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1.
Br J Haematol ; 193(4): 827-840, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33899219

RESUMO

A total of 244 patients with hereditary haemolytic anaemias (HHA) were screened for acute symptomatic human parvovirus B19 infection (HPV-B19) in a prospective study. To assess the risks associated with HPV-B19 infection, patients were classified into Group I and Group II according to presence or absence (symptoms, signs and specific serology) of acute HPV-B19 infection respectively. In all, 131 (53·7%) patients had ß-thalassaemia, 75 (30·7%) hereditary spherocytosis (HS), 27 (11·1%) sickle cell anaemia (SCA) and 11 (4·5%) glucose-6-phosphate dehydrogenase (G6PD) deficiency. Of 33 (13·5%) patients who presented with symptomatic HPV-B19 infection, 19 (57·5%) had HS, nine (27·3%) had ß-thalassaemia and five (15·2%) had SCA. In Group I, there were significant differences in the mean white blood cell, red blood cell and platelet counts, haemoglobin concentration, total bilirubin (TB), alanine aminotransferase, aspartate aminotransferase and serum creatinine (all P < 0·001) compared to Group II. In all, 27 (81·8%) patients had arthropathy and bone marrow failure (BMF); 13 (39·4%) had acute kidney injury (AKI), more in SCA (80%); and 12 (36·4%) patients had hepatitis, more in HS (66·8%). Five (15·2%) patients with HS had BMF, AKI, nervous system involvement and extreme hyperbilirubinaemia (TB range 26·3-84·7 mg/dl). Five (15·2%) patients had haemophagocytic syndrome. Two patients with HS combined with Type-I autoimmune hepatitis presented with transient BMF. Complete recovery or stabilisation was noted at 12 months in every patient except for one patient with SCA who died during the infection. HPV-B19 must be suspected and screened in patients with HHA with typical and atypical presentations with careful follow-up.


Assuntos
Anemia Hemolítica Congênita , Transtornos da Insuficiência da Medula Óssea , Eritema Infeccioso , Hepatite , Hiperbilirrubinemia , Parvovirus B19 Humano/metabolismo , Doença Aguda , Adolescente , Adulto , Anemia Hemolítica Congênita/sangue , Anemia Hemolítica Congênita/mortalidade , Anemia Hemolítica Congênita/virologia , Transtornos da Insuficiência da Medula Óssea/sangue , Transtornos da Insuficiência da Medula Óssea/mortalidade , Transtornos da Insuficiência da Medula Óssea/virologia , Criança , Eritema Infeccioso/sangue , Eritema Infeccioso/mortalidade , Feminino , Seguimentos , Hepatite/sangue , Hepatite/mortalidade , Hepatite/virologia , Humanos , Hiperbilirrubinemia/sangue , Hiperbilirrubinemia/mortalidade , Hiperbilirrubinemia/virologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos
2.
J Cardiothorac Surg ; 15(1): 195, 2020 Jul 28.
Artigo em Inglês | MEDLINE | ID: mdl-32723390

RESUMO

BACKGROUND: Hyperbilirubinemia is one of the common complications after cardiac surgery and is associated with increased mortality. However, to the best of our knowledge, the reports on clinical significance of postoperative severe hyperbilirubinemia in Stanford type A aortic dissection (AAD) patients were limited. METHODS: Patients who underwent surgical treatment for AAD in our center between January 2015 and December 2018 were retrospectively screened. In-hospital mortality, long-term mortality, acute kidney injury (AKI), and the requirement of continuous renal replacement therapy (CRRT) were assessed as endpoints. Univariate and multivariate regression models were employed to identify the risk factors of these endpoints. RESULTS: After screening, 271 patients were included in our present study. Of the included patients, 222 (81.9%) experienced postoperative AKI, and 50 (18.5%) received CRRT. The in-hospital mortality was 30.3%. The 1-year, 2-year, and 3-year cumulative mortality were 32.9, 33.9, and 35.3%, respectively. Multivariate Logistic regression analysis indicated that age (P < 0.033), AKI stage 3 (P < 0.001), the amount of blood transfusion after surgery (P = 0.019), mean arterial pressure (MAP) in the first postoperative day (P = 0.012), the use of extracorporeal membrane oxygenation (ECMO) (P = 0.02), and the peak total bilirubin (TB) concentration (P = 0.023) were independent risk factors of in-hospital mortality. The optimal cut-off value of peak TB on predicting in-hospital mortality was 121.2 µmol/L. Patients with post-operation TB ≥ 121 µmol/L was associated with worse long-term survival as well. CONCLUSIONS: Severe post-operation hyperbilirubinemia is a common clinical situation in patients had AAD repair. In AAD patients with severe post-operation hyperbilirubinemia, older age, lower MAP, increased blood transfusion, stage 3 AKI, the use of ECMO, and the increased peak TB lead to increase in-hospital mortality.


Assuntos
Aneurisma da Aorta Torácica/cirurgia , Dissecção Aórtica/cirurgia , Hiperbilirrubinemia/etiologia , Complicações Pós-Operatórias , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Aneurisma da Aorta Torácica/mortalidade , China/epidemiologia , Feminino , Mortalidade Hospitalar/tendências , Humanos , Hiperbilirrubinemia/mortalidade , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco
3.
Heart Vessels ; 35(8): 1102-1108, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32222801

