Simmons 501 Final Review: 4th Exam COMPREHENSIVE Question Bank – 625 Expert-Verified Q&A on Complex Case Studies, Diagnostic Reasoning & Patient Management (NCLEX-Aligned | Latest Guidelines)

Normal vitals and measurements newborn

Head: 12-15 inches

Weight: 5.8 - 8.12 lbs

Length: 18-22 inches

T: 97.7 - 99.0 F

HR: 80-180

RR: 30-60

BP: not routinely done in primary care unless there is an issue

Pain: FLACC scale

Respiratory rate assessment

Assess for Accessory muscle usage and/ or chest excursion

RR > 60 --

Tachypnea > 6 breaths per minute at rest, earliest sign of respiratory, metabolic, cardiac, infectious, or hypothermic condition. Evaluate pre/post ductal O2, POC blood sugar, referral neonatology/ pediatrics.

Apnea --

Cessation for 20+ seconds accompanied by physiologic changes, pallor, O2 desaturation.

Possibly metabolic or infectious, respiratory or neurologic, hypothermia. Evaluate pre/post ductal O2, POC blood sugar, referral neonatology/ pediatrics.

Cardiac assessment

Done when infant is quiet

-Auscultate 5 areas: aortic, pulmonic, erb's point, tricuspid and mitral areas.

-Observe S1 and S2 for normalcy and S3, S4 for presence

-Palpate for heaves, lifts and thrills

PDA - patent ductus arteriosus:

-machine like sound, crescendo decrescendo

- when blood is shunted from the aorta to the pulmonary artery

- best heard 4th ICD, LSB

-REFER to pediatric cardiologist

ASD -- atrial septal defect

- in the pulmonic area

VSD -- ventricular septal defect

- like a harsh systolic murmur

Systolic murmur

-- coarctation of the aorta

When to refer murmur:

- Grade 3+

- diastolic (never normal)

- SOB, accessory muscle use, decrease in feeding and energy and a color change

Normal benign murmur:

transient and benign in newborns

soft, grade 1-2, systolic, resolve in 2 weeks

Skin assessment

Cap refills less than two seconds

Pink well perfused skin

pink and moist mucus membranes

elastic skin turgor

Acrocyanosis -- not concerning

- bluish discoloration of the hands and feet

normal in the 1st 24-48 hrs of life due to immature cardiac circulation

Acrocyanosis in older infants --

- in relation to cold stress

Harlequin's sign --

- unilateral color change on one side of the body due to autonomic basomotor instability

- transient and harmless lasting 10-20 minutes

- risk factors -- LBW

- causes -- autonomic vasomotor instability

Erythema toxicum --

common skin finding 30-70% of newborns, yellow or white 1.3mm papules will appear over and erythematous base anywhere on the body with exception of palmar surfaces

Strawberry hemangioma --

- raised capillary nevi that can occur anywhere on the body

- often increases in size over the 1st few months of life

- normally disappears by 10 years of life

- REFER if -- orbital hemangiomas, those that are very large or with potential to interfere with airways

Stork bites --

- pale pink or reddish discoloration of skin that occur on the nape of the neck, lower axilla, nasal bridge and eyelids, becoming more evidence when child cries

- disappear age 2, no treatment needed

Vernix caseosa --

- cheesy grey white substance that covers and protects the skin during fetal life (intra uterine)

- disappear closer to birth in term infants

Neonatal acne --

- caused by maternal hormone stimulation of the sebaceous glands

- appears age 3-4 weeks

- risk factors - more common in male infants

- treatment - none, topical lotions and creams make it worse

Petichiae --

- pinpoint hemorrhage on the body

- concerning -- when it occurs on a non presenting portion of the body

Jaundice --

- yellowing of skin and eyes, most visible after blanching

- progresses head to toe

Cyanosis -- types - require assessment for pathological condition

peri orbital -

circum oral

chest/abdominal

central cyanosis

Cafe - au - lait spots --

- concern -- newborns with 6 or more spots that are over 1 cm require workup for neurofibromitosis

Mottling --

- marbling spider web type appearance on the skin

- can be caused by hypothermia or infection

- occurs in unhealthy infant

cutis marmorata --

- marbling spider webt type appearance on the skin

- disappears when skin is warmed

- occurs on healthy infants

Pale skin pallors --

- anemia or infection

- assess darker skin -- look at soles of their feet and their hands, and circumoral assessment

At what o2 sat is visible cyanosis occurring? and why?

<60%, due to fetal Hgb

Head

Anterior fontanel --

- It's the largest diamond shaped fontanelle, 2cm closes by 18 mos.

