Oral manifestations /certified fixed orthodontic courses by Indian dental academy

Oral manifestations of
systemic diseases
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Mucopolysaccharidiosis
Heterogenous group of metabolic disorders inherited as
autosomal recessive fashion
Characterized by lack of any one of enzymes –needed to
process intercellular substance.
The glycosaminoglycans –
heparan sulfate
dermatan sulfate
keratan sulfate
chondrotin sulfate
Frequency – 1 in 15000 persons


Clinical and radiographic features
Vary depending on particular syndrome
Mental retardation often seen in this disorder
Coarse with heavy brow ridges , stiff joints, cloudy
degeneration of corneas leading to blindness.
Macroglossia, gingival hyperplasia(anterior region), thin
enamel with pointed cusps (typeIV-A),
Numerous impacted teeth with prominent follicular spaces-due
to accumulation of glycosaminoglycans

Clinical findings sugesstive of mucopolysaccharidoses but
diagnosis is confirmed by levels of GAGS in urine
Treatment
No satisfactory treatment
Reduced life span with mental retardation are over come
to an extent by allogeneic bone grafts.
Enzyme replacement therapy
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Lipid ReticuloEndothelioses
Large group of inherited disorders
Conditions include –Gaucher disease, Nimann- Pick disease
Tay-sachs disease.
Seen more in Jewish heritage.
Lack certain enzymes necessary for processing lipidscausing accumulation of lipids within a variety of cells.
Storage disease as the cells attempting to store substance.


Gaucher Disease: common . Lack of glucocerebrosidase
results in accumulation of glucosylceramide in lysosomes of
macrophages and monocytes.
Nieman Pick Disease: deficiency of acid sphingomyelinaseaccumulation of sphingomyelin in lysosomes
Tay Sachs disease: lack of hexosaminidase causing
accumulation of ganglioside in neurons
All are inherited diseases


Gauchers disease
macrophages are non functional ,& accumulate in bone
marrow
Normal hematopoietic system is disrupted
Anemia Thrombocytopenia occurs
Bone infractions & bone pain. Erlenmeyer Flask deformity of
long bones
Visceral enlargement (spleen,liver)
Growth retardation neurologic deterioration
Jaw lesions –ill defined radiolucencies without devitaliztion of
teeth

Niemann Pick Disease
Four types
Type A& B caused by deficiency of sphingomeyelinase
Type C&D –mutations of NPC-1 gene in cholesterol processing
Type A,C&D –neuronopathic features –Psychomotor retardation
dementia, spasticity & hepatosplenomegaly.
Life span –first or second decades.
Type B- Visceral signs Primary hepatosplenomegaly &
pulmonary invovement.

Tay-sachs disease
Wide clinical range as genetically heterogenous.
Survival –adulthood.
Blindness developmental retardation and intractable seizures
Death occurs in 3-5 years.
HISTOPATHOLOGY
Sheets of lipid engorged macrophages ,exhibiting bluish
cytoplasm which has fine texture resembling wrinkled silk.
Niemann Pick – bone marrow shows sea blue histiocyte

TREATMENT
Gaucher Disease:
Mild expression – no RX
Severe forms – enzyme replacement. But too expensive
Graft-versus-host disease is problem.
Increased risk of hematologic malignanciesand multiple myeloma
Niemann-Pick Disease
Poor prognosis
Genetic counseling for affected families
Molecular Markers are present to identify & intervention which
allows reduction in prevalence of disease.

AMYLOIDOSIS
Heterogenous group of conditions characterized by
deposition of extra cellular proteinaceous substance –
amyloid
Amyl-strach; oid-resembling
Clinical features
Organ limited
Systemic forms


Organ limited amyloidosis
Ex.amyloid nodule
Systemic amyloidosis
Several forms
Primary &Myeloma associated: effects old people,male;
symptoms non specific, macro glossia mucocutaneous
lesions,hepatomegaly,carpel tunnel syndrome.
Secondary : characteristically develops as a result of chronic
inflammatory process –osteomylitis,tuberculosis,or
sarcoidosis,

Hemodyalysis associated : long renal dialysis,
identified B-2 microglobulin which is not removed by
dialysis.
Deposists are seen in bones & joints ,and tongue
involvement is also seen.
Hedero familial : uncommon
Inhereted as autosomal dominant
Recessive-familial mediterranean fever
Polyneuropathies,cardiomyopathy,CHF,renal failure


Histopathology :
Gingiva-shows extracellular deposition in submucosal
Connective tissue – an amorphous eosinophilic material
arranged in perivascular orientation or present diffusely.
Dyes-congo red-appears red
In polarised light-apple green birefriengence
Crystal violet-metachromasia
Staining with thioflavine T-gives a positive amyloid presence
Amyloid-7.5 to 10 nm diameter ,non branching,linear fibrils

Diagnosis:
After histopathological diagnosis medical evaluation
for the type of amyloid should be asessed.
TREATMENT
No effective therapy.
Debulking of tongue ,treatment of infection and
reduction of inflammation has proven clinical
improvement.
Familial mediterrenean is effective with colchicine
therapy, and prednisolone


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