6 Lect 6 Rickets

7/25/2019 6 Lect 6 Rickets

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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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+! /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



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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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6 Lect 6 Rickets