Showing papers by "Boston Children's Hospital published in 1980"
TL;DR: The hypothesis that some reading impairments are related to low-level auditory perceptual dysfunction that affects the ability to learn to use phonics skills adequately is discussed.
1,469 citations
TL;DR: This first demonstration of angiogenesis in vitro shows that all the information necessary to develop an entire capillary network in vitro is expressed by one cell type, suggests a mechanism for lumen formation, and offers a possibility of distinguishing between direct and indirectAngiogenesis factors.
Abstract: Cloned capillary endothelial cells, cultured in tumour-conditioned medium, form capillary tubes. By light and electron microscopy these tubes resemble capillaries in vivo. This first demonstration of angiogenesis in vitro: (1) shows that all the information necessary to develop an entire capillary network in vitro is expressed by one cell type; (2) suggests a mechanism for lumen formation; and (3) offers a possibility of distinguishing between direct and indirect angiogenesis factors.
1,065 citations
583 citations
TL;DR: The clinicopathologic features of 118 granular cell tumors encountered at two affiliated hospitals were reviewed and it was found that Schwann cell origin is favored in most cases.
Abstract: The clinicopathologic features of 118 granular cell tumors (GCT) encountered at two affiliated hospitals were reviewed. A total of 110 patients were affected over this 32-year period of study (71 men, 39 women), and in 5% GCT were multiple. Patients ranged in age from 16 to 58 years (average 32 years) and were symptomatic for an average duration of 11 months prior to diagnosis. There was a greater than expected frequency of GCT among black patients (29%). Although tongue was the single most common anatomic site involved, relatively more GCT (44%) occurred in skin or subcutaneous tissue. Less common locations were breast parenchyma (10 cases), rectal mucosa and anus (6), vulva (4), esophagus and larynx (2 cases each). The correct preoperative diagnosis of this protean tumor was made in only three patients. GCT were surgically treated with the average diameter of resected tumor being 1.2 cm (range 0.2--3.5 cm). Pseudoepitheliomatous hyperplasia was noted in 11 tumors and in one vulvar GCT there was overlying in situ squamous cell carcinoma. Tumors were incompletely excised in 24 of 56 patients having adequate followup; only five of these 24 patients experienced a local recurrence of tumor. Malignant behavior was not observed. Results of histochemical and ultrastructural study are briefly discussed. The precise histogenesis of GCT is uncertain but Schwann cell origin is favored in most cases.
526 citations
TL;DR: Six infants with a neonatally lethal malformation syndrome of hypothalamic hamartoblastoma, postaxial polydactyly, and imperforate anus are reported on, postulated that this is a previously apparently unreported syndrome of presently unknown cause.
Abstract: We report on six infants with a neonatally lethal malformation syndrome of hypothalamic hamartoblastoma, postaxial polydactyly, and imperforate anus Some, but not all, patients had laryngeal cleft, abnormal lung lobulation, renal agenesis and/or renal dysplasia, short 4th metacarpals, nail dysplasia, multiple buccal frenula, hypoadrenalism, microphallus, congential heart defect, and intrauterine growth retardation The infants also had hypopituitarism and hypoadrenalism
All were sporadic cases, parents were not consanguineous, chromosomes were apparently normal Family histories were unremarkable There was insecticide and/or herbicide exposure in several of the cases, but no exposures were common to all 6 mothers Five of the patients were born within an 8-month period, but all in different geographic locations It is postulated that this is a previously apparently unreported syndrome of presently unknown cause
309 citations
TL;DR: Two pat ients with cortical hyperost0sis which occurred following longterm adminis t ra t ion of PGE~ in low doses are described.
275 citations
TL;DR: Electroencephalographic and evoked potential data were recorded during behavioral testing from 8 dyslexic and 10 normal boys aged 9 to 11 years, suggesting relative cortical inactivity in that group.
Abstract: Electroencephalographic (EEG) and evoked potential data were recorded during behavioral testing from 8 dyslexic and 10 normal boys aged 9 to 11 years. Topographic mapping of their brain electrical activity revealed four discrete regions of difference between the two groups involving both hemispheres, left more than right. Aberrant dyslexic physiology was not restricted to a single locus but was found in much of the cortical region ordinarily involved in reading and speech. Prominent group differences were observed in the bifrontal area in addition to the more expected left temporal and left posterior quadrant regions. Although activation tests produced more prominent group difference, dyslexics differed from normal subjects at rest as well. EEG alpha activity was increased for the dyslexics, suggesting relative cortical inactivity in that group.
