RICKETS IN CHILDREN

RICKETS:
• Rickets ( from Greek word means -spinal column )
• Disease of growing bones.
• Occurs in children only before the fusion of
epiphyses.
• Due to unmineralised matrix at the growth plates.
• Inadequate mineralization Thick Growth Plate
• Bones become soft.
 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.
What is Rickets ?
 Impaired mineralisation of the growth plate &
osteoid
 Low serum phosphate is fundamental to
pathogenesis of rickets
Normal Growth Plate Rachitic Growth Plate

Apoptosis of Hypophosphatemia
Hypertrophic No Apoptosis of
Chondrocytes Hypertrophic
caused by Chondrocytes
PHOSPHATE ions
What is Rickets ?

Impaired Apoptosis of Terminally Differentiated Chondrocytes in the Growth Plate

Responsible for Clinical & Radiological Signs of Rickets
Vitamin D Metabolism
 Causes of Rickets
•VITAMIN D DISORDERS CALCIUM DEFICIENCY

•Nutritional Vitamin D deficiency •Low intake Diet

•Congenital Vitamin D deficiency • Premature Infant
•Secondary Vitamin D deficiency • Malabsorption
- Malabsorption • Primary Disease
-Increased degradation • Dietary inhibitors of calcium
-Decreased Liver 25-hydroxylase absorption
•Vitamin D dependent ricket Type 1
•Vitamin D dependent ricket Type 2 RENAL LOSSES
•Chronic Renal Failure
•X- linked hypophosphatemic ricket
•PHOSPHORUS DEFICIENCY •AD hypophosphatemic ricket
•Hereditary hypophosphatemic ricket
with hypercalcuria
• Inadequate intake •Fanconi syndrome
• Premature infants
• Aluminium containing antacids

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 Clinical Features of Rickets:

A)GENERAL
• Failure To Thrive
• Listlessness
• Protruding Abdomen,
UMBILICAL HERNIA due to
hypotonia of abdominal
wall muscles
• Muscle Weakness
(specially proximal)
• Fractures
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 B) HEAD
• Box-like square head

•CRANIOTABES (softening of cranial

bone)

•Delayed Fontanelle Closure

•Delayed Dentition (early

numerous caries,enamel
hypoplasia)

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 C)Chest:

1.RACHITICROSARY: Widening of
costochondral junction

2.Harrison Groove : pulling of softened ribs

by the diaphragm during inspiration, Muscle
traction on the softened rib cage.
3.Pectus carinatum
4.Thoracic asymmetry
5.Widening of thoracic bone
6.Respiratory Infection

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D) BACK
Scoliosis

Kyphosis

Lordosis
E) EXTREMITIES
Enlargement of wrists
and ankles
Growth plate widening

Valgus or varus
deformities
WINDSWEPT DEFORMITY
combination of varus deformity of 1 leg
with valgus deformity of other leg

Anterior bowing of tibia and

femur

Coxa Vara

Leg pain
HYPOCALCEMIC SYMPTOMS
Tetany
Seizures
Stridor due to laryngeal spasm
 Vitamin C - Ascorbic Acid
• Humans cannot synthesize vitamin C and must
obtain it from food
• It is important for hydroxylation of lysine and
proline for formation of collagen.

• Collagen-
– Helps support and protect blood vessels, bones,
joints, organs and muscles
– Protective barrier against infection and disease
– Promotes healing of wounds, fractures and bruises
 Scurvy – Signs & Symptoms

• Peak incidence: 6-24 months

• Vague irritability & loss of appetite
• The baby cries on handling
• Generalized tenderness : Pseudo-paralysis;
Frog position.
• Bluish purple, spongy swelling of gum mucosa
• Teeth loose
• Sharp painful scorbutic rosary is palpable in
costo-chondral junction & depression of
sternum.
• Delay in wound-healing
• Small blood vessels fragile leading to peri-
follicular hge, echymosis
 INVESTIGATIONS
RADIOLOGY (Rickets)
1. Cupping, fraying & widening of lower ends of
long bones.
2. Widening of the radiolucent space between
metaphysis and epiphysis due to non-
opaque osteoid tissue.
3. Generalized osteopenia
4. Deformities of bone.
• Edge of metaphysis loses its sharp border:
FRAYING
• Edge of metaphysis changes from convex or flat
surface to a more concave surface: CUPPING
• Widening of Metaphyseal end of bone:
SPLAYING
• Metaphyseal lines spread laterally forming
CORTICAL SPURS
 Radiographic features of Scurvy
• generalized osteopenia
• cortical thinning: “pencil-point” cortex
• periosteal reaction due to subperiosteal haemorrhage
• scorbutic rosary
• haemarthrosis
• Wimberger's ring sign: circular, opaque radiologic shadow
surrounding epiphyseal centers of ossification, which may
result from bleeding
• Frankel's line: dense zone of provisional calcification
• Trümmerfeld zone: lucent metaphyseal band underlying
Frankel's line
• Pelken spur: metaphyseal spurs which result in cupping of the
metaphysis
SCURVY RICKETS
Radiographs of the knee of a 3.6-year-old girl with
hypophosphatemia depict severe fraying of the metaphysis.

