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Metabolic Bone Disorders

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Presentation on theme: "Metabolic Bone Disorders"— Presentation transcript:

1 Metabolic Bone Disorders
Prof. Mamoun Kremli AlMaarefa College

2 Objectives Bone as an active tissue Calcium is an important mineral
Calcium metaboism – normal control Diseases Osteoporosis Rickets and Osteomalacia Hyperparathyroidism Scurvy

3 Functions of bone tissue
Mechanical: Support & protect soft tissue Load transmission Mediate movement Mineral reservoir Largest reservoir of Ca Regulation of Ca

4 Basic Anatomy of Bone

5 Bone components A: Matrix: Organic: (40% of dry weight)
Collagen fibers Cells Inorganic (Minerals): (60%) Ca hydroxyapetite, Ca phosphate Others B: Cells: Osteoblasts, osteoclasts, osteocytes, others

6 Bone cells Osteoblasts Osteoclasts Osteocytes Osteone:
a unit, not a cell

7 Bone is active Continuous activity and flow
Structure and composition changing all the time Regulations by regulating cellular activity: Osteoclasts & Osteoblasts Cellular Activity Modulation of Bone Structure & Composition Changes in mineral ion concentrations Hormones & Local Factors

8 Bone growth & remodelling
Epiphyseal: Endochondral ossification On surface: Oppositional ossification In Adults: Remodelling of existing bone (no growth) Annually: 4% of cortical and 25% of cancellous “old bone” continuously replaced by “new bone” Initially: formation slightly exceeds resorption Later: resorption exceeds formation Bone mass steadily declines

9 Age related bone changes
Childhood – adolescence: Growth ( size & change shape) Adolescence – 35 (40) years: Bones get heavier and stronger Annual bone mass gain: 3% 35 (40) – 50 years: Slow loss of bone mass annually: Men: 0.3% Women: 0.5% to menopause, then 3% for 10 years - (Why?) (↑ osteoclastic activity by ↓ hormones) 65 years – onwards: Loss of mass slows gradually to 0.5% (↓osteoblastic activity)

10 Body Calcium Most of Ca in body is present in bone
Release of Ca from bone is a slow process Serum calcium is essential for cell function, nerve conduction, and muscle contraction Normal level: mg/dl ( mmol/L) S. levels have to be controlled quickly Intestinal absorption Renal reabsorption

11 Causes of Calcium absorption
 intake of phosphates (as in soft drinks)  intake of oxalates (as in tea and coffee) Drugs: corticosteroids Intestinal malabsorption syndromes

12 Players in Ca regulation
Vit. D is the general crude regulator Target organs: Small intestines Bones PTH is the sensitive fine regulator Kidneys (v quick) Bones (slow) (indirectly): small intestine

13 Players in Ca regulation
Cacitonin: C cells of Thyroid Opposite PTH on bone and kidneys Oestrogen: Protects bone from PTH Corticosteroids: Bad to bone Local – BMP (Bone Morphogenic Proteins) Mechanical stress: Strengthens bone

14 Calcium metabolism

15 Hormonal regulation of Ca met.

16 Hormonal regulation of Ca met.
Mesutti, 2011

17

18 Laboratory investigations
X-rays Bone mineral density (BMD) DEXA scans: Dual Energy X0ray Absorptiometry Biochemical tests: Serum Ca, Phosphate Serum Alkalin Phosphatase Osteoclastic activity, measures bone turnover rate Vit. D levels Urine Ca and Phosphate excretion Renal profile Liver function test

19 Common Diseases Osteoporosis Rickets Osteomalacia
Hyperparathyroidism (osteitis fibrosa)

20 Osteoporosis Reduction of bone mass Types:
Bone minerals and matrix both reduced Matrix present is normally mineralized Types: Generalized: systemic disease Localized: disuse (e.g. in cast)

21 Osteoporosis More in women In men:
Post menopausal Oestrogen withdrawal Increased with: cigarette smoking when start menopause with weak bones In men: 15 years later In elderly, may be associated with osteomalacia

22 Osteoporosis – clinical features
Weak bones: easily fractures: Vertebral compression fractures Backache, kyphosis Colle’s fracture Neck of femur Proximal humerus Orthopedic Radiology, A Greenspan. lippincott

23 Osteoporosis – clinical features
Weak bones: easily fractures: Vertebral compression fractures Backache, kyphosis Colle’s fracture Neck of femur Proximal humerus Orthopedic Radiology, A Greenspan. lippincott

24 Osteoporosis – clinical features
Weak bones: easily fractures: Vertebral compression fractures Backache, kyphosis Colle’s fracture Neck of femur Proximal humerus Apley’s System of Prthop & Fractures Orthopedic Radiology, A Greenspan. lippincott

25 Osteoporosis – clinical features
Weak bones: easily fractures: Vertebral compression fractures Backache, kyphosis Colle’s fracture Neck of femur Proximal humerus Loss of cortical thickness seen on X-rays Apley’s System of Prthop & Fractures Orthopedic Radiology, A Greenspan. lippincott

26 Risk Factors for postmenopausal osteoporosis
Caucasian (white) or Asiatic ethnicity F.H. of osteoporosis H.O. anorexia nervosa or amenorrhea Low peak bone mass in third decade Early onset menopause Very slim built Oophorectomy and early hysterectomy Nutritional deficiency Chronic lack of exercise

27 Osteoporosis - Prevention
Good Ca and Vit. D intake Good physical activity Exposure to sun No smoking No alcohol

28 Osteoporosis - Prevention
If BMD low: Hormone replacement therapy (oestrogen): Effective early For initial five years Problems: Dysfunctional uterine bleeding Risk of uterine and breast cancer – on long use

29 Osteoporosis - treatment
Treat the fractures Maintain good Ca and Vit D intake May be associated with osteomalacia Maintain good physical activity Trying to reduce rate of further bone loss Hormone replacement therapy Bisphosphonates

30 Rickets & Osteomalacia

31 Rickets & Osteomalacia
Same disease: (children / adults) Inadequate absorption and/or utilization of Ca Common causes: Lack of Vit. D Sever Ca deficiency Hypophosphatemia Results in loss of mineralization of bone

32 Rickets - pathology Matrix forms, not calcified In growing physis
Widened physis (epiphyseal growth plate) Cupping of metaphyseal end (weak new bone) In all bone Osteopenia, Thin cortex, Deformity Harrisons sulcus, frontal bossing In sever cases: hypocalcaemia: Tetany, convulsions, failure to thrive

33 Rickets – clinical picture
Enlarged ends of long bones Wrists, knees Rickety rosary: costo-chondral junctions Harrisons sulcus Frontal bossing Bowing of legs: Localized – distal tibiae In sever cases: tetany, convulsions Orthopedic Radiology, A Greenspan. lippincott

34 Rickets – clinical picture
Enlarged ends of long bones Wrists, knees Rickety rosary: costo-chondral junctions Harrisons sulcus Frontal bossing Bowing of legs: Localized – distal tibiae In sever cases: tetany, convulsions

35 Rickets – clinical picture
Enlarged ends of long bones Wrists, knees Rickety rosary: costo-chondral junctions Harrisons sulcus Frontal bossing Bowing of legs: Localized – distal tibiae In sever cases: tetany, convulsions

36 Rickets – clinical picture
Enlarged ends of long bones Wrists, knees Rickety rosary: costo-chondral junctions Harrisons sulcus Frontal bossing Bowing of legs: Localized – distal tibiae In sever cases: tetany, convulsions

37 Rickets – clinical picture
Enlarged ends of long bones Wrists, knees Rickety rosary: costo-chondral junctions Harrisons sulcus Frontal bossing Bowing of legs: Localized – distal tibiae In sever cases: tetany, convulsions

