Immunoadsorption onto protein A induces remission in severe systemic lupus erythematosus.

BACKGROUND: Reduction of pathological autoantibodies and circulating immune complexes can be useful in the treatment of autoimmune disease. Plasmapheresis has been shown to reduce autoantibody levels in systemic lupus erythematosus (SLE), but its effect on patients' outcome was not better compared with conventional immunosuppression in the past. AIM OF THE STUDY: Immunoadsorption as a selective extracorporeal immunoglobulin elimination technique was evaluated as rescue therapy in patients suffering from SLE. METHODS: Eight patients with severe, therapy-resistant SLE underwent immunoadsorption onto protein A sepharose without concomitant immunosuppressants. RESULTS: Remission of the disease was achieved in seven patients. Therapy had to be stopped in one patient because of side-effects. The best results were obtained when immunoadsorption was carried out daily, without supplementary intravenous immunoglobulin therapy. Oral cyclophosphamide for 3-6 months during follow-up was used to suppress relapse. Autoantibodies and circulating immune complexes were effectively eliminated regardless of their IgG subclass. CONCLUSION: Immunoadsorption onto protein A might be used as an extracorporeal treatment option in SLE when other therapies are ineffective.     

Thoracic tumors in children with neurofibromatosis-1

Thoracic tumors have been infrequently reported as a complication of neurofibromatosis-1 (NF1). To determine the prevalence and clinical features of thoracic tumors seen in children with NF1, we reviewed medical records and imaging studies for a group of 260 pediatric patients with NF1 followed in a multidisciplinary NF Center. Extrapleural thoracic tumors were seen in nine patients with NF1, corresponding to a prevalence of 3.5% in this hospital-based series of patients. Pathological studies of the tumors demonstrated plexiform neurofibroma in four cases and neurofibrosarcoma in one case. The remaining four cases were suspected to be plexiform neurofibroma based on clinical features but have not been confirmed histologically. Three patients presented with symptoms of chest pain, syncope, or wheezing; six patients were asymptomatic at the time of diagnosis of the tumors. Physical findings frequently found in patients with thoracic tumors were scoliosis (especially focal scoliosis) and visible plexiform neurofibromas of the neck. We conclude that NF1 patients presenting with any of these signs and symptoms should be screened for thoracic tumors with chest X-ray and magnetic resonance imaging as needed. It is unknown whether screening asymptomatic NF1 patients with chest X-rays on a regular basis will result in an improved outcome. Am. J. Med. Genet. 74:533–537, 1997. © 1997 Wiley-Liss, Inc.     

Hepatocellular carcinoma in Gaucher disease: an international case series

Gaucher disease (GD) is associated with an increased risk for malignancies. Next to hematological malignancies, the development of solid tumors in several organs has been described. The liver is one of the major storage sites involved in GD pathogenesis, and is also affected by liver-specific complications. In this case series, we describe 16 GD type 1 (GD1) patients from eight different referral centers around the world who developed hepatocellular carcinoma (HCC). Potential factors contributing to the increased HCC risk in GD patients are studied. Eleven patients had undergone a splenectomy in the past. Liver cirrhosis, one of the main risk factors for the development of HCC, was present in nine out of 14 patients for whom data was available. Three out of seven examined patients showed a transferrin saturation?>?45%. In these three patients the presence of iron overload after histopathological examination of the liver was shown. Chronic hepatitis C infection was present in three of 14 examined cases. We summarized all findings and made a comparison to the literature. We recommend that GD patients, especially those with prior splenectomy or iron overload, be evaluated for signs of liver fibrosis and if found to be monitored for HCC development.     

Vacuum-assisted closure as a surgical assistant in life-threatening necrotizing fasciitis in children

Necrotizing fasciitis is a severe soft tissue infection that can involve skin, subcutaneous fat, fascia and muscle. It can result in devastating sequelae including tissue necrosis, sepsis, toxic shock syndrome, cardiopulmonary collapse and death. To control rapidly spreading necrosis, early diagnosis and aggressive surgical treatment with extensive radical debridement of the affected areas is necessary, as well as systemic administration of broad-spectrum antimicrobials and, very often, intensive care support.The subatmospheric negative pressure dressing has been previously used in acute and complex wounds management. The concept of using vacuum-assisted closure dressing as another management component is presented in the current article.     

Morbidity associated with heroin overdose presentations to an emergency department: A 10‐year record linkage study

Introduction: To examine hospitalizations in a cohort of 224 patients who presented with non‐fatal heroin overdose to an ED. Methods: A record linkage study, using the morbidity, mental health and mortality databases in the Data Linkage Unit of the Department of Health, Western Australia. The main outcome measures were hospital separations 5 years before and after entry into the cohort. Results: Before entry into the cohort, 199 (89%) patients had an admission to mental health services. These 199 had a combined total of 1367 separations, most commonly for a mental health condition, injury or poisoning. Women had more than twice the relative risk (RR) of men for all separations (RR 2.35, 95% confidence interval [CI] 1.96–2.82, P < 0.001) and for injury and poisoning separations (RR 2.04, 95% CI 1.56–2.66, P < 0.001). The highest concentrations of separations occurred within 1 year before and 1 year after entry into the cohort. There were 12 (5.4%, 95% CI 2.9–9.4%) deaths, most commonly from overdose. Conclusion: Non‐fatal heroin overdose ED presentations are associated with a cluster of hospitalizations around that episode, likely to be related to heroin availability. Presentation to hospital by heroin users represents an opportunity to counsel less risky behaviour.