Enfermedad hepática en la fibrosis quística pediátrica. Una revisión de los conocimientos actuales / Cystic fibrosis liver disease in children ­ A review of our current understanding

La fibrosis quística, la segunda enfermedad genética más frecuente, es el resultado de una proteína de canal mutada, la CFTR, que secreta iones de cloro que fluidifican las secreciones. La esperanza de vida en los pacientes ha aumentado en años recientes gracias a mejoras en el tratamiento. No obstante, las complicaciones hepáticas son la tercera causa de muerte y la comprensión de su fisiopatología es aún deficiente. Se considera que la obstrucción biliar secundaria a la presencia de secreciones espesas conduce a la cirrosis. Sin embargo, el ácido ursodesoxicólico no ha modificado la historia natural. Además, la presencia de hipertensión portal en ausencia de cirrosis no puede ser explicada. Se ha propuesto el rol de la CFTR como modulador de tolerancia inmune, que explica la presencia de una inflamación portal persistente que culmina en fibrosis. El eje intestino-hígado tendría un rol importante en la presentación y la progresión de esta enfermedad

Cystic fibrosis is the second most common genetic disease in infancy. It is the result of a mutated channel protein, the CFTR, which secretes chloride ions, fluidifying secretions. Recent improvements in the treatment have increased life expectancy in these patients. Nevertheless, liver involvement remains the third cause of death. Unfortunately, our understating of the physiopathology is still deficient. Biliary obstruction secondary to the presence of thick secretions is considered to lead to cirrhosis. However, treatment with ursodeoxycolic acid has not changed the natural history. Furthermore, the presence of portal hypertension in the absence of cirrhosis cannot be explained. Recently, the role of CFTR as modulator of immune tolerance has been proposed, which could explain the presence of a persistent portal inflammation leading to fibrosis, and the gut-liver axis would also have a role in disease presentation and progression.

Clinical analysis of liver dysfunction induced by SHR-1210 alone or combined with apatinib and chemotherapy in patients with advanced esophageal squamous cell carcinoma / 中华肿瘤杂志

Objective: To investigate the clinical characteristics of abnormal liver function in patients with advanced esophageal squamous carcinoma treated with programmed death-1 (PD-1) antibody SHR-1210 alone or in combination with apatinib and chemotherapy. Methods: Clinical data of 73 patients with esophageal squamous carcinoma from 2 prospective clinical studies conducted at the Cancer Hospital Chinese Academy of Medical Sciences from May 11, 2016, to November 19, 2019, were analyzed, and logistic regression analysis was used for the analysis of influencing factors. Results: Of the 73 patients, 35 had abnormal liver function. 13 of the 43 patients treated with PD-1 antibody monotherapy (PD-1 monotherapy group) had abnormal liver function, and the median time to first abnormal liver function was 55 days. Of the 30 patients treated with PD-1 antibody in combination with apatinib and chemotherapy (PD-1 combination group), 22 had abnormal liver function, and the median time to first abnormal liver function was 41 days. Of the 35 patients with abnormal liver function, 2 had clinical symptoms, including malaise and loss of appetite, and 1 had jaundice. 28 of the 35 patients with abnormal liver function returned to normal and 7 improved to grade 1, and none of the patients had serious life-threatening or fatal liver function abnormalities. Combination therapy was a risk factor for patients to develop abnormal liver function (P=0.007). Conclusions: Most of the liver function abnormalities that occur during treatment with PD-1 antibody SHR-1210 alone or in combination with apatinib and chemotherapy are mild, and liver function can return to normal or improve with symptomatic treatment. For patients who receive PD-1 antibody in combination with targeted therapy and chemotherapy and have a history of long-term previous smoking, alcohol consumption and hepatitis B virus infection, liver function should be monitored and actively managed in a timely manner.

Alleviation of diabetes-induced hepatotoxicity by date palm hydroalcoholic extract in rat model; a biochemical, immunohistochemical and stereological study / Alivio de la hepatotoxicidad inducida por diabetes mediante el extracto hidroalcohólico de palmera datilera en modelo de rata: un estudio bioquímico, inmunohistoquímico y estereológico

