Changes in the lymphocyte subsets during high dose immunoglobulin therapy for intractable childhood epilepsy.
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Child↗
Biomedical subjects
H Shiihara
Publications and source records attributed to H Shiihara.
Histologic activity of the liver in children with transfusion-associated chronic hepatitis C.
Adults with chronic hepatitis C develop cirrhosis over a period of 6 to 20 years, but there are few reports of this disorder in children. To determine the histologic activity of chronic hepatitis C in children, we examined 31 biopsy specimens from 25 children (age range 3-16 years) with this disease. All patients were seropositive for antibody to hepatitis C virus by second-generation testing, and for HCV-RNA by the polymerase chain reaction. All cases were transfusion-associated. Patients were divided into two groups according to underlying disease: malignant disease or aplastic anemia (Group A, 17 cases) and non-malignant disease (Group B, eight cases). All patients in Group A, but only one in Group B, had received multiple transfusions. All patients in Group A had received intensive courses of cytotoxic and immunosuppressive agents. The histologic diagnosis was made using the standard criteria and Knodell's histology activity index. Chronic persistent hepatitis was more common in Group B (six patients) than in Group A (three patients). Chronic aggressive hepatitis 2B was found only in Group A (five patients). The mean histology activity index score was higher in Group A than in Group B (8.5 vs. 5.7). Six patients (four in Group A and two in group B) subsequently had a liver biopsy. The pathological diagnosis did not change after the second biopsy in any patient in Group B, while two patients in Group A showed a rapid progression of hepatitis. In each category of the histology activity index, periportal necrosis and intralobular necrosis were more severe in Group A than in Group B.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Monomelic muscle atrophy.
Two patients with slowly progressive muscle atrophy limited to only one leg are reported. They had pes equinovarus deformity and muscle weakness in the affected leg but no symptom in the other limbs. Muscle biopsies from the affected leg showed dystrophic changes consisting of variation in muscle fiber size, endomysial fibrosis, and necrotic and regenerating fibers. Dystrophin was normally expressed at the surface membrane of the muscle fibers. These two patients possibly had a variant of distal muscular dystrophy, though a neural influence could not be completely excluded.
Biopsy↗
[Neurological complications of varicella-zoster virus (VZV) infection].
Sixty cases with varicella-associated neurological disorders from Japanese literature during recent 11 years were analyzed and compared with previous reports. Chief diseases were encephalitis (encephalopathy) (23.3%), cerebellar ataxia (21.7%), meningitis (18.3%), cerebral infarction (13.3%) and facial palsy (8.3%). Cerebellar ataxia, meningitis and cerebral infarction were found in young children under 9 years, and other disorders were seen also in older children and adults. Some cases had neurological symptoms before the appearance of skin rash. The number of cells in cerebrospinal fluid was increased in meningitis, encephalitis and myelitis. Though neurological complications due to varicella were rare, prognosis was not necessarily good, including several cases with death or severe sequelae. In our 4 cases of herpes zoster meningitis, marked intrathecal VZV-specific antibody production was found and they showed high antibody index. Oligoclonal band was found in one case. The pathogenesis of neurological complications of VZV infection was considered to be caused by direct viral invasion in herpes zoster and at least in some cases of varicella. Therapy with antiviral agents is necessary and vaccination is recommended for prevention.
Adolescent↗
Successful pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension associated with anticardiolipin antibodies: report of a case.
Chronic pulmonary thromboembolism with pulmonary hypertension is a rare but most unique syndrome in the broad spectrum of pulmonary embolism. This report describes a successful pulmonary thromboendarterectomy performed for a totally occluded right pulmonary artery on a 43 year old man who presented with positive cardiolipin antibodies. The surgery was performed through a median sternotomy with cardiopulmonary bypass and intermittent periods of deep hypothermic circulatory arrest. We are convinced that this method allows for complete removal of the thrombotic obstruction and should be the procedure of choice for patients with very proximal obstruction of a pulmonary artery.
Adult↗
[Time-course changes of regional cerebral blood flow by SPECT with N-isopropyl-p-[123I]-iodoamphetamine in childhood partial seizures].
