RICKETSRICKETS

LEARNING OBJECTIVESLEARNING OBJECTIVES• Define Rickets

• Enumerate types of Rickets

• Describe clinical features of nutritional Rickets

• Explain lab diagnosis and X-ray findings in nutritional rickets

• Describe preventive measures for nutritional rickets

• Enlist the post Rickets deformities in musculoskeletal system.

DEFINITIONDEFINITION

Rickets A disease of growing bones occurs in children before fusion of epiphysis due to un mineralized matrix at the growth plates..

OsteomalaciaOsteomalacia

Failure of mature bones to mineralize

due to

prolonged deficiency

dietary lack of vitamin D

or

lack of ultraviolet rays of sun.

VITAMIN D METABOLISMVITAMIN 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-hydroxycholicalciferol(25 OH-D)

• Converted in the kidney into 1-25-(OH)2-D• The most active metabolite of Vit D• It acts on GIT to increase calcium

absorption• On bone to increase calcium resorption• Parathyroid hormone activates Alpha-1

hydroxylase enzyme in the kidney.

Types of RicketsTypes of Rickets

• Vitamin D Deficient Rickets(nutritional)

• Vitamin D Dependent rickets

• Vitamin D Resistant Rickets

• Renal Rickets

• Hepatic Rickets

• Congenital Rickets

Normal bone developmentNormal bone development

• Bone consists of protein matrix –osteoid

• Mineral phase-calcium and phosphorus.

• Ossification

• Intramembranous ossification-flat bones

mesenchymal cells differentiate into osteoblasts

• Enchondral ossification –long tubular bones

Endochondral ossificationEndochondral ossification

• Growing cartilage at the epiphyseal plates

is mineralized and resorbed

and

replaced by osteoid matrix

which undergoes mineralization

to create bone.

In RicketsIn Rickets

Mineralization is delayed or inadequate

osteoid thickens and increase in

circumference of growth plate.

Softening of the bones-----Deformities

CLINICAL FEATURESCLINICAL FEATURES

• Peak incidence 6 months – 2 years• Irritability • profuse sweating while asleep• hypotonia • frequent respiratory infections.• Failure to thrive • Delay in walking,delayed dentition• Fits,tetany.

SIGNSSIGNS

HEAD

• Larger than normal.

• Frontal bossing (due to excess osteoid)

Craniotabes (ping pong ball sensation)

due to thinning of outer table of skull.

• Delayed closure of anterior fontanel

• caput quadratum (square like head)

THOREXTHOREX

• Rachitic Rosery (prominent costochondral junctions)

• Harrison’s sulcus (depression above the subcostal margin at the site of diaphragm) Pulling of softened ribs by the diaphragm during

inspiration.• Pigeon chest deformity.(The weakened ribs

bend inwards due to the pull of respiratory musclesand ,causing anterior protrusion of sternum.

ExtremitiesExtremities

1. Widening of wrists and ankles

2. Bending of long bones

results in

bow legs

knock knees,(genu valgum)

3. Green stick fractures

Widening of wrist jointsWidening of wrist joints

Widening of ankle jointsWidening of ankle joints

LAB DATALAB DATA

1.Serum Calcium low (normal 9-11mg/dl)

2.Serum phosphorus low (normal-5-7mg/dl

3.Alkaline phosphatase is raised.

This is the most striking feature,shows increased but ineffective activity of osteoblasts.

4. 25-(OH) D levels less than 20 ng/dl

Confirms of Vitamin D deficiency

TYPE 2 VIT D DEPENDENT TYPE 2 VIT D DEPENDENT

• End Organ resistance to effects to 1,25.(OH) –D3

• ALOPECIA

• 1-25(OH) VIT D is high

• TREATMENT

• Physiological doses of one alpha Leo

• 1-2 micrograms per day.

PREVENTIONPREVENTION

• To prevent rickets, health experts recommend

• a child should 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.

• Fish

PREVENTIONPREVENTION

• Daily intake of 400 i.u.vitamin D by supplemention.

• Lactating mothers should receive supplemention with milk or vitamin D to ensure prevention of rickets in their babies.

• Sun exposure to mothers.

• Calcium supplements such as lime can also be added to staple foods like rice and bread. Plenty of sunlight, fresh air and exercise are also necessary to ensure sufficient Vitamin D intake.

• A meeting of international experts on rickets held in Dhaka in 2006 identified community-based awareness as one of the most effective measures against the spread of rickets. Experts at the meeting said efforts to boost rice production over the years had influenced diets: there was less emphasis on calcium-rich foods such as dairy products and leafy green vegetables.

LEARNING OBJECTIVESLEARNING OBJECTIVES

• Describe clinical features of rickets

• Enumerate investigations of rickets

• Interpret investigation of rickets

• Describe management of rickets.

• Identify radiological findings in rickets, osteomalacia and osteoporosis

TREATMENTTREATMENT

• STOSS THERAPY• 300,000-600,000 units i/m• Indrop D 200,000 units• Repeat x-ray after 3 weeks• Another dose• HIGH DOSE VITAMIN D THERAPY• 2000-5000 IU/day over 4-6 weeks • Followed by intake o 400 I/U daily

VITAMIN D DEPENDENT VITAMIN D DEPENDENT RICKETSRICKETS

• Inborn error of vitamin D metabolism• Autosomal Recessive• Type 1 and 2• TYPE 1• Defect in 1 alpha-hydroxylase responsible for

the synthesis of 1-25-dihydroxy vit D• Symptoms in the 1st year of life• Tetany,convulsions,musle weakness andgrowth

failure

VITAMIN D RESISTANT RICKETSVITAMIN D RESISTANT RICKETS

• X linked dominant• Males are more severely effected than

females• Vitamin D activation & tubular

reabsorption of phosphate are impaired resulting in hypophosphatemia .

• TREATMENT• Oral Phosphate and 1 ,25-(OH)2 –D3

0.05micrograms /kg/day.

PREVENTIONPREVENTION

1.Exposure to sunlight (ultraviolet light)

Early morning and evening 30 minutes per week or 2 hours per week maintains adequate sun exposure.

2.Food fortified with Vit A and Vit D specially butter,ghee and milk.

Children under 5 should 500ml of milk daily or youghart or cheese daily.