PEDIATRIC 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 g rowth 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 A.Heart rate decreases with increasing age B.Blood pressure increases with age 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
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8/2/2019 Growth and Development with Pediatric Condition
Urinary System1. 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 valuesbetween 1-2 years ; gradually decreases after 20 years
Digestive System1. Stomach is small gradually increases during infancy and childhood
2. Peristaltic activity decreases with advancing age
3. Blood glucose level rise from infancy to adolescence4. 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 System1. 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 increase3. Maturation of the brainstem and spinal cord follows cepahlocaudal and proximodistal
laws
THE INFANT
1 monthA. 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 life3. 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 prone2. 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 presentC. Sensory
1. Eye movements coordinated most of the time; follows a light to midline
2. Visual acuity 20/100 to 20/50D. Socialization and vocalization
1. Watches face intently while being spoken to
2. Utters small, throaty sounds
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2. Build tower of two blocks; enjoys throwing objects and picking them up
3. Drinks from a cup and can use spoonB. 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 drawing3. Drinks well in cup; manages spoon well
4. Builds tower of three or four cubesC. Vocalization and socialization
1. Says 10 words or more
2. New awareness of strangers3. 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 cm3. Teeth: 16 temporary; begins visits to dentist
B. Motor
1. Gross motor skills quite refined2. Can walk up and down stairs
3. Builds tower of six to seven cubes or will make cube into a train
C. Sensory1. 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 pronouns2. Obeys simple commands, shows signs of increasing autonomy and individuality;
makes simple choices when possible
3. Can help undress self and pull on simple clothes4. Does not share possessions, everything is ―mine‖
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E. Imitating and make-believe play starts by end of the second year
F. Suggested toys: play furniture, dishes, cooking utensils, puzzles, pedal-propelled toys,clay, etc.
Health Promotion for toddlers
A. Nutritional objectives
1. Provide adequate nutrient intake to meet continuing growth and development2. 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 levelsB. Diet: calorie and nutrient requirements increase with age
Injury Prevention
A.
More than half of accidental child deaths are related to automobiles and fireB. Accidents can be viewed in terms of child’s growth and development, especially curiosity
about the environment1. Motor vehicle
2. Burns
a. Investigating: pulls off pot off the stove; plays with matches; inserts an object towall 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 mouthb. 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 waterb. Child is unaware of inability to breathe under water
5. Aspiration
a. Puts everything in mouthb. Very interested in body and newly found openings
6. Fractures
a. Climbing, running, and jumpingb. 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
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1. Vocabulary of about 2100 words; talks constantly; asks meaning of new words2. Generally cooperative and sympathetic towards others
3. Basic personality structure is well established
E. Mental abilities (Piaget’s phase of intuitive thought )
1.
Beginning understanding of time terms of days as part of a week 2. Beginning understanding the conversion of numbers
Play during Preschool Years (cooperative Play)
A. Loosely organized group play where membership changes readily, as do rulesB. Through play, child deals with reality, learns control of feeling, and expresses emotions
more through actions than through word
C. Play is still physically oriented but also imitative and imaginary
D. Increasing sharing and cooperation among preschool children, especially 5-year-old
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
Health Problems most common in school-aged children
A. Reactions of the school-aged child
1. Usually handles separation well but prefers parents to be near
2.
