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Cardiac abnormalities in neurofibromatosis. 神经纤维瘤病的心脏异常。
Neurofibromatosis Pub Date : 1988-01-01
A E Lin, K L Garver
{"title":"Cardiac abnormalities in neurofibromatosis.","authors":"A E Lin,&nbsp;K L Garver","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Cardiac abnormalities are potentially important features of neurofibromatosis (NF), but they are rarely discussed in this regard. Several literature reviews have suggested that congenital heart defects (CHD) occur with increased frequency in NF, and several isolated case reports have described hypertrophic cardiomyopathy (HCM) with NF. However, it is not clear whether the occurrence of CHD and HCM in patients with NF is coincidental or representative of an intrinsic, albeit uncommon, manifestation of the disorder. Adequate prospective studies documenting their true prevalence in NF are lacking. As an alternative, we reviewed the available relevant literature and, on that basis, have concluded that there is no clear evidence that CHD occurs with increased frequency among patients with NF, and that no statements can be made about the prevalence of HCM among patients with NF.</p>","PeriodicalId":77754,"journal":{"name":"Neurofibromatosis","volume":"1 3","pages":"146-51"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14282955","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Pathological features of optic nerve and chiasmatic gliomas. 视神经与交叉神经胶质瘤的病理特征。
Neurofibromatosis Pub Date : 1988-01-01
L J Rubinstein
{"title":"Pathological features of optic nerve and chiasmatic gliomas.","authors":"L J Rubinstein","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>This paper reviews optic pathway gliomas in terms of their incidence, gross pathologic features, microscopic features, biological behavior, malignant forms and association with neurofibromatosis.</p>","PeriodicalId":77754,"journal":{"name":"Neurofibromatosis","volume":"1 3","pages":"152-8"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14282957","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Cell culture studies on neurofibromatosis (von Recklinghausen). VI. No increased stimulation of cell proliferation by sera from neurofibromatosis patients. 神经纤维瘤病细胞培养研究(von Recklinghausen)。六、神经纤维瘤患者血清对细胞增殖的刺激不增加。
Neurofibromatosis Pub Date : 1988-01-01
K Binder, W Krone, R Hochsattel, H Gall
{"title":"Cell culture studies on neurofibromatosis (von Recklinghausen). VI. No increased stimulation of cell proliferation by sera from neurofibromatosis patients.","authors":"K Binder,&nbsp;W Krone,&nbsp;R Hochsattel,&nbsp;H Gall","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The growth-stimulating activity of sera from 10 patients with von Recklinghausen neurofibromatosis (NF-1) was compared with that of sera from 10 age- and sex-matched healthy donors. Cell cultures derived from peripheral neurofibromas and from skin biopsies of healthy persons were used as indicator cells. Stimulation of 3H-thymidine incorporation increased with the clotting time of the blood samples, reaching a plateau at about 35 h with no decline of activity during the following 48 h. There was no difference between the stimulating activity of sera from the NF-1 patients and that of sera from the healthy donors with either type of indicator cells. This held true when the indicator cells were stimulated during the exponential growth phase or when serum-starved confluent cells were exposed to the various sera. Three NF-1 strains used as indicator cells during this investigation showed a significantly less vigorous response to both types of human sera and to fetal calf serum than the respective control strains. All human sera collected after 42 h clotting time were superior to fetal calf serum with both types of indicator cells.</p>","PeriodicalId":77754,"journal":{"name":"Neurofibromatosis","volume":"1 2","pages":"85-92"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14283426","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Aqueductal stenosis in neurofibromatosis. 神经纤维瘤病中的导水管狭窄。
Neurofibromatosis Pub Date : 1988-01-01
J J Riviello, H G Marks, M S Lee, G A Mandell
{"title":"Aqueductal stenosis in neurofibromatosis.","authors":"J J Riviello,&nbsp;H G Marks,&nbsp;M S Lee,&nbsp;G A Mandell","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Macrocrania (i.e., macrocephaly) is a common manifestation of von Recklinghausen neurofibromatosis (NF-1), occurring in approximately 30% of patients with this disorder. Megalencephaly is the usual basis for NF-1-associated macrocrania, although obstructive hydrocephalus secondary to a brain tumor or cranial vault dysplasia may occur. We describe 2 patients with NF-1 and aqueductal stenosis documented by CT scan. The incidence of aqueductal stenosis among our patients with NF-1 is 1%.</p>","PeriodicalId":77754,"journal":{"name":"Neurofibromatosis","volume":"1 5-6","pages":"312-7"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14281732","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Segmental neurofibromatosis. Case report and review of the literature. 节段性神经纤维瘤。病例报告及文献复习。
Neurofibromatosis Pub Date : 1988-01-01
P G Calzavara, A Carlino, G P Anzola, M P Pasolini
{"title":"Segmental neurofibromatosis. Case report and review of the literature.","authors":"P G Calzavara,&nbsp;A Carlino,&nbsp;G P Anzola,&nbsp;M P Pasolini","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Segmental neurofibromatosis (NF) is characterized by the strictly unilateral occurrence of features that are typical of the more ordinary forms of NF (i.e., NF-1, NF-2), including schwannomas, cutaneous or plexiform neurofibromas, and/or café au lait spots. That is, these features are found in only one or several dermal segments. We describe a case of a 68-year-old woman affected by neurofibromas restricted to the right lumbar region. After extensive investigations, including magnetic resonance imaging, we could rule out the presence of additional, more widespread lesions. Previous reports of segmental NF are reviewed.</p>","PeriodicalId":77754,"journal":{"name":"Neurofibromatosis","volume":"1 5-6","pages":"318-22"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14281733","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Vascular changes in cutaneous neurofibromas. 皮肤神经纤维瘤的血管改变。
