Board Exam Flashcards
Reasons for high alpha-fetoprotein
RAIN: Renal (nephrosis, renal agenesis, polycystic kidney disease), Abdominal wal defects, Increased number of fetuses/Incorrect dates, Neuro (anencephaly and spina bifida)
Reasons for low alpha-fetoprotein
Trisomy 21 or Trisomy 18
What is a non-stress test and what does it measure?
Measures spontaneous fetal movements and heart rate; therefore, it measures fetal autonomic nervous system integrity
What is the contraction stress test and what does it measure?
Measures the fetal heart rate in response to uterine contraction; measures uteroplacental insufficiency and tolerance of labor
Most common cause of fetal bradycardia
Heart block, which may be seen with maternal lupus
When do you treat SVT in a fetus
With heart rates >240 bpm; treat with antiarrhythmic medication for the mother
What are the components of the biophysical profile?
Fetal movement, reactive heart rate, breathing, tone, volume of amniotic fluid
Causes of apnea in a neonate
APNEA: Abnormal metabolism (hypoglycemia, hypocalcermia, anemia, maternal medications); PDA and other cardiac causes; Neurological (seizures, intracranial hemorrhage, apnea of prematurity); Epidemiological/Infectious (sepsis, pertussis, RSV and other respiratory infections); Abnormal swallowing/GERD
Chest XR findings in RDS
Granular opacifications, air bronchograms, ground glass appearance. Typically symmetric appearance
One way to distinguish RDS from pneumonia secondary to GBS
Calculate the ratio of bands to total neutrophils; if greater than 0.2, sepsis or pneumonia is more likely. Also, temperature instability is a clue towards pneumonia
Interaction between bilirubin and RDS
When hyperbilirubinemia co-exists with RDS, the threshold for kernicterus is lowered
Factors that increase and decrease the risk of RDS
Increase: infants of diabetic mothers, c-section delivery, birth asphyxia; decrease: prolonged rupture of membranes, prenatally administered steroids
Changes expected after surfactant administration in RDS
Decreased oxygen requirement, reduced inspiratory pressure, improved lung compliance
Definition of chronic lung disease
Present in infants who still have an oxygen requirement 28 days after birth and/or continued oxygen requirement at 36 weeks corrected gestation
Typical CXR of BPD
Diffuse opacities as well as cystic areas with streaky infiltrates and ground glass appearance
How long should you monitor well appearing infants whose mothers had inadequately treated GBS?
48 hours
Drug of choice for treatment of proven GBS
Pencillin
Delayed detachment of the umbilical cord
Leukocyte adhesion deficiency or low WBC
Proper cord care
Wash with soap and water (Do not apply mercurochrome–can cause harm! and cases mercury)
What test should you do when you see a single umbilical artery?
Renal ultrasound
Should the obstetrician milk the cord?
No, it can lead to polycythemia
Physical findings in post-term babies
Dry skin that is peeling, long fingernails, and decreased lanugo on the back, the ears will have a strong recoil
Is bilateral ankle clonus normal in an infant?
Yes
CXR in meconium aspiration syndrome
Patchy areas of atelectasis alternating with areas of hyperinflation
Contraindications to phototherapy
Elevated direct bilirubin (can cause “Bronze Baby Syndrome”) or a family history of light-sensitive porphyria
Causes of indirect hyperbilirubinemia
YELLOW:
You never know: Gilbert Disease
Endocrine (hypothyroid-hypopituitarism)
Enterohepatic circulation increased (obstruction, pylori stenosis, meconium ileus, ileus, Hirshsprung’s)
Lucy Driscoll Syndrome
Lysed blood cells (hemolytic disease, defects of RBC metabolism, isoimmunization)
Overdrive (Galactosemia, tyrosinosis, hypermethionemia-cystic fibrosis)
Wasted blood (petechiae, hematomas, hemorrhages,cephalphematomas, swallowed maternal blood)
Risks in LGA infants
Hypoglycemia, polycythemia (possibly due to increased erythropoietin), hypoplastic left colon syndrome
When do full and preterm infants have their hemoglobin nadir?
Full term reach their nadir at 2-3 months (can go as low as 9-11), preemies reach it in 1-2 months (can go as low as 7-8)
What test is used to detect the presence of fetal cells in the mother’s blood and is used to evaluate neonatal anemia?
Kleihauer Betke Test
What Hgb is considered anemic at birth?
Less than 13
What is polycythemia and when and how should it be treated?
Central venous (not capillary) hematocrit of 65 or higher. Treat for 70 or higher with partial exchange transfusion. Polycythemia can lead to hypoglycemia, hyperbilirubinemia, and/or thrombocytopenia.
Lethargy, hypotonia, and irritability in an infant with a history of TTTS, delayed clamping of the cord, Down syndrome, or IDM
Hyperviscosity syndrome from polycythemia
What is the Apt Test?
Test if blood in the neonate’s gastric aspirate is actually maternal blood
What test should be performed in eery baby with omphalocele?
