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1.
J Med Case Rep ; 16(1): 159, 2022 Apr 21.
Artículo en Inglés | MEDLINE | ID: mdl-35443720

RESUMEN

BACKGROUND: Extragonadal choriocarcinoma is rare and can be associated with hyperthyroidism when producing very high levels of human chorionic gonadotropin. CASE PRESENTATION: A 62-year-old Hispanic female presented with a 3-week history of shortness of breath, palpitations, extreme weakness, new-onset hot flashes, and right flank pain. Her physical examination was remarkable for tachycardia, hepatomegaly, hyperreflexia, and tremor; goiter was absent. Laboratory studies revealed increased lactate dehydrogenase, alkaline phosphatase, suppressed thyroid stimulating hormone, very elevated T4, and absent thyroid stimulating immunoglobulin. 18F-fluorodeoxyglucose positron emission tomography-computed tomography exhibited hepatomegaly with multiple large fluorodeoxyglucose-avid liver masses and a focus of fluorodeoxyglucose avidity in the stomach with no structural correlate. A thyroid scan (99mTcO 4 - ) showed diffusely increased tracer uptake. She was started on propranolol and methimazole. Upon stabilization of severe thyrotoxicosis, upper endoscopy was performed, showing a ~ 5 cm bleeding lesion in the greater stomach curvature body; biopsy was consistent with choriocarcinoma; beta-human chorionic gonadotropin hormone was 2,408,171 mIU/mL. The patient received methotrexate followed by etoposide and cisplatin. Methimazole was titrated down, and upon liver failure the medication was stopped. The thyrotoxicosis was effectively controlled with antithyroid drug and concurrent chemotherapy. At ~ 1.5 months after initial diagnosis, the patient died due to bleeding/acute liver failure with coagulation defects followed by multiple organ failure. CONCLUSIONS: Severe thyrotoxicosis can represent an unusual initial presentation of metastatic choriocarcinoma in the setting of extreme elevation of beta-human chorionic gonadotropin. Primary gastric choriocarcinoma is an aggressive malignancy with very poor outcomes. The co-occurrence of severe thyrotoxicosis with advanced primary gastric choriocarcinoma and imminent liver failure complicates management options.


Asunto(s)
Coriocarcinoma , Hipertiroidismo , Fallo Hepático , Neoplasias Gástricas/patología , Tirotoxicosis , Coriocarcinoma/complicaciones , Coriocarcinoma/diagnóstico , Coriocarcinoma/tratamiento farmacológico , Gonadotropina Coriónica/uso terapéutico , Gonadotropina Coriónica Humana de Subunidad beta , Femenino , Hepatomegalia , Humanos , Hipertiroidismo/diagnóstico , Hipertiroidismo/tratamiento farmacológico , Hipertiroidismo/etiología , Fallo Hepático/complicaciones , Masculino , Metimazol/uso terapéutico , Persona de Mediana Edad , Neoplasias de Células Germinales y Embrionarias , Embarazo , Neoplasias Testiculares , Tirotoxicosis/complicaciones , Tirotoxicosis/diagnóstico , Tirotoxicosis/tratamiento farmacológico , Tomografía Computarizada por Rayos X
2.
World J Gastroenterol ; 28(8): 775-793, 2022 Feb 28.
Artículo en Inglés | MEDLINE | ID: mdl-35317103

RESUMEN

Diabetes mellitus (DM) is common in liver cirrhosis (LC). The pathophysiological association is bidirectional. DM is a risk factor of LC and LC is a diabetogenic condition. In the recent years, research on different aspects of the association DM and LC has been intensified. Nevertheless, it has been insufficient and still exist many gaps. The aims of this review are: (1) To discuss the latest understandings of the association of DM and LC in order to identify the strategies of early diagnosis; (2) To evaluate the impact of DM on outcomes of LC patients; and (3) To select the most adequate management benefiting the two conditions. Literature searches were conducted using PubMed, Ovid and Scopus engines for DM and LC, diagnosis, outcomes and management. The authors also provided insight from their own published experience. Based on the published studies, two types of DM associated with LC have emerged: Type 2 DM (T2DM) and hepatogenous diabetes (HD). High-quality evidences have determined that T2DM or HD significantly increase complications and death pre and post-liver transplantation. HD has been poorly studied and has not been recognized as a complication of LC. The management of DM in LC patients continues to be difficult and should be based on drug pharmacokinetics and the degree of liver failure. In conclusion, the clinical impact of DM in outcomes of LC patients has been the most studied item recently. Nevertheless many gaps still exist particularly in the management. These most important gaps were highlighted in order to propose future lines for research.


