STAT3 Gain of Function: A New Kid on the Block in Interstitial Lung Diseases

Alexandre Fabre (1, 2) , Sarah Marchal , Lisa Renee Forbes , Tiphanie Phillips Vogel , Vincent Barlogis , Valerie Triolo , Pierre-Simon Rohrlich , Etienne Berard , Diane Frankel (1, 3) , Damien Ambrosetti , Christine Soler , Marie Hoflack , Marie Baque , Emmanuelle Bosdure , Melisande Baravalle , Ania Carsin , Jean-Christophe Dubus (4) , Lisa Giovannini-Chami

1 MMG - Marseille medical genetics - Centre de génétique médicale de Marseille
2 Service de pédiatrie multidisciplinaire [Hôpital de la Timone Enfants - APHM]
3 Laboratoire de Biologie Cellulaire [Hôpital de la Timone - APHM]
4 MEPHI - Microbes évolution phylogénie et infections

A 5-year-old girl with failure to thrive and multiorgan disease was referred to our center for chronic hypoxemia. On evaluation, we noted tachypnea (respiratory rate 35/min), supraclavicular retractions, median diurnal oxygen saturation as measured by pulse oximetry (Sp O 2) = 91.7% at rest, percentage of time below Sp O 2 90% at 26% during sleep, and clubbing. A computed tomography scan showed diffuse interstitial lung disease (Figure 1). Spirometry was normal (TLC, 83% of predicted; FEV 1 , 83% of predicted; FEV 1 /FVC, 98%; and forced expiratory flow, midexpiratory phase, 142% of predicted), but it was not possible to measure DL CO .

Domaines: Maladies infectieuses

Alexandre Fabre, Sarah Marchal, Lisa Renee Forbes, Tiphanie Phillips Vogel, Vincent Barlogis, et al.. STAT3 Gain of Function: A New Kid on the Block in Interstitial Lung Diseases. American Journal of Respiratory and Critical Care Medicine, 2018, 197 (11), pp.E22-E23. ⟨10.1164/rccm.201707-1500IM⟩. ⟨hal-01858930⟩