RICKETS

DEFINITION

A disease of growing bones occurs in children

before fusion of epophysis due to un
mineralized matrix at the gwoth plate
VITAMIN D METABOLISM

Maintenance of normal
plasma levels of
calcium & phosphorus

Two forms of Vit D are

present
1. Vit D2 (Ergocalciferol)
2. Vit D3 (Cholecalciferol)
 In liver it is hydroxylated into 25-
hydroxycholecalciferol (25 OH-D)
 Converted in the kidney into 1-25-(OH)2-D
 The most active metabolite in vitamin D
 It acts on GIT to increase calcium absorbtion
 On bone to increase calcium resorption
 Parathyroid hormone activates Alpha-1
hydroxylase enzyme in the kidney
Types of Rickets

 Vitamin D deficient Rickets (Nutritional)

 Vitamin D dependent Rickets
 Vitamin D resistant Rickets
 Renal Rickets
 Hepatic Rickets
 Congenital Rickets
Normal bone development

 Bone consist of protein matrix –osteoid

 Mineral phase-calcium and phosphorus
 Ossification :
1. Intramembranous Ossification - flat bones
mesenchymal cells differentiate into osteblas
2. Endochondral Ossification – long tubular bone
Endochondral Ossification

Growing cartilage at epiphyseal plates is

mineralized and resorbed and replaced by
osteoid matrix which undergoes
mineralization to create bone
In Rickets

Mineralization is delayed or inadequate osteoid

thickens and increase in circumference of
growth plate

Softening of the bones -----Deformities

CLINICAL FEATURES

 Peak incidence 6 months – 2 year

 Irritability
 Profuse sweating while asleep
 Hypotonia
 Frequent respiratory infection
 Failure to thrive
 Delay in walking, delayed dentition
 Fits, tetany
SIGNS

HEAD :
 Larger than normal
 Frontal bossing (due to excess osteoid)
 Craniotabes (ping pong ball sensation) due to
thinning of outer table skull
 Delayed closure of anterior fontanel
 Caput quadratum (square like head)
THORAX

 Rachitic Rosery (prominent costochondral

junctions)
 Harrison sulcus (depression above the
subcostal margin at the site of diaphragm)
pulling of softened ribs by the diaphragm
during inspiration
 Pigeon chest deformity ( The weekend ribs
bend inward due to the pull of respiratory
muscle and causing anterior protrusion of
sternum
Extremities

 Widening of wrist and ankle

 Bending long bones result in bow leg, knock
knee
 Green stick fracture
LABORATORIUM

 Serum calcium low (N = 9-11mg/dl)

 Serum phosporous low (N= 5-7 mg/dl)
 Alkaline phosphatase is raised
 25-(OH) D level less than 20 ng/dl confirm of
vitamin D deficiency
PREVENTION
 A child shoul be breast fed
 Weaned and put on to cow’s milk and other
foods rich in vitamin D and calcium, like eggs and
dairy products such as butter and leafy
vegetables
 Consume fish
 Daily intake of 400 i.u vitamin D by
supplementation
 Sun exposure to mother
 Lactating mother should receive suplementation
to prevention rickets in their babies
TREATMENT

 STOSS THERAPY 300.000-600.000 i.u oral

 Indrop D 200.000 u.i
 Repeat X ray after 3 weeks
 HIGH DOSE VITAMIN D THERAPY 2000-5000
IU/day over 4-6 week, followed by intake 400
IU daily
VITAMIN D DEPENDENT RICKETS

 Inborn error of vitamin D metabolism

 Autosomal recessive
 Type 1 and 2
 Type 1 :
Defect in 1 alpha-hydroxylase responsible for
the syntesis of 1-25-dihydroxy vit D
 Symptom in the 1 year of life
 Tetany, convulsion, muscle weakness and
growth failure
VITAMIN D DEPENDENT RICKETS

 Type 2 :
- End organ resistance to effect to 1,25(OH)-D3
- Alopecia
- 1-25(OH) Vit D is high

Treatment :
 Supra Physiological doses of 1,25(OH)2 Vitamin D3
 High dose oral or intravenous calcium
VITAMIN D RESISTANT RICKETS

 X linked dominant
 Males are more severely effected than female
 Vitamin D activation & tubular reabsorbtion
of phosphate are impaired resulting in
hypophosphatemia
 Treatment :
Oral phosphate and 1,25 (OH)2 D3 0,05
microgram/Kg/day