Thrombosis and hemorrhage in the critically ill cirrhotic patients: five years retrospective prevalence study.

BACKGROUND: Cirrhotic patients present a complex interaction between deficient synthetic liver function, hemodynamic abnormalities and superimposed conditions that alter coagulation system. This alters both coagulation and fibrinolytic processes,increasing bleeding and thrombosis risks. Particularly, critically ill cirrhotic patients represent a diagnostic challenge since they have multiple comorbidities making the thrombotic and bleeding risks unpredictable. The prevalence of bleeding and thrombosis in this subset of patients remains poorly described. The main aim of this article is to describe the prevalence of thrombotic and hemorrhagic complications in cirrhotic patients admitted between 2007 and 2012 at Médica Sur Clinic and Foundation ICU. MATERIAL AND METHODS: We performed a five years retrospective study including every cirrhotic patient admitted to ICU between January 2007 and December 2012. RESULTS: The incidence of hemorrhage was 48.5%, the overall incidence of thrombotic complications was 13.66%. Variceal bleeding was the most prevalent hemorrhagic event and portal vein thrombosis the most common thrombotic event. Factors associated with presenting a bleeding episode included kidney injury, infection an thrombosis. Factors associated with increased thrombotic risk included ascitis,infection and bleeding. CONCLUSION: Critically ill cirrhotic patients have an high risk for both thrombotic and bleeding episodes. The association between the presence of bleeding and thrombotic events was statistically significant.

Coagulation abnormalities in the cirrhotic patient.

The clotting process is a dynamic array of multiple processes which can be described in four phases: platelet plug initiation and formation, clotting process propagation by the coagulation cascade, clotting termination by antithrombotic mechanisms and clot removal by fibrinolysis. The liver plays a central role in each of these phases of clotting process, as it synthesizes the majority of coagulation factors and proteins involved in fibrinolysis as well as thrombopoeitin, which is responsible for platelet production from megakaryocytes. Many pathological processes associated with cirrhosis, such as portal hypertension and endothelial dysfunction, as well as co-morbid conditions, may also alter the coagulation process. Consequently, patients with liver disease have a disturbed balance of procoagulant and anti-coagulant factors which deviates from the normal coagulation cascade. This situation poses an additional problem in the diagnostic and therapeutic approach to this group of patients, since traditional coagulation test may not be reliable for assessing bleeding or thrombotic risk and traditional transfusional strategies may not be applicable in cirrhotic patients. In this article, we review the pathophysiological bases of coagulation abnormalities, in cirrhotic patients, the diagnostic therapeutic strategies to be followed and its impact on the clinical outcome in the cirrhotic patient.

[DRESS syndrome. A clinical case report]. / Síndrome de DRESS. Reporte de un caso clínico.

BACKGROUND: DRESS syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) or reaction to drugs with eosinophilia and systemic symptoms is a serious drug reaction associated with the use of aromatic anticonvulsants and allopurinol. At least 44 drugs have been associated with DRESS. The aim was to present the case of a patient with DRESS syndrome associated with phenytoin. CLINICAL CASE: a 20 year old woman, with a history of seizures since childhood, presented generalised tonic-clonic seizures for the last three months. Therefore, she began treatment with 100 mg of phenytoin, administered orally, every 8 hours. Three weeks later, she developed fever up to 42 degrees, papules in the hands extending to trunk and extremities, generalized rubicund, pruritus, pain while urinating, adding hyperoxia, dysphagia and dry cough. Consequently, she went to the emergency room. DISCUSSION: the diagnosis is clinical and it is set according to the criteria of the scale of RegiSCAR. As the initial manifestations are unspecific, the diagnosis and treatment could be delayed. The importance of recognizing this syndrome is an early treatment to get better prognostics. The mortality is up to 10 %.

