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56 Cards in this Set
- Front
- Back
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Define "AMYLOIDOSIS":
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a heterogenous group of conditions characterized by the deposition of an extra-cellular proteinaceous material called amyloid
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What is common amongst all the different amyloid proteins?
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unique protein folding patterns known as "B-pleated sheet"
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What are the two main classifications of AMYLOIDOSIS?
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- Organ-limited amyloidosis
- systemic |
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What are the four types of SYSTEMIC AMYLOIDOSIS:
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- Primary and myeloma associated amyloidosis
- secondary amyloidosis - hemodialysis-associated amyloidosis - heredofamilial amyloidosis |
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What are some of the first indications of PRIMARY AMYLOIDOSIS?
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- fatigue
- weight loss - paresthesia - hoarseness - edema - orthostatic hypotension |
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What is a common (10-40%) in patients with primary amyloidosis?
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MACROGLOSSIA
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SECONDARY AMYLOIDOSIS develops as a result of what?
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chronic inflammatory process such as:
- long standing osteomyelitis - tuberculosis - sarcoidosis |
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How is a diagnosis of AMYLOIDOSIS made?
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- biopsy of rectal mucosa
- alternative tissue sources are the gingiva or labial salivary gland |
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Staining for AMYLOIDOSIS is done with what? How does it appear under different lighting?
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- Congo Red stain
- red - when viewing under polarized light it shows an apple-green birefringence |
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What is the treatment for AMYLOIDOSIS?
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- no effective therapy available
- prognosis is poor |
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Most patients with AMYLOIDOSIS die from what conditions within months to a few years after the diagnosis?
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- cardiac failure
- arrhythmia - renal disease |
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Define CROHN'S DISEASE (REGIONAL ENTERITIS):
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Inflammatory, probably an immunologically mediated condition of UNKNOWN CAUSE that primariy affects the distal portion of the small bowel and proximal colon.
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What age group are patients when CROHNS DISEASE first becomes evident?
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teenagers
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Why are oral lesions significant in CROHN'S DISEASE?
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they may precede the gastrointestinal lesions as many as 30% of cases
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What are the oral lesions of CROHN's DISEASE?
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- diffuse or nodular swelling of the oral and perioral tissues
- cobblestone appearance of the mucosa - ulcers: deep granulomatous-appearing ulcers, often linear ulcers on the buccal vestibule and apthous like ulcerations - soft tissue swelling resembling denture-related fibrous hyperplasia (dentate individuals, smaller mucosal tags) - patchy erythematous macules and plaques involving attached and unattached gingivae (termed mucogingivitis) |
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What is the treatment for CROHN's DISEASE?
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- Initially treated with sulfa-type drug. Corticosteroids used in more severe cases.
- Surgery may be required in some cases - oral lesions usually clear with treatment of the GI process |
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HYPOPHOSPHATASIA represents a deficiency in what enzyme?
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alkaline phosphatase
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What is the significance of alkaline phosphatase?
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- it participates in the process of calcification of bone and cementum
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The majority of HYPOPHOSPHATASIA cases are inherited as?
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autosomal RECESSIVE
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What are the common factors in all the types of HYPOPHOSPHATASIA?
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- reduced levels of alkaline phosphatase
- increased levels of blood and urinary phosphoethanolamine - bone abnormalities that resemble rickets |
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What are the four types of HYPOPHOSPHATASIA?
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- Perinatal
- Infantile - Childhood - Adult |
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Which is the most severe form of HYPOPHOSPHATASIA?
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- perinatal - infant rarely survives
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What is the most consistent features in CHILDHOOD HYPOPHOSPHATASIA:
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- premature loss of the primary teeth without evidence of a significant inflammator response
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How is the diagnosis of HYPOPHOSPHATASIA made?
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- clinical manifestations
- decreased levels of serum alkaline phosphatase - increased levels of phosphoethanolamine in both urine and blood |
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What is some histopathology of HYPOPHOSPHATASIA?
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- exfoliated teeth show absence or marked reduction in amounts of cementum covering root surface
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What is the treatment of HYPOPHOSPHATASIA?
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- symptomatic treatment
- genetic counselling shold be done. Cannot be corrected. |
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ACROMEGALY is caused by what?
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EXCESS production of growth hormone from the pituitary gland
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The increase in growth hormone in ACROMEGALY is usually due to what?
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functional pituitary adenoma
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How does GIGANTISM result?
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excess production of growth hormone that arises BEFORE THE EPIPHYSEAL PLATES CLOSE
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Patients with ACROMEGALY usually have signs similar to what?