Asunto(s)
Diabetes Mellitus Tipo 2 , Hepatopatías , Fallo Hepático , Diabetes Mellitus Tipo 2/complicaciones , Diabetes Mellitus Tipo 2/diagnóstico , Diabetes Mellitus Tipo 2/epidemiología , Humanos , Cirrosis Hepática/complicaciones , Cirrosis Hepática/diagnóstico , Cirrosis Hepática/terapia , Hepatopatías/complicaciones , Hepatopatías/diagnóstico , Hepatopatías/terapia , Fallo Hepático/complicaciones , Factores de Riesgo
3.
Rev Bras Ter Intensiva ; 32(3): 474-478, 2020.
Artículo en Portugués, Inglés | MEDLINE | ID: mdl-33053039

RESUMEN

This case report a severe case of yellow fever complicated by liver failure and disseminated intravascular coagulation. Thromboelastometry was capable of identifying clotting disorders and guiding hemostatic therapy. We report the case of a 23-year-old male admitted to the Intensive Care Unit with sudden onset of fever, generalized muscle pain associated with liver failure, and disseminated intravascular coagulation. The results of conventional laboratory tests showed thrombocytopenia, whereas thromboelastometry suggested coagulopathy with slight hypofibrinogenemia, clotting factor consumption, and, consequently, an increased risk of bleeding. Unlike conventional laboratory tests, thromboelastometry identified the specific coagulation disorder and thereby guided hemostatic therapy. Both fibrinogen concentrates and vitamin K were administered, and no blood component transfusion was required, even in the presence of thrombocytopenia. Administration of hemostatic drugs, including fibrinogen concentrate and vitamin K, improved thromboelastometric parameters, correcting the complex coagulation disorder. Blood component transfusion was not performed, and there was no bleeding.


Este relato de caso detalha um caso grave de febre amarela complicada por insuficiência hepática e coagulação intravascular disseminada. A tromboelastometria foi capaz de identificar os distúrbios da coagulação e orientar o tratamento hemostático. Relatamos o caso de um homem com 23 anos de idade admitido na unidade de terapia intensiva com quadro com início abrupto de febre e dor muscular generalizada associados a insuficiência hepática e coagulação intravascular disseminada. Os resultados dos exames laboratoriais convencionais revelaram trombocitopenia, enquanto a tromboelastometria sugeriu coagulopatia com discreta hipofibrinogenemia, consumo de fatores de coagulação e, consequentemente, aumento do risco de sangramento. Diferentemente dos exames laboratoriais convencionais, a tromboelastometria identificou o distúrbio de coagulação específico e, assim, orientou o tratamento hemostático. Administraram-se concentrados de fibrinogênio e vitamina K, não sendo necessária a transfusão de qualquer componente do sangue, mesmo na presença de trombocitopenia. A tromboelastometria permitiu a identificação precoce da coagulopatia e ajudou a orientar a terapêutica hemostática. A administração de fármacos hemostáticos, incluindo concentrados de fibrinogênio e vitamina K, melhorou os parâmetros tromboelastométricos, com correção do transtorno da coagulação. Não se realizou transfusão de hemocomponentes, e não ocorreu qualquer sangramento.


Asunto(s)
Trastornos de la Coagulación Sanguínea/diagnóstico , Coagulación Intravascular Diseminada/complicaciones , Fallo Hepático/complicaciones , Fiebre Amarilla/complicaciones , Trastornos de la Coagulación Sanguínea/etiología , Trastornos de la Coagulación Sanguínea/terapia , Coagulación Intravascular Diseminada/diagnóstico , Coagulación Intravascular Diseminada/virología , Hemostáticos/administración & dosificación , Humanos , Fallo Hepático/diagnóstico , Fallo Hepático/virología , Masculino , Tromboelastografía/métodos , Adulto Joven
4.
Rev. bras. ter. intensiva ; 32(3): 474-478, jul.-set. 2020. graf
Artículo en Inglés, Portugués | LILACS | ID: biblio-1138507

RESUMEN

RESUMO Este relato de caso detalha um caso grave de febre amarela complicada por insuficiência hepática e coagulação intravascular disseminada. A tromboelastometria foi capaz de identificar os distúrbios da coagulação e orientar o tratamento hemostático. Relatamos o caso de um homem com 23 anos de idade admitido na unidade de terapia intensiva com quadro com início abrupto de febre e dor muscular generalizada associados a insuficiência hepática e coagulação intravascular disseminada. Os resultados dos exames laboratoriais convencionais revelaram trombocitopenia, enquanto a tromboelastometria sugeriu coagulopatia com discreta hipofibrinogenemia, consumo de fatores de coagulação e, consequentemente, aumento do risco de sangramento. Diferentemente dos exames laboratoriais convencionais, a tromboelastometria identificou o distúrbio de coagulação específico e, assim, orientou o tratamento hemostático. Administraram-se concentrados de fibrinogênio e vitamina K, não sendo necessária a transfusão de qualquer componente do sangue, mesmo na presença de trombocitopenia. A tromboelastometria permitiu a identificação precoce da coagulopatia e ajudou a orientar a terapêutica hemostática. A administração de fármacos hemostáticos, incluindo concentrados de fibrinogênio e vitamina K, melhorou os parâmetros tromboelastométricos, com correção do transtorno da coagulação. Não se realizou transfusão de hemocomponentes, e não ocorreu qualquer sangramento.


