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Rickets
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Definition of Rickets ,causes ,presentation ,differential diagnosis ,management ,prevention

Definition of Rickets ,causes ,presentation ,differential diagnosis ,management ,prevention

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Rickets

  1. 1. Rickets Prof. Dr. Saad S Al Ani Senior Pediatric consultant Head Of Pediatric Department Khorfakkan hospital Sharjah ,UAE saadsalani@yahoo.com
  2. 2. Background Rickets is a disease of growing bone that is unique to children and adolescents. It is caused by a failure of osteoid to calcify in a growing person. Failure of osteoid to calcify in adults is called Osteomalacia. 3/8/2012 Rickets Prof. Dr.Saad S Al ani 2 Khorfakkan Hospital
  3. 3. Vitamin D deficiency rickets occurs when the metabolites of vitamin D are deficient. Less commonly, a dietary deficiency of calcium or phosphorus may also produce rickets 3/8/2012 Rickets Prof. Dr.Saad S Al ani 3 Khorfakkan Hospital
  4. 4. Vitamin D-3 (cholecalciferol) is formed in the skin from a derivative of cholesterol under the stimulus of ultraviolet- B light. Natural nutritional sources of vitamin D are limited primarily to fatty, ocean-going fish. 3/8/2012 Rickets Prof. Dr.Saad S Al ani 4 Khorfakkan Hospital
  5. 5. Source of vitamin D 1. Ultraviolet light 2. Cod liver oil 3. Ergosterol (vitamin D-2) Dairy milk is fortified with vitamin D (400 IU/L) Human milk contains little vitamin D(less than 20-40 IU/L) 3/8/2012 Rickets Prof. Dr.Saad S Al ani 5 Khorfakkan Hospital
  6. 6. Pathophysiology 3/8/2012 Rickets Prof. Dr.Saad S Al ani 6 Khorfakkan Hospital
  7. 7. Cholecalciferol (i.e., vitamin D-3) is formed in the skin from 5-dihydrotachysterol. 3/8/2012 Rickets Prof. Dr.Saad S Al ani 7 Khorfakkan Hospital
  8. 8. This steroid undergoes hydroxylation in 2 steps . The first hydroxylation Occurs at position 25 in the liver, producing calcidiol (25-hydroxycholecalciferol) 3/8/2012 Rickets Prof. Dr.Saad S Al ani 8 Khorfakkan Hospital
  9. 9. The second hydroxylation Occurs in the kidney at the 1 position, where it undergoes hydroxylation to the active metabolite calcitriol (1,25-dihydroxycholecalciferol ) 3/8/2012 Rickets Prof. Dr.Saad S Al ani 9 Khorfakkan Hospital
  10. 10. Calcitriol Acts at 3 known sites to tightly regulate calcium metabolism: (1) it promotes absorption of calcium and phosphorus from the intestine (2) it increases reabsorption of phosphate in the kidney (3) it acts on bone to release calcium and phosphate. 3/8/2012 Rickets Prof. Dr.Saad S Al ani 10 Khorfakkan Hospital
  11. 11. Epidemiology The frequency increasing internationally 1.Children to wear sunscreen while outdoors 2.Children spend more time indoors watching television or playing electronic games, instead of playing outdoors •Lowdon J. Rickets: concerns over the worldwide increase. J Fam Health Care. Mar-Apr 2011;21(2):25-9.[Medline]. 3/8/2012 Rickets Prof. Dr.Saad S Al ani 11 Khorfakkan Hospital
  12. 12. Clinical Presentation 3/8/2012 Rickets Prof. Dr.Saad S Al ani 12 Khorfakkan Hospital
  13. 13. Knock knee deformity (genu valgum) Bowleg deformity (genu varum) 3/8/2012 Rickets Prof. Dr.Saad S Al ani 13 Khorfakkan Hospital
  14. 14. Wrist enlargement Rib beading (rachitic rosary) 3/8/2012 Rickets Prof. Dr.Saad S Al ani 14 Khorfakkan Hospital
  15. 15. Frontal bossing Tibial bowing 3/8/2012 Rickets Prof. Dr.Saad S Al ani 15 Khorfakkan Hospital
  16. 16. Scoliosis Harrison's sulcus and pot belly 3/8/2012 Rickets Prof. Dr.Saad S Al ani 16 Khorfakkan Hospital
  17. 17. Differential Diagnoses 1. Hypophosphatasia 2. Jansen syndrome 3. Hypophosphatemic vitamin D–resistant rickets. 4. Severe calcium deficiency 5. Severe phosphorus deficiency 3/8/2012 Rickets Prof. Dr.Saad S Al ani 17 Khorfakkan Hospital
  18. 18. Approach Considerations Serum measurements in the workup for rickets may include the following: 1.Calcium.2.Phosphorus.3.Alkaline phosphatase4.Parathyroidhormone 5.25-hydroxy vitamin D 6.1,25-dihydroxyvitamin D Radiography is indicated in patients with rickets 3/8/2012 Rickets Prof. Dr.Saad S Al ani 18 Khorfakkan Hospital
  19. 19. Serum Chemistry Calcium (ionized fraction) is low Calcidiol (25-hydroxy vitamin D) is low Parathyroid hormone is elevated Phosphorus level is invariably low for age Alkaline phosphatase levels are uniformly elevated . 3/8/2012 Rickets Prof. Dr.Saad S Al ani 19 Khorfakkan Hospital