Introducción: el síndrome de DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) o la reacción a fármacos con eosinofilia y síntomas sistémicos es una reacción medicamentosa grave, asociada al uso de anticonvulsivos aromáticos y alopurinol. Se han descrito por lo menos 44 fármacos asociados a DRESS. El propósito es presentar el caso clínico de una paciente con síndrome de DRESS asociado a fenitoína. Caso clínico: paciente femenina de 20 años, con antecedente de crisis convulsivas desde la infancia, durante tres meses previos presentó crisis convulsivas tónico-clónicas generalizadas, por lo que inició tratamiento con fenitoína: 100 mg vía oral cada 8 horas. Tres semanas después presentó fiebre de hasta 42 grados, pápulas en manos con extensión a tronco y extremidades, rubicundez generalizada, prurito, dolor al orinar, además de hiporexia, disfagia y tos seca, por lo que acudió al servicio de urgencias. Discusión: el diagnóstico es clínico y se establece según los criterios de la escala de RegiSCAR. Debido a que las manifestaciones iniciales son poco específicas, el diagnóstico y el tratamiento definitivo pueden retrasarse. La importancia del reconocimiento y tratamiento temprano de esta entidad radica en la incidencia de mortalidad de hasta 10 %.

Acute kidney injury in critically ill cirrhotic patients: a review.

Acute kidney injury (AKI) is an important marker of morbidity and mortality in critically ill cirrhotic patients. The most common causes of AKI in cirrhotic patients include prerenal or hepatorenal syndrome (HRS). Diagnosis of AKI may be delayed by the lack of clinical, biochemical, and radiological markers with proven sensitivity and specificity in cirrhotic patients. In this review, we discuss the epidemiology, pathophysiology, diagnosis, and therapies for AKI in cirrhotic patients admitted to an intensive care unit (ICU).

Sarcoma granulocítico en una paciente con leucemia mieloide crónica / Granulocytic sarcoma in a female patient with chronic myeloid leukemia

El sarcoma granulocítico es un tumor localizado poco común, compuesto de células granulocíticas inmaduras. Generalmente se presenta en pacientes con leucemia mieloide aguda, síndromes mielodisplásicos o leucemia mieloide crónica. Puede ocurrir en cualquier localización anatómica. Reportamos el caso de una mujer de 41 años con el diagnóstico de leucemia mieloide crónica que presentó edema en brazo derecho que no remitía a pesar de tratamiento antibiótico y fasciotomía. El estudio histológico de la biopsia la lesión tomada mostró un sarcoma granulocítico. En este trabajo se revisa la citogenética, presentación clínica, diagnóstico y pronóstico de este síndrome tumoral.

Granulocytic sarcoma is an uncommon and localized extramedullary tumor composed of immature granulocytic cells. It may present in patiens with acute myeloid leukaemia, myelodysplastic syndrome or chronic myelogenous leukaemia. It may occur in any anatomical site. We report on the case of a 41 year old female diagnosed with chronic myelogenous leukaemia who presented with right arm swelling that did not resolve after antibiotic and surgical fasciotomy. The histological examination showed granulocytic sarcoma. In this report the citogenetics, clinical presentation, diagnosis and outcome of granulocytic sarcoma was reviewed.

Fragilidad y sarcopenia / Frailty and sarcopenia

La fragilidad es un síndrome geriátrico caracterizado por pérdida de peso, cansancio, debilidad, marcha lenta y disminución de la actividad física. Es más común en mujeres, obesos y diabéticos. Es secundaria a disregulación endócrina y a un estado proinflamatorio y protrombótico. La sarcopenia, pérdida de la masa muscular, es característica de la fragilidad. El tratamiento está encaminado a incrementar la masa y fuerza muscular mediante un mejor aporte calórico-protéico y un programa de ejercicios. El presente trabajo tiene como objetivo revisar conceptos actuales relacionados a la definición, epidemiología, fisiopatología y tratamiento del síndrome de fragilidad y la sarcopenia, así como su impacto en la población geriátrica.

Frailty is a geriatric syndrome characterized by weight loss, fatigue, weakness, slow walking and reduced physical activity. It is more common among women, obese people and diabetic patients. It is secondary to endocrine dys-regulation and a proinflammatory, prothrombotic status. Sarcopenia, loss of muscle mass, is characteristic of frailty. The treatment is focused on increasing muscle mass and strength by improving caloric-proteic intake and implementing a physical exercise program. The aim of the present article is to review the current concepts related to the definition, epidemiology, pathophysiology and treatment of frailty syndrome and sarcopenia, as well as its impact on geriatric population.

[Severe sepsis, septic shock and secondary multiple organ dysfunction in infection by Kluyvera ascorbata]. / Sepsis grave, choque séptico y disfunción orgánica múltiple secundaria a infección por Kluyvera ascorbata.

