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Rickets
Rickets is a disease of growing bone caused by unmineralized bone matrix at the growth plates, occurring only in children before fusion of the epiphyses. It is caused by vitamin D disorders, calcium deficiency, renal losses, or phosphorous deficiency. Clinical features include failure to thrive, bowed legs, widened wrists and ankles, and bone pain. X-rays show widened growth plates and fraying and cupping of the metaphysis. Treatment involves high dose vitamin D and calcium supplementation.
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Rickets
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- 2. Ca Ph BONE Inorganic OrganicWater matrix Bone cells
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- 4. Rickets - A diseaseof growing bone - Caused by unmineralized matrix at the growth plates - Occurs in children only before fusion of the epiphyses
- 5. ETIOLOGY Vitamin D disordersCalcium deficiency Renal Losses Phosphorous deficiency RICKETS
- 6. Cholecalciferol 25 hydroxycholecalciferol 1,25 Dihydroxycholecalciferol VitaminD–Dependent Rickets, Type 1-> mutation Vitamin D–Dependent Rickets, Type 2-> mutation
- 7. severe maternal vitaminD deficiency during pregnancy AR
- 9. PHEX gene mutationincreases FGF-23inhibition of ph. reabsorb in PT - FGF 23 -mutation - DMP1 and EMPP1 increase FGF 23 - mutations in the gene encoding a chloride channel that is expressed in the kidney - dysfunction of the renal proximal tubule rare complication polyostotic fibrous dysplasia, hyperpigmented macules, and polyendocrinopathy
- 10. Clinical Features GENERAL – -Failure to thrive - Protruding abdomen - Muscle weakness (especially proximal) - Fractures
- 11. HEAD • Craniotabes – -softening of the cranial bones - can be detected by applying pressure at the occiput or over the parietal bones. The sensation is similar to the feel of pressing into a ping-pong ball and then releasing. - Craniotabes may also be secondary to osteogenesis imperfecta, hydrocephalus, and syphilis. - It is a normal finding in many newborns, especially near the suture lines, but it typically disappears within a few months of birth
- 12. HEAD • Frontal andparietal bossing- box like appearance of head- hot cross bun appearance or caput quadratum • Delayed fontanel closure- anterior fontanelle remains wide open and its closure is delayed • Delayed dentition & caries • Craniosynostosis
- 13. CHEST Rachitic rosary • Wideningof the costochondral junctions • Feels like the beads of a rosary as the examiner’s fingers move along the costochondral junctions from rib to rib • Smooth angle ( Sharp angle in scurvy)
- 14. CHEST • Harrison groove -horizontal depression along the lower anterior chest - occurs from pulling of the softened ribs by the diaphragm during inspiration
- 15. CHEST • Respiratory infections •Softening of the ribs also impairs air movement and predisposes patients to atelectasis and pneumonia.
- 16. BACK • Scoliosis • Kyphosis( rachitic cat back) • Lordosis
- 17. EXTREMITIES • Enlargement ofwrists and ankles (Growth plate widening)
- 18. • Valgus orvarus deformities • Windswept deformity (One leg – valgus , other leg – varus) • Coxa vara
- 19. • Anterior bowingof the tibia and femur (general softening of the bones bend easily when subject to forces such as weight bearing)
- 20. • Double malleolisign – Widening of epiphysis above medial malleolus gives appearance of double malleoli
- 21. HYPOCALCEMIC SYMPTOMS • Tetany •Seizures • Stridor due to laryngeal spasm
- 23. History • Dietary history •Drug intake – - Anticonvulsants phenobarbital and phenytoin, increase degradation of vitamin D, - aluminum-containing antacids interfere with the absorption of phosphate
- 24. • A historyof renal disease (proteinuria, hematuria, urinary tract infections) • Polyuria can occur in children with chronic kidney disease or Fanconi syndrome • Fat malabsorption is often associated with diarrhea or oily stools • Alopecia suggests vitamin D–dependent rickets type 2.
- 26. Xray findings • Thickeningof the growth plate • Physis- Widening • Metaphysis- Cupping and Fraying • Rarefaction – decreased density and trabecula is prominent. Healing rickets – provisional zone of calcification Between shaft and epiphysis
- 27. Labs • Serum phosphate– Decreased • Serum calcium – normal or decreased • Serum alkaline phosphatase - increased
- 28. Diagnosis • Classically byxray • Supported by History + Physical examination + Lab findings
- 29. Treatment • Stoss therapy,300,000-600,000 IU of vitamin D are administered orally or intramuscularly as 2-4 doses over 1 day. • The alternative is daily, high-dose vitamin D, with doses ranging from 2,000-5,000 IU/day over 4-6 wk. • Either strategy should be followed by daily vitamin D intake of 400 IU/day if 1 yr old.
- 30. • Rickets ininfancy is characterised by the following except : ( AIIMS May 2007) a) Craniotabes b) Rachitic rosary c) Wide open fontanelles d) Bow legs
- 31. NEET – pediamcq 2.True regarding nutritional rickets is: a)Decreased cortical bone b)Decreased calcification of mature bone c)Decreased calcification of growing bone d) Decreased production of steroid
- 32. NEET 3) Basic pathologyin rickets- a)Defective bone matrix formation b)Defect in mineralization c)A defect in osteoid formation d) All of the above
- 33. NEET 4) Enlarged tenderepiphysis with bowing of long bones and xray evidence of delayed carpal ossification suggest the diagnosis a) Scurvy b) Infantile rickets c) Syphilis d) Osteogenesis imperfecta
- 34. NEET 5) Which statementis untrue in renal rickets? a) Results from renal insufficiency in infancy b) Is due to deficient phosphorus excretion c) Manifests itself by marked dwarfism d) Causes no deformity at puberty e) Ends fatally from uraemia at puberty
- 35. Renal rickets • CKD+ Hyperphosphatemia + secondary hyperparathyroidism • Clinically – growth retardation • Lower limb more involved than upper limb • Rx – Diet low in phosphorus • Dialysis • High dose of calcitriol
- 36. NEET • 6) A4 yr old rachitic child developed genu varum. The best line of Rx is by – a) Antirachitic treatment b) Repeated moulding c) Corrective splints d) Tibial osteotomy
- 37. 7) 13 yrold boy presented for consultation bcoz his knees tend to knock together & he tends to fall during running. Examination – Separation of medial malleoli by 3 inches when standing with knee in contact with each other and feet directed forwards. The correct diagnosis is? A) Coxa vara B) Genu valgum C) Genu varum D) Ctev E) Bilateral flat foot
- 38. 8) Inheritance ofvitamin D dependent rickets is a) AD b) AR c) X linked recessive d) X linked dominant
- 39. 9) Features ofrickets – a) Marfan sign b) Laryngismus stridulus c) Tackle deformity d) None of the above e) All of the above
- 40. • 10) Truestatements about rickets- a) Hypotonia occurs due to hypocalcaemia b) Green stick fractures can occur c) Short stature d) All of the above e) None of the above
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