RESUMO

Preoperative hyperbilirubinemia is associated with increased mortality and morbidity after cardiac surgery. However, this clinical significance is unclear with transcatheter aortic valve replacement (TAVR) procedures. The aims of this study were to determine the prevalence and prognostic implications of preoperative elevations of serum total bilirubin on TAVR outcomes. In 611 consecutive patients who underwent an elective TAVR procedure, 576 patients had recorded serum total bilirubin levels. Hyperbilirubinemia was defined as any value of serum total bilirubin ≥ 1.2 mg/dL obtained within 30-days prior to the TAVR procedure. The primary composite endpoint was post-TAVR all-cause in-hospital mortality or stroke. The overall prevalence of hyperbilirubinemia was 10% (n = 58). There were no patients with a prespecified diagnosis of liver cirrhosis. Pre-TAVR hyperbilirubinemia compared to normal bilirubin level was more common in younger (78 ± 10 vs. 82 ± 8 years old, p < 0.001) males (15 vs. 6%, p < 0.001), with history of pacemaker or ICD (33 vs. 18%, p = 0.005), congestive heart failure New York Heart Association class IV within 2 weeks from TAVR (35 vs. 14%, p < 0.001), severe tricuspid regurgitation (14 vs. 4%, p < 0.001), and atrial fibrillation or flutter (60 vs. 40%, p = 0.004, respectively). Pre-TAVR hyperbilirubinemia was independently associated with an increased post-TAVR in-hospital mortality (7 vs. 2% in normal bilirubin, p = 0.03), stroke (5 vs. 1%, p = 0.019, respectively), and a composite endpoint of death or stroke (12 vs. 3%, p < 0.001). Preoperative hyperbilirubinemia in patients undergoing TAVR is more prevalent than previously considered with multifactorial causes. Hyperbilirubinemia is independently associated with an increased post-TAVR in-hospital mortality and stroke.


Assuntos
Estenose da Valva Aórtica/cirurgia , Bilirrubina/sangue , Hiperbilirrubinemia/epidemiologia , Substituição da Valva Aórtica Transcateter , Idoso , Idoso de 80 Anos ou mais , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/mortalidade , Biomarcadores/sangue , Feminino , Mortalidade Hospitalar , Humanos , Hiperbilirrubinemia/sangue , Hiperbilirrubinemia/diagnóstico , Hiperbilirrubinemia/mortalidade , Masculino , Prevalência , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Acidente Vascular Cerebral/epidemiologia , Fatores de Tempo , Substituição da Valva Aórtica Transcateter/efeitos adversos , Substituição da Valva Aórtica Transcateter/mortalidade , Resultado do Tratamento
4.
Diabetes Res Clin Pract ; 159: 107971, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31805352

RESUMO

AIMS: To evaluate if neonatal complications or death were poorer for neonates born small for gestational age (SGA) than for those born with adequate weight or large for gestation age (LGA) to women with gestational diabetes mellitus (GDM). METHODS: Retrospective analysis of the clinical outcomes of neonates born to 3413 women with GDM. The prevalence of neonatal hypoglycaemia, hypocalcaemia, hyperbilirubinemia, polycythaemia, and death was compared among three birthweight groups: SGA, adequate, and LGA. A two-sided chi-squared or Fisher's exact test was used for between-group comparisons. A forward multiple logistic regression was performed to determine the odds ratio (OR) associated with SGA. RESULTS: Neonatal complications were more frequent in the SGA group (20.1%) than in the adequate (9.9%) or LGA (15.2%) groups. There were four deaths (1.6%) in the SGA group compared to one in the LGA (0.4%) and six in the adequate (0.2%) groups (P = 0.002). SGA was a risk factor for neonatal complications or death (OR. 2.122; 95% confidence interval, 1.552-2.899), independent of maternal age, weight gain, fasting glucose, glycaemic control, gestational hypertension, pre-eclampsia, smoking, or neonatal prematurity. CONCLUSION: SGA birthweight is an important risk factor for neonatal complications or death among neonates born to mothers with GDM.


Assuntos
Diabetes Gestacional/fisiopatologia , Doenças do Recém-Nascido/etiologia , Doenças do Recém-Nascido/mortalidade , Recém-Nascido Pequeno para a Idade Gestacional , Complicações na Gravidez/fisiopatologia , Adulto , Peso ao Nascer , Glicemia/análise , Feminino , Idade Gestacional , Humanos , Hiperbilirrubinemia/epidemiologia , Hiperbilirrubinemia/etiologia , Hiperbilirrubinemia/mortalidade , Hipocalcemia/epidemiologia , Hipocalcemia/etiologia , Hipocalcemia/mortalidade , Hipoglicemia/epidemiologia , Hipoglicemia/etiologia , Hipoglicemia/mortalidade , Incidência , Recém-Nascido , Doenças do Recém-Nascido/epidemiologia , Mães , Gravidez , Estudos Prospectivos , Estudos Retrospectivos , Fatores de Risco , Espanha/epidemiologia
5.
Eur J Cancer ; 100: 85-93, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-30014884

RESUMO

INTRODUCTION: Treatment of patients with advanced pancreatic carcinoma (APC) and hyperbilirubinaemia is problematic because these patients were regularly excluded from clinical studies. Nanoparticle albumin-bound paclitaxel and gemcitabine (nab-P/G) is an evidence-based treatment for patients with APC. This retrospective study investigated the safety and efficacy of nab-P/G in patients with APC and cholestatic hyperbilirubinaemia. METHODS: We screened our prospective database for patients with APC treated with nab-P/G at total bilirubin levels of ≥1.2 mg/dl. Patients were assigned into three groups according to their bilirubin level (A: 1.2-3 mg/dl, B: >3-5 mg/dl, C: >5 mg/dl). Analyses with regard to safety and survival were performed. RESULTS: Twenty-nine of 168 patients screened between Dec 2013 and Dec 2015 fulfilled the inclusion criteria. Most patients (83%) were male; median age was 63 [41-79] years. Nab-P/G administrations in patients with an elevated bilirubin level (median, range) did not result in unexpected toxicities assessed by predefined (non-)haematological parameters. Median overall survival (mOS) for the whole group was 11.7 (95% confidence interval [CI]: 6.8-14.0) months; for A: 11.8 (95% CI: 6.5-16.5), B: 9.2 (95% CI: 1.1 - NA) months and C 11.8 (95% CI: 5.9-20.0] months (p = 0.843). Again, mOS from the first application of nab-P/G did not differ between the groups (p = 0.13). CONCLUSION: Nab-P/G administrations in our pts with cholestatic hyperbilirubinaemia suffering from APC were feasible and safe with respect to individualised dose administrations. A multicenter phase 1 trial in pts with hyperbilirubinaemia is started (AIO-PAK-0117) to confirm these findings in a prospective setting.