Posterior fontanel --

- triangle shaped, smaller, closes by 4 months

Suture lines --

- 5 suture lines

- can be open or overlapped on newborn skull

Cephalohematoma --

- collection of blood under the periosteum caused by pressure during labor or operative measures

- WILL NOT cross suture lines

- 6-8 weeks to resolve

- often from vacuum or forceps used in delivery

Caput suucedaneum --

- localized swelling of soft tissue of the scalp caused by pressure during labor and birth

- WILL CROSS suture lines

- 24-48 hours after birth to resolve

Eyes/ vision assessment

Fix and focus on objects --

- 8-10 inches from the face

Permanent eye color --

- 3-6 months

Assess --

size, shape, placement

Iris --

blue grey iris in light skin

brown iris in dark skin

Hypertelorism --

- eyes too widely spaced

Hypotelorism --

- eyes too close together

Third eye --

- during eye assessment, third eye should fit easily between the two eyes, if not, could indicate chromosomal anomaly or syndrome

Red reflex --

- Absent : possible infection or cataracts

- grey/white pupil: retinoblastoma

Palpebral fissure --

- indicate eye opening is too small or that there is a chromosomal anomaly or syndrome

Epicanthal folds --

- slanting of the eyes that could indicate chromosomal anomaly or syndrome

Ear assessment

Normal --

Firm flexible cartilage, brisk recoil, line from inner canthus of the eye to outer canthus to the occiput should have the top of the pinna touching it in normal ear placement.

Ear placement --

- angle of placement of the ear should be vertical, or no more than 10 degrees from vertical

Pre auricular sinus node --

- concerns about renal abnormalities, outer ear and renal tissue form at the same time

Pre auricular sinus tag noted on newborn --

- repeat hearing test at 6 months, get renal functional evaluated

Nose assessment

obligate nose breathers

If nasal passage way obstructed --

- difficulty feeding and become very upset

Mouth assessment

Ankyloglossia --

- heart shaped tongue

- tongue tie

- puts infant at risk for feeding difficulty and latching problems

Macroglossia --

- enlarged tongue

- chromosomal anomalies, trisomy 21

Palatal isses --

- cleft palate

- submucous cleft

- high arched palate

Thoracic/ lung assessment

1:1 anterior posterior ratio, cylyndrical chest

chest size -- 33cm, or 2 cm less than head measurement

Witches milk --

- milk from lactating babies (male too)

- normal response to mom's hormones from exposure during birth

Clavicles/ chest --

- look for crepitus and asymmetrical findings (investigate further)

Abdomen assessment

Newborn is domed and rounded.

Umbilical cord --

- 2 arteries

- 1 vein

- 1% of babies have single vessel cord

- falls off 7-10 days

Male genitalia

Urethral opening should be at the tip of the penis, testes should be descended and palpable, hydroceles should be assessed for transillumination

Epispadia --

urethral tip on top of the penis

Hypospadia --

urethral opening on dorsal side of penis

Female infant genitalia

Size assessment of labia majora, minora and clitoris

Blood tinged mucousy or milky vaginal discharge -- normal findings

Pilondial dimple

A finding at the base of babies spine, observe that the base is visible.

Newborn extremities

Upper extremities --

Full ROM, Symmetry, and brachial pulses

Lower extremities --

Femoral pulses

Barlow and Ortolani maneuvers

Symmetry of skin folds (gluteal and femoral areas)

Size and shape of newborn feet

Measures that affect breastfeeding

What are some NON-SUPPORTIVE measure that affect breast feeding?

NON-SUPPORTIVE --

-oxytocin during labor

-epidural anesthesia

-operative delivery

-cesarean delivery

SUPPORTIVE --

1 or more support people present during the birth and care by doula

Milk supply

Maintaining adequate milk supply is dependent on what?

Adequate milk supply --

nutritive stimulation of the breast and removal of milk on a regular and frequent basis.

Indicated good milk removal --

Suck:swallow:breathe -- 1 sec : 1sec : 1 sec

Newborn gut

Becomes sterilized by --

Maternal bacteria through breastfeeding.

Infant's immune system comes through colonized gut --

70% of it

What resource is used to look effects of medication and therapies on breastfeeding?

LactMed

Breastfeeding

Contraindications --

-maternal substance abuse

-cytotoxic and immunosuppressive drugs

- active TB

- HSV lesion on breast

- active varicella

- HIV infections in a developed country

- neonatal galactosemia

Common problems --

- sore nipples

- inverted nipples

- low milk supply

- candidas

- mastitis

- feeding refusal

- late pre term

- tongue tie

- palatal issues

- hypoglycemia

Ensure you address --

- obtaining breast pump

- milk storage

- bottle introduction

Feeding issues common to infants include?

