Showing papers by "Boston Children's Hospital published in 1971"
TL;DR: A new palliative surgical procedure for interruption of the aortic arch was successfully performed in an infant, and may prove life-saving in the hypoplastic left heart syndrome, with the addition of atrial septal defect creation when left atrial decompression is indicated.
Abstract: A new palliative surgical procedure for interruption of the aortic arch was successfully performed in an infant. A Teflon graft was inserted from the main pulmonary artery to the descending thoracic aorta in order to bypass a stenotic ductus arteriosus, and both pulmonary artery branches were banded to reduce the pulmonary blood flow. This procedure may also prove life-saving in the hypoplastic left heart syndrome, with the addition of atrial septal defect creation when left atrial decompression is indicated. This syndrome was the commonest cause of death from congenital heart disease in the first month of life in our recent experience (31 percent), based on a consecutive autopsy series of 174 neonates with congenital heart disease. The anatomic findings in 10 autopsy cases of interrupted aortic arch are summarized. A new understanding of the morphogenesis of these anomalies is presented, based on correlation of embryologic and pathologic data. The literature on interruptions of the aortic arch is reviewed, and surgical experience with these malformations is considered in detail.
246 citations
TL;DR: In both normal and plexectomized animals, 24Na levels were found to be markedly greater in the gray matter surrounding the ventricles and in thegray matter bordering the subarachnoid space, suggesting that sodium exchanges in these two general areas of the brain may be linked to the formation of the cerebrospinal fluid.
Abstract: The production of cerebrospinal fluid and the transport of 24Na from the blood to the cerebrospinal fluid were studied simultaneously in normal and choroid plexectomized rhesus monkeys. Choroid plexectomy reduced the production of cerebrospinal fluid by an average of 33 to 40 percent and the rate of appearance of 24Na in the cerebrospinal fluid and its final concentration were proportionately reduced. In both normal and plexectomized animals, 24Na levels were found to be markedly greater in the gray matter surrounding the ventricles and in the gray matter bordering the subarachnoid space. That sodium exchanges in these two general areas of the brain may be linked to the formation of the cerebrospinal fluid is discussed here.
200 citations
TL;DR: This study collected more information on metabolic responses in children during exercise and found that children’s anaerobic capacity appears to be lower, at least to judge from data on blood lactate concentration after exhaustive exercise.
Abstract: Aerobic power is the same or greather in children expressed per kg body weight than in adults (1). On the other hand, children’s anaerobic capacity appears to be lower, at least to judge from data on blood lactate concentration after exhaustive exercise. The reason for this has been the subject of discussion but no satisfactory explanation has yet been produced. This study was performed in order to study this question further and to collect more information on metabolic responses in children during exercise. A more detailed presentation of the study can be found elsewhere (3).
141 citations
TL;DR: In the last decade, this work has speculated upon the functional implications of epileptic outbursts of laughing, in terms of the possible anatomic substrate and interconnections subserving this fairly unique human expression of emotion.
Abstract: SUMMARY
In the last decade we have encoutered 10 cases in whom we considered laughter to represent either the only or one of several peripheral expressions of an abnormal cerebral electrical discharge. We have distinguished between pathological laughter that may occur in the course of diffuse neurological diseases or as an expression of a dementing process, from that which can be properly called epileptic in nature. We have also distinguished between ictal and postictal laughter. Finally, we have speculated upon the functional implications of epileptic outbursts of laughing, in terms of the possible anatomic substrate and interconnections subserving this fairly unique human expression of emotion, and have pointed out differences in clinical and EEG characteristics that seem to correlate with the different location of the lesions responsible for the discharges.
RESUME
Pendant la derniere decade, nous avons observe 10 cas dans lesquels le rire representait soit la seule, soit une des expressions peripheriques 'une decharge electrique cerebrale pathologique.
Nous avons distingue le rire pathologique qui se voit au cours 'affections neurologiques diffuses ou comme expression 'un processus dementiel, du rire epileptique proprement dit.
