Pulmonary lymphangioleiomyomatosis: literature update.

Pulmonary lymphangioleiomyomatosis is an uncommon disease of unknown etiology characterized by the proliferation of abnormal smooth muscle cells in the lungs, leading to parenchymal destruction and progressive respiratory failure. The natural history of this disease remains poorly understood, primarily seen in women of childbearing age. The diagnosis can be difficult because symptoms are nonspecific and very similar to other respiratory diseases like asthma, emphysema and bronchitis. Lymphangioleiomyomatosis may not be diagnosed until a pneumothorax, chylothorax, interstitial lung disease or angiomyolipomas are discovered. The recent advances in genetic and molecular research provide new hope to discover the intricate mechanism of disease and evaluate new therapies. Internists, primary care physicians and pulmonologists should be aware of this condition in order to avoid delay in the diagnosis and institute appropriate therapy. The clinical features, pathophysiology, molecular genetics and medical treatment will be reviewed.

Challenges in critical care medicine: an overview of Puerto Rico's intensive care units.

OBJECTIVE: Puerto Rico (PR) has undergone rapid changes during the last decades. Some of these involve the health care system and the delivery of care to the critically ill patient. With this in mind, we investigated how the intensive care units throughout our island's hospitals are organized so that we could establish a profile of the adult intensive care units (ICU) in PR. METHODS: From January 1, 2010 through April 30, 2010, questionnaires were distributed by e-mail or fax to every hospital that maintained a critical care unit. The questionnaires asked for such details as the structure of the unit; whether is use on an open or closed model; the number of beds in the unit; the total number of faculty members in the unit; the credentials of the unit's medical faculty and nursing staff; whether critical care service was available, and the different people in-charge of the unit during the day and at night. RESULTS: A total of 33 questionnaires were distributed, of which 19 were collected and analyzed. Among the ICU directors who responded, the predominant specialty was cardiology. Surprisingly, only 26% of the hospitals had critical care specialists. In most of the institutions, an internist or a primary care physician was on site during the day, this individual directly supervised patients and had decision making authority. At night, however, patients were managed by supervising nurse with limited ability to medically identified patient complications, though primary care physician was always available by phone if a critical decision needed to be made. Some of the units used protocols as part of their medical-management armamentarium. CONCLUSION: Although only a small percentage of the island's ICUs participated in our project, the study's findings serve as evidence of the need to re-evaluate the delivery of care to the critically ill population.

A hematologic condition expressed as a lung disease.

Pleural involvement secondary to Multiple Myeloma is considered a very rare complication. According to the literature only 1% of these patients develop a myelomatous pleural effusion. We present a case of a 39 year old man with multiple myeloma diagnosed six years prior to our evaluation, which developed progressive dyspnea, dry cough and right pleuritic chest pain two weeks prior to admission. On physical examination the patient had decreased breath sounds over the right posterior hemithorax accompanied by dullness to percussion. The chest radiogram was consistent with a right sided pleural effusion. Pleural fluid analysis revealed the presence of abundant abnormal plasma cells. The patient died four weeks after hospitalization. The presence of myelomatous pleural effusion is considered to be a poor prognostic finding, no matter at what disease stage it develops. So far no definite treatment has been shown to improve survival.

Pleural effusion as the initial extramedullary manifestation of Acute Myeloid Leukemia.

Leukemias rarely debut by pleural involvement as the first manifestation of the hematologic malignancy. This complication is most commonly seen in solid tumors such as carcinomas of the breast, lung, gastrointestinal tract and lymphomas. We present a case of a 66 year old male who presented with a pleural leukemic infiltration of his undiagnosed Acute Myeloid Leukemia that was not a complication of the disease extension, but the acute presentation of the illness. Progressive shortness of breath for two weeks, cough, clear sputum and weight loss were the initial complaints. Serum dyscrasia suggested a hematologic abnormality. A chest x-ray performed demonstrated a buildup of fluid with layering in the left pleural cavity. Diagnostic thoracentesis suggested an exudative etiology with cytology remarkable for 62% leukemic myeloblast. The diagnosis was confirmed by bone marrow biopsy with expression of the antigens CD 34+ and CD13+, with unfavorable cytogenetic prognosis and a trisomy 21 chromosomal defect. Chemotherapy was initiated, though no remission achieved with induction chemotherapy. Complications and disease progression precludes in the patient's death. Although rare, due to the unusual presentation of the disease, this case clearly demonstrates the importance of biochemical analysis and cytopathology specimens obtained in pleural fluid.