GERD, constipation, diarrhea and colic

Newborn weights

1st days of birth infants lose --

5-8% of their birthweight

Breastfed infants return to birthweight --

at 2 weeks

3 weeks baby not returned to birthweight --

Failure to thrive

Infant doubles birth weight --

5-6 months

Infant triples birth weight --

12 months

Infant should gain daily --

0.5 - 1 ounce daily

Growth chart recommended by CDC until 24 months --

WHO growth charts

Minimum diaper output daily --

6 diapers daily

Infant growth spurts in breast fed --

- 1st few days

- 7-10 days

- 2-3 weeks

- 4-6 weeks

- 3/4/6/ and 7-9 months

Signs of growth spurt --

hungry

sleepy

grumpy

Poor weight gain linked to --

- infrequent feedings

- inadequate milk supply

- genetics

- infection, disease

- physical anomaly that prevents suck/swallow

Indications of poor weight gain --

- day 6 losing weight

- weight below third percentile

- lethargic, sleepy, inactive, unresponsive

- sleeping more than 5 hours between feedings

- dry mucous membranes

- poor skin turgor

- not maintaining 0.5 - 1 ounce weight daily

Sleeping through the night at 8-12 weeks old --

leads to poor weight gain

Ignoring hunger cues and scheduling feeds --

lead to poor weight gain

Amount of times to feed infant --

8 times a day

Maternal clinical findings of infant poor weight gain --

- recent use of COC

- illness or weight loss

- hectic schedule

- does not recognize infant hunger cues

Infant lost weight and is unable to feed with vigor --immediate referral to acute care center for hydration and infusion is indicated

Poor weight gain associated with --

- developmental delays

- poor bonding

- severe dehydration

Newborn screening/screens

Include in ALL screening --

- congenital hypothyroidism

- congenital adrenal hyperplasia

- hemoglobinopathies

- congenital heart disease

- galactosemia

-hearing

- severe combined immunodeficiencies

- PKU

-MSUD

Metabolic screening --

- congenital hypothyroidism

- PKU

- galactosemia

Hepatitis B vaccine

Administer --

Within the 1st 24 hours of birth

Weighs less than 2,000 grams --

- give at 1 month of age or discharge from hospital --whichever comes first

Maternal immunizations should include?

Influenza and Tdap

When does an infant become eligible for the flu vaccine?

At 6 months of age

Circumcision

Benefits --

Reduced rate of penile cancer

Reduced rate of UTI

Reduced STI

Risks --

inadequate or too much skin removal

bleeding

infection

urethral issues

injury

anesthetic complications

Bilirubin

- end product of heme metabolism

Low levels --

acts as antioxidant

High levels --

acts as a toxin

Jaundice

Frequency of occurrence --

80% of all US born infants

Peaks --

for most at 6mg/dl between 2 and 4 days of life

Less than 24 hours old newborn if jaundiced --

measure TSB, or TcB levels

Difficult to assess in --

dark skinned infants

Pathologic jaundice --

jaundice occurring in 1st 24 hours of life, or after the 1st week or lasts longer than 2 weeks

Causes pathologic jaundice --

isoimmunization

erythrocyte issues

structural issues

infections and sequestered blood

Pathologic jaundiced measured --

Evaluate all newborns whose TSB is not following the expected trajectory, or is not explained on physical exam should be evaluated for additional causes of hyperbilirubinemia.

isoimmunization

incompatible blood types or incompatible rH factors. (Maternal O, fetal A or B) or maternal Rh neg and fetal Rh positive.

erythrocyte issues

G6PD deficiency, an erythrocyte enzyme defect- evaluate for this if phototherapy response is poor.

What infections can cause jaundice

Sepsis impairs conjugation of bilirubin, increased hemolysis places newborn at risk for DIC.

Which newborns should have a measure of total and direct / conjugated bilirubin?

Sick newborns and those jaundiced at or beyond 3 weeks of age.

Hyperbilirubinemia

Causes --

Hepatic immaturity, decreased ability to conjugate bilirubin, decreased excretion of bilirubin, mild dehydration, low caloric intake

Risk factors --

less than 38 weeks gestation

exclusively breastfed

JAUNDICE risk factors acronym hyperbilirubenemia

- Jaundice in 1st 24 hours

- a sibling who was jaundiced

- unrecognized hemolysis

- non optimal sucking

- deficiency G6PD

- infection

- cephalohematoma

- east asian or mediterranean

How to screen --

heel stick or transcutaneous

- use transcutaneous when high possibility of anemia

Transcutaneous screen --

objective screen that looks at baby's color and gives you an objective number on their color

