Loss of Intellectual Function in Children with Phenylketonuria After Relaxation of Dietary Phenylalanine Restriction

PEDIATRICS ◽  
1985 ◽  
Vol 75 (2) ◽  
pp. 226-232
Author(s):  
Margretta R. Seashore ◽  
Estelle Friedman ◽  
Robert A. Novelly ◽  
Vijaya Bapat
Keyword(s):  
Dietary Therapy ◽  
Dietary Control ◽  
Restricted Diet ◽  
Intelligence Scale ◽  
Cognitive Problem ◽  
Intellectual Function ◽  
Developmental Age ◽  
The Mean ◽  
Initiation Of Therapy ◽  
Serum Phenylalanine

Fourteen patients with classic phenylketonuria (PKU) were treated with a phenylalanine restricted diet from early infancy. All had satisfactory dietary control, with serum phenylalanine concentrations ranging between 2 to 5 mg/dL. Dietary restriction was discontinued in all these children between ages 5 and 6 years, and a free diet allowed. Developmental testing was performed using the Cattell Infant Intelligence Scales (1 to 2 years), Stanford-Binet Intelligence Scale (2 to 4 years), Wechsler Intelligence Scale for Children (WISC) and the revised version (WISC-R) (<5 years). Mean IQ for the group (Stanford-Binet and WISC) at termination of dietary therapy was 104 ± 13. Four to 7 years after discontinuation of dietary therapy, mean IQ for the group was 90 ± 13. The severity correlated, to some degree, with duration of unrestricted diet, but not with initial serum phenylalanine concentrations, age at initiation of therapy, or IQ at time diet was discontinued. Several children are experiencing difficulties, both attentional and academic, in school. Two children have had a change in the EEG from normal to abnormal. Neurologic testing performed after 4 to 7 years off diet demonstrated deficits in visual-motor integration or cognitive problem-solving in most children. The mean developmental age for the group for perceptual-motor integration was 1.2 years below the mean chronologic age of the group. This deterioration in intellectual function suggests that discontinuation of the phenylalanine-restricted diet is hazardous for some children with classic phenylketonuria.

Intellectual Performance of 36 Phenylketonuria Patients and Their Nonaffected Siblings

PEDIATRICS ◽  
1976 ◽  
Vol 58 (1) ◽  
pp. 53-56
Author(s):  
James C. Dobson ◽  
Emily Kushida ◽  
Malcolm Williamson ◽  
Eva G. Friedman
Keyword(s):  
Mean Difference ◽  
Matched Pair ◽  
Dietary Therapy ◽  
Intellectual Impairment ◽  
Intelligence Scale ◽  
Intellectual Performance ◽  
The Mean ◽  
Normal Controls

Thirty-six patients with a confirmed diagnosis of phenylketonuria (PKU) were identified and placed on dietary therapy before reaching 121 days of age. These children are currently between 4 and 6 years old, and have been given the Stanford-Binet Intelligence Scale. Subsequently, their normal siblings of closest age were selected as matched-pair controls and were also tested with the Staford-Binet. The mean age of the PKU sample when tested was 50.0 months, and 50.9 months for the normal controls. The 36 index patients scored a mean IQ of 94 and their nonaffected siblings obtained a mean IQ of 99. This five-point mean difference was significant at the .02 level, and suggests the presence of a minimal intellectual impairment associated with PKU, even when treatment begins early and is rigorously monitored.

Phenylketonuria: Implications of Initial Serum Phenylalanine Levels on Cognitive Development

1988 ◽  
Vol 63 (1) ◽  
pp. 135-142 ◽  
Author(s):  
A. Barclay ◽  
O. Walton
Keyword(s):  
Cognitive Development ◽  
Age At Onset ◽  
Blood Levels ◽  
Dietary Control ◽  
Biochemical Control ◽  
Phenylalanine Level ◽  
Initiation Of Therapy ◽  
Serum Phenylalanine

This study investigated influences of diagnostic phenylalanines, age at initiation of diet, and dietary control, separately and in combination, on cognitive development for 29 phenylketonuric children (17 boys and 12 girls) who ranged in age from 1 to 1,184 days. Initial serum phenylalanine concentrations and age at initiation of therapy were significantly related to cognitive development, whereas neither dietary and biochemical control relative to blood levels of phenylalanine or for these variables in combination exerted a statistically significant effect on measures of functioning. The interaction between initial serum phenylalanine level and age at onset of diet did reach nearly significant proportions, suggesting that the relation between diagnostic phenylalanines and subsequent cognitive development in the phenylketonuric population warrants further study. Despite an approximately 13-point decrease in IQs between initial (1 yr.) and most recent measurements (3 yr.), all 29 children were functioning in the normal or near-normal intellectual range at the most recent testing.

