Comparison of clinical efficacies and safeties of lumen-apposing metal stent and conventional-type metal stent-assisted EUS-guided pancreatic wall-off necrosis drainage: a real-life experience in a tertiary hospital.

BACKGROUND: Endoscopic ultrasound (EUS)-guided drainage of pancreatic wall-off necrosis (WON) with transmural stent is regarded as firstline therapy. We aimed at comparing its efficacy and safety with using fully covered self-expandable metal stent (FCSEMS) and lumen-apposing metal stent (LAMS). METHODS: A retrospective review was performed on all consecutive patients with pancreatic WONs who underwent EUS-guided drainage by either FCSEMS or LAMS. RESULTS: From 2011 to 2016, 68 patients (66.2% male, median age, 66.5 years) underwent WON drainage (22/68 (32.4%) using FCSEMSs of size 10 × 60 mm (14/22, Hanarostent; 8/22 Wallflex); 46/68 (67.6%) using LAMSs (38/46 and 8/46 with AXIOS of size 15 × 10 mm and 10 × 10 mm, respectively). These two groups were matched for age (66 vs. 70 years, p 0.514), APACHE II (11.5 vs. 10, p 0.693), causes [72.7 vs. 80.4% by gallstone pancreatitis (p 0.472); 9.1 vs. 10.9% by alcoholism (p 0.818)], WON size (8.5 vs. 9 cm, p 0.322), location (36.4 vs. 26.1% at pancreatic head, p 0.384; 54.5 vs. 65.2% at body/tail, p 0.395), and enterostomy site [63.6 vs. 76.1% via transgastric (p 0.285); 31.8 vs. 19.6% via transduodenal (p 0.267)] and their number of necrosectomy (p 0.978). The technical (100 vs. 93.5%, p 0.219) and clinical (95.5 vs. 93.5%, p 0.749) success and adverse event (22.7 vs. 39.1%, p 0.180; 9.1 vs. 19.6% with bleeding, p 0.271; 4.5 vs. 13% with spontaneous stent migration, p 0.28; 9.1 vs. 6.5% with dislodgement during necrosectomy, p 0.704) of the two groups were comparable without significant different. However, the LAMS group associated with early stent revision compared with FCSEMS group (log rank p 0.048). CONCLUSIONS: EUS-guided drainage of WON using FCSEMSs and LAMSs are comparable in efficacy and safety; however, the latter is associated with early stent revision.

Acquired Fanconi syndrome associated with prolonged adefovir dipivoxil therapy in a chronic hepatitis B patient.

Adefovir dipivoxil (ADV) is one of the commonly used antiviral agents in the treatment of chronic hepatitis B infection. Nephrotoxicity is dose related and occurred at a daily dosage of >30 mg. However, it is now increasingly recognized that nephrotoxicity can occur at a daily dose of 10 mg. We present a case of acquired Fanconi syndrome in a patient with chronic hepatitis B who had been treated with ADV for 4 years. She presented with progressive muscle weakness and generalized bone pain. The laboratory results showed the feature of proximal renal tubule dysfunction, particularly severe hypophosphatemia. Diagnostic approach to hypophosphatemia and proximal renal tubular dysfunction is discussed. After switching over from ADV to entecavir, her symptoms and laboratory findings returned to normal. Acquired Fanconi syndrome can be associated with ADV at a conventional dosage, and therefore, patients treated with long-term ADV should have regular monitoring of renal function and calcium and phosphate levels.

Clinical characteristics of concurrent and sequentially presented lupus-related protein-losing enteropathy: what are their differences?

