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Our Diseases

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2. Our diseases

The liver vessels

The liver is the largest and most important organ of the body with the particular function of permanently processing the blood coming from the digestive system in order to adapt it to the needs of other organs. Like any other organ, it has an artery (input) and hepatic veins (output). But, and this is unique in the whole (of the) body, the liver also has a vein which penetrates it, coming from the intestine, the stomach and the spleen; it is the portal vein.

The total blood mass going to and from the liver represents a quarter of the overall blood mass that the heart pumps, (of which a third by the hepatic artery and two thirds by the portal vein).

 

Attacks of the vessel

The main problems are obstruction of the hepatic veins (Budd Chiari or portal cavernoma) .

In some cases, the obstruction results from a compression due to a nearby injury. Most of the time, the incident is said to be "primitive": thrombosis (blood clot), triggering a phlebitis (venous inflammation).

Thrombosis occurs when blood tends to clot (Prothrombic state) .

Some conditions are inherited, i.e.: like factor V Leiden, factor 2 mutation, shortage of proteins C and S or antithrombin .

Other conditions can appear throughout life, like: myeloproliferative and antiphospholipidic syndromes, nocturnal paroxystic hemoglobinuria, oral contraceptives or pregnancy.

Generally, thrombosis is due to a combination of several factors.

The reasons why thrombosis can occur in the hepatic or portal veins and not elsewhere in the body are still unknown.

 

Symptoms of our diseases

At the beginning of the thrombosis, patients may have no signs or very common ones.

In some instances, the signs may be very serious:

- extreme fatigue,
- pains, fever,
- abdominal swelling caused by liquid (ascites).
- All intermediate stages are possible.

The initial signs can spontaneously, reduce, disappear or become worse.

Later, the signs reflect the inconvenience of the blood flow in the liver vessels (portal hypertension): abdominal varicose veins, some of which -the oesophageal varicosis- can be the origin of a digestive haemorrhage.

In case of hepatic veins thrombosis, chronic ascites, jaundice, mental confusion, infections will confirm the hepatic functions are affected.

In case of a portal thrombosis, its extension can cause a severe intestine pain.

Nevertheless, even after a very acute start, the effect on the vessels can remain totally invisible. The body has, indeed, some very efficient means to compensate.

 

EARLY DIAGNOSIS

Early diagnosis can make a difference in our lives.

Quick identification of a vascular disease gives the best chance to identify the causes and then to stop the pernicious impacts to the liver or the intestine;
— to anticipate the extension of the thrombosis to other non-affected veins and even,
— to obtain the regression of certain thrombosis through treatments.

90% of the cases can be diagnosed by non invasive exams (Doppler ultrasound, scan or MRI) which allow the veins to be observed. The results of these exams are all the more trustworthy when the radiologist is well experienced and has been made aware by the physician of the hypothesis of a vascular risk.

Once the diagnosis of the liver vascular disease has been established, specific investigations will be performed by an haematologist to identify the prothrombic status.

 

Treatments

Treatments exist:

They require the cooperation of experts in several disciplines: specialized labs, hematologists, radiologists, hepatic surgeons, anesthetists, resuscitation specialists and of course hepatologists.

The first step is to:

(a) assess the specific treatment of the causes when they are known;
(b) to administer anticoagulants;
(c) to treat severe manifestations (ascites, kidney failure, infections, digestive hemorrhage)

Nowadays, these treatments are well recognised in liver and gastroenterology units.

The second step is to make the thrombosed veins patent again, once and for all (angioplasty of the hepatic veins or of the vena cava).

In case it fails, the third option is to make a derivation between the portal and the cava systems. This is currently what is done using TIPS. (This technique is far less invasive than surgery).

In case this fails as well, a hepatic transplant must be envisaged.

Thanks to these different treatments, there have been constant improvements in the last thirty years.

Some diseases which were potentially fatal after a few months or years have now become non visible in 85 % of the cases. This is the result of continuous treatment and regular checkups.

