Resolution of pancreatico-pleural fistula with endoscopic ultrasound-guided therapy.

Pancreatico-pleural fistula is an uncommon cause of recurrent pleural effusion. Delayed diagnosis may occur if fluid amylase level is not obtained early in the clinical course. As most cases of pancreatico-pleural effusion are due to chronic pancreatitis, endoscopic therapy may be effective if pancreatic fluid secretion can be diverted to a more physiologic pathway. However, when severe pancreatitis leads to disconnection of the pancreatic duct, it precludes conventional endoscopic treatment via transpapillary stenting of the pancreatic duct. We describe a patient with a chronic, refractory pancreatico-pleural fistula arising from chronic pancreatitis with a disconnected pancreatic duct syndrome, which resolved following endoscopic ultrasound-guided therapy.

Long-term versus short-term treatment with recombinant interferon alfa-2a in patients with chronic hepatitis B: a prospective, randomized treatment trial.

We conducted a prospective, randomized trial to study the efficacy and tolerance of long-term versus short-term treatment with recombinant interferon alfa-2a in patients with chronic hepatitis B. Ten patients were randomly assigned to a 6-month interferon regimen, and 10 patients were assigned to a 3-week interferon trial. Eleven patients (five assigned to long-term treatment and six to short-term treatment) did not complete interferon therapy: eight had either severe thrombocytopenia or neutropenia; one had pronounced fatigue in relationship to administration of interferon; one had spontaneous bacterial peritonitis and sepsis and died; and one had a massive fatal variceal hemorrhage during interferon therapy. Most of the serious hematologic complications occurred in patients with cirrhosis and hypersplenism. In one patient, seroconversion to hepatitis B virus DNA negativity occurred before the onset of treatment. Four of the five patients able to complete the 6-month interferon regimen and only one of four patients able to complete the 3-week trial had seroconversion to hepatitis B virus DNA negativity. Thus, we conclude that the therapeutic response was better among patients who were able to complete a 6-month interferon trial. In patients with cirrhosis and hypersplenism, development of either severe thrombocytopenia or leukopenia associated with interferon therapy precluded completion of treatment.

Endoscopic laser therapy for watermelon stomach.

Thirteen patients (9 women, 4 men) with anemia from acute and chronic gastrointestinal bleeding were found to have antral vascular disease consistent with watermelon stomach. The median age was 73 yr, with a range of 54-88 yr. Eight of the patients were transfusion-dependent, requiring a median of 5.5 U within the 12 mo before treatment. All were treated with endoscopic neodymium:yttrium-aluminum-garnet laser coagulation. Endoscopic and hematologic improvement were evident in 12 patients available for follow-up after a median period of 6 mo. There was a median increase of 4 g/dl in hemoglobin concentration; thus, the need for transfusion was eliminated. No major complications were encountered. Laser coagulation for watermelon stomach appears to be safe and efficacious and may be a therapeutic alternative for this disorder.

L-carnitine: effect of intravenous administration on fuel homeostasis in normal subjects and home-parenteral-nutrition patients with low plasma carnitine concentrations.

We studied the effects of intravenous L-carnitine on the metabolism of fatty acids, ketone bodies, glucose, and branched-chain amino acids in four normal volunteers and four patients on long-term home parenteral nutrition (HPN) with low plasma carnitine concentrations. Substrate kinetics were determined by use of [1-14C]palmitate, [3,4-13C2]-acetoacetate, [6,6-2H2]glucose, and [5,5,5-2H3]leucine before and during a 3-h intravenous infusion of L-carnitine. HPN patients were restudied after 1 mo of nightly intravenous carnitine administration. HPN patients tolerated the short-term fast well, exhibiting neither hypoglycemia nor hypoketonemia. Intravenous carnitine had no effect on rates of fatty acid oxidation, ketone body production, glucose production, or leucine kinetics in either group. Routine addition of carnitine to the HPN regimen does not appear to be necessary. The failure of L-carnitine administration to have discernable effects on intermediary metabolism in normal volunteers casts doubt on its role in the treatment of a variety of medical conditions.

