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Biomedical subjects

K Bork

Publications and source records attributed to K Bork.

At least 19 recordsLinked to original sources
PubMed1990

Pathogenesis of paraneoplastic follicular hyperkeratotic spicules in multiple myeloma. Follicular and epidermal accumulation of IgG dysprotein and cryoglobulin.

We describe a 62-year-old man with multiple myeloma who developed horny spicules on his face, particularly on his nose. IgG-lambda monoclonal gammopathy was detected, and the serum dysprotein was shown to be a cryoglobulin, which forms a cryogel at low temperatures. Light and electron microscopic and immunohistochemical examinations showed an intercellular precipitation and massive accumulation of the IgG dysprotein and cryoglobulin between the keratinocytes of the upper epidermis and the infundibular epithelium. The follicles were dilated and filled with parakeratotic cells, the protein deposits between them and a rudimentary hair thus resulting in the clinically visible symptoms of horny spicules. The limitation or the predominance of the symptoms in cold-exposed body regions, the morphological identification of the dysprotein deposits as cryoglobulin or cryogel, and the laboratory findings concerning the temperature and pH dependence of the precipitation of the IgG dysprotein reveal that the paraneoplastic horny spicules are a hitherto unknown clinical manifestation of cryoglobulinemia.

Cryoglobulinemia↗
PubMed1990

Fat-storing hamartoma of dermal dendrocytes. Clinical, histologic, and ultrastructural study.

A 30-year-old man is described who since birth had a peculiar symmetrical red-brown micronodular cutaneous plaque. Within the slightly raised plaque, small firm nodules were palpable. Histologic examination revealed phagocytic cells with the appearance of histiocytes between the collagen fibers of the reticular dermis. Immunohistochemical and electron microscopic examination confirmed that the cells of the plaque were dermal dendrocytes with phagocytic activities. The existence of the lesion since birth, the phagocytosis of lipid droplets by the cells, and the lack of associated metabolic disturbances suggest a fat-storing hamartoma of dermal dendrocytes that does not correspond to a known fat-storing or fat-producing tumor.

Adult↗
PubMed1989

Long-term prophylaxis with C1-inhibitor (C1 INH) concentrate in patients with recurrent angioedema caused by hereditary and acquired C1-inhibitor deficiency.

A case of hereditary angioedema (HAE) type I (inherited C1-inhibitor [C1 INH] deficiency) and a case of late-onset acquired C1 INH with angioedema is described. In both patients, long-term prophylaxis with C1 INH had become necessary because treatment with danazol and epsilon-aminocaproic acid was not effective or not tolerated. Consequently, both patients received a pasteurized concentrate of C1 INH continuously for a period of 1 year in a dosage that kept them free of symptoms. The patient with HAE was administered 500 units of C1 INH intravenously every 4 or 5 days, whereas the patient with acquired angioedema required 1000 units of C1 INH every 5 days. As a result of this long-term prophylaxis, both patients became free or nearly free from their episodes of cutaneous and internal edema. The low plasma levels of C1 INH, C4, and C2, rose. In the patient with acquired C1 INH deficiency, the swellings increasingly reappeared after 10 months, although the patient's antibody titer did not rise during treatment. No side effects were recorded during therapy. In particular, both patients remained HIV and hepatitis B antibody negative.

Angioedema↗
PubMed1989

Persistent skin ulcers, mutilations, and acro-osteolysis in hereditary sensory and autonomic neuropathy with phospholipid excretion. Report of a family.

We observed three children in a Turkish family who from early childhood had deformations of the feet and torpid ulcers with subfocal osteomyelitis and osteolysis, which subsequently led to amputations. The fingers showed ainhumlike constriction bands and spontaneous amputations. Neurologic studies revealed an almost complete sensory and autonomic loss affecting all modalities and a marked involvement of motor fibers. The clinical symptoms fulfill many of the hallmarks of hereditary sensory and autonomic neuropathy type II, including autosomal recessive inheritance, onset of symptoms in childhood, and mutilating acropathy. A high urinary excretion of sphingomyelin and lecithin suggests that the pathogenic mechanism may be a disorder of phospholipid metabolism.

Adult↗
PubMed1989

Hereditary angioedema associated with subacute cutaneous lupus erythematosus.

The increased occurrence of various autoimmune diseases has recently been reported in patients with hereditary angioedema (HAE). This is especially the case in different forms of lupus erythematosus, but also other autoimmune diseases. We report a 24-year-old female patient who 10 years ago developed the clinical symptoms of HAE which occurred at the same time as subacute cutaneous lupus erythematosus. The results of both immunological investigations and histocompatibility antigen genotyping gave no clear insight into the causal interrelationship of both diseases.

Adult↗
PubMed1989

Acquired C1 inhibitor (C1-INH) deficiency type II. Replacement therapy with C1-INH and analysis of patients' C1-INH and anti-C1-INH autoantibodies.

