[A case of familial occurrence of chronic hypoplastic neutropenia and aplastic anemia].
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Biomedical subjects
Publications and source records attributed to M Iida.
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Streptozotocin-diabetic rats were maintained on a 72% fructose diet for 4 weeks and some were treated with an aldose reductase inhibitor (either alrestatin: 0.9 g . kg-1 . day-1 or ONO-2235: 50 mg . kg-1 . day-1). Fructose feeding significantly influenced the development of impaired motor nerve conduction velocity in the diabetic rats and this effect was positively correlated with sorbitol accumulation in the sciatic nerve of diabetic rats maintained on a fructose-rich diet. Treatment with ONO-2235, a new aldose reductase inhibitor, prevented both slowing of motor nerve conduction velocity and elevation of nerve sorbitol concentration. On the other hand, erythrocyte sorbitol levels were significantly correlated to those of the sciatic nerve (r = 0.86, p less than 0.001) and the retina (r = 0.91, p less than 0.001) in these animals. Thus, our findings suggest that increased polyol pathway activity may be related to the pathogenesis of diabetic neuropathy and erythrocyte sorbitol concentrations may prove a useful indicator for the presence of diabetic complications.
We treated two patients with familial polyposis coli (FPC) who were found to have multiple adenomas in the ileostomy stoma, five and seven years after colectomy, respectively. These adenomas were dense and 3 to 10 mm in diameter. The adjacent ileal mucosa showed a colonic metaplasia. Ileal adenomas detected using intraoperative intestinal endoscopy at the time of colectomy were less than 2 mm in diameter and sparse in density. These findings suggest that ileal adenomas occurring after colectomy for FPC tend to increase in size and number and to resemble colonic adenomas.
In order to study the natural history of fundic gland polyposis, 23 patients with familial adenomatosis coli/Gardner's syndrome were examined over a follow-up period ranging from 17 mo to 13 yr (average 6 yr). Examinations included gastric radiography and endoscopy with biopsy. Fundic gland polyps were found in 10 individuals. The size and number of polyps varied considerably. During the follow-up period, there was an increase in number or size of polyps, or both, in 5 patients (aged 8-27 yr), a decrease or disappearance in 2 patients (aged 36 and 41 yr), an initial decrease or disappearance followed by a late-occurring increase in 2 patients (aged 28 and 35 yr), In addition, malignant or adenomatous changes of fundic gland polyps were not observed in any patient. Therefore, fundic gland polyposis in patients with familial adenomatosis coli/Gardner's syndrome may appear as early as 8 yr of age. In some patients there is a gradual increase in number and size of polyps, whereas in others, polyp proliferation ceases and polyps may even decrease in number and size. Our findings indicate that the fundic gland polyposis does not require prophylactic surgery and that careful periodic follow-up should suffice.
Gastroduodenoscopy was performed in 25 patients with various disorders, such as liver cirrhosis, nephrotic syndrome, and ulcerative colitis, to assess the effects of corticosteroids on the stomach and duodenum. The main criterion for entry into the trial was the absence of open ulcer, healed ulcer, erosion, or bleeding from the stomach or duodenum on pretreatment endoscopy performed within 48 hours before administration of corticosteroids. Endoscopy repeated at 2 to 4 weeks disclosed gastroduodenal lesions in 11 cases (44%)and no lesion in 14 cases (56%). The gastroduodenal lesions observed in 11 cases are as follows: one gastric ulcer (4.0%), six gastric erosions (24.0%), two gastroduodenal erosions (8.0%), and two duodenal erosions (8.0%). A lack of correlation between the patients' subjective complaints and endoscopic findings indicates the unreliability of patients' complaints and the importance of endoscopy in assessing gastroduodenal lesions. There were no differences in the total and average daily doses of corticosteroid between a group with gastric and/ or duodenal lesions and a group without such lesions. Corticosteroids may produce gastroduodenal lesions, regardless of the dose.
The characteristic findings of barium enema and colonofiberscopic studies and their changes over time were investigated in 10 patients with penicillin-related non-pseudomembranous colitis. Radiographic examination within 6 days of onset revealed abnormal findings such as narrowing of the lumen, loss of haustral markings, thumb-printing, transverse ridging, and saw-toothed irregularities. These findings tended to be more pronounced on the right than on the left side of the colon. Endoscopy revealed lesions characterized by mucosal reddening, edema, and hemorrhage, without definite ulceration or erosion. In 5 of the 10 patients, non-continuous distribution of the lesions was noted. In 4 patients, the rectum was unaffected. Reversion to normal occurred within an average of 15.7 days following onset of the symptoms. Thus, for an accurate diagnosis of this disease, barium enema and/or total colonoscopy should be performed within 6 days of onset.
We report the clinical and radiologic features of 12 patients with acute intestinal anisakiasis. Diagnosis of anisakiasis was made immunologically by positive antibody to Anisakis larvae and a recent history of raw fish intake. Severe abdominal pain was a major symptom in these patients-half experienced fluid levels indicating ileus on plain x-ray films of the abdomen. Radiologic findings included irregular thickening of the jejunum, ileum, or colon, with mucosal edema and luminal narrowing with dilatation of the proximal intestine. In two cases a thread-like filling defect suggesting a worm was visualized on the x-ray film. These findings were interpreted as anisakiasis. Although ileus developed in some patients, all were treated and cured completely without surgery.
The three-dimensional and surface structures of the simple conical papillae of the rat tongue have been demonstrated with scanning electron microscopy. The papillary projection was organized into the anterior, posterior and central core cell populations, whereas the basal region of the papilla which consisted of circularly arranged cells showed no differentiation into three autonomic cell populations. It is considered that the anterior and posterior cell populations around the central core tend to be mutually attached at the bilateral sides, and that the posterior and core cell contacts are rather close than the anterior one. The anterior papillary cells showed relatively smooth surface with little micropits and without microridges. The reticular microridges on the basal cell surface of the posterior papillary cells appear to later develop the micropits and linear microridges on the tip cell surface. These suggest that the anterior cell surface is more highly keratinized than the posterior one. The microridges or micropits on the outer cell surface and the microprojections on the inner cell surface organizing filiform papilla are considered to be the structures for the purpose of cell adhesion.
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A 49-year-old woman with Gardner's syndrome, who underwent total proctocolectomy in 1982, was found to have a cancer of the duodenal bulb. Subsequently, resection of the stomach and duodenal bulb was performed in 1983. The surgical specimen showed an ulcerating tumor in the duodenal bulb which was a moderately differentiated adenocarcinoma histologically. Multiple adenomas were present in the gastric antrum and the duodenum. Duodenal cancer so far reported has been mostly confined to the periampullary region, and cancer of the duodenal bulb associated with familial polyposis coli has not been reported.