In gastric inflammation, a distinction is made between the acute and the chronic form. Stomach inflammation occurs when the protective coat of the stomach becomes too thin due to increased use or reduced education and the stomach acid attacks, irritates or even destroys the wall (ulcer).
Acute inflammation of the stomach (gastritis)
Acute inflammation of the stomach is usually caused by external influences and is often caused by overeating, alcohol, medication (NSAIDs, aspirin, corticosteroids, cytostatics), infections or stress. In addition to the stress in daily life, such stress can also trigger surgery, trauma or competitive sports (runners stomach).
complaints
The symptoms range from stomach pain, loss of appetite, feeling of fullness, belching, nausea to unpleasant taste in the mouth. In the worst case, the stomach wall is eaten, which is called an ulcer and of course poses a risk of bleeding.
treatment
The therapy consists mainly in omitting the triggering cause, temporary food parole, therapy for nausea and the administration of acid blockers. This usually leads to rapid healing.
Chronic inflammation of the stomach (gastritis)
Chronic gastritis usually causes far fewer symptoms and has a long history. The complaints essentially correspond to the above.
Type B gastritis, Helicobacter pylori (80%)
Infection with Helicobacter pylori, a bacterium that can survive in the acidic environment of the stomach and causes chronic inflammation, is common (about 1% per year of life). This permanent inflammation leads to thinning of the stomach wall (atrophy) and a decrease in the acid-producing parietal cells in the stomach. Additional factors such as acidic foods (fruit juices, vinegar) or acid-producing foods and luxury foods (coffee, nicotine, wine, schnapps) then all the more cause complaints. Often gastric and duodenal (duodenal) ulcers (ventricular ulcers, duodenal ulcers) develop, and the infection apparently affects the immune system deeply. A connection with calcification of the arteries of the heart (coronary artery disease, CAD) is also discussed. Atrophy of the gastric mucosa also increases the risk of stomach cancer.
The disease is best diagnosed by gastroscopy with samples and is easily and efficiently treated with antibiotics and acid inhibitors.
Type A gastritis (5%), type C gastritis (15%)
Type A gastritis is an autoimmune disease against the acid-producing parietal cells of the stomach (parietal cells, ATPase) and the intrinsic factor. This leads to a reduction in acid formation in the stomach and, due to the lack of the intrinsic factor that sends the vitamin B 12 through the intestinal wall, to poor digestion and a vitamin B12 deficiency (pernicious anemia, anemia). Chemical gastritis (type C) is inflammation of the gastric mucosa through medication (NSAIDs – non-steroidal anti-inflammatory drugs, aspirin) or through the backflow of alkaline bile from the duodenum into the stomach. Since the stomach only provides protection against acid, there is also inflammation in the latter case, which does not respond to the administration of acid blockers.
Crohn’s disease, with ulcerative colitis, is a chronic inflammatory bowel disease. The incidence is approximately 5/100 000 / y with a disease peak between the ages of 20 and 40. There is a family cluster. The exact mechanism of disease development is still unknown. It is an autoimmune disease in which local tissue damage with inflammation and ulceration in the intestine occurs through activation of the lymphatic cells with the release of inflammatory messengers. Although Crohn’s disease can occur anywhere on the digestive tract, the terminal small intestine (terminal ileum) and the large intestine (colon) are usually affected. Unfortunately, Crohn’s disease is currently not curable, but it can be treated well.
complaints
The symptoms of Crohn’s disease are varied. In addition to a general feeling of illness, cramp-like complaints in the abdomen and diarrhea occur. Diarrhea is often associated with mucus and blood build-up. Slight temperature increases can occur. Deficiency is often caused by the lack of absorption of vitamin B12, zinc, folic acid and iron. This can cause growth and failure to thrive in children. None of the symptoms mentioned is always present. Furthermore, diseases outside of the intestine with eye infections (episcleritis), joint infections (arthritis), ankylosing spondylitis and liver infections (primarily sclerosing cholangitis) occur.
course
Crohn’s disease usually affects the intestine in individual sections, between which there is healthy intestinal tissue. Inflammation affects all layers of the intestinal wall. The course of the disease is batch-wise and varies greatly from individual to individual. Relapses are possible at intervals of years and decades, while other patients only have short disease-free periods. Complications can arise from the inflammatory shrinkage of the intestinal diameter (stenoses) or from the formation of fistula ducts. In any case, a close connection to the treating doctor is necessary.
