[Therapeutic options for portal hypertensive biliopathy: case series and literature review]. / Opciones terapéuticas de la biliopatía por hipertensión portal: serie de casos y revisión de la bibliografía.

BACKGROUND: Portal hypertensive biliopathy is an underdiagnosed condition because only some patients have symptoms. The major clinical manifestations include cholestasis and cholangitis. The aim of this study is to present a series of cases evaluated, treated and followed at a tertiary-care public institution. CLINICAL CASE: Four patients with portal hypertensive biliopathy were exposed to different therapeutic approaches focused on the management of portal hypertension and biliary decompression. They were followed for ~5 years. Three cases achieved a favorable outcome with symptom remission, but one patient died while attempting dilatation of the bile duct. Finally, we carried out a literature review about actual portal hypertensive biliopathy therapeutics. CONCLUSIONS: There is currently no consensus on the optimal treatment for this condition. The goal is to decompress the biliary tree. Each case should be individually evaluated to choose the best treatment option.

Antecedentes: la biliopatía por hipertensión portal es poco diagnosticada debido a que sólo algunos pacientes experimentan síntomas. Las manifestaciones clínicas más importantes son la colestasis y la colangitis. Objetivo: comunicar una serie de casos evaluados, tratados y seguidos en una institución pública de tercer nivel. Casos clínicos: cuatro pacientes con biliopatía por hipertensión portal se expusieron a diferentes métodos para tratar la hipertensión portal y la descompresión de la vía biliar. Se realizó seguimiento durante casi cinco años. Tres casos mostraron adecuada evolución, con remisión de los síntomas; un paciente falleció al intentar dilatarle la vía biliar. Finalmente, se revisa la bibliografía en relación con la terapéutica de la biliopatía por hipertensión portal. Conclusiones: no existe consenso para el tratamiento óptimo de este padecimiento, aunque el objetivo es descomprimir la vía biliar; cada caso plantea particularidades que guían el tratamiento.

Persistent hepatic encephalopathy secondary to portosystemic shunt occluded with Amplatzer device.

Hepatic encephalopathy is a frequent complication of cirrhosis, when this event becomes persistent, treatment compliance should be verified and any precipitating factor need to be identified. Also the presence of portosystemic shunts, which are a rare cause of decompensation or persistence hepatic encephalopathy need to be ruled out. In this paper we report the case of a 57 year old man with persistent hepatic encephalopathy secondary to the presence of a porto-onfalo-femoral shunt successfully closed with the placement of an Amplatzer device.

Granulomatous hepatitis caused by Q fever: a differential diagnosis of fever of unknown origin.

The differential diagnosis of fever of unknown origin (FUO) includes infectious, neoplastic, rheumaticinflammatory and miscellaneous diseases. We report the case of a 35-year-old man with FUO caused by Q fever. A liver biopsy showed the characteristic fibrin-ring lipogranulomas compatible with Q fever. The serologic tests confirmed the diagnosis of acute infection by Coxiella burnetii. The therapeutic response was excellent. In conclusion, we described a patient with acute Q fever and granulomatous hepatitis.

[Primary sclerosing cholangitis presenting as recurrent cholangitis and right hepatic duct outpouching]. / Quiste de la vía biliar y colangitis de repetición como manifestaciones iniciales de colangitis esclerosante primaria.

Primary sclerosing cholangitis (PSC) may have an atypical mode of presentation with recurrent cholangitis and diverticulum-like outpouchings of the hepatic ducts; a high clinical suspicion is required to confirm the diagnosis instead of ascribing cirrhosis to a secondary cause as a result of the biliary stasis propitiated by the biliary cysts. We describe the case of a 63-year old woman with a one-year history of episodes of cholangitis and a persistent elevation of alkaline phosphatase. The endoscopic retrograde cholangiopancreatography and a magnetic resonance cholangiography showed strictures of the intrahepatic biliary tract compatible with PSC and a diverticulum-like outpouching of the right hepatic duct. A liver biopsy revealed cirrhosis. Initial management consisted of antibiotics, a sphincterotomy and the placement of a biliary plastic stent, however, the patient continued to have recurrent cholangitis and finally, the cyst was resected.

Liver failure after an uncovered TIPS procedure associated with hepatic infarction.

Transjugular intrahepatic portosystemic shunt (TIPS) is a safe and effective procedure for the treatment of complications of liver cirrhosis, such as refractory ascites, hepatic hydrothorax and refractory variceal bleeding. The aim of this paper is to describe a rare case of liver failure after a TIPS procedure. A 38-year-old diabetic male with Child-Pugh C liver cirrhosis due to chronic hepatitis C infection who had developed refractory ascites was scheduled for a TIPS procedure. Within 24 h following TIPS placement, the patient developed distributive shock, jaundice, persistentgrade 3 hepatic encephalopathy, severe coagulopathy and acute renal failure. He was treated with lactulose enemas, broad-spectrum antibiotics and blood-derived products. Laboratory data revealed a 100-fold increase in aminotransferases and a non-enhanced computed tomography showed an irregular hypodense area in the right posterior segment of the liver. Despite being initially being in a stable condition, the patient developed progressive liver failure and died 2 mo later. Hepatic infarction is an uncommon phenomenon after a TIPS procedure; however, it can greatly complicate the course of a disease in a patient with an already compromised liver function.

[Renal hemodynamics and its correlation with the Child-Pugh stage in cirrhotic patients and their controls]. / Hemodinámica renal y su correlación con el estadio de Child-Pugh en pacientes cirróticos y controles sanos.

