[Case of branchio-oto-renal syndrome].
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Biomedical subjects
Publications and source records attributed to Y Mae.
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Quantitative and qualitative evaluations of erythrocyte ferritin in 161 patients with RA and RAEB in MDS, AML, CML, PV, PA, HS, IDA, chronic liver disease and alcoholic liver disease were carried out. Mean erythrocyte ferritin levels of patients with RA, AML, PA, HS and alcoholic liver disease were increased compared with normal subjects. On isoelectric focusing analyses (IEF), erythrocyte ferritin in normal subjects were detected between pI 5.1 and 5.7. In the cases of RA, pI ranges of erythrocyte ferritin may be divided into three groups, acidic, neutral, basic shift on IEF respectively. In these groups, the more acidic the ferritin shift, the higher the proportion of morphological abnormalities of the erythroid precursors in the bone marrow was observed. In patients with AML (M2, M3, M4), little difference was found among these three subtypes, and all of the cases showed similar pattern with normal subjects on IEF. The ferritin from IDA showed low levels and slight basic shift compared with normal subjects on IEF, and these features were also found in patients with CML (chronic phase) and PV. After iron supplementation, marked increase of acidic ferritin was detected on IEF indicating an intermediate store for iron destined for haem synthesis. It was clear that the stainable iron in liver parenchymal cells were found at erythrocyte ferritin concentration 20 ag/cell or over in patients with chronic liver disease. Measurement of erythrocyte ferritin concentration is a helpful method for evaluating iron deposition in hepatocyte non-invasively. From these results it is considered that quantitative and qualitative analyses of erythrocyte ferritin are very useful for evaluating erythropoiesis as well as iron metabolism.
We measured red blood cell counts, hemoglobin levels, hematocrit values, serum iron levels, total iron binding capacity, transferrin saturation, and serum ferritin levels in 364 elderly subjects in the eighth decade, who consisted of 283 subjects (171 males, 112 females) living in their own homes and 81 subjects (28 males, 53 females) living in an old-age home. The mean values of RBC, Hb, Ht, SI, s-Ft in both male and female subjects living in their own homes were 469 X 10(4)/microliters vs 439 X 10(4)/microliters, 14.6 g/dl vs 13.3 g/dl 44.3% vs 40.6%, 95.6 micrograms/dl vs 84.8 micrograms/dl, 50.2 ng/ml vs 42.6 ng/ml, respectively. On the other hand, the mean values of RBC, Hb, Ht, SI, s-Ft in both male and female subjects living in an old-age home were 421 X 10(4)/microliters vs 390 X 10(4)/microliters, 13.8 g/dl vs 12.6 g/dl, 39.9% vs 36.7%, 74.2 micrograms/dl vs 58.6 micrograms/dl, 98.8 ng/ml vs 69.1 ng/ml, respectively. All erythrocyte parameters were significantly lower in females than in males in both groups, and significantly lower in subjects living in an old-age home than in subjects living in their own homes. The erythrocyte parameters of subjects living in their own homes showed little difference compared with those of normal young adults. These results suggest that a sedentary lifestyle in the elderly people contribute to the tendency to have anemia and this is considered to relate to age-associated pathology such as inflammation. As anemia in elderly people often follows a underlying illness, we must always give attention to the factors which cause anemia.(ABSTRACT TRUNCATED AT 250 WORDS)
The authors reported a case of Bietti's crystalline retinopathy in 1981. In the present paper they report three additional cases studying the stages of this disease and the clinical findings at each stage. Conclusions obtained are as follows: This disease is a type of total RPE-choriocapillaris dystrophy, the process of which starts at the posterior pole. This disease can be divided into three stages, based on the clinical findings: Stage 1: RPE atrophy with uniform fine white crystalline deposits is observed at the macular area. Stage 2: RPE atrophy extends beyond the posterior pole. Choriocapillaris atrophy in addition to the RPE atrophy appears markedly at the posterior pole. Crystalline deposits in the lesion vary in shape and size and have a tendency to conflue. The number of crystalline deposits is less in the advanced atrophic areas of the RPE-choriocapillaris complex. Stage 3: RPE-choriocapillaris complex atrophy is observed throughout the fundus. The total number of crystalline deposits decreases because the crystalline deposits that disappear number more than those that appear in Stage 3. Crystalline deposits which are observed at the corneal limbus are possibly prominent in the advanced stage of atrophy of the RPE-choriocapillaris complex.
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The development of nerve fiber bundle defects in monkey eyes by means of intraocular retinal coagulation, such as endodiathermy, intraocular cryotherapy or endophotocoagulation was confirmed by ophthalmoscopy. Two to 4 days after the application of intraocular retinal coagulation, the findings could be already observed. Endodiathermy especially could provide the typical ophthalmoscopic findings of nerve fiber bundle defects without any abnormal fluorescein angiographic findings, while endophotocoagulation and intraocular cryotherapy induced hyperfluorescence due to window defect at the coagulation site. Therefore, endodiathermy in monkey eyes can provide an excellent ophthalmoscopic model of the nerve fiber bundle defects. Histological findings 8 weeks after the coagulation showed thinning of the retinal nerve fiber layer and gliosis occurring in the retinal portion corresponding to the lesion.
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