Docstoc

RICKETS

Document Sample
RICKETS Powered By Docstoc
					                            Submitted to thewingars by
                            Jahaanzeb Mughal.




        RICKETS
    PROF SAMIYA NAEEMULLAH
Diplomat American Board Of Pediatrics,
           FAAP’FCPS.
     Head of department of pediatrics
Islamic International Medical College Trust
      LEARNING OBJECTIVES
•   Identify cases of rickets clinically
•   Explain Lab Diagnosis
•   X-ray findings
•   Enumerate preventive measures
•   Define osteomalacia
              DEFINITION
              Rickets
    A disease of growing bones
         occurs in children
      before fusion of epiphysis
               due to
        un mineralized matrix
        at the growth plates.
.
          Osteomalacia

Failure of mature bones to mineralize
             due to
       prolonged deficiency
      dietary lack of vitamin D
               or
    lack of ultraviolet rays of sun.
  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)
             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 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 ossification
• Growing cartilage at the 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 years
•   Irritability
•   profuse sweating while asleep
•   hypotonia
•   frequent respiratory infections.
•   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 of skull.
•   Delayed closure of anterior fontanel
•   caput quadratum (square like head)
                  THOREX
• 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.
              Extremities
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 joints
Widening of ankle joints
               LAB 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
              TREATMENT
•   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
            RICKETS
• 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
  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.
VITAMIN 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.
            PREVENTION
• 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
            PREVENTION

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.
             PREVENTION

• 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.
• 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.

				
DOCUMENT INFO
Shared By:
Categories:
Tags:
Stats:
views:649
posted:7/27/2011
language:English
pages:39