Showing papers by "Children's Memorial Hospital published in 1979"
Journal Article•
TL;DR: Significant interactions between sex and age relationship to the child were noted on scales of social withdrawal, inhibition, immaturity, and irritability, and the differences between illness groups approached significance on measures of fear and inhibition.
Abstract: Although numerous references are made to the stressful, deleterious effects of chronic or terminal illnesses and handicaps on the siblings of pediatric patients, very few studies have been conducted using comparison groups. The present study compared the adjustment of 3- to 13-year-old siblings of pediatric hematology (N = 62), cardiology (N = 57), and plastic surgery patients (N = 37) with healthy siblings (N = 46). The adjustment measure was an objective, paper-and-pencil measure of children's emotional and behavioral problems, the Louisville Behavior Checklist. On analyses of covariance, the patient groups were more likely to show symptoms of irritability and social withdrawal, and the differences between illness groups approached significance on measures of fear and inhibition. Among the younger children, ages 3 to 6, there were significant group differences, with the siblings of patients undergoing plastic surgery showing the highest level of general psychopathology. Among children ages 7 to 13, male siblings of patients with blood disorders were more likely to show signs of emotional disturbance than female siblings. No group differences were noted on measures of aggression or learning problems. Significant interactions between sex and age relationship to the child were noted on scales of social withdrawal, inhibition, immaturity, and irritability.
204 citations
TL;DR: The data suggest that abnormalities of linear growth, which can occur in children with poorly controlled diabetes, may involve abnormalities in net somatomedin activity.
Abstract: To test the hypothesis that somatomedin activity is influenced by diabetes and its metabolic regulation, the relationship between somatomedin activity and diabetic control as assessed by hemoglobin A1C was investigated in 40 children with insulin-dependent diabetes. An inverse correlation between hemoglobin A1c and serum somatomedin activity was statistically significant. The data suggest that abnormalities of linear growth, which can occur in children with poorly controlled diabetes, may involve abnormalities in net somatomedin activity.
90 citations
TL;DR: The clinical score is a useful adjunct in the management of children with chronic inflammatory bowel disease and can be used in prospective studies of various therapeutic modalities.
Abstract: A clinical scoring system for the assessment of children with chronic inflammatory bowel disease has been devised. A close correlation is demonstrated between severity of disease and the level of serum albumin. The clinical score is simple to perform, sensitive to changes in clinical status, reproducible by different observers, and specifically designed to evaluate inflammatory bowel disease in children and adolescents. The clinical score is a useful adjunct in the management of children with chronic inflammatory bowel disease and can be used in prospective studies of various therapeutic modalities.
86 citations
TL;DR: The survival of children given total resection of the tumour was significantly longer, and females survived longer than males, and reexploration of the posterior fossa for recurrences failed to improve the prognosis.
Abstract: Fifty-one patients with medulloblastoma have been treated at the Children's Memorial Hospital during the past 11 years. The ratio between males and females was 2∶1. The ages ranged from 4 months to 12 years; 7 patients were less than 12 months old at the time of diagnosis. After 1969, prior to definitive surgery, all patients were given a ventriculo-peritoneal shunt to decompress the hydrocephalus which was invariably present. There were no cases of systemic metastasis related to these shunts. Subsequently, posterior fossa craniotomy was performed, with total or radical resection of tumour in 13 cases, subtotal resection in 20 cases, and partial resection or biopsy in 14 cases. After completion of surgical treatment and radiotherapy, the three-year survival rate was 45.6 per cent and the five-year survival 34.5 per cent. The survival of children given total resection of the tumour was significantly longer, and females survived longer than males. Local recurrence and spinal cord or systemic metastases are discussed. Reexploration of the posterior fossa for recurrences failed to improve the prognosis. Recurrent or metastatic medulloblastoma should be treated by radiotherapy or chemotherapy, or both.
64 citations
TL;DR: It is concluded that modest hyperlipidemia does exist in some children with growth hormone deficiency, and subclinical hypothyroidism may play a role in the hypercholesterolemia of some children, as may grow hormone deficiency itself.
