Rickets

Rickets

Prof. Dr. Saad S Al Ani

Senior Pediatric consultant

Head Of Pediatric Department

Khorfakkan hospital

Sharjah ,UAE

[email protected]

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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.

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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

04/12/2023 Rickets Prof. Dr.Saad S Al ani Khorfakkan Hospital

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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.

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Source of vitamin D1. 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)

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Pathophysiology

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Cholecalciferol (i.e., vitamin D-3) is formed in the skin from 5-dihydrotachysterol.

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Occurs at position 25 in the liver, producing calcidiol (25-hydroxycholecalciferol)

The first hydroxylation

This steroid undergoes hydroxylation in 2 steps.

04/12/2023 Rickets Prof. Dr.Saad S Al ani Khorfakkan Hospital

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Occurs in the kidney at the 1 position, where it undergoes hydroxylation to the active metabolite calcitriol

(1,25-dihydroxycholecalciferol )

The second hydroxylation

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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.

04/12/2023 Rickets Prof. Dr.Saad S Al ani Khorfakkan Hospital

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Epidemiology

2.Children spend more time indoors watching television or playing electronic games, instead of playing outdoors

The frequency increasing internationally

1.Children to wear sunscreen while outdoors

• Lowdon J. Rickets: concerns over the worldwide increase. J Fam Health Care. Mar-Apr 2011;21(2):25-9.[Medline].

04/12/2023 Rickets Prof. Dr.Saad S Al ani Khorfakkan Hospital

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Clinical Presentation

04/12/2023 Rickets Prof. Dr.Saad S Al ani Khorfakkan Hospital

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Knock knee deformity (genu valgum)

Bowleg deformity (genu varum)

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Wrist enlargement

Rib beading (rachitic rosary)

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Frontal bossing

Tibial bowing

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Scoliosis

Harrison's sulcus and pot belly

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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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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

04/12/2023 Rickets Prof. Dr.Saad S Al ani Khorfakkan Hospital

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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.

04/12/2023 Rickets Prof. Dr.Saad S Al ani Khorfakkan Hospital

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Radiography

1. Cupping of the metaphysis

2. Fraying of the edge

3. Widening of the osteoid tissue

4. Hypominiralization of bones

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Anteroposterior and lateral radiographs of the wrist of an 8-year-old boy with rickets demonstrates cupping and fraying of the metaphyseal region.

04/12/2023 Rickets Prof. Dr.Saad S Al ani Khorfakkan Hospital

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Radiograph in a 4-year-old girl with rickets depicts bowing of the legs caused by loading.

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Radiographs of the knee of a 3.6-year-old girl with hypophosphatemia depict severe fraying of the metaphysis.

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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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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

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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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Rickets Medications

Vitamin D is a fat-soluble vitamin used to prevent or treat vitamin D deficiency

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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

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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].

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Thank you