NEONATOLOGY M Flashcards
How much should TFI be increased by in order to ensure feed safety?
20-30 ml/kg/d –> if calculating this for oral feeds, divide by 8 for q3h feeds
What is the definition of polycythemia?
Hct > 65% or Hgb > 220
Omphalocele gastroschisis What are the features of omphalocele?
Omphalocele: -midline -protective cover (guts herniating through umblical cord) -25-40% have congenital anomalies (Beckwidth Wiedemann, Trisomy 13 & 18)
Omphalocele gastroschisis What are the features of gastroschisis?
Gastroschisis: -not midline (right sided most common) -no protective cover (full thickness abdo wall defect) -10-15% have intestinal atresia
What is the Kleihauer-Betke test?
Tests mom for baby’s blood -bleeding from the fetal into the maternal circulation (fetomaternal hemorrhage) -put mom’s blood into some filter thing and it breaks down mom’s beta chains but not fetus’ gamma chains
What are the causes of apnea in:
-prems -
terms
-both
Prem:
- apnea of prematurity & anemia
- IVH , PDA, RDS
- eye exams
Term: -cerebral infarct -polycythemia
Both:
- infection, hypothermia, NEC
- cardiac -asphyxia - -aspiration -seizure
Differential diagnosis for neonatal hematuria?
- Vascular: renal artery or venous thrombosis (common in infants of diabetic moms) -both can be caused by umbilical catheterization
- Coagulation disorder: vitamin K deficiency
- Renal:
a. Structural: -renal cortical necrosis -neonatal glomerulonephritis (seen in maternal syphillis)
b. Neoplasm/mass -Wilms -Neuroblastoma -Nephroblastoma -Rhabdomyosarcoma -Polycystic kidney disease - Trauma: birth, iatrogenic, perinatal asphyxia
- Infection - UTI
Complications seen in infants of diabetic mothers?
-
Size related:
a. Large for gestational age
b. Shoulder dystocia/birth trauma - Metabolic/blood:
Hypoglycemia
Hypocalcemia, Hypomagnesemia
Polycythemia –> hyperbilirubinemia
- Congenital anomalies (neural tube, renal, cardiac malformations
- Vascular: Renal vein thrombosis
What are prenatal signs of TEF (3)?
- Frequent fetal hiccups
- Polyhydramnios
- Microgastria
TEF What is the most common type of TEF? -second most common?
TEF What is the most common types of TEF? In order of frequency
Think from most common to least common:
CAE-BD (It’s CAE’S BIRTHDAY!)
Type C (85%) = Esophageal atresia with distal TEF -on CXR after insertion of NG tube, will see coiling -esophageal atresia -distal tracheoesophageal fistula -gastric distension -respiratory distress/aspiration secondary to GERD
Second most common: type A (9%) = isolated esophageal Atresia (think A for ATRESIA!) -esophageal atresia without tracheoesophageal fistula -no gas in abdomen -scaphoid abdomen
Third most common: type E (6%) = isolated TEF (no esophageal atresia) -presents later in life -recurrent pneumonias/persistent cough due to aspiration
Fourth most common: type B (1%) = Proximal TEF with esophageal atresia
Fifth most common: Type D (!) = Double TEF with esophageal atresia (tracheoesophageal fistula between proximal and distal esophageal ends) (Esophageal atresia: presents in neonate because they can’t eat and the food comes back up!)
What are the risk factors for neonatal polycythemia? (4)
- Endocrine:
IDM, Graves disease, Neonatal hypothyroidism
- Newborn reated:
SGA, Postmature,
- pregnancy/delivery issues: Maternal hypertension, Recipient of twin-twin transfusion, Delayed cord clamping
- Syndromic: Beckwith-Wiedemann syndrome, Trisomy 13, 18, 21
What are the clinical manifestations of neonatal polycythemia? (8)
***Many affected infants are asymptomatic ***Symptoms and complications secondary to stasis and hyperviscosity in vasculature
Clinical manifestations:
- General: Irritability, Lethargy, Poor feeding
- Blood: Hypoglycemia Hyperbilirubinemia Thrombocytopenia
- Respiratory: Respiratory distress, Cyanosis
What are the indications for partial exchange transfusion in neonatal polycythemia?
