Ricket and osteomalacia
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Ricket and osteomalacia

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different between rickets and osteomalacia

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Ricket and osteomalacia Ricket and osteomalacia Presentation Transcript

  • Rickets & Osteomalacia Hamad Emad Dhuhayr
  • CONTENTS 1. SOEPEL 2. DEFINITION 3. EPIDEMIOLOGY 4. BASIC BACKGROUND 5. AETIOLOGY 6. RISK FACTORS 7. CLINICAL MANIFESTATIONS 8. INVESTIGATION 9. MANAGEMENT 10.REFERENCES
  • SUBJECT: • S , a four year old girl is brought to the primary health centre for not being to walk properly. On examination, there are skeletal deformities of both upper and lower limbs with marked bowing General examination shows pallor, otherwise there are no other signs.The abdomen is distended, otherwise systemic examination is normal. SOEPEL
  • OBJECTIVE: taking history, physical examination ( musculoskeletal and neurological ) EVALUATION (DD): Ricket Hypophosphatamia Metaphyseal dysostoses Blount's syndrome PLAN: History, physical examination, plain X-ray ELABORATION: vit D SOEPEL
  • LEARNING GOALS: Rickets and Osteomalacia SOEPEL
  • Normal Development • calcium/ PO4 • Vit D • PTH • calcitonin Bones….What do they need to be strong?
  • VIT D LEVEL IN SERUM - • 25 (OH) D3 level ng/ml DEFICIENT < 10 INSUFFICIENT 10 - 20 OPTIMAL 20 - 60 HIGH 60 - 90 TOXIC >90
  • PARATHYROID HORMONE • Stimulus for its secretion : fall in serum Ca. • PTH promotes bone resorption process and is adversely affected by calcitonin. • PTH also stimulates the excretion of phosphates by the kidneys; this inhibition of phosphate resorption in turn enables calcium resorption. • In GIT - indirectly increases calcium absorption by increasing the synthesis of active vit D 3 by stimulating alpha hydroxylase
  • CALCITONIN • It is produced by para follicular c cells of thyroid. • It is a calcium lowering hormone in serum by inhibiting bone resorption by decreasing the no & activity of osteoclasts . • So calcitonin acts counter to PTH. Calcitonin inhibits bone resorption thus causing serum calcium levels to fall.
  • Metabolic bone diSeases
  • Metabolic bone diseases include: •Rickets •Osteomalacia •osteoporosis
  • Rickets • Disease of growing bones of children(in it epiphyseal plate not closed )in which defective mineralization occurs in both bone and cartilage of epiphyseal growth plate. Osteomalacia • Disorder of mature bones in adult (after epiphyseal plate closure )in which mineralization of new osteoid bone is inadequate or delayed
  • Types of Rickets • (1)Vitamin D deficient rickets: there is decrease in vitamin D inside body. • (2)Vitamin D dependent rickets: there is defect in the process of vitamen D activation. • (3)Vit D resistant rickets: either -Hypophosphatemic rickets • - End organ resistance to 1,25 Dihydroxy Vit D3
  • causes of rickets : • 1- Nutritional deficiency: commonest cause in the developing countries also Excess of phytate in diet which form insoluble compounds with calcium so prevent its absorption (chapati flour) • 2-Malabsorption as in Celiac disease,Pancreatic insufficiency • 3-Hepato-biliary disease • Biliary Artesia • Cirrhosis • neonatal hepatitis • 4-Drugs • Anti-convulsants • Phenobartbitone • Phenytoin 5-Renal causes : -Renal osteodystrophy - Renal tubular acidosis.
  • pathogenesis ofVitamin D deficient rickets • The predominant cause of rickets is a deficiency in vitamin D, which is required for normal calcium absorption from the gut. Malabsorption leads to low levels of calcium in the blood.This not only prevents proper bone growth, but can also lead to calcium being released form the bones to increase its blood level.
  • Hypophosphatemic rickets • Nutritional phosphate deficiency • Prematurity • Decreased intestinal absorption of phosphate • Ingestion of phosphate binders (aluminum hydroxide) • Renal phosphate wasting
  • Rickets Clinical feature General Failure to thrive Apathetic , listless, irritable Shorter, lower body weight and anemic Excessive sweating particularly at hand & face
  • Rickets Clinical feature Head • craniotabes(soft skull) • frontal bossing • Widening of suture, persistent fontanelae • Delayed dentition, caries
  • Rickets Clinical feature Chest • Rachitic rosary • Harrison groove • Pigeon chest • Respiratory infection and atelectasis
  • Rickets Characteristic feature • Widening of wrist, knee and ankle due to physeal over growth
  • Rickets Characteristic feature Abdomen - prominent muscle weakness (floppy baby, delayed walking) Pelvis - narrow inlet
  • Rickets Characteristic feature Deformity • Toddlers: Bowed legs (genu verum)
  • Rickets Characteristic feature Deformity • Older children: Knock-knees (genu velgum)
  • Rickets Characteristic feature Deformity • windswept knees • CoxaVera • String of pearls deformity
  • Rickets Characteristic feature • Thoracic kyphosis (rachitic cat – back) • increased tendency for fracture, especially green stick # • Growth disturbance • Bone pain or tenderness • Less common tetany, laryngeal, stridor and convulsion • Sign of PEM