The diagnosis is made using a colonoscopy (colonoscopy), in which tissue samples are painlessly taken from all parts of the intestine. These are examined finely (histologically), thereby ensuring the diagnosis. Since the disease can affect all parts of the intestine, an additional gastroscopy is also required after the diagnosis.
therapy
Regarding nutrition, the therapy is supportive, i.e. if lactose deficiency has been demonstrated (approx. 30% of patients), a lactose-free diet is advisable, missing vitamins and trace elements are substituted. There is no real Crohn’s diet. All foods that are tolerated can also be eaten (elimination diet). A fiber-free diet is necessary in the acute episode. If the course of the disease is very severe, short-term nutrition through the vein may also be necessary.
Acute relapses are treated by administering medications that suppress the body’s immune response. These include cortisone (local or systemic), azathioprine, TNF blockers (= biologicals, infliximab, adalimumab, golimumab and certolizumab), a4b7 integrin antibodies (vedolizumab), interleukin 12 and 23 antibodies (ustekinumab).
The medication is either given in short doses with rapid tapering (relapsing therapy for cortisone) or given permanently as maintenance therapy. If complications such as stenoses or fistulas occur, antibiotic treatment, endoscopic dilatation or surgical therapy may also be necessary. The therapy depends heavily on the existing symptoms, the course and the concomitant diseases. Here a close consultation, connection and cooperation with the treating doctor is necessary. It is important to be able to speak back quickly in the event of problems or symptoms.
Ulcerative colitis, with Crohn’s disease, is a chronic inflammatory bowel disease. The frequency is approximately 4/100 000 / year with a disease peak between the ages of 20 and 40. The exact mechanism of disease development is still unknown. It is an autoimmune disease in which local tissue damage with inflammation and ulceration in the intestine occurs through activation of the lymphatic cells with the release of inflammatory messengers.
Ulcerative colitis has continuous intestinal infestation with ulceration of only the superficial layers of the mucous membrane. It only affects the large intestine and begins in the rectum (rectum) and progresses upwards to different degrees in the colon. Unfortunately ulcerative colitis is currently not curable, but can be treated well.
complaints
Blood-mucous diarrhea and cramp-like abdominal pain (tenesmen) often appear as the main symptom, especially before and during bowel movements. Low temperatures, weight loss, heavy bleeding and growth and failure to grow in children can occur. Complaints outside the intestine are less common in ulcerative colitis (with the exception of the liver) than in Crohn’s disease. They mainly affect the skin (canker sores, erythema nodosum, pyoderma gangrenosum), the eyes with inflammation, the joints (arthritis and ankylosing spondylitis), and the liver (primarily sclerosing cholangitis).
Diagnostics and course
Ulcerative colitis affects the intestine continuously, the rectum is always affected. The course is batch-wise and individually very different. Flare-ups can occur here at intervals of years and decades, while other people only have short breaks. Relapses are often triggered by increased stressful situations.
Complications can arise from significant blood loss, from a very rare but then very pronounced colon inflammation with an enlarged bowel (toxic megacolon). Ulcerative colitis is at increased risk of developing colon cancer. The risk of colon cancer correlates with the extent of the length of the infection and the extent of the inflammation. The risk increases after about 10 years. Close consultation with the attending doctor is necessary here.
The diagnosis is made by means of a Dickdam mirror (colonoscopy), in which tissue samples are painlessly taken from all parts of the intestine. These are then examined in the tissue and enable the diagnosis to be confirmed.
therapy
Diet: There is no real colitis diet. Missing vitamins and trace elements are added, and additional drinking food is given in the event of malnutrition. In the acute episode, the diet is low or free of fiber. If the course of the disease is very severe, nutrition via the vein may also be necessary. Treatment of acute relapses and maintenance therapy is carried out by the administration of 5 amino salicylic acid (5-ASA) and, in the case of infestation in the left large intestine, the administration of locally effective steroids. After a remission has been reached, 5-ASA significantly reduces the recurrence of relapses and the risk of developing carcinoma (minus 75%!) And is therefore given as a low-dose continuous dose.
If the clinical picture is severe, there is also a short-term systemic administration of drugs that suppress the body’s immune response. These include cortisone (local or systemic), azathioprine, cyclosporin A and the TNF blockers (= biologicals, infliximab, adalimumab, golimumab and certolizumab), a4b7 integrin antibodies (vedolizumab), interleukin 12 and 23 antibodies (ustekinumab).