Assuntos
Adenocarcinoma/tratamento farmacológico , Albuminas/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Colestase/etiologia , Desoxicitidina/análogos & derivados , Hiperbilirrubinemia/etiologia , Paclitaxel/administração & dosagem , Neoplasias Pancreáticas/tratamento farmacológico , Adenocarcinoma/complicações , Adenocarcinoma/mortalidade , Adenocarcinoma/secundário , Adulto , Idoso , Albuminas/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Colestase/diagnóstico , Colestase/mortalidade , Bases de Dados Factuais , Desoxicitidina/administração & dosagem , Desoxicitidina/efeitos adversos , Estudos de Viabilidade , Feminino , Humanos , Hiperbilirrubinemia/diagnóstico , Hiperbilirrubinemia/mortalidade , Masculino , Pessoa de Meia-Idade , Paclitaxel/efeitos adversos , Neoplasias Pancreáticas/complicações , Neoplasias Pancreáticas/mortalidade , Neoplasias Pancreáticas/patologia , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Gencitabina
6.
Am J Surg ; 214(1): 93-99, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28010881

RESUMO

BACKGROUND: Post-hepatectomy hyperbilirubinemia is associated with liver insufficiency and failure. The highest survivable peak total bilirubin (ptbili) is not defined. This study aimed to identify the postop ptbili beyond which survival is improbable or impossible. METHODS: An institutional database of major hepatectomies (≥3 segments, no biliary resections), 2000-2012 was reviewed. Data were analyzed to find ptbili in the first 45 postop days. Factors associated with 90-day mortality (90 DM) and those predictive of ptbili were determined. RESULTS: 603 pts were analyzed with 90DM of 4.5%. 90 DM for a ptbili ≥ 18 (n = 15) was 86.6%, but only 2.5% for a ptbili < 18. All 6 pts with a ptbili ≥ 30 died. On multivariate analysis, postop ptbili ≥ 18 (HR34.95, CI 3.8-324; p = 0.002) and cirrhosis (HR6.4, CI 1.2-33.2; p = 0.027) were associated with 90DM. Factors associated with a ptbili ≥ 18 were age >65 (HR14.24, CI 2.9-70.5; p = 0.001), preop chemotherapy (HR4.77, CI 1.3-18.2; p = 0.02) and postop FFP (HR12.5, CI 2.6-56.2; p = 0.001). CONCLUSION: Postop ptbili ≥ 18 after major hepatectomy has an 86.6% risk of 90DM; there are no survivors for tbili ≥ 30. These values may guide postop counseling for prognosis. Future studies may evaluate tbili ≥ 18 as an indication for hepatic replacement therapy.


Assuntos
Bilirrubina/sangue , Hepatectomia/efeitos adversos , Hiperbilirrubinemia/mortalidade , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Quimioterapia Adjuvante/efeitos adversos , Feminino , Georgia/epidemiologia , Humanos , Cirrose Hepática/mortalidade , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Terapia Neoadjuvante/efeitos adversos , Plasma , Complicações Pós-Operatórias , Estudos Retrospectivos , Adulto Jovem
7.
Asian Cardiovasc Thorac Ann ; 23(9): 1039-43, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26405017

RESUMO

BACKGROUND: We aimed to assess the incidence and perioperative risk factors for hyperbilirubinemia after cardiac surgery, and determine its influence on early operative outcome. METHODS: This prospective observational study was conducted on 476 patients who underwent cardiac surgical procedures from January 2014 to March 2014. Postoperative hyperbilirubinemia was defined as serum total bilirubin >2.0 mg dL(-1). RESULTS: The overall incidence of postoperative hyperbilirubinemia was 25% (119 patients). Patients undergoing valve repair or replacement had the highest incidence of hyperbilirubinemia (36.2%), followed by coronary artery bypass grafting with concomitant valve surgery (34.1%), congenital heart surgery (23.1%), and coronary artery bypass alone (12.7%). Postoperative hyperbilirubinemia was associated with increased duration of inotropic support (p = 0.0001), mechanical ventilation (p = 0.0001), intensive care unit stay (p = 0.001), hospital stay (p = 0.006), and mortality (p = 0.014). The perioperative factors associated with postoperative hyperbilirubinemia were increased preoperative bilirubin level (p < 0.0001), preoperative prothrombin time (p < 0.0001), cardiopulmonary bypass time (p = 0.028), aortic crossclamp time (p = 0.004), and blood transfusion units (p = 0.0001). CONCLUSIONS: Postoperative hyperbilirubinemia is common in patients undergoing cardiopulmonary bypass and is associated with high hospital mortality. The factors associated with its occurrence are increased preoperative bilirubin level, preoperative prothrombin time, cardiopulmonary bypass time, aortic crossclamp time, and blood transfusion units. Persistent hyperbilirubinemia is associated with a worse outcome than early transient hyperbilirubinemia.