SUMMARY: The present study was aimed to investigate the hepatoprotective effects of date palm hydroalcoholic extract (DP)in diabetic rats using biochemical and histopathological approaches. Diabetes was induced by administration of 60 mg/kg of streptozotocin intraperitoneally. In this analysis 32 adult rats were randomly divided into four groups; group 1: non-diabetic control whic received 0.1 mL normal saline, group 2:served as non-diabetic control which treated with 270 mg/kg of DP, group 3: served as untreated diabetic, and group 4: diabetic rats treated with 270 mg/kg of DP. Diabetic rats treated with the DP extracts exhibited lower hepatic oxidative stress and lower hepatic enzymes level. Extract treatment decreased the level of malondealdehyde (MDA) as a marker of lipid peroxidation. Stereological estimations revealed a significant increase in the liver volume in diabetic rats which was reduced in DP-treated rats. Immunofluorescence staining showed high synthesis of acrolein as a byproduct of lipid proxidation. While, optical density measurement revealed significant decrease in acrolein after DP administration. Histopathological examination showed severe changes in untreated diabetic liver tissue manifested by dilated portal vein, leukocytic infiltration, fatty degeneration and necrotic nuclei, whereas, DP treatment attenuated the adverse effects of diabetes on the liver represented by relatively healthy hepatocytes and sinusoids. The obtained results indicated that date pam extract was beneficial in the prevention of diabetes-induced hepatotoxicity due to its natural antioxidant constituents. Further preclinical and clinical studies are needed for considering this plant in management of prediabetes and diabetes hepatic complications.

RESUMEN: El presente estudio tuvo como objetivo investigar los efectos hepatoprotectores del extracto hidroalcohólico (DP) de la palmera datilera en ratas diabéticas utilizando enfoques bioquímicos e histopatológicos. La diabetes fue inducida mediante la administración de 60 mg / kg de estreptozotocina por vía intraperitoneal. Se dividieron al azar 32 ratas adultas en cuatro grupos; grupo 1: control no diabético que recibió 0,1 mL de solución salina normal, grupo 2: control no diabético tratado con 270 mg / kg de DP, grupo 3: fue separado como diabético no tratado, y grupo 4: ratas diabéticas tratadas con 270 mg / kg de DP mg / kg de DP. Las ratas diabéticas tratadas con los extractos de DP mostraron menor estrés oxidativo hepático y menor nivel de enzimas hepáticas. El tratamiento con extracto disminuyó el nivel de malondealdehído (MDA) como marcador de la proxidación de lípidos. Las estimaciones estereológicas revelaron un aumento significativo en el volumen del hígado en ratas diabéticas que se redujo en las ratas tratadas con DP. La tinción por inmunofluorescencia mostró una alta síntesis de acroleína como subproducto de la proxidación de lípidos. Mientras que, la medición de la densidad óptica reveló una disminución significativa de la acroleína después de la administración de DP. El examen histopatológico mostró cambios significativos en el tejido hepático diabético no tratado manifestados por vena porta dilatada, infiltración leucocítica, degeneración grasa y núcleos necróticos, mientras que el tratamiento con DP atenuó los efectos adversos de la diabetes en el hígado representados por hepatocitos y sinusoides relativamente sanos. Los resultados obtenidos indicaron que el extracto de palmera datilera fue beneficioso en la prevención de la hepatotoxicidad inducida por diabetes debido a sus constituyentes antioxidantes naturales. Se necesitan más estudios clínicos para considerar esta planta en el manejo de la prediabetes y las complicaciones hepáticas de la diabetes.

Elevación de enzimas hepáticas inducida por COVID-19 en embarazada / Elevated liver enzimes induced by COVID-19 in pregnancy

INTRODUCCIÓN: Las alteraciones del perfil hepático durante el embarazo ocurren en 3-5% de las gestantes. Una nueva etiología que se ha presentado en el contexto de pandemia actual es el síndrome respiratorio agudo severo relacionado con el nuevo coronavirus (SARS-CoV-2). Éste es responsable de alteraciones hepáticas en 2 a 11% de la población general infectada por este virus, y de hasta un 30% en las embarazadas que se infectan con SARS-CoV-2. Con el objetivo de mostrar una presentación poco frecuente del SARS-CoV-2 se expone un caso clínico de elevación de transaminasas en embarazada inducida por este nuevo virus. CASO CLÍNICO: Paciente de 36 años, cursando embarazo de 20+6 semanas, consulta por dolor abdominal asociado a ictericia y coluria. Se solicita estudio donde destaca elevación de transaminasas. Ecografía abdominal con vía biliar fina. Se descartan diferentes etiologías de hepatitis aguda y crónica (dada la falta de antecedentes). Finalmente se solicita PCR para COVID-19 que resulta positiva. CONCLUSIÓN: Luego de un estudio exhaustivo de diferentes etiologías de elevación de transaminasas, se atribuye esta alteración enzimática a SARS-CoV-2. Se decide seguimiento ambulatorio estricto con pruebas hepáticas cada dos semanas. La paciente evoluciona estable con exámenes normales luego de un mes desde que se indica el alta hospitalaria. Después de descartar etiologías frecuentes de elevación de transaminasas durante el embarazo, sugerimos solicitar el estudio de este virus con PCR para COVID-19, ya que podría ser una presentación poco frecuente de SARS-CoV-2.

