Chapter 4 - Neonatology Flashcards

1
Q

What are apgar scores?

A
  • they are an assessment of intrapartum stress and neurologic depression at birth
  • when persistently low, the score indicates a need for resuscitation
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2
Q

How are apgar scores calculated?

A

assessed at 1 and 5 minutes after birth plus every five minutes until a score of 7 or more is reached

  • hear rate: absent, < 100 bpm, > 100 bpm
  • respirations: absent, slow or irregular, good/crying
  • muscle tone: limp, some flexion, active motion
  • reflex irritability: no response, grimace, cough/sneeze/cry
  • color: blue, body pink but extremities blue, all pink
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3
Q

What is vernix caseosa?

A

a benign, thick, white, creamy material found covering large areas of the skin in preterm in infants

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4
Q

What is acrocyanosis?

A

cyanosis of the hands and feet, which is frequent during the first 48-72 hours of life, and is indicative of vasomotor instability

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5
Q

What is cutis marmorata?

A

mottling of the skin with venous prominence, an indicator of vasomotor instability

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6
Q

Jaundice is always abnormal if what is true?

A

if it is detected in the first 24 hours of life

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7
Q

What is milia?

A

benign, self-resolving, small cysts formed around the pilosebaceous follicles, which appear as tiny, whitish papules over the nose, cheeks, etc.

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8
Q

What is pustular melanosis?

A

a benign, transient rash characterized by small, dry superficial vesicles over a dark macular base, which must be differentiated from viral and bacterial infections

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9
Q

What is erythema toxic neonatorum?

A

a benign rash characterized by erythematous macule, papules, and pustules, with lesions containing eosinophils and which spare the palms and soles

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10
Q

What is a nevus simplex?

A

a pink macular lesion on the neck or face, which is the most common vascular lesion of infancy

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11
Q

What is a nevus flammeus?

A
  • also known as a port wine stain
  • a vascular malformation composed of dilated capillary-like vessels, which often become darker with age
  • those associated with the ophthalmic branch of the trigeminal may be associated with CNS vascular malformations, seizures, and intracranial calcifications as part of Sturge-Weber syndrome
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12
Q

What is the difference between a strawberry and a cherry hemangioma?

A
  • strawberry: a benign capillary hemangioma of infancy that grows rapidly and regresses spontaneous by 5-8 years of age; requires intervention only if it compromises the airway or vision
  • cherry: a benign capillary hemangioma of the elderly, which does not regress
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13
Q

Compare and contrast a caput succedaneum and a cephalohematoma.

A
  • caput succedaneum are diffuse edema or swelling of the soft tissue of the scalp, which crosses the cranial sutures and usually the midline
  • cephalohematomas are subperiosteal hemorrhages secondary to birth trauma, which are confined and limited by the cranial sutures
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14
Q

What are craniotabes?

A

soft areas of the skull with a ping-pong ball feel, which usually disappear within weeks or months of brith

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15
Q

What is Pierre Robin sequence?

A
  • micrognathia
  • glossoptosis
  • cleft palate
  • obstruction of the upper airway
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16
Q

What are epstein pearls?

A

small, white, epidermoid-mucoid cysts on the hard palate, which usually disappear within weeks of birth

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17
Q

What is neonatal torticollis?

A

asymmetric shortening of the sternocleidomastoid, which may result from being in a fixed position in utero or from postnatal hematoma resulting from birth injury

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18
Q

What is the difference between pectus carinatum and pectus excavatum?

A
  • carinatum: prominent and bulging sternum

- pectus excavatum: depressed sternum

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19
Q

What are five signs of respiratory distress in a newborn?

A
  • respiratory rate greater than 60
  • deep respirations
  • cyanosis
  • expiratory grunting
  • retractions
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20
Q

What is a periodic breathing pattern?

A

apneic bursts lasting less than 10 seconds, seen in premature infants which have no clinical significance

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21
Q

What is a normal respiratory and heart rate in a newborn?

A
  • respiratory rate < 60

- heart rate between 95-180

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22
Q

During a newborn exam, what would diminished femoral pulses suggest? What would exaggerated femoral pulses suggest?

