Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone / Tratamento da doença de depósito de cadeia leve idiopática: remissão completa com bortezomib e dexametasona

Abstract Light chain deposition disease (LCDD) is a rare clinical entity characterized by the deposition of light chain immunoglobulins in different tissues and primarily affects the kidneys, followed by the liver and heart. This disease often manifests as nephrotic syndrome with marked proteinuria and rapid deterioration of renal function. More than 50% of cases are secondary to multiple myeloma or other lymphoproliferative diseases, with a well-established treatment aimed at controlling the underlying disease. In rare cases, there is no detection of an associated hematological disease, referred to as idiopathic LCDD. In these cases, there is no evidence-based consensus on the therapeutic approach, and management is based on the clinical experience of reported cases. Here we report a case of idiopathic LCDD treated with bortezomib and dexamethasone with complete hematologic responses, significant reduction of proteinuria, and improved renal function.

Resumo A doença de deposição de cadeia leve (DDCL) é uma entidade clínica rara caracterizada pela deposição de cadeias leves das imunoglobulinas em diferentes tecidos e afeta principalmente os rins, seguido pelo fígado e coração. Manifesta-se frequentemente como síndrome nefrótica com proteinúria marcante e rápida deterioração da função renal. Mais de 50% dos casos são secundários ao mieloma múltiplo ou outras doenças linfoproliferativas, tendo seu tratamento bem estabelecido, voltado para o controle da doença de base. Em casos raros, não há detecção de uma doença hematológica associada, sendo referida como DDCL idiopática. Nestes casos, não há um consenso baseado em evidências sobre a abordagem terapêutica, tendo sua conduta baseada na experiência clínica dos casos relatados. Aqui, nós relatamos um caso de DDCL idiopática tratado com bortezomib e dexametasona atingindo resposta hematológica completa, redução significativa da proteinúria e recuperação da função renal.

Aminotransferases analysis in patients with dengue serotype IV / Análise das aminotransferases em pacientes com dengue sorotipo IV

OBJECTIVE: To evaluate the aminotransferase levels inpatients with dengue serotype IV. METHODS: We conducteda cross-sectional analysis of 3,596 people with laboratory confirmed dengue. RESULTS: The analysis showed that 49.2%(1,768) had normal aminotransferase levels, 43.4% (1,559)had changes in levels by up to three times the upper limit ofnormal, 7.0% (253) had elevations three times the referencevalue and 0.4% (16) developed acute hepatitis. Patients withthrombocytopenia and hemoconcentration had major changesin the levels of enzymes (p<0.05). Women were more susceptibleto those changes than men (p<0,05). The average AST and ALTwere higher in dengue hemorrhagic type (147.51±137.74U/Land 111.54±81.27U/L) as compared to dengue fever(55.18±52.06U/L and 60.39±57.50U/L) (p<0.05). Leukopenicpatients had aminotransferases average lower compared toindividuals without any drop in overall white blood cellcount (p<0.05). A total of 224 patients were submitted tohospitalization, with the AST average of 88.18±100.47U/Land ALT 77.95±88.38U/L, which is higher than the average inambulatory patients (p<0.05). CONCLUSION: A significantnumber of patients was found with altered levels of enzymes,which requires clinical laboratory monitoring for an extendedperiod.

OBJETIVO: Avaliar os níveis de aminotransferases em pacientes com dengue sorotipo IV. MÉTODOS: Foi realizada uma análise transversal de 3.596 pessoas com dengue confirmada laboratorialmente. RESULTADOS: A análise mostrou que49,2% (1.768) tinham níveis normais de aminotransferases,43,4% (1.559) mostraram alterações nos níveis em até três vezes o limite superior da normalidade, 7,0% (253) apresentaram elevações três vezes o valor de referência e 0,4% (16) desenvolveram hepatite aguda. Os doentes com trombocitopenia e hemoconcentração tiveram grandes alterações nos níveis enzimáticos(p<0,05). As mulheres foram mais suscetíveis a essas alterações do que os homens (p<0,05). As médias de AST e ALT foram maiores na dengue do tipo hemorrágica (147,51±137,74U/Le 111,54±81,27U/L) em comparação com dengue clássica(55,18±52,06U/L e 60,39±57,50U/L) (p<0,05). Pacientes leucopênicos tiveram a média de aminotransferases menor em comparação com os indivíduos sem qualquer queda na contagem global de glóbulos brancos (p<0,05). Um total de 224pacientes foi submetido a hospitalização, com a média de AST88,18±100,47U/L e ALT 77,95±88,38U/L, o que é mais elevado do que a média em doentes ambulatórios (p<0,05). CONCLUSÃO:Um número significativo de pacientes foi identificado com níveis alterados de enzimas, o que exige acompanhamento clínico e laboratorial por um período prolongado.

