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      Disseminated Cryptococcosis in an HIV-Negative Patient With Liver Cirrhosis and Asplenia: A Rare but Dreadful Disease

      case-report
      1 , , 2 , 3 , 3
      ,
      Cureus
      Cureus
      multiorgan system failure, hiv negative, asplenia, liver cirrhosis, disseminated cryptococcal infection

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          Abstract

          Cryptococcosis (cryptococcal infection) is a severe life-threatening fungal infection. It is seen worldwide, specifically in immunocompromised, mainly in human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS)-infected individuals. Cryptococcal infection can present with meningitis, pneumonia, peritonitis, disseminated cryptococcosis, and cryptococcal fungemia. Here, we report the case of an HIV-negative Caucasian male in his early 50s with liver cirrhosis and asplenia who presented to our hospital with bilateral foot cellulitis and pneumonia. He was eventually diagnosed with disseminated cryptococcosis. Even with appropriate treatment, he developed multiorgan failure and finally expired. The disseminated cryptococcal infection has a very high mortality rate in patients with liver cirrhosis and asplenia. Liver cirrhosis is an independent risk factor, and asplenia is a comorbid condition for cryptococcal infection in HIV-negative patients. Healthcare providers should have a high suspicion of cryptococcosis in these patients. Early testing with cryptococcal antigen assay and initiation of an appropriate antimicrobial regimen can help minimize bad outcomes.

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          Most cited references21

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          Estimation of the current global burden of cryptococcal meningitis among persons living with HIV/AIDS.

          Cryptococcal meningitis is one of the most important HIV-related opportunistic infections, especially in the developing world. In order to help develop global strategies and priorities for prevention and treatment, it is important to estimate the burden of cryptococcal meningitis. Global burden of disease estimation using published studies. We used the median incidence rate of available studies in a geographic region to estimate the region-specific cryptococcal meningitis incidence; this was multiplied by the 2007 United Nations Programme on HIV/AIDS HIV population estimate for each region to estimate cryptococcal meningitis cases. To estimate deaths, we assumed a 9% 3-month case-fatality rate among high-income regions, a 55% rate among low-income and middle-income regions, and a 70% rate in sub-Saharan Africa, based on studies published in these areas and expert opinion. Published incidence ranged from 0.04 to 12% per year among persons with HIV. Sub-Saharan Africa had the highest yearly burden estimate (median incidence 3.2%, 720 000 cases; range, 144 000-1.3 million). Median incidence was lowest in Western and Central Europe and Oceania (
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            Cirrhosis-associated immune dysfunction

            The term cirrhosis-associated immune dysfunction (CAID) comprises the distinctive spectrum of immune alterations associated with the course of end-stage liver disease. Systemic inflammation and immune deficiency are the key components of CAID. Their severity is highly dynamic and progressive, paralleling cirrhosis stage. CAID involves two different immune phenotypes: the low-grade systemic inflammatory phenotype and the high-grade systemic inflammatory phenotype. The low-grade systemic inflammatory phenotype can be found in patients with compensated disease or clinical decompensation with no organ failure. In this phenotype, there is an exaggerated immune activation but the effector response is not markedly compromised. The high-grade systemic inflammatory phenotype is present in patients with acute-on-chronic liver failure, a clinical situation characterized by decompensation, organ failure and high short-term mortality. Along with high-grade inflammation, this CAID phenotype includes intense immune paralysis that critically increases the risk of infections and worsens prognosis. The intensity of CAID has important consequences on cirrhosis progression and correlates with the severity of liver insufficiency, bacterial translocation and organ failure. Therapies targeting the modulation of the dysfunctional immune response are currently being evaluated in preclinical and clinical studies.
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              The changing epidemiology of cryptococcosis: an update from population-based active surveillance in 2 large metropolitan areas, 1992-2000.

              To examine trends in the incidence and epidemiology of cryptococcosis, active, population-based surveillance was conducted during 1992-2000 in 2 areas of the United States (the Atlanta, Georgia, and Houston, Texas, metropolitan areas; combined population, 7.4 million). A total of 1491 incident cases were detected, of which 1322 (89%) occurred in HIV-infected persons. The annual incidence of cryptococcosis per 1000 persons with AIDS decreased significantly during the study period, from 66 in 1992 to 7 in 2000 in the Atlanta area, and from 24 in 1993 to 2 in 1994 in the Houston area. Poisson regression analysis revealed that African American persons with AIDS were more likely than white persons with AIDS to develop disease. Less than one-third of all HIV-infected persons with cryptococcosis were receiving antiretroviral therapy before diagnosis. Our findings suggest that HIV-infected persons who continue to develop cryptococcosis in the era of highly active antiretroviral therapy (HAART) in the United States are those with limited access to health care. More efforts are needed to expand the availability of HAART and routine HIV care services to these persons.
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                7 April 2023
                April 2023
                : 15
                : 4
                : e37243
                Affiliations
                [1 ] Internal Medicine, St. Vincent Medical Center, Evansville, USA
                [2 ] Internal Medicine, Indiana University, Indianapolis, USA
                [3 ] Hospital Medicine, University of Florida Health, Jacksonville, USA
                Author notes
                Article
                10.7759/cureus.37243
                10164342
                37162787
                d26bafa8-6563-42ce-8758-40c8db3d3acd
                Copyright © 2023, Chippa et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 7 April 2023
                Categories
                Internal Medicine
                Gastroenterology
                Infectious Disease

                multiorgan system failure,hiv negative,asplenia,liver cirrhosis,disseminated cryptococcal infection

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