Should be considered if:
- Hct > 70-75%
- S/S of hyperviscosity
In partial exchange transfusion for neonatal polycythemia, what is the formula for volume to be exchanged?
Volume of exchange (ml) = blood volume x (Observed-desired hematocrit)/observed hematocrit
***withdraw the blood slowly and replace with crystalloid (NS)
Describe the hyperoxic test.
Arterial gas is obtained in the right radial artery prior to placing the patient on 100% O2 via oxygen hood or I&V.
Leave 100% O2 on for 10 minutes. Then repeat the art gas.
- PaO2 > 150 post hyperoxic test: pulmonary disease
- PaO2 <100 post hyperoxic test: cardiac disease
- PaO2 100-150: inconclusive (could be cardiac or pulmonary)
***Keep in mind that hyperoxic test cannot differentiate between cardiac disease and severe PPHN
What are the side effects of PGE1 (6)?
- Apnea
- Cardiac: Hypotension ,Bradycardia or tachycardia
- Other: Fever, Seizures, Cutaneous flushing
What is the cause of hemolytic disease of the newborn aka erythroblastosis fetalis?
Rh incompatibility: pre-natal transplacental passage of maternal IgG antibodies active against paternal RBC Rh antigens on the fetus’ RBC ABO incompatability:
post-natal destruction of fetal RBCs by maternal IgM antibodies
What is RhoGAM?
Rho (D) immunoglobulin: IgG anti-D antibodies
-given to Rh negative mothers at approxiamtely 28 weeks and the Rho (D) immunoglobulin will remove any anti-D positive fetal RBCs before the maternal immune system can respond to them (prevents maternal sensitization)
During what events can maternal sensitization occur in hemolytic disease of the newborn caused by Rh incompatibility?
- Blood transfusion: if Rh-positive blood is infused into an Rh-negative woman through error
- During pregnancy: when fetal blood enters mom’s circulation through spontaneous or induced abortion
- At delivery: fetal blood enter’s mom’s circulation
Why is hemolytic disease of the newborn caused by Rh incompatibility worse in subsequent pregnancies?
In first pregnancy, usually the mother is exposed to fetal blood during delivery. Initially IgM antibodies are produced which are then later replaced by IgG antibodies over time (baby has already been delivered at this point).
In subsequent pregnancies, smaller amounts of antigen can stimulate IgG production which can then cross the placenta and cause hemolytic disease in subsequent pregnancies -this is why RhoGAM should be given to Rh negative moms at 28 weeks and also immediately post-partum
What are the complications of hemolytic disease of the newborn caused by Rh incompatibility? (4)
- Severe anemia –> heart failure –> hydrops fetalis
- Extramedullary hematopoiesis: hepatosplenomegaly
- Bone marrow hyperplasia with increased erythropoiesis and subsequently, thrombocytopenia
- Severe hyperbilirubinemia (within 1st day of life)
What lab test is the best predictor of severity of hemolytic disease of the newborn caused by Rh incompatibility of the newborn at delivery?
Cord blood bilirubin level: indicates severity of hemolysis in utero
- Cord blood Hgb is less reliable since it can be normal if baby has compensatory bone marrow and extramedullary hematopoiesis
- Mom’s anti-Rh titers correlate poorly to severity of disease
Describe the steps in antenatal diagnosis of hemolytic disease of the newborn caused by Rh incompatbility.
- Determine whether mom is Rh-negative or Rh-positive.
- If Rh-negative, take history of previous transfusions, abortion or pregnancies
- Test dad’s blood type to check for incompatibility
- Measure maternal titer of IgG antibodies to D antigen at 12-16, 28-32, 36 wks of gestation -presence of elevated antibody titers at the beginning of pregnancy, rapidly rising titers or titer of 1:64 or greater suggests significant hemolytic disease
What antenatal monitoring should occur for Rh negative mothers found to have Rh positive fetus?
If IgG antibodies to D antigen is found at a titer of 1:16 or more, monitor by
- Doppler US of middle cerebral artery flow of fetus
- real time fetal US for signs of hydrops and then
- percutaneous umbilical blood sampling if needed.