A
  • diminished: coarctation of the aorta

- increased: patent ductus arteriosus

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23
Q

Upon inspection of the umbilical cord, what would the presence of only one umbilical artery suggest?

A

congenital renal anomalies

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24
Q

What is diastasic rectified?

A
  • a separation of the left and right side of the rectus abdomens at the midline of the abdomen
  • gradually disappears as the infant develops and the rectus abdomens muscles grow
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25
Q

How are umbilical hernias treated?

A

most close spontaneously, so intervention is reserved for cases that persist or become symptomatic

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26
Q

What is a persistent urachus?

A

a failure of the tracheal duct to close, resulting in a fistula between the bladder and umbilicus; often urine drains from the umbilicus in these cases

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27
Q

What is the difference between a meconium plug and meconium ileum?

A
  • plug is obstruction of the left colon and rectum caused by dense, dehydrated meconium
  • ileus is the occlusion of the distal ileum caused by thickened, dried, and viscid meconium, usually secondary to a deficiency of pancreatic enzymes and an abnormally high protein content of intestinal secretions
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28
Q

What is hydrometrocolpos?

A
  • an imperforate hymen with retention of vaginal secretions

- may present as a small cyst between the labia at birth or as a lower midline abdominal mass during childhood

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29
Q

How do hypospadias and epispadias differ apart from the location of the meatus?

A
  • hypospadias is not associated with other urinary malformations
  • epispadias is often associated with bladder extrophy
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30
Q

How is cryptorchidism managed?

A
  • check for associated hernia, GU malformations, hypospadias, or other genetic syndrome
  • most resolve within 1 year without intervention
  • after 1 year, surgical correction is needed to maintain fertility and reduce the risk of future malignancy
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31
Q

Absence or hypoplasia of the radial at birth is associated with what three conditions?

A
  • TAR: thrombocytopenia absent radii
  • Fanconi anemia
  • Holt-Oram syndrome
32
Q

List frequent problems experienced by preterm infants.

A
  • disrupted mother-father-infant interaction
  • intracranial hemorrhage
  • retinopathy of prematurity
  • RDS or bronchopulmonary dysplasia
  • anemia
  • infection
  • hypothermia
  • hypoglycemia
  • hypocalcemia, fluid and electrolyte abnormalities
  • indirect hyperbilirubinemia
  • patent ductus arteriosus
  • perinatal asphyxia
  • NEC
33
Q

How do we define preterm and post-term deliveries?

A
  • preterm are those occurring less than 37 completed weeks of gestation
  • post-term are those occurring at 42 or more weeks gestation
34
Q

What are the most common complications of post-term delivery?

A

placental insufficiency and it’s consequences: intrauterine asphyxia, meconium aspiration syndrome, and polycythemia

35
Q

How do we define small-for-gestational age, large-for-gestational age, and high birth weight babies?

A
  • SGA: weigh below the 10th percentile
  • LGA: weigh above the 90th percentile
  • HBW: weigh more than 4 kg
36
Q

Large-for-Gestational Age

A
  • defined as any newborn with birth weight above the 90th percentile
  • many are simply constitutionally large but maternal diabetes is an important pathologic etiology
  • potentially complicated by need for delivery via C-section or using forceps; birth injuries like fractured clavicle, brachial plexus injury, or facial nerve palsy; or hypoglycemia
37
Q

Small-for-Gestational Age

A
  • defined as any newborn with birth weight below the 10th percentile
  • may be due to constitutional factors, prematurity, etc.
  • differs from intrauterine growth restriction because it is a diagnosis made at birth rather than during pregnancy
  • may be complicated by temperature instability, inadequate glycogen stores with hypoglycemia, or polycythemia and hyperviscosity syndrome
38
Q

What is cyanosis? At what level does it occur?

A
  • a bluish discoloration directly related to the absolute concentration of deoxygenated or reduced hemoglobin
  • appears when more than 3 g/dL of reduced hemoglobin is in arterial blood or more than 5 g/dL in capillary blood
39
Q

What are the etiologies for cyanosis in a neonate?