Fears the unknown, especially when dependency or loss of control is expected; fearsbodily harm, especially disfigurement 3. Possesses realistic concept of death by 9 to 10 years old
4. Wants to know scientific rationale fro treatments and procedures
B. If possible parents can be helped to prepare the child beforehand, since increased
cognitive and verbal ability makes explanations possible
COMMON PEDIATRIC CONDITIONS:
Chromosomal Aberrations
Trisomy 21 (down Syndrome)
Spontaneous chromosomal abnormality that causes characteristic facial features,
other distinctive physical abnormalities and mental retardation
Median life expectancy of 49 years (significantly increased [from 25 years in 1983]by improved treatment for heart defects, respiratory and other infections, and acute
leukemia)
Pathogenesis:
Nearly all cases of Down syndrome result from Trisomy 21- Produces three copies of chromosome 21 instead of normal two because of
faulty meiosis (nonjunction) of the ovum or, sometimes, the sperm
- Results in karyotype of 47 chromosomes instead of the usual 46
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May also result from an unbalanced translocation or chromosomal rearrangement
in which the long arm of chromosome 21 breaks and attaches to anotherchromosome
Some affected, may have chromosomal mosaicism, a mixture of normal cells and
cells are trisomic (usually leads to less severe phenotype)
Pathophysiology and clinical signs:
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 chromosomalaberration
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
Sex-chromosomal abnormality of XXY in males
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 variablephysical 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 .
Pathophysiology and clinical signs:
Skeletal and cardiovascular abnormalities can become increasingly severe.
Gonadal development is particularly susceptible to each additional X
chromosome, resulting in seminiferous tubule dysgenesis and infertility, as wellas hypoplastic and malformed genitalia in polysomy X males. Moreover, mental
capacity diminishes with additional X chromosomes. The intelligence quotient
(IQ) score is reduced by approximately 15 points for each supernumerary Xchromosome, but conclusions about reduced mental capacity must be drawn
cautiously. All major areas of development, including expressive and receptive
language and coordination, are affected by extra X chromosome material
The major consequences of the extra sex chromosome, usually acquired through
an error of nondisjunction during parental gametogenesis, includehypogonadism, gynecomastia, and psychosocial problems.
Klinefelter's syndrome is a form of primary testicular failure, with elevated
gonadotropin levels due to lack of feedback inhibition by the pituitary gland.
Androgen deficiency causes eunuchoid body proportions; sparse or absent facial,axillary, pubic, or body hair; decreased muscle mass and strength; feminine
distribution of adipose tissue; gynecomastia; small testes and penis; diminished
libido; decreased physical endurance; and osteoporosis. The loss of functionalseminiferous tubules and Sertoli cells results in a marked decrease in inhibin B
levels, which is presumably the hormone regulator of the follicle-stimulating
hormone (FSH) level. The hypothalamic-pituitary-gonadal axis is altered inpubertal patients with Klinefelter’s syndrome.
Gastrointestinal Malformations
Cleft Lip and Cleft Palate
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 thepalatine 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 andpalatine 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 alveoluson one or both sides of the premaxilla
Pathophysiology and clinical signs:
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
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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:
Mechanical obstruction: an intraluminal obstruction or a mural obstruction from
pressure on the intestinal wall occurs. Examples are stenosis, strictures, adhesions,
hernias, and abscesses
Functional obstruction: the intestinal musculature cannot propel the contentsalong the bowel. Examples are amyloidosis, muscular dystrophy, neurologic
disorders like Parkinson’s diseases. The blockage can also be temporary and theresult of manipulation of the bowel during surgery
Pathophysiology and clinical signs:
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. Thepatient 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
Anorectal Anomalies (imperforate anus)
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
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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 thebladder 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 portionacquires 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 theanal 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 cloacato descend and failure of the proctodeum to invaginate. Continued communication
between the urogenital tract and rectal portions of the cloacal plate causesrectourethral 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 orvestibular fistula) to virtually no muscle (complex long – common-channel cloaca,
prostatic or bladder-neck fistula).
Clinical signs:
Anal opening very near the vaginal opening in girls
Missing or misplaced opening to the anus
No passage of first stool within 24 - 48 hours after birth Stool passes out of the vagina, base of penis, scrotum, or urethra
Swollen belly area
Pyloric Stenosis
Congenital hypertrophy of muscular tissue of the pyloric sphincter, usually asymptomatic
until 2 to 4 weeks after birth
Pathogenesis:
The causes of infantile hypertrophic pyloric stenosis are multifactorial.1 Both
environmental factors and hereditary factors are believed to be contributory.Possible etiologic factors include deficiency of nitric oxide synthase containing
neurons, abnormal myenteric plexus innervation, infantile hypergastrinemia, and
exposure to macrolide antibiotics.
