PRKAG2 syndrome, a rare hypertrophic cardiomyopathy: a Brazilian long-term follow-up with extracardiac disorders.

OBJECTIVE: This study aimed to provide a long-term follow-up of PRKAG2 syndrome and describe the new phenotypic aspects of the condition. PRKAG2 syndrome is a rare autosomal-dominant glycogen storage disease characterized by cardiac hypertrophy, ventricular pre-excitation, and conduction system disease. Fatal arrhythmias occur frequently. METHODS: A family cohort of 66 participants was recruited. Clinical and genetic analyses were performed. RESULTS: Median age of 36.97±17.28 years, with 69.9% being men. Nineteen subjects carried the deleterious variant p.K290I of the PRKAG2 gene. This group experienced many malignant events, including eight pacemaker implants, three sudden cardiac deaths, five aborted cardiac arrests, four strokes, four premature neonatal deaths, two spontaneous abortions, five forceps deliveries, and 12 cesarean procedures. Extracardiac involvement, such as in neurocognitive and psychiatric disorders, has been observed only in carriers of mutations. Palpitations, Syncope, atrial fibrillation, atrial flutter, sinus pauses, and bradycardia were strongly and significantly associated with major or severe adverse events (sudden cardiac death, aborted cardiac arrest, pacemaker use, stroke, and congestive heart failure). Early diagnosis and intervention through antiarrhythmic drugs, anticoagulation, pacemaker implantation, radiofrequency catheter ablation, and cesarean section surgery improved the symptoms and survival rates. Mutations carriers were advised to avoid pregnancy. CONCLUSION: This study identified that the p.K291I_PRKAG2 mutation is associated with poor prognosis, highlighting the need for early intervention. Further research may uncover the potential connections between intellectual disability, miscarriage, and neonatal death in individuals with this syndrome.

Scintigraphic and Echocardiographic Study of Patients with Pathogenic or Probably Pathogenic Variants of the TTR Gene without Overt Cardiac Involvement. / Avaliação Cintilográfica e Ecocardiográfica em Portadores de Variantes Patogênicas ou Provavelmente Patogênicas do Gene TTR sem Envolvimento Cardíaco Manifesto.

BACKGROUND: Transthyretin amyloidosis (ATTR) is an infiltrative disease caused by abnormal protein deposition mainly in the heart and peripheral nervous system. When it affects the heart, the disease presents as restrictive cardiomyopathy; when it affects the peripheral and autonomic nervous system, it manifests as polyneuropathy, and is called familial amyloid polyneuropathy (FAP). There are two ATTR subtypes: wild-type ATTR, where there is no mutation, and mutant ATTR (ATTRm), which is characterized by a mutation in the gene encoding the transthyretin protein (TTR). In both subtypes, cardiac involvement is the major marker of poor prognosis. OBJECTIVES: To assess the prevalence of subclinical cardiac involvement in a sample of patients with TTR gene mutation by using pyrophosphate scintigraphy and strain echocardiography; to compare scintigraphy and strain findings; to evaluate the association between neurological manifestations (FAP) and subclinical cardiac involvement; and to analyze whether there is an association between any specific mutation and cardiac involvement. METHODS: This is a cross-sectional study with carriers of the TTR gene mutation, without cardiovascular symptoms or changes in electrocardiographic or conventional echocardiographic parameters. All patients underwent pyrophosphate scintigraphy and strain echocardiography. Subclinical cardiac involvement was defined as a Perugini score ≥ 2, heart-to-contralateral lung (H/CL) ratio ≥ 1.5 at 1 h, H/CL ≥1.3 at 3 h, or global longitudinal strain (GLS) ≤ -17%. Descriptive and analytical analyses were performed and Fisher's exact test and Mann-Whitney test were applied. A value of p < 0.05 was considered significant. RESULTS: The 23 patients evaluated had a median age of 51 years (IQR 37-57 years), 15 (65.2%) were female, 12 (52.2%) were Pardo, nine (39.1%) had systemic arterial hypertension, and nine (39.1%) had a previous diagnosis of FAP. Of the nine patients with FAP, 8 (34.8%) were on tafamidis. The associated mutations were Val142IIe, Val50Met, and IIe127Val. The median GLS in the sample was -19% (-16% to -20%). Of the 23 patients, nine (39.1%; 95% CI = 29-49%) met criteria for cardiac involvement, six (26%) by the GLS-based criteria only. There was no association between having FAP and being an asymptomatic carrier, as assessed by strain echocardiography and pyrophosphate scintigraphy (p = 0.19). The prevalence of systemic arterial hypertension, diabetes mellitus, dyslipidemia, smoking, and reduced GLS did not differ between groups. Septal e' wave velocity was the only variable that significantly differed between individuals with and without reduced GLS, with an area under the ROC curve of 0.80 (95% CI = 0.61-0.98, p = 0.027). The best diagnostic accuracy was achieved with a septal e' velocity ≤ 8.5 cm/s. There was no association between mutation type and preclinical cardiac involvement, nor between tafamidis use and lower degree of cardiac involvement (37.5% versus 40.0%, p = 0.90). CONCLUSION: Subclinical cardiac involvement was common in a sample of TTR mutation carriers without cardiac involvement. Reduced left ventricular GLS was the most frequent finding. There was no association between the presence of amyloid polyneuropathy and subclinical cardiac involvement. Type of mutation was not associated with early cardiac involvement. In this sample, the use of tafamidis 20 mg/day was not associated with a lower prevalence of subclinical cardiac involvement.

