ABSTRACT
BACKGROUND: Darier disease is an uncommon genodermatosis characterized by the symmetrical eruption of keratotic reddish-brown papules occurring in the seborrheic areas of the body. A unilateral, or localized, variant has been identified. We report 4 new cases of localized Darier disease and review the English-language literature. The implications of these cases on future genetic studies are also discussed. OBSERVATIONS: Localized Darier disease occurred with equal frequency in males and females. The average age at onset was 27 years. The most frequent site of involvement was the trunk (40% [16/40]). This condition was aggravated by sunlight, heat, or sweating in 42% (19/40) of reported cases, and 38% (15/40) of the patients responded to treatment with topical tretinoin. CONCLUSIONS: Many of the clinical features of localized Darier disease suggest that it is a genetic mosaic of generalized Darier disease. Further studies of localized Darier disease may therefore prove to be instrumental in the search for the Darier disease gene.
Subject(s)
Darier Disease/genetics , Abdomen , Adult , Axilla , Biopsy , Chromosomes, Human, Pair 12 , Darier Disease/pathology , Female , Humans , Leg , Male , Skin/pathology , ThoraxABSTRACT
There have been reports of an association between Darier's disease, an autosomal dominant genodermatosis, and psychiatric illness. Recently the gene causing Darier's disease has been mapped to an area on 12q, between D12S58 and D12S84. The findings of linkage analysis of 4 markers in the Darier's disease region on 12q in five families segregating schizophrenia are presented. They fail to support close linkage between schizophrenia and the Darier's disease region on 12q.
Subject(s)
Chromosomes, Human, Pair 12 , Darier Disease/genetics , Schizophrenia/genetics , Adult , Chromosome Mapping , Genetic Linkage , Genetic Markers , Humans , Lod Score , Models, Genetic , Nuclear Family , Psychotic Disorders/geneticsABSTRACT
A cDNA clone for the beta-chain of human alcohol dehydrogenase (ADH) was used to isolate several cross-hybridizing clones from a mouse liver cDNA library. Clones pADHm9 and a portion of pADHm12 were sequenced. pADHm9 coded for a sequence of 151 C-terminal amino acids and some untranslated sequences from the 3' end of its corresponding mRNA. This clone was identified as an Adh-1 cDNA clone. Consistent with the known expression of Adh-1, this gene was expressed constitutively in liver, whereas the Adh-3 gene product was found only in stomach, lung and reproductive tissues. Furthermore, the translated region of the cDNA shared 91% amino acid sequence homology with rat liver ADH. [32P]pADHm9 was used as a hybridization probe to study the mechanism of androgen induction of kidney ADH activity. Induction of A/J female mice by androgen resulted in a dramatic increase in the steady-state level of Adh-1 mRNA content which correlated with the level of enzyme induction. The size of the mRNA obtained from control or induced kidney and liver tissues was indistinguishable by Northern analysis. [32P]pADHm9 was also used to probe restriction fragments of genomic DNA obtained from several inbred mouse strains. The hybridization patterns, considered with the genetic evidence, suggested that pADHm9 recognized sequences which may be present as only a single copy in the genome. No restriction fragment length polymorphisms were observed among the several inbred mouse strains examined.
Subject(s)
Alcohol Oxidoreductases/genetics , Cloning, Molecular , DNA/analysis , Genes/drug effects , Kidney/enzymology , Testosterone/pharmacology , Alcohol Dehydrogenase , Alcohol Oxidoreductases/biosynthesis , Amino Acid Sequence , Animals , Base Sequence , DNA Restriction Enzymes , Enzyme Induction , Female , Humans , Kidney/drug effects , Kinetics , Liver/enzymology , Mice , Mice, Inbred A , Nucleic Acid Hybridization , Protein Biosynthesis , Sequence Homology, Nucleic AcidABSTRACT
To serve as the basis of cost comparison, USDA "moderate-cost" and "thrifty" menus for one week were modified to meet guidelines for a cholesterol-lowering diet. Prices of individual items on the menus at both cost levels were obtained from two supermarkets and averaged. Items on the moderate-cost, cholesterol-lowering diet were $1.37 less than the USDA menus. At the low-cost level, the modified menus cost 22 cents more. In neither case was the difference significant by the t-test.
Subject(s)
Cholesterol, Dietary , Heart Diseases/prevention & control , Hypercholesterolemia/prevention & control , Adult , Child , Costs and Cost Analysis , Humans , Male , Menu Planning , Middle Aged , Socioeconomic FactorsABSTRACT
A twelve-month pilot project was conducted to test public reaction to a special restaurant menu identifying food choices low in cholesterol and saturated fat. The Houston steak house in which the "Help Your Heart" menu was tested prepared foods according to guidelines from dietitians of the Diet Modification Clinic, Baylor College of Medicine. Two newspaper articles, a brief magazine feature, and regularly scheduled radio advertising spots publicized the special menu. At the end of each month of the test period, the number of orders from the special menu was tallied for fourteen randomly selected days. Monthly sales from the special menu ranged from 2.5 to 5.1 per cent, with an overall average of 3.4 per cent. There was no statistically significant change from the mean of the percentage of total sales from the special menu over the months. Despite the relatively low percentage of total sales, restaurant executives were pleased with the results of the study and planned to offer the low-cholesterol menu indefinitely, eventually in an expanded form.
