Linfangioleiomiomatosis pulmonar. Descripción y evolución de dos casos / Pulmonary lymphagioleiomyomatosis: description and evaluation of two cases

La linfangioleiomiomatosis pulmonar (LAM) es una enfermedad rara, de causa desconocida, crónica y grave, que de manera casi exclusiva afecta a mujeres jóvenes. Produce un cuadro clínico típico y un patrón radiológico característico. El diagnóstico requiere la confirmación histológica, que muestra una proliferación irregular de células de músculo liso en los pulmones. El tratamiento no suele ser efectivo, produciéndose un deterioro rápido de la función pulmonar, que produce insuficiencia respiratoria grave, a menudo fatal. Presentamos dos casos de LAM en mujeres de 33 y 38 años. En el primer caso la enferma tenía todas las manifestaciones pulmonares descritas en la literatura y en el segundo existían además angiomiolipomas renales bilaterales. Se discuten las características clínico-evolutivas, radiológicas e histológicas, así como el tratamiento instaurado en ambos casos, realizándose una revisión de la bibliografía. Se concluye que la LAM debe sospecharse en mujeres jóvenes que presenten disnea, tos y/o hemoptisis y un patrón radiológico intersticial, en especial si se asocia un derrame pleural o un neumotórax (AU)

[Pulmonary lymphangioleiomyomatosis. Description and course of 2 cases]. / Linfangioleiomiomatosis pulmonar. Descripción y evolución de dos casos.

Pulmonary lymphangioleiomyomatosis (LAM) is a rare, serious, chronic disease whose etiology is unknown and which affects young women almost exclusively. It produces typical clinical and radiological characteristics. Diagnosis is confirmed on finding histological evidence of irregular proliferation of the smooth muscle cells of the lungs. Treatment is not usually effective and there is rapid worsening of the pulmonary function leading to serious respiratory failure which often results in the patients death. We present two cases of LAM in women, one of whom was 33 and the other 38. In the first case the patient had all the pulmonary findings described in the literature, while the second also had bilateral renal angiomyolipomas. We discuss the histological, radiological and clinical characteristics and evolution of both cases, as well as the treatment given. We also review the literature. It is concluded that LAM should be suspected in young women who have dyspnea, cough and/or hemoptysis and an interstitial radiological pattern, especially when associated with a pleural effusion or pneumothorax.

[Tuberculosis outbreak at a public school]. / Brote epidémico de tuberculosis en un colegio público.

BACKGROUND: At school there are special circumstances of living together and a particular susceptibility, which favour the emergence of tuberculosis microepidemics. We report here the microepidemic occurred at a school among 9-year old children. METHODS: After ruling out a possible familiar source in a child with pulmonary tuberculosis, we detected a case with high bacillar shedding in a female teacher and conducted a tuberculin search among children and teachers, initially outlining the theoretical groups at risk. Tuberculin positive children underwent chest-X-ray and when abnormalities were found, children were derived to the pediatrician for chemotherapy. All converters received secondary chemoprophylaxis and all non-respondents primary chemoprophylaxis. RESULTS: The classroom where the teacher spent most of het time had a higher rate of converters (70%) than other classroom, where the index teacher spent only a partial time (40%; RR: 1.75; CI: 1.06-2.88) or the collective of teachers (45.4%; RR: 1.45; CI: 0.94-2.23). Three additional cases of secondary disease were detected, all of them children. The initial compliance with chemoprophylaxis was greater among (for) children (97.0%) than among teachers (41.6%). Among children there was one case of tuberculin conversion compared with three cases among teachers. No additional cases were detected; also, an abnormal rate of reactors outside the initially studied groups was also not detected. CONCLUSIONS: Our results somehow agree with those reported from other school outbreaks. To note the anergy and lack of symptoms in the index case and the suggestion to delineate the degree of spending hours together to identify groups with a higher theoretical risk of being infected. Thus, an unnecessary expense of resources and a social alarm would be avoided.

[Renopleural fistula secondary to xanthogranulomatous pyelonephritis]. / Fístula renopleural secundaria a pielonefritis xantogranulomatosa.

A case is presented of renopleural fistula secondary to xantogranulomatous pyelonephritis in an 18-year-old female patient. Although the renopleural communication was not observed radiologically, concomitant clinical signs, bacteriological isolation of E. coli in renal and pleural content and surgical findings, confirmed the existence of such fistula.