FUNDAMENTO: A amiloidose por transtirretina (ATTR) é uma doença infiltrativa causada pela deposição anormal de proteína principalmente no coração e no sistema nervoso periférico. Quando acomete o coração, a doença manifesta-se como uma cardiomiopatia restritiva e, quando afeta o sistema nervoso periférico e autônomo, apresenta-se como uma polineuropatia, podendo ser chamada de Polineuropatia Amiloidótica Familiar (PAF). Existem dois subtipos de ATTR, a ATTR selvagem, em que não há variantes genéticas, e a ATTR hereditária, caracterizada por uma variante no gene que codifica a proteína transtirretina (T\TR). Em ambos os subtipos, o envolvimento cardíaco é o principal marcador prognóstico. OBJETIVOS: Avaliar a prevalência do envolvimento cardíaco subclínico em uma amostra de pacientes com variantes genéticas no gene TTR usando a cintilografia com pirofosfato e o ecocardiograma com strain; comparar os achados cintilográficos e as medidas de strain; avaliar a associação entre PAF e o envolvimento subclínico; e analisar se existe uma associação entre uma variante genética específica e o envolvimento cardíaco. MÉTODOS: Estudo transversal com carreadores de variantes no gene TTR sem sintomas cardiovasculares e sem alterações nos parâmetros da eletrocardiografia ou do ecocardiograma convencional. Todos os pacientes foram submetidos à cintilografia com pirofosfato e à ecocardiografia com análise de strain. O envolvimento cardíaco subclínico, definido como um escore de Perugini ≥ 2, razão Coração (C)/ Hemitórax Contralateral (CL) ≥ 1,5 em uma hora, C/CL ≥ 1,3 na terceira hora, ou um strain longitudinal global (SGL) ≤ −17%. Realizadas análises descritiva e analítica, e aplicados o teste exato de Fisher e o teste de Mann-Whitney. Um valor de p<0,05 foi considerado significativo. RESULTADOS: Os 23 pacientes avaliados apresentavam uma idade mediana de 51 (37-57) anos, 15 (65,2%) eram do sexo feminino, 12 (52,2%) eram pardos, nove (39,1%) apresentavam hipertensão arterial sistêmica, e nove (39,1%) tinham um diagnóstico prévio de PAF. Dos nove pacientes com PAF, oito (34,8%) usavam tafamidis. As variantes genéticas identificadas foram Val142IIe, Val50Met e IIe127Val. O valor mediano do SGL foi −19% (-16% ­ −20%). Dos 23 pacientes, nove (39,1%; 95% CI = 29­49%) preencheram os critérios de envolvimento cardíaco, seis (26%) somente pelo critério do SGL. Não houve associação entre PAF e um carreador assintomático avaliado por ecocardiograma com análise de strain e pela cintilografia com pirofostato (p=0,19). A prevalência de hipertensão arterial sistêmica, diabetes mellitus, dislipidemia, tabagismo e SGL reduzido não foi diferente entre os grupos. A velocidade da onda e' septal foi a única variável que apresentou diferença significativa entre os indivíduos com e sem SGL reduzido, com uma área sob a curva ROC de 0,80 (IC95% = 0,61­0,98, p = 0,027). A melhor acurácia diagnóstica foi alcançada com uma velocidade e' septal ≤ 8,5 cm/s. Não houve associação entre o tipo de variante genética e o envolvimento cardíaco pré-clínico, nem entre o uso de tafamidis e este mesmo envolvimento (37,5% versus 40,0%, p = 0,90). CONCLUSÃO: O envolvimento cardíaco subclínico foi frequente em uma amostra de carreadores da variante genética do gene TTR. Um valor do SGL reduzido foi o achado mais comum. Não houve associação entre a presença de polineuropatia amiloidótica e o envolvimento subclínico. O tipo de variante genética não foi associado com envolvimento cardíaco precoce. Nesta amostra, o uso de tafamidis (20mg/dia) não foi associado com uma menor prevalência de envolvimento cardíaco subclínico.

ANALYSIS OF HEALTHCARE ASSOCIATED AND HOSPITAL ACQUIRED INFECTIONS IN CRITICALLY ILL PATIENTS WITH CIRRHOSIS.

BACKGROUND: Bacterial infections occur in 43-59% of cirrhotic patients admitted to the intensive care unit with impact in morbidity and mortality. An increase in the frequency of multidrug-resistant (MDRO) and extensively drug-resistant (XDRO) organisms has been described in bacterial infections in cirrhotic patients with an adverse impact on survival. OBJECTIVE: To characterize community-acquired (CA), healthcare-associated (HCA), and hospital-acquired (HA) infections in cirrhotic patients and their impact in the occurrence of adverse outcomes. METHODS: This study included all cirrhotic patients admitted in an intensive care unit specialized in liver and gastrointestinal diseases in Brazil between January 2012 and June 2018. Frequency and topography of infections were retrospectively evaluated, as well as the frequency of MDRO and XDRO organisms, and their impact in occurrence of acute kidney injury, hepatorenal syndrome, acute-on-chronic liver failure, sepsis and mortality. RESULTS: A total of 374 infections were observed and classified as CA (22%), HCA (34%) and hospital-acquired (44%). Eighty-nine (54%) episodes of hospital-acquired infections were second infections. Spontaneous bacterial peritonitis (32%) and urinary tract infection (23%) were the most common infections. Culture-proven infections were positive in 61% of the cases, mainly gram-negative bacteria (73%). Acute kidney injury, hepatorenal syndrome and sepsis were observed, respectively, in 48%, 15% and 53% of the cases. MDRO and XDRO were seen, respectively, in 35% and 16%, mainly in HCA (48% vs 26% in CA infections, P=0.02) and hospital-acquired (58% vs 26% in CA infections, P=0.0009). Adverse outcomes were more frequently observed in subjects with hospital-acquired infections when compared to HCA and CA infections. Hospital-acquired, HCA and second infections were independently associated with in-hospital mortality. CONCLUSION: Hospital-acquired, HCA and second infections are increasingly associated with either MDRO and/or XDRO and are independent predictors of in-hospital mortality. Their recognition and proper selection of appropriate empiric antibiotic regimens are important measures to reduce in-hospital mortality.

Avaliação Cintilográfica e Ecocardiográfica em Portadores de Variantes Patogênicas ou Provavelmente Patogênicas do Gene TTR sem Envolvimento Cardíaco Manifesto / Scintigraphic and Echocardiographic Study of Patients with Pathogenic or Probably Pathogenic Variants of the TTR Gene without Overt Cardiac Involvement

