Biomedical subjects
P Harten
Publications and source records attributed to P Harten.
[Hereditary angioedema. Diagnostic and treatment errors as systemic lupus erythematosus].
BACKGROUND: Symptoms of hereditary angioedema are intermittent edema of subcutaneous tissues, abdominal organs, upper airways, and brain. Because of spontaneous mutation, in 20% of patients a familial history is lacking. Serological hallmarks are diminished complement factor 4 and C1-esterase inhibitor. The heterogenicity of the clinical symptoms frequently leads to false or delayed diagnosis. CASE REPORT: We report on a 50-year-old male patient with intermittent joint swellings, abdominal complaints, pleural effusions, ascites and headaches with disturbances of consciousness since early adulthood. Diagnosis was systemic lupus erythematosus. Immunosuppressive therapy was ineffective over months. Careful re-evaluation of the patient's clinical history and further laboratory examinations led to the diagnosis of an hereditary angioedema. Anamnestic and laboratory exploration of family members disclosed four other cases. Two of them also were symptomatic for decades without adequate diagnosis. CONCLUSION: In case of intermittent swellings, abdominal complaints, laryngeal edema, pleural effusions or ascites, differential diagnosis should involve hereditary angioedema. With early diagnosis and adequate treatment, prognosis is good. Since ACE inhibitors can aggravate the disease they are contraindicated. Diagnosis, pathogenesis, and treatment are discussed by reviewing the literature.
Hepatocellular adenomas in patients taking danazol for hereditary angio-oedema.
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Thrombocytopenic thrombotic purpura: severe clinic with no CT, minor MRI, but a SPECT correlate.
A 28-year-old woman with primarily therapy refractory TTP was followed neuroradiologically over 6 months. Despite pronounced neurological and neuropsychiatric symptoms including hemiparesis and aphasia she had unremarkable CT scans on two occasions. Three MRI exams showed no correlate for her neurological symptoms except a small petechial cortical hemorrhage in the right parietooccipital gyrus which may account for her TTP-related anxiety disorder. A cerebral HMPAO-SPECT showed long-standing right-sided hypoperfusion compatible with residual vasculature changes. The possible causes for the clinico-neuroradiological discrepancies are discussed in view of the literature.
[Febrile neutropenia: practical aspects].
BACKGROUND: Infections are a major cause of mortality in neutropenic patients. They require long hospital stays and highly expensive therapeutic measures. In this review we discuss the practical and pharmaco-economic aspects of the management of febrile neutropenia. PREVENTION AND THERAPY: Prevention of fever of unknown origin (FUO) demands hygienic and antimicrobiotic measures. First-line antibiotic therapy consists of an aminoglycoside combined with an ureidopenicillin or a 3rd-generation cephalosporin. Double beta-lactam antibiotic combinations are equally effective and less toxic, but more expensive. Monotherapy with carbapenems, ceftazidime, or cefepime appear to offer comparable efficacy. Lung infiltrates require immediate treatment with amphotericin B. If the initial therapeutic regime fails, a carbapenem plus a glycopeptide antibiotic and a parenteral antimycotic drug should be applied after 3 to 4 days. The prophylactic or interventional administration of hematopoietic growth factors is only indicated in special high-risk situations. CONCLUSIONS: Using the described therapeutic procedure, the response rate exceeds 90%. Consistent, step-wise escalating administration of antibiotics is essential. More evaluation is needed to determine whether selected patients with febrile neutropenia can be treated on an outpatient basis.
[Antimicrobial therapy of neutropenia].
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Thrombocytosis in polymyalgia rheumatica: an additional diagnostic criterion and possible risk factor for ischemic complications?
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[The diagnosis of pulmonary embolism].
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Recombinant human granulocyte colony stimulating factor in patients with systemic lupus erythematosus associated neutropenia and refractory infections.