Our objective was to compare patients with concurrent and sequentially presented systemic lupus erythematosus (SLE)-related protein-losing enteropathy (PLE). Patients with history of SLE admitted for PLE were selected and their clinical, laboratory, endoscopic and imaging characteristics, treatment and outcome were analyzed. From 2001 to 2010, 21 and 27 patients had concurrent and sequentially presented SLE-related PLE, respectively, and their clinical characteristics were comparable except the following: the concurrent group had more pleural effusion (P < 0.01), cutaneous (P < 0.03), neurological (P = 0.02) manifestations, higher creatine phosphokinase (127.6 IU/L vs. 105.7 IU/L, P < 0.05) and lactate dehydrogenase (504.0 IU/L vs. 422.2 IU/L, P < 0.05); whereas the sequential group had higher anti-double strand DNA titer (179.8 vs. 100.4, P < 0.05), 24-h urine protein excretion (1.1 g/d vs. 0.6 g/d, P < 0.05) and increased proteinuria after onset of PLE (0.21 g/d vs. 1.1 g/d, P < 0.04). The endoscopic, histological and radiological features were comparable between the two groups. More patients from the sequential group required more potent immunosuppressive therapy for induction (55.6% vs. 14.3%, P = 0.002) and maintenance (48.2% vs. 9.5%, P < 0.01).The concurrent group associated with better treatment outcomes, with requiring shorter mean time (4.5 months vs. 7.9 months, P = 0.03) for normalbuminemia and more individuals (90.5% vs. 63%, P < 0.02) achieving normalbuminemia in first year. The complications were infrequent: two drug-related adverse events from each group, one patient each from the concurrent group developed shingle and SLE nephropathy. PLE associated with concurrent and sequentially presented of SLE are comparable in clinical behavior; and the immunosuppressive therapy is generally well-responded and tolerated. However, the concurrent group is associated with better disease activity control.

Diagnostic challenges of tuberculosis peritonitis in patients with and without end-stage renal failure.

BACKGROUND: Information about diagnostic features of tuberculosis (TB) peritonitis among patients with end-stage renal failure (ESRF), compared with those without ESRF, is limited. Here, we review our experience with TB peritonitis in patients with and without ESRF over a period of 11 years, with special reference to the clinical features of and diagnostic tools for TB peritonitis. METHODS: The records of all patients with TB peritonitis who were seen at United Christian Hospital (Hong Kong) from 1996 through 2006 were reviewed. The diagnosis of TB peritonitis was based on (1) detection of mycobacteria on smear and/or Mycobacterium tuberculosis on culture of ascitic fluid and/or peritoneal biopsy specimens and/or (2) demonstration of caseating granulomata on histological assessment of peritoneal biopsy specimens. RESULTS: During the study period, 52 patients (19 without ESRF and 33 with ESRF) had TB peritonitis. Patients with ESRF tended to have more-acute onset of symptoms and neutrophil-predominant peritoneal fluid, which mimicked bacterial peritonitis. Diagnosis was made earlier among patients without ESRF than among those with ESRF. Laparotomy or laparoscopy was the initial diagnostic tool for 12 patients (63%) without ESRF, whereas mycobacterial culture was the diagnostic method for 18 patients (55%) with ESRF. CONCLUSIONS: TB peritonitis has nonspecific and protean manifestations. It should be considered in the context of culture-negative peritonitis and in the context of culture-positive peritonitis that fails to respond to appropriate antibiotics. Laparoscopy with biopsy should be considered at an early stage when TB peritonitis is suspected.

Comparison of efficacy and renal safety of telbivudine and entecavir in treatment-naive elderly patients with chronic hepatitis B.

BACKGROUND: Data comparing the clinical outcomes of telbivudine (LdT) and entecavir (ETV) in elderly patients with chronic hepatitis B are limited. GOALS: The aim of the present study was to compare the efficacy and renal safety of LdT and ETV in treatment-naive elderly (≥ 60 years) patients with chronic hepatitis B. METHODS: A total of 33 patients treated with LdT were consecutively enrolled in the study. Each patient was matched on the basis of age, sex, and baseline hepatitis B virus (HBV) DNA levels with three to four randomly selected controls treated with ETV. RESULTS: Clinical characteristics were comparable between the two groups. Higher cumulative HBeAg-seroconversion rates were observed in the LdT group than in the ETV group after 2 years (50 vs. 20%) and 3 years (50 vs. 26.67%) of treatment (all P<0.0001). Virological response rate at week 24 was significantly lower in the LdT group than it was in the ETV group (54.55 vs. 70.87%, P<0.0001), but no significant difference was noted in long-term cumulative rates of undetectable HBV DNA levels between the two groups (P=0.562). Virological breakthrough occurred in six (18.18%) LdT patients, with no such cases reported in the ETV group (P<0.0001). Antiviral resistance was strongly associated with LdT use and the absence of undetectable HBV DNA at weeks 12 and 24 (P<0.0001). During the study, significant improvement was observed in the estimated glomerular filtration rate and model for end-stage liver disease score in LdT versus ETV group. CONCLUSION: LdT has a lower clinical efficacy for viral suppression and a higher risk of antiviral resistance than does ETV. However, LdT resulted in higher HBeAg-seroconversion rates and better renoprotective effects than did ETV.

