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METABOLIC BONE DISEASE

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Presentation on theme: "METABOLIC BONE DISEASE"— Presentation transcript:

1 METABOLIC BONE DISEASE
Dr.Ahmed Saied MBChB FIBMS

2 General Bone tissue consists of: Extracellular substance * Osteoid: collagen, mucopolysaccharide * Crystalline component: calcium phosphate, hydroxyapatite Cells * Osteoblasts * Osteoclasts

3 Bone is constantly absorbed and replaced with new bone.
Disturbances in this equilibrium result in either too much bone (increased radiodensity, osteosclerosis) or too little bone (decreased density = osteopenia).

4 that influence the radiographic appearance.
OSTEOPENIA Osteopenia is a nonspecific radiographic finding that indicates increased radiolucency of bone. Bone density may be difficult to assess because of technical factors (kVp, mA) that influence the radiographic appearance.

5 1- Osteoporosis: decreased amount of normal bone
Types 1- Osteoporosis: decreased amount of normal bone 2- Osteomalacia: decreased bone mineralization 3- Marrow replacement: bone replaced by tumor, marrow hyperplasia,or Metabolic products 4- Hyperparathyroidism: increased bone resorption

6 Specific radiographic clues
Disorder Looser zones osteomalacia subperiosteal resorption hyperparathyroidism focal lytic lesions disseminated multiple myeloma

7 OSTEOPOROSIS Classification Primary osteoporosis (most common): unassociated with an underlying illness * Type I osteoporosis: postmenopausal * Type II osteoporosis: senile -Idiopathic juvenile osteoporosis

8 Secondary osteoporosis (less common)
1- Endocrine disorders(Hypogonadism , Hyperthyroidism, Cushing's disease, Acromegaly, Nutritional, Malabsorption syndromes, Alcoholism, Scurvy.

9 2- Hereditary metabolic or collagen disorder
(Osteogenesis imperfecta, Marfan syndrome, Ehlers-Danlos syndrome, Homocystinuria, Hypophosphatasia, Wilson's disease, Alkaptonuria, Menkes' syndrome) 3- Drugs (Heparin Exogenous steroids

10 * Osteopenia: 30%-50% of bone has to be lost to
Radiographic features * Osteopenia: 30%-50% of bone has to be lost to be detectable by plain film * Diminution of cortical thickness: width of both MCP cortices should be less than half the shaft diameter * Decrease in number and thickness of trabeculae in bone Vertebral bodies show earliest changes: resorption of horizontal trabeculae Empty box vertebra: apparent increased density of vertebral endplates due to resorption of spongy bone Vertebral body compression fractures: wedge, biconcave codfish bodies, true compression * Pathological fractures

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12 Qualitative assessment:
Singh index is based on trabecular pattern of proximal femur. Patterns: 1-Mild: loss of secondary trabeculae 2-Intermediate: loss of tensile trabeculae 3-Severe: loss of principal compressive trabecular Quantitative bone densitometry Predicts the risk for developing fractures 3 methods are available: * Single-photon absorption Measures cortical bone density of radial shaft * Dual-photon absorption with radionuclide or dual-energy x-ray Measures vertebral and hip Quantitative CT with phantom Measures vertebral body density (trabecular only) Most effective technique for evaluation of bone density

13 Indications for measurements:
Initiation of estrogen replacement therapy or phosphonate therapy * Establish diagnosis of osteoporosis * Assess severity of osteoporosis *Monitor treatment efficacy

14 OSTEOMALACIA Abnormal mineralization of bone is termed Osteomalacia in adults and rickets in children. In the past, the most common cause was deficient intake of vitamin D. Today, absorption abnormalities and renal disorders are more common causes.

15 Nutritional deficiency of:
*Vitamin D * Calcium * Phosphorus Absorption abnormalities *Gastrointestinal (GI) surgery * Malabsorption *Biliary disease Renal *Chronic renal failure *Renal tubular acidosis *Proximal tubular lesions * Dialysis induced Abnormal vitamin D metabolism *Liver disease * Hereditary metabolic disorders Drugs * Dilantin * Phenobarbitol

16 Rickets

17 Normal Osteomalacia

18 Normal Osteoporosis

19 frogleg view looser’s zone
AP view looser’s zone

20 Osteomalacia: Bone scan

21 Radiographic features
1-Generalized osteopenia 2-Looser's zones (pseudofractures): cortical stress fractures filled with poorly mineralized osteoid tissue. * Milkman's syndrome: osteomalacia with many Looser's zones Typical location of Looser's zones (often symmetrical) Axillary margin of scapula Inner margin of femoral neck Rib Pubic, ischial rami * Osteomalacia may be indistinguishable from osteoporosis; however, Looser's zones are a reliable differentiating feature.