  20. 20. Radiography 1. Cupping of the metaphysis 2. Fraying of the edge 3. Widening of the osteoid tissue 4. Hypominiralization of bones 3/8/2012 Rickets Prof. Dr.Saad S Al ani 20 Khorfakkan Hospital
  21. 21. Anteroposterior and lateral radiographs of the wrist of an 8-year-old boy with rickets demonstrates cupping and fraying of the metaphyseal region. 3/8/2012 Rickets Prof. Dr.Saad S Al ani 21 Khorfakkan Hospital
  22. 22. Radiograph in a 4-year-old girl with rickets depicts bowing of the legs caused by loading. 3/8/2012 Rickets Prof. Dr.Saad S Al ani 22 Khorfakkan Hospital
  23. 23. Radiographs of the knee of a 3.6-year-old girl with hypophosphatemia depict severe fraying of the metaphysis. 3/8/2012 Rickets Prof. Dr.Saad S Al ani 23 Khorfakkan Hospital
  24. 24. Treatment & Management Treatment for rickets may be administered gradually over several months or in a single-day dose of 15,000 mcg (600,000 U) of vitamin D 3/8/2012 Rickets Prof. Dr.Saad S Al ani 24 Khorfakkan Hospital
  25. 25. If the gradual method is chosen, 125-250 mcg (5000-10,000 U) is given daily for 2-3 months until: 1. Healing is well established 2. Alkaline phosphatase concentration is approaching the reference range 3/8/2012 Rickets Prof. Dr.Saad S Al ani 25 Khorfakkan Hospital
  26. 26. If the vitamin D dose is administered in a single day, it is usually divided into 4 or 6 oral doses. An intramuscular injection is also available In nutritional rickets: 1.Phosphorus level rises in 96 hrs 2. Radiographic healing is visible in 6-7 days 3/8/2012 Rickets Prof. Dr.Saad S Al ani 26 Khorfakkan Hospital
  27. 27. Rickets Medications Vitamin D is a fat-soluble vitamin used to prevent or treat vitamin D deficiency 3/8/2012 Rickets Prof. Dr.Saad S Al ani 27 Khorfakkan Hospital
  28. 28. Cholecalciferol (Vitamin D3, D drops Kids, Delta-D3) 1.single-day dose of 15,000 mcg (600,000U), which is usually divided into 4 or 6 oral doses An intramuscular injection is also available. 2.An alternative regimen is to give 125-250 mcg (5000-10,000 U) daily for 2-3 months 3/8/2012 Rickets Prof. Dr.Saad S Al ani 28 Khorfakkan Hospital
  29. 29. References 1. McKay CP, Portale A. Emerging topics in ediatric bone and mineral disorders 2008. Semin Nephrol. Jul 2009;29(4):370-8. 2. Lowdon J. Rickets: concerns over the worldwide increase. J Fam Health Care. Mar-Apr 2011;21(2):25-9.[Medline]. 3. Chapman T, Sugar N, Done S, Marasigan J, Wambold N, Feldman K. Fractures in infants and toddlers with rickets. Pediatr Radiol. Dec 9 2009;[Medline]. 4. Casey CF, Slawson DC, Neal LR. VItamin D supplementation in infants, children, and adolescents. Am Fam Physician. Mar 15 2010;81(6):745-8. [Medline]. 5. Greer FR. Issues in establishing vitamin D recommendations for infants and children. Am J Clin Nutr. Dec 2004;80(6 Suppl):1759S- 62S. [Medline]. 6. [Guideline] Wagner CL, Greer FR. Prevention of rickets and vitamin D deficiency in infants, children, and adolescents. Pediatrics. Nov 2008;122(5):1142-52. [Medline]. 3/8/2012 Rickets Prof. Dr.Saad S Al ani 29 Khorfakkan Hospital
  30. 30. 3/8/2012 Rickets Prof. Dr.Saad S Al ani 30 Khorfakkan Hospital

Notas do Editor

  • which circulates in the plasma as the most abundant of the vitamin D metabolites and is thought to be a good indicator of overall vitamin D status
  • *Calcidiol circulates in the plasma as the most abundant of the vitamin D metabolites and is thought to be a good indicator of overall vitamin D status
  • This cholecalciferol, which circulates in the bloodstream in minute amounts, is not technically a vitamin but a hormone.
  • which circulates in the plasma as the most abundant of the vitamin D metabolites and is thought to be a good indicator of overall vitamin D status
  • Rare metabolic bone diseases is a rare autosomal dominant form of short-limbed dwarfism in which infants present with metaphyseal chondroplasia. Hereditary disorders of vitamin D metabolism 5. Premature infants who are breast fed and do not receive mineral supplements
  • Infants who are breastfed are at risk for rickets, especially those who: a. receive no oral supplementation b. have darkly pigmented skin
  • Infants who are breastfed are at risk for rickets, especially those who: a. receive no oral supplementation b. have darkly pigmented skin
  • Infants who are breastfed are at risk for rickets, especially those who: a. receive no oral supplementation b. have darkly pigmented skin

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