Internal Medicine

0521779407-09 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:12

Hemophilia A and B Hepatic Encephalopathy 677

➣Acute management of bleeding episodes in inhibitor patients

Low titer inhibitor (<5 Bethesda units [BU]), infuse high doses

of concentrate to overcome inhibitor. Other options as below.

High titer inhibitor (>5 BU). Best option is recombinant FVIIa,

activates FX directly. Other options include activated pro-

thrombin complex concentrates.

➣Long-term management of inhibitors

Immune tolerance regimen, daily infusion of high doses

of FVIII results in disappearance of inhibitor in 70–90% of

inhibitor patients, but therapy may be required for up to 2

yrs. Success rate lower with FIX inhibitors.

Hepatic Encephalopathy...............................

ANDY S. YU, MD and EMMET B. KEEFFE, MD

history & physical

History

■risk factors for liver disease: excessive alcohol use, injection drug use,

blood transfusion, multiple sexual partners, Asian country of origin,

family history

■risk factors for portal hypertension: cirrhosis

Physical Signs

■stigmata of chronic liver disease: gynecomastia, jaundice, spider

nevi, firm liver, splenomegaly, ascites, testicular atrophy, palmar ery-

thema,

■fetor hepaticus, altered consciousness, wide range of neuropsychi-

atric presentations

■staging of HE

➣Stage 1: impaired attention, personality change, sleep reversal

➣Stage 2: drowsiness, disorientation, confusion

➣Stage 3: stupor, somnolence, but arousable

➣Stage 4: unresponsiveness, coma

■movement disorders nonspecific for HE, including asterixis, hyper-

reflexia, hypertonia, extensor plantar response, slow monotonous

speech

tests

■diagnosis of HE is clinical and is based on (1) identifying significant

liver dysfunction, and (2) excluding other causes of CNS dysfunction