Bone Abstracts

Since 1997, GH therapy has been approved for skeletal disorders including ACH and HCH patients in Japan. The efficacy of GH therapy for short period has been reported in several study. However, few study about efficacy and safety of GH therapy for long term have been shown. The aim of our study is to examine that GH therapy for HCH patients is efficient and safe enough and improve final height and proportion.18HCH patients (11 males and seven females) wh...

Objectives: Endochondral ossification in the growth plate cartilage (GPC) plays a crucial role in the determination of the length and shape of long bones. Many skeletal dysplasias are caused by GPC dysfunction, associated with short stature. We have already reported that human iPS cell-derived cartilage (hiPSC-Cart), when implanted into the subcutaneous spaces of the SCID mice for 4 weeks, formed skeletal tissue like GPC. This model could also recapitulate the pathology of FGF...

Background: Hypophosphatasia (HPP) is characterized by defective mineralization of bone and/or teeth in the presence of low serum alkaline phosphatase (ALP) activity and caused by mutations in the ALPL gene encoding tissue-nonspecific ALP. Odontohypophosphatasia (odonto HPP) is the mildest form of hypophosphatasia and characterized by dental complications without abnormalities of the skeleton system.Objectives: We aimed to investigate biochemical and gen...