Term
| Pituitary Dwarfism definition |
|
Definition
| Failure or decreased production of growth hormone in a child |
|
|
Term
| Types of pituitary dwarfism |
|
Definition
| Panhypopituitarism & isolated GH deficiency |
|
|
Term
| Definition of panhypopituitarism |
|
Definition
| Deficit of all pituitary hormones - results in no puberty |
|
|
Term
| Etiology of Pituitary Dwarfism |
|
Definition
| Genetics, pituitary adenoma; other intrasellar/parasellar tumors; inflammatory and infectious destruction; surgical removal; radiation-induced destruction; traumatic brain injury; subarachnoid hemorrhage, and postpartum pituitary necrosis (Sheehan syndrome); similar conditions in hypothalamus or pituitary stalk |
|
|
Term
| Clinical presentation of pituitary dwarfism |
|
Definition
| Depends if isolated GH deficiency vs. pan-hypopituitarism; normal proportions overall; face may be smaller in proportion to skull; delayed tooth eruption; proportionally smaller teeth; 3rd molars missing |
|
|
Term
| Definition of pituitary adenoma |
|
Definition
| Benign neoplasm of anterior pituitary; age 20-50, more common in men, circumscribed mass in sella turcica |
|
|
Term
|
Definition
| = small (<10 mm); often non-functioning (don't produce hormones); "Incidentalomas" Non-functioning adenomas = ~25% of adults at autopsy |
|
|
Term
|
Definition
| = large (>=10 mm); "Functioning" excess secretion of pituitary hormones (prolactin, ACTH, growth hormone); subclassified by type of hormone released (prolactinoma); Mass effect - symptoms/signs due to size of lesion - may impinge on optic chiasm (altered/loss of vision); invasion of cavernous sinuses (oculomotor palsies); severe headaches; +/- effect from excess hormone produced (galactorrhea in prolactinoma; gigantism in GH secreting tumor) |
|
|
Term
| Treatment for pituitary adenomas |
|
Definition
| Surgery, radiation (esp. recurrent tumors after surgery), medication (e.g. octreotide: binds to somatostatin receptor - GH inhibiting hormone - signals pituitary to suppress secretion of GH) |
|
|
Term
|
Definition
| GH secreting adenoma (before closure of epiphyseal plates, growing individual); 10-20% assoc w/ McCune-Albright syndrome |
|
|
Term
|
Definition
| Generalized increase in size of body; disproportionaly long arms and legs; very tall (>3SD from"average"); enlarged sella turcica; macrodontia; enlarged mandible; +/- effects from reduced secretion of other pituitary hormones |
|
|
Term
|
Definition
| Surgical removal of causative adenoma |
|
|
Term
|
Definition
| Primary GH excess: excess GH after growth is complete; >90% = benign GH-secreting pituitary adenoma; also "familial acromegaly syndromes (v. Rare) |
|
|
Term
| Other less common etiologies of acromegaly |
|
Definition
Extrapituitary "ectopic" hypersecretion of GH: other tumors secreting GH - pancreatic islet-cell tumors, lymphoma (uncommon)
Excess production of GHRH (rare): central hypothalamic tumors; peripheral neuroendocrine tumors |
|
|
Term
| Demographics of acromegaly |
|
Definition
Incidence 3/1,00,000/year
Prevalence 60/1,000,000 |
|
|
Term
| Oral signs and symptoms of acromegaly |
|
Definition
| Thickening/enlargement of membranous bones of skull/jaws; mandibular prognathism; anterior open bite; +/- spacing of teeth; large tongue (macroglossia) |
|
|
Term
| Morbidity/Mortality of acromegaly |
|
Definition
| Overall standardized mortality ratio of patients w/ acromegaly is 1.48; factors contributing to increased mortality - higher prevalence of hypertension, hyperglycemia, cardiomyopathy, sleep apnea |
|
|
Term
| Background on adrenal cortex - cortisol |
|
