Home » Search Results for: hypophosphatasia

Search Results for: hypophosphatasia

Hypophosphatasia: Advocating for the Patient

In the last of four segments dedicated to hypophosphatasia (HPP), Debbie Taillefer, President and Founder, Soft Bones Canada, speaks to Dr. Suham Alexander about the importance of creating a community and patient advocacy forum for patients afflicted with soft bone diseases. Highlights Soft Bones Canada was created to fill the void that Debbie and other patients required when they were newly diagnosed with HPP. Soft Bones Canada works to help support and educate patients as well as to bridge the gap with medical professionals. Dentists can play an important role in early diagnosis of the disease since the earliest signs ...

Read More »

Hypophosphatasia: A General Dentist’s Experience

Dr. Suham Alexander speaks to Dr. Bob Schroth in this third segment about hypophosphatasia (HPP) and the dental implications and management of patients with this condition. Children with HPP have some definite dental challenges. The developing teeth in these children lack cementum on the roots which leads to premature exfoliation. Additionally, large pulp chambers and irregular dentin and enamel defects increase the extent and risk of caries development. Radiographic tell-tale signs in HPP include: enlarged pulp horns, lower bone height interproximally as well as bony defects. Prudent use of dental radiographs is recommended in these patients as determined on a ...

Read More »

The Genetic Foundation of Hypophosphatasia

Dr. Cheryl Greenberg, a pediatrician and geneticist, speaks to Dr. Suham Alexander about the clinical history and manifestations of HPP in her unique patient population in Winnipeg, MB. HPP is over-represented in the Mennonite population in Manitoba due to a “founder” mutation. Highlights HPP is categorized by age of onset: perinatal, infant, juvenile, adult (systemic forms) and odonto-hypophosphatasia (dental form). All patients with HPP must have a low level of alkaline phosphatase. There is a strong genetic basis for the various forms of HPP. There are more than 300 mutations described in the literature. However, one gene is responsible for ...

Read More »

What is Hypophosphatasia (HPP)?

Highlights In the first segment of a 4-part series, Dr. Marc McKee introduces Oasis Discussions followers to a soft bone disease which also has a significant dental impact. Hypophosphatasia (HPP) is a very rare disease that has a prevalence of  approximately 1:100,000 live births.  Much progress has been made in recent years in understanding HPP, and in treating patients with this condition. HPP is a disease which negatively affects mineralization due to an increase in inhibitory pyrophosphate levels caused by decreased activity of an enzyme called tissue non-specific alkaline phosphatase (TNAP, TNSALP). This results in the inhibition of mineralization causing ...

Read More »