Comparison of clinical efficacy and renal safety of telbivudine and entecavir in chronic hepatitis B patients receiving cytotoxic chemotherapy.

OBJECTIVE: Limited data is available on the clinical outcomes of telbivudine (LdT) and entecavir (ETV) in pre-emptive antiviral chemoprophylaxis. This study aimed to evaluate the clinical efficacy and renal safety of LdT and ETV in patients with chronic hepatitis B (CHB) who received cytotoxic chemotherapy. METHODS: Altogether 290 treatment-naïve CHB patients undergoing intense chemotherapy were enrolled to receive daily 600 mg of LdT or 0.5 mg of ETV as pre-emptive antiviral chemoprophylaxis. RESULTS: The ETV group had significantly higher proportion of patients with undetectable hepatitis B viral (HBV) DNA load compared with LdT at week 24 (73.0% vs 50.3%, P = 0.000). The cumulative rates of virological breakthrough in the LdT and ETV groups were 9.15% and 3.65% at the second year of therapy, respectively (P = 0.059), which was associated with detectable HBV DNA at week 24 (P = 0.000). The MELD score of the LdT group was significantly lower than that of the ETV group after the first year (4.53 vs 7.53, P = 0.002) and the second year (1.96 vs 7.09, P = 0.000) of antiviral therapy. Moreover, the estimated glomerular filtration rate (eGFR) was significantly improved in the LdT group than in the ETV group after two years of antiviral therapy. CONCLUSION: LdT has a lower clinical efficacy in viral suppression than ETV, but LdT is associated with greater extent of improvement in liver and renal functions of patients in pre-emptive prophylaxis for cytotoxic chemotherapy.

Role of C-reactive protein in response-guided therapy of pyogenic liver abscess.

BACKGROUND: Protocols for antibiotic treatment of pyogenic liver abscess (PLA) are usually based on clinicians' own experience without any validation. Our study was to evaluate the clinical implication of C-reactive protein (CRP) in predicting treatment outcome and adequacy of antibiotic therapy of PLA. PATIENTS AND METHODS: Patients with PLA in whom white blood cell (WBC) count, erythrocyte sedimentation rate (ESR), and CRP were checked regularly during the clinical course were included. The prolife of CRP during the clinical course was compared with that of ESR and WBC. The usefulness of CRP in predicting the chance of recovery and adequacy of antibiotic therapy was examined. RESULTS: From 2000 to 2011, 109 patients with PLA underwent regular monitoring of WBC, ESR, and CRP. Except for ESR, both WBC and CRP showed an initial rapid reduction in first 3 weeks, followed by a relatively slow decrease. From week 3 to week 6, the CRP ratio (relative to CRP at week 1) of patients with and without adverse events (i.e. including mortality) was compared; a significant difference was found at week 3 (P=0.001), week 4 (P=0.004), week 5 (P=0.011), and week 6 (P=0.018), whereas no statistically significant difference was found in the WBC ratio over the same period. By week 3, a CRP ratio of 0.423 or less was a marker of good outcome (sensitivity 0.846; specificity 0.667) and was also a marker of adequacy of antibiotic therapy of 5 weeks or less (sensitivity 0.786; specificity 0.714) if the ratio was 0.278 or less. CONCLUSION: Weekly CRP measurement was useful in the identification of patients with PLA with good outcome and adequacy of antibiotic therapy of 5 weeks or less.

