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J Invest Dermatol ; 130(9): 2173-8, 2010 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-20428190


NF-1 is associated with a 15-year decrease in life expectancy. Internal neurofibromas are associated with increased morbidity and mortality through malignant transformation and compression of neighboring organs. Our purpose was to develop and to validate a clinical score for predicting internal neurofibromas in adults. The development sample comprised 208 patients and the validation sample 191 patients. The score was developed using logistic regression. Discrimination and calibration of the model were evaluated. Four variables were independently associated with internal neurofibromas: at least two subcutaneous neurofibromas (odds ratio (OR)=4.7, [2.1-10.5]), age < or =30 years (OR=3.1, [1.4-6.8]), absence of cutaneous neurofibromas (OR=2.6, [0.9-7.5]), and fewer than six café-au-lait spots (OR=2.0 [0.9-4.6]). The score computed by linear combination of the rounded coefficients of these four variables ranged from 0 to 40 (mean, 12.8+/-10.8). The probability of internal neurofibromas was computed as exp (-2.93+0.11Score)/exp (1+(-2.93+0.11Score)). Probabilities agreed well with the observed frequencies indicating good calibration, and discrimination was adequate (AUC-ROC, 0.75) in both data sets. The presence of internal neurofibromas can be accurately predicted using a simple clinical score. Further work will establish the score threshold that identifies patients at high risk for complications.

Neurofibromatose 1/mortalidade , Neurofibromatose 1/patologia , Exame Físico/normas , Índice de Gravidade de Doença , Adulto , Idoso , Manchas Café com Leite/mortalidade , Manchas Café com Leite/patologia , Feminino , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Morbidade , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Fatores de Risco , Sensibilidade e Especificidade , Tela Subcutânea/patologia , Adulto Jovem
Orphanet J Rare Dis ; 5: 3, 2010 Feb 03.
Artigo em Inglês | MEDLINE | ID: mdl-20128925


OBJECTIVE: To document the epidemiological, clinical, histological and radiological characteristics of aggressive vascular abnormalities of bone in children. STUDY DESIGN: Correspondents of the French Society of Childhood Malignancies were asked to notify all cases of aggressive vascular abnormalities of bone diagnosed between January 1988 and September 2009. RESULTS: 21 cases were identified; 62% of the patients were boys. No familial cases were observed, and the disease appeared to be sporadic. Mean age at diagnosis was 8.0 years [0.8-16.9 years]. Median follow-up was 3 years [0.3-17 years]. The main presenting signs were bone fracture (n = 4) and respiratory distress (n = 7), but more indolent onset was observed in 8 cases. Lung involvement, with lymphangiectasies and pleural effusion, was the most frequent form of extraosseous involvement (10/21). Bisphosphonates, alpha interferon and radiotherapy were used as potentially curative treatments. High-dose radiotherapy appeared to be effective on pleural effusion but caused major late sequelae, whereas antiangiogenic drugs like alpha interferon and zoledrenate have had a limited impact on the course of pulmonary complications. The impact of bisphosphonates and alpha interferon on bone lesions was also difficult to assess, owing to insufficient follow-up in most cases, but it was occasionally positive. Six deaths were observed and the overall 10-year mortality rate was about 30%. The prognosis depended mainly on pulmonary and spinal complications. CONCLUSION: Aggressive vascular abnormalities of bone are extremely rare in childhood but are lifethreatening. The impact of anti-angiogenic drugs on pulmonary complications seems to be limited, but they may improve bone lesions.

Doenças Ósseas/epidemiologia , Malformações Vasculares/epidemiologia , Doenças Ósseas/diagnóstico , Doenças Ósseas/tratamento farmacológico , Criança , Difosfonatos/uso terapêutico , Feminino , França/epidemiologia , Humanos , Interferon-alfa/uso terapêutico , Estimativa de Kaplan-Meier , Masculino , Estudos Retrospectivos , Malformações Vasculares/diagnóstico , Malformações Vasculares/tratamento farmacológico
Hist Sci Med ; 39(3): 285-90, 2005 Jul-Sep.
Artigo em Francês | MEDLINE | ID: mdl-17152774


Four practitioners, Victor Menard, François Calot, Jacques Calvé, Etienne Sorel were the prominent surgeons in the Hospital of Berck sur Mer. They tried to set a real resistance against the terrible disease : the articular tuberculosis. Each of them proposed a means either of diagnosis or classification or surgical treatment.

Ortopedia/história , França , História do Século XIX , História do Século XX , Hospitais Especializados/história , Humanos , Doenças Musculoesqueléticas/história , Doenças Musculoesqueléticas/terapia