摘要:

Increased incidence of malignant disorders is noted in patients with both primary and acquired immunodeficiency diseases. The pathogenetic mechanism(s) for these disorders remain unclear. Defective immunosurveillance of these patients, however, is mainly postulated to be responsible for the increased risk of these malignant disorders. Of the malignant disorders, Epstein‐Barr virus (EBV)‐induced lymphoproliferative disorders (LPD) have been increasingly reported, possibly due to improved therapeutic management techniques such as bone marrow transplantation, which results in prolonged survival periods for the primary immunodeficiency; the dramatic development of immunosuppressive treatments for transplant recipients; and the growing numbers of acquired immunodeficiency syndrome (AIDS) patients.This review focuses on the primary immunodeficiency diseases and EBV‐induced LPD, and discusses pathogenetic mechanism(s) for the increased incidence of these malignant diso

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00976.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

Denopamine is an orally active β1agonist whose cardiovascular action in the newborn is unknown. We evaluated its circulatory effects during normoxia in newborn piglets less than 7 days of age. The piglets were acutely instrumented under general anesthesia with an electromagnetic flow probe around the main pulmonary artery and catheters in the main pulmonary artery, aorta, left ventricle, and the right and left atria. A Millar high‐fidelity catheter was used to measure left ventricular dp/dt. The ductus arteriosus was ligated. Denopamine was administered in the right atrium as a continuous infusion of 2, 4, and 8μg/kg per min for 10 min each. Although cardiac index, heart rate and left ventricular dp/dt increased dose‐dependently by 46.0 ± 18.2%, 87.1 ± 34.9% and 159.9 ± 42.4%, respectively, stroke index was not significantly altered. Unlike pulmonary artery pressure (which increased dose‐dependently), aortic pressure increased with 2 and 4μg/kg per min denopamine, respectively, it fell with 8μg/kg per min denopamine. Similarly, the systemic vascular resistance decreased with the high dose (8 μg/kg per min). There was no significant change in pulmonary vascular resistance. Denopamine is potently inotropic in the adult. However, its circulatory effect in the neonate is dependent on its chronotropic action. Furthermore, denopamine is a systemic vasodilator at high doses in the neonata

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00977.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

Controversy exists as to whether high frequency oscillation (HFO) increases the risk of intraventricular hemorrhage (IVH) compared with conventional mechanical ventilation (CMV). We compared the risk of intracranial hemorrhage after phenylephrine‐induced hypertension, combined with a hypovolemic, hypotensive insult followed by rapid volume replacement in two groups of newborn beagle puppies (one group on a piston pump HFO and the other on CMV).A total of 12 beagle puppies (6 on HFO and 6 on CMV) survived the protocol. Arterial blood gas analysis and arterial blood pressures through the study were of the same magnitude in both groups. The length of time for which the puppies remained hypertensive and hypotensive also did not vary significantly between the groups.Intraventricular hemorrhages were observed in two of six CMV puppies and two of six HFO puppies. One animal in each group had a white matter hemorrhage. Subarachnoid hemorrhages were seen in 4 animals on CMV and 3 on HFO.This study indicated that HFO does not increase the risk of any kind of intracranial hemorrhage, including IVH, in beagle puppie

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00978.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

Severity of viremia and neutralizing antibody responses were compared between nine young infants ( 10 months) with measles and 18 infants and children ( 11 months) with ordinary measles. Peripheral blood mononuclear cell (PBMC)‐associated viremia was detected between the first day of elevation of fever (day 0) and day 5 of the disease in the former group, whereas PBMC‐associated and cell‐free viremia were detected between day 0 and day 14 in the latter group. The number of infected PBMC during the first 7 days of the disease was 3.22 ± 1.07 (log10, mean ± s.d.) per 10 million PBMC in the former group, which was significantly smaller (P= 0.02) than that of the latter group (4.21 ± 1.18). The former group reached the maximum level of antibody earlier than the latt

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00979.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

We examined neuroradiological computerized tomography (CT) findings and the clinical course of four Japanese children with glutaric aciduria type I (GA1) whose enzyme activity of glutaryl‐CoA dehydrogenase was undetectable. Brain CT in all cases examined showed low density white matter, fluid collection in bilateral frontotemporal regions (particularly surrounding the Sylvian fissures), enlargement of the lateral ventricles and slight atrophy of the basal ganglia. Although these findings seemed to be characteristic for GA1, they were unlikely to be more extended, at least over 2 years after infancy. The low density white matter was observed more evidently in the neonatal or early infantile periods than in later periods. The degree of enlargement of fissures in bilateral frontotemporal regions about the Sylvian fissures appeared to correlate with the severity of symptoms such as dystonia or choreo‐athetosis. Magnetic resonance images (MRI) in one case showed bilateral linear‐shaped low intensity in areas of the external capsules and putamen on a Tl‐weighted image. These CT and MRI findings, as well as clinical symptoms such as choreoathetosis or dystonia, may suggest that metabolic abnormalities in GA1, such as glutaconate, are toxic to the extrapyramidal tract system in the central nervous system, and that the clinical symptoms of the patients are attributable to atrophy of basal ganglia. Brain CT may be useful in diagnosis and evaluation of the clinical course of GA1 p

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00980.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