22 RENAL OSTEODYSTROPHY Renal osteodystrophy is a general term that refers to a myriad of radiographic osseous changes in patients with renal failure. Radiographically, these changes are secondary to osteomalacia, secondary hyperparathyroidism, and aluminum intoxication. Radiographic features -Changes of osteomalacia Osteopenia and cortical thinning Looser's zones occur but are uncommon -Changes of hyperparathyroidism * Subperiosteal resorption (e.g., SI joint resorption) * Rugger jersey spine * Brown tumors * Osteosclerosis * Soft tissue calcification &Chondrocalcinosis

23 HYPERPARATHYROIDISM (HPT)
Parathyroid hormone stimulates osteoclastic resorption of bone. HPT is usually detected by elevated serum calcium during routine biochemical screening. Three types: Primary HPT: Adenoma, 85% (single 90%, multiple 10%) Hyperplasia, 12% Parathyroid carcinoma, 1%-3% Secondary HPT: most often secondary to renal failure; rarely seen with ectopic parathyroid production by hormonally active tumor Tertiary HPT: results from autonomous glandular function following longstanding renal failure

24 Radiographic features:
Clavicuiar lysis, Subperiosteal resorption Brown tumors ,General osteopenia (Bone resorption is virtually pathognomonic(Subperiosteal resorption Radial aspect of middle phalanges (especially index and middle finger) Phalangeal tufts Trabecular resorption Salt and pepper skull Cortical resorption Tunneling of MCP bones (nonspecific) Subchondral resorption Widened SI joint Distal end of clavicle Widened symphysis pubis Can lead to articular disease Brown tumors (cystlike lesions) may be found anywhere in the skeleton but especially in the pelvis, jaw, and femur. 0 Loss of the lamina dura Soft tissue calcification Chondrocalcinosis 0 Complication: fractures

25 Hyperparathyroidism Xrays: sub-periosteal resorption pepper pot skull
rugger jersey spine cystic brown tumours

26 Hyperparathyroidism Xrays: sub-periosteal resorption pepper pot skull
rugger jersey spine cystic brown tumours

27 Hyperparathyroidism Xrays: sub-periosteal resorption pepper pot skull
rugger jersey spine cystic brown tumours

28 Hyperparathyroidism Xrays: sub-periosteal resorption pepper pot skull
rugger jersey spine cystic brown tumours

29 Hyperparathyroidism Xrays: sub-periosteal resorption pepper pot skull
rugger jersey spine cystic brown tumours

30 Marrow Disease Malignant infiltration * Myeloma * Leukemia/lymphoma
CLASSIFICATION Malignant infiltration * Myeloma * Leukemia/lymphoma *Metastases (small cell tumors) Secondary marrow hyperplasia *Hemoglobinopathies *Hemolytic anemias Lysosomal storage diseases * Gaucher's disease *Niemann-Pick disease

31 SICKLE CELL ANEMIA Structural defect in hemoglobin (hemoglobin S; point mutation). Most hemoglobinopathies (over 250 are known) result in rigid hemoglobin and hemolysis. Incidence: 1% of blacks. Diagnosis is confirmed by hemoglobin electrophoresis. Sickle cell disease (IlbSS) has many bone findings, whereas sickle cell trait (HbAS) is only occasionally associated with bone infarcts. Hemoglobin sickle cell disease has same bone findings but the spleen is enlarged.

32 Radiographic features
Hyperplasia of marrow * Hair-on-end appearance of skull Pathological fractures @ Biconcave H-shaped vertebra Q Osteopenia ! Vascular occlusion AVN occurs primarily in medullary space of long bones, hands, growing epiphyses * Bone sclerosis from infarctions * H-shaped vertebral bodies involvement of growing epiphyses leads to growth disturbances * Dactylitis (hand-foot syndrome): bone infarcts of hands and feet Osteomyelitis I * High incidence: most are caused by Staphylococcus Salmonella infection more common than in general population Most commonly at diaphysis of long bones Osteomyelitis and infarction may be difficult to distinguish. * Small calcified fibrotic spleen due to autoinfarction * Cholelithiasis * Progressive renal failure Papillary necrosis * Cardiomegaly: high output congestive heart failure (CHF) Pulmonary infarcts

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34 Plain radiography. Anterior-posterior view of bilateral knees
Plain radiography. Anterior-posterior view of bilateral knees. Note the irregular areas of lacy and serpentine calcific deposits in bilateral distal femurs and proximal tibias typical for bone infarcts.

35 THALASSEMIA (COOLEY'S ANEMIA)
Genetic disorder characterized by diminished synthesis of one of the globin chains. Radiographic features Hyperplasia of marrow is the dominant feature. * Expands the marrow space: hair-on-end skull * Modeling deformities of bone: Erlenmeyer flask deformity * Premature closure of growth plates * Paravertebral masses due to extramedullary hematopoiesis Vascular occlusion * Scattered bone sclerosis * H-shaped vertebral bodies * AVN less common than in sickle cell Other * Cardiomegaly and CHF * Secondary hemochromatosis Cholelithiasis

36 Radiographic features Hyperplasia of marrow is the dominant feature.
THALASSEMIA (COOLEY'S ANEMIA) Genetic disorder characterized by diminished synthesis of one of the globin chains. Radiographic features Hyperplasia of marrow is the dominant feature. * Expands the marrow space: hair-on-end skull * Modeling deformities of bone: Erlenmeyer flask deformity * Premature closure of growth plates * Paravertebral masses due to extramedullary hematopoiesis Vascular occlusion * Scattered bone sclerosis * H-shaped vertebral bodies * AVN less common than in sickle cell Other * Cardiomegaly and CHF * Secondary hemochromatosis Cholelithiasis

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