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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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  • 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

Transcript

  • 1. RicketsProf. Dr. Saad S Al AniSenior Pediatric consultantHead Of Pediatric DepartmentKhorfakkan hospitalSharjah ,UAEsaadsalani@yahoo.com
  • 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. 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 rickets3/8/2012 Rickets Prof. Dr.Saad S Al ani 3 Khorfakkan Hospital
  • 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. Source of vitamin D1. Ultraviolet light2. Cod liver oil3. Ergosterol (vitamin D-2)Dairy milk is fortified with vitamin D(400 IU/L)Human milk contains littlevitamin D(less than 20-40 IU/L)3/8/2012 Rickets Prof. Dr.Saad S Al ani 5 Khorfakkan Hospital
  • 6. Pathophysiology3/8/2012 Rickets Prof. Dr.Saad S Al ani 6 Khorfakkan Hospital
  • 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. 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. The second hydroxylation Occurs in the kidney at the 1 position,where it undergoes hydroxylation tothe active metabolite calcitriol(1,25-dihydroxycholecalciferol )3/8/2012 Rickets Prof. Dr.Saad S Al ani 9 Khorfakkan Hospital
  • 10. CalcitriolActs at 3 known sites to tightly regulatecalcium 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 releasecalcium and phosphate.3/8/2012 Rickets Prof. Dr.Saad S Al ani 10 Khorfakkan Hospital
  • 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. Clinical Presentation3/8/2012 Rickets Prof. Dr.Saad S Al ani 12 Khorfakkan Hospital
  • 13. Knock knee deformity (genu valgum) Bowleg deformity (genu varum)3/8/2012 Rickets Prof. Dr.Saad S Al ani 13 Khorfakkan Hospital
  • 14. Wrist enlargement Rib beading (rachitic rosary)3/8/2012 Rickets Prof. Dr.Saad S Al ani 14 Khorfakkan Hospital
  • 15. Frontal bossing Tibial bowing3/8/2012 Rickets Prof. Dr.Saad S Al ani 15 Khorfakkan Hospital
  • 16. Scoliosis Harrisons sulcus and pot belly3/8/2012 Rickets Prof. Dr.Saad S Al ani 16 Khorfakkan Hospital
  • 17. Differential Diagnoses1. Hypophosphatasia2. Jansen syndrome3. Hypophosphatemic vitamin D–resistant rickets.4. Severe calcium deficiency5. Severe phosphorus deficiency3/8/2012 Rickets Prof. Dr.Saad S Al ani 17 Khorfakkan Hospital
  • 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 rickets3/8/2012 Rickets Prof. Dr.Saad S Al ani 18 Khorfakkan Hospital
  • 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. Radiography 1. Cupping of the metaphysis 2. Fraying of the edge 3. Widening of the osteoid tissue 4. Hypominiralization of bones3/8/2012 Rickets Prof. Dr.Saad S Al ani 20 Khorfakkan Hospital
  • 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. 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. Radiographs of the knee of a 3.6-year-old girl withhypophosphatemia depict severe fraying of the metaphysis.3/8/2012 Rickets Prof. Dr.Saad S Al ani 23 Khorfakkan Hospital
  • 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 D3/8/2012 Rickets Prof. Dr.Saad S Al ani 24 Khorfakkan Hospital
  • 25. If the gradual method is chosen, 125-250 mcg(5000-10,000 U) is given daily for 2-3 monthsuntil:1. Healing is well established2. Alkaline phosphatase concentration is approaching the reference range3/8/2012 Rickets Prof. Dr.Saad S Al ani 25 Khorfakkan Hospital
  • 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 days3/8/2012 Rickets Prof. Dr.Saad S Al ani 26 Khorfakkan Hospital
  • 27. Rickets Medications Vitamin D is a fat-soluble vitamin used to prevent or treat vitamin D deficiency3/8/2012 Rickets Prof. Dr.Saad S Al ani 27 Khorfakkan Hospital
  • 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 dosesAn intramuscular injection is also available.2.An alternative regimen is to give 125-250mcg (5000-10,000 U) daily for 2-3 months3/8/2012 Rickets Prof. Dr.Saad S Al ani 28 Khorfakkan Hospital
  • 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. 3/8/2012 Rickets Prof. Dr.Saad S Al ani 30 Khorfakkan Hospital