INTRODUCTION: Approximately 3-5% of women present alterations of hepatic enzymes during pregnancy. Under the new circumstances that the world is facing with the SARS-COV2 pandemic, a new etiology for hepatic enzyme alterations has risen. The severe acute respiratory syndrome that the novel coronavirus causes is responsible for hepatic enzyme alterations in 2 to 11% of the sick population that did not have a previous underlying hepatic condition. Furthermore, hepatic enzyme alterations in pregnant women infected with SARS-COV2 presents in up to 30% of the cases. An infrequent presentation of SARS-COV2 is presented as our clinical case. CLINICAL CASE: A 36-year-old patient with a 20+6 week pregnancy presents abdominal pain, jaundice and choluria. General blood workup shows elevated transaminases. The abdominal ultrasound revealed a thin bile duct. Acute and chronic hepatitis etiologies were discarded. Finally, a PCR of COVID-19 was solicited, which turned out to be positive. CONCLUSIÓN: After an exhaustive study to determine the etiology of the elevated transaminases, the hepatic alterations were attributed to SARS-COV2 infection. A conservative management was adopted, with outpatient follow-up with liver testing every two weeks. The patient progresses with a stable steady decline in hepatic enzyme levels, and one-month post hospital discharge, her transaminases had reached normal values. Based on this clinical case, after ruling out frequent etiologies for elevated transaminases during pregnancy, it seems reasonable to request a PCR for COVID-19, since it could be a rare presentation of SARS-CoV-2.

Comportamiento de las hepatopatías en el embarazo valoradas por gastroenterología / Behavior of liver diseases in pregnancy valued by Gastroenterology

RESUMEN Introducción: el embarazo supone cambios en la fisiología de la mujer. Estos cambios pueden llevar a la aparición de enfermedades que afectan el hígado como: síndrome de HELLP, colestasis gravídica intrahepática, esteatosis hepática aguda del embarazo, entre otras, que pueden repercutir en el curso de la gestación. Material y métodos: se realizó un estudio observacional, descriptivo, retrospectivo de corte transversal en 52 gestantes que fueron valoradas en gastroenterología por sospecha de hepatopatías durante el embarazo en el año 2018, que constituyeron el universo de estudio, con el objetivo de determinar el comportamiento de las hepatopatías durante la gestación en las embarazadas valoradas por el servicio de Gastroenterología del Hospital Universitario "Comandante Faustino Pérez Hernández" de Matanzas. Se estudiaron las variables: trimestre gestacional, síntomas y signos, resultados analíticos, entidad nosológica, tipo de parto y complicación neonatal. Se elaboró una planilla para la recolección de los datos. Los resultados se analizaron en frecuencias absolutas y porcientos y se expusieron en tablas. Resultados: predominaron las gestantes del tercer trimestre con 61.6 %. El síntoma más frecuente fue el prurito en 33 gestantes (63.6%). La hipertransaminasemia se manifestó en el 100% de las gestantes. La entidad más frecuente fue la hepatitis crónica por virus B en 19 gestantes (36.5%) seguida de la colestasis intrahepática del embarazo con un 25 %. La mayoría de los partos fueron realizados por cesárea (94.2 %). La principal complicación neonatal fue el bajo peso al nacer en 26 (39.4 %). Conclusiones: las hepatopatías propias de la gestación se comportaron con igual frecuencia descrita en la literatura de acuerdo al trimestre que predominó, aunque fue significativa la incidencia de gestantes valoradas con infección por virus de la hepatitis b cuyo diagnóstico se hizo durante el embarazo siendo la principal causa de las complicaciones neonatales observadas (AU).

SUMMARY Introduction: Pregnancy supposes changes in the woman's physiology. These changes can lead to the appearance of illnesses affecting the liver, such as Hellp syndrome, intrahepatic cholestasis gravidarum, acute hepatic steatosis of pregnancy, among others, that can rebound in the course of the gestation. Materials and methods: a cross-sectional retrospective, descriptive, observational study was carried out in 52 pregnant women that were valued in Gastroenterology due to the suspicion of liver diseases during pregnancy in 2018; they were the universe of the study and the aim was determining the behavior of liver diseases during pregnancy in pregnant women valued in the service of Gastroenterology of the university hospital "Comandante Faustino Pérez Hernández" of the province of Matanzas: The studied variables were: gestational trimester, symptoms and signs, analytical results; nosological entity, type of delivery and neonatal complications. A form was draw up to collect data. The results were analyzed in absolute frequencies and percentages and showed in tables. Results: The third semester pregnant women predominated with 61.7 %. Pruritus was the most frequent symptom in 33 pregnant women (63.6 %). High levels of serum transaminases were present in 100 % of the studied women. The most frequent disease was chronic hepatitis caused by virus B in 19 pregnant women (36.5 %), followed by intrahepatic cholestasis of pregnancy with 25 %. Most of deliveries were performed by cesarean (94.2 %). The main neonatal complication was low weight at birth in 26 (39.4 %). Conclusions: liver diseases that are proper of gestation behaved in the same frequency as they are described in literature in relation to the predominating semester, although it was significant the incidence of assessed pregnant women with infection caused by the virus of hepatitis B diagnosed during pregnancy; it was the main cause of the observed neonatal complications (AU).

