Tousif M. Pasha

Incidence and Risk Factors for Cholangiocarcinoma in Primary Sclerosing Cholangitis

Cholangiocarcinoma (CCA) is a dreaded complication of primary sclerosing cholangitis (PSC); however, marked variability in the incidence of CCA in PSC is reported. Furthermore, limited information exists on risk factors for the development of CCA in PSC. The aim of this study was to determine the incidence of CCA in patients with PSC and to evaluate baseline risk factors for the later development of CCA. From a previous study of the natural history of PSC, we identified 161 patients with PSC who did not have CCA at study entry. Patients were followed until a diagnosis of CCA was established, liver transplantation was performed, or death occurred. Patients were followed for a median of 11.5 yr (interquartile range 4.0–16.1 yr). Fifty-nine patients (36.6%) died, 50 patients (31.1%) underwent liver transplantation, and 11 patients (6.8%) developed CCA. The rate of CCA developing was approximately 0.6% per year. Compared to the incidence rates of CCA in the general population, the relative risk of CCA in PSC was significantly increased (RR = 1,560; 95%CI = 780, 2,793; p < 0.0001). On univariate analysis, a history of variceal bleeding (p < 0.001), proctocolectomy (p = 0.01), and lack of symptoms (p = 0.02) were significant risk factors for CCA with the Mayo Risk Score being marginally significant (p = 0.051). Multivariate analysis determined only variceal bleeding to be a significant risk factor for CCA (RR 24.2; 95%CI: 3.3–67.1). No association was found between the duration of PSC and the incidence of CCA. In conclusion, approximately 7% of PSC patients later developed CCA over a mean follow-up of 11.5 yr, which is dramatically higher than the rates in the general population. Variceal bleeding is a major risk factor for the later development of CCA.

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.

Impact of nutritional status on outcomes after liver transplantation.

Background. Poorpreoperative nutritional status has been reported to be associated with adverse outcomes after liver transplantation. Published data are, however, conflicting, with methods of preoperative nutritional assessment and postoperative outcomes varying between studies. Methods. We prospectively studied the predictive value of preoperative nutritional status for adverse outcomes after liver transplantation. Assessment of preoperative nutritional status included: body cell mass determination, subjective global assessment, anthropometry, handgrip dynamometry, biochemical and amino acid profile, Child’s score, and dual-energy x-ray absorptiometry. Death, intensive care unit (ICU) length of stay ≥4 days, hospital length of stay ≥15 days, blood usage ≥36 U of blood products, infection, rejection, and global resource utilization (an index of cost) greater than the median were considered poor outcomes. Results. Fifty-three patients were studied. Longer ICU stay was associated with lower handgrip strength (P <0.01) and lower aromatic amino acid levels (P <0.01). Longer total hospital stay and the development of infections were associated with lower branched chain amino acid levels (P <0.01 and <0.001, respectively). Acute cellular rejection was associated with lower total body fat (P <0.001) and higher triglyceride levels (P <0.02). Neither death nor higher global resource utilization was associated with any preoperative nutritional parameter. Conclusions. Lower preoperative handgrip strength and branched chain amino acid levels are associated with longer ICU stays and increased likelihood of posttransplant infections. In our program, in which nutritional support was provided to potential recipients exhibiting malnourishment, none of the measured nutritional parameters were associated with mortality or greater global resource utilization.

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.

Crohn's disease of the esophagus: Clinical features and outcomes

ObjectivesCrohn’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. MethodsPatients with esophageal Crohn’s disease evaluated at Mayo Clinic Rochester between 1976 and 1998 were identified. ResultsTwenty 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. ConclusionEsophageal 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.

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.