Neurocognitive outcome and mental health in children with tyrosinemia type 1 and phenylketonuria:A comparison between two genetic disorders affecting the same metabolic pathway

Tyrosinemia type 1 (TT1) and phenylketonuria (PKU) are both inborn errors of phenylalanine-tyrosine metabolism. Neurocognitive and behavioral outcomes have always featured in PKU research but received less attention in TT1 research. This study aimed to investigate and compare neurocognitive, behavioral, and social outcomes of treated TT1 and PKU patients. We included 33 TT1 patients (mean age 11.24 years; 16 male), 31 PKU patients (mean age 10.84; 14 male), and 58 age- and gender-matched healthy controls (mean age 10.82 years; 29 male). IQ (Wechsler-subtests), executive functioning (the Behavioral Rating Inventory of Executive Functioning), mental health (the Achenbach-scales), and social functioning (the Social Skills Rating System) were assessed. Results of TT1 patients, PKU patients, and healthy controls were compared using Kruskal-Wallis tests with post-hoc Mann-Whitney U tests. TT1 patients showed a lower IQ and poorer executive functioning, mental health, and social functioning compared to healthy controls and PKU patients. PKU patients did not differ from healthy controls regarding these outcome measures. Relatively poor outcomes for TT1 patients were particularly evident for verbal IQ, BRIEF dimensions "working memory", "plan and organize" and "monitor", ASEBA dimensions "social problems" and "attention problems", and for the SSRS "assertiveness" scale (all p value

Ondervoeding als importziekte

SCOPUS: ar.jinfo:eu-repo/semantics/publishe

What else? Development, Gender, and Human-Rights

The issues raised in this book about globalization and commodification of the human body are extremely vast and complex, and have significant human, social, medical, economic, legal, religious, and ethical implications. It is clear from the previous chapters that the questions are many and that much more research is necessary and of crucial importance. While the four previous parts are dedicated each to one specific topic involving globalization and commodification of the human body, this par..

Le dépistage néonatal des erreurs innées du métabolisme

info:eu-repo/semantics/publishe

Nutritional problems caused by anticancer treatment in children

English AbstractJournal ArticleResearch Support, Non-U.S. Gov'tSCOPUS: NotDefined.jinfo:eu-repo/semantics/publishe

Problèmes métaboliques posés par l'inotxication a l'aspirine.

Journal Articleinfo:eu-repo/semantics/publishe

Validation d'un score d'évaluation nutritionnelle rapide. Application à 80 cas de diarrhée du retour.

Pediatricians often have to assess the nutritional status of their patients from unprecise anamnestic information, unreliable clinical signs or biological data difficult to interpret. None of these parameters can be used alone to establish the nutritional status of a sick child. The validity of a simple and rapid nutritional score was tested. The score consisted of 3 anthropometric parameters (weight, height, triceps skinfold) and 4 biological analyses frequently used in pediatric practice (albumin, transferrin, alkaline phosphatase and ferritin serum determinations). The usefulness of the score was established through the important nutritional deficiencies observed in 80 children admitted for "homing diarrhea" requiring early nutritional support.English AbstractJournal ArticleResearch Support, Non-U.S. Gov'tSCOPUS: NotDefined.jinfo:eu-repo/semantics/publishe

Aluminum and infants.

CommentLetterSCOPUS: le.jinfo:eu-repo/semantics/publishe

Humanitarian aid and health services in Eastern Kivu, Zaïre: collaboration or competition

The population of eastern Kivu, like that of Rwanda, has been surviving in critical conditions for many years. The Rwandan refugees of 1994 fled into an area with its own serious problems. The Zairean health services in the health districts of Rutshuru, Kirotshe and Masisi, in spite of the political and socioeconomic disintegration of the country, were still functioning, and the local hospitals and health centres, although overwhelmed, contributed to a large extent to the disaster response. Prominent among the major problems facing the local health services were their limited adaptability, the inadequate coordination and collaboration offered to them by the humanitarian aid agencies, and the discrimination between the direct and secondary victims of the emergency. The public health consequences of the Rwandan refugee crisis for the Zairean population constitute an integral part of the disaster

Antigenic variation of vibrio el Tor during an epidemic in Kivu.

Journal Articleinfo:eu-repo/semantics/publishe