Assuntos
Bilirrubina/sangue , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Ponte Cardiopulmonar/efeitos adversos , Hiperbilirrubinemia/etiologia , Adolescente , Adulto , Idoso , Biomarcadores/sangue , Procedimentos Cirúrgicos Cardíacos/mortalidade , Ponte Cardiopulmonar/mortalidade , Criança , Pré-Escolar , Feminino , Mortalidade Hospitalar , Humanos , Hiperbilirrubinemia/sangue , Hiperbilirrubinemia/diagnóstico , Hiperbilirrubinemia/mortalidade , Incidência , Índia/epidemiologia , Masculino , Pessoa de Meia-Idade , Duração da Cirurgia , Estudos Prospectivos , Tempo de Protrombina , Medição de Risco , Fatores de Risco , Fatores de Tempo , Reação Transfusional , Resultado do Tratamento , Adulto Jovem
8.
Pediatr Blood Cancer ; 61(7): 1257-62, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24535916

RESUMO

BACKGROUND: Despite several advances in the treatment of Epstein-Barr virus (EBV) in recent years, patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) do not always show satisfactory outcomes. We here conducted a nationwide survey in Japan to identify prognostic factors of EBV-HLH in children with this disease in an effort to improve the management and the outcomes of these patients. PROCEDURE: Between January 2003 and June 2008, we enrolled 98 children younger than 18 years of age who were diagnosed with EBV-HLH. We then studied the clinical characteristics and laboratory findings at the time of diagnosis with the aim to identify prognostic factors for EBV-HLH. RESULTS: The mean age of onset of EBV-HLH was 3.9 ± 2.8 years. Most of our patients presented with fever, hepatosplenomegaly, lymphadenopathy, and hemophagocytosis of bone marrow. Sixty-two percent of patients showed T cell clonality, and 97% had EBV infection in either T or natural killer cells. Most patients (60%) were treated with a multi-agent chemotherapeutic regimen, including corticosteroid, etoposide, and cyclosporine. After initial treatment, 90.3% of patients were in remission, and 7 patients (8.2%) experienced recurrence of EBV infection. Among several prognostic factors, patients with both hyperbilirubinemia (>1.8 mg/dl) and hyperferritinemia (>20,300 ng/ml) at the time of diagnosis had significantly poorer outcomes than those with low serum bilirubin and ferritin levels. CONCLUSIONS: These findings suggest that the therapeutic strategy for children with EBV-HLH could be tailored according to the laboratory findings at diagnosis.


Assuntos
Coleta de Dados , Infecções por Vírus Epstein-Barr , Herpesvirus Humano 4 , Linfo-Histiocitose Hemofagocítica , Corticosteroides/administração & dosagem , Idade de Início , Antineoplásicos Fitogênicos/administração & dosagem , Povo Asiático , Bilirrubina/sangue , Criança , Pré-Escolar , Ciclosporina/administração & dosagem , Intervalo Livre de Doença , Infecções por Vírus Epstein-Barr/sangue , Infecções por Vírus Epstein-Barr/diagnóstico , Infecções por Vírus Epstein-Barr/tratamento farmacológico , Infecções por Vírus Epstein-Barr/mortalidade , Etoposídeo/administração & dosagem , Humanos , Hiperbilirrubinemia/sangue , Hiperbilirrubinemia/diagnóstico , Hiperbilirrubinemia/tratamento farmacológico , Hiperbilirrubinemia/mortalidade , Imunossupressores/administração & dosagem , Lactente , Japão/epidemiologia , Linfo-Histiocitose Hemofagocítica/sangue , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Linfo-Histiocitose Hemofagocítica/mortalidade , Indução de Remissão , Estudos Retrospectivos , Taxa de Sobrevida
9.
Cardiology ; 122(1): 12-9, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22652820

RESUMO

OBJECTIVES: Despite recent advances in cardiovascular surgery, hyperbilirubinemia after heart valve surgery occurs often and the mechanism is not well clarified. We evaluated the incidence and nature of postoperative hyperbilirubinemia in heart valve surgery patients to analyze its risk factors and to identify its clinical significance for associated morbidity and mortality. METHODS: We investigated 334 consecutive patients who underwent heart valve surgery. Postoperative hyperbilirubinemia was defined as total serum bilirubin concentration greater than 3 mg/dl at any postoperative measurement point. RESULTS: Postoperative hyperbilirubinemia developed in 63 patients (19.0%) and was associated with greater in-hospital mortality (15.9 vs. 1.5%, p < 0.01). Univariate analysis revealed congestive heart failure history, cardiogenic shock, infective endocarditis, preoperative liver dysfunction, longer cardiopulmonary bypass time and transfusion as significantly related to postoperative hyperbilirubinemia. In multivariate analysis, preoperative liver dysfunction [odds ratio (OR) 6.5, p < 0.05] and longer cardiopulmonary bypass time (OR 1.007, p < 0.01) were independent predictors. In postoperative hyperbilirubinemia patients, preoperative New York Heart Association status and history of heart failure were associated with outcome. CONCLUSIONS: Postoperative hyperbilirubinemia remains common in patients undergoing heart valve surgery and is associated with a high rate of in-hospital mortality. For patients with heart failure or liver dysfunction, meticulous operative management is required to reduce cardiopulmonary bypass time and transfusion.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Doenças das Valvas Cardíacas/cirurgia , Hiperbilirrubinemia/etiologia , Complicações Pós-Operatórias/etiologia , Idoso , Procedimentos Cirúrgicos Cardíacos/mortalidade , Endocardite/complicações , Feminino , Insuficiência Cardíaca/complicações , Doenças das Valvas Cardíacas/mortalidade , Mortalidade Hospitalar , Humanos , Hiperbilirrubinemia/mortalidade , Tempo de Internação , Hepatopatias/complicações , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/mortalidade , Estudos Prospectivos , Fatores de Risco , Choque Cardiogênico/complicações
10.
Biol Blood Marrow Transplant ; 18(10): 1589-99, 2012 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22531490