Nous avons fait egalement la difference entre rire epileptique critique et postcritique. Enfin, nous avons discute les implications fonctionnelles des bouffees epileptiques de rire, en rapport avec le substratum anatomique possible et les connections qui concernet cette expression specifiquement humaine de 'emotion et nous avons mis en evidence des differences dans les caracteristiques cliniques et EEG qui semblent en relation avec les differentes localisations des lesions responsables des decharges.
141 citations
TL;DR: The prognosis in neonates presenting with Ebstein's anomaly is relatively poor in the presence of complicating cardiac lesions, more marked cyanosis and, to a lesser extent, extreme cardiomegaly.
Abstract: The clinical features, cardiac catheterization data and natural history of 55 patients with Ebstein's anomaly are presented; 18 had a significant associated cardiac anomaly. The clinical features are usually clear-cut. Cardiac catheterization and angiography demonstrate downward displacement of the tricuspid valve, a large right atrium, no important elevation of the right ventricular pressures, decreased pulmonary blood flow and right to left shunt through the foramen ovale. Right ventricular outflow obstruction may be masked by the low level of pulmonary blood flow and resistance. Intracardiac electrocardiogram is usually diagnostic. The prognosis in these children is relatively poor in the presence of (1) complicating cardiac lesions, (2) more marked cyanosis and (3), to a lesser extent, extreme cardiomegaly. The appearance of congestive heart failure foretells early death ( In neonates presenting with Ebstein's anomaly the incidence of important associated cardiac anomalies is higher, and the clinical picture more variable because of the physiologic adjustments of birth. Cyanosis may be severe only to improve later. The electrocardiogram may show atypical features and exhibit the classic pattern within weeks. Radiographic cardiomegaly may be dramatically out of proportion to the apparent well-being of the infant. Surprisingly gross cardiomegaly is compatible with survival.
140 citations
TL;DR: These studies show that as early as 8–12 weeks after birth, focal areas can be identified in the grossly normal pig aorta which show an increased uptake of cholesterol.
104 citations
TL;DR: This analysis used electrophoresis of red blood cell haemolysates on cellulose acetate to identify electrophoretic phenotypes of euros and red kangaroo populations, which were characterized by a single slow moving band and those of wallaroos had a single fast moving band.
Abstract: THE production of glucose-6-phosphate dehydrogenase (EC 11149, G6PD) in human1, horse and donkey2 and brown and blue hare3 cells is governed by genes carried by the X chromosome. Two electrophoretic forms of G6PD have been found in wallaroos and euros (Macropus robustus Gould) and one in red kangaroos (Macropus rufus (Desm.)); members of the marsupial family Macropodidae (kangaroos). This analysis used electrophoresis of red blood cell haemolysates on cellulose acetate4. No polymorphic populations were found5 but electrophoretic phenotypes of euros (Macropus robustus erubescens Sclater) were characterized by a single slow moving band (G6PD-S) while those of wallaroos (Macropus r. robustus Gould) had a single fast moving band (G6PD-F). Red kangaroo populations were uniformly G6PD-S.
97 citations
TL;DR: A low concentration of platelet serotonin has been demonstrated in 88 of 25 children who have symptoms of hyperactivity without classical evidence of neurologic dysfunction and in 2 children, during residence in the research ward of the hospital, the serotonin concentration rose toward the normal range and the children's hyperactivity lessened.
95 citations
TL;DR: Ten of 12 mothers who had deprived their infants were found to have a characterdisorder and the basis for the diagnosis and the implications for intervention strategies are discussed.
86 citations
TL;DR: Spectra obtained from mitochondrial granules of chondrocytes in situ confirm the suspected presence of calcium and phosphorus in living tissue.
