Metabolic bone disease and osteoporosis (inc pagets)

Question 1

aetiology of pagets

Answer 1

15-30% familial, loci of SQSTMI, anglosaxon origins, chronic viral infections within osteoclasts may induce it.

Question 2

symptoms of pagets

Answer 2

bone pain, deformity, heat, neruological complications (nerve deafness)

TIBIAL BOWING

Question 3

presentation of pagets

Answer 3

LEG BOWING

elevated serum ALKphosphatase, hearing loss, rare development of osteosarcoma in the effected bone.

Question 4

Ix of pagets

Answer 4

xray, serum Calcium and ALKPhosphate, urinary hydroxyproline.

Question 5

complications of pagets?

Answer 5

osteosarcoma,

deafness,

high output cardiac failure

bone pain,

Question 6

tx of pagets

Answer 6

iv bisphosponate therapy. Iv zoledronic acid, bed rest and analgesia, calcitonin too.

Question 7

What is Rickets and Osteomalacia?

Answer 7

Severe nutritional vitamin D or Calcium deficiency causes insufficient mineralisation and thus rickets in a growing child and Osteomalacia in the adult when the epiphyseal lines are closed. Vitamin D stimulates the absorption of calcium and phosphate from the gut and calcium and phosphate then become available for bone mineralisation. Muscle function is also impaired in low vitamin D states

Question 8

signs of rickets

Answer 8

large forehead, stunted growth, odd curve to the spine, odd shaped ribs and breast bones, large abdomen, wide bones, odd shhaped legs, wide ankles, wide joints at thhe elbow and wrist.

Question 9

What is Osteogenesis Imperfecta?

Answer 9

Genetic disorder of connective tissue characterised by fragile bones from mild trauma and even acts of daily life Broad clinical range from those which are prenatally fatal to those only presenting in 40s with early ‘osteoporosis’

Question 10

name thhe types of OI

Answer 10

Defects in type 1 collagen twenty-eight different types exist/ first 4 most common: Type 1: milder form-when child starts to walk and can present in adults Type 11: lethal by age 1 Type 111: progressive deforming with severe bone dysplasia and poor growth Type 1V : similar to type 1 but more severe

Question 11

signs and symptoms of OI

Answer 11

growth deficiency, defective tooth formation, hearing loss, blue sclera, scoliosis, barrel chest, ligamentous laxity, easy bruising. (use the Beighton score)

Question 12

Mx of OI

Answer 12

surgical, iv bisphosphonates, genetic counselling, social and educational adaptations.

Question 13

What is Osteoporosis?

Answer 13

A metabolic bone disease characterised by low bone mass and micro architectural deterioration of bone tissue, leading to enhanced bone fragility and a consequent increase in fracture risk A result on DXA bone scanning

Question 14

how is fracture risk assessed?

Answer 14

age, BMD, falls, bone turnover, FRAX tool by WHO

Question 15

who is at risk of osteoporosis/ osteoporotic fracture?

Answer 15

1 in 2 women over 50, 1 in 5 men over 50. If you suffer 1 vertebral fracture you are 5 times more likely to have another and twice as likely to have hip fracture than if you had no vertebral fractures. Poor dietary calcium, premature menopause, lack of weight bearing exersice, prolongued amenhorrea, anorexia nervosa.

Question 16

what are the edocrine causes of osteoporosis?

Answer 16

thyrotoxicosis, hyper/hypothyroidism, cushings,hyperprolactinaemia, hypopituitarism, early menopause,

Question 17

rheumatic causes of osetoporosis?

Answer 17

RA, ankylosing spondylitis, polymyalgia rheumatica.

Question 18

GI causes?

Answer 18

inflammatory disease (UC or crohns), liver diseases, malabsorption: chronic pancreatitis, coeliac disease, whipples, short gut, ischaemic bowel

Question 19

medications which cause osteoporosis

Answer 19

steroids, PPI, enzyme inducting antiepileptic medications, aromatase inhibitors, GnRH inhhibitors, Warfarin.

Question 20

what does a BMD of -1 to -2.5 indicate?

Answer 20

osteopenia

Question 21

what does a BMD of below -2.5 indicate?

Answer 21

osteoporosis

Question 22

what is the sign guideline for osteoporosis?

Answer 22

refer for a dexa scan based of FRAX or qfracture score above 10% fracture risk at any site over the next 10 yrs. Tx decisions after dexa scanning and individual report-fracture risk around 20% 10yr risk would be tx threshold.

Question 23

Tx of osteoporosis

Answer 23

Bisphosphonates (alendronate, risedronate, raloxifene),

Hormone replacement therapy,

selective estrogen receptor modulator (SERM’s),

Denosumab (monoclonal antibody against RANKL)

Teriparatide (intermittent parathyroid hormone, increases bone density)

Calcitonin.

Analgesia.

Question 24

advice to patients and family

Answer 24

exersice regularly for weight bearing, calcium in diet, avoid smoking and alcohol excess.

Question 25

what are the se’s of hrt?

Answer 25

increased risk of blood clots, breast cancer, heart disease and stroke.

Question 26

what are the negative effects of SERM’s?

Answer 26

hot flushes, clotting risks, lack of protection at the hip site.

Question 27

se’s of bisphosphonates

Answer 27

oesophagitis, uveitis, ONJ, atypical shaft fractures. Need to take a drug holiday for 1-2 yrs typically after 10yrs of oral bisphosphonates.

Question 28

what is denosumab and how is it good?

Answer 28

monoclonal antibody against RANKL, it binds the RANK ligand and inhibits osteoclast formation, function and survival. Reduces osteoclastic bone resorption, subcutaneous injection every 6 months, safer in patients with a significant renal impairment than bisphosphonates.

Question 29

se’s of denosumab

Answer 29

allery/ rash, symptomatic hypocalcaemia if given when vit D depletes.

Question 30

what is Teriparatide?

Answer 30

reduces fracture risk, it is an intermittent parathyroid hormone, increases bone density

Question 31

se’s of Teriparatide

Answer 31

injection site irritation, rarely hypercalcaemia, allergy, costly.

Question 32

what do osteoblasts, clasts do?

Answer 32

blasts - bone production, clasts - bone destruction.

Question 33

go over hormonal regulation of calcium (vit d)

Answer 33

Question 34

what is Pagets disease?

Answer 34

disorder of bone turnover, increased bone resorption followed by increased bone formation.DECREASED MINERALISATION. Leads to disorganised bone (bigger, less compact, more vascular and more suceptible to deformity and fractures).