Surgical therapy may also be necessary if the inflammation is very pronounced or the megacolon is toxic. Since ulcerative colitis affects only the large intestine, healing by colon removal is possible in principle. However, this is reserved for very severe cases and acute complications.
The therapy depends heavily on the existing symptoms, the course and the concomitant diseases. Here a close consultation, connection and cooperation with the treating doctor is necessary. It is important to be able to speak back quickly in the event of problems or symptoms.
Microscopic colitis is a trigger for unclear diarrheal diseases, which are accompanied by a completely normal appearance of the intestinal mucosa in the performed colonoscopy. Diagnosis is only possible on the basis of detailed tissue samples from all sections of the intestine and analysis thereof under the pathologist’s microscope. The origin is not entirely clear, a cause is assumed over several factors that have to come together (multifactorial genesis). Drugs (NSAIDs) are a possible cause. A distinction is made between two forms: firstly, collagenous colitis with a thickening of the connective tissue fibers in the intestinal wall and secondly, lymphocytic colitis with development of an inflammatory reaction in the intestinal wall (frequency for both 2-3 / 100,000). The disease in each case means that fluid from the intestine can no longer be absorbed back into the body, causing diarrhea. Both diseases have increased in frequency significantly for years and affect more women than men (5: 1). The age is usually over the 60th year of life.
symptoms
The main symptom is the development of watery diarrhea over 4 weeks, which can also occur in episodes. An infectious cause, sprue or lactose intolerance should be ruled out before a possible diagnosis is made. Weight loss, abdominal pain, nausea, gas and nighttime diarrhea are common. The diagnosis is only possible via colonoscopy (colonoscopy).
treatment
The therapy consists in the administration of a locally acting cortisone in the intestine (budesonide), which is administered in slowly descending doses. The disease often responds very well and quickly. Since the therapy is symptomatic, i.e. not healing, if there is a relapse, especially at the beginning, after the first diagnosis. However, these respond well to the therapy. Since the treatment is carried out with a locally effective cortisone, the overall tolerance is good and the side effects of the therapy for the body are minor. Frequently, there are also long-term symptom-free intervals.
Hepatitis B is a virus-related inflammation of the liver. 300 to 400 million people are affected worldwide, with considerable regional differences. In parts of Africa or Asia, the disease affects up to 30% of the population, while in North America or Western Europe it occurs much less frequently at around 0.6%.
Infection occurs either during birth from mother to child, through sexual intercourse or through direct blood contact, e.g. by infected needles (for drug addicts or when using insufficiently sterilized needles for multiple use). Risk groups are people who use needles, employees in hospitals or medical practices as well as people with frequently changing sex partners or life partners of infected people. There is also an increased risk for people who frequently come into contact with blood products such as hemophilia or dialysis patients.
In earlier years, one of the most common transmission routes was the administration of contaminated blood products. Due to the high safety standards, this route of infection practically no longer occurs in industrialized countries. The disease is more contagious than e.g. Hepatitis C. An infected person with very strong virus formation in the blood can even pass on the disease through close physical contact. The usual interpersonal and family contact – including e.g. B. sharing cutlery or toilets – is harmless.
Acute illness
Much of the infection in adulthood heals completely. For one part, the disease itself is not noticed at all. It is only during a blood test that you notice that there has been contact with the pathogen, but the infection has healed without consequences and there is usually immunity to hepatitis B. Another part of the acute infections leads to a pronounced course of the disease Feeling unwell, weakness, tiredness, headache and joint pain and possibly jaundice (jaundice) as well as greatly increased liver values. However, this usually does not last longer than 6 weeks and then usually leads to healing.
Chronic disease in adults
About 10% of patients become infected in adulthood and the disease remains chronically active. Often you don’t notice the hepatitis infection. The disease usually runs from the beginning only with moderately elevated liver values and without symptoms. There is an increased risk of this in dialysis patients, transplant patients and drug use. In the case of chronic illness, depending on the amount of virus (more than 10,000 virus copies / ml blood, corresponding to 2000 IU / ml) and / or activity and fibrosis> = 1, there is an indication for treatment.
Chronic disease in infants
With infections in infancy or childhood the course of the illness is different from that with the adult: here over 90% of the infected develop a chronic, however mostly less aggressive course. As a virus carrier, these patients are a source of infection throughout life, but often have only minor liver damage.
