WHIPPLE'S DISEASE (INTESTINAL LIPODYSTROPHY): REVIEW OF THE LITERATURE AND REPORT OF A CASE SUCCESSFULLY TREATED WITH ADRENOCORTICOTROPIN (ACTH) AND CORTISONE
Authors: J RADDING, M J FIESE
Journal: Annals of Internal Medicine (1954)

Case Reports1 November 1954WHIPPLE'S DISEASE (INTESTINAL LIPODYSTROPHY): REVIEW OF THE LITERATURE AND REPORT OF A CASE SUCCESSFULLY TREATED WITH ADRENOCORTICOTROPIN (ACTH) AND CORTISONEJEROME RADDING, M.D., MARSHALL J. FIESE, M.D.JEROME RADDING, M.D.Search for more papers by this author, MARSHALL J. FIESE, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-41-5-1066 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptIn 1907 Whipple1 reported a previously undescribed disease which occurred in a 36 year old physician and was manifested by vague abdominal distress, progressive weight loss, diarrhea, arthralgia and generalized pigmentation. Stool examination showed a great increase in neutral fat and fatty acids. The patient died following an exploratory laparotomy, and microscopic examination revealed an accumulation of mononuclear foam cells in the lamina propria of the small intestine and in the enlarged mesenteric lymph nodes. Whipple named the disease intestinal lipodystrophy, and felt that it was due to some disorder of fat metabolism.Since Whipple's description of this disease 52...Bibliography1. Whipple GH: A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues, Bull. Johns Hopkins Hosp. 18: 382, 1907. Google Scholar2. Blumgart HL: Three fatal adult cases of malabsorption of fat, Arch. Int. Med. 32: 113, 1923. CrossrefGoogle Scholar3. Fahr T: Zur Frage des Zusammenhanges zwischen Stoffwechselstörungen und morphologischen Veranderungen im reticuloendothelialen System, Klin. Wchnschr. 7: 1787, 1928. CrossrefGoogle Scholar4. Fleischmann R: Ueber tumorbildende Fettgewebsgranulome im Gokörse des Dünndarms, Arch. f. klin. Chir. 158: 692, 1930. Google Scholar5. CollinsBerdez ANGL: Chyle cysts of mesentery, Arch. Surg. 28: 335, 1934 (case 2). CrossrefGoogle Scholar6. Jarcho S: Steatorrhea with unusual intestinal lesions, Bull. Johns Hopkins Hosp. 59: 275, 1936. Google Scholar7. Hill JM: Mesenteric chyladenectasis: report of a case, Am. J. Path. 13: 267, 1937. MedlineGoogle Scholar8. Boeck , in discussion on Bargen, J. A., Bollman, J. L., and Kepler, E. J.: Diarrhea of pancreatic insufficiency, Am. J. Digest. Dis. 4: 728, 1938. Google Scholar9. Gaertner K: Über einen Fall von hochgradiger Fettspeicherung in den Mesenteriallymphknoten, Frankfurt. Ztschr. f. Path. 52: 529, 1938. Google Scholar10. GlynnRosenheim LEML: Mesenteric chyladenitis with steatorrhea and features of Addison's disease, J. Path. and Bact. 47: 285, 1938. CrossrefGoogle Scholar11. Korsch JH: Fettstoffwechselstorung mit Granulombildung in Mesenterium, Zentralbl. f. allg. Path. u. path. Anat. 71: 337, 1938. Google Scholar12. Kloos K: Ueber eine eigenartige Fettresorptionsstörung und ihre Beziehung zur Sprue, Virchows Arch. f. path. Anat. 304: 625, 1939. CrossrefGoogle Scholar13. ReinhartWilson HLSJ: Malabsorption of fat (intestinal lipodystrophy of Whipple): report of a case, Am. J. Path. 15: 483, 1939. MedlineGoogle Scholar14. Pearse HE: Whipple's disease, or intestinal lipodystrophy, Surgery 11: 906, 1942. Google Scholar15. SailerMcGann SRJ: Lipophagic granulomatosis of enteric tract, Am. J. Digest. Dis. 9: 55, 1942. CrossrefGoogle Scholar16. ApperlyCopley FLEL: Whipple's disease (lipophagia granulomatosis), Gastroenterology 1: 461, 1943. Google Scholar17. Vaux DM: Chyladenectasis with steatorrhea, J. Path. and Bact. 55: 93, 1943. CrossrefGoogle Scholar18. AmsterdamGrayzel JJDM: Intestinal lipodystrophy (lipophagia granulomatosis or Whipple's disease), Am. J. M. Sc. 210: 605, 1945. CrossrefGoogle Scholar19. Clemmesen J: Steatorrhea arthro-pericarditica (mesenteric chyladenectasis): review and report of a case, Acta med. Scandinav. 