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Titolo:
LYMPHANGIOLEIOMYOMATOSIS - THE PATHOPHYSIOLOGY OF DIMINISHED EXERCISECAPACITY
Autore:
CRAUSMAN RS; JENNINGS CA; IRVIN CG; KING TE;
Indirizzi:
NATL JEWISH CTR IMMUNOL & RESP MED,ILD SCOR PROGRAM,DEPT MED,1400 JACKSON ST DENVER CO 80206 NATL JEWISH CTR IMMUNOL & RESP MED,ILD SCOR PROGRAM,DEPT MED DENVER CO 80206 UNIV COLORADO,HLTH SCI CTR,DEPT MED,DIV PULM SCI & CRIT CARE MED DENVER CO 80262
Titolo Testata:
American journal of respiratory and critical care medicine
fascicolo: 4, volume: 153, anno: 1996,
parte:, 1
pagine: 1368 - 1376
SICI:
1073-449X(1996)153:4<1368:L-TPOD>2.0.ZU;2-0
Fonte:
ISI
Lingua:
ENG
Soggetto:
PULMONARY DIFFUSING-CAPACITY; TUBEROUS SCLEROSIS; FORME-FRUSTE; LYMPHANGIOMYOMATOSIS; ANGIOMYOLIPOMA; LESIONS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
35
Recensione:
Indirizzi per estratti:
Citazione:
R.S. Crausman et al., "LYMPHANGIOLEIOMYOMATOSIS - THE PATHOPHYSIOLOGY OF DIMINISHED EXERCISECAPACITY", American journal of respiratory and critical care medicine, 153(4), 1996, pp. 1368-1376

Abstract

Dyspnea with exertion is nearly always present in patients with pulmonary lymphangioleiomyomatosis, but the mechanisms underlying exercise impairment have not been well defined. Spirometry, lung volumes, lung mechanics, and exercise physiology were performed on a cohort of 16 patients. We determined the relative contribution of airflow limitation,gas exchange abnormalities, and pulmonary vascular abnormalities to the exercise performance achieved. The patients had normal TLC and Vtg,but RV was elevated in 88% of the subjects. A moderate to severe obstructive pattern was present in 69% of the subjects, and the DL(CO) wasreduced, often markedly, in 81% of the subjects. Exercise performancewas limited (work load, 68% +/- 6) with abnormalities of ventilatory function and gas exchange present. Strong correlations between overallexercise performance (percent predicted Vo(2)max and maximal work load achieved) and indices of airflow and vascular involvement were present. Poor exercise performance was due primarily to ventilatory limitation. The etiology of this ventilatory limitation appears twofold. First, subjects had a reduced ventilatory ceiling because of airflow limitation. Second, subjects demonstrated an excessive ventilatory responseas a result of increased dead-space ventilation thought to be due to disease-associated cystic changes and associated pulmonary vascular dysfunction or destruction.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 09/07/20 alle ore 01:02:25