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Biomedical subjects

W Küster

Publications and source records attributed to W Küster.

At least 19 recordsLinked to original sources
PubMed1992

Indication for the identity of palmoplantar keratoderma type Unna-Thost with type Vörner. Thost's family revisited 110 years later.

Palmoplantar keratoderma (PPK) type Unna-Thost is known to be the most common form of a hereditary disorder of keratinization of palms and soles. The disease is clinically identical with PPK type Vörner which is histologically characterized by epidermolytic hyperkeratosis. By reinvestigation of the family originally seen by Thost in 1880, the features of epidermolytic hyperkeratosis were found histologically confirming the diagnosis PPK of Vörner. This proves the identity of PPK type Thost with PPK of Vörner. Because of the histological variability of epidermolytic hyperkeratosis, detailed light and electron microscopic studies are necessary in cases of diffuse types of PPK.

Adult↗
PubMed1992

[Schöpf syndrome. Clinical, genetic and lipid biochemical studies].

Schöpf syndrome is an unusual genodermatosis categorized within the heterogeneous group of ectodermal dysplasias. Since the first description of this syndrome in 1971, ten further cases have been published. The diagnostic features include eyelid cysts, hypotrichosis, hypodontia, nail dystrophy, and keratosis of palms and soles. When the signs and symptoms noted in all published cases are taken together, considerable clinical variability in the combinations and in the age of onset is observed. The frequent occurrence of benign and malignant tumours of the palms and soles deserves particular attention. Schöpf syndrome is probably not as rare as is commonly believed. A woman with typical clinical features of this syndrome is presented. Schöpf syndrome is assumed to be passed on as an autosomal recessive trait; however the present and two further case reports are not compatible with this mode of inheritance, because family members in several generations are affected. Schöpf syndrome is probably a heterogeneous disorder. Lipid biochemical investigations of stratum corneum by high-performance thin-layer chromatography (HPTLC) show a decrease in the ceramide fraction and an increase in free fatty acids as a percentage of total lipids. The pathophysiological significance of these findings needs further investigation.

Adult↗
PubMed1991

Cytogenetic effects during extracorporeal photopheresis treatment of two patients with cutaneous T-cell lymphoma.

The effect of extracorporeal photopheresis (EP) on various cytogenetic parameters has been investigated. During EP the photoactivatable agent 8-methoxypsoralen (8-MOP) was administered orally. After 2 h a leukocyte-enriched blood fraction was collected by haemocentrifugation, irradiated with UVA extracorporeally, and reinfused to the patient. Two patients suffering from cutaneous T-cell lymphoma showed a marked clinical improvement in response to therapy. In order to investigate the cytogenetic effects and mutagenic risk of EP, the mitotic index (MI), the type and number of chromosomal aberrations and the rate of sister chromatid exchanges (SCE) were studied. Following EP treatment the patients' lymphocytes were cultured and stimulated with phytohaemagglutinin (PHA) for 48 or 72 h. The cultured lymphocytes showed a decreased MI after 48 h as an indicator of cytotoxic effects, but not after 72 h. In lymphocyte cultures not stimulated with PHA, the MI was decreased even after 72 h. The number of chromosomal aberrations and SCE were increased upon treatment, but only transiently, returning to basal levels between consecutive treatments. Our data provides no evidence for increased mutagenic risk as a consequence of effective EP treatment.

Administration, Oral↗
PubMed1991

[Nuclear medicine kidney diagnosis in progressive systemic scleroderma].

Kidney involvement is one of the most frequent causes of death in progressive systemic scleroderma (PSS). It is therefore important to detect potential impairment of renal function in PSS very early. In 76 patients referred for nuclear medicine diagnostic procedures, 42 pathologic results were found with 131I-hippurate clearance, while only 14 abnormal results were detected by static 99mTc-DMSA scans. Hippurate clearance is thus a sensitive method of functional renal evaluation in PSS.

Adolescent↗
PubMed1991

[Acne inversa. Pathogenesis and genetics].

Acne inversa is a chronic inflammatory disorder of sebaceous follicles and terminal hair follicles and is one type of acne diseases. The pathogenesis of acne inversa is identical with that of the other types: Hyperkeratosis of the follicular infundibulum leads to a comedo. Bacterial infections result in a rupture of the follicular canal followed by a granulomatous inflammatory reaction with abscesses, panniculitis and draining sinuses. Acne inversa has so far only occasionally been observed in two or more members of the same family. The familial presentations of acne inversa published in the literature and two observations of familial occurrence among the authors' own patients reveal an autosomal dominant inheritance with high penetrance.

Acne Vulgaris↗
PubMed1991

[Acne inversa. Diagnosis and therapy].

Acne inversa (synonyms: acne triad, acne tetrad or hidradenitis suppurativa, and others) is an inflammatory disease that develops mostly in the axillae, under the breasts and in the anogenital region. The new term, acne inversa, encompasses what was previously called the occlusion triad or tetrad. The disease is relatively common, but the diagnosis is frequently missed. Many different modalities of treatment are recommended in the literature. Radical surgical intervention should be performed at the earliest recognized stage. In the light of experience with our own patients, we propose surgical intervention with wide excision and subsequent healing by secondary intent.

