Statistical study of aca-talasemia, a review of thirty-eight cases appearing in the literatures

1. With a view to grasp more simply and clearly the characteristics of this disease and in order to find a clue for prompt discovery of cases when encountered in future, the authors undertook a statistical study of the cases already reported by various authors. 2. The cases reported so far amount to 17 familial groups which consisted of 38 acatalasemic cases. These groups were distributed widely throughout Japan. The disease seemed to be prevalent in the rural communities where adherence to the custom of consanguineous marriage occurs. As yet, we have not heard of the occurrence of this disease in other countries. 3. The disease has equal distribution in both sexes. About one half of patients showed a peculiar oral gangrene (Takahara's disease). The great majority of these were noted in those less than 10 years of age. 4. The great majority of them were children whose parents were united in consanguineous marriage and have siblings with acatalasemia. 5. As for the treatment of oral lesions in this disease, extraction of tooth at the site of the lesions, removal of the diseased tissues en masse by resection, and penicillin treatment given concomitantly are effective. The course and the length of time required in healing of the wound due to the operation are about the same as in the case of normal persons. 6. The authors wish to call special attention to the phenomenon peculiar to the acatalasemic blood. The blood of acatalasemic individuals changes to brownish-black color in the absence of foaming or bubble formation upon the application of hydrogen peroxide to blood.</p

Bilateral Serous Retinal Detachments Associated with Accelerated Hypertensive Choroidopathy

Purpose. We report a case of hypertensive choroidopathy with bilateral serous retinal detachments. Patient. A 50-year-old man underwent bilateral serous retinal detachments. Retinal arteriolar narrowing, vascular tortuosity, and arteriovenous nicking were identified in both eyes. The blood pressure was 206/125 mmHg. The patient was diagnosed with bilateral hypertensive choroidopathy and treated with oral antihypertensive treatment. Results and discussion. One month after antihypertensive treatment, the serous retinal detachments resolved and the visual acuity improved. A patient with those findings should be considered as having hypertensive choroidopathy and treated as soon as possible

Optical coherence tomography guided peeling of macular epiretinal membrane

Optical coherence tomography (OCT) has emerged as a powerful diagnostic aid in disorders of the vitreoretinal juncture. The purpose of this study is to determine whether OCT can be used as an additional tool for evaluating an architecture including the thickened area, and the identifiable edge of a macular epiretinal membrane (ERM), and helping us to dissect the ERM from the retinal surface more easily and safely. In two cases with ERM, the edges of the membranes were detected by OCT, and the peeling of the membrane was started at the area easily. OCT guided ERM peeling might be useful for dissecting ERM membranes without any hesitation

Transient tractional retinal detachment in an eye with retinitis pigmentosa

We present a case of retinitis pigmentosa with vitreoretinal traction-associated retinal detachment. The retinal detachment was detected in the nasal periphery. No retinal breaks and no active vascular leakage were observed by fundus scopy and fluorescein angiography, respectively. However, 8 months later, the tractional retinal detachment was spontaneously resolved with posterior vitreous detachment

Histological development of stapes footplate in human embryos.

Normal development of the human stapes footplate was investigated in serial sections by light microscopy. Materials were obtained from 35 Japanese embryos from the 6th to 32nd week of embryonal age. Eighteen embryos up to 16 weeks of age (3.5mm to 105mm in crown-rump length) were examined, focusing particularly on the lamina stapedialis of the otic capsule. The present study showed that primordial formation of the lamina stapedialis appeared in 16mm embryo and that the lamina was completely formed and fused to the base of the annular stapes in a 35mm embryo. In a 50mm embryo, the adult form of stapes was found with a rim and annular ligament. The results, therefore, seemed to essentially agree with the theory of dual origin and development of the footplate proposed by Cauldwell and Anson, and teratogenic agents might affect any stage of the process producing anomalies,</p

The Microvascular Architecture of the Vestibule and the Endolymphatic Duct and Sac of the Rat in Vascular Corrosion Casts

The blood vessels of the vestibule and the endolymphatic duct and sac (ES) of the rat were reproduced with methacrylate casting medium and observed under a scanning electron microscope. Dense capillary networks of the macula utriculi and the macula sacculi were observed. The collecting venules from the vestibule emptied into the vein of the vestibular aqueduct (VVAQ). The plexus of the vessels in the ES was triangular in shape and had anastomoses with vessels of the bone and dura and drained into the VVAQ. The posterior meningeal artery (PMA) gave off two branches to the ES. These findings supported the similarity of the vascularization of the vestibule and the ES between the human and the rat

Unilateral cystic inner ear anomalies in siblings.

Unilateral cystic inner ear anomalies were diagnosed in two siblings, a 9 year old boy and a 6 year old girl. X-ray examination of the temporal bone was performed, together with audiological examinations and vestibular function tests. The common tomographic X-ray findings consisted of an enlarged solitary sac type deformity of the vestibule with narrowing of the internal auditory canal, severe hypoplasia of the anterior semicircular canal and no visualized cochlea. Pure-tone audiometry revealed severe mixed type of hearing loss in the right ear in both children. The test for vestibular function showed no response to caloric testing.</p

α-Naphthylisothiocyanate (ANIT) Induced Cholestasis in Rats

In order to distinguish the disorder of bile acid and cholesterol metabolism in α-naphthylisothiocyanate (ANIT)-induced cholestasis, we examined changes in bile acid levels and compositions in bile, serum, feces and urine, as well as cholesterol levels in bile, serum, liver and feces in Wistar male rats (10-13 weeks) after a single oral administration of 100 mg/kg of ANIT. The bile flow and the biliary secretions of cholesterol, phospholipids and bile acids markedly decreased on days 1 and 2 but increased over the normal values on day 4 and then returned to the normal ranges. The fecal excretion of bile acids decreased after the treatment and remained low by day 4 but markedly increased thereafter. The urinary excretion of bile acids changed almost in parallel with serum bile acid level, increasing to 37 mg/day on day 2, 28 mg/day on days 3-4 but to a trace on days 5-6. The urinary bile acids on day 2 mainly consisted of cholic acid while those on days 3-4 and biliary bile acids on day 4 were mostly β-muricholic acid. The serum cholesterol level markedly increased maximally on day 2 and decreased thereafter. The fecal excretion of sterols, cholesterol and coprostanol, decreased on days 1-2 but rather increased thereafter. These data suggest that the cholestasis induced by ANIT is very similar to that in bile duct ligated rats for a short period but not to those ligated for long periods. In addition, the present data suggest that the bile acid independent bile flow is impaired and the daily synthesis of bile acids, especially β-muricholic acid, is increased in the ANIT induced cholestasis