a case of recurrent low trauma fracture
DESCRIPTION
Case presentation on 1st October, 2013TRANSCRIPT
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Dr. Sonjoy Dey Resident, Phase A (Hepatology)
Dr. Md. Zahidul Amin FCPS Student
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Master P male 11 years From c
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Pain in the front of chest and lower back for 5 days
History of repeated fracture at multiple sites from infancy
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According to the statement of the patient he was relatively alright up to last Sunday
When he was playing in school , he felt into ground & felt pain in the front of the chest and lower back region
The pain was sharp catching localized in nature aggravate in activity and relieved by rest .
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According to the statement of his mother her baby suffered from fracture in left lower forearm without any trauma at the age of the 6 days .
Then he suffered from repeated fracture in multiple sites over the next one year involving rt upper forearm , rt mid forearm & left upper thigh .
Last fracture occurred at the age of 2 year . in every occasion he was treated with plaster immobilization .
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His birth was uneventful through normal per vaginal delivery .
He had normal weight gain throughout the childhood . His appetite was normal , no history of altered bowel
habit. He had no history of inadequate sun light exposure or
childhood abuse . There was no history of abnormal dentations or hearing
difficulty.
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He had 2 sisters , no one was suffering from such disease .
No maternal and paternal uncles and aunts had suffered from such illness .
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Appearance – normal Built – normal Nutrition – average Anaemia : absent Jaundice : absent Pulse : 84/ min RR :16/min Temp : 98.4 F
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Height :128 cm Weight : 43 kg BMI : 25.84
Skin & nail : healthy No lymph node palpable No dental abnormality
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There is deformity in rt arm . No shortening of any limb Tenderness over rt 5th rib in mid clavicular line & over 2nd
lumber spine . Gait –Normal Leg - Normal Spine – normal Range of movement is normal in all joints
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Higher cerebral function and memory normal All cranial nerves are normal No conductive or sensory neuronal deafness Fundoscopy normal Sensory and motor system intact .
Examination of other systems reveals no abnormality
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RicketsHyperparathyroidism Osteogenesis ImperfectaChildhood Paget’s disease
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Date Name of inv result
1 24/09/13 CBC Hb:12.1gm/dl , ESR :45 mm in 1st hourTc:9000/cmm , N :65% ,L:29% ,Pl count 2,40,000/cmm
2 24/09/13 Urine RME Protein : nil, sugar: nil , Pus cell :3-6/HPF ,RBC: nil , epi cell : 3-5/HPF
3 24/09/13 RBS 4.4 mmol/L
4 24/09/13 S creatinine 0.6 mg/dl
5 24/09/13 S calcium 9.4 mg/dl
6 24/09/13 S alkaline phosphatase
378 U/L (50 -135U/L)
7 24/09/13 S inorganic phosphate
4.2 mg/dl
8 24/09/13 S albumin 45 gm/L
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Date Name of inv result
9 24/09/13 Usg of whole abdomen normal
10 24/09/13 Chest X-ray PA view normal
11 24/09/13 X-ray lumbo sacral spine normal
12 24/09/13 S PTH Pending….
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Date Name of inv result
1 27/12/05 CBC Hb:10.6gm/dl , ESR :25 mm in 1st hourTc:10,500/cmm , N :50% ,L:45% ,Pl count 2,40,000/cmm
2 27/12/05 PBF Non specific
3 27/12/05 S bilirubin T .5mg/dl
4 27/12/05 S creatinine 0.8 mg/dl
5 27/12/05 S calcium 9.5 mg/dl
6 27/12/05 S inorganic phosphate
5.1mg/dl
7 27/12/05 SGPT 22U/L
8 27/12/05 S electrolytes Na : 143 , k : 3.8 , cl :106 , Tco2 :24.50
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Date Name of inv result
1 27/12/05 X-ray of both Wrist , elbow and forearm
Suggestive of Scurvy
2 16/06/11 X ray pelvis with both hip A/P view
Femoral head & neck is wide, osteopanic with thin outer cortex . Sclerotic reaction is seen in acetabulum mostly in upper part . Suggestive of Osteogenesis Imperfecta
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What the actual diagnosis? What should be the next management plan?
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Recurrent fracture with any one of the following: Blue sclerae
Dentinogenesis Imperfecta Family H/O disease
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No curative RX Supportive treatment Avoid contact sports Sweeming Physiotherapy Psychological support Surgery Regular follow up
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THANK YOU
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Oral alendronate — The effect of daily oral alendronate (5 mg or 10 mg based on body weight: below or above 40 kg, respectively)