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BUDD CHIARI SYNDROME

DEFINITION

It is a liver disease caused by the obstruction of hepatic veins.   It is a rare disorder.  

Symptoms
 

Manifestations may vary a lot:                                                           

-  no symptoms,  fatigue, abdominal pain, progressive increase of the volume of the abdomen,leg oedema.

Less frequently:

-  Gastro intestinal bleeding, acute hepatitis, jaundice, ascites.                                                                               

 Diagnosis

Radiology procedures such as Doppler hepatic ultrasonography, Hepatic Magnetic Resonance

Imaging (MRI) and hepatic computerised tomography usually visualize hepatic veins.                                                                            

 Causal Factors

The combination of several predisposing risk factors is often necessary. The main one is a blood disorder: myeloproliferative disorder (overproduction of white or red blood-cells or of platelets). This disease is acquired, it is not transmitted by parents at birth.

Other predisposing risk factors are congenital anomalies. The main anomalies are the mutation of factorV Leiden (prothrombin gene mutation) and natural anticoagulant deficiency (protein C, protein S, antithrombin).

Other risk factors are exogenous: oral contraceptives containing oestrogen, pregnancy.

Diagnosis fo the causes       

A blood test is performed to make a diagnosis.
In the case of myeloproliferative disorder, thorough tests such as osteo-medullary biopsy or blood volume measurement may also be necessary
 
 Treatment
 

 Anticoagulation therapy:

It fluidifies the blood and thus helps to prevent the formation of new thrombosis at risk of new ovein obstruction. There are two types of anticoagulants: those which are taken orally and those which are given by subcutaneous injections.

Posology must be strictly adapted to avoid two risks: insufficient anticoagulation (risk of thrombosis ) and   excessive anticoagulation (risk of bleeding). The oral treatment is monitored by regular blood tests to measure your INR.

Thrombolysis :

It is an injectable pharmacologic therapy which is mainly used locally on the site of the thrombus so as to  disolve it. This therapy is only used in the case of recent thrombosis.

Angioplasty and insertion of a STENT:

Such procedure is used to widen (angioplasty) a narrowed vein (“stenosed”) and to place a metal scaffold (stent) at the site of dilation to prevent restenosis.

Transjugular intrahepatic portal-systemic shunt (TIPS):

This procedureconsists in creating a bypass between the portal vein in the liver and the hepatic veins or the inferior vena cava.

Transplantation: Transplantation is performed when  angioplasty or TIPS have failed.

The above mentioned  procedures are performed one after the other, as needed, starting from the simplest and less invasive: anticoagulants, angioplasty and placement of a stent, then TIPS, and finallyliver transplantation. Going on to the next step will depend on the response to the previous treatment (if it was technically feasible).

Side effects               

Complications of Budd Chiari syndrome can be due to the disease itself (apparition of ascites, varices bleeding, liver failure, infection…).
Other side-effects may result from some treatments (anticoagulant-related bleeding, complications after angioplasty or TIPS).
 

Recurrences 

Recurrences are unlikely to happen if the anticoagulant drugs are taken as prescribed, the treatment well-monitored, if the cause of the disease is treated. To avoid the recurrence of thrombosis, it is thus essential to take medications as directed. If any problems should arise, speak about them to your practitioner without delay.

Prognosis

The first two years can be difficult and specialised treatments are required. After this period, liver condition ceases to be a problem for 80% of the patients. Future treatments will focuse on the cause of the disorder. A near-normal family and professional life is absolutly possible. 

Source : Hepatic Vascular Disorder Reference Centre . Hepatology Unit Beaujon Hospital.

