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Plexiform neurofibromas in children with neurofibromatosis type 1: frequency and associated clinical deficits.

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Plexiform neurofibromas in children with neurofibromatosis type 1: frequency and associated clinical deficits. / Nguyen, Rosa; Kluwe, Lan; Fuensterer, Carsten; Kentsch, Michael; Friedrich, Reinhard; Mautner, Viktor Felix.

in: J PEDIATR-US, Jahrgang 159, Nr. 4, 4, 01.10.2011, S. 652-652.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Nguyen, R, Kluwe, L, Fuensterer, C, Kentsch, M, Friedrich, R & Mautner, VF 2011, 'Plexiform neurofibromas in children with neurofibromatosis type 1: frequency and associated clinical deficits.', J PEDIATR-US, Jg. 159, Nr. 4, 4, S. 652-652. https://doi.org/10.1016/j.jpeds.2011.04.008

APA

Nguyen, R., Kluwe, L., Fuensterer, C., Kentsch, M., Friedrich, R., & Mautner, V. F. (2011). Plexiform neurofibromas in children with neurofibromatosis type 1: frequency and associated clinical deficits. J PEDIATR-US, 159(4), 652-652. [4]. https://doi.org/10.1016/j.jpeds.2011.04.008

Vancouver

Nguyen R, Kluwe L, Fuensterer C, Kentsch M, Friedrich R, Mautner VF. Plexiform neurofibromas in children with neurofibromatosis type 1: frequency and associated clinical deficits. J PEDIATR-US. 2011 Okt 1;159(4):652-652. 4. https://doi.org/10.1016/j.jpeds.2011.04.008

Bibtex

@article{debcf60e66944f18a430e15e9f3a6909,
title = "Plexiform neurofibromas in children with neurofibromatosis type 1: frequency and associated clinical deficits.",
abstract = "OBJECTIVES: To define the frequency and clinical features of plexiform neurofibromas (PN) in children with neurofibromatosis type 1.STUDY DESIGN: Sixty-five children received whole-body magnetic resonance imaging (MRI) and clinical-neurologic examination. Tumor sizes were calculated volumetrically with the program MedX v3.42. χ(2) test, Fisher exact test, t test, and Spearman rank correlation were used for statistical analysis.RESULTS: Seventy-three tumors were detected in 37 of these 65 children. The mean volume of the tumors was 145.4 mL or 4.8 mL/kg body weight. Eighteen of the 73 PNs caused clinical deficits in 17 children, and the other 56 PNs in 20 children were asymptomatic. Symptomatic tumors were larger than asymptomatic ones (9.6 vs 3.3 mL/kg body weight; P = .01). However, in certain body regions, for example, the head, small tumors also caused clinical deficits. Ten of 18 children ≥11.5 years (median age of the 37 children with PNs) had symptomatic PNs compared with 7 of 19 who were <11.5 years (P = .25).CONCLUSION: PNs cause clinical deficits in young children. Early detection and regular MRI monitoring help to estimate growth and possible upcoming complications, and are thus beneficial for optimizing treatment and management.",
keywords = "Humans, Male, Female, Adolescent, Age Factors, Child, Child, Preschool, Infant, Magnetic Resonance Imaging, Body Weight, Whole Body Imaging, Musculoskeletal Diseases/etiology, Neurofibroma, Plexiform/complications/*pathology, Neurofibromatosis 1/*pathology, Pain/etiology, Reflex, Abnormal, Humans, Male, Female, Adolescent, Age Factors, Child, Child, Preschool, Infant, Magnetic Resonance Imaging, Body Weight, Whole Body Imaging, Musculoskeletal Diseases/etiology, Neurofibroma, Plexiform/complications/*pathology, Neurofibromatosis 1/*pathology, Pain/etiology, Reflex, Abnormal",
author = "Rosa Nguyen and Lan Kluwe and Carsten Fuensterer and Michael Kentsch and Reinhard Friedrich and Mautner, {Viktor Felix}",
note = "Copyright {\textcopyright} 2011 Mosby, Inc. All rights reserved.",
year = "2011",
month = oct,
day = "1",
doi = "10.1016/j.jpeds.2011.04.008",
language = "English",
volume = "159",
pages = "652--652",
journal = "J PEDIATR-US",
issn = "0022-3476",
publisher = "Mosby Inc.",
number = "4",

}

RIS

TY - JOUR

T1 - Plexiform neurofibromas in children with neurofibromatosis type 1: frequency and associated clinical deficits.

