Pediatric Nursing Allan C.

Santos, MD Growth measurements Recumbent length for infants up to age 36 months + weight and head circumference Standing height + weight after age 37 months GROWTH Ethnic differences Expected growth rates at various ages Skin fold thickness and arm circumference Head circumference measurements Infant and Toddler Vital Signs 1) Count respirations FIRST (before disturbing the child) 2) Count apical HR SECOND 3) Measure BP (if applicable) THIRD 4) Measure temperature LAST Pediatric Blood Pressures Measurement devices Cuff selection Cuff placement Interpretation of BP measurement Physical Assessment General appearance Skin Hair, nails, hygiene Lymph nodes

Head and neck EENT Physical Assessment (contd) Chest Heart Lungs Abdomen Genitalia Back and extremities Neurologic assessment Developmental Assessment Screening procedures To identify children whose developmental level is below normal for chronologic age and who therefore require further investigation Denver Developmental Screening Test II AKA Denver II Widely used, standardized measures Examiners must be specifically trained and certified in use of the tools Interpretation of test Recommendations/referrals Growth and Development Growth- an increase in the number and size of cells as they divide and synthesize new proteins; results in an increased size and weight of the whole or any of its parts Development- a gradual change and expansion; the emerging and expanding of individuals capacities through growth, maturation and learning. Maturation- an increase in competence and adaptability; to function at a higher level; a qualitative change

 Differentiation- development from simple to more complex activities and functions Developmental Task- a set of skills and competencies peculiar to each developmental stage that children must accomplish or master in order to deal effectively with their environment Growth and Development- must be continuous, orderly and progressive Directional Trends: 1. Cephalocaudal 2. Proximodistal * the first two appear symmetric and bilateral 3. Differentiation - from simple to more complex activities and functions - from very broad, global patterns of behavior, more specific, refined patterns emerge Sequential Trends: - in all dimensions of growth and development there is a definite, predictable sequence with each child normally passing through every stage. Developmental Pace - it does not progress at the same rate or pace in all individuals - there are periods of accelerated or decelerated growth of the whole body or its systems Sensitive Periods - are those times in a lifetime of an organism when it is more susceptible to positive and negative influences. Biologic Growth and Physical Development External Proportions - cephalocaudal trend - growth of the head is fastest during fetal life - growth of the trunk predominates during infancy

- growth of the head is fastest during childhood - during adolescence, trunk elongates again Linear growth (height) - result of skeletal growth and considered a stable measurement of skeletal growth - the maximum growth in length occurs before birth, but the newborn continues to grow at a rapid but at a relatively slower rate. Weight - reflective of the intrauterine environment to a greater extent Weight - doubles by 4th to 7th month - triples by the end of the first year - quadruples by the end of 2nd year - there will be a steady annual increase thereafter (2 to 2.75kg/year until adolescent growth spurt) Indicators of Development 1. bone age- most accurate measure of development ( radiologic determination of osseous maturation) 2. dentition Bone Formation - starts during the second month of intrauterine life (appearance of first centers of ossification) - at birth, approximately 400 o.c. appear; half remains at maturity - provides basis for assessment of bone age - the earliest to appear are those of the hamate and capitate (5th to 6th months of age) - radiographs of the hands and wrists provide the most useful areas for screening to determine skeletal age before the age of 6 years

 Neurologic Maturation: - the nervous system grows more rapidly before birth than organ systems - two periods of rapid brain growth occurs during; 1. 15th to 20th week AOG 2. 30th week AOG -extends till 1 year of life - continues during early childhood and then slows to a more gradual rate during later childhood and adolescence - no new nerve cells appear after the 6th month of fetal life Lymphoid Tissues: - lymph nodes, thymus, spleen, tonsils, adenoids and blood lymphocytes follow a growth pattern different from that of other body tissues - 6th to 12th years of age, twice the size than the adult size Physiologic Changes Metabolism: - BMR highest in a newborn infant - BMR slightly higher in boys than girls at all ages and further increases during pubescence over that in girls - BMR; 1. infants- 108kcal/kgBW 2. maturity- 45kcal/kgBW - water requirement; *remain at 1.5ml/cal of energy throughout life Temperature: - body temperature, reflecting metabolism, during the process of development decreases expended

- females maintain a relatively higher body than males throughout life Sleep and Rest: - a protective function on all human organisms, recovery and repair of tissues after activity Sleep and Rest:

temperature

allow for

- as infant grows older, the total time spent in sleep gradually decreases, they remain awake for longer periods, and they sleep longer at night. - the time spent in deep, restful sleep, increases 50% during infancy to 80% in older child. Temperament The manner of thinking, behaving or reacting characteristic of an individual. Refers on the way a person deals with life. Easy child and a difficult child Provides a useful tool for caregivers in anticipating probable areas of difficulty or risks associated with development. *difficult children may be more prone to colic in infancy *active children require more vigilance to prevent injury Development of Personality and Mental Function Theoretical Foundations of Personality Development 1. Psychosexual Development (Freud) 2. Psychosocial Development (Erickson) Theoretical Foundations of Mental Development 1. Cognitive Development (Piaget) 2. Language Development 3. Moral Development (Kohlberg) from

4. Spiritual Development Development of Self Concept Body Image Self-Esteem Theory of Freud All human behavior is energized by psychodynamic forces -3 components: 1. id- the unconscious mind - inborn component that is by instincts - the pleasure principle 2. ego- the conscious mind, serves principle 2. ego- conscious or the controlling self that is able to find realistic means for gratifying the instincts while blocking the irrational thinking of the id. 3. superego- the conscience - functions as the moral arbitrator and represents the ideal. Psychosexual Development of Freud Oral Stage: - from birth to 1 year - sucking, biting, chewing and vocalizing are the sources of pleasure Anal Stage: - from 1 to 3 years - interest centers in the anal region as the muscles develop sphincter major the reality driven

 Phallic Stage: - from 3 to 6 years - genitals become an interesting and area of the body - recognition of sexual differences and curiosity dissimilarities - Oedipus and Electra complexes - penis envy and castration anxiety Latency Period - from 6 to 12 years old - elaborate on previously acquired traits and skills - physical and psychic energy are channeled into acquisition of knowledge and vigorous play Genital Stage - 12 years old and above - begins at puberty with maturation of the reproductive system and production of sex Genital Stage - the genital organs become the major source of tension and pleasures, but energies are also invested in forming friendships and preparation for marriage. Psychosocial Development by Erickson The most widely accepted of personality development. Built on Freudian Theory. Emphasizes a healthy personality as opposed to the pathologic approach. Stages: 1. Trust versus Mistrust - from birth to 1 year sexual hormones sensitive about the

- establishment of the basic trust dominates the first year of life and describes all the childs satisfying experiences of this age. - it is a time of getting and taking in senses - consistent, loving care by the mother - the result is faith and optimism 2. Autonomy versus Shame and Doubt - from 1 to 3 years - the problem of autonomy can be holding on and letting go the sphincters -centered on the childrens increasing themselves, their bodies and their environment - they want to do things for themselves - much of their learning is acquired through activities and behavior of others. - the favorable outcomes are self-control and 3. Initiative versus Guilt - from 3 to 6 years - characterized by vigorous and intrusive enterprise and a strong imagination - explore the physical world with all their powers. - they develop a conscience. - they must learn to retain a sense of impinging on the rights and privileges of others - the lasting outcomes are direction and 4. Industry versus Inferiority - from 6 to 12 years initiative without purpose behavior, senses and imitating the will power symbolized by the ability to control through all the

- they are ready to be workers and - they want to engage in tasks and activities through to completion. - they want and need real achievements - children learn to compete and cooperate - a decisive period of their social with others - the ego developed from a sense of industry 5. Identity versus Role Confusion - from 12 to 18 years old - characterized by a rapid and marked - overly preoccupied with the way they of others as compared to their own self concept

producers they can carry

with others relationship is competence

physical changes appear in the eyes

- struggle to fit the roles they have played and those they hope to play with the current roles and fashion adopted by their peers - the outcome of successful mastery is devotion and fidelity to others and to Cognitive Development by Piaget Cognition - refers to the process developing individuals become acquainted with the world and the object it contains. - language, morals and spiritual development emerge as cognitive abilities advance. Intelligence enables individuals to make adaptations to the environment that increase the probability of survival and through their behavior individuals establish and maintains equilibrium with the environment. Stages: 1. Sensorimotor - from birth to 2 years the values and ideologies

- governed by sensations in which simple learning takes place - develop a sense of cause and effect as they direct behavior toward objects - problem solving is primarily trial and error - they display a high level of curiosity, experimentation and enjoyment of novelty and begin to develop a sense of self vs environment - awareness of permanence of objects - begin to use language and representational 2. Preoperational Thought - from 2 to 7 years - the predominant characteristic of development is egocentrism - view things in their own perspective - thinking is concrete and tangible - in the latter stage, reasoning is intuitive transductive 3. Concrete Operations - from 7 to 11 years - thought becomes increasingly logical and coherent and also intellectual thought.

- ability to classify, sort, order, and otherwise organize facts about the world to use in problem solving - develop a new concept of permanence - do not have the capacity to deal in - reasoning is inductive - thought becomes less self centered 4. Formal Operations - from 11 to 15 years - characterized by adaptability and flexibility (conservation) abstraction

- can think in abstract terms, use abstract symbols and draw logical conclusions from a set of observations. Cognitive Impairment Any type of mental difficulty or deficiency Mental retardation (MR) Early Behavioral Signs of Cognitive Impairment Non-response to contact, voice, movement Irritability Poor/slow feeding Poor eye contact during feeding Diminished spontaneous activity Mental Retardation -American Academy of Mental Retardation Sub-average intellectual function Onset before age 18 Functional impairments (IQ <75)

Functional Impairments (at least 2 of these 10) Communication Home living Community use Leisure Health and safety Self care Social skills Functional academics

Work Self-direction Causes of Mental Retardation Intrauterine infection and intoxication Trauma (prenatal, perinatal, postnatal) Metabolic or endocrine disorders Inadequate nutrition Postnatal brain disease Causes of Mental Retardation (contd) Chromosomal anomalies Prematurity, LBW, postmaturity Environmental influences Unknown prenatal influences Psychiatric disorders with onset in childhood Classification of MR Educable/mildIQ 50-75 Trainable/moderateIQ 35-55 SevereIQ 20-40 ProfoundIQ <20-25 Primary Prevention of Mental Retardation Rubella immunization Genetic counseling Use of folic acid supplements Educate about fetal alcohol syndrome Educate about lead exposure Language Development

 The environment must provide a means for them to acquire and develop speech and language skills. By the time they begin to walk, children are able to attach a name to objects and persons. Moral Development by Kohlberg Preconvention Level - culturally oriented to the labels of good/bad and right/wrong, children integrate this in terms of the physical or pleasurable consequences of their actions. Conventional Level - concerned with conformity and loyalty - value the maintenance of family, group or national expectations regardless of consequences - behavior that meets with approval and pleases or helps others is considered to be good Postconventional Level - autonomous or principled level - correct behavior tends to be defined in terms of general individual rights and standards that have been examined and agreed on by the general society. *The most advanced level of moral development is one in which self chosen ethical principles guide decisions of conscience. (Justice and Human Rights) Self Concept Describes how an individual describes himself or herself. Includes all the notions, beliefs and convictions that constitutes an individuals relationship with others. Components: 1. Body image- refers to the subjective concepts and attitudes that individuals have toward their own bodies. - physiologic, psychologic, and social

- adolescence is the age when children become most concerned about the physical self. 2. Self-esteem- is the value that each individual places on oneself and refers to an overall evaluation of oneself. - the affective component of the self, whereas self concept is the cognitive component - positive feedback enhances self-esteem; they are vulnerable to feelings of worthlessness and are anxious about failure. Role of Play in Development Types of Play 1. Onlooker play 2. Solitary play 3. Parallel play 4. Associative play 5. Cooperative play Functions of play 1. Sensorimotor development 2. Intellectual Development 3. Socialization 4. Creativity 5. Self Awareness 6. Therapeutic Value 7. Moral value Selected Factors that Influence Development 1. Heredity 2. Neuroendocrine Factors 3. Nutrition 4. Interpersonal relationship

5. Socioeconomic level 6. Diseases 7. Stresses of childhood 8. Environmental hazards Developmental Milestones Neonate - complete head lag - hands fisted, thumbs in - cry without tears - visual fixation for human face 1 month - dance reflex disappears - looks at mobile objects 2 months - baby coos - closure of the posterior fontanel - head lag when put into sitting - holds head up when in prone position - social smile 3 months - hand regard - hold head and chest up when in prone position - follows object when in past midline - grasp and tonic reflex fading 4 months - babbling sound

- laugh aloud - head control complete - turns from front to back - needs space to turn 5 months - handles rattle well - moro reflex disappears - turn both sideways/roll over 6 months - reaches out in anticipation - sits with support - says vowel sounds - uses palmar grasp - eruption of first temporary teeth (lower central incisors) 7 months - beginning fear of the stranger - transfer objects from hand to hand

8 months - sits without support - peak of stranger anxiety - plantar reflex disappears 9 months - creeps and crawls - needs space for creeping

- combine two syllables - neat pincer grasp reflex

10 months - puts self to stand - respond to own name - understands the word no - plays: peek a boo, 11 months - stands with assistance - cruises 12 months - cooperate in dressing - stands alone, take the first step - walk with assistance 15 months - can speak 4-6 words - creeps upstairs - holds spoon well - puts small pellet into small pots - scribbles with pencil voluntarily - seats self on chair - walks alone 18 months - can speak 7-20 words - bowel control achieved

- can run and jump in place - favorite word is mine - name one body part - typically places both feet in 1 step before advancing - walks up and down stairs holding on to a persons hands or railings 2 years - 50-200 words - daytime bladder control achieved - still uses both feet on the same step at the same time - walks upstairs alone 30 months - make simple line or stroke - copy a circle - jumps down from stairs - knows full name - temporary teeth complete 36 months - 300-900 words - draw a cross - know how to unbutton - night time bladder control achieved - speaks fluently 4 years - 1500 words - jumps and skips - laces shoes

 5 years - 2,100 words - imaginary playmates 6 years - beginning interest in God - temporary teeth begins to fall - permanent teeth begins to appear - recognizes all shapes 7 years - age of assimilation - copy a diamond 8 years - can count backwards - expansive age - normal homosexual 9 years - coordination improves - hero worship - stealing and lying are common - tells time correctly - takes care of body needs completely - difficult to handle in school 10 years - writes legibly - ready for competitive play - more considerate and cooperative

- joins organizations - well mannered with adult 10-12 years - energetic and active - secret language - share secrets with peers - social and cooperative Signs of Sexual Maturity Females T thelarche A adrenarche M menarche Males - increase in the size of the testes and penis - deepening of the voice - appearance of axillary and pubic hair - production of viable sperm 1. The nurse is seeing an adolescent boy and his parents in the clinic for the first time. Which of the following should the nurse do first? A. Introduce self. B. Make family comfortable. C. Explain purpose of interview. D. Give assurance of privacy. 2. Which of the following is most likely to encourage parents to talk about their feelings related to their childs illness? A. Be sympathetic. B. Use direct questions.