Treatment --

phototherapy

exchange transfusion

Primary care management --

- come back for 1st visit 1-3 days after discharge

- 2nd visit 3-5 days after discharge

- monitor infant weight, intake, output, voids, jaundice

- if in doubt obtain TSB or TcB

- biliblankets, home phototherapy or sunlight exposure

HSV in infants

Prevalence --

1 of 3000-20000 births

Types that appear first --

disseminated appears earlier in the 1st weeks of life

Incidence increases with --

prematurity

30-50% acquire it by --

maternal genital exposure

Local infection characterized by --

- skin vesicles or bullae

- conjunctivitis, keratitis or retinitis

- mouth ulcers

- initial lesions will present at areas of trauma (i.e. scalp electrode locations)

- presents during 2nd week of life

CNS infection characterized by --

- seizure and encephalitis

- occurs during 2nd - 4th week of life

Disseminated HSV characterized by --

- bacterial sepsis plus hepatits

- pneumonitis

- DIC

- can affect all major organs

- present during 1st 2 weeks of life

Diagnosis --

- cultures of vesicles

- nasopharyngeal and conjunctival swabs

- urine

- stool

- tracheal secretions

- CSF

Rapid dx test --

- vesicle scrapings

When to get suspected HSV cutaneous and mucosal cultures? --

- 24-48 hours after delivery to differentiate colonization from infection

DO IMMEDIATELY FOR SYMPTOMATIC INFANTS

Viral identification --

takes 1-3 days

Skin or mucous membrane Treatment --

Acyclovir, IV 20mg/kg/dose q8h x 14 days

CNS treatment --

Acyclovir, IV 20mg/kg/dose q8h x 21 days

At risk infant for HSV but negative culture --

- follow closely for 6 weeks

Primary prevention --

- empiric acyclovir IV q8h after birth pending neonatal cultures

- consider c-section

Cold sores when handling infants --

- wear mask until lesions crusted over

- strict handwashing

- avoid contact with infant

- dont breast feed if lesion is on breast

When to initiate HSV re activation therapy --

- symptomatic infant

- premature

- has acquired an open wound during delivery

Neonate born to mother of active HSV --

- mother with active lesions then baby should be isolated from other newborns and handled with contact precautions

Chlamydia

What percentage born to chlamydia infected mothers acquire chlamydia --

50%, although 70% will be asymptomatic

How is it acquired --

- during delivery via inoculation of the infants eyes or respiratory tract

- maternal secretions

2 most common manifestations --

- conjunctivitis

- pneumonia with delayed onset (RLL)

Risk for developing conjunctivitis/pneumonia --

25-50%

5-20%

Prevention --

- screen and treat during pregnancy

- re screen and re treat high risk women in third trimester

Do NOT treat with PO erythromycin as prophylaxis in asymptomatic infant born to chlamydia positive mother --

- increase risk of hypertrophic pyloric stenosis

Gonorrhea

When do they contract it --

During birth, most infected women are asymptomatic

Maternal infection increases risk for --

- spontaneous abortion

- PROM

- premature delivery

- chorioamnionitis

- can result in perinatal distress, neonatal sepsis, arthritis, meningitis, scalp abscess, and ophthalmia

Treatment for infants w/ mother active gonorrhea -- Ceftriaxone 125 mg IV / IM x1

Why are all infants given eye prophylaxis at birth?

Because Gonorrhea conjunctival infection rapidly causes blindness.

GBS - Group B Strep

Early onset --

- 1st 24 hours of life

- 50% are symptomatic at birth indicating intrauterine infection

Late onset --

infection during 2nd week of life

How is it contracted --

- vertical transmission is the passage of a disease from mother to baby during the period immediately before and after birth

Highest rates of GBS --

- in high risk deliveries, premature infants, low birth weight, and prolonged PROM

Colonization of pregnant women and newborns with GBS --

15-40%

Chemoprophylaxis has decreased transmission rates from --

4/1,000 - 0.3/1,000

Clinical findings --

- < 37 weeks

- ROM > 18 hrs

- maternal fever during delivery > 100.4

- previous delivery with GBS +

- maternal chorio, including ROM and maternal fever plus 2 of the following::

- maternal HR > 90

- fetal HR > 170

- maternal WBC > 15,000

- uterine tenderness

- foul smelling amniotic fluid

Neonatal clinical findings --

- poor feedng

- temp instability

- cyanosis, apnea, tachypnea, grunting, flaring, retracting

- seizures

lethargy

- bulging fontanels

- rapid onset and deterioration

Diagnostic studies --

- CSF culture

- Blood culture

- CBC/ dff

- CRP

- urine culture

Differential diagnosis --

- RDS

- amniotic fluid aspiration syndrome

- persistant fetal circulation

- meningitis

- osteomyelitis

- septic arthritis

- sepsis from other infections

- metabolic problems

Treatment sepsis --

- Ampicillin 50mg/kg/dose IV q12h and

- Gentamicin 2.5 mg/kg/dose IV q12-24 hrs

depending on gestational age

Treatment meningitis --

- Ampicillin 100mg/kg/dose IV q12h and

- Gentamicin 2.5 mg /kg/dose IV q12-24 hrs

depending on gestational age

Why two antibiotics?