Abstract: To test the hypothesis that hyperlipidemia is characteristic of growth hormone deficiency in childhood, we have measured serum cholesterol and triglyceride concentrations in 24 euthyroid children with growth hormone deficiency. Although modest elevations of cholesterol and/or triglyceride above the 95th percentile for age, race, and sex were present in 46% of the children studied, the mean (+/- 1 SD) cholesterol of 173 +/- 36 mg/dl and the mean triglyceride of 80 +/- 42 mg/dl were not significantly different from published normal mean values. Administration of human growth hormone for 4 mo to 15 of these subjects did not alter these mean cholesterol and triglyceride values, but did result in a marked improvement in the growth rate. Some individuals (n = 6) with a subnormal growth response to therapy and/or a low serum thyroxine and high serum cholesterol were treated for an additional 4 mo with growth hormone and thyroid hormone together. There was a statistically significant decrement in serum cholesterol in this group. We conclude that modest hyperlipidemia does exist in some children with growth hormone deficiency. Subclinical hypothyroidism may play a role in the hypercholesterolemia of some children, as may growth hormone deficiency itself. Any association of growth hormone and lipid metabolism remains to be clarified.
53 citations
TL;DR: The morphology was studied in postmortem specimens of children with and without paraureteral diverticula and urethral obstruction, and function was determined by deductive reasoning from radiographic and surgical observations in living children.
48 citations
TL;DR: Preoperative surface electromyography of the urinary sphincters may prove to be a useful screening test to detect occult dyssynergia in patients who have had failed ureteral reimplants.
48 citations
TL;DR: Positional abnormalities (straddling or overriding) of the atrioventricular (A-V) valves were studied using angiographic, sector scan echocardiographic and postmortem anatomic data in 10 patients with transposition of the great arteries including double outlet right ventricle.
Abstract: Positional abnormalities (straddling or overriding) of the atrioventricular (A-V) valves were studied using angiographic, sector scan echocardiographic and postmortem anatomic data in 10 patients with transposition of the great arteries including double outlet right ventricle. Group I included six patients with tricuspid valve abnormalities. This group was further classified into: (a) patients with anular straddling and abnormal attachment of portions of the tricuspid leaflets in the left ventricle (malattachment) or on the ventricular septum, or both (four patients with d-transposition of the great arteries and hypoplastic right ventricle); and (2) patients with malattachment of portions of the tricuspid valve leaflet to the crest or left ventricular surface of the septum (septal malattachment) without significant anular straddling (two patients with d-transposition of the great arteries). Group II included four patients with mitral valve abnormalities without significant anular straddling, further classified into: (1) patients with septal malattachment of the portions of the mitral valve to the crest and right ventricular surface of the ventricular septum (two patients with double outlet right ventricle); and (2) patients with ventricular malattachment of portions of the mitral valve leaflets to the papillary muscles in the right ventricle (two patients with ventricular septal defect and discordant criss-cross atrioventricular connections). On the basis of these observations and reported experience, two main types of positional abnormalities of the A-V valves were recognized: (1) anular straddling in which the A-V anulus straddled the ventricular septum above the contralateral ventricle, together with ventricular leaflet malattachments, and (2) leaflet malattachment of either the septal or ventricular type without significant straddling of the valve anulus. Angiocardiography and sector scan echocardiography helped to identify these abnormalities of the A-V valves.
37 citations
TL;DR: Dermatitis herpetiformis and SLE were diagnosed in a 15-year-old girl with Marfan's syndrome who died of cardiac tamponade secondary to cystic medial necrosis of the ascending aorta, suggesting that predisposition to immune-mediated disorders may be associated with the expression of multiple clinical entities.
Abstract: There is increasing evidence that, as in systemic lupus erythematosus (SLE), deposition of immune complexes plays a role in the pathogenesis of dermatitis herpetiformis (DH). Dermatitis herpetiformis and SLE were diagnosed in a 15-year-old girl with Marfan's syndrome who died of cardiac tamponade secondary to cystic medial necrosis of the ascending aorta. The concurrence of these diseases suggests that predisposition to immune-mediated disorders may be associated with the expression of multiple clinical entities.