Rickets Prof. Dr.Saad S Al ani Khorfakkan

08/14/2020 30
Hospital
 Approach to Rickets:
Diet history- vit-D deficiency.
Cutaneous sun exposure: culture ,clothing etc.
Maternal risk factors for vit-D
Child is on anticonvulsant, Al-containing antacids
Malabsorption: GI symptoms ,liver disease
Fat Malabsorption: Look for ADEK defi.
Renal disease: CRF, Polyuria -Fanconi syn.
Family h/o short strature ,bone disease, unexplained death of
sibling cystinosis  M.C.C. Fanconi syn.
Alopecia  Vit-D dep. Type-2 rickets

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 VITAMIN- D DEFICIENCY
ETIOLOGY

VITAMIN D DEFICIENCY is cause of Rickets

Worldwide.
•
Most commonly occur in infancy due to poor
intake and inadequate cutaneous synthesis
•
Formula fed infants- receive adequate vit D
even without cutaneous synthesis
•
Breast fed infants rely on cutaneous synthesis
or vitamin D supplements
LABORATORY FINDINGS
Rachitic index: Serum Ca× Serum Phosphate
S. Ca: Hypocalcemia –due to action of ↑PTH
S. Phosphate: Hypophosphatamia - due to PTH
induced renal loss of phosphate and decreased
intestinal absorption
S. PTH: High
S. Alkaline Phosphatase: High
1,25D level-N,↑,↓- secondary to up regulation of
renal 1 α hydroxylase due to hypophosphatemia
and hyperparathyroidism
Depending Upon The Cause:
• S. creatinine: High in CKD & RTA
• S. Electrolytes: Altered in CKD & RTA
• ABG: Metabolic acidosis in RTA, CKD
• Urine for glucose, amino acid: Glycosuria,
aminoaciduria seen in Fanconi Syndrome
• 24 hrs urinary calcium level & Urinary
Calcium/ creatinine ratio: ↑ed in Fanconi
Syndrome
• 24 hrs urinary Phosphate level: ↑ed in RTA,
Familial hypophosphatemic rickets, Fanconi Syn
Treatment of Vitamin D Deficiency Rickets
 STOSS Therapy:
Oral vitamin D3, (Cholecalciferol) 300000 - 600000 IU./day is
given IM or orally 2-4 doses over 1 day
OR
Gradual therapy 2000-5000 IU/day over 4-6 weeks.
 Oral calcium supplements if necessary
 Provide vitamin D supplements (~ 400 iu/day) after the rickets
has healed
Radiological Changes

Rx
Vitamin D3
+
Calcium
 PREVENTION OF VITAMIN D
DEFICIENCY
Universal administration of a daily
multivitamin containing 200-400 IU of
Vitamin D to children who are breast feed.

For older children, the diet should be

reviewed to ensure that there is a source of
Vitamin D
Rickets Due to Calcium Deficiency

Wind-swept Abnormality due to Calcium Oginni et al Archives of Disease in Childhood. 2003;88:812-8

Deficiency Rickets –
16 month old child with severe Rickets
 Treatment of Scurvy
• Oral vitamin C 200mg daily for several weeks.
• 3-4 ounces tomato or orange juice are equally
effective
• Clinical recovery occurs 24-48 hours
• Radiological change 1-2 week
• Sub-periosteal hge takes months
• After cure of scurvy, 35-50 mg of vit-C should
be taken daily with diet.
 Prevention

• EBF
• Supplementation of lactating mother with vitC
• Formula feeding should be supplemented
with vit C.
• Encourage intake of vitamin –C rich food`