Echocardiogram
Treatment for neonatal seizures
Phenobarbital
Grading of IVH
Grade 1- Germinal matrix
Grade 2- IVH without dilation
Grade 3- IVH with dilation
Grade 4- Parenchymal involvement
What to watch for if mother is given terbutaline as tocolysis during labor?
Hypoglycemia (terbutaline stimulates fetal insulin)
Baby withdrawal symptoms if mother was using alcohol
Hyperactivity, irritability. May also see hypoglycemia
Baby withdrawal symptoms if mother was using cocaine
No official withdrawal or abstinence syndrome exists. But are at increased risk for cerebral infarctions, limb anoalies, and urogenital defects.
Baby withdrawal symptoms if mother was using marijuana
None
Baby withdrawal symptoms if mother was using amphetamine
None. Though are often irritable and easily agitated with routine environmental stimulation. Often experience IUGR, and are prone to developmental and cognitive impairment.
Baby withdrawal symptoms if mother was using barbituates
hyperactivity, hyperphagia, irritability, crying and poor suck swallow coordination
Baby withdrawal symptoms if mother was using opioids
Hyperirritability, tremors, jitteriness, hypertonia, loose stools, emesis, and feeding difficulties. Can also have seizures. May need methadone and/or oral morphine to manage withdrawal
Sequence of male pubertal development
Testicular growth–> pubarche–> penile growth –> peak height velocity
What testicular length indicates the onset of puberty
Greater than 2.5 cm
What should you think about if there is pubic hair development and penis enlargement in the absence of testicular enlargement
Androgen stimulation from outside the gonadal area
Sequence of female pubertal development
Breast budding–> pubarche –> peak height velocity –> menarche
What is considered delayed puberty?
No pubertal signs by age 14 in boys and age 13 in girls
Treatment of delayed puberty
Should be evaluated by endocrinologist. Monthly IM injections of testosterone for boys and oral estrogen for girls
What is premature adrenarche?
The presence of androgenic sexual characteristics (axillary hair, pubic hair, acne, and/or adult body odor) without estrogenic sexual characteristics (breast development and menarche) and without the growth spurt of puberty
Lab findings in premature adrenarche
Elevated serum DHEA and DHEA-S, Low testosterone
Causes of premature adrenarche
exogenous androgen, endogenous androgen-secreting tumor, late-onset CAH, polycystic ovary syndrome
What is the bone age in premature adrenarche or premature thelarche
Normal (no more than one year advanced)
What is central precocious puberty
Development of secondary sexual characteristics accompanied by acceleration in linear growth or advanced bone age. Precocious if starts before 9 in boys or before 8 in girls
What is the consequence of central precocious puberty?
Short adult height
What should you think of with precocious puberty and cafe au lait spots?
McCune Albright Syndrome.
Other signs include Cushing syndrome in infants
Treatment of precocious puberty
Leuprolide (GnRH agonist)
What syndromes can have panhypopituitarism?
Prader-Willi, Kallmann, and septooptic dysplasia
Genetics of androgen insensitivity syndrome?
X-linked
Why do patients with androgen insensitivity syndrome have a vagina ending in a blind pouch?
Because Mullerian inhibiting factor is still produced by the Y chromosome, so no uterus or ovaries develop
What should you think of if a newborn male has excessive scrotal pigmentation?
Congenital adrenal hyperplasia
What levels are high in 21-hydroxylase deficiency?
17-hydroxyprogesterone
What are the two main hormones produced by the adrenal glands and what do they do?
Aldosterone, which helps control the amount of salt and fluid in the body, and cortisol, which helps control how the body uses sugar and how it responds to stress
How do you treat adrenal crisis?
20 mL/kg of D5NS (with NO potassium) IV over one hour. Then add IV hydrocortisone. Then add glucocorticoid replacement.
What is the cosyntropin stimulation test (ACTH stimulation test)?
Used to test the adrenals.
With primary adrenal deficiency–> no rise in cortisol levels (b/c adrenals are broken and can’t respond)
With secondary disease–> the adrenal usually have normal cortisol stores and cortisol will be released during the test
What is the difference between Cushing syndrome and Cushing disease?
Cushing syndrome refers to glucocorticoid excess of any origin. Cushing disease speciically refers to the excessive production of corticotropin by the pituitary gland, which leads to excess cortisol production by the adrenal gland
How does Cushing syndrome present in children?
Increased BMI with growth arrest. Will also have delayed bone age
Can also see classic acne, purple striae, hirsuitism, virilization, and buffalo hump.
Labs to test for Cushing syndrome?
24 hour urine free cortisol excretion, midnight sleeping plasma cortisol level (greatest sensitivity of all tests for CS in children), dexamethasone suppression (should lead to undetectable plasma cortisol levels after dexamethasone administration
What two hormones is the posterior pituitary responsible for?
ADH and oxytocin
What presents as diabetes insipidus, exophthalmos, and lytic bone lesions?
Langerhans cell histiocytosis
Bone age in children with growth delay due to hypothyroidism
Delayed
Clues to Marfan syndrome in a patient with tall stature
Disproportionately long arms (arm span to height ratio >1.05) and significantly long tapering fingers described as arachnodactyly, or spider fingers
What is the most common preventable cause of intellectual disability worldwide?
