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H Date

Publications and source records attributed to H Date.

At least 37 records · Page 2Linked to original sources

Early-onset ataxia with ocular motor apraxia and hypoalbuminemia is caused by mutations in a new HIT superfamily gene.

Friedreich ataxia (FRDA), the most common autosomal recessive neurodegenerative disease among Europeans and people of European descent, is characterized by an early onset (usually before the age of 25), progressive ataxia, sensory loss, absence of tendon reflexes and pyramidal weakness of the legs. We have recently identified a unique group of patients whose clinical presentations are characterized by autosomal recessive inheritance, early age of onset, FRDA-like clinical presentations and hypoalbuminemia. Linkage to the FRDA locus, however, was excluded. Given the similarities of the clinical presentations to those of the recently described ataxia with oculomotor apraxia (AOA) linked to chromosome 9p13, we confirmed that the disorder of our patients is also linked to the same locus. We narrowed the candidate region and have identified a new gene encoding a member of the histidine triad (HIT) superfamily as the 'causative' gene. We have called its product aprataxin; the gene symbol is APTX. Although many HIT proteins have been identified, aprataxin is the first to be linked to a distinct phenotype.

Amino Acid Sequence↗

Microvascular angina in a patient with aortic stenosis.

A 39-year-old woman had exercise-induced ST segment depression associated with chest pain. Cardiac evaluation revealed moderate aortic stenosis (AS), related to the bicuspid valves, with an aortic mean pressure gradient of 22 mmHg, a calculated aortic valve area of 1.3 cm2 and normal left ventricular (LV) peak systolic and end-diastolic pressures, but no LV hypertrophy, resulting in normal LV wall stress. Although the coronary arteries were angiographically normal, rapid atrial pacing and an intracoronary papaverine injection revealed a significantly decreased coronary flow reserve (CFR), which may have played an important role in the pathogenesis of angina pectoris in this patient. Though the CFR is usually decreased in patients with AS, as well as in microvascular angina, in this particular case, it appeared to have decreased as a consequence of microvascular dysfunction rather than of AS-related mechanisms.

Adult↗

Current status and future of lung transplantation.

Lung transplantation has been performed successfully outside Japan since 1983 in patients with end-stage lung disease. More than 9,000 lung transplants have been reported in The Registry of the International Society for Heart and Lung Transplantation. In contrast, a transplant law became effective in Japan only recently, and four universities were designated as official lung transplant centers (Okayama, Osaka, Kyoto and Tohoku Universities). In October 1998, the first successful living-donor lobar lung transplantation was performed at Okayama University. Since then, seven lung transplants (four from living donors and three from cadaveric donors) have been successfully performed in Japan. Although lung transplantation offers acceptable prospects for 5-year survival, chronic rejection and donor shortage remain to be major problems. In an effort to address the donor shortage issue, living-donor lobar lung transplantations have been performed with satisfactory intermediate survival and functional results.

Adult↗

[Current status and future of lung transplantation].

Lung transplantation has been performed successfully since 1983 in patients with various end-stage lung diseases including primary pulmonary hypertension. More than 10,000 lung transplants have been reported in The Registry of the International Society for Heart and Lung Transplantation. In contrast, a transplant law became effective in Japan only recently and 11 lung transplants have been performed with excellent results. We performed the first successful living-donor lobar lung transplantation for a 19-y-o-f with primary pulmonary hypertension on January 5, 2001 using her father's right lower lobe and her mother's left lower lobe. When the patients with primary pulmonary hypertension do not respond to prostacyclin therapy, lung transplantation is a workable option.

Adult↗

Involvement of two different mechanisms in trigeminal ganglion-evoked vasodilatation in the cat lower lip: role of experimental conditions.

