Showing papers by "Montreal Children's Hospital published in 1979"
01 Jan 1979
TL;DR: It is evident that a good deal of confusion and disagreement remains about the exact nature of the deficit being attributed to hyperactive children.
Abstract: Over the past several years, acceptance of the central importance of an attentional deficit in hyperactive children has been emerging in the clinical, educational, and research literature. When we examine the definitions of attentional deficit being used by different authors, however, it becomes evident that a good deal of confusion and disagreement remains about the exact nature of the deficit being attributed to hyperactive children.
337 citations
TL;DR: The data indicate that the 4‐day plasma t½ of caffeine characteristic of the newborn depends in large part on slow urinary excretion of unchanged drug since there is little or no metabolism.
Abstract: The concentrations of caffeine and metabolites in urine have been examined as a function of age to explore the remarkably slow elimination of caffeine by human infants. Urine samples were obtained from 3 adults and 10 infants aged 8 days to 8 months during therapeutic treatment with caffeine. A high-performance liquid chromatographic (HPLC) procedure involving reversed-phase partition chromatography was developed to separate caffeine and 13 of its metabolites. During the first month of life, caffeine accounted for more than 85% of the identifiable products in urine. Caffeine remained the predominant component for the first 3 months, but its percentage decreased gradually to the adult value of less than 2% by the age of 7 to 9 months. This change reflected increasing metabolite production, not decreasing urinary caffeine concentration. The adult metabolite pattern of partially demethylated xanthines and urates was attained by 7 to 9 months. The data indicate that the 4-day plasma t1/2 of caffeine characteristic of the newborn depends in large part on slow urinary excretion of unchanged drug since there is little or no metabolism. Subsequent decrease in the t1/2 to about 4 hr by the age of 8 months correlates closely with the rise in metabolite production.
260 citations
TL;DR: The effects of prolonged food deprivation on plasma GH, pituitary GH, and tissue somatostatin levels were investigated in the male rat and a rebound response was observed in rats allowed to refeed for 3 days after 72-h food deprivation, as evidenced by an increased number of GH secretory episodes and a shorter period of the GH rhythm.
Abstract: The effects of prolonged food deprivation on plasma GH, pituitary GH, and tissue somatostatin levels were investigated in the male rat. Six-hour GH secretory profiles obtained from normal animals bearing chronic intracardiac venous cannulae showed the typical ultradian GH rhythm, with most peak GH values being >600 ng/ml. Exposure to 24-h food deprivation resulted in a significant depression in amplitude of GH pulses, with peak values not exceeding 300 ng/ml. The amplitude and duration of the GH secretory episodes declined progressively after 48 and 72-h food deprivation, and normal periodicity was not evident. The mean 6-h GH level in each of the food-deprived groups was significantly less than that of normal animals. A rebound response was observed in rats allowed to refeed for 3 days after 72-h food deprivation, as evidenced by an increased number of GH secretory episodes and a shorter period of the GH rhythm. In a second series of experiments, pituitary GH and tissue levels of somatostatin-like immuno...
221 citations
TL;DR: This analysis of counselees' perceptions of the problems created by being at genetic risk suggests that parents may process the disparate facts of their situation in common ways that emphasize their uncertainty, and it indicates that how parents perceive factual information may be more important in orienting their deliberations than what these facts actually are.
Abstract: To investigate how parents who have had genetic counseling perceive the problems created by being at risk, transcripts of open-ended, semistructured follow-up interviews with 53 counselees were analyzed qualitatively. Rate information, though recalled accurately by parents considering further childbearing, was discounted as impersonal, and subjects overwhelmingly perceived the chance of recurrence in binary form -- it either will or will not happen. By processing rates this way, they simplified probabilistic information and shifted their focus to the implications of being at risk and the potential impact of that which might or might not occur. The many uncertainties they faced, the "consequences" of being at risk that parents felt had to be resolved during the decision-making process, fell into 3 major categories: uncertainty that arose because of the ambiguous impact and meaning of having an affected child; uncertainty about how to make a choice and how others would view it, the burden of decision-making; and uncertainty about their ability to fulfill their roles as parents. These issues were perceived as part of the problem to be resolved and were consolidated into "scenarios" in which the parents "tried out the worst." This analysis of counselees' perceptions of the problems created by being at genetic risk suggests that parents may process the disparate facts of their situation in common ways that emphasize their uncertainty, and it indicates that how parents perceive factual information may be more important in orienting their deliberations than what these facts (diagnosis, prognosis, risks) actually are.
