hrp0092rfc3.4 | Multi-system Endocrine Disorders | ESPE2019

Peripheral Glucocorticoid Metabolim May Reflect Resolution of Inflammation in Kawasaki Disease

Sai Shuji , Tamura Takuya , Nagumo Kiyoshi , Chapman Karen

Background: Kawasaki disease (KD) is an acute inflammatory disorder, associated with systemic vasculitis including coronary artery aneurysms (CAA). Treatment with intravenous immunoglobulin (IVIG) can resolve inflammation. However, about 20% patients show resistant to IVIG treatment and some of the cases required additional treatments. Recently, IVIG plus adjuvant glucocorticoid hormones (GC) has been shown to be an effective therapy for these patients, su...

hrp0092p2-30 | Bone, Growth Plate and Mineral Metabolism | ESPE2019

A Rare case of Neonatal Hypocalciuric Hypercalcemia Complicated with Arrhythmia

Arai Yuto , Okumura Ryo , Wada Soichiro , Haseyama Keiji , Sai Shuji

Background: Familial hypocalciuric hypercalcemia is a rare, lifelong, but benign hereditary disorder due to its mild, often asymptomatic phenotype. Here we present a rare case of neonatal hypocalciuric hypercalcemia complicated with arrhythmia.Case: A healthy male infant weighing 2636g was delivered by spontaneous vaginal delivery at term. The pregnancy had progressed normally, but neonatal arrhythmia was found by physic...

hrp0098p1-2 | Adrenals and HPA Axis 1 | ESPE2024

Reciprocal regulation of 11b-HSD1/2 correlates initial glucocorticoids responsiveness in childhood acute lymphoblastic leukemia

Sai Shuji , Hirabayashi Shinsuke , Saito Yusuke , Chapman Karen , Manabe Atsushi

Background: Synthetic glucocorticoid (GC) form crucial first-line treatment for childhood acute lymphoblastic leukemia (ALL). However, some patients reveal GC resistance with unknown mechanism. We have previously shown that reciprocal regulation of the GC metabolizing enzymes, 11beta-hydroxysteroid dehydrogenase types 1 and 2 (11b-HSD1 and 11b-HSD2) is associated with GC sensitivity/resistance in ALL at diagnosis. 11b-HSD1 predominantly regenerates active GC f...