0521779407-17 CUNY1086/Karliner 0 521 77940 7 June 13, 2007 7:57
Porphyria, Acute Portal Hypertensive Bleeding 1195■Reverse fasting state by giving carbohydrate, by mouth if possible,
otherwise as D10W by vein; monitor for hyponatremia.
■Give analgesia; generally parenteral opiates (meperidine, fentanyl).specific therapy
■If seizures present, control with short-acting benzodiazepine (e.g.,
i.v. diazepam) or magnesium.
■If neurological signs present or pain persists over 24–48 hours, start
Panhematin (Ovation Pharmaceuticals; 800-455-1141) by slow i.v.
push. Use a large peripheral vein or central line. Panhematin is sup-
plied as powder, which is reconstituted immediately prior to infu-
sion.follow-up
■Adequate hematin will produce a sharp drop in urinary PBG after 2–
3 days, and resolution of pain after 3–5 days. Discontinue infusions
when a clinical response is evident or after 8 days.
■Educate patient as to porphyria-inducing medications.
■Determine the type of acute porphyria (urine and stool porphyrins;
RBC uroporphyrinogen-1-synthetase), and offer genetic screening
to first-degree relatives.complications and prognosis
■Outlook for acute abdominal attacks is good, particularly if an incit-
ing agent is identified. Most genetic carriers have no symptoms pro-
vided they avoid inducing drugs. Spontaneous attacks can recur.
Psychosis, if present, resolves completely; chronic mental illness not
seen. Recovery from motor neuropathy is slow (many months) but
in many cases is complete.PORTAL HYPERTENSIVE BLEEDING
EMMET B. KEEFFE, MD
history & physical
History
■Likelihood of developing varices in patients with cirrhosis: 35–80%
■25–35% of cirrhotic patients with large varices will bleed
■risk of recurrent variceal bleeding:∼70% within 2 years of index bleed
■for each bleeding episode, mortality ranges 35–50%