Clinical Risk Factors for Mortality in Patients With Neurofibromatosis 1

Khosrotehrani, Kiarash; Bastuji‐Garin, Sylvie; Zeller, J; Revuz, J; Wolkenstein, Pierre

doi:10.1001/archderm.139.2.187

Cited by 50 publications

(46 citation statements)

References 19 publications

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“…Subcutaneous neurofibromas often appear during childhood. Even though subcutaneous neurofibromas are linked to more severe cases [6] , and are often symptomatic, they were not associated with early diagnosis in our study. Numerous neurofibromas and large CLM ( 6 15 mm) were associated with early diagnosis, small CLM were not.…”

Section: Methodscontrasting

confidence: 65%

Age at Diagnosis of Neurofibromatosis 1: An Audit of Practice

et al. 2008

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Section: Methodscontrasting

confidence: 65%

Age at Diagnosis of Neurofibromatosis 1: An Audit of Practice

et al. 2008

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“…In the MRI study of the spine in children with NF1, characteristics found more often in patients with than without internal neurofibromas included scoliosis (71.4% vs. 30.4%), subcutaneous neurofibromas (71.4% vs. 39.1%), and plexiform neurofibromas (28.6% vs. 8.7%); none of these differences was statistically significant, ( P = 0.08, P = 0.22, and P = 0.17, respectively), possibly because statistical power was limited [28]. Furthermore, patients with subcutaneous neurofibromas were at higher risk for mortality in two different NF1 populations, from France [33] and North America [21], respectively. Finally, xanthogranulomas were independently associated with internal neurofibromas in our study.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics predicting internal neurofibromas in 357 children with neurofibromatosis-1: results from a cross-selectional study

Sbidian

Hadj‐Rabia

Riccardi³

et al. 2012

Orphanet J Rare Dis

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ObjectiveTo identify clinical characteristics associated with internal neurofibromas in children with NF1, as a means of ensuring the early identification of patients at high risk for malignant peripheral nerve-sheath tumors developed from preexisting internal neurofibromas.Patients and methodsWe used data from two NF1 populations, in France and North America, respectively. The French database comprised 1083 patients meeting NIH diagnostic criteria for NF1 and the Neurofibromatosis Institute Database of North America comprised 703 patients. Patients younger than 17 years of age were eligible for our study if they had been evaluated for internal neurofibromas using computed tomography and/or magnetic resonance imaging. Clinical characteristics associated with internal neurofibromas by univariate analysis (P ≤ 0.15) were entered into a multiple logistic regression model after checking for potential interactions and confounding. Multiple imputation was used for missing values.ResultsAmong the 746 children in the two databases, 357 (48%) met our inclusion criteria. Their mean age was 7.7 ± 5.0 years and there were 192 (53.8%) males. Internal neurofibromas were present in 35 (9.8%) patients. Internal neurofibromas developed earlier in females than in males and their prevalence increased during adolescence. Factors independently associated with internal neurofibromas were age (OR = 1.16 [1.07-1.27]), xanthogranulomas (OR = 5.85 [2.18-15.89]) and presence of both subcutaneous and plexiform neurofibromas (OR = 6.80 [1.52-30.44]).ConclusionsSeveral easily recognizable clinical characteristics indicate a high risk of internal neurofibromas in children with NF1 and, therefore, a need for very close monitoring.

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“…Bu olgularda 14'ünün anne baba veya kardeşinde NF-1 tanısı mevcuttu. Yapılan çalışmalarda %39-%54 arasında değişen farklı oranlarda pozitif aile öyküsü saptanmıştır [6][7][8] . Belirlediğimiz %28.6 oran literatür verileri ile uyumlu olduğu gözlendi.…”

Section: Discussionunclassified

“…Kutanöz ve subkutanöz nörofibromlar benign tümörler olup NF-1 olgularda görülme sıklıkları yaşla birlikte artar [1][2][3][4][5][6][7][8][9][10] . Çalışmamızda 10 olguda subkutan nörofibrom tespit edildi.…”

Section: Discussionunclassified

Neurofibromatosis type-1: Evaluation of 49 cases

Çarman¹

2017

Haydarpasa Numune Med J

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Clinical Risk Factors for Mortality in Patients With Neurofibromatosis 1

Cited by 50 publications

References 19 publications

Age at Diagnosis of Neurofibromatosis 1: An Audit of Practice

Age at Diagnosis of Neurofibromatosis 1: An Audit of Practice

Clinical characteristics predicting internal neurofibromas in 357 children with neurofibromatosis-1: results from a cross-selectional study

Neurofibromatosis type-1: Evaluation of 49 cases

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