This study describes the chest radiographs of 50 adult patients with histologically verified histiocytosis X, proposes a radiological classification, and examines the role of radiology in assessing the prognosis of the disease. Radiologically the lesions predominate in the middle and lower lung fields, usually sparing the costophrenic angles, and are typically micronodular, reticular, or cystic...

The immunological phenotype of the cells involved in skin and lymph node lesions from two cases of histiocytosis X (H-X) were analysed by immunofluorescence techniques using combinations of heterologous and monoclonal antisera to Ia-like antigen and human cortical thymocyte (HTA-1) determinant. These cells were also characterised by a new technique using simultaneous immunofluorescence and enzy...

SYNTHESIS Vasopressin is synthesised in hypothalamic neurones, predominantly concentrated in the supraoptic and paraventricular nuclei, which appear to be separate from those concerned in oxytocin synthesis. After synthesis, which may begin with the formation of a high molecular weight, biologically-inactive precursor molecule (Sachs and Takabatake, 1964), the vasopressin is packaged together w...

Five cases of bronchogenic carcinoma were observed among 93 patients with pulmonary histiocytosis X (Hx). Mean age at the time of diagnosis of Hx was 42 years; on the average, cancer occurred 10.5 years later. All patients were smokers and continued to smoke heavily at the time of diagnosis of cancer. Comparison of the five cases associating Hx and lung carcinoma with a group of 88 control pati...

Division and proliferation of dendritic cells (DCs) have been proposed to contribute to homeostasis and to prolonged antigen presentation. Whether abnormal proliferation of dendritic cells causes Langerhans cell histiocytosis (LCH) is a highly debated topic. Transgenic expression of simian virus 40 (SV40) T antigens in mature DCs allowed their transformation in vivo while maintaining their phen...

Langerhans cell histiocytosis syndromes have been described as encompassing a range of disorders, such as eosinophilic granuloma, Letterer-Siwe syndrome and Hand-Schüller-Christian disease. These disorders have been mainly diagnosed at early ages of life and are relatively rare entities in adult age groups. In this study, we aimed to retrospectively evaluate the patients with Langerhans cell hi...

A 10-year-old boy presented with torticollis and neck pain for 2 months without fever. His physical examination showed torticollis and limitation of flexion/extension and cervical rotation without neurological deficit. Family and past histories were unremarkable. Cervical spine radiographs showed a cervical scoliosis with loss of the normal cervical lordosis and partial collapse of C3 vertebral...

There is no single agreed definition of a rare disease. The European Commission on Public Health defines rare diseases as those which are ‘life-threatening or chronically debilitating and which are of such low prevalence that special combined efforts are needed to address them’. The term ‘orphan’ has been used in association with rare diseases in order to emphasise their relative neglect in ter...