Abstract This case report a severe case of yellow fever complicated by liver failure and disseminated intravascular coagulation. Thromboelastometry was capable of identifying clotting disorders and guiding hemostatic therapy. We report the case of a 23-year-old male admitted to the Intensive Care Unit with sudden onset of fever, generalized muscle pain associated with liver failure, and disseminated intravascular coagulation. The results of conventional laboratory tests showed thrombocytopenia, whereas thromboelastometry suggested coagulopathy with slight hypofibrinogenemia, clotting factor consumption, and, consequently, an increased risk of bleeding. Unlike conventional laboratory tests, thromboelastometry identified the specific coagulation disorder and thereby guided hemostatic therapy. Both fibrinogen concentrates and vitamin K were administered, and no blood component transfusion was required, even in the presence of thrombocytopenia. Administration of hemostatic drugs, including fibrinogen concentrate and vitamin K, improved thromboelastometric parameters, correcting the complex coagulation disorder. Blood component transfusion was not performed, and there was no bleeding.


Asunto(s)
Humanos , Masculino , Adulto Joven , Fiebre Amarilla/complicaciones , Trastornos de la Coagulación Sanguínea/diagnóstico , Fallo Hepático/complicaciones , Coagulación Intravascular Diseminada/complicaciones , Tromboelastografía/métodos , Trastornos de la Coagulación Sanguínea/etiología , Trastornos de la Coagulación Sanguínea/terapia , Hemostáticos/administración & dosificación , Fallo Hepático/virología
5.
Nutrition ; 60: 235-240, 2019 04.
Artículo en Inglés | MEDLINE | ID: mdl-30682545

RESUMEN

OBJECTIVES: Vitamin D deficiency may be associated with comorbidities and poor prognosis. However, this association in patients in the intensive care unit (ICU) has not been fully elucidated. The aim of this study was to investigate whether the serum concentrations of 25-hydroxyvitamin D (25[OH]D) within the first 48 h after ICU admission are associated with prognostic indicators (Acute Physiology and Chronic Health Evaluation [APACHE] II, Sequential Organ Failure Assessment [SOFA] score, Charlson comorbidity index [CCI]), clinical complications, serum C-reactive protein (CRP) concentrations, mechanical ventilation duration, and mortality. METHODS: Seventy-one patients were admitted to the ICU, and their concentrations of 25(OH)D in the first 48 h were analyzed. To evaluate the prognostic factors in the ICU, APACHE II scores, SOFA scores, CCI questionnaires, mechanical ventilation time, CRP, and mortality were used. RESULTS: The mean concentration of 25(OH)D was 17.7 ± 8.27 ng/mL (range 3.5-37.5 ng/mL), with 91.6% presenting with deficiency at admission. Although no associations were found between serum 25(OH)D concentrations with mechanical ventilation time, CRP, mortality, and APACHE II and SOFA severity scores, we found associations with the CCI when adjusted by age (model 1: odds ratio [OR], 1.64; 95% confidence interval [CI], 1.14-2.34) and by age, sex and body mass index (model 2: OR, 1.59; 95% CI, 1.10-2.34). In addition, among the comorbidities present, 25(OH)D concentrations were inversely associated with cancer (crude model OR, 3.42; 95% CI, 1.21-9.64) and liver disease (crude model OR, 9.64; 95% CI, 2.28-40.60). CONCLUSION: We found a strong association between 25(OH)D concentrations and the prognostic indicator CCI and clinical complications (acute respiratory insufficiency, acute liver failure, and infections), but no associations with the prognostic indicators APACHE II and SOFA score, CRP, mechanical ventilation duration, or mortality. The main comorbidities associated with low 25(OH)D were cancer and liver disease, suggesting that the determination of 25(OH)vitamin D is relevant during the ICU stay.


Asunto(s)
Enfermedad Crítica/mortalidad , Admisión del Paciente/estadística & datos numéricos , Índice de Severidad de la Enfermedad , Deficiencia de Vitamina D/mortalidad , Vitamina D/análogos & derivados , APACHE , Enfermedad Aguda , Adulto , Anciano , Proteína C-Reactiva/análisis , Comorbilidad , Estudios Transversales , Femenino , Mortalidad Hospitalaria , Humanos , Infecciones/sangre , Infecciones/complicaciones , Infecciones/mortalidad , Unidades de Cuidados Intensivos , Fallo Hepático/sangre , Fallo Hepático/complicaciones , Fallo Hepático/mortalidad , Masculino , Persona de Mediana Edad , Neoplasias/sangre , Neoplasias/complicaciones , Neoplasias/mortalidad , Puntuaciones en la Disfunción de Órganos , Pronóstico , Respiración Artificial , Insuficiencia Respiratoria/sangre , Insuficiencia Respiratoria/complicaciones , Insuficiencia Respiratoria/mortalidad , Factores de Tiempo , Vitamina D/sangre , Deficiencia de Vitamina D/sangre , Deficiencia de Vitamina D/complicaciones
6.
Rev. Hosp. Clin. Univ. Chile ; 29(3): 198-205, 2018. Tab.
Artículo en Español | LILACS | ID: biblio-999257

RESUMEN

The gastrointestinal system is closely related to the skin. Multiple disorders of the gastrointestinal tract that manifest clinically on the skin surface are recognized, which can even occur before establishing the definitive diagnosis of the disease. For this reason, it is necessary that the dermatologist is internalized regarding the wide variety of clinical signs that can guide the etiological study, which requires in most cases, a multidisciplinary management. (AU)