- Antibiotic therapy with a penicillin and aminoglycoside (amp and gent) until differentiated between E. coli and Listeria sepsis or meningitis

Prevention --

Screen ALL pregnant women between 35-37 weeks unless they have GBS detected in urine or previous child with GBS then just treat with abx during labor

Treatment for positive GBS culture --

IV penicillin or ampicillin at onset of labor

Repeated q4h until baby is born except in c-section before onset of labor and without rupture of membranes

Women GBS unknown --

Antibiotics if labor starts before 37 weeks, prolonged ROM > 18 hours, fever during labor

Intrapartum abx is the only --

-proven method of preventing early onset GBS infection in newborns

Intrapartum abx will not prevent --

- late stage GBS infection, there has been no proven way to prevent late stage GBS

Vaccine currently being worked on for GBS

Alcohol

Most dangerous substance fetuses are exposed to.

Leads to serious and permanent impairment

Tobacco use

Most common cause of LBW in infants

Drugs in pregnancy

When to consider drug abuse --

- poor nutrition, not gaining weight, malnourished

- track marks, nasal hyperemia, septal defects

- high risk factors like absent prenatal care, family hx, previous child abuse or neglect, poor support system, legal issues, psych problems, nose bleeds, HIV positive

Children of these mothers are at higher risk for --

physical, sexual, and emotional abuse

Obstetric complications --

Abruption of the placenta, unexplained preterm labor, uterine trauma, IUGR, previous poor birth outcome

:::Neurological issues:::

-Cocaine use -- seizures, postpartum intracerebral hemorrhage

- Amphetamine/LSD -- psychosis

- Heroin -- abstinence syndrome, mononeuritis, polyneuritis, transverse myelitis

::: Cardiovascular issues:::

- Cocaine/Amphetamine -- HTN, infarction, cardiomyopathy, arrhythmias, sudden death

- IV drugs -- bacterial endocarditis

:::Infectious issues:::

- IV drugs -- Hep B/C, HIV, cellulitis

- All drugs -- pneumonia, UTI, STIs

:::GI issues:::

- Cocaine, IV -- intestinal infarction, acute and chronic hepatitis

Common maternal complications of drug use --

poor nutrition

How to obtain substance abuse history --

Verbally, with kindness and in a private setting, be non-judgmental, be direct.

Make it clear that this is about the best interest of the child not punishing mom.

Also ask about household drug use

Gather -- Type, dose, route, duration of drug use

Drug screen --

On mothers -- Not without consent -- cannot report drug use to authorities

On neonates -- if presents with unexplained neurological symptoms -- drug test can be done on the infant without parental consent, can use U/A, meconium, hair or cord blood to test for drugs. Meconium is the most accurate and will detect substance abuse in last 1/2 of pregnancy

Contact CPS on maternal drug use?--

yes

Urine toxicology times::

- ETOH -- hours

- amphetamines/cocaine -- 1-3 days

- opiates -- 2-4 days

- LSD -- 2-3 days

- marijuana -- 7-30 days

To identify infant exposure use either maternal or fetal urine

Negative tests for narcotics --

infant will test negative by the time it develops symptoms of withdrawal

Screening tools to assess maternal substance abuse --

Ewings 4 p's (Parents, Partners, have any drug problems in the Past and Present)

CRAFFT substance abuse screen for adolescents and young adults

What should be done prior to accusing Mom of drugs exposure?

A through review of her medical records looking for ordered narcotics and sedatives

How do you detect drug abuse during the second half of pregnancy?

Meconium and hair screenings, collect meconium within 1st 48 hours of life

Crack/Cocaine

local anesthetic and CNS stimulant

Withdrawal in neonate --

none

Pregnancy --

teratogen that crosses the placenta

S/S of abuse during pregnancy --

premature labor, placental abruption, fetal asphyxia

Clinical findings in neonates --

LBW, IUGR, prematurity, fetal distress, mec staining, microcephaly, anomalies of urinary and GI tracts, feeding issues, irritability, abnormal sleep patterns, hypertonia

Neurobehavioral findings --

present 2-3 days after birth

Treat --

Suck, swaddle, side or stomach, swing and shush

Breastfeeding --

no

If tests positive consider --

testing for STIs

Long term effects --

Change in IQ or behavior

Neurobehavioral dysfuncton

Hyperactivity

Aggression

Short term attention span issues

What are some naturally occurring narcotics?

