Immunoglobulin G4-related sclerosing disease: experience with this novel entity in a local hospital.

OBJECTIVE: To review the site of involvement, clinical presentation, and treatment outcome of patients having immunoglobulin G4-related sclerosing disease in a local regional hospital. DESIGN: Retrospective case series. SETTING: Pamela Youde Nethersole Eastern Hospital, Hong Kong. PATIENTS: All patients with a diagnosis of immunoglobulin G4-related sclerosing disease in the hospital diagnosed in the period from April 2008 to March 2010. RESULTS: A total of 12 patients with involvement of various organs were identified. There was a male predominance (male-to-female ratio=5:1). The mean age at diagnosis was 65 years. The salivary glands, biliary tract, pancreas, and cervical lymph nodes were the commonest involved sites. The immunoglobulin G4 level was elevated in 83% of the patients. Patients usually appeared to respond well to steroid treatment. CONCLUSION: Immunoglobulin G4-related sclerosing disease is a systemic disease and can involve various systems.

Longitudinal study of Chinese patients with pernicious anaemia.

BACKGROUND: The survival of whites who have been treated for pernicious anaemia (PA) is unaffected, apart from incurring a greater risk of gastric cancers. The long term outcome of PA in Chinese is unknown. METHODS: A hospital based prospective longitudinal study of Chinese PA patients was conducted. Patients with known cancers were excluded. RESULTS: From 1994 to 2007, 199 intrinsic factor antibody (IFA) positive and 168 IFA negative patients were recruited. Both cohorts had similar baseline characteristics, except the IFA positive patients had more severe haematological findings and more thyrogastric immune features; also more IFA negative patients had type 2 diabetes mellitus and gastrointestinal (GI) disease or GI surgery. Both cohorts had a good haematological response but an unsatisfactory neurological response to treatment. Hypothyroidism developed in patients of both cohorts during follow-up. 24 IFA positive patients and 7 IFA negative patients developed cancers (p = 0.007) during follow-up. 20% of all cancers were gastric carcinoma. Mean survival of both cohorts was similar. Mean survival of IFA positive patients with and without cancers was 64 and 129 months, respectively (p<0.001), and that of IFA negative patients 36 and 126 months, respectively (p<0.001). Death rates were 31% in the IFA positive cohort and 21% in the IFA negative cohort (p = 0.028). Cancer related death rates of IFA positive and IFA negative cohorts were 37% and 14%, respectively (p = 0.014). CONCLUSION: The survival period of Chinese with PA who have received treatment is good, but there is an increased risk of gastric cancers. IFA positive patients have a higher risk of developing all types of cancers and cancer related deaths than IFA negative patients.

Computer analysis of prolonged lower oesophageal sphincter pressure recordings.

The aim of the study was to validate a recently developed computer program for the analysis of prolonged recordings of lower oesophageal sphincter pressure. Thirty 1-hour stretches were selected from sets of 24-h pressure signals recorded from the pharynx, oesophagus, lower oesophageal sphincter (LOS) and stomach in 10 ambulant patients with gastrooesophageal reflux disease. Three experienced investigators visually analysed end-expiratory LOS pressures and transient lower oesophageal sphincter relaxations (TLOSRs), using published criteria. A computer program was developed for calculation of an end-expiratory pressure curve and detection of TLOSRs using the same criteria. Although the results showed an maximum deviation from the mean of 11.1% and 14.8% for manually calculated LOS pressures and visually detected TLOSRs, respectively only 62.1% of the detected TLOSRs were detected by all three observers. LOS pressure as measured by the computer closely approximated the mean of the LOS pressures calculated by the three observers. Although the total number of TLOSRs was comparable to that assessed by visual analysis, the computer detected only 46% of the TLOSRs detected by each observer and 56.8% of the TLOSRs detected by all observers. It is concluded that automated calculation of end-expiratory LOS pressure is feasible and yields reliable results, whereas automated detection of TLOSRs could not be satisfactorily accomplished. Our study showed that improvement of computer algorithms for TLOSR detection is desirable. However, the previously described criteria for detection of TLOSRs are insufficiently precise; further refinement of these criteria will be necessary to reduce the large discrepancies between the outcome of detection of TLOSRs by computer and by humans, and to reduce the equally large discrepancies between the results of detection by different human observers.