Rare occurrence of Whipple Disease in a young female patient with a fatal outcome

Maresi, Emiliano; Argo, Antonina; Portelli, Francesca; Busardò, Francesco Paolo; Raoult, Didier; Lépidi, Hubert

doi:10.1016/j.ejfs.2013.04.004

Cited by 7 publications

(6 citation statements)

References 35 publications

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“…Determined by the conditions or illness requiring invasive/surgical procedure, even rare and/or undiagnosed illness or conditions to death (Abate et al 2014;Maresi et al 2013;Maresi et al 2006;Rancati et al 2018), especially preventable conditions and complex clinical interactions, account for overall 90% of PODs. Myocardial infarction (MI) in the perioperative period, even in patients without medical history or ischemic heart disease, takes import role.…”

Section: Definitions Of Anesthetic Perioperative-related Mortality Anmentioning

confidence: 99%

Perioperative and anesthetic deaths: toxicological and medico legal aspects

Argo

Zerbo

Lanzarone

et al. 2019

Egypt J Forensic Sci

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Background: Anesthesia has become safer during decades, though there is still a preventable mortality; the complexity of medical and surgical interventions, increasingly older and sicker patients, has created a host of new hazards in anesthesiology. In this paper, some of these perioperative (PO) fatal adverse events are investigated in terms of health responsibility. Selective literature research in several data bases, concerning perioperative and anesthetic deaths and medical responsibility, was performed. Main text: A generally accepted definition of the anesthesia and perioperatory-related death still remains one of the major concerns in forensic pathology, and the terms "operative deaths" and "anesthetic deaths" are usually applied inaccurately within the medico-legal literature. Such events involve comprehensively PO fatalities and allow for subtle separation of natural and unnatural death, at least from the prospective of forensic pathology. Iatrogenic deaths in this field can be separated into some major categories, as attributable to previous patient's unfavorable conditions or depending from surgical procedure per se (such as PO cardiac and cerebrovascular events). In this review, the authors carried out syntheses of specific research areas regarding epidemiology, complications of general and spinal anesthetic, failure in airway management and patient's circulatory homeostasis, and adverse drugs reactions; analysis considering the challenge of anesthetic-related mortality, epidemiology and classifications, by indicating causal chain of death, in respect of both contributing and associated anesthetic and surgery facts. Conclusions: Perioperative quality control programs and its relevance for medico-legal evaluation are emphasized as, although mortality rates have decreased worldwide over the last decades, however, preventable drug-related deaths still happen. Such fatal events have to be considered within the field of forensic pathology experts, with regard of malpractice claims, to implement a strategy for preventing potentially fatal complications.

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Section: Definitions Of Anesthetic Perioperative-related Mortality Anmentioning

confidence: 99%

Perioperative and anesthetic deaths: toxicological and medico legal aspects

Argo

Zerbo

Lanzarone

et al. 2019

Egypt J Forensic Sci

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“…[19][20][21] Surprisingly, rare reported cases of WD diarrhea culminating in hypovolemic shock did not involve suspected rheumatologic etiologies nor immunosuppressive therapies at all. 22,23 Several of these studies highlight that WD may be commonly misdiagnosed as inflammatory rheumatoid disease and that WD symptoms may be exacerbated with immunosuppressive therapies. This then leaves in question how many similar WD cases are actually fatal and never received a diagnostic post-mortem examination.…”

Section: Discussionmentioning

confidence: 99%

Whipple s disease: a fatal mimic

et al. 2020

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Whipple's Disease, a rare diagnosis caused by the slow-growing bacterium Tropheryma whipplei, most often presents with the classically described signs of malabsorption due to gastrointestinal colonization. However, it can also have signs and symptoms that clinically overlap with rheumatic diseases, potentially resulting in misdiagnosis. Furthermore, treatment with modern potent biologic immunosuppressive agents and classic disease modifying anti-rheumatic drugs (DMARDs) can lead to serious exacerbation of undiagnosed infections. We present the case of a middle-aged woman with long term complaints of arthalgias, who was diagnosed with seronegative rheumatoid arthritis and subsequently treated for almost 7 years with such immunosuppressive therapies. The patient's disease course included chronic diarrhea that abruptly intensified and culminated in fatal hypovolemic shock/sepsis. A diagnosis of WD was made by autopsy examination, wherein several organ systems were found to be heavily involved by Tropheryma whipplei organisms, and their identification was confirmed with histochemical and molecular evaluation. Notably, most bacterial organisms were located deeply in the submucosa/muscularis of affected organs, a practical reminder to practicing pathologists that challenges the classic histopathologic description of Whipple disease as an infiltration of predominantly lamina propria, and the potential for sampling bias in typically superficial endoscopic biopsies during routine procedures.

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“…The exact prevalence of Whipple’s disease (WD) is unknown but it is uncommon [4] . For an excellent overview of published case reports, see the recent summary by Maresi et al [2] . Here we report a case of classic Whipple’s disease presenting with mesenteric lymphadenitis.…”

Section: Discussionmentioning

confidence: 99%

“…Whipple’s disease (WD) is a diagnostic challenge because it is a “great imitator” and relatively uncommon, with less than 1000 cases described [2]. All clinicians and pathologists must, therefore, be aware of its features and have a high index of suspicion so that antibacterial treatment can be initiated in a timely manner; a missed diagnosis can be fatal.…”

Section: Introductionmentioning

confidence: 99%

“…Whipple’s disease, first described by George H. Whipple over a hundred years ago, is a multisystemic disease caused by the Gram-positive bacterium Tropheryma whipplei [1] . Whipple’s disease (WD) is a diagnostic challenge because it is a “great imitator” and relatively uncommon, with less than 1000 cases described [2] . All clinicians and pathologists must, therefore, be aware of its features and have a high index of suspicion so that antibacterial treatment can be initiated in a timely manner; a missed diagnosis can be fatal.…”

Section: Introductionmentioning

confidence: 99%

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Mesenteric lymphadenitis as a presenting feature of Whipple’s disease

Chizinga

Schiliro

Mullin

et al. 2017

IDCases

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Detecting Whipple’s disease, a “great imitator”, requires a high index of suspicion so that antimicrobial treatment can be initiated in a timely manner; a missed diagnosis can be fatal. Although an uncommon cause, Whipple’s disease must be considered in adults with mesenteric lymphadenitis. We report the case of a 39-year-old African American man who presented with chronic joint pain, chronic weight loss, and acute onset epigastric pain. Contrast-enhanced computed tomography of the abdomen and pelvis showed extensive mesenteric lymphadenopathy. A diagnosis of Whipple’s disease was made based upon demonstration of PAS-positive macrophages in the mesenteric lymph node and duodenal biopsies. Antimicrobial therapy resulted in weight gain and resolution of abdominal pain and arthralgia at six months follow-up.Whipple’s disease can be fatal without antibacterial therapy and it always needs to be considered in individuals presenting with any combination of abdominal pain, weight loss, and diarrhea in the background of nonspecific arthritis or arthralgia. Whipple’s disease must also be considered in adults presenting with mesenteric lymphadenitis. Review of CT scans may be helpful, as Whipple’s disease characteristically causes low attenuation mesenteric lymphadenopathy.

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Rare occurrence of Whipple Disease in a young female patient with a fatal outcome

Cited by 7 publications

References 35 publications

Perioperative and anesthetic deaths: toxicological and medico legal aspects

Perioperative and anesthetic deaths: toxicological and medico legal aspects

Whipple s disease: a fatal mimic

Mesenteric lymphadenitis as a presenting feature of Whipple’s disease

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