Cholestasis of pregnancy code for ICD 10. Liver diseases associated with pregnancy

Pregnancy is often accompanied by various complications. Among them, intrahepatic cholestasis of pregnant women is one of the most severe and common. The disease is accompanied by liver damage.

Most often, the pathology responds well to treatment, and all symptoms of cholestasis disappear after childbirth. But sometimes the development of the disease can lead to dangerous consequences - premature birth or intrauterine death of the fetus.

Cholestasis: what is this phenomenon?

Cholestasis is a pathological process associated with a violation of the formation and, accompanied by its stagnation and toxic damage to liver cells by bile components.

Various reasons can trigger the development of pathology, which can be conditionally divided into:

intrahepatic;
extrahepatic.

Extrahepatic cholestasis is associated with a violation of the outflow of bile from the gallbladder. It is caused by dyskinesia (impaired motor activity) of the biliary tract or a mechanical obstruction (stone, trauma, tumor) that clogged the bile ducts.

Intrahepatic cholestasis is a more complex phenomenon. It is caused by a violation of the production and movement of bile through the intrahepatic ducts. This may be due to insufficient intake of necessary substances into the body, pathological changes in the liver tissues, or may be the result of long-term extrahepatic cholestasis (this phenomenon rarely occurs during pregnancy).

Why does it occur in pregnant women?

Cholestasis of pregnancy (ICD-10 code - K83.1) is singled out as a separate disease (as opposed to bile stasis of any other origin). Its peculiarity is that the disease occurs in the third (rarely in the second) trimester of pregnancy and goes away on its own after childbirth. However, pathology can significantly worsen the patient's well-being during pregnancy.

The exact reason why this condition develops is unknown. It is assumed that sex hormones are involved in this. Gestagens (hormones of the second phase of the menstrual cycle and pregnancy) have a versatile effect on the body, which can sometimes lead to the development of pathologies. In particular, progesterone increases the formation of bile and reduces the motor activity of the biliary tract.

It is also assumed that an increase in the uterus and the associated change in the position of the abdominal organs may play a role. In particular, in late pregnancy, intestinal loops are displaced, which put pressure on the liver parenchyma, due to which the outflow of bile through the intrahepatic ducts is disturbed.

Hereditary factors, the peculiarities of the metabolism of sex hormones, the distribution of substances supplied with food, and other factors also have a certain influence. , which accompanies cholestasis, is observed in 80-90% of pregnant women.

Risk factors that can provoke the development of cholestasis include:

carrying a multiple pregnancy;
hereditary factor (the risk of cholestasis is high in those women whose closest relatives have had this disease);
pregnancy resulting from artificial insemination (IVF);
chronic liver diseases (cirrhosis, hepatitis, tumor processes);
toxic or alcoholic liver damage;
a history of unsuccessful pregnancies that ended in miscarriage or intrauterine fetal death in the early stages.

Other provoking factors that can play a role in the development of the pathological process include taking certain medications (oral contraceptives, hormones), congenital liver pathologies, and a sharp decrease in immunity during pregnancy.

Symptoms

When bile stagnates inside the liver, it does not enter the duodenum, while some of the bile acids and bilirubin are absorbed back into the blood. In addition, stagnant bile damages the liver parenchyma.

In this regard, a set of symptoms of cholestasis in pregnant women develops:

cholemic syndrome (associated with the entry of bile acids into the blood);
a symptom complex associated with a lack of bile in the feces and with damage to the hepatic parenchyma.

Holemia

The entry of bile acids and bilirubin into the blood causes unbearable skin itching. At the same time, in the early stages, the skin can look completely healthy. Itching is very intense, it is impossible to get rid of it, it causes insomnia, provokes mood swings and can even cause neuroses.

Yellowness of the skin appears a few days after itching. With cholestasis of pregnancy, this symptom may be mild. A characteristic feature that allows you to distinguish jaundice from natural fluctuations in skin tone is a yellowish tint of the sclera (normally it does not happen).