C. Use open-ended questions. D. Avoid periods of silence. 3. What is the single most important factor to consider when communicating with children? A. The childs physical condition B. Presence or absence of the childs parent C. Developmental level of the child D. Nonverbal behaviors of the child 4.Which of the following approaches would be best to use to ensure a positive response from a toddler? A. Assume an eye-level position and talk quietly. B. Call the toddlers name while picking him or her up. C. Call the toddlers name and say, Im your nurse. D. Stand by the toddler, addressing him or her by name. 5. Because children younger than 5 years are egocentric, the nurse should do which of the following when communicating with young children? A. Focus communication on child. B. Explain experiences of others to child. C. Use easy analogies when possible. D. Assure child that communication is private. 6. The nurses approach, when introducing hospital equipment to a preschooler who seems afraid, should be based on which of the following principles? A. The child may think the equipment is alive. B. The child is too young to understand what the equipment does. C. Explaining the equipment will only increase the childs fear. D. One brief explanation will be enough to reduce the childs fear. 7. An 8-year-old girl asks the nurse how the blood-pressure apparatus works. The most appropriate nursing action is which of the following?

A. Ask her why she wants to know. B. Determine why she is so anxious. C. Explain in simple terms how it works. D. Tell her she will see how it works as it is used. 8. When the nurse interviews an adolescent, which of the following is especially important? A. Focus the discussion on the peer group. B. Allow an opportunity to express feelings. C. Emphasize that confidentiality will always be maintained. D. Use the same type of language as the adolescent. 9. The nurse must assess 10-month-old Chad. He is sitting on his fathers lap and appears to be afraid of the nurse and of what might happen next. Which of the following initial actions by the nurse would be most appropriate? A. Initiate a game of peek-a-boo. B. Ask father to place Chad on the exam table. C. Undress Chad while he is still sitting on his fathers lap. D. Talk softly to Chad while taking him from his father. 10. The nurse is taking a sexual history on an adolescent girl. Which of the following is the best way to determine whether she is sexually active? A. Ask her, Are you sexually active? B. Ask her, Are you having sex with anyone? C. Ask her, Are you having sex with a boyfriend? D. Ask both the girl and her parent if she is sexually active. 11.Which of the following parameters correlates best with measurements of the bodys total protein stores? A. Height B. Weight C. Skin-fold thickness

D. Upper arm circumference 12.Which of the following is characteristic of development of a 2 -year-old child? A. Birth weight has doubled. B. Primary dentition is complete. C. Anterior fontanel is open. D. Binocularity may be established. 13.At what age should the nurse expect a toddler to give both first and last names when asked? A. 15 months B. 18 months C. 24 months D. 30 months The Newborn Immediate Adjustments 1. The Respiratory System - the most critical and immediate physiologic change required of the newborn is the onset of breathing - triggering factors: * chemical factors * thermal stimulus * tactile stimulus - the importance of surfactant production 2. The Circulatory System - transition from fetal to postnatal circulation involves the functional closure of the fetal shunts: foramen ovale, ductus arteriosus and eventually the ductus venosus Physiologic Status of Other Systems

1. Thermoregulation - is the 2nd most critical factor to the newborn survival - factors that predispose the newborn to excessive heat loss; * relatively larger surface area * only with thin layer of * nonshivering thermogenesis - the principal thermogenic sources are the lungs, liver and the brain. - presence of brown fat the axillae, and * superficial (around the scapulae, behind the sternum) around the neck, in trachea, subcutaneous fat

* deeper layers (around the kidneys, esophagus, some major arteries and adrenals)

- also at risk of hyperthermia because of inefficient dissipation of heat in hot environment 2. Hemopoietic System - blood volume of the newborn full-term infant is 80-85ml/kgBW - blood volume depends on the placental transfer of blood *delayed clamping of the cord can add as much as 100ml of blood 3. Fluids and Electrolytes - 73% of the total body weight is water - higher ratio of extracellular fluid than adults (higher ratio of sodium and chloride than potassium, phosphate and magnesium) - relatively faster rate of fluid exchange - relatively faster metabolic rate - relatively more acid production - still with an immature renal function

4. Gastrointestinal System - the liver is the most immature of the digestive organs - deficiency of glucoronyl transferase results in the physiologic jaundice of the newborn - prone to hypoglycemia - salivary glands mature in 2nd to 3rd month (drooling) - stomach capacity (up to 90ml) - more feeding (breast milk), more stools - longer intestines in relation to body size - more gastric regurgitation - change in stool patterns in the newborn A. Meconium - should occur during the first hours, though may be delayed up to 7 days in VLBW infants B. Transitional Stools - usually appear by third day after initiation of feeding; greenish brown to yellowish brown, thin and less sticky than meconium; may contain some milk curds C. Milk Stools - usually appears by fourth and with sour milk odor in consistency and infants 5. Renal System - urine volume/24hours *200-300ml by the end of 1st week have - yellow to golden, pasty in breastfed infants - pale yellow to light brown, a more offensive odor in formula fed day consistency firmer in 24 to 48

*15ml/voiding; 20 voiding/day - first voiding should occur during the first 6. Integumentary System - many functions are still immature - epidermis and dermis are loosely bound pegs are not yet developed) - sebaceous glands *influenced initially by maternal *very active in late fetal life and *plugging causes milia - the eccrine sweat glands *are functional at birth (palmar sweating equivalents to an anxious adult at 3 weeks of age) *retention of sweat results in - the apocrine glands remain nonfunctional slower at the - boys hair grows faster than girls and in crown both sexes, hair growth is miliaria androgens early infancy with each other (rete 24 hours

- less melanin, more predisposed to harmful effects of the sun 7. Musculoskeletal System A. Skeletal System- larger amounts of cartilage than of ossified bone, rate of ossification is fairly rapid during the first year B. Muscular System- is almost completely formed at birth. 8. Defenses Against Infection - although the phagocytic properties of the blood are present in the infant, the inflammatory response of the tissues to localize an infection is immature. - 2nd month: ability to make own antibodies - 1st 3 months of infancy: protected by maternal antibodies 9. Endocrine System

- adequately developed but functionally still immature - ADH, sex hormones 10. Neurologic System - still incompletely integrated but sufficiently developed to sustain extrauterine life - most functions are primitive reflexes - ANS are crucial during transition *regulates initial respirations *thermoregulation *acid-base balance - myelination: cephalocaudal proximo-distal - all cranial nerves are myelinated except the olfactory and optic nerves 11. Sensory Functions - have a significant effect on growth and - vision: *tear glands begin to function at 2nd to 4th week of age the development

*has the ability to focus momentarily on a bright or moving object that is within 20 cm and in the midline of the visual field *visual acuity is 20/100 or 20/400 -hearing: *hearing probably similar to an - smell: *can smell breast milk - taste: adult

*during early childhood, the taste concentrated mostly on tip of the tongue - touch: *face, hands and soles are the sensitive APGAR Scoring System

buds are

most

The APGAR score is affected by degree of physiologic immaturity, infection, congenital malformation, maternal sedation and analgesia and neuromuscular sedation. Scoring significance: 0 to 3: severe distress 4 to 6: moderate difficulty 7 to 10: absence of difficulty in adjusting extrauterine life Clinical Assessment of Gestational Age Ballard Scale - the revised scale can be used to newborns weeks of gestation -appropriate for 20-44 weeks' gestation -perform in immediate post delivery period accuracy * Gestational age is more closely related to weight. Dubowitz Scale Weight Related to Gestational Age Birth weight is poor indicator of gestational and fetal maturity Gestational age reflects fetal maturity AGAgrowth between 10th and 90th percentile SGA<10th percentile fetal maturity than is birth for highest as young as 20 to

LGA>90th percentile Ballards Scoring System Neuromuscular Maturity a. Posture b. Square Window c. Arm Recoil d. Popliteal Angle e. Scarf Sign f. Heel to Ear Physical Maturity a. Skin b. Lanugo c. Plantar Surface d. Breast e. Eye/Ear f. Genitals Other Descriptions of Newborn Maturity Term = 36-42 completed weeks gestation

Preterm = <36 completed weeks gestation Post term = >42 completed weeks gestation Vital Signs Measurement in Newborns Temperature Axillary (benefit vs. risk) Rectal (benefit vs. risk) First temp to evaluate patency of anus

HRApical; 120-160 bpm WNL RR30-60 breaths/minute BPBaseline evaluation of CV status Assessment of Reflexes in the Newborn Localized Eyes 1. Blinking or Corneal Reflex 2. Pupillary Reflex 3. Dolls Eye - if persistent, indicates neurologic damage Nose 1. Sneeze 2. Glabellar Mouth and Throat 1. Sucking reflex - persists during infancy 2. Gag reflex 3. Rooting reflex - should disappear at 3rd to 4th month but may persist till the 12th month 4. Extrusion reflex - disappears by the 4th month 5. Yawning reflex 6. Coughing reflex Extremities 1. Grasping reflex

voluntary

*palmar grasp- lessens after the 3rd grasp *plantar grasp- lessens by 8th month of

month, to be replaced by age

2. Babinski reflex - disappears by one year of age 3. Ankle Clonus reflex Mass 1. Moro reflex - disappears at the 3rd to 4th month - strongest at the 2nd month 2. Startle reflex - disappears at the 4th month 3. Perez reflex - disappears by 4th to 6th month 4. Asymmetric tonic neck reflex - disappears at 3rd to 4th month - replaced by symmetric positioning of the 5. Trunk Incurvation reflex - disappears at the 4th week 6. Dance or Step reflex - disappears at the 3rd to 4th week - to be replaced by deliberate movement 7. Crawling reflex - disappears at 6th week 8. Placing reflex - age of disappearance varies Nursing Interventions in the Newborn Period body

 Maintain a patent airway Maintain a stable body temperature Protect from infection and injury - Identification - Eye Care Erythromycin Tetracycline Silver nitrate solution

- Vitamin K Administration - Hepatitis B Vaccine Administration - Newborn Screening for Disease (phenylketonuria, galactosemia, sickle cell - Universal Newborn Hearing Screening - Bathing - Care of the Umbilicus - Circumcision Provide Optimal Nutrition - Breast feeding versus Bottle (milkNewborn Nutrition Comparison of human milk and cows milk Recommendation: No cow milk before 1 year of age Commercial formulas modified from cow milk Other commercial formulas formula) feeding anemia)

Physiologic Benefits of Human Milk Species specific food for newborn humans Digestibility Immunologic properties cannot be duplicated in commercial formulas Availability/Infection control More Benefits of Breastfeeding Economical Always available Breast cancer incidence significantly lower in women who have breastfed May also offer protection to child from obesity, allergy, diabetes, atherosclerosis Breastfeeding Disadvantages Cultural perspectives on infant feeding Need for support, encouragement, and assistance Keys to Breastfeeding Success Correct sucking technique Correct positioning of infant at breast Absence of a rigid feeding schedule Promotion of Successful Breastfeeding Frequent and early breastfeeding (within first hr of life is important) Promotion of skin-to-skin contact Feeding on demand schedule Careful control of drugs (maternal and newborn) Significance of nurses in breastfeeding success Health Problems of Newborns Soft Tissue Injury