Gender-related differences in clinical course of crohn?s disease in an Asian population: a retrospective cohort review.

CONTEXT: Data from Asian populations about gender-related differences in Crohn's disease are few. Objectives This study was to analyze the clinical characteristics between women and men affected by Crohn's disease. METHODS: This was a retrospective cohort study to analyze consecutive Crohn's disease patients from Jan 2000 to Dec 2012. Clinical and phenotypic characteristics and treatment outcomes were evaluated. RESULTS: 79 patients (55 male and two of them with positive family history) were diagnosed with Crohn's disease. Ileocolonic disease and inflammatory lesion was the most dominant site of involvement and disease behavior respectively in both men and women. Apart from higher frequency of nausea (45.83 vs 23.64%, P 0.024) and lower body mass index (19.44 vs 22.03 kg/m2, P 0.003) reported in women, no significant gender-related differences in clinical characteristics were observed. Women were more associated with delay use of immunosuppressive therapy (12 vs 36 months, P = 0.028), particularly for those aged less than 40 years old (85 vs 62.6%, P = 0.023). Cox proportional hazard regression analysis revealed that active smoking (HR, 4.679; 95% CI, 1.03-21.18) and delayed use of immunosuppressive therapy (HR, 4.13; 95% CI, 1.01-16.88) were only independent risk factors associated with increased risk of complications. CONCLUSIONS: There were no significant gender-specific differences in clinical and phenotypic characteristics between male and female Crohn's disease patients. Smoking history and delay use of immunosuppressive therapy were associated with higher risk of complications.

Intestinal tuberculosis complicated with perforation during anti-tuberculous treatment in a 13-year-old girl with defective mitogen-induced IL-12 production.

Interleukin-12 (IL-12) is a cytokine which is secreted by activated phagocytes and dendritic cells and promotes cell-mediated immunity to intracellular pathogens, by inducing type 1 helper T cell (TH1) responses and interferon- γ (IFN- γ) production. Defects in the IL-12 may cause selective susceptibility to intracellular pathogens, such as mycobacteria. We herein report on a 13-year-old girl with defective mitogen-induced IL-12 production, who developed intestinal tuberculosis with wide dissemination involving the lung and urinary tract. She improved gradually, but developed terminal ileal perforation approximately 6.1 months following initiation of anti-tuberculous treatment. The paradoxical response phenomenon was suspected. The girl subsequently underwent surgical resection of the affected bowel segment with a temporary double barrel stoma, and ileocolonic anastomosis was performed after the completion of the anti-tuberculous therapy. The patient remained well, with no evidence of recurrent tuberculosis in the past 5 years. This case illustrates the possibility of underlying primary immunodeficiency in a patient with disseminated tuberculosis; delayed tuberculous intestinal perforation can develop during chemotherapy for tuberculosis.

Is there any difference in pyogenic liver abscess caused by Streptococcus milleri and Klebsiella spp?: retrospective analysis over a 10-year period in a regional hospital.

