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ACUTE NONLIPID DISSEMINATED RETICULOENDOTHELIOSIS

Authors: R, BATSON; J, SHAPIRO; A, CHRISTIE; H D, RILEY;

ACUTE NONLIPID DISSEMINATED RETICULOENDOTHELIOSIS

Abstract

THE NONLIPID reticuloendothelioses are usually considered to include Hand-Schuller-Christian's disease, eosinophilic granuloma of the bone, and acute disseminated reticuloendotheliosis (Letterer-Siwe's disease). The latter is the least common of this group, approximately 41 cases having been previously reported. It is the purpose of this paper to contribute to the knowledge of this disease by reviewing the literature and reporting 15 additional cases. Our patients were 13 months or younger at the onset of the disease and illustrated classical features of acute disseminated reticuloendotheliosis. These characteristics included hepatosplenomegaly, anemia, skin lesions, adenopathy, bone lesions, and recurring secondary infections. With one exception all diagnoses were confirmed pathologically. HISTORICAL REVIEW The earliest reports of nonlipid reticuloendotheliosis concerned Hand-Schuller-Christian's disease. These were by Smith, 2 Hand, 3 Kay, 4 Schuller 5 and Christian 6 and resulted in characterization of the disease as a triad consisting of diabetes insipidus, exophthalmos, and defects in membranous bones. Epstein

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Keywords

Histiocytosis, Langerhans-Cell, Leukemia, Hairy Cell, Humans, Infant, Genetic Diseases, X-Linked, Severe Combined Immunodeficiency, Child, Lymphatic Diseases

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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
50
Average
Top 1%
Average
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