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)