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For a better knowledge of the illnesses of the blood vessels of the Liver  

From the Medical Service to Everyday Life

The Blood Vessels of the Liver, the Blood Vessels of Life 

The liver plays a major part in permanently overhauling the blood, and in particular the blood coming from the digestive tract, so as to adapt it to the needs of the other organs. To do so, as for any other organ, it disposes of an artery (which enters the liver), and of hepatic veins (which leave the liver). However, as a unique exception in the organism, the liver also disposes of a vein which enters it (from the intestine, from the stomach and from the spleen): this is the portal vein. The total quantity of blood reaching the liver (and leaving it) represents at any given time one quarter of the amount which is pumped by the heart. Of this quantity, one third comes by the hepatic artery and two thirds by the portal vein.

  The Assault on our Blood Vessels

The main damage is the obstruction of the hepatic vein (Budd-Chiari syndrome) or of the portal vein (pylephlebitis or cavernoma of the portal vein). In certain cases, the obstruction is subordinate to a compression due to a close injury. More often than not, the impairment is called primary: thrombosis (formation of a blood clot) triggering off a phlebitis (inflammation of the vein). This thrombosis happens in a situation where there is a tendency for an exces of blood to coagulate, or in a prothrombotic state. Certain states are hereditary: facteur V Leiden, mutation of factor 2, or a deficiency of protein C, of protein S or of anti thrombin. Others are acquired during the course of a lifetime: myeloproliferative disorder, antiphospholipid syndrome, paroxysmal nocturnal hemoglobinuria (PNH), oral contraception, or pregnancy.

 As a general rule, it is necessary for there to be a combination of several causes. The reasons for which a thrombosis occurs in the hepatic vein, or the portal vein, and not elsewhere in the organism, are still not clarified.

Signs of our illnesses

 When a thrombosis is taking place, the signs can be very different: either there are no signs, or they can be commonplace; or they can be quite outstanding like an extreme fatigue, pains, temperature, abdominal swelling due to liquid (ascites), or jaundice. Any or all of these symptoms may occur. The initial signs may lessen or disappear or worsen. Later, these signs show the difficulty in the bloodflow through the hepatic vessels (portal hypertension): numerous various parts in the abdomen of which some, the esophageal varices, may be the cause of a digestive haemorrhage. In the case of a thrombosis of the hepatic veins, a chronic ascites may set in as well as signs showing that certain functions of the liver have been affected (jaundice, sensitivity to bacterial infections, cerebral functions impaired, decrease of coagulation factors, and blood albumin). In the case of portal thrombosis, spreading of the thrombosis can cause serious intestinal pain. However, even if the onset was very pronounced, damage to the blood vessels may go completely unnoticed. Indeed, the organism disposes of very efficient means of compensation.

An early diagnosis is decisive for our future

 An early recognition of a vascular injury enables us: 

  • To property identify the causes, which will be treated ;
  • To put a stop to any deleterious consequences for the  liver or for the intestine ;
  • To avoid the spreading of the thrombosis to the undamaged reins ;
  • Or even to obtain a regression of certain thrombosis by the treatment. 

In 90% of cases, a diagnosis is obtained by non-agressive tests (Doppler ultrasound, Scanner or MRI) which give a view of the vessels. The quality of the tests is increased by having an experienced radiologist who is informed by the doctor of a possible vascular injury. Once the diagnosis of a hepatic vascular injury has been made, often an opinion should be given by a hemotologist who is familiar with the particular nature of hepatic thrombosis or portal vein thrombosis in order to identify the pro-thrombotic state by means of the appropriate examination.

