Professional Documents
Culture Documents
Principles of Growth
A. Children are individuals, not little adults, who must be seen as part of the family
B. Children are influenced by genetic factors, home, and environment , and parental attitude
C. Chronologic and developmental ages of children are the most important contributing
factors influencing their care
D. Play is a natural medium for expression, communication, and growth in children
E. Growth id complex, with all aspects closely related
F. Growth is measured both quantitatively and qualitatively over a period of time
G. Although the rate is uneven , growth is a continuous and orderly process
1. Infancy: most rapid period of growth
2. Preschool to puberty: slow and uniform rate of growth
3. Puberty (growth spurt) second most rapid growth period
4. After puberty : decline in growth rate till death
H. There are regular patterns in the direction of growth and development ,such as
cephalocaudal law and proximodistal
I. Different parts of the body grow at different rates
1. Prenatally: head grows the fastest
2. During first year: elongation of trunk dominates
J. Both rate and pattern of growth can be modified, most obviously by nutrition
K. Each individual proceeds at own rate
L. Development is closely related to the maturation of the nervous system; as primitive
reflexes disappear, they are replaced by voluntary activity
Characteristic of Growth
Circulatory system
Respiratory System
A. Rate decreases with increase age
B. Vital capacity
1. Gradual increase throughout childhood and adolescence, with a decrease in later life
2. Capacity in males exceeds that in females
C. Basal metabolism
1. Highest rate is found in the newborn
2. Rate declines with increase in age, higher in males than females
Urinary System
1. Premature and full-term newborn have some inability to concentrate urine
a. Specific gravity (newborn): 1.001 to 1.02
b. Specific gravity (others) 1.001 to 1.030
2. Glomerular filtration rate greatly increased by 6 months of age; reaches adult values
between 1-2 years ; gradually decreases after 20 years
Digestive System
1. Stomach is small gradually increases during infancy and childhood
2. Peristaltic activity decreases with advancing age
3. Blood glucose level rise from infancy to adolescence
4. Premature infants have lower blood glucose than full-term
5. Enzymes are present at birth to digest proteins and a moderate amount of fats, but only
simple sugars (amylase is produced as starch is introduced)
6. Secretion of hydrochloric acid and salivary enzymes increases with age until
adolescence; then decreases with advancing age
Nervous System
1. Brain reaches 90% of total size by 2 years of age
2. All brain cells are presents by the end of the first year, although their size and complexity
will increase
3. Maturation of the brainstem and spinal cord follows cepahlocaudal and proximodistal
laws
THE INFANT
1 month
A. Physical
1. Weight: gains about 150- 210 g weekly during first 6 months of life
2. Height: grows about 2.5cm (I inch) a month for the first 6 months of life
3. Head circumference: grows about 1.5 cm (1/2 inch) a month for the first 6 months
B. Motor
1. Assumes flexed position with pelvis high, but knees not under abdomen, when prone
2. Holds the head parallel with the body when suspended in prone position
3. Can turn head from side to side when prone; lifts head momentarily from bed
4. Asymmetric position dominates, such as tonic neck reflex
5. Primitive reflexes still present
C. Sensory
1. Eye movements coordinated most of the time; follows a light to midline
2. Visual acuity 20/100 to 20/50
D. Socialization and vocalization
1. Watches face intently while being spoken to
2. Utters small, throaty sounds
2 to 3 months
4 to 5 months
A. Physical
1. Birth-weight doubles
2. Drools because salivary glands are functioning but child does not have sufficient
coordination to swallow saliva
B. Motor
1. Can sit when the back is supported; knees will be flexed and back rounded; balances
the head well
2. Symmetric body position predominates
3. Can sustain a portion of own weight when held in a standing position
4. Reaches for and grasps an object with the whole hand but misjudges distances
5. Can carry hand or an object to the mouth at will
6. Can roll over from abdomen to back
7. Lifts head and shoulders at 90◦ angle when prone
8. Primitive reflexes (e.g. grasp, tonic neck, and moro) have disappeared
9. Neurologic reflexes
a. Landau (from 6- 8 months to 12-24 months): when suspended in a horizontal
prone position, the head is raised , legs and spine are extended
b. Parachute (7-9 months ,persists indefinitely) when the infant is suspended in a
horizontal prone position and suddenly thrust prone position and suddenly thrust
forward, hands and fingers extend forward as if to protect from falling.
