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My name is Jason Bell. I was born in Saint Louis at St. John’s Mercy Medical Center, but only lived here for a year. I grew up in Eastern Pennsylvania, and my family relocated to Austin, TX after high school. I have an undergraduate degree from Texas State University and a masters degree from The University of Nevada, Las Vegas in Kinesiology/Exercise Physiology. From my background in studying human movement and applied physiology, I chose to attend D.O. school at the Arizona College of Osteopathic Medicine, Glendale, AZ. I enjoy using musculoskeletal manipulation or manual medicine when indicated to augment patient care. From 2008 to 2012, I completed my residency and chief resident year at The University of Texas Medical Branch (UTMB) in Galveston, TX. While at UTMB under Dr. Nicola Abate, I was involved in research exploring metabolic and genetic factors that contribute to the development of diabetes after renal transplant.
My hobbies include spending time with my wonderful wife, Huong and our dogs. I also enjoy aerobic exercise, weight lifting, trying worldwide cuisine, reading about topics in Endocrinology and Internal Medicine, and watching college and professional football. We are excited about owning our first home and beginning the next phase of our lives in the Midwest. We are curious to compare the summer heat in St. Louis to past summers spent in Las Vegas, Phoenix, and Galveston.
X-linked hypophosphatemia (XLH) and hypophosphatasia (HPP) are heritable forms of rickets. XLH is treated with calcitriol and inorganic phosphate (Pi) supplementation during childhood to overcome intrinsic vitamin D resistance and the pathogenetic renal Pi wasting. This therapeutic approach aims to control lower extremity bowing and improve short stature. However, the regimen is often stopped partly for safety purposes after puberty when growth plates fuse and adult height is reached. HPP is characterized by alkaline phosphatase deficiency leading to accumulation of inorganic pyrophosphate, an inhibitor of mineralization, which causes to rickets. There is no established medical treatment for HPP. Now, however, experimental bone-targeted alkaline phosphatase-replacement is showing promise for HPP. During the past 29 years, the Research Center at Shriners Hospital in St. Louis has been caring for the largest number of children with XLH and HPP followed at one facility.
Specific Aim #1 will gather information concerning XLH that is crucial to know whether stopping calcitriol and Pi treatment in young adult life is a correct strategy. Also, the prevalence of XLH complications (previously identified for adults who did not receive calcitriol and Pi during childhood) will be assessed. To accomplish these two goals, an outcome study will be performed using former XLH patients of the Research Center who will return for the outpatient studies described below.
Specific Aim #2 will gather important information concerning the outcome and prognosis of pediatric HPP during adult life. Such natural history information is essentially unavailable, yet critical for understanding the objectives for, and documenting any complications from, emerging medical treatments for HPP.
Accordingly, the 131 former XLH patients and 43 former HPP patients of the Research Center will be invited to return over the 3-year grant cycle for a one-day study that will involve an interval medical history, physical examination, blood and urine studies to define mineral and skeletal homeostasis, and select radiographs and bone densitometry using DXA to understand skeletal mass and quality. The findings for these XLH adults will then be contrasted to findings published in 1989 – 1991 for XLH adults of the same age who did not receive calcitriol and Pi treatment. Investigation of the adult HPP patients will use the same techniques to provide their natural history outcome.