Single photon emission computed tomography (SPECT) with N-isopropyl-p-[123I]-iodoamphetamine was performed twice at different times in 18 children suffering from partial seizures to evaluate the time-course changes of the hemodynamics in the focal region. Comparison of the 2 SPECT images revealed that a decreased regional cerebral blood flow (CBF) in the first was normalized in 4 cases (22.2%) and contracted in 7 cases (38.9%). The region of decreased blood flow in the second was contralateral to that in the first in 1 case (5.6%) and unchanged in 6 cases (33.3%). Most of the normalized and contracted cases were those in which clinical seizures disappeared and the EEG findings were normalized. In these cases which responded to treatment, the decreased regional CBF was also found to be improved. Repeated SPECT appears to be useful for evaluating the therapeutic efficacy. On the other hand, most of the unchanged cases were those in which clinical seizures were frequent or uncontrolled, or which persistently showed the abnormalities in their EEG. SPECT was considered to reflect well the conditions of the epileptic foci. These results indicate that repeated SPECT for observing the time-course changes of the regional CBF represents a useful technique with can be applied in evaluating the therapeutic efficacy or deciding the time to discontinue treatment and for pathogenetic elucidation of the epileptic foci.
Adolescent↗
Central nervous system damage following surgery using cardiopulmonary bypass--a retrospective analysis of 1386 cases.
In order to determine the incidence and risk of central nervous system damage (CNSD) which accompanies cardiovascular surgery, a retrospective analysis was carried out on 1386 patients who received surgery utilizing cardiopulmonary bypass. CNSD occurred in 32 of the 1386 patients, the total incidence being 2.3 per cent. The major risk factors which led to a high incidence of CNSD were found to be reoperation, thrombus in the left atrium (TLA) and calcification of the ascending aorta (Cal aAo). In the primary surgical series, CNSD was found in only 29 of 1350 patients (2.2 per cent), however, of a total 36 patients who underwent reoperation, 3 (8.3 per cent) patients developed CNSD (p less than 0.05). In a surgical series done on 562 patients with mitral valve disease, the prevalence of CNSD was much higher in patients who suffered TLA than in those who did not, being 4/85 cases (4.7 per cent) vs 11/477 cases (2.3 per cent), respectively. Moreover, a significantly higher incidence of CNSD was noted in patients with Cal aAo, occurring in 6/10 cases (60 per cent) with Cal aAo vs 4/333 cases (1.2 per cent) without Cal aAo (p less than 0.001). The results of this study showed that patients with CNSD, who had received coronary artery bypass surgery and aortic arch replacement tended to follow a poor clinical course, suggesting that atheromatous plaque embolization may be the leading cause of the poor prognosis following CNSD in patients having undergone cardiopulmonary bypass.
Adult↗
[Pre- and postoperative nutritional assessment of cardiac cachexia].
Pre- and postoperative nutritional states were studied in fifty patients undergoing cardiac surgery. Seven of them were cardiac cachectic patients. There was no hospital death except one patient with cardiac cachexia. Preoperative measurements showed decreased %standard body weight (80.7%), %standard AMC (86.1%), and %standard TSF (61.5%) in cachectic patients. Resting energy expenditure (REE) and circulating blood volume were significantly higher in cachectic patients throughout the pre- and postoperative periods, and a significant correlations were admitted between them (p less than 0.05). REE increased to 33.6 kcal/kg/day 1 week after operation. Serum proteins, including rapid turnover proteins (RTP), when expressed in g/dl or mg/dl, did not show any difference between two groups. However by correction with circulating plasma volume and body weight (mg/kg), cardiac cachectic patients had significantly higher protein contents preoperatively and seventh post-operative day (p less than 0.05). There was no difference in urinary excretion of 3-methylhistidine between two groups throughout the study period, but urinary excretion of urea was higher in 1st and 3rd postoperative days in non cachectic patients. Serum cortisol and urinary excretion of vanillymandelic acid were higher, and serum insulin level tended to be lower in cachectic patients postoperatively. These results indicate that, in cardiac cachexia, increased blood volume and hypermetabolic state cause higher energy expenditure, resulting in weight loss and chronic heart failure.
Adult↗
[Surgical results of pulmonary thromboendarterectomy for chronic pulmonary thromboembolism].
Chronic pulmonary thromboembolism may produce severe respiratory insufficiency and progressive pulmonary hypertension resulting in an intractable right ventricular failure. Although the pathogenesis of this syndrome has not been completely understood, medical treatment usually has little effect and only surgery can be a life saving remedy. Since 1986, seven patients with this syndrome have had pulmonary thromboendarterectomy at the First Department of Surgery, Chiba University Medical Center. Surgery is indicated mainly for patients who present with a mean pulmonary arterial pressure greater than 30 mmHg. All procedures were performed through a unilateral thoracotomy with a cardiopulmonary bypass on stand-by use. Two patients (#1 and #6) needed bilateral surgery which was carried out in two stages, in the remainder only a right side being operated on. When a major pulmonary artery was clamped, the systolic pulmonary arterial pressure rose to equalize or even exceeded that of systemic artery, however since no further hemodynamic deterioration was observed, cardiopulmonary bypass was not used in any case. Although the key feature of this procedure is commonly said to establish an exact cleavage plane between thrombus and arterial wall, it can be carried out without difficulty through thoracotomy approach. Grasping the thrombus, blunt dissection is further proceeded until the entire thrombotic material comes free as a cast of the vessels. All patients survived the operation without major complications including severe reperfusion pulmonary edema. Postoperative catheterization, performed one month following surgery, demonstrated a marked reduction of pulmonary arterial pressure with significantly reduced pulmonary arterial resistance (PAR).
