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Rickets:Rickets:Etiology, pathogenesis, clinical features,Etiology, pathogenesis, clinical features,
diagnostics, treatment and preventiondiagnostics, treatment and prevention
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Rickets is a childhood disorderinvolving softening and weakening of
the bones.
It is primarily caused by lack of
vitamin D, calcium, or phosphate.
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Vitamin D is a fat-soluble vitamin that may be absorbed from
the intestines or may be produced by the skin when the skin isexposed to sunlight ultraviolet light of sunlight helps the
body to form vitamin D!.
"he absorbed vitamin D is converted into its active form to act
as a hormone to regulate calcium absorption from the
intestine and to regulate levels of calcium and phosphate in
the bones.
If there is a deficiency of Vitamin D, the body is unable toproperly regulate calcium and phosphate levels. #hen the
blood levels of these minerals become too low, it results in
destruction of the support matrix of the bones.
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Sunlight as a source of vitamin D
Lack of vitamin D production by the skin may occur if a
person is confined indoors, or works indoors during the
daylight hours, or lives in climates with little exposure
to sunlight.
Sunlight is important to skin
production of
vitamin D and environmental
conditions where sunlight
exposure is limited may reduce
this source of vitamin D.
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Sunlight as a source of vitamin D
Adeuate supplies of
vitamin D!can "e
synthesi#ed with sufficient
exposure to solarultraviolet $ radiation
%elanin, clothing orsunscreens that a"sor"
&'$ will reduce cutaneous
production of vitamin D!
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Rickets In Rickets, another mechanism in the body works to
increase the blood calcium level.
The parathyroid gland may increase its functioning rate tocompensate for decreased levels of calcium in the
bloodstream.
To increase the level of calcium in the blood the hormonedestroys the calcium present in the bones of the body andthis results in further loss of calcium and phosphorous from
the bones.
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Etiology
$.%ack of sunshine due to&
1) Lack of outdoor activities
2) Lack of ultraviolet light in fall and winter ) Too much cloud! dust! vapour and smoke
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Etiology
'. Improper feeding&
1) Inade"uate intake of #itamin $
%reast milk &'1&I(1&&ml *ow+s milk &.',I(1&&ml
-gg yolk 2I(average yolk
/erring 1&&I(1&&g2) Improper *a and 0 ratio
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Etiology
(. )ast growth, increased re*uirement
relative deficiency!
+. Diseases and drug&
Liver diseases! renal diseases
astrointestinal diseases ntiepileptic
lucocorticosteroid
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Pathogenesis
#itamin $ deficiency
bsorption of *a! 0
3erum *a
4unction of 0arathyroid
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Pathogenesis "
igh secretion
in urine Decalcification of old bone
in blood a in blood normal or low slightly
*a! 0 product
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Pathogenesis Low secretion of 0T/
4ailure of decalcification of bone
Low serum *a level
5achitic tetany
/pasmophylia!
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(n the vitamin D deficiencystate, hypocalciemia develops,
which stimulates excess
parathyroid hormone, which
stimulates renal phosphorus
loss, further reducing deposition
of calcium in the "one.
Excess parathyroid hormone
also produces changes in the
"one similar to those occurring
in hyperparathyroidism.
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0arly in the course of rickets,the calcium concentration in theserum decreases.
1fter the parathyroid response,
the calcium concentrationusually returns to the referencerange, though phosphoruslevels remain low.
1lkaline phosphatase, which isproduced by overactiveosteoblast cells, leaks to theextracellular fluids so that itsconcentration rises to anywherefrom moderate elevation to very
high levels.
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"he history in patients with rickets mayinclude the following&
"he infant2s gestational age, diet and degree ofsunlight exposure should be noted.
1 detailed dietary history should include
specifics of vitamin D and calcium intake.
1 family history of short stature, orthopedicabnormalities, poor dentition, alopecia, parental
consanguinity may signify inherited rickets.
0valuation
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Vitamin D dependent
Vitamin D-dependent rickets, type I is secondary to
a defect in the gene that codes for the productionof renal '34!D(-$-alpha-hydroxylase.
