0521779407-09 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:12
696 Hepatorenal Syndrome Hereditary Elliptocytosis (HE)■Diagnose and treat infection aggressively
■Discontinue any potential nephrotoxic therapy
■Avoid large volume paracentesis, as it may precipitate further reduc-
tion of the central blood volume; use only for tense ascites if the
rupture of an umbilical hernia seems likely
specific therapy
■No specific proven medical therapy is available (terlipressin effective,
but not yet licensed in U.S.)
■Catecholamines (midodrine alone, or with octreotide infusion, may
be beneficial based on limited studies; dose range of midodrine is
2.5 to 7.5 mg/d, increased up to 12.5 mg/d; octreotide may be admin-
istered 100 ug subcutaneously three times daily, and increased to
200 ug if needed; treatment course is 1–2 weeks
■Albumin infusion (1 g/kg) on the first day, followed by 25–50 g/d,
should be considered in all patients
■Evaluate for liver transplantation – the treatment of choice
■Dialysis is ineffective in the long-term management but may provide
support until a transplant is possible
follow-up
■Daily monitoring of renal function, central venous pressures, urine
output in type 1 HRS
■Serial monitoring of renal function in outpatient with type 2 HRS
complications and prognosis
Complications
■Renal failure with associated electrolyte and metabolic complica-
tions
■DeathPrognosis
■Survival in type 1 HRS is less than 20% 4 weeks after the diagnosis is
made, and less than 5% at 10 weeks.HEREDITARY ELLIPTOCYTOSIS (HE) AND HEREDITARY
PYROPOIKILOCYTOSIS (HPP)
PATRICK G. GALLAGHER, MD
history & physical
History
■history of anemia, jaundice, gallstones, splenectomy