Resumo Fundamento: A amiloidose por transtirretina (ATTR) é uma doença infiltrativa causada pela deposição anormal de proteína principalmente no coração e no sistema nervoso periférico. Quando acomete o coração, a doença manifesta-se como uma cardiomiopatia restritiva e, quando afeta o sistema nervoso periférico e autônomo, apresenta-se como uma polineuropatia, podendo ser chamada de Polineuropatia Amiloidótica Familiar (PAF). Existem dois subtipos de ATTR, a ATTR selvagem, em que não há variantes genéticas, e a ATTR hereditária, caracterizada por uma variante no gene que codifica a proteína transtirretina (T/TR). Em ambos os subtipos, o envolvimento cardíaco é o principal marcador prognóstico. Objetivos: Avaliar a prevalência do envolvimento cardíaco subclínico em uma amostra de pacientes com variantes genéticas no gene TTR usando a cintilografia com pirofosfato e o ecocardiograma com strain; comparar os achados cintilográficos e as medidas de strain; avaliar a associação entre PAF e o envolvimento subclínico; e analisar se existe uma associação entre uma variante genética específica e o envolvimento cardíaco. Métodos: Estudo transversal com carreadores de variantes no gene TTR sem sintomas cardiovasculares e sem alterações nos parâmetros da eletrocardiografia ou do ecocardiograma convencional. Todos os pacientes foram submetidos à cintilografia com pirofosfato e à ecocardiografia com análise de strain. O envolvimento cardíaco subclínico, definido como um escore de Perugini ≥ 2, razão Coração (C)/ Hemitórax Contralateral (CL) ≥ 1,5 em uma hora, C/CL ≥ 1,3 na terceira hora, ou um strain longitudinal global (SGL) ≤ −17%. Realizadas análises descritiva e analítica, e aplicados o teste exato de Fisher e o teste de Mann-Whitney. Um valor de p<0,05 foi considerado significativo. Resultados: Os 23 pacientes avaliados apresentavam uma idade mediana de 51 (37-57) anos, 15 (65,2%) eram do sexo feminino, 12 (52,2%) eram pardos, nove (39,1%) apresentavam hipertensão arterial sistêmica, e nove (39,1%) tinham um diagnóstico prévio de PAF. Dos nove pacientes com PAF, oito (34,8%) usavam tafamidis. As variantes genéticas identificadas foram Val142IIe, Val50Met e IIe127Val. O valor mediano do SGL foi −19% (-16% - −20%). Dos 23 pacientes, nove (39,1%; 95% CI = 29-49%) preencheram os critérios de envolvimento cardíaco, seis (26%) somente pelo critério do SGL. Não houve associação entre PAF e um carreador assintomático avaliado por ecocardiograma com análise de strain e pela cintilografia com pirofostato (p=0,19). A prevalência de hipertensão arterial sistêmica, diabetes mellitus, dislipidemia, tabagismo e SGL reduzido não foi diferente entre os grupos. A velocidade da onda e' septal foi a única variável que apresentou diferença significativa entre os indivíduos com e sem SGL reduzido, com uma área sob a curva ROC de 0,80 (IC95% = 0,61-0,98, p = 0,027). A melhor acurácia diagnóstica foi alcançada com uma velocidade e' septal ≤ 8,5 cm/s. Não houve associação entre o tipo de variante genética e o envolvimento cardíaco pré-clínico, nem entre o uso de tafamidis e este mesmo envolvimento (37,5% versus 40,0%, p = 0,90). Conclusão: O envolvimento cardíaco subclínico foi frequente em uma amostra de carreadores da variante genética do gene TTR. Um valor do SGL reduzido foi o achado mais comum. Não houve associação entre a presença de polineuropatia amiloidótica e o envolvimento subclínico. O tipo de variante genética não foi associado com envolvimento cardíaco precoce. Nesta amostra, o uso de tafamidis (20mg/dia) não foi associado com uma menor prevalência de envolvimento cardíaco subclínico.

Abstract Background: Transthyretin amyloidosis (ATTR) is an infiltrative disease caused by abnormal protein deposition mainly in the heart and peripheral nervous system. When it affects the heart, the disease presents as restrictive cardiomyopathy; when it affects the peripheral and autonomic nervous system, it manifests as polyneuropathy, and is called familial amyloid polyneuropathy (FAP). There are two ATTR subtypes: wild-type ATTR, where there is no mutation, and mutant ATTR (ATTRm), which is characterized by a mutation in the gene encoding the transthyretin protein (TTR). In both subtypes, cardiac involvement is the major marker of poor prognosis. Objectives: To assess the prevalence of subclinical cardiac involvement in a sample of patients with TTR gene mutation by using pyrophosphate scintigraphy and strain echocardiography; to compare scintigraphy and strain findings; to evaluate the association between neurological manifestations (FAP) and subclinical cardiac involvement; and to analyze whether there is an association between any specific mutation and cardiac involvement. Methods: This is a cross-sectional study with carriers of the TTR gene mutation, without cardiovascular symptoms or changes in electrocardiographic or conventional echocardiographic parameters. All patients underwent pyrophosphate scintigraphy and strain echocardiography. Subclinical cardiac involvement was defined as a Perugini score ≥ 2, heart-to-contralateral lung (H/CL) ratio ≥ 1.5 at 1 h, H/CL ≥1.3 at 3 h, or global longitudinal strain (GLS) ≤ −17%. Descriptive and analytical analyses were performed and Fisher's exact test and Mann-Whitney test were applied. A value of p < 0.05 was considered significant. Results: The 23 patients evaluated had a median age of 51 years (IQR 37-57 years), 15 (65.2%) were female, 12 (52.2%) were Pardo, nine (39.1%) had systemic arterial hypertension, and nine (39.1%) had a previous diagnosis of FAP. Of the nine patients with FAP, 8 (34.8%) were on tafamidis. The associated mutations were Val142IIe, Val50Met, and IIe127Val. The median GLS in the sample was −19% (−16% to −20%). Of the 23 patients, nine (39.1%; 95% CI = 29-49%) met criteria for cardiac involvement, six (26%) by the GLS-based criteria only. There was no association between having FAP and being an asymptomatic carrier, as assessed by strain echocardiography and pyrophosphate scintigraphy (p = 0.19). The prevalence of systemic arterial hypertension, diabetes mellitus, dyslipidemia, smoking, and reduced GLS did not differ between groups. Septal e' wave velocity was the only variable that significantly differed between individuals with and without reduced GLS, with an area under the ROC curve of 0.80 (95% CI = 0.61-0.98, p = 0.027). The best diagnostic accuracy was achieved with a septal e' velocity ≤ 8.5 cm/s. There was no association between mutation type and preclinical cardiac involvement, nor between tafamidis use and lower degree of cardiac involvement (37.5% versus 40.0%, p = 0.90). Conclusion: Subclinical cardiac involvement was common in a sample of TTR mutation carriers without cardiac involvement. Reduced left ventricular GLS was the most frequent finding. There was no association between the presence of amyloid polyneuropathy and subclinical cardiac involvement. Type of mutation was not associated with early cardiac involvement. In this sample, the use of tafamidis 20 mg/day was not associated with a lower prevalence of subclinical cardiac involvement.

PRKAG2 syndrome, a rare hypertrophic cardiomyopathy: a Brazilian long-term follow-up with extracardiac disorders

ABSTRACT Objective This study aimed to provide a long-term follow-up of PRKAG2 syndrome and describe the new phenotypic aspects of the condition. PRKAG2 syndrome is a rare autosomal-dominant glycogen storage disease characterized by cardiac hypertrophy, ventricular pre-excitation, and conduction system disease. Fatal arrhythmias occur frequently. Methods A family cohort of 66 participants was recruited. Clinical and genetic analyses were performed. Results Median age of 36.97±17.28 years, with 69.9% being men. Nineteen subjects carried the deleterious variant p.K290I of the PRKAG2 gene. This group experienced many malignant events, including eight pacemaker implants, three sudden cardiac deaths, five aborted cardiac arrests, four strokes, four premature neonatal deaths, two spontaneous abortions, five forceps deliveries, and 12 cesarean procedures. Extracardiac involvement, such as in neurocognitive and psychiatric disorders, has been observed only in carriers of mutations. Palpitations, Syncope, atrial fibrillation, atrial flutter, sinus pauses, and bradycardia were strongly and significantly associated with major or severe adverse events (sudden cardiac death, aborted cardiac arrest, pacemaker use, stroke, and congestive heart failure). Early diagnosis and intervention through antiarrhythmic drugs, anticoagulation, pacemaker implantation, radiofrequency catheter ablation, and cesarean section surgery improved the symptoms and survival rates. Mutations carriers were advised to avoid pregnancy. Conclusion This study identified that the p.K291I_PRKAG2 mutation is associated with poor prognosis, highlighting the need for early intervention. Further research may uncover the potential connections between intellectual disability, miscarriage, and neonatal death in individuals with this syndrome.