Risk of chronic hepatitis B disease
The risk of chronic hepatitis B lies – over years or decades – in the development of cirrhosis of the liver and primary liver cancer (hepatocellular carcinoma, HCC). This development makes hepatitis B the tenth leading cause of death in the world.
therapy
There are now very good therapeutic options for chronic hepatitis B, which either lead to complete healing or at least control. This includes therapy with interferon, nucleoside or nucleotide analogs.
Interferon therapy only works if the hepatitis B e antigen is positive (HBe positive). About a third of these patients respond to interferon with a complete cure. In addition to interferon, there are the substance groups of the nucleoside and nucleotide analogs (lamivudine, adefovir, tenofovir, telbivudine, entecavir and others), which are easy to take as a tablet once a day and, with adequate therapy, lead to complete suppression of the virus. Here there are increasing healing rates over time. However, there is usually a need for lifelong therapy.
Whether and with what medication a patient with chronic hepatitis B should be treated depends on many circumstances, such as the individual’s medical history and blood values. Therapy decisions and planning are complex here and should only be made by an experienced doctor.
We would be happy to advise you competently and comprehensively.
Prevention / vaccination
Prevention by vaccination is better than any antiviral treatment for infection. The currently used vaccine, which is genetically engineered, is well tolerated and shows a high vaccine response. The vaccination then reliably protects against infection. Vaccination against hepatitis B is recommended in newborns (in children of positive mothers combined active / passive). However, every adult who has an increased risk of infection should also be vaccinated (partners and children of infected people, healthcare workers, dialysis patients).
Hepatitis C is a real mass disease and can be treated very well. In total, around 1-2% of the world’s population is infected with chronic hepatitis C. The frequency in Europe and America is between 0.5 and 2% (worldwide locally up to 20%).
The hepatitis C virus is an RNA virus that mainly settles in the liver and multiplies there. The transfer takes place directly by transferring blood to blood (parenterally), as possible with sexual contacts or also e.g. sharing needles among drug addicts. A possible risk of infection is also due to improper tattooing or piercing. The risk of infection from a blood transfusion was minimized by the high safety standards to a probability of 1: 1,000,000.
Acute hepatitis C.
The acute illness usually runs without symptoms, at most unspecific complaints similar to flu occur. However, the disease becomes chronic in approximately 70-80% of cases. Therefore, early therapy is recommended, which prevents the development of a chronic disease in 98%. In a study, 100% of genotype 1 patients were healed by giving sofosbuvir / ledipasvir for 6 weeks. (Deterding K. et al. Lancet Infect Dis 2016, 1473)
Chronic hepatitis C.
In chronic hepatitis C (disease with virus detection in the blood for more than 6 months), there are usually no or only mild symptoms, such as Fatigue or upper abdominal pain. However, the liver responds to the proliferation of viruses by developing inflammation and the accumulation of connective tissue. After a disease duration of 20-40 years, about 2/3 of the patients develop manifest cirrhosis with the corresponding risks.
There may be an increase in liver values in the blood, especially the so-called transaminases (GOT, GPT). The form of the disease and the level of transaminase deflection are not related.
Manifest chronic hepatitis C is only present when the virus RNA is detected in the blood. The antibodies to hepatitis C only become positive or remain positive after a delay, even if the disease has already healed spontaneously.
Therapy options (DAA)
Chronic hepatitis C, which was considered difficult to treat in previous years, can now be successfully cured in most cases by means of direct-acting antiviral combination therapy.
Therapy criteria are the detection of chronic hepatitis C and the patient’s request for therapy. The previously necessary evidence of higher-grade liver damage is no longer necessary. However, existing cirrhosis of the liver can affect the therapy regimen, so performing an elastography is always recommended. This measurement of liver damage is carried out non-invasively as part of an ultrasound examination.
Therapy of chronic hepatitis C – chances of recovery
There are different subtypes of the hepatitis C virus (genotypes, 1-6). The therapy of the subtypes was different for a long time (individualized therapy). However, modern therapy no longer has to make a difference here. In the past, hepatitis C was difficult to treat and not always successful. This has changed drastically with the introduction of the so-called “Direct Acting Antivirals” (DAA) of the second generation.
With the drug combinations sofosbuvir / velpatasvir or glecaprevir / pibrentasvir for 8-12 weeks, permanent healing occurs in 95-100% of cases. The therapies are well tolerated and have no noticeable side effects. Therapy failing is therapy with sofosbuvir / velpatasvir / voxilaprevir.