257 citations
TL;DR: The 25 affected males with macro-orchidism and Xq27 fra had some minor clinical features in common: there was an increase in birth weight, high forehead, prognathism, pale irides, big ears, and an increased head circumference in infancy and childhood which did not persist into adult life.
235 citations
TL;DR: The shiverer mouse is unique in showing a striking alteration in myelin protein composition that does not significantly affect the gross morphology and lamellar organisation of the myelin sheath, and this results question the proposed role of basic proteins15–19 in Myelin as ‘structural cement’.
Abstract: The myelin sheath is a multilamellar membrane system which surrounds axons in vertebrates and provides the electrical insulation necessary for saltatory nerve impulse conduction. Myelin forms from its cell of origin as a flattened, membrane-bound cytoplasmic process which wraps spirally around the axon; a periodic compact array of membrane pairs is produced from the wrappings as the cytoplasmic contents are extruded, and the external surfaces of membranes become apposed1,2. Neurological mutant mice which show myelin abnormalities are useful models for examining the formation, stability and breakdown of myelin. For example, the shiverer mouse carries an autosomal recessive mutation3 (shi)4 that results in severe myelin deficiency in the central nervous system (CNS)5,6, apparently due to a defect in myelin formation5,6. The small amount of myelin that does form in the CNS is generally not compacted at its cytoplasmic surfaces6, possibly due to the low level of basic protein in shiverer CNS tissue7. In the peripheral nervous system (PNS), in contrast, amounts of compact myelin seem to be normal6. The coarse tremor and convulsions that begin at about 2 weeks of age in the shiverer are presumably due to the severe CNS deficiency of myelin, as similar neurological signs are shown by other mutants with reduced CNS myelin8. Most studies on such mutants have concentrated on those regions of the nervous system which are grossly deficient in myelin5–10. In the other regions myelin seems by light microscopy to be normal. At the ultrastructural and molecular level, however, this myelin sometimes shows abnormalities11–14, and this has prompted us to examine intensively such myelin in several neurological mutants. For this we have used X-ray diffraction, electron microscopy and SDS-polyacrylamide gel electrophoresis (SDS-PAGE). We report here that, of the mutants we have examined so far, the shiverer mouse is unique in showing a striking alteration in myelin protein composition that does not significantly affect the gross morphology and lamellar organisation of the myelin sheath. Our results thus question the proposed role of basic proteins15–19 in myelin as ‘structural cement’.
226 citations
222 citations
TL;DR: A 5-year-old boy, excreting large amounts of 2-hydroxyglutaric acid in the urine, found to have thel-configuration, as analysed by capillary gas chromatography of theO-acetylated di-(-)-2-butyl ester derivative.
Abstract: A 5-year-old boy, excreting large amounts of 2-hydroxyglutaric acid in the urine (3.3–7.6 mmol/l), is described. The patient presented with psychomotor retardation and dystrophy. His skeletal age was delayed. The EEG was not well differentiated; it resembled that observed in 2-year-old children. There was a severe anaemia, which reacted well to iron supplements. The 2-hydroxyglutaric acid was found to have thel-configuration, as analysed by capillary gas chromatography of theO-acetylated di-(-)-2-butyl ester derivative. The relation ofl-2-hydroxyglutarate excretion to known metabolic pathways is discussed.
TL;DR: Treatment patterns revealed that surgery remains the most common treatment modality, and review of patterns of survival suggested that children in the other nonwhites category with unilateral disease had poorest survival rates.
Abstract: • Data from the population-based Surveillance, Epidemiology, and End Results Program of the National Cancer Institute were used to calculate the incidence of retinoblastoma for the years 1974 through 1976. Each year 3.58 cases occurred for each million children under the age of 15 years. Incidence was markedly age related, with over 90% of the cases being diagnosed before the age of 5 years. Although no difference in incidence was found for whites and blacks, other nonwhites had rates greater than four times those of whites. Twenty percent of patients had bilateral disease. Treatment patterns revealed that surgery remains the most common treatment modality. Review of patterns of survival suggested that children in the other nonwhite category with unilateral disease had poorest survival rates.
TL;DR: It is proposed that hydroxypyridinium residues are formed by spontaneous interaction of two residues of hydroxylysino-5-ketonorleucine, which provides a novel mechanism for lateral crosslinking within and between fibrils which may account for some of the unique physical properties of hard tissue collagens.