Asunto(s)
Humanos , Masculino , Femenino , Manifestaciones Cutáneas , Enfermedades Gastrointestinales/complicaciones , Enfermedades Inflamatorias del Intestino/complicaciones , Fallo Hepático/complicaciones , Hemorragia Gastrointestinal/complicaciones
7.
World J Gastroenterol ; 23(5): 776-791, 2017 Feb 07.
Artículo en Inglés | MEDLINE | ID: mdl-28223722

RESUMEN

Celiac disease (CD) is a systemic immune-mediated disorder triggered by dietary gluten in genetically predisposed individuals. The typical symptoms are anemia, diarrhea, fatigue, weight loss, and abdominal pain. CD has been reported in patients with primary sclerosing cholangitis, primary biliary cholangitis, autoimmune hepatitis, aminotransferase elevations, nonalcoholic fatty liver disease, hepatitis B, hepatitis C, portal hypertension and liver cirrhosis. We evaluate recommendations for active screening for CD in patients with liver diseases, and the effect of a gluten-free diet in these different settings. Active screening for CD is recommended in patients with liver diseases, particularly in those with autoimmune disorders, steatosis in the absence of metabolic syndrome, noncirrhotic intrahepatic portal hypertension, cryptogenic cirrhosis, and in the context of liver transplantation. In hepatitis C, diagnosis of CD can be important as a relative contraindication to interferon use. Gluten-free diet ameliorates the symptoms associated with CD; however, the associated liver disease may improve, remain the same, or progress.


Asunto(s)
Anticuerpos/sangre , Enfermedad Celíaca/complicaciones , Enfermedad Celíaca/diagnóstico , Hepatopatías/complicaciones , Hepatopatías/inmunología , Enfermedad Celíaca/inmunología , Colangitis Esclerosante/complicaciones , Colangitis Esclerosante/inmunología , Dieta Sin Gluten , Glútenes/efectos adversos , Glútenes/inmunología , Hepatitis B/complicaciones , Hepatitis B/inmunología , Hepatitis C/complicaciones , Hepatitis C/inmunología , Hepatitis Autoinmune/complicaciones , Hepatitis Autoinmune/inmunología , Humanos , Hipertensión Portal/complicaciones , Hipertensión Portal/inmunología , Cirrosis Hepática/complicaciones , Cirrosis Hepática/inmunología , Cirrosis Hepática Biliar/complicaciones , Cirrosis Hepática Biliar/inmunología , Fallo Hepático/complicaciones , Fallo Hepático/inmunología , Trasplante de Hígado , Enfermedad del Hígado Graso no Alcohólico/complicaciones , Enfermedad del Hígado Graso no Alcohólico/inmunología
8.
Liver Transpl ; 23(6): 762-768, 2017 06.
Artículo en Inglés | MEDLINE | ID: mdl-27935212

RESUMEN

Hepatitis C virus (HCV) infection is the leading cause of liver disease in hemophilia patients. In those with human immunodeficiency virus (HIV)/HCV coinfection, the rate of liver disease progression is greater than in HCV monoinfected individuals. Despite antiretroviral therapy, which slows HCV liver disease progression, some require transplantation. Whether transplant outcomes are worse in hemophilic (H) rather than nonhemophilic (NH) candidates is unknown. In order to determine rates and predictors of pretransplant and posttransplant survival, we conducted a retrospective observational study using United Network for Organ Sharing national transplant registry data, comparing HCV+ H and NH candidates. We identified 2502 HCV+ liver transplant candidates from 8 US university-based transplant centers, between January 1, 2004 to December 31, 2010, including 144 HIV+ (6%) and 2358 HIV-; 36 H (1%) and 2466 NH; 1213 (48%) transplanted and 1289 not transplanted. Other than male predominance and younger age, each were P < 0.001. Baseline data were comparable between H and NH. In univariate analysis, 90-day pretransplant mortality was associated with higher baseline Model for End-Stage Liver Disease (MELD; hazard ratio [HR] = 1.15; P < 0.001), lower baseline platelet count (HR = 0.9 per 25,000/µL; P = 0.04), and having HIV/HCV+ hemophilia (P = 0.003). In multivariate analysis, pretransplant mortality was associated with higher MELD (P < 0.001) and was significantly greater in HIV+ than HIV- groups (P = 0.001). However, it did not differ between HIV+ H and NH (HR = 1.7; P = 0.36). Among HIV/HCV+, posttransplant mortality was similar between H and NH, despite lower CD4 in H (P = 0.04). In conclusion, this observational study confirms that hemophilia per se does not have a specific influence on transplant outcomes and that HIV infection increases the risk of mortality in both H and NH patients. Liver Transplantation 23 762-768 2017 AASLD.


