ABSTRACT
UNLABELLED: The aim of this study was to analyse the outcome of optic pathway gliomas in 30 children with neurofibromatosis type 1, the indications of treatment, and the follow-up and screening protocol. PATIENTS AND METHODS: All patients with a minimal two years follow-up (median six years, range two to 19 years), in two multidisciplinary consultations of Saint-Vincent-de-Paul (Paris) and Purpan (Toulouse) hospitals, were included in the study. In our series, we practiced systematic screening MRI in children under six years' of age or with neuropsychological deficiency that may imply an unreliable ophthalmological examination. RESULTS: Thirty-seven percent (11 patients) had progressive ophthalmological signs and were treated, and 63% (19 patients) were not progressive. Our study confirmed that most of optic pathway gliomas were stable during evolution, but rare cases may have bad prognosis. CONCLUSION: Our study supported the importance of close ophthalmological follow-up during childhood for which screening methods are discussed. There is a consensus to limit treatment for patients with progressive ophthalmological symptoms.
Subject(s)
Neurofibromatosis 1/complications , Optic Nerve Glioma/etiology , Adolescent , Child , Child, Preschool , Disease Progression , Female , Follow-Up Studies , Humans , Infant , Magnetic Resonance Imaging , Male , Optic Nerve Glioma/pathology , Prognosis , Retrospective StudiesABSTRACT
The authors reported two recent cases of broncho-pulmonary amylosis with monoclonal gammapathy. The first observation concerned a diffuse amylosis characterized by a bilateral pulmonary infiltration with pleural effusions and secretion of IgGlambda with a Bence Jones proteinuria. Pleural and pulmonary biopsies will provide the proof of amylosis. Evolution will rapidly be fatal in case of renal insufficiency. Post-mortem sampling will confirm the amyloid involvement (muscle, liver, kidney). The second observation described a localized amylosis developed in contact with a mediastino-pulmonary lymphoplasmocytic lymphoma with tumoral secretion of IgMlambda. Although pneumonectomy brought out the disappearance of the abnormal paraportein, death occurred within a few months, linked either to the spreading of the lymphomatous process or to the onset of a bacillary surinfection. The authors then recalled the main clinical and diagnostic data of the broncho-pulmonary amylosis. They underlined the importance of GLENNER'S work who, by proving the immunoglobulinic nature of amyloid, explained that in both cases, amylosis appeared as the consequences of an abnormal immunoglobulins synthesis.
