Extreme lymphoplasmacytosis and hepatic failure associated with sulfasalazine hypersensitivity reaction and a concurrent EBV infection--case report and review of the literature.

We present an unusual case of a patient with extreme lymphoplasmacytosis and hepatic failure in association with a reaction to sulfasalazine and a concurrent Epstein-Barr virus (EBV) infection. Sulfa drugs can cause a wide range of allergic and hypersensitivity reactions and occasionally can lead to a fulminant illness. In the case under discussion the patient had hepatotoxicity, skin rash, fever, and peripheral blood atypical lymphocytosis. Initial impressions suggested the possibility of a malignant lymphoproliferative disorder. Flow cytometry of peripheral blood and a bone marrow biopsy provided clear evidence for a reactive, polyclonal process as opposed to a malignant disorder. Cessation of the offending drug and administration of steroids led to dramatic improvement. This case illustrates that drug hypersensitivity reactions can be manifested by an extreme lymphocytoid leukemoid reaction.

Transjugular intrahepatic portosystemic shunt for treatment of bleeding ectopic varices with portal hypertension.

PURPOSE: In the setting of hepatic failure and portal hypertension, hemorrhage from stomal and rectal varices is a well-described problem. It has recently been suggested that transjugular intrahepatic portosystemic shunting may be useful in the therapy of bleeding from parastomal or anorectal varices in patients unresponsive to conservative therapy. METHODS: We retrospectively review our institution's experience of five patients with parastomal varices and seven patients with anorectal varices who underwent transjugular intrahepatic portosystemic shunting for hemorrhage refractory to conservative management between 1994 and 1998. RESULTS: The study group consisted of four Child's A, five Child's B, and three Child's C patients. The mean age of the patients was 60.3 (range, 37-85) years. Mean follow-up was 15 (range, 5-27) months. The mean portosystemic pressure gradient before transjugular intrahepatic portosystemic shunting was 17.4+/-3.1 mm Hg. After transjugular intrahepatic portosystemic shunting, the mean portosystemic pressure gradient was reduced to 5.8+/-1.8 mm Hg (P<0.05). Transjugular intrahepatic portosystemic shunting were successful in complete resolution of bleeding in all patients. Three patients had encephalopathic changes after transjugular intrahepatic portosystemic shunting. Two patients died within 30 days of transjugular intrahepatic portosystemic shunting of causes unrelated to the procedure. Four patients required shunt revision within one year of placement. CONCLUSION: The transjugular intrahepatic portosystemic shunting procedure is an effective modality in the therapy of cirrhotic patients with bleeding stomal or anorectal varices unresponsive to conservative management. There is an acceptable procedure-related morbidity and mortality.

The successful treatment of symptomatic, refractory hepatic hydrothorax with transjugular intrahepatic portosystemic shunt.

Hepatic hydrothorax is a rare complication of portal hypertension. Conservative therapy may be successful but refractory hepatic hydrothorax is not uncommon. Management of refractory hydrothorax is usually ineffective and can result in a worsened clinical status. Transjugular intrahepatic portosystemic shunts (TIPS) lower portal pressure and have been used in the treatment of refractory ascites. The aim of this study was to determine the efficacy of TIPS in the treatment of symptomatic refractory hepatic hydrothorax. A TIPS was placed in 24 consecutive cirrhotic patients with symptomatic refractory hepatic hydrothorax. Five patients (20.8%) were Child's/Pugh class B and 19 (79.2%) were class C. All had undergone multiple thoracenteses and were hypoalbuminemic. Mean follow-up was 7.2 months (range, 0.25-49 months). Fourteen (58.3%) of 24 patients had complete relief of symptoms after shunt placement and did not require further thoracentesis. Five (20.8%) additional patients required fewer thoracenteses. Five (20.8%) patients developed worsening liver function and died within 45 days. In eight (66.7%) of 12 patients with > or = 60 days of follow-up, the serum albumin increased by a mean of 1.2 g/dL (range, 0.1-2.2 g/dL). The Child's-Pugh score improved in 7 (58.3%) of these 12 patients and two patients improved from class C to class A. These two patients no longer require liver transplantation. This study shows that TIPS can be effective in the management of symptomatic, refractory hepatic hydrothorax. Clinical and laboratory improvement may be seen and liver transplantation may become unnecessary.

Severe ascites: efficacy of the transjugular intrahepatic portosystemic shunt in treatment.