37 citations
TL;DR: Findings lend support to a hypothesis that the development of Wilms' tumor requires the occurrence of two successive mutational events, one of which may be a germinal mutation.
Abstract: Wilms' tumor was diagnosed in two children each of whom has an identical twin. In one of the pairs of twins the aniridia syndrome with psychomotor retardation was present in both children, but Wilms' tumor was found in only one. In the other twins hemihypertrophy as well as Wilms' tumor were identified in one child, whereas neither of these abnormalities was present in her twin sister. These findings lend support to a hypothesis that the development of Wilms' tumor requires the occurrence of two successive mutational events, one of which may be a germinal mutation. The presence of aniridia, hemihypertrophy, or other associated congenital abnormalities may aid in distinguishing between hereditary and sporadic forms of Wilms' tumor.
35 citations
TL;DR: Four infants with d-transposition of the great arteries and intact ventricular septum who manifested early clinical symptoms and deterioration due to dynamic left ventricular outflow stenosis are presented and the Mustard operation relieved symptoms in all infants.
Abstract: Four infants with d-transposition of the great arteries and intact ventricular septum who manifested early clinical symptoms and deterioration due to dynamic left ventricular outflow stenosis are presented. All four had an anatomically adequate atrial septal defect, made at the initial balloon atrial septostomy, that was later confirmed intraoperatively. Two infants continued to have a low arterial oxygen saturation level because of inadequate interatrial mixing, and one of these had severe persistent cyanosis and was treated with the Mustard operation at age 4 days. The other two infants subsequently presented with hypercyanotic spells at age 3 months. All four infants had features of dynamic left ventricular outflow stenosis on hemodynamic, angiocardiographic and echocardiographic studies. The left ventricular outflow pressure gradient was shown to increase after administration of isoproterenol in one infant, and relief of a cyanotic spell with reduction of left ventricular systolic pressure was achieved in another after intravenous administration of propranolol. The Mustard operation relieved symptoms in all infants. The effect of left ventricular outflow tract stenosis on the mechanisms responsible for interatrial mixing in d-transposition of the great arteries with intact ventricular septum is discussed.
TL;DR: Experimental observations indicate the absence of free putrescine, spermidine and spermine throughout gestation, which is compatible with an in vivo function in the regulation of embryonic growth and development.
TL;DR: Percutaneous nephrostomy drainage may be established rapidly using ultrasonic guidance in severely ill pediatric patients with obstructive uropathy using B-mode ultrasonography.
TL;DR: Evidence is given that the degree of abnormality in uric acid and nucleoside concentrations in plasma and urine reflect the severity of the enzymatic deficiency and structural alterations of the mutant enzymes result from structural gene mutations and demonstrate genetic heterogeneity in the disease purine nucleosid phosphorylase deficiency.
Abstract: The biochemical features of two families with purine nucleoside phosphorylase deficiency are compared. Laboratory studies and an evaluation of kinetic and physical properties of erythrocyte purine nucleoside phosphorylase give evidence that a) the degree of abnormality in uric acid and nucleoside concentrations in plasma and urine reflect the severity of the enzymatic deficiency and b) structural alterations of the mutant enzymes result from structural gene mutations and demonstrate genetic heterogeneity in the disease purine nucleoside phosphorylase deficiency.
TL;DR: Results suggest a possible alteration in the carbohydrate moiety of alpha 2-macroglobulin in cystic fibrosis, presumably due to a defective posttranslational process.
TL;DR: It is concluded that the end-diastolic h r ratio and the VOT Ao ratio are quantitatively useful in the assessment of subaortic stenosis, whereas the commonly present motion abnormalities of the aortic valve are not indicative of the severity of the subAortic lesion.
