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Biomedical subjects

S Wakai

Publications and source records attributed to S Wakai.

At least 19 recordsLinked to original sources
PubMed1992

Successful surgical obliteration of a huge intradural arteriovenous fistula of the spinal cord in a child.

We report the case of an 8-year-old boy with a huge intradural spinal arteriovenous fistula (AVF), which was successfully obliterated by surgery. The symptoms were episodic headaches and progressive motor and sensory deficits. He had suffered a subarachnoid hemorrhage twice before but no ensuing neurological deficits. Neuroradiological examinations revealed an intradural AVF with a huge venous aneurysm ventral to the spinal cord of C7 to T2, which was fed by the left 5th intercostal artery and the right thyrocervical artery. These two feeding arteries were occluded intradurally just at the venous aneurysm emerging point. MR images taken sequentially after surgery demonstrated complete thrombosis and subsequent disappearance of the thrombosed malformation. Neurological symptoms improved gradually. Treatment of such malformations is discussed.

Angiography↗
PubMed1992

Delayed expression of dystrophin on regenerating muscle from two siblings with Becker muscular dystrophy.

We present here a unique expression of dystrophin on biopsied muscle from 2 siblings with Becker muscular dystrophy (BMD). They had neither muscle weakness nor atrophy. Clustered dystrophin-deficient fibers were constituted to regenerating basophilic fibers (mainly type 2C fiber) based on histochemical stainings. We speculate that the developmental delay in the expression of dystrophin is a characteristic finding in regenerating fibers from asymptomatic and young BMD patients, such as the siblings in this report.

Child↗
PubMed1992

Focal glomerulosclerosis in idiopathic membranous glomerulonephritis.

The significance of the finding of focal glomerulosclerosis (FGS) in idiopathic membranous glomerulonephritis (MGN) is uncertain. Twenty-seven patients with mixed FGS and MGN (MGN-FGS) were compared to 25 patients with MGN alone (generally matched for age, sex and stage of glomerular lesion) with respect to pathology, presenting clinical and laboratory features, and course of disease. Biopsies from the MGN-FGS patients showed significantly more extensive tubulointerstitial disease (P less than 0.001) than did those with MGN alone. At the time of biopsy, the MGN-FGS group had a significantly higher proportion of patients with hypertension (P = 0.006) and microhematuria (P = 0.006), a marginally higher percentage of patients with the nephrotic syndrome (P = 0.051), and a greater mean 24-hour urinary protein excretion (P = 0.004). A similar proportion of patients in each group were treated with either prednisone alone or prednisone with an immunosuppressive. Forty-eight percent of MGN-FGS patients and 13% of the MGN patients developed established renal failure in the follow-up period (P = 0.008). The renal survival rate for the MGN-FGS group was significantly lower at 24 months (0.61 vs. 0.93, P less than 0.05), 60 months (0.48 vs. 0.88, P less than 0.025), and over the entire follow-up period (P less than 0.05). The results indicate that FGS in MGN is associated with a significantly poorer prognosis than MGN without this lesion.

Acute Kidney Injury↗
PubMed1992

[Effects of smoking and drinking habits and vitamin A intake on serum concentrations of beta-carotene and retinol].

Serum samples from 341 males aged 10 to 59 years were obtained and stored at -40 degrees C until examined for retinol and beta-carotene concentrations by HPLC, and their relationships to smoking habit, alcohol drinking habit and vitamin A intake were studied. In univariate analysis the serum beta-carotene level was significantly lower in the smokers than in the non-smokers (smokers: 4.6 micrograms/dl, non-smokers: 7.1 micrograms/dl, p less than 0.01) and lower in the drinkers than in the non-drinkers (drinkers: 4.6 micrograms/dl, non-drinkers: 7.3 micrograms/dl, p less than 0.01). The serum retinol level was not different by smoking habit but was higher in the drinkers than in the non-drinkers (drinkers: 80.4 micrograms/dl, non-drinkers: 67.0 micrograms/dl, p less than 0.01). Serum beta-carotene was higher in the group with a greater intake of vitamin A of vegetable origin (6.1 micrograms/dl) than in the group with a smaller intake of it (4.7 micrograms/dl) (p less than 0.01), but serum retinol was not different by the amount of vitamin A intake of animal food origin. To estimate the respective effects and interactions of the above factors on serum beta-carotene and retinol levels by adjusting for the confounding effects of age, serum total cholesterol, HDL-cholesterol and triglyceride, analysis of covariance was performed. For serum beta-carotene, smoking habit (p less than 0.01), drinking habit (p less than 0.01) and the amount of vitamin A intake of vegetable food origin (p less than 0.05) had significant main effects.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1992

Lobar intracerebral hemorrhage. A clinical, radiographic, and pathological study of 29 consecutive operated cases with negative angiography.

