Retrospective study of children with hypophosphatasia with reference to dental changes 2/5/10

Department of Pediatric Dentistry
Resident’s Name: Murphy Program: Lutheran Medical Center - Providence
Article title: Retrospective study of children with hypophosphatasia with reference to dental changes
Author(s): Lundgren, Ted, Otto Westphal, Per Bolme, Thomas Moderer, et al
Journal:
Year. Volume (number). Page #’s: Jan 1991. 357-364
Major topic: Hypophosphatasia
Minor topic(s): Dental implications of HP
Main Purpose: Discuss the effects HP had on 17 children
Overview of method of research: Eval. Of 17 children
Findings: Hypophosphatasia(HP) is lack of the bone/liver/kidney isoenzyme alkaline phosphatase(AP). There are infantile, childhood, and adult forms of the disease. There is a hereditary component. Clinical expressions range from
1. intrauterine death
2.severe skeletal hypomineralization/numerous fractures
3. Leg symptoms such as hip, knee, ankle problems, trouble walking, valgus(the outward angulation of the distal segment of a bone or joint) feet o r knees, limited motion, limping, and no babinski sign
4. Premature loss of one or more primary incisors with no hx of trauma
5. Insufficient weight gain and GI issues
6. Changes in skull sutures and fontanelle
7. Eye anomalies in the retina and sclera
8. Marginal bone loss in anterior mandible region
9. Enlarged pulp cavities
10.Phenylethylamine (PEA) in the urine
Due to its early oral manifestations, dentists are usually the first ones to suspect and/or diagnose the disorder. A typical scenario is a child presents after losing his 4 mand. incisors with no history of trauma. The most common finding on dental radiographs is marginal bone loss in the mandibular anterior region, along with cementum aplasia.
Key points/Summary: Measuring the PEA in the urine is the best test for HP. An exam of the legs and radiographic exam of the long bones is essential. A Dental exam with special attention to premature loss of primary teeth and assessment of anterior mandibular bone an be an early predictor.
Assessment of Article: Good article. Quick and dirty review of HP and it’s oral manifestations

http://upload.wikimedia.org/wikipedia/commons/0/0a/Valgus.jpg