Enfermedades hepáticas y su relación con hiperglicemia / The relationship between liver diseases and hyperglycemia

La Diabetes Mellitus tipo 2 (DM2) y las enfermedades crónicas del hígado(ECH), definida para esta revisión como cualquier alteración funcional o estructural de este órgano, desde inflamación hasta fibrosis, son patologías que frecuentemente se asocian, y su coexistencia se relaciona con peor pronóstico y mayores complicaciones de ambas entidades. El objetivo de este artículo es describir la relación entre hiperglicemia y enfermedades del hígado, sus procesos fisiopatológicos comunes y tratamiento, distinguiendo las patologías más relevantes, entre ellas la Diabetes Hepatogénica (DH), la enfermedad hepática por Virus Hepatitis C (VHC) y la Enfermedad Hepática Grasa No Alcohólica (EHGNA). La DH es aquella diagnosticada en pacientes con cirrosis asociada a insuficiencia hepática, sin antecedentes previos de alteración de la glicemia. En la actualidad el diagnóstico se realiza en etapas tardías de la enfermedad. El VHC tiene un efecto diabetogénico conocido. Algunas terapias antivirales usadas para VHC evidencian mejoría de las alteraciones metabólicas al lograr respuestas virológicas sostenidas. En DM2, la EHGNA es frecuente, con mayor incidencia de fibrosis, hepatocarcinoma (HCC) y riesgo cardiovascular (RCV). Es necesario realizar una pesquisa e intervención precoz de EHGNA a los pacientes con DM2. En el manejo de éstos, la baja de peso ha demostrado ser efectiva en el control glicémico y en la mejoría histológica. Dentro de las terapias antidiabéticas, además del uso de metformina, debería considerarse aquellas que han demostrado a la fecha beneficios en EHGNA, como son tiazolidinedionas (pioglitazona) y/o análogos de GLP-1 (liraglutide) y optimizar el control de otros factores de RCV.

Type 2 Diabetes Mellitus (DM2) and chronic liver diseases (CLD) defined in this revision as any functional or structural alteration in the organ, covering from inflammation to fibrosis, are pathologies that are frequently associated, and when found together are related to worse prognosis and higher complications in both conditions. The objective of this article is to describe the relationship between hyperglycemia and liver diseases, their common physio-pathological processes and treatments, identifying the most important pathologies, including Hepatogenic Diabetes (HD), Hepatitis C Virus (HCV) liver disease and Non-Alcoholic Fatty Liver Disease (NAFLD). Hepatogenic diabetes (HD) is diagnosed in patients with liver failure associated to cirrhosis with no previous record of impaired glycemia. Currently, diagnosis is made during the late stages of the disease. Hepatitis C virus (HCV) has a known diabetogenic effect. Some antiviral therapies used for HCV show improvement in metabolic alterations by achieving sustained virological responses. Non-alcoholic fatty liver disease (NAFLD) in DM2 patients is common, presenting higher risk for fibrosis, hepatocellular carcinoma (HCC) and increased cardiovascular risk (CVR). Early screening and interventions for NAFLD in DM patients are necessary. Weight loss has been shown to be effective in glycemic control and histological improvement. Anti-diabetic therapies, in addition to the use of metformin, should consider therapies that have shown benefits for managing NAFLD, such as thiazolidinedione (pioglitazones) and/or aGLP-1 (Liraglutide), and optimally controlling other cardiovascular risk (CVR) factors.

Déficit de Alfa-1 antitripsina, una causa de hepatopatía crónica comúnmente insospechada en el adulto: presentación de 9 casos con revisión de literatura / ALFA-1 antitrypsin deficiency, a commonly missed cause of chronic liver disease in the adult: presentation of 9 cases with review of current literature