121: 495, 1945. CrossrefMedlineGoogle Scholar20. EscuderoMostoLandabure PDP: Lipodistrofia intestinal de Whipple, Dia Méd. 17: 915 (Aug. 27) 1945. Google Scholar21. FitzgeraldKinney PJTD: Intestinal lipodystrophy (Whipple's disease), Am. J. Path. 21: 1069, 1945. MedlineGoogle Scholar22. Hansenvan Staa KH: Die einheimische Sprue und ihre Folgekrankheiten (sekundäre Avitaminosen), 1936, G. Thieme, Leipzig, p. 39, cited by Clemmesen in Acta med. Scandinav. 121: 495, 1945. Google Scholar23. Frei R: Über die histopathologischen Veränderungen am Dünndarm bei Störungen der Fettresorption (sog. Xanthomatose des Dünndarms), Schweiz. Ztschr. f. Path. u. Bakt. 10: 685, 1947. Google Scholar24. Oliver-PascualGalanOliver-PascualCastillo EJAE: Un caso de lipodistrofia intestinal con lesiones ganglionares mesenterica de granulomatosis lipofagia (enfermedad de Whipple), Rev. españ. enferm. ap. digest. y nutrición 6: 213, 1947. MedlineGoogle Scholar25. PembertonComfortFairZaslow JdMWEJ: Intestinal lipodystrophy (Whipple's disease): preliminary report of three cases in early stage of disease, Surg., Gynec. and Obst. 85: 85, 1947. MedlineGoogle Scholar26. RosenRosen MSSH: Intestinal lipodystrophy of Whipple: report of a case and analysis of literature, Am. J. Path. 23: 443, 1947. MedlineGoogle Scholar27. Chapnick HA: Idiopathic steatorrhea, with report of a case of Whipple's disease, Ann. Int. Med. 29: 549, 1948. LinkGoogle Scholar28. Fernandez LunaLatienda DRI: Lipodistrofia Generalizada, Rev. Asoc. méd. argent. 62: 590, 1948. MedlineGoogle Scholar29. NewmanPope BRH: A case of intestinal lipodystrophy (Whipple's disease) simulating Boeck's sarcoid, Gastroenterology 11: 120, 1948. MedlineGoogle Scholar30. RutishauserDemoleMach EMRS: Sprue due to intestinal lipostasis (intestinal lipodystrophy; Whipple's disease), Rev. méd. de la Suisse Rom. 68: 573, 1948. MedlineGoogle Scholar31. AveryPaulley JFJW: Intestinal lipodystrophy (Whipple's disease), Lancet 1: 214, 1949. MedlineGoogle Scholar32. CoplandColvinEssrigHeibner SMSHIMWC: Intestinal lipodystrophy or Whipple's disease, Surgery 26: 688, 1949. MedlineGoogle Scholar33. KampmeierPeterson RHJC: Whipple's disease; its relationship to the rheumatic state, Tr. Am. Clin. and Climatol. A. 61: 248, 1949. MedlineGoogle Scholar34. SchutzBennerChristian PJWHWA: Intestinal lipodystrophy (Whipple's disease), Am. J. Med. 7: 553, 1949. CrossrefMedlineGoogle Scholar35. Black-Schaffer B: Tinctorial demonstration of glycoprotein in Whipple's disease, Proc. Soc. Exper. Biol. and Med. 72: 225, 1949. CrossrefMedlineGoogle Scholar36. HendrixBlack-SchafferWithersHandler JPGRWP: Whipple's intestinal lipodystrophy: report of 4 cases and discussion of possible pathogenic factors, Arch. Int. Med. 85: 91, 1950. CrossrefGoogle Scholar37. OdesskyBurdison LWR: Intestinal lipodystrophy (Whipple's disease) occurring with parathyroid hyperplasia and nephrosis; case with autopsy, Arch. Path. 49: 307, 1950. Google Scholar38. PlummerRussiHarrisCaravati KSWHCM: Lipophagic intestinal granulomatosis (Whipple's disease): clinical and pathologic study of 34 cases, with special reference to clinical