The third manifestation of cholemia is the darkening of the urine. It is also caused by high amounts of bilirubin in the blood and exceeding the renal threshold. Usually appears a few days after jaundice. Analyzes show a high content of bilirubin and bile acids in the blood and urine.

Acholia

A failure in the flow of bile into the duodenum leads to digestive disorders. In particular, with a lack of bile, it is impossible to fully digest fats. As a result, taste preferences change - a pregnant woman develops an aversion to fatty foods, and when she eats, dyspeptic disorders occur (diarrhea, pain in the abdomen and right hypochondrium). But since changes in taste and indigestion are considered normal by many pregnant women, such symptoms often go unnoticed.

A more reliable sign is a change in the appearance of the feces. Excrement becomes greasy due to undigested lipids and whitish due to a lack of stercobillin, a product of bilirubin metabolism, which gives stool its characteristic color.

Other specific features characteristic of cholestasis include:

weight loss;
development of hypovitaminosis due to malabsorption of fat-soluble vitamins A, E, D, K;
general weakness, fatigue;
deterioration of the skin, hair, nails;
decreased vision.

Liver damage

With a long course of the pathological process, bile begins to damage the hepatic parenchyma. In this case, there are pains in the right hypochondrium - non-intense, pulling, constant, aggravated after eating and physical activity. In this case, the phenomena of cholemia and acholia to varying degrees may be present. Laboratory blood parameters acquire changes characteristic of liver damage.

Why is cholestasis dangerous?

Stagnation of bile, in the first place, is a danger to the developing fetus, as toxins penetrate the placenta to the unborn child. The progression of cholestasis can lead to hypoxia (oxygen starvation) of the fetus and its intrauterine death, or to premature birth.

With a long course of cholestasis and the absence of timely treatment, a bacterial infection in the biliary tract may develop, which can lead to intrauterine infection of the fetus.

Subsequently, children born to mothers with diseases of the liver and biliary tract lag behind in mental and physical development, often get sick against the background of reduced immunity. Such babies suffer from diseases of the respiratory and digestive systems and are prone to various neuropsychiatric disorders.

For a woman, the development of cholestasis during pregnancy can later lead to the formation of gallstones, liver failure, or cirrhosis of the liver.

Diagnostic methods

During the appointment, the doctor listens to the patient's complaints, collects an anamnesis (finds out the presence of provoking factors and concomitant diseases), during a visual examination, pays attention to the yellowness of the skin, scratching, and palpation reveals an enlarged liver.

If cholestasis is suspected, a woman will have to undergo a series of laboratory and instrumental studies. Among them:

(allows you to determine the presence of an inflammatory process;
biochemical blood test;
urinalysis for bilirubin content.

A blood test necessarily includes liver tests, determination of the level of bilirubin and the concentration of bile acids. An increase in the level of bilirubin in the blood serum indicates stagnation of bile and damage to liver cells. The level of alkaline phosphatase is taken into account as a marker of bile synthesis disorders, the indicators of ALT and AST enzymes, the level of which increases with the destruction of liver cells.

In addition to laboratory tests, to clarify the diagnosis, instrumental research methods are prescribed - ultrasound or MRI of the liver. When conducting an ultrasound examination, the degree of damage to the organ is assessed, pathological changes in its tissues are revealed - dilated bile ducts, the presence of stones, cysts, tumor neoplasms that interfere with the outflow of bile.

In doubtful cases, when ultrasound does not give a reliable picture of pathological changes, they resort to the MRI method or examine the bile ducts using endoscopic cholangiography.

Treatment

Treatment of cholestasis of pregnant women is complex, including not only drug therapy, but also lifestyle and nutrition adjustments. In most cases, it is symptomatic, that is, it is aimed at alleviating the patient's well-being and eliminating the unpleasant manifestations of the disease. Usually, all symptoms of the disease disappear within a few days after childbirth, however, this does not mean that it is not necessary to treat the disease. Even in late pregnancy, steps should be taken to ensure that cholestasis does not cause serious liver damage.

