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J - Fixed Prosthodontics
J - Fixed Prosthodontics
FIXED PROSTHODONTIC
REHABILITATION IN A
WEAR PATIENT WITH
FABRY`S DISEASE
Avinash S. Bidra
Journal of prosthodontics: 2011,S2-S8.
DR. P.AKANSHA
M.D.S. 1ST YEAR
DEPARTMENT OF PROSTHODONTIC
CONTENT
Introduction
Clinical
report
Discussion
Reference
INTRODUCTION
Fabry`s disease is a rare X-linked recessive,
lysosomal storage metabolic disorder described by
FABRY AND ANDERSON in 1898.
A mutation in the gene that controls the
Galactosidase A enzyme causes insufficient
breakdown of lipids that builts up harmful levels of
glycosphingolipids in the kidneys, eyes ,autonomus
nervous system, cardiovascular system.
The incidence has been estimated at 1: 40,000 to
1:117,000 people worldwide, but absolute numbers
are unavailable.
Systemic manifestation-
Cardiovasular disease with susceptible to stroke at
young age,
Renal dysfunction,
Corneal dystrophy,
Cutaneous angiokeratomas,
1. Perioral angiokeratomas,
5. Bimaxillary prognathism,
6. Xerostomia.
Management –
Patients with fabry`s disease requires special
considerations as they already medically
compromised one.
Prosthodontic considerations includes the
Management of diastema for esthetic purpose and
optimal occlusal scheme,
Management of bilateraly prognathism and
accompanying “gummy smile”.
Several reports described fixed prosthodontic
management of patients with generalized wear of
dentition.
The purpose of the report is to describe the
prosthodontic management of an American College
of Prosthodontists Prosthodontic diagnostic
index(ACP PDI) class IV dentate patient with
fabry`s disease.
Criterias-
Severely compromised tooth condition
• Insufficient tooth structure to retain or support
intracoronal or extracoronal restorations—in three
or more sextants.
• Pathology that affects the coronal morphology of
four or more teeth in all sextants.
• Teeth require localized adjunctive therapy, i.e.,
periodontal, endodontic or orthodontic procedure
for teeth in three or more sextants.
Severely compromised occlusal scheme
• Occlusal scheme requires major therapy to
reestablish the entire occlusal scheme including
any changes in the occlusal vertical dimension.
Other Class IV characteristics
• Severe manifestations of local or systemic disease,
including the sequelae from oncologic treatment.
• Maxillomandibular dyskensia and/or ataxia
• A refractory patient—a patient who presents with
chronic complaints following appropriate treatment.
CLINICAL REPORT
Patient History -
Medication –
Immunosuppressants
Antihypertensives
Gingiva –
minimal inflammation
maximum probing
depth of 3 mm.
Xerostomia .
2) Hard tisssue –---
Diastema in maxillary and mandibular anterior
region of tooth.
Generalized sever attrition -occlusal surfaces of
posteriors .
Generalized sever erosion on :