Investigation of risk factors related to the development of hepatic dysfunction in patients with a low and moderate cardiac risk during open-heart surgeries

Abstract Objective: To determine the possible risk factors associated with hepatic dysfunction during open-heart surgeries. Methods: After excluding 71 patients, 307 patients with possible low and moderate cardiac risk who underwent either coronary artery bypass graft surgery (CABG) (n=176) or valve repair surgery (mitral valve, mitral and aortic valves and/or tricuspid valve) (n=131) were investigated prospectively during a 6-month period. Hyperbilirubinemia is defined as an occurrence of a plasma total bilirubin concentration >34 µmol/L (2 mg/dL) in any measurement during the postoperative period; the patients were divided into groups with or without postoperative hyperbilirubinemia. The collected parameters were: alanine transaminase (ALT), aspartate transaminase (AST), alkaline phosphatase (ALP), total bilirubin (TBil), gamma-glutamyl transpeptidase (GGT) and albumin. The parameters were collected preoperatively and postoperatively on days 1, 3 and 7. Preoperative, intraoperative, and postoperative risk factors were investigated. Logistic regression analysis was performed to identify the risk factors for postoperative hyperbilirubinemia. Results: Postoperative hyperbilirubinemia was observed in 7 of 176 patients (4%) who underwent CABG, and in 11 of 131 patients (8.4%) who underwent valve replacement surgeries. Independent risk factors for early postoperative hyperbilirubinemia were found as: ejection fraction (EF), aortic cross-clamp (ACC) time, intensive care unit stay and extubation time (P<0.001). In comparison to CABG procedures, postoperative hyperbilirubinemia was observed more frequently in patients undergoing valve surgeries (P=0.027). Conclusion: Low EF and prolonged ACC time are significant independent risk factors for early postoperative hyperbilirubinemia during open-heart surgeries with cardiopulmonary bypass. Valve surgeries show a higher incidence of hyperbilirubinemia in comparison to CABG.

Coledocolitiasis múltiples y colangitis en paciente con quiste de colédoco: a propósito de un caso / Multiple choledocholithiasis and cholangitis in a patient with choledochal cyst: a case report

INTRODUCCIÓN: El quiste de colédoco (QC) es una patología infrecuente, caracterizada por una dilatación de vía biliar intra o extrahepática. Constituye una lesión congénita, representado 1% de las lesiones biliares benignas. Tiene una incidencia de 1 en 100.000 a 150.000 habitantes. Es más frecuente en mujeres, y su etiología es desconocida. En adultos los síntomas son inespecíficos; predominando dolor abdominal e ictericia. PRESENTACIÓN DEL CASO: Mujer de 61 años con cólico abdominal en hipocondrio derecho de tres días, vómitos e ictericia. Al ingreso hospitalario presentaba leucocitosis, hiperbilirrubinemia, aumento de fosfatasa alcalina, transaminasas y amilasa. Se plantearon los diagnósticos de ictericia obstructiva, pancreatitis y quiste hidatídico complicado, por lo que se realiza tomografía computada (TC) de abdomen evidenciando dilatación sacular intra y extrahepática, compatible con QC tipo IV-a. Se realizó colecistectomía y coledocostomía con sonda T de urgencia por evolución a colangitis con resultados favorables. DISCUSIÓN: Los QC son una causa rara de ictericia obstructiva. En Chile existen escasos datos estadísticos al respecto. Se manifiesta con una sintomatología inespecífica, sobretodo en adultos. El diagnóstico se realiza con hallazgos de laboratorio concordantes con ictericia colestásica, donde los estudios imagenológicos como ultrasonido y TC tienen un rol importante, pese a que en algunas ocasiones pueden pasar inadvertido. Es primordial un alto índice de sospecha para el diagnóstico y un tratamiento oportuno debido a su importante riesgo de progresión a colangiocarcinoma

