Diet and compliance in phenylketonuria.

Abstract:

UNLABELLED:In phenylketonuria, compliance and diet is a difficult issue; it is hard to quantify and is under-researched. It is influenced by many factors. Failure to consume prescribed quantity of protein substitute has been commonly reported and is probably affected by their acceptability, format and timing of administration. There are few reports documenting actual phenylalanine intake and blood phenylalanine control, but it is possible that the more rigorous systems for allocation of phenylalanine are associated with worse compliance. The dry, hard and insipid nature of many low protein products may lead to their under usage and consequent boredom and hunger. The diet becomes increasingly harder to maintain as children grow older and seek fewer constraints in their meals. CONCLUSION:In phenylketonuria, encouraging adherence to diet requires continual education, reinforcement and support from the family and professionals within the support team.

journal_name

Eur J Pediatr

authors

MacDonald A

doi

10.1007/pl00014375

keywords:

subject

Has Abstract

pub_date

2000-10-01 00:00:00

pages

S136-41

eissn

0340-6199

issn

1432-1076

journal_volume

159 Suppl 2

pub_type

杂志文章,评审
  • A fatal case of portal hypertension complicating systemic mastocytosis in an adolescent.

    abstract::Portal hypertension was observed in a 17-year-old girl with urticaria pigmentosa since 2 months of age. Liver biopsies showed portal and sinusoidal infiltration with mast cells although spleen biopsies showed only fibrosis. CONCLUSION. Portal hypertension is a complication of systemic mastocytosis that can lead to dea...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF01959789

    authors: Fonga-Djimi HS,Gottrand F,Bonnevalle M,Farriaux JP

    更新日期:1995-10-01 00:00:00

  • Revised recommendations for the management of Gaucher disease in children.

    abstract:UNLABELLED:Gaucher disease is an inherited pan-ethnic disorder that commonly begins in childhood and is caused by deficient activity of the lysosomal enzyme glucocerebrosidase. Two major phenotypes are recognized: non-neuropathic (type 1) and neuropathic (types 2 and 3). Symptomatic children are severely affected and m...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章,评审

    doi:10.1007/s00431-012-1771-z

    authors: Kaplan P,Baris H,De Meirleir L,Di Rocco M,El-Beshlawy A,Huemer M,Martins AM,Nascu I,Rohrbach M,Steinbach L,Cohen IJ

    更新日期:2013-04-01 00:00:00

  • Pneumomediastinum: a rare complication of anorexia nervosa in children and adolescents. A case study and review of the literature.

    abstract::Spontaneous pneumomediastinum is uncommon in paediatric practice. We describe two cases of spontaneous pneumomediastinum in a child and an adolescent with anorexia nervosa. Thorough investigation failed to reveal any underlying cause for secondary pneumomediastinum. Pneumomediastinum in anorexia nervosa can be caused ...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-007-0444-9

    authors: van Veelen I,Hogeman PH,van Elburg A,Nielsen-Abbring FW,Heggelman BG,Mahieu HF

    更新日期:2008-02-01 00:00:00

  • COVID-19 Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in children and adolescents: a systematic review of critically unwell children and the association with underlying comorbidities.

    abstract::Data show that children are less severely affected with SARS-Covid-19 than adults; however, there have been a small proportion of children who have been critically unwell. In this systematic review, we aimed to identify and describe which underlying comorbidities may be associated with severe SARS-CoV-2 disease and de...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章,评审

    doi:10.1007/s00431-020-03801-6

    authors: Williams N,Radia T,Harman K,Agrawal P,Cook J,Gupta A

    更新日期:2020-09-10 00:00:00

  • Latent chronic cholangitis in congenital hepatic fibrosis.

    abstract::In a five-year-old girl with congenital hepatic fibrosis raised erythrocyte sedimentation rate and serum gammaglobulins were noted for 3 years. Needle liver biopsy showed histological features of cholangitis and Klebsiella oxytoca was cultured from a liver biopsy specimen. Antibiotic treatment resulted in normalizatio...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF01377359

    authors: Alvarez F,Hadchouel M,Bernard O

    更新日期:1982-11-01 00:00:00

  • Influence of blood pressure level and age on within-visit blood pressure variability in children and adolescents.