BACKGROUND/PURPOSE: To compare the clinical characteristics of patients with Streptococcus milleri (SM) and Klebsiella spp. associated pyogenic liver abscess (PLA). METHODS: A retrospective study of patients with PLA due to SM and Klebsiella spp. was conducted. Clinical characteristics, laboratory and radiological features, management and outcomes were analyzed. RESULTS: From 2000 to 2009 inclusive, 21 and 140 patients had SM and Klebsiella spp. associated monomicrobial infected PLA, respectively. A higher incidence of active malignancy occurred in the SM group (14.3% vs. 3.6%, p < 0.03). The common clinical features of the patients were fever, chill and right upper quadrant pain. A longer duration (6.3 vs. 4.4 day, p = 0.04) of symptoms and a higher incidence of hepatomegaly (14.3% vs. 2.9%, p < 0.01) occurred in the SM group. Common laboratory and imaging abnormalities included: anemia, leukocytosis, high erythrocyte sedimentation rate and C-reactive protein, hypoalbuminemia, elevated total bilirubin and alanine aminotransferase, right hepatic lobe involvement, hypoechoic in ultrasonograpghy, rim enhancement and septal lobulation in computed tomography. The biliary tract disorder was the most common cause of the disease in the two groups. Patients with Klebsiella spp. associated PLA tended to have more complications: bacteremia (61.6% vs. 31.6%, p < 0.01) septic shock (33.6% vs. 19%, p = 0.11), disseminated intravascular coagulation (20.7% vs. 4.8%, p = 0.04), metastatic infections (10.7% vs. 0%, p = 0.06), acute renal and respiratory failure (5% vs. 0%, p = 0.14). However, both were effectively managed by the combination of antibiotics and image-guided aspiration with/without drainage, and their mortality rates were comparable to each other. Those patients with metastatic infection might need a longer duration (6.07 vs. 5.32 week, p = 0.144) of antibiotic therapy, which was due to the longer mean duration (3.85 vs. 2.86, p < 0.04) of an intravenous counterpart. CONCLUSION: SM associated PLA tends to have a distinct clinical syndrome as compared with that of Klebsiella spp. with regard to risk factors, clinical manifestations and complications. However, both can be effectively treated with a combination of antibiotics and image-guided aspiration with/without drainage.

Older age as a poor prognostic sign in patients with pyogenic liver abscess.

BACKGROUND: Previous studies have focused on the role of age as a prognostic factor in pyogenic liver abscess (PLA) and results have been controversial. The aim of this study was to compare the clinical characteristics of PLA in elderly (age ≥65 years) and non-elderly patients in order to identify any differences so that an early diagnosis can be made and appropriate therapeutic measures can be instituted promptly. METHODS: We performed a retrospective analysis of patients with PLA. The demographic and clinical features, laboratory and imaging findings, management, and clinical outcomes of elderly and non-elderly patients were studied. RESULTS: Between January 2000 and December 2010 inclusive, 319 patients had PLA; 168 (52.7%) were aged ≥65 years. Elderly patients were more likely to have acute onset of symptoms (4.2 vs. 5.3 days, p < 0.04), co-morbidities, and respiratory symptoms (25.6% vs. 14.6%, p < 0.01). They also had lower serum bilirubin (28.1 vs. 37.1 µmol/l, p < 0.04), alanine aminotransferase (71.6 vs. 94.3 U/l, p < 0.02), and glycosylated hemoglobin (8.1% vs. 10%, p < 0.01), and more often had septal lobulation (64.3% vs. 54.3%, p < 0.04) and pneumobilia (10.1% vs. 4.0%, p < 0.02). Moreover, they required a longer duration of oral antibiotics (2.63 vs. 2.05 weeks, p < 0.01) and had a higher incidence of acute coronary syndrome during the illness (7.7% vs. 2.0%, p < 0.01). Gram-negative organisms were the dominant isolates in both groups, but the elderly had a lower incidence of Gram-positive infections (5.4% vs. 13.2%, p < 0.01). Lastly, old age was associated with local recurrence of PLA (odds ratio (OR) 3.1, 95% confidence interval (CI) 1.0-9.7, p < 0.04) and mortality (OR 3.17, 95% CI 1.25-8.04, p = 0.015). CONCLUSIONS: Elderly patients tend to have a more atypical presentation in PLA, for which clinicians should be on high alert. We found older age to be associated with a higher recurrence of PLA and a higher mortality rate.

Age-related differences in the clinical course of Crohns disease in an Asian population: a retrospective cohort review.