RESUMO

Allogeneic hematopoietic cell transplantation (HCT) is commonly associated with hepatic complications. Patients with myelofibrosis (MF) often develop liver dysfunction in the early posttransplantation period; however, this has not yet been studied in a systematic fashion. We retrospectively evaluated 53 patients with MF who underwent HCT to assess the prevalence of acute liver toxicity and risk factors and the impact on survival. We compared the prevalence of acute hepatic complications in that group and a matched control group of 53 patients with myelodysplastic syndrome (MDS). In the MF group, during the first 6 weeks after HCT, the incidence of mild (34.2-102.6 µM), moderate (102.6-342 µM), and severe (>342 µM) hyperbilirubinemia was 34%, 40%, and 4%, respectively (normal, <22 µM). The incidence of mild/moderate transaminitis (2-10 times the upper limit of normal) was 23%, and that of severe transaminitis (>10 times the upper limit of normal) was 6%. Veno-occlusive disease as defined by the Baltimore criteria was observed in 19 patients (36%) in the MF group. Compared with MDS, MF was associated with a significantly higher incidence of moderate/severe hyperbilirubinemia (44% versus 21%; P = .02) and veno-occlusive disease (36% versus 19%; P = .05). A history of portal hypertension, biopsy-proven hepatic iron overload, or splanchnic vein thrombosis was a strong predictor of moderate/severe hyperbilirubinemia (P = .02). Acute hepatocellular injury with moderate/severe hyperbilirubinemia or transaminitis was associated with inferior survival at 12 months (P = .02) in the MF group. We conclude that patients with MF are at significant risk of early hepatotoxicity after HCT, which is associated with an adverse impact on survival.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Hiperbilirrubinemia/patologia , Fígado/patologia , Síndromes Mielodisplásicas/patologia , Mielofibrose Primária/patologia , Condicionamento Pré-Transplante , Adulto , Idoso , Canadá/epidemiologia , Feminino , Humanos , Hiperbilirrubinemia/epidemiologia , Hiperbilirrubinemia/mortalidade , Hiperbilirrubinemia/terapia , Incidência , Fígado/imunologia , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/epidemiologia , Síndromes Mielodisplásicas/mortalidade , Síndromes Mielodisplásicas/terapia , Mielofibrose Primária/epidemiologia , Mielofibrose Primária/mortalidade , Mielofibrose Primária/terapia , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Análise de Sobrevida , Fatores de Tempo , Transplante Homólogo
11.
Bone Marrow Transplant ; 47(10): 1343-9, 2012 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22388280

RESUMO

To analyze the incidence, characteristics and risk factors of hyperbilirubinemia after allogeneic hematopoietic cell transplantation with reduced-intensity conditioning (allo-RIC), we conducted a retrospective study in three Spanish centers. We analyzed 452 consecutive patients receiving allo-RIC. Of these, 92 patients (20%) developed marked hyperbilirubinemia (>4 mg/day or >68.4 µM) after allo-RIC. The main causes of marked hyperbilirubinemia after transplant were cholestasis due to GVHD or sepsis (n=57, 62%) and drug-induced cholestasis (n=13, 14%). A total of 22 patients with marked hyperbilirubinemia (24%) underwent liver biopsy. The most frequent histological finding was iron overload alone (n=6) or in combination with other features (n=6). In multivariate analysis, the risk factors for marked hyperbilirubinemia after allo-RIC were non-HLA-identical sibling donors (hazard ratio (HR) 2.2 (95% confidence interval (CI) 1.4-3.6) P=0.001), female donors to male recipients (HR 2.1 (95% CI 1.3-3.3) P=0.003) and high levels of bilirubin and γ-glutamyl transpeptidase before transplant (HR 4.5 (95% CI 2.5-8.4) P<0.001 and HR 4.6 (95% CI 2.6-8.1) P<0.001, respectively). Patients with marked hyperbilirubinemia showed higher 4-year nonrelapse mortality (HR 1.3 (95% CI 1-1.7), P=0.02) and lower 4-year OS (HR 1.4 (95%CI 1.3-1.7), P<0.001) than patients without. In conclusion, we confirm that marked hyperbilirubinemia is frequent and diverse after allo-RIC. Development of marked hyperbilirubinemia after allo-RIC is associated with worse outcome of the procedure.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Hiperbilirrubinemia/mortalidade , Irmãos , Doadores de Tecidos , Condicionamento Pré-Transplante , Adolescente , Adulto , Idoso , Biópsia , Intervalo Livre de Doença , Feminino , Humanos , Hiperbilirrubinemia/sangue , Hiperbilirrubinemia/etiologia , Hiperbilirrubinemia/patologia , Incidência , Fígado/metabolismo , Fígado/patologia , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Taxa de Sobrevida , Transplante Homólogo
12.
Transplant Proc ; 43(10): 3819-23, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22172853

RESUMO

INTRODUCTION: Liver transplant recipients are at high risk for Clostridium difficile infection. We have recently encountered multiple cases of CDI in our liver transplant recipients and for some of them it led to severe hyperbilirubinemia, liver failure, and even death. Our goals are to report our experience and analyze the factors that contributed to unfavorable outcomes. MATERIAL AND METHODS: All liver transplant recipients diagnosed with CDI between December 1, 2007, and January 30, 2009, were included and retrospectively reviewed. RESULTS: Twenty-four patients were identified, 14 men and 10 women. Fourteen patients experienced hyperbilirubinemia after the infection and 7 progressed to liver failure. Pre-CDI biopsy-proven liver abnormality, use of extended-criteria donors (ECDs) and a donor risk index (DRI) greater than 1.9 were associated with a higher risk of graft failure (P<.05). Hepatitis C, inpatient versus outpatient diagnosis, and a donor age greater than 50 years were not associated with a higher risk of graft failure. Use of ECDs and timing of the infection at more than 1 month but less than 1.5 years posttransplant were also associated with higher chances of sustained hyperbilirubinemia (P<.05). CONCLUSION: CDI in liver transplant patients can be very serious and may lead to sustained hyperbilirubinemia or graft failure. Marginal grafts are more susceptible to decompensate after such an infection than standard criteria grafts; moreover, already abnormal grafts do not tolerate this infection well and decompensate to complete failure in 85% of the cases.


