Neonatology Flashcards

1
Q

Investigation for biliary atresia*

A

> Blood

  • FBC: anemia of chronic disease
  • LFT
  • Coagulation profile: decrease VitK dependent clotting factor -> PT increase

> Imaging

  • Abdominal ultrasound: triangular cord sign, gallbladder ghost sign
  • Hepatobiliary scintigraphy

> Definitive

  • Percutaneous liver biopsy: bile duct proliferation, perilobular edema, fibrosis
  • IntraOp cholangiogram
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2
Q

Describe Kasai procedure**

A
  • Jejunum is anastomosed to patent ducts of portal hepatis with removal of gallbladder and bile duct
  • The distal duodenum is anastomosed to the jejunal limb to create a Roux-en-Y configuration
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3
Q

Why Kasai best done before 2 months old

A
  • Excessive backflow of bile will injure hepatocytes thus causing permanent hepatic injury
  • Success rate for establishing good bile flow is much higher
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4
Q

Conjugated vs Unconjugated bilirubin

A

> Conjugated (with glucuronic acid)

  • Soluble in water, insoluble in fat and alcohol
  • Present in bile
  • Not toxic to tissue

> Unconjugated

  • Insoluble in water, soluble in fat and alcohol
  • Not present in bile
  • Toxic to tissue, can lead to kernicterus
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5
Q

Etiology of neonatal jaundice***

A

< 24 hours

  • Unconjugated: ABO/ Rhesus incompatibility, G6PD/ Pyruvate kinase deficiency
  • Conjugated: TORCHES

24 hours - 2 weeks

  • Unconjugated: physiological, breastfeeding jaundice, hemorrhage, septicemia
  • Conjugated: TORCHES

> 2 weeks (Prolonged)

  • Unconjugated: congenital hypothyroidism, infection, breastmilk jaundice
  • Conjugated: biliary atresia, TORCHES
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6
Q

Investigation for neonatal jaundice**

A

> Acertain underlying cause and extend of hemolysis (eg: severe, early onset)

  • G6PD testing
  • Mother’s and baby’s blood group
  • Direct Coombs test - Rh incompatibility
  • Full blood count + peripheral blood picture
  • Reticulocyte count
  • Septic workup (IF infection is suspected)

> TORCHES screening - antibody test
Ultrasound: choledochal cyst, gallstones
HIDA Scan (Hepatobiliary iminodiacetic acid)

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7
Q

What is HIDA scan

A
  • Radioactive tracer is injected into a vein in arm, travels through bloodstream to liver, then travels with the bile into gallbladder and through bile duct into small intestine
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8
Q

Complication of Kasai procedure

A
  • Cholangitis
  • GI bleeding form varices
  • Intestinal obstruction
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9
Q

Signs of neonatal sepsis*

A
  • Hypo/ Hyperthermia
  • Behavior change: lethargy, irritability, change in tone
  • Skin: poor perfusion, pallor, jaundice, petechiae
  • Feeding problem: poor feeding, vomiting, diarrhea
  • Cardiovascular: tachycardia, hypotension
  • Respiratory: tachypnea, cyanosis, respiratory distress
  • Metabolic: hypo/ hyperglycemia, metabolic acidosis
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10
Q

Risk factor for acute bilirubin encephalopathy/ Kernicterus

A
  • Hemolytic disease
  • Sepsis
  • Low gestational age
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11
Q

Complication of G6PD

A
  • Severe neonatal hyperbilirubinemia -> Kernicterus
  • Kidney failure
  • Splenomegaly
  • Cholelithiasis
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12
Q

Management of acute hemolysis in G6PD

A
  • Inciting agent should be removed as soon as possible
  • Aggressive hydration for acute intravascular hemolysis or transfusion for severe anemia -> prevent possible kidney damage from the precipitation
  • Blood electrolyte should be checked and correction of abnormalities made
  • Metabolic acidosis is usually observed but mostly resolved by transfusion support and fluid therapy
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13
Q

Advice for patient with G6PD

A
  • Avoidance of unsafe drugs and chemicals (eg: Primaquine, chloroquine, hydroxychloroquine)
  • Dietary restriction (eg: Fava bean, bitter melon)
  • If have symptoms caused by medication taken, inform doctor or nurse and stop taking the medication
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14
Q

Neonatal jaundice can be detected clinically at which level

A
  • Serum bilirubin rises above 5mg/dl
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15
Q

Causes of neonatal hypoglycemia*

A

> Inadequate glucose supply
- Inadequate glycogen store (eg: prematurity)

> Impaired glucose utilization

  • Excessive insulin secretion
  • Sepsis
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16
Q

Management of neonatal jaundice*

A

> Phototherapy

  • For TB >20mg/dL, administered continuously until falls below this level
  • Once reached, can be interrupted for feeding or parental holding

> Exchange transfusion
Indication:
- symptomatic infants with sign of bilirubin-induced neurologic dysfunction
- when intensive phototherapy fails to effectively reduce TB in infants
- TB >25mg/dL
- Isoimmune hemolysis - remove circulating antibodies and sensitized RBC