What is the antenatal treatment for hemolytic disease of the newborn secondary to Rh incompatibility? -indications?
In utero transfusion of PRBCs through the umbilical vein
- maternal/fetal sedation with diazepam and fetal paralysis with pancuronium
- may need to be repeated every 3-5 wks
- indications: 1. Hydrops 2. Fetal anemia (Hct < 30%)
What is the postnatal treatment for hemolytic disease of the newborn secondary to Rh incompatibility?
- Resuscitation if severe hemolytic anemia is present
- IVIG
- May require exchange transfusion if severe hyperbilirubinemia
What is the cause of the classic form of galactosemia?
Galactose-1-phosphate uridyl transferase deficiency (GALT)
- enzyme responsible for metabolizing galactose-1-phosphate
- autosomal recessive
- without this enzyme, galactose-1-phosphate accumulates and causes injury to kidney, liver and brain
What is the dietary source of galactose?
-when do infants with classic galactosemia typicaly present and why?
Milk and dairy products - lactose is converted to glucose and galactose in our bodies!
-infants with classic galactosemia present usually within the 1st week of life since they are receiving high amounts of lactose through breast milk or formula
What are the clinical features of galactosemia?
-acute (7) -if untreated,
chronic complications? (5)
Newborn or young infant with:
- GIT/Liver: Jaundice, Hepatomegaly, Vomiting, Lethargy/poor feeding
- Metabolic: . Hypoglycemia
- CNS: Seizures
- E coli sepsis (thought to be secondary to phagocytic and neutrophil dysfunction due to impaired glycolysis secondary to galactosemia)
Later in infancy or childhood if untreated:
- Neuro/eyes; Mental disability, Cataracts, Vitreous hemorrhage 5. 2. 2. Other: Renal failure Hypergonadotrophic hypogonadism
***Galactose-1-phosphate accumulates in liver, brain and kidney!
What are the diagnostic tests for galactosemia (2)?
- Urine for reducing substances: galactosuria is present
- Direct enzyme assay for GALT in RBCs - will be deficient
What are the diagnostic tests for galactosemia (2)?
- Urine for reducing substances: galactosuria is present 2. Direct enzyme assay for GALT in RBCs
What is the treatment for galactosemia?
Elimination of galactose from the diet
- galactosemia is a contraindication for breastfeeding
- will need lactose free formulas
What are the acute issues seen in babies born with maternal heroine use?
- Respiratory depression and RDS
- Lower birth weight
- Jaundice
What are the 3 main categories of neonatal coagulopathy? -
what are the 3 forms of hemorrhagic disease of the newborn?
-
Hemorrhagic disease of the newborn: secondary to vitamin K deficiency
a. -early form (within 1st 24 hrs):
maternal meds affecting vitamin K levels
i. Anticonv:Phenytoin 2. Isoniazid
ii. ABx: 3. Rifampin 4. Barbituates
iii) Warfarin -
b. classic form (DOL 2-7): vitamin K injection not given at birth -
c. Late form (weeks 2-6):
decreased vitamin K intake secondary to breastfeeding or hepatobiliary disease
- Congenital coagulopathy: bleeding in deep tissues -hemophilia A or B
- DIC: bleeding everywhere -decreased fibrinogen -increased PTT -increased INR -decreased platelets
What maternal meds may lead to early hemorrhagic disease of the newborn? (5)
- Phenytoin 2. Isoniazid 3. Rifampin 4. Barbituates 5. Warfarin
What are the causes of neonatal DIC? -maternal (2) -infant (4)
Maternal 1. Eclampsia 2. Placental abruption
Infant: 1. Sepsis 2. Asphyxia 3. Severe acidosis 4. NEC
How long does it take umbilical cord to slough off?
2 weeks -worry if it takes longer than 1 month since it might indicate neutrophil chemostatic defects
What is 2 vessel umbilical cord associated with? (4)
- Renal abnormalities
- Congenital malformations
- IUGR
- Trisomy 18
What are the recommendations for safe sleep practices (3)?