Assuntos
Clostridioides difficile/patogenicidade , Enterocolite Pseudomembranosa/microbiologia , Hiperbilirrubinemia/microbiologia , Falência Hepática/microbiologia , Transplante de Fígado/efeitos adversos , Distribuição de Qui-Quadrado , Enterocolite Pseudomembranosa/complicações , Enterocolite Pseudomembranosa/mortalidade , Feminino , Humanos , Hiperbilirrubinemia/mortalidade , Estimativa de Kaplan-Meier , Falência Hepática/mortalidade , Transplante de Fígado/mortalidade , Masculino , New York , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento
13.
J Pediatr Surg ; 46(6): 1052-6, 2011 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-21683197

RESUMO

PURPOSE: Multidisciplinary treatment of pediatric intestinal failure has shown promising results. However, there are limited data as to the optimal time frame for referral of patients to intestinal failure programs. The aim of this study was to explore the relationship of hyperbilirubinemia at referral with patient outcomes in a multidisciplinary program. METHODS: A retrospective analysis was performed of a prospectively collected database from a multidisciplinary intestinal failure program. Multivariable logistic regression adjusted for age at referral was used to model the association between the conjugated bilirubin at referral and risk of mortality. Median values with range are reported. RESULTS: Sixty-two patients were referred from 2005 to 2009. Patients presented at age 6.4 months (0.4-261.4 months) and were followed up for 16.8 (0.3-53.0) months. Nine subjects (14.5%) died, and 12 subjects (19.4%) were listed for combined liver-intestine transplant. A 50% mortality was seen in patients referred with a conjugated bilirubin ≥ 7.2 mg/dL (n = 12), whereas mortality at referral bilirubin levels <7.2 mg/dL was 6%. After adjusting for age at referral, patients with a conjugated bilirubin ≥ 7.2 mg/dL at referral were 15.4 times more likely to die than patients who presented with lower bilirubin levels (P = .001; 95% confidence interval, 2.8-83.4). CONCLUSION: Within a pediatric intestinal failure program, mortality is associated with the degree of hyperbilirubinemia at time of referral. These data strongly suggest that these patients should be referred to a multidisciplinary program early in the evolution of their liver disease.


Assuntos
Hiperbilirrubinemia/diagnóstico , Hiperbilirrubinemia/mortalidade , Enteropatias/mortalidade , Enteropatias/cirurgia , Hepatopatias/diagnóstico , Encaminhamento e Consulta/organização & administração , Centros Médicos Acadêmicos , Pré-Escolar , Estudos de Coortes , Comorbidade , Bases de Dados Factuais , Progressão da Doença , Feminino , Hospitais Pediátricos , Humanos , Hiperbilirrubinemia/cirurgia , Lactente , Recém-Nascido , Enteropatias/diagnóstico , Estimativa de Kaplan-Meier , Hepatopatias/mortalidade , Hepatopatias/cirurgia , Modelos Logísticos , Masculino , Insuficiência de Múltiplos Órgãos/mortalidade , Insuficiência de Múltiplos Órgãos/fisiopatologia , Insuficiência de Múltiplos Órgãos/cirurgia , Análise Multivariada , Transplante de Órgãos/métodos , Transplante de Órgãos/mortalidade , Avaliação de Programas e Projetos de Saúde , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Análise de Sobrevida , Taxa de Sobrevida , Estados Unidos
14.
World J Surg ; 31(6): 1256-63, 2007 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-17453285

RESUMO

BACKGROUND: Surgery is the only potentially curative treatment for hilar bile duct cancer. This study sought to evaluate the efficacy and feasibility of surgical management of hilar bile duct carcinoma, including radical hepatectomy, at a single institution. METHODS: We performed a retrospective review of 49 consecutive patients who underwent surgery at our hospital between 1990 and 2003. RESULTS: Altogether, 44 of 49 patients underwent radical hepatectomy combined with caudate lobectomy and lymphadenectomy. One and four patients underwent partial hepatectomy or bile duct resection, respectively. No patients underwent preoperative portal vein embolization. The 5-year survival rate was 39.7%, with a median survival time of 3.75 years. The postoperative morbidity and mortality rates were 46.8% and 2.0%, respectively. Cox's proportional hazard model revealed that lymph node status and the residual tumor factor were independent prognostic factors. Multivariate analysis revealed that preoperative hyperbilirubinemia, postoperative complications, and extended surgical procedures were independently associated with postoperative hyperbilirubinemia. After potentially curative resection, 39.4% of patients suffered from disease recurrence. In 60% of the total cases, the sites of recurrence were distant metastases. CONCLUSION: Surgery, including radical hepatectomy combined with caudate lobectomy and lymph node dissection, is a feasible, effective treatment for hilar bile duct cancer.