INTRODUCTION: Choledochal cysts (CCs) is a rare disease characterized by dilatation of the intrahepatic or extrahepatic bile duct, which is about 1% of all benign biliary lesions. Its incidence is 1:100,000 to 150,000 habitants. It is more common in females, and its etiology is unknown. In adults the symptoms are nonspecific, predominantly abdominal pain and jaundice. CASE REPORT: 61 year old female patient with three days of severe abdominal colic in the right upper quadrant, whit both vomiting and jaundice. On admission, she presents leukocytosis, hyperbilirubinemia, and increased levels of alkaline phosphatase, transaminases and amylase. Diagnosis of obstructive jaundice, pancreatitis and complicated hydatid cyst arising. The abdominal CT Scan reveals intra and extrahepatic saccular dilatations, compatible with a type IV-a CCs. Both cholecystectomy and T-tube choledochotomy were done by evolution to cholangitis with favorable results and satisfactory postoperative. DISCUSSION: CCs is a rare cause of obstructive jaundice, and in this regard, there are few data described in Chile, Its diagnosis requires a high index of suspicion because of its nonspecific symptoms found mostly in adults. Despite this, the diagnosis is determined with laboratory findings consistent with cholestatic jaundice and support diagnostic imaging such as ultrasound, CT Scan, among others. Although the imaging findings, it may not be detected. A correct diagnosis and appropriate treatment is essential because of its high risk of progression to cholangiocarcinoma. Currently the patient is waiting for resection of extrahepatic bile duct and Roux-en-Y hepatic jejunostomy which is the optimal treatment.

Hepatitis alcohólica severa no respondedor a a glucocorticoides: reporte de un caso / Alcoholic hepatitis not responding to corticosteroids: a case report

INTRODUCCIÓN: La hepatitis alcohólica corresponde a un daño inflamatorio agudo sobre un hígado progresivamente dañado por la ingesta excesiva y prolongada de alcohol. Puede presentar ictericia, manifestaciones de alcoholismo crónico e insuficiencia hepática progresiva. PRESENTACIÓN DEL CASO: Varón de 60años con antecedentes de daño hepático crónico secundario a alcoholismo activo, que presentó cuadro de dos semanas de ictericia progresiva, prurito y bradipsiquia, asociado a leucocitosis, hiperbilirrubinemia, y elevación discreta de transaminasas, con predominio de GOT sobre GPT. Hemocultivos, urocultivo y serologías para virus hepatotropos fueron negativos. La ecografía abdominal mostró signos de hepatopatía crónica, sin dilatación de vía biliar. Con una función discriminante de Maddrey de 106 puntos se inició pentoxifilina, evolucionando tórpidamente. Se agregó prednisona durante siete días; se obtiene una puntuación de Lille de 0,99 (no respondedor), suspendiendo los corticoides. Progresó la insuficiencia hepática, con posterior insuficiencia renal aguda, acidosis metabólica, trastornos hidroelectrolíticos y fallecimiento al mes de evolución. DISCUSIÓN: La hepatitis alcohólica posee alta mortalidad. Existen múltiples escalas pronósticas. Los corticoides están indicados en casos severos, sin embargo hasta un 40 por ciento se catalogan como no respondedores. Se requieren nuevos tratamientos para mejorar la supervivencia de estos pacientes.

INTRODUCTION: Alcoholic hepatitis constitutes an acute inflammatory episode due to prolonged alcohol abuse on a previously damaged liver. Clinical features include jaundice, signs of chronic alcoholism and progressive liver failure. CASE REPORT: A 60-yearold male with a history of cirrhosis due to ongoing excessive intake of alcohol presented a two week history of progressive jaundice, pruritus, and bradypsychia. Laboratory tests showed leukocytosis, hyperbilirubinemia and a mild elevation of liver enzymes (GOT over GPT). Blood and urine cultures as well as serological markers for viral hepatitis were negative. Abdominal ultrasound showed signs of chronic liver disease, with no bile duct dilatation. A modified Maddrey’s discriminant function of 106 was determinant on starting therapy with pentoxifyline. However, patient’s status deteriorated. Prednisone was added to the treatment but seven days later, the patient was categorized as a non-responder (Lille score of 0.99), so the glucocorticoids were suspended. The patient’s liver failure progressed, after which renal failure, metabolic acidosis and electrolytic abnormalities developed; that led to his death after one month from admission. DISCUSSION: Alcoholic hepatitis requires prompt diagnosis and treatment, due to its high death rate. There are various prognostic scales available, one of which is the modified Maddrey’s discriminant function. The fundamental therapeutic measure is the use of intravenous glucocorticoids; yet up to 40 percent of patients qualify as non-responders.