Análise das infecções relacionadas aos cuidados de saúde e hospitalares nos pacientes cirróticos críticos / Analysis of healthcare associated and hospital acquired infections in critically ill patients with cirrhosis

ABSTRACT Background Bacterial infections occur in 43—59% of cirrhotic patients admitted to the intensive care unit with impact in morbidity and mortality. An increase in the frequency of multidrug-resistant (MDRO) and extensively drug-resistant (XDRO) organisms has been described in bacterial infections in cirrhotic patients with an adverse impact on survival. Objective To characterize community-acquired (CA), healthcare-associated (HCA), and hospital-acquired (HA) infections in cirrhotic patients and their impact in the occurrence of adverse outcomes. Methods This study included all cirrhotic patients admitted in an intensive care unit specialized in liver and gastrointestinal diseases in Brazil between January 2012 and June 2018. Frequency and topography of infections were retrospectively evaluated, as well as the frequency of MDRO and XDRO organisms, and their impact in occurrence of acute kidney injury, hepatorenal syndrome, acute-on-chronic liver failure, sepsis and mortality. Results A total of 374 infections were observed and classified as CA (22%), HCA (34%) and hospital-acquired (44%). Eighty-nine (54%) episodes of hospital-acquired infections were second infections. Spontaneous bacterial peritonitis (32%) and urinary tract infection (23%) were the most common infections. Culture-proven infections were positive in 61% of the cases, mainly gram-negative bacteria (73%). Acute kidney injury, hepatorenal syndrome and sepsis were observed, respectively, in 48%, 15% and 53% of the cases. MDRO and XDRO were seen, respectively, in 35% and 16%, mainly in HCA (48% vs 26% in CA infections, P=0.02) and hospital-acquired (58% vs 26% in CA infections, P=0.0009). Adverse outcomes were more frequently observed in subjects with hospital-acquired infections when compared to HCA and CA infections. Hospital-acquired, HCA and second infections were independently associated with in-hospital mortality. Conclusion Hospital-acquired, HCA and second infections are increasingly associated with either MDRO and/or XDRO and are independent predictors of in-hospital mortality. Their recognition and proper selection of appropriate empiric antibiotic regimens are important measures to reduce in-hospital mortality.

RESUMO Contexto As infecções bacterianas ocorrem em 43—59% dos pacientes cirróticos internados em unidade de terapia intensiva com impacto na morbimortalidade. Um aumento na frequência de bactérias multirresistentes e com resistência estendida foi descrito em infecções bacterianas em pacientes cirróticos, com um impacto adverso na sobrevida. Objetivo Caracterizar as infecções adquiridas na comunidade, relacionadas aos cuidados de saúde (RCS) e hospitalares em pacientes cirróticos e seu impacto na ocorrência de desfechos adversos. Métodos Este estudo incluiu todos os pacientes cirróticos internados em uma unidade de terapia intensiva especializada em doenças hepáticas e gastrointestinais no Brasil entre janeiro de 2012 e junho de 2018. A frequência e topografia das infecções foram avaliadas retrospectivamente, bem como a frequência de bactérias multirresistentes e resistência estendida, e seu impacto na ocorrência de lesão renal aguda, síndrome hepatorrenal, insuficiência hepática crônica agudizada, sepse e mortalidade. Resultados Um total de 374 infecções foram observadas e classificadas como infecções adquiridas na comunidade (22%), RCS (34%) e infecções hospitalares (44%). Oitenta e nove (54%) episódios de infecções hospitalares foram identificadas como segunda infecção. Peritonite bacteriana espontânea (32%) e infecção do trato urinário (23%) foram as infecções mais comuns. As infecções comprovadas por cultura foram positivas em 61% dos casos, principalmente ocasionadas por bactérias gram-negativas (73%). Lesão renal aguda, síndrome hepatorrenal e sepse foram observados respectivamente, em 48%, 15% e 53% dos casos. Bactérias multirresistentes e resistência estendida foram observadas respectivamente, em 35% e 16%, principalmente nos RCS (48% vs 26% em infecções adquiridas na comunidade, P=0,02) e infecções hospitalares (58% vs 26% em infecções adquiridas na comunidade, P=0,0009). Os resultados adversos foram observados com mais frequência em indivíduos com infecções nosocomiais em comparação com infecções relacionadas aos cuidados de saúde e comunitárias. Infecções hospitalares, RCS e ocorrência de uma segunda infecção foram independentemente associadas à mortalidade intra-hospitalar. Conclusão Infecções hospitalares, relacionadas aos cuidados de saúde e reinfecções estão cada vez mais associadas a bactérias multirresistentes e/ou resistência estendida e são preditores independentes de mortalidade intra-hospitalar. Seu reconhecimento e seleção adequada de regimes antibióticos empíricos apropriados são medidas importantes para reduzir a mortalidade intra-hospitalar.

Dysautonomia Evaluation by Holter in Chagas Heart Disease

Abstract Background Sudden cardiac death is the main lethal mechanism associated with Chagas cardiomyopathy. Studies suggest that dysautonomia may represent a relevant, intense, independent, and early phenomenon in the natural history of the disease, even when ventricular systolic function is preserved, and may also be the mechanism that triggers malignant ventricular arrhythmias. Objective To evaluate the degree of dysautonomia and its possible association with ventricular arrhythmias in patients with Chagas cardiomyopathy, according to different categories of mortality risk, as defined by the score proposed by Rassi, used as a surrogate outcome for death. Methods A cross-sectional study involving 43 patients with Chagas cardiomyopathy stratified into risk categories based on the Rassi score, with 23 being classified as low risk and 20 as intermediate-to-high risk. Heart rate variability (HRV) was assessed using Holter monitoring for long-term recordings of 24 hours (time domain) and for short-term recordings of 5 minutes (frequency domain) at rest and after autonomic tests: deep breathing and Valsalva maneuver. The HRV variables were compared between the groups using the Student's t-test and α=0.05. Results Comparison of the results between the risk stratification groups showed no differences in HRV indexes, either in the time or frequency domain. However, results showed a significant increase in the number of arrhythmias as a function of increased risk (p=0.02). Conclusion There was no association between the degree of dysautonomia, evaluated by Holter monitoring, and the categories of mortality risk, despite a direct association between the rate of arrhythmias and the higher risk group.

Decompensated chagasic heart failure versus non-chagasic heart failure at a tertiary care hospital: Clinical characteristics and outcomes.