 Head Trauma 1. Caput succedaneum - vaguely outlined area of edematous tissue situated over the portion of the scalp that presents in a vertex delivery - extends beyond the bony margins - swelling accumulated in the tissues above - subsides in few days 2. Cephalhematoma - bleeding into the area between the bone - occurs most often with primiparous often associated with forceps delivery and vacuum extraction - the boundaries are sharply demarcated beyond the true limits of the bone - usually at the parietal area - swelling subsides in 2 weeks to 3 months 3. Subgaleal hemorrhage - associated with the use of vacuum Fractures - the clavicle is the most frequently during the birthing process - may have no symptoms - any newborn more than 8.5lbs, large for gestational age and is delivered vaginally should be evaluated for a fractured clavicle High Risk Infants Related to Dysmaturity 1. Preterm Infants - incidence is highest in the lower socioeconomic class fractured bone extractor at birth and its periosteum delivery and is the bone

and do not extend

- attributable to multiple pregnancy, other placental problems

pregnancy induced hypertension and

- is largely, but not entirely due to anatomic and physiologic immaturity of the various organs and systems at the time of birth 2. Postmature Infants - of unknown cause - may be described as absence of lanugo, caseosa, abundant scalp hair, and long fingernails. - may have a wasted physical appearance intrauterine deprivation - some indications of the presence of amniotic fluid - at risk of fetal distress because: *decreasing efficiency of the placenta *macrosomia, *meconium aspiration syndrome - greatest risk is during the process of especially in primigravidas High Risk Due To Physiologic Factors 1. Hyperbilirubinemia - benign in most cases - indirect hyperbilirubinemia is the most - possible causes of hyperbilirubinemia: *physiologic factors *excess production of bilirubin *decreased capacity of the liver to secrete conjugated bilirubin *combined overproduction and undersecretion (sepsis) common type parturition little vernix that reflects meconium in the

*hypothyroidism, galactosemia - 2 phases of physiologic jaundice a. 3rd day- 6mg/dl 5th day- 2-3mg/dl b. steady level till the 12th-14th day - entero-hepatic circulation or shunting - feeding; *stimulates peristalsis and produces more rapid of meconium, thus facilitating the passage of unconjugated bilirubin *introduces bacteria used to aid in reduction of bilirubin to urobilinogen. - breastfeeding-associated jaundice *begins at 2-4 days of age *occurs in approximately 10-15% of newborns *due to decreased caloric & fluid intake - breast milk jaundice *begins at 5-7days of age *occurs in 2-3% of breast fed infants *peak @ the 2nd week(3-12 wk) *due to the presence of pregnanediol, fatty acids and - unconjugated bilirubin *.2 to 1.4mg/dl (>5mg/dl-jaundice) - conditions that warrant the investigation of jaundice: beta glucuronidase the

a. appearance of jaundice within hours b. persistence of jaundice up to 1 weeks c. total serum bilirubin level of 12 13mg/dl d. increase of serum bilirubin level 5mg/dl/day e. direct bilirubin of 1.5 to 2mg/dl - complications: a. bilirubin encephalopathy - factors that would contribute to development of kernicterus other than indirect hyperbilirubinemia *metabolic acidosis *decreased serum albumin *intracranial infections *abrupt increases in blood pressure - management: *phototherapy- main form of treatment *exchange transfusion *phenobarbital - for infants with hemolytic disease - should be given several days prior to delivery 2. Hemolytic Disease of the Newborn a. Rh Incompatibility the

24 2

of

b. ABO Incompatibility - diagnostic evaluation: *indirect Coombs test *direct Coombs test -therapeutic management: *prevention of Rh isoimmunization *intrauterine transfusion *exchange transfusion -nursing consideration: *watch out for jaundice *watch out for hypothermia (increases the O2 and CHO requirement, acidosis, decreased and bilirubin) *watch out for hyperthermia 3. Metabolic Complications a. Hypoglycemia -types: *Increased or impaired glucose utilization *Decreased glucose stores -clinical manifestations: *nonspecific s/sx *cerebral signs- jitteriness, tremors, twitching, high pitched cry, lethargy, limpness, apathy, convulsions and coma -screening: *serum glucose determination of all infants at risk binding of albumin

-treatment: *early feeding in normoglycemic patients *IV glucose administration if feeding not tolerated b. Hypocalcemia -types: *Early Onset- 1st 48 hours *Late Onset- apparent after 3-4 -clinical manifestations: pitched cry, *jitteriness, apnea, cyanosis, abdominal distention (early *twitching, tremors, seizures -laboratory diagnosis: *determination of serum calcium -treatment: administration of *early onset- increase formula calcium supplements *late onset- calcium gluconate, -correct hypoparathyroidism 4. Respiratory Distress Syndrome - also known as Hyaline Membrane - almost exclusively in preterm infants Pulmonary Interstitial Emphysema- develops in preterm infants with RDS - s/sx *tachypnea, dyspnea *retractions Disease feedings, vitamin D level edema, high onset) (late onset) days

*fine inspiratory crackles *audible expiratory grunt *flaring of the external nares *cyanosis or pallor -manifestations of disease progression *apnea *flaccidity *absent spontaneous movement *unresponsiveness *diminished breath sounds mottling -manifestations associated with a severe *shock-like state *decreased cardiac output *bradycardia and low BP -diagnosis: *based on the s/sx *radiography *blood gas analysis -management: *maintain adequate ventilation and perfusion *maintain acid-base imbalance *maintain a neutral thermal environment *maintain adequate tissue perfusion oxygenation and disease

*prevent hypotension *maintain adequate hydration and electrolyte status *maintain proper nutrition *exogenous surfactant administration -pulmonary hemorrhage and mucus plugging -nursing responsibilities: 1. arterial blood gas determination 2. monitoring of oxygenation 3. assessment of the infants tolerance to the procedure 4. adjustment of the ventilator setting 5. delay suctioning -prevention: *avoidance of premature delivery *combination of maternal steroid administration and postnatal administration of surfactant -prognosis: *this is a self limiting disease (72 hours) 6. Respiratory Complications a. Meconium Aspiration Syndrome -treatment: *suction hypopharynx at birth *intubate and suction trachea (drawbacks)

*may use exogenous surfactant b. Apnea of Prematurity - treatment: *check thermal stability and apnea *administer theophylline and caffeine *administer nasal continuous positive airway pressure -nursing management: *observe for signs of theophylline or caffeine toxicity c. Pneumothorax -treatment: *needle aspiration *chest tube insertion -nursing management: *close vigilance *provide appropriate care of chest tube drainage *emergency setup at bedside 7. Chronic Lung Disease -also called as bronchopulmonary dysplasia -pathologic process related to: *alveolar damage from lung disease *prolonged peak inspiratory pressures and oxygen *immature alveoli and respiratory -prevention: tract

*administer maternal steroids, exogenous surfactant postnatally *provide early detection of pulmonary function tests *prevent or control respiratory or systemic infections -nursing management: *support respiratory efforts *maintain adequate oxygenation and hypoxemia *administer steroids, diuretics and bronchodilators *provide supplemental oxygen 8. Cardiovascular Complications a. Patent Ductus Arteriosus -treatment: *regulate parenteral fluids *provide respiratory support *administer Indomethacin or perform surgical ductal ligation b. Persistent Pulmonary Hypertension -treatment: *regulate IV fluids *provide supplemental oxygen and assisted ventilation *administer vasodilators *maintain acid-base balance *prevent hypoxemia and hypercarbia avoid

*administer nitric oxide c. Anemia -treatment: *administer volume expanders *transfuse with packed RBC *administer with recombinant erythropoietin d. Polycythemia/Hyperviscosity Syndrome -risks: *cardiovascular symptoms *seizures *hyperbilirubinemia *gastrointestinal abnormalities -treatment: *implement partial exchange transfusion with blood products or *therapy for associated problems e. Vitamin K Deficiency -formerly known as the hemorrhagic the newborn -treatment: *administer vitamin K 9. Cerebral Complications a. Retinopathy of Prematurity -management: *prevent preterm birth *early detection in <28 weeks AOG <1500g weight and disease of volume expanders

*decrease exposure to direct bright *use supplemental oxygen judiciously *arrest vascular proliferation (cryotherapy, laser photocoagulation, surgical repair b. Hypoxic-Ischemic Brain Injury -treatment: *prevent hypoxia *provide supportive care *provide adequate ventilation *maintain cerebral perfusion *prevent cerebral edema *treat the underlying cause *administer anti-seizure drugs c. Germinal Matrix/Intraventricular Hemorrhage -management: *provide ventilatory support *maintain oxygenation *regulate fluids and electrolytes acid base balance *suppress and prevent seizures *ventricular shunting or drainage d. Intracranial Hemorrhage -types: *subdural *subarachnoid

light

of the detached retina)

and

*intracerebellar 10. Neonatal Seizures -most commonly caused by Hypoxic-Ischemic Encephalopathy secondary to perinatal asphyxia -other causes: *metabolic *toxic

*prenatal infections *postnatal infections *trauma at birth *malformations *narcotic withdrawal, degenerative disease, benign familial seizures -types: 1. Clonic- slow rhythmic jerking -approximately 1-3/second 2. Tonic- extension/stiffening movements 3. Subtle- more common in preterm infant - often overlooked -signs: *horizontal eye deviation *repetitive blinking or fluttering of the eyelids, staring *sucking or other oral-buccallingual movements *arm movements that resemble rowing or swimming movements

*leg movements described as pedaling or bicycling *apnea (common) -signs may appear alone or in combination 4. Myoclonic- rapid jerks that involve the muscle groups Seizure vs. Jitteriness Jitteriness or tremulousness in the newborn is a repetitive shaking of an extremity or extremities that may be observed with crying, occurs with sleeping state or elicited with stimulation. Seizures vs. tremors Tremor is the repetitive movements of both hands (with or without movements of the jaws or legs) at a frequency of 25/second and lasting more than 10 minutes. -diagnosis: *physical examination *comprehensive health history *blood chemistry *CSF examination *EEG *CT scan, MRI -management: *prevention of cerebral damage *correction of metabolic derangement *respiratory support *cardiovascular support *suppression of seizure activity flexor

High Risk Related to Infectious Processes 1. Sepsis - also called as septicemia -refers to generalized bacterial infection of the -causes: *transplacental transfer *during labor (ingestion or aspiration) -types: *early sepsis- less than 3 days -acquired as a perinatal infection -most commonly caused by GBS and E. coli -other organisms include H. influenzae and coagulase aureus (VLBW) *late onset sepsis -1-3 weeks after birth -is primarily nosocomial -diagnosis: *laboratory and radiographic exams (anemia, leucocytosis, leucopenia) negative Staphylococcus blood stream

*laboratory -anemia, leucopenia, leucocytosis, metabolic acidosis electrolyte imbalance -thrombocytopenia and coagulopathy (severe cases) -positive blood culture and

-management: *prevention of enteral (milk formula feeding) for 1-2 days *abdominal decompression *IV antibiotics 2. Necrotizing Enterocolitis -is an acute inflammatory disease of the an increased incidence in preterm infants. -3 factors in its causation: *intestinal ischemia *invasion by pathogenic bacteria *formula feeding in the intestinal lumen -with pneumatosis intestinalis -diagnosis: *radiographic -prevention: *good hand-washing techniques *isolation precautions *adoption of appropriate spacing of hospital infants beds -management: *antibiotic therapy -empirical -C/S guided *immunotherapy *circulatory and ventilatory support *IV fluids bowel with

*correction of hypoxia High Risk Due To Maternal Conditions 1. Infants of Diabetic Mothers -clinical manifestations of infants born to mother *LGA *very plumped and full faced *abundant vernix caseosa *listlessness and lethargy *large placenta and umbilical cord *possibly meconium stained at birth -RDS in a neonate from a diabetic -high susceptibility to hypoglycemia, hypocalcemia, hypomagnesemia, cardiomyopathy -management: *early examination for congenital anomalies, respiratory or *adequate thermoregulation *early introduction of carbohydrate feedings *monitoring of blood glucose level *monitor for birth related injuries 2. Drug Exposed Infants -withdrawal reaction (involves the CNS, and the Respiratory Systems) *heroin- after 12-24 hours *methadone- after 2-3days -drug therapy: GIT, ANS cardiovascular problems mother hyperbilirubinemia a diabetic

and

*phenobarbital *clonidine a. Cocaine

*morphine, methadone *diazepam

- smaller head circumference - decreased birth weight - decreased birth length 3. Maternal Infections a. HIV -depressed nasal bridge -mild upward or downward obliquity of the -long palpebral fissures with blue sclera -ocular hypertelorism -prominent upper vermillion border b. VZV -limb dysplasia -microcephaly -cortical atrophy -chorioretinitis -cataracts - auditory nerve palsy -mental retardation c. Chlamydia -conjunctivitis and pneumonia c. Neisseria gonorrhea -ophthalmitis -neonatal gonococcal arthritis, septicemia, arthritis eyes

d. Cytomegalovirus -microcephaly -cerebral calcifications -chorioretinitis -seizure disorder -deafness -mental retardation e. Rubella -cataract -microphthalmia -retinitis -glaucoma -microcephaly -seizures -severe mental retardation -patent ductus arteriosus -hearing loss -intrauterine growth retardation f. Toxoplasmosis -hydrocephaly, cerebral calcifications, chorioretinitis (classic triad) -microcephaly, seizures, mental retardation, -encephalitis, myocarditis, hepatosplenomegaly, anemia, jaundice, purpura 4. Chromosomal Abnormalities a. Cri Du Chat Syndrome deafness diarrhea, vomiting,