144 citations
TL;DR: Assessment of the effectiveness and side-effects of antenatal administration of betamethasone in the prevention of respiratory distress syndrome in potentially premature infants found that prolonged rupture of the membranes played no protective role against RDS, and the incidence of infection was similar in both groups.
Abstract: One hundred forty-six pregnant women were enrolled in a prospective double-blind study to assess the effectiveness and side-effects of antenatal administration of betamethasone in the prevention of respiratory distress syndrome (RDS) in potentially premature infants. On admission to the study, the women were given, at random, either 12 mg of betamethasone or placebo. The same dose was repeated 24 hours later and then weekly up to 34 weeks of gestation. Gestational age of the infants ranged from 25 to 34 weeks, and birth weights ranged between 730 and 2,650 gm. Statistically significant differences in favor of the infants in the betamethasone group were found in the incidence of RDS, 20.7% in the betamethasone group compared with 59.5% in the control group (P less than .005); in the severity of RDS (P less than .05); and in the death rate (P less than .05). A higher incidence of hypoglycemia was found among infants in the betamethasone group (P less than .05). Prolonged rupture of the membranes played no protective role against RDS, and the incidence of infection was similar in both groups.
122 citations
TL;DR: In this paper, transcripts of 30 tape-recorded or observed genetic counseling sessions were analyzed qualitatively to explore verbal communication between family and counselor, and the findings suggest that strategies for processing information are an important element influencing parental perspectives on and approaches to the problems created by being at risk and to possible courses of action.
Abstract: To explore verbal communication between family and counselor, transcripts of 30 tape-recorded or observed genetic counseling sessions were analyzed qualitatively. In half the cases, available data did not allow counselors to give parents a single estimate of the recurrence rate. Moreover, limits on the language available for communicating small probabilities made common the use of nonnumerical statements about a family's chance of having an abnormal child. Counselees processed the factual information they were given, most commonly translating recurrence rates in ways that emphasized the uncertainties associated with them. They tended to view these rates in binary form and requested guidelines for their behavior, indicating uncertainty about how to proceed with reproductive decision-making. The findings suggest that strategies for processing information are an important element influencing parental perspectives on and approaches to the problems created by being at-risk and to possible courses of action.
122 citations
TL;DR: It is concluded that this syndrome can best be understood in terms of interactions between social, psychological, and biological variables.
Abstract: The behavioral characteristics of the hyperactive child are described at different stages of development. Recent and sometimes controversial research findings are discussed with respect to prevalence, etiology, treatment, and outcome. It is concluded that this syndrome can best be understood in terms of interactions between social, psychological, and biological variables.
99 citations
TL;DR: The cognitive styles of reflection-impulsivity, field-dependence-independence, and constricted-flexible control were studied in hyperactive adults and matched controls and indicated that the hyperactive Adults were more field-dependent and more constricted than the controls.
Abstract: SUMMARY
The cognitive styles of reflection-impulsivity, field-dependence-independence, and constricted-flexible control were studied in 70 hyperactive adults and 42 matched controls as part of a follow-up study. Results indicated that the hyperactive adults were more field-dependent and more constricted than the controls. They were also less accurate although they did not respond more quickly than the controls on a visual matching task. The educational implications of this are discussed.
87 citations
TL;DR: Articles in this section should require 3 Journal pages or less; the text 1,000 words or less and a combined total of 2 illustrations or tables with up to 10 references.
81 citations
TL;DR: It is concluded that the ileal mechanism for active transport of taurocholate is undeveloped in the fetus and newborn infant and losses of bile salt from the immature intestine may contribute to the steatorrhea and so-called diarrhea of newborn infants.
80 citations
TL;DR: Qualitative analysis of transcripts of follow-up interviews with 53 parents who had had genetic counseling was undertaken to characterize the process by which childbearing decisions were made and to determine how counselees resolved the problems created by being at risk.