C. Sensory
1. Recognizes familiar objects and people
2. Has coupled eye movements, accommodation is developing
3. Socialization and vocalization
4. Coos and gurgles when talked to ; enjoys social interaction
5. Vocalization displeasure when an object is taken
6 to 7 months
A. Physical
1. Wt: gains about 90 to 150 g weekly during second 6 months of life
2. Height: grows about 1.25 cm (1/2 inch) a month
3. Head circumference : grows about 0.5 (1/5 inch) a month
4. Teething may begin with eruption of two lower central incisors, followed by upper
incisors
B. Motor
1. Can turn over equally well from stomach or back
2. Sit fairly well unsupported
3. Lifts head off table when supine
4. Can transfer a toy from one hand to the other and from hand to mouth
5. Plays with feet and puts them in mouth
C. Sensory
1. Has taste preferences; will spit out disliked food
2. Recognize that things are still present even though they cannot be seen
D. Socialization and vocalization
1. Shows stranger anxiety
2. Makes polysyllabic vowel sounds
3. Vocalizes ―m-m-m-m‖ when crying; cries easily on slightest provocation but laughs
just as quickly
8 to 9 months
A. Motor
1. Sits steadily alone; pulls self to standing position; stands holding onto furniture
2. Good hand-to-mouth coordination
3. Developing pincer grasp
4. Crawls may go backward at first
B. Sensory
1. Depth perception is increasing
2. Displays interest in small objects
C. Socialization and vocalization
1. Social attachment is evident (e.g. stretches out arms to loved ones)
2. Responds to own name
3. Imitative and repetitive speech, using vowels and consonants such as ―dada‖, no true
words as yet but comprehend words such as ―bye-bye‖
10 to 12 months
A. Physical
1. Weight: birth-weight triples
2. Height: birth-length increases by 50%
3. Head and chest circumference are equal
4. Upper and lower lateral incisors usually have erupted , for total of 6to 8 teeth
B. Motor
1. Creeps
2. Stands alone for a short times, walks with help
3. Can sit down from a standing position without help
4. Can eat from spoon and cup but needs help
5. Can play peek-a-boo; holds a crayon to make a mark on a paper
6. Helps in dressing, such a putting arm through sleeves
C. Sensory
1. Visual acuity 20/50+; amblyopia (lazy eye) may develop with lack of binocularity
2. Discriminates simple geometric forms
D. Socialization and vocalization
1. Shows emotions such as jealousy, affection, anger
2. Enjoys familiar surroundings and will explore away from mother
3. Fearful of strange situations or with strangers
4. May develop habit of ―security‖ blanket
5. Can say two words besides Dada and Mama with meaning; understands simple verbal
request, such as, ―give it to me.‖
A. Safety is the chief determinant in choosing toys ( aspirating small objects is one of
accidental death
B. Mostly used for physical development
C. Toys needs to be simple because of short attention span
D. Visual and auditory stimulation is important
E. Suggested toys: rattles soft, stuffed toys; mobiles; push-pull toys; simple musical toys
Feeding Milestones
A. Newborn feels hunger and indicates desire for food by crying; expresses satiety by
contentedly falling asleep
B. At 1 month has strong extrusion reflex
C. 5 to 6 month can use fingers to eat teething cracker or toast
D. 6 to 7 months developmentally ready to chew solid foods
E. 8 to 9 months can hold a spoon and play with it during feeding
F. 9 months can hold own bottle
G. 12months can drink from a cup
Injury prevention
A. Accidents are one of the leading causes of death during infancy
1. Mechanical suffocation causes most accidental deaths in children under 1 year of age
2. Aspiration is common during second half of first year and into early childhood
3. Trauma from rolling off a bed or falling down stairs can occur at any time
The Toddler
15 months
A. Motor
1. Walks well alone by 14 months with a wide based gait; creeps upstairs
2. Build tower of two blocks; enjoys throwing objects and picking them up
3. Drinks from a cup and can use spoon
B. Vocalization and socialization
1. Can use four to six words, including name
2. Has learned ―no‖ which may be said while doing a requested demand
18months
A. Physical
1. Growth decreased and appetite lessened – ― physiologic anorexia‖
2. Anterior fontanel is usually closed
3. Abdomen protrudes, larger than chest circumference
B. Motor
1. Runs clumsily; climbs stairs or up on furniture
2. Imitates strokes in drawing
3. Drinks well in cup; manages spoon well
4. Builds tower of three or four cubes
C. Vocalization and socialization
1. Says 10 words or more
2. New awareness of strangers
3. Temper tantrums