Abstract: The validity of Laplace's relation for assessing peak left ventricular pressure has previously been demonstrated in children with congenital aortic valve s tenosis by using the echocardiographically determined end-diastolic h r ratio (that is, the ratio of mean left ventricular septal and posterior wall thicknesses to half the minor left ventricular cavity axis). This report examines the applicability of this ratio to the preoperatlve assessment of left ventricular pressure in 17 children with discrete subaortic stenosis. Because the regression equation derived from previously published data on patients with aortic valve stenosis and the present data on children with subaortic stenosis were similar, a pooled estimate of peak left ventricular pressure (LVP) in children w ith left ventricu l ar outflow tract obstruction is presented: LVP = 312.23 ( h r ) ± 28.01 ( h r ) . Additionally, the left ventricular outflow tract was measured as the anteroposterior dimension between the first recognizable closure point of the mitral valve and the interventricular septum and expressed as the left ventricular outflow tract/aortic root dimension ( LVOT Ao ) ratio. This ratio was tower in patients with subaortic stenosis than in control subjects and was helpful in differentiating patients with mild stenosis from those with more severe stenosis. The prevalence of echocardiographically observed features such as early systolic closure of the aortic valve and fluttering of the aortic valve leaflets could not be correl a ted with the severity of stenosis. It is concluded that the end-diastolic h r ratio and the VOT Ao ratio are quantitatively useful in the assessment of subaortic stenosis, whereas the commonly present motion abnormalities of the aortic valve are not indicative of the severity of the subaortic lesion.
TL;DR: An abnormal cystometrogram was found in 61% of the children with a voiding abnormality and helped to establish a basis for successful therapy and facilitated the management of childhood ureteral reflux.
TL;DR: Echocardiography provides anatomic diagnosis of a supracristal ventricular septal defect with prolapsed right coronary aortic leaflet in four children aged 2 to 10 years and the severity and progression can be assessed by quantitation of the left ventricular size and performance.
Abstract: Anatomically diagnostic echocardiographic features of a supracristal ventricular septal defect with prolapsed right coronary aortic leaflet are described in four children aged 2 to 10 years. Both single crystal M mode as well as 80 ° phased array sector scan techniques were used. The echographic features in the M mode scan from the aorta to the left ventricle in three of four patients included (1) the position of the ventricular septal defect as a clear space between the interrupted septal echoes below the aortic root, and (2) the prolapsed right coronary aortic leaflet as anomalous linear echoes in the right ventricular outflow tract. Angiographic, intraoperative and echocardiographic contrast studies were used to establish the diagnosis. On sector scanning using the long axis view, the supracristal ventricular septal defect was recognized as a clear space between the top of the ventricular septum and the anterior segment of the aortic root in three of four patients. The right coronary aortic leaflet was seen to prolapse into the right ventricular outflow tract through this defect, and its motion could be clearly followed during systole and diastole. It is concluded that echocardiography provides anatomic diagnosis of this lesion. Furthermore, the severity and progression of this lesion can be assessed by quantitation of the left ventricular size and performance.
TL;DR: These teenagers are living with, not dying of, cancer, and the authors must direct their efforts towards helping them to live with their disease and grow toward maturity.
Abstract: Adolescence is a time of tremendous change, characterized by conflicting high and low periods as the individual seeks to find his own unique identity, establish his autonomy, and determine his place in the world. The adolescent is no longer a child and not yet an adult. The feelings generated by this unique position in the life cycle create conflicts between the dependency needs of his childhood past and the natural striving for the independence of his adult future.
TL;DR: Both low- and high-molecular-weight inhibitors of serine proteases were found to inhibit chemotaxis by human polymorphonuclear leukocytes totally at widely varying concentrations.
