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Paroxysmal nocturnal hemoglobinuria (PNH), a clonal hematopoietic stem cell disorder, arises from the increased sensitivity of red blood cells (RBC) to complement due to an acquired deficiency of certain glycosylphosphatidylinositol (GPI)-linked proteins, resulting in chronic intravascular hemolysis, arterial and venous thrombotic phenomena, multi-organ damage, and failure. We present an intriguing case of hemolytic anemia, initially suspected to be drug-induced, and later found to be associated with PNH, despite being a subclinical clone. A clinician should not hesitate to repeat fluorescent-labeled aerolysin (FLAER) cytometry if the clinical picture strongly favors a diagnosis of PNH. This case marks the importance of testing for PNH clones in autoimmune hemolytic anemia (AIHA) since their prevalence is not negligible and may correspond to a prominent hemolytic pattern, a higher thrombotic risk, and a higher therapeutic indication, such as eculizumab. This underscores the significance of conducting a thorough evaluation for occult causes of treatment-unresponsive hemolytic anemia, paving options for an early and alternative therapeutic approach.
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Introduction: Melioidosis is caused by Burkholderia pseudomallei, a Gram-negative, saprophytic bacillus, commonly found in soil or contaminated water. As infection with this bacterium produces a wide variety of clinical manifestations the organism is aptly called the 'great mimicker'. Even though it is non-fastidious and an easily cultivable organism, it can be misidentified in automated identification systems. Case report: A 24-year-old primigravida presented with complaints of fever and myalgia of 45 days' duration. She was diagnosed to have haemophagocytic lymphohistiocytosis (HLH) based on clinical and laboratory parameters. Blood and bone marrow culture sent to the microbiology laboratory grew non-fermenting Gram-negative bacilli which were misidentified as Burkholderia cepacia by matrix-assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF MS) technology. It was subsequently identified as B. pseudomallei by 16S rRNA gene sequencing. The patient was commenced on intensive phase therapy with intravenous ceftazidime for 2 weeks, followed by maintenance therapy with oral trimethoprim and sulfamethoxazole for 3 months. In view of HLH, she was treated with intravenous dexamethasone for 2 weeks which was later switched to oral dexamethasone for a period of 6 weeks. She responded well to the treatment, but had to undergo medical termination of her pregnancy as there was severe intrauterine growth restriction of the fetus. Conclusion: Prognosis of melioidosis is excellent if early diagnosis and appropriate antibiotic treatment is provided. In this era of automation, it is important to determine if the suspected pathogen is listed in the database of the automated identification system.
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Diabetics who develop severe acute respiratory syndrome coronavirus 2 (SARS CoV-2) are more likely to have severe disease, higher odds of intensive care requirement and mortality. Fifteen percent of patients have new onset hyperglycemia. We studied the comparative outcomes between prior DM, newly detected hyperglycemia and assessed role of secondary sepsis on mortality. RWe performed a r etrospective study of confirmed SARS-CoV-2 patients at a tertiary care hospital in Chennai, India. Patients were divided as 2 groups (Group 1: With preexisting diabetes mellitus, Group 2: With newly diagnosed hyperglycemia due to newly detected diabetes mellitus or non-diabetic hyperglycemia. Clinical and laboratory data was analysed. Two hundred and thirty eight patients had prior-diabetes mellitus (Group 1) and 40 had newly diagnosed hyperglycemia (Group 2). Thirty four of group 1 and 7 of group 2 patients required intensive care. Mean capillary blood glucose (MCBG) during hospital stay was 207 mg/dl (Group 1) and 192 mg/dl (Group 2). Twentysix patients (9.3%) had secondary sepsis of which sixteen died. Logistic regression identified secondary sepsis(p<0.0001), elevated D-dimer >6 fold (p= 0.0001), elderly p=0.0045), male (p=0.0006), NLR >5 (p=0.01),serum creatinine ≥2 mg/dl (p=0.0004), FiO2 requirement >0.6 in first 48 hours (p=0.001) as mortality predictors.Our study observed a 14.38 % prevalence of newly diagnosed DM or non-diabetic hyperglycemia. Secondary sepsis and >6 fold elevation in D-dimer were strong predictors of mortality. Steroid use possibly contributed to secondary sepsis. Early identification and aggressive management of secondary sepsis are necessary for diabetics.
