Question
A 7 year old girl suffering from malabsorption,preseted with the following condition as shown in the X ray.Regarding this condition,which would be the earliest manifestation?
A. Craniotabes
B. Harrison groove
C. Pigeon chest
D. Delayed dentition
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Correct Answer » A Explanation |
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Ans:A. Craniotabes.
The patient in question is suffering from Rickets.
Image shows:
- First:Rickets of the knees demonstrates bowing of the femurs, metaphyseal cupping and fraying,coarsening of the trabecular pattern, increase in distance between end of shaft and epiphyseal center, poorly ossified epiphyseal centers.
- Second:There is cupping and fraying of all of the metaphyses (white arrows) in this skeletally-immature child.
RICKETS
- Osteomalacia during enchondral bone growth
- Age
- 4-18 months
Histology
- Zone of preparatory calcification does not form resulting in build-up of maturing cartilage cells
- Also occurs in shafts so that osteoid production elevates periosteum
Clinical findings
- Irritability
- Bone pain
- Tenderness
- Craniotabes:(areas of thinning and softening of bones of the skull) manifests early in infants with vitamin D deficiency
- Rachitic rosary
- Bowed legs
- Delayed dentition
- Swelling of wrists and ankles
Location
- Metaphyses of long bones subjected to stress are particularly involved
- Wrists
- Ankles
- Knees
Imaging findings
- Cupping and fraying of metaphysis
- Poorly mineralized epiphyseal centers with delayed appearance
- Irregular widened epiphyseal plates (increased osteoid)
- Increase in distance between end of shaft and epiphyseal center
- Cortical spurs projecting at right angles to metaphysis
- Coarse trabeculation (not the ground-glass pattern found in scurvy)
- Periosteal reaction may be present
- Deformities common
- Bowing of long bones
- Molding of epiphysis
- Fractures
- Frontal bossing
Causes Of Rickets
- Abnormality In Vitamin D Metabolism
- Associated with hyperparathyroidism
- Vitamin D deficiency
- Lack of sunshine exposure
- Malabsorption of vitamin D
- Defective conversion of vitamin D to 25-OH-cholecalciferol in liver
- Liver disease
- Defective conversion of 25-OH-D3 to 1,25-OH-D3 in kidney
- Chronic renal failure = renal osteodystrophy
- Vitamin D-dependent rickets = autosomal recessive enzyme defect of 1-OHase
- Abnormality In Phosphate Metabolism
- Calcium Deficiency