a 37-year-old man with bow-leg & kyphoscoliosis
DESCRIPTION
A case presented in Medicine Grand Round in BSMMU on 2nd June, 2013 by Dr. Suman Kanti Chowdhury (FCPS course student)TRANSCRIPT
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A 37-year-old man with bow-leg & kyphoscoliosis
PresenterDr. Suman Kanti Chowdhury
FCPS course studentDepartment of Endocrinology
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Particulars of the patient
• 37-year
• Male
• Unmarried
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Chief complaints
• Pain in lower limb with difficulty in walking &
progressive loss of height for last 11 years
• Progressive chest deformity for last 6 years
• Bowing of both legs & shortness of breath for last 1
year
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H/O presenting complaints:
• Bony pain
– Started 11 years back
– 1st felt stress pain in right foot during walking & relieved
after rest, included left foot 1 & ½ months later
– Extended progressively at both ankle, leg, knee, thigh &
finally at lower back region with increasing severity
– Felt only immediately after start to walk & relieved at rest
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H/O presenting complaints: cont…
– For last 7 years, cant walk un-aided & cant
stand from squatting position & climb up
stairs
– No H/O joint swelling or morning stiffness
or claudication distance
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H/O presenting complaints: cont…
• Chest deformity – 7 years back, developed progressive deformity – associated pain over upper back of chest without
any radiation but good response to painkiller
• Exertional dyspnoea– For last 1 year, developed exertional dyspnoea – felt after walking around 15 minutes with cratch
without any central chest pain, orthopnoea, cough or hemoptysis
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H/O presenting complaints: cont…
• Bowing of both legs– for last 1 year
• Loss of height – Within this 11 years, lost 28 cm of his height
• Weight loss– Within this 11 years, 20.5 kg of his weight
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H/O presenting complaints: cont…
• No H/O
– generalized swelling, change in urine volume or
prolong fever,
– Steatorrhoea , lacking of sunlight exposure,
– not used to take Aluminium containing drugs, long
time anticonvulsant therapy & fluoride containing
water before this illness
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• H/O past illness – nothing significant
• Family history – 1 brother & 1 sister, mother is alive,
father died of CVD
• Social history - average diet pertaining to lower
middle class with adequate intake of calcium & vit D.
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Drug historyName Dose Frequency Duration
Cap rocaltrol (Calcitriol) 0.25 µg 12 hourly For 1-2 month infrequently
Calcium & vit D 500 mg calcium+ Vit D
1 tab 12 hourly
From 2006-2013
Tab salazine (sulfsalazine) 500 mg 12 hourly For 2 months at 2003
Tab Nodia (leflunomide) 10 mg 2 tab daily From 22.4.2006 for 1 & ½ years
Tab alendronate 70 mg weekly For 2 months at 2006
Cap Bonviva (Ibandronate) 150 mg 1 cap monthly From 30.5.2007 for 6 months
Tab Risefos (risedronate) 35mg weekly From dec,2007 for 2 years
Inj Arachitol (cholecalciferol) 6 lakh unit weekly From 26.12.2007-27.9.2008
Minirin spray (calcitonin) 200 U intranasaly
Daily For 8 months at march,07
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General examPulse – 76/min, peripheral pulses - intact BP – 120/80 mm of HgTemp-normal,RR- 18/minCyanosis-absent
Weight- 38.5 kg, Height - 140 cm, BMI - 19.64.No lymphadenopathy, thyroid-not palpable.
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Musculoskeletal examination
Kyphoscoliosis of dorsal spine, lumber lordosis
Genu varus deformity,
Planter arch- Right flat foot, Left foot arch-height reduced,
No joint swelling or deformity
True leg length- 68cm(right), 69cm(left)
Apparent leg length- 85cm(right), 86cm(left)
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Gait- cant walk without aidArm- muscle wasting present, movement not restricted,No joint swelling or deformitySpine-movement restriction in dorsal & lumber spine due to deformityLeg-muscle wasting present, movement not restricted, No joint swelling.
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• Respiratory system examination:
Kyphoscoliosis of D/S, expansion- 5 cm,
Ant-post -19 cm, Transverse - 28 cm
Percussion – resonant, vocal resonant-normal,
vesicular breath sound, no added sound
• Nervous system examination:
Muscle power- 4/5 over lower limbs,
Muscle bulk-reduced, Muscle tone, Jerks- normal.