  Existing treatments

 Cooperation of experts of many disciplines is necessary: specialised laboratories, hematologists, radiologists, hepatic surgeons, anaesthetists, resuscitators, and of course hepatologists.
 The first stage is to introduce the specific treatment for any existing causes; to administer anticoagulants; to treat severe symptoms (acites, renal insufficiency, infections, and digestive haemorrhage). These treatments are codified today in the department of the Digestive Surgery Department (Services d’hépato-gastroentérologie).
 The second stage is to consider restoring permanently the permeability of the vessels affected by thrombosis (percutaneous angioplasty of the hepatic veins or of the vena cava).
 In case of failure, the third stage, for the hepatic veins, consists of creating a derivation between the portal system and the cava system which short-circuits the obstacle of the hepatic veins. At present this technique of TIPS is used (thus avoiding any surgery).
 In case of a new set-back, a liver transplant must be considered. The evolution has been transformed by these different treatments since, over 30 years of constant improvement, the picture of potentially lethal illnesses after a few months or years has changed to illnesses  which, in 85% of cases, have become inconspicuous, thanks to continuous treatment and regular supervision.
 

  Downtown Reference Center for Liver Diseases

 This is a multidisciplinary team which includes hepatologists, radiologists, surgeons, hematologists, and anatomical pathologists. This team has a particular experience in several fields : 

  • The medical care of the patients suffering from an illness of the hepatic vessels.
  • Research on the diagnostic and therapeutic techniques, and the identification of the causes. Laboratory research on animals regarding liver diseases, or on the taking of blood samples or biopsies of patients.

 The Center’s mission is to facilitate patient care as close as possible to their home, and at the highest standards, by offering advice and logistical or technical help to the local doctors. The team also has a mission to increase its knowledge regarding these illnesses through its work on research, and to disseminate such information.

 The Center can be contacted at Hôpital BEAUJON, Clichy (tél : +331 40 87 51 60)

Numerous problems to be overcome

 Immediate medical requirements are to : 

  • Identify the factors of thrombosis yet unknown.
  • Have simpler tests at our disposal to identify the factors known more particularly the myeloproliferative disorder.
  • Have at our disposal anticoagulants more easy to handle by a greater interval between the therapeutic effect and overdose.
  • Have at our disposal more efficient treatments which are better tolerated for certain causes, notably for blood disorders.
  • Be able to judge more accurately the perfect time for the various therapeutic procedures (sooner or later).
  • Be able to raise the level of successes and diminish the complications due to therapeutic procedures (angioplasty and TIPS).

 

Regardingthe patients’ problems 

  • Assure a medical follow-up wherever we live.
  • Relieve the patients (suffering caused by ascites) and support them.
  • Make available a more humane and modern hospitalisation which respects both the patient and relatives.
  • Increase the information to be received by patients and their families.
  • Take into account the geographical distances between the specialised medical centers.
  • Reduce our difficulties in continuing our professional activities.
  • Take into account the impact that the illness has on the families of the diseased.
  • Improve any financial aid and reduce the reimbursement period.

 

Regarding the development of medical research 

  • Understand why a thrombosis arises in the hepatic veins or the portal vein, and not elsewhere in the organism.
  • Understand by which mechanisms certain blood diseases lead to a thrombosis.
  • Understand how the irreversible after-effects (or sequelae) of thrombosis arise, and how to prevent them.
  • Understand how ascites arise during the Budd-Chiari syndrome.
  • Understand how the naturel mechanisms, for compensation after a veinal thrombosis develop, so as to be able to stimulate them.
  • Understand what part the hepatic vascular obstructions play in the aggravation of frequent liver diseases, like cirrhosis of the liver due to a viral hepatitis or to an intake of alcohol. 

 

Our Association 

Created in October of 2005, AMVF has its Head Office at Hôpital BEAUJON, Department of Hepatology, 100 bd du Général LECLERC, CLICHY 92110.

 The Association gathers together the sicks, their families and friends confronted by their illnesses. Its objectives are :

 To increase the knowledge of these diseases amongst the public concerned (doctors, the sick, the general public, government representatives and the public authorities).

  • To create a bond between the patients to avoid loneliness, to inform them and to help and support them.
  • To promote medical research to fight against the disease of liver vessels and to promote the creation of reference centers on these patholologies.
  • To assure the financial support of the specific characteristics of the illnesses of the vessels of the liver, and to develop it.
  • To reenforce the link between patients and medical practitioners, to insure a better all-round patient care. 

