Neonatal Jaundice Flashcards
biliary atresia common in
female
idiopathic neonatal hepatitis common in
male
Lethargy associated with
galactosemia
itching
Cholestasis
vomiting
galactosemia
dark urine and pale stools: Pale stool persistent vs intermittent
Pale stool persistent BA , intermittent NHS
.Fever , rash in pregnancy with and miscarriage with
TORCH, metabolic
AGA
BA
SGA
INH
Poor feeding ,lethargy, hoarse cry constipation
hypothyroidism
Convulsion
congenital infection
Normal, Delayed development
BA , congenital
Poor feeding
Galactosemia
Consanguinity: ,Affected sib :
metabolic disease, INH
Irritable , lethargic
congenital infection , metabolic disease
Large anterior fontanelle
Hypothyroidism , down syndrome
Microcephaly, Macrocephaly
CMV, Toxoplasmosis
Cataract
galactosemia
choreo-retinitis
CMV, rubella and toxoplasma.
Cherry red spots:
Nieman –pick disease.
Posterior emryotoxo
alagille syndrome.
Optic nerve
hypoplasia
pan- hypopitutarism
FTT
INH and congenital infection
heart rate and RR.
increased in infections
dysmorphic features.
(Downs syndrome , hypothyroidism, alagille syndrome, cleft lip or palate
with BA)
RTI
downs syndrome , cystic fibrosis
Potty belly, umbilical hernia
hypothyroidism
Simian crease & clinodactly and sandal gap
.
in Down syndrome
US: Absent/Small/non visualized gall bladder Ø Lack of post prandial contraction of GB. Ø Triangular cord sign(a cone shaped fibrotic mass cranial to the bifurcation of portal vein
BA
Hepatobiliary Scintigraphy: delayed uptake, normal excretion, normal uptake, absent excretion
Neonatal Hepatitis, Biliary Atresia
Percutaneous Liver Biopsy
Most important investigation in differentiating INH and BA.
Prerequisites: Normal CBC, PT, APTT & USG
Percutaneous Liver Biopsy could lead to bleeding, so these tests are needed
Kasai procedure followed by Liver Transplantation
BA
life long thyroxine
Hypothyroidism
Kasai Procedure statistics
Bile flow re-established in 90% if performed prior to
8 weeks of life and less than 20% if performed
after 12 weeks of life.
Complications of Kasai procedure
ascending cholangitis and re-
obstruction as well as failure to re-establish bile
flow.
Liver Transplantation
Indications: u Biliary atresia is the most common indication for transplant.
u When initial treatment was given lately/ Portoenterostomy not done
u Failed portoenterostomy
u Decompensated cirrhosis and end stage liver disease despite initial successful Kasai.
the single most important determinant in successful management of BA.
Early kasai Age(< 8 wks)
hepatobiliary disorders resulting in Cholestasis characterized by
Jaundice Ø High/dark colored urine Ø Pale stools + Ø Direct bilirubin > 1.5 mg% or > 20% of total, beyond 14 days of life
How does it differ from neonatal jaundice? onset Type of bilirubin Severity complications
BA types
Biliary Atresia
Biliary atresia: 1 in 10000 to 15000 infants. Female predominance
u It is a progressive obliterative inflammatory process involving the bile ducts, resulting in
obstruction of bile flow leading to cholestasis, hepatic fibrosis, and eventually cirrhosis.
u Average birth weight , hepatomegaly with firm to hard consistency
u Associated with many anomalies
u No well-documented familial cases
Idiopathic Neonatal Hepatitis
Idiopathic neonatal hepatitis: 1 in 5000 to 10000 live
birth. With male predominance and familial cases
(15-20%)
u Generally normal stools or acholic stools with onset
at one month-old ,Low birth weight .
u Normal liver on exam or hepatomegaly with normal
to firm consistency. u characteristic “giant cell hepatitis” lesion is present
on liver biopsy & for which no infectious, genetic,
metabolic or anatomic cause is identified
BA VS INH
Choledochal Cyst
Localized cystic dilatation of common bile
duct is called choledochal cyst.
u •25% patient present in neonates with prolonged jaundice & cholestasis.
Triad of choledochal cyst
- Intermittent jaundice 2. Recurrent abdominal pain 3. Abdominal mass
Galactosemia
Here galactose-1-phosphate accumulation occur due to galactose-1- phosphate Uridyl transferase enzyme deficiency.
u• Present with vomiting, loose motion, persistent jaundice, FTT, hepato- splenomegaly, septicemia, cataract, repeated hypoglycemia and convulsion.
Inspissated Bile Syndrome
Conjugated hyperbilirubinemia resulting from severe jaundice associated
hemolysis due to Rh or ABO incompatibility is termed as inspissated bile
syndrome.
Alpha-1-antitrypsin Deficiency
Alpha-1-antitrypsin makes up 90% of alpha-1- globulin fraction
u Biopsy also shows accumulation of PAS-positive, diastase-resistant eosinophilic granule.
u Varying degrees of fibrosis correlate with disease prognosis.
TPN Related Cholestasis
u It develops in >50% of infants with birth weight <1000 gram & in <10% of
term infants after giving prolonged parenteral feeding.
u This may be due to lack of enteral feeding →reduction of gut hormone
secretion → reduce bile flow → biliary stasis.
Murmur
: congenital rubella syndrome ,down syndrome , alagille
syndrome)
choledochal
firm hepatomegaly,
splenomegaly
BA
Right hypochondric mass:
choledochal cyst.
Ascites
late
stage 3-4 months later