Introducción: El déficit de alfa-1 Antitripsina (a1AT) es una de las enfermedades genéticas más prevalentes en el ser humano, lastimosamente como entidad clínica tiende a ser pobremente sospechada. Con más de 100 mutaciones conocidas, las que se encuentran asociadas a enfermedad hepática son los homocigotos Z en el alelo del gen a1AT que ocurre en 1 a 2000- 3500 nacimientos. A diferencia de la enfermedad pulmonar donde las secuelas ocurren primordialmente por el déficit propio de la a1AT con destrucción enzimática de la microestructura de la vía aérea, el compromiso hepático ocurre por acúmulo intracelular de la proteína Z mutante, que se desdobla de forma aberrante, en vez de ser secretada. Esta acumulación produce lesión celular, hepatitis, fibrosis, cirrosis y carcinoma hepatocelular (CHC) por desencadenar una serie de eventos que culminan con la apoptosis hepatocitaria, regeneración e injuria crónica. Materiales y métodos: Se presentan 9 casos de pacientes que se han encontrado bajo nuestro cuidado, con edades variadas desde infantes hasta adultos mayores. Resultados: Cada uno con una presentación clínica distinta que va desde la elevación de enzimas hepáticas y otros como cirrosis que se han trasplantado con diagnóstico confirmatorio postquirúrgico. Conclusión: Se comenta acerca del manejo de la hepatopatía y su progresión a lo largo del tiempo que se han mantenido en la clínica de hígado a nuestro cargo.

Introduction: Alfa 1-antitrypsin deficiency is one of the most prevalent genetic diseases in the human being, sadly it is not a commonly suspected clinical entity. With more than 100 known mutations, those associated with hepatic disease are the Z homocygote allele mutations in the gene a1AT which occur in every 2000-3500 births. Opposing to the pulmonary disease, in which de sequelae are caused by the deficit of this protein which in turn fastens the enzymatic destruction of the airway microstructure, the hepatic compromise is secondary to the intracellular accumulation of the aberrant misfolded protein. This accumulation causes cellular damage, hepatitis, fibrosis, cirrhosis and hepatocellular carcinoma through activation of a series of mechanisms which culminate in hepatocitary apoptosis, regeneration and chronic cellular injury. Materials and methods: 9 cases of confirmed a1AT deficiency are presented, from different ages ranging from adolescence through elderly patients. Results: Each of one of them with different clinical presentation going from asymptomatic liver enzyme elevations to transplanted cirrhosis in which the diagnosis was post procedural. Conclusion: We comment about the management of the chronic liver disease and the evolution of these patients through time in the liver clinic.

Effects of different periods of gastric ischemia on liver as a remote organ

Abstract Purpose: To evaluate the hepatic changes associated with gastric ischemia. Methods: Thirty male rabbits were studied, distributed in 3 groups (n=10). Group 1: ligature and section of the gastric vasculature and removal of the liver after three hours; Group 2: ligature and section of the gastric vasculature and removal of the liver after 6 hours; Group 3: ligature and section of the gastric vasculature and removal of the liver after 12 hours. Blood samples were collected immediately before surgery and after the determined time of ischemia in each group to evaluate the hepatic function. After the death of the rabbits, the liver was removed for macro and microscopic study. Results: An increase in aminotransferases and bilirubin occurred in groups 2 and 3. Total protein and albumin diminished in all of the animals. All of the rabbits from groups 2 and 3 presented hepatocellular necrosis. Conclusion: The devascularization of the stomach for a period of above three hours is associated with hepatic morphological and functional disorders.

Hepatobiliary disease in children and adolescents with cystic fibrosis / Doença hepatobiliar em crianças e adolescentes com fibrose cística

Abstract Objectives: The aims of the study were to determine the frequency of hepatobiliary disease in patients with cystic fibrosis and to describe the sociodemographic, clinical, and laboratory profile of these patients. Methods: This was a retrospective, descriptive, and analytical study of 55 patients diagnosed with cystic fibrosis, aged between 3 months and 21 years, followed-up from January 2008 to June 2016 in a referral center. Medical records were consulted and sociodemographic, clinical and laboratory data, including hepatobiliary alterations, imaging studies, genetic studies, liver biopsies, and upper digestive endoscopies were registered. Results: Hepatobiliary disease was diagnosed in 16.4% of the patients and occurred as an initial manifestation of cystic fibrosis in 55.6% of these cases. The diagnosis of hepatopathy occurred before or concomitantly with the diagnosis of cystic fibrosis in 88.9% of the children. All patients with hepatobiliary disease were considered non-white, with a predominance of females (77.8%) and median (IQR) of 54 (27-91) months. Compared with the group without hepatobiliary disease, children with liver disease had a higher frequency of severe mutations identified in the CFTR gene (77.8% vs. 39.6%, p = 0.033) and severe pancreatic insufficiency (88.9% vs. 31.6%, p = 0.007). Conclusion: The frequency of hepatobiliary disease was high, with a very early diagnosis of the disease and its complications in the studied series. A statistical association was observed between the occurrence of hepatobiliary disease and the presence of pancreatic insufficiency and severe mutations in the CFTR gene. It is emphasized that cystic fibrosis is an important differential diagnosis of liver diseases in childhood.