Vanishing bile duct syndrome in a Hodgkin's lymphoma patient with fatal outcome despite lymphoma remission

Vanishing bile duct syndrome [VBDS] is a condition resulting from severe bile duct injury, progressive destruction, and disappearance of intrahepatic bile ducts [ductopenia] leading to cholestasis, biliary cirrhosis, and liver failure. VBDS can be associated with a variety of disorders, including Hodgkin's lymphoma [HL]. We describe a 33-year-old male patient who presented with lymphadenopathy and jaundice, and was diagnosed to have HL. Serum bilirubin worsened progressively despite chemotherapy, with a cholestatic pattern of liver enzymes. Diagnosis of VBDS was established on liver biopsy. Although remission from HL was achieved, the patient died of liver failure. Presence of jaundice in HL patients should raise the possibility of VBDS. This report discusses the difficulties of delivering chemotherapy in patients with liver dysfunction. HL-associated VBDS carries a high mortality but lymphoma remission can be achieved in some patients. Therefore, liver transplantation should be considered early in these patients.

A Case of Hereditary Spherocytosis Coexisting with Gilbert's Syndrome / 대한소화기학회지

We recently encountered a case of hereditary spherocytosis coexisting with Gilbert's syndrome. Patient was initially diagnosed with Gilbert's syndrome and observed, but other findings suggestive of concurrent hemolysis, such as splenomegaly and gallstones were noted during the follow-up period. Therefore, further evaluations, including a peripheral blood smear, osmotic fragility test, autohemolysis test, and red blood cell membrane protein test were performed, and coexisting hereditary spherocytosis was diagnosed. Genotyping of the conjugation enzyme uridine diphosphate-glucuronosyltransferase was used to confirm Gilbert's syndrome. Because of the high prevalence rates and similar symptoms of these 2 diseases, hereditary spherocytosis can be masked in patients with Gilbert's syndrome. In review of a case and other article, the possibility of the coexistence of these 2 diseases should be considered, especially in patients with unconjugated hyperbilirubinemia who also have splenomegaly and gallstones.

Hiperbilirrubinemia en apendicitis: es un factor predictivo de perforación / Hyperbilirubinemia in appendicitis: is a predictive factor of perforation

Background: An elevated total bilirubin level can be a marker for perforated appendicitis. Aim: To assess and compare the predictive value of total bilirubin, C-reactive protein (CRP), white-blood cell count, the lapse of symptoms evolution, and systemic inflammatory response syndrome (SIRS) for the diagnosis of perforated appendicitis. Material and Methods: Prospective study of 134 consecutive patients aged 33 +/- 16 years (63 males) operated for acute appendicitis of whom 49 had a perforated appendix. A preoperative blood sample was obtained to measure total bilirubin, C reactive protein and complete blood count. A systemic inflammatory response score was calculated. Results: The lapse of symptoms before operation was higher in patients with perforated appendicitis compared with their counterparts without perforation (105.2 +/- 79.3 and 38.6 +/- 17.5 hours respectively). C reactive protein values were 176 +/- 82.6 and 80 +/- 76 mg/1 respectively, (p = 0.01). Serum bilirubin values were 0.7 +/- 0.3 and 1.0 +/- 0.5 mg/dl, respectively (p = 0.05). Sixty five percent of patients with perforated appendicitis had a SIRS score between 3 and 4 points. A C reactive protein over 76.7 mg/1, a lapse of symptoms over 34.5 hours and a SIRS score of three or more had the best performance for the prediction of perforated appendicitis. Conclusions: The diagnosis of perforated appendicitis may be suspected based on CRP, SIRS, and the lapse of symptoms before operation. We do not recommend the use of total bilirubin to predict perforation in appendicitis.

