1 Systemic Diseases with Oral Manifestations All pictures are intellectual property of the Division of Oral and Maxillofacial Pathology or its Fa culty. Duplication or any unauthorize d use is prohibited. Amyloidosis • Heter ogen eous gr oup of con ditio ns • Diffe rent typ es but essen tially two forms • Organ -limi ted (loca lized ) – Senile cerebral: Alzheimer Aβ (pleated configuration) – Thyroid: Medullary carcinoma of the thyroid – Pancreas: Isle ts of L angerhans (DMII) – Heart – Prion disea ses (PrP; rese mblan ce of amyloi d) – Rarely in the mouth; presents as nodule Amyloidosis • Sys temic amyl oid osi s – Famili al – Myelo ma-as socia ted – Hemo dialys is-ass ociat ed (A β2m) Familial Amyloidosis Kidney failure United States, Europe Apolipo protei n AI Hypertension, Kidney failure United States, Europe Fibrinogen Neuropathy, Heart failure, Diarrhea, Kidney failure Most common, worldwide Transthyretin USUAL CLINICAL FEATURES DISTINGUISHING FEATURES TYPE Familial Amyloidosis Kidney failure United States, Russia Apolip oprote in AII Intra-cranial hemorrhage Iceland Cys tat in C Corneal changes (eye), Occasionally heart and kidney disease Finland Gelsolin Kidney failure, Liverfailure Europe, Canada Lysozyme Myeloma-associated • AL type; l igh t cha ins (λ ) • Old er indiv idu als • Fatigu e, weight loss, pare sthes ia, hoarse ness, edema orthostatic hypotension • Macroglossia • Eyeli ds, re troau ricu lar regio n, ne ck, li ps • Petec hiae, ecchy mose s • Xeros tomia a nd xer opht halmia
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• Biopsy, special stains (Congo Red with polarization,crystal violet, thioflavine T), electron microscopy
• Depending on the clinical findings tests may revealmalfunction of organs
Vitamin A (retinol)
• Red and yellow vegetables, liver
• Fat-soluble
• Deficiency – Blindness due to ulceration
– Problems in keratinization
• Hypervitaminosis – Bulging fontanelles (infants), bone pain or swelling, skin and hair
changes and hair loss, skin peeling, angular cheilitis-like, yellowdiscoloration of the skin, higher sensitivity to sunlight, irritability,decreased appetite, vomiting, drowsiness, double vision (youngchildren), gynecomastia (swelling of breast tissue in men),impotence and ejaculation failure