-
Bed details:
a. Sleep in a crib that meets government standards
b. Crib in parent’s room for 6 months -room sharing protects
c. Baby should not be in a non-crib surface (water bed, couch) against SIDS -
Sleeping details:
a. 3. Back to sleep
b. No heavy blankets, toys, etc. Can have thin blanket and baby can be in a sleeper
3. Environment
a. Decreased second hand smoke
b. In hospital, baby and mom should not share a bed
What are the risk factors for contracting RSV bronchiolitis? (8)
- Age: Prematurity , Less than 2 years old with cardiac issues 2. Environment: Second hand smoke, School age sibling at home
- Child: Boys, IUGR/SGA, Multiple gestation (< 2.5 kg), Underlying lung pathology
What are the etiologies of polyhydramnios? (4)
- GI obstruction: esophageal, duodenal, intestinal obstruction 2. Hypotonia (difficulty with swallowing): anencephaly, neural tube defects
- Diseases: GDM, Non-immune fetal hydrops, Bartter syndrome
4 Idiopathic (most common)
What is Bartter syndrome? -clinical features
Chloride channel abnormality
Metabolic: polyuria, hypokalemia, hyponatremia, hypercalcemia
CNS -often presents with seizures secondary to hyponatremia
What are the indications for exchange transfusion? (2)
- Metabolic: Severe hyperbilirubinemia, Polycythemia Hyperkalemia
- Hemolytic disease of the newborn
What are the side effects of exchange transfusion? (3)
- Cardiovascular: Thrombosis/air embolism, Volume overload
- Other: NEC, Hyperkalemia, Infection
- Death
Distinguish between G6PD, alpha, and beta thalassemia in terms of clinical presentation
G6PD: presents at 1 week Alpha thalassema: presents at birth with hydrops and death in neonate
Beta thalassemia: presents at 2-3 months
How is G6PD inherited?
X-linked recessive
What is Erb palsy? -associated complication?
C5-C6 nerve palsy:
waiter’s tip
- assymetric moro
- palmar grasp present
- shoulder won’t move but hand can move
-associated complication:
5% can ipsilateral diaphragmatic paralysis (C5 keeps you alive)
What is Klumpke’s palsy?
-associated complication?
C8-T1 nerve palsy:
Klumpke’s Klaw
-associated complication: 33% have Horner’s syndrome
How do you treat brachial plexus palsy?
Immobilize x 7-10 days,
then physiotherapy for mobilization
What are favorable prognostic signs for brachial plexus palsy?
- Recovery within 2 wks
- Only involvement of proximal upper extremity
What percentage of babies with brachial plexus will have residual deficits?
20-30%
What is the most common cause of neonatal conjugated hyperbilirubinemia?
Maybe now Biliary Atresia…was Neonatal hepatitis
What are the causes of conjugated hyperbilirubinemia? (4)
**Top 3 most common:
- Liver problems:
a. Biliary atresia
b. Cholestasis -Alagille -congenital
c. Idiopathic neonatal hepatitis
d. Choledochal cyst - Infections
- TPN
- Diseases: Galactosemia , Alpha-1-antitrypsin
What is the pathophysiology of congenital lobar emphysema? -what are the CXR findings?
unilateral enlargement due to accumulation of air and/or fluid
- valve effect, hyperexpansion and air trapping
- left upper lobe most commonly affected
- on CXR: hyperlucency, hyperinflation of one lobe with mediastinal shift and compression of remaining lung
What are the 4 categories of lung bud abnormalities?
- Congenital lobal emphysema
- Congenital pulmonary adenomatoid malformation (CPAM) (used to be known as CCAM)
- Pulmonary sequestration
- Bronchogenic cyst
What is the pathophysiology of CPAM? -CXR findings?
Congenital pulmonary adenomatoid malformation
- Proliferation of bronchiole structures but no alveoli -a bunch of cysts of lung tissue that communicates with tracheobronchial tree
- CXR findings: cysts (white)
What is the pathophysiology of pulmonary sequestration? -CXR findings?
-most common location?
Lung tissue not connected to bronchi and trachea
-blood supply is from systemic circulation and not pulmonary circulation
-most common location: left costophrenic sulcus
What is the major complication of subcutaneous fat necrosis?
