Pediatric emergency medicine trisk 2086 2086
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diuretic (e.g., spironolactone) to achieve a slow, gradual change in ascites is
all that is initially required. Aggressive diuresis may precipitate or worsen
hepatorenal syndrome.
Patients with encephalopathy should be frequently monitored for changes in
neurologic function. Bowel therapy with lactulose is a common therapy
directed at hyperammonemia. Patients with HE should also have medications
reviewed for any medication that may worsen their mental status. Blood
within the GI tract, if present, provides a high protein load and may also
worsen HE. Patients should also be monitored for signs of elevated ICP.
Cerebral edema is a major cause of mortality. Invasive monitoring, however,
has not been shown to improve outcome and can be difficult in the setting of
intractable coagulopathies. Patients should be monitored in an intensive care
setting and neurosurgical consultation is necessary.
Additional medical treatments include steroids, N-acetylcysteine, and
plasmapheresis. Steroids have been shown to be beneficial in the setting of
adrenal insufficiency, autoimmune hepatitis, or with medication reactions.
Steroids should not be used nonspecifically with other general causes of ALF.
Acetaminophen toxicity should be treated aggressively with N-acetylcysteine
(see Chapter 102 Toxicologic Emergencies ). N-acetylcysteine may have some
benefit in certain groups of patients with non–acetaminophen-induced liver
failure, however evidence is lacking for its broad use in all cases of ALF.
Plasmapheresis has been used to perform the detoxification functions of the
liver, however evidence of its benefit on patient outcome is lacking. Finally, if
medical management has failed, patients may require liver transplant. Ten
percent to 15% of pediatric liver transplants in the United States are secondary
to ALF (see Chapter 125 Transplantation Emergencies ).
PORTAL HYPERTENSION
Portal hypertension is a clinical syndrome caused by either increased or
restricted blood flow within the portal vasculature leading to a splenomegaly
and the development of portosystemic blood vessel collaterals as blood is
shunted to the systemic vasculature. The disease becomes clinically
significant when effects of this elevation in pressure or collateral vessels
become evident. Most patients present with splenomegaly, however some will
also present with GI bleeding or ascites. The management of these
complications is important for the ED provider as they may be life
threatening.