Codeine, morphine, opium

What are semi/ synthetic narcotics?

fentanyl, heroine, hydromorphone, dilaudid, methadone, demerol, oxycodone, percocet, darvon

What qualifies as narcotics dependence?

Tolerance to narcotics or any type with symptoms of withdrawal upon discontinuation

Heroin withdrawal

occurs in 75% of infants

Methadone withdrawal symptoms occur in what percentage of infants?

90%

What should be considered instead of heroin or methadone?

Suboxone

Narcotic effects on the fetus includes?

Increased rates of spontaneous abortion, stillbirth, IUGR

NAS - Neonatal abstinence syndrome

Symptoms --

High pitched cry, tremulousness, sleeplessness, difficult feedings, sweating, nasal stuffiness, sneezing, vomiting, cramping, diarrhea, seizures, EEG abnormalities, CNS S/S, HYPOTINIC, IUGR, lethargic, temperature fluctuations

Symptoms and gestational age --

less pronounced in pre term infants due to immature CNS system

Differential diagnosis --

sepsis, hypoglycemia, CNS hemorrhage, infection, CNS disorder

Onset --

usually devleops within 48 hours of life

96% by 4 days

can depend on half life of drugs and when mother had last dose of drug ( 48-72 hours for most, can be 28 days)

Can be delayed up to 4 weeks

Symptoms last --

4-6 months after birth

Maternal screening --

HIV, Hep B/C, polydrug abuse

Supportive treatment --

Frequent small feedings, Suck, swaddle, shush, swing, side, stomach, IV fluids electrolyte replacement, High cal food 150-250 cal/kg/day

What initiates supportive care measures --

Finnegan >8 on 3 consecutive scores or a score >24, dehydration, weight loss, seizures, and poor feeding

Initiate treatment when --

Three Finnegan scores are >8 or a total of the 3 consecutive scores is 24+.

Pharm treatment benefits --

Short term amelioration of the clinical signs

Pharm treatment risks --

Prolonged exposure to drugs

prolonged time in the hospital

Impaired maternal bonding

Reinforces idea that annoying behaviors should be treated with drugs

Clinical indications for pharm treatment --

Seizure, poor feeding, diarrhea, vomiting, weight loss, dehydration, inability to sleep, and fever unrelated to infection

Types of seizures associated with NAS withdrawal --

Myoclonic, they typically respond to opiates and carry no increased risk of poor outcome.

Facial dysmorphia findings --

Underdeveloped philtrum

Thin Upper lip

Flat mid face

short upturned nose, low nasal bridge

ear anamolies

short palpebral fissures

ptosis

Micrognathia

epicanthal folds

Railroad track ears

Which withdrawal begins later heroin or methadone?

Methadone due to the longer half life of the drug, although both typically begin before 48 hours of life

Methadone

mom's daily intake should be reduced to --

<20 mg/kg daily

some OB's dont reduce because --

have concerns that mom may turn to street drugs or other illicit drugs

some providers increase methadone because --

mom will have lower maternal plasma levels of the drug

Modified Finnegan Score sheet

How neonatal abstinence syndrome is scored

Perform by --

Assess infant at 2 hours then q4h after birth

If score is > 8 change to q2h assessment until 24 hours after last score of >8 then go back to q4h

Change sheets daily

What is the Lipsitz Tool?

A simple numeric NAS scoring system, scores >4 indicate withdrawal

What is the OSTREA system?

1 6 criteria scoring system, limited because you can't summarize the severity of the scores

Withdrawal from what can be life threatening?

Hypnotic drugs or narcotics

An infant is born to a mother with confirmed polysubstance abUse, the Finnegan score has not exceeded 6. How is infant treated for NAS?

This infant is asymptomatic and does not require treatment for NAS.

What is the only drug in the US approved for treatment of ETOH withdrawal?

Benzo's

What is the only drug in the US that is approved for treatment of opioid withdrawal?

Methadone

What si the 1st line drug of choice for treating opioid withdrawal?

Morphine

What is the backup drug they used to add to morphine for withdrawal for opiates?

Phenobarbital

What is the morphine dose for NAS?

0.24-1.3 mg/kg/day

Decreasing NAS pahrma treatment is decided by?

Decreasing scores on Finnegan Scale

SSRI

Examples --

prozac, celexa, lexapro, zoloft, luvox,

Third trimester use leads to neonatal --

excessive crying, irritability, jitteriness, shivering, restlessness, feeding difficulties, hypoglycemia and seizures.