Medical treatment

All drugs for the treatment of cholestasis should be selected by a specialist, taking into account possible contraindications. Many medicines during childbearing are prohibited for use, so the doctor must choose those medicines that will help relieve unpleasant symptoms without harming the health of the mother and the unborn baby.

The scheme of drug therapy includes choleretic drugs and hepatoprotectors. Cholagogues improve the production of bile, increase the tone of the biliary tract, and stimulate the secretion of bile. They are taken regardless of the meal, but it is advisable to observe strict intervals between taking the tablets.

A good effect is achieved when taking Hofitol. This is a safe natural herbal remedy, which contains artichoke extract. The principle of action of the drug is aimed at normalizing the production of bile, eliminating biliary dyskinesia and congestion.

To suppress intolerable skin itching, a pregnant woman may be prescribed medications based on ursodeoxycholic acid. The most commonly used drugs are Ursofalk, which provide a choleretic effect, prevent stagnation of bile and eliminate itchy sensations on the skin.

Hepatoprotectors are drugs that reduce the toxic effects of stagnant bile on the liver parenchyma. You need to take them before the birth of the child and for some time after (until the tests normalize). Among the most prescribed drugs in this group are Essentiale Forte, Heptral, Gepabene.

To improve the functioning of the gastrointestinal tract, digestive enzymes are prescribed - Creon, Pancreatin, Festal. To bind bile acids in the intestine, it is recommended to take enterosorbents - Polyphepan, Polysorb. Vitamins E and C are prescribed as antioxidants. To prevent bleeding, it is recommended to select multivitamin complexes containing vitamin K, which improves blood clotting.

In severe pathology, threatening the development of complications, a pregnant woman is hospitalized and detoxification procedures are performed in a hospital - plasmapheresis and hemosorption.

Nutrition Features

The diet for cholestasis of pregnant women occupies an important place in the complex therapy. A woman is advised to consume more easily digestible low-fat foods - vegetable dishes, fresh fruits, low-fat dairy products, dietary meat and fish.

Grains are good too. During this period, it is important to avoid overeating. The amount of food at one meal should be small, and the meals themselves - from 5 to 6 times a day. It is recommended to increase the amount of fluid (as much as the state of the kidneys allows). Freshly squeezed juices, diluted in half with water, green, herbal and fruit teas, compotes, fruit drinks, mineral waters without gas are useful.

The list of prohibited foods for cholestasis of pregnancy includes:

animal fats, butter;
fatty meats and fish;
rich broths;
spicy seasonings and spices;
pickles, marinades;
canned food, smoked products, semi-finished products;
fatty sauces;
legumes, mushrooms;
vegetables with coarse fiber (radish, turnip, radish, horseradish, bell pepper, white cabbage, etc.);
flour and confectionery (especially with cream);
strong black tea and coffee;
ice cream.

The diet should include vegetable soups, side dishes of cereals and vegetables, dietary meats (chicken, rabbit), lean fish. Dishes should be served boiled, stewed or steamed. Fatty and fried foods should be banned.

Butter should be replaced with vegetable oil (olive, sunflower) and used in salad dressings made from fresh vegetables and ready meals. Chicken eggs can be eaten, but not more than 1 piece per day. Almost all fruits are useful (except melons and avocados), as well as any berries that are a source of antioxidants and vitamins.

Folk remedies

In addition to the main course of treatment, a woman can, after consulting a doctor, use safe, time-tested folk recipes based on herbal and natural ingredients.

To cope with the incessant itching, you can put compresses with a decoction of chamomile, sage, birch leaves, just wipe the very itchy areas with an ice cube, make lotions with cold water or oatmeal broth.

Instead of tablets, many experts advise taking decoctions of choleretic plants. Today, pharmacies offer a wide range of various herbal preparations. Herbs are packaged in convenient filter bags that can be brewed and drunk like tea.

The patient should pay attention to her daily routine - a rationally composed regime of work and rest, dosed physical activity will make her feel better. A woman is recommended daily walking in the fresh air, water aerobics or fitness for pregnant women, light housework. Hard physical labor and professional sports should be completely excluded.