Kluyvera, a new genus of Enterobacteriaceae, is an emergent pathogen. Kluyvera species have been isolated from sputum, urine, stools, and blood. Kluyvera strains are infrequent but potentially dangerous pathogens in the immunocompetent or immunocompromised host due to their potential to provoke a wide range of infections and their ability to transfer extended spectrum beta lactamase genes. We herein report the case of a teenage male with severe sepsis and septic shock due to K. ascorbata.

Hígado graso y esteatohepatitis no alcohólica: Conceptos Actuales

El hígado graso no alcohólico (HGNA) incluye dentro de su presentación evolutiva a la esteatosis hepática, esteatohepatitis no alcohólica (EHNA), cirrosis y hepatocarcinoma. Se relaciona a obesidad, preferentemente abdominal, diabetes mellitus tipo II y síndrome metabólico (SM). En su fisiopatología están involucrados la sobrenutrición, vida sedentaria, factores genéticos y resistencia a la insulina. Su prevalencia es del 17 al 33%. La EHNA se presenta en el 30% de estos casos, de los cuales un 20 a 25% evoluciona a hepatocarcinoma. El HGNA es una de las causas más frecuentes de alteraciones en las pruebas de función hepática en pacientes asintomáticos. En su fase inicial, se caracteriza por malestar abdominal, fatiga, elevación de alanin aminotransferasa (AAT), gamaglutamil transpeptidasa (GGT), hepatomegalia, e hiperecogenicidad hepática en el ultrasonido. No es una enfermedad benigna, ya que el 32% de los enfermos progresan a fibrosis, el 20% a cirrosis y el riesgo de muerte relacionada a disfunción hepática es del 12% a 10 años. Las alternativas terapéuticas están dirigidas a modificar el estilo de vida, la dieta y el empleo de medicamentos, que en conjunto impactan en la fisiopatología de la enfermedad, en especial en la resistencia a la insulina y SM.

Non-Alcoholic Fatty Liver Disease (NAFLD) includes hepatic steatosis, non-alcoholic steatohepatitis (NASH), cirrhosis, and hepatocellular carcinoma in its clinical presentation and evolution. It is related to obesity, especially abdominal, type 2 diabetes mellitus, and Metabolic Syndrome (SM). Overnutrition, sedentarism, genetic factors, and insulin resistance have been involved in its physiopathology. The prevalence of NAFLD ranges from 17 to 33%. NASH is present in 30% of these cases, 20 to 25% of which become hepatocellular carcinoma. NAFLD is one of the most frequent causes of alterations in hepatic function tests in asymptomatic patients. In its early stage, its main features are abdominal discomfort, fatigue, alanine-aminotransferase (ALAT) increase, gamma-glutamyl transpeptidase (GGT), hepatomegaly, and hepatic hyperechogenicity on ultrasound scan. It is not a benign disease, since 32% of patients develop fibrosis, 20% fibrosis, and death risk related to hepatic dysfunction is 12% at 10 years. Therapeutic alternatives aim at modifying the life style and diet, and also include the prescription of drugs, all this affects the disease physiopathology, especially insulin resistance and metabolic syndrome.

Gallbladder disease in patients with primary sclerosing cholangitis.

BACKGROUND/AIMS: Gallbladder abnormalities may be part of the spectrum in primary sclerosing cholangitis (PSC). The aim of the present study was to evaluate the occurrence and prognostic importance of gallbladder abnormalities in patients with PSC. METHODS: Presence of gallbladder abnormalities was assessed in 286 patients with PSC treated at the Liver Unit, Karolinska University Hospital, Huddinge, between 1970 and 2005. RESULTS: One or more gallbladder abnormalities were found in 41% of the patients. Gallstones were found in 25% and cholecystitis in 25%. Cholecystitis among patients with extrahepatic involvement of PSC (30% (65/214)) was significantly higher than among those with intrahepatic involvement (9% (6/70)) (P<0.0001). A gallbladder mass lesion with a mean size of 21 (+/-9) mm (S.D.) was found in 18 (6%) patients, in 56% (10/18) of whom it constituted gallbladder carcinoma. In 9 patients without a gallbladder mass lesion, histological re-evaluation disclosed epithelial dysplasia of the gallbladder. CONCLUSIONS: Gallbladder disease is common in patients with PSC. Dysplasia and carcinoma are commonly found in gallbladder epithelium, suggesting that regular examination of the gallbladder in PSC patients could be of value for early detection of a gallbladder mass lesion. Cholecystectomy is recommended when such a lesion is detected, regardless of its size.