RESUMO
Lymphangioleiomyomatosis (LAM) is a rare lung disease of unknown etiology, described since 1918 associated with tuberous sclerosis complex (TSC-LAM) and are reported sporadically (S-LAM). It is classified within the group of interstitial lung diseases (ILD) and currently the European Respiratory Society (ERS) has published guidelines for diagnosis and treatment. The objective is to evaluate the clinical presentation of two patients, evolution, management, and review of current treatment. Both patients admitted in our hospital for examination, presenting with spontaneous pneumothorax. Their CT scan shows characteristic cystic lesions and thoracotomy with lung biopsy was performed because lack of expansion and to confirming the diagnosis. Despite the controversy about the optimal management of these patients, there is evidence about the use of progesterone routinely in patients with rapid deterioration of respiratory function when it was provided for a period of at least 12 months. Due to the rareness of the disease, it requires a patient registry to evaluate the use of experimental drugs or include them in research protocols to improve their prognosis.
Assuntos
Linfangioleiomiomatose/diagnóstico , Adulto , Feminino , HumanosRESUMO
BACKGROUND: Despite the severity of infection with influenza virus, little has been reported about its presence in pregnant women. OBJECTIVE: To communicate our experience on influenza virus H1N1 in pregnant women. METHODS: We conducted a retrospective cohort study on 16 hospitalized patients with diagnosis of pregnancy and probable or confirmed case of infection with H1N1 influenza virus. SUSPECTED CASE: Sudden onset of respiratory infectious process, in 2009, in Mexico City. All cases underwent the fast detection test and subsequently confirmation by PCR test. Discharge criteria: clinical improvement, PaO(2) above 60 mm Hg and absence of bilateral consolidation on chest radiographs. Severity criteria: type I respiratory failure (PaO(2) < 50 mm Hg) who required mechanical ventilatory support, radiographic pattern compatible with multisegmental pneumonia and PaO(2)/FiO(2) < 200 mm Hg. RESULTS: The pregnant patient mortality was 4.25%. The average age was 25.8 years, with 24.13 weeks of gestation with 5.6 days average of hospital stay. The main co-morbidity was obesity. CONCLUSIONS: The fatal cases were those with longer time of evolution and late diagnosis.