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Biomedical subjects

K Konrad

Publications and source records attributed to K Konrad.

At least 127 records · Page 7Linked to original sources

Photochemotherapy for pustular psoriasis (von Zumbusch).

Photochemotherapy (PUVA) with oral methoxsalen and UV-A was instituted in 8 patients with generalized pustular psoriasis (von Zumbusch). Complete clearing of skin lesions and of systemic symptoms was achieved in all patients. Patients who had been on systemic treatment prior to PUVA had to be treated with considerably more UV-A energy than patients who had received topical therapy alone. Seven patients were kept in complete remission by maintenance therapy for an observation period of up to 1 1/2 years. Side effects of systemic pre-PUVA treatment resolved during several months of maintenance therapy.

Adolescent↗

Histiocytosis X in adult with skin and uncommon central nervous system involvement.

Report of a case of histiocytosis X with adult onset, widespread skin lesions and rare central nervous system involvement resulting in dizziness and ataxia. On autopsy histiocytosis X infiltrates in the central nervous system were demonstrated with granuloma formation in the temporal lobe and nucleus dentatus of the cerebellum.

Central Nervous System Diseases↗

[Photochemotherapy for psoriatic patients treated with corticosteroids and methotrexate].

The effect of photochemotherapy with oral administration of 8-methoxypsoralen followed by irradiation with UVA (PUVA) was investigated in 34 cases of severe, generalized psoriasis which could be controlled only by systemic administration of corticosteroids and cytotoxic agents. In each case clearing of psoriatic lesions was achieved under PUVA treatment. As expected, the average duration of the initial PUVA treatment period was longer when compared to patient groups not pretreated with corticosteroids and cytotoxic agents. In order to avoid relapses, maintenance treatment was installed after complete healing of psoriatic lesions comprising an average of one PUVA exposures per week. Eighty percent of such treated patients are continuously completely clear; the remaining patient group is 70--90% improved. The side effects, elicited by the previous administration of corticosteroids and/or cytotoxic agents are improved or have resolved under PUVA therapy. The present study indicates, that PUVA treatment of psoriasis is not only an alternative to the use of systemic corticosteroids and cytotoxic agents but is superior to these treatment modalities due to its lack of side effects and its higher effectiveness.

Adrenal Cortex Hormones↗

The pigmentary response to photochemotherapy.

Previous studies on skin topically photosensitized with trimethylpsoralen and subsequently irradiated with long-wave UV light have demonstrated an increase in melanosome size and changes in the distribution patterns of melanosomes, suggesting the possibility of gene derepression or the induction of a somatic mutation of melanocytes. The present investigation was performed to determine whether identical changes are induced by systemic photochemotherapy using 8-methoxypsoralen and UVA (PUVA) under therapeutic conditions. Our results show that PUVA stimulates melanogenesis but does not induce significant changes in the average size of melanosomes nor in their distribution patterns within keratinocytes. Thus they indicate that under therapeutic conditions PUVA does not induce morphologically detectable cytogenetic changes in pigment cells.

Adult↗

Mouse model for protoporphyria. III. Experimental production of chronic erythropoietic protoporphyria-like skin lesions.

Albino mice were made protoporphyric with griseofulvin according to an established procedure. Photosensitivity flares were elicited once a week throughout a 10-month period, using black light as a source for 410 nm radiation and the flares were monitored by the intravenous injection of vascular tracers and by light and electron microscopy. Each irradiation led to a selective destruction of the endothelial cells of superficial capillaries which was followed by massive vascular leakage. The basal lamina remained largely intact, providing the scaffold for regenerating endothelial cells which deposited new basal lamina material at their periphery. Subsequent exposures to 410 nm radiation reproduced the endothelial damage and subsequent basal lamina formation; multiple irradiations thus resulted in excessive, concentric, tubelike basal lamina deposits around dermal vessels which light microscopically appeared as PAS-positive hyaline material and clinically gave the skin a thickened, waxy appearance. This model thus reproduced the skin of erythropoietic protoporphyria clinically, microscopically, and at the ultrastructural level.

Animals↗

Experimental induction of hepatocellular hyalin (Mallory bodies) in mice by griseofulvin treatment. 1. Light microscopic observation.

Griseofulvin (GF) feeding of mice resulted in protoporphyria, liver cell damage, bile duct alterations, and finally hepatoma formation. In addition, hepatocellular hyalin developed, resembling in its morphology classic Mallory bodies (MB) as seen in alcoholic and nonalcoholic liver disorders in man. Liver cells containing MB often displayed features of severe cell damage and MB were finally released into the sinusoids and degraded by macrophages. The rapid disappearance of MB following GF discontinuation and the reappearance after resumption of GF feeding suggest an intimate relationship between metabolic alterations in the hepatocytes exerted by the drug and MB formation. This assumption is further supported by the fact that MB change their tinctoreal properties in chromotrope aniline blue-stained sections after GF discontinuation, possibly relfecting degeneration. Long term GF treatment apparently primed the liver for MB formation since the cells were able to respond almost instantly with MB to a GF challenge after a 1-month GF-free period.

Animals↗

[Photochemotherapy of psoriasis: clinical experiences with 152 patients (author's transl)].