As a preventive measure for the disease, doctors recommend leading an active and healthy lifestyle, eating right and fully, treating concomitant diseases of the biliary system in a timely manner and eliminating risk factors that can lead to the development of cholestasis.

Cholestasis of pregnancy - causes and symptoms. Consequences of gestational cholestasis on mother and child. Methods for the correct treatment of cholestasis of pregnancy.

Intrahepatic cholestasis of pregnancy affects 2% of expectant mothers in Russia. The proportion of women who suffer from cholestasis during pregnancy is much higher in South America, reaching 25%.

Pregnant women suffer from intrahepatic cholestasis (ICH). This occurs as a result of impaired bile secretion at the level of liver cells - hepatocytes. This disease threatens the unborn child.

Cholestasis of pregnant women mkb 10- K.83.1.

Causes of cholestasis of pregnancy

Cholestasis of pregnancy symptoms

Symptoms of cholestasis are not dangerous, just unpleasant. They appear in the third trimester of pregnancy. Symptoms of intrahepatic cholestasis of pregnancy include:

Itching of the skin increases with the development of pregnancy and is aggravated immediately before childbirth. The feeling of itching first appears on the arms and legs, and eventually covers the torso, abdomen, neck and face. Symptoms of cholestasis disappear within three weeks after the birth of the child.

Internet discussions

Treatment of cholestasis of pregnancy. Top 8 ways

Exclude the causes of yellowing of the skin: hepatitis and the effects of certain drugs

Start pharmacological relief of the symptoms of the disease in the Department of Pathology of Pregnancy

Use cholestyramine and antihistamine in case of persistent itching

Take vitamin K to minimize the risk of bleeding. Pregnancy cholestasis causes liver dysfunction and leads to impaired blood clotting - hence an increased tendency to bleed

Take in parallel with the diet, if it occurs

Do not eat a lot of fatty foods, as the liver is weakened

Avoid sodas, sweets, and high-carb foods

Give preference to baked dishes, fruits, vegetables and compotes

Impact of gestational cholestasis on mother and child

Cholestasis does not interfere with the expectant mother and is soon forgotten after childbirth. But liver problems interfere with a normal pregnancy.

Risks for a mother with gestational cholestasis:

A pregnant woman with cholestasis needs:

perform fetal monitoring. The risk of rapid maturation of the placenta is increased, therefore NCT, CTG, Manning test and amnioscopy are very important

assess the mobility of the fetus by its intrauterine movements. Cholestasis threatens fetal death

make a decision to accelerate delivery in case of severe manifestation of cholestasis at the 25th week of pregnancy

will decide on artificial stimulation of labor and caesarean section with intrahepatic cholestasis

do not forget that the percentage of complications increases in proportion to the duration of pregnancy

remember that severe cholestasis leads to termination of pregnancy before the 36th week

do not use oral hormonal contraception for intrapeptic cholestasis. The disease often reappears with repeated pregnancies and occurs in 40% of women who give birth to a second child.

Pregnant women need to be screened. Early diagnosis and proper treatment of cholestasis results in natural childbirth without a threat to the mother and child.

RCHD (Republican Center for Health Development of the Ministry of Health of the Republic of Kazakhstan)
Version: Clinical Protocols of the Ministry of Health of the Republic of Kazakhstan - 2016

Medical abortion (O04), Liver damage during pregnancy, childbirth and the puerperium (O26.6), Singleton birth, delivery by caesarean section (O82), Singleton birth, spontaneous delivery (O80), Excessive vomiting of pregnancy (O21)

Gastroenterology

general information

Short description

Approved
Joint Commission on the quality of medical services
Ministry of Health and Social Development of the Republic of Kazakhstan
dated September 29, 2016
Protocol #12

This protocol addresses the following diseases/conditions associated with pregnancy:
uncontrollable vomiting of pregnant women;
Intrahepatic cholestasis of pregnant women;
Acute fatty degeneration of the liver of pregnant women;
HELLP syndrome.

