An unusual cause of meningitis.

A 57 year old man patient presented with fever and frontal headache. He had a background history of sero-positive rheumatoid arthritis which was well controlled on immunomodulatory disease modifying anti-rheumatoid drugs (DMARDS) including methotrexate and lef lunomide. Six months earlier he had returned from Massachussetts in the USA after a one year period of residence there. On examination his vital signs were within normal limits and he was afebrile with a temperature of 36.1oC. His left elbow joint was warm, tender and swollen; examination was otherwise normal.

Crohn's disease of the esophagus: clinical features and outcomes.

OBJECTIVES: Crohn's disease of the esophagus is rare. We sought to determine the clinical features and outcome of patients with esophageal Crohn's disease seen at our institution. METHODS: Patients with esophageal Crohn's disease evaluated at Mayo Clinic Rochester between 1976 and 1998 were identified. RESULTS: Twenty patients (0.2%) with esophageal involvement were identified. Median age at diagnosis was 31 years (range, 7-77 years). Eleven patients (55%) were female. Extraesophageal Crohn's disease preceded or was found at the same time as the diagnosis of esophageal Crohn's in all cases. Sixteen patients (80%) had symptoms referable to the esophagus. Endoscopic findings included ulcers in 17 (85%), erythema or erosions in 8 (40%), and strictures in 4 patients (20%). One patient had a fistula. The most common histological findings were active chronic inflammation (75%) and ulcer (30%). No granulomata were identified. Approximately one-half of our patients improved with first-line therapy. Eleven patients (55%) received immune modifier therapy. Six showed significant improvement on azathioprine, 6-mercaptopurine, or cyclosporine. Esophageal dilatation was required in six patients, and three patients required surgery. CONCLUSION: Esophageal Crohn's disease may be underdiagnosed. Patients with Crohn's disease complaining of esophageal symptoms should undergo upper endoscopy with biopsies, and the diagnosis of esophageal Crohn's disease should be entertained if aphthous or deep ulcers or strictures are present. Immune modifier therapy should be considered for steroid-dependent and steroid-resistant cases.

Gastrointestinal basidiobolomycosis in Arizona: clinical and epidemiological characteristics and review of the literature.

Gastrointestinal basidiobolomycosis (GIB) is an unusual fungal infection that is rarely reported in the medical literature. From April 1994 through May 1999, 7 cases of GIB occurred in Arizona, 4 from December 1998 through May 1999. We reviewed the clinical characteristics of the patients and conducted a case-control study to generate hypotheses about potential risk factors. All patients had histopathologic signs characteristic of basidiobolomycosis. Five patients were male (median age, 52 years; range, 37--59 years) and had a history of diabetes mellitus (in 3 patients), peptic ulcer disease (in 2), or pica (in 1). All patients underwent partial or complete surgical resection of the infected portions of their gastrointestinal tracts, and all received itraconazole postoperatively for a median of 10 months (range, 3--19 months). Potential risk factors included prior ranitidine use and longer residence in Arizona. GIB is a newly emerging infection that causes substantial morbidity and diagnostic confusion. Further studies are needed to better define its risk factors and treatment.

Utility of standard nutritional parameters in detecting body cell mass depletion in patients with end-stage liver disease.

Protein-calorie malnutrition, best measured by body cell mass (BCM) depletion, has been associated with adverse outcomes in patients with end-stage liver disease. We prospectively measured BCM and multiple standard nutritional parameters in patients with end-stage liver disease to determine which, if any, of the traditionally measured nutritional parameters correlate with BCM. A detailed nutritional assessment, including BCM analysis, subjective global assessment, anthropometry, handgrip dynamometry, laboratory tests, and body composition measured by dual-energy X-ray absorptiometry was performed in 69 sequential patients awaiting liver transplantation. The frequency of abnormalities of specific parameters of nutritional status varied between 19% and 99%. Most of the commonly measured parameters of nutritional status correlated poorly with BCM. Patients with depleted BCM (lowest quartile for sex) had midarm circumference (P <.01), arm-muscle circumference (P <.001), handgrip strength (P <.001), blood urea nitrogen (P <.01), and creatinine (P <.01) values less than those for patients with greater BCM (highest 3 quartiles for sex). In multivariate analysis, arm-muscle circumference and handgrip strength were the best predictors of BCM. The combined criteria of handgrip strength less than 30 kg and arm-muscle circumference less than 23 cm have a sensitivity of 94% and a negative predictive value of 97% in identifying patients with depleted BCM. Although abnormalities of nutritional parameters are highly prevalent among patients with end-stage liver disease, most parameters of nutritional status do not correlate with BCM. In patients with end-stage liver disease, arm-muscle circumference and handgrip strength are the most sensitive markers of BCM depletion.