Therapeutic decision
Whether, when, for how long and with what medication a patient with chronic hepatitis C should be treated, despite all the simplifications, depends on the elastography and many other circumstances. The therapy decision and planning should only be made by an experienced doctor. We would be happy to advise you comprehensively.
Autoimmune hepatitis is a rare chronic liver disease. It accounts for about 5% of all chronic liver diseases. The terms autoimmune (components of the immune system are directed against your own body) and hepatitis (inflammation of the liver) describe the idea about the development of this disease. It is believed that the body acts in the sense of an immune response with antibody formation against its own liver cells and destroys them. The result is, over the long-term inflammation, an accumulation of scar tissue (fibrosis) with the possible end stage of cirrhosis.
Women, whose age is often under 30, are particularly affected. A familial clustering of the disease can be observed. Hepatitis A, B, C and D viruses, as well as herpes viruses, pharmaceuticals and environmental toxins, are discussed as triggers of the disease. However, an exact explanation of the origin of the disease has so far been lacking. Sick people usually show very non-specific symptoms such as tiredness, fever, nausea, weight loss, possibly also joint pain or repeated eye infections. In about 10% of the cases there are no symptoms. The disease is often combined with autoimmune thyroid diseases, rheumatism, vitiligo, inflammatory bowel diseases and vascular inflammation.
The autoimmune hepatitis is divided into 2 subtypes, which are defined with the help of the detection of different antibodies. Type 1 with detection of ANA and SMA is by far the most common. The diagnosis of the disease is mainly based on increased liver enzyme values, the detection of autoantibodies and a histological liver examination.
The therapy for autoimmune inflammation of the liver consists of medicinal inhibition of the immune system. This is done by immunosuppressive therapy, consisting of cortisone preparations and, for example, azathioprine, which must be taken over several years. An attempt to omit the medication is possible after 2 years with normal liver values, but has a high relapse rate, so that lifelong therapy often has to be carried out. The prognosis is very good with successful therapy and with a normal life expectancy.
Primarily biliary cholangitis (chronic non-purulent destructive cholangitis) is an autoimmune disease, the cause of which is unclear. This leads to a progressive inflammatory destruction of the small bile ducts, which can result in the final appearance of cirrhosis of the liver.
The PBC affects 80-90% women after the age of 40. In Europa, the occurrence is approximately 5 / 100,000 inhabitants. First-degree relatives and patients with indigenous sprue (celiac disease) have an increased risk of developing PBC.
symptoms
The symptoms of PBC are uncharacteristic. Above all, there is increased tiredness or excruciating itching of the skin. These symptoms can clearly precede an increase in bile and liver values. As a result of cholestasis (stasis of the bile), skin changes (xanthelasms = love handles), vitamin deficiency symptoms (vitamins A, D, E, K), increased stool (steatorrhea) and osteoporosis develop. The disease is also associated with other extrahepatic immune disorders, e.g. Sicca syndrome, Sjögren syndrome, rheumatism or Hashimoto’s thyroid disease.
diagnosis
Diagnosis is based on the detection of special antibodies, which are directed against mitochondria (AMA) and are present in> 95% of the cases, whereby the AMA subgroup M2 is considered pioneering. Laboratory chemistry has shown an increase in the so-called cholestase parameters over the years: alkaline phosphatase (AP), bilirubin and gamma-glutamyl transferase (g-GT).
A liver biopsy is not necessary to make a diagnosis, but it can be recommended to record the activity and stage of the disease. In stage I there is an inflammatory reaction of the so-called portal fields, in stage II additional necrosis and in stage III the formation of connective tissue septa, while in stage IV there is a cirrhotic liver tissue remodeling.
course
PBC is a creeping disease that can often go undetected clinically for years. In stage IV (cirrhosis), as with cirrhosis of other causes, there is an increased risk of developing hepatocellular carcinoma (HCC).
A therapy that can cure the PBC does not currently exist. However, the relevant data show that ursodeoxycholic acid (UDC) has a prognostically positive effect. This is a bile acid, which is non-toxic and largely free of side effects. The UDC is used in a dose of 10-15 mg / kg body weight / day for life in all stages of PBC. There is a reduction in cholestasis and liver inflammation parameters. If cirrhosis has occurred, the treatment of choice is liver transplantation. In 20-45% of cases, a recurrence (recurrence) of the PBC develops after transplantation, but this then usually proceeds very slowly. Determining the time for liver transplantation is difficult and requires a great deal of experience from the attending doctor and the sensitivity that is developed as a result.