Resumo Objetivos: Os objetivos do estudo foram determinar a frequência da doença hepatobiliar em pacientes com fibrose cística e descrever o perfil sociodemográfico, clínico e laboratorial destes. Métodos: Estudo retrospectivo, descritivo e analítico de 55 pacientes com diagnóstico de fibrose cística, entre três meses e 21 anos, acompanhados de janeiro de 2008 a junho de 2016 em um centro de referência. Foi realizada consulta aos prontuários médicos, registrando-se os dados sociodemográficos, clínicos e laboratoriais, incluindo-se alterações hepatobiliares, exames de imagem, estudos genéticos, biópsias hepáticas e endoscopias digestivas altas. Resultados: A doença hepatobiliar foi diagnosticada em 16,4% dos pacientes e ocorreu como manifestação inicial da fibrose cística em 55,6% destes casos. O diagnóstico da hepatopatia ocorreu antes ou concomitante ao diagnóstico da fibrose cística em 88,9% das crianças. Todos os pacientes com doença hepatobiliar foram considerados não brancos, havendo predominância do sexo feminino (77,8%) e mediana (I.I.Q) de idade de 54 (27-91) meses. Em comparação com o grupo sem doença hepatobiliar, as crianças com hepatopatia tiveram maior frequência de mutações graves no gene CFTR identificadas (77,8% vs 39,6%; p = 0,033) e de insuficiência pancreática grave (88,9% vs 31,6%; p = 0,007). Conclusão: A frequência de doença hepatobiliar foi elevada, observando-se um diagnóstico muito precoce da mesma e de suas complicações na casuística estudada. Houve associação estatística entre a ocorrência de doença hepatobiliar e a presença de insuficiência pancreática e de mutações graves do gene CFTR. Enfatiza-se que a fibrose cística represente um importante diagnóstico diferencial de hepatopatias na infância.

Hematoma subcapsular hepático roto en síndrome HELLP / Ruptured subscapular liver hematoma in context of HELLP syndrome

Background: Ruptured subscapular liver hematoma in context of HELLP syndrome is a rare complication with high mortality. Clinical presentation is nonspecific: epigastric pain, right upper quadrant pain and shoulder pain, should however provide high index of suspicion. Termination of pregnancy is the only definitive treatment with a beneficial effect on the mother and the fetus. According to the hemodynamic status of the patient, treatment can be conservative through hepatic artery embolization, or surgical management, via contained packing of the bleeding area. Aim: To report seven cases of ruptured subscapular liver hematomas treated in a period of 13 years. Material and Methods: Review of medical records of patients discharged with the diagnosis from 2002 to 2015. Results: We identified seven women aged 30 to 44 years, for a total of 86,858 live births. At presentation, mean gestational age was 36 weeks. Five patients reported epigastric pain and three shoulder pain. All patients met the criteria for HELPP syndrome. All patients were operated on, and packing of the bleeding zone was carried out in six. One patient died. Two newborns died. Conclusions: The features of these patients may help in the management of this uncommon but severe condition.

Chronic hepatothorax due to right diaphragmatic rupture: an anesthetic challenge in a rare case / Hepatotórax crônico devido à ruptura do diafragma direito: um desafio anestésico em um caso raro

Abstract Background: Diaphragmatic rupture is an uncommon condition, with 90% of ruptures occurring on the left side. However, its incidence on the right side is increasing along with the increase in traffic accidents. Liver herniation may become progressive causing severe atelectasis of the right lung, resulting in impaired respiratory status and hemodynamic changes. Case report: We report the case of a 40 years old female, ASA III, scheduled for hepatothorax repair that evolved from right diaphragmatic hernia after a car accident when she was 8 years old. Clinically, she had severe restrictive respiratory syndrome caused by the hepatothorax. The anesthetic evaluation was normal, except for the chest X-ray showing elevation of the dome of the right hemidiaphragm without tracheal deviation. Diagnosis was confirmed by CT scan. After liver replacement in the abdominal cavity, a transient increase in central venous pressure, stroke volume index and flow time corrected (35%), and a decrease in systemic vascular resistance were observed. After complete hemodynamic and hepatosplenic stabilization, as well as ventilation, the patient was transferred intubated, under controlled ventilation and monitored, to the liver transplant unit. Conclusions: Hepatothorax is a rare condition and its repair may represent an anesthetic challenge. After liver replacement in the abdominal cavity during corrective surgery under general anesthesia complications may occur, particularly associated with pulmonary re-expansion. Effective teamwork and careful planning of surgery, between the surgical and anesthetic teams, are the key to success.

