hrp0089p2-p048 | Bone, Growth Plate & Mineral Metabolism P2 | ESPE2018

Infantile Arterial Calcification and Subsequent Hypophosphatemia due to ENPP1 Mutation – A Case Followed through to Adulthood

Nour Munier , Inman Mark , Arnason Terra

Introduction: Infantile Arterial Calcification (IAC) is a rare and frequently lethal condition. Children who survive the infantile period may develop fibroblast growth factor 23 (FGF23) mediated hypophosphatemia and rickets when IAC is due to mutations in the ENPP1 gene.Case: We present a female patient born to a family whose previous child died at birth with extensive vascular calcifications. Akin to the first sibling, our case presented with e...

hrp0089p2-p005 | Adrenals and HPA Axis P2 | ESPE2018

Perioperative Care of CAH – Incongruencies of Practices among Canadian Specialists

Nour Munier , Gill Hardave , Mondal Prosanta , Inman Mark , Urmson Kristine

Introduction: In pediatric years, the most common cause of primary adrenal insufficiency is congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. Current Endocrine Society guidelines advocate for the use of perioperative supraphysiologic (often referred to as ‘stress dose’) glucocorticoids for children with primary adrenal insufficiency undergoing general anesthesia or surgery. We perceived a difference in practice patterns amongst pediatric subspeci...

hrp0082fc7.5 | Growth promoting therapies | ESPE2014

Impact of GH on Adult Bone Quality in Turner Syndrome: a High Resolution Peripheral Quantitative Computed Tomography Study

Nour Munier A , Perry Rebecca J , Stephure David K , Hanley David A , Boyd Steven K

Background: Women with Turner syndrome (TS) are known to be at risk of osteoporosis and fracture. While childhood GH treatment is common in TS, the impact of this therapy on bone health has been poorly understood.Objective: The purpose of this study was to determine the effect of childhood GH-treatment on adult bone quality in TS women using dual X-ray absorptiometry (DXA) and high resolution peripheral quantitative computed tomography (HR-pQCT).<p c...