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COVID-19 , Diabetes Mellitus , Hiperglucemia , Sepsis , Humanos , Masculino , Anciano , COVID-19/complicaciones , SARS-CoV-2 , India/epidemiología , Hiperglucemia/epidemiología , Diabetes Mellitus/epidemiología , Sepsis/complicaciones , GlucemiaRESUMEN
BACKGROUND: Arterial and venous thrombosis is one of the major complications of coronavirus disease 2019 (COVID-19) infection. Studies have not assessed the difference in D-dimer levels between patients who develop thrombosis and those who do not. METHODS: Our study retrospectively assessed D-dimer levels in all virus confirmed hospitalized patients between May to September, 2020. Patients were divided into three groups: group 1 with normal D-dimer of < 0.5 µg/mL, group 2 with elevation up to six folds, and group 3 with more than six-fold elevation. Statistical analysis was done using SPSS software 23.0. RESULTS: Seven hundred twenty patients (group1 (n = 414), group 2 (n = 284) and group 3 (n = 22)) were studied. Eight thrombotic events were observed. Events were two with stroke, two non-ST elevation myocardial infarction and one each of ST elevation myocardial infarction, superior mesenteric artery thrombosis with bowel gangrene, arteriovenous fistula thrombus and unstable angina. No significant difference (P = 0.11) was observed between median D-dimer levels among patients who developed thrombosis (1.34) and those who did not develop thrombosis (0.91). Twenty-nine patients died. The adjusted odds of death among those with a six-fold or higher elevation in D-dimer was 128.4 (95% confidence interval (CI): 14.2 - 446.3, P < 0.001), while adjusted odds of developing clinical thrombosis was 1.96 (95% CI: 0.82 - 18.2, P = 0.18). CONCLUSIONS: Our study observed a 1.1% in-hospital incidence of clinical thrombosis. While, a six-fold elevation in D-dimer was significantly associated with death; the same was not a strong predictor of thrombosis; an observation which implies that dose of anticoagulation should not be based on absolute D-dimer level.
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Background The purpose of this study was to investigate the correlation between the computed tomography pulmonary artery obstruction index and parameters of functional lung impairment in acute pulmonary embolism, and establish the value of these parameters in prognosticating right ventricular dysfunction and 30-day mortality. Methods This study included 322 consecutive patients (mean age 45.6 ± 13.2 years, 46.9% male) with acute pulmonary embolism, free of other cardiopulmonary disease, who underwent computed tomography pulmonary angiography. Correlations of arterial CO2, O2, and alveolar-arterial oxygen gradient with the computed tomography pulmonary artery obstruction index, measured using the Qanadli score, were analyzed. Logistic regression was used to identify independent predictors of right ventricular dysfunction and 30-day mortality. Results Of the 322 patients, 196 (60.9%) had right ventricular dysfunction, and 58 (18.0%) died within 30 days. The pulmonary artery obstruction index had a significant correlation with partial pressures of arterial O2 ( r = -0.887, p < 0.001) and CO2 ( r = -0.618, p = 0.019) and alveolar-arterial oxygen gradient ( r = +0.874, p < 0.001). Arterial O2 pressure had a good predictive accuracy and discriminative power for both right ventricular dysfunction (sensitivity 80.6%, specificity 85.1%, area under the curve 0.91) and 30-day mortality (sensitivity 77.8%, specificity 82.0%, area under the curve 0.89). Conclusions In patients with acute pulmonary embolism, free of other cardiopulmonary disease, parameters of functional impairment have a strong correlation with computed tomography pulmonary artery obstruction index. Hypoxia is an independent predictor of both right ventricular dysfunction and 30-day mortality in these patients.