-distinctive weak, high pitched, mew-like cry resembling the cry of the cat, small head, hypertelorism, failure to thrive, severe mental retardation. b. Trisomy 13 (Patau Syndrome) -multiple anomalies, including cleft lip and cleft palate, ear malformations, microphthalmia, polydactyly, eye defects, mental retardation, and early death

c. Trisomy 18 (Edwards Syndrome) -deformed and low set ears, micrognathia, overlapping fingers, prominent occiput, hypertelorism, failure to thrive and early death, mental retardation d. Trisomy 21 (Down Syndrome) -brachycephaly with flat occiput, epicanthal ears, nose, and protruding tongue folds, small

-broad, short hands with stubby fingers and transverse palmar crease, cardiac defects, mental retardation, variable life expectancy Fragile X Syndrome Second most common genetic cause of mental retardation after Down syndrome X-linked Dominance with Reduced Penetrance Differs from X-linked recessive pattern Abnormal gene on the lower end of the long arm of the X chromosome More common in males; sometimes females Fragile X Syndrome: Gender Differences Most of males are mentally deficient 30% of females are mentally deficient Males have only the nonfunctioning X Females have one normally functioning X and one nonfunctioning X

Classic Physical Appearance Large HC/long face/prognathism/large ears/long palpebral fissures/strabismus Mitral valve prolapse Macro-orchidism Classic Physical Appearance (contd) Palatehigh arched Hyperextensible finger joints/palmar crease Flat feet Classic Behavioral Features Mild to severe mental retardation/Normal IQ with LD Delayed speech and language Hyperactivity Autistic-like behaviors Aggressive behaviors Therapeutic Management of Fragile X Syndrome Tegretol/Prozacbehavioral control Stimulants for hyperactivity (similar to ADHD management) Mimic behaviormainstream management Inborn Errors of Metabolism 1. Congenital hypothyroidism 2. Phenylketonuria 3. Galactosemia Congenital Hypothyroidism Can be transient or permanent Risk factors: -iodine deficiency

-genetics -Downs syndrome -Prematurity Thyroid Function Test -T4(<6ug/dl) -TSH(>60uU/ml) Manifestations: >excessive sleeping >reduced interest in nursing >poor muscle tone >low or hoarse cry >infrequent bowel movements >exaggerated jaundice >low body temperature >large anterior fontanel >persistence of a posterior fontanel athyreosis >umbilical hernia & macroglossia Treatment: -lifelong thyroid hormone replacement -DOC is synthetic thyroid hormone Levothyroxine Sodium (Synthroid and Prognosis -early treatment: normal physical growth and intelligence Levothroid)

Phenylketonuria Inherited as autosomal recessive trait

 Due to the absence of phenylalanine hydroxylase Results to hyperphenylalaninemia and urinary excretion of its metabolites (phenyllactic acid, phenylacetic acid and phenylpyruvic With a characteristic musty odor of the urine Lack of tyrosine > lack of melanin and catecholamines Manifestations -blond hair -blue eyes -fair skin particularly susceptible to -failure to thrive -frequent vomiting -irritability, hyperactivity, unpredictable -erratic behavior -mental retardation > due to decreased level of neurotransmitters Diagnostic Evaluation -GUTHRIE BLOOD TEST > the most commonly used test for newborns > only the heel blood can be used screening (not the cord blood) > can detect serum phenylalanine beyond 4mg/dl (n.v. is 1.6mg/dl) -the objective of early screening is to mental retardation -other methods: Quantitative Fluorometric Tandem Mass Spectrophotometry Management for levels prevent Assay and screening eczema

acid)

-restriction of phenylalanine from the diet -2 criteria in the dietary management 1. meet the childs optimum need for growth 2. maintain phenylalanine levels within the safe range (2-6mg/dl from birth to 12 years)(2-10mg/dl through adolescence) and (2-15mg/dl in adults) -foods low in phenylalanine (vegetables, some cereals, breads, and starches) fruits, juices,

-foods to avoid> high protein foods and the sweetener aspartame (contain two amino acids: aspartic acid and phenylalanine) Galactosemia A rare autosomal recessive disorder A deficiency of the enzyme galactose-1-phosphate uridyltransferase Manifestations: -hepatic dysfunction > cirrhosis (jaundice by the second week of life) -portal hypertension > splenomegaly -cataracts at 1 2 months of age -cerebral damage > lethargy and -vomiting and weight loss -E. coli sepsis Diagnosis -history -physical assessment -galactosuria and galactosemia hypotonia

-decreased galactose-1-phosphate uridyltransferase in RBC -malnutrition -dehydration, decreased muscle mass Therapeutic Management -eliminating all milk from the diet Prognosis -ovarian dysfunction, cataracts, abnormal speech, cognitive impairment, growth retardation and motor delay Vitamins and Their Nutritional Significance Water Soluble Vitamins 1. Vitamin B1 -also called thiamin -coenzyme in carbohydrate metabolism -deficiency can result to beriberi -excess can result to headache, irritability, weakness 2. Vitamin B2 -also called riboflavin -coenzyme in carbohydrate, fat and protein metabolism the eyes, insomnia, rapid pulse and

-maintains healthy skin especially around nose and mouth -deficiency can result to ariboflavinosis -excess can result to paresthesia and pruritus. 3. Vitamin B3 -also called Niacin or Nicotinamide -coenzyme in protein and fat metabolism

-needed for a healthy nervous system, skin normal digestion -may lower blood cholesterol level -deficiency can lead to pellagra -excess can lead to release of histamine, increased gastric acidity, hepatotoxicity, increased serum uric acid level, elevated certain cardiac arrhythmias 4. Vitamin B6 -also called Pyridoxine -coenzyme in protein and fat metabolism -needed for the formation of antibodies and -needed for the utilization of copper and iron -aids in the conversion of tryptophan to niacin - deficiency can lead to dermatitis, weight loss anemia, retarded growth, irritability, and peripheral neuritis -excess can lead to peptic ulcer disease 5. Folic Acid -also called Folacin -coenzyme in single carbon transfer thymine, hemoglobin) -necessary in the formation of red cells -may prevent neural tube defects

and for a

plasma glucose level, and

hemoglobin

convulsions, and seizures

(purines,

-deficiency can lead to macrocytic anemia, bone marrow depression, glossitis, intestinal malabsorption -excess may cause insomnia and 6. Vitamin B12 irritability

-also called as Cyanocobalamin -coenzyme in protein synthesis, indirect formation of red cells (particularly on acids and folic acid metabolism) -needed for the normal functioning of the system -deficiency may lead to pernicious anemia, yellow tinge on the skin, spinal cord degeneration and delayed 7. Biotin -coenzyme in carbohydrate, fat and protein -interrelated with functions of other B -deficiency is uncommon since this is by the bacterial flora 8. Pantothenic Acid -coenzyme in carbohydrate, fat and protein -synthesis of amino acids, fatty acids and -deficiency is rare since this is synthesized by flora 9. Vitamin C -also called Ascorbic Acid -essential for collagen formation -increases absorption of iron for hemoglobin -enhances the conversion of folic acid to -play a role in the hydroxylation of adrenal -may have stimulating effect on the activity of leucocytes and antibody formation -antioxidant agent formation folinic acid steroids phagocytic metabolism steroids the bacterial metabolism vitamins synthesized lemonbrain growth effect on the formation of nucleic nervous

-deficiency may lead to scurvy, signs of anemia, decreased wound healing, increased susceptibility to infection -excess may lead to diarrhea, excretion of uric acid and acidification of the urine, hemolysis, impaired leucocyte activity, damage to beta cells of the pancreas, reproductive failure and rebound scurvy Water Insoluble Vitamins 1. Vitamin A -Retinol, Retinoic Acid and Retinaldehyde -necessary in the formation of pigment -formation and maintenance of the epithelial -for normal bone growth and development -needed for normal growth and spermatogenesis -involved in thyroxine formation -antioxidant -deficiency can lead to night blindness, keratinization of the epithelium, xeropthalmia, defective tooth enamel, retarded growth, impaired bone formation, decreased thyroxine, and decreased resistance to infection -excess can lead to birth defects, fissures at the corners of the mouth and lips, hepatomegaly, retarded growth, thickening of the cortex of long bones 2. Vitamin D -also called ergocalciferol or cholecalciferol -for the absorption of calcium and phosphorus and decreased renal excretion of phosphorus -deficiency may lead to rickets: craniotabes, rachitic rosary, pigeon chest, rachitic tetany, vertebral abnormalities -excess may lead to calcification of tissues, tissue rhodopsin

of calcium and 3. Vitamin E

osteoporosis of long bones, elevated phosphorus

serum levels

-also called tocopherol -production of red blood cells and protection hemolysis -for muscle and liver integrity -coenzyme factor in tissue respiration -minimizes oxidation of PUFA and vitamins tract and tissues -deficiency can cause hemolytic anemia 4. Vitamin K -catalyst for the production of clotting -deficiency can lead to hemorrhage -excess can lead to hemolytic anemia in 6-phosphate dehydrogenase activity Minerals And Their Nutritional Significance 1. Calcium -bone and tooth development and maintenance phosphorus -muscle contractions -blood clotting -absorption of Vit.B12 -enzyme activation -nerve conduction 2. Chromium -involved in glucose metabolism and energy production 3. Copper in the combination of patients lacking glucosefactors A&C in intestinal from

-production of hemoglobin -essential element of several enzyme system 4. Fluoride -formation of caries resistant teeth -strong bone development 5. Iodine -production of normal thyroid hormones -normal reproduction 6. Iron -formation of hemoglobin and myoglobin -essential part of several enzymes and proteins 7. Selenium -antioxidant, especially protective of vit. E -protects against the toxicities of heavy metals -associated with fat metabolism 8. Zinc -components of about 100 enzymes -synthesis of nucleic acids and protein in immune -release of vitamin A from the liver -improved wound healing with Vit. C -normal taste sensitivity Protein and Energy Malnutrition 1. Kwashiorkor 2. Marasmus Food Sensitivity 1. Food Allergy or Hypersensitivity system and coagulation

2. Food Intolerance Kwashiorkor Primarily a deficiency of protein with an Typical characteristics: -thin wasted extremities and a edema -the skin is scaly and dry and has areas -dermatosis due to vitamin deficiency -permanent blindness due to vit. A def -iron, calcium and zinc deficiencies -thin, dry, coarse and dry hair -depigmentation and patchy alopecia -diarrhea Maramus Due to general malnutrition of proteins and calories of depigmentation prominent abdomen from adequate supply of calories

Is usually a syndrome of physical and emotional deprivation Typical characteristics: -gradual wasting and atrophy of body -appears old, with flabby and wrinkled -fat metabolism is less impaired > fat vitamin deficiency is less likely -apathetic, withdrawn and lethargic -intercurrent infections Management 1. dietary management -vit. A, vit. E, selenium and zinc 2. protection from infection skin soluble tissues

3. adequate hydration 4. skin care 5. restoration of physiologic integrity Lactose Intolerance Congenital Lactase Deficiency -manifestations occur soon after birth after lactose containing milk -complete absence or severely reduced presence of lactase -requires lifelong lactose free or reduced Late Onset Lactase Deficiency -referred to as primary lactase deficiency -is the most common type of lactose -manifested usually around 3 7 years of although the time of onset is variable Secondary Lactose Intolerance -occur secondary to the damage of the intestinal lumen which decreases or lactase. Manifestations -abdominal pain, bloating, flatulence and -onset is 30minutes to an hour after the of lactose Diagnosis Hydrogen Breath Test -will yield a higher percentage of (20parts/million or more above baseline) Management 1. Elimination of lactose containing foods (dairy products) hydrogen diarrhea consumption destroys the enzyme intolerance age lactose diet consuming

2. Supplementation of calcium and vitamin D 3. Yogurt fresh vs. frozen 4. Lactase treated dairy products 5. Lactase tablets Heavy Metal Poisoning Can occur from the ingestion of a variety of substances Most common is Lead Poisoning Types 1. Lead poisoning -sources: paint and gasoline (through inhalation, ingestion and transplacental) city dwellers

-risk factors: poverty, less than 6 years of age, -clinical manifestations: renal, hematologic and

neurologic system -young children are more vulnerable than children and adults the older

-lead (blood) > soft tissues and organs > intracellular compartment > competes with molecules of calcium -lead (blood) > blood brain barrier > brain edema and encephalopathy -lead (blood) > interfere with iron binding anemia Diagnostic Evaluation -lead testing of venous blood (level of concern and up) -determination of Erythrocyte level Protoporphyrin is 10ug/dl > cerebral into heme >

*sensitive to blood lead level 35ug/dl Management -treatment actions depend on the lead level -use of chelating agents a. calcium disodium edetate b.succimer Sudden Infant Death Syndrome -unknown cause of death of babies less than

above

childs blood

1 year of age abnormality

-theorized to be related to a brainstem in the neurologic regulation of the cardio-respiratory control -signs: *prolonged sleep apnea *increased frequency of brief pauses *excessive periodic breathing *impaired arousal responsiveness to carbon dioxide and decreased O2 -risks: *maternal smoking *poor prenatal care *bed sharing *sleeping in the prone position *sleeping in the side lying position Apnea of Infancy - refers to pathologic apnea in infants of more weeks of gestation

inspiratory

increased

than 37

-can be a sign of: *sepsis *seizures *upper airway abnormalities *gastro-esophageal reflux *hypoglycemia or other metabolic disorder Eating Disorders Obesity -currently defined as an excessive amount of in relation to lean body mass Overweight -refers to the state of increased body weight in Common emotional consequences of obesity: 1. Poor body image 2. Low self esteem 3. Social isolation 4. Feelings of depression and rejection Etiology and pathogenesis: 1. Hypothyroidism 2. Adrenal hypercorticoidism 3. Hyperinsulinism 4. Dysfunction of the CNS 5. Heredity Management: 1. Diet 2. Exercise relation to height body fat or adipose tissue

