume reduction developed by Brantigan in the 1950s and rediscovered by Cooper is the only successful surgical approach to an essentially incurable pulmonary disease.

History of surgery for emphysema.

Throughout the 20th century, several operations have been advocated as methods of treatment for patients with emphysema and, often, they were promoted as offering potential cures. Unfortunately, most of these procedures attempted to treat the wrong physiological or anatomic deficit so that mid- or long-term results were unpredictable or frankly disastrous. Procedures such as costochondrectomy were designed to permit further enlargement of the lungs, whereas thoracoplasty was designed to reduce lung volume. Operations were performed to restore the curvature of the diaphragm or devised to increase blood supply to the lung. Almost every thoracic structure including chest wall, diaphragm, pleura, nerves, airways, lung, or esophagus became "at risk" for surgical intervention. Short of bullectomy for emphysematous bullous lung disease and perhaps volume reduction for diffuse emphysema, none of these procedures has stood the test of time.

Contrastes radiológicos: enfisema con imagen intersticial, fibrosis con imagen de enfisema / Radiological contrasts: Enphysema wiht interstitial imagen, fibrosis with enphysema imagen

La radiografía de tórax es uno de los estudios de gabinete más útiles para diagnóstico de las enfermedades pulmonares. Tanto las enfermedades obstructivas como las restrictivas muestran un patrón radiográfico característico. En este trabajo se reportan dos casos de imágenes radiográficas contrastantes con el reporte histológico. El primero correspondió a una paciente con una placa radiográfica compatible con bronquitis crónica y/o enfisema y cuyo reporte histológico fue de fibrosis en más de 70%. El segundo saso, correspondió a una paciente cuya placa mostró una imagen estersticial difusa y en quien el reporte histológico fue de enfisema. En este trabajo se discuten las posibles explicaciones por las que se pueden dar estos contrastes. Se hace hincapié en el contexto con que la radiografía es interpretada y en la importancia de la semiología, de los antecedentes exposicionales y del análisis de las pruebas de función pulmonar, en particular de la curvapresión-volumen como apoyo para el diagnóstico de certeza.