Subclinical pulmonary haemorrhage causing a restrictive lung defect in three siblings with a unique urticarial vasculitis syndrome.

Three siblings with urticarial vasculitis syndrome (UVS) are described. All had restrictive lung function abnormalities caused by subclinical pulmonary haemorrhage. The latter was suspected after finding haemosiderin-laden macrophages and a friable bronchial mucosa during elective bronchopulmonary alveolar lavage (BAL). The chest radiographs were normal at presentation but after steroid withdrawal symptoms worsened, haemoglobin levels fell, and Case 1 developed acute pulmonary haemorrhage. This was documented by lung biopsy, which also revealed evidence of old haemorrhage and fibrosis. We concluded that these patients had a unique familial variant of UVS with a previously unreported restrictive lung disease due to subclinical pulmonary haemorrhage.

Tetanus. A clinical review.

OBJECTIVE: Although tetanus is now uncommon in Oman (The Expanded Program on Immunization was introduced in 1981), patients continue to present from time to time at an average rate of 6 cases per year. Worldwide, the mortality rate for tetanus remains high (ranging from 15-20% in developed countries). At the Sultan Qaboos University Hospital, Muscat, Oman, prolonged Intensive Care Unit treatment and multi-disciplinary management is invariably required for tetanus patients. This study was carried out to evaluate our results over the past decade. METHODS: All tetanus patients admitted to the Sultan Qaboos University Hospital from 1991 up to the end of 1999 were retrospectively reviewed. Patients were diagnosed early, and aggressive treatment in the Intensive Care Unit was instituted, with a coordinated multidisciplinary management. RESULTS: Ten cases were identified and included in the review, comprising 9 adults and one infant aged 2 weeks. Adult patients were aged 36-75 years (mean 59 years), and the average Intensive Care Unit stay of the 9 surviving patients was 5.5 weeks (range 3-7 weeks). All patients presented with severe generalized tetanus. Two patients with traditional cautery marks developed tetanus. A focus of infection could not be found in 2 patients. All patients had early tracheostomy and assisted ventilation with appropriate sedation. One patient required almost 45 gm of diazepam throughout his Intensive Care Unit stay. One adult patient died on the 6th day of admission following myocardial infarction. The neonatal case survived after 35 days care in the Intensive Care Unit. The mortality rate for our patients was therefore 10%. CONCLUSION: Tetanus in Oman remains an infrequent but important disease requiring costly and prolonged Intensive Care Unit treatment. We attribute the comparatively low mortality rate (10%) in this study, to early diagnosis, institution of aggressive treatment, good nursing as well as a well-coordinated multi-disciplinary management.

Tetanus: A clinical review.

OBJECTIVE: Although tetanus is now uncommon in Oman (The Expanded Program on Immunization was introduced in 1981), patients continue to present from time to time at an average rate of 6 cases per year. Worldwide, the mortality rate for tetanus remains high (ranging from 15-20% in developed countries). At the Sultan Qaboos University Hospital, Muscat, Oman, prolonged Intensive Care Unit treatment and multi-disciplinary management is invariably required for tetanus patients. This study was carried out to evaluate our results over the past decade. METHODS: All tetanus patients admitted to the Sultan Qaboos University Hospital from 1991 up to the end of 1999 were retrospectively reviewed. Patients were diagnosed early, and aggressive treatment in the Intensive Care Unit was instituted, with a coordinated multidisciplinary management. RESULTS: Ten cases were identified and included in the review, comprising 9 adults and one infant aged 2 weeks. Adult patients were aged 36-75 years (mean 59 years), and the average Intensive Care Unit stay of the 9 surviving patients was 5.5 weeks (range 3-7 weeks). All patients presented with severe generalized tetanus. Two patients with traditional cautery marks developed tetanus. A focus of infection could not be found in 2 patients. All patients had early tracheostomy and assisted ventilation with appropriate sedation. One patient required almost 45 gm of diazepam throughout his Intensive Care Unit stay. One adult patient died on the 6th day of admission following myocardial infarction. The neonatal case survived after 35 days care in the Intensive Care Unit. The mortality rate for our patients was therefore 10%. CONCLUSION: Tetanus in Oman remains an infrequent but important disease requiring costly and prolonged Intensive Care Unit treatment. We attribute the comparatively low mortality rate (10%) in this study, to early diagnosis, institution of aggressive treatment, good nursing as well as a well-coordinated multi-disciplinary management.

Serum levels of soluble Fas correlate with indices of organ damage in systemic lupus erythematosus.

OBJECTIVE: To evaluate whether the levels of soluble form of the Fas apoptosis antigen (sCD95/sFas) varied from those of healthy control subjects in a group of patients with systemic lupus erythematosus (SLE). This was done to determine whether sFas has a role in either the disease activity or the organ damage in SLE. METHODS: Serum levels of sFas were measured over a period of 4 y (277 determinations) in 39 Arab patients with SLE and 22 age-, gender-, and race-matched healthy controls using double antibody ELISA. SLEDAI scores for disease activity and SLICC/ACR scores for cumulative organ damage were determined. Serum levels of acute phase reactants, complement, inflammatory cell counts, levels of autoantibodies, and kidney and liver function test results were obtained retrospectively from clinical records. RESULTS: sFas levels were significantly higher in patients with SLE (n = 39, 277 determinations) (0.60 ng/ml +/- 0.38) than in healthy controls (n = 22) (0.26 ng/ml +/- 0.11) (P < 0.00001). The levels of sFas correlated with SLICC/ACR (r = 0.36; P < 0.02), but not with SLEDAI. sFas correlated with renal and liver function tests measured by s-creatinine (r = 0.38; P < 0.0001), creatinine clearance (r = -0.30, P < 0.001), s-albumin (r = -0.28, P < 0.0001), and ALT (r = 0.35; P < 0.00001), but did not correlate with the levels of acute phase reactants. CONCLUSION: sFas is elevated in sera of SLE patient. Since sFas correlates with indices of organ damage but not with disease activity, it may be a marker of organ damage in SLE and may act to protect certain organs from further damage by inhibiting Fas-mediated apoptosis.