Hypercalcemia
Differentiate between jiterriness and seizures.
- Eye movements: -normal in jitteriness -horizontal deviation, nystagmus and staring and blinking in seizures
- Movement: -fine movements in jitteriness -coarse movements in seizures
- Able to stop movements: -yes in jitteriness -no in seizures
How long does erythema toxicum last for?
What do the papules contain?
-what about pustular melanosis?
What do the pustules contain?
Erythema toxicum = develops 1-3 d after birth, lasts up to 1 week,
contains eosinophils
Pustular melanosis = present at birth, lasts 2-3 d,
contains neutrophils
What are some conditions associated with a large anterior fontanelle?
- Hypothyroidism
- Syndromes: Apert syndrome, Trisomy 13, 18, 21, Osteogenesis imperfecta
- Hydrocephalus
Where is the position of a cephalohematoma vs. subgaleal hematoma vs. caput?
-Cephalohematoma: subperiosteal hemorrhage (doesn’t cross suture lines)
-Subgaleal: collection of blood BELOW the aponeurosis that covers the scalp and the entire length of the occipito-frontalis muscle = can lead to extensive bleeding, develop a consumptive coagulopathy leading to hypotension
-Caput: soft tissue swelling of the scalp (crosses suture lines)
What is Mobius syndrome?
Absence of hypoplasia of the facial nerve leading to symmetrical facial palsy
If retinal hemorrhages occur during delivery from instrumentation or C-section, when should they resolve by?
85% resolve by 2 weeks,
they should ALL resolve by 4 weeks
What is the management of gastroschisis?
- Temperature monitoring since can lose heat from exposed bowel
- NG decompression , NPO = TPN & V fluids
- Protective covering and keep bowels in right lateral position to protect kinking of bowel until surgical repair
- Broad spectrum antibiotic coverage
- Surgical intervention ASAP
What are the main differences between breast milk and cow’s milk? (5)
Breast milk:
- Lower protein content
- Greater whey:casein ratio (70:30 as opposed to 60:40)
- Higher fat content (30-50% compared to 3-4%)
- Richer in Vitamins A, C, E; lower in Vitamins D and K
- Lower iron but better absorption
What is the calculation for determining umbilical catheter length? -UVC -UAC
UVC: (3 x wt in kg) + 9 UAC: (0.5 x UVC insertion depth) + 1
What are the increased risks of twin pregnancies? (8)
- Prematurity
- Growth restriction
- Spontaneous abortion
- Placental abnormalities (abruption, previa)
- Increased perinatal mortality
- Birth asphyxia
- Fetal malposition
- Polyhydramnios
If maternal drug abuse is suspected, which specimen from the infant is most accurate for detecting exposure?
Meconium = greater sensitivity than urine with positive findings persisting longer -contains metabolites gathered over as much as 20 weeks (as opposed to urine with more recent exposure)
What is the best way to assess gestational age in the fetus?
***Historically Nagele’s rule is the best way:
1st day of last menstrual period If this is not available:
- before 12 weeks gestation: crown-rump length on US
- beyond 12 weeks: biparietal diameter on US
What are the 5 components of the biophysical profile?
- Fetal breathing movements
- Gross movements
- Amniotic fluid level
- Fetal HR
- Fetal tone
***2 points each for possible total of 10 points
At what gestational age does pupillary reaction to light develop?
As early as 29 weeks but not consistently present until 32 weeks
When do premature infants “catch up” on growth charts?
Most catch up growth occurs during the 1st 2 years of life with most catching up by 3 years of age
What are risk factors for NEC in preterm infants? (5) -one protective risk factor against NEC?
- Prematurity & Low birth weight
- Use of steroids or NSAIDs
- Requiring ventilator support
- . Symptomatic PDA requiring surgery
Protective risk factor: breast milk
How long should infants with NEC receive nothing by mouth?
Infants with true confirmed NEC (radiographic or surgical evidence): NPO + TPN x 2-3 wks
-infants with suspected NEC can be fed after 3-7 days of bowel rest
What are the 3 stages of NEC?