Endoscopic perforation of the colon: lessons from a 10-year study.

OBJECTIVES: To assess the incidence, clinical features, and management of endoscopic colon perforations in a large number of patients at a major medical teaching center. METHODS: A retrospective review of medical records of all patients with colon perforations from endoscopy over a 10-yr period. RESULTS: A total of 10,486 colonoscopies were performed over a 10-yr period. There were 20 (0.19%) perforations and two (0.019%) deaths related to colonoscopy and two perforations with no deaths in 49,501 sigmoidoscopies (0.004%). The majority of perforations (65%) occurred in the sigmoid colon. The mean age of the patients was 72 yr (range, 48-87 yr). Multivariate analysis using gender and age showed that female gender was an independent predictor of a higher risk of perforation (p < 0.05). Electrocautery injury (36%) and mechanical injury (32%) from the tip and shaft of the endoscope were the major causes for perforation. Most patients (91%) presented within 48 h of endoscopy. Nine patients (47%) required a surgical resection with primary anastomosis; seven (37%) required a simple closure. The average hospital length of stay was 7.7 +/- 2.8 days. Although trainee endoscopists were involved in only 20% of the colonoscopies performed, eight (40%) perforations occurred while the training fellow was involved in the case. However, this increased risk of perforation with a training fellow was not statistically significant (p = 0.625). CONCLUSIONS: Colonoscopy can result in significant morbidity and carries a small risk of death. Sigmoidoscopy has lower risk. The following situations may represent increased risk to colonoscopy patients: unusual difficulty in traversing the sigmoid colon; difficult examinations in female patients, and difficult examinations performed by trainee physicians.

A randomized prospective study comparing rigid to balloon dilators for benign esophageal strictures and rings.

BACKGROUND: The optimum choice of dilator (rigid vs. balloon) for benign esophageal strictures has not been well studied. The aim of this study was to compare the immediate relief of dysphagia and the incidence of repeat dilatation within the first year with the use of either a rigid (Savary) dilator or balloon dilator for benign lower esophageal strictures. METHODS: Patients with dysphagia found to have benign esophageal strictures during endoscopy were randomized to undergo dilation with a rigid (Savary) or a balloon dilator (Microvasive or Bard). The 1-year incidence of repeat dilatation was estimated by the Kaplan-Meier method. RESULTS: A total of 251 subjects were stratified at entry according to the type of stricture (peptic vs. Schatzki ring) and severity of stricture (mild vs. moderate/severe) and then randomized to either a Savary (n = 88), Microvasive (n = 81), or Bard (n = 82) dilator. There were no significant differences between the rigid dilator or the two balloons with regard to immediate relief of dysphagia or the need for repeat dilatation at one year. Patients with moderate/severe strictures required repeat dilatation at one year twice as often as those with mild strictures. There were no significant complications reported in these patients. CONCLUSIONS: Both rigid and balloon dilators are equally effective and safe in the treatment of benign lower esophageal strictures caused by acid reflux and Schatzki rings.

An open-label study of the safety and tolerability of converting stable liver transplant recipients to neoral.

Neoral is a new formulation of cyclosporine based on microemulsion technology, designed to provide increased and more reliable absorption of the medication. The aim of this study was to assess whether conversion from Sandimmune to Neoral provides safe and effective oral immunosuppression in stable liver transplant recipients. We studied 59 stable liver transplant recipients (being treated with prednisone, azathioprine, and Sandimmune). All patients were enrolled in an open-label study in which they were converted from Sandimmune to Neoral therapy at a dose ratio of 1:1. Thirty-nine patients underwent duct-to-duct bile duct anastomoses, and 20 underwent Roux-en-Y bile duct anastomoses. After conversion, the Neoral dosage was adjusted on the basis of trough levels measured at weeks 1, 2, 3, 4, 6, 8, and 12. To assess safety and tolerability, we prospectively obtained serial information, including laboratory data and information on side effects. Standard statistical methodology was used. A total of 59 patients (23 men, 36 women; mean age, 55 years; mean follow-up after liver transplantation, 5.7 years) completed 3 months of follow-up after conversion from Sandimmune to Neoral. There were 32 dosage changes; 22 (69%) required reduction of the Neoral dose. Mean cyclosporine trough levels remained above 100 ng/mL during the follow-up period. There were no significant differences between cyclosporine levels in patients with duct-to-duct or Roux-en-Y bile duct anastomoses. There were no episodes of rejection during the 3-month follow-up period. The side effect profile was similar in both groups, except for a significant reduction in the number of patients with headaches after Neoral conversion. Liver transplant recipients can safely be converted from Sandimmune to Neoral. Neoral was well tolerated in this population.