The present study was designed to examine the vasodilator mechanisms elicited by electrical stimulation of trigeminal ganglion (TG) in cat lower lip of the cats. When vago-sympathectomized cats were fixed into a stereotaxic frame by means of ear-bars, etc., the lip blood flow (LBF) increase evoked by lingual nerve (LN) stimulation (parasympathetic reflex response) was almost abolished in 15 out of 34 animals, but unaffected in the other 19. With the animal in the stereotaxic frame, electrical stimulation at sites within the TG evoked an LBF increase whether or not the LN stimulation-induced reflex response was intact. However, hexamethonium abolished the TG stimulation-induced LBF increase in animals whose brainstem parasympathetic reflex was intact, but reduced it by only 50% in animals whose reflex was impaired. This difference was seen in all experiments in which the electrode site was within the TG proper, regardless of its exact position. Although the underlying mechanism is unclear, these data suggest that when the TG is stimulated the LBF increase is entirely mediated via the parasympathetic reflex mechanism in animals whose brainstem reflex is intact, and that an antidromic vasodilatation occurs only in animals whose brainstem parasympathetic reflex is impaired.

Adrenergic Antagonists↗

Elucidation of accuracy in calibration of MR signal intensity based on transmission amplitude method.

To calibrate magnetic resonance (MR) signal intensity that depends on radio frequency (RF) coil loading, the transmission amplitude (TRA) for the excitation in the transmit-receive RF coil has been used as a good index in the so-called TRA method. As this TRA method needs neither an internal reference nor an additional external reference for the calibration, its accuracy is free from reference measurements. This study elucidated the calibration accuracy of MR signal intensities based on the TRA method. A cylindrical gel phantom was used for accuracy measurements with a 1.5-T MRI unit with conventional T1 imaging as a simple pulse sequence for various loading conditions. The brain parenchyma of eight healthy volunteers also showed calibrated MR signal deviations. The error of the phantom calibration measurements was 2.18% (S.D.%). The background noise intensity of images was theoretically derived to correlate with the impedance mismatching of the RF coil, which is inevitable for fixed tuning, even for automatic tuning that is not always exact. Taking into account this noise intensity, the calibration method was modified to reduce its error to 1.50%. The standard deviations of the calibrated values in the thalamus and frontal white matter were 2.9 and 3.8%, respectively. We suggest that the modified TRA method is a practical and reliable technique to obtain clinical numeric evidence.

Brain↗

Referrals for lung transplantation in Japan. Unique indications and necessity of living-donor lobar lung transplantation.

OBJECTIVES: The purpose of this study was to review all referrals to our lung transplant program and to find realistic options for accepted candidates in Japan. METHODS: During the period from April 1992 to August 1999, 45 referrals were received. After screening, 22 of the referred patients were admitted for an extensive inpatient evaluation, and 15 of these were accepted as candidates for transplantation. RESULTS: The indication was primary pulmonary hypertension in 9, bronchiectasis in 2, lymphangioleiomyomatosis in 2, idiopathic pulmonary fibrosis in 1, and pneumoconiosis in the other 1. Two of those with primary pulmonary hypertension went to the USA and there received bilateral lung transplant. One with bronchiectasis received living-donor lobar lung transplantation in our center. These three recipients are alive and doing well during the follow-up period of 11 to 69 months. Among the 12 patients who have not received lung transplant, 5 patients have died while waiting. CONCLUSIONS: Indications for lung transplant are quite distinct in Japan, and primary pulmonary hypertension is the most frequent indication. Living-donor lobar lung transplantation is a realistic option for properly selected candidates.

Adolescent↗

One year follow-up of the first bilateral living-donor lobar lung transplantation in Japan.

One-year follow-up results on Japan's first bilateral living-donor lobar lung transplantation concern a 24-year-old woman who had become ventilator-dependent due to severe bronchiectasis associated with primary ciliary dyskinesia. Surgery was conducted using her sister's right lower lobe and her mother's left lower lobe. Total forced vital capacity of the 2 transplanted lobes was 51.7% of the recipient's predicted forced vital capacity. One-year after transplantation, her forced vital capacity was 2,160 ml--73.2% of her predicted forced vital capacity. The recipient's sister's decrease in forced vital capacity was 410 ml and that of her mother 440 ml. The recipient and donors have since returned to normal, unrestricted lives.

Adult↗

Effects of inhaled nitric oxide in a canine living-donor lobar lung transplant model.