OBJECTIVE: To evaluate clinical and epidemiological characteristics and clinical outcomes in patients hospitalized with decompensated heart failure (DHF), with a comparison between Chagas and non-Chagas disease. METHOD: This is a retrospective cohort study involving 136 patients consecutively admitted with DHF between January 1 and December 31, 2011, with the following outcomes: acute renal failure, cardiogenic shock, rehospitalization, and hospital death. Individuals aged ≥ 18 years with DHF were included while those with more than 10% of missing data regarding outcomes were excluded. Statistical analysis was performed using SPSS version 17.0. Chi-squared test was used to compare proportions. Student's T test was used to compare means. Kaplan-Meier and log-rank tests were used to compare rehospitalization rates between the two groups over time. RESULTS: Chagasic and non-chagasic patients were compared. The first had lower mean systolic blood pressure (111.8±18.4 versus 128.8±24.4, p<0.01), lower mean diastolic blood pressure (74.5±13.6 versus 82.0±15.2, p<0.01) and lower left ventricular ejection fraction (26.5±6.2 versus 41.5±18.9, p<0.01). In all, 20 patients with Chagas (50.1%) were rehospitalized, compared to 35 patients in the non-Chagas group (35.4%, p=0.04). Log rank test = 4.5 (p<0.01) showed that rehospitalization rates between the two groups over time (Kaplan-Meier curves) differed. CONCLUSION: Chagas disease was associated with lower systolic and diastolic blood pressure and lower left ventricular ejection fraction. The rehospitalization rate was higher in Chagas disease.

Predictive performance of BI-RADS magnetic resonance imaging descriptors in the context of suspicious (category 4) findings.

OBJECTIVE: To determine the positive predictive value (PPV) and likelihood ratio for magnetic resonance imaging (MRI) characteristics of category 4 lesions, as described in the Breast Imaging Reporting and Data System (BI-RADS(®)) lexicon, as well as to test the predictive performance of the descriptors using multivariate analysis and the area under the curve derived from a receiver operating characteristic (ROC) curve. MATERIALS AND METHODS: This was a double-blind review study of 121 suspicious findings from 98 women examined between 2009 and 2013. The terminology was based on the 2013 edition of the BI-RADS. RESULTS: Of the 121 suspicious findings, 53 (43.8%) were proven to be malignant lesions, with no significant difference between mass and non-mass enhancement (p = 0.846). The PPVs were highest for masses with a spiculated margin (71%) and round shape (63%), whereas segmental distribution achieved a high PPV (80%) for non-mass enhancement. Kinetic analyses performed poorly, except for type 3 curves applied to masses (PPV of 73%). Logistic regression models were significant for both patterns, although the results were better for masses, particularly when kinetic assessments were included (p = 0.015; pseudo R(2) = 0.48; area under the curve = 90%). CONCLUSION: Some BI-RADS MRI descriptors have high PPV and good predictive performance-as demonstrated by ROC curve and multivariate analysis-when applied to BI-RADS category 4 findings. This may allow future stratification of this category.

OBJETIVO: Determinar o valor preditivo positivo (VPP) e a razão de verossimilhança positiva de características de ressonância magnética (RM) de lesões da categoria 4, como descritas no léxico do Breast Imaging Reporting and Data System (BI-RADS®), e testar o desempenho preditivo dos descritores por meio de análise multivariada e área sob a curva derivada da curva receiver operating characteristic (ROC). MATERIAIS E MÉTODOS: Foi realizado um estudo revisional duplo-cego de 121 achados suspeitos em 98 mulheres examinadas entre 2009 e 2013. A terminologia foi baseada na edição de 2013 do BI-RADS. RESULTADOS: Dos 121 achados suspeitos, 53 (43,8%) eram de fato lesões malignas, sem diferença significativa entre nódulos e realce não nodular (p = 0,846). Nódulos com margem espiculada (71%) e forma redonda (63%) apresentaram os maiores VPPs, ao passo que a distribuição segmentar teve alto VPP para realce não nodular (80%). Apenas a curva cinética do tipo 3 teve bom desempenho quando aplicada a nódulos (VPP = 73%). Modelos de regressão logística foram significantes para os dois padrões principais, embora os nódulos tenham apresentado resultados melhores, particularmente com a introdução da análise cinética (p = 0,015; pseudo-R2 = 0,48; área sob a curva = 90%). CONCLUSÃO: Alguns descritores de RM do BI-RADS têm alto VPP e bom desempenho preditivo - demonstrado por curva ROC e análise multivariada - quando aplicados a achados da categoria 4 segundo o BI-RADS. Isso pode permitir futura estratificação dessa categoria.

Septic versus non-septic acute kidney injury in critically ill patients: characteristics and clinical outcomes.

OBJECTIVE: This study aimed to describe and compare the characteristics and clinical outcomes of patients with septic and non-septic acute kidney injury. METHODS: This study evaluated an open cohort of 117 critically ill patients with acute kidney injury who were consecutively admitted to an intensive care unit, excluding patients with a history of advanced-stage chronic kidney disease, kidney transplantation, hospitalization or death in a period shorter than 24 hours. The presence of sepsis and in-hospital death were the exposure and primary variables in this study, respectively. A confounding analysis was performed using logistic regression. RESULTS: No significant differences were found between the mean ages of the groups with septic and non-septic acute kidney injury [65.30±21.27 years versus 66.35±12.82 years, respectively; p=0.75]. In the septic and non-septic acute kidney injury groups, a predominance of females (57.4% versus 52.4%, respectively; p=0.49) and Afro-descendants (81.5% versus 76.2%, respectively; p=0.49) was observed. Compared with the non-septic patients, the patients with sepsis had a higher mean Acute Physiology and Chronic Health Evaluation II score [21.73±7.26 versus 15.75±5.98; p<0.001)] and a higher mean water balance (p=0.001). Arterial hypertension (p=0.01) and heart failure (p<0.001) were more common in the non-septic patients. Septic acute kidney injury was associated with a greater number of patients who required dialysis (p=0.001) and a greater number of deaths (p<0.001); however, renal function recovery was more common in this group (p=0.01). Sepsis (OR: 3.88; 95%CI: 1.51-10.00) and an Acute Physiology and Chronic Health Evaluation II score >18.5 (OR: 9.77; 95%CI: 3.73-25.58) were associated with death in the multivariate analysis. CONCLUSION: Sepsis was an independent predictor of death. Significant differences were found between the characteristics and clinical outcomes of patients with septic versus non-septic acute kidney injury.

Prognostic factors in patients with acute coronary syndrome without ST segment elevation.

