ABNORMALITIES OF BONE IN CALCIUM

DEFICIENCYCMCH

DEPARTMENT OF ORTHOPAEDICS

RICKETS

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

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

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

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

ACQUIRED NUTRITIONAL RENAL HYPOCALCAEMIA → ↓MINERALISATION

GASTROINTESTINAL DRUGS

PHENOBARBITONE, PHENYTOIN

CONGENITAL FAMILIAL HYPOPHOSPHATAEMIC RICKETS ENZYME DEFICIENCIES

AETIOPATHOGENESIS

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

◦ EXAMINATION LOWER LIMB DEFORMITIES – BOWING OF

FEMUR/TIBIA, GENU VALGUM RACHITIC ROSARY HARRISON SULCUS KYPHOSCOLIOSIS (ESP. ABOVE 2 YEARS)

MANAGEMENT

MANAGEMENT

GENU VARUM

RACHITIC ROSARY

SERUM CALCIUM SERUM PHOSPHATE Ca x P PRODUCT ALKALINE PHOSPHATASE 25-HYDROXYCHOLECALCIFEROL SERUM E/U/Cr URINALYSIS

MANAGEMENT - BIOCHEMICAL ASSESSMENT

SPLAYING, CUPPING AND FRAYING OF METAPHYSES

OSTEOPENIA BONY DEFORMITIES CRANIOTABES PATHOLOGIC FRACTURES

MANAGEMENT – RADIOGRAPHIC ASSESSMENT

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

CLINICAL COMPLIANCE IMPROVEMENT IN DEFORMITY COMPLICATIONS

INVESTIGATIONAL SERUM CALCIUM SERUM PHOSPHATE FORTNIGHTLY ALKALINE PHOSPHATASE

FOLLOW-UP

DUE TO DISEASE◦ RESIDUAL DEFORMITY◦ PATHOLOGIC FRACTURE◦ COMPLICATED CHILDBIRTH◦ RESPIRATORY FAILURE

DUE TO TREATMENT◦ HYPERCALCAEMIA◦ NEPHROCALCINOSIS◦ UROLITHIASIS

COMPLICATIONS

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

THANK YOU

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