Withdrawal symptoms start --

several hours to several days after birth and they can last 1-2 weeks

Adverse neonatal outcome --

no adverse neurodevelopment outcomes

Recommendation in pregnancy --

continue during pregnancy at lowest possible dose as withdrawal can have harmful effects on mother baby dyad

Fetal Alcohol Spectrum disorders include

FAS/ Partial FAS

Alcohol related neurodevelopemental disorders

alcohol related birth defects

When to stop ETOH --

when decide to try to get pregnant

How much ETOH is too much --

no known dose relationship response to alcohol and FAS

Maternal FAS risk factors --

advanced maternal age

increased parity

poor maternal nutrition

african american or native descent

binge drinking

FAS statistics --

0.3-1.9 per 1,000 children worldwide

Alcohol effects on fetus --

IUGR

Increased rates of malformation

Chronic fetal hypoxia

Percentage of fetal anomalies related to alcohol --

5%

Clinical findings --

IUGR post natal growth restriction

CNS issues

Microcephaly

delays, retardation

poor motor control

attention deficits

hyperactivity

muscle weakness

Facial dysmorphia --

Underdeveloped philtrum

Thin Upper lip

Flat mid face

short upturned nose, low nasal bridge

ear anamolies

short palpebral fissures

ptosis

Micrognathia

epicanthal folds

Railroad track ears

Diagnostic criteria FAS --

Confirmed fetal ETOH exposure

Characteristic facial anomalies

Evidence of growth retardation

Evidence of CNS neurodevelopment abnormalities

Ocular effects --

strabismus, retinal vascular abnormalities, refractive problems secondary to small globes

Auditory effects --

Conductive hearing loss, neurosensory hearing loss

Cardiac --

ASD, VSD, aberrant great vessels, ToF

Renal --

Urethral duplications, horseshoe kidneys, aplastic, dysplastic and hypoplastic kidneys, hydronephrosis

Skeletal complications --

Hypoplastic nails, short 5th digit, flexion contractures, scoliosis, pectus excavatum

Differential diagnosis --

chromosomal abnormality, maternal PKU defects, fragile X, turner syndrome

Prevention --

abstinence of alcohol

Trisomy 21 / Down Syndrome

Three types --

Trisonomy nondisjunction, translocation and mosaicism

How many people --

400,000 or 1 in 691 babies

Life expectancy --

60 years

80% born to mothers --

under 35 years of age

Physical characteristics --

protruding tongue, simian crease, brushfield spots, epicanthal folds

Screening levels -

AFP- Decreased

UE3- Decreased

hcg- Elevated

DIA- Elevated

Screenings --

1st trimester nuchal translucency test

2nd trimester QUAD screen

Screening recommendations --

Blood Karotyping

Cardiac echo

Screen for mitral valve prolapse at adolescents

Hearing @ birth and 6 mos

Eyes screenign @ 4, 12, 24 mos and 2 yrs and then prn

Thyroid TSH as newborn, 6, 12 mos and annually

Cervical spine eval at 3 and 5 yrs

Growth chart --

down syndrome growth chart

Hip dysplasia --

screen annually through 10 years

Common adult conditions --

Atlantoaxial subluxation

hypothyroidism

early dementia

leukemia

epilepsy

spinal stenosis

cataracts and hearing loss

Differential diagnosis --

Turner syndrome (no X)

Klinefelter Syndrome (Extra X, XXY)

Leukemia chances --

10-20%

Hypothyroidism screening --

6 mos, 12 mos and annually from 1-18 years

SIDS?

Sudden unexplained death before 1 year of age, in a previously healthy infant, remains unexplained despite autopsy and crime scene investigation

3rd leading cause of infancy, 2300 deaths annually

Prevention --

A safe sleep environment, back to sleep, room share, firm bed, nothing in crib, oscillating fan

Rates --

have dropped by > 50% since 1993

Risk factors --

prone and side sleeping

soft bedding

tobacco exposure

prematurity

Protective factors --

breast feeding, pacifier use, room sharing, immunizations

Pathophysiology --

Respiratory center signals the sub cortex which sends wake up signals to the cortex. The cortex can fail to respond when an infant overheats, is affected by drugs and smoking, or sleeping prone, or on their side.

Cardiac issues associated with it --

Long QT often caused by overheating

Racial disparities --

non hispanic black and alaskan nativ es have much higher mortality rate

What contributes to the rates --

Unsafe sleeping environments propagated by the media in TV, sales, everywhere...

Another common SIDS like cause of death is?

ASSB accidental suffocation and strangulation in bed, rates of this have quadrupled in recent years

How many genes are associated with human development?

