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Biomedical subjects

H H Euler

Publications and source records attributed to H H Euler.

At least 19 recordsLinked to original sources
PubMed1996

[Cryofibrinogenemia caused by monoclonal anti-fibrinogen antibodies].

HISTORY AND CLINICAL FINDINGS: For nine years a 54-year-old woman had been suffering from worsening treatment-resistant cold-dependent purpura of the limbs as well as cutaneous ulcerations and arthralgia, which recently had occurred even at a even slight decrease in room temperature. INVESTIGATIONS: A special form of cryofibrinogenemia was identified by affinity-chromatographic separation of a plasma cryoprecipitate. From this cryoprecipitate a monoclonal antifibrinogen antibody (IgG-kappa) was isolated which, in the cold, formed a precipitating complex with fibrinogen. Paraproteinaemia was not demonstrated by conventional serum and plasma electrophoresis. There was no evidence of neoplasma. TREATMENT AND COURSE: Attempted treatment with steroids, fibrinolytic agents and intravenous cyclophosphamide was unsuccessful. But long-term repeated plasmaphereses and anti-immunoglobulin adsorption improved the symptoms. After 5 years of this treatment-14 years after onset of symptoms-the patient died of the consequences of fulminant pulmonary embolism. CONCLUSION: To establish the diagnosis of monoclonal cryofibrinogenemia it is necessary, first, to identify the cryoprecipitate in plasma; secondly, to undertake affinity-chromatographic separation of the cryoprecipitate with subsequent analysis of its components.

Antibodies, Monoclonal↗
PubMed1996

Monoclonal cryo-antifibrinogenemia.

We report on a 54-year-old female patient with arthritis and a severe cold-induced leukocytoclastic vasculitis of the skin caused by a rare form of cryofibrinogenemia ("type II" cryofibrinogen). Affinity chromatography of cryoprecipitates from the patient's plasma revealed reversible cryoprecipitability of complexes composed of fibrinogen and a monoclonal antifibrinogen antibody (IgG3 kappa). Conventional serum and plasma electrophoresis did not detect the paraprotein. Control of symptoms was achieved by long-term plasmapheresis.

Cryoglobulins↗
PubMed1996

High dose intravenous immunoglobulins in systemic lupus erythematosus: clinical and serological results of a pilot study.

OBJECTIVE: To investigate the effect of high dose intravenous immunoglobulins (IVIG) in systemic lupus erythematosus (SLE). METHODS: Twelve patients with mildly to moderately active disease were given 30 g of sulfonated IVIG preparation on each of Days 1-4 and 21-24. RESULTS: Within 6 weeks the mean disease activity score, the Systemic Lupus Activity Measure (SLAM), declined from 7.33 (range 3-15) to 5.25 (range 0-10) (p < 0.01). In 9/12 patients the SLAM dropped by at least 2 points. In 3/12 patients the improvement lasted 5 to 12 months. Within 1 week after initiation of therapy most patients showed a decline in ds-DNA antibodies, whereas titers of antinuclear antibodies and complement proteins were not affected. The treatment was well tolerated, with the exception of transient hypotension in one patient. CONCLUSION: In this uncontrolled study, IVIG had temporary beneficial effects in mildly to moderately active SLE.

Adolescent↗
PubMed1995

[Churg-Strauss syndrome. A differential diagnosis in chronic polypoid sinusitis].

BACKGROUND: The Churg-Strauss syndrome (CSS) is a rare multiple organ disease, that belongs to the group of systemic granulomatous vasculitis. The initial symptoms are often bronchial asthma and allergic rhinitis; later in the course of the disease, the patients exhibit lung, heart and kidney manifestations. PATIENTS: We report on a thirty-one year old female patient who initially presented with asthma and polypous sinusitis. Three years later, after undergoing endonasal sinus surgery three times, she was diagnosed with CSS. Within a review of the literature, we discuss symptomatology, differential diagnosis, and therapy of the CSS. RESULTS: Worldwide about 200 cases are published. The patient presented in this case report is now in a good health condition after a four-year therapy with steroids. The patient is free of sinusitis symptoms without the need of renewed surgical intervention. CONCLUSIONS: CSS should be considered in the differential diagnosis if bronchial asthma and resistant polypous sinusitis occur coincidentally. Often it is very difficult to diagnose CSS. Frequently it is only diagnosed after several years. Therapy of choice is the treatment with steroids, sometimes supplemented by cytotoxical drugs.