    abstract::Blood pressure (BP) is variable in children and this could affect BP assessment, but the magnitude of within-visit BP variability (BPV) over consecutive measurements has never been investigated. This study aimed to determine the direction and magnitude of, and factors affecting, within-visit BPV in children and adoles...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-017-3049-y

    authors: Veloudi P,Blizzard CL,Srikanth VK,Schultz MG,Sharman JE

    更新日期:2018-02-01 00:00:00

  • A case of perineal hypospadias with paracentric inversion of chromosome 14.

    abstract::Abnormalities of chromosome 14 are encountered infrequently in clinical cytogenetics and only few studies of paracentric inversion have been published. This paper reports the first case of hypospadias associated with paracentric inversion of chromosome 14. ...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF00441748

    authors: Leung AK,Hoo JJ

    更新日期:1986-02-01 00:00:00

  • Familial hemophagocytic lymphohistiocytosis.

    abstract::Familial hemophagocytic lymphohistiocytosis (FHL) is probably a genetically transmitted disease affecting infants and very young children. Cardinal symptoms are fever, hepatosplenomegaly, and pancytopenia. Frequently meningeal involvement is seen, manifested by neurologic symptoms and a lymphohistiocytic pleocytosis w...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章,评审

    doi:10.1007/BF00443367

    authors: Janka GE

    更新日期:1983-06-01 00:00:00

  • Click-evoked oto-acoustic emission measurement in preterm infants.

    abstract:UNLABELLED:Click-evoked oto-acoustic emissions (CEOAE) are acoustic responses produced by the inner ear, reflecting functional auditory integrity. We studied both the success rate of the CEOAE method and the CEOAE presence in preterm infants during their stay at the Neonatal Intensive Care Unit (NICU), by analysis of t...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s004310050986

    authors: Brienesse P,Maertzdorf WJ,Anteunis LJ,Manni JJ,Blanco CE

    更新日期:1998-12-01 00:00:00

  • Childhood brucellosis in north-western Greece: a retrospective analysis.

    abstract:UNLABELLED:Fifty-two cases of childhood brucellosis which occurred in north-western Greece during the 15-year period 1979-1993, are reviewed. It is believed that they represent very closely the total incidence of the disease in the region which has a population of 100,000 children aged 0-14 years old. Brucellosis-affec...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF02115616

    authors: Galanakis E,Bourantas KL,Leveidiotou S,Lapatsanis PD

    更新日期:1996-01-01 00:00:00

  • Validation of a neonatal skin color scale.

    abstract::Ethnic classification does not correlate well with skin tone. As there are no neonatal skin color scales, we aimed to create and validate one of our own. After creating the scale and briefly training our staff, we conducted a prospective, observational study to assess reproducibility and correlation of each scale colo...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-020-03623-6

    authors: Maya-Enero S,Candel-Pau J,Garcia-Garcia J,Giménez-Arnau AM,López-Vílchez MÁ

    更新日期:2020-09-01 00:00:00

  • Effects of bilirubin on visual evoked potentials in term infants.

    abstract:UNLABELLED:To determine bilirubin-induced neurotoxicity, serial visual evoked potentials (VEPs) of 72 infants with neonatal hyperbilirubinaemia and 22 controls were evaluated and compared in four sessions for 8 weeks after birth. The levels of maximal serum bilirubin were found positively related to the wave latencies ...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF02079073

    authors: Chen YJ,Kang WM

    更新日期:1995-08-01 00:00:00

  • Copper and liver disease.

    abstract::There has been some limited progress in the understanding of the basic defect in Wilson disease and the gene concerned has been located to the chromosome region 13q14. Treatment with zinc has emerged as a definite alternative to penicillamine administration and some shortcomings and/or hazards of both forms of therapy...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章,评审

    doi:10.1007/BF01963553

    authors: Danks DM

    更新日期:1991-01-01 00:00:00

  • Everything the pediatrician ever wanted to know about HLA but was afraid to ask.

    abstract::Following a description of the genetic aspects of the human histocompatibility antigens system HLA and its principle typing methods, this paper reviews the relationship between HLA antigens, transplantation immunology and certain diseases. In particular, the role of the lymphocyte-defined antigens of the HLA-D system ...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章,评审

    doi:10.1007/BF00441576

    authors: Passarge E,Valentine-Thon E

    更新日期:1980-03-01 00:00:00

  • Osteoarticular infections due to Kingella kingae in children.