Introducción: Se ha propuesto a la hiperbilirrubinemia como un marcador específico de apendicitis perforada. El objetivo del presente estudio es el de comparar el rendimiento para la predicción de perforación de la bilirrubina total (BT) y la proteína C reactiva (PCR), leucocitosis, el tiempo de evolución del cuadro clínico y el síndrome de respuesta inflamatoria sistémica (SIRS). Métodos: Se diseñó un estudio prospectivo y observacional, en el que se aplican curvas Receiver Operating Characteristics para comparar la sensibilidad y especificidad de las variables investigadas, se determinaron los mejores puntos de corte con la mejor sensibilidad y especificidad. Resultados: El período de tiempo de evolución del cuadro clínico se encontraba prolongado en los pacientes con apendicitis perforada (105,2 +/- 79,3 h y 38,6 +/- 17,5 h) y los niveles de PCR se encontraban muy elevados (176 +/- 82,6 mg/1 y 80 +/- 76 mg/1). La mayoría de los pacientes con apendicitis perforada tuvieron una puntuación SIRS entre 3 y 4 puntos. El valor de la PCR mayor a 76,7 mg/1, el tiempo de evolución de los síntomas mayor a 34,5 h y una puntuación SIRS de 3 puntos o más obtuvieron los mejores puntos de corte con el mejor rendimiento para la predicción de apendicitis perforada. Conclusiones: El diagnóstico de apendicitis perforada puede sospecharse cuando la PCR, SIRS y el período de tiempo de evolución del cuadro clínico están elevados. No recomendamos la medición de la BT como factor predictivo de perforación en pacientes con apendicitis.

Colestasias familiares: una causa común de hiperbilirrubinemia conjugada / Familial cholestasis: a comon cause of conjugated hyperbilurubinemia

Cholestasis is defined as a disorder affecting the production of bile resulting in the retention of its components in the liver and blood. In children, this disorder is almost always due to genetic alterations. Functionally, cholestasis may be the result of hepatic failure to secrete bile due to decrease in transport, synthesis or biliary obstruction. Extrahepatic cholestasis may be caused by biliary atresia and other obstructions of the bile ducts. Intrahepatic cholestasis may be the result of several disorders including progressive familial intrahepatic cholestasis (PFIC) types 1, 2 and 3, an autosomal recessive disease due to mutations in the genes ATP8B1, ABCBll and ABCB4 respectively. Pathophysiology and clinical presentation of this disease are now well understood. Clinically, these patients may present with jaundice, itching, anorexia, and generally unwell. Laboratory tests may disclose conjugated bilirubin over lmg/dl or larger than 20 percent of total bilirubin. Ursodeoxycholic acid, cholestiramine and biliary diversion may help in some of these conditions. Ongoing research into the mechanisms of genetic cholestasis could be key to therapy.

La Colestasia corresponde a un trastorno en la formación y excreción de la bilis que provoca retención de sus componentes y daño en hígado y sangre. La colestasia en el niño casi siempre se debe a una alteración hepática secundaria a causas ahora mayormente conocidas a nivel molecular. Desde el punto de vista funcional la colestasia resulta de una insuficiencia secretora del hígado debido a una disminución del flujo biliar por falla en los procesos de transporte o síntesis o a una obstrucción de la vía biliar. La colestasia extrahepática incluye la atresia de vías biliares y otras obstrucciones de la vía biliar. La colestasia intrahepática incluye las colestasias progresivas familiares PFIC 1, 2 y 3 causadas por fallas en los genes ATP8B1, ABCBll y ABCB4 respectivamente. Clínicamente pueden presentarse con ictericia, prurito, anorexia y compromiso del estado general. Desde el punto de vista del laboratorio las enfermedades colestásicas se caracterizan por hiperbilirrubinemia conjugada mayor a 1 mg/dl o mayor a 20 por ciento de bilirrubina total.

"Síndrome pequeño para su tamaño", (small-for-size), como complicación de un trasplante hepático con donante vivo adulto relacionado / Small-for-size syndrome: report of one case

Although the use of cadaveric split or living donor liver transplantation is a valid option for liver transplants, they have several complications, being the "small-for-size syndrome" one of the most frequent. This entity is mainly due to the incapacity that the graft has to meet the blood drainage demands. We report a 61 year-old patient with sub-acute liver failure, transplanted with a partial liver graft that developed hyperbilirubinemia, ascites and liver function deterioration. A meso-caval shunt was performed, after which the ascites resolved, serum bilirubin normalized and the synthetic function of the liver improved. After one month, a follow-up CT seen showed the absence of blood flow in the shunt, possible due to the reduction of the hyper-perfusion of the liver. The clinical and biochemical condition of the patient continued improving despite the lack of flow through the shunt.

Sickle cell hepatopathy.