Uncontrollable vomiting of pregnant women - a pregnancy-related pathology that occurs in the first trimester and is characterized by severe nausea and vomiting leading to malnutrition, weight loss, dehydration, electrolyte imbalance, ketacidosis, and often an increase in transaminase activity.
Note*: Frequency of NRP: 1-20 cases per 1000 pregnancies.

Benign cholestasis of unclear etiology, occurring in the second and third trimesters (more often after 30 weeks), very rarely in the first trimester and characterized by itching that is felt on any part of the body, but more often on the soles and palms, especially at night.
Note*: Frequency of ICP: 1 case per 2000-6000 pregnancies.

A life-threatening complication for the mother and fetus that occurs in the second half of pregnancy (more often in the third trimester), sometimes in the postpartum period, characterized by microvesicular steatosis of hepatocytes due to impaired fatty acid metabolism, and leading to acute liver failure.
Note*: Frequency of AFLD: 1 case per 10,000-15,000 pregnancies.

HELLP-syndrome- a life-threatening complication for the mother and fetus that occurs in the second or third trimesters of pregnancy and in the postpartum period, characterized by hemolysis, increased activity of liver enzymes, a decrease in the number of platelets, and leading to the development of acute liver failure, multiple organ failure, DIC, liver ruptures and hematomas of various localization. The HELLP abbreviation includes: H emolysis (hemolysis), E levated L iver enzymes (increased activity of liver enzymes), L ow P lateles (decrease in the number of platelets).
Note*: HELLP frequency: 1-6 cases per 1,000 pregnant women.

Correlation between ICD-10 and ICD-9 codes

ICD-10		ICD-9
The code	Name	The code	Name
O21	Excessive vomiting during pregnancy	-	-
O21.1	Excessive or severe vomiting of pregnant women with metabolic disorders	-	-
O21.2	Late pregnancy vomiting	-	-
O26.6	Intrahepatic cholestasis of pregnancy	-	-
O26.6	Acute fatty degeneration of the liver of pregnant women	-	-
O14.2	HELLP syndrome	-	-
O80	Singleton birth, spontaneous delivery	-	-
O82.0	Conducting an elective caesarean section. Repeat caesarean section NOS	-	-
O82.1	Performing an emergency caesarean section	-	-
O82.2	Cesarean section with hysterectomy	-	-
O82.8	Other singleton births by caesarean section	-	-
O82.9	Birth by caesarean section, unspecified	-	-
O04	medical abortion	-	-

Protocol development date: 2016

Protocol Users: GPs, therapists, gastroenterologists, obstetricians-gynecologists, infectious disease specialists.

Evidence level scale:

A	High-quality meta-analysis, systematic review of RCTs, or large RCTs with a very low probability (++) of bias whose results can be generalized to an appropriate population.
V	High-quality (++) systematic review of cohort or case-control studies or High-quality (++) cohort or case-control studies with very low risk of bias or RCTs with low (+) risk of bias, the results of which can be generalized to the appropriate population .
WITH	Cohort or case-control or controlled trial without randomization with low risk of bias (+), whose results can be generalized to the appropriate population or RCTs with very low or low risk of bias (++ or +), whose results cannot be directly distributed to the relevant population.
D	Description of a case series or uncontrolled study or expert opinion.

Classification

UXO classification

Symptoms	Light	Medium	heavy
Appetite	moderately reduced	markedly reduced	missing
Nausea	moderate	significant	persistent, painful
salivation	moderate	pronounced	thick viscous
Frequency of vomiting (per day)	3-5 times	6-10 times	11-15 times or more (up to continuous)
Pulse rate	80-90	90-100	over 100
Systolic BP	120-110 mmHg	110-100 mmHg	less than 100 mm. rt. Art.
food retention	mostly keep	partially hold	don't hold back
Weight loss	1-3 kg (up to 5% of the original weight)	3-5 kg (1 kg per week, 6-10% of the original weight)	more than 5 kg (2-3 kg per week, more than 10% of the initial weight)
Dizziness	rarely	in 30-40% of patients (moderately expressed)	in 50-60% of patients (expressed significantly)
Subfebrile condition	norm	rarely seen	in 35-80% of patients
Jaundice of the sclera and skin	missing	in 5-7% of patients	in 20-30% of patients
Hyperbilirubinemia	missing	21-40 µmol/l	above 40 µmol/l
Dry skin	-/+	++	+++
Chair	norm	once every 2-3 days	stool retention
Diuresis	900-800 ml	800-700 ml	less than 700 ml
Acetonuria	missing	periodically at 20-50%	at 70-100%

