RESUMO
Rationale: Pulmonary ionocytes are a newly discovered airway epithelial cell type proposed to be a major contributor to cystic fibrosis (CF) lung disease based on observations they express the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel at a higher level than any other cell type in the airway epithelia. Moreover, genetically manipulated experimental models that lack ionocytes develop NaCl transport abnormalities and airway surface liquid (ASL) dehydration consistent with CF. However, no direct evidence indicates ionocytes engage in NaCl transport or contribute to ASL formation, questioning the relevance of ionocytes to CF lung disease. Objectives: To determine the ion transport properties of pulmonary ionocytes and club cells in genetically intact healthy and CF airway epithelia. Methods: We measured ion transport at the single-cell level using a self-referencing ion-selective microelectrode technique in primary human bronchial epithelial cell culture. Measurements and Main Results: cAMP-stimulated non-CF ionocytes do not secrete Na+ or Cl- into the ASL, but rather modulate its pH by secreting bicarbonate via CFTR-linked Cl-/bicarbonate exchange. Non-CF club cells secrete Na+ and Cl- to the lumen side after cAMP stimulation. CF ionocytes and club cells do not transport ions in response to cAMP stimulation, but incubation with CFTR modulators elexacaftor/tezacaftor/ivacaftor restores transport properties. Conclusions: We conclude that ionocytes do not contribute to ASL formation but regulate ASL pH. Club cells secrete the bulk of airway fluid. In CF, abnormal ionocyte and club cell function results in acidic and dehydrated ASL, causing reduced antimicrobial properties and mucociliary clearance. This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives License 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/).
RESUMO
Se presenta el caso clínico de un paciente de 30 años de edad que consulta por un cuadro de pujos y tenesmo rectal de dos meses de evolución. Al examen físico se constata una lesión submucosa en recto inferior de 3 cm de diámetro aproximado. Se realiza RMI de alta resolución con difusión sin imágenes de restricción por lo cual se decide tratamiento quirúrgico mediante un abordaje transanal a través del plano interesfintérico. El análisis Inmunohistoquímico de la pieza quirúrgica muestra positividad difusa para Proteína S-100 y Vimentina y negativa para otros marcadores, hallazgos compatibles con Schwannoma rectal.
The clinical case of a 30 year old who consults for pushing and rectal tenesmus of over two months of evolution is presented. On physical examination a small submucosal lesion in the lower rectum is found. RMI is performed with diffusion which show no images restraining which surgical treatment is decided by a transanal approach. Immunohistochemical analysis of the surgical specimen shows diffuse positivity for S-100 protein and vimentin, findings consistent with rectal schwannoma.
Assuntos
Humanos , Masculino , Adulto , Neurilemoma/diagnóstico , Neurilemoma/cirurgia , Neoplasias Retais/cirurgia , Cirurgia Endoscópica Transanal , Diagnóstico por Imagem , Intervalo Livre de Doença , Complicações Pós-Operatórias , Resultado do TratamentoRESUMO
El sistema de evaluación ictiopatológico, diseñado a los fines de poder desarrollar un plan de monitoreo constante de diversos cuerpos de agua de la Provincia de Buenos Aires, comprende la estandarización de la toma de muestras, del relevamiento de las variable ictiométricas, de los datos derivados del examen de autopsia y del análisis histopatológico de las biopsias de los órganos seleccionados por sus características "indicadoras"