The aim of this study was to compare the clinical characteristics and treatment outcomes of patients with young and adult onset Crohns disease. Among 79 consecutive Crohns disease patients (11 (13.92%) with onset =16 years old), young onset Crohns disease was significantly associated with fever (36.36 vs. 14.71%, P 0.041), weight loss (72.7 vs. 29.4%, P 0.003), isolated abdominal pain (45.45 vs. 16.18%, P 0.013), lower body mass index ( 17.32 vs. 21.29 kgm2, P 0.019), and extraintestinal manifestation, particularly oral (45.5% vs. 22.1%, P 0.049) and perianal lesion (63.6% vs. 36.8%, P 0.046). In both groups, ileocolonic disease and inflammatory lesion were the most prevalent site of involvement and dominant disease behavior respectively. Their complication and bowel resection rate were similar but the former took a longer period of time to develop in the young onset group (84 vs. 24 month, P 0.018). Cox proportional hazard regression analysis revealed that active smoking and delayed use of immunosuppressive therapy were the only independent risk factors associated with increased risk of complications.

Spontaneous abdominopelvic abscess as an initial manifestation of Crohn's disease: a retrospective cohort review.

OBJECTIVE: The study aimed to compare the clinical characteristics of abdominopelvic (AP) abscess in patients with and without newly diagnosed Crohn's disease (CD). METHODS: We investigated 91 consecutive patients with AP abscess (14 newly diagnosed with CD) from January 2001 to December 2010. The clinical, radiological, microbiological and pathological features and patients' outcomes were evaluated. RESULTS: Patients with newly diagnosed CD were younger (42.5 vs. 57.9 years, P = 0.004), more associated with fever (50.0% vs. 13.0%, P = 0.004) and weight loss (21.4% vs. 3.9%, P = 0.045), and normoglycemic (5.2 mmol/L vs. 6.7 mmol/L, P = 0.001). Their abscesses, located predominantly at the paracolic gutter region (P = 0.002) were associated with fistulae (P = 0.001) and caused by transmural bowel wall inflammation. More patients in the CD group required temporarily parenteral nutritional support (P = 0.000) and had a longer hospital stay (P = 0.045) and a higher local recurrent rate (P = 0.000). CONCLUSION: AP abscesses associated with newly diagnosed CD display distinct clinical behavior with a high risk of local recurrence.

Is hepatic neoplasm-related pyogenic liver abscess a distinct clinical entity?

AIM: To compare the clinical characteristics of pyogenic liver abscess (PLA) in patients with and without hepatic neoplasm (HN). METHODS: Authors performed a retrospective analysis involving patients with PLA. The demographic, clinical features, laboratory and imaging findings, management and outcome of patients with and without HN were studied. RESULTS: From January 2000 to December 2009 inclusive, 318 patients (35 with HN) had PLA, and mean age and comorbidity were comparable between the two groups. More patients with HN experienced right upper quadrant pain (68.6% vs. 52.7%, P < 0.04), developed jaundice (14.3% vs. 5.7%, P < 0.03) and hepatomegaly (17.1% vs. 3.9%, P < 0.01), and had higher serum total bilirubin level (43.3 µmol/L vs. 30.0 µmol/L, P = 0.05). Most patients in both groups had PLAs in the right hepatic lobe, and biliary tract disorder was the most common underlying cause (71.4% and 61.8%). However, more PLAs in the HN group were associated with thicker abscess wall (37.1% vs. 19.4%, P < 0.01), septal lobulation (77.1% vs. 58%, P < 0.02), gaseous cavitation (17% vs. 7.8%, P = 0.03), portal thrombophlebitis (11.4% vs. 1.8%, P < 0.01) and aerobilia (25.9% vs. 5.5%, P < 0.01). Mixed bacterial growth (40% vs. 15.2%, P < 0.01) and Gram-negative bacilli (22.8% vs. 60.4%, P < 0.01) were dominant isolates in PLAs with and without HN, respectively. Although incidence of the complications was comparable between the two groups, patients with HN had a higher mortality rate than those without (71.4% vs. 8.8%, P < 0.01). Multivariate logistic regression analysis revealed underlying active malignancy [odds ratio (OR): 40.45, 95% CI: 14.76-111.65], hypoalbuminemia (OR: 1.22, 95% CI: 1.14-1.38), disseminated intravascular coagulation (OR: 3.32, 95% CI: 1.19-9.69) and acute coronary syndrome (OR: 4.48, 95% CI: 1.08-17.8) were independent risk factors associated with mortality. However, several HN cases, presented concurrently with PLAs, were found to have curative resectable tumors and had good prognosis after surgery. CONCLUSION: PLA associated with HN tends to form a distinct clinical syndrome with a different extent of clinical manifestations, radiological and microbiological features and complications.