Vitamin D-dependent rickets, type II is a rare
autosomal disorder caused by mutations in thevitamin D receptor. "ype II does not respond to
vitamin D treatment5 elevated levels of circulating
calcitriol differentiate this type from type I.
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4ther onditions "hat an ause Rickets
6edications6 1ntacids6 1nticonvulsants6 orticosteroids6 %oop diuretics
6alignancy rematurity Diseases of organs associated with vitamin D and
calcium metabolism6 7idney disease
6 %iver and biliary tract disease 6alabsorption syndromes
6 eliac disease6 ystic fibrosis rare!
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Clinical signs
Rickets
is a systematic disease with
skeletons involved most, but the
nervous system, muscular system
and other system are also involved.
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)raniota"es manifests early in infants, althoughthis feature may be normal in infants, especially for
those born prematurely.
linical signs
If rickets occurs at a later age,thickening of the skull
develops.
*rontal "ossinganddelaysthe closure of the anterior
fontanelle.
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rotruding foreheadasymmetrical or odd-shaped skull
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/keletal deformities8ow legs.
)orward pro9ection of the
breastbone
igeon chest or pectuscarinatum!.
)unnel chest pectus
excavatum!.
8umps: in the rib cage rachiticrosary!.
1symmetrical or odd-shaped
skull.
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hest deformity
)unnel chest ; pectus
excavatum
igeon chest
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linical signs
In the chest, knobbydeformities results in therachitic rosary along thecostochondral 9unctions.
"he weakened ribs pulled bymuscles also produce flaringover the diaphragm, which is
known as arrison groove.
Rib beading
rachitic rosary!
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,athwayofVitaminD,roduction
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$owlegs andknock+knees.
linical signs
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7nock knee deformity
genu valgum!
8owleg deformity
genu varum!
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Vitamin D Defciency - Rickets
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8owlegs and knock-knees
elvic deformities
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1 teenage male with rickets.
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linical signs
he ends of the long"ones demonstrate thatsame kno""y thickeningat the ankle.
-alpation of the ti"ialgives the impression of adou"le epiphysis
%arfan sign/.
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linical signs
Increased tendency toward
bone fractures. 8ecause the softened long
bones may bend, they mayfracture one side of thecortex greenstick fracture!.
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/pine deformities spinecurves abnormally,including scoliosis orkyphosis!.
In more severe instancesin children older than 'years, vertebral softeningleads to kyphoscoliosis
linical signs
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-ain in the "ones of Arms, 0egs, Spine, -elvis.
Dental deformities
Delayed formation of teeth
Defects in the structure of teeth
1oles in the enamel (ncreased incidence of cavities in the teeth
dental caries/
linical signs
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-rogressive weakness
Decreased muscle tone loss of muscle
strength/ %uscle cramps
(mpaired growth
Short stature adults less than 2 feet tall/ *ever or restlessness, especially at night
linical signs
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8owlegs and knock-knees
/hort stature
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=ait disturbances andneurologic abnormalitiessuch as hyperreflexia! in
all children should besought.
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%aboratory findings
%aboratory investigation may include&
/erum levels of calcium total and ioni>edwith serum albumin!, hosphorus, 1lkaline phosphatase 17!
arathyroid hormone, ?rea nitrogen, alcidiol ?rine studies include urinalysis and levels
of urinary calcium and phosphorus.
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Decreases
in serum calcium,serum phosphorus,
calcidiol, calcitriol,urinary calcium.
"he most common laboratory findings innutritional rickets are&
-arathyroid hormone,
alkaline phosphatase,
urinary phosphoruslevels are elevated.
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lassic radiographic findings include
Anteroposterior and lateral radiographs of the wrist of an 3+year+
old "oy with rickets demonstrates cupping and fraying of the
metaphyseal region
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lassic radiographic findings include&
Radiographs of the knee of a !+year+old girl with hypophosphatemia
depict severe fraying of the metaphysis.
