ORAL ASPECTS OFTHE METABOLIC DISEASESpptx

It is believed by many that the intraoral production of acids from carbohydrate, arising from incomplete oxidation, is a factor in the production of dental caries, periodontal diseases, oral malodor, bone loss and other associated metabolic diseases .The cluster of metabolic factors include abdominal obesity, high blood pressure, impaired fasting glucose, high triglyceride levels, and low HDL cholesterol levels. Metabolic syndrome greatly raises the risk of developing diabetes, heart disease, stroke, or all three.Metabolic syndrome is a collection of conditions that often occur together and increase your risk of diabetes, stroke and heart disease. The main components of metabolic syndrome include obesity, high blood pressure, high blood triglycerides, low levels of HDL cholesterol and insulin resistance.

1. ORAL ASPECTS OF METABOLIC DISEASES

2. HURLER SYNDROME Type 1 MPS Abnormal in chromosome arm 4p 16.3

3. CLINICAL FEATURES Occurs within first 2 years of life Seen in childhood and adolescence Large head Prominent forehead Broad saddle nose and wide nostrils Puffy eyelids with bushy eyebrows T hick lips Large tongue Open mouth Nasal congestion with noisy breathing Hepatosplenomegaly – protruberant abdomen Claw hand Mentally retarded

4. ORAL MANIFESTATION Short and broad mandible with prominent gonions Spacing of teeth Localized bone destruction in the jaw Small teeth and wide spaced between Gingival hyperplasia Enlarged tongue

5. HISTOLOGIC FEATURES Hurler cells or clear gargoyle -Abnormal deposits with involved fibroblast Seen gingival tissues of affected patients Large with meta chromatically stained cytoplasm Either agranular or finely granular Crescent shaped nucleui

6. TREATMENT No treatment

7. Gaucher disease Common lysosomal storage disease Deficiency of lysosomal hydrolase , glucocerebrosidase

8. CLINICAL FEATURES Three forms: Type I : chronic non –neuropathic form Type II: infantile or acute neuroneopathic form Type III: juvenile or Norrbottnian form( intermediate between type I and type II)

9. TYPE I Present in childhood Hepatospenomegaly Pancytopenia Skeletal disease- Erlenmeyer flask (deformity of distal femur)

10. TYPE II Causes rapidly progressive neurovisceral involvement Result in death of infant

11. TYPE III Present in juvenile patients with systemic involvement

12. All three types are autosomal recessive inheritance Bone involved-bone marrow shows diffuse changes Gaucher cells- numerous large , foamy , slightly granular cells with round pyknotic nuclei . Gaucher cell infiltration in the long bone-common feature

13. RADIOGRAPHICALLY Pseudo cystic/honeycomb radiolucent lesion-premolar region Result in enlargement of bone marrow spaces Widening of lamina dura Displacement of mandibular canal Apical root resorption

14. TREATMENT ERT(Enzyme replacement therapy) Prognosis – very poor

15. NIEMANN –PICK DISEASE Common of genetic disturbances of lipid metabolism. Autosomal recessive trait. Results from lysosomal accu m ulation of sphingomyelin

16. TYPES Type A- Acute neuronopathic form Type B- C hronic non-neuropathic form (less common) Type C - Intracellular cholesterol esterification

17. Clinical features Type A –severe infantile form with extensive neurologic involvement Hepatosplenomegaly Minor dysmorphy and brownish pigmentation of skin Neurological examination- H ypotonia Progressive loss of acquired motors skills R eduction of spontaneous movements

18. Retinal examination – macular cherry-red spot Type B- seen in early childhood Organomegaly –no nervous involvement Bruising Epistaxis Retarded body growth Delayed skeletal age and puberty Joint pain Headache Abdominal pain Diarrhea

19. Type C Shows visceral and neurological manifestation Cerebellar ataxia Dysarthria Dysphagia Dementia Seizure dystonia

20. Histological features Niemann –Pick cells Cells- Foamy cytoplasm

21. Treatment Enzyme replacement therapy Organ transplantation

22. LETTERE-SIWE DISEASE Histiocytic disorder belongs to Langerhans cell disorder Occurs- before 3 years of age

23. CLINICAL FEATURES Skin rash Erythematous with ulcerations Low grade fever with malaise Irritability Splenomegaly Hepatomegaly Lymphedenopathy

24. Oral manifestation Ulcerated lesion-gingival hyperplasia Destruction of maxilla and mandible Premature loss and loosening of teeth

25. LABORATORY FINDINGS Progressive anemia Leukopenia / thrombocytopenia

26. Treatment and prognosis Chemotherapy –vincristine and prednisolone Prognosis-extremely poor