Definition
Corticosteroids are subset of steroids that regulate body's ability to respond to stress
Essential for survivial; main one is "cortisol" |
|
|
Term
| Pathway of production of cortisol |
|
Definition
Hypothalamus (CRH) --> Pituitary (ACTH) --> Adrenal Cortex (cortisol)
Epinephrine and NE are made froma adrenal medulla |
|
|
Term
| T/F: Cushing's disease causes hypertrophy of the adrenal gland. |
|
Definition
|
|
Term
| T/F: Addison's disease causes hypertrophy of adrenal gland |
|
Definition
|
|
Term
| Etiology of Addison's disease |
|
Definition
| Primary adrenal cortical insufficiency: Usually autoimmune, infections (TB, deep fungal); metastatic tumors to adrenal gland (rare), leads to overproduction of ACTH; Secondary hypoadrenocorticism: ACTH deficiency; destructive lesions of hypothalamus or pituitary due to neoplasia, inflammation; trauma; idiopathic; Iatrogenic (=secondary adrenal suppression): drugs - long-term CS, ketoconazole |
|
|
Term
| Addison's disease and mechanism of hyperpigmentation |
|
Definition
| Leads to overproduction of ACTH/MSH precursor in POMC - MSH stimulates melanocytes --> more pigmentation |
|
|
Term
| Extra-oral clinical features of Addison's disease |
|
Definition
| Requires >90 & gland destruction, bronzing skin, signs/symptoms of decreased cortisol levels - weakness, hypotension, nausea/vomiting, weight loss, low sodium levels, elevated potassium, hypovolemia |
|
|
Term
| Intra-oral clinical features |
|
Definition
| Oral melanotic macule-like lesions; oral pigmentation; often precedes skin changes |
|
|
Term
| Dental significance of Addison's disease -- |
|
Definition
| Caution re: dental tx involving SIGNIFICANT surgical stress can lead to acute adrenal crisis |
|
|
Term
| Definition of hypercortisolism |
|
Definition
| Any condition in which body is exposed to elevated levels of cortisol (or equivalent) |
|
|
Term
| Etiology of hypercortisolism |
|
Definition
| Most common cause = long term use of SYSTEMIC corticosteroids (prednisone, inhaled steroid medicines for asthma, topical steroids usually not a cause); adrenal cortex tumor producing excess cortisol; ectopic cortisol-producing malignancies; ectopic ACTH-producing malignancies (e.g. some lung cancers); pituitary tumor producing excess ACTH |
|
|
Term
| Clinical findings in hypercortisolism |
|
Definition
= Result of long-term increased levels of glucocorticoids:
Hypertension, weight gain, moon faces (fat in facial area), buffalo hump; diabetes, osteoporosis, depression, Hirsutism, striae, acne, poor wound healing, muscle wasting, depression |
|
|
Term
| Acute adrenal insufficiency background |
|
Definition
| Body produces ~20 mg per day of cortisol ("endogenous"); when body is faced with stress anterior pituitary releases ACTH sent to the adrenal gland which makes cortisol |
|
|
Term
| Risk factors of acute adrenal insufficiency |
|
Definition
| Patients taking high doses of supplemental corticosteroids for long periods (>2 weeks); or by depot injection ("release active compound over a long period of time") |
|
|
Term
| Mechanism of acute adrenal insufficiency |
|
Definition
Patients taking corticosteroids for many conditions (in high doses, corticosteroids suppress immune system; therefore used to treat many "immune-mediated Dxs) - be aware many formulations of "exogenous" corticosteroids, diff formulations all have diff strengths
Long-term use of "exogenous" corticosteroid causes adrenal gland "hypofunction" therefore adrenal can't make additional cortisol in response to stress; result = potential for "acute adrenal insufficiency" in situations requiring more cortisol production |