38 Rickets – clinical picture
Enlarged ends of long bones Wrists, knees Rickety rosary: costo-chondral junctions Harrisons sulcus Frontal bossing Bowing of legs: Localized – distal tibiae In sever cases: tetany, convulsions N Engl J Med 2009

39 Orthopedic Radiology, A Greenspan. lippincott
Rickets – X-rays Widened physis (epiphyseal growth plate) metaphyseal end of physis Cupping of (weak new bone) Irregular Deformed bones Orthopedic Radiology, A Greenspan. lippincott

40 Orthopedic Radiology, A Greenspan. lippincott
Rickets – X-rays Orthopedic Radiology, A Greenspan. lippincott

41 Orthopedic Radiology, A Greenspan. lippincott
Rickets – X-rays Orthopedic Radiology, A Greenspan. lippincott

42 Rickets – lab results Serum Ca: Serum Phsphate: Alk Phosphatase:
slightly low /or normal Serum Phsphate: Alk Phosphatase: High – a lot of bone turnover Vit. D level: low PTH level: Increased – scondary effect – to keep s. Ca level Urinary Ca: V. low

43 Rickets - treatment Vit. D and Calcium
Most deformities correct gradually Sever deformities might need surgical correction

44 Hopophsphataemic rickets
Vit. D resistant rickets Familial, X-linked Impaired renal tubular reabsorption of phosphate Lab. Results: Serum Phosphate: low Urinary phosphate: high Treatment: High dose Vit. D Phosphate

45 Orthopedic Radiology, A Greenspan. lippincott
Osteomalacia Caused by defective Vit. D: Deficiency – lack of sun exposure Intestinal malabsorption Defective formation of active Vit. D: Liver or Renal disease Clinical features Bone aches – backache, hip pain Compressed vertebral fracture Insufficiency fractures of femur / tibia Orthopedic Radiology, A Greenspan. lippincott

46 Apley’s System of Prthop & Fractures
Osteomalacia – X-rays Weak osteopaenic bone Biconcave vertebrae & compression fractures Trefoil pelvis – acetabular protrusion Typically: Looser’s zones: Poorly healing stress fractures Neck of scapula Neck of femur Pubic bones Apley’s System of Prthop & Fractures

47 Apley’s System of Prthop & Fractures
Osteomalacia – X-rays Weak osteopaenic bone Biconcave vertebrae & compression fractures Trefoil pelvis – acetabular protrusion Typically: Looser’s zones: Poorly healing stress fractures Neck of scapula Neck of femur Pubic bones Apley’s System of Prthop & Fractures

48 Orthopedic Radiology, A Greenspan. lippincott
Osteomalacia – X-rays Weak osteopaenic bone Biconcave vertebrae & compression fractures Trefoil pelvis – acetabular protrusion Typically: Looser’s zones: Poorly healing stress fractures Neck of scapula Neck of femur Pubic bones Orthopedic Radiology, A Greenspan. lippincott

49 Apley’s System of Prthop & Fractures
Osteomalacia – X-rays Weak osteopaenic bone Biconcave vertebrae & compression fractures Trefoil pelvis – acetabular protrusion Typically: Looser’s zones: Poorly healing stress fractures Neck of scapula Neck of femur Pubic bones Apley’s System of Prthop & Fractures

50 Orthopedic Radiology, A Greenspan. lippincott
Osteomalacia – X-rays Looser’s zone Orthopedic Radiology, A Greenspan. lippincott

51

52 Hyperparathyroidism Primary: Secondary: Tertiary:
Parathyroid adenoma / hyperplasis Secondary: Hyperplasia due to hypocalcaemia Tertiary: Autonomous activity after secondary hyperplasis

53 Orthopedic Radiology, A Greenspan. lippincott
Hyperparathyroidism Effect of PTH Target organs: Kidneys Bones Intestines (indirect) Bone weakens, resorption Increased serum Ca Orthopedic Radiology, A Greenspan. lippincott