A middle-aged lady with a pyogenic liver abscess caused by Clostridium perfringens.

The pyogenic liver abscess caused by Clostridium perfringens (C. perfringens) is a rare, but rapidly fatal infection. It is usually associated with malignancy and immunosuppression. We report the case of 50-year-old lady with the secondary liver metastases from rectal cancer presented with fever and epigastric pain. The identification of Gram-positive bacilli septicaemia, the presence of gas-forming liver abscess and massive intravascular hemolysis should lead to the suspicion of C. perfringens infection. Here we review twenty cases published since 1990 and their clinical features are discussed. The importance of "an aggressive treatment policy" with multidisciplinary team approach is emphasized.

A gentleman with an unusual cause of hypoalbuminemia.

Protein-losing enteropathy (PLE) is a rare syndrome of gastrointestinal protein loss that may complicate a variety of diseases. The primary causes can be divided into erosive gastrointestinal disorders, nonerosive gastrointestinal disorders, and disorders involving increased central venous pressure or mesenteric lymphatic obstruction. Herein, we report on a 65-year-old man with PLE caused by invasive gastrointestinal stromal tumor (GIST). To our best knowledge, this is the first reported association between GIST and PLE. A brief review of the literature on the incidence, pathogenesis and management of GIST is also presented.

Protein-losing enteropathy associated with or without systemic autoimmune disease: what are the differences?

OBJECTIVE: The aim of our study was to compare protein-losing enteropathy (PLE) associated with or without systemic autoimmune (SA) diseases. METHODS: Patients diagnosed with PLE were selected, and their clinical characteristics, laboratory, endoscopic and imaging characteristics, treatment, and outcome were analyzed. RESULTS: From 2001 to 2010, 74 patients (60 patients with SA disease) with a female predominance were diagnosed with PLE. The SA group tended to be younger, presented early (4.3 vs. 7 weeks, P=0.08), and had significantly more mucocutaneous-articular involvement (16.7 vs. 0%, P<0.05; 50 vs. 0%, P<0.02; 43.3 vs. 0%, P<0.01), compared with the other group, which showed more weight loss (64.3 vs. 25%, P<0.01), malaise and fatigue (57.1 vs. 28.3%, P<0.02), and tended to have more gastrointestinal (GI) symptoms. The SA group was associated with lymphopenia (0.8 vs. 2.7 × 109/l, P<0.01), hyperglobulinemia (43 vs. 31.2 IU/l, P<0.04), lactate dehydrogenase (511.1 vs. 393.5 IU/l, P<0.05), hematuria (48.3 vs. 7.1%, P<0.01), and pyuria (23.3 vs. 0%, P<0.03), whereas the non-SA group had a higher platelet count (402 vs. 262.5 × 109/l, P<0.01) and alkaline phosphatase (111 vs. 78.2 IU/l, P<0.03) on admission. A subgroup analysis of patients with SA disease showed that more lupus patients had pericardial effusion (14.6 vs. 0%, P=0.08), polyarthritis (50 vs. 16.7%, P=0.02), lower C3 level (0.5 vs. 0.85 mg/l, P<0.01), antinuclear factors (89.6 vs. 58.3%, P<0.01), and antiextractable nuclear antigen antibody (73.3 vs. 37.5%, P<0.03), whereas nonlupus patients had higher C-reactive protein (87.9 vs. 40 mg/l, P<0.01) and more antineutrophil cytoplasmic antibody (ANCA) (60 vs. 3%, P=0.00). Thirty-seven (71%) patients with SA disease had diffuse nonerosive erythematous GI mucosa with chronic inflammatory cells in the lamina propria layer, and 12 (85.7%) patients without SA disease had focal lesions. The treatment response was comparable between the two groups. However, the time required to normalize the serum albumin level (6.3 vs. 12.3 months, P=0.02) of patients with SA disease was much shorter than that of the non-SA group and those of inflammatory markers, specifically, C-reactive protein and complement C3, of its own group (6.3 vs. 11.6 vs. 12.1 months, P<0.04). More patients without SA disease had infective episodes during the management period (14.3 vs. 1.7%, P<0.01). CONCLUSION: Patients with PLE associated with SA disease tend to have a distinct clinical syndrome with regard to the extent of clinical manifestations and laboratory, endoscopic, and histological features compared with those without. Patients without SA disease are more prone to develop complications and mortality. However, both can be effectively treated with comparable treatment response.

