Uploaded on

 

  • Full Name Full Name Comment goes here.
    Are you sure you want to
    Your message goes here
    Be the first to comment
No Downloads

Views

Total Views
1,240
On Slideshare
0
From Embeds
0
Number of Embeds
0

Actions

Shares
Downloads
23
Comments
0
Likes
1

Embeds 0

No embeds

Report content

Flagged as inappropriate Flag as inappropriate
Flag as inappropriate

Select your reason for flagging this presentation as inappropriate.

Cancel
    No notes for slide

Transcript

  • 1. Case 1 Jagdish K Prof. Dr. A. Gowrishankar’s unit
  • 2.
    • A 45yrs old lady presented to the opd with the chief complaints of
      • Pain in the knee, low backache, pain in the thigh for the past 1yr.
      • Breathlessness on & off for 6months .
  • 3.
    • Patient was apparently alright 1yr ago following
    • which patient developed pain in those areas,
    • insidious in onset, gradually progressive, no specific
    • character attributed. No specific aggravating / relieving factors .
  • 4.
    • Patient has breathlessness for the past 6months, while
    • doing her household activity and increases more
    • during exertion & relieved by rest.
    • Patient complains of occasional light headedness
  • 5.
    • No h/o
      • Chest pain
      • Palpitation
      • Syncope
      • Presyncope
      • Abdominal pain
      • Nausea
      • Vomiting/ diarrhoea/ constipation/ bleeding from orifices.
  • 6.
    • No h/o
      • Diplopia
      • Dysphagia
      • Dysarthria
      • Motor deficits
      • Sensory deficits
      • Unconsciousness/ altered sensorium
      • Involuntary movements
  • 7.
    • Attained menopause 2 yrs back, cycles regular during premenstrual period. No gynaec complaints in the post menopausal period
    • Vegan by diet,
    • Not a k/c/o DM/SHT/TB/Asthma
  • 8.
    • Patient has lost 10-15 cm of height in the past 2- 3 yrs.
  • 9. Examination
    • Comfortable at rest,
    • Conscious, oriented, afebrile
    • No pallor/ icterus/ cyanosis/ clubbing/ lymphadenopathy/ edema
    • Pulse: 110/min;
    • BP: 120/60 mm of hg, rt arm, supine position, SBP 130 mm of hg in lower limb by palpatory method.
    • JVP not elevated
    • RR : 16/min
  • 10.
    • Patient has fixed flexion deformity of hips
    • She has kyphosis , no tenderness of spine
    • There is Genu varum / tibia vara .
    • Other systems : normal
  • 11.  
  • 12.  
  • 13.  
  • 14.
    • Problems:
      • Bone pain
      • Loss of height
      • Breathlessness
      • Limb deformities
      • Probable High output state
  • 15. Investigations
    • CBC
      • Hb: 12.2
      • TC : 7,200
      • DC : P60 L 40
      • ESR : 6/12
      • PCV : 36%
      • Plt: 1.5
      • MCV: 32
      • MCH: 29
    • RFT
      • RBS: 84
      • Urea: 23
      • Creatinine: 0.6
    • Serum electrolytes:
      • Na + : 136
      • K + : 4.5
      • Cl - : 96
      • HCO 3- : 24
  • 16.
    • Peripheral smear : normocytic normochromic blood picture, adequate RBCs, WBCs & platelets
    • ECG : sinus tachycardia
  • 17. CXR
  • 18. X ray LS spine
  • 19.  
  • 20.  
  • 21. X ray skull
  • 22.
    • Serum calcium : 8.8 mg/dl ( total & corrected)
    • Phosphate : 4 (N:2.4-4.1mg/dl)
    • ALP : 880 (N: 40-140 U/L)
    • PTH : 55 pg/ml (N:10-60 pg/ml)
    • Vitamin D3: 40 (N: 20-76 pg/ml)
  • 23.
    • TFT :
      • T3 : 196 (N: 75 - 220 ng/dL)
      • T4 : 7.2 (N: 4 - 11 μg/dL)
      • TSH : 3.8 (N: 0.5-5.0 mIU/L)
    • RBC transketolase: 500 (N:440± 120 μ g/ml/hr )
  • 24.
    • ECHO :
      • EF 66%
      • No RWMA
      • Normal LV systolic function
      • Calcific aortic sclerosis
    • ABG : mild respiratory alkalosis
    • PFT : normal
  • 25.
    • Pelvic bone biopsy:
      • Increase in no & nuclei of osteoclasts
      • Jigsaw puzzle pattern appreciated
      • Picture consistent with Paget’s.
  • 26. Pagets disease
    • Sir James Paget first described it due to an inflammatory process
    • Used to be called as osteitis deformans , now called as osteodystrophica deformans.
  • 27.
    • A chronic disorder that can present as enlarged & misshapen bones
    • Excessive breakdown & formation of bone causes affected bone to weaken, presenting with pain, misshapen bone, fracture & osteoarthritis.
    • Disease typically affects one or few bones as opposed to osteopetrosis which affects all bones.