Acne Vulgaris↗
PubMed1990

A family study of atopic dermatitis. Clinical and genetic characteristics of 188 patients and 2,151 family members.

The history of 188 caucasian patients with atopic dermatitis (AD) and of 2,151 family members has been analyzed. Of the AD patients 48% suffered from respiratory atopy (36% rhinitis, 28% asthma, and 15% both). AD showed by far the earliest onset of all atopic diseases: 50% of our patients had skin lesions before the age of 2 years and 60% before the age of 5 years. In contrast, symptoms of allergic asthma developed in 40% of AD patients before the age of 5 years in comparison with only 25% who had allergic rhinitis. AD affects females more frequently than males (male to female ratio 1:1.5), regardless of whether additional respiratory atopies are present or not. In contrast, respiratory atopies develop more frequently in males than in females (male to female ratio 1.5:1). Mothers with respiratory atopy more often have atopic children (26%) than do fathers with respiratory atopy (13%). Finally, risk figures for genetic counselling are given. In short, the general risk of developing AD (3%) and atopy (7%) increases by a factor of two with each first-degree family member already suffering from atopy.

Adolescent↗
PubMed1990

[Pregnancy in Crohn disease and ulcerative colitis. Maternal and fetal risks].

Familial occurrence in chronic inflammatory bowel disease is well established. Recurrence risks for first degree relatives range from 1 to 5%. A pregnancy is usually not negatively influenced by the disease. The risks for miscarriages are slightly increased, if conception takes place during periods of inflammatory activity. In the case of first manifestation during pregnancy, however, mother and child are exposed to increased risks. The course of the disease is usually not negatively influenced by pregnancy. The rate of relapse is similar to that of comparable samples without pregnancy. In women with an active disease, the prognosis is considerably poorer. The risk to children as a result of drug therapy can be regarded as low. Family planning, pregnancy and birth entail an increased psychological demand, which, in turn, necessitates specific therapy in some cases.

Colitis, Ulcerative↗
PubMed1990

[Skin changes in alimentary zinc deficiency caused by artificial infant food].

We report on 2 non-related newborn children who developed erythematosquamous skin eruptions on both their faces and the extensor sides of their fingers at the 4th and 10th week after birth, resp. Both children had been fed upon hypoallergenic formulas since birth. Serum investigations revealed decreased zinc levels: 0.4 and 0.17 mg/l, resp. (normal values: 0.59-0.96 mg/l). Following zinc substitution with zinc sulphate 10 mg daily for 3 days, the serum zinc levels of both children had increased to normal values and remained stationary during continued treatment with 3 mg zinc daily. The skin eruptions disappeared a few days after the start of treatment.

Acrodermatitis↗
PubMed1989

The genetics of Crohn disease: complex segregation analysis of a family study with 265 patients with Crohn disease and 5,387 relatives.

We have analysed the pedigrees of 265 probands with Crohn disease, collected from a specialty clinic at the University of Düsseldorf. Complex segregation analysis suggests the presence of a recessive gene with incomplete penetrance for susceptibility to the disease with no residual causes of family resemblance. However, a proportion of the isolated cases are probably due to phenocopies, this proportion being greatest among cases with an advanced age of onset.

Chromosome Mapping↗
PubMed1989

Autosomal dominant lamellar ichthyosis exhibits an abnormal scale lipid pattern.

Autosomal dominant lamellar ichthyosis (ADLI) is a recently recognized genetic skin disorder. Clinically and histologically, it cannot be distinguished with certainty from the more frequent autosomal recessive lamellar ichthyosis (ARLI), which in itself may still be heterogeneous. By ultrastructural examination of ADLI a prominent transforming zone between the stratum granulosum and stratum corneum and lipid inclusions in the stratum corneum have been observed. Using sequential high-performance thin-layer chromatography, we studied the plantar scale lipid pattern of two patients, mother and daughter, affected with ADLI. We found a distinctive alteration in the relative composition of the scale lipid pattern characterized by excessive amounts of free fatty acids, triglycerides, elevated n-alkanes, reduced free sterols and decreased total ceramides. This scale lipid profile clearly differs from that of the erythrodermic and non-erythematous variants of ARLI and confirms that this disorder is a distinct entity of the heterogeneous group of lamellar ichthyoses.

Alkanes↗
PubMed1989

[Nuclear medicine diagnosis of heart involvement in progressive systemic scleroderma].

The frequency and extent of heart involvement in progressive systemic scleroderma (PSS) can be underestimated when current clinical diagnostic methods are used. We therefore studied 53 PSS patients with planar (201Tl) chloride myocardial scintigraphy, and 44 PSS patients with radionuclide ventriculography using red blood cells labeled in vitro with 99mTc. Comparison of maximal exercise and resting myocardial scans demonstrated pathologic findings in 18 of 38 patients. In 10% of the patients abnormal ejection fraction values (less than 50%) and Fourier analysis were found on radionuclide ventriculography. Myocardial scintigraphy in conjunction with other diagnostic modalities thus provides useful information for the evaluation of heart involvement in PSS.

Cardiac Output↗