Your Epoch is not for Trying. It's for Living and for Dying...

The article is devoted to biographies of three Russian physicians of the Silver Age (a period in the History of Russian culture between 1890 and 1917). They made early, significant and internationally recognized contribution into medical science and became eponymous, although social disasters of the twentieth century caused deep impact on their subsequent lives and careers, so their role was shadowed from global medical community. The article analyzes biographies and academic achievements of A-F.K. Siewert (aka: Zivert, Ziwert, von Siewert) (1872-1922), known for first description of the hereditary dyskinesia of cilia (as a triad of: situs inversus of the viscera, abnormal frontal sinuses producing sinusitis and bronchiectasis); S.S. Abramov (1875-1951), discoverer of primary idiopathic myocarditis, and N.I. Taratynov (1887-1919), who was the first in description of a local form of histiocytosis X (solitary eosinophilic granuloma) and predicted the eosinophilic origin of Charcot-Leyden crystals. The contribution of these scientists into Medicine is reviewed in context of historical epoch, on background of their different individual social choices and the fate of their families. Besides their eponymous descriptions, other medical priorities of these scholars are analyzed. Some previously unpublished materials from their family archives are presented, which witness for possible existence of unknown prototype for the main hero of 'Doctor Zhivago' novel by B.L. Pasternak and for probable priorities of doctor Zivert - in active diastole concept, or doctor Abramov - in description of dilated cardiomyopathy. The factors facilitating rapid development of theoretical and practical Medicine in imperial Russia of late XIX - early XX centuries are discussed. The conclusion of the author is that in any epoch, even the most cruel and unfavorable one, the creative activity is a way to social immortality (19 figs, 68 refs).

Langerhans cell histiocytosis.

The clinical manifestations of Langerhans cell histiocytosis have been recognized for more than a century. For most of that time, physicians have viewed the disease from different perspectives, interpreting portions of its clinical spectrum as if they were distinct and unrelated entities. More recently, Langerhans cell histiocytosis has been unified into a single concept, though the disease continues to defy traditional classification. By most accounts, Langerhans cell histiocytosis appears to be a morphologically benign proliferation of inflammatory cells that escapes regulatory control mechanisms. Studies from patients with all stages of the disease, however, document clonal proliferation of immune processing cells (i.e., Langerhans cells), suggesting a malignant disease process. The most common ophthalmic manifestation of Langerhans cell histiocytosis is a solitary lesion of orbital bone, which typically responds to minimally invasive therapy. The best management of solitary orbital Langerhans cell histiocytosis is debatable and has been complicated by its recent designation as a risk factor for central nervous system disease. This article summarizes recent developments in understanding the biology of Langerhans cell histiocytosis, reviews its ophthalmic manifestations, prognosis, and the controversy surrounding treatment of isolated orbital disease.

Histiocitosis de celulas de Langerhans en el raquis infantil / Langerhans cell histiocytosis of the spine in children

Introduccion: La histiocitosis de celulas de Langerhans incluye un amplio espectro de enfermedades de etiologia desconocida, observada preponderantemente en los niños. El objetivo del presente trabajo es analizar las formas de presentacion, los estudios complementarios, los procedimientos diagnosticos, los criterios de estadificacion y los resultados de distintas modalidades terapeuticas en su ubicacion espinal. Materiales y metodos: Doce pacientes evaluados retrospectivamente con diseño de casos y controles, tratados entre 1983-2004. Seguimiento promedio 5 años. La estadificacion incluyo la extension anatomica (Weinstein-Boriani-Biagini) y el comportamiento biologico (Enneking). El centellograma, la TC y la RM fueron los estudios de eleccion. El tratamiento incluyo corticoides, radioterapia, quimioterapia y cirugia. Significación estadistica p ≤ 0,05. Resultados: Relacion masculino-femenino 10-2, media de edad 9 años 5 meses. Datos clínicos relevantes: dolor, limitación funcional o deformidad y compromiso neurologico. Predominio de formas unicas en el raquis lumbar y el cuerpo vertebral. Las variables clinicas fueron modificadas favorablemente con significacion estadistica. Se obtuvo la remision de la enfermedad sin excepciones, pero persistieron distintas secuelas morfologicas vertebrales, sin traduccion clinica.Conclusiones: Se obtuvo la curacion de las lesiones, independientemente del tratamiento realizado. En el momento del diagnostico, ningun criterio clinico, histologico o por imagenes pudo predecir el curso ulterior de la enfermedad

Jacques Calvé.

Jacques Calvé was born August 18, 1875, In Paris, France, and died on March 10, 1954. His main interest in medicine was the treatment of tuberculosis of the spine. Therefore, he established a very differentiated plan for the treatment of the Pott's disease. Calvé was also one of the pioneers in the radiologic evaluation of the skeletal system. He was among the first to describe the osteonecrosis of the hip and its radiological features-with his name still a part of the disease name today: the Morbus Legg-Calvé-Perthes. Furthermore, he was the first to describe the radiologic image of histiocytosis X of the vertebral body, but he interpreted it as a special form of tuberculosis of the spine. It was years later that the correct diagnosis of this particular radiologic change was found. This paper provides a short account of Jacques Calvé's life as a medical scientist.

Langerhans cell histiocytosis--clinical and epidemiological aspects.

Langerhans cell histiocytosis is a disease which frustrates both clinician and scientist. Its aetiology is unknown, its pathogenesis is ill understood and the clinical course is unpredictable. Historically, the different nomenclatures reflecting the first clinical descriptions by Hand (1893, 1921), Schuller (1915) and Christian (1920), and subsequently by Letterer (1924) and Siwe (1933), led to confusion only partially resolved by Lichtenstein (1953) who recognised that the disease in each of these clinical syndromes were components of a spectrum of disease involving the histiocyte. He proposed his unifying concept of Histiocytosis X--'X' being the unknown aetiological factor. In 1973, Nezelof recognised the lesional cell as a 'Langerhans-like' cell but it took another decade for the disease to be recognised as a single entity and the term Langerhans cell histiocytosis to be internationally accepted. The publication, by the Histiocyte Society (1987), of their classification of the histiocyte disorders together with criteria for pathological diagnosis and clinical evaluation of Langerhans cell histiocytosis have consolidated the position. This article details the wide variety of clinical manifestations of the disease and its sequelae and discusses possible epidemiological factors. Finally it looks at the potential implications of recent scientific research on the management of the disease.

Historical perspectives of Langerhans cell histiocytosis.

This brief review of the history of Langerhans cell histiocytosis brings us into touch with the history of medicine of the last 100 years and the opportunity to reflect on the progress in medicine in general. Hand, Christian, Schüller, Letterer, and Siwe represent far more than names to attach to eponyms. This historical overview takes us from the first descriptions of the disease in the literature through the modern efforts to take the X out of histiocytosis-X.