The term NASH (non-alcoholic steatohepatitis) was introduced in the 1980s to classify liver damage that occurs without drinking alcohol. Histologically, however, the same criteria exist as for alcohol-related fatty liver: honeycomb cells that have changed, honeycomb cells, inflammatory cells, and necrotic changes in the liver tissue. These characteristics seem to be less pronounced in NASH than in alcohol-induced fatty liver. So-called Mallory bodies (= alcoholic hyaline) occur less frequently.
causes
It is clear that fatty liver formation, inflammatory reactions and also hyperinsulinemia are involved. The main causes are overweight, high blood lipids (hyperlipidemia) and high blood sugar (diabetes mellitus). However, drugs such as amiodarone, estrogens or glucocorticoids can also trigger NASH.
NASH is a frequent coincidental finding that is unclear due to an enlarged liver (hepatomegaly) or due to an increase in liver values (GOT, GPT).
symptoms
The majority of the patients are symptom-free. Atypical symptoms, such as feeling of fullness or right-sided, oppressive upper abdominal complaints could certainly be an indication.
Diagnosis
Before the diagnosis of NASH is made, other viral, autoimmune and metabolic diseases must be excluded. Furthermore, a detailed medical history, a careful clinical examination and possibly a liver biopsy are required to assess the severity of the damage.
course
NASH’s findings are diverse. They range from changes requiring control to cirrhosis, with the majority of patients having a good prognosis. Older, overweight patients have the greatest risk of increasing scarring (fibrosis), especially if an advanced inflammatory response is already evident in the histology.
therapy
The treatment of a NASH is problematic. Many patients in whom the liver changes are found to be minor and are not progressive do not require therapy. Careful weight loss, optimal diabetes control, and treatment for hypercholinesterinaemia are conducive to the successful regression of NASH. Radical diets are not recommended as this may increase the inflammatory response and thus have the opposite effect. The use of drugs that affect the metabolic situation is still the subject of studies.
The term liver cirrhosis stands for a predominantly scarred remodeling of the liver with the destruction of liver cells, impairment of the vascular supply and an increase in non-functional connective tissue. This condition can be compared to a pronounced scarring. Statistically speaking, men are affected much more often than women.
causes
The underlying causes are diverse. Chronic alcohol abuse is the most common cause of cirrhosis at around 40%, followed by chronic hepatitis B and C at around 35%. Metabolic diseases can also lead to cirrhosis of the liver. These include, for example, hemochromatosis (iron metabolism disorder) and Wilson’s disease (copper metabolism disorder). An autoimmune cause of cirrhosis is e.g. B. autoimmune hepatitis (AIH), in which antibodies against liver cells are formed. Bile drainage disorders can also lead to cirrhosis, such as primary biliary cholangitis (PBC) or primary sclerosing cholangitis (PSC), both of which can cause inflammation and subsequent destruction of the liver. In addition, medication or toxic substances can cause cirrhosis of the liver. In some cases, no cause can be found. This form is called cryptogenic (hidden) liver cirrhosis.
symptoms
Some of the patients complain of uncharacteristic symptoms such as reduced performance and a feeling of pressure in the right upper abdomen at an early stage. Often there are no symptoms. In the later course of the disease, jaundice, ascites or (due to the formation of bypass circuits) bleeding from the esophagus can develop. The main focus of the investigations is the clarification and treatment of the cause, the assessment of liver function and the detection of complications. Since cirrhosis of the liver is defined as tissue remodeling, a liver puncture is usually performed to secure it.
severity
The so-called Child-Pugh classification, which includes various clinical and laboratory parameters and has been awarded points, has proven itself for the division into degrees of severity. The grades Child-Pugh A – C can be divided according to this point system.
treatment
It is always important to treat the root cause, as this usually improves liver function significantly. In stage A, semi-annual sonographic monitoring is also recommended. In stage B and C, the therapy of complications is also in the foreground.
With existing cirrhosis of the liver, all additional poisons such as alcohol and non-essential medication should be strictly avoided, and the underlying disease should be treated. Close contact with the attending doctor (adjustment of the therapy, implementation of the corresponding preventive examinations etc.) is necessary.
Liver transplantation may be necessary in severe cases. Determining the time is difficult and requires a great deal of experience from the attending physician and the sensitivity that is developed as a result.
