Lymphangioleiomyomatosis: clinical course in 32 patients

Article Abstract:

Lymphangioleiomyomatosis (LAM) is a rare lung disease which involves a proliferation of the smooth muscle within the lungs. Because the condition seems to affect only premenopausal women, it is thought to involve estrogens. Since the condition is so uncommon, there have been no controlled studies of therapeutic methods and there is no consensus on the best mode of treatment for these patients. A review of 32 patients seen since 1976 reveals some frequent observations associated with the disease and dispels some common misconceptions about the condition. The diagnosis of LAM was delayed an average of 44 months from the time of the appearance of the first symptoms. This is not surprising, as one of the standard symptoms of the condition is pneumothorax. Spontaneous pneumothorax is much more common than LAM, and only a specialist is likely to recognize LAM early on. The suspicion that the condition is associated with estrogens has prompted the therapeutic removal of the ovaries; 16 patients were so treated in the present series. One patient continued to take estrogen against medical advice. None of the patients improved, four remained stable, and 11 deteriorated. This observation is in contrast with some previously reported cases. Similar observations were made among the nine patients taking the antiestrogen drug tamoxifen. None improved, three remained stable, and six deteriorated. The course of the disease was not altered among patients who underwent menopause. Rapid progression of the disease was observed only in the first years after diagnosis, and many patients lived over two decades of slow progression. Overall, the average survival of the patients in the present series was 8.5 years after diagnosis. At present, 78 percent of the 32 patients remain alive. (Consumer Summary produced by Reliance Medical Information, Inc.)

Muscle diseases, Muscular diseases

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Withdrawing life support: how is the decision made?

Article Abstract:

The decision-making process in withdrawing life support from critically ill patients needs to be better understood in terms of the values and belief systems of health care workers. Although physicians and nurses are assumed to consider patient condition and characteristics when deciding to continue or limit care, a Canadian study found that their personal characteristics may also affect treatment choices. The survey showed a great variability in the level of care physicians and nurses would provide for patients in 12 intensive-care scenarios. In eight of the 12 patient scenarios, over 10% of respondents chose opposite extremes of care, that is, aggressive treatment versus comfort measures only. In only one scenario did over half the respondents agree on treatment choice. Further study is necessary to clarify how health care workers reach treatment decisions and communicate with patients and family.

Editorial, Ethical aspects, Critical care medicine, Life support systems (Critical care), Life support care

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Case 23-2004: a 50-year-old woman with low oxygen saturation

Article Abstract:

A case study of a 50-year old Puerto Rican woman with low oxygen saturation and a history of uterine fibroids and asthma is presented. The key findings in the patient's preoperative evaluation included low SpO(sub 2) values on multiple occasions in the absence of pulmonary or cardiac disease.

Health aspects, Asthma, Aged women, Elderly women, Uterine fibroids

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