AU - Nguyen, Rosa

AU - Kluwe, Lan

AU - Fuensterer, Carsten

AU - Kentsch, Michael

AU - Friedrich, Reinhard

AU - Mautner, Viktor Felix

N1 - Copyright © 2011 Mosby, Inc. All rights reserved.

PY - 2011/10/1

Y1 - 2011/10/1

N2 - OBJECTIVES: To define the frequency and clinical features of plexiform neurofibromas (PN) in children with neurofibromatosis type 1.STUDY DESIGN: Sixty-five children received whole-body magnetic resonance imaging (MRI) and clinical-neurologic examination. Tumor sizes were calculated volumetrically with the program MedX v3.42. χ(2) test, Fisher exact test, t test, and Spearman rank correlation were used for statistical analysis.RESULTS: Seventy-three tumors were detected in 37 of these 65 children. The mean volume of the tumors was 145.4 mL or 4.8 mL/kg body weight. Eighteen of the 73 PNs caused clinical deficits in 17 children, and the other 56 PNs in 20 children were asymptomatic. Symptomatic tumors were larger than asymptomatic ones (9.6 vs 3.3 mL/kg body weight; P = .01). However, in certain body regions, for example, the head, small tumors also caused clinical deficits. Ten of 18 children ≥11.5 years (median age of the 37 children with PNs) had symptomatic PNs compared with 7 of 19 who were <11.5 years (P = .25).CONCLUSION: PNs cause clinical deficits in young children. Early detection and regular MRI monitoring help to estimate growth and possible upcoming complications, and are thus beneficial for optimizing treatment and management.

AB - OBJECTIVES: To define the frequency and clinical features of plexiform neurofibromas (PN) in children with neurofibromatosis type 1.STUDY DESIGN: Sixty-five children received whole-body magnetic resonance imaging (MRI) and clinical-neurologic examination. Tumor sizes were calculated volumetrically with the program MedX v3.42. χ(2) test, Fisher exact test, t test, and Spearman rank correlation were used for statistical analysis.RESULTS: Seventy-three tumors were detected in 37 of these 65 children. The mean volume of the tumors was 145.4 mL or 4.8 mL/kg body weight. Eighteen of the 73 PNs caused clinical deficits in 17 children, and the other 56 PNs in 20 children were asymptomatic. Symptomatic tumors were larger than asymptomatic ones (9.6 vs 3.3 mL/kg body weight; P = .01). However, in certain body regions, for example, the head, small tumors also caused clinical deficits. Ten of 18 children ≥11.5 years (median age of the 37 children with PNs) had symptomatic PNs compared with 7 of 19 who were <11.5 years (P = .25).CONCLUSION: PNs cause clinical deficits in young children. Early detection and regular MRI monitoring help to estimate growth and possible upcoming complications, and are thus beneficial for optimizing treatment and management.

KW - Humans

KW - Male

KW - Female

KW - Adolescent

KW - Age Factors

KW - Child

KW - Child, Preschool

KW - Infant

KW - Magnetic Resonance Imaging

KW - Body Weight

KW - Whole Body Imaging

KW - Musculoskeletal Diseases/etiology

KW - Neurofibroma, Plexiform/complications/pathology

KW - Neurofibromatosis 1/pathology

KW - Pain/etiology

KW - Reflex, Abnormal

KW - Humans

KW - Male

KW - Female

KW - Adolescent

KW - Age Factors

KW - Child

KW - Child, Preschool

KW - Infant

KW - Magnetic Resonance Imaging

KW - Body Weight

KW - Whole Body Imaging

KW - Musculoskeletal Diseases/etiology

KW - Neurofibroma, Plexiform/complications/pathology

KW - Neurofibromatosis 1/pathology

KW - Pain/etiology

KW - Reflex, Abnormal

U2 - 10.1016/j.jpeds.2011.04.008

DO - 10.1016/j.jpeds.2011.04.008

M3 - SCORING: Journal article

C2 - 21621223

VL - 159

SP - 652

EP - 652

JO - J PEDIATR-US

JF - J PEDIATR-US

SN - 0022-3476

IS - 4

M1 - 4

ER -