54 Hyperparathyroidism Bones Stones Moans Groans Rarefaction
Subperiosteal resorption (middle phalanges) Reorption of lateral end clavicle Brown tumors Stones Kidney stones and nephroclacinosis Moans Abdominal pain, renal pain Groans Pschological depression, stress

55 Hyperparathyroidism – x-rays
Bones Rarefaction Bone resorption Subperiosteal resotption middle phalanges Tibial shaft lateral end clavicle Brown tumors Skull: salt & pepper Soft tissue calcification

56 Hyperparathyroidism – x-rays
Bones Rarefaction Bone resorption Subperiosteal resotption middle phalanges Tibial shaft lateral end clavicle Brown tumors Skull: salt & pepper Soft tissue calcification Orthopedic Radiology, A Greenspan. lippincott

57 Hyperparathyroidism – x-rays
Subperiosteal bone resorption Orthopedic Radiology, A Greenspan. lippincott

58 Hyperparathyroidism – x-rays
Bones Rarefaction Bone resorption Subperiosteal resotption middle phalanges Tibial shaft lateral end clavicle Brown tumors Skull: salt & pepper Soft tissue calcification Orthopedic Radiology, A Greenspan. lippincott

59 Hyperparathyroidism – x-rays
Subperiosteal bone resorption Orthopedic Radiology, A Greenspan. lippincott

60 Hyperparathyroidism – x-rays
Bones Rarefaction Bone resorption Subperiosteal resotption middle phalanges Tibial shaft lateral end clavicle Brown tumors Skull: salt & pepper Soft tissue calcification Orthopedic Radiology, A Greenspan. lippincott

61 Hyperparathyroidism – x-rays
Bones Rarefaction Bone resorption Subperiosteal resotption middle phalanges Tibial shaft lateral end clavicle Brown tumors Skull: salt & pepper Soft tissue calcification Orthopedic Radiology, A Greenspan. lippincott

62 Hyperparathyroidism – x-rays
Bones Rarefaction Bone resorption Subperiosteal resotption middle phalanges Tibial shaft lateral end clavicle Brown tumors Skull: salt & pepper Soft tissue calcification

63 Hyperparathyroidism – x-rays
Bones Rarefaction Bone resorption Subperiosteal resotption middle phalanges Tibial shaft lateral end clavicle Brown tumors Skull: salt & pepper Soft tissue calcification Orthopedic Radiology, A Greenspan. lippincott

64 Hyperparathyroidism – x-rays
Bones Rarefaction Bone resorption Subperiosteal resotption middle phalanges Tibial shaft lateral end clavicle Brown tumors Skull: salt & pepper Soft tissue calcification Orthopedic Radiology, A Greenspan. lippincott

65 Hyperparathyroidism - treatment
Hydration Reduced calcium intake If adenoma: Surgical removal Beware of the “hungry bone” syndrome post operatively – severe hypocalcaemia (why?)

66 Scurvy – Vit. C deficiency
First discovered in sailors Failure of collagen fibers formation Weak osteoid matrix Clinical picture: Child irritable, anemia Bleeding gums Pain and swellings at ends of long bones

67 Scurvy – Vit. C deficiency
X-rays: Osteopaenia – more at mataphysis Sub-periosteal bleeding Periosseous calcification Ring epiphysis Sclerosis at juxtaepiphyseal metphysis Treatment: Vit C (large doses)

68 Scurvy – Vit. C deficiency
Orthopedic Radiology, A Greenspan. lippincott

69 Scurvy – Vit. C deficiency
Orthopedic Radiology, A Greenspan. lippincott

70 Scurvy – Vit. C deficiency
X-rays: Osteopaenia – more at mataphysis Sub-periosteal bleeding Periosseous calcification Ring epiphysis Sclerosis at juxtaepiphyseal metphysis Treatment: Vit C (large doses)

71 Summary Bone is an active tissue Calcium is an important mineral
Continuous absorption and rebuilding Calcium is an important mineral Calcium control Diseases Osteoporosis Rickets and Osteomalacia Hyperparathyroidism Scurvy

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