4. Very ritualistic, has favorite toy or blanket, thumb-sucking may be at peak
2 years
A. Physical
1. Weight: 11 to 12 kg
2. Height: 80 to 82 cm
3. Teeth: 16 temporary; begins visits to dentist
B. Motor
1. Gross motor skills quite refined
2. Can walk up and down stairs
3. Builds tower of six to seven cubes or will make cube into a train
C. Sensory
1. Accommodation well developed
2. Visual acuity 20/40
D. Vocalization and socialization
1. Vocabulary about 300 words, uses short, two-to-three-word phrases, also pronouns
2. Obeys simple commands, shows signs of increasing autonomy and individuality;
makes simple choices when possible
3. Can help undress self and pull on simple clothes
4. Does not share possessions, everything is ―mine‖
30 months
A. Physical
1. Full set of 20 temporary teeth
2. Decreased need for naps
B. Motor
1. Walks on tiptoe; stands on one foot momentarily
2. Builds tower of eight blocks
3. Copies horizontal or vertical lines
4. May attend to own toilet needs
C. Vocalization and socialization
1. Begins to see self as separate individual
2. Still see other children as objects
3. Increasingly independent, ritualistic, and negativistic
A. Nutritional objectives
1. Provide adequate nutrient intake to meet continuing growth and development
2. Provide a basis for support of psychosocial development in relation to food patterns,
eating behavior, and attitude
3. Provide sufficient calories for increasing physical activities and energy levels
B. Diet: calorie and nutrient requirements increase with age
Injury Prevention
A. More than half of accidental child deaths are related to automobiles and fire
B. Accidents can be viewed in terms of child’s growth and development, especially curiosity
about the environment
1. Motor vehicle
2. Burns
a. Investigating: pulls off pot off the stove; plays with matches; inserts an object to
wall socket
b. Climbing: reaches the stove, oven, ironing board and iron, cigarettes on the table
3. Poisons
a. Learning new tastes and textures, puts everything into mouth
b. Developing fine motor skills; able to open bottles, cabinets, jars
c. Climbing to previously unreachable shelves
4. Drowning
a. child and parents do not recognize the danger of water
b. Child is unaware of inability to breathe under water
5. Aspiration
a. Puts everything in mouth
b. Very interested in body and newly found openings
6. Fractures
a. Climbing, running, and jumping
b. Still developing sense of balance
The Preschooler
3 years
A. Physical
1. Usual weight gain 1.8 to 2.7 kg
2. Usual height gain 7.5 cm
B. Motor
1. Jumps off bottom stair; walks upstairs alternating feet
2. Rides a tricycle using pedals
3. Constructs three-block bridge; builds tower of 9 to 10 cubes
4. Can unbutton front or side button; uses a spoon
5. Usually toilet trained at night
C. Sensory: visual acuity 20/30
D. Vocalization and socialization
1. Vocabulary of about 900 words; uses three-to-four-word sentences; uses plurals; may
have normal hesitation in speech pattern
2. Begins to understand ideas of sharing and taking turns
E. Mental Abilities
1. Beginning understanding of the past ,present, future, or any aspect of time
2. Stage of magical thinking
4 years
A. Physical
1. Height and weight increases are similar to previous year
2. Length at birth is doubled
B. Motor
1. Skips and hops on one foot; walks up and down stairs like an adult
2. Can button buttons and lace shoes
3. Throw ball overhand; uses scissors to cut out-line
C. Vocalization and socialization
1. vocabulary of 1500 words or more
2. may have an imaginary companion
3. tends to be selfish and impatient but takes pride in accomplishments; exaggerates,
boast, and tattles on others
D. Mental Abilities
1. Unable to conserve matter
2. Can repeat four numbers and is learning number concept
3. Knows which is longer lines; has poor space perception
5 years
School-Aged Children
A. Physical growth
1. Permanent dentition, beginning with 6-year molars and central incisors at 7 or 8 years
of age
2. Tends to look lanky because bone development precedes muscular development
B. Motor
1. Refinement of coordination, balance, and control occurs
2. Motor development necessary for competitive activity becomes important
C. Sensory: visual acuity 20/20
D. Mental Abilities
1. Readiness for learning, especially in perceptual organization: names months of year,
knows right from left, can tell time, can follow directions at once
2. Acquires use of reason and understanding of rules; needs consistency
3. Trial-and-error problem solving becomes more conceptual rather than action oriented
4. Reasoning ability allows greater understanding and use of language