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Arteriopatías Oclusivas/complicaciones , Hipoxia/complicaciones , Arteria Pulmonar/fisiopatología , Embolia Pulmonar/complicaciones , Disfunción Ventricular Derecha/etiología , Función Ventricular Derecha , Adulto , Área Bajo la Curva , Arteriopatías Oclusivas/diagnóstico por imagen , Arteriopatías Oclusivas/mortalidad , Arteriopatías Oclusivas/fisiopatología , Análisis de los Gases de la Sangre , Angiografía por Tomografía Computarizada , Femenino , Humanos , Hipoxia/sangre , Hipoxia/diagnóstico , Hipoxia/mortalidad , India , Modelos Logísticos , Pulmón/fisiopatología , Masculino , Persona de Mediana Edad , Oportunidad Relativa , Valor Predictivo de las Pruebas , Pronóstico , Estudios Prospectivos , Arteria Pulmonar/diagnóstico por imagen , Embolia Pulmonar/diagnóstico por imagen , Embolia Pulmonar/mortalidad , Embolia Pulmonar/fisiopatología , Curva ROC , Factores de Riesgo , Índice de Severidad de la Enfermedad , Factores de Tiempo , Disfunción Ventricular Derecha/diagnóstico por imagen , Disfunción Ventricular Derecha/mortalidad , Disfunción Ventricular Derecha/fisiopatologíaRESUMEN
Accurate identification of low-risk patients with acute pulmonary embolism (PE) who may be eligible for outpatient treatment or early discharge can have substantial cost-saving benefit. The purpose of this study was to derive and validate a prediction model to effectively identify patients with PE at low risk of short-term mortality, right ventricular dysfunction, and other nonfatal outcomes. This study analyzed data from 400 consecutive patients with acute PE. We derived and internally validated our prediction rule based on clinically significant variables that are routinely available at initial examination and that were categorized and weighted using coefficients in the multivariate logistic regression. The model was externally validated in an independent cohort of 82 patients. The final model (HOPPE score) consisted of 5 categorized patient variables (1, 2, or 3 points, respectively): systolic blood pressure (>120, 100 to 119, <99 mm Hg), diastolic blood pressure (>80, 65 to 79, <64 mm Hg), heart rate (<80, 81 to 100, >101 beats/min), arterial partial pressure of oxygen (>80, 60 to 79, <59 mm Hg), and modified electrocardiographic score (<2, 2 to 4, >4). The 30-day mortality rates were 0% in low risk (0 to 6 points), 7.5% to 8.5% in intermediate risk (7 to 10), and 18.2% to 18.8% in high-risk patients (≥11) across the derivation and validation cohorts. In comparison with the previously validated PESI score, the HOPPE score had a higher discriminatory power (area under the curve 0.74 vs 0.85, p = 0.033) and significantly improved both the discrimination (integrated discrimination improvement, p = 0.002) and reclassification (net reclassification improvement, p = 0.003) of the model for short-term mortality. In conclusion, the HOPPE score accurately identifies acute patients with PE at low risk of short-term mortality, right ventricular dysfunction, and other nonfatal outcomes. Prospective validation of the prediction model is necessary before implementation in clinical practice.
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Angiografía por Tomografía Computarizada/métodos , Embolia Pulmonar/diagnóstico , Medición de Riesgo , Enfermedad Aguda , Femenino , Estudios de Seguimiento , Humanos , Incidencia , India/epidemiología , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Pronóstico , Estudios Prospectivos , Embolia Pulmonar/epidemiología , Curva ROC , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Tasa de Supervivencia/tendenciasRESUMEN
Increase in the incidence of drug resistance and association with HIV has led to a resurgence of tuberculosis. However, tubercular arteritis continues to remain a rare entity with a prelidection for the thoracic aorta. We report a tubercular ascending aortic pseudoaneurysm in a patient already on treatment for disseminated tuberculosis who underwent successful surgical repair and also review literature pertaining to this entity.
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Aneurisma Falso/etiología , Aorta/patología , Tuberculosis Cardiovascular/complicaciones , Adulto , Aneurisma Falso/cirugía , Antituberculosos/uso terapéutico , Aorta/cirugía , Arteritis/etiología , Femenino , Humanos , Tuberculosis Cardiovascular/tratamiento farmacológico , Tuberculosis Cardiovascular/patologíaRESUMEN
Accurate diagnosis of glucose-6-phosphate dehydrogenase (G6PD) deficiency is required to avoid the risk of acute hemolysis associated with 8-aminoquinoline treatment. The performance of the BinaxNOW G6PD test compared with the quantitative spectrophotometric analysis of G6PD activity was assessed in 356 Plasmodium vivax-infected subjects in Brazil, Peru, Thailand, and India. In the quantitative assay, the median G6PD activity was 8.81 U/g hemoglobin (range = 0.05-20.19), with 11 (3%) subjects identified as deficient. Sensitivity of the BinaxNOW G6PD to detect deficient subjects was 54.5% (6 of 11), and specificity was 100% (345 of 345). Room temperatures inadvertently falling outside the range required to perform the rapid test (18-25°C) together with subtlety of color change and insufficient training could partially explain the low sensitivity found. Ensuring safe use of 8-aminoquinolines depends on additional development of simple, highly sensitive G6PD deficiency diagnostic tests suitable for routine use in malaria-endemic areas.