Adult↗
A chronic contained rupture of an abdominal aortic aneurysm complicated with severe back pain.
Chronic contained rupture of an abdominal aortic aneurysm is an uncommon occurrence with the aneurysms usually small-to-moderate in size. Diagnosis may be difficult because patients present with both atypical and chronic symptoms. Pressure erosion of the lumbar spine is presumably a highly significant associated disorder, but an enhanced computed tomographic scan is the most reliable method for the correct diagnosis. We report on a 46-year-old man who developed severe back pain which was initially thought to result from spinal disease. Retrospective review of computed tomographic scans taken two years before admission revealed the beginning of the leakage of the aneurysm. Remarkably, the patient remained stable two years after the rupture.
Aorta, Abdominal↗
[Open aortic technique for DeBakey type II dissection].
Two patients with chronic dissecting aneurysm (DeBakey type II) underwent graft replacement using open aortic technique. During proximal anastomosis, the body temperature of the patients was lowered to 23 degrees C and open distal anastomosis was performed under circulatory arrest of the lower body using selective perfusion to the brain. Graft-to-aorta anastomosis was performed in distal ascending aorta (case 1) and in aortic arch (case 2), after the approximation of inner and outer layer of the false lumen. Two patients survived operations, and the postoperative courses and postoperative angiograms were satisfactory. Open aortic technique was considered to be applied not only to patients with arch aneurysm or arch dissection but also to patients with DeBakey type II or type I dissection.
Aged↗
[A case report of hypertrophic obstructive cardiomyopathy after the correction of atrioventricular septal defect].
A 47-year-old woman who had closure of an atrioventricular septal defect and repair of mitral cleft developed severe dyspnea on effort 6 years after surgery. An echocardiography showed asymmetric left ventricular hypertrophy and a left cardiac catheterization revealed marked intraventricular pressure gradient and moderately severe mitral regurgitation. The patient underwent mitral valve replacement through transseptal approach during empty beating heart. Symptomatic relief was obtained and marked reduction of intraventricular systolic pressure gradient was noted in postoperative cardiac catheterization about 40 days after the operation. Mitral valve replacement should be considered for the treatment of hypertrophic obstructive cardiomyopathy in severe cases with associated morphological abnormality of mitral leaflet or severe mitral regurgitation.
Cardiomyopathy, Hypertrophic↗
[A case of traumatic tricuspid regurgitation].
Tricuspid regurgitation due to nonpenetrating trauma occurred in a 60-year-old male patient who had received chest trauma in a motorcycle accident. He was admitted because of shortness of breath and palpitation on exertion. On admission physical examinations revealed pulsated and dilated jugular veins, hepatomegaly, and systolic murmur. The chest X-ray film showed an enlarged heart and electrocardiograms revealed complete right bundle branch block. Echocardiography demonstrated systolic prolapse of the tricuspid anterior leaflet into the right atrium. Right atrial v wave pressure was 20 mmHg. Tricuspid valve replacement with a Carpentier-Edwards 33 mm using super interpose method was performed successfully 13 years after the trauma. At operation, it was found that the chordae tendineae of the anterior leaflet was ruptured.
Chordae Tendineae↗
[Long-term results of the 19 mm low-profile bileaflet valve prosthesis in the small aortic anulus].
Between April, 1979, and November, 1986, 20 patients underwent aortic valve replacement (AVR) in the small aortic anulus with either 19 mm St. Jude Medical valve prosthesis or 19 mm Duro-Medics valve prosthesis, which are relatively new, low-profile bileaflet valve prostheses. There were two male and 18 female patients ranging from 35 to 69 years old (mean, 54.7 years). Average body surface area was 1.37 +/- 0.11 m2 (range 1.20 to 1.55 m2). One patient died of arrhythmia at 22 postoperative day. The 19 survivors have been followed up for as long as 61 months (mean, 31.2 months). There were two late complications, cerebral infarctions, and event free ratio was 0.85 at five years. All long-term survivors were in New York Heart Association Functional Class I (15 patients) and Class II (three patients). Preoperative and postoperative echocardiograms demonstrated significant decreases in mean left ventricular end-diastolic diameter (LVDd) (48.9 +/- 8.3 mm vs 42.2 +/- 5.7 mm; p less than 0.01) and in left ventricular end-systolic diameter (LVDs) (32.2 +/- 8.2 mm vs 25.7 +/- 4.9 mm; p less than 0.01). Mean left ventricular wall thickness was decreased to 24.5 +/- 3.7 mm from 25.8 +/- 6.4 mm. The average peak systolic gradient at rest with Doppler ultrasound was 26.0 +/- 9.3 mmHg (range nine to 36 mmHg). Though transprosthetic gradient did occur in patients who received 19 mm low-profile bileaflet valves in narrow aortic roots, progressive prosthetic stenosis was not observed and small aortic prostheses provide acceptable palliation for long-term results clinically.