OBJECTIVE: In living-donor lobar lung transplantation, early severe graft dysfunction can occur if the size or amount of transplanted lung tissue is insufficient. The purpose of this study was to evaluate the effects of inhaled nitric oxide on early pulmonary function in a canine bilateral living-donor lobar lung transplant model. METHODS: Sixteen pairs of mongrel dogs with a donor/recipient weight ratio less than 1.2 were used. The donor lung bloc was extirpated after heparinization. Right middle, lower and cardiac lobes were implanted as a right lung of the recipient and left lower lobe was implanted as a left lung without cardiopulmonary bypass. In Group 1 (n = 9), nitric oxide gas was administered continuously at a concentration of 40 parts per million prior to reperfusion of the right lung throughout the 6-hour assessment period after transplantation. In Group 2 (n = 7), nitrogen gas was administered in the same manner as nitric oxide, for control. RESULTS: At the end of assessment, the survival rate was 89% (8/9) in Group 1 and 57% (4/7) in Group 2. The arterial oxygen tension in Group 1 was significantly higher than that in Group 2. The pulmonary arterial pressure and pulmonary vascular resistance index were significantly lower in Group 1 than in Group 2. The aortic pressure and cardiac index did not differ significantly between the two groups. The wet-to-dry weight ratio and myeloperoxidase activity were significantly lower in Group 1 than in Group 2. CONCLUSIONS: These data suggested that inhaled nitric oxide improved early pulmonary function in living-donor lobar lung transplantation by vasodilatating the pulmonary vasculature and inhibiting neutrophil activation.

Administration, Inhalation↗

Inhaled nitric oxide ameliorates postoperative acute graft dysfunction after living-donor lobar lung transplantation.

Inhaled nitric oxide, a selective pulmonary vasodilator, decreases mean pulmonary artery pressure and pulmonary vascular resistance and improves oxygenation in previously unobtainable ways without significant changes in systemic hemodynamics. We report successful treatment of a patient with acute graft dysfunction after living-donor lobar lung transplantation using nitric oxide inhalation.

Acute Disease↗

Predictors of improvement in FEV1 (forced expiratory volume in 1s) after lung volume reduction surgery.

We evaluated various preoperative and operative factors to identify the predictors of improvement in forced expiratory volume in 1s (FEV1) after lung volume reduction surgery (LVRS). Fifty-eight emphysema patients received bilateral LVRS either via a sternotomy (n = 53) or by thoracoscopy (n = 5). The patients were divided into the following two groups: group I (n = 17), patients whose FEV1 improved by less than 20%; group II (n = 41), patients whose FEV1 improved by more than or equal to 20%. The preoperative factors (age, degree of dyspnea, oxygen use, steroid use, pulmonary function test, arterial blood gas, pulmonary hemodynamics, 6-min walking distance) and operative factors (removed lung weight, number of staplers) were both similar between the two groups. Upper lobe type emphysema was more frequently seen in group II and the average improvement in FEV1 was significantly better in the patients with upper lobe type emphysema (62.8% +/- 8.5%) than lower lobe type (36.9% +/- 7.6%) and mixed type (35.5% +/- 6.5%), P < 0.01. Severe pleural adhesion was more frequently found in group I and the average improvement in FEV1 was also significantly lower in patients with severe pleural adhesion (19.8% +/- 6.4%) than mild-to-moderate adhesion (46.8% +/- 5.3%) and no-to-minimal adhesion (62.3% +/- 10.4%), P < 0.01. These results lead us to conclude that upper lobe type emphysema may thus be a predictor of better FEV1 improvement while severe pleural adhesion is considered to be a predictor of a poorer FEV1 improvement after bilateral LVRS.

Aged↗

Short-term inhaled nitric oxide in canine lung transplantation from non-heart-beating donor.

BACKGROUND: Use of lungs harvested from non-heart-beating donors (NHBDs) would increase the pulmonary donor pool; however, this strategy would have higher risk of early postoperative graft dysfunction due to unavoidable warm ischemic time. We evaluated the effects of short-term inhaled nitric oxide (NO) during reperfusion in canine left single-lung allotransplantation from a non-heart-beating donor. METHODS: The donor dogs were sacrificed without heparinization and left at room temperature for 3 hours. Then, recipient dogs received a left single-lung allotransplantation. After implantation, the right bronchus and pulmonary artery were ligated. In group 1 (n = 6), NO gas was administered continuously at a concentration of 40 parts per million throughout a 6-hour assessment period. In group 2 (n = 6), NO gas was administered for the initial 1 hour during reperfusion. In group 3 (n = 6), nitrogen gas was administered for control. RESULTS: Groups treated with NO exhibited lower pulmonary vascular resistance, as well as improved survival and oxygenation. There was no significant difference in these parameters between group 1 and group 2. Myeloperoxidase activity was significantly lower in NO-treated groups. CONCLUSIONS: Inhaled NO during reperfusion is beneficial in lung transplantation from non-heart beating donors. The beneficial effect is obtained mainly during the first hour of reperfusion.

