Newborn infant_pre-test

Question 1

Congenital syphillis presentation

Answer 1

Varied. Many newborns will appear asymptomatic for first few weeks/months. But most untreated infants will develop skin lesions (infiltrative, maculopapular skin peeling rash on face, palms and soles), rhinitis with serous, purulent blood tingied discharge. Other common findings are hepatosplenomegaly, jaundice due to syphilitic hepatitis. LFTs are elevated. Saddle nose due to destrction of bone from syphilitc rhinits. Later manifestation, interstitial keratitis.

Question 2

Later congenital syphilis presentation

Answer 2

Intersitital keratitis begins in early childhood (6-14 yo), photophobia, corneal haziness, eventual scarring, lacrimation. Hutichison teeth, abnormal enamel, mulberry molars.

Question 3

Congenital rubella

Answer 3

Small/IUGR. Affects all organ systems. deafness, Cataracts, micropthalmia, myocarditis, red/purple macular rash (blueberry muffin rash), structural heart defects (PDA, PA stenosis). Labs may show hemolytic anemia with thrombocytopenia, elevated liver functions, pleocytosis in spinal fluid.affected children have poor prognosis.

Question 4

How do you get congenital HSV

Answer 4

Can happen in utero, intrapartum and postnatally. Infants born vaginally to mom with primary genital herpes infection has highest risk for disease with up to 50% chance of perinatal infection.

Question 5

Ppt of congenital HSV

Answer 5

Isolated CNS involvement, isolated cutaneous involvement, or systemic generalized infection. Tx is with acyclovir and even with therapy, morbitidy is high in infants with CNS invovlemnt.

Question 6

Classic triad for toxoplasmosis

Answer 6

hydrocephalus, chorioretinitis and intracranial calcification. May also display sx similar to toher congenital infections - anemia, petechial rash, organomegaly, jaundice and seizures..

Question 7

Cytomegalic incluion disease findings?

Answer 7

Hepatomegaly, splenomegaly, jaundice, petechiae, purpura and microcephaly, chorioretinitis and intracranial calcifications. More than half present with senorineural hearing loss.

Question 8

What is the most common cause of congenital infection

Answer 8

CMV infection. Symptomatic congenital disease usually occurs if mom has primary cmv infection during first trimester.

Question 9

CMV excretion can least for

Answer 9

Months to years

Question 10

Layers of skull starting from the skin to the meninges

Answer 10

Skin, connective tissue, aponeurotic layer, connective tissue, periosteum, bone, dura, subararachnoid space, leptomeninges

Question 11

Caput succednaeum, cephalohematoma, subgaleal hemorrhage, subdural hematoma. Describe them (where in the layers do they occur, do they cross midline etc)

Answer 11

Caput succadaneum: soft boggy mass. Right under skin, involves delivery portion of head. Lesion is sometimes ecchymotic and an extend across suture lines. Edema may be pitting and resolves within first few days of life. Cephalohematoma: sub-periosteal and do not cross suture lines. no discoloration fo scap seen. swelling progrsses over first few hours. most resolve within first few weeks/month. Subgaleal/subapnoeurotic hemorrhage will feel like cephalothematoma and crosses midline. can be life threatining if infants lose a lot of blood into this space and can become hypovelemic. subdural hematoma common seen as part of abuse head trauma. occurs when bridge cortical veings that drain the cerebral cortex have been ruptures leading to collection of blood between the dura and cerebral mantle.

Question 12

What is Alport syndrome

Answer 12

Most common of the hereditary nephritis conditions, frequently ends in ESRD. X linked dominant form of inheritance in 85% of causes, the rest are A/R. all cause hematuria and progressive nephritis. Other findings, deafness, ocular defects.

Question 13

CHARGE syndrome

Answer 13

Coloboma, heart disese, atresia of the choanae, retarded growth and development, genital hypoplasia and ear abnormalities

Question 14

In what circumstances should an infant get varicella IG?