A
  • respiratory pathology
  • the 5 T’s of cyanotic congenital heart disease: ToF, transposition of the great vessels, truncus arteriosus, tricuspid atresia, and total anomalous pulmonary venous connection
  • CNS pathology like intraventricular hemorrhage
  • hematologic disorders like polycythemia
40
Q

What is the 100% oxygen test used to evaluate cyanosis? What do the various results indicate?

A

it is an arterial blood gas performed after administration of 100% oxygen used to evaluate whether cyanosis is cardiac or respiratory in nature

  • congenital heart disease with reduced pulmonary blood flow (e.g. ToF) will see only a slight increase in PaO2 (10-15 mmHg)
  • congenital heart disease associated with normal or increased pulmonary flow (e.g. truncus arteriosus) will see a more substantial increase (15-20 mmHg)
  • respiratory etiologies will have a considerable increase in PaO2, often reaching more than 150 mmHg
  • the exception to respiratory pathology is infants with severe lung disease causing pulmonary hypertension, which generates a right-to-left shunt through the foramen ovale or ductus arteriosus
41
Q

How does respiratory distress in preterm infants differ from that experienced by term infants?

A
  • RDS is the more common etiology in preterm infants

- meconium aspiration and persistent pulmonary hypertension of the newborn are more common in term

42
Q

What are signs of respiratory distress in a newborn?

A
  • tachypnea
  • decreased air entry or gas exchange
  • retractions
  • grunting
  • stridor
  • flaring of the alar nasi
  • cyanosis
43
Q

Respiratory Distress Syndrome

A
  • respiratory distress caused by lack of surfactant, which is most often seen in preterm neonates less than 37 weeks
  • lack of surfactant leads to collapse of air sacs and formation of hyaline membranes
  • most often due to prematurity (insufficient time to produce), c-section (less stress-induced steroid release, which otherwise triggers surfactant production and secretion), or maternal diabetes (insulin inhibits surfactant production)
  • other risk factors include white, preterm, males; mother with previous RDS infant; and hypothermia or asphyxia
  • CXR is diagnostic as it will show diffuse atelectasis with increased density in both lungs, a ground-glass appearance, and air bronchograms
  • treat with supplemental oxygen, CPAP, or mechanical ventilation and with exogenous surfactant
  • may be complicated by air leaks, NEC, respiratory acidosis, bronchopulmonary dysplasia, retinopathy of prematurity, intraventricular hemorrhage, sepsis, and patent ductus arteriosus
44
Q

What is pulmonary surfactant? How do we assess production?

A
  • a product of type II pneumocytes, primarily composed of dipalmitoylphosphatidylcholine (aka lecithin)
  • first seen around 28 weeks gestation; adequate levels are usually reached around week 34
  • can assess production using the L/S ratio since sphingomyelin levels in amniotic fluid remain relatively stable through pregnancy
  • a ratio of more than 2 indicates fetal lung maturity
45
Q

Persistent Pulmonary Hypertension of the Newborn

A
  • any condition other than congenital heart disease that is associated with low blood flow to the lungs after birth
  • this includes perinatal asphyxia, meconium aspiration, diaphragmatic hernia, hypoplastic lungs,
  • these etiologies increase pulmonary vascular resistance, causing significant right-to-left shunting through the foramen ovale or ductus arteriosus, resulting in hypoxemia
  • presents with respiratory distress, cyanosis, and notably different pre- and post-ductal PaO2
  • diagnosed with CXR for which findings differ based on etiology and echocardiogram, which rules out heart disease and assess the degree of right-to-left shunting
  • prevent hypoxemia with oxygen or ventilation and use nitric oxide as a pulmonary vasodilator
46
Q

What is meconium?

