Cholestasis - a pathological condition in which the marked decrease in secretion of bile into the small intestine, resulting from breach of its products or excretion.Depending on the etiopathogenetic mechanisms violation of the flow of bile into the duodenum share extrahepatic cholestasis and intrahepatic its shape with fundamental differences in clinical manifestations.
In most situations, experts use the term "cholestasis syndrome" as a pathological condition, this is not an independent nosological form.Pathomorphological this pathology manifests itself in the form of a drip of bile in the liver cells and around dilated kanalikulov (intrahepatic cholestasis).If there is a variant of extrahepatic cholestasis, bile droplets accumulate mainly around the dilated bile duct, as well as a small amount directly into the hepatic parenchyma.
cholestasis liver with prolonged duration provokes the development of structural damage to the structure of the liver reversible and irreversible
Chronic cholestasis in addition to the above changes characterized by the formation of irreversible disturbances in the form of bile infarcts, areas of sclerosis and form microabscesses.Gastroenterological profile Experts are of the opinion that almost any form of liver disease, as a structural unit, inevitably provokes the pathogenic mechanisms of cholestasis.In addition, there are a number of pathologies involving both intrahepatic ducts lesion or extrahepatic localization.
Among the pathological conditions that can provoke the development of cholestasis released a wide range of diseases, but unites all these etiological factors of a single etiopathogenetic mechanism of cholestasis.So, the main link in the pathogenesis is the damaging impact of bile acids in the liver cells.All diseases and pathological conditions are the background for the development of cholestasis are divided into two pathogenetic categories: diseases associated with impaired bile production in the required quantity and pathological conditions that violate the normal current of bile.
Violation zhelcheobrazovatelnoy liver function observed in alcoholic liver parenchyma lesions, viral and toxic effects on the body with toxic agents preferentially localized in the liver.In addition, the process of formation of bile adversely affects disturbed microflora in the intestine, liver and cirrhotic changes endotoksemicheskie state.To this category belongs etiopathogenetic and intrahepatic cholestasis of pregnant women.
In the normal process of outflow of bile have a negative impact pathological changes of bile ducts (cholangitis, duktopatiya, Caroli's disease), congenital anomalies of the biliary duct system, and the system biliary cirrhosis.Such systemic diseases such as tuberculosis, lymphoma, and sarcoidosis will inevitably provoke a change in the normal functioning of the structures of hepatobiliary system, but these disorders are developed mainly in the late stages of the underlying disease.
Intrahepatic cholestasis in all cases accompanied by an excessive accumulation of bile acids not only in the circulating blood, but also in various types of tissues with simultaneous lack of content in the lumen of the duodenum of the small intestine.Hepatic cholestasis can occur in several forms.Thus, in his functional variant showed a significant slowdown in tubular bile flow, cholestasis and morphological accompanied by a significant deposit of bile components in the projection of hepatocytes and bile ducts.Clinical intrahepatic cholestasis occurs at a delay of bile components in circulating blood, while under normal conditions bile acids are excreted in the bile.
development of intrahepatic cholestasis, it is possible even with the total absence of luminal obstruction of the main bile duct.However, available intrahepatic bile duct obstruction order inevitably provokes the development of cholestasis syndrome.
Symptoms and signs of cholestasis
cholestatic syndrome, caused by excessive excretion of bile acids and other components of bile in the liver and other tissues of the human body, is inevitably accompanied by the development of pathological structural changes in target organs with specific clinical and laboratory manifestations.
the basis of clinical variants of cholestasis and their intensities laid three main mechanisms: excessive deposition of bile components in the circulating blood and tissues, a significant decrease in the concentration of bile in the duodenum, the damaging influence of toxic bile acids in the hepatocytes.The intensity of those or other clinical symptoms depends on the underlying disease, severity of liver cell failure and the severity of a violation of the excretory function of hepatocytes.
Itching in cholestasis par with signs of disorders of digestion and absorption of nutrients in the intestine are the main clinical criteria of the disease state, regardless of the form in which proceeds cholestasis.Pathognomonic symptoms of chronic cholestasis current version is a typical skin lesions in the form of hyperpigmentation and education xanthelasma and hepatic dystrophy, as a reflection of the bone lesions.It contributes to the development of hepatic osteodystrophy vitamin deficiency group D and E, and initial manifestations of this complication is a severe pain in the projection of the thoracolumbar spine.In addition, this category of patients are at risk for the occurrence of spontaneous pathological fractures caused by diffuse osteoporosis.
cholestasis peculiarity is that the development of manifestations of asthenic syndrome only occurs with concomitant hepatocellular lesions and manifests itself in the form of appearance is not motivated by weakness, dizziness, inability to perform usual exercise.When existing intrahepatic cholestasis, a change in the shape and size of the liver in the direction of its significant increase, with the complete absence of pain and disruption of the structure of hepatic parenchyma.Increasing the parameters of the spleen occurs only as a comorbidity biliary cirrhotic liver damage that is extremely rare.
One of the more specific manifestations of cholestasis, allowing an experienced set a preliminary diagnosis without conducting specific laboratory examination of the patient chair is a change in the form of an increase in its daily amount, the stench, discoloration and disturbance patterns.Due to the fact that the restriction in cholestasis noted receipt of bile acids in the duodenum of the small intestine, in the stool accumulates a large concentration of uncleaved fats.