Asunto(s)
Infecciones por VIH/cirugía , Hemofilia A/cirugía , Hepatitis C Crónica/cirugía , Fallo Hepático/cirugía , Trasplante de Hígado , Adulto , Antirretrovirales/uso terapéutico , Linfocitos T CD4-Positivos/citología , Coinfección/mortalidad , Interpretación Estadística de Datos , Progresión de la Enfermedad , Femenino , Infecciones por VIH/complicaciones , Infecciones por VIH/mortalidad , Hemofilia A/complicaciones , Hemofilia A/mortalidad , Hepatitis C Crónica/complicaciones , Hepatitis C Crónica/mortalidad , Humanos , Fallo Hepático/complicaciones , Fallo Hepático/mortalidad , Masculino , Persona de Mediana Edad , Análisis Multivariante , Oportunidad Relativa , Complicaciones Posoperatorias , Sistema de Registros , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento , Estados Unidos
9.
Liver Transpl ; 22(5): 615-26, 2016 05.
Artículo en Inglés | MEDLINE | ID: mdl-26684547

RESUMEN

Infection with carbapenem-resistant Acinetobacter baumannii (CRAB) after liver transplantation (LT) is associated with high mortality. This study aimed to identify risk factors for post-LT CRAB infection, as well as to evaluate the impact of pre-LT CRAB acquisition on the incidence of post-LT CRAB infection. This was a prospective cohort study of all patients undergoing LT at our facility between October 2009 and October 2011. Surveillance cultures (SCs) were collected immediately before LT and weekly thereafter, until discharge. We analyzed 196 patients who were submitted to 222 LTs. CRAB was identified in 105 (53.6%); 24 (22.9%) of these patients were found to have acquired CRAB before LT, and 85 (81.0%) tested positive on SCs. Post-LT CRAB infection occurred in 56 (28.6%), the most common site being the surgical wound. Multivariate analysis showed that the risk factors for developing CRAB infection were prolonged cold ischemia, post-LT dialysis, LT due to fulminant hepatitis, and pre-LT CRAB acquisition with pre-LT CRAB acquisition showing a considerable trend toward significance (P = 0.06). Among the recipients with CRAB infection, 60-day mortality was 46.4%, significantly higher than among those without (P < 0.001). Mortality risk factors were post-LT infection with multidrug-resistant bacteria, LT performed because of fulminant hepatitis, retransplantation, prolonged cold ischemia, longer LT surgical time, and pre-LT CRAB acquisition, the last showing a trend toward significance (P = 0.08). In conclusion, pre-LT CRAB acquisition appears to increase the risk of post-LT CRAB infection, which has a negative impact on recipient survival. Liver Transplantation 22 615-626 2016 AASLD.


Asunto(s)
Infecciones por Acinetobacter/complicaciones , Antibacterianos/farmacología , Carbapenémicos/farmacología , Farmacorresistencia Bacteriana , Fallo Hepático/complicaciones , Fallo Hepático/cirugía , Infecciones por Acinetobacter/tratamiento farmacológico , Acinetobacter baumannii/efectos de los fármacos , Adolescente , Adulto , Anciano , Electroforesis en Gel de Campo Pulsado , Femenino , Humanos , Trasplante de Hígado , Masculino , Persona de Mediana Edad , Análisis Multivariante , Complicaciones Posoperatorias , Estudios Prospectivos , Reoperación , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento , Adulto Joven
11.
Liver Transpl ; 20(10): 1193-201, 2014 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-24916539

RESUMEN

The Model for End-Stage Liver Disease (MELD) score has reduced predictive ability in patients with cirrhosis and MELD scores ≤ 20. We aimed to assess whether a 5-stage clinical model could identify liver transplantation (LT) candidates with low MELD scores who are at increased risk for death. We conducted a case-control study of subjects with cirrhosis and MELD scores ≤ 20 who were awaiting LT at a single academic medical center between February 2002 and May 2011. Conditional logistic regression was used to evaluate the risk of liver-related death according to the cirrhosis stage. We identified 41 case subjects who died from liver-related causes with MELD scores ≤ 20 within 90 days of death while they were waiting for LT. The cases were matched with up to 3 controls (66 controls in all) on the basis of the listing year, age, sex, liver disease etiology, presence of hepatocellular carcinoma, and MELD score. The cirrhosis stage was assessed for all subjects: (1) no varices or ascites, (2) varices, (3) variceal bleeding, (4) ascites, and (5) ascites and variceal bleeding. The MELD scores were similar for cases and controls. Clinical states contributing to death in cases were: sepsis 49%, spontaneous bacterial peritonitis 15%, variceal bleeding 24%, and hepatorenal syndrome 22%. In a univariate analysis, variceal bleeding [odds ratio (OR) = 5.6, P = 0.003], albumin (OR = 0.5, P = 0.041), an increasing cirrhosis stage (P = 0.003), reaching cirrhosis stage 2, 3, or 4 versus lower stages (OR = 3.6, P = 0.048; OR = 7.4, P < 0.001; and OR = 4.1, P = 0.008), a sodium level < 135 mmol/L (OR = 3.4, P = 0.006), and hepatic encephalopathy (OR = 2.3, P = 0.082) were associated with liver-related death. In a multivariate model including the cirrhosis stage, albumin, sodium, and hepatic encephalopathy, an increasing cirrhosis stage (P = 0.010) was independently associated with liver-related death. In conclusion, assessing the cirrhosis stage in patients with low MELD scores awaiting LT may help to select candidates for more aggressive monitoring or for living or extended criteria donation.