 

To become a member of the Association means: 

  • To allow the Association to expand in terms of numbers, and thus be recognised by the public authorities and the medical profession.
  • To participate in the accomplishment of our actions, through your help, your suggestions or your acts.
  • To have the opportunity of sharing your difficulties, to formulate any questions you may have, to find a listening ear from people who, like you, have suffered from the various degrees of the illness.
  • To be kept regularly informed, thanks to the news-letters issued with our Scientific Advisory Board and at the general meeting, regarding the different actions and projects carried out within our association, and the impact they have on patients’ lives.

 

WE, as well as the sicks and their relations, can act .

 Source : Hepatic Vascular Disorder Reference Centre . Hepatology Unit Beaujon Hospital.

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PORTAL HYPERTENSION

  THE PORTAL VENOUS  SYSTEM 

All the organs of the body receive their blood via an artery coming from the aorta. For most of the organs, the blood coming from the vena caval system drains into the inferior or superior vena cava.  However, for the digestive system located in the abdomen (stomach, pancreas, small and large intestine) as well as for the spleen, the blood is collected by a particular venous system, the portal venous system, which drains into the liver . The portal venous system brings most of the blood from the digestive tract to the liver. This blood carries around all food absorbed by the intestine, medicines taken orally, but also many hormones and substances secreted by the digestive organs, for example insulin from the pancreas. 

In the liver, the portal vein branches out in many little veins similar to the arteries in order to irrigate liver capillaries. The level of blood flow in the portal vein is very high; it equals to about 20% of the cardiac output and corresponds to 2/3 rd of blood flow received by the liver, the liver artery bringing the remaining 1/3. 

 PORTAL HYPERTENSION 

Portal hypertension is characterised by an increase in the blood pressure in the portal veinsystem.

 CAUSES OF PORTAL HYPERTENSION 

There must be an obstacle to the blood flow, in a strategic place, to cause portal hypertension. This strategic obstacle can be: 

  1.  An obstacle of the portal vein trunk which is mainly represented by the thrombosis of the portal vein or its consequence, the entity called a cavernoma or cavernous trnasformation.  Such a thrombosis is often due to a blood disorder which increases the risk of venous thrombosis in general.                                                                                      

  2. The most frequent obstacle on the branches of the portal vein or liver capillaries is cirrhosis which is the result of a long lasting disease of the liver.  These long lasting diseases of the liver are generally associated to drinking alcohol, diabetes or obesity, a chronic viral hepatitis B or C. However,  there exists also damages to the portal vein ramifications or liver capillaries causing a portal hypertension without being due to a cirrhosis. These diseases proper to the small vessels have been called with different names which shows our lack of knowledge in regards to them. They were named as idiopathic portal hypertension, portal liver sclerosis, intra-hepatic portal hypertension non cirrhotic, occlusive portal venous disorder, or regenerative nodular hyperplasia.

3.The obstacle on the hepatic veins is represented by the syndrome of Budd-Chiari which is generally caused by a thrombosis. Thrombosis of the hepatic veins is often due to a blood disorder which increases the risk of thrombosis.

Blood disorders increasing the risk of thrombosis of the portal vein or the liver veins include mainly the myeloproliferative disorders, the antiphospholipid syndrome, inherited coagulation defects (V factor Leiden, factor II Leiden, deficiency in antithrombin, in protein C, or  in protein S ), or chronic inflammatory diseases, in particular those related to the digestive track. 

