rickets
TRANSCRIPT
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ABNORMALITIES OF BONE IN CALCIUM
DEFICIENCYCMCH
DEPARTMENT OF ORTHOPAEDICS
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RICKETS
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RICKETS IS A METABOLIC BONE DISEASE UNIQUE TO CHILDREN AND ADOLESCENTS, RESULTING FROM INADEQUATE MINERALISATION OF OSTEOID
IT IS THE COMMONEST METABOLIC BONE DISEASE IN CHILDREN AND FREQUENTLY ATTENDED BY DEFORMITIES .
INTRODUCTION
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COMMONEST METABOLIC BONE DISEASE GLOBALLY
NUTRITIONAL TYPE IS COMMONEST IN AFRICA, MIDDLE EAST & SE ASIA
RENAL RICKETS COMMONEST IN THE WEST
INCIDENCE RISING IN THE WEST DUE TO INCREASED USE OF SUNSCREEN LOTIONS & MORE CHILDREN STAYING INDOORS AND WATCHING TV OR PLAYING VIDEO GAMES
EPIDEMIOLOGY
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METABOLIC BONE PHYSIOLOGY – BONE COMPOSITION
BONE
MATRIX
COLLAGEN
GROUND SUBSTANCE
MUCOPOLYSACCHARIDES (CHONDROITIN SULFATE)
PROTEINS (OSTEOPONTIN, OSTEOCALCIN,
OSTEONECTIN, BMP)
MINERALS (CALCIUM, PHOSPHATE, MAGNESIUM)
CELLS
OSTEOBLASTS
OSTEOCLASTS
OSTEOCYTES
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SOURCE & REGULATION OF BONE MINERALISATION
ROLE OF VITAMIN D ROLE OF PARATHYROID HORMONE
ROLE OF CELLS IN BONE TURNOVER OSTEOBLASTS OSTEOCLASTS
METABOLIC BONE PHYSIOLOGY – BONE MINERALISATION
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ACQUIRED NUTRITIONAL RENAL HYPOCALCAEMIA → ↓MINERALISATION
GASTROINTESTINAL DRUGS
PHENOBARBITONE, PHENYTOIN
CONGENITAL FAMILIAL HYPOPHOSPHATAEMIC RICKETS ENZYME DEFICIENCIES
AETIOPATHOGENESIS
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CLINICAL ASSESSMENT◦ HISTORY
AGE – CHILD/ADOLESCENT DEFORMITY OF THE LOWER LIMBS SHORT STATURE LISTLESSNESS SEIZURES NUTRITION – MALNUTRITION, PROLONGED BREASTFEEDING
WITHOUT SUPPLEMENTATION FAMILY HISTORY DRUG HISTORY
◦ EXAMINATION ↓HEIGHT-FOR-AGE GENERALISED HYPOTONIA DELAYED CLOSURE OF ANTERIOR FONTANELLE THICKENING OF WRIST JOINT, KNEE, ANKLE
MANAGEMENT
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◦ EXAMINATION LOWER LIMB DEFORMITIES – BOWING OF
FEMUR/TIBIA, GENU VALGUM RACHITIC ROSARY HARRISON SULCUS KYPHOSCOLIOSIS (ESP. ABOVE 2 YEARS)
MANAGEMENT
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MANAGEMENT
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GENU VARUM
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RACHITIC ROSARY
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SERUM CALCIUM SERUM PHOSPHATE Ca x P PRODUCT ALKALINE PHOSPHATASE 25-HYDROXYCHOLECALCIFEROL SERUM E/U/Cr URINALYSIS
MANAGEMENT - BIOCHEMICAL ASSESSMENT
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SPLAYING, CUPPING AND FRAYING OF METAPHYSES
OSTEOPENIA BONY DEFORMITIES CRANIOTABES PATHOLOGIC FRACTURES
MANAGEMENT – RADIOGRAPHIC ASSESSMENT
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TREATMENT IS MEDICAL
CHOLECALCIFEROL 2000 – 5000IU/DAY + CALCIUM SUPPLEMENTS
IN RENAL RICKETS, HIGH DOSES – CHOLECALCIFEROL 150,000IU + Ca. CALCITRIOL MAY BE GIVEN AS WELL
RESIDUAL DEFORMITY IS CORRECTED SURGICALLY (CORRECTIVE OSTEOTOMY)
TREATMENT
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CLINICAL COMPLIANCE IMPROVEMENT IN DEFORMITY COMPLICATIONS
INVESTIGATIONAL SERUM CALCIUM SERUM PHOSPHATE FORTNIGHTLY ALKALINE PHOSPHATASE
FOLLOW-UP
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DUE TO DISEASE◦ RESIDUAL DEFORMITY◦ PATHOLOGIC FRACTURE◦ COMPLICATED CHILDBIRTH◦ RESPIRATORY FAILURE
DUE TO TREATMENT◦ HYPERCALCAEMIA◦ NEPHROCALCINOSIS◦ UROLITHIASIS
COMPLICATIONS
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PARENT EDUCATION
ADEQUATE EXPOSURE TO SUN
AVOID USE OF SUNSCREEN LOTIONS
DIETARY SOURCES - OILY FISH (MACKEREL, TUNA), COD LIVER OIL, EGG YOLK, BEEF LIVER
BREAST MILK CONTAINS VIT D 20-40IU/L
THE AMERICAN ACADEMY OF PAEDIATRICS (2008) RECOMMENDS:◦ BREASTFED INFANTS TO RECEIVE 400IU VIT D/DAY
◦ WHEN WEANED INFANTS SHOULD BE PLACED ON VIT D-FORTIFIED FORMULA OR FOR AN OLDER CHILD FORTIFIED MILK
PREVENTION
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THANK YOU
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APLEY’S SYSTEM OF ORTHOPAEDICS & FRACTURES, 9th Ed, pp 117-142
PRINCIPLES & PRACTICE OF SURGERY, BADOE et al, 4th Ed, pp 1167-1169
BAILEY & LOVE’S SHORT PRACTICE OF SURGERY, 25TH Ed, pg 566
CHAPMAN’S ORTHOPAEDIC SURGERY, 3RD Ed, pp 4588 – 4590
http://emedicine.medscape.com/article/985510-overview
http://www.cdc.gov/breastfeeding/recommendations/vitamin_d.htm
REFERENCES