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Nitric oxide has been demonstrated as a major inhibitory nonadrenergic,noncholinergic neurotransmitter in the GI tract, causing relaxation of smooth
muscle of the myenteric plexus upon its release. Impairment of this neuronal
nitric oxide synthase (nNOS) synthesis has been implicated in infantile
hypertrophic pyloric stenosis, in addition to achalasia, diabetic gastroparesis,and Hirschsprung disease
Pathophysiology and clinical signs:
Marked hypertrophy and hyperplasia of the 2 (circular and longitudinal) muscular
layers of the pylorus occurs, leading to narrowing of the gastric antrum. Thepyloric canal becomes lengthened, and the whole pylorus becomes thickened. The
mucosa is usually edematous and thickened. In advanced cases, the stomach
becomes markedly dilated in response to near-complete obstruction.
Vomiting — The first symptom of pyloric stenosis is usually vomiting. At first it
may seem that the baby is simply spitting up frequently, but then it tends toprogress to projectile vomiting, in which the breast milk or formula is ejected
forcefully from the mouth, in an arc, sometimes over a distance of several feet.Projectile vomiting usually takes place soon after the end of a feeding, although in
some cases it may be delayed for hours. Rarely, the vomit may contain blood.
Changes in stools — Babies with pyloric stenosis usually have fewer, smallerstools because little or no food is reaching the intestines. Constipation or stools
that have mucus in them may also be symptoms.
Failure to gain weight and lethargy — Most babies with pyloric stenosis will fail
to gain weight or will lose weight. As the condition worsens, they are at risk fordeveloping fluid and salt abnormalities and becoming dehydrated.
Visible peristalsis: palpable olive-shaped mass in the right upper quadrant as the
stomach tries to empty itself against the thickened pylorus.
Megacolon (Hirschprung’s Disease)
Absence of parasympathetic ganglion cells in a portion of the bowel, which causes the
enlargement of the bowel proximal to the defect
Pathogenesis:
Congenital aganglionosis of the distal bowel defines Hirschsprung disease.
Aganglionosis begins with the anus, which is always involved, and continuesproximally for a variable distance. Both the myenteric (Auerbach) plexus and the
submucosal (Meissner) plexus are absent, resulting in reduced bowel peristalsis
and function. The precise mechanism underlying the development of Hirschsprung disease is unknown.
Enteric ganglion cells are derived from the neural crest. During normal
development, neuroblasts will be found in the small intestine by the 7th week of gestation and will reach the colon by the 12th week of gestation. One possible
etiology for Hirschsprung disease is a defect in the migration of these neuroblasts
down their path to the distal intestine. Alternatively, normal migration may occur
with a failure of neuroblasts to survive, proliferate, or differentiate in the distalaganglionic segment. Abnormal distribution in affected intestine of components
required for neuronal growth and development, such as fibronectin, laminin,
neural cell adhesion molecule (NCAM), and neurotrophic factors, may beresponsible for this theory
Pathophysiology and clinical signs:
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 bowelfunction, including absorption, secretion, motility, and blood flow.
Normal motility is primarily under the control of intrinsic neurons. Bowel
function is adequate, despite a loss of extrinsic innervations. These ganglia
control both contraction and relaxation of smooth muscle, with relaxationpredominating. Extrinsic control is mainly through the cholinergic and adrenergic
fibers. The cholinergic fibers cause contraction, and the adrenergic fibers mainly
cause inhibition.
No peristaltic occurs in the affected portion of intestine (spastic and contracted)
the section is usually narrowed; therefore no fecal material passes through it. The
intestine above the affected section has an accumulation of fecal material.