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- brain tumour (headaches, visual disturbances)
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What are the oral findings of ACROMEGALY?
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- hypertrophy of soft palate (cause or accentuate sleep apnea)
- diffuse macroglossia - mandibular prognathism (Class III) - apertognathia (ant. open bite) - diastema formation |
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How is ACROMEGALY treated?
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removal of the pituitary tumour (by transsphenoidal approach)
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Define CRETINISM:
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When insufficient production of thyroid hormone occurs during infancy
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Define MYXEDEMA:
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If an adult has markedly decreased thyroid hormone levels (HYPO) for a prolonged period then deposition of GLYCOSAMINOGLYCAN ground substance is seen in the subcutaenous tissue, producing a "non-pitting edema"
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What are the two types of HYPOTHYROIDISM?
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PRIMARY: thyroid gland itself is abnormal, usually hypoplasia or agenesis
SECONDARY: pituitary gland does not produce enough TSH, often after radiation therapy for brain tumours |
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What are two oral findings of HYPOTHYROIDISM?
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- thick lips
- macroglossia - if during childhood TEETH MAY FAIL TO ERUPT |
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How is a diagnosis of HYPOTHYROIDISM made?
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- assaying free thyroxine (T4)
- if those levels low then TSH levels measured to determine whether primary or secondary disease |
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What is the treatment for HYPOTHYROIDISM?
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- thyroid replacement therapy
- Good prognosis for adults |
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Define HYPERPARATHYROIDISM?
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Hypersecretion of PARATHYROID HORMONE (responds to decrease in serum calcium levels)
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How does SECONDARY HYPERPARATHYROIDISM develop?
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- in response to chronic low levels of serum calcium
- most cases this is a reflection of chronic renal disease |
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How does PRIMARY HYPERPARATHROIDISM result?
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- Parathyroid adenoma
- parathyroid hyperplasia - rarely carcinoma - Unfrequently by MEN type 1 or 2a |
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What is the CLASSIC TRIAD of signs and symptoms of HYPERPARATHYROIDISM?
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STONES: tendency for nephrolithiasis (metastatic calcifications)
BONES: generalized loss of lamina dura, alterations in trabecular pattern, brown tumours ABDOMINAL GROANS: tendency for development of duodenal ulcers |
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What is the treatment of HYPERPARATHYROIDISM?
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PRIMARY: surgery
SECONDARY: management of kidney disease |
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Define ADDISON'S DISEASE:
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- Adrenal gland insufficient production of adrenal corticosteroid hormones caused by destruction of adrenal cortex
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What are the causes of PRIMARY HYPOADRENOCORTICISM:
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- autoimmune destruction
- infections (TB, AIDS, deep fungal) - rarely metastatic tumours, amyloidosis, sarcoidosis |
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What are the causes of SECONDARY HYPOADRENOCORTICISM?
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- decreased production of ACTH
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Symptoms of HYPOADRENOCORTICISM appear when how much of the gland is destroyed?
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approx. 90%
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What are some clinical features of HYPOADRENOCORTICISM?
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- skin bronzing
- weight loss - peculiar craving for salt - anorexia - nausea - |
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What are ORAL FINDINGS of HYPOADRENOCORTICISM?
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- diffuse or patchy brown macular pigmentation (final manisfestation of disease)
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How is a diagnosis of HYPOADRENOCORTICISM confirmed?
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- rapid ACTH stimulation test to examine response of adrenal gland to exogenously administered doses of ACTH
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What is the difference in plasma levels of ACTH in PRIMARY vs SECONDARY HYPOADRENOCORTICISM?
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PRIMARY: Plasma ACTH is high
SECONDARY: Plasma ACTH is low (reduced production by pituitary) |
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What are the two types of DIABETES MELLITUS?
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TYPE 1: insulin-dependent DM
TYPE 2: non-insulin dependent DM |
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What are the different symptoms for each type of DIABETES MELLITUS?
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TYPE 1 - weight loss, polyphagia, polyuria, thirst, polydypsia
TYPE 2 - symptoms more subtle |
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ORAL MANIFESTATIONS of DIABETES MELLITUS are generally limited to which class of patients?
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TYPE 1 Insulin dependent DM
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What are the oral manifestations of DIABETES MELLITUS?
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- periodontal disease
- healing delayed - diabetic sialadenosis - oral candidiasis - xerostomia - infections increased - microangiopathy |
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What are the two different types of treatments for each DIABETES MELLITUS?
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TYPE 1: injections of insulin.
TYPE 2: Dietary modifications and lifestyle |
