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W Küster

Publications and source records attributed to W Küster.

At least 73 records · Page 4Linked to original sources
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↗
PubMed1988

[Basal cell nevus syndrome with squamous cell carcinoma of the larynx].

We report on a 54-year-old patient suffering from basal cell nevus syndrome. At the age of 51, squamous cell carcinoma of the larynx had been diagnosed. The frequent occurrence of various kinds of benign and malignant neoplasms in patients with basal cell nevus syndrome illustrates the close relationship between this syndrome and phacomatoses.

Basal Cell Nevus Syndrome↗
PubMed1988

Congenital scalp defects with distal limb anomalies (Adams-Oliver syndrome): report of ten cases and review of the literature.

We describe one family with 5 affected persons in 4 generations, another family with 2 affected brothers and 3 sporadic cases of the rare syndrome of congenital scalp defects with distal limb deficiency. The manifestations of this syndrome are highly variable. Review of the literature showed 11 families and 19 sporadic cases. In most families the disorder clearly follows an autosomal dominant pattern of inheritance, but in some families with reduced penetrance. Important differential diagnoses are the syndrome of scalp defect and postaxial polydactyly, the syndrome of scalp defect and split-hand defect, amniotic band sequence, and epidermolysis bullosa dystrophica type Bart.

Female↗
PubMed1988

Progressive erythrokeratodermia and cochlear hearing impairment. A case report and review of the literature.

A female child with congenital progressive erythrokeratodermia combined with sensory hearing loss observed through a period of 5 years is reported. She demonstrates symmetrical hyperkeratotic skin changes, verrucous plaques on her nose cheeks, ears, chin, knees, elbows, and heels. Electron microscopic studies of her skin did not reveal qualitative changes, her moderate to severe hearing impairment is of cochlear origin, moderately progressive, and particularly affecting the high frequencies. Up to now a vascularizing keratitis could not be detected. Her family history is not contributory. Twenty-eight similar cases from the literature, mainly reported as 'KID' syndrome, are reviewed. There are two familial instances. Autosomal dominant inheritance is assumed. We consider the acronymic designation 'KID' syndrome misleading, since the main features of the disorder are a progressive erythrokeratodermia, cochlear deafness, and non-obligatory vascularizing keratitis.

Child, Preschool↗
PubMed1988

Cleft lip and palate, lower lip pits, and limb deficiency defects.

Cleft lip or palate and lower lip pits are typical features of the autosomal dominantly inherited Van der Woude syndrome. Limb defects have not been reported in this syndrome so far. A girl with a unilateral complete cleft lip and palate, bilateral lower lip pits, and amniotic deformities of all four limbs is reported and the possibility of chance occurrence of cleft lip and palate, lower lip pits, and limb defects is discussed.

Abnormalities, Multiple↗
PubMed1988

[Triploidy in newborn infants].

Human triploidy, a common condition occurring in about 1 to 2% of all clinically recognizable pregnancies, is a rare finding in live-born children. Not more than 44 live-born triploid infants have been reported in the available literature. Triploid infants surviving for more than a few days have been suspected to be hidden mosaicisms and mostly have turned out to be diploid-triploid mosaics. We observed two live-born female infants with a complete triploidy and describe the typical clinical picture.

Abnormalities, Multiple↗
PubMed1988

[Clinical aspects and genetics of congenital skin defects].

Congenital skin defects are uncommon anomalies in neonates, confronting the clinician with diagnostic problems because of their etiologic heterogeneity. The classifications currently in use are didactically unsatisfactory. Therefore, we propose a new classification taking account of the localization of the congenital skin defect (head, trunk, extremities) and the criterion of isolated or associated occurrence. The groups clinically defined in this way, tables showing the pathognomonic symptoms and, in addition, a short review of all syndromes involving congenital skin defects should allow a rapid diagnosis. Clinical and genetic features of the isolated congenital skin defects are also reviewed.

Abnormalities, Multiple↗
PubMed1988

EEC syndrome sine sine? Report of a family with oligosymptomatic EEC syndrome.

We report a family with oligosymptomatic EEC syndrome. Whereas the mother had most symptoms of this syndrome, one son presented a minimal ectrodactyly and a highly arched palate and one daughter showed only a unilateral stiff thumb. The variability of this syndrome is discussed. The penetrance of this dominantly inherited disorder is judged to be reduced to about 78%.

Adult↗