Abstract: Both low- and high-molecular-weight inhibitors of serine proteases were found to inhibit chemotaxis by human polymorphonuclear leukocytes totally at widely varying concentrations. Synthetic low-molecular-weight substrates with trypsin-like or chymotrypsin-like specificity were also shown to be potent inhibitors of chemotaxis. Chemotactic inhibition was reversible except with a titrant for the active site of a serine protease. N-acetyl-L-tyrosine ethyl ester was found to be a suitable substrate for measuring protease activity of polymorphonuclear leukocytes. Concentrations of the various protease inhibitors that caused 100% chemotactic inhibition caused 80%-100% inhibition of protease activity of polymorphonuclear leukocytes. Chemotaxis is the directional movement of polymorphonuclear leukocytes (PMNLs) along a chemical gradient. Ward and Becker, based on their work with a variety of inhibitors, have suggested that serine esterases are associated with chemotax
TL;DR: Second remission induction rates for vincristine and prednisone alone were compared for children with acute lymphocytic leukemia as mentioned in this paper, and no evidence of a significant difference between the second induction complete remission rate for VP (78.6%) and VLP (73.7%) was found.
Abstract: Second remission induction rates for vincristine and prednisone alone (VP) and vincristine, L-asparaginase, and prednisone (VLP) are compared for children with acute lymphocytic leukemia. No evidence of a significant difference between the second induction complete remission rate for VP (78.6%) and VLP (73.7%) was found. Duration of first remission and prognostic group at initial diagnosis (defined on the basis of age and white blood count at initial diagnosis) are shown to be significant prognostic factors for second remission induction; and three second remission induction risk groups are defined on the basis of these two factors. Periodic reinforcement with prednisone in first remission does not appear to lower second induction complete response (CR) rates for VP. There was no evidence of a significant difference in the frequency of occurrence of severe toxicity between the VP and VLP regimens.
TL;DR: Investigation of two infants suggested that peak ventricular fluid levels of chloramphenicol after intravenous administration were achieved after 3 h, which may explain some of the past treatment failures when chlorampshenicol was utilized in patients with gram-negative ventriculitis and meningitis.
Abstract: Investigation of two infants suggested that peak ventricular fluid levels of chloramphenicol after intravenous administration were achieved after 3 h. The penetration of chloramphenicol into ventricular fluid may be unpredictable. In one patient, the peak ventricular fluid level was 57.5% of the peak serum level, and it was only 22.5% in the other patient. This observation may explain some of the past treatment failures when chloramphenicol was utilized in patients with gram-negative ventriculitis and meningitis.
TL;DR: Results indicate that anxious/inhibited boys are more likely than less inhibited boys to engage in prosocial behavior, which may be due to their greater empathy in observing the needs of others or to an effort at ingratiation or an overreaction to social distress.
Abstract: Relationships between dimensions of problem behavior and observations of prosocial behavior were explored among a sample of emotionally disturbed boys. Results indicate that anxious/inhibited boys are more likely than less inhibited boys to engage in prosocial behavior. This may be due to their greater empathy in observing the needs of others or, alternatively, to an effort at ingratiation or an overreaction to social distress. The latter possibility suggests that prosocial behavior may reflect or compound the interpersonal difficulties of anxious/inhibited boys.
TL;DR: It appears that mothers who desire to hold their babies immediately after delivery can be permitted to do so without concern by the staff that the babies will become chilled.
TL;DR: Glucose 6‐phosphate dehydrogenase deficiency was identified in three children who were evaluated because of chronic nonspherocytic hemolytic anemia and the electrophoretic, functional, and kinetic properties of the mutant enzymes allowed each to be distinguished from G‐6‐PD variants previously described.
Abstract: Glucose 6-phosphate dehydrogenase (G-6-PD) deficiency was identified in three children who were evaluated because of chronic nonspherocytic hemolytic anemia. One child is of German extraction, another Puerto Rican, and the third Mexican. In each of the patients the hemolytic process was well compensated, but each had one or more episodes of anemia following exposure to an oxidant drug or with infections. The electrophoretic, functional, and kinetic properties of the mutant enzymes, derived both from the patients' erythrocytes and from cultured fibroblasts, allowed each to be distinguished from G-6-PD variants previously described.
TL;DR: The findings indicate that the mutation in Krabbe disease leads to synthesis of normal quantities of catalytically and structurally altered protein.