Resumo Justificativa: A ruptura diafragmática é uma condição incomum e ocorre em 90% no lado esquerdo. No entanto, a incidência de ruptura à direita tem vindo a aumentar junto com o aumento dos acidentes automobilísticos. A herniação do fígado pode tornar-se progressiva, causar atelectasia grave do pulmão direito, resultar num débil estado respiratório e alterações hemodinâmicas. Relato de caso: Mulher de 40 anos, estado físico ASA 3, marcada para reparação de hepatotórax que evoluiu de hérnia diafragmática direita, adquirida aos 8 anos, após um acidente automobilístico. Clinicamente apresentava síndrome respiratória restritiva grave, causada pelo hepatotórax. A avaliação anestésica era normal, com exceção da radiografia do tórax, que evidenciava elevação da hemicúpula diafragmática direita, sem desvio traqueal. Diagnóstico foi confirmado por tomografia computadorizada. Depois da recolocação do fígado na cavidade abdominal foram observados um aumento transitório da pressão venosa central, do Stroke Volume Index e Flow Time Corrected (35%) e uma diminuição da resistência vascular sistêmica. Uma vez alcançada a estabilização hemodinâmica geral e hepatoesplênica, bem como da ventilação, a paciente foi transferida entubada, sob ventilação controlada e monitorada para a Unidade de Transplantação Hepática. Conclusões: O hepatotórax é uma condição rara e a sua correção pode representar um desafio anestésico. Após a recolocação abdominal do fígado, durante uma cirurgia corretiva, sob anestesia geral, podem ocorrer complicações, principalmente as associadas à reexpansão pulmonar. Um trabalho em equipe eficaz e o planejamento cuidadoso da cirurgia, entre as equipes cirúrgica e anestésica, são a chave para o sucesso.

El análisis de orina como herramienta diagnóstica en casos de malaria grave / Urinalysis as a diagnostic tool in severe malaria

Resumen La malaria produce complicaciones y muerte especialmente en poblaciones con acceso limitado a la atención en salud. La malaria grave puede reconocerse tempranamente mediante la detección en la orina de hallazgos como la hematuria, la coluria y la proteinuria. Se hizo una revisión narrativa basada en estudios sobre malaria grave y el empleo del análisis de orina mediante la consulta de 91 publicaciones. Mediante el análisis de la orina, se pueden detectar alteraciones metabólicas y lesiones en distintos órganos. En estudios recientes en Colombia se ha confirmado su utilidad como apoyo en el diagnóstico de la disfunción renal, la disfunción hepática y la anemia asociada con hemólisis, las cuales son complicaciones frecuentes en la malaria. El examen constituye una herramienta de fácil aplicación en la consulta ambulatoria y en pacientes hospitalizados para reconocer tempranamente casos complicados, y permite la detección oportuna de diferentes lesiones en el paciente con malaria, contribuyendo así a la reducción de la morbilidad grave y la mortalidad.

Abstract Malaria accounts for a significant morbidity and mortality rate around the world, especially in communities with limited access to healthcare. Some clinical signs in urine, like haematuria, coluria and proteinuria, help for the early diagnosis of severe malaria cases. A narrative review was conducted by analyzing 91 publications on studies about severe malaria cases and the use of urinalysis. A urinalysis can detect metabolic disturbances and organ injury. Its diagnostic utility for frequent complications caused by malaria, such as hepatic injury, kidney dysfunction and hemolysis, has been confirmed by recent Colombian studies. This test is an easy-to-use tool in outpatient clinics and with hospitalized patients to promptly recognize complicated cases, allowing the timely identification of different lesions in patients with malaria, thus contributing to the reduction of severe morbidity and mortality.

Mucopolisacaridosis (síndrome de Hunter) / Mucopolysaccharidosis (Hunter syndrome)

Se realiza una presentación de un caso interesante, no comúnmente visto en la práctica médica, de unos de los tipos de mucopolisacaridosis, específicamente de un síndrome de Hunter. Se hace esta presentación con el objetivo de dar a conocer a estudiantes y profesionales de la salud, mediante fotos, las características físicas del paciente con dicho sídrome, quien llegó desnutrido al hospital; se le operó de la hernia umbilical y se mejoró su estado nutricional al compensarse su hepatopatía. Se le da el alta médica en mejores condiciones(AU)

We present here an interesting case of mucopolysaccharidoses, which is not commonly seen in medical practice, specifically a Hunter syndrome. This presentation is done in order to make known to students and health professionals, through photos, the physical characteristics of the patient with such syndrome. This patient arrived malnourished at the hospital, he was operated on the umbilical hernia and improved his nutritional status by compensating for his liver disease. This patient had medical discharge in better conditions(AU)

Hematoma hepático asociada a eclampsia y síndrome de Hellp / Liver hematoma associated with eclampsia and HELLP syndrome

El hematoma subcapsular es una complicación grave elevado riesgo de mortalidad materna y neonatal, se observa como complicación de patologías hipertensivas más frecuentemente, además de otras patologías graves como hígado graso agudo del embarazo, traumatismos, patologías infecciosas. Se instala durante el embarazo o post parto, con manifestaciones clínicas inespecíficas, con grave y rápido deterioro materno que de no diagnosticar y tratar en tiempo y forma. Produce muerte materna en un 75% y fetal 60%. Se presenta un caso de hematoma hepático como complicación de una eclampsia y síndrome de Hellp, que evolucionó en forma favorable. Tratada con electrocoagulación el cual usamos para tratamiento de la hemorragia.