22 autosomes (same in boy/ girl) and the 23rd pair is sex chromosomes

What protects individuals from misuse of genetic information?

2008 passage of GINA (Genetic information Nondiscrimintaion Act) Does not apply to protection in regards to life insurance or disability, only protects for health insurance and employment.

Family history risk factors for genetic disorders include?

Mental retardation, metabolic disorders, delayed puberty, sensory deficits, progressive disorders

Family ethnic backgrounds including Northern *European* descent have increased likelihood of?

Cystic Fibrosis, PKU

Jewish ashkenazi descent predisposes what genetic disorders?

Tay-Sachs, Canavan, Gaucher

West african descent predisposes to what genetic disorders?

Sickle cell, sickle cell hemoglobin C

Mediterranean descent predisposes to?

bet thalassemia, sickle cell

French Canadian heritage predisposes on to?

Tay-Sachs, branched chain ketoaciduria

What do you need to have a referral for genetic testing for malformations?

2 major malformations

1 major & 1 minor

>3 minor malformations

What newborn malformations dysmorphic features warrant further evaluation?

short stature, hypertonia, missing vessels in umbilicus, low set ears

All states require the following for metabolic newborn screening?

PKU, congenital hypothyroidism, galacotsemia, sickle cell, nearly all states require CF

How and when do you do metabolic newborn screening?

Heel blood sample, sent to lab takes 1 week, preform as close to hospital discharge as you can, if done before 24 hrs, re-screen within 1-2 weeks

What 10 disorders are included in the RUSP recommended Uniform Screening Panel?

hypothyroidism

adrenal hyperplasia

hemoglobinopathies

CCHD

CF

Galactosemia

hearing loss

Severe combined immunodeficiencies

PKU

maple syrup urine disease

What metabolic screening results require immediate attention?

Galactosemia and maple syrup urine disease

What is autosomal recessive mean?

BOTH parents carry the defected gene, 50% chance of carying gene, 25% of being affected, 25% chance of not getting it

What is an example of an autosomal recessive gene?

CF

What is dormant mean in response to genes?

Only one parent needs to pass the gene to offspring and they have a 50/50 chance

Examples of dormant gene diseases include?

Huntington, Polycystic kidney disease

In regards to X-linked disorders what is relevant?

These only occur in males because females have no X chromosomes

Xlinked diseases:

Hemophilia A, Duchene MD, G6PD

How are X-linked genes passed down?

Never father to son, always female carrier passes to son, all daughters of affected males are carriers

Klinefelter syndrome presentation includes:

Long limbs, decreased testicular size, small penis, gynecomastia

PKU

Type of disorder --

Autosomal recessive, inability to break down amino acids phenylalanine in protein

Treatment --

No phenylalanine for life or under < 15/20 mg/dl

S/S --

musty urine, fair skin, blue eyes, hypo pigmentation, feeding troubles, irritability and eczema

Galactosemia

Type of disorder --

Autosomal recessive, deficiency in galactose enzyme, inability to digest milk sugar, can't breakdown lactose

S/S --

vomiting, diarrhea, age 3 months cataracts, lethargy

Restrictions --

lactose lifelong restriction, cannot be breast fed -- do not breastfeed

put baby on soy formula

if tests positive -- immediately treat it can be life threatening

Maple syrup urine disorder

Inherited gene defect r/t a deficiency in branched chain ketoacid decarboxyylase enzyme affecting , metabolism of amino acids

Treatment --

Peritoneal dialysis

Hemodialysis to clear accumulated acids

Discontinue protein intake

CF - Cystic Fibrosis

Autosomal recessive, altered protein synthesis in the transport of chloride ions

S/S --

meconium ileus

poor nutrition

pulmonary dysfunction

increased susceptibility to infections

Treatment --

Lung transplant, replacement enzymes, abx, fat soluable vitamins, pre-digested formula

Education --

Infant needs to pass meconium by 48 hours

Sweat testing can be done at 2 weeks old

Sickle cell disease

Inherited sickling gene

S/S --

Splenomegaly, massive pooling of blood in the spleen can lead to vascular collapse, anemia, hypotension

Hand foot syndrome painful swelling

Frequent life threatening infections

Treatment --

restoration of intravascular volume

RBC transfusion

splenolectomy

prophylactic ABX

Pneomococcal immunization

Education --

watch out for infections, splenic sequestration crisis, and cardiovascular accident

Congenital hypothyroidsism

Autosomal recessive, inability to produce adequate amounts of thyroid hormone, thyroid agenesis

S/S--

asymptomatic macroglossia, umbilical hernia, developmental delays, mental retardation

elevated TSH, low serum T4 or free T4

Treatment --

T4 replacement therapy (levothyroxine)