Answer 14

if mom had onset within 5 days prior to delivery and dx within 2 days post delivery. IVIG not necessary if baby is older than 2 days old because they are not at increased risk compared to older children.

Question 15

What is the Apt Downey test

Answer 15

Differentiates maternal from fetal hemoglobin in a bloody specimen. Fetal Hg is alkali resistant whreas adult will convert to hematin unpon exposure to alkai.

Question 16

Drugs contraindicated in breastfeeding

Answer 16

Cyclosporin, lithium antineoplastic, illicit durgs like heroin and cocaine, AMPHETAMINES, ergotamine (used for acute migrain attacks, has biologic role of causing vasoconstriction)

Question 17

Metabolic patterns in infants born to diabetic moms, in particular glucose, calcium and magnesium?

Answer 17

Hypoglycemic in first 24 hours. In utero exposure to high glucose from mom leads to fetal islet cell hypertrophy, leading to increased insulin production. Abrupt cut off of glucose after birth and high insulin leads to hypoglycemia. Between 48 hours and 96 hours => acheive glycemic control but may now develop hypocalcemia and hypomagnesemia. Hypocalcemia due to delayed PTH syntehsis or responsiveness.

Question 18

Signs and symptoms of hypocalcemia

Answer 18

Irritability, sweating, tremors, twitches, seizures and arrhythmias.

Question 19

Evidence of hemolysis in newborns

Answer 19

Even if HCT is normal, nucleated RBCs, elevated retic count, microspherocytes in blood smear is evidence

Question 20

What is pneumtosis intestinalis. Found in what condition

Answer 20

Distended loops of bowel with air in bowel wall. NEC

Question 21

How should pneumatosis intestinalis be managed?

Answer 21

Check for free peritonieal air. If none, start systemic abx, monitor lytes and VS closely, perform serial abdominal firlms to evaluate for perforation.

Question 22

What is an apneic episode

Answer 22

absence of respirations for 20 seconds, may be accompanied by cyanosis and bradycardia. In otherwise well appearing premature infant, this is due to incompletely developed respiratory center.

Question 23

Most commonly fractured bone during delivery

Answer 23

Clavicle

Question 24

Presentation of broken clavicle

Answer 24

Pseudoparalysis mimicking erb duchene paralysis

Question 25

What nerves are effected in Erb Duchene and Klump paralsis

Answer 25

1) 5th and 6th cervical nerves 2) 7th and 8th and first thoracic nerve (can produce horner syndrome)

Question 26

Sub galeal hemorrhage aka

Answer 26

Subaponeurotic hemorrhage. Below the aponeurotic layer and above periosteum. Crosses midline. Infants can lose a third of their blood into this space and cause hypovolemic shock.

Question 27

Venous crit of above 65% in neonate

Answer 27

Polycythemia, accompanied by increase in blood viscosity

Question 28

Manifestations of hyperviscosity syndrome

Answer 28

tremuslousness, jitteriness => seizure due to sludging of blood in cerebral micocirculation or thrombus formation, renal veing thrombois, NEC, tachypnea. Better therapy is partial exhange transfusion with saline or LR not with phlebotomy

Question 29

What is the mgmt of healthy asymptomatic infants born to HIV+ moms. What tests do they need, if any

Answer 29

No special monitoring or radiographs needed. HIV Elisa antibody test will be positive anyway due to maternal transfer of antibodies. Confirmatory western blot also not helpful.

Question 30

What vaccine cannot be given before 6 weeks of age.

Answer 30

Hib, DTAP and IPV vaccines

Question 31

Hep A vaccine is not given until what age

Answer 31

12 years

Question 32

What is ECMO stand for

Answer 32

Extracorporeal membraine oxygenation

Question 33

How should infants of mom with Hep BsAg be treated?

Answer 33

Hep B immunogloubine and Hep B vaccine for protection not only from immediate perinatal infection but also infectation that may have been acquired as a result of continued exposure in utero.

Question 34

Is proponalol contraindicated during pregnancy?

Answer 34

yes

Question 35

What effects does proponalol have on fetus?

