Clinicopathologic features of antineutrophil cytoplasm autoantibody (ANCA)-associated acute renal failure in Chinese patients

Summary: The clinical course and renal pathologic features of anti-neutrophil cytoplasm auto-antibody (ANCA)-associated renal disease were studied among Chinese patients from a single centre. Eight ANCA positive patients with acute renal impairment were studied, four of whom required dialysis shortly after presentation. Their mean age at presentation was 61.6 ± 4.2 years. Renal histology, obtained in seven patients, showed paucummune crescentic glomerulonephritis in five patients, interstitial nephritis in two patients, and small vessel vasculitis in one patient. Pulmonary baemorrhage was the other common disease manifestation, present in four of the eight patients, necessitating ventilatory support in three patients. Neurologic, cutaneous, and gastrointestinal involvement were also observed. Seven of the eight patients tested positive for pANCA and anti-myeloperoxidase, while cANCA was detected in one patient of the eight patients, six (75%) responded to therapy, consisting of prednisolone and cyclophosphamide in five patients, and antibacterial therapy alone in one patient, who had interstitial nephritis but no evidence of vasculitis. Two patients died from sepsis and severe debilitation one month after presentation. of the other six patients, five had significant improvement of renal function, while one became dialysis-dependent. the levels of ANCA and C-reactive protein remained normal, and disease reactivation was not observed during follow-up for 32.4 ± 6.1 months. Patient and renal survival rates at one year were 75% and 62.5%, respectively. It was concluded that the clinical and pathologic features of ANCA-associated renal disease in Chinese patients are, in general, similar to those described in Caucasians. Nevertheless, cANCA-positivity is distinctly uncommon. the demonstration of interstitial nephritis in two of the eight patients underlines the importance of renal biopsy for correct histologic diagnosis. Early institution of aggressive immunosuppression and supportive therapies are essential for the achievement of favourable outcome in patients with vasculitis.     

Hemolytic-uremic syndrome following bone marrow transplantation in adults for hematologic malignancies

One hundred and sixty eight adult patients with B-cell non-Hodgkin's lymphoma (NHL) and other hematologic malignancies who underwent autologous or allogeneic bone marrow transplantation (BMT) were investigated for the subsequent development of hemolytic-uremic syndrome (HUS). All patients were conditioned with cyclophosphamide and total body irradiation. When examined at 3-month intervals for the first year post-BMT, all patients had uniform measurements of hematocrit (Hct) and serum creatinine. Sixteen patients who initially exhibited Hct and creatinine values that were normal range for the BMT populations developed a sudden decrease in Hct and increase in creatinine between 3 and 11 months post-BMT and fulfilled the clinical and laboratory criteria for HUS. None of these patients had known active cytomegalovirus infection, graft-versus-host disease, or cyclosporine administration. The degree of decrease in Hct and creatinine elevation ranged from solely laboratory abnormalities to a clinically significant syndrome. Twelve of the 16 patients developed acute clinical complications of congestive heart failure, hypertension (HTN), or peripheral edema. Twelve patients required red blood cell support, whereas only four patients required platelet transfusions. Both hemolytic anemia and thrombocytopenia have resolved in virtually all cases. At a mean follow up of 18 months postdiagnosis, creatinine elevations have persisted along with HTN. All patients have survived without life-threatening long-term sequelae. With the increasing use of BMT as a curative modality for patients with hematologic malignancies, it becomes important to prospectively monitor patients for the development of HUS and its potential long-term impact on renal function.     

Sleep apnoea caused by rheumatoid arthritis

Sleep apnoea syndrome (SAS) is a rarely documented, but possibly lethal, complication of the instability of the cervical spine in rheumatoid arthritis. Five patients with SAS of a central or peripheral origin are presented, and the problems of recognizing and diagnosing the syndrome are discussed. We hope that clinicians will become more aware of the existence and the different aetiologies of SAS, thus improving early recognition and appropriate treatment. Adequate treatment has proven to increase survival in peripheral SAS and seems to be successful in doing so in central SAS.      

Reduction of patient exposure by use of heavy elements as radiation filters in diagnostic radiology

The effect on patient exposure of x-ray filters made of heavy elements has been studied in excretory urography and lumbar spine examinations. Several elements with atomic numbers between 62 and 74 were tested in experiments using radiographic phantoms and in patient studies. A holmium filter reduced skin exposure by more than 50% in excretory urography. Similar results were obtained with ytterbium and tungsten in lumbar spine studies.