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

RicketsRickets

OsteomalaciaOsteomalacia

Defective Mineralization of CartilageDefective Mineralization of Cartilage Growth PlateGrowth Plate

Defective Mineralization of Bone Defective Mineralization of Bone MatrixMatrix

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 : NL

P:

P urinary clearance

BONE Demineralization

P:

Classification of Rickets & OsteomalaciaClassification 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 RicketsClinical Manifestation in Rickets

o Skull

o Swelling Epiphysis at wristo Richet,s rossaryo Harrison,s Sulcuso Bow or Knock Kneeo Wind Swept Lego Ricket , s Myopathyo Pelvic Deformity

CraniotabesCranial 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 )

RicketsRicketsLeg bowingLeg bowing

Hereditary resistance to vit D

Clinical Manifestation in OsteomalaciaClinical Manifestation in Osteomalacia

o generalized Bone Pain

o Localized Bone Pain in Groino Osteomalacia Myopathyo Secondary Osteoarthritis o Marked enthesopathy o waddling gait

SpineRibesPelvis

Sacroiliac Wrist Knee Facet Joints

Radiology Of osteomalacia or ricketsRadiology 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 & FractureBowing & Fracture

Fraying& WideningFraying& Widening

Hypophosphatemic osteomalacia

Fraying

Loozer ZoneLoozer Zone

Loozer ZoneLoozer Zone

Loozer ZoneLoozer Zone

Pseudofracture

OsteomalaciaOsteomalacia

Biochemical Change in OsteomalaciaBiochemical Change in Osteomalacia

1- ca P = Nl Alk ph1- ca P = Nl Alk ph2- ca = Nl P Alk ph2- ca = Nl P Alk ph3- ca P Alk ph3- ca P Alk ph

24 h Urinary ca < 100 mg / 24 h24 h Urinary Hydroxyproline Excretion

Work up for OsteomalaciaWork up for Osteomalacia

Ca , P , Alk ph Ca , P , Alk ph 24 h urinary Ca24 h urinary Ca 25 ( OH ) D25 ( OH ) D 1 , 25 ( OH ) D1 , 25 ( OH ) D PTHPTH Bone BiopsyBone Biopsy

Treatment of osteomalacaTreatment 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 orμ 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)