Primarily sclerosing cholangitis (PSC) is a chronic autoimmune inflammation of the biliary tract. The frequency in Switzerland is approximately 1/100 000 / year with a frequency peak between 30 and 50 years of age. Men are affected twice as often. Most patients (80%) also have ulcerative colitis (i.e., inflammatory bowel disease), with bowel manifestation generally preceding PSC. PSC narrows the bile ducts, and chronic bile congestion leads to increasing scarred tissue remodeling (fibrosis) of the liver with the possible end stage of cirrhosis. The cause is currently unknown.
There is a frequent combination with other diseases such as ulcerative colitis, Sicca syndrome, immune thyroiditis (Gravis, Hashimoto), rheumatic diseases or retroperitoneal and mediastinal fibrosis (M. Ormond).
symptoms
The clinical symptoms are quite unspecific, with increasing tiredness, itching and yellowing of the skin often occurring. But symptoms such as upper abdominal pain, weight loss, liver enlargement and fever can also go hand in hand with the PSC. Approximately 20% of the patients remain asymptomatic.
diagnosis
The diagnosis is based on an increase in the bile values (alkaline phosphatase (AP), gamma-glutamyl transferase (GGT) and bilirubin) as well as possibly the appearance of pANCA antibodies (in 70-80%, less specific) and typical changes in the bile duct in the ERC (endoscopic Retrogrades Cholangiography)
The ERC is currently the method of choice among the imaging methods, since the typical image with pearl-string-like multiple strictures (constrictions) of different lengths, sagging and diffuse reductions in the bile ducts can best be represented. MRCP (imaging of the bile ducts using magnetic resonance imaging) can be an alternative. Liver histology is not diagnostic, but can help to classify the stages (I: portal cholangitis; II: periportal hepatitis and / or fibrosis; III: septal fibrosis and / or bridge necrosis; IV: cirrhosis).
course
The course of the disease is characterized by repeated bacterial inflammation of the biliary tract (cholangitis). The main complication is bile duct carcinoma (cholangiocarcinoma, CCC), which is observed in 4-20% of patients with primary sclerosing cholangitis.
therapy
There is currently no curative therapy. Bile duct infections are treated with antibiotics, the deficiency symptoms are compensated. Regular checks are necessary. The use of ursodeoxycholic acid (UDC) in a dose of 10-20 mg / kg body weight / day has a positive effect on the cholestasis parameters and significantly reduces the risk of bile duct cancer. In addition to this medicinal therapy, the endoscopic expansion of constrictions and, if necessary, the temporary insertion of plastic tubes (stents) to bridge the disease could have slowed the progression of the disease.
In the advanced stage of cirrhosis, the only alternative therapy is liver transplantation. After a successful liver transplant, life expectancy and quality are generally very good. Determining the time for liver transplantation is difficult and requires a great deal of experience from the attending doctor and the sensitivity that is developed as a result.
Hereditary (genetic) hemochromatosis is an inherited (autosomal recessive) iron metabolism disease with a frequency of approximately 1: 1000. A gene mutation causes an increased and not adapted iron absorption from the intestine and leads to iron deposition and subsequent damage in various organs, in particular the liver (liver value increase, risk of liver cirrhosis, liver cell carcinoma), pancreas (diabetes), heart (heart muscle weakness, Arrhythmia), joints (arthrosis) and skin (darkening).
The genetic defect affects the HFE gene, which is located on chromosome 6. If the defect affects only one chromosome, this is called heterozygous, it affects both chromosomes 6, homogzygot. In the European population, there is a single homogzygote point mutation in over 90% of people with hemochromatosis (Cys-282-Tyr), in 5-10% of hemochromatosis patients there is a second point mutation (His-63-Asp) in the HFE gene. This second point mutation (His-63-Asp) can also, in combination with a heterozygous status for the Cys-282-Tyr mutation, cause hemochromatosis (so-called compound heterozygote).
The laboratory determination of ferritin and iron transferrin saturation has been shown to be a useful screening test. These parameters can be used to assess the body’s iron stores by taking blood samples. The increase in iron transferrin saturation usually precedes the iron loading of the organs and the increase in ferritin. Iron transferrin saturation greater than 60% (normal 25-50%) raises the suspicion of hemochromatosis. However, false high or false normal values also occur in 5-10% of cases. The ferritin should be used as a measure of the total iron storage for assessment.
Unfortunately, the detection of a mutation in the HFE gene does not automatically allow the diagnosis of hemochromatosis. On the other hand, the lack of a mutation does not exclude hemochromatosis.
