Schweizerische medizinische Wochenschrift最新文献

There are still several problems surrounding the diagnosis of cerebrospinal fluid leak. Currently the method of choice for cerebrospinal fluid detection is qualitative determination of beta-2-transferrin. Faster and more efficient methods (beta-trace) are under clinical investigation. The major problem is localisation of the site of leakage. Combination of several radiological methods increases the rate of correct diagnosis. In surgery the use of intrathecal sodium-fluorescein improves visualisation of the site of leakage and thus increases the chances of secure and stable closure of the cerebrospinal fluid fistula.

The Swiss HIV Cohort Study (SHCS) is a prospective cohort study of HIV-infected adolescents and adults seen at 7 outpatient clinics (Swiss University Hospitals in Basle, Berne, Geneva, Lausanne, Zurich, the St. Gall Cantonal Hospital and the Civico Hospital in Lugano). The SHCS serves as an infrastructure for different research projects and includes about 70% of all patients with advanced disease in Switzerland. From April 1984 to November 1995 3120 HIV-infected patients of the SHCS died. Autopsies were performed in 314 of these patients. The aim of our study is to analyse autopsy findings as well as causes of death in those 314 HIV-infected patients. An HIV-related cause of death was found in 271 (86%) of the patients, 12 patients (4%) died of a drug overdose, and 3 (1%) of the patients committed suicide. 28 (9%) died either from an HIV unrelated or unidentified cause. The five most frequent causes of death were: bacterial pneumonia (52 patients, 17%), Pneumocystis carinii pneumonia (40 patients, 13%), lymphoma (34 patients, 11%), cytomegalovirus infection (33 patients, 11%), and toxoplasmosis (30 patients, 10%). During our study marked progress occurred in treating HIV-infected patients and preventing opportunistic infections. These improvements have further changed the natural course of acquired immunodeficiency syndrome. They are reflected in the falling rate of Pneumocystis carinii pneumonia and toxoplasmosis, as well as an increase in lymphoma as a cause of death over the period of our study.

Familial hypokalaemic periodic paralysis is an autosomal dominant muscle disease which has been linked to point mutations in the skeletal muscle L-type calcium channel alpha 1 subunit (alpha 1 s). It consists of muscular weakness episodes due to hypokalaemia caused by intracellular shifting of potassium. We describe the case of a young man of Kurdish origin, with a history of Graves' disease, who was admitted to the emergency room with hypotonic tetraplegia associated with severe hypokalaemia.

A case of focal myositis in a healthy 68-year-old woman is described. The patient was admitted for evaluation of a painful soft-tissue mass localised on the medial side of the left thigh, initially misdiagnosed as thrombophlebitis of the v. saphena magna. Laboratory data were normal, in particular sedimentation rate and muscle enzyme levels. After exclusion of venous thrombosis, the mass localised in the left m. gracilis was surgically removed. Histologic examination of the biopsy specimen showed muscle cell necrosis and severe inflammation, with lymphocytic infiltration leading to the diagnosis of focal myositis. This is a rare benign inflammatory pseudotumour of skeletal muscle. The aetiology and pathogenesis of the disease remain unclear. It is most commonly seen in the lower extremities and may mimic thrombophlebitis or soft-tissue neoplasm. Ultrasound and magnetic-resonance scans are helpful, but definitive diagnosis is obtained only by histology. Because recurrent lesions in other skeletal muscles are possible, and a third of patients develop polymyositis, a follow-up of several years is recommended.

Bowel obstruction, causing repetitive vomiting and reduced quality of life, is a common complication in patients with intraabdominal malignancies. Conservative treatment with nasogastric tubes is limited by patient discomfort. Antisecretory drug treatment with octreotide may be insufficient. We describe the application of percutaneous endoscopic gastrostomy (PEG) in 3 terminally ill cancer patients as simple and effective method for decompression in the upper gastrointestinal tract.

We report on conservative management of 2 patients with spontaneous splenic rupture associated with infectious mononucleosis. Both patients had an unremarkable hospital course and were discharged within 7 days of admission. Resolution of the haematoma was followed by ultrasound monitoring during the hospital stay. A literature review to 1999 shows that approximately 45 patients with serologically proven infectious mononucleosis have suffered spontaneous rupture of the spleen. Spontaneous splenic rupture is a rare but potentially fatal complication of infectious mononucleosis. Although splenectomy has been advocated in the past as the definitive therapy, we recommend that non-surgical management be considered in haemodynamically stable patients, to avoid the complications of splenectomy (e.g. post-splenectomy sepsis).

Paraproteinaemias may be associated with benign or malignant proliferations of lymphocytes or plasma cells, including multiple myeloma, monoclonal gammopathy of undetermined significance (MGUS) and Waldenström's macroglobulinaemia. Primary amyloidosis may be associated with multiple myeloma and rarely with lymphoid malignancies, but most cases can be considered as a particular form of monoclonal gammopathy of undetermined significance, where the paraprotein causes damage by virtue of its amyloidogenic properties. This article discusses recent advances in understanding of the biology of multiple myeloma. Multiple myeloma is now known to arise from a post-germinal centre B cell in the lymph node which homes to the bone marrow. Interactions with stromal cells in the marrow facilitate homing and growth of the myeloma cells. The stromal cells produce IL-6, which is an important growth factor for myeloma cells, while the myeloma cells produce factors such as TNF-alpha and IL-1 beta that activate osteoclasts, resulting in myeloma bone disease. Myeloma cells also produce vascular endothelial growth factor which results in increased microvessel formation in the marrow, promoting tumour growth. There has been interest in the possible role of the Kaposi's sarcoma associated herpes virus (HHV8) in multiple myeloma, following the demonstration of viral gene sequences in multiple myeloma marrow. However, results of further studies have been conflicting and at present there is no clear evidence for an aetiological role of HHV8 in multiple myeloma. Cytogenetic studies using modern techniques have demonstrated that almost all multiple myeloma cases are cytogenetically abnormal, the predominant abnormalities being various translocations involving chromosome 14q and deletions of chromosome 13. 14q translocations are equally common in monoclonal gammopathy of undetermined significance, but deletions of chromosome 13 seem to be associated with progression to multiple myeloma, and also have powerful prognostic significance for survival in multiple myeloma patients.

The chief advances observed over the last 15 years in the treatment of multiple myeloma arise from intensive procedures and in particular autologous bone marrow transplantation. However, even if autologous bone marrow transplantation increases the rate of total remissions, no plateau is yet observable in the survival curve and transplantation is probably not a curative therapy. Hopes for the future seem to rest on intensive chemotherapy combined with innovative therapeutic approaches such as diphosphonates, thalidomide or immunotherapy.