Comparison of clinical and laboratory characteristics between children and adults with dengue

Over the past several years, the epidemiological profile of dengue has been changing progressively and is currently characterized by an increase in the number of cases in children under 15 years of age. This study was aimed at comparing the clinical and laboratory features between adults and children with dengue; therefore, we performed a cross-sectional analysis of 5686 individuals with laboratory-confirmed dengue who sought treatment at a healthcare facility in Rio de Janeiro, Brazil from 2010 to 2011. A multivariate analysis indicated that myalgia (OR = 2.58; CI 95% = 2.08-3.18), retro-orbital pain (OR = 1.36; CI 95% = 1.15-1.62), nausea (OR = 1.92; CI 95% = 1.60-2.30), and arthralgia (OR = 3.64; CI 95% = 2.72-4.89) were the most frequent clinical symptoms in adults, whereas vomiting (OR = 0.52; CI 95% = 0.43-0.61) and skin rash (OR = 0.46; CI 95% = 0.25-0.85) were the most prevalent symptoms in children. Adults exhibited a higher hemoconcentration (OR = 3.04; CI 95% = 2.53-3.65), thrombocytopenia (OR = 2.17; CI 95% = 1.80-2.60), increased erythrocyte sedimentation rate (OR = 1.81; CI 95% = 1.53-2.14), and increased ALT (OR = 3.13; CI 95% = 2.44-4.02) than did children. In addition, adults exhibited a higher frequency of the severe forms of the disease (OR = 1.74; CI 95% = 1.12-2.72) and hospitalization (OR = 2.21; CI 95% = 1.59-3.06) relative to children. Based on these results, this study demonstrated significant differences in the clinical and laboratory presentations and disease severity between adults and children affected by dengue.

Aminotransferase changes and acute hepatitis in patients with dengue fever: analysis of 1, 585 cases

INTRODUCTION: Type 3 dengue virus caused an extensive epidemic in the state of Rio de Janeiro in summer 2002. In some of the patients, it was found in an atypical form with increased aminotransferase levels and acute hepatitis. MATERIAL AND METHODS: An analysis was made of 1,585 serologically confirmed dengue cases at the Dengue Reference Center in Campos dos Goytacazes, Rio de Janeiro state. The grade of hepatic aggression was established according to the alterations in the aminotransferase levels: grade A - normal levels of aminotransferase; grade B - elevated aminotransferase, with increased levels of at least one of the enzymes; grade C - elevated aminotransferase, with the levels of at least one of the enzymes increased to more than three times the reference values; grade D - acute hepatitis, with aminotransferase levels increased to at least 10 times their normal values. RESULTS: Among the 1,585 serologically confirmed dengue cases, 44.5 percent presented alterations in the aminotransferase levels (grade B), 16.9 percent presented grade C liver involvement and 3.8 percent of the patients had progressed to acute hepatitis (grade D). The average values for the rise in aspartate aminotransferase and alanine aminotransferase were 93.3 U/L and 86.0 U/L. The greatest alterations were observed among females (p<0.001), cases of dengue hemorrhagic fever (p<0.001), and cases with sequential infections (p=0.001). CONCLUSIONS: Liver damage with elevation of aminotransferases and reactive hepatitis was a common complication of dengue virus infection in these patients.

Prevalência de dislipidemia e fatores de risco em Campos dos Goytacazes - RJ / Prevalence of dyslipidemia and risk factors in Campos dos Goytacazes, in the Brazilian State of Rio de Janeiro

OBJECTIVE: To determine the prevalence of dyslipidemias in adults in the city of Campos dos Goytacazes, in the Brazilian state of Rio de Janeiro, and to identify its relation to risk factors. METHODS: Cross-sectional, population-based, observational study with sampling through conglomerates and stratified according to socioeconomic levels, sex, and age, with 1,039 individuals. Risk factors, familial history, blood pressure, anthropometric measurements, glucose, triglycerides and cholesterol were determined. RESULTS: The following prevalences were observed: of dyslipidemias 24.2 percent; of hypercholesterolemia, 4.2 percent; of elevated LDL-C, 3.5 percent; of low HDL-C, 18.3 percent; and of hypertriglyceridemia, 17.1 percent. The following mean levels were observed: cholesterol, 187.6± 33.7 mg/dL; LDL-C, 108.7±26.8 mg/dL; HDL-C, 48.5±7.7 mg/dL; and triglycerides, 150.1±109.8 mg/dL. The following variables showed a positive correlation with dyslipidemia: increased age (P<0.001), male sex (P<0.001), low familial income (P<0.001), familial history (P<0.01), overweight/obesity (P<0.001), waist measure (P<0.001), high blood pressure (P<0.001), and diabetes mellitus (P<0.001). The following variables had no influence on dyslipidemias: ethnicity, educational level, smoking habits, and sedentary lifestyle. CONCLUSION: The frequency of lipid changes in the population studied was high, suggesting that measures for the early diagnosis should be taken, in association with implementation of programs for primary and secondary prevention of atherosclerosis

Hepatitis in Dengue Shock Syndrome

Dengue fever is the most frequent arbovirus disease in the world and the most important one in terms of morbidity and mortality. Atypical manifestations of dengue have become commonplace during the last few years, including hepatic damage, which manifests mainly by pain in the right hypochondrium and an increase in the levels of aminotransferases. We describe a case of acute hepatitis in a patient with Dengue Shock Syndrome Grade III. We analyzed the clinical and laboratory aspects of this atypical complication of dengue as well as the differential diagnoses.