BACKGROUND: In patients with acute coronary syndromes (ACS) without ST segment elevation (SSE), it is suggested that a series of markers (inflammatory cells, hyperglycemia and renal function) can identify individuals with increased risk for cardiovascular events. OBJECTIVE: To evaluate the impact of these laboratory parameters on intra-hospital outcomes of patients with ACS with no SSE. METHODS: We prospectively evaluated 195 patients consecutively admitted with ACS with no SSE. Demographic and clinical laboratory data were recorded during the course of the hospitalization period in relation to the occurrence of combined events. RESULTS: Mean age was 67 ± 12 years, and 52% were men. In analyzing the area under the ROC curve, only neutrophil/lymphocyte ratios (AUC: 70%, CI 95% 56%-82%, p = 0.006) and creatinine (AUC: 62%, CI 95% 50%-80%, p = 0.03) discriminated those patients with ACS with no SSE who presented an adverse outcome. The patients who suffered an adverse event during hospitalization had lower lymphocyte counts (1502 ± 731/mm ³ vs. 2020 ± 862/mm³; p = 0.002), lower glomerular filtration rates (51 ± 27 mL / min vs. 77 ± 34 mL/min; p < 0,001) and higher serum creatinine levels (2.1 ± 2.7 mg/dL vs. 1.1 ± 1.3 mg/dL; p = 0.047) than those who progressed through hospitalization without incident. The logistic regression analysis showed that variables remaining as independent and significant predictors were: glomerular filtration rate (OR: 1.03; CI 95%: 1.00 - 1.13, p = 0.002), and lymphocyte count (OR: 1.02; CI95%: 1.01 to 1.04, p = 0.03). CONCLUSION: Assessment of renal function and lymphocyte count provide potentially useful information for the prognostic stratification in patients with ACS with no SSE (Arq Bras Cardiol. 2013; [online].ahead print, PP.0-0).

Extubation failure influences clinical and functional outcomes in patients with traumatic brain injury.

OBJECTIVE: To evaluate the association between extubation failure and outcomes (clinical and functional) in patients with traumatic brain injury (TBI). METHODS: A prospective cohort study involving 311 consecutive patients with TBI. The patients were divided into two groups according to extubation outcome: extubation success; and extubation failure (defined as reintubation within 48 h after extubation). A multivariate model was developed in order to determine whether extubation failure was an independent predictor of in-hospital mortality. RESULTS: The mean age was 35.7 ± 13.8 years. Males accounted for 92.3%. The incidence of extubation failure was 13.8%. In-hospital mortality was 4.5% and 20.9% in successfully extubated patients and in those with extubation failure, respectively (p = 0.001). Tracheostomy was more common in the extubation failure group (55.8% vs. 1.9%; p < 0.001). The median length of hospital stay was significantly greater in the extubation failure group than in the extubation success group (44 days vs. 27 days; p = 0.002). Functional status at discharge was worse among the patients in the extubation failure group. The multivariate analysis showed that extubation failure was an independent predictor of in-hospital mortality (OR = 4.96; 95% CI, 1.86-13.22). CONCLUSIONS: In patients with TBI, extubation failure appears to lengthen hospital stays; to increase the frequency of tracheostomy and of pulmonary complications; to worsen functional outcomes; and to increase mortality.

Association between the rapid shallow breathing index and extubation success in patients with traumatic brain injury.

OBJECTIVE: To investigate the association between the rapid shallow breathing index and successful extubation in patients with traumatic brain injury. METHODS: This study was a prospective study conducted in patients with traumatic brain injury of both genders who underwent mechanical ventilation for at least two days and who passed a spontaneous breathing trial. The minute volume and respiratory rate were measured using a ventilometer, and the data were used to calculate the rapid shallow breathing index (respiratory rate/tidal volume). The dependent variable was the extubation outcome: reintubation after up to 48 hours (extubation failure) or not (extubation success). The independent variable was the rapid shallow breathing index measured after a successful spontaneous breathing trial. RESULTS: The sample comprised 119 individuals, including 111 (93.3%) males. The average age of the sample was 35.0 ± 12.9 years old. The average duration of mechanical ventilation was 8.1 ± 3.6 days. A total of 104 (87.4%) participants achieved successful extubation. No association was found between the rapid shallow breathing index and extubation success. CONCLUSION: The rapid shallow breathing index was not associated with successful extubation in patients with traumatic brain injury.

Decompensated chagasic heart failure versus non-chagasic heart failure at a tertiary care hospital: Clinical characteristics and outcomes / Insuficiência cardíaca descompensada chagásica versus não chagásica em um hospital de atenção terciária: características e desfechos clínicos

Summary Objective: To evaluate clinical and epidemiological characteristics and clinical outcomes in patients hospitalized with decompensated heart failure (DHF), with a comparison between Chagas and non-Chagas disease. Method: This is a retrospective cohort study involving 136 patients consecutively admitted with DHF between January 1 and December 31, 2011, with the following outcomes: acute renal failure, cardiogenic shock, rehospitalization, and hospital death. Individuals aged ≥ 18 years with DHF were included while those with more than 10% of missing data regarding outcomes were excluded. Statistical analysis was performed using SPSS version 17.0. Chi-squared test was used to compare proportions. Student's T test was used to compare means. Kaplan-Meier and log-rank tests were used to compare rehospitalization rates between the two groups over time. Results: Chagasic and non-chagasic patients were compared. The first had lower mean systolic blood pressure (111.8±18.4 versus 128.8±24.4, p<0.01), lower mean diastolic blood pressure (74.5±13.6 versus 82.0±15.2, p<0.01) and lower left ventricular ejection fraction (26.5±6.2 versus 41.5±18.9, p<0.01). In all, 20 patients with Chagas (50.1%) were rehospitalized, compared to 35 patients in the non-Chagas group (35.4%, p=0.04). Log rank test = 4.5 (p<0.01) showed that rehospitalization rates between the two groups over time (Kaplan-Meier curves) differed. Conclusion: Chagas disease was associated with lower systolic and diastolic blood pressure and lower left ventricular ejection fraction. The rehospitalization rate was higher in Chagas disease.

Resumo Objetivo: Avaliar características clínico-epidemiológicas e desfechos clínicos em pacientes internados por insuficiência cardíaca descompensada (ICD), estabelecendo uma comparação entre pacientes chagásicos e não chagásicos. Método: Trata-se de um estudo de coorte retrospectivo abrangendo 136 pacientes internados consecutivamente com ICD entre 1 de janeiro e 31 de dezembro de 2011, tendo como desfechos: lesão renal aguda, choque cardiogênico, reinternamento e óbito hospitalar. Foram incluídos indivíduos com idade ≥ 18 anos com ICD e excluídos aqueles com mais de 10% de dados faltantes em relação aos desfechos. Para a análise estatística, foi utilizado o SPSS® versão 17.0. Para a comparação entre proporções, foi utilizado o teste Qui-quadrado. O teste T de Student foi utilizado para comparar médias. Utilizamos as curvas de Kaplan-Meier e o teste do log rank para comparar as taxas de reinternações entre os dois grupos ao longo do tempo. Resultados: Na comparação entre chagásicos e não chagásicos, os primeiros apresentaram menor média de pressão arterial sistêmica (111,8±18,4 versus 128,8±24,4; p<0,01), menor média de pressão arterial diastólica (74,5±13,6 versus 82,0±15,2; p<0,01) e menor fração de ejeção do ventrículo esquerdo (26,5±6,2 versus 41,5±18,9; p<0,01). Um total de 20 chagásicos (50,1%) reinternaram contra 35 não chagásicos (35,4%; p=0,04). O teste do log rank = 4,5 (p<0,01) mostrou que as taxas de reinternações entre os dois grupos ao longo do tempo (curvas de Kaplan-Meier) diferiram. Conclusão: A doença de Chagas associou-se a menores valores de pressão arterial sistólica e diastólica, além de menor fração de ejeção do ventrículo esquerdo. A taxa de reinternamento foi maior em chagásicos.