A

material in the fetal gut composed of water, mucopolysaccharides, desquamated skin and GI mucosal epithelial cells, vernix, bile salts, and amniotic fluid

47
Q

Meconium Aspiration Syndrome

A
  • meconium, often passed as a consequence of distress in the fetus at term, becomes more frequent after 42 weeks; meconium-stained amniotic fluid may then reach distal airways and alveoli in utero if the fetus becomes hypoxic and develops gasping or deep respiratory movements
  • presents with signs and symptoms of respiratory distress
  • diagnosed based on history of meconium at, or before, delivery and on increased lung volume with diffuse patchy areas of atelectasis and parenchymal infiltrates on CXR
  • managed with suctioning of the perineum and trachea in addition to oxygen administration
48
Q

Idiopathic Apnea of Prematurity

A
  • defined as a respiratory pause without airflow lasting more than 20 seconds or a pause of any duration accompanied by bradycardia, cyanosis, or desat
  • can be central, in which there is a complete cessation of chest wall movements and no airflow, or secondary to airway obstruction, in which the chest wall continues to move but without airflow
  • many etiologies of apnea so idiopathic apnea of prematurity is a diagnosis of exclusion
  • frequency decreases with gestational age and usually resolves by 38-44 weeks corrected gestational age
  • manage by maintaining a neutral thermal environment, treating hypoxia, and using caffeine, a respiratory stimulant
49
Q

What is the cutoff for distinguishing indirect from direct hyperbilirubinemia?

A
  • indirect: elevated bilirubin in which conjugated/direct is less than 15% of the total bilirubin
  • direct: elevated bilirubin in which conjugated/direct is more than 15% of the total bilirubin
50
Q

What are the most common causes of direct hyperbilirubinemia?

A
  • biliary atresia
  • neonatal infection (sepsis or hepatitis)
  • metabolic disorders including galactosemia, hereditary fructose intolerance, tyrosinemia, a1AT deficiency
51
Q

Jaundice should always be evaluated if one of what three conditions is met?

A
  • jaundice appearing in the first 24 hours
  • bilirubin rises more than 5-8 mg/dL in a 24 hours period or more than 0.5 mg/dL per hour (these suggest hemolysis)
  • direct hyperbilirubinemia
52
Q

How should indirect hyperbilirubinemia be evaluated from a diagnostic stand point? Direct hyperbilirubinemia?

A
  • indirect: CBC, reticulocyte count, and smear to evaluate for sepsis and hemolysis
  • direct: hepatic ultrasound, serologies for viral hepatitis, radioisotope scans of the hepatobiliary tree
53
Q

How should hyperbilirubinemia be managed?

A
  • requires serial bilirubin assessments; get a direct bilirubin if total bilirubin is persistently elevated
  • observe, particularly for pale stools or dark urine, suggestive of biliary atresia
  • reassurance can be used for physiologic jaundice with low risk of neurologic effects
  • phototherapy may be indicated based on risk
  • exchange transfusion is performed for rapidly rising bilirubin levels secondary to hemolytic disease
54
Q

Describe bilirubin metabolism.

A
  • protoporphyrin is converted to UCB
  • UCB is transported by albumin to the liver
  • there, it is conjugated by uridine diphosphate glucuronyl tansferase to glucuronide
  • this water soluble CB is excreted in bile and into the intestine
  • most CB is metabolized by intestinal flora to urobilin and excreted in stool while some is deconjugated via beta-glucuronidase and reabsorbed
55
Q

How does bilirubin metabolism differ between neonates and adults?

A
  • they have lower UDPGT activity
  • they lack the intestinal flora that metabolize CB to urobilin for excretion
  • they have meconium in the GI tract, containing beta-glucuronidase, which deconjugates the bilirubin and allows it to be reabsorbed
56
Q

What are the features of early bilirubin-induced neurologic dysfunction, late BIND, and kernicterus?

A
  • initially, poor suck, high-pitched cry, hypotonia, lethargy, seizures
  • later, extensor hypertonia and opisthotonus
  • kernicterus: abnormalities in tone and reflexes, choreoathetosis, tremor, oculomotor paralysis, sensorineural hearing loss, cognitive impairment
57
Q

What is the difference between an ABO mismatch, incompatibility, and set-up?

