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Introduction and importance: Kaposi sarcoma (KS) is an angioproliferative disease, that mostly affects HIV-infected patients with a high viral load and a low CD4 count. In rare cases, the paradoxical worsening of a pre-existing or previously unrecognized opportunistic infection occurs in a phenomenon known as immune reconstitution inflammatory response (IRIS). Case presentation: The authors presented a male patient in his 30s with HIV, who developed a series of complications caused by KS following the initiation of antiretroviral therapy. Despite ongoing antiretroviral therapy (ART), chemotherapy, and supportive measures, the patient developed KS-related IRIS, characterized by rapid clinical deterioration, multiorgan failure, and ultimately succumbed to the disease. Clinical discussion: To the best of our knowledge, very rare cases have been reported with KS-IRIS after the initiation of ART. Many predictors of KS-IRIS development have been identified. Patients must meet the known diagnostic criteria to be diagnosed with IRIS. The treatment of KS-IRIS depends on the stage of KS. ART alone is usually adequate in mild cutaneous KS. Chemotherapy and ART are recommended for patients with severe cutaneous and visceral KS. Conclusion: HIV patients with KS undergoing ART initiation or modification should be closely monitored, particularly during the early stages and in those with extensive disease. Treating opportunistic infections before ART initiation may reduce the risk of KS-IRIS. The increasing prevalence of KS in ART-treated patients with HIV warrants further attention and highlights the need for better management strategies in this population.
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Introduction and importance: Birt-Hogg-Dube (BHD) is a rare genetic disorder that results from a mutation in the folliculin (FLCN) gene. Manifestations include pulmonary cysts, fibrofolliculomas, renal tumors, and pneumothoraces. Genetic testing can be used to confirm the diagnosis when suspected. BHD syndrome is diagnosed in patients with negative FLCN gene results using diagnostic criteria. Case presentation: A male in his 20s presented with recurrent pneumothoraces. A physical examination revealed bumps on his face and upper body. A chest computed tomography scan revealed cystic lesions. Blood tests, ESR, and CRP levels were unremarkable. Punch skin biopsy revealed fibrofolliculomas. Genetic testing for the FLCN mutation returned negative. His history, physical exam, imaging, and histopathology suggested BHD syndrome despite having a negative family history and genetic analysis. Eventually, the patient was diagnosed with FLCN gene-negative BHD syndrome. Clinical discussion: More than a hundred families have been identified to have BHD worldwide. There are a few cases in the literature describing patients phenotypically presenting with BHD despite having a negative genetic analysis. One study in Japan found 16 out of 157 individuals having a clinical presentation of BHD with no mutations. Also, decreased expression of the FLCN mRNA may lead to BHD. Conclusion: BHD syndrome can present with a negative FLCN gene mutation; however, patients must meet the known diagnostic criteria such as criteria made by Menko et al., Gupta et al., and Schmidt et al. in order to have a diagnosis of BHD syndrome. Also, a qualitative decrease of FLCN with the absence of mutations may also lead to BHD.
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INTRODUCTION: Acute kidney injury (AKI) remains a critical issue for cancer patients despite recent treatment improvements. This study aimed to assess the incidence of AKI in cancer patients and its related risk factors. METHODS: A Retrospective cohort study was conducted at tertiary hospitals in the period 2016-2018. A data abstraction sheet was used to collect related variables from patients' records. During admission, the incidence of AKI was assessed using creatinine measurements. RIFLE criteria were used to classify it into five categories of severity: risk, injury, failure, loss, and end-stage renal disease. RESULTS: Using RIFLE (Risk, Injury, Failure, Loss, and End-stage renal disease) criteria, 6.9% of admissions were complicated with AKI. The severity of these fell into the categories of risk, injury, and failure, 3.3%, 1.7%, and 1.9%, respectively. In the multivariate model, the odds for developing AKI was significantly higher for patients with congestive heart failure (AOR = 17.1, 95% CI 1.7-80.1), chronic kidney disease (adjusted OR = 6.8, 95% CI 1.4-32.2 (P value 0.017)), sepsis (AOR = 4.4, 95% CI 1.9-10.1), hypercalcemia (AOR = 8.4, 95% CI 1.3-46.1), and admission to the ICU (AOR = 5.8, 95% CI 2.1-16.2). In addition, the mortality rate was nearly seven times higher for patients complicated by AKI (relative risk = 7.6, 95% CI 3.2-18.2). CONCLUSION: AKI was significantly associated with congestive heart failure, chronic kidney disease, sepsis, ICU admission, and hypercalcemia in cancer patients, resulting in poorer outcomes and higher mortality rates. AKI assessment for hospitalized cancer patients should be performed regularly, especially for patients at increased risk.