Abstract: A scanning electron microscope was converted to an electron microprobe with high spatial resolution by the addition of a transmitted electron detector and a solid-state x-ray detector. Spectra obtained from mitochondrial granules of chondrocytes in situ confirm the suspected presence of calcium and phosphorus. Contamination during analysis can lead to false indications of silicon in living tissue.
82 citations
TL;DR: Exogenous phospholipase A activity in the microsomal membranes of the myocardium may have relevance to in vitro studies of Ca2+ permeability and Ca2-dependent ATPase changes previously noted during snake venom phospholIPase A treatment of muscle membranes.
TL;DR: Six patients with partial deletion of the long arm of chromosome No. 18 are described, with characteristic features of this syndrome including typical facies with reduced prominence of the midface region, prominent antihelix, “carp-shaped” mouth, nystagmus, and funduscopic abnormalities.
TL;DR: It is concluded that there is a correlation between a diagnosis of infantile autism by the E-2 score and enhanced release of radioactive 5-HT from 5- HT-loaded blood platelets in vitro.
Abstract: Experiments were conducted to determine the possibility of predicting a diagnosis of autism on the basis of an abnormally high release of14C-5-hydroxy-tryptamine from 5-HT loaded blood platelets of children. Such increased 5-HT release was previously reported by the authors for blood platelets of children diagnosed as autistic according to the Rimland E-2 score. The platelets of 10 psychotic children (including a number of subjects diagnosed as autistic by the E-2 score) were examined without knowledge of the diagnosis, in order to determine whether the biochemical results correlated with the E-2 score. On the basis of experimental data it was possible to predict that six children were autistic and four were nonautistic psychotics. According to the E-2 score, seven children were autistic and three nonautistic psychotics. The authors conclude that there is a correlation between a diagnosis of infantile autism by the E-2 score and enhanced release of radioactive 5-HT from 5-HT-loaded blood plateletsin vitro.
TL;DR: Observations so far have been very limited, but it could well be that transplants in cases of esophageal atresia, even with major gaps, could become superfluous.
TL;DR: This study suggests that reliable guides to myocardial contractility can readily be obtained from high fidelity pressure recordings alone.
Abstract: The index V pm (the actual or physiologic maximal shortening velocity of the contractile element) has been obtained from the analysis of left ventricular pressure recordings and then applied to the assessment of myocardial contractility in man Pressures during the isovolumic period of ventricular systole were recorded during routine catheterization of 46 patients with a variety of cardiac lesions The quantity V pm , that is (dp/dt/kp) max , was compared with the hemodynamic evaluation of each patient based on the left ventricular end-diastolic pressure, ejection fraction and left ventricular end-diastolic volume V pm was selected for this study since it appeared to be largely independent of load and does not require biplane angiocardiography and extrapolation analyses Preliminary results suggest that a value of V pm greater than 16 sec −1 indicates normal myocardial function; lower values indicate abnormal function In several instances this index was not in agreement with the hemodynamic measures, but in most cases the clinical assessment supported the conclusion drawn from V pm This study suggests that reliable guides to myocardial contractility can readily be obtained from high fidelity pressure recordings alone
TL;DR: A specific cause for the diarrhea in the 3 infants who recovered could not be demonstrated, but each had an adverse reaction temporally associated with the change from Nutramigen to Enfamil or whole milk feedings, thus suggesting the presence of milk protein allergy.
TL;DR: Severe pulmonary vascular obstruction in the absence of obvious pulmonary parenchymal pathology was found in 5 full-term neonates and was characterized clinically by cyanosis and tachypnea and the possible causes of the persistence are discussed.
TL;DR: A comparison of pigmented and unpigmented tissues revealed a quantitative difference in the distribution of the basal dendritic cells, which did not vary with the degree of pigmentation.
TL;DR: Of the 20 babies with adequate follow-up, approximately ⅓ recovered by 6 months with minimal deficit, over ½ recovered by 1 year with moderate residua to include persistent weakness, delay in bone growth, dislocation, and peculiar posturing of the arm; and the other three infants showed significant handicaps.