TL;DR: Compensatory renal growth does not involve the formation of new nephrons in the postnatal kidney of the rat and the potentiation of compensatory changes after neph rectomy at an early age is largely dependent on the developmental stage of the kidney at the time of nephrectomy.
TL;DR: Genetic types of plasminogen were determined from a donor and a recipient before and after hepatic homotransplantation and demonstrated that the liver is the principal site of synthesis of human plAsminogen.
Abstract: Genetic types of plasminogen were determined from a donor and a recipient before and after hepatic homotransplantation. Examination of the plasminogen types demonstrated that the liver is the principal site of synthesis of human plasminogen.
TL;DR: Damage to the sinus node remains the most common cause of arrhythmias after the Mustard operation and delayed atrial conduction may predispose to atrial muscle reentrant tachycardia.
Abstract: To determine the mechanisms of the cardiac arrhythmias frequently seen after the Mustard operation for transposition of the great arteries, intracardiac electrophysiologic studies were performed in 52 children 1 to 8 years after the Mustard operation. Sinus nodal automaticity as judged from the response to rapid atrial pacing was abnormal in 28 of the 52 children. Sinoatrial conduction (conduction of the sinus impulse to the atrium) was found to be abnormal in three of nine patients studied with the atrial extrastimulus method. Conduction of the sinus impulse from the high right atrium to the atrioventricular (A-V) node was abnormally delayed in only 2 of 41 subjects. The low lateral wall of the right atrium was depolarlzed late in 3 of 11 subjects (including the preceding 2). Two subjects showed delayed A-V nodal conduction and one delayed His-Purkinje conduction. The mechanism of supraventricular tachycardia induced in the laboratory was determined to be sinoatrial nodal reentry in four subjects and atrial muscle reentry in four. Two of the four with atrial muscle reentry had prolonged high right atrium to low lateral right atrium intervals during sinus rhythm. Thus, damage to the sinus node remains the most common cause of arrhythmias after the Mustard operation. In addition, delayed atrial conduction may predispose to atrial muscle reentrant tachycardia.
TL;DR: A clear age development in capacity for sustained attention (vigilance) and in motor inhibition between 4 and 6 years of age, in normal children is demonstrated.
Abstract: SUMMARY
A normative study of 230 children from Sydney pre-schools and primary schools (aged 3–7 years) was carried out using a Continuous Performance Test of Vigilance (CPT), the Draw-a-Line Slowly test (DALS) for motor inhibition, and the Sprague Ballistographic Chair for motility. The study demonstrated a clear age development in capacity for sustained attention (vigilance) and in motor inhibition between 4 and 6 years of age, in normal children. Correlations between components of the CPT, Mean Reaction Time (MRT), and the DALS test were shown suggesting a need for further multivariate studies. Sex differences were not found for the CPT, or DALS tests, but were shown for Chair Score and for motor components of the DALS test (DALO and DALF), for which boys scored higher motility scores and faster DALO and DALF scores.
TL;DR: Results indicate that the major factor in determining the frequency of SCEs is the concentration of BUdR in the medium, and that mutagenesis can be suppressed by deoxycytidine (dC) without changing the amount of BUDR in DNA.
Abstract: The halogenated thymidine (dT) analogue, 5-bromodeoxy-uridine (BUdR), has a variety of effects on mammalian cells, including toxicity, suppression of differentiation, and mutagenesis. Although it is generally assumed that the effects of BUdR are due primarily to its presence in DNA, results from our laboratory have raised doubts about such assumptions1–4. We have shown, for example, that BUdR mutagenesis in mammalian cells is determined by the concentration of BUdR in the medium rather than in DNA3, and that mutagenesis can be suppressed by deoxycytidine (dC) without changing the amount of BUdR in DNA4. BUdR has also been shown to induce sister chromatid exchanges (SCEs) in mammalian cells5–7. Initial results suggested that the relationship between BUdR and SCEs might not be explained by a single factor5, and various correlations between BUdR and SCEs have been proposed8,9. However, the results to date have been inconclusive, because the experiments did not resolve as independent variables the concentration of BUdR in the medium and the amount of BUdR incorporated into nuclear DNA. We have now carried out experiments to resolve these two factors; the results indicate that the major factor in determining the frequency of SCEs is the concentration of BUdR in the medium.
