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Biomedical subjects

G Goerz

Publications and source records attributed to G Goerz.

261 records · Page 15Linked to original sources

Multiple exercise-related mononeuropathy with abdominal colic.

Hereditary neuropathy with liability to pressure palsies (NLPP) is a rare disease characterized by recurrent sensory-motor deficits precipitated by exposure to minor pressure. This report describes a variant of this neuropathy in 5 siblings suffering from painful palsies after strenuous work with concurrent episodes of abdominal colic resembling that of acute intermittent porphyria. Electrophysiological studies of the index case showed the typical abnormalities of motor and sensory nerve conduction, including clinically non-affected nerves. Light and electron-microscopic examination showed the characteristic lesions of the NLPP with sausage-like swelling of the myelin sheaths. In addition, non-compacted, "loose" myelin lamellae were frequently observed in association with distended Schmidt-Lantermann incisures. Non-compacted myelin was a prominent finding in this type of demyelinating neuropathy. We suggest that an unknown metabolic factor may induce both demyelination of peripheral nerve fibers and functional disturbance in autonomic nerves leading to attacks of abdominal pain.

Abdomen↗

[Lupus erythematodesus profundus].

A 4 year old boy with lupus panniculitis since the third month is presented. Typical discoid lupus erythematosus lesions were also present. There were no signs of systemic involvement. Lupus panniculitis is an uncommon subtype of cutaneous lupus erythematosus. Our case is very unusual as the lesions developed already 3 month after birth. Clinical manifestation, therapy and histopathology of lupus panniculitis are discussed.

Adipose Tissue↗

Green light is effective and less painful than red light in photodynamic therapy of facial solar keratoses.

Photodynamic therapy (PDT) with topically applied delta-aminolevulinic acid (ALA) is increasingly employed to treat patients with multiple solar keratoses and superficial skin tumors. For these indications, ALA-PDT has been shown to be highly efficient. Treatment of multiple or extended lesions, however, is substantially hampered by the fact that ALA-PDT is associated with burning pain during the irradiation procedure. The standard irradiation devices commonly used for ALA-PDT emit red light around 630 nm. In the present half-side comparison study we have observed that ALA-PDT employing a green light irradiation device (543-548 nm) is equally effective, as compared with standard red light ALA-PDT. In contrast to red light ALA-PDT, however, green light ALA-PDT caused only little tingling and burning but no pain. These observations indicate that green light ALA-PDT is superior to standard ALA-PDT, because it is associated with less unwanted side effects.

Aged↗

Treatment of porphyria cutanea tarda with oral thalidomide.

Eight male patients with overt clinical and biochemical features of porphyria cutanea tarda (PCT) were orally treated with 300 mg/day thalidomide for 1 week and with 200 mg/day for 3 more weeks. Already after the first week of treatment no new vesicles and/or bullae could be observed. Spontaneous blisters completely disappeared, increased skin fragility subsided and skin hyperpigmentation receded about 2 months after completion of therapy, whereas hypertrichosis persisted. There was a rapid decrease in the urinary total porphyrin excretion which reached normal levels in all patients by the end of the fourth week of therapy, whereas the posttreament chromatographic pattern of urinary porphyrins revealed a slight reduction of higher carboxylated porphyrin metabolites and an increase in the amount of the excreted coproporphyrin, as compared to the pretreatment period. Somnolence, intermittent constipation and dry mouth occurred in all patients, 2 patients additionally experienced dizziness. No evidence of peripheral neuropathy could be detected and laboratory investigations revealed no abnormalities, as compared to the pretreatment period. During the 16- to 28-month follow-up of the patients, no clinical or biochemical relapse was observed. In view of the encouraging results of the present investigation, further studies are now warranted in order to definitely answer the question whether oral thalidomide may be regarded as an effective alternative approach to the treatment of PCT.

Administration, Oral↗

Alterations of rat liver phospholipid composition induced by oral thalidomide.

In an attempt to elucidate the effects of oral thalidomide on liver phospholipid composition, doses of 1 and 3 mg/kg/day of thalidomide were orally administered to two groups of female Wistar rats (7 animals each), respectively, over a period of 60 days. Control animals (n = 7) received corresponding quantities of the vehicle alone. Chromatographic analysis and quantitative determination of the isolated phospholipid classes revealed statistically significant alterations of phospholipid fractions in the liver of the animals treated with the higher thalidomide dose (3 mg/kg/day). These alterations may be associated with changes in the metabolic activity, ionic transport and cell-cell interactions of the hepatic cellular components.

Administration, Oral↗

[Animal experiment model of the effect of ultraviolet irradiation on hexachlorobenzene-induced porphyria].

The Effect of UV-Irradiation on the Hexachlorobenzene-induced Porphyria in an Experimental Animal Model Hexachlorobenzene is able to induce in humans and different animal species a hepatic porphyria, which is comparable to porphyria cutanea tarda. We studied whether hexachlorobenzene feeding could induce this porphyria also in hairless rats (Rowett-Nude), as these are suitable for photobiologic studies. After development of hepatic porphyria the rats were irradiated either with UV-A or UV-B light. Biochemical effects on the porphyrin metabolism and clinical as well as histologic skin changes were examined. In the liver of the porphyric rats, UV-A and UV-B irradiation induced increased aminolevulinic acid synthase activity accompanied with augmentation of hepatic porphyrin content. UV-A or UV-B irradiation also lead to increased total porphyrin contents in the skin of porphyric compared to non-irradiated animals. Clinically and histologically, however, only UV-A irradiation induced lesions, which were comparable to skin manifestations of porphyria cutanea tarda.

Animals↗

[Do not forget: lead poisoning].

3 patients with lead intoxication are demonstrated. The analysis of porphyrins and porphyrin precursors led to the tentative diagnosis which was confirmed by the increased lead levels. The increased urinary excretion of porphyrins and of delta-aminolevulinic acid, the decreased activity of uroporphyrinogen synthase and the increased protoporphyrin content (absorption maximum 595 nm) in the red cells suggested the diagnosis before the lead values of the patients were known. Clinical signs of the patients included gastrointestinal and neurological symptoms. It is assumed that the lead intoxication in these patients was caused by occupational exposition.

Adult↗

[Allergic contact dermatitis caused by Asteraceae. Identification of an 8,9-epoxythymol-diester as the contact allergen of Arnica sachalinensis].

A pharmacist developed an allergic contact dermatitis while working with Arnica sachalinensis. Patch testing with sesquiterpene lactones was negative. The contact allergen of A. sachalinensis was identified as 10-acetoxy-8,9-epoxy-thymol-isobutyrate. This compound, or structurally related 8,9-epoxythymol-diesters, are often found in Asteraceae. In addition to sesquiterpene lactones, epoxythymol-diesters should be considered as contact allergens. Cross reactions between the two groups of compounds are not expected and could not be demonstrated in our patients.

Adult↗

Gradient centrifugation analysis of steroid-hormone binding in human malignant melanoma.

Vertical tube rotor sucrose gradient centrifugation in the presence of the protease inhibitor sodium molybdate provide to give a deeper insight into the steroid hormone receptor status of human malignant melanoma (MM) as did previous studies using dextran-coated charcoal procedure only. As compared to endocrine dependent breast cancer, the oestrogen binding capacity of MM is low. Although in some biopsies fairly high concentrations of progestin binders were detected, the sedimentation properties were not receptor typic. Androgen binding was found to be negligible. In contrast, the presence of glucocorticoid receptors is a common feature of human MM. A human MM cell line was demonstrated to contain glucocorticoid receptors only.

Adult↗