PURPOSE: To evaluate the transjugular intrahepatic portosystemic shunt (TIPS) as primary treatment in patients with cirrhosis and severe ascites. MATERIALS AND METHODS: A TIPS placement was attempted in 54 consecutive patients with intractable ascites. Clinical assessment findings, shunt patency, complications, and survival were analyzed. RESULTS: A TIPS was successfully placed in 50 patients (93%). Follow-up for clinical effectiveness in 51 patients was a mean of 285 days (range, 1-981 days). Forty patients (78%) gained clinical benefit from the shunt. Of these, 29 (57%) had a complete response (required no further paracentesis) and 11 patients (22%) had a partial response (required less frequent but additional paracentesis for control of ascites). The absence of preprocedure renal insufficiency (creatinine < 1.5 mg/dL [< 130 mumol/L]) was the only characteristic identified as an indicator of clinical success (P < .05). Eleven patients (22%) required shunt revision during follow-up to gain or prolong control of symptoms. Cumulative survival in the population evaluated for clinical efficacy was 53% at 6 months and 48% at 1 year. A complete response was the only variable that indicated increased survival (P < .05; R2 = 12%) with a 6-month survival rate of 76% and a 1-year rate of 71%. CONCLUSION: TIPS placement appears to be effective as a primary treatment of patients with cirrhosis and severe ascites.

n-3 fatty acids decrease colonic epithelial cell proliferation in high-risk bowel mucosa.

The n-3 fatty acids (C20:5, eicosapentaenoic acid; c22:6, docosahexaenoic acid) may be important in the development, growth, and metastasis of colon cancer, a leading cause of death in North America. Patients who have had a bowel neoplasm have a high risk of developing a second neoplasm, and this risk is associated with a high percentage of cells correspond to the S phase of bromodeoxyuridine (BrdUrd) labeling in mucosal epithelial cells. To determine the effect of n-3 fatty acid supplementation on DNA synthesis of rectal mucosa, patients with stage 1 or stage 2 colon carcinoma or adenomatous polyps were randomized to consume either 9 g/d n-3 fatty acid capsules or 9 g/d placebo capsules. Plasma phospholipid fatty acid analysis and proctoscopic mucosal biopsies were performed at baseline, 3, and 6 mon. Colonic crypts were isolated from the mucosa, disassociated with enzymes, and incubated with BrdUrd, and %S phase was measured by flow cytometry. The plasma phospholipid n-6/n-3 ratio was determined by gas chromatography. Supplement compliance was assessed by plasma phospholipid n-6/n-3 ratio. Mean capsule consumption in these two group was 82%. Prior to supplementation, there were no significant differences in the %S phase and the plasma n-6/n-3 ratio between these groups. Patients whose colonic epithelial cells indicated hyperproliferation at baseline showed a strongly positive correlation to the %S phase of BrdUrd uptake and the n-6/n-3 ratio. There was no significant change after n-3 treatment in patients with low baseline. Those in the placebo group showed no significant difference in n-6/n-3 ratio, although there was an increase in the %S phase of BrdUrd uptake at 6 mon. The n-3 group did not have significant side effects, and polyps were not found after completing 12 mon of n-3 fatty acid supplementation. This study suggests that n-3 fatty acid may be a useful chemopreventive agent in some patients as reflected in a plasma biomarker of colon tumor growth and metastasis. A low plasma phospholipid n-6/n-3 fatty acid ratio may serve as a nutritional marker that is associated with colonic epithelial cell hyperproliferation in the n-3-supplemented group as compared with the placebo group. Characteristics of mucosal proliferation at baseline may be a crucial factor for the effect of n-3 fatty acid supplementation.

Hepatitis C infection: a rare cause of fulminant hepatic failure.

Fulminant hepatic failure is caused by a variety of viruses, toxins, and metabolic derangements. The hepatitis C virus (HCV) causes indolent development of cirrhosis and has not been associated with fulminant hepatic failure. We report the first documented case of fulminant hepatitis C in the United States. The patient developed jaundice and stage IV encephalopathy. Initial laboratory evaluation did not reveal the etiology. The patient survived without liver transplantation. Three wk later he was found to have a positive HCV RNA and anti-HCV antibody seroconversion. He continued to improve with alpha-interferon treatment and has normal liver function and a negative HCV RNA 15 months later.

Toxicity associated with severe inhalational and dermal exposure to dimethylacetamide and 1,2-ethanediamine.

We present the case of a worker who was accidentally exposed (inhalational and dermal routes) to the chemicals dimethylacetamide and ethylenediamine for 90 minutes in a confined space. Clinical effects included delirium, hallucinations, skin burns, cellulitis, bilateral conjunctivitis, hepatitis, secondary coagulopathy, rhabdomyolysis, and a grade 2 esophagitis. Urinary monomethylacetamide levels 6 days after the exposure were 61 ppm.