Mentally normal 6–9 year old children with or without convulsive disorders were examined using the procedures of the five soft signs (SS) selected from conventional soft neurological signs and Berges' gesture imitation tasks. In comparisons of SS between the 60 children with convulsive disorders (CD group) and the 38 without (N group), the average number of SS detected in each case and the incidence ratio of the two signs ‐ partial imitation (PI) and partial sensory agnosia (PSA) ‐ were higher in the CD group than the N group. Examinations of relationships between age, IQ, error index (EI; an index taken from the Continuous Performance Test) and SS, and of correlations among the SS, found two kinds of SS: age‐related and El‐related signs. The former included right‐left confusion, clumsiness and PI, which correlated with each other, while the latter included unstableness of lateral gazing and PSA, which did not correlate with each other. Even though several factors belonging to characteristics of convulsive disorders were evaluated concerning the presence or absence of PI and PSA, there was only one relationship: PSA was more common in children with febrile convulsions than in those with epilepsy. We speculated that PSA reflected an immaturity of the brain in children with febrile

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00981.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

Oscillatory variations of cutaneous blood flow were studied using laser Doppler flowmetry in 20 newborns. Simultaneous monitoring of cardiorespiratory state was also performed. The frequency and amplitude of oscillatory curve varied, even on identical skin regions in each infant. When their respiration and heart rate were irregular, the frequencies of oscillatory curves were significantly higher and with a larger amplitude than when cardiorespiratory function was regular. The frequencies of oscillatory curves at two symmetrical points of an individiual were not different, but a significant difference in the oscillatory cycle was recognized at two asymmetrical points. Distributions of peak‐to‐peak time periods of oscillatory curves were studied in nine healthy and four sick newborns. Seven of nine healthy newborns had more than two peaks in their distributions, while only one peak with a narrowly‐spread distribution was observed in the four sick newborns with almost exclusively periodic breathing throughout monitoring.In conclusion, we speculate that cardiorespiratory state and some neurogenic control affects the variations observed. In addition, the narrowly‐spread distribution of each time period appeared to be an important indicator of mo

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00982.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

Percutaneous balloon aortic valvuloplasty (BAV) was performed in 14 patients, including one critically ill infant with congenital valvular aortic stenosis (AS). BAV was effective in 13 patients (except the infant). The peak systolic pressure gradient between the left ventricle (LV) and the ascending aorta decreased from 76.6 ± 21.6 to 29.5 ± 15.3 mmHg (P<0.001). Follow‐up cardiac catheterization was performed for eight patients between 1 and 3 years (1.6 ± 1.1 years) after BAV. Restenosis was found in only one patient, and the efficacy of BAV continued significantly. Aortic regurgitation developed or increased in severity in 5 of 13 children immediately after BAV. Any other severe complication was not observed.Dilatation by BAV was not sufficient for the infant with critical AS, and acute myocardial infarction (AMI) in the lateral wall of the LV occurred during the BAV procedure. The infant died 3 days after the procedure due to AMI.It was concluded that the retrograde double balloon technique was superior to the retrograde single balloon technique. In two cases, the single balloon technique was ineffective because it was impossible to fix the balloon at the aortic annulus. However, the double balloon technique was effective in every patient.BAV is effective for AS in children, and an optional repeat trial may enable BAV to be the first choice for AS. Although BAV may be effective for neonates and infants with critical AS as an emergency treatment, much attention must be paid during the proce

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00983.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

Bronchial reactivity to inhaled methacholine (MCH) was evaluated in 32 patients with ‘idiopathic’ chest pain. Each pain was recurrent in nature. The incidence of cases with a provocative concentration causing a 20% fall in the forced expiratory volume in 1 sec (PC20) of 10 mg/ml or less was 62.5% (20 cases), while it was only 11.1% (three cases) in 27 healthy controls. Seventeen patients had no personal history of allergic diseases, elevated serum lgE level or positive house dust mite‐specific IgE antibody. Among these 17, eight (47.1%) had a PC20of 10 mg/ml or less, the incidence of which was also higher than that of the healthy controls. During the challenge, eight patients complained of chest pain similar to that experienced before. The present results indicate that bronchial hyper‐reactivity is an important cause of ‘idiopathic’ chest pain. Patients with unexplained chest pain should be considered for inhalatio

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00984.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

A measles outbreak occurred in the Chikuhou district of Fukuoka, Japan from May to October 1990, during which 71 patients were cared for at the Itoda Public Hospital. Hospital records revealed a large outbreak in the adjacent town of Kanada. In order to characterize the outbreak, questionnaires were sent to all preschool‐age children in Itoda (73% effective response) and in Kanada (76% effective response) requesting information about their vaccination and/or history of measles. The number of patients was 22 (4%) in Itoda and 63 (14%) in Kanada, most of these being preschoolers, while the vaccination rate was 61% and 44%, respectively. The herd immunity levels in age‐specific groups were compared between the two towns. Before the epidemic, the immunity level of 1 year old children in Kanada, who showed the higher attack rate, was lower (18%) than that in Itoda (39%), while after the epidemic it rose above 60% in both towns. When we studied the correlation between the attack rate and the vaccination rate, or the number of children susceptible to measles (susceptibility rate) in each preschool, the attack rate correlated negatively with the vaccination rate (correlation coefficient [CC] = ‐ 0.818;P<0.01), and positively with the susceptibility rate (CC 0.860;P<0.01). The regressed equation on the correlation indicated that the immunity level should be more than 70% to keep the attack rate under 5% in preschoois. After the epidemic, the immunity levels of all preschoolers reached above 70%. Early vaccination should be given to at least 70% of young preschoolers in order to prevent a large outbreak of measles under the present vaccination program in

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00985.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY