Transcript Hypophosphotemic Vitamin D MH

Hypophosphatemic Vitamin D
Resistant Rickets
Mallory Hornberger:
Date: 10/22/2008
Name of Syndrome:
Patient:
Jordan Hutchings
Age:
11
Gender:
Male
Height:
4’0”
Weight:
65lbs
Vital Signs:
HR: 85B.P.M. Normal
Respiratory rate: 18 R.P.M. Normal
Blood Pressure: 102/60 mmHg
Temperature: 97°F
Chief
complaint
Medical
Alert
Complains of tooth ache in mandibular #22
canine.
None
Hypophosphatemic Vitamin D Resistant
Rickets
Cause of Syndrome - Medical History (if
applicable)
X linked dominant inheritance pattern and is the result of the
mutation of the PHEX gene. The protein is thought to be involved
in bone and dentin mineralization and renal phosphate
absorption.
Calcitriol: Vitamin D Analog; dose:.25 mcg
Metallic taste/ xerostomia
Human Growth Hormone: Hormone; 5 mg; no adverse affects
Age/race/sex predilections and Systemic
Clinical Features:
Age: Can occur in early childhood or have
an adult onset. Most common in children
though. Most people with this will be
short in stature and possibly struggle with
back problems. This syndrome is common
in males and females equally.
Systemic: Patients undergoing extreme
treatment for this would exhibit bone
deformations such as areas of weak
formation and areas of too much.
Commonly during extractions these
patients are at a higher fracture risk.
Clinical: Cracks in teeth or broken teeth.
Loose teeth. Gingival abscesses.
Orofacial Clinical Features
There would be no real facial disfigurements
noted with a patient with this problem.
Radiographic Features of this Syndrome
Many periapical abscesses are common, possible
cracks in dentin or enamel, jaw bone may also be
weak. Root dysplasia and enlarged pulp champers and
pulp horns have also been noted.
Special considerations in Treatment of this patient? How is it Diagnosed?
How common is it? Is it a horse or zebra?
The patient would need to have a pano and periapical radiographs fairly often to check for bone
health and if periapical abscesses are developing in more areas. This would also check to see if
there are cracks in dentin and if they are spreading through the dentinoenamel junction. These
cracks can induce fractures of the enamel and have exposures to the pulp causing infection. The
patient can also show gingival abscesses as well that can be viewed clinically. During extraoral
examination lymphadenitis, swelling of the glands, can be found often. These features are very
common in people with vitamin d resistant rickets and should be watched closely. Treatment as far
as medication is concerned is usually ineffective with vitamin D treatment. Calcium bone building
medications and phosphorous pills are often seen on a patients medical history. Some children
may be on the human growth hormone as well. Many of the above features are horses. They
should be continually watched for good reason that this horse could become a zebra.
Intraoral Findings
List Sources and References (This should be more than your text book!)
Textbook!
Google Images
http://www.wheelessonline.com/ortho/hypercalcemia