[Langerhans cell histiocytosis].
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Biomedical subjects
Publications and source records attributed to M Kousa.
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The acute clinical picture and long-term prognosis of 160 patients with Reiter's disease (RD), and 144 patients with reactive arthritis triggered by yersinia infection (YA), was analysed. Most of the patients with RD were men, while YA was as common among male and female patients. YA manifested in a third of patients as incomplete or complete RD. The long-term prognosis in RD was less favourable than in YA. Recurrent arthritis, development of chronic destructive arthritis and radiologic sacroiliitis were more frequent in RD than in YA. The presence of HLA-B27 was the major factor determining extra-articular inflammatory symptoms and the development of chronic problems.
The hospital records of 242 patients with diagnostic chlamydial complement fixation (CF) titres (seroconversion and/or titre greater than or equal to 64) found among 60,000 patients screened for suspected viral illnesses were reviewed to study the clinical conditions associated with positive CF serology for Chlamydiae. After excluding typical genital C. trachomatis infections, the majority of the remainder were considered to represent C. psittaci infections. Respiratory symptoms were the most common clinical manifestations of chlamydial infections detectable by CF, but the majority (58%) of the patients did not have pneumonia. Abdominal, neurological as well as urinary tract symptoms were common. Cutaneous, joint, cardiac, genital and ocular manifestations were also noted. Fever (greater than or equal to 38.5 degrees C) was present in 62% of the patients. The ESR was raised (greater than or equal to 20 mm/h) in the majority of the patients (83%), but the leucocyte count was usually (86%) within normal limits. Because the clinical spectrum of C. psittaci infections is apparently broad, serological tests for detecting antibodies to C. psittaci (e.g. CF) should be used widely in various clinical conditions and not for patients with pneumonia alone.
An analysis of 160 patients with Reiter's disease, 144 with yersinia arthritis, and 9 with salmonella arthritis was performed, Complete or incomplete Reiter's syndrome was observed in one-third of the patients with yersinia arthritis and in most of those with salmonella arthritis. During the followup period, chronic back pain and joint symptoms were frequent in all the patient groups. Patients who were HLA-B27 positive had a more severe acute disease (more frequent back pain, urologic symptoms, mucocutaneous manifestations, and a longer duration of the disease) and more frequent chronic back pain and sacroiliitis.
Penile lesions from six patients with balanitis circinata were examined with the light and electron microscopes. The epithelium showed slight parakeratosis, acanthosis, and elongation of rete ridges. Neutrophil pustules occupied the upper epidermis. Prickle cells formed a spongiform net around the pustules. The most prominent features detected by the electron microscope were the small-to-medium-sized pustules in the upper epidermis. The thin flattened keratinocytes formed a sponge-like trabecular network. Neutrophils were not found inside the keratinocytes. Chlamydia were not present in the lesions, which indicates that balanitis circinata is due to some reactive mechanism. The fine structure of balanitis circinata resembles that of pustular psoriasis.
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The isolation and immunological studies reviewed here appear to have proved that there is an association with Chlamydia trachomatis in reactive arthritis, and particularly in Reiter's disease. This condition, which occurs predominantly in young men, usually is precipitated by infection, sometimes enteritic but at present more commonly sexually transmitted. In the case of nongonococcal urethritis, C. trachomatis has been shown to be an agent that can trigger Reiter's disease. Patients with HLA B27 antigen are especially at risk. Clinical observations suggest that long-term tetracycline treatment may have a beneficial effect.
Treatment with an oral aromatic retinoid (etretinate) and with PUVA significantly reduced the elevated levels of putrescine, spermidine and spermine in psoriatic lesions. Both treatments also significantly reduced the spermidine/spermine ratio, which is considered to be an indicator of proliferation activity. Although both regimens produced a roughly parallel reduction of epidermal polyamines the initial fall of putrescine was much more rapid in patients receiving retinoid. This may indicate that one of the primary targets of retinoids could be ornithine decarboxylase.
Human epidermis, obtained in vivo by the suction blister method, was dissociated with trypsin and desoxyribonuclease into a single-cell suspension. Autoradiographic analysis of the blister roof epidermis and of the epidermal cell suspension was performed to show that neither the suction procedure nor the enzymatic dissociation affected DNA synthesis of the epidermal cells.
The effect of partially purified human leucocyte interferon on natural killer activity (NK activity) of peripheral blood in six healthy volunteers was tested in this study. After 4 h of intradermal injection of interferon, a rapid transient decline in NK activity and in the number of NK cells (large granular lymphocytes) in peripheral blood was observed. The decline was most distinct at 20 h and recovered at 72 h. Since no clear activation of Nk cells in peripheral blood was detected, the results suggest an extravasation of NK cells as a result of interferon injection. NK cells did not accumulate at the injection site on the skin, because the suction blister fluids over the injection sites did not contain increased numbers of NK cells.
Epicutaneous tests with 0.1% merthiolate in petrolatum showed hypersensitivity in 96 of 4647 eczema patients (2.0%) and in seven of 105 healthy recruits (7%). There was a marked preponderance of young age classes in the eczema group. Twelve of 41 merthiolate-positive patients tested reacted to mercury alone, three to thiosalicylic acid alone and one to both. The remaining 25 patients reacted to neither of the individual components although the merthiolate complex as a whole gave a positive test result. Forty-five of the merthiolate-positive patients were tested subcutaneously with 0.5 ml of a 0.01% merthiolate solution, i.e. a dose equal to that contained in one shot of tetanus toxoid, for example. Nine patients developed a local reaction at the site of the injection, and the area became eczematous in four cases. In one of the patients the eczema spread over the body, causing fever. Since merthiolate-sensitive patients also react to merthiolate administered intracutaneously, the vaccinator should avoid the use of a needle whose outer surface has been contaminated when the vaccine was aspirated from the bottle. However, even when this precautionary measure is taken, local reactions can be expected in such a high percentage of merthiolate-sensitive persons that merthiolate in vaccines should be replaced by another antibacterial agent.
Celery and parsley were confirmed as etiological agents in 14 patients with severe attacks of angio-edema and urticaria. All of the patients proved to be atopic. Without exception they all had positive prick test reactions to mugwort. The patients were examined using the scratch - chamber test method and all of them showed positive reactions to celery and 12 patients to parsley. No peroral provocation tests were performed because of the severity of the reactions in the past. Passive transfer by the prausnitz-Küster technique was positive in two patients.
Fourteen eczema patients, 13 women and one man, were found to have contact allergy to a brown stocking dye which is a mixture of nitro- and aminodiazobenzene and aminoanthraquinone dyes. Eleven of the patients reacted to p-phenylenediamine; nine of 12 tested reacted to aminoazobenzene, nine to Disperse Orange 3 and four to Disperse Yellow 3. Positive epicutaneous test reactions to the dye were found in five of 362 consecutive eczema patients tested (1.4%).
The effect of sulphadiazine and trimethoprim (T-S) was compared with that of a placebo in the treatment of 75 men with nongonococcal urethritis (NGU) and their female sexual partners. Forty (53%) men and 30 (40%) women had positive culture results for Chlamydia trachomatis before treatment was started. Thirty-four patients of each sex received active treatment. Of these, 19 men and 15 women had positive culture results for C trachomatis before treatment with T-S and one of each sex had a positive culture result after treatment. C trachomatis had been isolated in 21 men and 15 women before treatment with placebo. At follow up four weeks after treatment had been started, 16 men and 15 women still harboured C trachomatis. Five of the chlamydia-positive patients treated with placebo developed complications (epididymitis, arthritis, or salpingitis) so the trial was discontinued.