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
Hirschsprung disease should be considered in any newborn with delayed passage
of meconium or in any child with a history of chronic constipation since birth.Other symptoms include bowel obstruction with bilious vomiting, abdominal
distention, poor feeding, and failure to thrive. Ribbon-like or pellet stool may also
be noted caused by contracted affected portion and failure of internal rectal
90% of all Congenital Heart Disease (defects) is unknown but is associated to
fetal or maternal infection during the first trimester, chromosomal abnormalities,or exposure to teratogens during fetal development.
Left to Right Shunts
Ventricular Septal Defect
Ventricular septal defect is an abnormal communication between the right and left
ventricle. Accounts for 25% of all CHDs.
Pathophysiology and clinical signs:
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 rightventricle 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 sweatingassociated with feeding, poor weight gain, there will also be low, harsh murmur
heard throughout systole.
Atrial Septal Defect
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 openingin 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.
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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 volumeoverload 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 beheard high on chest, with fixed splitting of the second heart sound.
Patent Ductus Arteriosus
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 pulmonarycirculation (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 inthe 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 orthird interspace.
Right to Left Shunt
Tetralogy of Fallot
Tetralogy of fallot is the most common complex congenital heart defect; it accounts for
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 resultsin 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 followingthree levels: pulmonary valve stenosis, infundibular hypertrophy, or supravalvular
stenosis
The right ventricle becomes hypertrophied as a result of the increased gradientacross the RVOT
Transposition of the Great Arteries/ Vessels
TGA occurs when the pulmonary artery arises off the left ventricle and the aorta arisesoff the right ventricle. It accounts for 5% to 10% of congenital heart defects.
Pathophysiology and clinical signs:
This defect results in two parallel circulations:a. The right atrium receives deoxygenated blood from the IVC and SVC; blood flow
continues through the tricuspid valve into the right ventricle and is pumped back
to the aorta.
b. The left atrium receives richly oxygenated blood from the pulmonary veins; bloodflow continues through the mitral valve into the left ventricle and is pumped back
into the pulmonary artery
To sustain life, there must be an accompanying defect ( PDA, ASD, or VSD) that
allows mixing of deoxygenated blood and oxygenated blood between the two circuit Symptoms evident soon after birth; clinical scenario is influenced by the extent of
intercirculatory mixing. Cyanosis, tachypnea, and metabolic acidosis may be noted.
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:
Unknown etiology but generally thought to result from genetic predisposition
triggered by something in the environment.
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a. Certain drugs, including valporic acid, have been known to cause neural tube
defects if administered during pregnancyb. Women who have spina bififda and parents who have one affected child have an
increased risk of producing children with neural tube defects
Several types of spina bifida are recognized of which three are most common:
a.
Spina bifida occulta The defect is only in the vertebrae.the spinal cord and meninges are
normal
b. Meningocele The meninges protrude through the opening in the spinal canal. This form
a cyst filled with CSF and covered with skin
c. Myelomeningocele Both the spinal cord and cord membranes protrude through thr defect in
the laminae of the vertebral column.
Pathophysiology and clinical signs:
Possibly ,a depression or dimple,tuft hair, soft fatty deposits,port wine nevi, or acombination of these abnormalities on the skin over the spinal defect due to
incomplete closure of one or more vertebrae without protrusion of spinal cord or
meninges ( spina bifida occulta)
Saclike structure that protrudes over the spine,trophic skin disturbances, and
ulcerations (myelomeningocele,meningocele) due to incomplete closure of one or
more vertebrae with protrusion of spinal content
Depending on the level of the defect, permanent neurologic dysfunction, such asflaccid or spastic paralysis and bowel and bladder incontinence.`
Hydrocephalus:
Is a condition of latered production, flow, or absorption of cerebrospinal fluid (CSF). It is
characterized by an abnormal increase in CSF volume within the intracranial cavity and
by enlargement of the head in infancy.
Pathogenesis:
Noncommunicating hydrocephalus – obstruction in the system between the source
of CSF production (ventricles) and the area of its reabsorption (subarachnoid
space)
a.