Abstract: Qualitative analysis of transcripts of follow-up interviews with 53 parents who had had genetic counseling was undertaken to characterize the process by which childbearing decisions were made and to determine how counselees resolved the problems created by being at risk. Although specific issues to be resolved varied with a parent's perception of his or her situation, all those who considered having subsequent children attempted to limit the uncertainties they faced and to neutralize those consequences perceived as most problematic. To do so, counselees uniformly inferred from factual information and experiences available to them how they could manage the possible consequences of taking a chance. Factors influencing a parent's ability to make a clear decision included the presence of a previous normal child, the diffusion of decision-making responsibility to others, and recognition that one had already managed the worst. When these "facts" could not be processed to provide a sense of coping, parents either decided against reproduction and took appropriate action or made a "non-decision" about reproduction by choosing to leave conception to chance. These various tactics allowed parents to create a "least-lose" option in terms of their child-bearing choices. This analysis of how parents make reproductive decisions, along with previous findings, suggests that being at risk both creates common problems and elicits common responses from counselees. Moreover, it highlights the importance of recognizing parents' perceptions of their situation to understand how their deliberations are structured and how factual information influences their ultimate choices.
TL;DR: Liver explants obtained from human fetuses with gestational ages of 12 to 20 weeks were incubated with theophylline and produced caffeine and, in lesser amounts, 1,3-dimethyluric acid and 3-methylxanthine, suggesting that the predominant pathway in theophyLLine metabolism in the fetus and newborn infant is the methylation reaction producing caffeine.
Abstract: Caffeine (1,3,7-trimethylxanthine) is a biotransformation product of theophylline (1,3-dimethylxanthine) in the human fetus. Liver explants, obtained from human fetuses with gestational ages of 12 to 20 weeks, were incubated with theophylline and produced caffeine and, in lesser amounts, 1,3-dimethyluric acid and 3-methylxanthine. These findings suggest that the predominant pathway in theophylline metabolism in the fetus and newborn infant is the methylation reaction producing caffeine. This may contribute to the neonate's exceedingly slower elimination of caffeine relative to theophylline. Caffeine produced from theophylline may add to the pharmacologic effects of theophylline in newborn infants with apnea.
TL;DR: The polymorphic cellular composition lends support to the concept that the myxoid variant of malignant fibrous histiocytoma (MFH) is probably derived from a primitive multipotent mesenchymal cell capable of structural and functional modulation toward more cytodifferentiated forms.
Abstract: The ultrastructural findings in 4 cases of the myxoid variant of malignant fibrous histiocytoma (MFH) are described. Although this neoplasm is characterized by a high rate of local recurrence, the overall prognosis is considerably better than the usual nonmyxoid MFH. The neoplasm is composed of an admixture of round, stellate and multinucleated giant cells within a myxoid and well-vascularized stroma. Electron microscopy demonstrated four principal cell types: a primitive mesenchymal cell, spindle cells of fibroblastic and histiocytic nature, and multinucleated giant cells. These observations complement the light microscopic features but probably are insufficient to differentiate critically this entity from other myxomatous lesions of mesenchymal soft tissue such as myxoma, pseudosarcomatous fasciitis, myxoid liposarcoma. The polymorphic cellular composition lends support to the concept that the neoplasm is probably derived from a primitive multipotent mesenchymal cell capable of structural and functional modulation toward more cytodifferentiated forms.
TL;DR: The ability of the Hyp/Y mouse to adapt to phosphorus deprivation is reported here on; the response is observed both in vivo in net reabsorption of Pi and in vitro in BBMV transport of Pi.
Abstract: Human X-linked hypophosphataemia (XLH)1 involves a partial loss in net renal reabsorption of phosphate anion (Pi). The mutant male hemizygote has about half the normal renal transport activity2 and this is quite insensitive to circulating parathyroid hormone (PTH)2,3. The renal defect is sufficient to explain persistent hypophosphataemia in XLH. The hypophosphataemic mouse (Hyp)4 also has an X-linked defect in renal reabsorption of Pi (refs 4–7). The mutant male hemizygote (Hyp/Y) has about half the normal renal reabsorption activity and this abnormality persists after parathyroidectomy7. Conservation of the X chromosome during mammalian evolution and evidence for functional homology between species for specific X-linked genes8 have encouraged us to use the Hyp mouse as a model for the renal defect in XLH. We have studied Pi transport in purified renal brush border membrane vesicles (BBMV) prepared from Hyp/Y mice5,9. A selective loss involving almost half the Na+-dependent Pi co-transport activity is present in Hyp/Y membranes. These findings, genetic studies in man2,10 and recent physiological evidence11 suggest that Pi transport in the mammalian nephron occurs by at least two processes: one under the control of an X-linked gene that is more sensitive to PTH inhibition and another process, controlled by an autosomal locus, that is less sensitive to the hormone. We report here on the ability of the Hyp/Y mouse to adapt to phosphorus deprivation; the response is observed both in vivo in net reabsorption of Pi and in vitro in BBMV transport of Pi.