Predictive performance of BI-RADS magnetic resonance imaging descriptors in the context of suspicious (category 4) findings / Desempenho preditivo dos descritores de ressonância magnética do BI-RADS no contexto de achados suspeitos (categoria 4)

Abstract Objective: To determine the positive predictive value (PPV) and likelihood ratio for magnetic resonance imaging (MRI) characteristics of category 4 lesions, as described in the Breast Imaging Reporting and Data System (BI-RADS®) lexicon, as well as to test the predictive performance of the descriptors using multivariate analysis and the area under the curve derived from a receiver operating characteristic (ROC) curve. Materials and Methods: This was a double-blind review study of 121 suspicious findings from 98 women examined between 2009 and 2013. The terminology was based on the 2013 edition of the BI-RADS. Results: Of the 121 suspicious findings, 53 (43.8%) were proven to be malignant lesions, with no significant difference between mass and non-mass enhancement (p = 0.846). The PPVs were highest for masses with a spiculated margin (71%) and round shape (63%), whereas segmental distribution achieved a high PPV (80%) for non-mass enhancement. Kinetic analyses performed poorly, except for type 3 curves applied to masses (PPV of 73%). Logistic regression models were significant for both patterns, although the results were better for masses, particularly when kinetic assessments were included (p = 0.015; pseudo R2 = 0.48; area under the curve = 90%). Conclusion: Some BI-RADS MRI descriptors have high PPV and good predictive performance-as demonstrated by ROC curve and multivariate analysis-when applied to BI-RADS category 4 findings. This may allow future stratification of this category.

Resumo Objetivo: Determinar o valor preditivo positivo (VPP) e a razão de verossimilhança positiva de características de ressonância magnética (RM) de lesões da categoria 4, como descritas no léxico do Breast Imaging Reporting and Data System (BI-RADS®), e testar o desempenho preditivo dos descritores por meio de análise multivariada e área sob a curva derivada da curva receiver operating characteristic (ROC). Materiais e Métodos: Foi realizado um estudo revisional duplo-cego de 121 achados suspeitos em 98 mulheres examinadas entre 2009 e 2013. A terminologia foi baseada na edição de 2013 do BI-RADS. Resultados: Dos 121 achados suspeitos, 53 (43,8%) eram de fato lesões malignas, sem diferença significativa entre nódulos e realce não nodular (p = 0,846). Nódulos com margem espiculada (71%) e forma redonda (63%) apresentaram os maiores VPPs, ao passo que a distribuição segmentar teve alto VPP para realce não nodular (80%). Apenas a curva cinética do tipo 3 teve bom desempenho quando aplicada a nódulos (VPP = 73%). Modelos de regressão logística foram significantes para os dois padrões principais, embora os nódulos tenham apresentado resultados melhores, particularmente com a introdução da análise cinética (p = 0,015; pseudo-R2 = 0,48; área sob a curva = 90%). Conclusão: Alguns descritores de RM do BI-RADS têm alto VPP e bom desempenho preditivo - demonstrado por curva ROC e análise multivariada - quando aplicados a achados da categoria 4 segundo o BI-RADS. Isso pode permitir futura estratificação dessa categoria.

Simple magnetic resonance imaging criteria can differentiate ductal carcinomasin situ from invasive carcinomas / Critérios simples de imagem por ressonância mangética podem diferenciar carcinomas ductais in situ de carcinomas invasivos

Objective: To investigate if apparent diffusion coefficient (ADC) values can discriminate ductal carcinomas in situ (DCIS) from invasive carcinomas and to test the incremental gain of a model combining these measurements to dynamic contrast-enhanced (DCE) main pattern (mass versus non-mass). Methods: Forty-four lesions (12 DCIS and 32 invasive cancers) were reviewed by two examiners, their ADCs were averaged, and they were classified according to enhancement patterns. A logistic regression model with ADC values and enhancement patterns was devised. Receiver operating characteristic (ROC) curves were used to compare the discriminative performance of isolated ADCs to the regression model by their areas under the curve (AUCs). Results: ADC values were significantly different between lesion types (p=0.034), with mean of 1.23x10-3 mm2/s for DCIS and 1.05x10-3 mm2 /s for invasive cancers. The model grouping enhancement patterns and ADC values had better performance AUC=0.80) than isolated ADCs (AUC=0.71), though the difference was not statistically significant (p=0.105). Conclusion: ADC measurements of pre-invasive breast lesions are substantially different from those of invasive cancers. When ADC measurements are associated with main enhancement patterns, the performance of the technique is increased.

Objetivo: Investigar se valores de coeficiente de difusão aparente (ADC) podem discriminar carcinomas ductais in situ (CDIS) de carcinomas invasivos e testar o ganho incremental de modelo combinando tais medidas ao padrão principal de realce (nódulo versus realce não nodular) do estudo contrastado dinâmico (ECD). Métodos: Quarenta e quatro lesões (12 CDIS e 32 cânceres invasivos) foram revisadas por dois examinadores, seus ADCs médios calculados e elas foram classificadas de acordo com padrões de realce. Um modelo de regressão logística com valores de ADC e padrões de realce foi delineado. Curvas receiver operating characteristic (ROC) foram utilizadas para comparar a performance discriminativa dos ADCs isolados ao modelo de regressão através de suas áreas sob a curva (AUCs). Resultados: Os valores de ADC foram significantemente diferentes entre tipos de lesão (p=0,034), com média de 1,23x10-3 mm2 /s para CDIS e 1,05x10-3 mm2 /s para cânceres invasivos. O modelo agrupando padrões de realce e valores de ADC teve melhor performance (AUC=0,80) do que ADCs isolados (AUC=0,71), ainda que a diferença não tenha sido estatisticamente significante (p=0,105). Conclusão: Medidas de ADC de lesões mamárias pré-invasivas são substancialmente diferentes daquelas de cânceres invasivos. Quando medidas de ADC são associadas aos principais padrões de realce, a performance da técnica é aumentada.

Nova mutação no gene PRKAG2 associado a parada cardiorrespiratória, insuficiência cardíaca congestiva e múltiplas vias acessórias / A novel mutation in the PRKAG2 gene associated with cardiorespiratory arrest, congestive heart failure and accessory pathways

Relata-se o caso de paciente do sexo masculino portador de síndrome de Wolff-Parkinson-White,hipertrofia ventricular e doença do sistema de condução, que apresentou duas paradas cardiorrespiratórias,insuficiência cardíaca congestiva e uma nova variação no gene PRKAG2.

