Guidelines for the prevention of sepsis in asplenic and hyposplenic patients.

Asplenic or hyposplenic patients are at risk of fulminant sepsis. This entity has a mortality of up to 50%. The spectrum of causative organisms is evolving as are recommended preventive strategies, which include education, prophylactic and standby antibiotics, preventive immunizations, optimal antimalarial advice when visiting endemic countries and early management of animal bites. However, there is evidence that adherence to these strategies is poor. Consensus-updated guidelines have been developed to help Australian and New Zealand clinicians and patients in the prevention of sepsis in asplenic and hyposplenic patients.

Prolonged prodrome, systemic vasculitis, and deafness in Cogan's syndrome.

Cogan's syndrome is a rare, multisystem disease which occurs predominantly in children and young adults. It was originally described as the combination of interstitial keratitis and audiovestibular disturbance, but other forms of ocular disease, as well as systemic vasculitis, have since been recognised as part of the syndrome. Diagnosis can be difficult if the various manifestations occur separately, but early recognition is important because prompt treatment may prevent deafness. Two cases are presented here illustrating the features of this disease, and providing histological evidence of systemic vasculitis in both.

CD4 lymphocyte numbers after splenectomy in patients infected with the human immunodeficiency virus.

Splenectomy has been reported to alter inconsistently the CD4 lymphocyte numbers in patients infected with the human immunodeficiency virus (HIV). To further assess the effect of splenectomy we have retrospectively examined the charts of 10 patients who were infected with HIV and who had undergone splenectomy. There was a significant increase in the mean CD4 numbers following splenectomy (mean increase of 326/microliters, or 2.1-fold, P = 0.0009), the total lymphocyte numbers (mean increase of 1.55/ml, or 2.2-fold, P = 0.001) and in the CD8 lymphocyte count (mean increase of 968/microliters, or 2.3-fold, P = 0.014). No significant difference was observed in the percentage CD4 lymphocytes (P = 0.95) or in the CD4:CD8 lymphocyte ratio (P = 0.76). In two patients, symptoms suggestive of impaired immune function developed post-splenectomy, at a time when their CD4 lymphocyte numbers were markedly higher than their pre-splenectomy values. One developed oral candidiasis (CD4 960/microliters, percentage CD4 32%), and in one patient a 7 kg weight loss was associated with recurrent mouth ulcers (CD4 680/microliters, percentage CD4 7%). We conclude that the total CD4 count increases significantly after splenectomy while the percentage CD4 lymphocyte count and CD4:CD8 lymphocyte ratio do not. Our data suggest that the CD4 lymphocyte count overestimates the immune function in these patients, although our findings are not conclusive.

Low frequency maintenance ganciclovir for cytomegalovirus retinitis.

45 patients on maintenance ganciclovir for treated cytomegalovirus (CMV) retinitis were reviewed retrospectively. Treatment was given at 30 mg/kg/week in 3 divided doses: Monday, Wednesday, Friday. The median time to clinical relapse was 5.4 months. This is similar to that reported with higher frequency maintenance regimens. The high degree of immunodeficiency (median CD4 count 16 cells/microliters) and poor prognosis (median survival 8.2 months) associated with CMV retinitis were confirmed.

Chronic meningococcal disease.

Chronic meningococcal septicaemia is an unusual and infrequently recognised presentation of disease caused by Neisseria meningitidis. Clinical features are immunologically mediated and include fever, rash and arthritis, which may mimic cutaneous vasculitis or reactive gonococcal arthritis. Diagnosis is difficult to confirm as blood cultures commonly do not grow the organism despite weeks of symptoms. Culture of the organism from the nasopharynx may provide supportive evidence for diagnosis. Chronic meningococcaemia should be considered in the differential diagnosis of a cutaneous vasculitis. In the clinical setting of an undiagnosed fever with vasculitic rash and joint symptoms an empirical trial of intravenously administered penicillin should be considered before steroid therapy as a rapid response may simplify the diagnostic dilemma.

Cytomegalovirus cultures during maintenance DHPG therapy for cytomegalovirus (CMV) retinitis in acquired immunodeficiency syndrome (AIDS).

Nine patients with acquired immunodeficiency syndrome (AIDS) and cytomegalovirus (CMV) retinitis on maintenance therapy with ganciclovir: 9(1,3-dihydroxy-2-propoxymethyl) guanine (DHPG) at high dose (30 mg/kg/week) or low dose (20 mg/kg/week) were tested every 1-2 weeks for CMV isolation from blood, saliva, and urine. Duration of therapy ranged from 1.5 to 12 months (average 5.3 months). During pretreatment and low-dose and high-dose maintenance therapy, CMV was isolated from 48/59 (81%), 90/211 (43%), and 40/290 (14%) of specimens, respectively. Three patients with progressive retinitis had viraemia more frequently than did six patients with stable retinitis, CMV being isolated from 29/47 (62%) and 17/121 (14%) of blood samples, respectively.

Plasma exchange in dermatomyositis/polymyositis: beneficial effects in three cases.

We describe ameliorative effects of plasmapheresis in three patients with dermatomyositis-polymyositis resistant to other therapy, particularly prednisolone. Serologically, the cases were marked, respectively, by a weakly-reactive speckled-type ANA; a strongly reactive speckled-type ANA and an unidentifiable precipitin line by CIEP; and an antibody to cytoplasm and to Jo1. The remission induced by plasmapheresis was temporary in one case and lasting, with low doses of prednisolone, in two. The nuclear autoantibody in case two became undemonstrable after plasmapheresis but recurred during a subsequent relapse. This experience with plasmapheresis in myositis supplements a substantial reported experience of benefit from this therapy.