Adult↗
[A new trial assessment of left ventricular function before and after aorto coronary bypass surgery--application of ejection vector (E.V.)].
In 34 patients underwent aorto coronary bypass, left ventriculography was analyzed and ejection indexes (E.F. 100, E.F. 50, E.V. = 2 X E.F. 50/E.F. 100) were calculated. There was not difference in ejection indexes of patients between who had been complicated with preoperative myocardial infarction and not. E.F. 100 was not changed postoperatively, otherwise, E.F. 50 and E.V. showed significant increase. In 15 patients whose quality of life remarkably was improved postoperative period, E.F. 100 was not changed but E.F. 50 and E.V. were significantly increased. Four patients could not perform exercise test to full dose because of intractable ischemic calf pain. In twelve patients, whose left ventricular function was improved at postoperative exercise test, their E.F. 100 was found to be unchanged, but in contrast, E.F. 50 and E.V. were increased significantly. In conclusion, we found that E.V. could become to be more sensitive indicator of left ventricular function compared with E.F. 100 before and after aorto coronary bypass surgery.
Adult↗
Early and late results of valvular surgery in the elderly.
From January, 1979 to June, 1986, 70 consecutive elderly patients (30 males and 40 females) age 65 years or older underwent open heart surgery for valvular heart diseases at Mitsui Memorial Hospital in Tokyo. Seventeen patients (24.3%) were septuagenarians. Porcine bioprosthesis (Carpentier-Edwards) was inserted for the mitral and the tricuspid position, and mechanical valve (Björk-Shiley or Duromedics) for the aortic position. Every patients received an anticoagulant (warfarin) postoperatively all through the follow-up period. The mean duration of follow-up was 43 months and the cumulative follow-up was 195 patient-years. Fifteen patients died in hospital after operation, giving an operative mortality rate of 21.4%. Cardiac death occurred in only 5 and the other 10 patients died of other causes such as sepsis, cerebrovascular accident, and agranulocytosis, showing that these patients were already in an advanced stage of cardiac failure. Late death occurred in 5 patients, but there was no clear-cut cardiac death documented. The actuarial survival rates at 3 years and 5 years were 71.6% and 65.9% for all patients, and 90.5% and 83.3% for early survivors. The functional and symptomatic improvement of 49 late survivors was remarkable. Thirty-eight patients (77.6%) are now NYHA class I and II. The incidence of thromboembolic and hemorrhagic episodes was fairly low--2% and 3% per patient year, respectively. Although the operative mortality is rather high in this study, it is clear that the surgical management of elderly patients with life-threatening valvular lesions results in substantial functional improvement and good prognosis in surviving patients.(ABSTRACT TRUNCATED AT 250 WORDS)
Actuarial Analysis↗
[Successful conservative treatment of chylorrhea in the anterior mediastinum after mitral valve replacement].
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Chylothorax↗
Immunoglobulin therapy in the West syndrome.
An examination was made of high dose non-treated immunoglobulin (NTIG) therapy at an early stage of the West syndrome (WS). Six patients with cryptogenic WS who suffered attacks ranging from 15 days to 6 months (mean 70 days) and 5 patients with symptomatic WS who suffered attacks ranging from 14 days to 4 months (mean 32 days), were administered NTIG 6-10 times intravenously at 100-200 mg/kg of body weight at intervals of 2 or 3 weeks. All patients with cryptogenic WS showed complete remission in accordance with normalized electroencephalogram (EEG) without following anticonvulsants medication. Of the 5 patients with symptomatic WS one patient showed cessation of clinical seizures in agreement with EEG improvement and 2 other patients revealed transient cessation of clinical seizures with recurrence. In cases of complete remission, the energy percentage of the power spectrum for each frequency band displayed a tendency toward gradual decrease of delta wave band in correspondence with the increase of theta wave band percentage after NTIG therapy, suggesting that high dose NTIG may be useful for early treatment of cryptogenic WS and for inhibiting brain deterioration owing to epileptic encephalopathy.
Anticonvulsants↗