Niemann-Pick's Disease*†
Authors: ROBERT A. KNOX
Journal: Annals of Internal Medicine (1932)

Article1 August 1932Niemann-Pick's Disease(Essential Lipoid Histiocytosis)ROBERT A. KNOX, M.D., F.A.C.P., GEORGE W. RAMSEY, M.D., F.A.C.P.ROBERT A. KNOX, M.D., F.A.C.P.Search for more papers by this author, GEORGE W. RAMSEY, M.D., F.A.C.P.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-6-2-218 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptNiemann-Pick's disease is a disease of infancy characterized clinically by an enlargement of the liver, spleen and lymph glands, associated with yellowish brown pigmentation of the skin and accompanied by an enlarged abdomen and more or less emaciation. Cranial and skeletal changes may be associated. It is a fatal condition, being characterized by progressive wasting. Death may be due to intercurrent affections. "It is a congenital, familial, constitutional, systemic disease which manifests itself as a disturbance of the lipoid metabolism and leads to a deposit of complex cellular substances in the cells of the reticulo-endothelial apparatus. Its etiology and pathogenesis...References1 STEINER WR: Diseases of the spleen, Nelson's Loose Leaf Med., 1929, 609-614. Google Scholar2 NIEMANN A: Ein unbekanntes Krankheitsbild, Jahrb. f. Kinderh. 1914, xxix, 1-10. Google Scholar3 KNOXWAHLSCHMEISSER JHHRHC: Gaucher's disease: a report of two cases in infants, Johns Hopkins Hosp. Bull., 1916, xxvii, 1-12. Google Scholar4 SIEGMUND-KÖLN : Lipoidzellenhyperplasie der Milz und Splenomegalie Gaucher, Verhandl. d. deutsch. path. Gesellsch, 1921, xviii, 59-62. Google Scholar5 BLOOM W: Splenomegaly (type Gaucher) and lipoid-histiocytosis (type Niemann), Am. Jr. Path., 1925, i, 595-626. Google Scholar6 HENSCHEN F: Contribution à l étude de la maladie de Gaucher, Acta Med. Scandinav., 1925, xvi, 593. Google Scholar7 SCHIFF E: Im Leben diagnostizierte lipoidzellige Splenohepatomegalie (Typus Niemann-Pick) bei einem 17 Monate alten Knaben, Jahrb. f. Kinderh., 1926, lxii, 1-6. Google Scholar8 HAMBURGER R: Lipoidzellige Splenohepatomegalie (Typus Niemann-Pick) in Verbindung mit amaurotischer Idiotie bei einem 14 Monate alten Mädchen, Jahrb. f. Kinderh., 1927, cxvi, 41-50. Google Scholar9 CORCANOBERLINGDIENST PCG: La maladie de Niemann-Pick, Rev. franç. de pédiat., 1927, iii, 789-829. Google Scholar10 BERMAN SL: A case of lipoid histiocytosis, Am. Jr. Dis. Child., 1928, xxxvi, 102-114. CrossrefGoogle Scholar11 KRAMER B: Lipoid-cell splenohepatomegaly, Niemann-Pick type; a clinical analysis of nine cases with a report of one additional case, Med. Clin. N. Am., 1928, xi, 905-918. Google Scholar12 DIENSTHAMPERL H: Berichten über einen Fall von lipoidzelliger Splenohepatomegalie vom Typus Niemann-Pick, Wien. klin. Wchnschr., 1927, xi, 1432-1433. Google Scholar13 ABTBLOOM AFW: Essential lipoid histiocytosis (type Niemann-Pick); congenital disturbance in lipoid metabolism in infants associated with splenohepatomegaly, Jr. Am. Med. Assoc., 1928, xi, 2076-2080. CrossrefGoogle Scholar14 ABRIKOSSOFF A: Demonstration eines eigenartigen Falles der Niemann-Pickschen Krankheit, Centralbl. f. allg. Path. u path Anat., 1928, xliii, 7. Google Scholar15 BLOOMKERN WR: Spleen from Gaucher's disease and lipoid histiocytosis, Arch. Int. Med., 1927, xxix, 456. CrossrefGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Washington, Pa.†From the Departments of Pediatrics and Pathology, Washington Hospital, Washington, Pa.*Received for publication, September 2, 1931. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited ByNiemann-Pick DiseaseFamiliäre amaurotische IdiotieLes manifestation oculaires des troubles primitifs du metabolisme des lipides: Étude clinique, génétique et anatomo-pathologiqueNiemann-Pick's Disease Acute and Chronic Type?Niemann-pick diseaseErbpathologie des Blutes und der blutbildenden OrganeDie Speicherkrankheiten (Thesaurismosen) 1 August 1932Volume 6, Issue 2Page: 218-226KeywordsCellsEtiologyLiverLiver diseasesLymphPathogenesisPediatricsSpleenSystemic diseases Issue Published: 1 August 1932 PDF DownloadLoading ...