In the case of increased laboratory values, the diagnosis is clearly confirmed by a liver biopsy with determination of the iron content in the liver tissue (ashing). A statement about the extent of the liver damage can be made by an ultrasound examination with an elastography, i.e. without puncture, possible.
For siblings / children of hemochromatosis patients, the examination for the presence of a mutation in the HFE gene (Cys-282-Tyr) for early detection is highly recommended, since it is very quick to differentiate between a heterozygous and homozygous form. The therapy is carried out by bloodletting, with 500 ml of blood (corresponding to 250 mg of iron) being drawn weekly until the ferritin level returns to normal, and later quarterly. Due to the genetic increase in iron absorption, bloodletting therapy must never be stopped completely. In the long term, serum ferritin values of 50 ng / ml should be aimed for.
If this therapy is started early and is carried out for life, there is no organ damage and there is a completely normal life expectancy.
Wilson’s disease is an autosomal recessive inherited metabolic disorder that leads to copper accumulation in the body, primarily in the liver and brain. The genetic defect in humans lies on chromosome 13 and causes a reduced excretion of copper with the bile.
With food, 0.8 to 2.0 mg of copper are taken in daily. The transport is bound to ceruloplasmin. The copper is almost completely absorbed by the liver cells after absorption. Excess copper is bound to the stool and excreted in the bile.
root cause
The Wilson gene (ATP7B) located on chromosome 13 codes for an ATP-dependent copper transport protein (the Wilson protein). Patients with Wilson’s disease have a mutation in this gene that leads to reduced copper excretion in the bile. A strong reduction in coeruloplasmin leads to increased concentrations of free copper in the blood and subsequently to copper deposits in all organs.
symptoms
As a rule, abnormalities do not occur before the age of 6 and not after the age of 40. The majority of patients become symptomatic in adolescence or young adulthood, although a distinction must be made between liver-specific and other (extrahepatic) symptoms. Children are more likely to have exclusively liver problems, while with increasing age neuropsychiatric symptoms with asymptomatic cirrhosis are more likely to be noticed.
Liver (hepatic)
The liver symptoms are very variable and range from no symptoms to liver inflammation (hepatitis) or liver fibrosis / cirrhosis.
extrahepatically
The extrahepatic symptoms include neurological disorders such as lumpy language, trembling of the hands, gait insecurity (ataxia), difficulty swallowing (dysphagia) or coordination problems in the foreground. Late symptoms include spasticity, muscle rigidity and bulbar paralysis (lesion of the motor cranial nuclei in the area of the medulla oblongata). Various clinical pictures with aggressive or distant behavior can show up psychiatrically.
The eyes often show the so-called Kayser-Fleischer corneal ring, a brown ring around the cornea, which is due to the copper deposition. Furthermore, the kidneys (prox.-tubular dysfunction), the bones (osteopenia / osteoporosis), the heart (cardiomyopathy) or rarely also the skin (hyperpigmentation, bluish discolouration of the lunulae – crescents in the area of the fingernails) can be affected. Abdominal pain occurs as a non-specific symptom in approx. 60%.
diagnosis
The disease can be diagnosed by determining copper levels and coeruloplasmin in blood and urine. In addition, a liver biopsy should be performed, showing a histologically similar picture to alcohol hepatitis with a fine-dripping fatty liver with Mallory bodies and fibrosis. Inflammatory infiltrates, however, rarely occur. Using a special coloring (rhodamine), stored copper can be displayed. The copper content of the liver should also be determined. Values> 250 µg / g dry weight speak for a M. Wilson.
Molecular biological investigations to determine mutations of the Wilson gene are not part of the routine tests, since the variety of mutations (currently more than 370) cannot yet replace conventional diagnostics in a meaningful way. Clinically, an ophthalmological examination is also necessary to show a possible Kayser-Fleischer corneal ring.
therapy
The treatment is carried out with trientine (trieethylene tetramine) or D-penicillamine, which bind the copper in water-soluble form and lead to an increased copper excretion via the urine and the bile in the stool. Significant improvement in symptoms only occurs after some time, a balanced copper balance after 12-18 months. Unfortunately, lifelong therapy must be carried out.
With advanced cirrhosis of the liver, there may also be a need for a liver transplant. The new liver also eliminates the defect in protein formation in Wilson’s disease (correction of the gene defect because the new liver has a functional gene).