The subcapsular hematoma is a serious complication, with high risk of maternal and neonatal mortality, it is seen as a complication of hypertensive diseases more frequently, busides another serious diseases such as acute fatty liver of pregnancy, trauma, infectious diseases. It is onset occurs during pregnancy or postpartum, with nonspecific clinical manifestations, with severe and rapid material deterioration which of not diagnosed and treated from the beginning could produce maternal death in 75% and fetal 60%. We present a case of liver hematoma as a complication of eclampsia and Hellp syndrome, whose evolution was favorable, treated with electrocoagulation which we use for the treatment of hemorrhage.

Etiology and epidemiology of patients presenting with liver disease during pregnancy

We conducted this study to elaborate the etiology and epidemiology of liver diseases presenting during pregnancy. It was a prospective, observational study. This study was conducted in the Department of Gastroenterology, Fatima Memorial Hospital in collaboration with the Department of Obstetrics and Gynaecology from June 2011 -May 2013. 73 patients were evaluated. Data was evaluated for quantitative and qualitative variables. Outcome of mother, pregnancy and neonates was also recorded were available. During the study period 73 pregnant patients were evaluated for the presence of liver disease giving an incidence of 3.6%. The mean age of the patients was 26.3 +/- 3.8 [median: 26, range: 16 - 45] years. The patients presented at a mean gestational age of 5.8 +/- 2.4 [median: 6, range: 1- 9] months. The mean STB and ALT levelswere 11.5 +/- 8.5 [median: 9.3, range: 1.4 - 48] mg/dL and 943.5 +/- 887.4 [median: 765, range: 13 - 4810] IU/Lrespectively. The mean duration of jaundice and mean hospital stay were 6.82 +/- 5.32 [median: 5, range: 2 - 30] days and 5.6 +/- 2.7 [median: 5, range: 1-18] days. Fulminant hepatic failure was seen in 6.8% of the patients. Acute Hepatitis E was the most frequent diagnosis in our population. Maternal deaths were seen in 8.2%, foetal deaths were seen in 12.3% and neonatal deaths in 6.5%. Liver diseases during pregnancy can have multiple causes and may predict poor outcomes for the patients and neonates

Personalized treatment of hepatitis B

There are seven approved drugs for treatment of hepatitis B. Professional guidelines provide a framework for managing patients but these guidelines should be interpreted in the context of the individual patient's clinical and social circumstances. Personalized management of hepatitis B can be applied based on prediction of the individual patient's risk of cirrhosis and hepatocellular carcinoma to guide the frequency and intensity of monitoring and urgency of treatment. It can also be applied to decisions regarding when to start treatment, which drug to use, and when to stop based on the individual patient's disease characteristics, preference, comorbidities and other mitigating circumstances.

Diagnostic evaluation of hepatopulmonary syndrome in egyptian children with chronic liver disease

There are few data on prevalence of hepatopulmonary syndrome [HPS] in children with chronic liver disease [CLD]. This prospective study evaluated the prevalence and diagnostic procedures of HPS in Egyptian children with CLD. One hundred twenty [120] children with CLD were subjected to room-air pulse oximetry in supine and upright position, contrast enhanced echocardiography [CEE] and technetium-99m-labeled macroaggregated albumin [99m]Tc-MAA] perfusion lung scan. Arterial blood gas [ABG] analysis in upright position was performed for all children with identified intrapulmonary vascular dilatation [IPVD]. Clinical, laboratory, imaging and endoscopic data were recorded and analyzed. Hypoxemia was found in 14 cases [11.7%] of the total cohort all of them had IPVD, whereas 6 cases [5%] of the patients had EPVD without hypoxemia. Therefore, HPS and subclinical HPS were diagnosed in 11.7% and 5% of CLD patients, respectively. Only 10 HPS patients had a pathological arterial oxygen saturation [SaOi] in the supine position [<97%] but all showed a pathological SaO[2] decrease [>4%] after changing from supine to upright position.[99m]Tc-MAA perfusion lung scan revealed IPVD in 16.7% whereas CEE detected IPVD in 10% only of enrolled patients. There were strong correlations between shunt index estimated by lung scintiscan and oxygenation parameters in HPS patients. The characteristics of HPS patients were similar to that of non-HPS patients except for clubbing, dyspnea, cyanosis, orthodoexia and bleeding varices that were more associated with HPS patients as well as well as the Child-Pugh grades, which tended toward higher scores in HPS patients