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Biomedical subjects

I Kikuchi

Publications and source records attributed to I Kikuchi.

At least 19 recordsLinked to original sources

BCC-associated amyloidosis with a peculiar pattern of deposition.

An 83-year-old Japanese woman with lepromatous leprosy had been treated in a leprosarium. More than 10 years ago, she developed a dome-like brown tumor on the dorsum of the nose which showed the histology of basal cell carcinoma. The lesion was a well bordered, rounded tumor with tumor cell nests on its periphery extending toward the center as cords of tumor nest and with amyloid depositions between the cords. The tumor nests and amyloid lessened toward the center of the tumor, being replaced by collagen fibers. Amyloidosis showing this peculiar pattern of transition was reported with a review of published cases.

Aged

Squamous-cell carcinoma arising in solar keratosis of a leprosy patient: relationship with positive skin smear examination.

A 70-year-old Japanese female with lepromatous leprosy developed squamous-cell carcinoma within a long standing area showing solar keratoses on her head. Alopecia had been present at least for the preceding 34 years on her forehead and frontal area of the head, and solar keratoses had existed at least for the previous 9 years. Skin smear examination for acid-fast bacilli which had continually been negative for the previous 2 years (during which 8 examinations were done) became positive. We assume that there may be some relationship between the onset of the tumor and the positive skin smear examination, although the exact connection remains unclarified.

Aged

Congenital onychodysplasia of the index fingers: a case involving the thumbnails.

A case of congenital onychodysplasia of the index fingers involving the nails of both thumbs is presented, and the characteristics and various hypotheses of this most distal congenital reduction malformation are reviewed. The presence of such an atypical case as this may suggest that there is heterogeneity of this congenital condition, in agreement with Ohtsu's classification of this condition into (1) common type, (2) secondary type, and (3) hereditary type. An abnormal hand grip hypothesis, proposed by the author, could not be substantiated by Ohtsu, although it cannot be denied, for instance, based on the presence of a sucking blister. Three groups of congenital distal malformations can be differentiated by the pattern of involved fingers.

Adult

The human class I alcohol dehydrogenase gene cluster: three genes are tandemly organized in an 80-kb-long segment of the genome.

The class I alcohol dehydrogenases (ADH; EC 1.1.1.1) play a key role in hepatic alcohol catabolism. Three human class I ADH genes, ADH1, ADH2, and ADH3, which encode the alpha, beta, and gamma subunits respectively, have been isolated and mapped on chromosome 4q21-q23. Genomic cloning using a cosmid vector allowed us to obtain an 88-kb-long genomic segment, which was found to include an entire 80 kb of the class I ADH gene cluster. All three genes lie in the same transcriptional orientation and the order of genes is 5'-ADH3-ADH2-ADH1-3'. It may be of some significance that the order of transcriptional activation in the hepatic development, alpha----beta----gamma, is opposite to the order of gene arrangement. Several members of the AluI family and the KpnI (L1) family of interspersed repetitive sequences were mapped in this region. The divergence of insertional sites suggested that gene multiplication of the class I ADH genes had taken place in the earlier stages of human (or primate) evolution.

Alcohol Dehydrogenase

Congenital fascial dystrophy: stiff skin syndrome--a human counterpart of the tight-skin mouse.

Four patients are described with stony-hard induration of the skin and deeper tissues, most pronounced on the buttocks, thighs, and legs, and with limitation of joint mobility and contractures of the lower limbs. Two patients were siblings and one was the product of a consanguineous marriage. The disorder appears to be genetically determined, but the mode of inheritance has not been established. The disease was noticed in the patients' early infancy and was not progressive. Except for functional impairment of the lungs caused by an underdeveloped thorax that resulted from pressure of the thickened thoracic fascia, there was no involvement of the viscera or muscles and no immunologic abnormalities. The most important finding was markedly thickened fascia. This hereditary connective tissue disorder has all the characteristics of the tight-skin mouse.

Adult

HLA-linked immune suppression in humans.

There is no doubt that HLA-DR molecules are acting as the products of HLA-linked immune response genes (Ir-genes), because (i) HLA-DR molecules are the restriction elements in the interaction between CD4+ helper T cells and antigen-presenting cells (APC) to respond to many antigens such as streptococcal cell wall antigen (SCW) (Nishimura & Sasazuki, 1983; Sone et al., 1985; Hizayama et al., 1986), schistosomal antigen (Sj) (Hirayama et al., 1987), Mycobacterium leprae antigen (ML) (Kikuchi et al., 1986) and so on; and (ii) anti-HLA-DR monoclonal antibodies completely abolish the immune response to those antigens (Nishimura & Sasazuki, 1983; Sone et al., 1985). However, genetic analysis of the immune response to those antigens in families or populations revealed that responsiveness is recessive and non-responsiveness to those antigens is a dominant genetic trait that is tightly linked to HLA (Sasazuki et al., 1980a, 1983; Watanabe et al., 1988). This is completely opposite to the situation under the Ir-gene control where responsiveness is dominant and non-responsiveness is recessive. In this paper, we report evidence of how we came across the concept of HLA-linked immune suppression genes (Is-genes) besides Ir-genes, and show evidence for the epistatic interaction between HLA-DR and DQ to determine the immune response to several antigens in humans.

Genes, MHC Class II

Smoke inhalation injury from newer synthetic building materials--a patient who survived 205 days.

A 25-year-old factory worker sustained inhalation injury and 2 per cent deep burns while fighting a fire in his factory (LSI factory) which was made of new synthetic building materials. He became unconscious and inhaled noxious substances from the smoke. Although he survived the acute stages following injury, his pulmonary function subsequently deteriorated with a damaged trachea and bronchi, repeated attacks of pneumonia, atelectasis, pneumothorax and lung fibrosis, leading to death with hypercapnia (PaCO2 more than 100 mmHg) after 205 days in hospital. Autopsy revealed scarring contraction and dilatation of the trachea and bronchi, acquired bronchiectasis, lobular pneumonia, bleeding, hyperaemia and oedema of the lungs. Systemic administration of corticosteroids was only transiently beneficial in reducing the increased airway resistance.

Adult

Stiff skin syndrome.

Stiff skin syndrome was first documented by Esterly and McKusick. We saw a patient with this disease, as well as another who had a similar condition but a somewhat different histology. These two patients shared heritable stiff skin, restricted joint mobility, and absent mucopolysacchariduria. In stiff skin syndrome, which may have two variants, skin biopsy demonstrates large cells stained metachromatically by toluidine blue. These cells are shaped like dermal melanocytes or rounded cells and their presence may be directly related to the deposition of mucopolysaccharide in the dermis. In some cases they may invade the deeper tissues. Two cases of stiff skin syndrome that were in the Japanese literature are also discussed.

Adolescent

Incontinentia pigmenti achromians as part of a neurocutaneous syndrome: a case report.

A case of incontinentia pigmenti achromians associated with mental retardation, epilepsy, short stature and ocular anomalies was reported. A study of this case together with a review of the 38 cases in the literature revealed that this entity has been associated with central nervous system involvements at a high frequency. It is necessary, therefore, to consider incontinentia pigmenti achromians as a neurocutaneous syndrome from the viewpoint of pediatric neurology.

Child