RICKETS

BY Dr.

HAMDY ABO
HAGAR
ASSISTANT PROFESSOR
OF PEDIATRICS

Definition

Defective mineralization of growing

bones, due to vitamine D deficiency
or abnormal metabolism.

Vitamin D
Sources:
A: Diets such as fats and oils.
B: Vitamin D supplements.
C: Ultraviolet sunrays effect
on a precursor in the skin (7dehydrocholesterol).
Types:
Vitamin D2 of plant origin
Vitamin D3 of animal origin
and that naturally formed in
the skin.

Vitamin D
Metabolism:
After absorption from the gut or
formation in the skin, vitamin D passes
through two steps of activation before it
becomes ready to act:
- In the liver: It is hydroxylated to 25hydroxy Vit D.
- In the kidney: It is furtherly
hydroxylated to 1,25 dihydroxy Vit D (the
active form).

Vitamin D
Actions:
In the intestine: It increases absorption
of Ca and P.
In the kidney: It increases reabsorption
of Ca and P.
In bones: It stimulates mineralization
of bones.

Growth plate in normal bone

Growth plate in normal bone

:shows

Zone of resting
cartilage (one
layer of cells).

Zone of
proliferating
cartilage: regular
columns of cells
originating from
resting layer).

Growth plate in normal bone

:shows

Zone of degeneration (cells

become swollen with
glycogen, glycolytic
enzymes and alkaline
phosphatase. Calcium is
deposited in the matrix. It is
sharply demarcated in X ray
film).

Zone of ossification (blood

vessels invade the
developing bone with
ossification and remodeling
resulting in mature bone).

In rickets

Zone of proliferation increases and

becomes very vascular causing
enlargement of metaphyseal area and
invades the adjacent zone of
degeneration.

Zone of degeneration fails to mineralize

and the newly formed tissue called osteoid
is excessively deposited and being soft it
gives way with pressure causing bulging
and deformity of metaphyseal area of long
bones (this is responsible for flaring of the
ends of long bones and rachitic rosary).

In rickets

In the shaft: bone is resorbed and new

osteoid is formed around the shaft
from the periosteum

During healing of rickets: a new line

of calcified bone (line of provisional
calcification) appears at the end of
zone of degeneration out standing
from rarefied osteoid then the area
between it and the diaphysis
gradually fills with normal density
bone.

Radiologic Changes
Active rickets:
They occur early, are pathognomonic
and diagnostic, and help in follow up.
Distal ends of long bones appear
flared, frayed and cupped.

Distance between the distal end of

radius and metacarpal bones appears
wider than normal (by the area filled
with osteoid).

Diaphysis appears rarefied and may

show double contour or deformity.

Radiologic Changes
Healing rickets:
Occurs 2-3 weeks after
successful treatment.
Appearance of the line of
provisional calcification
at the end of metaphysis,
then the osteoid in
between this line and
diaphysis gradually
ossifies.

Radiologic Changes
Healed rickets:
Bone density returns to normal with
slight cupping remains as a stigma of
previous rickets.

Biochemical (laboratory)
:manifestations

Serum alkaline phosphatase is elevated due to over

activity of osteoblasts during the formation of
excessive osteoid (normal 5-15 Bodansky units /dl).

Serum inorganic phosphorus is decreased (normal

4.5-6.5 mg/dl).

Serum calcium is maintained within normal values

(9-11 mg/dl) due to compensatory hyperactivity of
parathyroid gland.

Vitamin D and its metabolites are decreased.

Clinical manifestations

Vitamin D deficiency rickets most

commonly occurs at the end of the
first year and during the second year
of life.

Clinical manifestations:
Early rickets
1.Craniotabes:
Occurs due to thinning of the inner table
of occipital bone under the pressure of
intracranial contents with failure of
mineralization.
It can be elicited by gentle pressure by
both thumbs of the occipital bone, which
produces a dent with crackling sensation
(ping pong ball like).
This can be elicited from 3 to 12 months
of life.