Photochemotherapy, a recently developed method of treatment, is based on the effect of light on a systemically administered photo-active substance. 152 patients with severe generalized psoriasis were exposed, after oral administration of 8-methoxy-psoralen, to a high-intensity UVA light source radiating at a continuous spectrum between 320 and 390 nm (maximum at 365 nm). Radiation doses ranged from 1.4 to 4.8 J/cm2 and were progressively increased. Complete remission occurred in 141 patients, satisfactory improvement in the remaining 11. It required 12+/-6 radiation sessions (-/x +/- s) to obtain remission, mean duration of treatment being 22 +/- 14 days. Apart from a neutral ointment no other local or systemic therapy was used. Photochemotherapy completely healed the psoriatic foci leaving evenly tanned, cosmetically appealing skin. All patients were treated at intervals as out-patients (average once a week or ev-ry other week), and remained without recurrence for up to 400 days. No local treatment is required with photochemotherapy, which is very effective, simple, harmless and does not stress patient or doctor. Interval treatment maintained the cure.

Adolescent↗

[Local therapy of extensive severe burns: an illustrative review (author's transl)].

Apart from improvements in general management, the implementation of modern local therapy of burns, including topical chemotherapy and early surgical coverage of the burned surface, has significantly reduced the morbidity and mortality of severely burned patients. Furthermore, the average time of hospitalization has decreased (for example: 3rd degree burn, 60% of body surface involved, hospitalization in 1953: 11 months; in 1974: 5 1/2 months) and the rehabilitation period has also been shortened. This paper deals with current management at this department in respect to the local treatment of extensive 3rd degree burns. Special consideration is given to the different possibilities of wound closure and their indications, to therapeutic problems inherent in the special localization of certain burns and to the importance of physical therapy for early rehabilitation. In severe burns additional injuries should be considered and excluded by careful examination.

Adrenal Cortex Hormones↗

[Giant melanosomes in moles and in normal human epidermis (author's transl)].

This is the first report on the occurrence of giant melanosomes in normal human epidermis and in moles. Due to their characteristic ultrastructural features these large pigment organelles are of special interest for the research in melanin pigmentation. The diferentiation of giant malanosomes from other types of large melanin granules is discussed.

Adult↗

Mouse model for protoporphyria. I. The liver and hepatic protoporphyrin crystals.

Outbred albino mice were rendered protoporphyric by a diet containing 2.5% (weight) of griseofulvin. There was a 5-fold increase in liver weight, hepatocellular degeneration and necrosis, cholestasis, ductular proliferation and cirrhosis. Liver protoporphyrin values were elevated and brown pigment granules were present in hepatocytes, Kupffer cells, and bile ducts. The granules showed red fluorescence, birefringence, and, at the ultrastructural level, consisted of aggregates of needle-like crystals. Crystals isolated from such livers showed solubility and absorption characteristics of protoporphyrin; in vitro recrystallization of protoporphyrin, extracted from protoporphyric mouse livers, yielded crystals identical with those observed in vivo, and commercial protoporphyrin exhibited similar morphologic features. The liver pathology and protoporphyrin crystals observed in these animals are identical to the liver pathology and crystals observed in the human disease, erythropoietic protoporphyria. In this mouse model, protoporphyrin crystals are intimately associated with hepatocellular injury and it appears that their accumulation within hepatocytes leads to hepatocellular destruction. A similar pathogenesis is postulated for the hepatic damage that occurs in some cases of erythropoietic protoporphyria.

Animals↗

Mouse model for protoporphyria. II. Cellular and subcellular events in the photosensitivity flare of the skin.

Acute phototoxic reactions were induced by long-wave ultraviolet light (UV-A) in mice with griseofulvin-induced protoporphyria. The clinical response was characterized by erythema, pronounced edema, and purpura. Tracer experiments and electron microscopy revealed pronounced vascular damage and leakage of vascular contents, whereas the epidermis and all other dermal components were intact. There was selective destruction of endothelial cells and damage of the basal lamina of the vessels. This striking vascular injury was absent from nonprotoporphyric UV-A-irradiated mice and from protoporphyric and nonprotoporphyric mice exposed to short-wave ultraviolet light (UV-B). Patients with erythropoietic protoporphyria (EPP) exhibit an identical, selective damage of blood vessels when irradiated with UV-A or sunlight but not with UV-B alone. It is hypothesized that in both murine protoporphyria and EPP, endothelial cells are photosensitized by protoporphyrin circulating in the serum and that photosensitized endothelia represent the primary cellular target of the photochemical reaction induced by UV-A.

Animals↗

Erythropoietic protoprophyria--submicroscopic events during the acute photosensitivity flare.

The immediate response of erythropoietic protoporphyria (EPP) skin to long-wave ultraviolet radiation (UVR) was studied with the electron microscope. The main finding was severe vascular injury. This was confined to the superficial vessels of the dermis and consisted of endothelial cell degeneration and a pronounced leakage of vascular contents. In contrast, the epidermis showed no abnormalities. Short-wave UV irradiation of EPP skin resulted in epidermal changes typical for the usual sunburn reaction and spared the dermal blood vessels. The following conclusions are drawn: (i) Endothelial cells are the primary cellular target for the photodynamic reaction in EPP. (ii) The fibrillar material, characteristic for chronic EPP lesions, originates from the vessels and vascular contents. (iii) The multilayered basement membranes observed in such lesions reflect multiple consecutive reparative processes that follow endothelial injuries.

Adult↗