Hypothyroidism
How should babies take levothyroxine?
Crushed and mixed in formula, human milk, or water, but NOT with soy formulas because it reduces absorption
How is thyroxine binding globulin deficiency inherited?
X-linked
Antibodies seen in Hashimoto’s
Anti-thyroglobulin and anti-thyroid peroxidase antibodies
What antibodies are seen in Graves disease?
Thyroid-stimulating immunoglobulin
Signs and symptoms of Graves disease?
Bulging eyes, emotional lability, weight loss, sleep disturbance, and/or heat intolerance, lid lag, increased appetite with weight loss, decreased muscle strength or endurance, itching, tremors, sweating, increased urination at night, decreased menstrual flow and/or decreased frequency of menses
First line agent for Graves and how does it work
Methimazole, blocks the organification of iodide and so decreases thyroid hormone synthesis
Symptoms of neonatal thyrotoxicosis
Irritability, tremors, and tachycardia, failure to thrive, feeding problems, and hyperbilirubinemia
What causes neonatal thyrotoxicosis?
Exposure to maternal thyroid stimulating antibodies crossing the placenta. Be careful that mom may be on synthroid from having thyroid ablated, but still has circulating antibodies
How to correct sodium for elevated glucose
Corrected (actual) Na= measured Na +0.3(glucose-5.5)
What should you think about if sodium does not increase as glucose falls during treatment for DKA?
Overzealous fluid correction. This represents as increased risk for cerebral edema. Important if there is a patient with mental status change during DKA correction
When do you put dextrose in the fluids during DKA correction?
After glucose drops below 300
Definition of metabolic syndrome
- Hyperinsulinemia or insulin resistance
- Dyslipidemia
- Hypertension
- Obesity, particularly central adiposity
Treatment of metabolic syndrome
Weight loss, regular exercise. NO recommendation for the use of metformin
Definition of hypercalcemia
Serum calcium greater than 11 mg/dL
Causes of hypercalcemia
WISH:
Williams syndrome
Ingestion (vitamin D and A intoxicaton, thiazide diuretics), Immobilization
Skeletal disorders (dysplasias and immobilization/body casts)
Hyperparathyroidism
Treatment of hypercalcemia
High volume fluid, lasix, and EKG monitoring. In rare cases calcitonin is used.
Presentation of hypocalcemia
Painful muscle spasms, generalized seizures (can be resistant to diazepam), vomiting, prolonged QT interval on EKA, Chvostek and Trousseau signs, hypomagnesemia
Causes of hypocalcemia
PINK:
Pseudohypoparathyroidism (peripheral tissue resistant to the effects of PTH)
Intake (nutritional deficiency), Immune deficiency (DiGeorge syndrome)
Nephrotic syndrome (with a lowered albumin level, there is a lower calcium level)
Kidney (renal insufficiency results in higher phosphate, lower calcium, and a secondary hypoparathyroidism
What should you think of if you see hypocalcemia and hypophosphatemia?
Vitamin D deficiency
What do you see on XR of patients with longstanding hypoparathyroidism?
Short 4th and 5th metacarpals and metatarsals and calcification of the basal ganglia
When might calcium be needed emergently?
Hypocalcemia, hyperkalemia, hypermagnesemia, calcium channel blocker ingestion
What is rickets?
The deficient mineralization of bone at the growth plate. Thus, it cannot occur after the growth plates are closed.
What is the alk phos level in rickets?
Elevated in all forms of rickets
Signs and symptoms of rickets?
Bone pain, anorexia, decreased growth rate, widening of the wrist and knees, delayed eruption of teeth, bowed legs, enlarged costochondral junctions (“rachitic rosary”), and softening of the skull bones (craniotabes)
Causes of calcipenic rickets?
Vitamin D deficient rickets, vitamin D dependent rickets, hereditary vitamin D resistant rickets
Why might you see rickets in chronic liver disease?
Usually a result of reduced availability of bile salts in the gut and subsequent decreased absorption of vitamin D
What causes vitamin D dependent rickets?
Inadequate renal production of 1,25 dihydroxy vitamin D. (Autosomal recessive disorder). Also known as pseudo-vitamin D resistant rickets
Treatment of vitamin D dependent rickets?
Vitamin D2 and 1,25-dihydroxy vitamin D
What is hereditary vitamin D resistant rickets?
Autosomal recessive hereditary condition due to end organ resistance to vitamin D
How do you treat wound botulism?
Antitoxin, then penicillin or metronidazole
What are the non-treponemal tests for syphillis?
RPR and VDRL
When do you treat the infant for syphillis if the other had syphillis?
If the mother was treated within the past month; if she was treated with erythromycin (does not cross the placenta); or if the baby’s tigers are higher than the mothers
Treatment of corynebacterium diphtheria
Antitoxin and antibiotics (penicillin or erythromycin)
What should you think of in a baby with positive reducing substances in the urine?
Galactosemia