Intellectual Assessment of 111 Four-Year-Old Children With Phenylketonuria

PEDIATRICS ◽  
1977 ◽  
Vol 60 (6) ◽  
pp. 822-827
Author(s):  
James C. Dobson ◽  
Malcolm L. Williamson ◽  
Colleen Azen ◽  
Richard Koch
Keyword(s):  
Psychosocial Factors ◽  
Collaborative Study ◽  
Dietary Treatment ◽  
Dietary Therapy ◽  
Intelligence Scale ◽  
Intellectual Assessment ◽  
Treatment Groups ◽  
Serum Phenylalanine ◽  
Low Serum

Of the 216 children with phenylketonuria (PKU) who were initially enrolled in the Collaborative Study of Children Treated for Phenylketonuria, 203 were placed on dietary therapy between 3 and 92 days of age. Of these, 111 are now at least 4 years of age and constituted the sample for the present analysis. Their mean IQ on the Stanford Binet Intelligence Scale was 93 (1972 norms). The children assigned to two treatment groups based on "moderate" and "low" serum phenylalanine levels were comparable on their IQs at age 4, although many of the children could not be maintained in the specified categories. Females scored a significantly higher mean IQ than males (97 vs. 90). Those children for whom dietary treatment was initiated during the first month of life scored a mean IQ of 95, compared with 85 for those initially treated from 31 to 65 days. However, the interpretation of dietary inception data may have been contaminated by familial and psychosocial factors. The PKU Collaborative Study is still in progress in 15 clinics located in 11 states.

Rorschach Characteristics in Adolescents with Systemic Lupus Erythematosus

2006 ◽  
Vol 28 (1) ◽  
pp. 100-118 ◽  
Author(s):  
J. Santoantonio ◽  
L. Yazigi ◽  
E. I. Sato
Keyword(s):  
Lupus Erythematosus ◽  
Case Control Study ◽  
Activity Index ◽  
Disease Activity Index ◽  
Female Adolescents ◽  
Control Group ◽  
Intelligence Scale ◽  
The Mean ◽  
Experimental Group ◽  
Control Study

The purpose of this study was to investigate the personality characteristics in adolescents with SLE. The research design is a case-control study by means of the Rorschach Method and the Wechsler Intelligence Scale. Study group: 30 female adolescents with lupus, 12–17 years of age. The SLE Disease Activity Index was administered during the period of psychological evaluation. Control group: 32 nonpatient adolescents were matched for age, sex, and socioeconomic level. In the Wechsler Intelligence Scale the mean IQ of the experimental group was significantly lower than that of the control group (77 and 98, respectively, p < .001). In the Rorschach, the lupus patients showed greater difficulty in interpersonal interactions, although they displayed the resources to process affect and to cope with stressful situations. A positive moderate correlation (p = .069) between the activity index of the disease and the affect constriction proportion of the Rorschach was observed: the higher the SLEDAI score, the lower the capacity to process affect. There is a negative correlation between the activity index of the disease and the IQ (p = .001): with a higher activity index of the disease, less intellectual resources are available.

Evaluation of Low-Dose Gentamicin Once Daily Dosing Regimen at A General Hospital in Malaysia

2018 ◽  
Vol 9 (01) ◽  
Author(s):  
Ab Rahman A F ◽  
Md Sahak N. ◽  
Ali A. M.
Keyword(s):  
Medical Records ◽  
Public Hospital ◽  
Low Dose ◽  
Dosing Regimen ◽  
Once Daily ◽  
Neutropenic Sepsis ◽  
Wide Acceptance ◽  
The Mean ◽  
Initiation Of Therapy ◽  
Almost All