Assuntos
Neoplasias dos Ductos Biliares/cirurgia , Ductos Biliares Intra-Hepáticos/cirurgia , Colangiocarcinoma/cirurgia , Hepatectomia , Complicações Pós-Operatórias/etiologia , Idoso , Idoso de 80 Anos ou mais , Neoplasias dos Ductos Biliares/mortalidade , Neoplasias dos Ductos Biliares/patologia , Ductos Biliares Intra-Hepáticos/patologia , Colangiocarcinoma/mortalidade , Colangiocarcinoma/patologia , Estudos de Viabilidade , Feminino , Seguimentos , Humanos , Hiperbilirrubinemia/etiologia , Hiperbilirrubinemia/mortalidade , Hiperbilirrubinemia/patologia , Fígado/patologia , Excisão de Linfonodo , Metástase Linfática/patologia , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica , Recidiva Local de Neoplasia/etiologia , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/patologia , Neoplasia Residual/etiologia , Neoplasia Residual/mortalidade , Neoplasia Residual/patologia , Complicações Pós-Operatórias/mortalidade , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Análise de Sobrevida
15.
World J Gastroenterol ; 12(41): 6722-6, 2006 Nov 07.
Artigo em Inglês | MEDLINE | ID: mdl-17075992

RESUMO

AIM: To clarify the incidence and nature of postoperative hyperbilirubinemia in patients after modern extracorporeal circulation, to analyze possible perioperative risk factors, and to elucidate the clinical significance of postoperative hyperbilirubinemia associated mortality and morbidity. METHODS: Between March 2005 and May 2006, three hundred and eighty six consecutive patients undergoing extracorporeal circulation surgery due to a variety of cardiac lesions were investigated prospectively. The incidence of postoperative hyperbilirubinemia was defined as a serum total bilirubin concentration of more than 51 micromol/L. Several perioperative parameters were compared by logistic regression between hyperbilirubinemia and non-hyperbilirubinemia patients to determine possible risk factors contributing to postoperative hyperbilirubinemia and mortality. RESULTS: Overall incidence of postoperative hyperbilirubinemia was 25.3% (98/386). In patients with postoperative hyperbilirubinemia, 56.2% reached peak total bilirubin concentration on the first postoperative day, 33.5% on the second day, and 10.3% on the seventh day. Eighty percent of the increase of total bilirubin resulted from an increase of both conjugated and unconjugated bilirubin. Development of postoperative hyperbilirubinemia was associated with a higher mortality (P<0.01), longer duration of mechanical ventilation (P<0.05) and longer ICU stay time (P<0.05). Preoperative total bilirubin concentration, preoperative right atrium pressure, numbers of valves replaced and of blood transfusion requirement were identified as important predictors for postoperative hyperbilirubinemia. CONCLUSION: Early postoperative hyperbilirubinemia after modern extracorporeal circulation is mainly caused by an increase in both conjugated and unconjugated bilirubin, and is associated with a high mortality. Important contributing factors are the preoperative total bilirubin concentration, preoperative severity of right atrial pressure, numbers of valve replacement procedures, and the amount of blood transfusion requirement during and shortly after surgery. We suggest that postoperative hyperbilirubinemia is a multifactorial process, which is caused by both the impaired liver function of bilirubin transport and the increased production of bilirubin from haemolysis.


Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Circulação Extracorpórea/efeitos adversos , Hiperbilirrubinemia/etiologia , Fígado/metabolismo , Adolescente , Adulto , Idoso , Pressão Sanguínea/fisiologia , Procedimentos Cirúrgicos Cardíacos/métodos , Circulação Extracorpórea/métodos , Feminino , Implante de Prótese de Valva Cardíaca/efeitos adversos , Humanos , Hiperbilirrubinemia/metabolismo , Hiperbilirrubinemia/mortalidade , Incidência , Fígado/fisiopatologia , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/metabolismo , Estudos Prospectivos , Fatores de Risco , Reação Transfusional
17.
Hepatogastroenterology ; 51(59): 1434-8, 2004.
Artigo em Inglês | MEDLINE | ID: mdl-15362770

RESUMO

BACKGROUND/AIMS: To predict the occurrence of postoperative liver failure after hepatectomy, the clinical significance of monitoring alkaline phosphatase (ALP) has been studied, and the relationship between the change of total bilirubin and the level of ALP or gamma-glutamyl transpeptidase (gamma-GTP) after hepatectomy was evaluated. METHODOLOGY: The 163 patients, who underwent hepatectomy at our institute for the past 12 years, were divided into three groups according to the postoperative events. HF consisted of 5 patients with liver failure, HB showed the postoperative high bilirubinemia (over 5mg/dL) in 13 cases and GP were 145 cases without any postoperative problems. RESULTS: 1. The postoperative highest level of total bilirubin (T-Bil) correlated with the decreasing rate of ALP, prothrombin time (PT), total cholesterol (T-CHO) or gamma-GTP and total blood loss (p<0.01). 2. The level of ALP decreased after hepatectomy significantly and the decreasing rate was serious in HB and HF (p<0.05). 3. The recovering time to preoperative level of ALP was clearly shorter in GP than in HB and HF. 4. The level of ALP and gamma-GTP at the point where the level of T-Bil increased over 5mg/dL, was useful to distinguish between HF and HB. 5. The good correlation between postoperative level of ALP and gamma-GTP was noted. The decreasing levels of ALP and gamma-GTP were found to be critical below 80% and 55% after bisegmentectomy. Furthermore, the recovered levels of ALP and gamma-GTP were important to distinguish between HF and HB. CONCLUSIONS: Monitoring the ALP level was indicated to be useful to estimate the postoperative course of bilirubin.


Assuntos
Fosfatase Alcalina/sangue , Hepatectomia , Falência Hepática/diagnóstico , Testes de Função Hepática , Neoplasias Hepáticas/cirurgia , Complicações Pós-Operatórias/diagnóstico , Alanina Transaminase/sangue , Aspartato Aminotransferases/sangue , Bilirrubina/sangue , Causas de Morte , Colesterol/sangue , Humanos , Hiperbilirrubinemia/diagnóstico , Hiperbilirrubinemia/enzimologia , Hiperbilirrubinemia/mortalidade , L-Lactato Desidrogenase/sangue , Falência Hepática/enzimologia , Falência Hepática/mortalidade , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/secundário , Complicações Pós-Operatórias/enzimologia , Complicações Pós-Operatórias/mortalidade , Valor Preditivo dos Testes , Tempo de Protrombina , Valores de Referência , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Taxa de Sobrevida
18.
Ren Fail ; 24(3): 297-305, 2002 May.
Artigo em Inglês | MEDLINE | ID: mdl-12166696

