Reversible airflow obstruction, proliferation of abnormal smooth muscle cells, and impairment of gas exchange as predictors of outcome in lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a rare disease, occurring in women, characterized by cystic degeneration of the lungs, abdominal tumors, and proliferation of abnormal smooth muscle cells. Lung function abnormalities consist of impairment of the diffusion capacity (DL(CO)) and airflow obstruction. The objective of this study was to correlate the functional impairment with histologic measures of disease severity to identify predictors of disease outcome. Lung function of 143 patients and lung biopsies of 74 of these patients were reviewed for evidence of airway disease and scoring of disease severity. A positive response to bronchodilators was associated with more severe airflow obstruction, a predominantly solid pattern of LAM lesions in the lung biopsy, and greater rate of decline in expiratory flow. Airway inflammation, present in 61% of the lung specimens, was not associated with reversible airway obstruction and did not correlate with the severity of airflow obstruction. DL(CO) correlated best with the LAM histologic score (LHS), a demonstrated predictor of outcome. We conclude that reversible airway obstruction is found in LAM patients with accelerated loss of lung function and a predominantly solid pattern of LAM lesions. Impairment of DL(CO) correlates with LHS, a predictor of survival and time to lung transplantation.

National trends in the management of cardiovascular disease risk factors in children: second NHLBI survey of primary care physicians.

BACKGROUND: The evidence that atherosclerosis begins during adolescence has led to the belief that primary prevention of cardiovascular disease (CHD) should commence in childhood. Although several national guidelines have been issued for the detection and treatment of CHD risk factors in children, concerns continue to be expressed regarding what constitutes appropriate measures and when to institute such measures in children. A 1988 national survey of primary care physicians revealed variation in the management of CHD risk factors in children by physician categories, which suggested the underlying quandary among physicians regarding CHD risk factors in children. OBJECTIVE: To assess current clinical management of pediatric CHD risk factors in the primary care setting and also to evaluate time trends between the current and 1988 surveys. METHODS: A 25-minute telephone survey was conducted with 1036 of eligible physicians (ie, >20 hours per week direct patient care including at least five pediatric patient contacts) selected from a national probability sample in three practice categories (family practitioners, pediatricians, and general practitioners). The questionnaire assessed the current practice of these physicians in the primary care setting regarding cholesterol and blood pressure (BP) screening and treatment, both nonpharmacologic and pharmacologic, and physician attitude and knowledge. RESULTS: Cholesterol screening in children was performed by 75.7% of all physicians. Nonscreening was highest among general practitioners (38%) and lowest among pediatricians (12%). BP was measured by almost all physicians. The majority of physicians (71%) prescribed diet as the first cholesterol-lowering step, but approximately 16% also used pharmacologic therapy. Cholesterol synthesis inhibitors and bile acid sequestrants were the drugs used most commonly. Approximately 25% of physicians have used drugs in children to treat high BP. Diuretics and beta-blockers were used most frequently. More than one fourth of the physicians had some concern about identifying and treating children for CHD risk factors. There was slightly less cholesterol measurement in 1995 compared with 1988, and no notable increase in the knowledge regarding major CHD risk factors. CONCLUSION: The results of the second National Heart, Lung, and Blood Institute survey of primary care physicians suggest that additional inroads need to be made in the dissemination of the national guidelines for the management of CHD risk factors in children, including appropriate use of pharmacologic agents.