Term
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Definition
• Heterogeneous group of conditions
characterized by deposition of extracellular
material called amyloid
• Amyloid: multiple myeloma, rheumatoid
arthritis or chronic infections including
tuberculosis • Divided into organ‐limited and systemic
forms
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Term
| • ORGAN‐LIMITED AMYLOIDOSIS |
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Definition
– Rarely been reported in oral soft tissues
– Most consist of aggregates of immunoglobulin light chains and not associated systemic alteration -AL = _____ light
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Term
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Definition
• Occurs in several forms:
» primary
» myeloma‐associated
» secondary
» hemodialysis‐associated
» heredofamilial |
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Term
Primary & Myeloma‐Associated
Amyloidosis |
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Definition
• Affect older adults (av. 65)
• Mucocutaneous lesions and macroglossia
from amyloid deposits
• Skin: smooth‐surfaced, firm, waxy papules
and plaques.
• Macroglossia 12‐40%, diffuse or nodular
enlargement of tongue. -related to most common cause of macroglossia
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Term
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Definition
• Characteristically develops as result of
chronic inflammatory process‐ chronic
osteomyelitis, tuberculosis or
sarcoidosis
• Liver, kidney, spleen and adrenal
typically involved
• Biopsy of rectal mucosa, gingiva and
labial salivary gland confirm diagnosis |
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Term
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Definition
• H/E‐ extracellular deposition of amorphous
eosinophilic material within submucosa
• Identify amyloid using Congo red dye and
also crystal violet
• Treatment of infection and reduction of
inflammation results in clinical
improvement |
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Term
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Definition
• Anterior pituitary hypofunction‐ growth
failure (pituitary dwarfism)
• Diminished GH ‐ reduced capacity of tissues to
respond
• Gonadotropin ‐ leads to amenorrhea and
infertility in women/ decreased libido,
impotence, and loss of pubic and axillary hair
in men -doesn't develope mental retardation
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Term
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Definition
• Shorter height/body proportion normal
• Growth history‐consistent failure
• Skull size/mental status near normal
• Max/mand smaller, late teeth eruption
• ( 1‐3yrs for 1st decade & 3‐10yrs for 2nd decade)
• Shedding delay, 3rd molars fail to dev |
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Term
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Definition
• Radioimmunoassay‐ GH below normal
• Replacement therapy
– Human GH, if detected before closure of
epiphyseal plates
– If hypothalamic defect – GH releasing hormone is
used
– If lack GH receptors – no tx |
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Term
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Definition
• Excess production of growth hormone
after/before closure of epiphyseal plates
• Increase in growth hormone due to
pituitary adenoma
• 20% gigants are McCune‐Albright syndrome
cases |
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Term
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Definition
• Headaches and visual disturbances and
other signs of a brain tumor
• HTN,HD,hyperhidrosis,arthritis, p.
neuropathy
• Renewed growth in small bones of hands
and feet and membranous bones of skull
and jaws -has larger jaw sizes
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Term
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Definition
• Gloves or hats become too small
• Coarse facial appearance
• Sleep apnea bc hyperplasua or palatal tissues • Thick lips and enlarged tongue
• Dental: mandibular prognathism due to
>>mandibular growth
• Open‐bite and spacing between teeth |
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Term
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Definition
• Dignosis late‐9yrs, 42yrs
• GH assay‐glucose oral, MRI
• Treatment involves removal of the pituitary
tumor, 1% mortality
• Pharmacotherapy with a somatostatin
analogue may be used when surgery is
contraindicated, pegvisomant inj |
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Term
HYPOTHYROIDISM
(cretinism/myxedema) |
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Definition
• Reduced level of thyroid hormone
• In infancy ‐ cretinism
• Adults ‐ deposition of glycosaminoglycans in
subcutaneous tissue – myxedema (nonpitting edema)
• Primary ‐ thyroid gland abnormal
• Autoimmune ‐ hashimoto’s thyroiditis
• Secondary ‐ pituitary, lack of TSH
• (radiation therapy for brain tumors) |
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Term
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Definition
| Reduced level of thyroid hormone In infancy |
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Term
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Definition
• Reduced level of thyroid hormone • Adults ‐ deposition of glycosaminoglycans in
subcutaneous tissue – myxedema (nonpitting edema) |
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Term
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Definition
Primary HYPOTHYROIDISM‐ thyroid gland abnormal Autoimmune |
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Term
| radiation therapy for brain tumors |
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Definition
Secondary HYPOTHYROIDISM ‐ pituitary, lack of TSH |
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Term