Abstract: Experience in the diagnosis, treatment, and evaluation of residua of brachial plexus paralysis in 25 infants is presented. Associated defects substantiate the traumatic nature of the deliveries. The electromyogram proved a valuable tool in the exact delineation of the pathology and in determination of prognosis. Early optimum treatment prevented atrophy and contractures. Of the 20 babies with adequate follow-up, approximately ⅓ recovered by 6 months with minimal deficit, over ½ recovered by 1 year with moderate residua to include persistent weakness, delay in bone growth, dislocation, and peculiar posturing of the arm; and the other three infants showed significant handicaps.
TL;DR: Angiographic demonstration of bilateral brachial and coronary artery aneurysms associated with rhinitis, conjunctivitis, erythematous macular skin rash, fever, anemia, leukocytosis, and congestive heart failure supported the clinical diagnosis of periarteritis in an infant.
TL;DR: Det. 562 is a permanent heteroploids human cell line that is similar to HeLa and other heteroploid cell lines in morphology, growth, and virus susceptibility, but differs from similar permanent human cell Lines in having G-6-PD type B isozyme.
Abstract: SummaryDet. 562 is a permanent heteroploid human cell line that is similar to HeLa and other heteroploid cell lines in morphology, growth, and virus susceptibility.It differs from similar permanent human cell lines in having G-6-PD type B isozyme. The implications of this finding are discussed. The importance of cell line monitoring is stressed.
TL;DR: Fractionation of specific rubella antibodies is important not only in all cases where a serum sample was taken too late and a rise of antibody titre missed, but also in cases with significant antibody rise to distinguish a secondary immune response after rubella reinfection.
TL;DR: Evidence is presented which suggests that although protein sulfhydryl groups may be involved in the dissociation-reassociation phenomenon, hydrophobic bonding is probably a more critical factor.
TL;DR: The clinical course and the results of laboratory investigations of 5 children withunilateral hyperlucent lung syndrome are presented and it is suggested that the pulmonary artery hypoplasia, in part, results from a failure of growth on the affected side secondary to the bronchopulmonary pathology.
TL;DR: In this paper, the renal action of parathyroid hormone (PTH) is known to be mediated via 3′, 5′-adenosine monophosphate (cyclic AMP), which is used to determine proximal tubular maturation.
Abstract: Since the renal action of parathyroid hormone (PTH) is known to be mediated via 3′, 5′-adenosine monophosphate (cyclic AMP), urinary cyclic AMP studies were used to determine proximal tubular maturation. Ten formula fed full-term male infants showed a 30 to 60 fold increase in phosphate clearance and excretion with a 3–4 fold increase in urinary cyclic AMP comparing their first and third day 24-hour urines. This 3–4 fold increase in cyclic AMP could reflect increasing pTH renal responsiveness and/or increasing secretion of PTH. One and 3 day old infants and adults were given a one hour PTH infusion (5 μ/kg/hr) measuring urinary cyclic AMP in time periods before and after the infusions. Peak increases in responses from baseline of cyclic AMP were 1.64 ± 0.34, 7.15 ± 1.15 and 36.30 ± 0.73 μmoles/gm creatinine mean ± range on first day, third day and adults respectively with similar relationships of increasing phosphate excretion and decreasing % TRP. Thus, the development of newborn renal responsiveness to parathyroid hormone is on the cellular cyclic AMP level suggesting increasing maturation of the enzyme adenyl cyclase which forms cyclic AMP from ATP.
TL;DR: Cholics acid was found to be a metabolic end product, whereas 4 to 7 per cent of chenodeoxycholic acid was transformed prior to urinary excretion, which led to reduced excretion in urine and feces.
TL;DR: Cholesteryl ester exchange between erythrocytes and lipoproteins was negligible and maximum exchange, in vitro, occurred between 6 and 8 h without equilibration of [ 3 H] cholesterol.