3. Behavioral therapy 4. Group involvement 5. Medical therapies 6. Prevention Anorexia Nervosa -an eating disorder characterized by a refusal to maintain a minimally normal body weight and by severe weight loss in the absence of obvious physical causes. Management: 1. Reinstitution of normal nutrition or reversal -tube feedings or IV fluids (severe) -2000 to 3000 kcal/day -weight gain of .5 to 2 lbs a week 2. Resolution of disturbed patterns of family interaction 3. Individual psychotherapy Prognosis: -25% attain full recovery, 50% improve but may despite adequate treatment Bulimia Nervosa -is an eating disorder characterized by eating Congenital Heart Disease Common in boys -Transposition of Great Vessels -Truncus Arteriosus -Tetralogy of Fallot Common in girls -Patent Ductus Arteriosus frequent binge relapse, 25% do poorly of malnutrition

-Atrial Septal Defect Causes -Familial -Exposure to rubella Two Major Types: 1. Cyanotic Heart Disease 2. Acyanotic Heart Disease A. Acyanotic Heart Disease with Increased Pulmonary Blood Flow 1. Ventricular Septal Defect -systolic murmur at the left lower border of the significant sign -increased oxygen saturation at the right side sternum and no other of the heart -Failure of heart structure to progress

-ECG reveals hypertrophy of the right side of the heart -only 50% of the oxygenated blood will go to the -mgt: surgery- complete repair is the procedure of (Dacron patch or a purse string) long term antibiotic therapy 2. Atrial Septal Defect -systolic murmur at the upper border of the sternum with no significant sign the heart heart atrium choice aorta

-increase oxygen saturation at the right side of -ECG reveals hypertrophy of the right side of the -50% of the oxygenated blood goes into the right -mgt: surgery- use of Dacron patch long term antibiotic therapy 3. Endocardial Cushion Defect

-AV canal affecting both the tricuspid and the -confirmed by cardiac catheterization -valves are closed -mgt: surgery 4. Patent Ductus Arteriosus -failure of the ductus arteriosus to close -continuous machinery-like murmur -ECG reveals hypertrophy of the left ventricle -mgt: Indomethacin Surgical ligation by 2 4 years old

mitral valve

B. Acyanotic Heart Disease with Decreased Pulmonary Blood Flow 1. Pulmonic Stenosis -narrowing of the valve of pulmonary artery -typical systolic ejection murmur -S2 sound is widely split -ECG reveals right ventricular hypertrophy -mgt: Balloon Stenotomy Surgery 2. Aortic Stenosis -narrowing of the valve of the aorta -ECG reveals left ventricular hypertrophy -systolic murmur -anginal pain

-mgt: Balloon Stenotomy Surgery 3. Duplication of the Aortic Arch -doubling of the arch of aorta causing compression to the trachea and esophagus -dysphagia and dyspnea -left ventricular hypertrophy -mgt: surgery 4. Coarctation of the Aorta -narrowing of the arch of aorta -outstanding signs: *absent femoral pulse *Higher BP of the upper extremities -lower BP in the lower extremities -Mgt: BP monitoring of four extremities Surgery Cyanotic Heart Disease with Increased Pulmonary Blood Flow 1. Transposition of Great Arteries -aorta is arising from the right ventricle while arising from the left -cyanosis after the first cry -polycythemia, a compensatory mechanism to supply to the body -consequences of polycythemia: the decreased oxygen the pulmonary artery is

*thrombus *embolus *cerebro-vascular accident -ECG reveals cardiomegaly -mgt: surgery- Senning, Mustard or Rastelli 2. Total Anomalous Pulmonary Venous Connection -pulmonary veins enter the right atrium or the left atrium -open foramen ovale -mild to moderate cyanosis -absent spleen -mgt: surgery 3. Truncus Arteriosus -pulmonary and aorta arise in one common -cyanosis is present -with polycythemia *can lead to thrombus, embolus and -mgt: surgery 4. Hypoplastic Left Heart Syndrome -nonfunctioning left ventricle -with cyanosis -with polycythemia and its consequences -mgt heart transplantation CVA trunk with ventricular defect superior vena cava instead of Procedure

 Cyanotic Heart Disease with Decreased Pulmonary Blood Flow 1. Tricuspid Atresia -failure of the tricuspid valve to open -with open foramen ovale -with cyanosis -with polycythemia and its consequences -mgt *Pulmonary to systemic artery *Pulmonary artery banding *Bidirectional Glenn Shunt anastomosis) -may be performed at 6 to 9 months *Modified Fontan Procedure 2. Tetralogy of Fallot -anomalies present: *pulmonic stenosis *ventricular septal defect *overriding of the aorta *right ventricular hypertrophy -with high degree of cyanosis -with polycythemia and its consequences -with severe dyspnea >relieved by squatting (in older children) >relieved by knee chest position (in -with growth retardation -with Tet spells or blue spells -with syncope and mental retardation infants) (cavopulmonary anastomosis

-with clubbing of the fingernails -radiography shows a boot-shaped heart -mgt *oxygen therapy *morphine sulfate *propanolol *palliative repair >Blalock Taussig or Modified Taussig Procedure *complete repair >Brock Procedure Acquired Heart disease 1. Rheumatic Heart Disease -inflammatory disease following an infection caused by group A beta hemolytic streptococcus -initial inflammatory process could be tonsilitis, pharyngitis or both Blalock

-group A beta hemolytic streptococcus is an aerobic microorganism > will stay at the left side of the heart or mitral valve as an Aschoff Bodies -Aschoff bodies * rounded nodules with multinucleated cell fibroblast that stay in the mitral valve -with left sided heart failure because of increase in size of the Aschoff bodies -Jones Criteria for the diagnosis -mgt: *bed rest *avoid contact sports *throat swab for culture and sensitivity and

mitral stenosis due to the

*antibiotic therapy *aspirin therapy or salicylates Jones Criteria Major Criteria 1. Polyarthritis 2. Chorea /Syndenhamms chorea/St. Vitous Dance 3. Carditis 4. Erythema marginatum 5. Subcutaneous nodules Minor Criteria 1. Low grade fever 2. Diagnostic Evaluation *antibody *C reactive protein *ESR *antistreptolysin O titer Kawasaki Disease -acute systemic vasculitis; mucocutaneous lymph -common in <5y/o -not spread by person to person contact -s/sx: >fever for 5 days or more >bilateral conjunctival injection without >changes oral mucous membrane *erythema *dryness *oropharyngeal reddening exudate node syndrome

*fissuring

*strawberry tongue

>changes in the extremities *peripheral edema *erythema of the palms and soles *periungual desquamation >polymorphous rash on the trunk >cervical lymphadenopathy (1 lymph node >1.5cm) -with 3 phases: 1. acute phase -abrupt onset of high fever unresponsive symptomatic treatment -very irritable 2. subacute phase -resolution of fever -irritability persists 3. convalescent phase -all signs resolved but lab values not returned to normal -6 to 8 weeks from the time of onset -management: >high dose IV gamma globulin with salicylate therapy (first 10 days of illness) >anticoagulation with coumadin for patients aneurisms >supportive and symptomatic nursing care Blood Disorders Anemia with giant to

 Polycythemia Bleeding Disorders 1. Hemophilia -sex linked recessive disorders *females are carriers *males manifest the disease -clotting factors deficient: * clotting factors 8, 9 or 11 -omphalagia *is the earliest sign / >30cc of blood -the maternal clotting factors are present in the is delayed diagnosis of hemophilia - sudden bruising and hemarthrosis -diagnostic exam: *partial thromboplastin time -goal: *prevention of injury -health teaching: *avoid contact sports *determine the case before doing any procedure *in case of fracture/injury Hemolytic Anemias Sickle Cell Anemia -a result of inheritance of sickle hemoglobin gene -sickled Hgb *crystal formation when exposed to low oxygen tension invasive newborn thats why there

deformed, rigid and sickled RBC > ischemia&infarction -sickling process is intermittent (with exposure to cold environment) -assessment and diagnostic findings >sickle cell trait: *normal RBC *normal WBC *normal platelets >sickle cell anemia *decreased Hct *sickled cells on smear -confirmatory test: Hemoglobin electrophoresis -clinical manifestations: *Hgb 7-10mg/dl *jaundice *enlargement of the bones of the face and the *tachycardia, cardiac murmurs and enlarged *dysrhythmias and heart failure *susceptible to infection primarily pneumonia osteomyelitis *chronic hemolysis and thrombosis >> ischemia and necrosis of organs with (spleen, lungs and CNS) -complications: 1. cerebral infarction 2. infection and heart skull low oxygen tension and

slowed circulation

3. renal failure 4. impotence 5. heart failure 6. pulmonary hypertension Sickle Cell Crisis -3 types: 1. Very painful sickle cell crisis > due to tissue hypoxia and necrosis 2. Aplastic crisis > due to infection of human parvovirus > Hgb decreases rapidly that the BM cannot 3. Sequestration Crisis >pooling of sickled cells in an organ *spleen >> splenic infarction >> autosplenectomy Acute Chest Syndrome -decreased hemoglobin and hematocrit (rapid) -fever -tachycardia -bilateral chest film infiltrate -causes: *infection *fat embolism -treatment: *antibiotics det *fluid restriction -diagnostic tests: *CXR *incentive spirometry *bronchoscopy *phospholipase A2 compensate

*corticosteroid *transfusion >decreases phospholipase A2 -prognosis: >usually diagnosed during childhood -medical management: >3 treatment modalities: a. BM transplantation b. Hydroxyurea *increases the concentration of fetal Hgb *decreases vaso-occlusive crisis c. Long term RBC transfusion Adverse Effects of Hydroxyurea 1. chronic suppression of WBC formation 2. teratogenesis 3. potential for later development of malignancy Complications of Transfusion 1. Iron Overload -needs chelation therapy 2. poor venous access 3. alloimmunization due to repeated transfusion *exchange transfusion Treatment: 1. Supportive >pain management *folic acid supplementation

2. Prompt use of antibiotics -nursing diagnosis: 1. Pain related to tissue hypoxia 2. Risk for infection 3. Powerlessness related to illness induced powerlessness

4. Knowledge deficit regarding prevention of crisis -goals: 1. Relief of pain 2. Decreased incidence of crisis 3. Enhanced self esteem and power 4. Absence of complications -nursing interventions: 1. Management of pain 2. preventing and managing infections 3. promoting coping skills 4. Minimizing knowledge deficit 5. Monitoring and managing potential complications -leg ulcers -priapism leading to impotence -chronic pain and substance abuse Thalassemias -a hereditary disorder associated with defective synthesis -characterized by microcytosis and hypochromia -imbalance in the configuration of hemoglobin >> increased rigidity of RBC >> hemolysis -2 types: hemoglobin chain

(according to the globin chain diminished) a. alpha thalassemia -milder and often without symptoms b. beta thalassemia -more severe and lethal Alpha Thalassemia Clinical Syndromes: 1. Silent Carrier -asymptomatic -deletion of a single alpha globin gene -barely detectable reduction in alpha globin synthesis 2. Alpha Thalassemia Trait -deletion of 2 globin gene -minimal or no anemia and no abnormal signs 3. Hemoglobin H Disease -deletion of the 3 out of 4 alpha globin gene -hemoglobin H has extremely high O2 affinity therefore not useful for O2 exchange -with moderately severe anemia 4. Hydrops fetalis -most severe form -deletion of the 4 alpha globin gene @ 16 -in the fetus > gamma globin chains in excess form tetramers (hemoglobin Bart) > extremely increased oxygen affinity > unable to deliver oxygen to the tissues > severe anoxia chromosome and physical gene

Beta Thalassemia Clinical Syndromes: 1. Thalassemia Major -B+/B+ or B0/B0 -lead to severe transfusion dependent anemia -severe anemia and first become manifested 6birth (hemoglobin synthesis switches from HgbF to HgbA) 9mos after

-if untransfused, Hgb may range between 36gm/dl (with marked anisocytosis and microcytic, hypochromic anemia) -increased reticulocytes but erythropoiesis is -clinical course: short or brief because of death at an 2. Thalassemia Minor -resistant against falcifarum malaria -asymptomatic and anemia is mild if present G6PD Deficiency (G6PD is essential for membrane stability) -would result to chronic hemolytic anemia -hemolysis only when under stress, infection or use of certain medications (oxidant drugs) -inherited as X-linked disease -oxidant drugs: >anti-malarial agents >sulfonamides >nitrofurantoin >analgesics (ASA, Phenacetin) >thiazides due to the early age ineffective

>chloramphenicol >para-amino-salisylic acid >vitamin K -clinical manifestations: >s/sx of hemolysis *reticulocytosis *hemoglobinuria *jaundice *pallor spleen)

-presence of Heinz bodies (taken up by the -assessment and diagnostic findings: >qualitative assay for G6PD -medical management: a. withdrawal of the offending agent b. transfusion -only in severe hemolytic states c. health education -avoid triggering factors Hereditary Spherocytosis -Is also a type of hemolytic anemia

-Abnormal permeability of RBC membrane > spherocytosis > taken up by the liver > hemolysis Croup Syndromes Characterized by hoarseness, barking cough, inspiratory stridor, and varying degrees of respiratory distress Croup syndromes affect larynx, trachea, and bronchi Epiglottitis, laryngitis, LTB, tracheitis Acute Epiglottitis Clinical manifestations

Sore throat, pain, tripod positioning, retractions Inspiratory stridor, mild hypoxia, distress Therapeutic management Potential for respiratory obstruction Nursing considerations Prevention: Hib vaccine Acute Laryngitis More common in older children and adolescents Usually caused by virus Chief complaint is hoarseness Generally self-limiting and without LT sequelae Treatment: symptomatic Acute LTB LTB = Laryngotracheobronchitis Most common of the croup syndromes Generally affects children <5 yrs Organisms responsible RSV, parainfluenza virus, Mycoplasma pneumoniae, influenza A and B Manifestations of LTB Inspiratory stridor Suprasternal retractions Barking or seal-like cough Increasing respiratory distress and hypoxia Can progress to respiratory acidosis, respiratory failure and death Therapeutic Management Airway management

Maintain hydrationPO or IV High humidity with cool mist Nebulizer treatments Epinephrine Steroids Acute Spasmodic Laryngitis AKA spasmodic croup, midnight croup Paroxysmal attacks of laryngeal obstruction Occur chiefly at night Inflammation: mild or absent Most often affects children ages 1-3 Therapeutic management Bacterial Tracheitis Infection of the mucosa of the upper trachea Distinct entity with features of croup and epiglottitis Clinical manifestations similar to LTB May be complication of LTB Thick, purulent secretions result in respiratory distress Therapeutic Management Humidified oxygen Antipyretics Antibiotics May require intubation Infections of the Lower Airways Considered the reactive portion of the lower respiratory tract Includes bronchi and bronchioles

Cartilaginous support not fully developed until adolescence Constriction of airways Bronchitis AKA tracheobronchitis Definitions Causative agents Clinical manifestations Bronchiolitis and RSV Definitions RSV = Respiratory syncytial virus Pathophysiology Diagnostics Therapeutic management Prevention of RSVprophylaxis Nursing considerations Pertussis (Whooping Cough) Caused by Bordetella pertussis In U.S. it occurs most often in children who have not been immunized Highest incidence in spring and summer Highly contagious Risk to young infants Vaccines Tuberculosis (TB) Caused by Mycobacterium tuberculosis human and bovine varieties Transmission modes Clinical manifestations

TB Testing Recommended procedure is Mantoux test Uses purified protein derivative (PPD) Standard dose and administration technique Positive reaction 5-mm induration 10-mm induration 15-mm induration Recommendations for TB testing Therapeutic Management of TB INH Rifampin PZA 6 month regimen Multi-drug resistantstreptomycin IM Prophylaxis for high-risk pt: INH 9-12 mos TB (contd) Prognosis Prevention Nursing considerations Pneumonia -acute inflammation of the pulmonary parenchyma -with alveolar consolidation -as a primary disease or a complication -bacterial/viral -types:

>lobar >interstitial >bronchial -s/sx: >cough >malaise >pleuritic pain Viral Pneumonia -most commonly caused by RSV -prognosis: good -treatment: symptomatic promote comfort oxygen inhalation(mist tent) chest physiotherapy fluid intake family support >fever >anorexia >tachypnea >wheezing

>headache >GI complaints

Primary Atypical Pneumonia -usually affects 5 to 12 years old children -usually lasts for 7 to 10 days -outcome: good -treatment:

symptomatic hospitalization Bacterial Pneumonia -with abrupt onset -usually preceded by a viral infection -treatment: antibiotic therapy bed rest liberal fluid intake/ IVF with O2 symptomatic hospitalization *infants *complcations Complications: a. empyema b. pyopneumothorax c. tension pneumothorax d. meningitis e. acute otitis media f. pleural effusion Prevention: pneumococcal polysaccharide vaccine Nursing Considerations: a. respiratory assessment b. oxygen administration (mist tent) c. antibiotics

d. balance between rest and activity e. cough remedies (antitussives, mucolytics) f. IV fluid infusion oral fluids g. postural drainage Foreign Body Aspiration -most common under the age of 3 -hot dogs, round candy, peanuts, grapes -manifestations: cannot speak

becomes cyanotic collapses -diagnosis: history >> physical assessment Bronchoscopy Fluoroscopic exam -management: a. Heimlich maneuver -over the age of one back blows and chest thrusts -under the age of one b. laryngoscopy c. bronchoscopy Asthma Chronic inflammatory disorder of airways

Bronchial hyper-responsiveness Episodic Limited airflow or obstruction that reverses spontaneously or with treatment Etiology and pathophysiology Asthma Severity Classification in Children 5 yrs and Older Step I: mild, intermittent asthma Step II: mild, persistent asthma Step III: moderate, persistent asthma Step IV: severe, persistent asthma Clinical features of each classification Asthma Diagnostic evaluation Therapeutic management Nursing considerations Implementation Drug Therapy for Asthma LT control meds Quick relief meds MDI Corticosteroids Cromolyn sodium Albuterol, metaproterenol, terbutaline Drug Therapy for Asthma (contd) LT bronchodilators (Serevent) Theophyllinemonitor serum levels Leukotriene modifiers

Others Asthma Interventions Exercise Chest physiotherapy (CPT) Hyposensitization Prognosis Status Asthmaticus Respiratory distress continues despite vigorous therapeutic measures Emergency treatmentepinephrine 0.01 mL/kg SQ (max dose 0.3 mL) Concurrent infection in some cases Therapeutic intervention Goals of Asthma Management Avoid exacerbation Avoid allergens Relieve asthmatic episodes promptly Relieve bronchospasm Monitor function with peak flow meter Self-management of inhalers, devices, and activity regulation Cystic Fibrosis (CF) Exocrine gland dysfunction that produces multi-system involvement Most common lethal GENETIC illness among white children Approximately 3% of U.S. Caucasian population are symptom-free carriers Cystic Fibrosis (contd) Incidence in U.S. Live Births 1 in 3300 Caucasians (95% of cases)

1:16,000 African-Americans 1:32,000 Asians Etiology of CF Autosomal recessive trait Inherits defective gene from both parents with an overall incidence of 1:4 Pathophysiology of CF Characterized by several unrelated clinical features Increased Viscosity of Mucous Gland Secretion Results in mechanical obstruction Thick inspissated mucoprotein accumulates, dilates, precipitates, coagulates to form concretions in glands and ducts Respiratory tract and pancreas are predominately affected Increased Sweat Electrolytes Basis of the most reliable diagnostic proceduresweat chloride test Sodium and chloride will be 2-5 times greater than the controls Other Factors Increased organic/enzymatic constituents of saliva Abnormalities of the autonomic nervous system Respiratory Manifestations Present in almost all CF patients but onset/extent is variable Stagnation of mucus and bacterial colonization result in destruction of lung tissue Tenacious secretions are difficult to expectorate-obstruct bronchi/bronchioles Respiratory Manifestations (contd) Decreased O2/CO2 exchange Results in hypoxia, hypercapnea, acidosis Compression of pulmonary blood vessels and progressive lung dysfunction lead to pulmonary hypertension, cor pulmonale, respiratory failure, and death

Infectious Pathogens Pseudomonas aeruginosa Burkholderia cepacia Staphylococcus aureus Haemophilus influenzae Escherichia coli Klebsiella pneumoniae Respiratory Progression Gradual progression follows chronic infection Bronchial epithelium is destroyed Infection spreads to peribronchial tissues weakening bronchial walls Peribronchial fibrosis Decreased O2/CO2 exchange Further Respiratory Progression Chronic hypoxemia causes contraction/hypertrophy of muscle fibers in pulmonary arteries/arterioles Pulmonary hypertension Cor pulmonale Pneumothorax Hemoptysis GI Tract Thick secretions block ductscystic dilation degeneration diffuse fibrosis Prevents pancreatic enzymes from reaching duodenum Impaired digestion/absorption of fat-steatorrhea Impaired digestion/absorption of protein-azotorrhea GI Tract (contd) Endocrine function of pancreas initially stays unchanged

Eventually pancreatic fibrosis occurs; may result in diabetes mellitus Focal biliary obstruction results in multilobular biliary cirrhosis Impaired salivation Clinical Manifestations of GI Tract Pancreatic enzyme deficiency Progressive COPD associated with infection Sweat gland dysfunction Failure to thrive Increased weight loss despite increased appetite Gradual respiratory deterioration Presentation Wheezing respiration, dry nonproductive cough Generalized obstructive emphysema Patchy atelectasis Cyanosis Clubbing of fingers and toes Repeated bronchitis and pneumonia Presentation (contd) Meconium ileus Distal intestinal obstruction syndrome Excretion of undigested food in stool increased bulk, frothy, and foul Wasting of tissues Prolapse of the rectum Presentation (contd) Delayed puberty in females

Sterility in males Parents report children taste salty Dehydration Hyponatremic/hypochloremic alkalosis Hypoalbuminemia Diagnostic Evaluation Quantitative sweat chloride test Chest x-ray PFT Stool fat and/or enzyme analysis Barium enema Goals Prevent/minimize pulmonary complications Adequate nutrition for growth Assist in adapting to chronic illness Respiratory Management CPT Bronchodilator medication Forced expiration Aggressive treatment of pulmonary infections Home IV antibiotic therapy Aerosolized antibiotics Respiratory Management (contd) Pneumothorax Hemoptysis Nasal polyps

Steroid use/nonsteroidal anti-inflammatory Transplantation GI Management Replacement of pancreatic enzymes High protein high calorie diet as much as 150% RDA Intestinal obstruction Reduction of rectal prolapse Salt supplementation Prognosis of CF Estimated life expectancy for child born with CF in 2003 is 40-50 years Maximize health potential Nutrition Prevention/early aggressive treatment of infection Pulmonary hygiene New researchhope for the future Gene therapy Bilateral lung transplants Improved pharmacologic agents Family Support Coping with emotional needs of child and family Child requires treatments multiple times/ day Frequent hospitalization Implications of genetic transmission of disease Respiratory Failure Defined as inability of respiratory system to maintain adequate oxygenation Terms To Know

WOB = work of breathing Apnea = absence of breathing Respiratory insufficiency Respiratory arrest = cessation of respiration Management of Respiratory Failure Clinical manifestations of respiratory failure Diagnostic evaluation Therapeutic management Ventilation techniques and strategies Nursing considerations Gastrointestinal Disorders 1. Dehydration 2. Water Balance in Infants -greater need for water -greater fluid intake and output relative to size -water and electrolyte imbalance develop more rapidly -increased ECF than ICF; Increased NaCl *until 2 years old -insensible fluid losses *2/3 > skin *1/3 > respiratory tract fever -may result to dehydration (7ml/kg/24 hours for each degree rise in body 37.2C) 3. Body Surface Area -premature > 5x higher than an older child temperature above

-term

> 2-3x higher than an older child

4. Basal Metabolic Rate -higher in infancy > increased production of metabolic wastes > increased heat production > increased insensible fluid loss > increased need for water 5. Kidney Function -inability to concentrate or dilute urine -inability to conserve or excrete sodium -inability to acidify the urine -inability to handle large quantity of solute free water or concentrated solutions 6. Fluid Requirements Factors: -hydration status -size -environmental factors -underlying disease >20kgBW Dehydration: -occurs in two ways: 1. out of the body into the stools 2. into the ICF compartment to replace electrical equilibrium -types: 1. Isotonic (isosmotic or isonatremic) potassium to maintain 20ml/kg = 100ml/kg= 10kgBW 50ml/kg = 11-20 kgBW

-the primary form of dehydration in children -water and salt are lost in approximately amount -no osmotic force between the ECF and -major loss is sustained from the ECF -significantly reduces the plasma volume >decreased blood volume > shock 2. Hypotonic (hyposmotic or hyponatremic) -electrolyte deficit exceeds the water -ICF is more concentrated than ECF -greater proportional loss of ECF -more severe form of dehydration even small volume of fluid losses 3. Hypertonic (hyperosmotic or hypernatremic) -water loss in excess of electrolyte loss -proportionately larger loss of water or intake of electrolytes -is the most dangerous type of dehydration -may result from excessive solute load in fluids or high protein content of NGT feedings -fluid shifts from lesser concentration the ECF -cerebral changes are more serious and result to permanent damage -diagnostic evaluation: a. weight monitoring may oral ICF to larger with deficit hypovolemic ICF equal

-most important determinant of the percent of the total body fluid loss children b. Level of Consciousness c. Response to Stimuli d. Skin Elasticity and Skin Turgor e. Capillary Refill f. Heart Rate g. Eyes and Fontanels -management: a. assessment Disorders of Motility 1. Diarrhea -acute diarrhea -acute infectious diarrhea -chronic diarrhea -intractable diarrhea -chronic non-specific diarrhea (irritable colon of infants and toddlers)

in infants and younger

*common cause of diarrhea in children 6months of age -consequences: *dehydration *shock -diagnostic evaluation: 1. History -history of recent travel *acid-base balance

54

-exposure to an untreated water -contact with an animal or birds -day care center attendance -antibiotic use -recent diet changes -associated s/sx 2. CBC 3. Stool Culture 4. Serology 5. Stool Exam 6. Stool pH 7. Stool electrolyte 8. Urine specific gravity 9. Serum electrolyte and waste product -management: a. assessment of F&E imbalance b. rehydration *ORS *IVF therapy volume per volume replacement c. maintenance fluid therapy d. reintroduction of an adequate diet *BRAT diet should be avoided -nursing consideration 1. maintenance of adequate hydration 2. maintenance of adequate nutrition determination

3. prevention of spread of infection 4. appropriate support and education -prevention a. personal hygiene b. protecting the water supply from contamination c. careful food preparation -nursing alert: a. quickly freeze all ground meat and other b. never thaw food on the counter or let it sit refrigerator for more than 2 hours c. wash hands or utensils and work areas with water d. proper cooking (do not eat raw meat) Constipation -alteration in the frequency, consistency or ease in passing out stools -3 or more days of without passing out stools -painful bowel movements, blood streaked stools -obstipation (long intervals in between defecation) -encopressis (constipation with fecal soiling) -usually due to idiopathic or functional Hirschsprung Disease -congenital aganglionic megacolon -diagnostic evaluation: a. clinical s/sx: *distended abdomen *contracted anal sphincter constipation perishable foods out of the hot, soapy

*small caliber empty rectum b. radiography c. ano-rectal manometric examination d. full thickness rectal biopsy -management: A. surgery -removal of the aganglionic portion -restore normal bowel motility -restore the function of the internal anal sphincter *one stage surgery *two stage surgery >for severely distended abdomen >involves temporary osteotomy then corrective surgery -treatment of anal stricture and incontinence

-nursing considerations: (neonatal) 1. help parents adjust to congenital defect 2. foster an infant-parent bonding 3. prepare for medical/surgical interventions 4. assist in colostomy care after discharge A. Pre Operative Care -symptomatic treatment with enemas -low fiber high calorie high protein diet

-total parenteral nutrition -measurement of abdominal circumference -explanation of the procedure B. Post Operative Care -abdominal surgery care Gastroesophageal Reflux Disease -may develop even without regurgitation - becomes a disease if with: >failure to thrive >bleeding >dysphagia >respiratory symptoms *apnea *bronchospasm -causes: >dysfunction of the LES >delayed in gastric emptying >poor clearance of the esophageal acid >susceptibility of the mucosa to acid injury -risks: a. prematurity Cleft lip -facial malformation occurs during embryonic -failure of the maxillary and median nasal process -occurs during 5-6 wks. of fetal life -causes: development to fuse. *laryngospasm *pneumonia

a. maternal factors b. hereditary influence c. environmental factor -diagnosis: a. sonogram while in utero b. physical features -signs and symptoms: a. readily visible at birth b. difficulty feeding especially bottle feeding c. abdominal distention d. dried cracked oropharynx -management: a. assess ability to suck or swallow secretions breathing without distress b. modify feeding technique c. hold upright position, hold formula on the the mouth d. feed small amount gradually and BURP e. position on side after feeding f. keep suction equipment and bulb syringe g. encourage breast feeding h. surgical repair between 1-3mos Z-plasty Cheiloplasty B. Post-op care: a. NPO 4 b. place on lateral position, avoid prone pos. on bed side frequently side and back of and

c. LOGAN bar to protect surgical incision d. restraint elbow e. after feeding clean suture line with dipped with saline f. can feed normally when sutures are g. no hard objects h. anticipate needs Cleft palate -failure of the union of the palatal process that 9-12 wks gestation. -signs and symptoms: *infection especially aspiration pneumonia *altered speech *hearing problems -diagnosis: a. apparent at birth b. use of finger to inspect the palate c. tongue blade and flash light. -management: a. surgical repair after the child has grown before speech is developed 12-18 wks. PALATOPLASTY >Post-op care a. lie on abdomen prone b. feeding resume by bottle breast or cup c. secure oral packing remove after 2-3 d. do not allow to brush teeth days but not normally close during the cotton swab removed

e. avoid hard foods f. restraint and remove every 2 hrs g. avoid using of oral suction or placing object in the mouth h. analgesics for pain i. refer to speech pathologist Pyloric stenosis -hypertrophy of the circular muscle of the pylorus -occurs on the first few wks. of life -more common in males than in females -causes: a. unknown b. genetic predisposition -diagnosis: a. UTZ b. upper GI X-ray c. endoscopy d. electrolyte study -signs and symptoms: a. projectile vomiting (3-4 ft.) shortly after 60 mins.) b. hunger and irritability olive shape mass epigastrium just right of the umbilicus c. signs of dehydration and malnutrition d. dry mucus membrane e. decrease urine output f. poor skin turgor in the feeding (30and causes obstruction pacifier

g. sunken fontanels h. fever i. weight loss j. electrolytes imbalance k. metabolic alkalosis -management: a. monitor VS b. monitor intake and output c. monitor signs of dehydration and imbalance d. surgery PYLOROMYOTOMY (fredet-ramstedt procedure) >Post-op care: -maintain IVF -feed slowly and burp frequently -monitor surgical wound for signs of infection Intussusception -invagination of one portion of the intestine into -occurs between 3 months to 5y/o -common in males -cause: >idiopathic -signs and symptoms: *colicky abdominal pain severe every 15-20 *vomiting bile stained mins. another electrolyte

*fecal emesis *currant jelly like stools containing blood and mucus *hypo/hyper active bowel sounds *tender distended abdomen and sausage mass on the URQ *increase temp. *peritoneal irritation -complications a. ischemia b. perforation c. peritonitis d. shock -diagnosis: a. barium enema b. bowel examination c. blood studies especially WBC -management: a. monitor signs of perforation or shock b. increase HR c. fever d. change in LOC e. decrease BP f. increase respiration g. prepare for hydrostatic reduction h. monitor for passage of normal brown stool i. surgical operation shaped

 IMPERFORATED ANUS -incomplete development or absence of the anus in in the perineum -etiology: congenital -diagnosis: a. X-ray b. sonogram -signs and symptoms: a. (-) meconium in 24hrs. b. absence or stenosis of the anorectal c. abdominal distention -management: a. repairs depend on the site b. (upper) palliative colostomy if there is until 6-12 mos. c. post-op care d. 1 wound care e. keep area clean f. zinc- oxide g. hydrocolloids h. pos. in side- lying with head suspended i. formula feeding when normal peristalsis j. bowel training Hydrocephalus -increased production or decreased absorption of CSF at 90 present fistula canal its normal position,

-leads to increased ICP >> passive dilation of the ventricles -may occur in children <10-12y/o -diagnosis: a. MRI b. CT Scan c. Echoencephalography -in neonates -ratio of the lateral ventricle vs. cortex -causes: a. congenital >maldevelopment (most common) *Arnold-Chiari Malformation *aqueduct stenosis *aqueduct gliosis *atresia of the foramina of Lushka and Magendie >acquired *neoplasm *hemorrhage *infection -2 types: A. Communicating B. Non communicating -clinical picture depends on 2 factors: a. time of onset b. presence of pre-existing structural lesions -management:

Goals: 1. Relief of hydrocephalus 2. Treatment of complications 3. Management of the problems related to the of the disorder on psychomotor development -surgical management: a. removal of obstruction b. shunting -ventricular catheter -unidirectional flow valve -distal catheter with pre-determined intraventricular pressure Complications of Shunting *infection >most common >1-2months after placement (greatest risk) septicemia shunt nephritis effect

bacterial endocarditis meningitis wound infection ventriculitis

c. Endoscopic Third Ventriculostomy -alternative to shunting -for children with non communicating hydrocephalus Complications: *hemorrhage *sterile meningitis

*septostomy failure -prognosis: depends on a. rate of development b. duration of increased ICP greatest risk c. frequency of complications d. cause of hydrocephalus -nursing management: a. head circumference monitoring >occipito-frontal circumference b. palpation of fontanels and suture lines >assoc. with straining and crying c. LOC monitoring >most reliable indicator in older children d. nutrition >small, frequent feedings e. ventricular tap >with sedation -post op care: a. position to unaffected side b. flat on bed c. prevent subdural hematoma d. pain management NSAIDs narcotics e. monitoring for the s/sx of inc ICP *80% survival *Ist year of death

f. monitoring for abdominal distention peritonitis post op ileus g. I and O monitoring h. monitoring infection i. skin care Spina Bifida -a neural tube defect -with multifactorial inheritance Myelodysplasia >malformation of the spinal canal and cord 1. Spina Bifida Occulta -not visible externally -most frequent in the lumbosacral area -may not be apparent 2. Spina Bifida Cystica -2 types: a. meningocoele b. myelomeningocoele -pathophysiology: 1. failure of closure of the neural tube (first 3-5 weeks AOG) 2. multifactorial etiology drugs radiation pre-pregnancy obesity

maternal malnutrition previous NTD preg

chemicals -diagnosis: a. clinical s/sx b. examination of the meningeal sac c. MRI d. CT scan e. Myelography f. Lab exams for complications meningitis UTI -prenatal detection a. prenatal fetal UTZ b. alpha fetoprotein elevation (gamma globulin) >should be done between 1618weeks AOG *anencephaly *myelomenigocoele c. chorionic villus sampling >drawbacks (avoid before 10 weeks) 1. SKELETAL LIMB DEPLETION -management: a. early fetal surgery b. collaborative management of neurology, neurosurgery, urology, pediatrics, rehabilitation, social services INFANCY: a. surgery (first 24-72 hours) orthopedics, PT,

first 1218 hours >> best b. antibiotic treatment ORTHOPEDIC a. prevention of joint contractures b. correction of existing deformity c. prevention of sensory and motor deficits d. prevention of skin breakdown -common problems: a. hips b. spine GUT -neurogenic bladder >>>Urinary System Distress -ureterohydronephrosis -symptomatic UTI -vesicoureteral reflux -renal insufficiency txt: antibiotics, intermittent cath, vesicostomy BOWEL CONTROL a. diet modification b. regular toilet habits c. prevention of constipation and fecal impaction MACE- Malone Antegrade Continence Enema -prognosis: variable c. knees d. feet

-prevention a. folic acid supplementation .4mg/day age b. folic acid 4mg/day women of childbearing with previous NTD preg (1 month prior to preg till first semester) Developmental Dysplasia of the Hip -previously known as the congenital hip dysplasia -with shallow acetabulum, subluxation or -left hip in 60% of cases right hip in 20% of cases both hips in 20% of cases -causes: unknown more common in girls family history intrauterine position type of delivery -predisposing factors: a. physiologic -maternal hormones -intrauterine positioning b. mechanical -breech presentation -multiple fetuses -oligohydramnios joint laxity post natal positioning birth order dislocation

-large infant size c. genetics -6% higher in infants with affected -36% higher in infants with one affected parent -2 major groups: A. typical -with intact CNS B. teratologic -with neuromuscular defect -3 degrees: a. acetabular dysplasia (preluxation) b. subluxation -incomplete dislocation -largest percentage of DDH c. dislocation -diagnosis: a. physical assessment b. ortolani and barlows test c. radiography -not reliable <3mos d. UTZ -for screening only e. CT scan -treatment: A. newborn to 6 months -Pavlik harness

-skin traction -hip spica cast B. 6-18 months -traction(3 weeks time) -closed reduction -hip spica cast c. older child -operative reduction >with pre op traction -casting >> cast removal >> ROM ex >>weight bearing -at 4 years old (difficult) -at 6 years old (impossible) Congenital Clubfoot -types: A. Talipes Varus B. Talipes Valgus -more common in boys -most common >> Talipes Equinovarus -unilaterality >> more common -may be associated with other abnormalities: a. chromosomal abnormalities b. arthrogryposis c. cerebral palsy d. spina bifida -causes: unknown C. Talipes Calcaneus D. Talipes Equinuus

abnormal positioning in utero >> flexible deformity arrest in embryonic development >> rigid def -classification A. positional clubfoot -also known as transitional, mild, or postural -treated by simple stretching and casting B. Syndromic or Teratologic -assoc with other anomalies -more severe form -more resistant to treatment C. Congenital Clubfoot -idiopathic -may occur in a normal child -wide range of rigidity and prognosis -diagnosis: a. UTZ (prenatal) b. physical assessment -at birth -management: 3 stages: A. correction of the deformity B. maintenance of the correction until normal balance is regained C. follow up observation to avert possible of deformity 1. casting muscle recurrence clubfoot

-8-12 weeks (Dennis Brown Splint) 2. Surgery -6-12 months of age -followed by immobilization by 6-12 weeks Acute Post Streptococcal Glomerulonephritis -features: A. oliguria B. edema C. hypertension -most are post infectious pneumococcal streptococcal viral -mostly affects school age children (6-7 y/o) >> males: females (2:1) -etiology: streptococcal (pharyngitis and pyoderma) -pathophysiology: immune basement deposition in the membrane inflammation features of AGN -diagnosis: 1. urinalysis >hematuria >proteinuria >no bacteria glomerular basement D. circulatory congestion E. hematuria F. proteinuria

