Pediatric emergency medicine trisk 0881 0881
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procoagulation factors (as measured by high INR), they maintain a balance
because they also have low levels of anticoagulation factors which are seldom
measured. Plasmapheresis, exchange transfusion, and hemodialysis may be
necessary depending on the severity of the case. Mortality with acute liver failure
can be 80% or higher; however, even with severe dysfunction, full recovery can
occur. No reliable way currently exists to predict which patients presenting with
acute liver failure will have a poor prognosis.
Congenital TORCHS Infections and Sepsis
One of the first signs of sepsis in a newborn could be conjugated
hyperbilirubinemia, which may occur antecedent to more recognizable physical
findings of sepsis or blood cultures becoming positive. Infants who have
congenital infections may also have a low birth weight. They generally present in
the immediate newborn period with cholestatic jaundice, irritability, jitteriness,
and/or seizures. On examination, microcephaly, hepatomegaly, splenomegaly, and
petechiae may be seen with the perinatal TORCHS complex. These include
perinatal infections from toxoplasmosis, parvovirus B19, rubella, CMV, herpes
simplex, varicella zoster, and syphilis. Jaundice may also be an early diagnostic
sign of urinary tract infection (UTI) in the neonatal period. Gram-negative
infections such as UTI and sepsis can lead to cholestasis as bile flow is sensitive
to circulating endotoxins.
Biliary Atresia
Infants with BA present with a mild conjugated hyperbilirubinemia, sometimes
under the typical cutoff of 1 mg/dL. They initially feed well and thrive. Their
stools may be intermittently pigmented early in life and then become permanently
without pigment usually by 4 to 6 weeks. The medical history and physical
examination at presentation are generally reassuring, with the exception of
jaundice and hepatomegaly. However, if these infants are not diagnosed early,
they will have too much liver injury to be able to benefit from the Kasai
hepatoportoenterostomy (HPE) and will need liver transplantation for survival
(see Chapter 91 Gastrointestinal Emergencies ).
Evaluation for BA varies from institution to institution. Abdominal ultrasound
is neither sensitive nor specific, though absence of gall bladder, irregular gall
bladder walls, and polysplenia are findings that may be seen. The gold standard
for diagnosis is an intraoperative cholangiogram to demonstrate obstruction of
bile flow. Treatment includes the Kasai HPE to allow direct flow into the
intestines, and has the highest success rate when performed earlier. Ideal timing
for the Kasai procedure with highest success of reestablishing bile flow is <30 to