diagnosis and pathogenesis, Arch. Int. Med. 86: 280, 1950. CrossrefGoogle Scholar39. Reveno WS: Intestinal lipodystrophy (Whipple's disease): report of a case, New England J. Med. 243: 216, 1950. CrossrefMedlineGoogle Scholar40. TraceyBrolsma MLMP: Whipple's disease (lipophagia granulomatosis); report of one case, Gastroenterology 15: 366, 1950. CrossrefMedlineGoogle Scholar41. PalumboRugtiv LTGM: Intestinal lipodystrophy (Whipple's disease), Arch. Surg. 62: 604, 1951. CrossrefGoogle Scholar42. Albertini B: Contributo morfologico e clinico sulla lipodistrofia intestinale o morbo de Whipple, Arch. ital. mal. app. diger. 18: 195, 1952. MedlineGoogle Scholar43. ChristieDalton ACDA: A fatal case of intestinal lipodystrophy of Whipple investigated during life, J. Path. and Bact. 64: 351, 1952. CrossrefMedlineGoogle Scholar44. Inguscio L: Lipodistrophia intestinale o sindrome de Whipple, Athena 18: 315, 1952. MedlineGoogle Scholar45. Janowitz HD: Personal communication. Google Scholar46. Luton EF: A possible cause of death in Whipple's disease, Gastroenterology 20: 327, 1952. CrossrefMedlineGoogle Scholar47. Mendes De LeonCoenegracht CJ: Lipodystrophia intestinalis (ziekte van Whipple), Nederl. tijdschr. v. geneesk. 96: 560, 1952. MedlineGoogle Scholar48. Paulley JW: A case of Whipple's disease (intestinal lipodystrophy), Gastroenterology 22: 128, 1952. CrossrefMedlineGoogle Scholar49. Russo RR: Whipple's disease: review of the literature and report of two cases, Arch. Int. Med. 89: 600, 1952. CrossrefGoogle Scholar50. Upton AC: Histochemical investigation of the mesenchymal lesions in Whipple's disease, Am. J. Clin. Path. 22: 755, 1952. CrossrefMedlineGoogle Scholar51. Wallace JL: Intestinal lipodystrophy, Whipple's disease, Texas State J. Med. 48: 771, 1952. MedlineGoogle Scholar52. JonesBensonRoque CMJAAL: Whipple's disease, New England J. Med. 248: 665, 1953. CrossrefMedlineGoogle Scholar53. StorySagild RDU: Whipple's disease (intestinal lipodystrophy) and serum glycoproteins, J. A. M. A. 152: 312, 1953. CrossrefGoogle Scholar54. PlummerWeisigerCaravati KBBCM: Successful corticotropin (ACTH) therapy of Whipple's disease refractory to cortisone, Arch. Int. Med. 91: 784, 1953. CrossrefGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Fresno, California*Received for publication March 5, 1954.From the Medical Service, Veterans Administration Hospital, Fresno. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byWhipple's Disease: Clinical, Biochemical, and Histopathologic Features and Assessment of Treatment in 29 PatientsDiarrhea and ConstipationBACTERIA IN WHIPPLE'S DISEASEThe arthritis of Whipple's diseaseWhipple's diseaseProphylaxis in patients receiving adrenal steroid therapyWhipple's diseaseWhipple's diseaseAntibiotic Control of Diarrhea in Whipple’s DiseaseDie Whipplesche KrankheitWhipple's DiseaseThe Use of ACTH and Adrenocorticosteroids in Diseases of the Digestive SystemGastrointestinal malabsorptive syndromesIntestinal lipodystrophy (Whipple's disease) treated with ACTH and corticosteroidsThe Use of the Adrenocorticosteroids in GastroenterologyObliterative Lymphangitis of the Mesentery In Whipple's DiseaseIntestinal Lipodystrophy (Whipple's Disease) Amenable to Corticosteroid Therapy 1 November 1954Volume 41, Issue 5Page: 1066-1075KeywordsAdrenocorticotropic hormoneFatsFatty acidsLaparotomyLipodystrophyLymph nodesMedical servicesMucous membranesSmall intestineWeight loss ePublished: 1 December 2008 Issue Published: 1 November 1954 PDF downloadLoading ...