TL;DR: In this article, the injured finger was immersed in physiologically normal saline for approximately 2/3 of its length, and the probe was moved across the surface of the wound to measure the currents recorded a remarkable similarity to those obtained by Borgens et al. (1977) on salamanders.
Abstract: Measurements were made on 10 children who had finger tip amputations. They were made at 1 to 7 day intervals until 2 successive zero readings were obtained. The injured finger was immersed in physiologically normal saline for approximately 2/3 of its length, and the probe was moved across the surface of the wound. The currents recorded a remarkable similarity to those obtained by Borgens et al. (1977) on salamanders, rising to a peak average current density of 22 mu A cm-2 after an average of 8 days.
TL;DR: Gray-scale ultrasound examination of the gallbladder was successfully performed in 226 patients with sickle cell hemoglobinopathy from 2 to 18 years of age, and sixty-three demonstrated the presence of gallstones.
TL;DR: In this paper, the authors examined the social validity of a parent training program and found that parents perceived their children as less compliant and more deviant before treatment but not after treatment or at the follow-up than the non-clinic children.
TL;DR: The overall incidence of calculus disease in patients was higher than reported previously from other centers in this country andImmobilization was an important factor in the etiology of stone disease.
Abstract: Sixty-one children who presented with urinary calculi between 1967 and 1977 were reviewed. The overall incidence of calculus disease in our patients was higher than reported previously from other centers in this country. Because presenting symptoms varied, a high index of suspicion was often required for diagnosis. Stones of infection were the most common type identified. Inasmuch as the recurrence rate of calculi in children was low (6.5%), careful consideration of time and cost is warrented before extensive evaluation for metabolic causes of stone formation is undertaken. Immobilization was an important factor in the etiology of stone disease. Most stones less than 5 mm in size passed without surgery.
TL;DR: Orthopedic surgery can alleviate the hip flexion, adduction, and medial rotation deformities of the hip and improve the function and appearance of gait and the goal should be optimal independence for the child and adolescent during development, and freedom from pain with deteriorating function due to degenerative arthritis in the adult.
TL;DR: Phenotypic features and functions known to depend on the presence of the Y chromosome or the H-Y antigen are discussed in relation to structural anomalies of theY chromosome and other abnormalities of sexual and somatic development.
Abstract: Phenotypic features and functions known to depend on the presence of the Y chromosome or the H-Y antigen are discussed in relation to structural anomalies of the Y chromosome and other abnormalities of sexual and somatic development.
TL;DR: Recognition of reasons underlying parents' sense of the child's special status may do much to allay unnecessary fears and promote more appropriate use of health care facilities.
Abstract: In a study of use of five general pediatric services, 750 parents were interviewed on site about their decisions on how and when to seek medical care for their children. Parents' fears that a particular child was "vulnerable"--ie, uniquely threatened by an episode of illness--was a recurrent concern (reported by 27%), explaining many of their medical visits. Medical record review indicated that in 40%, there was no clinical basis for these parent concerns. One important source of these unwarranted concerns was fear of recurrence of an earlier medical problem, long since resolved. In some families, the role of social and environmental issues in generating a high level of concern was evident. Vulnerable children made more visits per year and made many more of their visits to the emergency room, and their parents more often expressed dissatisfaction with care received. Recognition of reasons underlying parents' sense of the child's special status may do much to allay unnecessary fears and promote more appropriate use of health care facilities.
TL;DR: Biochemical data fromSerial biochemical studies of a rat cortical tissue culture system in which synapses regularly form showed that gamma-aminobutyric acid (GABA) is present in the cultures and increases with their maturation, supporting the concept that GABA is the transmitter for many cortical synapses.
TL;DR: Clinical characteristics of pseudoseizures and epileptic seizures documented by TEEG-VR were compared and degree and duration of the postictal state, incontinence, combativeness, relationship to stress, and response to anticonvulsant medication were useful differentiating criteria.
TL;DR: The EEG maturation in girls up to 11 years seems to be retarded compared with boys: the mean percentage of slower activity is higher, and of faster activity is lower, in girls than boys.
TL;DR: Important differences between the metabolic activation of 7,12-dimethylbenz[a]anthracene in intact cellular systems and in liver homogenate suggest that the use of homogenates in conjunction with short-term assays for carcinogens could yield misleading results.
Abstract: Important differences between the metabolic activation of 7,12-dimethylbenz[a]anthracene in intact cellular systems and in liver homogenates suggest that the use of homogenates in conjunction with short-term assays for carcinogens could yield misleading results.