fml metabolic

Question 1

Paget’s Disease

1) another name?
2) genetic or metabolic?
3) monostatic or poluystotic?
4) what IS this disease?
5) where is it common geographically? race?
6) etiology
7) sex and age
8) location
9) common stance
10) facial features?
11) neurological symptoms?
12) people complain about what the most?
13) radio
14) what is skin covering the lesion like?
15) lab finding? what is it? must be at what threshold to initiate treatment?
16) D/d
17) histo
18) txt
19) classic description
20) complications
21) What is a late stage risk of Paget’s
22) What percent of lesions turn into osteosarcoma? age? cause?

Answer 1

1) Ostetitis Deformans
2) Metabolic
3) Poly (remember P for P)
4) Abnormal resorption and deposition of bone
* and in the case of pagets, its just the abnormal deposition - the osteoblast is over active
5) more common in Britain; has great geographic variance
* Caucasians
6) unknown etiology… maybe inflammatory
7) 2M:1F and greater than 40yrs (very rare to be found in someone under 40)
8) Jaws 10-15%; lumbar vertebrae, skull, pelvis and femur
9) Simian stance due to the bowing like deformity of the femur
10) “LION” like face (due to enlargement of maxilla) + diastema between the teeth + uniform enlargement of the jaws that might cause dentures not to fit, the increase in the circumference of the head
11) Deafness and blindness due to the compression of the nerves since the foramina decreased in size
12) bone pain, most consistent symptom
13) They will start out as radiolucent with altered trabecular pattern (osteoclasts working - resorption of bone and replacing with fibrovascular tissue)
* Over time, you get to become radiopaque, eventually becoming a COTTON ROLL like appearance with HYPERCEMENTOSIS
14) feels warm to touch since the bone is replaced with a fibrovascular tissue
15) Increase in the alkaline phosphatase (ALP) levels while Calcium and phosphate stay the same
* ALP is a marker of bone tumor, bone deposition and a by product of osteoblastic bone deposition
* Elevated levels of urinary hydroxyproline since this is also a break down product of protein
* 25% or GREATER
16) radiopaque caused by hypercementosis. Must differenciate from: Florid cementoosseous dsyplasia and Osteomas
17) Alternating levels of bone resorption and formation . Bone marrow is replaced by vascular fibrous connective tissue
* blue basophilic reversal lines
* prominent osteoblasts in the periphery as well as multi-nucleated giant cells in periphery
* stroma is full of fibrous tissue
18) if limited (ALP 25% or less) there is no treatment. Otherwise they need calcitonin and bisphosonates to DEcrease bone turn over
19) Mosaic and jigsaw puzzle
* this pattern is from basophilic (blue colored) reversal lines that are alternating zones of bone deposition/resorption and giant cells or multi-nucleated cells floating in fibrous stroma
20) complications:
1. Neurological due to shrinking of the foramina
2. Dental - discussed above
3. Osteosarcoma
21) Osteomyelitis due to perforation of the bone
22) 1% of cases will turn into osteosarcoma… If you see someone over 50 yrs with osteosarcoma think Paget’s bc that is uncommon OR radiation

Question 2

Hyperparathyroidism

1) what is it?
2) what do you need to rule out?
3) what hormone is overproduced?
4) causes for hormone overproduction
5) what is elevated in serum?
6) compare to Paget’s
7) secondary causes
8) relate to Vitamin D3
9) is inherited autosomal common?
10) catch phrase and describe it
11) early symptoms
12) How do the “bones” look radiogrphcailly? What other disease shares this? distinguish from fibrous dysplasia
13) histo
14) complication of the secondary disease of hyperparatyyoridism?
15) What is the treatment of the primary disease?
16) What is the treatment of the secondary disease

Answer 2

1) syndrome where the parathyroid glands are overproducing parathyroid hormones
2) central giant cell granuloma
3) PTH hormones
4) causes:
- most common: benign glandular neoplasm such as a paratyhroid adenoma
- hyperplasia
- least common: adenocarcinoma (less than 1%) or autosomal dominant
5) Serum will see an elevated PTH (obviously) as well as calcium and alkalkine phosphtase
* this makes sense since the entire point of the PTH is to unregulated calcium
6) Paget’s does not see an elevation in calcium - will only see an elevated ALP
7) Renal failure, dialysis, and malabsorption = all of this is due to the lack of calcium and this will then trigger the PTH
- common causes for renal failure is diabetes, hypertension, SLE
8) Someone with poor absorption of Vitamin D3 can also have poor calcim resorption
9) No, inherited autosomal dominant NOT common
10) Stones, bones, abdominal groans and moans
- stones: renal calculi because there is elevated calcium’s levels, especially in the kidney
- bones: brown tumors, looks like central giant cell granuloma - can occur anywhere in the long skeleton
- abdominal groans: Peptic ulcers, anorexia, polyuria, polydipsia and nausea
- moans: depression, memory and psychosis
11) No early symptoms! All show up later
12) Radiolucent tumor with ground glass radiopaque center and loss of lamina dura or ORANGE PEEL
* distinguish from fibrous dyplasia or hyperparathyroidism when you see ground glass look
* Remember FD is in younger patients in the first and second decade
13) Histopathology of central giant cell and brown tumor identical
* result of osteoclasts
14) pulp obliteration
15) primary disease txt: Surgery to eliminate the paratyhroid pathology
16) 2nd txt: Manage the renal disease

Question 3

Hyperthyroidism

1) what is it?
2) incidence?
3) sex and age
4) What is the most common presentation of the hyperthyroidism
5) Pathophys of grave’s disease?
6) What happens clinically IN GENERAL HYPERTHYROIDISM
7) classic clinical presentation of the Grave’s disease
8) oral presentations of Grave’s disease
9) txt
10) What do you want to rule out if you have a goiter?
11) If people have a thyroid storm, what do you want to avoid?

Answer 3

1) excessive production of thyroid hormone (T3 or T4) or TSH (thyroid stimulating hormone)
2) 3/10,000
3) Females - 20s or 30s
4) Grave’s disease - 70 to 85% of cases
5) LATS (long acting thyroid stimulator) IgG that binds to the TSH receptor - autoimmune
* TSH receptor on the surface of glandular cells - normally TSH (from anterior pituitary) binds to a TSH receptor and this will stimulate the thyroid cells to produce colloid
6) Remembrer, thyroid is your thermostat and regulator of your metabolism so you will see:
- Heat intolerance
- Hyperhidrosis- excessive sweating
- Heart palpitations
- Palmar erythema
- Diarrhea
- Anxiety
7)
* Exopthalamos
* Lig lag - due to scarring
* Acropachy or clubbing
* Pretibial myxema
* Increase in pulse, stroke volume, and cardiac out put
8) Oral presentations
- Premature exfoliation of the deciduous teeth increasing metabolism
- Rapid eruption of the permeant teeth - increasing metabolism
- Osteoporosis of the jaws
- burning tongue
- increased erosion
9) Thyroid suppressive meds, Radioactive iodine, Surgery and remove it entirely if there is a GOITER
10) thyroid cancer because it will be in the same demographic
11) IF poorly controlled, avoid epinephrine and avoid atropine because you can induce major cardiac issues