2. serum chemistry >azotemia >may have an associated nephrotic syndrome 3. ASO titer antihyaluronidase antiDNAse B streptozyme 4. decreased serum complement >guide to assess improvement (should gradually elevate 8 weeks after 5. CXR >cardiomegaly >pulmonary congestion >pleural effusion 6. renal biopsy >seldom required -management: 1. sodium and fluid restriction potassium restriction 2. vital signs, I & O, body weight monitoring 3. peritoneal/ hemodialysis 4. antibiotics Wilms Tumor -most common malignant renal and -3 y/o (peak of diagnosis) -more common in boys intraabdominal tumor of childhood

-more common in the left -diagnosis: 1. comprehensive health history 2. physical assessment 3. radiographic work ups A. abdominal UTZ B. abdominal and chest CT scan 4. hematologic studies 5. biochemical studies 6. urinalysis 7. BMA -management: 1. surgery- ASAP 2. chemotherapy *chemotherapy >6-15 months >Actinomycin D >Vincristine >Adriamycin + Cyclophosphamide -prognosis: Good Pre-op Care: a. surgery in 24 to 48 hours -no sufficient time for preparation -no palpation Post op Care: 3. radiotherapy

*complications a. Vincristineb. Radiationinduced ulcers induced edema

c. Post surgical adhesion formation Several children and their mothers are waiting for their medical appointments in the pediatric cardiology clinic. Cindy is 2 years old and has patent ductus arteriosus. Billy is 6 months old and has a ventricular septal defect. Harold is 4 years old and has tetralogy of Fallot. 1. Mothers accompanying these children are discussing when their childrens cardiac defects were first recognized. Cindys mother narrates that Cindys PDA was discovered at 4 weeks of age during her first pediatric examination when the nurse noted; A. Clubbing of her fingers B. A machinery-like murmur C. Reddening of her lips when crying D. A resting respiratory rate of 35 breaths per minute 2. Harolds mother tells the other mothers that the congenital heart disease was discovered in the nursery when the nurse reported that; A. His heart rate was 180 beats/min B. He continuously drooled mucus, even between feedings C. His fingers and toes were cyanotic D. He appeared tense, with contracted muscles even when sleeping 3. Billy has been gaining weight very slowly. Because he feeds so poorly, Billys mother asks the nurse how to increase his food intake. The nurse should suggest that she may; A. Offer nutritious finger foods between feedings B. Enrich the formula and enlarge the nipple hole C. Feed him only when he is hungry and cries vigorously for food D. Speak to the doctor about learning how to tube feed him

4. Billys mother tells the nurse that since learning of her sons diagnosis, she has problems in concentrating and difficulties in sleeping. Which response by the nurse will best help her validate her feelings? A. Do you feel that your son will not recover B. This must be a rough time for you C. You havent been able to concentrate or sleep D. Since your sons diagnosis you have been depressed Harold has been admitted to the hospital for correction of the tetralogy of Fallot. 5. Before surgery, a cardiac catheterization is performed. After the cardiac catheterization, the nurse takes the pedal pulse on the affected side. Noting that there is no pulse, the nurse should; A. Take the pulse 10 minutes later because pulse strength gradually returns during the first few hours B. Check the pedal blood pressure because the blood pressure returns faster than the pulse C. Ask the child to move the affected leg because there may be a complication of peripheral nerve damage D. Notify the physician because an absent pulse is a sign of blood vessel obstruction 6. Harold has been receiving digoxin elixir. One morning he refuses to take the prescribed dose. The nurse should ask if he would like to; A. Take his medicine after his bath B. Have apple juice or water to wash it down C. Pour the correct amount himself D. Have a special toy after he takes his medicine 7. While walking from the playroom to his room, Harold suddenly squats. The nurse should; A. Ask him if he has to go to the bathroom B. Pick him up and carry him to his room C. Allow him to squat until he is ready to continue walking

D. Take his pulse and blood pressure immediately Harold had open heart surgery. 8. Two days after surgery, a dark bloody fluid is draining from the chest tube. The nurse should; A. Report this, because bleeding should start to diminish after 24 hours B. Check that the seals of the underwater drainage bottles are air tight C. Record this, because bleeding is expected to continue for 72 hours D. Observe if the fluid in the drainage tube rises and falls with each breath 9. Discharge planning for Harold includes teaching his parents the importance of A. Continuation of restricted activity for at least 6 months B. Gradual increase in activity with expanding responsibilities and social contacts C. Encouraging all activity that is expected of a healthy 4 year old child D. Regular short periods of activity with rest periods in between The nurse is caring for several infants in the newborn nursery. The nurse observes that 10 hour old Jennifer has difficulty maintaining a patent airway despite frequent suctioning. 9. At her first feeding, Jennifer gags and regurgitates after each swallow. The nurse should immediately; A. Suction before continuing with the feeding B. Take an apical pulse C. Notify the physician D. Check the amount of maternal analgesics (labor) A diagnosis of esophageal atresia has been made. 10. Which maternal complication should alert the nurse to observe the newborn for this problem? A. Hydramnios B. Breech presentation C. Prolapsed cord

D. Pregnancy induced hypertension 11. If Jennifer had a tracheoesophageal fistula in addition to esophageal atresia, the nurse would have observed; A. Reverse peristaltic waves B. Thoracic breathing C. Abdominal distention D. Pronounced tachycardia 12. Which nursing action would be most helpful in meeting Jennifers developmental needs in the first few days after surgery to repair the esophageal atresia? A. Hold her frequently to prevent crying B. Position her on her abdomen to promote active movement C. Offer her a pacifier to provide sucking D. Change her soiled diapers immediately to maintain comfort Richard is 3 months old. He has gained 1 pound since his last visit to the well baby clinic 1 month ago. His mother tells the nurse that he has several episodes of constipation alternating with diarrhea. 13. Which observation by the nurse would assist in the diagnosis of Hirschprungs disease? A. Low grade fever B. Abdominal distention C. Dry, scaly skin D. Amber colored urine A diagnosis of Hirschprungs disease is confirmed. 14. Richards newborn chart is reviewed. His birth weight was 3.4kg. Which note by the nurse would indicate that Richard had an early sign of megacolon while he was in newborn nursery? A. day1: suctioned twice, thick mucus return B. day 2: passed first meconium stools

C. day 3: axillary temp- 36C D. day 4: weight 3.2 kg 15. Daily enemas have been ordered for Richard. The nurse should teach Richards mother how to prepare a/an; A. Hypotonic enema B. Isotonic enema C. Hypertonic enema D. Soapsuds enema 16. Surgical resection has been recommended for Richard. His parents ask the nurse how they will know for certain that the surgery has been completely successful? The nurse responds that the surgery is successful if he is able to; A. Defecate without straining B. Eat without vomiting and his appetite improves C. Have at least one normal bowel movement each day D. Maintain bowel control when he is toilet trained A live baby boy, term by LMP, 34weeksAOG by first trimester ultrasound has been born from a mother through NSD who is admitted because of Pneumonia with Sepsis. The neonate was born cyanotic, flaccid with meconium stained amniotic fluid. The vital signs were as follows: RR=70, PR=180, T=35.6C. Apgar Score=2,3. 1. The baby is primarily at risk of; a. meconium aspiration syndrome b. hyaline membrane disease c. retrolental fibroplasia d. neonatal seizures 2. In Hyaline Membrane Disease, dyspnea is due to what mechanism/s; a. lack of surfactant

b. intense inflammation damaging the AC2 c. ventilation/perfusion mismatch d. hypoxemia 3. Due to the immaturity of the neonatal liver, the baby developed jaundice in the first 18hours of life; a. physiologic jaundice b. pathologic jaundice c. nutritional jaundice d. breastmilk associated jaundice 4. The highest priority should be focused on; a. suctioning b. surfactant replacement c. mechanical ventilation d. administration of steroids 5. Jaundice is primarily treated by using phototherapy. Phototherapy will enhance the conversion of; a. conjugated to unconjugated bilirubin b. fat soluble to water soluble bilirubin c. direct to indirect bilirubin d. any of the above 6. Early onset sepsis is most commonly caused by; a. a maternal GIT bacteria b. an airborne bacteria c. a fetal GIT bacteria d. a droplet bacteria 7. All of the following are true to caput succedaneum, except; a. resolution will take 3 days

b. limited by the margins of bony structure c. usually associated with difficult labor and delivery d. usually seen among multiparous women 8. All of the following reflexes may be lost by the end of the 4th month, except; a. extrusion reflex b. moro reflex c. startle reflex d. babinski reflex

9. The first sign of female sexual maturity; a. growth of pubic hairs b. first appearance of menses c. development of the breast d. deepening of the voice 10. Hemorrhagic Disease of the Newborn is; a. Rh incompatibility b. ABO incompatibility c. Vitamin K deficiency d. Hemophilia A 11. The clinical picture of a mother and baby with the baby suffering from ABO inc; a. baby A, mother B b. baby O, mother A c. baby A, mother O d. baby B, mother A 12. Clinical picture of RH inc; a. baby Rh+, mother Rhb. baby Rh-, mother Rh+ c. baby Rh+, mother B

d. baby Rh-, mother O 13. Low APGAR score of the premature neonate is secondary to poor adjustment to extrauterine life. This is due to the poor compensation of; A. Cardiovascular System B. Respiratory System C. Neuroendocrine System D. Thermoregulatory System 14. Meconium aspiration syndrome of the neonate on the same case is due to; A. cardio-respiratory distress B. fetal hypoxemia C. overwhelming infection D. decreased tissue perfusion 15. The single most important nursing management in a newborn with sepsis is; A. Strict handwashing technique B. Administration of prophylactic antibiotics C. Administration of vaccines D. Use of antibiotics and vaccines 16. A machinery-like murmur of patent ductus arteriosus was appreciated upon auscultation. It was confirmed after echocardiography. Four days already passed and still the patency remains. How are you supposed to intervene? A. Watch out umbilical cord bleeding B. Wait for 4 years before you decide to intervene C. Talk to the physician about the planned surgery D. Reassure the parents about the spontaneous closure of the defect 17. The mother in a hurry approached the nurse about a current seizure episode. What should be done immediately?

A. Provide supplemental oxygen B. Administer prn diazepam C. Insert a tongue depressor wrapped in gauze by mouth D. Reassure the mother that nothing bad will happen to the baby A live baby boy was born through NSD to a 45 years old primigravid mother at 37 weeks AOG. APGAR is 4,6. Cyanosis was noted on the extremities and trunk. Throughout the course of assessment, he was seen to have low set ears, with Simian creases, large protruding tongue and with flat occiput. The umbilical cord contains only 1 artery and 1 vein. His mother further verbalized previous rubella infection during the 10th week of pregnancy. 1. Which of the following should raise your suspicion as a nurse about the possibility of cardiovascular congenital anomaly? A. Low set ears with large protruding tongue B. 1 umbilical artery and 1 vein C. APGAR score of 4 and 6 D. Cyanosis on the trunk and lower extremity 2. The signs presented are highly suggestive of a chromosomal defect. Most likely it would be? A. Trisomy 18 B. Trisomy 21 C. Edwards Syndrome D. Patau Syndrome 3. What is the most likely cause of cyanosis if the baby was diagnosed to have pulmonary congestion after right sided cardiac catheterization? A. Patent Ductus Arteriosus B. Total Anomalous Pulmonary Venous Connection C. Tetralogy of Fallot

D. Ventricular Septal Defect The nurse in the well baby clinic is caring for several infants aged 1 to 6 months. 4. Joan is 1 month old. Her mother tells the nurse that Joan is an exceptionally good baby who sleeps well and rarely cries. The nurse should carefully observe for signs of cretinism when Joans mother add that her baby; A. feeds well, quickly finishing all the formula in her bottle B. very active when awake, making crawling in her crib C. seems constipated and has one firm yellow stool every other day D. is usually warm, flushing and perspiring readily 5. Dessicated thyroid is ordered for Joan. Her mother asks how long Joan must take this medication. How should the nurse respond? A. until after she finishes her prepubertal growth spurt B. when the pubescent period has been completed and she enters adulthood C. annual evaluation of T3 and T4 levels will be used to determine discontinuation D. indefinitely, probably for the rest of her life 6. Three months later, during a clinic visit, the nurse evaluates Joans developmental status to determine the effectiveness of the thyroid therapy. Which observation would indicate that the therapy is effective? A. laughs readily and can turn from back to side B. has strong Moro and tonic neck reflexes C. can lift her head and chest a short distance when lying on her abdomen D. keeps his fists clenched, opens her hand when grasping an object An infant born at 38 weeks AOG was fed with formula milk right after delivery. 30 minutes after feeding, the baby became irritable and passed out watery stools. Diagnosis was made and it was Lactose Intolerance. 7. What is the most likely type of lactose intolerance on the said case? A. Congenital Lactase Deficiency B. Primary Lactase Deficiency

C. Secondary Lactase Deficiency D. Virally induced Lactase Deficiency 8. As nurses, management should be focused on; A. Feeding using lactose free milk formula B. Feeding using lactase treated formula C. Avoiding milk then resumption of regular feeding after 24 hours D. Breastfeeding 9. The nurse should be concerned on; A. Susceptibility to infection B. Malnutrition C. Dehydration D. Enterocolitis 10. A four year old child developed Rheumatic Heart Disease. He developed a systolic murmur due to aortic valve stenosis. As a nurse, what should be your highest concern? A. Antibiotic therapy B. Provision of bed rest C. Nutritional supplementation D. Rehydration