Sickle cell hepatopathy is a well-documented entity that ranges from the self-limiting hepatic right upper quadrant syndrome to the potentially lethal intrahepatic cholestasis and acute hepatic sequestration syndromes. We describe a 26-year-male with homozygous sickle cell disease who had this unique hepatic presentation and was documented to have characteristic findings of cholestasis, portal inflammation and sinusoidal dilatation on histopathology.

Dubin-johnson syndrome

A young man presented with recurrent episodes of mild jaundice. Apart from conjugated hyperbilirubinemia, other liver function tests were always normal. Clinical suspicion of Dubin-Johnson syndrome was raised. Liver biopsy showed diffuse deposition of coarse granular dark brown pigment in hepatocytes. Dubin-Johnson syndrome is a benign condition, which results from a hereditary defect in biliary secretion of bilirubin pigments, and manifests as recurrent jaundice with conjugated hyperbilirubinemia. The defect is due to the absence of the canalicular protein MRP2 located on chromosomes 10q 24, which is responsible for the transport of biliary glucuronides and related organic anions into bile. No treatment is necessary and patients have a normal life expectancy

Central Pontine Myelinolysis in a Patient with Acute Lymphoblastic Leukemia after Hematopoietic Stem Cell Transplantation: A Case Report

We describe a 37-yr-old man who developed central pontine myelinolysis (CPM) after allogeneic hematopoietic stem cell transplantation (HSCT) for acute lymphoblastic leukemia. After HSCT, desquamation developed on the whole body accompanied by hyperbilirubinemia. The liver biopsy of the patient indicated graft-versus-host disease- related liver disease, and the dose of methylprednisolone was increased. Then, the patient developed altered mentality with eye ball deviation to the left, for which electroencephalogram and magnetic resonance imaging (MRI) scans were done. Brain MRI scan demonstrated the imaging findings consistent with central pontine myelinolysis and extrapontine myelinolysis. He did not have any hyponatremia episode during hospitalization prior to the MRI scan. To the best of our knowledge, presentation of CPM after allogeneic HSCT is extremely rare in cases where patients have not exhibited any episodes of significant hyponatremia. We report a rare case in which hepatic dysfunction due to graft-versus-host disease has a strong association with CPM after HSCT.

Acute Hemolysis and Renal Failure caused by Hepatitis A Infection with Underlying Glucose-6-Phosphate Dehydrogenase Deficiency / 대한진단검사의학회지

Although glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme disorder worldwide, it has rarely been reported among Korean. The patient was previously healthy 39 yr old male who showed severe hemolytic anemia and acute renal failure accompanied by hyperbilirubinemia after hepatitis A infection. The additional studies for differential diagnosis of hemolytic anemia showed a moderate deficiency of G6PD enzyme. Because hepatitis A infection in patient with G6PD deficiency present much more severe clinical symptoms, G6PD enzyme should be examined in patients with triggering factors of hemolysis such as hepatitis A infection.

Sobrevida de pacientes com estenose biliar maligna baseada no escovado endoscópico e na bilirrubinemia / Survival of patients with malignant biliary stricture based on endoscopic brushing and on bilirubinemia

RACIONAL: As principais causas de estenose biliar maligna são câncer de pâncreas e colangiocarcinoma. A definição do prognóstico dos pacientes no momento da pancreatocolangiografia retrógrada endoscópica é importante na escolha da conduta mais adequada. OBJETIVO: Avaliar a importância do escovado endoscópico e da bilirrubinemia na determinação da sobrevida dos pacientes com estenose biliar maligna. MÉTODOS: Os pacientes com estenose biliar diagnosticados durante pancreatocolangiografia retrógrada endoscópica foram submetidos a duplo escovado. Amostras de sangue de todos eles foram obtidas para dosagem das bilirrubinas. Os pacientes foram acompanhados para determinar o diagnóstico final e a sobrevida. RESULTADOS: Diagnóstico final de doença maligna foi obtido em 40 pacientes de um total de 50 casos de estenose biliar. Os níveis séricos elevados das bilirrubinas ou a citologia por escovado positiva para malignidade estava relacionada a menor sobrevida. CONCLUSÃO: Os dados desta pesquisa demonstram a possibilidade de determinar o prognóstico em casos de estenoses biliares malignas através do resultado do escovado endoscópico ou da bilirrubinemia.