5. Concrete operations (piaget) : know that quantity remains the same even though
appearance differs
A. Number of play activities decreases, whereas the amount of time spent in one particular
activity increases
B. Likes games with rules
C. Likes athletic competition because of increase motor ability
D. In beginning of school years, boys and girls play together but gradually separate into sex-
oriented type of activities
E. Suggested play for 6-9 years old
1. More housekeeping toys that work, paper-doll set, sewing machine, building toys,
simple work and number games, physical active games such as climbing trees, jump
rope, bicycle riding
F. Suggested play for 9-12 years old
1. Handicraft of all kinds; model kits, pottery clay, archery, dart games, chess, jigsaw,
science toys
Chromosomal Aberrations
Pathogenesis:
Small head with distinctive facial features (low nasal bridge, epicantric folds,
protruding tongue, and low-set ears); small, open mouth and disproportionately
large tongue due to chromosomal aberration
Small, short, thick fingers and hands; single transverse crease on the palm (simian
crease); small white spot on the iris (brushfield’s spots) due to chromosomal
aberration
Mental retardation (estimated IQ of 30 to 70) and congenital defects (heart
defects, duodenal atresia, hirshsprung’s disease, polydactyly, syndactyly) due to
chromosomal aberration
Developmental delay due to hypotonia and decreased cognitive processing
Impaired reflexes due to decreased muscle tone in limbs
Klinefelter’s Syndrome
Pathogenesis:
The X chromosome carries genes that play roles in many body systems,
including testis function, brain development, and growth.2 The addition of more
than one extra X or Y chromosome to a male karyotype results in variable
physical and cognitive abnormalities. In general, the extent of phenotypic
abnormalities, including mental retardation, is directly related to the number of
supernumerary X chromosomes. As the number of X chromosomes increases,
somatic and cognitive development are more likely to be affected.
Gastrointestinal Malformations
Abnormalities involving the lip and palate (may occur separately or in combination)
develop in the second month of pregnancy , when the front and sides of the face and the
palatine shelves fuse imperfectly
Cleft lip (with or without cleft palate) occurs twice as often in males than as females;
cleft palate alone (without cleft lip) is more common in females
Pathogenesis:
During the second month of pregnancy ,the front and sides of the face and
palatine shelves develop; because of a chromosomal abnormality , exposure to
teratogens, genetic abnormality, or environmental factors, the lip or palate fuses
imperfectly
Deformity may range from a simple notch to a complete cleft, cleft palate may be
partial or complete
Complete cleft includes the soft palate, the bones of the maxilla, and the alveolus
on one or both sides of the premaxilla
Difficulty feeding because infant cannot form a vacuum with the mouth to suck;
may be able to breastfeed ( breast may fill the cleft, making sucking easier)
Mouth breathing results in : increased swallowed air, causing distended abdomen
, and mucous membranes of the oropharynx become dried and cracked with
increased risk of infection
Altered speech; palate is needed to trap air in the mouth
Dental development ;excessive dental caries; malocclusion from displacement of
the maxillary arch
Hearing problems caused by recurrent otitis media (Eustachian tube connects the
nasopahrynx and middle ear and transports pathogens to ear)
Intestinal Obstruction
A blockage prevents the normal flow of intestinal contents through the intestinal tract
Pathogenesis:
Intestinal contents, fluids, and gas accumulate above the intestinal the intestinal
obstruction. The abdominal distention and retention of fluid reduce the absorption
of fluids and stimulate more gastric secretions. With increasing distention,
pressure within the intestinal lumen increases, causing a decrease in venous and
arteriolar capillary pressure. This causes edema, congestion, necrosis, and
eventual rupture or perforation of the intestinal wall, with resultant peritonitis.
The initial symptom is usually crampy pain that is wavelike and colicky. The
patient may pass blood and mucus but no fecal matter and flatus. Vomiting
occurs. If obstruction is complete the peristaltic waves becomes extremely
vigorous and eventually assume a reverse direction. If obstruction is in the ileum,
fecal vomiting takes place.
First the patient vomits the stomach contents, then the bile-stained contents of the
duodenum and jejunum, and finally the darker, fecal matter contents of the ileum.