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Rickets in wrist + uncalcified lower ends of "ones
are porous, ragged, and saucer+shaped
A/ Rickets in ! month old infant
$/ Healing after 28 days of
treatment
)/ After 41 days of
treatment
A
$ )
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@-ray in rickets
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Clinical Stages
0arly stage ?sually begin at ( months old
/ymptoms& mental psychiatric symptoms
Irritability, sleepless, hidrosis
/igns& occipital bald
%aboratory findings& /erum a, normal or
decreased slightly, 17 normal or elevated
slightly, '34!D( decreased
Roentgen-graphic changes& normal or
slightly changed
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Clinical manifestation
1dvanced stage 4n the base of early rickets, osseous changes
become marked and motor developmentbecomes delayed.
$. 4sseous changes&$! ead& craniotabes, frontal bossing, boxlike
appearance of skull, delayed closure ofanterior fontanelle
'! "eeth& delayed dentition with abnormal order,defects
(! hest& rachitic rosary, arrisonAs groove,pigeon chest, funnel-shaped chest, flaring of
ribs
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Clinical manifestation
+! /pinal column& scoliosis, kyphosis, lordosis3! 0xtremities& bowlegs, knock knee,
greenstick fracture
B! Rachitic dwarfism
'. 6uscular system& potbelly, late in standing andwalking
(. 6otor development& delayed
+. 4ther nervous and mental symptoms
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%aboratory findings& /erum a and decreased
a and product decreased
17 elevated
Roentgen-graphic changesrist is the best site for watching the changes#idening of the epiphyseal cartilage
8lurring of the cup-shape metaphyses of long bone
Clinical manifestation
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Clinical manifestation
ealing stage& /ymptoms and signs of Rickets alleviate ordisappear by use of appropriate treatment.
"he blood chemistries become normal, except17, that may be slightly elevated.
/e*uelae stage& 1ll the clinical symptoms and signs disappear.
8lood hemistries and @-ray changes arerecovered, but osseous deformities may be left.
?sually seen in hildren after ( years old.
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"ypes of Rickets
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reatment 4 %anagement
reatment for rickets may "e
administered gradually over several
months orin a single+day doseof 52,666
mcg 766,666 &/ of vitamin D
&
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(fthe gradual method is chosen, 582+826 mcg
2666+56,666 &/ is given dailyfor 8+! monthsuntil:
5.1ealing is well esta"lished
8. Alkaline phosphatase concentration is
approaching the reference range
1
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(fthe vitamin D dose is administered in a
single day, it is usually divided into 9 or 7
oral doses. An intramuscularinection is
also availa"le
(n nutritional rickets:
5.-hosphorus level rises in ;7 hrs
8. Radiographic healing is visi"le in7+< days
2
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Rickets %edications
'itamin D is a fat+solu"le vitamin used to
prevent or treat vitamin D deficiency
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)holecalciferol
'itamin D!, D drops =ids, Delta+D!/5.single+day dose of 52,666 mcg 766,666&/,
which is usually divided into 9 or 7 oral doses
An intramuscular inection is also availa"le.
8.An alternative regimen is to give 582+826
mcg 2666+56,666 &/ daily for 8+! months
,
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Treatment
+. alcium supplementation&Dosage& $-( gCday
only used for special cases, such as baby
fed mainly with cereal or infants under (months of age and those who have already
developed tetany.
3. lastic therapy& In children with bone deformities after +
years old plastic surgery may be useful.
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Prevention
$. ay much attention to the health care of
pregnant and lactating women, instruct themto take ade*uate amount of vitamin D.
'. 1dvocate sunbathing
(. 1dvocate breast feeding, give supplementary
food on time
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/ources of Vitamin D/ources of Vitamin D
/unlight is the most important source
)ish liver oil
)ish sea food herring salmon!
0ggs
lants do not contain vitamin D(
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Preventionalcium supplementation&
&.'1gmday! for premature! weak babies and babies fed mainly with
cereal
Recommended daily intake of calcium is as follows& $ to ( years of age.&& mg 7two servings of dairy products a day)
+ to E years of age.8&& mg 7two to three servings of dairy products aday) F to $E years of age.1!&& mg 7four servings of dairy products a day) $F to 3G years of age.1!&&& mg a day 7three servings of dairy products
a day)
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