Evaluation of performance of measurement of faecal α(1)-antitrypsin clearance and technetium-99m human serum albumin scintigraphy in protein-losing enteropathy.

BACKGROUND AND AIM: Our study aimed to compare the performance of faecal α(1)-antitrypsin clearance (AATC) and radiolabelled human serum albumin (HSA) scintigraphy in protein-losing enteropathy (PLE). METHODS: Patients studied by both AATC and technetium-99m ((99m)Tc)-labelled HSA scintigraphy were recruited and categorized into PLE and non-PLE groups based on clinical and laboratory findings. The performance of AATC and (99m)Tc-labelled HSA scintigraphy was evaluated using clinical diagnosis of PLE as a gold standard. RESULTS: 29 patients were recruited and 13 patients were considered to have definite PLE (PLE group). In the PLE group, all patients had a positive HSA scinigraphy and 10 (77%) had demonstrable positive tracing in the early phase. Conversely, only 6 of them (46%) had elevated AATC level (>13 m/day). Results of (99m)Tc-labelled HSA scan (but not AATC) showed significant agreement with the clinical diagnosis (κ 0.35, p = 0.013). (99m)Tc-labelled HSA scintigraphy carried higher sensitivity (100 vs. 46%) and negative predictive value (100 vs. 63%) compared to AATC in diagnosing PLE. The correlation between the results of these two investigations was only modest (κ 0.27, p = 0.04). The area under the receiver operating characteristic curve of AATC level showed no optimal diagnostic cut-off for PLE. CONCLUSION: (99m)Tc-labelled HSA scintigraphy was superior to AATC in diagnosing PLE.

Unique hepatic imaging features in a patient with nodular regenerative hyperplasia of the liver associating with systemic lupus erythematosus.

Nodular regenerative hyperplasia (NRH) of the liver, characterized by regenerative nodules distributed throughout the liver in the absence of fibrosis, is a rare but important complication of systemic lupus erythematosus (SLE). The main consequence of NRH is non-cirrhotic portal hypertension. We describe a female patient who had immune thrombocytopenia in association with elevated liver enzymes and evidence of portal hypertension as initial manifestations of SLE. Computed tomographic scan and magnetic resonance imaging of the liver in this patient showed enhancing periportal tubular structures distributed throughout the liver. The diagnosis of NRH was eventually disclosed by transcutaneous needle liver biopsy. The pattern of radiological abnormality in this patient has not been described previously in NRH. In addition, we believe this is a first reported case of SLE presenting with immune thrombocytopenia in association with NRH.

Oesophageal tuberculosis mimicking oesophageal carcinoma.

Tuberculous involvement of the oesophagus is rare, and is usually caused by direct spread from adjacent afflicted structures. We report an 83-year-old male patient with oesophageal tuberculosis secondary to tuberculous mediastinal lymphadenitis who presented with non-specific symptoms of anorexia and lethargy. Upper gastro-intestinal endoscopy revealed an ulcerative tumour-like lesion in the mid-oesophagus suggesting oesophageal carcinoma. Repeated endoscopic biopsies revealed a non-specific acute-on-chronic inflammation consisting of non-caseating granulomas, with no evidence of malignancy. Endoscopic ultrasonography demonstrated that the oesophageal lesion was secondary to direct extension of mediastinal lymphadenopathy. The diagnosis of tuberculosis was eventually confirmed by histological and microbiological analysis of a surgically excised cervical lymph node. The patient responded promptly to treatment with antituberculous drugs. We suggest that oesophageal tuberculosis has to be kept in mind in the differential diagnosis of oesophageal ulcerohypertrophic lesions.

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.

Clopidogrel-induced hepatotoxicity after percutaneous coronary stenting.

Clopidogrel, an adenosine diphosphate receptor blocker, is widely used as an adjunctive antiplatelet therapy in acute coronary syndrome and percutaneous coronary stenting. The occurrence of hepatotoxicity is rare. We describe the occurrence of symptomatic liver disease in a 74-year-old man 5 weeks following commencement of therapy with clopidogrel. The reported cases of clopidogrel-induced hepatotoxicity are reviewed and the clinical significance of this event are discussed.