Mucosal vascular malformations of the gastrointestinal tract: clinical observations and results of endoscopic neodymium: yttrium-aluminum-garnet laser therapy.

Ninety-three consecutive patients with transfusion-dependent gastrointestinal bleeding from vascular malformations (VMs) underwent systematic assessment in our gastrointestinal laser suite with extended upper gastrointestinal endoscopy and colonoscopy. Of these patients, 83 had angiodysplasia and 10 had the Osler-Weber-Rendu (OWR) syndrome. The median age in each of these groups was 70 and 63 years, respectively. Cardiovascular disease, especially valvular disease, was common. A poor correlation existed between the results of endoscopically identified VMs and visceral angiography in patients with angiodysplasia. A combination of upper and lower gastrointestinal VMs was found in 11% of patients with angiodysplasia and 60% of those with the OWR syndrome. All identified VMs were completely obliterated by photocoagulation with use of a neodymium:yttrium-aluminum-garnet (Nd:YAG) laser. Bleeding was successfully controlled in 9 patients with the OWR syndrome and in 72 patients with angiodysplasia (range of follow-up, 1 to 39 months). In 243 laser treatments, 3 perforations and 5 episodes of delayed bleeding occurred. This experience demonstrates that extended upper endoscopy is useful in identifying VMs and that gastrointestinal bleeding from VMs can be safely and successfully controlled with use of endoscopic Nd:YAG laser therapy.

L-carnitine therapy in home parenteral nutrition patients with abnormal liver tests and low plasma carnitine concentrations.

Persistent abnormalities of liver function tests occur in approximately 15% of home parenteral nutrition (HPN) patients and are associated with steatosis, steatohepatitis, and, rarely, fibrosis or cirrhosis. Approximately one-third of patients with gut failure on long-term HPN have low total and free plasma carnitine concentrations, and it has been suggested that a deficiency of L-carnitine may be responsible for the steatosis and steatohepatitis in HPN patients. To determine whether administration of L-carnitine is capable of reversing steatosis in HPN patients, 4 adult women on HPN for a mean of 53 mo (range 21-80 mo) were studied before and after 1 mo of intravenous L-carnitine supplementation (1 g/day). All patients had abnormalities in standard liver function tests and low total and free plasma carnitine values. The mean total and free plasma carnitine concentrations and the mean total hepatic carnitine concentration were reduced before supplementation and rose to normal values after treatment (27.4 +/- 2.3 to 35.5 +/- 3.1 nmol/ml, 19.4 +/- 2.8 to 25.7 +/- 2.5 nmol/ml, and 3.5 +/- 0.65 to 6.5 +/- 1.2 nmol/mg of noncollagen protein, respectively). However, there were no significant changes in mean serum aspartate aminotransferase and alkaline phosphatase levels (65 +/- 21 vs. 54 +/- 12 IU and 429 +/- 220 vs. 472 +/- 224 IU, respectively), plasma free fatty acids, plasma triglycerides, hepatic free fatty acid and triglyceride concentrations, or the grade of hepatic steatosis on light microscopy. These results suggest that carnitine deficiency is not a major cause of steatosis and steatohepatitis in patients receiving HPN.

Plasma carnitine levels in patients receiving home parenteral nutrition.

Patients on long-term home parenteral nutrition (HPN) are known to frequently develop hepatic steatosis or steatohepatitis. The etiology of this steatosis or steatohepatitis is unknown, but carnitine deficiency has been one of the postulated mechanisms. The importance of L-carnitine in hepatic fatty acid oxidation and the steatosis observed in primary and acquired carnitine deficiencies prompted us to determine plasma carnitine levels in 37 patients receiving long-term HPN. Thirteen patients (35%) had low total and free plasma carnitine levels. Fifteen of the 37 HPN patients were matched for age and sex with 15 patients with Crohn's disease who did not require HPN. Mean total and free plasma carnitine values were significantly lower (p less than 0.001) in these 15 HPN patients (32.2 +/- 11.9 and 28.4 +/- 10.8) when compared to Crohn's patients not requiring HPN (49.1 +/- 10.9 and 46.4 +/- 11.5). Associations were not detected between plasma carnitine and clinical or biochemical parameters that might have explained the low values.