May be partial,intermittent,or complete.b. Occurs in the majority of cases
c. Caused by congenital defects such as Arnold-Chiari malformation,Dandy-
walker cyst, aqueductal stenosis (neurofibromatosis)d. Also caused by acquired conditions such as infections,trauma,spontaneous
intracranial bleeding,and neoplasms.
Communicating hydrocephalus:
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a. Failure in the absorption system; cause is unknown
b. Excessive production of CSF- tumor or unknown causes (rare)
Pathophysiology and Clinical Signs:
The ventricular system becomes greatly distended.a. The increased ventricular pressure results in thinning of the cerebral cortex
and cranial bones, especially in the frontal, parietal, and temporal areas.
b. The floor of the third ventricle commonly bulges downward, compresses theoptic nerves, dilates the sella turcica, and often compresses the hypophysis
cerebri.
c. The basal ganglia, brain stem, and cerebellum remain relatively normal butcompressed.
d. The choroid plexus is usually atrophied at some degree.
In infants clinical signs may show signs of increased intracranial pressure such as
high-pitched,shrill cry, restlessness and irritability, excessive head growth due to
open sutures, bulging of fontanels, papilledema of retina, seizures, sunset eyes,head lags may also be noted especially important after 4 to 6 months.
Meningitis
Inflammation of the brain and spinal meninges
Pathogenesis:
Usually results from bacterial, virus, fungus, parasite, or other toxin- Acute pyrogenic,which is commonly caused by streptococcus pneumonia,
heamophilus influenza, neisseria minigitidis, or escheriachia coli
-
Acute lymphatic ,which is viral in origin
Inflammation of the pia-arachnoid and subarachnoid space progresses to
congestion of adjacent tissues and nerve cell destruction
ICP increases because of exudates Theses changes lead to engorged blood vessels, disrupted cerebral blood supply,
possible thrombosis, or rupture and cerebral infarction
Pathophysiology:
Fever, chills,malaise, petechial rash, and tachycardia resulting from from
infection and inflammation
Headache, vomiting and rarely,papilledema ( inflammationand edema of the optic
nerve) due to increased ICP
Nuchal rigidity,positive Brudsinki’s and kergnig’s signs, exaggerated andsymmetrical deep tendon reflexes, and opisthotonos due to meningeal irritation
Photophobia, diplopia,and other viosion problems resulting from cranial nerve
irritation
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The cause in unknown; although it appears to require interaction between a
number of intrinsic factors ( genetic susceptibility, activation of immune system)
and extrinsic factors (gluten and possibly other environmental factors). Both
cellular immunity and humoral immunity are implicated. There is a familialtendency with association of specific HLA antigens.
Pathophysiology and Clinical Signs:
Characterized of celiac disease include the following:
a. Impaired intestinal absorption probably results from decreased area of absorption in small bowel and impaired enzyme activity
b. Histologic abnormalities of the small intestine
c. Clinical and histologic improvement with wheat, rye-, barley, and possibly
oat-free diet
d.
Recurrence of clinical manifestations and histologic changes afterreintroduction of dietary gluten
e. Clinical manifestations may include anorexia, muscle wasting,watery,pale,foul-smelling stool, celiac crises may also be noted: severe
episode of dehydration and acidosis from diahhrea, the body is also unable to
absorb fats: steatorrhea.
HEMATOLOGIC CONDITIONS
Leukemia
Group of malignant disorder characterized by abnormal proliferation and maturation of
lymphocytes and nonlymphocytic cells, leading to suppression of normal cells
Pathogenesis:
Cause is unknown but is linked to exposure to radiation, certain chemical anddrugs 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 wherethey originate,such as lymphocytes in lymph tissue and granulocytes in bone
marrow
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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
Pathophysiology and clinical signs:
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
A condition in which there is defect in clotting mechanism of blood; genetic disorder, x-
linked recessive transmission
Usually occurs in males; females are carriers but do not have the disease
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 Xchromosome (hemophilia B)
Classificationa. 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 anessential factor, and factor VIII is a critical cofactor ( accelerates the activation of
factor X by several thousand-fold)
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