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Biomedical subjects

I Hashimoto

Publications and source records attributed to I Hashimoto.

At least 361 records · Page 20Linked to original sources
PubMed1977

Familial intracranial aneurysms and cerebral vascular anomalies.

The author reports a family in which four members had intracranial aneurysms and one additional member was suspect. One member had multiple aneurysms that were successfully treated surgically. Elective angiography on five asymptomatic members of the family disclosed asymptomatic aneurysms in two. In addition, cerebrovascular anomalies were found in many of the family members. The parents of the family were consanguineous. High incidence of these associated anomalies and consanguinity in the parents tend to suggest the hereditary basis of the disease. Banding analysis of chromosomes in three siblings with aneurysms and three siblings without aneurysms was carried out. Elective investigation of the asymptomatic members should be considered where there are already two or more affected in a family. The indications for surgical prophylaxis on asymptomatic aneurysms in other members of the family are discussed.

Adult↗
PubMed1977

The true sagittal diameter of the cervical spinal canal and its diagnostic significance in cercical myelopathy.

A method is presented for measuring the true sagittal diameter of the cervical spinal canal on routine lateral radiograms by the use of a midline perforated ruler. Sagittal measurements were made in 92 normal adults to establish a range of normal values. The lower and upper limits were 10 mm and 17 mm, respectively, at the levels of C-3 to C-7. Comparison of the results with the anatomical measurements of dried specimens showed good agreement. Four cases of cord compression were presented and the diagnostic significance of the method in cervical myelopathy was discussed.

Adult↗
PubMed1976

Ultrastructural studies in epidermolysis bullosa hereditaria. IV. Recessive dystrophic types with junctional blistering. (Infantile or Herlitz-Pearson type and adult type).

Recessive dystrophic epidermolysis bullosa with junctional blisters includes both the classical epidermolysis bullosa hereditaria letalis Herlitz (Herlitz-Pearson type) and a recently separated more benign adult type. Ultrastructural examination was performed of 13 skin specimens from 3 cases of the Herlitz-Pearson-type and one case of the adult type. Principal ultrastructural changes in involved, intact and experimentally frictioned skin regions, common to all patients, are as follows: In nonseparated areas hypoplasia of the hemi-desmosomes and mild decrease of the tonofibrils are found. Hypoplasia of hemi-desmosomes consists of a marked rudimentary structure of the sub-basal dense plaque and the attachment plaque. Focal widening of the lamina lucida suggesting early blistering occurs exclusively in the areas devoid of hemidesmosomes. In separated areas cleavage always occurs in the plane of lamina lucida, viz. the mode of blistering is junctional. Fragments of basal cells are often encountered still remaining attached to the blister floor ("epidermolytic torn-off phenomenon"). These torn-off portions of basal cells are characterized by relatively rich distribution of hemidesmosomes. Basal cells forming the blister roof frequently show small gaps of basal plasma membrane and rarefaction of the basal part of the cytoplasm, which are thought to be secondary changes. Among the observed alterations, structural defects of hemidesmosomes are considered to play the most important role in the pathogenesis of junctional blisters.

Adult↗
PubMed1976

Ultrastructural studies in epidermolysis bullosa hereditaria. III. Recessive dystrophic types with dermolytic blistering (Hallopeau-Siemens types and inverse type).

Ultrastructural examination was performed in 42 biopsy specimens from 22 patients with the Hallopeau-Siemens types or with the inverse type of epidermolysis bullosa dystrophica recessiva. The patient group consists of 8 cases of the localized Hallopeua-Siemens type, 9 of the generalized Hallopeau-Siemens type and 5 of the inverse type. The origins of the biopsy specimens are involved, intact and experimentally frictioned skin from blister-predilected sites, as well as clinically normal skin from nonpredilection sites. It is confirmed that all the blisters initiate below the basal lamina. Anchoring fibrils are moderately to markedly decreased in most cases, while they are normal in 3 other cases. It is thought that secondary degradation of anchoring fibrils and/or collagen fibrils plays an important role in blistering mechanism in the Hallopeau-Siemens and inverse types of recessive dystrophic epidermolysis bullosa, whereas a primary aplasia of anchoring fibrils as causative defect has been out ruled.

Collagen↗
PubMed1976

Ultrastructural studies in epidermolysis bullosa hereditaria. II. Dominant dystrophic type of Cockayne and Touraine.

Ultrastructural examination was performed in 9 biopsy specimens from 4 patients with the Cockayne-Touraine type of epidermolysis bullosa dystrophica dominans. The specimens were taken from: 1. clinically normal skin from the blister-nonpredilected sites (trunk) as well as 2. atrophic, 3. intact, and 4. experimentally frictioned skin regions from the blister-predilected sites (extremities). In the frictioned skin a dermolytic blister formations was observed. Development of anchoring fibrils showed a marked regional difference, the counts of fibrils being significantly lower (40%) in the predilection sites than in the nonpredilection sites. In addition the anchoring fibrils showed a variable degree of abnormal structure. The low frequency of often abnormally structured anchoring fibrils in the blister-preferred sites provides a good explanation for the clinical features. More studies are needed to see if regional differences in fibril frequency is a feature also of normal skin, in which case the dominant epidermolysis gene may represent a mutated structural anchoring fibril gene.

Connective Tissue↗
PubMed1976

Epidermolysis bullosa dystrophica dominans (Pasini)-a primary structural defect of the anchoring fibrils.

In epidermolysis bullosa dystrophica dominans Pasini a structural defect of the anchoring fibrils, a structural protein of the epidermo-dermal junction, has been demonstrated by electron microscopy to be constantly present not only in involved skin but also in intact skin of nonpredilection sites of blister formation. These findings render support to the concept that in dominant disorders an abnormality in a non-enzymic structural protein is more likely than in an enzymic protein.

Collagen↗
PubMed1976

Epidermolysis bullosa hereditaria with junctional blistering in an adult.

A 38-year-old patient with epidermolysis bullosa is described, in whom junctional blister formation is revealed by electron microscopy. Clinical and ultrastructural differences from the recessive dystrophic type (Hallopeau-Siemens) and from the lethal type (Herlitz) of epidermolysis bullosa are discussed in detail.

Adult↗
PubMed1976

[Epidermolysis bullosa dystrophica inversa: report on 2 sisters].

Two sister cases of epidermolysis bullosa dystrophica inversa are reported. This type, first described by Gedde-Dahl (1971), is characterized by the inverse site of skin involvement, the intermittent course, frequent traumatic corneal erosions, retarded development of skin atrophy and absence of milia formation. Our present cases had all these characteristics except the corneal involvement. The pedigree of this family favors an autosomal recessive mode of inheritance; no consanguinity is demonstrable in the ancestors over four generations. Electron microscopic examination in one of the patients reveals the blistering beneath the basal lamina, which suggests that the pathogenesis may be similar to the Hallopeau-Siemens type rather than to the junctional type of epidermolysis bullosa.

Epidermolysis Bullosa↗