TL;DR: A bacteriologic survey was performed to estimate the prevalence and duration of meningococcal carriage in children in Montreal, Canada and there was no difference in carriage rates between the sexes and between hospitalized and non-hospitalized children in all age groups.
Abstract: A bacteriologic survey was performed to estimate the prevalence and duration of meningococcal carriage in children in Montreal, Canada. Infants and children with proven meningococcal infection, or with asymptomatic meningococcal nasopharyngeal (NP) carriage, and their household contacts, were also studied to define communicability. N. meningitidis was present in 30 (2.4%) of the NP cultures from 1238 asymptomatic infants and children in this civilian population during a non-epidemic period. Meningococcal carriage was not found in 278 subjects 1--60 days of age; there was no difference in carriage rates between the sexes and between hospitalized and non-hospitalized children in all age groups. Meningococci were initially isolated from 11 of 106 household contacts of 29 ill index cases and from 15 of 104 contacts of 29 asymptomatic carriers; 35% of all contacts (index cases and carriers) were colonized by the eighth week of surveillance. Duration of NP carriage was longer (mean 15.2 weeks) in disease-free families than in families of ill patients (mean 5.5 weeks). Serogroups B and C were most commonly isolated from both ill and asymptomatic subjects. Resistance to sulfadiazine (MIC greater than or equal to mg/100 ml) was present in 6.5% and 39.4% of group B and group C strains, respectively. Although chemoprophylaxis was not used, there were no secondary cases among the 29 families of index cases.
TL;DR: The hand pattern profiles of 18 previously published and 10 new cases of de Lange syndrome were compared to those of cases referred as suspects, but judged clinically and by numerical taxonomic methods not to have de Lange Syndrome (non-de Lange).
Abstract: The hand pattern profiles of 18 previously published and 10 new cases of de Lange syndrome were compared to those of cases referred as suspects, but judged clinically and by numerical taxonomic methods not to have de Lange syndrome (non-de Lange). Based on a Poznanski metacarpophalangeal profile of the 2 groups of patients, a simple scoring system using 11 measurements was devised using the 16 most marked differences within and between metacarpals and phalanges. Of the metacarpals, the first is shorter than the second, third, fourth, or fifth; and the second and fifth are shorter than the third or fourth. Of the middle phalanges, the third and fourth are shorter than the respective metacarpal and than the second and fifth middle phalanx. Of the distal phalanges, the fifth is shorter than the second. The mean Z score for these 11 measurements is further below normal in the de Lange group than in the non-de Lange group. All 11 non-de Lange patients had a score less than 17, and all 28 de Lange patients had a score of 18 or more.
TL;DR: The effect of growth hormone on somatomedin levels and growth rate during the first year of life in one of the infants is described.
Abstract: . Two infants with idiopathic panhypopituitarism presented with severe neonatal hypoglycaemia, hepatomegaly and hyperbilirubinaemia (direct and indirect). Abnormal liver function tests returned to normal over a 5–8 month period. The growth rate in the absence of detectable growth hormone was 50% of normal during the first 6 months. The effect of growth hormone on somatomedin levels and growth rate during the first year of life in one of the infants is described.
TL;DR: To date, simple skin testing using recall antigens has proved to be the most accurate method of assessing preoperative risk for serious infectious complications.
TL;DR: Ch Chromosomal analysis of girls with short stature, normal pubertal development, and regular menses may reveal that minor deletions of the short arm of the X chromosome are more frequent than has been previously reported.
TL;DR: The findings suggest that three gene products serve net tubular reabsorption of imino acids and glycine in human kidney, which is likely to reflect deviant and specific brush border membrane carrier activity toward these amino acids.