  • 28.
    • Rarely diagnosed before 40 yrs
    • Prevalence 1.5 – 8% depending on the age & the local prevalence
  • 29. Etiology
    • Viral
      • Paget's disease may be caused by a slow virus infection (i.e ., paramyxoviruses ) present for many years before symptoms appear.
      • Measles though recent evidence has cast some doubt upon the measles association.[4]
      • Canine distemper virus
      • Respiratory syncytial virus
  • 30.
    • Genetic
    Name OMIM Locus Gene PDB1 167250 6p ? PDB2 18q22.1 RANK PDB3 5q35 SQSTM1 PDB4 606263 5q31 ?
  • 31. Pathogenesis
    • Increased no & size of osteoclasts
    • Osteoclasts hypersensitive to Vitamin D3 & RANK ligand
    • Marrow stromal cell – increased expression of RANK-Ligand.
  • 32. Pathogenesis
    • Phases
      • 1 : osteoclastic
      • 2 : osteoclastic & osteoblastic
      • 3 : exhaustive & burnt out phase
      • All three phases may be seen at same time at different sites.
  • 33. Symptoms
    • Bone pain is the most common symptom. It can occur in any bone affected by Paget's disease. It often localizes to areas adjacent to the joints.
    • Headaches and hearing loss may occur when Paget's disease affects the skull.
    • Pressure on nerves may occur when Paget's disease affects the skull or spine .
  • 34. Symptoms
    • Hip pain may occur when Paget's disease affects
    • the pelvis or thighbone.
    • Damage to joint cartilage may lead to arthritis.
    • Teeth may spread intraorally due to the intraoral force placed on the anterior teeth (especially maxillary central and lateral incisors) by the labial tissues, especially the muscles.
  • 35. Symptoms
    • Somnolence (drowsiness) may be due to vascular steal syndrome of the skull .
    • Paralysis may be due to vascular steal syndrome of the vertebrae.
    • Increased head size, bowing of limb, or curvature of spine may occur in advanced cases.
    • Hypercementosis in teeth may occur.
  • 36. Diagnosis
    • Calcium
    • Phosphate
    • ALP
    • Hydroxyproline
    • N telopeptide
    • C telopeptide
  • 37. Differential diagnosis
    • Fibrogenesis imperfecta ostium
    • Osteoblastic mets
    • Sickle cell anemia
    • Osteopetrosis
    • Pyknodysostosis
  • 38. Treatment
    • Decision about treating patients can be complicated because
      • No two people are affected exactly the same way by the disease
      • Difficulty to predict the progress.
  • 39. When to treat???
    • Bone pain
    • Headache
    • Nerve related symptoms
    • Elevated SAP
    • High output state
  • 40. Drugs
    • Oral:
      • Etidronate : 200-400mg OD for 6months
        • Precautions
      • Alendronate
      • Tiledronate
      • Risendronate
  • 41. Drugs
    • IV
      • Pamidronate: 30-60 mg iv in NS OD for 3days
      • Zolendronate : 5mg infusion in NS stat. once a year dose.
  • 42.
    • Calcitonin : s/c thrice a week for 18months. Nasal spray not approved for treatment of Paget’s but for osteoporosis.
    • Surgery
  • 43. Calcium levels
    • Normal
    • Hypo
    • Hyper
  • 44. Diet & Exercise
    • 1000-1500 mg of calcium
    • Adequate sunshine
    • 400 units of Vitamin D3
    • Especially when treating with bisphosphonates.
    • Administration of calcium & bisphosphonates should be separated by 2 hours.
  • 45. Prognosis
    • The outlook is generally good, particularly if treatment is given earlier.
    • Earlier the disease onset,morbidity is more ;
    • Disease does not spread to normal bones
    • Treatment can control Paget's disease and lessen symptoms, but is not a cure
    • Osteogenic sarcoma can occur
    • Life expectancy is not altered .
  • 46. Physician & Paget’s disease
    • Headache
    • Nerve problems
    • Vascular steal phenomenon
    • calcific aortic valve sclerosis;
    • High output state
  • 47. Ivory vertebra
  • 48. Picture frame vertebra
  • 49. Osteoporosis circumscripta
  • 50. carry home points
    • Middle age – elderly ;
    • Bone pain,deformity ;nerve compression ;high output state;
    • Increased osteoclastic resorption ,compensatory osteoblastic activity ;
    • Normal electrolytes ;increased ALP & increased urinary markers ;
    • Bisphosphonates are the treatment ;
    • Prognosis is usually good ;
    • ZOLENDRONATE IS AVAILABLE IN STANLEY