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GI II Exam 2 > Tieman Brandau Neonatal Case > Flashcards

Tieman Brandau Neonatal Case Flashcards

1
Q

Tests based on substances released from damaged tissue

A

endogenous substances released by damaged hepatocytes;
ALT
AST
Lactic dehydrogenase

Endogenous substances reflecting impaired bile flow:
GGT
Alk Phos
5'-nucleotidase
Leucine aminopeptidase
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2
Q

Tests based on substances synthesized by teh liver

A 
albumin
coag factors
serum lipids and lipoproteins
triglycerides
cholesterol
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3
Q

Cholestasis

A

Impaired bile flow caused by either intrahepatic or extrahepatic disorders
Laboratory studies will show increased direct bilirubin (conjugated bilirubin) measurements

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4
Q

Physiologic jaundice

A

defined as jaundice in an otherwise healthy infant
NOT physiologic if develops before 36 hours of age, persists beyond 10 days or if direct bilirubin is > 20 % of total bilirubin

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5
Q

Breast milk jaundice

A

Approximately 2% of breast fed infants will have prolonged unconjugated hyperbilirubinemia with levels ranging from 10-15 mg/dL

be wary of kernicterus

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6
Q

important test to get with infants

A

ammonia levels– important for inborn errors of metabolism

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7
Q

Differential Diagnosis for Neonatal Cholestasis

A

Infection
Metabolic disorders (20%)
Endocrine
Genetic (25%)
Familial intrahepatic cholestatic syndromes
Anatomical or obstructive disorders (25-35%)

Number of unique disorders causing cholestasis in the neonatal period is greater than in any other time of life

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8
Q

Infectious Causes of Neonatal Cholestasis

A

Viral-adenovirus, CMV, coxsackievirus, EBV, enterovirus, hepatitis A, B, C, herpes simplex, HIV, parvovirus, reovirus, rubella

Bacterial-UTI, sepsis, listerosis, TB

Spirochete-syphilis, leptospirosis

Parasites-toxoplasmosis, malaria, toxocariasis, histoplasmosis

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9
Q

congenital rubella syndrome- association

A

microcephaly
heart disease
petechiae and purpura

cataracts, glaucoma, strabismus, nystagmus, microphthalmia, iris dysplasia

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10
Q

early congenital syphilis

A

presents before 2 years
prematurity and intrauterine growth retardation
hepatosplenomegaly
nasal chondritis (snuffles)
skin rash
osteochondritis
neurological symptoms and signs, including hydrocephalus and cranial nerve palsies

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11
Q

late congenital syphilis

A 
presentation after 2 years
craniofacial malformation
dental abnormalities
interstitial keratitis
deafness
neurosyphilis
paroxysmal cold haemoglobinuria
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12
Q

Toxoplasmosis

A

most common intrauterine infection in developing countries
affects more than one billion in the world
most common cause of posterior uveitis

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13
Q

Metabolic Causes of Neonatal Cholestasis

A

Galactosemia
Glycogen storage disease type 4
Fatty acid oxidation defects (SCAD, LCAD)
Urea cycle defects, arginase deficiency
Tyrosinemia
Lysosomal storage diseases (Gaucher, Farber Wolman and Niemann-Pick)

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14
Q

Galactosemia effects

A 
brain damage
cataracts
jaundice
enlarged liver
kidney damage

if given milk, unmetabolized milk sugars build up and damage the liver, eyes, kidneys and brain

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15
Q

Glycogen storage disease Type IV

A

Andersen disease
autosomal recessive due to deficiency of glycogen banching enzyme

deficiency of GBE results in formation of an amylopectin-like compact glycogen molecule with fewer branching points and longer outer chains

patients with the classical form of GSDIV develop progressive liver disease early in life

the non-progressive hepatic variant of GSD IV is less frequent and these patients usually survive into adulthood

besides these liver related presentations, there are rare neuro muscular forms of GSD IV