OBJECTIVE: To investigate whether human recombinant granulocyte colony stimulating factor (GCSF) is capable of inducing increased neutrophil granulocyte (polymorphonuclear leukocytes, PMN) counts in patients with systemic lupus erythematosus (SLE) associated neutropenia and refractory infections. METHODS: Nine patients with SLE associated neutropenia and concomitant refractory infections received a total of 12 cycles of 48 Mio U GCSF per day subcutaneously for an average of 6 days (range 1-17 days) as an adjunct to antibiotic treatment. In one case of impaired wound healing, longterm GCSF was applied over 148 days. RESULTS: In each case, the average PMN count increased distinctly within 2 days from 1.3 per nl (range 0.7-2.4) to 8.4/nl (3.2-19.4). Major adverse events were exacerbation of central nervous system symptoms in 2 patients and leukocytoclastic vasculitis in one. CONCLUSION: GCSF induces a rapid increase in PMN counts in patients with lupus associated neutropenia and normal or hyperplastic granulopoiesis. In 3 of 9 patients we observed a flare of lupus associated symptoms.
[Thrombocytosis in polymyalgia rheumatica].
BASIC PROBLEM AND OBJECTIVE OF STUDY: The diagnosis of polymyalgia rheumatica is made difficult by the absence of any pathognomonic findings. Erythrocyte sedimentation rate (ESR) and concentration of C-reactive protein are used as diagnostic criteria and to monitor the disease's course. It was the purpose of this study to evaluate prevalence of thrombocytosis and whether there is any correlation between thrombocytosis and ischaemic complications. PATIENTS AND METHODS: In a retrospective study the data of 23 patients (14 women, 9 men; mean age 67.3 [48-84] years) with polymyalgia rheumatica were analysed: platelet counts, ESR and C-reactive protein had been measured at the time of diagnosis and 7, 14 and 21 days after start of glucocorticoid treatment. At the time of diagnosis 17 of 23 patients (74%) had a thrombocytosis (> 400,000/microliter), at a mean value of 511,000 (418,000-638,000)/microliter. The platelet count decreased more slowly than the inflammatory parameters. On average the platelet count returned to normal on the 14th day. Ischaemic complications developed in three patients (visual disturbances, blindness, cardiac arrhythmias, neurological deficits): all had a high platelet count (462,000-524,000/microliter). CONCLUSIONS: Thrombocytosis can serve as an additional criterion in the diagnosis and follow-up of polymyalgia rheumatica. An association with ischaemic complications is especially likely in the elderly, in case of immobility and if there is evidence of giant-cell arteritis. Treatment with platelet aggregation inhibitors should be considered.
Multiple organ manifestations in thromboangiitis obliterans (Buerger's disease). A case report.
Thromboangiitis obliterans (TAO) occurs almost exclusively in young male smokers. Its involvement of the small and medium-sized arteries and veins leads to ischemic complaints and/or changes in the extremities. The possibility of organ involvement is a matter of controversy. The authors report a case of TAO with multiple organ involvement, including myocardial, splenic, and cerebral infarctions; pulmonary embolisms; and probable intestinal ischemia during a twenty-three-year course.
[New knowledge on HIV pathogenesis].
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Liposomal amphotericin B therapy in disseminated histoplasmosis.
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Lupus anticoagulant and heparin-associated bleeding.
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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.
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.
[Septic histoplasmosis with disseminated osteosclerosis].
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[Intensified therapy of severe lupus erythematosus].
14 female patients (mean age 28 [18-56] years) with severe systemic lupus erythematosus (SLE) were treated after discontinuing previous immunosuppressive therapy, according to an intensified protocol, with three plasmaphereses (days 1-3), followed by pulse cyclophosphamide (12 mg/kg i.v. each on days 3-5) and then oral immunosuppression (cyclophosphamide 1-5 mg/kg daily, depending on white blood cell count; prednisone equivalent 2.0 decreasing to 0.1 mg/kg, according to response, for 6 months). The aim of "synchronization" of plasmaphereses with subsequent cyclophosphamide pulse-therapy is to damage pathogenic lymphocyte clones during maximal compensatory activation induced by plasmapheresis. In all patients there was rapid improvement from the nephritis, pneumonitis, cytopenia, CNS abnormalities and polyserositis. The lupus activity index (SLAM) fell clearly, from initially 28.4 (13-37) points to 8.9 (2-13) after 6 months. Treatment was discontinued after this fall in 12 patients. A recurrence was observed in two patients, at 12 and 39 months respectively. Another patient died from liver cirrhosis of unknown aetiology. Nine patients are under observation but without treatment at present, in essential remission after 2 years (5-51 months), with a SLAM of 2.8 (0-7) points. "Synchronization" of plasmaphereses with subsequent pulse cyclophosphamide achieved rapid improvement and it resulted, for the first time, repeatedly in long-term treatment-free clinical remissions.