Subgaleal hematoma and seven exchange transfusions.

A 3 kg baby was delivered by cesarean section after prolonged labor. He had massive subgaleal hematoma. He developed anemia requiring packed cell transfusions and hyperbilirubinemia requiring a total of seven exchange transfusions and highly intensive phototherapy. There were no adverse complications of the hyperbilirubinemia or the exchange transfusion.

Malarial hepatitis.

AIMS AND OBJECTIVE: To study the clinical, biochemical and histopathological changes in the liver of patients of Plasmodium falciparum malaria with jaundice. MATERIAL AND METHOD: This study was conducted on 50 PBF confirmed cases of Plasmodium falciparum malaria with jaundice. Detailed history, clinical examination, biochemical parameters for liver function test and blood for hepatitis B and C was done in all patients. Liver biopsy was done for detailed histopathological examination in all the 20 patients having serum bilirubin between 3 to 10 mg%. All patients were treated by IV/oral quinine using standard regimen. RESULTS: Age of the patient was ranging from 15-45 years. All patients had jaundice, 70% had pallor, 56% had splenomegaly, 48% had hepatomegaly and 24% of cases had coma. Based on serum bilirubin level, the patients were categorized in group A (18 patients, serum bilirubin < 3 mg%), in group B (20 patients, serum bilirubin 3-10 mg%) and in group C (12 patients, serum bilirubin >10 mg%). Histopathological examination done in all the 20 patients of group B, showed evidence of swollen hepatocytes (100%), malarial pigment deposition (75%), inflammatory infiltrates (60%), congestion of hepatocyte (50%) alongwith centrizonal necrosis in 25% of cases. CONCLUSION: The evidence of predominant conjugated hyperbilirubinemia, increased levels of AST and ALT along with evidence of hepatocellular necrosis in histopathological examination are strong evidence of gross hepatocytic dysfunction in patients of Plasmodium falciparum malaria with jaundice. Therefore the term malarial hepatitis should not be taken as a misnomer.

Essential fatty acid status in infants and children with chronic liver disease

The relationship between essential fatty acid [EFA] status and degree of hyperbilirubinaemia and oxidant stress in infants and children with chronic liver diseases was evaluated. Thirty patients with chronic cholestasis and 30 with liver cirrhosis were examined; 30 healthy subjects served as controls. Patient groups had significant decreases in EFAs and significant elevation of total bilirubin. Levels of thiobarbituric acid reactive substances were significantly raised and were significantly inversely correlated to decreased EFA levels. There were also significant decreases in retinol, alpha-tocopherol and alpha-tocopherol/total lipids ratio, which had significant positive correlations with decreased EFA levels. Infants and children with chronic liver diseases have a high risk of EFA deficiency correlated with progressive elevation of serum bilirubin and progressive deterioration of oxidant status

Ictericia neonatal I. fisiopatología, etiología, diagnóstico y encefalopatía bilirrubínica / Neonatal jaindice I. phisiology, etiology, diagnosis, and bilirubin encephalopathy

La ictericia neonatal es uno de los principales problemas a que se enfrentan los pediatras y neonatólogos en la consulta externa y en las áreas de urgencias y hospitalización. Decidir cuándo esta ictericia es fisiológica y cuándo requiere un abordaje completo a fin de determinar el origen y establecer el tratamiento adecuado, es uno de los retos más grandes en los últimos años debido a la falta de consenso entre los diferentes grupos de especialistas. En esta revisión se presentan aspectos de fisiopatología para entender mejor los mecanismos de producción de hiperbilirrubinemia indirecta en el neonato, se analizan las principales causas de ésta y se hace hincapié en los problemas hemolíticos y los conceptos actuales con relación a la ictericia por leche materna. Asimismo se presentan algunas entidades no tan comunes pero que deben considerarse en el diagnóstico diferencial de estos pacientes. Se presentan de manera modificada las recomendaciones de 1994 de la Academia Americana de Pediatría para el abordaje diagnóstico de estos niños al tomar en cuenta las características particulares de nuestra población y también se analizan los mecanismos implicados en la encefalopatía bilirrubínica, el cuadro clínico y algunas controversias relacionadas con el neurodesarrollo de estos niños.