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16
Q

Tyrosinemia 1 in Infants

A

Common Manifestations: Diarrhea and bloody stools
Vomiting
Poor weight gain
Extreme sleepiness
Irritability
“cabbage-like” odor to the skin or urine

Liver problems :
	Enlarged liver 
	Jaundice
	Tendency to bleed and bruise easily
	Swelling of the legs and abdomen
17
Q

Gaucher Disease Subtypes

A

get giant liver and spleen

18
Q

Genetic Causes of Neonatal Cholestasis

A

Cystic fibrosis
Trisomy 13, 18 or 21
Turner syndrome
α-1 antitrypsin deficiency (10% of all causes of neonatal cholestasis)

19
Q

Trisomy screening

A

21- down
18- edwards
13- patau

20
Q

Turner syndrome

A 
low posterior hairline
webbed neck and extra skin
wide-set nipples
swollen hands
discolored spots on skin
swollen feet
21
Q

Endocrine Causes of Neonatal Cholestasis

A

Hypothyroidism

Hypopituitarism

22
Q

untreated congenital hypothyroidism

A 
jaundice
poor feeding
hypotonia
macroglossia
large fontanelles, delayed closure
course facial features
mental retardation
short stature
23
Q

Familial Intrahepatic Cholestatic Syndromes

A

Alagille syndrome (arteriohepatic dysplasia)
Byler disease (Progressive Familial Intrahepatic Cholestasis)
Carioli disease
“Idiopathic” neonatal hepatitis

24
Q

Alagille Syndrome

A 
Autosomal dominant disorder
Associated findings include:
- Cholestatic liver disease
- Pulmonary valvular stenosis or atresia
- Vasuculopathy
- Renal disease
25
Q

3 most common forms of neonatal cholestasis

A

idiopathic neonatal hepatitis
extrahepatic biliary atresia
alpha-1-antitrypsin deficiency

26
Q

when to do the Kasai procedure?

A

first 4 weeks of life

taking a loop of small bowel, putting it up into the porta hepatis, and hoping it will drain the bowel. This is all we had before liver transplants for babies came around.

27
Q

CHOLESTASIS defn

A

The systemic retention of biliary constituents as a result of failure of formation and (or) the flow of bile

28
Q

intrahepatic vs extrahepatic cholestasis

A

INTRAHEPATIC
Usually a failure of formation of bile at the hepatocyte level
Elevated transaminases and unconjugated bilirubin
Can also be an obstructive process confined to the intrahepatic bile ducts

EXTRAHEPATIC
Obstructive process of the extrahepatic bile ducts
Elevated AP, GGTP and conjugated bilirubin

29
Q

NEONATAL OBSTRUCTIVE JAUNDICE - defn and most common causes

A

Must be considered in any newborn jaundice presenting or persisting after 14 days old
Elevated conjugated bilirubin, AP, GGTP
Often, but not always, with acholic stools

Most common causes are
Biliary atresia
Less common causes are congenital biliary tract anomalies, choledochal cysts, and infections

30
Q

BILIARY ATRESIA

A

have a high index of suspicion

Post-natal destruction of EH bile ducts with resultant injury and fibrosis of IH bile ducts
Unknown etiology
~30% of neo-natal cholestatic jaundice
Females > males
Associated with extra-hepatic anomalies
Most common cause of hepatic death and reason for liver transplantation in children
Diagnosed by lab, US, MRC and liver bx
Treated by Porto-enterostomy (Kasai procedure)
Best results if done at < 60 days of age
If Kasai procedure fails, liver transplant

31
Q

CHOLEDOCHAL CYSTS

A

Less common cause of pediatric obstructive jaundice (2-4%)
5 types—type V (intrahepatic) called Caroli’s disease***

60-70% are found at age under 10

  • Present with obstructive jaundice, acholic stools, pruritis, abd. pain, and/or fever
  • Diagnosed by typical lab pattern of obstructive jaundice, US, MRCP, and ERCP
  • Should usually be surgically resected because of risk of malignancy and cholangitis
32
Q

type 5 choledochal cysts

A

Caroli’s disease

GI II Exam 2 (16 decks)

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