|
|
Term
| Clinical presentation of acute adrenal insufficiency |
|
Definition
| Rare, patient will have hx of Addison's dx or hx of taking supplemental corticosteroids; mental confusion; intense pain in abdomen, back, legs; rapid LOC; death untreated |
|
|
Term
| Causes of adrenal insufficiency (acute adrenal insufficiency) |
|
Definition
| 2. secondary (pts taking high doses of supplemental corticosteroids); rule of 2's - concern if pt has taken 20 mg+ of cortisol EQUIVALENT/day; for 2 weeks or long within last 2 years |
|
|
Term
| Prevention of acute adrenal insufficiency |
|
Definition
| Consider need for additional corticosteroids if significant stress (esp. surgical) is anticipated |
|
|
Term
|
Definition
| Synthesis: complex feedback mechanism involving hypothalamus (brain), pituitary gland (base of brain) and thyroid gland |
|
|
Term
| Causes of thyroid enlargement |
|
Definition
| iodine deficiency: uncommon in US, iodine routinely added to table salt, other foods; neoplasms: adenomas, thyroid carcinomas; thyroid cyst: commonly result from degenerating thyroid adenomas; chronic inflammation (thyroiditis): Hashimoto's; multinodular goiter |
|
|
Term
| Multinodular goiter AKAs: |
|
Definition
| Simple, colloid or nontoxic vs. toxic |
|
|
Term
| Non-toxic multinodular goiter: |
|
Definition
| Euthyroid producing iodine hormone |
|
|
Term
| Toxic multinodular goiter |
|
Definition
| Autonomously functionoing thyroid nodules, hyperthyroid, etiology unclear, 5-20% of cases of hyperthyroidism in US |
|
|
Term
| Clinical and significance of multinodular goiter non-toxic |
|
Definition
| Most common disease of thyroid, diffuse enlargement of thyroid gland, dysphagia, cough, and hoarseness; women 8:1 men; be aware of this as part of routine head and neck exam, small % are found to harbor a thyroid carcinoma |
|
|
Term
| Etiology of multinodular goiter non-toxic |
|
Definition
| Pathogenesis not entirely clear - familial predisposition, +/- iodine deficiency (less common in US); result = markedly enlarged, inborn errors of thyroid hormone biosynthesis leading to goiter=rare |
|
|
Term
|
Definition
| Inadequate T3/T4 = EVERYTHING SLOWS DOWN |
|
|
Term
|
Definition
| Excess thyroid hormone levels = EVERYTHING SPEEDS UP; e.g. Graves dx |
|
|
Term
| Clinical features of hypothyroidism |
|
Definition
| Arthritis, gold intolerance, depression, dry skin, fatigue, forgetfulness, heavy menstrual periods, infertility, muscle aches |
|
|
Term
| Hyperthyroidism clincial features |
|
Definition
| Difficult sleeping, heat intolerance, infertility, irritability, muscle weakness, nervousness, scant menstrual periods |
|
|
Term
| Etiology of hypothyroidism |
|
Definition
| 1. inadequate functioning gland due to thyroiditis, surgery or radioactive iodine therapy; 2. inadequate secretion of TSH by pituitary gland or TRH by hypothalamus |
|
|
Term
| Cretinims (etiology, clinical, and tx) |
|
Definition
| Low T3/T4 in childhood; 1 in 4,000; delayed growth, impaired development of CNS, mental retardation if no tx; girls 2:1, infants = sluggish, pale, cole, short stature (Defects in osseous maturation); thyroid replacement hormone (synthroid) |
|
|
Term
| Oral manifestations of cretinism |
|
Definition
| Protruding/enlarged tongue (macroglossia); failure of permanent teeth to erupt; retained deciduous teeth |
|
|
Term
| etiology of myxedema hypothyroidism |
|
Definition
| low T3/T4; AKA = myxedema |
|
|
Term
|
Definition
| Women>men 10:1; thickening of facial features (edema effecting facial skin; puffy eyelids), weight gain, lethargy, cold intolerance, macroglossia, coma = very rare; Increased sensitivity to CNS depressants |