The authors operated consecutively on 50 patients with lobar intracerebral hemorrhage during a prospectively designed study period from January, 1986, to March, 1990. They investigated the correlations between the underlying causes and the clinicoradiographic features in 29 patients who showed no angiographic vascular abnormalities, in order to elucidate the operative indication for such cases. Patients with ruptured saccular aneurysm or trauma were not included in this study. There were 15 males and 14 females, ranging in age from 7 to 76 years (mean 52.4 years). Histological diagnoses of the surgical specimens were as follows: vascular malformation in nine cases (arteriovenous malformation (AVM) in six and cavernous malformation in three), microaneurysm in 11, cerebral amyloid angiopathy in six, and brain tumor in two; in the remaining case the cause was not verified histologically. The underlying cause was determined in 96.5% of cases. The mean patient age was lowest in the cavernous malformation group (27.0 years), followed by the AVM (45.8 years), microaneurysm (59.8 years), and cerebral amyloid angiopathy (70.0 years) groups. Four patients with vascular malformation (three AVM's and one cavernous malformation) had previous episodes of bleeding at the same site, whereas none of those with microaneurysms or cerebral amyloid angiopathy had such episodes. On computerized tomography (CT) scans, the round to oval hematoma was related to the presence of an AVM or cavernous malformation in contrast to microaneurysms and cerebral amyloid angiopathy. Upon infusion of contrast material, variable enhancement was seen in five (two AVM's and three cavernous malformations) of the nine vascular malformations while no enhancement was noted in any patient with microaneurysm or cerebral amyloid angiopathy at the acute stage. Subarachnoid extension of the hematoma was associated with cerebral amyloid angiopathy significantly more frequently than with AVM's (p less than 0.05) and microaneurysms (p less than 0.01). The results suggest that clinicoradiographic pictures in cases with negative angiography are quite different among the three major pathological categories; namely, vascular malformation (AVM and cavernous malformation), microaneurysm, and cerebral amyloid angiopathy. It is suggested that the underlying etiology of a given lobar intracerebral hemorrhage with negative angiography may be predicted by a combination of patient age, history of previous bleeding at the same site, hematoma shape, and subarachnoid extension of the hematoma on CT scans. Based upon these findings, the authors discuss operative indications for such cases.

Adolescent↗
PubMed1992

Severe neonatal nemaline myopathy--histological and histochemical studies of respiratory muscles.

The histological and histochemical findings in the respiratory muscles of a patient with severe neonatal nemaline myopathy are described. The patient suffered from frequent pneumonia associated with vomiting due to gastroesophageal reflux and died at 3 months from respiratory failure. The diaphragm was moderately involved and the intercostal muscles mildly involved. Core/targetoid structures were observed in the diaphragm and intercostal muscles.

Comorbidity↗
PubMed1991

Benign familial neonatal convulsions: clinical features of the propositus and comparison with the previously reported cases.

A family with benign familial neonatal convulsions (BFNC) was presented. The propositus had his first episodes of cyanosis on the second day after birth. Thereafter, he also experienced multifocal clonic and/or focal clonic seizures. Between the seizures he appeared well and was essentially normal upon physical examination. Treatment with phenobarbital (4 mg/kg/day, p.o.) was started, and subsequently, he had no further seizures until 3 months of age. At the age of 4 months, he was again admitted to the hospital because of generalized tonic-clonic seizures. The findings of ictal EEG at that time were characterized by fast spiking with increasing amplitude during the tonic phase. During the clonic phase, there were repetitive bursts of spikes or sharp waves mixed with persisting muscle potentials. The termination of the convulsion was characterized by general voltage depression. Reference to previously reported cases of BFNC revealed that 10-15% of patients with this disorder had epilepsies later in life.

Electroencephalography↗
PubMed1991

Ruptured distal anterior cerebral artery aneurysms presenting as acute subdural hematoma--report of three cases.

The authors report three cases of distal anterior cerebral artery aneurysm presenting as acute subdural hematoma (SDH). Two patients were comatose on admission and died of massive SDH. One patient underwent aneurysmal neck clipping in the chronic stage and returned to normal daily life. A convexity SDH continuous with a wedge-shaped interhemispheric SDH was the characteristic computed tomographic appearance in all cases. There was no accompanying subarachnoid or intracerebral hemorrhage in one case (pure SDH). These cases are 9.4% of 32 ruptured distal ACA aneurysms treated in our institute in the last 14 years, a higher incidence than reported previously.

