CDA Essentials 2017 • Volume 4 • Issue 7

32 | 2017 | Issue 7 S upporting Y our P ractice awareness and fundraising efforts. “I myself could have been diagnosed earlier had my dentist known of HPP.” Historically, dentists have often been the first to witness the manifestations of the milder forms of HPP, acknowledges Dr. Robert Schroth, associate professor and clinical scientist at the University of Manitoba. “It probably stumps a few dentists as to why a child would be losing teeth early when there are no caries, no abscesses, no trauma history,” he says. “Dentists can be instrumental in the chain of investigation when they encounter such a scenario. They need to learn about the conditions that might lead to premature loss of baby teeth, and to connect that child with his or her family doctor or pediatrician for laboratory testing.” A diagnosis of HPP is established based on the patient’s: • medical and dental history • clinical manifestations, including radiographic assessments • laboratory tests revealing a low serum alkaline phosphatase level and raised levels of phosphorylated substrates (such as pyridoxal-5- phosphate) accumulating in blood or urine Dental management and treatment Some of the oral manifestations of the disease— including the enlarged dental pulp areas, irregular dentin formation, and enamel defects—may increase the risk for tooth decay and cavities, or speed up the carious process. “I strongly encourage the use of dental sealants for both primary and permanent teeth, especially if there are deep grooves and fissures,” says Dr. Schroth, who has been actively involved in caring for children with HPP. He also recommends tailoring each child’s prevention and treatment plans using risk assessment and risk management protocols such as those developed by the American Academy of Pediatric Dentistry. Recommendations regarding nutrition and oral care still apply for children with HPP (e.g., use of fluoride toothpaste, consumption of teeth-friendly snacks, limited ingestion of foods and drinks containing sugar between meals). “It may also important to tell parents that they will likely need to assist their child in their oral hygiene routine until about 8 years of age, as children with HPP might not have the arm strength to brush their teeth properly,” notes Dr. Schroth. It is also important to take into consideration the disease’s various repercussions. “Individuals with HPP often face other challenges such as speech impediment, bullying, and anxiety. They’re not just affected by mechanical issues with their teeth; there are also financial, emotional, social, and quality-of-life ramifications,” emphasizes Ms. Taillefer. It is therefore crucial to discuss these concerns with parents to craft the best restorative plan. “This may mean covering defects with resin fillings or, if the kid is very self-conscious in school, having a fixed dental appliance cemented onto the posterior teeth with archwires and acrylic teeth locked in place to replace the missing anterior teeth,” says Dr. Schroth. HPP treatment A new enzyme-replacement therapy has been developed for patients with deficient or ineffective TNAP, and it is showing encouraging results in bones. “Studies on the dental aspects are still being conducted as we speak,” says Dr. McKee. “We’re hopeful that this new treatment may also alleviate some of the HPP dental manifestations.” This enthusiasm is echoed by Dr. Schroth, who is providing care for some of the study participants using the new drug: “We see that permanent teeth are now developing more, and that there’s stronger enamel formation and good root development. That’s very encouraging!” a To learn more about HPP and how to care for patients with HPP, watch our 4-part series at: cdaadc/hpp Hypophosphatasia (HPP): signs and symptoms Oral and dental manifestations Other manifestations ❘  Soft and irregular dentin ❘  Atraumatic, premature loss of primary teeth with roots on them ❘  Lack of cementum covering the roots ❘  Malformed teeth (often) ❘  Enlarged pulp chambers ❘  Lesser bone height ❘  Bony defects with mobile primary incisors (sometimes) ❘  short stature and bowed legs ❘  delayed developmental milestones (e.g., walking) ❘  chest deformity ❘  hypoplastic lungs and pulmonary insufficiency ❘  chronic muscle or bone pain ❘  joint pain and/or swelling ❘  pseudofractures ❘  neurological manifestations ❘  nephrocalcinosis