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

1. Rickets
Prof. Dr. Saad S Al Ani
Senior Pediatric consultant
Head Of Pediatric Department
Khorfakkan hospital
Sharjah ,UAE
saadsalani@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
rickets
3/8/2012 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 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. Pathophysiology
3/8/2012 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 to
the active metabolite calcitriol
(1,25-dihydroxycholecalciferol )
3/8/2012 Rickets Prof. Dr.Saad S Al ani 9
Khorfakkan Hospital

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. 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 Presentation
3/8/2012 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 bowing
3/8/2012 Rickets Prof. Dr.Saad S Al ani 15
Khorfakkan Hospital

16. Scoliosis
Harrison's sulcus and pot belly
3/8/2012 Rickets Prof. Dr.Saad S Al ani 16
Khorfakkan Hospital

17. Differential Diagnoses
1. Hypophosphatasia
2. Jansen syndrome
3. Hypophosphatemic vitamin D–resistant
rickets.
4. Severe calcium deficiency
5. Severe phosphorus deficiency
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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 rickets
3/8/2012 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 bones
3/8/2012 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.
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Khorfakkan Hospital

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. 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
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Khorfakkan Hospital

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. 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
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Khorfakkan Hospital

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. 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. 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

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Khorfakkan Hospital