    abstract::By the description of two cases of osteoarticular infections due to Kingella kingae in two young children we wish to draw the attention of clinicians to invasive infections due to this micro-organism. Since its biological characterization in 1976, K. kingae has been increasingly reported as a human pathogen. Most comm...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章,评审

    doi:10.1007/BF02072618

    authors: Lacour M,Duarte M,Beutler A,Auckenthaler R,Suter S

    更新日期:1991-07-01 00:00:00

  • Sciatic nerve palsy complicating umbilical arterial catheterization.

    abstract::A term newborn infant developed a right sciatic nerve palsy after ischaemic necrosis of the gluteal region following umbilical arterial catheterization. The nerve lesion was believed to be caused by entrapment and compression by scar tissue. Recovery was slow and remained incomplete up to 6 months of age. ...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF00439407

    authors: Fok TF,Ha MH,Leung KW,Wong W

    更新日期:1986-09-01 00:00:00

  • Design of a multiple longitudinal study of growth and health in teenagers.

    abstract::This paper describes the design of a study to follow the development of boys and girls in secondary schools from the age of 12 through 17 on an annual basis, in order to acquire more information concerning the growth and development of teenagers. In this study, both physical and psychological characteristics are measu...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF00442158

    authors: Kemper HC,van 't Hof MA

    更新日期:1978-10-12 00:00:00

  • Clinical and laboratory features of children with community-acquired pneumonia are associated with distinct radiographic presentations.

    abstract::Chest radiographs from children with community-acquired pneumonia (CAP) were categorized into three distinct presentations and each presentation was correlated to clinical and laboratory findings. Children < 59 months with CAP presenting to pediatric emergency rooms during two years were enrolled prospectively in eigh...

    journal_title:European journal of pediatrics

    pub_type: 临床试验,杂志文章,多中心研究

    doi:10.1007/s00431-018-3165-3

    authors: Falup-Pecurariu OG,Diez-Domingo J,Esposito S,Finn A,Rodrigues F,Spoulou V,Syrogiannopoulos GA,Usonis V,Greenberg D,CAP-PRI.

    更新日期:2018-07-01 00:00:00

  • Deficiency of vitamins E and A in cystic fibrosis is independent of pancreatic function and current enzyme and vitamin supplementation.

    abstract:UNLABELLED:The aim of this study was to evaluate to what extent serum vitamins A and E cystic fibrosis are affected by the underlying disease, pancreatic sufficiency or insufficiency, meconium ileus, nutritional status, age and treatment (enzyme and vitamin supplementation). Serum vitamin A and E levels were determined...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF02002713

    authors: Lancellotti L,D'Orazio C,Mastella G,Mazzi G,Lippi U

    更新日期:1996-04-01 00:00:00

  • A developmental approach to the management of children with sleep disturbances in the first three years of life.

    abstract::A developmental approach to the management of sleep disturbances was successful in 85% (44/52) of the children (aged 2-36 months) within 2-6 weeks. No drugs were used. The approach was based on the following two principles: taking into account the characteristics of sleep behavior in infants and young children by sett...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF00442443

    authors: Largo RH,Hunziker UA

    更新日期:1984-08-01 00:00:00

  • Stress response and procedural pain in the preterm newborn: the role of pharmacological and non-pharmacological treatments.

    abstract:UNLABELLED:Repeated invasive procedures occur routinely in neonates who require intensive care, causing pain at a time when it is developmentally unexpected. Multiple lines of evidence suggest that repeated and prolonged pain exposure alters their subsequent pain processing, long-term development, and behaviour. Primar...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章,随机对照试验

    doi:10.1007/s00431-011-1655-7

    authors: Gitto E,Pellegrino S,Manfrida M,Aversa S,Trimarchi G,Barberi I,Reiter RJ

    更新日期:2012-06-01 00:00:00

  • IgG subclass deficiency in children with recurrent bronchitis.

    abstract::We studied the incidence of IgG subclass deficiency in children with recurrent bronchitis. Recurrent bronchitis was defined as three or more episodes a year during at least 2 consecutive years, of bronchopulmonary infection, productive cough with or without fever and/or diffuse râles by physical examination in the abs...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF02072228

    authors: DeBaets F,Kint J,Pauwels R,Leroy J

    更新日期:1992-04-01 00:00:00

  • Late renal sequelae in intravenously treated complicated urinary tract infection.

