Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Amicar may affect estimated blood loss (EBL) and blood transfusion in patients with adolescent idiopathic scoliosis (AIS) undergoing posterior spinal fusion (PSF) with pedicle screws and Ponte osteotomies. We performed a retrospective analysis of a prospectively collected, single-center database of 33 patients with main thoracic AIS treated with greater than 80% pedicle screws. Patients were divided into two groups based on whether they received Amicar (Yes), or did not receive any antifibrinolytics and Ponte osteotomies (No). Demographic, radiographic, and intraoperative data were compared between the two groups. Seventeen patients were treated with Amicar (Yes) (10 of whom had Ponte osteotomies) and 16 patients had neither antifibrinolytics nor Ponte osteotomies (No). The two groups had similar preoperative main Cobb angles, major curve flexibility, and gender. Despite longer operating times and a majority of patients receiving Ponte osteotomies, the Amicar group had a significantly lower EBL and homologous blood transfusion rate. Autologous transfusion volume was less in the Amicar group and trended toward significance. There were no differences in mean arterial pressure during surgery. There were no complications in either group. Amicar reduces EBL and homologous transfusion requirements in patients with main thoracic AIS undergoing PSF with pedicle screws and Ponte osteotomies.
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PMID:Does Amicar affect blood loss in patients with adolescent idiopathic scoliosis treated with pedicle screws and Ponte osteotomies? 2397 47

Congenital scoliosis is a lateral curvature of the spine that is due to the presence of vertebral anomalies. Although genetic and environmental factors are involved in the pathogenesis of congenital scoliosis, the specific cause of only a small number of individuals has been identified to date. We identified a de novo missense mutation in the olfactomedin-like 1 (OLFML1) gene by whole-exome sequencing of a patient with congenital scoliosis. Then, we carried out further functional investigation in mice. An assessment of the tissue distribution of Olfml1 revealed it to be prominently expressed in developing skeletal tissues, specifically osteoblasts. Short hairpin RNA-mediated knockdown of Olfml1 in osteoblasts induced the translocation of Yes-associated protein (YAP) transcriptional coactivator from the cytoplasm to the nucleus, which accelerated the Hippo signaling pathway to promote osteoblast mineralization. In contrast, experimentally induced gain of function of Olfml1 retained YAP in the cytoplasm. There appears to exist a novel cell-autonomous mechanism by which osteoblasts avoid excess mineralization through Olfml1. Our results also indicate that mutation of OLFML1 leads to impaired osteoblast differentiation and abnormal development of bone tissue.
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PMID:Olfactomedin-like protein OLFML1 inhibits Hippo signaling and mineralization in osteoblasts. 3026 5