Recurrent variceal bleeding in a young woman.

Congenital hepatic fibrosis is an uncommon fibrocystic disorder affecting the intrahepatic bile ducts. It has autosomal recessive inheritance. The main consequence of this condition is portal hypertension and it is often misdiagnosed as cirrhosis. Patients with congenital hepatic fibrosis usually present during childhood or early adolescence with oesophageal variceal bleeding. Portosystemic shunt surgery is the treatment of choice for these patients as the risk of postoperative hepatic encephalopathy is low. We report a patient with congenital hepatic fibrosis who presented with oesophageal variceal bleeding at the age of 16 years, initially misdiagnosed as having cryptogenic liver cirrhosis. The patient experienced two further episodes of oesophageal variceal bleeding in subsequent years. She eventually underwent portosystemic shunt surgery. One year after the operation, the shunt remained patent on Doppler ultrasonography, and there had been no further episodes of variceal bleeding post-surgery.

Epigastric pain in a patient with neurofibromatosis type 1.

Patients with neurofibromatosis type 1 are at increased risk of developing carcinoid tumours of the duodenum, particularly in the ampulla of Vater. Aggressive surgery with pancreatoduodenectomy is recommended for all ampullary carcinoid tumours because of their propensity to metastasise. We report a case of a 43-year-old woman with neurofibromatosis type 1, who presented with recurrent epigastric pain for 15 months. Evaluation using magnetic resonance cholangiopancreatography and side-viewing duodenoscopy revealed a submucosal tumour at the ampulla of Vater causing pancreatobiliary ductal obstruction and dilation. The ampullary tumour was overlooked initially by forward-viewing endoscopy. The patient subsequently underwent pancreatoduodenectomy. Histological examination of the surgically resected specimen confirmed the presence of a carcinoid tumour, with metastasis to peri-pancreatic lymph nodes. She remained asymptomatic 10 months after surgery.

Cholangiocarcinoma presenting as pseudoachalasia and gastroparesis.

Cholangiocarcinoma most commonly presents as painless progressive jaundice. We report a case occurring in a 56-year-old Chinese woman with an unusual presentation of progressive dysphagia and vomiting. Oesophageal manometric and barium studies were indicative of achalasia, and computed tomography confirmed the presence of cholangiocarcinoma extending to the gastroesophageal junction and proximal lesser curve of the stomach. In this case, a constricting tumour at the gastroesophageal junction with probable invasion of the vagus nerves led to features of achalasia and gastroparesis.

Diagnosis of gastroesophageal varices and portal collateral venous abnormalities by endosonography in cirrhotic patients.

BACKGROUND AND STUDY AIMS: The role of endosonography (EUS) in the management of portal hypertension is not well defined. We aimed to study the use of a new generation video-echo endoscope in the diagnosis of gastroesophageal varices (GEV) and extraluminal venous abnormalities in cirrhotic patients. PATIENTS AND METHODS: Cirrhotic patients were studied by echo endoscopy to assess esophageal varices endoscopically, and gastric varices and extraluminal venous abnormalities sonographically. The results were compared with esophagogastroduodenoscopy (EGD) examination. Dyspeptic patients served as controls. RESULTS: A total of 52 cirrhotic and 166 dyspeptic patients were studied. EUS identified esophageal varices (EV) endoscopically in 28 patients (53.8 %), which showed a good correlation with EGD findings (r = 0.855, P < 0.001). The red color sign and portal hypertensive gastropathy were diagnosed in six and seven patients, respectively, by both methods. EUS detected gastric varices sonographically in 16 patients (30.8 %), compared with detection in nine patients by EGD. Extraluminal venous abnormalities were detected in 48 cirrhotic patients (92 %) and in only nine dyspeptic patients (5.4 %) (P < 0.001). The size of extraluminal adventitial venous dilatation was significantly correlated with the severity of GEV and cirrhosis (P < 0.001). Perforating veins were identified in all patients with GEV. CONCLUSION: The new generation video-echo endoscope could be used as a single investigation in assessing both the intraluminal GEV and extraluminal venous abnormalities in cirrhotic patients. It may improve the management of patients with portal hypertension.