Asunto(s)
Hemorragia Gastrointestinal/mortalidad , Cirrosis Hepática/diagnóstico , Fallo Hepático/diagnóstico , Trasplante de Hígado , Selección de Paciente , Listas de Espera , Adolescente , Adulto , Anciano , Causas de Muerte/tendencias , Colorado/epidemiología , Femenino , Hemorragia Gastrointestinal/etiología , Humanos , Cirrosis Hepática/complicaciones , Cirrosis Hepática/mortalidad , Fallo Hepático/complicaciones , Fallo Hepático/cirugía , Masculino , Persona de Mediana Edad , Modelos Estadísticos , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Tasa de Supervivencia/tendencias , Factores de Tiempo , Adulto Joven
13.
J Pediatr ; 164(3): 553-9.e1-2, 2014 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-24321534

RESUMEN

OBJECTIVE: To determine the frequency of mitochondrial DNA depletion syndrome (MDS) in infants with cholestasis and liver failure and to further clarify the clinical, biochemical, radiologic, histopathologic, and molecular features associated with MDS due to deoxyguanosine kinase (DGUOK) and MPV17 gene mutations. STUDY DESIGN: We studied 20 infants with suspected hepatocerebral MDS referred to our tertiary care center between 2007 and 2013. Genomic DNA was isolated from blood leukocytes, liver, and/or skeletal muscle samples by standard methods. Mitochondrial DNA copy number relative to nuclear DNA levels was determined in muscle and/or liver DNA using real-time quantitative polymerase chain reaction and compared with age-matched controls. Nuclear candidate genes, including polymerase γ, MPV17, and DGUOK were sequenced using standard analyses. RESULTS: We identified pathogenic MPV17 and DGUOK mutations in 11 infants (6 females) representing 2.5% of the 450 cases of infantile cholestasis and 22% of the 50 cases of infantile liver failure referred to our center during the study period. All of the 11 patients manifested cholestasis that was followed by a rapidly progressive liver failure and death before 2 years of life. Mitochondrial DNA depletion was demonstrated in liver or muscle for 8 out of the 11 cases where tissue was available. Seven patients had mutations in the MPV17 gene (3 novel mutations), 4 patients had DGUOK mutations (of which 2 were novel mutations). CONCLUSION: Mutations in the MPV17 and DGUOK genes are present in a significant percentage of infants with liver failure and are associated with poor prognosis.


Asunto(s)
Colestasis/complicaciones , Fallo Hepático/complicaciones , Proteínas de la Membrana/genética , Proteínas Mitocondriales/genética , Mutación , Fosfotransferasas (Aceptor de Grupo Alcohol)/genética , Acidosis Láctica/complicaciones , Alanina Transaminasa/sangre , Aspartato Aminotransferasas/sangre , Bilis , Colestasis/mortalidad , ADN Mitocondrial/análisis , Femenino , Humanos , Lactante , Recién Nacido , Leucocitos/química , Hígado/química , Fallo Hepático/mortalidad , Masculino , Enfermedades Mitocondriales/genética , Enfermedades Mitocondriales/mortalidad , Músculo Esquelético/química , alfa-Fetoproteínas/análisis , gamma-Glutamiltransferasa/sangre
14.
Acta Gastroenterol Latinoam ; 43(2): 113-20, 2013 Jun.
Artículo en Español | MEDLINE | ID: mdl-23940911

RESUMEN

UNLABELLED: After the introduction of high active antiretroviral therapy (HAART), the human immunodeficiency virus (HIV) was no longer considered a contraindication for transplantation. Yet, liver disease in this population is characterized by an accelerated course that may impact on the waiting list. OBJECTIVE: To evaluate the experience in Argentina with HIV positive patients listed for liver transplantation. PATIENTS AND METHODS: We analyzed 52 HIV positive patients listed between July 2005 and March 2010 (Group HIV positive). Results were compared with 462 HIV negative patients included during the same period (Group HIV negative). Data were obtained from INCUCAI, the Argentinian procurement organism and from the Transplantation Centers. RESULTS: The etiology of liver disease in the Group HIV positive was hepatitis C 40, HBV 3, fulminant hepatitis 3, alcohol 2, retrasplant 2 and others 2. The mean MELD at the time of listing was 1615 (lower than 19 in 40 cases, higher than 19 in 8, emergency in 3) in the group HIV positive and 16.64 in the group HIV negative (NS). The outcome in the waiting list for HIV positive and negative patients respectively was: death 14 (27%) vs 61 (18.7%) (P < 0.05), cadaveric donor transplant 10 (13%) vs 95 (29.4%) (P < 0.001), living donor transplant 0 (0%) vs 5 (1.1%) (NS), mean time from listing to death 270.70 298.11 days vs 267.29 266.53 days (NS), mean time from listing to transplant 70.26 74.05 vs 261 187.6 days (P < 0.01), mean MELD at the time of death 12.54 (13 cases lower than 15, 1 higher than 19) vs 19.6 9.7 (P < 0.05), mean MELD at the time of transplantation 24.33 vs 24.1 7.6 (NS). CONCLUSION: HIV positive patients have high mortality in the waiting list and low access to liver transplantation. MELD score underscores the severity of liver disease in this population when compared to HIV negative patients.