If the obstruction is not corrected , hypovolemic shock occurs from dehydration
and loss of plasma volume
Failure of the membrane separating the rectum from the anus to absorb during eight week
of fetal life; fistulas within the vagina, urinary tract, or scrotum are common; most
frequent intestinal anomaly
Pathogenesis:
The embryogenesis of these malformations remains unclear. The rectum and anus
are believed to develop from the dorsal potion of the hindgut or cloacal cavity
when lateral ingrowth of the mesenchyme forms the urorectal septum in the
midline. This septum separates the rectum and anal canal dorsally from the
bladder and urethra. The cloacal duct is a small communication between the 2
portions of the hindgut. Downgrowth of the urorectal septum is believed to close
this duct by 7 weeks' gestation. During this time, the ventral urogenital portion
acquires an external opening; the dorsal anal membrane opens later. The anus
develops by a fusion of the anal tubercles and an external invagination, known as
the proctodeum, which deepens toward the rectum but is separated from it by the
anal membrane. This separating membrane should disintegrate at 8 weeks'
gestation.
Interference with anorectal structure development at varying stages leads to
various anomalies, ranging from anal stenosis, incomplete rupture of the anal
membrane, or anal agenesis to complete failure of the upper portion of the cloaca
to descend and failure of the proctodeum to invaginate. Continued communication
between the urogenital tract and rectal portions of the cloacal plate causes
rectourethral fistulas or rectovestibular fistulas.
The external anal sphincter, derived from exterior mesoderm, is usually present
but has varying degrees of formation, ranging from robust muscle (perineal or
vestibular fistula) to virtually no muscle (complex long–common-channel cloaca,
prostatic or bladder-neck fistula).
Clinical signs:
Pyloric Stenosis
Pathogenesis:
Absence of parasympathetic ganglion cells in a portion of the bowel, which causes the
enlargement of the bowel proximal to the defect
Pathogenesis:
Three neuronal plexus innervate the intestine: the submucosal (i.e., Meissen)
plexus, the intermuscular (i.e., Auerbach) plexus, and the smaller mucosal plexus.
All of these plexus are finely integrated and involved in all aspects of bowel
function, including absorption, secretion, motility, and blood flow.
Proximal to the narrow affected section, the colon is dilated filled with fecal
material and gas; hypertrophy of muscular coating; ulceration of mucosa may be
seen in newborn
Pathogenesis:
Ventricular septal defect is an abnormal communication between the right and left
ventricle. Accounts for 25% of all CHDs.
Blood flows from the high-pressure left ventricle across the VSD into the low-
pressure right ventricle and into the pulmonary artery, resulting in pulmonary
over-circulation
Due to higher pressure in the left ventricle, a shunting of blood from left to right
ventricle occurs during systole. If pulmonary vascular resistance produces
pulmonary hypertension, the shunt of blood is then reversed from the right to left
ventricle with cyanosis resulting.
Commons signs and symptoms are tachypnea, tachycardia, excessive sweating
associated with feeding, poor weight gain, there will also be low, harsh murmur
heard throughout systole.
An abnormal communication between the left and right atrias. ASDs account for 9% of
congenital heart defects.
Types:
1. Ostium secundum ASD: the most common type of ASD; abnormal opening
in the middle of the arterial septum.
2. Ostium primum ASD: abnormal opening at the bottom of the atrial septum
3. Sinus venosus ASD: abnormal opening at the top of the atrial septum.
Pathophysiology and clinical signs:
Blood flow from the higher-pressure left atrium across the ASD into the lower-
pressure left atrium (left to right shunt)
Increased blood return to the right heart leads to right ventricular volume
overload and right ventricular dilation
Atrial septal defect is usually asymptomatic but may also manifest frequent
URIs, poor weight gain and decreased exercise tolerance. Murmur may also be
heard high on chest, with fixed splitting of the second heart sound.
Failure of the fetal connection between the aorta and pulmonary artery to close
Pathophysiology and clinical signs:
During fetal life, the ductus arteriosus allows blood to bypass the pulmonary
circulation (fetus receives oxygen from the placenta) and flow directly into the
systemic circulation.
After birth the ductus arteriosus is no longer needed. The PDA usually closes in
the first few days of life.
When the ductus aretriosus fails to close, blood from the aorta (high pressure)
flows into the low-pressure pulmonary artery, resulting in pulmonary
overcirculation.
Small to moderate size PDA is usually asymptomatic while large PDA may
manifest CHF, tachypnea, poor weight gain, failure to thrive. Decreased exercise
tolerance.
Machinery-type murmur heard throughout the heartbeat in the left second or
third interspace.