Stage 1: suspected NEC -clinical signs: abdo distention, bloody stools, emesis, gastric residuals, apnea, lethargy -radiological signs: ileus, distension
Stage 2: Proven NEC -clinical signs + abdo tenderness +/- metabolic acidosis, thrombocytopenia -radiological signs: pneumatosis intestinalis, portal venous gas
Stage 3: Advanced NEC -clinical features in stage 2 + hypotension, significant acidosis, DIC, neutropenia -radiological features: stage 2 findings with pneumoperitoneum
When should iron supplementation be initiated for preterm babies?
4-8 weeks of age and maintained x 12-15 months
A newborn presents with thrombocytopenia. What is the most likely etiology in each of the following if present?
- blueberry muffin rash
- absence of radii
- palpable flank mass and hematuria
- large hemangioma
- abnormal thumbs
-blueberry muffin rash: think TORCH or viral infection
- absence of radii: think TAR (thrombocytopenia with absent radii)
- palpable flank mass and hematuria: renal vein thrombosis
- large hemangioma: Kasabach-Merritt syndrome
- Abnormal thumbs: Fanconi syndrome
What are the lab findings of DIC?
- Decreased fibrinogen
- Increased PT/PTT
- RBC fragmentation on peripheral smear from microangiopathic hemolytic anemia
What is the differential diagnosis of hypocalcemia in the neonate? -early (first 3 days of life) (3) -late (after 1st week of life) (8)
Early:
- Premature infants
- Infants with birth asphyxia
- Infants of diabetic moms
Late:
- Intake: High phosphate cow milk formula, Citrate (from exchange transfusion)
- Intestinal malabsorption
- Postdiarrheal acidosis
- Metabolic/Endocrine: Hypomagnesemia , Alkalosis, Neonatal hypoparathyroidism, Rickets
8.
What is the treatment for hypocalcemia in an infant presenting with seizures?
10% calcium gluconate, 2 ml/kg over 10 minutes
What is the most common pathogen causing late-onset sepsis in the prem infant in NICU? -treatment?
Coag negative staph (nosocomial infection)
-treatment: vancomycin
What is the classification of IVH?
Grade 1: germinal matrix hemorrhage
Grade 2: IVH without ventricular dilatation
Grade 3: IVH with ventricular dilatation
Grade 4: IVH with parenchymal involvement
Fill in the blank: in more than 90% of cases, IVH in prem infants occurs during the first ____ days of life.
-what is a common complication of severe IVH?
3 days!
-complication: posthemorrhagic hydrocephalus
What are the benefits of breastfeeding? (9)
Baby:
-
Immune:
a. Fewer episodes of otitis media, resp, GI illness
b. Increased immune protection - Human milk facilitates growth of beneficial, nonpathogenic flora
- Improved neurodevelopment
Mother:
- Social: Economic, Improved maternal-child bonding
- Medcial:
a. . Reduction in diabetes mellitus
b. Protective for mom against certain cancers (ovarian, breast)
c. Mom returns to pre pregnancy weight faster
What are contraindications to breastfeeding? (4 major categories)
- Metabolic d/o: galactosemia, PKU, urea cycle defects
- Infections: HIV, TB before treatment, Human t-cell lymphotropic viruses, HSV if present on breast
- Substance use by mom
- Medications: chemotherapy, radiation, lithium,
What are 3 clinical features of vitamin E deficiency?
- Hemolytic anemia (vitamin E is important for stabilizing the RBC membrane)
- Peripheral edema
- Thrombocytosis (increased platelets!!!)
In utero exposure to cocaine results in increased risk of which complications?
- Preterm labor
- Placental abruption
- Neonatal irritability
- SGA
- Microcephaly
***Usually requires no treatment
What is the timing of amniocentesis during pregnancy?
-what about CVS?
- Amniocentesis: between 15-17 wks typically
- CVS: after 10 wks
What causes the following?
- early decels
- variable decels
- late decels
- Early decels: head compression during contractions = not concerning
- Variable decels: most common, due to cord compression, may be concerning
- late decels: MOST concerning, due to uteroplacental insufficiency
When is the onset of neonatal acne?