|
|
Term
| Hashimoto thyroiditis (chronic lymphocytic thyroiditis) etiology: |
|
Definition
| Autoimmune disease; destroys normal thyroid gland - auto-antibody and T cell-mediated destruction of follicular cells; antibodies against thyroglobulin and thyroid peroxidases; eventually hypothyroid, +/- occasional periods of hyper T3 |
|
|
Term
| Clinical features of Hashimoto Thyroiditis |
|
Definition
| Age 30-40, women>men; diffuse, symmetrical thyroid enlargement |
|
|
Term
| Etiology of hyperthyroidism |
|
Definition
| Prolonged hypersecretion of thyroid hormones T4/T3; graves disease, toxic multinodular goiter, excess intake of thyroid meds, thyroiditis, thyroid adenoma/carcinoma; pituitary adenoma (TSH releasing) |
|
|
Term
| Clinical features of hyperthyroidism |
|
Definition
| Women 7:1, often enlarged gland, symptoms reflect hypermetabolic state = weight loss, increased aditation, increased heart rate, increased susceptibility to epinephrine |
|
|
Term
| Graves disease (hyperthyroidism) definition and etiology |
|
Definition
| most frequent cause of hyperthyroidism in patients under 40; 2% of women > 40 years; autoimmune disorder, stimulatory IgG antibodies directed against TSH receptor of thyroid follicle, increase hormone production |
|
|
Term
| Clinical features of graves disease |
|
Definition
| Diffusely enlarged thyroid gland, exophthalmos (fibroblasts in orbit are activated, GAG deposition causes eyes to bulge); nervousness, temor, irritability, tachycardia, palpitations; weight loss w/ increased appetite, night sweats, diarrhea, NB: Ig may cross placental barrier, leading to temporary hyperthyroidism in 1-5% of children born to affected mothers |
|
|
Term
| Treatment for graves disease |
|
Definition
| Drugs that block hormone synthesis (propylithiouracil, can cause hepatic failure long term); radioactive iodine therapy (side effect hypothyroidism); surgery |
|
|
Term
| Significance of graves disease |
|
Definition
| Exophthalmos irreversible; prior to Tx = risk of thyroid storm - massive release of large amounts of thyroid hormone (leads to delirium, tachycardia, elevated temperature; mortality rate is 20+%); epinephrine injection can trigger thyroid storm; infection, psychological trauma, or stress too |
|
|
Term
| Thyroid adenomas (follicular adenoma) |
|
Definition
| Discrete, solitary benign thyroid neoplasms; "Factoids" most non-functional, usually take up less radioactive iodine on scans = "cold nodules"; many undergo cystic degeneration; less common functional ones are assoc w/ mutations taht cause activating "gain of function" somatic mutations of TSH receptor or assoc G-protein; Dx of benign follicular adenoma requires histologic evaluation of capsule; so FNA alone not sufficient |
|
|
Term
| Papillary thyroid carcinoma |
|
Definition
| Most common thyroid carcinoma: 75-85% of cases; increased risk from exposure to ionizing radiation (diagnostic or therapeutic radiation to neck); esp females 20s-40s; clinical - asymmetrical enlargement; prognosis is excellent >95% at 10 years |
|
|
Term
| Medullary Thyroid carcinoma |
|
Definition
| Neoplasms derived from parafollicular "C" cells; result = excess secretion of calcitonin, <5% of thyroid cancers; familial form: multiple endocrine neoplasia (MEN) type IIb; dev early in life; multiple mucosal neuromas: oral cavity + eyelids |
|
|
Term
| Etiology of hypoparathyroidism |
|
Definition
| Decreased secretion of PTH; most common cause is removal of parathyroids during thyroidectomy; rarely: autoimmune destruction, syndromes (DiGeorge, endocrine-candidiasis syndrome) |
