[Carpentier's procedure for Ebstein's anomaly: successful and failed cases].

Two adult cases of Ebstein's anomaly underwent Carpentier's procedure. In the first case longitudinal plication limited to free wall of atrialized ventricle was performed and postoperative course was uneventful. In the second case preoperative echocardiography showed apparently restricted movement of anterior leaflet of the tricuspid valve which was compatible with intraoperative findings.

[Staged sternal closure for the case of postoperative severe cardiac failure: a convenient method in the intensive care unit].

A convenient method of staged sternal closure in the intensive care unit for a patient with severe cardiac failure was reported. According to the patient's cardiac function, optimal intersternal space can be adjusted. When using this method on a infant, the adjustment has shown to be very easy and take only a short time.

[An acute axonal polyneuropathy affecting intrinsic hand muscles following Campylobacter infection--a case report].

A 24-year-old carpenter had the shakes and fever on March 13, 1990. He suffered from watery diarrhea on March 14 and 15. He left muscle weakness in his thumbs and fingers when he drove nails with a hammer on March 24.

[Hereditary dentatorubropallidoluysian atrophy--clinical variants in a family and degeneration of cerebral white matter in a proband].

We describe a family with hereditary dentatorubropallidoluysian atrophy (DRPLA). 4 patients through 3 successive generations showed a wide clinical variety. The female proband with onset in the elderly developed choreiform involuntary movement, dementia, hyperreflexia and, at the progressive stage, mild ataxia.

[Extrapleural approach for patent ductus arteriosus and coarctation of the aorta].

During the period of 1989 to 1990, 6 consecutive children with patent ductus arteriosus or coarctation of the aorta underwent operative therapy. Ligation or division of patent ductus arteriosus or subclavian flap angioplasty for coarctation of the aorta were performed by extrapleural approach with axillary or posterolateral incision. Patients' ages ranged from 9 days to 8 years.

The particle size of hepatitis C virus estimated by filtration through microporous regenerated cellulose fibre.

To estimate the particle size of hepatitis C virus (HCV), a major causative agent of post-transfusion non-A, non-B hepatitis, we filtered plasma or serum samples through microporous cellulose fibres with different pore sizes. The amount of HCV particles in samples before and after filtration was determined by a quantitative reverse transcriptase polymerase chain reaction (PCR) method. Since there is no quantitative biological assay for HCV, except for that in chimpanzees, the HCV titre obtained from the PCR method was used in an equation constructed previously for application to filtration experiments with a flavivirus which is distantly related to HCV.

Preferential expression of the large hepatitis B virus surface antigen gene by an adenovirus-hepatitis B virus recombinant.

Using an adenovirus-hepatitis B virus (HBV) recombinant, expression of the HBV surface antigen (HBsAg) genes was examined in various cell lines using S1 nuclease mapping and radioimmunoassay. The steady-state level of the 2.4 kb RNA encoding the large HBsAg was much greater than, or the same as, that of the 2.

[Analysis of factors influencing early patency of saphenous vein grafts].

To determine factors affecting early patency of saphenous vein grafts, 140 grafts in 65 patients were studied angiographically within 2 months after operation. Twenty of the 140 grafts were occluded. Sixteen variables were extracted from the angiographic findings, intraoperative measurements, clinical characteristics, and biochemical data.

Stereographic demonstration of the nasal cavity of the rat with reference to the density of blood vessels.

Stereographic demonstration of the nasal cavity and distribution of the density of blood vessels were analyzed by computer simulation of serial sections of the rat nose. The nasal cavity was lined by 3 types of epithelium: Stratified epithelium covering the nasal vestibule, the anterior part of the maxilloturbinale, and the anterior part of the septum; respiratory epithelium covering most of the naso- and maxilloturbinalia, and middle part of the septum; and olfactory epithelium covering most of the ethmoturbinale and postero-dorsal part of the septum. Distribution of densities of blood vessels in the lamina propria of the nasal mucosa was roughly correlated with the distribution of the 3 types of epithelium: 194 blood vessels/mm2 of lamina propria beneath the olfactory epithelium; 82 vessels/mm2 beneath the stratified epithelium; and 66 vessels/mm2 beneath the respiratory epithelium.

[A case of cerebrotendinous xanthomatosis with spastic paraparesis, epilepsy, and bradykinesia].

A 26-year-old female developed mental deterioration, general convulsion, cataract and spastic gait in order since her entrance into elementary school. A diagnosis of cerebrotendinous xanthomatosis (CTX) was made because of hypercholestanolemia. At the time of admission, cataract, a mild thickening of Achilles tendons, mental deterioration, spastic paraparesis, truncal ataxia, and bradykinesia were noted.

Study on the erythrocytes from myotonic dystrophy with multi-nuclear NMR.

We have studied the water permeability through membranes, the function of the Na pump, and glucose metabolism of erythrocytes of patients with myotonic muscular dystrophy (MyD) using 1H--, 23Na, and 13C-NMR techniques. A significant decrease in water permeability was recognized in the MyD erythrocyte membrane, and impaired Na pumping was suspected to be correlated with the former biochemical abnormalities in band III protein of MyD erythrocyte membrane. Significant acceleration of glycolysis in the erythrocyte for the first 160 minutes was also recognized in MyD; however, the production of lactate showed no difference between MyD and controls.

Sequence analyses of the 3' genome end and NP gene of human parainfluenza type 2 virus: sequence variation of the gene-starting signal and the conserved 3' end.