Planter - flexor B/L, Sensory, cerebellar function-normal.
cranial nerves- intact, Fundoscopy - Normal
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Past
Photographs were used with permission of the patient
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Present
Photographs were used with permission of the patient
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Chest deformity
Photographs were used with permission of the patient
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Bow leg and flat feet
Photographs were used with permission of the patient
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Provisional diagnosis
Osteomalacia due to-
Vit D – resistant rickets type IVit D – resistant rickets type IIHypophosphataemic ricketsHypophosphatasia
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Investigations
• CBC (18.5.13): Hb - 14.2 gm/dl, ESR – 5 mm in 1st hour, WBC – 5000/cmm, platelet- 2,70,000/cmm,N-53%, L-40%, M-5%, E-2%.
• Urine R/E (18.5.13)– normal.
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InvestigationsInvestigation
18.5.2013 26.9.2012 17.7.7 26.12.2007 27.9.2008 23.10.2007
S Ca 7.8 mg/dl 9.2 mg/dl 8.4 mg/dl(8.5-10.5)
PTH 124 pg/ml(15-68 pg/ml)
148 pg/ml (11-67 pg/ml)
S. PO4 2 mg/dl 1.1 mg/dl 0.9 mg/dl(2.5-4.5 mg/dl)
S ALP 334 U/L(50-136U/L)
415 U/L(38-126 U/L)
455 U/L(32-92)
1.25(OH)2D3
34.5 pg/ml(29.6-65.1 pg/ml)
64 pg/ml(19.6-54.3 pg/ml)
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InvestigationsInvestigation 18.5.2013 26.9.2012 17.7.7 8.1.2008
24 hr urinary ca 32.4 mg/day(100-300 mg/day)
24 hr urinary PO4
236.72 mg/dl(400-1300 mg/day)
S. electrolyte Na-138, K-4.1, Cl-105, Co2-26 mmol/l
S. creatinine 0.6 mg/dl 0.5 mg/dl
S. magnesium 2.2 mg/dl(1.8-2.5 mg/dl)
s. albumin 37 gm/l
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InvestigationsInvestigation 2.2.04 4.2.2004 13.2.2006
SGPT 36 U/L
SGOT 25
S bilirubin 0.47 mg/dl
Bld urea 18 mg/dl
S uric acid 3.6 mg/dl
RA test Negative Negative
ANA Negative
CRP Negative Negative
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InvestigationsInvestigation 1.12.2005 13.2.2006 18.5.2013
TSH 1.44 µIU/mL(0 .27- 4.2 µIU/mL)
FT4 1.05 ng/dl (0.8-1.9 ng/dl)
CPK 75.6 U/L (24-190 U/L)
RBS 4 mmol/L
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Investigations• X-ray of pelvis : (21.5.13)Pelvis is deformed. Both the hip joints are protruded
medially into the pelvis cavity. Multiple fractures are seen at both rami of both pubis & both femoral neck with superior displacement at both greater trochanter.
• X-ray of both knee – lat view: (21.5.13)Bone density is reduced. visible joints appear normal.
Evidence of muscular atrophy is seen.
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Investigations• X-ray of both legs with ankle joints: (26.5.13)Osteopenia with old fracture of left fibula & lower end
of both tibia.
• CXR P/A view: (26.5.13)Multiple ribs fractures at right 2nd,6th,7th & left 2nd,7th,9th
& 10th ribs.
• X-ray of both hands: (26.5.13)Osteopenia with multiple fractures at right 1st & 2nd
metacarpal bones.
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Investigations• X-ray dorso-lumber spine (B/V): (18.5.13)Compression collapse at multiple dorsal vertebrae.
• Xray Lumbo-sacral spine(B/V): (18.5.13)Collapse of L2,L3 & L4 & deformed sacrum.
Xray skull (lat view): (18.5.13)Normal.Xray KUB region: (18.5.13)Normal.
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• USG of neck (26.9.2012) : Slightly hypoechoic right inferior parathyroid gland.• Nerve conduction study upper & lower limbs
(2.2.2004) – normal.• BMD (22.10.2007)– T score -2.8, Z score – 2.3.• Spirometry: (27.5.13) Severe restrictive abnormality.
Pred. Best % Pred
FVC (L) 2.65 0.96 36
FEV1 (L) 2.36 0.90 38
FEV1/FVC (%)
80.2 93.7 116.9
PEF (L/sec) 7.01 2.49 35.5
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Problem list:
• Diagnostic dilemma
• Future management plan
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Thank you