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

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Presentation transcript:

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

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

Vitamin D

Function of vit D

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

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

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

SKULL Parietal flattening Frontal bossing Softening of calvariae Widening of suture

Prominance of costocondral junction ( rachitic rosary )

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

Rickets Leg bowing

Hereditary resistance to vit D

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

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

Widening

Bowing & Fracture

Fraying& Widening

Hypophosphatemic osteomalacia

Fraying

Loozer Zone

Pseudofracture

Osteomalacia

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

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

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 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 IU / d ) μ 0/5 – 1 Calcitriol / d μ1-2/5 Calcitriol 3 / W in dialysed patient)