The evolving role of endoscopic retrograde cholangiography before and after cholecystectomy.

Laparoscopy is the preferred approach for cholecystectomy; however the indications for pre- versus postoperative endoscopic retrograde cholangiography (ERC) are still evolving. The records of patients who had ERC performed one month before (n = 119) or after (n = 35) laparoscopic or open cholecystectomy from January 1990 to August 1992 (period 1), and 66 patients who had preoperative ERC from November 1995 to October 1996 (period 2) are reviewed, ERC indications, findings and outcomes were reviewed, and trends in the use of preoperative ERC from 1990 to 1996 were sought. Between periods 1 and 2 the yield of preoperative ERC increased from 36% to 58% (P < 0.01). The use of preoperative ERC for the indication of abnormal liver enzymes declined (P < 0.05), while the yield increased (25% versus 61%, P < 0.01). Both the use of ERC and the yield remained stable for other indications. ERC was felt to have changed the surgical approach in 5% of patients. Primary indications for postoperative ERC (n = 35) included abnormal intraoperative cholangiography (43%), liver function test abnormalities (23%) and recurrence of preoperative symptoms (14%); stones were found in 60%, 50% and 20% of patients with these indications, respectively. Overall, 46% of postoperative studies revealed duct stones. The roles for pre- and postoperative ERC are changing and depend greatly on the relative laparoscopic and endoscopic expertise at a given institution.

Survival algorithms and outcome analysis in primary biliary cirrhosis.

The natural history of primary biliary cirrhosis (PBC) is one of slowly progressive cholestasis with liver damage, development of cirrhosis with its concomitant complications, and death unless the patient undergoes liver transplantation. Natural history studies have identified several variables associated with a decreased survival in patients with PBC. The course of the disease can be divided into three time periods: (1) a presymptomatic phase, probably lasting up to 20 years; (2) a symptomatic phase, with anicteric or mild jaundice, lasting up to 5 to 10 years; and (3) a preterminal or accelerated phase with marked jaundice, lasting up to 2 years. Since the course of the disease is one of slow progression leading to liver failure and death unless liver transplantation intervenes, several investigators have developed statistical models to predict survival. The ability to predict survival for individual patients with PBC has been valuable in the management of these patients, particularly in patient selection and timing of liver transplantation. In addition, survival estimates can be utilized in education and counseling patients and their families. These models may also be used to evaluate the efficacy of new treatments by comparing natural history survival with the survival achieved by therapeutic effect. Over the past several decades, the natural history models of PBC have been developed in the absence of effective medical therapy. The efficacy of liver transplantation and survival following liver transplantation has now been quantitatively established. Future efforts should be aimed at determining not only survival of patients with primary biliary cirrhosis in the presence of effective medical therapy but also at assessing the quality of life and cost-effectiveness of medical therapy and liver transplantation in the management of patients with primary PBC.

Basidiobolomycosis: an unusual fungal infection mimicking inflammatory bowel disease.

Basidiobolus ranarum is a fungus belonging to the Entomophthoraceae family and is mainly associated with subcutaneous soft tissue infection. The disease is usually characterized by an insidious onset of massive induration of the subcutaneous tissue involving the limbs, trunk, or buttocks. Most cases of basidiobolomycosis have been reported from Africa, South America, and tropical Asia. Visceral involvement is extremely rare. Only 4 cases with involvement of the gastrointestinal tract, including 1 fatal case originating in the United States, have been well documented in the English-language literature. This case report describes the first successfully treated patient residing in the United States who had B. ranarum infection involving the gastrointestinal tract.

Diagnosis and therapy of cholestatic liver disease.

The majority of cholestatic liver diseases can be diagnosed with a carefully performed history taking, physical examination, and appropriate imaging studies. In a minority of cases, however, liver biopsy may be necessary to establish the diagnosis. In addition to the treatment of the specific liver disease, therapy should address the management of complications unique to cholestasis and progressive liver failure.