We cloned and determined the nucleotide sequences of cDNAs against nucleocapsid protein (NP) mRNA and the genomic RNA of human parainfluenza type 2 virus (PIV-2). The 3' terminal region of genomic RNA was compared among PIV-2, mumps virus (MuV), Newcastle disease virus (NDV), measles virus (MV), PIV-3, bovine parainfluenza type 3 virus (BPIV-3), Sendai virus (SV), and vesicular stomatitis virus (VSV), and an extensive sequence homology was observed between PIV-2 and MuV. Although no significant sequence relatedness was observed between PIV-2 and other viruses, the terminal four nucleotides were identical in the viruses compared, implying a specific role of these nucleotides on the replication of paramyxoviruses.

Computed tomographic findings of progressive supranuclear palsy compared with Parkinson's disease.

We investigated computed tomographic (CT) films of 4 pathologically documented cases of progressive supranuclear palsy (PSP) in which the clinical presentations were atypical and compared the findings with those of 15 patients with Parkinson's disease (PD). Dilatation of the third ventricle, atrophy of the midbrain tegmentum, and enlargement of the interpeduncular cistern toward the aqueduct were found to be the characteristic findings in PSP. Thus, radiological findings can be useful when the differential diagnosis between PSP and PD is clinically difficult.

[Clinical features of uveitis in childhood during the past 20 years].

One hundred and fifty five cases (237 eyes) of children with uveitis were clinically observed in the past 20 years. There was no fluctuation in the number of patients during the period. Concerning the age as the first visit, the smallest group was under 4 years of age and the numbers of patients increased at ages 14 and 15.

[Auditory brainstem response and somatosensory evoked potential in Machado-Joseph disease in Japanese families].

To clarify physiological aspects of Machado-Joseph disease (MJD), we studied auditory brainstem response (ABR) and somatosensory evoked potential (SEP) in 17 clinically diagnosed patients with MJD aged 32-64 in Japanese families. ABR was recorded in 13 patients. In 8 patients, ABR were abnormal.

Detection of hepatitis C virus cDNA sequence by the polymerase chain reaction in hepatocellular carcinoma tissues.

We found the presence of hepatitis C virus (HCV) infection in liver tissues of hepatocellular carcinoma (HCC) patients who had antibodies to HCV but no serological markers for hepatitis B virus infection by the sensitive reverse transcription/polymerase chain reaction (R/PCR) method. The primers used were derived from the non-structural (NS) 3 and/or the structural (C/E) region. Amplified cDNA sequences of HCV were detected in either cancerous or non-cancerous portion of liver tissues from four out of eight HCC patients with primers of NS3 region.

[An autopsied case of manifesting chorea, serum antibody to brain proteins, neuronal degeneration in striatum and grumose degeneration in dentate nucleus].

A 49-year-old man complained of difficulty in writing due to choreic involuntary movement in the right hand, which spread gradually to his left hand, face, lower extremities and trunk during next nine years. He also showed difficulties in speech and swallowing. His family noticed his memory disturbance and change of his character.

Effect of the preS1 RNA sequence on the efficiency of the hepatitis B virus preS2 and S protein translation.

The gene coding for hepatitis B virus surface antigen consists of preS1, preS2, and S regions. Two species of mRNAs of this gene are transcribed. The larger species covers all three regions and is translated solely into preS1 protein, whereas the smaller one covers the preS2 and S regions and is translated into preS2 and S proteins.

Sequence determination of the hemagglutinin-neuraminidase (HN) gene of human parainfluenza type 2 virus and the construction of a phylogenetic tree for HN proteins of all the paramyxoviruses that are infectious to humans.

The nucleotide sequence of the hemagglutinin-neuraminidase (HN) gene of human parainfluenza type 2 virus (PIV-2) was determined. The PIV-2 HN gene was 2112 nucleotides excluding poly(A) tail. There was a single large open reading frame in the mRNA which encoded a protein of 571 amino acids with a calculated molecular weight of 63,262.

[Subacute sensory neuropathy manifestated by tonic pupils associated with small cell carcinoma of the lung].

Only a few cases of carcinomatous neuropathy with tonic pupils have been reported. In the present paper we described a 53-year-old woman with subacute sensory neuropathy who had presented with bilateral tonic pupils. She noticed numbness over the medial aspect of the left thigh in March, 1988, and then developed dysesthesia over the left forearm and hand, mild weakness of left upper and lower extremities and urinary disturbance.

[Acute autonomic and sensory neuropathy associated with galactorrhea-amenorrhea syndrome and intractable anorexia].

Acute autonomic and sensory neuropathy (AASN), one subtype of acute pandysautonomia, in which dorsal root ganglia and autonomic ganglia are involved is uncommon. Little is so far known on central nervous system involvement in AASN. In the present paper we described a rare case of AASN associated with the central nervous system manifestations such as galactorrhea-amenorrhea syndrome and intractable anorexia.

NMR study on [1-13C]glucose metabolism in the rat brain during hypoxia.

The metabolism of [1-13C]glucose in the rat brain during hypoxia was investigated by 13C NMR spectroscopy. Male Wistar rats, weighing 100-120g, were anesthetized with ketamine (50 approximately 75 mg/kg i.p.

[A case of amyotrophic lateral sclerosis associated with clinical features of progressive supranuclear palsy].

A 64-year-old male patient of amyotrophic lateral sclerosis (ALS) with frozen gait, axial rigidity and supranuclear upper gaze palsy was reported. We have followed this patient more than four years. He was well until November 1982, when he noticed weakness of left arm.