RESUMO
CFTR function is required for normal mucociliary clearance (MCC) and cough-assisted clearance (CC). Lumacaftor-ivacaftor is approved for use in people with cystic fibrosis (CF) carrying two copies of F508del-CFTR. In this observational study performed at four study sites, we characterized the effect of lumacaftor-ivacaftor on mucociliary and cough clearance and related this to other clinical and research endpoints after one month of treatment. Twenty-five adolescents and adults were enrolled. No effect on whole lung MCC was observed, but CC was significantly increased. Sweat chloride improved by 18 mEq/L in this group, indicating a modest restoration of CFTR activity, but no demonstrable change in FEV1 or lung clearance index was observed. We speculate that the modest effect of lumacaftor-ivacaftor on CFTR function was insufficient to yield an improvement in MCC.
Assuntos
Aminofenóis/uso terapêutico , Aminopiridinas/uso terapêutico , Benzodioxóis/uso terapêutico , Fibrose Cística/tratamento farmacológico , Depuração Mucociliar/efeitos dos fármacos , Quinolonas/uso terapêutico , Adolescente , Adulto , Criança , Agonistas dos Canais de Cloreto/uso terapêutico , Estudos de Coortes , Regulador de Condutância Transmembrana em Fibrose Cística/uso terapêutico , Combinação de Medicamentos , Feminino , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Adulto JovemAssuntos
Isquemia Miocárdica , Imagem de Perfusão do Miocárdio , Amônia , Circulação Coronária , Humanos , Isquemia , Isquemia Miocárdica/diagnóstico por imagem , Imagem de Perfusão do Miocárdio/métodos , Radioisótopos de Nitrogênio , Músculos Papilares/diagnóstico por imagem , Tomografia por Emissão de Pósitrons/métodos , Tomografia Computadorizada de EmissãoRESUMO
18F-FDG uptake in enlarged lymph nodes is always suspicious, especially in cancer patients. We report a case of hypermetabolic internal mammary lymphadenopathy in a breast cancer patient who was previously treated with bilateral mastectomy and implants. The abnormal nodal uptake turned out to be due to foreign body-induced inflammation, as confirmed histopathologically. Vigilant PET interpretation is required in breast cancer patients who have evidence of breast implant rupture.
Assuntos
Implantes de Mama , Neoplasias da Mama/terapia , Fluordesoxiglucose F18 , Granuloma/diagnóstico por imagem , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Falha de Prótese , Silicones/efeitos adversos , Neoplasias da Mama/complicações , Granuloma/complicações , Granuloma/etiologia , Humanos , Pessoa de Meia-IdadeRESUMO
In cases of nonhereditary osteomalacia associated with hypophosphatemia and inadequate response to vitamin D supplementation, one should consider the possibility of tumor-induced osteomalacia, a paraneoplastic syndrome caused by small mesenchymal tumors often found in obscure locations. We present a case of tumor-induced osteomalacia in which (111)In-pentetreotide scintigraphy aided in accurate localization of the culprit brachial plexus tumor and cure after resection.