Acute Disease↗
PubMed1991

[Occipital horn syndrome (Ehlers-Danlos syndrome type IX) with severe psychomotor retardation and muscle atrophy--a first Japanese case].

Occipital horn syndrome (OHS; Ehlers-Danlos syndrome type IX) belongs to the category of the copper metabolism disorders and is at present being investigated biochemically as is Menkes disease. We report a case of OHS in a 34-year-old male, which we believe to be the first Japanese case. He had been noted to have psychomotor retardation since his early childhood and now presents severe psychomotor retardation and muscle atrophy. He shows characteristic facial appearance, hyperelasticity of the skin, joint subluxation and generalized muscular atrophy. Laboratory investigations revealed a low serum copper and ceruloplasmin level as well as intestinal non-absorption of copper. Radiologic imagings showed occipital exostoses and bladder diverticula. The activity of lysyl oxidase, a copper-dependent enzyme involved in cross-link formation in collagen, was decreased in a skin-biopsied specimen. Electronmicroscopic investigation of a muscle biopsy showed irregularity of the myofibrillar network and accumulation of the concentric laminated bodies in the subsarcolemmal regions.

Adult↗
PubMed1991

[Anti-cardiolipin antibody and renal microthrombi in lupus nephritis].

To evaluate the relationship between anticardiolipin (ACL) antibody and microthrombi in renal tissue, we examined sera and renal biopsies from 47 patients of systemic lupus erythematosus (SLE) with lupus nephritis (LN). ACL antibody was measured by an enzyme-linked immunosorbent assay (ELISA). Their renal tissues were examined for histological types of lupus nephritis according to the WHO classification and appearance of renal microthrombi. Positive ACL had been shown in 28 of 47 patients (60%) with LN and in 15 of 18 patients (83%) with WHO IV (diffuse proliferative LN: DPLN). Incidences of renal microthrombi were significantly higher in patients with DPLN (61%) than in patients with all LN (34%) (p less than 0.01). The prevalence of renal thrombosis in patients with ACL (46%) was significantly higher than without ACL (16%) (p less than 0.05). Incidence of positive ACL was 81% in the cases with renal microthrombi and 48% in the cases without them. These findings suggest that there is strong association between ACL and the renal microthrombi in active LN.

Autoantibodies↗
PubMed1991

[A case of Moebius syndrome--electrophysiological studies of facial nerve and brainstem].

A five-year old boy was the product of a 40 week pregnancy by vertex presentation complicated only by threatened abortion at approximately 8 weeks gestation. Apgar score was 5 after one minute. At birth he was noted to have a generalized hypotonia associated with facial diplegia, small mandible, weak suck and swallow reflexes. Admission examination revealed small mandible, mask-like facial expression and mild mental retardation. Cranial nerve examination showed bilateral blepharoptosis and facial nerve palsies. Pupil reflexes were normal, but corneal reflexes were impaired bilaterally. Diplopia due to the left abducens nerve palsy was suggested. There was no atrophy of the tongue. Motor tone, strength, and deep tendon reflexes were normal. A normal 46 XY karyotype was present. The other clinical and laboratory findings were normal. MRI of the brain was unremarkable. The characteristics of electrophysiological studies were summarized as follows: 1) Auditory brainstem evoked responses demonstrated waveforms IV-V were abnormal because their amplitudes were less than 30% of wave I bilaterally. 2) Somatosensory evoked potentials documented by central conduction times from cervical region to sensory cortex were prolonged on both sides. 3) Facial nerve conduction velocity was calculated by evoked EMGs of the mentalis muscle electrically stimulated at two distal points over the marginal mandibular branch. MCV of the left side was reduced (34.2 m/sec). 4) The amplitude of the facial muscle potentials evoked by facial nerve stimulation was reduced on both sides. 5) Blink reflex responses documented by the latency difference of R1 responses between the two sides were prolonged.(ABSTRACT TRUNCATED AT 250 WORDS)

Blepharoptosis↗
PubMed1990

Loss of constitutional heterozygosity in chromosome 10 in human glioblastoma.

Gene deletion of chromosome 10 in 11 tumor tissues removed from nine patients with malignant glioma (seven glioblastomas and two malignant astrocytomas) was investigated. Loss of heterozygosity was found in four of the seven (57%) cases with glioblastoma, whereas heterozygosity was preserved in two malignant astrocytome cases. One glioblastoma case showed loss of heterozygosity only in the recurring tumor tissue, although heterozygosity was maintained in the initial tumor tissue. The significance of loss of heterozygosity in the growth and recurrence of glioblastoma is discussed.

Adolescent↗