Objective: Once daily dosing (ODD) aminoglycoside is gaining wide acceptance as an alternative way of dosing. In our setting it is the regimen of choice whenever gentamicin is indicated. The objective of this study was to evaluate the practice of gentamicin ODD in a public hospital in Malaysia. Methods: We conducted a retrospective review of medical records of patients on gentamicin ODD who were admitted to Hospital Melaka during January 2002 until March 2010. All adult patients who were on ODD gentamicin with various level of renal function were included in the study. Patients on gentamicin less than 72 hours and pregnant women were excluded. Results: From 110 patients, 75 (68.2%) were male and 35 (31.8%) were female. Indications for ODD gentamicin included pneumonia, 34 (31.0%) neutropenic sepsis, 27 (24.5%) and sepsis, 11 (10.0%). The mean dose and duration of gentamicin was 3.2 mg/kg/day and 7 days, respectively. Almost all patients were on gentamicin combined with other antibiotics. Clinical cure based on fever resolution was found in 89.1% of patients treated with ODD. Resolution of fever took an average of 48 hours after initiation of therapy. The evaluation for bacteriologic cure could not be performed because of insufficient data on culture and sensitivity. Out of 38 patients with analyzable serum creatinine data, four patients might have developed nephrotoxicity. Conclusion: In our setting, lower dosages of ODD gentamicin when used in combination with other antibiotics seemed to be effective and safe in treating most gram negative infections.

Can maintaining cognitive function at 65 years old predict successful ageing 6 years later? The PROOF study

2011 ◽  
Vol 40 (2) ◽  
pp. 259-265 ◽  
Author(s):  
Karine Castro-Lionard ◽  
Catherine Thomas-Antérion ◽  
Emilie Crawford-Achour ◽  
Isabelle Rouch ◽  
Béatrice Trombert-Paviot ◽  
...  
Keyword(s):  
Cognitive Abilities ◽  
Well Being ◽  
Successful Ageing ◽  
Adult Intelligence Scale ◽  
Intelligence Scale ◽  
Wechsler Adult Intelligence Scale ◽  
Benton Visual Retention Test ◽  
The Mean ◽  
Wechsler Adult Intelligence

Abstract Background: preservation of cognitive abilities is required to have a good quality of life. The predictive value of cognitive functioning at 65 years old on successful ageing 6 years later is not established. Methods: nine hundred and seventy-six questionnaires were sent by mail to a sample of healthy and voluntary French pensioners. Successful ageing was defined through health status and well-being. Cognitive abilities had been assessed 6 years earlier according to an objective method (Free and Cued Selective Recall Reminding Test (FCSRT), the Benton visual retention test and the similarities subtest of the Wechsler Adult Intelligence Scale-Revised) and a subjective one (Goldberg's anxiety scale, Mac Nair's scale and a Visual Analogue Scale to evaluate memory abilities change in the last 5 years). Results: six hundred and eighty-six questionnaires could be analysed. The mean age was 72.9 ± 1.2 years old with 59% of women and 99% lived at home. Well-being was negatively correlated with the FCSRT (r = −0.08, P = 0.0318) but positively related with the Benton (r = 0.09, P = 0.0125) and the similarities tests (r = 0.09, P = 0.0118). There is a negative correlation between anxious and cognitive complaints measured at baseline, and successful ageing indicators 6 years later. Conclusion: preservation of cognitive abilities at the age of retirement can predict a successful ageing 6 years later. ClinicalTrials.gov Identifier: NCT00759304.

Wisc Factor Structure for Normal Negro Pre-School Children

1964 ◽  
Vol 15 (2) ◽  
pp. 543-548 ◽  
Author(s):  
R. Travis Osborne
Keyword(s):  
Factor Analysis ◽  
School Children ◽  
Digit Span ◽  
Block Design ◽  
School Level ◽  
School Factors ◽  
Intelligence Scale ◽  
Picture Arrangement ◽  
The Mean ◽  
Intercorrelation Matrix

In order to estimate the dimensionality of the Wechsler Intelligence Scale for Children, factor analysis was applied to a 30 by 30 intercorrelation matrix of the WISC and four reference tests. The 10 standard WISC subtests, except Coding, were split into two, three, or four parts to yield as many variables as possible. Ss, were 111 Negro pre-school children; mean age, 6 yr. 1 mo.; the mean full scale IQ was 84. Evidence is presented supporting 10 statistically significant orthogonal dimensions. Not all factors are perfectly congruent with the subtest structure of the WISC or concordant with the results of prior factorizations of the WISC at the pre-school level. There is no factor for Block Design apart from Picture Arrangement. The Digit Span subtest splits involve three different WISC factors, two are from the performance section of the test and one is from the verbal section. Coding is involved in only one factor; Manipulation of Areas, one of the non-verbal reference tests. At least 7 of the 10 significant pre-school factors are readily identified by WISC subtests or combinations of WISC subtests.

