Erfolgreiche Langzeittherapie bei Stewart-Bluefarb-Syndrom

The Stewart-Bluefarb syndrome is defined as an unilateral angiodermatitis due to multiple arterio-venous fistules accompanied by acroangiodermatitis resembling Kaposi sarcoma (pseudo-kaposi sarcoma). The acroangiodermatitis is most common on the lower limb. It leads to ulcerated nodules with a high risk of bleeding and infection, as well as edema, pain and seldom limb hypertrophy. Curative therapy requires elimination of the arteriovenous shunts. Surgical destruction of the multiple small fistulae is a limitating factor. A better alternative is embolisation, but this approach carries the risk of ischemia and necrosis. A 32 year old female patient with Stewart-Bluefarb syndrome is presented; she has been successfully treated with embolisation on eight occasions.     

Chlamydia trachomatis in Andrologic Patients – Direct and Indirect Detection

Background: Chlamydia trachomatis is considered to be the most common sexually transmitted disease in Germany. It is currently unclear whether chlamydial infection causes pathological conditions of the male accessory glands with consequences for male infertility. Patients and Methods: Within the framework of several prospective studies the association between sperm quality, male accessory gland function and infection with C. trachomatis was investigated in men of couples with unexplained infertility. Chlamydial infection was determined by serologic methods and by proof of Chlamydia-specific DNA. As a marker of infection the direct determination of granulocytes in the ejaculate or the measurement of the polymorphonuclear (PMN) elastase concentration was used. The male accessory gland function was evaluated using the markers fructose, citric acid and α-glucosidase in the seminal plasma. Results: Chlamydia-specific DNA in the ejaculate was present in between 3–5% of the subjects, which corresponds to it s prevalence in the normal population. Chlamydia IgA antibodies were demonstrated with a frequency of 38% in seminal plasma (n = 834) using a genus-specific test (rELISA). Using other species-specific tests (MIF, SeroCT, IgA pELISA and ImmunoComb), Chlamydia IgA antibodies were found at frequencies of between 8 and 22%. Conclusion: Only in a few individual cases was it possible to show a connection between reduced sperm quality, disturbed male accessory gland function and indication of infection with Chlamydia, bacteria or Ureaplasma. Received: May 23, 2000 · Revision accepted: December 20, 2000     

Autografting of highly purified peripheral blood progenitor cells following myeloablative therapy in patients with lymphoma: a prospective study of the long-term effects on tumor eradication, reconstitution of hematopoiesis and immune recovery.

In a prospective study, we have investigated CD34+ selection of peripheral blood progenitor cells (PBPC) for autotransplantation in patients with lymphoma. Twenty-six consecutive patients (10 follicular lymphomas, seven mantle cell lymphomas, seven B-CLL, two immunocytomas) were mobilized using chemotherapy plus G-CSF. Sufficient numbers of PBPC could be collected from 24 patients and were immunoselected with the semiautomated Isolex 300 (n = 17) or the fully integrated Isolex 300i (n = 7) devices. The selection products were assayed by PCR amplification of clonal CDRIII or t(14;18) rearrangements for residual tumor cell content. Residual disease and long-term hematopoietic and immune recovery were studied by assessing the following parameters at 3, 6, and 12 months post-transplant: CDRIII or t(14;18) PCR, platelet count, lymphocyte subsets, serum IgG, serum IgA, and measles titer. With the Isolex 300 device 26% (10-65) of input CD34+ cells were recovered with a median purity of 89.2% (49.4-98.9) after CD34+ selection. The Isolex 300i device allowed significantly better recoveries (46% (22-86)) and purities of CD34+ cells (98.8% (92.2-99.2)). The overall purging efficacy was 3.2 (0.6-5.1) log. Twenty patients have been reinfused with CD34+ selected grafts after myeloablative preparation. Rapid engraftment occurred in all patients. With a median follow-up of 28 (19-42) months, 14 patients are alive without clinical or molecular evidence of disease recurrence, whereas five have relapsed and one additional patient shows persistent presence of the disease-specific molecular marker without clinical progression. Cellular and serological parameters of hematopoietic and immune functions were largely normal at 12 months post-transplant including the measles titer which was present in all patients. Kinetics of immunohematopoietic recovery were similar to those of 12 control patients who had received unmanipulated PBPC during the same time period except for the recovery of CD4+ CD45RA+ T cells which was significantly delayed in the CD34+ group. During the first year post-transplant, transient monoclonal or oligoclonal gammopathies were observed in seven of 16 study patients. We conclude that CD34+ selection with the Isolex system allows preparation of highly purified CD34+ fractions and effective tumor cell depletion. The CD34+ products can be reinfused safely after myeloablative treatment and result in sustained hematopoietic and immune recovery. The fact that all patients retained their specific measles immunity suggests that myeloablative treatment with reinfusion of highly purified CD34+ PBPC is not immunoablative.     

Current status of vaccine development in sexually transmissible diseases

A prophylactic vaccine represents a major hope for the control of sexually transmitted diseases. The current general vaccine strategies and the status of vaccine development against infections with Neisseria gonorrhoeae, Treponema pallidum, Chlamydia trachomatis and Herpes simplex virus are described. Vaccines consisting of whole infectious agents are replaced by protective subunits. A subunit vaccine has the advantage to be free from other components, which are not relevant for protection and which may confer unwanted side effects. At the present time vaccine development against infections with Neisseria gonorrhoeae and Herpes simplex virus seems to be the most progressed. With monoclonal antibodies several surface components could be identified, which are of importance for the pathomechanism. With Treponema pallidum and Chlamydia trachomatis the development is delayed by unsolved problems of immunity. For the production of vaccines molecular-biologic methods, like protein synthesis or gene-cloning will be used. Genetically modified live vaccines or polytope hybrid vaccines will gain importance in the future.     