Is pyogenic liver abscess associated with recurrent pyogenic cholangitis a distinct clinical entity? A retrospective analysis over a 10-year period in a regional hospital.

BACKGROUND: Recurrent pyogenic cholangitis (RPC) is recognized as an important cause of pyogenic liver abscesses (PLA). Although it is endemic to Southeast Asia, it is seen increasing in the west mainly owing to immigration. The aim of this study is to compare the clinical characteristics of PLA in patients with RPC and without RPC. METHODS: We performed a retrospective analysis involving patients with PLA. The demographic, clinical features, laboratory and imaging findings, management strategy, and outcome of patients with and without RPC were studied. RESULTS: From January 2000 to December 2009 inclusive, 319 patients (27 with RPC and 292 without RPC) had PLA. Patients with RPC had higher mean age (71.7 vs. 64.9 years; P=0.03) and tended to have more acute onset of presentation than it did in those without RPC. The common clinical features of the two groups were fever, chill, and right upper quadrant pain. Patients without RPC tended to have more respiratory symptoms and signs. The laboratory abnormalities of both groups shared common features such as anemia, leukocytosis, high erythrocyte sedimentation rate and C-reactive protein, hypoalbuminemia, elevated total bilirubin, and alanine aminotransferase. Left lobe was predominantly involved in patients with RPC (51.9% vs. 23.6%; P<0.01) whereas those without RPC usually had PLA at right lobe (67.5 vs. 40.7%; P<0.01). Both groups also shared common radiological features such as hypoechoic in ultrasonograpphy, rim enhancement and septal lobulation in computed tomography. However, aerobila was found more in patients with RPC than those without RPC (25.9 vs. 5.5%; P<0.01) and the latter tended to have more abscess ruptures. The biliary tract disorder was the most common cause of the disease in the two groups. The microorganisms involved had distinct patterns between these two groups. Patients with RPC tended to have fewer complications than patients without RPC as there were fewer occurrences in metastatic infections, disseminated intravascular coagulation, and acute renal and respiratory failure. PLA in patients with RPC had higher recurrence rate than those without RPC (37 vs. 2.4%; P<0.01). The odds ratio of local recurrence between RPC and non-RPC group was 23.95 (95% confidence interval, 8.11-70.72). However, both were effectively managed by the combination of antibiotic and image-guided aspiration with/without drainage, and their mortality rates are comparable with each other. CONCLUSION: PLA associated with RPC tends to have distinct clinical syndrome in regards to different extent of clinical manifestations, radiological and microbiological features, and complications. Its recurrent rate is higher than that of non-RPC type. However, both can be effectively treated with a combination of antibiotic and image-guided aspiration with/without drainage.

A gentleman with anemia and cholestasis.

Primary sclerosing cholangitis is a rare cause of cholestasis caused by progressive inflammation and fibrosis of both intrahepatic and extrahepatic bile ducts leading to multifocal ductal strictures. Herein, we report a case of primary sclerosing cholangitis and inflammatory bowel disease. The concomitant diagnosis of these two diseases is not typical. The management includes the treatment of inflammatory bowel disease and potential complications of primary sclerosing cholangitis, including dominant strictures of bile duct, portal hypertension, gallbladder diseases, cholangiocarcinoma, and colonoscopic surveillance.