Administration, Inhalation↗

Increased plasma antigen levels of monocyte chemoattractant protein-1 in patients with restenosis after percutaneous transluminal coronary angioplasty.

Monocyte chemoattractant protein-1 (MCP-1) plays an important role in the progression of atherosclerosis in coronary arteries. To examine whether or not plasma antigen levels of MCP-1 are related to restenosis after percutaneous transluminal coronary angioplasty (PTCA), the plasma antigen levels of MCP-1 were measured by enzyme-linked immunosorbent assay (pg/ml) before, 24 and 48 h, and 3 months after elective PTCA for stable exertional angina performed between June 1997 and March 1998. Restenosis was defined as recurrence of stenosis greater than 50% of the diameter in the dilated segment at 3-month follow-up angiography. There were no differences in plasma MCP-1 antigen levels before and at 24 h after PTCA between restenosis (R; n=27) and no-restenosis (N; n=43) groups (R vs N: 633+/-35 vs 589+/-34, and 669+/-41 vs 575+/-36 pg/ml before and at 24 h after PTCA, respectively), but plasma MCP-1 antigen levels were higher at 48 h and 3 months after PTCA in the R than in N group (R vs N: 678+/-41 vs 558+/-35, and 735+/-35 vs 571+/-32 pg/ml at 48 h and 3 months after PTCA, respectively). These data suggest that the MCP-1 production and macrophage accumulation in the balloon-injured site is partially associated with restenosis after PTCA.

Aged↗

[Two cases of thymic carcinoma effectively treated by preoperative chemotherapy].

A 67-year-old man was admitted with left pleural effusion on chest X-ray film. Chest CT scans showed an anterior mediastinal tumor, left pleural dissemination and pleural effusion. Percutaneous needle biopsy yielded a diagnosis of epitherial carcinoma. The patient underwent 3 courses of systemic chemotherapy (CDDP + ADM + VCR + CPA). At achievement of partial response, the tumor was completely resected. Examination of the resected tumor revealed partial response to chemotherapy. A 49-year-old woman was admitted our hospital with supraclavicular lymph nodes swelling. Chest CT scans showed an large mediastinal tumor. Percutaneous needle biopsy yielded a diagnosis of squamous cell carcinoma of thymus. The patient underwent 3 courses of systemic chemotherapy. At achievement of partial response, the tumor was completely resected. Examination of the resected tumor revealed partial response to chemotherapy. These cases indicate the usefulness of preoperative chemotherapy for advanced thymic carcinoma to reduce tumor size and to control local invasion, distant metastasis before operation.

Aged↗

[Three cases of pulmonary mucoepidermoid carcinoma].

Three cases of pulmonary mucoepidermoid carcinoma, which were surgically treated in our hospital, were reported. The first case was 28-year-old male, who had hemoptysis, underwent right upper lobectomy and mediastinal lymph node dissection on October 31, 1973. The tumor located and obstructed the ostium of right B2. The diameter was 2.3 x 1.8 cm. The second case was 63-year-old male, who had obstructive pneumonia, underwent left upper lobectomy and mediastinal lymph node dissection on November 18, 1998. The tumor located and obstructed the ostium of lingular bronchus, and the diameter was 1.8 x 1.2 cm. The third case was 25-year-old male, who had obstructive pneumonia, underwent left sleeve lower lobectomy and mediastinal lymph node dissection on May 26, 1999. The tumor located in ostium of left B6, and it obstructed lower bronchus and expanded into the lung. The diameter was 4 x 2 cm. All cases were diagnosed as low grade malignancy with no lymph node metastasis. The first case survived without recurrence at least 5 years as far as we followed, and the others are surviving until now without any signs of recurrence. These three cases were 0.19% of total resected lung cancers in our hospital from 1969 to 1999.

Adult↗