Utility of the plain abdominal radiograph for diagnosing ureteral calculi.

Our study was designed to measure sensitivity, specificity, predictive value, and accuracy of the plain abdominal radiograph (PAR) for diagnosing ureteral calculi (UC) in our emergency department (ED), and to determine if obtaining this test in the ED provides clinically useful diagnostic information. Records of 206 adult patients (147 men, 59 women) who underwent emergency excretory urography as part of an ED evaluation for possible ureteral stones were reviewed. A retrospective scoring index was devised to estimate the clinical probability of a ureteral stone for each patient. All patients had a PAR shortly before the excretory urogram (EXU) was performed. Each PAR was separated from the corresponding EXU, and both were independently reviewed for radiographic evidence of ureterolithiasis. The results of the PAR and EXU interpretations were compared. There were 92 true-positive PARs (45%), 19 false positives (9%), and 39 true negatives (19%), yielding a sensitivity of 62% and a specificity of 67%. In patients with a high estimated clinical probability of a ureteral calculus, the PAR had a positive predictive value of 86% and a negative predictive value of 22%. In the remaining patients, the positive and negative predictive values of the PAR were 69% and 72%, respectively. Each patient's clinical index score was compared to his or her EXU results. For detecting UC, the sensitivity of our scoring index was 82%, and the specificity was 51%; the positive predictive value of the scoring index was 82%, and the negative predictive value was 52%.(ABSTRACT TRUNCATED AT 250 WORDS)

Does long-term home parenteral nutrition in adult patients cause chronic liver disease?

Sixty patients with gut failure were treated with home parenteral nutrition for 2000 patient months. Fifty-one of these 60 patients had either no abnormalities or mild and transient elevations of their liver chemistries and did not have liver biopsies. Nine (15%) of 60 patients had abnormalities of liver tests that persisted from 8 to 95 months (median, 18 months) which prompted one or more liver biopsies per patient. Three patients had prolonged jaundice, one died of hepatic encephalopathy, and another with protracted intrahepatic cholestasis died following a biliary tract exploration. A third patient remains ill with signs and symptoms of chronic liver disease. Steatohepatitis was found in eight of the nine patients and was characterized by centrilobular and midzonal microvesicular and macrovesicular fatty changes with fat cysts, focal necrosis, and mixed inflammatory infiltrates. Centrilobular fibrosis was present in three patients and evidence of nodular regeneration in one. In the three patients demonstrating cholestasis, bile pigment was identified both in hepatocytes and canaliculi. Ceroid pigment in Kupffer cells was a consistent finding and much more severe than expected from the mildness of the hepatitis. Persistent abnormalities of liver chemistries in nine patients and progressive liver disease while receiving home parenteral nutrition in three patients are quite worrisome and suggest that home parenteral nutrition-associated steatohepatitis with or without cholestasis may progress to chronic liver disease.

Pulmonary infiltrates and eosinophilia associated with sulfasalazine.

Asymptomatic pulmonary infiltrates and eosinophilia developed in a patient with chronic ulcerative colitis 1 month after therapy with sulfasalazine had been instituted. The abnormalities resolved completely after use of the drug was discontinued. The sulfapyridine component of the sulfasalazine was the likely causative agent because 41 years earlier, the patient had experienced fever, myalgias, and eosinophilia after taking a sulfonamide. Ten previous cases of sulfasalazine pulmonary toxicity, including one fatality, have been reported.