Tetralogy of Fallot
Tetralogy of fallot is the most common complex congenital heart defect; it accounts for
6% to 10% of all CHD
Types:
Degree of cyanosis depends on the size of the VSD and the degree of right
ventricular outflow tract obstruction (RVOTO)
Obstruction of blood flow from the right ventricle to the pulmonary artery results
in deoxygenated blood being shunted across the VSD and into the aorta (right-to-
left shunt cyanosis)
Right ventricular outflow tract obstruction can occur at any or all of the following
three levels: pulmonary valve stenosis, infundibular hypertrophy, or supravalvular
stenosis
The right ventricle becomes hypertrophied as a result of the increased gradient
across the RVOT
TGA occurs when the pulmonary artery arises off the left ventricle and the aorta arises
off the right ventricle. It accounts for 5% to 10% of congenital heart defects.
NEUROLOGIC MALFORMATIONS/CONDITION
Spina Bifida
Refers to malformation of the spine in which the posterior portion of the laminae of the
vertebrae fails to close.
Pathogenesis:
Hydrocephalus:
Pathogenesis:
Meningitis
Pathogenesis:
Pathophysiology:
Hypospadias:
a. In males the urethra open on the lower surface of the penis from just behind
the glans to the perineum (placement varies)
b. In females the urethra opens into vagina ( rare)
Epispadias:
Pathogenesis:
Possibly caused by decreased testosterone production in early gestation
Clinical Signs:
Inability to void with penis in the normal elevated position
In females, urine dibbling from vagina
Usually not difficult to diagnose, because anomaly is very visual.
Cryptorchidism
Congenital disorder in which one or both testes fails to descend into scrotum, remaining
in the abdomen or inguinal canal or at the external ring of the inguinal canal
Pathogenesis:
SKELETAL MALFORMATION
Pathogenesis:
Exact etiology is unknown. Suggested contributing factors include:
a. Intrauterine positioning
b. Primary arrest in fetal development
c. Familial tendency (about 10% of cases)
d. Neuromuscular defect
Celiac Disease
Also called gluten-sensitive enteropathy or celiac sprue, is a disease of the small intestine
characterized by a permanent inability to tolerate dietary gluten.
Pathogenesis:
HEMATOLOGIC CONDITIONS
Leukemia
Pathogenesis:
Cause is unknown but is linked to exposure to radiation, certain chemical and
drugs such as benzene, and some chemotherapeutic agents,viruses, and genetic
abnormalities (down syndrome)
Classifications:
a. Acute lymphocytic leukemia: abnormal growth of lymphocytic precursors
(lymphoblasts) –most common type in young children and adults age ^% and
older; better prognosis than myelogenous. About 85 % incidence.
b. Acute myelogenous leukemia: rapid accumulation of myeloid precursors
(myeloblasts)- can affect children and adults; about 15% incidence and with
poorer prognosis
Immature nonfunctioning WBCs appear to accumulate first in the tissue where
they originate,such as lymphocytes in lymph tissue and granulocytes in bone
marrow
Immature, nonfunctioning WBCs spill into the bloodstream and overwhelm red
blood cells and platelets; from there, they infiltrate other tissues
Because of possible relapse, it commonly takes years to determine whether a
patient is cured or is just in long-term remission
Sudden onset of high fever resulting from bone marrow invasion and cellular
proliferation within the bone marrow
Thrombocytopenia and abnormal bleeding secondary to bone marrow suppression
Weakness and lassitude related to anemia from bone marrow invasion
Pallor and weakness related to anemia
Chills and recurrent infections related to proliferation of immune, nonfunctioning
WBCs
Bone pain from leukemic infiltration of bone
Liver, spleens, and lymph node enlargement related to leukemic cell infiltration
Hemophilia
Pathogenesis:
Defect in specific gene on the X chromosome that codes for factor VIII synthesis
(Hemophilia A)
More than 300 different base-pair substitution involving factor IX gene on the X
chromosome (hemophilia B)
Classification
a. Hemophilia A (classic Hemophilia): deficiency of clotting factor VIII; more
common than type B, affecting more than 80% of all patients with hemophilia
b. Hemophila B (chirstmas Disease): affects 15% of all patients with hemophilia
and a result from a factor IX defiency.
A low level of the blood protein necessary for clotting causes disruption of the
normal intrinsic cpagulation cascade
Factors VIII and IX are components of intrinsic clotting pathway, factor IX is an
essential factor, and factor VIII is a critical cofactor ( accelerates the activation of
factor X by several thousand-fold)
Excessive bleeding occurs when these clotting factors are reduced by more than
75%; bleeding may be mild, moderate, or sever, depending on the degree of
activation of clotting factors.
A person with hemophilia forms a platelet plug at the bleeding site, but clotting
factor deficiency impairs the ability to form a stable fibrin clot
Delayed bleeding s more common than immediate hemorrhage
References:
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