  • Rickets Radiographic feature
  • Rickets Radiographic feature
  • Rickets Radiographic feature
  • Rickets Radiographic feature
  • Rickets Diagnosis • History & physical examination finding • Radiographic abnormality • Special etiology confirmed with lab. test
  • Diagnosis of rickets • A-Clinical features of rickets: (1)Skeletal manifestations (2) extraskeletal manifestations • B-investigations
  • (1) Skeletal manifestations • The earliest sign of rickets in infant is craniotabes (abnormal softness of skull) • Delayed closure of anterior fontanel • Frontal and parietal bossing :Rounded prominence of the frontal and parietal bones in an infant’s cranial vault • Delayed eruption of primary teeth • Enamel defects and caries teeth. • Rachitic rosary • Swelling of the costo-chondral junction • Harrison’s groove • Lateral indentation of the chest wall at the site of attachment of diaphragm because the patients lack the mineralized calcium in their bones necessary to harden them; thus the diaphragm, which is always in tension, pulls the softened bone inward. • Enlargement of long bones around wrists and ankles • Bow legs, • knock knees • green stick fractures • Deformities of spine, pelvis and leg – rachitic dwarfism
  • (2)Extra – skeletal manifestations • SEIZURES • TETANY i.e periodic painful muscular spasms and tremors, caused by faulty calcium metabolism and associated with diminished function of the parathyroid glands. • HYPOTONIA AND DELAYED MOTOR DEVELOPMENT Muscle weakness • PROTUBERANT ABDOMEN, BONE PAIN, WADDLING GAIT AND FATIGUE. In older children presenting with rickets
  • B - Investigations • BASIC INVESTIGATIONSTO CONFIRM RICKETS • SerumCa, P and X rays of ends of long bones at knees or wrists • Hypocalcemia If Serum Calcium less than 8.0 mg/dl • Widening, fraying, cupping of the distal ends of shaft. • Tetracycline-labelled bone biopsy. Gold stansar.
  • Difference Between Osteoporosis & Osteomalacia • Osteoporosis refers to the degeneration of already constructed bone, making them brittle, • while osteomalacia is an abnormality in the building process of bone, making them soft.
  • Osteomalacia Clinical feature Insidious course Pt may present with bone pain, back ache and bone tenderness proximal muscle weakness Fracture may be first sing of Osteomalacia Vertebral collapse, kyphosis or knock knee perhaps due to adolescent rickets- may increase in later life.
  • Osteomalacia Clinical feature Long standing case sign of secondary hyperparathyroidism • Depression • Polyuria • Increased thirst • Constipation • Nephrolithiasis • ?Peptic Ulcer Disease
  • Osteomalacia X- ray • Looser zone
  • Osteomalacia • X- ray -Looser zone
  • Osteomalacia X- ray • lateral indentation of the acetabulam (trefoil pelvis) • Biconcave vertebrae
  • Osteomalacia • Long standing case sign of secondary hyperparathyroidism
  • Osteomalacia • Long standing of Osteomalacia Cortical erosion Pathological # Brown tumor
  • Osteomalacia Deferential diagnosis 1. Osteomalacia osteoporosis • Unwell well • Generalized chronic ache pain after # • Muscles weakness muscle normal • Looser’s zone absent • Ph3 decrease normal • Alk. Ph3ase increase normal
  • Osteomalacia Deferential diagnosis 2. Primary Hyperparathyroidism Hypercalcaemia hypophosphaetemia Raised PTH & alkaline phosphatase 3. Myeloma  Anemia Increase ESR Blood and urine electrophoresis raised of single Ig Bence jones protein
  • Rickets & Osteomalacia Treatment • Depending on etiology, severity and metabolic abnormality • In general the combination ofVit-D, Ca and phosphate • Orthopedic measure require in very less no. of cases 1 mg of vit-D = 40,000 IU 1 IU = 0.025 microgram
  • Rickets & Osteomalacia Target of therapy low -N = Ca N- = phosphate high - N = alkaline phosphatase Over dosing side effect ofVit – D prevented
  • Rickets & Osteomalacia Treatment 1.Vit-D deficiency state Vit –D 1,000 – 10,000 I.U./day4- 6 wk Adherence is poor (stoss therapy) Vit –D 300,000-600,000 IU Im /Orally in a day (2-4dose) Calcium --- 1g/ day General nutrition , sunlight ? Followed by 400 IU / day
  • Rickets & Osteomalacia Treatment 2. Absorption defect Vit- D 1,500 – 25,000 IU / day Calcium 1 g/ day Treatment of underling pathology; where appropriat, low fat or gluten free diet
  • Rickets & Osteomalacia Treatment 3.Vit – D resistant Vit – D 20,000- 60,000 IU/day Or dihydrotachysterol (dose 1/3 of vit D) Neutral phosphate-1.5- 6 g/ day (4-5 dose) Calcium – 1 g / day
  • Rickets & Osteomalacia Treatment 4.Vit – Dependent type – I 1, 25Vit – D 250 IU – 800 IU /day Calcium 1 g/day
  • Rickets & Osteomalacia Treatment 5.Vit – Dependent type – II Respond with high dose of  1, 25 Vit – D 1,000- 20,000 IU /day for 3-6 mth Calcium - - 1-3 g / day i/v Ca with oral supplement
  • Rickets & Osteomalacia Treatment 6. Renal tubular Vit- D 1,000 – 4,000 IU/ day Alkalizing solution; K supplement