Violations intestinal absorption inevitably affect the vitamin balance in the body.So, even for a short period of cholestasis provokes the development of vitamin K in the body, manifesting excessive bleeding of mucous membranes.Chronic cholestasis same in all situations is accompanied by symptoms of beriberi group A.
result of digestive disorders and intestinal absorption, which is observed in all pathogenic variants cholestasis is prolonged debilitating diarrhea.Abundant diarrhea syndrome provokes dehydration and release of large amounts of electrolytes and other nutrients.The outcome of this condition is the development of malabsorption syndrome and a progressive decrease in the patient's weight even when the normal power supply.
important clinical criteria of chronic cholestasis is pathognomonic lesion of the skin in the form of xanthomas, which are represented by yellow tumor-like spots that result from lipid metabolism.Favourite localization of these changes is the skin of the upper half of the body, as well as the area of the natural skin folds.The appearance and disappearance of xanthomas may be regarded as a diagnostic feature, which reflects the level of cholesterol in the blood.In a situation where the patient's liver spots are localized exclusively paraorbital, experts use the term "xanthelasma."
cholestasis is also accompanied by symptoms characteristic of disorders of copper metabolism in the body, because under normal conditions the copper excretion and absorption in the small intestine takes place in conjunction with the bile.Prolonged cholestasis accompanied by a significant increase in the concentration of copper and depositing it in the target organs by the type of Wilson's disease.
major complications of cholestasis, observed in 70% of cases provided that the duration of its flow is the formation of pigment stones in the structures of the biliary system and its attendant symptoms of bacterial cholangitis.Furthermore, the clinical picture in each patient may vary, depending on the underlying disease manifestations.
addition to typical clinical manifestations, enabling experienced correctly establish the diagnosis already in the primary contact with the patient cholestasis, there is a certain algorithm of examination of patients.All laboratory and instrumental techniques used in this situation, are aimed not so much at the diagnosis of cholestasis as to identify its causes.So, holding ultrasound or cholangiography in most cases allows you to set the exact location of the blockade in the lumen of mechanical structures of hepatobiliary system.If you suspect the presence of a patient intrahepatic cholestasis option in the absence of information content during other instrumental methods of research, it is recommended the implementation of needle biopsy.
cholestasis in pregnant
prevalence of this disease among the general population of pregnant women is 1: 500, and a high rate of inheritance caused family type.In addition, cholestasis of pregnancy is prone to recurrent course, so subsequent pregnancy in 80-90% of cases and occurs with signs of cholestasis.Mortality in cholestasis fetus in a pregnant woman is not more than 10% and in most cases, priority is caused massive uterine bleeding due to vitamin A deficiency of K.
pathogenetic mechanisms of cholestasis in a woman during pregnancy explains the violation of estrogen metabolism, exerts an inhibitory effect on the formation ofand bile flow.In a situation where there is a significant deposition of bile acids and other components of bile in the placenta, significantly increases the risk of provoking premature births in the early stages of fetal development, incompatible with life.Among gastroenterologists it is believed that pregnancy acts as a provoking factor in the development of existing from birth genetic defects products and bile flow.
risk of developing this disease are women, are in the third trimester of pregnancy and in most cases, the only clinical manifestation of cholestasis is a common agonizing itching.The preferential localization of cutaneous manifestations of cholestasis of pregnancy is the upper half of the body and especially the palms of the hands.In most situations, itching independently leveled postpartum women, not later than two weeks.
most informative regarding the diagnosis of cholestatic syndrome in pregnancy have blood chemistry, the study of which has been a significant increase in direct bilirubin, and alkaline phosphatase activity under normal indicator of ALT and AST.When carrying out a specific analysis of the content of fractions of bile acids in the blood of pregnant women in cholestasis there is a significant increase in their concentration in excess of the normal value of 100 or more.
Despite the fact that cholestasis at birth abortion is a common pathology, even in completely healthy women, in the identification of its features is necessary to carefully examine the patient to determine the presence of other diseases with similar clinical and laboratory picture (parenhematoznaya and jaundice, hereditary diseasesmetabolic disorders, acute fatty liver).
Provided slightly pronounced clinical and laboratory criteria cholestasis pregnant treatment is solely to use a number of symptomatic drugs (Promethazine in a daily dose of 75 mg, a daily dose of phenobarbital 45 mg orally).In a situation where a pregnant woman has been a significant excess of the level of bile acids and are completely absent indications for delivery in a planned manner, we recommend the use of drugs, the effect of which is aimed at reducing the level of serum bile acids (Holestiramin a daily dose of 12 g) with simultaneous application phytomenadione on10 mg subcutaneously once weekly, and folic acid in a daily dose of 1 mg.
Due to an increased risk of uterine bleeding diet for cholestasis of pregnancy is important.A woman should eat enough of foods containing a high concentration of vitamin K, as well as limit the amount of fatty foods.
Cholestasis in children
incidence of cholestasis in infants in the neonatal period and chest period varies considerably in the population of various countries and regions, however, there is some tendency to increase in the incidence of extrahepatic cholestasis origin in these patients.We should not lose sight of the transient variants of cholestatic syndrome newborns, even though substantial harm to the health and development of the child, he does not bring.
Determination etiopathogenetic variants cholestasis in children it is critical, as it affects the further patient management.Currently, most of the options extrahepatic cholestasis in infants is an absolute indication for surgery, since modern methods of surgical treatment of extrahepatic biliary atresia procedure allows to achieve good results in the elimination of signs of cholestasis.
Common features characteristic of both etiopathogenetic variants of cholestasis is the appearance of clinical and laboratory signs of violation of the outflow of bile.Clinical criteria in this case is the appearance of jaundice staining of the skin and mucous membranes, discoloration of feces and dark urine.The most important diagnostic sign of cholestasis in the newborn period is the color of the stool, as it allows us to estimate changes in the dynamics of development of cholestasis.For newborn babies is not typical appearance of pronounced skin itch, and in older age pronounced itching observed in intrahepatic cholestasis version.Note that in the neonatal period are always flows cholestasis with jaundice, which is a specific pathognomonic clinical marker of the disease.