17
Q

Complication of phototherapy

A
  • Hyperthermia, hypovolemia
  • “Bronze baby syndrome” - deposition of bronze-color photoisomers; usually resolve without sequelae within weeks
  • Seizure
  • Childhood cancer
  • Skin manifestation - café-au-lait?
18
Q

Complication of neonatal jaundice

A
  • Liver cirrhosis
  • Acute bilirubin encephalopathy
  • Kernicterus (chronic)
19
Q

Definition of failure to thrive

A
  • Persistently below 3rd percentile

- Deceleration that crosses two major percentile

20
Q

Pathophysiology of breast milk jaundice

A
  • Human milk contains high concentrations of beta-glucuronidase, which catalyzes the hydrolysis of beta-D-glucuronic acid
  • The loss of beta-D-glucuronic acid due to increased degradation is thought to promote an increase in intestinal absorption of unconjugated bilirubin
21
Q

How to diagnosis neonatal sepsis*

A
  • Elevated I/T ratio (>=0.2): immature to total neutrophil counts
  • Total WBC count in neonate is physiological higher -> less sensitive?
22
Q

Which medication can increase breast milk production

A
  • Domperidone

- MOA: increases the level of hormone prolactin which is involve in breast milk production

23
Q

Conditions associated with Down syndrome*

A
  • Congenital cataract, squint and glaucoma
  • Congenital heart defect (AVSD most common, VSD, PDA, ASD, TOF)
  • Duodenal atresia, pyloric stenosis, tracheoesophageal fistula
  • Hypothyroidism, hypogonadism
24
Q

Causes of Down syndrome**

A
  • Non-disjunction (95%): paired chromosome 21 failed to separate -> fertilization of gamete with 2 chr 21 -> trisomy 21
  • Translocation (4%): result from unbalanced Robertsonian translocation where extra chr21 translocated onto chr14
  • Mosaicism (1%)
25
Q

Clinical features of Down syndrome*

A

> Facial

  • Round face and short neck
  • Flat occiput (brachycephaly and third fontanelle)
  • Hypertelorism
  • Bilateral upward slanting of eyes
  • Flat nasal bridge
  • Big, wrinkled tongue

> Upper and lower limbs

  • Single palmar crease (simian crease)
  • Clinodactyly
  • Short, stubby fingers
  • Hypotonia
  • Sandal gap between big toe and second toe
26
Q

Management of Down syndrome

A
  • Careful examination to look for associated complications
  • Investigations
    § Echocardiogram by 2 week (if clinical examination or ECG were abnormal) or 6 weeks
    § Chromosomal analysis
    § T4/ TSH at birth or by 1-2 weeks of life
  • Early intervention program should begin at diagnosis if health conditions permit
  • Assess strength and needs of family
  • Health surveillance and monitoring
27
Q

Describe Kramer’s rule

A
  • Blanching skin with slight finger pressure & noting underlying color of skin
  • Area of body (mg/dL)
    Hand and neck: 4-8
    Upper trunk (above umbilicus): 5-12
    Lower trunk (below umbilicus) 8-16
    Arms and lower legs: 11-18
    Palms & soles: >=18
28
Q

Causes of FTT

A

> Inadequate intake

  • Cleft lip, cleft platelet
  • Inadequate frequency of feeding

> Inadequate absorption

  • Inborn error of metabolism
  • Pyloric stenosis
  • GI atresia
  • IBD/ Celiac disease (older children)

> Excessive loss

  • Chronic diarrhea
  • Parasitic infestation

> Increase demand for energy

  • Chronic illness
  • Thyroid disorder
  • Malignancy
29
Q

Features of common chromosomal defect

  • Turner
  • Klinefelter
  • Edward
  • Patau
A

> Turner (XO)
- Short stature, “shield” chest (widely spaced nipple, a short and webbed neck)

> Klinefelter (XXY)

  • Neonatal: micropenis, hypospadias, or cryptorchidism
  • Teenage: delayed puberty
  • Men: small testes, androgen deficiency, infertility

> Edward (Trisomy 18)

  • Small physical size, decreased muscle tone
  • Low-set ears, overlapping fingers, clubfeet
  • Heart/ lung abnormalities

> Patau (Trisomy 13)

  • Small head, absent eyebrows, cleft lip/ platelet
  • Dysplastic/ malformed ears
  • Clenched hands and polydactyly
  • Undescended testes
30
Q

Clinical features for malnutritient

A

> Marasmus

  • Inadequate energy intake including protein
  • Low weight-for-height
  • Reduced mid-upper arm circumference

> Kwashiorkor

  • Protein deficiency with adequate energy intake
  • Symmetric peripheral pitting edema
31
Q

Prolonged jaundice in term vs preterm

A

> Term: >2 weeks

> Preterm: >3 weeks

32
Q

Definition of direct and indirect bilirubin

A
  • Direct = Conjugated

- Direct/ Total bilirubin ratio >15-20% -> conjugated hyperbilirubinemia

33
Q

Cut off point for

  • Low birth weight
  • Very low birth weight
  • Extremely low birth weight
  • Small for gestational age
A
  • LBW: <2500g
  • VLBW: <1500g
  • ELBW: <1000g
  • SGA: <10 centile of birth weight for age