At around 3 weeks of life = from sebaceous gland stimulation by maternal androgens
-usually resolves by 4 mo of age
What are the manifestations of neonatal lupus erythematous? (4)
- Cardiac: congenital heart block (usually 3rd degree), occurs during 18-30th week gestation, can lead to fetal hydrops
- Skin: annular erythematous scaling plaques on sun exposed areas during first 2 weeks of life and spontaneously disappears by 6 months when maternal abs are decreasing
- Thrombocytopenia
- Hepatitis
What is commonly seen in infants born to moms who were on the following medications during pregnancy?
- lithium
- phenytoin
- tetracycline
- phenytoin: digit and nail hypoplasia
- lithium: ebstein anomaly
- tetracycline: yellow-brown discoloration of teeth
Why do babies grunt in resp distress?
They are closing their glottis to maintain lung volume and gas exchange during exhalation
What is the most common neonatal cystic lung malformation?
Congenital lobar emphysema
What is the most common form of spina bifida?
Myelomeningocele: failure of the posterior neural tube to close
What is VACTERL syndrome?
Vertebral abnormalities
Anorectal malformations
Cardiac anomalies
TEF
Renal or radial anomalies
Limb malformations
What is the double bubble sign?
Represents duodenal atresia
-the 1st bubble on the left is the gas in the stomach and the 2nd bubble on the right is gas in the duodenum since it cannot pass through the atresia (no gas in the small or large bowel distal to the level of the obstruction)
What is the management of meconium ileus?
Diagnostic gastrografin enema (hypertonic, water soluble) = washes out meconium
-OR surgical management if this doesn’t work
What is the underlying pathophysiology of Hirschsprung disease?
-associated with what conditions? (3)
Failed migration of neural crest cells = affected bowel fails to relax causing a functional obstruction (dilated proximal colon, distal small caliber lumen)
- associated with:
1. Trisomy 21
2. Smith-Lemli-Opitz syndrome
3. Congenital central hypoventilation syndrome
What is the most severe complication of neonatal immune-mediated thrombocytopenia?
IVH (can occur in utero)
What are clinical features of congenital toxoplasmosis infection? -treatment?
Hallmark is CNS involvement!!
- Chorioretinitis
- Diffuse intraparenchymal calcifications
- Hydrocephalus
- treatment: 1. Pyramethamine 2. Sulfadiazine 3. Leucovorin
The mother of a newborn baby develops varicella shortly after birth.
What is the timing for which you should give VZIG to the infant?
Give VZIG if mother develops varicella 5 days before or 2 days after delivery
What is the most common cardiac defect seen in infants of diabetic moms?
Hypertrophic cardiomyopathy
What is the classic triad of congenital rubella syndrome?
- Sensorineural hearing loss
- Cataracts 3.
Congenital heart disease
Which infection is prevented by newborn eye prophylaxis with 0.5% erythromycin, 1% silver nitrate or 1% tetracycline?
N. gonorrhea! -these reduce the incidence of C. trachomatis but does not PREVENT infection
What is the difference between neonatal alloimmune thrombocytopenic purpura vs. neonatal autoimmune thrombocytopenic purpura?
-treatment of each?
Neonatal alloimmune thrombocytopenic purpura:
maternal antibodies directed against paternal antigen on baby’s platelets = see low plts in baby, normal plts in mom.
-tx:
- planned C section to decrease risk of intracranial hemorrhage
- Maternal IVIG prenatally
- Washed maternal plts given to baby postnatally
Neonatal autoimmune thrombocytopenic purpura:
maternal ITP with autoantibodies crossing placenta and destroying baby’s plts = see low plts in baby, low plts in mom. -
tx:
- Maternal steroids prenatally
- IVIG/steroids for baby postnatally
Omphalocele gastroschisis What are the features of gastroschisis?
not midline (right sided most common)
- no protective cover (full thickness abdo wall defect)
- 10-15% have intestinal atresia
- not associated with congenital abnormalities
- prognosis depends on integritiy of bowel
Polycythemia What are the complications of neonatal polycythemia?
Complications:
- Neuro: Seizures Stroke
- Renal- Renal failure Renal vein thrombosis
- Other: Pulmonary hypertension Necrotizing enterocolitis