False-positive results in the detection of anti-LAV/HTLV-III antibodies by enzyme immunoassay

Two groups of sera were investigated with two different enzyme immunoassays for the detection of anti-LAV/HTLV-III-antibodies: 50 sera collected from patients with lupus erythematosus and 5 sera containing anti-HLA-DR4 antibodies. Of the 50 sera of the lupus erythematosus patients, 2 were positive in each of the tests. Of the 5 sera that contained anti-HLA-DR4 antibodies, one was positive. In addition, 11 sera showed positive results after heat treatment (56 degrees C, 1 h) in one of the tests. However, all sera in this group gave negative results in the other test. The positive results could not be confirmed with the immunoblot. These findings show that one must be aware of false-positive results when the sera of certain types of patients are investigated in anti-LAV/HTLV-III enzyme immunoassays. The sera of patients with lupus erythematosus, as well as that of polytransfused persons or multiparous women, that have been sensitized with HLA-DR4 antigens; heat treatment in one test has an effect on sera.     

The value of symptoms and findings in urethral Chlamydia trachomatis infection

The history and the results of clinical investigations in 74 patients with and 262 men without urethral Chlamydia (C.) trachomatis infection were evaluated: symptoms such as dysuria, discharge and/or burning/itching in the genital region were reported by 77% (n = 57) of the patients with an infection and 63.4% (n = 166) of the men without an infection. Upon clinical investigation, discharge was found in 55.4% (n = 41) of the chlamydia-positive patients and in 47.7% (n = 125) of the chlamydia-negative men. Only when yellow, whitish yellow and clear discharge were distinguished from one another was the difference between the two groups of patients (56.1% versus 12%) significant. Microscopic signs of urethritis, i.e. significant numbers of polymorphonuclear leukocytes (greater than 4 per high-power field and oil immersion) were detectable in the smears of 59.5% (n = 44) of the chlamydia-positive patients, in contrast to 15.6% (n = 41) in the smears of chlamydia-negative men. Overall, 67.6% (n = 50) of the patients with a C. trachomatis infection reported symptoms in the anamnesis combined with signs of urethritis upon clinical and microscopic investigation. There were 9.5% (n = 7) of the patients who either mentioned symptoms or showed clinical signs, and 13.5% (n = 10) who neither mentioned symptoms nor showed clinical signs. These results indicate that microbiological detection of the infectious agent is obligatory for diagnosis of urethral infection with C. trachomatis.     

Netherton's syndrome: ultrastructure of the active lesion under retinoid therapy

Summary A young female patient, expressing the symptom triad of Netherton's syndrome, i.e., ichthyosis linearis circumflexa Comèl, trichorrhexis invaginata and other hair shaft defects, and atopic diathesis, has been treated successfully with the new retinoid preparation Etretin. Our electron microscopical study especially focused on the ultrastructural effect on the characteristic, active part of the skin lesions, which is only found within a narrow borderline just preceding the lesion's margin. In untreated skin, this part is characterized by dermal inflammation, immigrating inflammatory cells, and specific keratinization disturbances: synthesis of keratinization proteins is suppressed, serum exudates invade the epidermis, either filling the intercellular spaces of the upper spinous and the granular layer as finely granular, amorphous material, or they are partly phagocytosed and lie within intracellular, round-oval inclusions. The portions of the lesions lying towards the center are unspecific and represent recovery stages, ultrastructurally resembling stages of normal wound repair. Oral therapy with Etretin did not heal the basic defect, but drastically reduced exoserosis and the deposition of intra- and extracellular material. Keratinization seemed to normalize. The condition of the hair was also improved.     

Serovar distribution of urogenital C trachomatis isolates in Germany.

Using monoclonal antibodies in a microimmunofluorescence test the distribution of C trachomatis serovars isolated in the STD out-patient clinic of the Department of Dermatology of the University of Heidelberg was determined. Of 56 isolates investigated the most frequent was serovar E with 35.7%, followed by serovar D with 28.6% and serovar F with 26.8%. This shows that the distribution of C trachomatis in Germany is very similar to that in other Western countries.     

Rosiglitazone plus metformin is effective and well tolerated in clinical practice: results from large observational studies in people with type 2 diabetes

This study investigated the efficacy during daily practice of rosiglitazone (RSG) added to metformin (MET) in poorly controlled type 2 diabetes mellitus. Two post-marketing observational studies were conducted in Germany over 6 months. RSG (4 mg/day titrated to 8 mg/day as required) was added to existing MET in 11,014 subjects. Subjects were maintained on diet and exercise. Addition of RSG to MET significantly reduced median HbA 1c by 1.3% (8.1 vs. 6.8%; p < 0.0001) and median fasting blood glucose (FBG) by 47.0 mg/dl (171.0 vs. 124.0 mg/dl; p < 0.0001) after 6 months. The proportion of subjects achieving HbA(1c) targets of < or = 6.5 and < or = 7.0% increased from 3.5 to 38.8% and from 13.5 to 63.7%, respectively. Mean systolic and diastolic blood pressure decreased by 7 and 3 mmHg, respectively (p < 0.0001). Mean weight decreased by 1.7 kg and was constant or reduced in most (74.1%) subjects. Addition of RSG to MET significantly reduces median HbA 1c and FBG in clinical practice and is generally well tolerated.