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Hamburg, Germany

Granstrom S.,University of Hamburg | Friedrich R.E.,Neurofibromatosis Laboratory | Friedrich R.E.,University of Hamburg | Langenbruch A.K.,University of Hamburg | And 2 more authors.
Anticancer Research | Year: 2014

Aim: To analyze psychosocial burdens associated with neurofibromatosis type-1 (NF1) phenotype - visible symptoms, medical complications, learning disabilities (LD) - from patients' perspective with focus on LD. Patients and Methods: A survey of 228 adult patients with NF1 was carried-out. Symptoms to estimate disease severity and visibility, and learning disability were assessed. Outcome parameters were social situation and psychosocial aspects. Results: Social situation and psychosocial aspects differed depending on NF1 phenotype. Patients with LD (n=55) were less frequently in a partnership (p=0.005) or had children (p=0.015) than those without (n=132). They also reported a higher frequency of depression (p=0.019) and sensitivity to stress (p<0.001) and more uncertainty regarding NF1-associated symptoms. These differences were significant when adjusting for disease severity and self-perceived disease visibility. Conclusion: Beside the psychosocial needs of patients with LD with NF1, medical management of this sub-group should include doctor-patient communication in easy language to compensate for patients' lack of knowledge about symptoms associated with cancer. Source

Friedrich R.E.,Neurofibromatosis Laboratory | Friedrich R.E.,University of Hamburg | Rother J.,Neurofibromatosis Laboratory | Rother J.,University of Hamburg | And 8 more authors.
Anticancer Research | Year: 2013

Neurofibromatosis type 1 (NF1) is an autosomal dominant inherited disease. Some stigmata of NF1 occur in the orbital region. The aim of this study was to reveal whether alterations of the orbital rim visible on plain radiographs may indicate the presence of a plexiform neurofibroma (PNF), a tumour almost exclusively diagnosed in NF1. Material and Methods: The plain orbital radiographs of 73 patients with NF1 (female: N=37, male: N=36) were investigated for alterations of the orbit. The group was further distinguished according to the presence of orbital PNF (N=53) and/or sphenoid wing dysplasia (N=30). Radiographs from patients with NF1 and with exclusion of PNF in the orbitofacial region were used for comparison (N=20). A special cephalometric analysis (Dental Vision) was adapted to the demands of this study. Results: Patients with NF1 not affected by an orbitofacial PNF exhibited symmetrical orbits. Unilateral increase in orbital height was associated with ipsilateral PNF. The width of orbits affected by a PNF was often slightly increased compared to the non-affected side. The determination of cephalometrically-defined angles disclosed an erection of the PNF-affected orbit compared to the medio-sagittal plane. Conclusion: Plain radiographs are often the first diagnostic measure used to determine skeletal alterations. This study shows that certain parameters of the orbital rim are useful indicators of a PNF in patients who are unilaterally affected by this lesion in the orbital or orbitotemporal region. Source

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