title: Whipple's disease infection surgical treatment: presentation of a rare case and literature review.
abstract: The Whipple' Disease (W.D.) is a very rare disease with an incidence of 1 per 1.000.000 inhabitants; it is a systemic infection that may mimic a wide spectrum of clinical disorders, which may have a fatal outcome and affects mainly male 40-50 years old. The infective agent is an actinomycete, Tropheryma Whipplei (T.W.) that was isolated 100 years after first description by Wipple, and identified in macrophages of mucosa of the small intestine by biopsy which is characterized by periodic acid-Schiff-positive, products of the inner membrane of his polysaccharide bacterial cell wall. The multisystemic clinical manifestations evolve rapidly towards an organic decay characterized by weight loss, malabsorption, diarrhea, polyathralgia, opthalmoplegia, neuro-psychiatric disorders and sometimes associated to endocarditis. Early antibiotic treatment with trimethoprim and sulfometathaxazole reduces the fatal evolution of the disease. The authors present a rare experience about a female subject in which the clinical gastrointestinal signs were preceded by neuro-psychiatric disorders, and evolved into obstruction and intestinal perforation which required an emergency surgery with temporary ileostomy, recanalized only after adequate medical treatment with a full dose of antibiotic and resolution of clinical disease for the high risks of fistulae for the edema and lymphadenopathy of mucosa. The diagnosis was histologically examined by intestinal biopsy performed during surgery, which showed PAS-positive histiocytes, while PRC polymerase RNA was negative, which confirms the high sensibility of PAS positive and low specificity of RNA polymerase for T.W.
authors: Surgical Department, Division of Chioggia-Mestre, Chioggia Hospital, Italy.
authors: M
authors: M
authors: Amendolara
authors: C
authors: C
authors: Barbarino
authors: D
authors: D
authors: Bucca
authors: G
authors: G
authors: Stevanato
authors: M
authors: M
authors: Zucchelli
authors: F
authors: F
authors: Romano
authors: L
authors: L
authors: Baiano
authors: M
authors: M
authors: Bernardi
authors: A
authors: A
authors: Broggiato
authors: S
authors: S
authors: Ramuscello
authors: M
authors: M
authors: Rizzo
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007077:Ileal Diseases / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D007081:Ileostomy; D007415:Intestinal Obstruction / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000601:surgery*; D007416:Intestinal Perforation / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D008875:Middle Aged; D035583:Rare Diseases; D016896:Treatment Outcome; D054851:Tropheryma / Q000187:drug effects / Q000302:isolation & purification; D008061:Whipple Disease / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000601:surgery*
chemical_list: D000900:Anti-Bacterial Agents
references: Bull Johns Hopkins Hosp. 1961 Aug;109:80-98
references: 13787237
references: J Clin Microbiol. 2007 Jun;45(6):2078-81
references: 17446327
references: Curr Opin Gastroenterol. 2008 Mar;24(2):141-8
references: 18301263
references: JAMA. 2001 Feb 28;285(8):1039-43
references: 11209175
references: N Engl J Med. 2007 Jan 4;356(1):55-66
references: 17202456
references: Lancet. 1991 Aug 24;338(8765):474-5
references: 1714530
references: Clin Exp Med. 2004 Sep;4(1):39-43
references: 15598084
references: Gastroenterol Clin Biol. 2007 Aug-Sep;31(8-9 Pt 1):729-39
references: 17925777
references: Acta Gastroenterol Belg. 1992 Mar-Apr;55(2):209-19
references: 1378675
references: Lancet. 2003 Jan 18;361(9353):239-46
references: 12547551
references: Proc Soc Exp Biol Med. 1960 Nov;105:411-4
references: 13694342
references: Ann Intern Med. 2001 Jan 16;134(2):115-9
references: 11177314
references: Mayo Clin Proc. 1988 Jun;63(6):539-51
references: 2453762
references: Lancet Infect Dis. 2008 Mar;8(3):179-90
references: 18291339
references: Neurosurgery. 1999 Jan;44(1):203-9
references: 9894983
references: Medicine (Baltimore). 2002 Nov;81(6):443-57
references: 12441901
references: Dig Dis Sci. 1994 Aug;39(8):1642-8
references: 7519538
references: Neth J Med. 2008 Oct;66(9):392-5
references: 18931401
references: Dig Dis. 2009;27(4):494-501
references: 19897965
references: Curr Opin Rheumatol. 2001 Jan;13(1):74-9
references: 11148719
references: QJM. 2006 Sep;99(9):609-23
references: 16905752
references: Clin Gastroenterol Hepatol. 2004 Oct;2(10):849-60
references: 15476147
references: World J Gastroenterol. 2009 May 7;15(17):2078-80
references: 19418579
references: Medicine (Baltimore). 2005 May;84(3):162-173
references: 15879906