A
  • a mismatch is when the maternal and fetal blood types are different
  • setup is a specific mismatch in which the mother has blood group O and the baby has a or B, but the Coombs test is negative
  • incompatibility occurs when the mother has blood group O, the baby has a or B, and the direct Coombs test is positive
58
Q

Physiologic Jaundice of the Newborn

A
  • defined by a total bilirubin less than 15 in full-term infants who are otherwise healthy and have no other demonstrable cause for elevated bilirubin- it is benign and self-limited- usually recognized on the 2nd or 3rd day of life- pathogenesis involves increased bilirubin production since fetal RBCs are shorter-lived, relative UDPGT deficiency, lack of intestinal flora to metabolize bile, high levels of beta-glucuronidase in meconium, minimal oral intake which slows excretion of meconium
59
Q

Breastfeeding Jaundice

A
  • happens early in the first week of life when milk supply is relatively or absolutely low
  • this limited quantity reduces oral intake, decreases GI motility, promotes retention of meconium, and increases beta-glucuronidase levels in the GI tract
  • beta-glucuronidase deconjugates bilirubin and allows it to be reabsorbed via enterohepatic circulation, elevating bilirubin levels
60
Q

Breast Milk Jaundice

A
  • begins in the first 4-7 days of life, peaks around days 10-14, and may persist for up to 12 weeks
  • most likely explanation is beta-glucuronidase present in breast milk aids enterohepatic circulation of bilirubin
61
Q

What are three causes of antibody-positive hemolytic jaundice and two causes of antibody-negative hemolytic jaundice? What are four causes of non-hemolytic jaundice?

A
  • Ab+: Rh incompatibility, ABO incompatibility, minor blood group antigen incompatibility
  • Ab-: membrane defects like spherocytosis or enzyme defects like G6PDH
  • non-hem: extensive bruising from birth trauma, large cephalohematoma, polycythemia, swallowed blood during delivery
62
Q

List eight etiologies for jaundice.

A
  • physiologic
  • breastfeeding
  • breast milk
  • hemolysis
  • non-hemolytic RBC breakdown
  • metabolic errors (Crigler-Najjar, Gilbert syndrome, galactosemia, hypothyroidism)
  • neonatal sepsis
  • congenital infection
63
Q

Which inborn error of metabolism is often associated with hyperbilirubinemia in the newborn?

A

galactosemia

64
Q

What are the most common presenting signs of neonatal abstinence syndrome?

A
  • jitteriness
  • hyperreflexia
  • irritability
  • tremulousness
  • feeding intolerance
  • excessive wakefulness
65
Q

Fetuses and newborns of drug-abusing mothers are at greater risk of mortality due to what causes?

A
  • obstetric complications like placental abruption
  • fetal demise in utero from withdrawal
  • perinatal asphyxia
  • congenital anomalies
  • child abuse and SIDS
66
Q

Esophageal Atresia with Distal Tracheoesophageal Fistula

A
  • the most common TE anomaly
  • presents with polyhydramnios in utero
  • neonates drool, choke, and vomit at the time of their first feeding; cyanosis can occur secondary to laryngospasm, which occurs to avoid reflux-related aspiration
  • the TEF allows air to enter the stomach and duodenum, which can be seen on x-ray; this helps differentiate it from esophageal atresia alone
  • often associated with VACTERL defects
67
Q

Congenital Diaphragmatic Hernia

A
  • results from abnormal development of the diaphragm between weeks 5 and 8 of gestation and allows abdominal contents to enter the thorax
  • most commonly involves the posterolateral area of the left diaphragm
  • presents with scaphoid abdomen; respiratory insufficiency, hypoxemia, and respiratory acidosis from pulmonary hypoplasia; and bowel sounds within the chest
  • diagnosis is typically made by ultrasound; CXR will show little or no gas in the abdomen, an absent diaphragmatic dome, significant contralateral mediastinal shift, and bowel loops in the thorax
  • should never use bag-and-mask ventilation to treat as this may distend the bowel and increase compression of the lung; instead, intubate and mechanically ventilate while correcting acidosis and hypercarbia until surgical intervention is possible
68
Q

Omphalocele

A
  • results from a disturbance of the midgut’s re-entry into the abdomen around week 10 of gestation
  • it is a central defect and there is a true hernia sac as the abdominal contents are covered by the peritoneal sac
  • frequently associated with other congenital anomalies
69
Q

Gastroschisis

A
  • results from a disturbance of the midgut’s re-entry into the abdomen around week 10 of gestation
  • it is located in the right paraumbilical area, is not mid-line, and has no true hernia sac
  • there is an increased risk of bowel damage from exposure to amniotic fluid, but unlike omphalocele, there is no increased risk for other congenital anomalies
70
Q

What are the three most significant differences between omphalocele and gastroschisis?