Adult↗
PubMed1994

Treatment-free remission in severe systemic lupus erythematosus following synchronization of plasmapheresis with subsequent pulse cyclophosphamide.

OBJECTIVE: To investigate the effect of an intensified treatment protocol synchronizing plasmapheresis with subsequent pulse cyclophosphamide for severe systemic lupus erythematosus (SLE). METHODS: A protocol of plasmapheresis (3 x 60 ml/kg) and subsequent high-dose pulse cyclophosphamide (36 mg/kg) followed by 6 months of peroral immunosuppression was used to treat 14 patients with severe SLE. RESULTS: Rapid improvement was achieved in all patients. Immunosuppressants, including corticosteroids, were withdrawn at month 6 in 12 patients. Eight patients continued without treatment for a mean observation period of 5.6 years (46-91 months). CONCLUSION: The results demonstrate that treatment-free clinical remission can be achieved in some patients with severe SLE.

Adolescent↗
PubMed1994

Disseminated histoplasmosis in a non-immunocompromised host.

Histoplasma infections in Europe are rare, and acute disseminated histoplasmosis has only been observed in immunocompromised persons. We describe a case of acute disseminated histoplasmosis in a young, nonimmunocompromised European woman. The probable source of infection was Sri Lanka or the Maldives. At presentation she was severely ill with fever, lymphadenopathy, anemia, thrombocytopenia, hepatosplenomegaly, and polyserositis. Histologically, myelofibrosis and osteosclerosis were observed with extramedullary hematopoiesis. Histoplasma capsulatum yeasts were detected in bone marrow trephine biopsy by methenamine silver staining. Treatment with conventional and liposomal amphotericin B and subsequent itraconazole led to rapid and complete recovery.

Acute Disease↗
PubMed1994

Plasmapheresis and subsequent pulse cyclophosphamide in severe systemic lupus erythematosus. An interim report of the Lupus Plasmapheresis Study Group.

The rarity with which therapy-free remission is achieved in patients with severe SLE demand the continued search for improved treatment strategies. Possibly, therapeutic results can be improved by means of a "synchronized" combination of plasmaphereses followed by pulse cyclophosphamide (Ctx). If the hypothesis is correct, that plasmapheresis induces a compensatory "rebound" proliferation of pathogenic clones, then pulse Ctx applied immediately after plasmapheresis should attack these activated clones when they are most vulnerable and, ideally, eliminate them. Employing a strict variant of this strategy in a pilot study, treatment-free remission lasting now for up to 7 years were achievable in 8 of 14 SLE patients. The Lupus Plasmapheresis Study Group (LPSG) is currently conducting a randomized international multicenter trial comparing a synchronized procedure with the administration of pulse Ctx alone. The present paper explains the rationale of synchronization and reports on the interim status of the LPSG Study.

Combined Modality Therapy↗
PubMed1993

A method for chronic membrane plasmapheresis in the rat.

Therapeutic apheresis as applied to humans may encompass a single treatment or numerous treatments for disorders ranging from acute poisoning to severe chronic autoimmune disease. However, the mechanisms of beneficial effects of apheresis are not well characterized. Utilizing a miniaturized hollow-fiber membrane system, we have developed a reliable technique for long-term vascular access in the rat that permits repetitive plasmapheresis. We established vascular access in 14 animals, with 8 and 6 rats randomized to 3- and 7-wk experimental periods, respectively. Immunoglobulin levels of blood samples obtained immediately before and after each plasmapheresis were measured to examine membrane filtration characteristics. Overall, 100% of the animals survived and 93% successfully completed their assigned experimental periods. Mean decrease of immunoglobulin G and M levels for 28 plasmapheresis treatments in five rats was 66.9 +/- 8.1 and 61.0 +/- 7.3% (SD), respectively, indicating effective membrane filtration. This model of apheresis can be applied to several disorders in the rat, including, but not limited to, spontaneous insulin-dependent diabetes mellitus and experimental inflammatory bowel disease.

Animals↗
PubMed1993

Effect of antigen-specific immunoadsorption on antibody kinetics in a rat model.

The investigation of antibody kinetics following antigen-specific immunoadsorption in alkaline phosphatase immunized rats revealed significantly lower antibody levels than in untreated controls over a follow-up period of 6 weeks. A rebounding antibody synthesis as a result of specific depletion was not observed. Non-adsorption of specific antiidiotypic antibodies may explain these findings.

Alkaline Phosphatase↗