The response of two patients with autoantibody-mediated C1-inhibitor (C1-INH) deficiency to replacement therapy with C1-INH was studied over a period of 3 d. In patient 1 an acute attack of angioedema was successfully managed by infusion of 1,000 U of C1-INH concentrate. C1-INH function returned to normal levels within 30 min, while CH50 and C4 peaked after 6-7 h and C1 hemolytic activity reached 50-60% of normal after 3 d. Immediately after the injection an increase in C1-INH-anti-C1-INH complexes was observed. Based on NH2-terminal sequence analysis of the patients' Mr 96,000 C1-INH, it is concluded that this fragment is generated after cleavage of C1-INH in its active site by one of its target proteases without generating a covalent C1-INH-enzyme complex. In a second patient with a four to five times higher anti-C1-INH antibody titer, the infusion of 500 ml of plasma or of 2,000 U of C1-INH concentrate influenced neither the severity of the patient's angioedema nor the tested parameters, except for an increase in the amount of C1-INH-anti-C1-INH complexes. Analysis of patients' anti-C1-INH antibodies revealed that the antibodies recognize different epitopes within the C1-INH. This suggests that patients with acquired angioedema type II are a heterogenous group with respect to the C1-INH autoantibodies.

Amino Acid Sequence↗
PubMed1989

Efficacy and safety of 2% mupirocin ointment in the treatment of primary and secondary skin infections--an open multicentre trial.

Staphylococci and beta-haemolytic streptococci are usually responsible for causing common primary and secondary skin infections. Mupirocin (Bactroban, Eismycin; trademarks of Beecham Group plc), a new antibiotic unrelated to any other antibacterial agent and developed for topical use only, shows a high level of activity against these bacteria. In an open multicentre study the efficacy and safety of mupirocin (2% in a polyethylene glycol vehicle) was evaluated in 1,391 general practice patients with superficial skin infections. The most common skin infections treated were pyoderma (eg, impetigo, folliculitis) and secondarily infected skin lesions. Treatment consisted of application of the ointment three times daily for an average of nine days. A total of 1,304 patients were evaluable for post-treatment clinical assessment. At the end of the treatment 961 (73.7 per cent) patients were cured and in 293 (22.5 per cent) patients the symptoms of the infection had markedly improved. In total, 525 bacterial strains were isolated from the wounds of 445 patients, predominantly staphylococci (n = 344) and streptococci (n = 93). Local side effects such as burning, itching and reddening were observed in 39 (2.9 per cent) of 1,357 patients. No evidence of systemic toxicity or abnormal laboratory data was noted. Mupirocin 2% ointment proved to be effective and safe in the treatment of primary and secondary skin infections.

Anti-Bacterial Agents↗
PubMed1988

[Regional intra-arterial chemotherapy with Dacarbazine in isolated liver metastases of a malignant melanoma].

A 58-year-old woman underwent regional intra-arterial chemotherapy for hepatic metastases of a malignant melanoma, because at the time no other organ metastasis was demonstrable. Dacarbazine was injected as a bolus on five successive days, four weeks apart, after which there were several days of fever and the urinary melanogen test became positive. At first the patient clearly improved and was again able to walk. But four months after the start of treatment she died in a hepatic coma.

Chemotherapy, Cancer, Regional Perfusion↗
PubMed1988

Increasing incidence of eczema herpeticum: analysis of seventy-five cases.

Clinicians at the Department of Dermatology at the University of Mainz saw 63 patients with 75 episodes of eczema herpeticum. An analysis of these cases shows an exceedingly high rate of increased incidence of the disease. From 1969 through 1981, 13 cases were registered, whereas in the years 1982 to 1986 the number of cases was 62. The mean age of the patients was 22.7 years, and 42 patients (56%) were between 15 and 24 years of age. This finding shows that the affected patients are not predominantly infants, as has been reported up to now. Of these patients 5 had one recurrence, two had two recurrences, and one had three recurrences. Acyclovir, used for treatment in 38 cases, led to rapid improvement, whereas therapy with immunoglobulins, antibiotics, and so-called immunostimulating agents did not clearly influence disease duration.

Acyclovir↗
PubMed1988

[Flagellar motility amd movement of boar spermatozoa during epididymal transit].

The motility of boar spermatozoa during epididymal transit was analysed in vitro using various techniques. From the head to the cauda there was an increase of the percentage of motile and progressive spermatozoa. During maturation there was a progressive reduction of flagellar bend curvature while flagellar beat frequencies increased. A three dimensional pattern of flagellar beating responsible for cell rotation and straight line progression of spermatozoa was observed only in caudal epididymis. The addition of epididymal fluid protein to the media could increase the number of motile cells at the various levels but had no influence on the characteristics of flagellar bending.

Animals↗
PubMed1988

[Prurigo and further diagnostically significant skin symptoms in strongyloidosis].

An increasing incidence of strongyloidosis must be expected in European countries as a result of the increasing numbers of immigrants, as well as holiday-makers returning from tropical regions. In addition to gastrointestinal symptoms, dermatological complaints are predominant. Only rarely are cutaneous symptoms the only clinical manifestation of disease. The penetration of filariform larvae may cause "ground itch." In cases of chronic disease, larva currens is the most obvious sign and consists of linear urticarial wheals evoked by larva migration. The most common non-specific symptoms are rashes, pruritus and urticaria. A further symptom of strongyloidosis, intensely itching prurigo, is described in a 20-year-old female Thai. Remission was achieved following tiabendazole therapy.

Adult↗