Oral manifestations of
systemic diseases
INDIAN DENTAL ACADEMY
Leader in continuing dental education
www.indiandentalacade...
Mucopolysaccharidiosis
Heterogenous group of metabolic disorders inherited as
autosomal recessive fashion
Characterized by...
Clinical and radiographic features
Vary depending on particular syndrome
Mental retardation often seen in this disorder
Co...
Clinical findings sugesstive of mucopolysaccharidoses but
diagnosis is confirmed by levels of GAGS in urine
Treatment
No s...
Lipid ReticuloEndothelioses
Large group of inherited disorders
Conditions include –Gaucher disease, Nimann- Pick disease
T...
Gaucher Disease: common . Lack of glucocerebrosidase
results in accumulation of glucosylceramide in lysosomes of
macrophag...
Gauchers disease
macrophages are non functional ,& accumulate in bone
marrow
Normal hematopoietic system is disrupted
Anem...
Niemann Pick Disease
Four types
Type A& B caused by deficiency of sphingomeyelinase
Type C&D –mutations of NPC-1 gene in c...
Tay-sachs disease
Wide clinical range as genetically heterogenous.
Survival –adulthood.
Blindness developmental retardatio...
TREATMENT
Gaucher Disease:
Mild expression – no RX
Severe forms – enzyme replacement. But too expensive
Graft-versus-host ...
AMYLOIDOSIS
Heterogenous group of conditions characterized by
deposition of extra cellular proteinaceous substance –
amylo...
Organ limited amyloidosis
Ex.amyloid nodule
Systemic amyloidosis
Several forms
Primary &Myeloma associated: effects old pe...
Hemodyalysis associated : long renal dialysis,
identified B-2 microglobulin which is not removed by
dialysis.
Deposists ar...
Histopathology :
Gingiva-shows extracellular deposition in submucosal
Connective tissue – an amorphous eosinophilic materi...
Diagnosis:
After histopathological diagnosis medical evaluation
for the type of amyloid should be asessed.
TREATMENT
No ef...
Thank you
For more details please visit
www.indiandentalacademy.com

www.indiandentalacademy.com
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Oral manifestations /certified fixed orthodontic courses by Indian dental academy

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The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and offering a wide range of dental certified courses in different formats.

Indian dental academy provides dental crown & Bridge,rotary endodontics,fixed orthodontics,
Dental implants courses.for details pls visit www.indiandentalacademy.com ,or call
0091-9248678078

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Oral manifestations /certified fixed orthodontic courses by Indian dental academy

  1. 1. Oral manifestations of systemic diseases INDIAN DENTAL ACADEMY Leader in continuing dental education www.indiandentalacademy.com www.indiandentalacademy.com
  2. 2. 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 www.indiandentalacademy.com
  3. 3. 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 www.indiandentalacademy.com
  4. 4. 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 Dental considerationswww.indiandentalacademy.com
  5. 5. 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. www.indiandentalacademy.com
  6. 6. 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 www.indiandentalacademy.com
  7. 7. 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 www.indiandentalacademy.com teeth
  8. 8. 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. www.indiandentalacademy.com
  9. 9. 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 www.indiandentalacademy.com
  10. 10. 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. www.indiandentalacademy.com
  11. 11. AMYLOIDOSIS Heterogenous group of conditions characterized by deposition of extra cellular proteinaceous substance – amyloid Amyl-strach; oid-resembling Clinical features Organ limited Systemic forms www.indiandentalacademy.com
  12. 12. 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, www.indiandentalacademy.com
  13. 13. 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 www.indiandentalacademy.com
  14. 14. 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 www.indiandentalacademy.com
  15. 15. 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 www.indiandentalacademy.com
  16. 16. Thank you For more details please visit www.indiandentalacademy.com www.indiandentalacademy.com

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