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Showing papers by "Montreal Children's Hospital published in 1971"



Journal ArticleDOI
TL;DR: When the effect of methylphenidate on the cognitive styles of hyperactive children was examined, using a double blind, own control design, the drug resulted in less impulsive responding and improved ability to inhibit incorrect responses.
Abstract: Hyperactive and matched normal children were compared on four dimensions of cognitive style: reflection-impulsivity, field dependence-independence, constricted-flexible control, and automatization. The hyperactive group was more impulsive, more field dependent, more constricted in ability to control attention, and slower on measures of automatization than the control group. When the effect of methylphenidate on the cognitive styles of hyperactive children was examined, using a double blind, own control design, the drug resulted in less impulsive responding and improved ability to inhibit incorrect responses.

226 citations



Journal ArticleDOI
TL;DR: A new form of maple-syrup-urine disease in which the hyperaminoacidaemia is completely corrected by thiamine hydrochloride without recourse to dietary restriction is illustrated.

147 citations


Journal ArticleDOI
27 Aug 1971-Science
TL;DR: Serum immunoreactive parathyroid hormone (IPTH) is normal in patients with X-linked hypophosphatemic rickets who are not treated with phosphate salts and endogenous IPTH does not influence the existing defect in tubular reabsorption of phosphate in male patients.
Abstract: Serum immunoreactive parathyroid hormone(IPTH) is normal in patients with X-linked hypophosphatemic rickets who are not treated with phosphate salts. Phosphate raises IPTH in these patients. Endogenous IPTH does not influence the existing defect in tubular reabsorption of phosphate in male patients.

119 citations


Journal ArticleDOI
TL;DR: Although methylphenidate appeared to produce smaller phasic and tonic ORs, this finding may have resulted from the effect of the drug on basal conductance level rather than on the OR measures per se.
Abstract: The effects of methylphenidate on the performance of 22 hyperactive children on a delayed reaction time (RT) task were investigated using a double blind cross over design. In addition, the influence of the drug on resting basal skin conductance and heart rate, and on the skin conductance component of the orienting response (OR) to nonsignal and signal stimuli were considered. RTs were faster and less variable and task-irrelevant motor responses less frequent when hyperactives were receiving methylphenidate than when they were receiving placebo. These results were taken as evidence that the drug improved attention. Methylphenidate increased basal skin conductance and heart rate levels during relaxation and also increased basal conductance during periods of stimulation. Although methylphenidate appeared to produce smaller phasic and tonic ORs, this finding may have resulted from the effect of the drug on basal conductance level rather than on the OR measures per se.

99 citations



Journal ArticleDOI
TL;DR: Two adolescent siblings with a new form of cystinosis are distinctive because of their present age, their mild nephropathy, and the absence of retinopathy and pigmentary changes.
Abstract: Two adolescent siblings with a new form of cystinosis are distinctive because of their present age (15 and 14 years), their mild nephropathy, and the absence of retinopathy and pigmentary changes. The intracellular concentration of free cystine in their fibroblasts and leukocytes is 3 to 6 µmoles/gm protein, which is lower than in fatal infantile cystinosis and slightly higher than in the benign adult trait. The cystine is localized predominantly in the "granular" fraction. Both parents of the adolescent cystinotic siblings have excessive cystine retention in fibroblasts and leukocytes in the range which is typical of infantile cystinotic heterozygotes but cystine storage is not directly proportional to gene dosage. Dithiothreitol reduces the cystine content of "adolescent" fibroblasts, as it does in "infantile" cells. The three cystinotic traits indicate that several mutant alleles, at one or more gene loci, affect one or more gene products which control the steady-state of cystine metabolism in the cell. Clinical prognosis is apparently influenced to some extent by the severity of cystine retention.

61 citations


Journal ArticleDOI
TL;DR: The assessment of reserve albumin binding capacity is non-specific for bilirubin or for any of its competing anions and cannot distinguish between competitive and non-competitive binding to albumin.

48 citations


Journal ArticleDOI
TL;DR: Newborn Long-Evans rat pups have reduced tubular reabsorption of proline, hydroxyproline and glycine in vivo, and net uptake of amino acids, after long incubation, is usually greater in newborn kidney than in mature tissue.

44 citations


Journal ArticleDOI
TL;DR: Transport in newborn kidney by the high -K m system is relatively protected from anoxia, indicating that ontogeny affects both total and specific activity of membrane transport systems in kidney.

Journal ArticleDOI
TL;DR: Diazoxide was given intravenously in 188 hypertensive episodes to 17 children with renal disease, and it is considered that diazoxide is the drug of choice in severe hypertensive crises in children.


Journal ArticleDOI
TL;DR: It is suggested that apneic spells and/or convulsions in the initial hours following otherwise uneventful ACD exchanges may be due to this reactive hypoglycemia, an event exaggerated in infants who are hypoglycemic to begin with.

Journal ArticleDOI
TL;DR: The effects of exchange transfusion with either acid citrate dextrose (ACD) blood or fresh heparinized blood were studied, and 2-(4 hydroxybenzeneazo) Benzoic acid binding rose more strikingly with ACD blood than with hepar inized blood.

Journal ArticleDOI
TL;DR: The successful reconstruction of the aortic arch in an infant with Type A aortsic arch interruption involved the division of the left carotid, vertebral, and subclavian arteries and was followed by pulmonary artery banding for a ventricular septal defect.

Journal ArticleDOI
TL;DR: Isolated rat kidney glomeruli were used to study amino acid transport in vitro and found that α-Aminoisobutyric acid uptake does not reach a steady state, behaving as if efflux were deficient.

Journal ArticleDOI
01 Jan 1971-Steroids
TL;DR: A procedure permitting the simultaneous calculation of the isotopic content and of the mass of steroid metabolites produced upon incubation of a radioactive precursor with rat adrenal tissue was applied to the steroids produced from progesterone or 11-deoxycorticosterone in incubations of whole and of quartered adrenals, giving the following results.

Journal ArticleDOI
TL;DR: In three patients with excessive dynamic tracheal compression with forced expiration, it seemed that the tracheostomy was only an incidental event in the etiology of this condition.