Answer 35

may cause growth retardation, diminishes ability of an asphyixated infant to increase HR and cardiac output. Also a/w hypoglycemia and apnea.

Question 36

Jaundice that usually appears in first 24 hours is usually a feature of?

Answer 36

Hemolytic states and is accompanied by indirect hyperbilirubinemia, reticulocytosis, and evidence of red cell destruction on smear. If there is no Rh or blood group incompatability considere G6PD deficiency or congenital hemolytic states like spherocytosis should be considered.

Question 37

Midgut volvulus presentation (age)

Answer 37

affected infant at 3-4 days old, bilious vomitus. Obstructs bowel in duodenojejunal junction.

Question 38

Goat’s milk is inadequate as infant nutrition because?

Answer 38

inadequate iron and folate, can contribute to anemia.

Question 39

Are siblings of person born with cleft palate and lift have greater change of being affected?

Answer 39

yes. Occurs in 4% of siblings of affected infants. Usual occurrence is 1:1000. evaluate for other structural and chromosomal abnormalities.

Question 40

Complications of cleft lip and palate

Answer 40

Recurrent otitis media and hearing loss as well as speech defects despite good anatomic closure. Repair of cleft lip usually within first 2-3 months of life, cleft palate is repaired later between 6 months and 5 years.

Question 41

Transient tachypnea of the newborn is usually due to what?

Answer 41

Condition due to retained fetal lung fluid. Tachypnea, retractions, grunting and sometimes cyanosis. Chest exam is normal, chest radiograph shows incrased pulmonary vascular markings with fluid in fissues and hyperexpansion (flat diaphragms). Therapy is supportive with maintenance of normal oxygen saturation. resolution usually occurs in first 3 days.

Question 42

Hyaline membrane disease aka

Answer 42

Idiopathic repsiratory distress syndrome. “ground glass appearance.”

Question 43

Onset of narcotic withdrawal symptom of infants and sx?

Answer 43

within first 2 days, hyperirritability, coarse trmors, V/D, fever, high pitched cry and hyperventilation. Seizures and respiratory depression are less common.

Question 44

What is Harlequin syndrome.

Answer 44

Transient change of skin color of otherwise asymptomatic newborn

Question 45

Kernig and and Brudzinki signs

Answer 45

Meningitis signs. Kernig is when hip is flexed, patient cannot straighten leg beyond 135 degrees. Brudzinki is when patient is supine, when neck is flexed, neck stiffness causes hip and knees to flex.

Question 46

When twins hemotocrits differ by more than 15mg/dL, what hsould you suspect?

Answer 46

twin to twin transusion. Donor twin likely to have oligohydramnios, anemia and hypovolemia with evidence of shock if HCT is significantly reduced. In recipient twin, polyhdramnios, plethora. If crit goes above 65% hyperviscosity syndrome.

Question 47

Cardiac defects are common in which TORCH infection

Answer 47

Rubella but not in others.

Question 48

Infants with upper brachial plexus injury can also have?

Answer 48

Ipsilateral phrenic nerve paralysis.

Question 49

What is the problem in galactosemia

Answer 49

Deficiency in galactose 1 phosphate uridyl transferase results in accumulation of galatose 1 phosphate in the tissues. Causes severe damage to liver, brian and eyes after eating lactose.

Question 50

PKU inheritance pattern and absence of what?

Answer 50

A/R. absence of enzyme that converts phenylalanine to tyrosine, resulting in accumulation of phenylalanine and its metabolites in the blood leading to severe mental retardation.

Question 51

Maple syrup urine disease tx?

Answer 51

Diet that consists of careful regulation of intaken of leucine, isoleucine and valine

Question 52

Waardenburg syndrome inheritance and ppt

Answer 52

A/D with variable penetrance. Lateral displacement of medial canthi, broad nasal bridge, medial hyperplasia of eyebrows, partial albinism commonly expressed by white forelock or heterochromia (or both) and deafness in 20% of cases.