We report the case of a male patient suffering from Wolff-Parkinson-White syndrome, ventricularhypertrophy, cardiac conduction system disease, who presented two cardiorespiratory arrests, congestive heartfailure and a new variation in the PRKAG2 gene.

Anticorpos anti-C1q e sua correlação com atividade do lúpus eritematoso sistêmico / Anti-C1q antibodies and systemic lupus erythematosus activity

JUSTIFICATIVA E OBJETIVO: Anticorpos Anti-C1q têm sido fortemente associados a envolvimento renal por lúpus eritematoso sistêmico. A principal hipótese para explicar a patogênese dos anticorpos anti-C1q no lúpus eritematoso sistêmico é a de que a doença é exacerbada por um decréscimo na depuração de células apoptóticas. O objetivo deste estudo foi verificar a associação entre títulos séricos de anti-C1q em portadores de lúpus eritematoso sistêmico, quanto à atividade da doença e quanto às suas manifestações clínicas, com ênfase para manifestações renais. MÉTODOS: Estudo de corte transversal realizado com 62 pacientes com diagnóstico recente de lúpus eritematoso sistêmico, quanto à presença ou não de autoanticorpos anti-C1q no soro, no período de junho a dezembro de 2012. Os critérios clínicos utilizados para caracterizar nefrite lúpica foram: (1) anormalidades ao sumário de urina, como proteinúria de 24 horas >500mg/24 horas ou (2) cilindrúria (cilindros granulosos, hemáticos ou leucocitários), ou (3) aumento da creatinina sérica em relação à creatinina imediatamente anterior >50%. RESULTADOS: A amostra foi constituída predominantemente por mulheres jovens e afrodescendentes. Observou-se que os títulos de anti-C1q se associaram à atividade de lúpus eritematoso sistêmico de maneira geral, uma vez que a correlação de Sperman apresentou correlação moderada positiva entre o escore Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) e a titulação de anti-C1q sérico...

BACKGROUND AND OBJECTIVE: Anti-C1q antibodies have been strongly associated with renal involvement in systemic lupus erythematosus. The main hypothesis to explain the pathogenesis of anti-C1q antibodies in systemic lupus erythematosus is that the condition is exacerbated by a decrease in the clearance of apoptotic cells. This study aimed to assess the association between serum titers of anti-C1q with activity systemic lupus erythematosus and with its clinical manifestations, with emphasis on renal manifestations. METHODS: This was a cross-sectional study conducted on 62 patients with newly diagnosed systemic lupus erythematosus for the presence or absence of anti-C1q autoantibodies in serum, in the period June to December 2012. The clinical criterioa used to characterize lupus nephritis were: (1) presence of proteinuria 24 hours >500mg/24 hours, or (2) urinary casts (granular cylinders, hematic or leukocyte), or (3) increased serum creatinine >50% related to the basal levels. RESULTS: The sample was mainly constituted by young females and afrodescendent. We observed that the titers of anti-C1q were associated with the activity of systemic lupus erythematosus in general, since the Spearman correlation showed moderate positive correlation between the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score and the titration of anti-C1q serum...

Lesão renal aguda em pacientes em uso de inibidor da enzima conversora de angiotensina para tratar insuficiência cardíaca descompensada / Acute kidney injury in patients using angiotensin-converting enzyme inhibitors to treat decompensated heart failure

JUSTIFICATIVA E OBJETIVO: Esse estudo procurou descrevera incidência de lesão renal aguda (LRA) em pacientes com insuficiência cardíaca (IC) descompensada pós uso de inibidor de enzima conversora de angiotensina (IECA) e o perfil clínico-epidemiológico desses pacientes. MÉTODOS: Trata-se de um estudo de coorte prospectiva. Foram incluídos no estudo pacientes com insuficiência cardíaca classe IV segundo critérios doNew York Heart Association (NYHA) descompensada admitidos nas enfermarias de Clínica Médica do Hospital Santo Antônio no período de 01/03/2011 a 30/10/2012. Foram excluídos pacientescom doença renal crônica estágios III, IV, V e com dados incompletos. A lesão renal aguda foi definida de acordo como critério RIFLE (Risk/Injury/Failure/Loss/End-stage). Os dados foram analisados através do programa estatístico SPSS 14.0.Esse projeto foi aprovado pelo Comitê de Ética e Pesquisa do Hospital Santo Antônio. RESULTADOS: Dos 100 pacientes estudados, a maioria era do sexo masculino, de etnia afrodescendentee apresentavam como etiologia da insuficiência cardíaca amiocardiopatia chagásica crônica. O sexo feminino, a presença de hipertensão arterial prévia, maiores valores médios basais depressão arterial sistólica (PAS) e pressão arterial diastólica (PAD)e maiores valores médios de idade foram associados à ocorrência de lesão renal aguda, bem como valores médios mais elevados de creatinina sérica basal. Doses médias maiores de inibidores de enzima conversora de angiotensina e de furosemida venosadurante a primeira semana de tratamento foram associadas à ocorrência lesão renal aguda. A área sob a curva ROC (Receiver Operating Characteristic) Curve (AuROC) para uso de inibidores da enzima conversora de angiotensina foi de 0,70 com p=0,001...

BACKGROUND AND OBJECTIVE: This study sought to describe the incidence of acute kidney injury (AKI) in patients with decompensated heart failure after angiotensin converting enzyme (ACE) inhibitors and the clinical - epidemiological profile of these patients. METHODS: This is a prospective cohort study. Patients with New York Heart Association (NYHA) class IV were included in the study. They were admitted in thewards of Internal Medicine, Hospital Santo Antonio in theperiod from 01/03/2011 to 30/10/2012. Patients with chronic kidney disease stages III, IV, V, and without complete data were excluded. Acute kidney injury was defined according to the RIFLE (Risk/Injury/Failure/Loss/End-stage) criteria. Data were analyzed using SPSS 14.0 statistical program. This project was approved by the Ethics and Research Comitee of Hospital St. Anthony. RESULTS: Of the 100 patients, the majority were male, of african descente and and had Chagas´ cardiomyopathy as a cause of heart failure. Females, the presence of previous hypertension and higher baseline mean of diastolic or sistolic pressure and higher mean values of age were associated with the occurrence of acute kidney injury, as well as higher values of baseline serum creatinine. Higher doses of angiotensin converting enzyme inhibitors and furosemide were associated with the occurrence of renal injury. The area under the Receiver Operating Characteristic (ROC) Curve (AUROC) for angiotensin converting enzyme inhibitors (ACEI) was 0.70 with p-0.001. CONCLUSION: High doses of angiotensin converting enzyme inhibitors and intravenous furosemide are associated with acute kidney injury especially in the first week of introduction of angiotensin converting enzyme inhibitors...