A
  • omphaloceles are midline where as gastroschisis is usually located in the right paraumbilical area
  • omphalocele has a true hernia sac while gastroschisis does not
  • omphaloceles are frequently associated with other congenital anomalies while there is no such risk with gastroschisis
71
Q

Meconium Ileus

A
  • CF leads to an abnormal accumulation of intestinal secretions and to a pancreatic enzyme deficiency, which both increase viscosity of meconium and contribute to an occlusion of the distal ileum
  • presents with abdominal distension, lack of meconium passage, and vomiting
  • abdominal radiographs reveal distention with minimal air-fluid levels since air remains trapped in the meconium, sometimes giving it a soap-bubble appearance
  • treat with enemas to relieve the obstruction
  • monitor for complications like intestinal perforation, meconium peritonitis, and volvulus
72
Q

Hirschsprung Disease

A
  • defective relaxation and peristalsis of the rectum and distal sigmoid colon
  • due to a congenital failure of ganglion cells (neural crest derived) to descend into the myenteric and submucosal plexuses
  • strongly associated with Down syndrome and RET mutations
  • clinical features, based on obstruction, include failure to pass meconium, an empty rectal vault of digital exam, megacolon, and bilious emesis
  • requires a rectal SUCTION biopsy to sample the two plexuses, which aren’t biopsied normally
  • treatment involves resection of the involved bowel
73
Q

Necrotizing Enterocolitis

A
  • presents with abdominal distention and tenderness, residual gastric contents, bilious aspirate, bloody stools, abdominal erythema, metabolic acidosis, oliguria, and thrombocytopenia
  • radiographs demonstrate abdominal distension, air-fluid levels, thickened bowel walls, pneumatosis intestinal, and venous portal gas
  • treat with bowel rest, no PO feeds, gastric decompression, antibiotics, and parenteral nutrition; surgical management is indicated for complicated cases
  • late complications include intestinal obstruction, nutritional deficiencies, and cholestasis
74
Q

Neonatal Hypoglycemia

A
  • defined as a glucose level below 45 mg/dL
  • may be caused by maternal diabetes, insulin-producing tumors, islet cell hyperplasia (nesidioblastosis), SGA with limited hepatic glycogen stores, prematurity and poorly developed glucokneosis, inborn errors of metabolism, and endocrinopathies
  • presents with diaphoresis, jitteriness, feeding problems, tachycardia, hypothermia, hypotonia, and seizures
  • screen for using glucometer with a cutoff below 40, but confirm with serum or plasma glucose
  • problematic even if asymptomatic because glucose is the brain’s primary unit of energy, so it is important to treat even before confirmatory test comes back
  • asymptomatic can be breast fed or given pumped breast milk or formula; otherwise, treat with IV dextrose
  • monitor until glucose is stable and above 45 mg/dL
75
Q

What are the complications often associated with maternal diabetes?

A
  • various congenital anomalies, including cardiac
  • large for gestational age or small for gestational age if there is placental insufficiency secondary to diabetic-induced vascular complications
  • visceromegaly
  • small left colon syndrome
76
Q

What is small left colon syndrome?

A
  • a potential complication unique to maternal diabetes

- presents with abdominal distension and failure to pass meconium due to decreased caliber of the left colon

77
Q

Polycythemia

A
  • defined as a central venous hematocrit greater than 65%
  • may be caused by increased EPO secondary to placental insufficiency or increased placental transfusion from delayed cord clamping amongst other things
  • presents with plethora, poor perfusion, cyanosis, poor feeding, respiratory distress, lethargy, jitteriness, seizures, renal vein thrombosis, and metabolic acidosis
  • increases the risk for NEC
  • treat with partial exchange transfusion