The Maternal Phenylketonuria International Study: 1984–2002

PEDIATRICS ◽  
2003 ◽  
Vol 112 (Supplement_4) ◽  
pp. 1523-1529 ◽  
Author(s):  
Richard Koch ◽  
William Hanley ◽  
Harvey Levy ◽  
Kim Matalon ◽  
Reuben Matalon ◽  
...  
Keyword(s):  
Outcome Measures ◽  
Birth Outcomes ◽  
International Study ◽  
Care Program ◽  
Nonparametric Test ◽  
Maternal Blood ◽  
Fetal Outcome ◽  
Dietary Control ◽  
Maternal Phenylketonuria ◽  
Restricted Diet

Objective. The purpose of this report is to review the obstetric medical, psychological, and nutritional aspects and outcome of the women and offspring enrolled in the Maternal Phenylketonuria Study, which was established to assess the efficacy of a phenylalanine (Phe)-restricted diet in preventing the morbidity associated with this disorder. Methods. A total of 382 women with hyperphenylalaninemia (HPA) were enrolled in the study and completed 572 pregnancies. Outcome measures were analyzed with χ2, Fisher exact text, analysis of variance, t test, Wilcoxon nonparametric test, and multiple logistic regression. Outcome measures were stratified according to maternal HPA classification and the time when dietary control was achieved. Results. Optimal birth outcomes occurred when maternal blood Phe levels between 120 and 360 μmol/L were achieved by 8 to 10 weeks of gestation and maintained throughout pregnancy (trimester averages of 600 μmol/L). Mothers with mild HPA achieved similar birth outcomes as mothers who were in control preconceptually and those in control by 8 to 10 weeks of pregnancy. Conclusions. Before conception, counseling and early entrance into a prenatal care program is essential in achieving optimal fetal outcome in women with HPA. The achievement of pre- and periconceptional dietary control with a Phe-restricted diet significantly decreased morbidity in the offspring of women with HPA.

scholarly journals Influence of restricting the diet on breeding in genetically obese mice

1972 ◽  
Vol 6 (1) ◽  
pp. 9-18 ◽  
Author(s):  
A. Eastcott
Keyword(s):  
Food Intake ◽  
Optimal Conditions ◽  
Obese Mice ◽  
Male Mice ◽  
Restricted Diet ◽  
The Mean ◽  
Set Up ◽  
Mean Time ◽  
Obese Male

A system for breeding from obese male mice ( ob/ob) by restricting food intake is described. It is shown that a 40% reduction in food intake at 6&frac12;-8&frac12; weeks of age is optimal in the colony under review. Males on restricted diet did not mate immediately but went through a period of adaptation; the mean time before vaginal plugs were observed was 30 days. Subsequent vaginal plugs occurred on average every 17 days and 69% of these matings resulted in litters. Under optimal conditions over 90% of all obese males set up on restricted diets mated and sired young.

Individual Differences and Reliability of Information Transmission in Absolute Judgments of Loudness, Brightness and Line Lengths

1974 ◽  
Vol 39 (1) ◽  
pp. 239-246 ◽  
Author(s):  
Stephen G. Landau ◽  
Monte S. Buchsbaum ◽  
Richard Coppola ◽  
Miriam Sihvonen
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Information Transmission ◽  
Digit Span ◽  
Line Length ◽  
Intelligence Scale ◽  
Transmission Of Information ◽  
Processing Facility ◽  
The Central Nervous System ◽  
The Mean

Information transmission, as estimated from absolute judgments of loudness, brightness and line length, was measured in 35 normal Ss. Each S was tested on all modalities on each of three days. Individuals varied widely in their ability to transmit information and were consistent across modalities and days; reliabilities for loudness, brightness and line length between Days 2 and 3 were 0.72, 0.81, and 0.89 respectively. The mean intercorrelation between modalities was 0.40. WAIS Digit Span but no other intelligence scale was positively correlated with transmission of information. The results suggest the existence of a single information-processing facility in the central nervous system.

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