Abstract: Seven infants (two French Canadian, four Ashkenazi Jewish, and one Greek) with massive selective hyperiminoglycinuria (proline, hydroxyproline, and glycine) were detected by urine screening in the second week of life. Follow-up investigations and family studies revealed that each subject had a benign condition, familial renal iminoglycinuria, an autosomal recessive condition. The family studies (Table 1 and Fig. 1) indicate the presence of at least two different mutant alleles segregating in this small group of probands. Hmozygotes of two forms and one genetic compound were identified. Quantitative studies revealed normal concentrations of proline and glycine in plasma (Fig. 2), normal maturation of creatinine clearance (as an index of glomerular filtration rate) (Fig. 3), and elevated renal clearance of proline and glycine (Table 2). Fractional excretion (CAA/CCR) of both proline and glycine in the probands was far in excess of that expected for the normal postnatal infant; FFPro and FEGly approached 100% of the filtered load on occasion (Fig. 4). A schedule of maturing tubular reabsorptive activity was apparent in the proband group. Proline reabsorption matured earlier than glycine reabsorption in the homozygotes (and the genetic compound) as it does in the normal infants (Fig. 5). Our findings suggest that three gene products serve net tubular reabsorption of imino acids and glycine in human kidney. One, affected by mutation in our patients, is responsible for a shared transport activity; a second with preference for proline, and not affected by the mutation, has an "early" schedule of postnatal maturation; and a third with preference for glycine, also not affected by the mutation, has a "late" schedule of maturation.
TL;DR: It is suggested that in children with malignant hypertension who are resistant to conventional modes of antihypertensive therapy, the use of the oral angiotensin I-convert ing enzyme inhibitor (Squibb 14225, captopril) may be a valuable and perhaps life-saving treatment.
TL;DR: A case of the G syndrome is reported in a baby boy who had an unusual facies (prominent forehead, telecanthus, posteriorly rotated ears, and anteverted nostrils) possibly secondary to birth asphyxia and numerous medical and surgical complications.
Abstract: A case of the G syndrome is reported in a baby boy who had an unusual facies (prominent forehead, telecanthus, posteriorly rotated ears, and anteverted nostrils), laryngeal cleft, hypospadias, cryptorchidism, and psychomotor development delay, possibly secondary to birth asphyxia and numerous medical and surgical complications. The mother had a similar facies.
TL;DR: A crude muscle extract infused into rats produced oliguria, a precipitous drop in total hemolytic complement, and in circulating white cell and platelets counts, and a mild vaso-depressor effects was noted.
Abstract: A crude muscle extract infused into rats produced oliguria, a precipitous drop in total hemolytic complement, and in circulating white cell and platelets counts. A mild vaso-depressor effects was noted. These changes were not produced by myoglobin or saline infusion. Muscle constituents other than myoglobin are responsible for the systemic and renal nephrotoxic effects observed.
TL;DR: It is concluded that the teaching of an oral means of communication which facilitates education within a mainstreamed system, is compatible with good psychosocial adjustment.
Abstract: There is a general pessimistic view found in the literature that defends a hypothesis that the consequences of a severe hearing impairment on psychosocial development aare many and severe. This pilot study of 20 adolescents who had experienced a profound hearing impairment from birth had the purpose of clarifying this issue. The findings of the study were not compatible with such pessimism. It is concluded that the teaching of an oral means of communication which facilitates education within a mainstreamed system, is compatible with good psychosocial adjustment. To confirm this optimism and to delineate the variables in good development, further study employing comparison groups is needed.
TL;DR: A family is presented in which the propositus has brachydactyly C, short stature, and hip dysplasia.
Abstract: Short stature may be a component of the brachydactyly C syndrome. A family is presented in which the propositus has brachydactyly C, short stature, and hip dysplasia.
TL;DR: It is proposed that this condition can be managed by suprapubic cystotomy with the fulguration of obvious bleeding points and the placement of two suprapUBic tubes.
TL;DR: Monitoring of changes occuring in two biologically active thymic stromal fractions during a state of chronic GvH reaction observed changes are interpreted as being possibly related to the state of immunosuppression by interfering with normal T cell differentiation and traffic through the thymus.