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M.Sait  Gönen  MD Department M.Sait  Gönen  MD Department

M.Sait Gönen MD Department - PowerPoint Presentation

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M.Sait Gönen MD Department - PPT Presentation

of the Endocrinology and Metabolism Metabolic Bone Disorders amp Osteoporosis Metabolic Bone Disease 1Mineralization Osteomalacia Rickets 2Bone Turnover ID: 1045208

disease bone age osteoporosis bone disease osteoporosis age mineralization osteomalacia fracture formation increased secondary caused strength characterized risk vitamin

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1. M.Sait Gönen MDDepartment of the Endocrinology and MetabolismMetabolic Bone Disorders &Osteoporosis

2. Metabolic Bone Disease1-Mineralization: Osteomalacia/Rickets2-Bone Turnover: Pacets, primary hyperparathyroidism, Secondary Hyperparathyroidism3-Low Bone Content: Osteoporosis/ generalized and localized4-High Bone Content: Osteopetrosis

3. Osteitis fibrosa CysticaPaget’s disease of bone Osteogenesis imperfectaosteoporosisBone diseases caused by disturbed bone metabolismBone loss due to menopause in womenDecreasing bone mass with age in men

4. Osteitis Fibrosa CysticaBrown tumor of hyperparathyroidismExcessive bone resorption (mediated by osteoclast activity) Hyperparathyrodism—› İncreased PTH —›resorp bonePathology —› diminished bone strength Subperiostal (below connective tissue) resoption Bone cystsRadiology:cyst formation,mimics bone neoplasmsHistology:fibrous tissue,hemorrhage(hemosiderin pigment)Treatment:underlying cause of hyperparathyroidism

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6. Paget’s DiseaseA common disorder of unknow etiology that is characterized by excessive and abnormal remodeling of boneMen>womenAge >40 yearsPolyostotic>monostotic

7. High bone turnoverHigh blood flowDisordered bone architectureWeakness of bonePaget’s Disease

8. AsymptomaticBone pain, progressive bony enlargement, bowing of long bones, or fractures at presentationDeafness -from cranial-nerve compression at the skull base or from middle-ear ossicle involvementThe spinal cord compressed -basilar invagination of the skull or enlargement of the vertebrae Paget’s Disease

9. Paget’s DiseaseExcess bone resorption and formation Repetition and acceleration of cyclic osteon breakdown and rebulding Result in disorganised boneEpi: prevalence increases with age Pathology: larger, weaker, less dense, more vascular bones Affects axial skleteon and proximal long bones

10. Clinical: AP increase Symptoms:usually asemptomatic, but some get bone pain/fracture/deformity,arthritis, neuro complications(compression)Radiology:cotton wool spots(sclerosis,whiter areas)Nuc:increased uptake in affected bonesTreatment:bisphosphonates(antiresorptive)Paget’s Disease

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13. Osteogenesis imperfectaBrittle bone disease or lobstein syndromeİnadequate bone formation with defective bone matrices Due to mutation in bone collagen gene(>8 variants)Clinical:unexpected fractures, family hx, blue scleraDiagnose with DNA tests or collagen analysisTreatment: bisphosphonates, surgery, no cure

14. Diseases of Mineralization:Osteomalacia and RicketsOsteomalacia is a term that is used to encompass a group of disesases in adults, the essential feature of which is a delay in mineralization as osteoid is laid down.Ricets is the equivalent term used in children where the disease manifests particularly at the growth areas of bone

15. Osteomalacia and RicketsDisease is diagnosed by histology and in sever cases by radiologyEtiology is diagnosed by biochemistry, history and examination

16. Osteomalacia/ricketsİnaduquate bone formation with defective bone matrix mineralization Due to insufficient calcium or phosphorus Most frequently caused by vitamin D deficiency Also caused by chronic kidney disease —› kidney converts inactive to active vitamin D 25-OH-D —› 1,25-OH2-DPathology: softening of bone Adults(osteomalacia) —› bone pain, muscle weakness, fracture risk

17. Children(rickets) —› bone deformities(ie bowing), dental issuesTreatment: restore vitamin D levels( or active form calcitriol ,calcium and phosphorus if necessary

18. OsteoporosisRossini M et al, Reumatismo 2016Osteoporosis is a systemic skeletal disorder characterized by decreased bone mass and qualitative alterations (macro- and micro-architecture, bone material properties) associated with increased fracture risk. Primary osteoporosis is defined as osteoporosis occurring after menopause (postmenopausal osteoporosis) or with advancing age (senile osteoporosis). Secondary osteoporosis is caused by a number of disorders and drugs.Definition

19. OsteoporosisReduced bone matrix and mineralization secondary to various factors No single etyoloji —›50% hereditary, 50% acquired(diet,age,exercise,comorbidities)Epi: Most common metabolic bone disorderPathology: İncreased fragility, fractures Decrease in bone density(> -2,5 std dev) Osteopeni is milder form(-1 to -2,5 std dev)

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22. Definition of OsteoporosisA skeletal disorder characterized by -Compromised bone strength predisposing to -An increased risk of fractureBone strength reflects the integration of two main features: -Bone Density -Bone quality

23. Evaluating Bone StrengthOsteoporosis is defined as a skeletal disorder characterized by compromised bone predisposing a person to an increased risk of fracture. Bone strength primarily reflects the integration of BMD and bone quality

24. Bone mineral density(BMD) Measured with dual energy X-ray Absorptiometry (DEXA) scan T-score is std multipl difference from avg ethnicity,/gender-matched 30 yo Z-score is std dev multipl difference from avg ethnicity/gender/age-matched ptOsteoporosis

25. Generalized Cortical TrabecularLokalized Rheumatoid arthritisBone Marrow disease Myeloma secondary cancer lymphoma and leukemia HistiocytosisOsteoporosis

26. OsteoporosisPrimary Osteoporosisİnherint risks : fracture hx, female, white, age, genetics Modifiable risks: smoking, alcohol, low estrogen, low calcium,Vit D sedentary lifestyle low BMI

27. OsteoporosisSecondary Osteoporosis —›due to other disease, surgerios,medicationHormones: corticosteroids(net resoption); thyroid/growth hormone(net formation) Dzs: Cushing’s; hyper-parathyroid/thyroid/prolaktin; hypo-gonad/pituitary; gastric bypass and other malabsorbtion; Ehler Danlos; OI;myeloma;lymphoma/leukemia Drugs; ethanol,glucocorticoids,heparin

28. Clinical:kyphosis(hump-back), scoliosis(S-spine), height loss, gait/balance issuesRadiology: x-ray only detects 30-50% —›Dexa preferred

29. TreatmentCa,Vitamin D,Estrogen replacementCalcitonin,Selective estrogen reseptor modulators(raloxifene and tamoxifen), bisphosfonates, Denosumab(RANK ab)Teriparatide(PTH analog,anabolic)