Onyx Embolization of a Distal Middle Cerebral Artery Pseudoaneurysm in a Five-Week-Old: A Case Report and Review of Current Treatment Options.

Intracranial pseudoaneurysms secondary to traumatic birth are a rare finding in infants. Definitive diagnosis of such findings is challenging, and no standard management is delineated for management of pseudoaneurysms in the pediatric population. Commonly attempted treatments include endovascular embolization or surgical clipping. A 5-week-old female presented with a two day history of right hand focal seizures. The patient was found to have a dysplastic superficial intra-axial aneurysm arising from the distal left middle cerebral artery (MCA) branch in the setting of a left posterior frontal lobe hemorrhage noted on brain magnetic resonance imaging/magnetic resonance angiography (MRI/MRA). The patient underwent diagnostic cerebral angiogram demonstrating a left distal MCA pseudoaneurysm, which was treated with Onyx embolization. Post-embolization period was complicated by recurrent left central localized seizures and a left hemispheric temporoparietal hemorrhagic infarction. The patient was managed on levetiracetam, phenytoin, phenobarbital with stable seizure control. Herein, we highlight the youngest case to date of a 5-week-old infant with a left distal MCA pseudoaneurysm treated with Onyx embolization. Pseudoaneurysmal incidence, diagnosis and accepted management is discussed.

Augmentation of CFTR maturation by S-nitrosoglutathione reductase.

S-nitrosoglutathione (GSNO) reductase regulates novel endogenous S-nitrosothiol signaling pathways, and mice deficient in GSNO reductase are protected from airways hyperreactivity. S-nitrosothiols are present in the airway, and patients with cystic fibrosis (CF) tend to have low S-nitrosothiol levels that may be attributed to upregulation of GSNO reductase activity. The present study demonstrates that 1) GSNO reductase activity is increased in the cystic fibrosis bronchial epithelial (CFBE41o(-)) cells expressing mutant F508del-cystic fibrosis transmembrane regulator (CFTR) compared with the wild-type CFBE41o(-) cells, 2) GSNO reductase expression level is increased in the primary human bronchial epithelial cells expressing mutant F508del-CFTR compared with the wild-type cells, 3) GSNO reductase colocalizes with cochaperone Hsp70/Hsp90 organizing protein (Hop; Stip1) in human airway epithelial cells, 4) GSNO reductase knockdown with siRNA increases the expression and maturation of CFTR and decreases Stip1 expression in human airway epithelial cells, 5) increased levels of GSNO reductase cause a decrease in maturation of CFTR, and 6) a GSNO reductase inhibitor effectively reverses the effects of GSNO reductase on CFTR maturation. These studies provide a novel approach to define the subcellular location of the interactions between Stip1 and GSNO reductase and the role of S-nitrosothiols in these interactions.