2. Rosary:
Clinical manifestations
Early rickets
Enlargement of
costochondral junction of
ribs giving the appearance
of beads due to excessive
osteoid formation.
3. Radiological finding of
active rickets.
4. Rise of serum alkaline
phosphatase enzyme.

Advanced rickets
Head:
Bossing of skull: excessive
proliferation of cartilage at occipital
and parietal eminences makes the
skull looks like a box.
Enlargement of head circumference.
Delayed closure of anterior
fontanels, which remains widely
open.
Delayed eruption of primary
dentition with possible enamel
hypoplasia.

Advanced rickets
Thorax:
Rosary beads.
Longitudinal

sulcus: appears lateral

to the rosaries due to compression
of rib cage by atmospheric pressure
at weakest point.

Harrison's

sulcus: A transverse
sulcus along the lower border of the
costal margin due to inward
traction of the ribs at sites of
diaphragmatic insertion.

Advanced rickets
Thorax:
Forward

protrusion of sternum
and adjacent costal cartilage.

Everted

costal margin below

Harrison's sulcus.

The

overall shape of the chest

wall is called pigeon chest,
which is nearly triangular in
cross section.

Advanced rickets

Abdomen:

Liver and spleen become palpable due

to deformed chest and weak abdominal
muscles. The abdomen appears
protruded.

Pelvis:

Pelvic inlet is narrowed by forward

protrusion of sacral promontory, while
pelvic outlet is narrowed by forward
projection of the coccyx.

This might be very hazardous in females

during labor in the future.

Advanced rickets
Spinal column:
Correctable kyphosis
in the dorsal region
and lordosis in the
lumbar region due to
muscle weakness and
laxity of ligaments.

Scoliosis

Advanced rickets

Extremities:
Enlargement of metaphyseal region
especially at wrists and ankles
Marfan's sign: transverse groove above the
medial and sometimes also the lateral
maleolus.
Deformities of long bones due to weight
bearing.
Greenstick fracture.

Advanced rickets

Rachitic dwarfism: due to

spinal and lower limb
deformities.

Weak muscles and lax

ligaments causing
delayed locomotor
milestones.

Complications

Respiratory: infections or atelectasis due to

chest deformities.

GIT: diarrhea or constipation.

Bony deformities or fractures.

Anemia: due to chronic infection or deficiencies.

Tetany: due to hypocalcaemia in late cases after

exhaustion of parathyroids.

Prognosis

Usually good with improvement after

exposure to sun light in the morning or
afternoon or after administration of
Vitamin D.

Deformities improve with normal growth

but very slowly.

Sometimes, severe skeletal deformities

require orthopedic correction.

Prevention

Exposure to ultraviolet rays in

sunshine (10 to 20 minutes/day).

Daily requirements of vitamin D are

400-800 i.u /day.

For low birth weight infants, and

patients of malnutrition or
hypothyroidism during receiving
their specific treatment, 1000-1500
i.u /day are needed for the
accelerated rate of growth.

Treatment

Oral Vitamin D in a dose of 15005000 i.u/day for 6-8 weeks.

Shock therapy: Vit D 600,000 i.u by

I.M. injection or orally single dose.

After 2-4 weeks, if no radiologic or

laboratory evidence of complete
healing occurs, the dose can be
repeated.

Non-Vitamin D Deficiency
Rickets

Vitamin D dependent type: due to defective 1alpha-hydroxylase in the kidney or failure of end
organ response to it.

Familial hypophophatemic resistant rickets: affects

girls more, there is tubular defect in phosphate
retention resulting in excessive urinary phosphate
losses.

Tubular defects such as Fanconi syndrome with

urinary loss of phosphate, glucose, amino acids
and bicarbonates.

Chronic renal failure.

Acquired renal tubular damage, e.g.
drugs.
Malabsorption syndromes: such as
celiac disease, cystic fibrosis of the
pancreas, cholestasis.
Anticonvulsant therapy causing
increased metabolism of Vitamin D.

THANK YOU