HYPOTHYROIDISM
(cretinism/myxedema) |
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Definition
• Lethargy, dry, coarse, extremeties
• Huskiness, constipation, weakness, fatigue
• Bradycardia, hypothermia, cold skin
• Swollen lips, diffuse tongue enlargement,
failure of eruption but normal tooth formation |
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Term
HYPOTHYROIDISM
(cretinism/myxedema) |
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Definition
• Lab ‐ Free thyroxine, T4 levels
• If low‐ TSH is measured
• Primary‐ TSH is elevated
• Secondary ‐ TSH is borderline/normal
• Levothyroxine – replacement
• Prognosis‐ good for adult/child‐early
• Late diagnosis in children‐ MR |
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Term
HYPERTHYROIDISM
(THYROTOXICOSIS / GRAVE’S DISEASE) |
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Definition
• Elevated prodn‐ thyroid hormone
• Elevated metabolism
• _________ ‐ 60‐90%, auto ‐
antibodies against TSH receptors on
thyroid cell surface
• Hyperplastic thyroid tissue/ tumors
(benign & malignant >> thyroid hormone)
• Pituitary adenoma‐>>TSH>>Thyroid
hormone |
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Term
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Definition
• C/F – 5‐10X, F:M, 2% of adult females
• Grave’s‐3rd/4th decades, diffuse thyroid
enlargement
• Palpit, nervousness, heat intolerance,
emotional lability, muscle weakness
• Wt loss, tachhycardia, perspiration,
• Widened pulse pr, warm, smooth skin, tremor -thyroid storm: elevated temperature
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Term
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Definition
• Ocular involvement
– 20‐40% of patients
– Stare with eyelid retraction/lid lag
– Protrusion of eyes‐exopthalmous/ proptosis
(deposition of glycosaminoglycans in retro‐orbital
connective tissue) |
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Term
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Definition
• Lab – T4 & TSH, T4 elevated and TSH
depressed
• Tx‐Radioactive Iodine, thyroid storm‐20‐
40% mortality |
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Term
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Definition
• Relatively rare caused by << PTH
• Related to parathyroid gland removal during
thyroidectomy
• Autoimmune destruction of the gland
(syndromal)
• PTH+ vit D‐ Ca+ levels, hypocalcemia PTH
stimulates renal Ca+ reabsorption &
osteoclastic resorption |
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Term
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Definition
• Tetany ‐Chvostek’s sign‐ twitching of
upper lip
-latent tetany
• Pitting enam hypo, eruption failure
• Persistent oral candidiasis (endocrine –
candidiasis syndrome |
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Term
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Definition
• Lab‐<<PTH, <<serum Ca+, >>serum
phosphate (normal renal function)
• Tx‐Ergocalciferol‐vit D2 , dietary Ca+ |
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Term
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Definition
• Excess production of parathyroid hormone
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Term
| Primary hyperparathyroidism |
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Definition
uncontrolled production of PTH, usually
due to parathyroid adenoma (80 to 90%) 60+ years |
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Term
| Secondary hyperparathyroidism |
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Definition
• PTH continuously produced, chronic low levels
of serum calcium
• Associated with chronic renal disease.
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Term
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Definition
• Kidney produces vitamin D, necessary for
calcium absorption from gut.
• In chronic renal disease, no vitamin D and
less calcium is absorbed |
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Term
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Definition
• Women two to four times more often
• Classic triad "stones, bones, and
abdominal groans.
• "Stones: marked tendency for renal calculi
due to elevated serum calcium
• Metastatic calcifications are seen involving
soft tissues (pulp stones)
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Term
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Definition
• Bones: variety of osseous changes.
• Generalized loss of lamina dura
surrounding roots of teeth, early sign • Alterations of trabecular pattern develop
next, ground glass appearance.
• With persistent disease, other osseous
lesions develop, such as brown tumor of ________ |
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Term
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Definition
• Bones: variety of osseous changes. • Named for dark reddish‐brown color
(contains hemorrhage and hemosiderin)
• Well‐demarcated unilocular or multilocular
radiolucencies. • Commonly affect mandible, clavicle, ribs,
and pelvis.
• Longstanding lesions produce significant
cortical expansion. |
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Term
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Definition
• Abdominal groans: develop duodenal
ulcers.
• Histopathologic Features
– Identical to central giant cell granuloma of
jaws. |
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Term
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Definition
Treatment and Prognosis
• Primary _____: hyperplastic tissue
or tumor must be surgically removed
• Secondary _____: not managed
aggressively unless patient has symptomatic renal
calculi. • Control pharmacologically: active vitamin D
• Renal transplantation usually restores normal
physiology
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