Asunto(s)
Seropositividad para VIH/virología , Fallo Hepático/cirugía , Trasplante de Hígado , Listas de Espera , Adolescente , Adulto , Anciano , Argentina , Femenino , Humanos , Fallo Hepático/complicaciones , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Factores de Tiempo , Adulto Joven
16.
Transplant Proc ; 45(5): 1907-9, 2013 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-23769069

RESUMEN

Hepatoportal sclerosis (HPS), first reported by Mikkelsen et al in 1965, is a pathologic condition that does not cause cirrhotic portal hypertension. The primary hepatic lesion in HPS is found in portal vein branches with preserved synthetic function. Rarely do patients with HPS need liver transplantation. The aim of this study was to describe the clinical and pathologic features of 6 HPS cases who underwent liver transplantation (OLT). From 2000 to 2008, 6 OLT candidates were diagnosed with HPS: 3 displayed bleeding varices and 4 ascites. Child-Pugh evaluation was class B (n = 4) or C (n = 2). The Model for End-stage Liver Disease scores were 18 (n = 2), 20 (n = 3), and 22 (n = 1). Cirrhosis resulted from presumed diagnoses of alcohol n = (1), autoimmune n = (2) or cryptogenic cirrhosis n = (3). On histologic examination, there was marked phlebosclerosis in all cases, including nonocclusive portal vein thrombosis (n = 3), intense portal fibrosis (n = 1), moderate portal fibrosis (n = 5), and uniform moderate sinusoidal dilatation without megasinusoid formation, but with ductal biliary proliferation and ductal biliary fibrosis in all cases. Cholestasis was observed in 1 and incomplete septal cirrhosis in 4 cases. None of the subjects showed histological features of the presumed underlying liver disease. The overall survival of this group was no different from that of other OLT patients. HPS causing hepatic failure may require liver transplantation. Fhlebosclerosis andportal fibrosis may contribute to the loss of hepatic synthesis leading to the need for hepatic transplant. Significant portal fibrosis and phlebosclerosis can contribute to hepatic parenchymal and posterior synthetic loss.


Asunto(s)
Fallo Hepático/cirugía , Trasplante de Hígado , Vena Porta/cirugía , Esclerosis/cirugía , Adulto , Femenino , Humanos , Fallo Hepático/complicaciones , Masculino , Persona de Mediana Edad , Esclerosis/complicaciones
17.
Acta gastroenterol. latinoam ; 43(2): 113-20, 2013 Jun.
Artículo en Español | BINACIS | ID: bin-132991

RESUMEN

UNLABELLED: After the introduction of high active antiretroviral therapy (HAART), the human immunodeficiency virus (HIV) was no longer considered a contraindication for transplantation. Yet, liver disease in this population is characterized by an accelerated course that may impact on the waiting list. OBJECTIVE: To evaluate the experience in Argentina with HIV positive patients listed for liver transplantation. PATIENTS AND METHODS: We analyzed 52 HIV positive patients listed between July 2005 and March 2010 (Group HIV positive). Results were compared with 462 HIV negative patients included during the same period (Group HIV negative). Data were obtained from INCUCAI, the Argentinian procurement organism and from the Transplantation Centers. RESULTS: The etiology of liver disease in the Group HIV positive was hepatitis C 40, HBV 3, fulminant hepatitis 3, alcohol 2, retrasplant 2 and others 2. The mean MELD at the time of listing was 1615 (lower than 19 in 40 cases, higher than 19 in 8, emergency in 3) in the group HIV positive and 16.64 in the group HIV negative (NS). The outcome in the waiting list for HIV positive and negative patients respectively was: death 14 (27


) vs 61 (18.7


) (P < 0.05), cadaveric donor transplant 10 (13


) vs 95 (29.4


) (P < 0.001), living donor transplant 0 (0


) vs 5 (1.1


) (NS), mean time from listing to death 270.70 298.11 days vs 267.29 266.53 days (NS), mean time from listing to transplant 70.26 74.05 vs 261 187.6 days (P < 0.01), mean MELD at the time of death 12.54 (13 cases lower than 15, 1 higher than 19) vs 19.6 9.7 (P < 0.05), mean MELD at the time of transplantation 24.33 vs 24.1 7.6 (NS). CONCLUSION: HIV positive patients have high mortality in the waiting list and low access to liver transplantation. MELD score underscores the severity of liver disease in this population when compared to HIV negative patients.


Asunto(s)
Seropositividad para VIH/virología , Fallo Hepático/cirugía , Trasplante de Hígado , Listas de Espera , Adolescente , Adulto , Anciano , Argentina , Femenino , Humanos , Fallo Hepático/complicaciones , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Factores de Tiempo , Adulto Joven
18.
Acta gastroenterol. latinoam ; Acta gastroenterol. latinoam;43(2): 113-20, 2013 Jun.
Artículo en Español | LILACS, BINACIS | ID: biblio-1157369