CONSEQUENCES OF PORTAL HYPERTENSION AND ITS TREATMENTS

a)    The increase in the portal pressure leads to a blood congestion  upstream of the obstacle, which can manifest in two ways; 

  1. Ascites: This is a watery liquid produced by the congested liver which accumulates  in the abdomen. The congestion of the liver is due to the obstacle on the liver veins or the small ramifications of the vessels in the liver. However, in case of thrombosis on the portal vein there is no liver congestion and no ascites. The first treatment implemented for ascites is the intake of diuretics and a low-salt diet. 
  2. Splenomegaly:  This is the increase of the size of the spleen, which is partly due to the congestion and partly to other more complicated mechanisms. In the case of portal hypertension, the splenomegaly is accompanied by a decrease of white cells and platelets in the blood, which mechanism is once more complicated. We call hypersplenism, the association of a splenomegaly and the decrease of white cells and platelets. This decrease, when it is specifically due to the portal hypertension is never dangerous even when very noticeable 

 

a)    The difference of pressure between the portal vein system and the lower and higher cava veins system fosters the formation of veins between these two systems. These veins are called shunts (or collateral veins, portacaval or portasystemic derivations)

These shunts can be seen as means used by the body to maintain the blood return towards the heart despite the obstacle on the portal system. These veins divert, direct away from the liver and into the general bloodstream, a part of the blood that should have gone first through the liver. These shunts can cause 2 types of complications: gastrointestinal bleeding and changes in the metabolism of some substances.

 1. Gastrointestinal bleeding; some collateral veins run within the wall of the oesophagus or the stomach  where they form varicose veins . Varicose veins can crack and cause bleeding in the digestive track which can translate in fainting due to a strong decrease of the blood pressure or simply in an  anaemia, or just with black stools (called melena)  but sometimes with a more frightening vomiting of gastric liquid strongly coloured in red by red blood  (called hematemesis).

 Most of these bleedings stop by themselves. However they require a quick admission to hospital to take medicine to reduce the portal pressure and to have an endoscopy which will prevent a recurrence of the bleeding using simple mechanicals means. It may be necessary to perform a red cells transfusion.

 These bleedings or their recurrence can be prevented, to a large extent, because they only affect the varicose veins that are large in size or look fragile, or those which already bled. An endoscopy done regularly (every 1 to 3 years) allows to detect them when no bleeding has occurred. Two types of treatments can then be implemented: medicinal or endoscopic.

 The medicinal treatment: consists in the daily and permanent intake of Propranolol or nadolol without any risk in the absence of asthma, Raynaud syndrome, or some other heart problems. We think these medicines work in decreasing the portal pressure. They can affect some patients with a discomfort leading to stop the treatment for 15 to 20% of the patients. 

The endoscopic treatment consists in fixing on the varicose veins small rubber bands which strangle them and make them disappear but this treatment is rarely definitive and a regular endoscopic check up of the oesophagus and stomach remains necessary. These treatments, (eventually combined with one another), prevent haemorrhages provided the program is properly followed. 

A major metabolic changePortacaval shunts carry blood from the gastrointestinal track to the general bloodstream without having been modified by the liver. Substances that are totally captured by the liver the first time they go through it, can then reach other organs, something which would not have happened in a normal situation. The mechanism can disrupt the elimination of medicines but also the elimination of the substance produced by the digestive track. This is how we explain the drowsiness of the brain, reversible, called hepatic encephalopathy, due to the lack of elimination of ammoniac and other substances. This serious complication is thankfully rare in its permanent or recurrent form. The treatment of the encephalopathy consists of 2 medicines: lactulose or/and rifaximin are very efficient and very well tolerated. The mechanism of their beneficial action on the hepatic encephalopathy is still not well known.  

A drastic way of correcting portal hypertension and to prevent bleeding is to connect directly the portal vein with the lower cava vein (anastomosis  portacava) in building a bypass in the liver using a stent. This method called TIPS is done with radiology reaching the venous systems by puncturing the right jugular vein at the neck level. The difficulty is to divert all the portal blood towards the general bloodstream with the metabolic consequences described above and the high risk of hepatic encephalopathy. The decision for this type of chirurgical intervention requires a careful analysis of the situation in a specialised centre.

 Source : Hepatic Vascular Disorder Reference Centre . Hepatology Unit Beaujon Hospital.

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