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RicketsOsteomalacia Defective Mineralization of Cartilage Growth Plate Growth Plate Defective Mineralization of Bone Matrix.

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Presentation on theme: "RicketsOsteomalacia Defective Mineralization of Cartilage Growth Plate Growth Plate Defective Mineralization of Bone Matrix."— Presentation transcript:

1 RicketsOsteomalacia Defective Mineralization of Cartilage Growth Plate Growth Plate Defective Mineralization of Bone Matrix

2 7 dehydrocholesterol Pre- vitamin D 3 Vitamin D 3 Dietary source : Vit D2 (ergocalciferol) Vit D3 (cholecalciferol)

3 Vitamin D

4 Function of vit D

5 Lack of VIT-D Intestinal Ca, p absorption Hypocalcemia PTH secretion Ca released from bone Ca : NLP: P urinary clearanc e BONE Demineralization P:

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11 Classification of Rickets & Osteomalacia Vit D deficiency GI disorder Disorder of vit D metabolism Acidosis CRF Generalized renal tubular disorder Primary Mineralization defect Defective Matrix synthetase Others

12 Clinical Manifestation in Rickets o Skull o Swelling Epiphysis at wrist o Richet,s rossary o Harrison,s Sulcus o Bow or Knock Knee o Wind Swept Leg o Ricket, s Myopathy o Pelvic Deformity Craniotabes Cranial Vault

13 SKULL Parietal flattening Frontal bossing Softening of calvariae Widening of suture

14 Prominance of costocondral junction ( rachitic rosary )

15 Indentation of lower ribs at the site of attachment to diaphragm ( harrison’s groove )

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17 Rickets Leg bowing

18 Hereditary resistance to vit D

19 Clinical Manifestation in Osteomalacia o generalized Bone Pain o Localized Bone Pain in Groin o Osteomalacia Myopathy o Secondary Osteoarthritis o Marked enthesopathy o waddling gait Spine Ribes Pelvis Sacroiliac Wrist Knee Facet Joints

20 Radiology Of osteomalacia or rickets Craniotabes Widening, cupping and ragging of Growth plate Bowing of Leg pelvic Abnormalities Kyphoscoliosis Bone fractures ( spine ) Looser Zone protruacetabuli

21 Widening

22 Bowing & Fracture

23 Fraying& Widening

24 Hypophosphatemic osteomalacia

25 Fraying

26 Loozer Zone

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29 Pseudofracture

30 Osteomalacia

31 Biochemical Change in Osteomalacia 1- ca P = Nl Alk ph 2- ca = Nl P Alk ph 3- ca P Alk ph 24 h Urinary ca < 100 mg / 24 h 24 h Urinary Hydroxyproline Excretion

32 Work up for Osteomalacia Ca, P, Alk ph Ca, P, Alk ph 24 h urinary Ca 24 h urinary Ca 25 ( OH ) D 25 ( OH ) D 1, 25 ( OH ) D 1, 25 ( OH ) D PTH PTH Bone Biopsy Bone Biopsy

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34 Treatment of osteomalaca Sun light or Dietary Children with tetany Malabsorption Anticonvulsant RTA Nephrotic Syn CRF( ( Vit D 800 – 4000 IU / d ) ( Vit D 800 – 4000 IU / d ) ( Ca + Large dose Vit D ) ( Vit D 50000- 100000 IU / d or μ 0/5 – 1 Calcitriol + 15 g / d calcium lactate or 4g/d calcium carbonate ) ( Vit D 1000 IU / d ) ( Inorganic Phosphate 1- 3/6 g / d + μ 0/5 – 2 Calcitriol / d ) ( Vit D 800-1000 IU / d ) μ 0/5 – 1 Calcitriol / d μ1-2/5 Calcitriol 3 / W in dialysed patient)

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