    abstract:BACKGROUND:The treatment of complicated urinary tract infection in children is still a matter of debate. In our hospital, antimicrobial treatment is initiated intravenously, and the duration of this treatment is adapted according to the results of a Tc-99m dimercaptosuccinic acid (DMSA) scintigraphy. AIM:This study wa...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-013-2024-5

    authors: Ferreiro C,Piepsz A,Nogarède C,Tondeur M,Hainaut M,Levy J

    更新日期:2013-09-01 00:00:00

  • Cardiac involvement in glycogen storage disease type III.

    abstract::Twenty patients with enzymatically proven glycogen storage disease type III (GSD III) aged 3-30 years underwent cardiological evaluation. Seventeen showed subclinical evidence of cardiac involvement in form of ventricular hypertrophy on ECG. Of 16 patients in whom an ECG examination was performed, 13 had abnormal echo...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF00443106

    authors: Moses SW,Wanderman KL,Myroz A,Frydman M

    更新日期:1989-08-01 00:00:00

  • Growth, puberty and hypothalamic-pituitary function in children with suprasellar arachnoid cyst.

    abstract:UNLABELLED:A suprasellar arachnoid cyst may cause disorders of growth, puberty and hypothalamic-pituitary function, due to the proximity of the cyst to the hypothalamic-pituitary area. A total of 30 patients (17 boys) with cyst diagnosed at 4.3 +/- 1 years were routinely evaluated at 5.4 +/- 1 years; 24 of them had one...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s004310051285

    authors: Adan L,Bussières L,Dinand V,Zerah M,Pierre-Kahn A,Brauner R

    更新日期:2000-05-01 00:00:00

  • Heme oxygenase-1 expression in premature and mature neonates during the first week of life.

    abstract::Newborns are exposed to mechanical and oxidative stress during labor and to relative hyperoxia thereafter during the course of adaptation to the extrauterine conditions. Part of the adaptation mechanism is the rapid degradation of fetal hemoglobin and the oxidation of its heme moiety by heme oxygenases (HOs). Heme oxy...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-006-0375-x

    authors: Maróti Z,Katona M,Orvos H,Németh I,Farkas I,Túri S

    更新日期:2007-10-01 00:00:00

  • Tetra-amelia with multiple malformations in six male fetuses of one kindred.

    abstract::An Arab Moslem kindred is reported in which six abnormal male fetuses were born in three closely related sibships. They had amelia, malformed head and other severe skeletal and visceral malformations. The similarities and differences between the developmental and inherent characteristics found in our patients and in f...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF00441792

    authors: Zimmer EZ,Taub E,Sova Y,Divon MY,Pery M,Peretz BA

    更新日期:1985-11-01 00:00:00

  • The utility of FDG PET in diagnosis and follow-up of lymphoma in childhood.

    abstract::Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL) are among the most common malignancies of childhood. (18)F-fluorodeoxyglucose (FDG) positron emission tomography (PET) can be employed for accurate staging, treatment planning, and response assessment in pediatric patients with lymphomas, taking advantage of the inc...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章,评审

    doi:10.1007/s00431-013-1993-8

    authors: Sioka C

    更新日期:2013-06-01 00:00:00

  • Analysis of medication prescribing errors in critically ill children.

    abstract:UNLABELLED:Medication prescribing errors (MPE) can result in serious consequences for patients. In order to reduce errors, we need to know more about the frequency, the type and the severity of such errors. We therefore performed a prospective observational study to determine the number and type of medication prescribi...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-015-2542-4

    authors: Glanzmann C,Frey B,Meier CR,Vonbach P

    更新日期:2015-10-01 00:00:00

  • Invasive pneumococcal disease in children: geographic and temporal variations in incidence and serotype distribution.

    abstract:UNLABELLED:We examined published studies from the United States, Europe, and Latin America to better understand geographic and temporal variability in the epidemiology of invasive pneumococcal disease (IPD) in young children. A comparison of IPD incidence levels reported for children <5 years of age within the United S...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-002-1066-x

    authors: Hausdorff WP

    更新日期:2002-12-01 00:00:00