RESUMO

During a recent outbreak of leptospirosis in northeastern Thailand, 148 patients with serologically diagnosed leptospirosis were seen in Loei hospital. The common serotypes were L. pyrogenes, and L. sejroe. Hypotension with a mean arterial pressure less than 70 mmHg upon admission or within 24 h after admission was observed in 94 patients or 64%. 30 patients had normal renal function; 30 patients had prerenal azotemia with mild pulmonary complication in 2; and 34 patients had acute renal failure. 29 patients with acute renal failure had pulmonary complications including pulmonary hemorrhage in 8, pulmonary edema in 3, acute respiratory distress syndrome (ARDS) in 14 and interstitial pneumonitis in 4. 54 patients had normal blood pressure. In this group 5 patients had acute renal failure; 16 had prerenal azotemia and 33 had normal renal function. Interstitial pneumonitis was noted in one patient with prerenal azotemia. Less renal complication and minimal pulmonary complication were seen in leptospirosis patients with normal blood pressure. The patients with normal renal function had no pulmonary complication. Good association existed between hypotension, renal failure and pulmonary complications.


Assuntos
Injúria Renal Aguda/etiologia , Hipotensão/etiologia , Leptospirose/complicações , Pneumopatias/etiologia , Injúria Renal Aguda/mortalidade , Injúria Renal Aguda/fisiopatologia , Adolescente , Adulto , Idoso , Biomarcadores/sangue , Pressão Sanguínea/fisiologia , Nitrogênio da Ureia Sanguínea , Criança , Creatinina/sangue , Feminino , Humanos , Hiperbilirrubinemia/etiologia , Hiperbilirrubinemia/mortalidade , Hiperbilirrubinemia/fisiopatologia , Hipotensão/mortalidade , Hipotensão/fisiopatologia , Rim/irrigação sanguínea , Rim/metabolismo , Rim/fisiopatologia , Leptospirose/mortalidade , Leptospirose/fisiopatologia , Pneumopatias/mortalidade , Pneumopatias/fisiopatologia , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Análise de Sobrevida , Tailândia/epidemiologia , Resultado do Tratamento , Fator de Necrose Tumoral alfa/metabolismo , Uremia/etiologia , Uremia/mortalidade , Uremia/fisiopatologia
19.
Surg Today ; 32(8): 679-84, 2002.
Artigo em Inglês | MEDLINE | ID: mdl-12181716

RESUMO

PURPOSE: The aim of this study was to evaluate the risk factors and outcome of patients with hyperbilirubinemia after surgery for generalized peritonitis. METHODS: We retrospectively analyzed data from 229 patients with generalized peritonitis caused by gastrointestinal (GI) perforations. RESULTS: Postoperative hyperbilirubinemia defined as a value of > or =5 mg/dl, within 1 month, developed in 39 patients. Postoperative hyperbilirubinemia was related to age ( P = 0.0102), poor nutritional status ( P = 0.0388), decreased base excess ( P = 0.0037), delay until surgery ( P = 0.0276), preoperative serum bilirubin ( P = 0.0321) and postoperative persistent infection ( P < 0.0001). Higher mortality was seen in patients with hyperbilirubinemia (59%) than in those without hyperbilirubinemia (4%). The patients with hyperbilirubinemia who survived had decreased serum bilirubin levels after 3-5 postoperative days whereas a continuous increase was seen in the patients who ultimately died. Preoperative shock ( P = 0.0003), a decreased preoperative platelet count ( P = 0.0152), postoperative infection ( P = 0.0050), and postoperative hyperbilirubinemia ( P < 0.0001) were risk factors for overall mortality. CONCLUSION: These results indicate that postoperative hyperbilirubinemia in patients with GI perforation is related to persistent postoperative infection and associated with poor prognosis.


Assuntos
Hiperbilirrubinemia/etiologia , Perfuração Intestinal/cirurgia , Peritonite/cirurgia , Complicações Pós-Operatórias , Gastropatias/cirurgia , Adulto , Feminino , Humanos , Hiperbilirrubinemia/mortalidade , Perfuração Intestinal/etiologia , Masculino , Pessoa de Meia-Idade , Estado Nutricional , Complicações Pós-Operatórias/mortalidade , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Gastropatias/etiologia
20.
Haematologica ; 76 Suppl 1: 60-2, 1991 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-1677914

RESUMO

Six patients suffering from refractory malignancies (3 NHL, 1 MM, 1 AML, 1 neuroblastoma) received high dose of chemotherapy and autologous peripheral blood stem cell transplantation (APBSCT). The recruitment of PBSC was performed using conventional salvatage schedules of therapy. The patients received a median of 8.69 MNC/kg bw and 20.87 CFU-GM x 10(4)/kg bw. Prompt engraftment occurred in all patients and the median number of days to achieve WBC greater than 1 x 10(9)/l was 16.5 (range 7-26), PMN greater than 0.5 x 10(9)/l was 21.5 (range 6-37) and PLTs greater than 50 x 10(9)/l was 17.5 (range 4-31). Four patients achieved a complete remission. One patient (neuroblastoma) died of progressive disease after a partial response. One patient died in relapse because of drug related toxicity.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Neoplasias/terapia , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Medula Óssea/patologia , Terapia Combinada , Estudos de Avaliação como Assunto , Feminino , Hematopoese , Humanos , Hiperbilirrubinemia/induzido quimicamente , Hiperbilirrubinemia/mortalidade , Itália/epidemiologia , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/mortalidade , Neoplasias/sangue , Neoplasias/tratamento farmacológico , Pancitopenia/induzido quimicamente , Indução de Remissão , Transplante Autólogo
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