Neurofibromatosis Pub Date : 1988-01-01
F Teixeira, A Martinez-Palomo, V M Riccardi, J Fernandez-Diez
{"title":"Vascular changes in cutaneous neurofibromas.","authors":"F Teixeira,&nbsp;A Martinez-Palomo,&nbsp;V M Riccardi,&nbsp;J Fernandez-Diez","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>A variety of vascular changes has been associated with neurofibromatosis, and morphological alterations of arteries have been described previously. However, little attention has been paid to structural modifications of the microvasculature in neurofibromas themselves. Small vessels of 10 cutaneous neurofibromas, excised from patients with neurofibromatosis, were studied by using transmission electron microscopy and compared with normal skin vessels. The major alterations were: (1) increased thickness of the cytoplasm of endothelial cells which send numerous long processes to the lumen and, in some instances, obliterate it; (2) larger numbers of endothelial cell processes on the abluminal surface which are apposed to pericyte processes; (3) increased amounts of filaments and free ribosomes in the cytoplasm of endothelial cells, and (4) abundant pericytes with bundles of cytoplasmic filaments and many small subplasmalemmal densities. Mitotic figures were not seen in any of the vascular elements. These changes were interpreted to be the result of a generalized malformative process of blood vessels in neurofibromas rather than a consequence of the action of a tumor angiogenesis factor.</p>","PeriodicalId":77754,"journal":{"name":"Neurofibromatosis","volume":"1 1","pages":"5-16"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14400021","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Avoidable complications of resection of major nerve trunk neurofibromas and schwannomas. 大神经干神经纤维瘤和神经鞘瘤切除术可避免的并发症。
Neurofibromatosis Pub Date : 1988-01-01
L R Pickard, J E Rose
{"title":"Avoidable complications of resection of major nerve trunk neurofibromas and schwannomas.","authors":"L R Pickard,&nbsp;J E Rose","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Neurofibromas and schwannomas of major nerve trunks may present with a variety of symptoms and other clinical concerns. These include: (1) the question of malignancy, (2) pain and paresthesias, (3) cosmesis, and (4) symptoms and impending problems related to compression of adjacent structures. For these reasons, patients with neurofibromas and schwannomas may have valid reasons for surgery. Precise delineation of the anatomical relationships of the lesions and their location within peripheral nerve trunks is essential for decision making regarding when and how they should be excised; judgement regarding risks vs. benefits must be made carefully. Microsurgical dissection can be utilized to remove some nerve trunk lesions with preservation of most or all motor and sensory functions. An important goal in resecting benign lesions is to avoid sacrificing major motor and sensory functions. Three cases are presented to illustrate these concepts: 2 cases with significant motor loss following partial resection of large neurofibromas involving peripheral nerve trunks and 1 case with a large femoral nerve neurofibroma excised from the nerve trunk with microsurgical dissection, leading to relief of symptoms and complete preservation of motor function.</p>","PeriodicalId":77754,"journal":{"name":"Neurofibromatosis","volume":"1 1","pages":"43-9"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14283416","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Guidelines for organizing a comprehensive neurofibromatosis program. 组织一个综合性神经纤维瘤病项目的指南。
Neurofibromatosis Pub Date : 1988-01-01
V M Riccardi
{"title":"Guidelines for organizing a comprehensive neurofibromatosis program.","authors":"V M Riccardi","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>As neurofibromatosis receives increasing amounts of attention in both clinical and research terms, there is an even greater need to organize comprehensive neurofibromatosis programs that will capitalize on the advances being made. Based on the experience of the Baylor NF Program, a model for establishing and operating comprehensive neurofibromatosis programs is presented and discussed.</p>","PeriodicalId":77754,"journal":{"name":"Neurofibromatosis","volume":"1 2","pages":"105-19"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14283421","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Optic pathway gliomas. 视神经胶质瘤。
Neurofibromatosis Pub Date : 1988-01-01
{"title":"Optic pathway gliomas.","authors":"","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":77754,"journal":{"name":"Neurofibromatosis","volume":"1 4","pages":"197-251"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14281767","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
NFormation: NF information on-line. 信息:在线的NF信息。
Neurofibromatosis Pub Date : 1988-01-01
A X Safina
{"title":"NFormation: NF information on-line.","authors":"A X Safina","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":77754,"journal":{"name":"Neurofibromatosis","volume":"1 3","pages":"188"},"PeriodicalIF":0.0,"publicationDate":"1988-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"14281766","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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