|
|
Term
| Pathogenesis and treatment of hypoparathyroidism |
|
Definition
| Normal function of PTH - PTH acts on kidney + osteoclasts; Kideny - increased calcium reabsorption, increased phosphate excretion, production of vitamin D (increases Ca2+ absorption from gut); osteoclasts stimulated to resorb bone, elevated serum calcium; tx: oral calcium supplements, vitamin D precursor |
|
|
Term
| Clinical of hypoparathyroidism |
|
Definition
| Hypocalcemia: increased neuromuscular excitability, mild tingling of hands and feet, muscle cramps, Chvostek's sign (twitching of upper lip when facial nerve tapped); if during tooth development period: pitting enamel hypoplasia; failure of tooth eruption; elevated serum phosphate |
|
|
Term
| Definition of hyperparathyroidism |
|
Definition
|
|
Term
| Etiology of hyperparathyroidism |
|
Definition
Primary: hyperplasia of parathyroid gland (18%); parathyroid adenoma (benign tumor, 80%), parathyroid carcinoma (malignant 2%); Secondary: chronic renal dx, e.g. renal osteodystrophy (=bone changes assoc w/ ESRD, =compensatory hyperPTH due to kidney dx); affects 90% of pts on long term renal dialysis (decreased renal conversion vit D, decreased phosphate excretion); chronic hypocalcemia, continuous production of PTH; Tertiary: continued hyperPTH following renal transplantation
|
|
|
Term
| Lab findings of hyperparathyroidism |
|
Definition
| Elevated PTH, hypercalcemia, hypophosphatemia |
|
|
Term
Hyperparathyroidism: excess PTH leads to excess loss of calcium from bones leads to:
|
|
Definition
| 1. Painful bones: resorption of phalanges of index and middle finger, generalized loss of lamina dura, blurring of the trabecular pattern leads to "ground glass" appearance; 2. renal stones: renal calculi dev due to elevated serum calcium, metastatic calcification in other soft tissue, 3. Abdominal groans duodenal ulcer; 4. Moans: depression and lethargy |
|
|
Term
| Bone effects of hyperparathyroidism |
|
Definition
| Decalcification of bones (prone to fracture); cyst formation in bones ("osteitis cystica"); "Brown tumor" of hyperPTH (histologically = aggregates of giant cells, histologically = identical to CGCGs of jaws (multinucleated giant cells in background of extravasated RBCs) |
|
|
Term
| Hyperparathyroidism, characteristics + histology |
|
Definition
| Brown tumor = deep red to brown color; well-demarcated uni- to multi-locular radiolucency (mandible, clavicle, ribs, pelvis); Histology = multinucleated giant cells in background of extravasated RBCs |
|
|
Term
| Brown tumor of HyperPTH (Chronic Kidney Disease-Mineral and Bone disorder) |
|
Definition
| Renal osteodystrophy; =spectrum of skeletal seen in renal Dx; seen in primary/secondary hyperPTH; includes "brown tumor" of hyperPTH = deep red to brown color; clinical presentation: 3-4% of primary hyperPTH patients, 1.5% of dialysis pts with secondary HPT, solitary or multiple lesions (ribs, clavicle, hands, feet, pelvic girdle, mandible ~4%) |
|
|
Term
|
Definition
| Of bone lesions: correct underlying hyperPTH (resolution of bone lesions); primary - neoplastic: surgical removal of affected gland; hyperplasia: removal of 3 of 4 enlarged glands; secondary - renal transplant, vs. removal of 3 of 4 glands |
|
|
Term
|
Definition
| generalized loss of lamina dura, blurring of trabecular pattern = "ground glass" appearance; may also see distinct radiolucent lesions |
|
|
Term
| Dental significance of hyperparathyroidism |
|
Definition
| Bone lesion in jaws w/ histology of CGCG (could be 1st manifestation of hyperPTH) |
|
|