INTRODUCTION: Portal hypertension is associated with splanchnic vasodilatation and baroreceptors activation with secondary renal vasoconstriction. OBJECTIVE: To measure the pulsatility and resistance index in the renal arteries: Segmentaries and arcuates of both kidneys in cirrhotic patients Child-Pugh A, B, C classes and compared them with healthy controls. PATIENTS AND METHODS: Thirty patients with cirrhosis were included: Ten patients Child A, 10 were Child B and 10 Child C class and 10 healthy patients, we measured the resistance index (RI = [systolic peak velocity-minimum diastolic velocity]/systolic peak velocity) and the pulsatility index (PI = [systolic peak velocity-minimum diastolic velocity]/medium velocity) with Doppler spectral analysis. RESULTS: The RI in controls had an average of 0.55 +/- 0.11. In child A: 0.79 +/- 0.114, in Child B 0.77 +/- 0.104 and in Child C 0.85 +/- 0.037. For the pulsatility index the values were: 0.98 +/- 0.21, 1.4 +/- 0.105, 1.72 +/- 0.206, and 2.04 +/- 0.346, respectively. The resistances and pulsatility index were higher in patients with cirrhosis at the time to be compared with healthy controls, which represent a diminishment in blood flow. At the time to compare each one of the Child stages with controls, both index resulted higher in cirrhotic patients, but when we compared the cirrhotic patients between them, only difference for RI in child B vs. C was evident. The differences in PI were evident between each one of the Child classes. DISCUSSION: The present study proved that a non invasive method like Doppler ultrasound is useful in the evaluation of the renal hemodynamic changes in cirrhotic patients with a good correlation between Child class and IP.

Early experience of Budd-Chiari syndrome treatment with transjugular intrahepatic portosystemic shunt.

The Budd-Chiari syndrome is a heterogeneous group of disorders characterized by obstruction of hepatic venous outflow at any level from the small hepatic veins to the junction of the inferior vena cava with the right atrium. We present two cases of Budd- Chiari syndrome with severe ascites associated with polycythemia vera in first case and protein C deficiency in the second, in both cases transjugular intrahepatic portosystemic shunt were placed, with excellent control of symptoms, no mortality were observed, and just one episode of pulmonary venous thrombosis was observed. To our knowledge this is the first time that transjugular intrahepatic portosystemic shunt are used and reported in Budd-Chiari syndrome in Mexico.

Pregnancy and portal hypertension a pathology view of physiologic changes.

The coexistent of pregnancy and liver disease represent a complex clinical situation, besides the liver complications that present in pregnancy with a previous health liver, like intrahepatic cholestasis of pregnancy, acute fatty liver of pregnancy or HELLP syndrome with bleeding disorders and viral hepatitis, the previous liver damage with portal hypertension associated represent a clear stated of hemodynamic changes which increased risk of variceal bleeding. The portal hypertension syndrome has a splanchnic blood flow increase. During pregnancy an hypervolemic stated developed as consequence there is an increased in portal flow that contributed to more portal pressure transmitted to the collaterals veins which increase variceal bleeding risk in this group of patients. The present review will focus on treatment options to prevent variceal bleeding in this clinical situation.

Beta blockers in portal hypertension. Are they really a good option?

Non-selective beta blockers are very useful drugs in preventing first variceal bleeding and re-bleeding in patients with cirrhosis. These drugs work in two ways: 1) by blocking beta1 receptors and reducing cardiac output, and 2) by blocking beta2 receptors, producing splanchnic vasoconstriction and reducing portal flow. Consequently, they reduce portal pressure. In primary prophylaxis, beta blockers reduced the bleeding risk from 30 to 15%; in secondary prophylaxis, this risk decreased from 60 to 42% in the first year. Heart rate decrease does not necessary correlate with reduction in hepatic venous pressure gradient (HVPG). When this gradient is reduced to less than 12 mmHg, the patient will not bleed; when this is reduced > 20% from basal values bleeding risk is extremely low, estimated at 9% at 2 years. The only way to know whether the patient has become a responder is to measure the HVPG. Additionally, by means of this method we also can identify the non-responders, who have a higher rate of re-bleeding, between 54 and 64%, and can attempt to utilize a more aggressive therapy, such as adding isosorbide mononitrate to the beta blocker or combining the beta blocker with endoscopic ligation. These options are discussed in the present review.

A 57 year old man with chronic renal failure and cardiac tamponade who developed ischemic hepatitis.

Ischemic hepatitis is an infrequent entity, usually associated with low cardiac out put. We present a case of a 57 year-old man with chronic renal failure and cardiac tamponade who developed elevation of serum alanine transferase level of 5,054 U/L, aspartate transferase level of 8,747 U/L and lactate dehydrogenasa level of 15,220 U/L. The patient developed hepatic encephalopathy and hypoglycemia. Liver Doppler ultrasound was normal. He was seronegative for HBV and HCV, drugs list was scrutinized for the names of known hepatotoxins. Ischemic hepatitis was diagnosed. The hypoglycemia and encephalopathy were solved and the patient was discharged with normal transaminase levels. Ischemic hepatitis is typically preceded by hypotension, hypoxemia, or both. As one would expect, the most common cause of sustained systemic hypotension is cardiovascular disease. Liver biopsy is usually not necessary. The best treatment is support measures and correct the underlying condition.

Biliary stricture caused by portal biliopathy: case report and literature review.

Portal biliopathy is a rare condition that is usually not diagnosed and only in few cases causes symptoms. Those symptoms are caused by vascular obstruction of the biliary tree in patients with portal hypertension. We report a case of a 29 years man who presented with history of intermittent jaundice, persistent elevation of hepatic function test and hematemesis as a manifestation of portal hypertension without liver damage. We present the clinical, radiological and pathological characteristics and literature review of the cases that had been reported, their diagnoses, treatment and clinical implication.