RESUMEN

UNLABELLED: After the introduction of high active antiretroviral therapy (HAART), the human immunodeficiency virus (HIV) was no longer considered a contraindication for transplantation. Yet, liver disease in this population is characterized by an accelerated course that may impact on the waiting list. OBJECTIVE: To evaluate the experience in Argentina with HIV positive patients listed for liver transplantation. PATIENTS AND METHODS: We analyzed 52 HIV positive patients listed between July 2005 and March 2010 (Group HIV positive). Results were compared with 462 HIV negative patients included during the same period (Group HIV negative). Data were obtained from INCUCAI, the Argentinian procurement organism and from the Transplantation Centers. RESULTS: The etiology of liver disease in the Group HIV positive was hepatitis C 40, HBV 3, fulminant hepatitis 3, alcohol 2, retrasplant 2 and others 2. The mean MELD at the time of listing was 1615 (lower than 19 in 40 cases, higher than 19 in 8, emergency in 3) in the group HIV positive and 16.64 in the group HIV negative (NS). The outcome in the waiting list for HIV positive and negative patients respectively was: death 14 (27


) (NS), mean time from listing to death 270.70 298.11 days vs 267.29 266.53 days (NS), mean time from listing to transplant 70.26 74.05 vs 261 187.6 days (P < 0.01), mean MELD at the time of death 12.54 (13 cases lower than 15, 1 higher than 19) vs 19.6 9.7 (P < 0.05), mean MELD at the time of transplantation 24.33 vs 24.1 7.6 (NS). CONCLUSION: HIV positive patients have high mortality in the waiting list and low access to liver transplantation. MELD score underscores the severity of liver disease in this population when compared to HIV negative patients.


Asunto(s)
Fallo Hepático/cirugía , Listas de Espera , Seropositividad para VIH/virología , Trasplante de Hígado , Adolescente , Adulto , Adulto Joven , Argentina , Estudios Retrospectivos , Fallo Hepático/complicaciones , Factores de Tiempo , Femenino , Humanos , Anciano , Masculino , Persona de Mediana Edad , Índice de Severidad de la Enfermedad
19.
Ann Hepatol ; 10 Suppl 1: S21-8, 2011 May.
Artículo en Inglés | MEDLINE | ID: mdl-21566251

RESUMEN

Extracorporeal liver support has been a much studied topic throughout the last 50 years. Albumin dialysis as a therapeutic option for patients with acute liver failure or acute decompensation of chronic liver disease was introduced in the mid-nineties. The Molecular Adsorbent Recirculating System (MARS) is based on the concept of albumin dialysis and allows for the removal of protein-bound as well as water-soluble toxins. Besides its role as a sufficient volume expander human serum albumin is an important scavenger for molecules with pathophysiological relevance in liver failure. Albumin dialysis enables the selective regeneration of patient's albumin resulting in an increase of albumin binding capacity. Clinically, an improvement of central and local hemodynamics as well as liver-, brain-, and kidney-functions were observed. Thus, the treatment can contribute to liver regeneration and stabilization of vital organ functions and thus help to bridge patients to liver transplantation or to recovery of native liver function. Proper patient selection is critical for clinical success. Aggressive treatment of infections and sepsis seems to be a decisive pre-requisite for its safe and efficient use. Cautious anticoagulation with heparin is the common standard. Citrate use is recommended for patients prone to bleeding. Today, albumin dialysis MARS is among the best studied liver support methods. It appears as a valuable therapeutic tool for the treatment of various complications of of liver failure, especially hemodynamic instability and hepatic encephalopathy. Further studies will need to help defining the optimal patient selection and technical process parameters such as session length and frequency of treatment.


Asunto(s)
Albúminas/uso terapéutico , Fallo Hepático/terapia , Diálisis Renal/instrumentación , Diálisis Renal/métodos , Encefalopatía Hepática/etiología , Encefalopatía Hepática/terapia , Humanos , Hiperbilirrubinemia/etiología , Hiperbilirrubinemia/terapia , Fallo Hepático/complicaciones , Resultado del Tratamiento
20.
Ann Hepatol ; 10 Suppl 2: S66-9, 2011 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-22228885

RESUMEN

Introduction. Hepatic encephalopathy (HE) refers to a complex neuropsychiatric syndrome that is progressive but potentially reversible and may have a significant impact on quality of life, as it is characterized by alterations in cognitive function, behavior and personality as well as transient neurological symptoms and electroencephalographic abnormalities. Objective. The aim of this study was to evaluate scientific evidence for the effectiveness and safety of LOLA infusions for treatment of clinical hepatic encephalopathy in patients with chronic liver disease. Material and methods. We included all randomized, controlled, double-blind, and humans' studies that were published in indexed journals. Results. Were identified 48 references (17 using PubMed, 12 using Medline and 19 using the Cochrane database). Of these, six were selected as having met the inclusion criteria. A total of 623 patients were randomized in these publications. Conclusion. The available scientific evidence supports the adoption of LOLA infusion as a treatment for clinical encephalopathy in patients with liver failure, because it has been shown to improve neuropsychiatric status and decrease serum levels of ammonia with a low incidence of adverse effects (less than 5%).


Asunto(s)
Dipéptidos/uso terapéutico , Encefalopatía Hepática/tratamiento farmacológico , Encefalopatía Hepática/etiología , Fallo Hepático/complicaciones , Adulto , Anciano , Amoníaco/sangre , Dipéptidos/administración & dosificación , Dipéptidos/efectos adversos , Encefalopatía Hepática/sangre , Humanos , Infusiones Intravenosas , Persona de Mediana Edad , Ensayos Clínicos Controlados Aleatorios como Asunto , Resultado del Tratamiento
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