The clinical characteristics of lupus related protein-losing enteropathy in Hong Kong Chinese population: 10 years of experience from a regional hospital.

OBJECTIVE: The aim of our study was to investigate systemic lupus erythematosus (SLE) related protein-losing enteropathy (PLE) in the following areas: clinical features, laboratory, endoscopic and imaging characteristics, treatment and outcome. METHOD: A retrospective analysis was performed. RESULTS: From 2001 to 2010, 48 patients had SLE related PLE and their clinical characteristics were: age 40.8 ± 14.3 years, male-to-female ratio 1:8.6, mean symptom duration 4.3 ± 3.4 weeks, initial presentation and concomitant activity of SLE in 21(43.8%) and 37 (77.1%) patients, <20% patients developed gastrointestinal (GI) symptoms, mean serum albumin level 24.4 ± 5 g/L. Thirty (62.5%) patients had diffuse non-erosive erythematous GI mucosa with chronic inflammatory cells in lamina propria. Protein leakage was at the small bowel in 15 (31.3%) patients, terminal ileum/caecum in 16 (33.3%) patients and ascending colon in 11 (22.9%) patients. Thirty (62.5%) patients responded initially well to a combination of prednisolone and azathioprine (AZA) and 33 (68.8%) patients were maintained well by the above therapy. Higher potent induction and maintenance therapy were required in patients with: proteinuria (p < 0.01), history of previous immunosuppressive therapy (p < 0.02) and requirement of higher potent induction therapy (p < 0.01). PLE as initial SLE presentation was associated with better prognosis. Four reversible adverse events were reported: one had AZA-induced pancreatitis, two developed AZA-induced hypoplastic anemia and one developed steroid psychosis. One patient developed shingles in the fourth month and responded to oral acyclovir. No thromboembolic events were reported and one patient died of SLE nephropathy. CONCLUSION: There appears to be increasing prevalence of SLE related PLE. A diagnosis can be made using 99m Tc-labeled HSA scintigraphy. PLE generally responds well to immunosuppressive therapy.

Nephrotoxicity, including acquired Fanconi's syndrome, caused by adefovir dipivoxil - is there a safe dose?

WHAT IS KNOWN AND OBJECTIVE: Adefovir dipivoxil (ADV) is an oral bioavailable prodrug of adefovir that possesses potent in vitro activity against hepadnaviruses, retroviruses and herpes viruses. ADV is excreted unchanged in the urine through glomerular filtration and tubular secretion and is known to be nephrotoxic at doses of 60mg daily and above. Thus, the long-term safety of ADV, particularly nephrotoxicity, is a major concern. Our objective is to comment on the nephrotoxcicity of low-dose (10mg daily) ADV through a case report. COMMENT: The clinical features of nephrotoxicity because of ADV are described. A case report of acquired Fanconi's syndrome in a chronic hepatitis B patient treated with ADV 10mg daily is used to illustrate several key aspects. WHAT IS NEW AND CONCLUSION: Adefovir dipivoxil can be nephrotoxic at conventional dosage and therefore, patients treated with long-term ADV should have regular monitoring of renal function, and calcium and phosphate levels.

An unusual cause of obstructive jaundice.

Small-cell carcinomas of lung origin have been well characterised for their clinico-histopathological features. However, extrapulmonary small-cell carcinomas are rare, and in particular, they are extremely rare at the ampullary region. We report herein a case of small-cell carcinoma of ampulla of Vater and review its clinical, histological, and immunohistochemical features.

Nodular regenerative hyperplasia of the liver associated with systemic lupus erythematosus: three cases.

Nodular regenerative hyperplasia of the liver, characterised by regenerative nodules distributed throughout the liver in the absence of fibrosis, is a rare but important complication of systemic lupus erythematosus. The main consequence of nodular regenerative hyperplasia of the liver is non-cirrhotic portal hypertension. This condition is probably underdiagnosed, as many of these patients may remain asymptomatic. Furthermore, nodular regenerative hyperplasia of the liver may be misdiagnosed as cirrhosis. We describe three female patients with nodular regenerative hyperplasia of the liver associated with systemic lupus erythematosus. All three patients have clinical manifestations of portal hypertension, and all were initially misdiagnosed as having cryptogenic cirrhosis.

Hepatic portal venous gas complicating septic thrombophlebitis of the superior mesenteric vein.

Hepatic portal venous gas is a rare radiological finding with a wide spectrum of underlying pathologies. We describe a case of hepatic portal venous gas due to septic thrombophlebitis of the superior mesenteric vein. The clinical management of portomesenteric venous gas and the importance of computed tomography in delineating its underlying causes are discussed.

Intestinal tuberculosis in a regional hospital in Hong Kong: a 10-year experience.

OBJECTIVE: To study the clinical and pathological characteristics of patients with intestinal tuberculosis. DESIGN: Retrospective study. SETTING: United Christian Hospital, Hong Kong. PATIENTS: Patients with intestinal tuberculosis diagnosed between January 1995 and December 2004 inclusive. RESULTS: The median age of the 13 male and 9 female patients was 53 years (range, 12-81 years). Nineteen (86%) had a definitive diagnosis of intestinal tuberculosis confirmed by the presence of caseating granulomas and/or acid-fast bacilli in histological specimens. In three (14%) the diagnosis was based on histology revealing non-caseating granulomas and a positive response to anti-tuberculous treatment. Common symptoms included abdominal pain (82%), diarrhoea (55%), weight loss (55%), and fever (45%). Three (14%) of the patients were complicated by intestinal obstruction, and another two (9%) had intestinal perforation. Four (18%) had concomitant active pulmonary tuberculosis. The most frequently involved site was the ileocaecal region, which was affected in 19 (86%) patients. Other sites included the jejunum, ascending and sigmoid colon. The diagnosis of intestinal tuberculosis was facilitated by examination of colonoscopic biopsy specimens (11 patients), and by examination of resected surgical specimens in the remainder. Two patients died from terminal malignancy. The remainder completed anti-tuberculous therapy and responded satisfactorily. CONCLUSIONS: The diagnosis of intestinal tuberculosis is difficult due to the lack of specific signs or symptoms. Colonoscopy with ileoscopy are useful tools in the search for colonic and terminal ileal tuberculosis. Surgical exploration is reserved for equivocal cases and for those who present as emergencies.

Tuberculosis intestinal perforation during anti-tuberculosis treatment.

Intestinal perforation is an uncommon but potentially fatal complication of intestinal tuberculosis. We report on a 63-year-old HIV-negative man who developed terminal ileal perforation approximately 3.5 months following initiation of anti-tuberculous treatment for pulmonary tuberculosis and a concomitant tuberculous perianal abscess. Clinical and radiological improvements were initially evident following commencement of anti-tuberculous treatment, and the paradoxical response phenomenon was suspected. The patient subsequently underwent surgical resection of the affected bowel segment with primary anastomosis, and made an uneventful recovery. Anti-tuberculous medication was continued for another 12 months, and after a further 12 months there was no evidence of recurrent tuberculosis. This case illustrates that tuberculous intestinal perforation can develop during chemotherapy for tuberculosis. Prompt diagnosis and appropriate surgical treatment are essential to avoid morbidity and mortality.