In addition, a modified index is used to assess the severity of vomiting in pregnant women. PUQE (Pregnancy- Unique Quantification of Emesis/ Nausea) :
Question 1: How long have you had nausea, discomfort, or stomach pain in the last 24 hours?

Question 2: Have you vomited and how many times in the past 24 hours?

Question 3: How many times have you had the urge to vomit or vomit without spitting up in the last 24 hours?

How many hours did you sleep in the last 24 hours? ___________________________________________
Name the causes of poor sleep __________________________________________________
On a scale of 0 to 10, how would you rate your well-being? ________________________ ,
where 0 is the worst possible and 10 is the best.
Name the reasons for feeling unwell _____________________________________________

Assessment of nausea and vomiting during pregnancy:

Risk factors for NRH are:
NRP in history;
hyperthyroidism;
· mental disorders;
· SD;
high BMI;
a female fetus
Hp infection (requires further study);

WCB classification: there is no generally accepted classification of ICP.
Risk factors for ICP are:
cholestasis against the background of taking contraceptives in history;
a family history of ICP;
multiple pregnancy.

OJBP classification: there is no generally accepted classification of OLFS.
Risk factors for AFLD are:
a history of AFLD in a previous pregnancy;
a history of impaired FA oxidation and Reye syndrome in children;
older age;
Multiple pregnancy
· preeclampsia;
a male fetus
low BMI;
taking NSAIDs.

Classification of the HELLP syndrome: There is no generally accepted classification of the HELLP syndrome.
Risk factors for HELLP syndrome are:
primiparous older patients;
the presence of preeclampsia;
Multiple pregnancy
polyhydramnios;

· family history of preeclampsia, diabetes mellitus, arterial hypertension.

Diagnostics (outpatient clinic)

DIAGNOSTICS AT OUTPATIENT LEVEL

A number of normal physiological and biochemical changes occur during pregnancy (Table 1), which should be kept in mind when interpreting the results of the examination.

Anatomical, physiological and biochemical changes during normal pregnancy:

Diagnostic criteria:

Diagnostic criteria for liver disease associated with pregnancy:

Nosology	Complaints and anamnesis	Physical examination	Laboratory research	Instrumental Research
NRB	Severe nausea and vomiting that occurs in the 1st trimester (usually between 4-10 weeks) resolves by 20 weeks weight loss drooling	signs of dehydration, hypersalivation, malnutrition, weight loss	change in FPP (in 67% of cases, more often with a later onset, severe ketonuria, normalize after rehydration) - transaminases, AST>ALT (2-5 ULN); electrolyte disorders: - hypokalemia; - hyponatremia; - hypochloremia; - hypomagnesemia; KOS: - metabolic acidosis ketonuria	· Ultrasound of the OBP; ECG signs of hypokalemia: flattening or inversion of the T wave and an increase in the amplitude of the U wave); EGDS.
VHB	itching, which is felt in any areas, more often on the soles and palms, especially at night	jaundice (in 17-75% of cases, more often 1-4 weeks after the onset of itching), excoriations (traces of scratching) are found on the skin	total bilirubin (often< 85 мкмоль/л); ALP (up to 4 norms and >, but this is not indicative due to its placental production); GGTP (more indicative, but occurs< 30% чаще при наличии генетической предрасположенности); · ALT, AST > 40 IU/ml; ↓ Albumin; · INR.	OB ultrasound MRCP; EGDS.
OZhPB	. symptoms develop over several days or weeks; . weakness; . lack of appetite, nausea and vomiting (70%); . pain in the right upper quadrant and epigastrium (50-80%); . polyuria and polydipsia. Women with existing liver and gastrointestinal disease may have atypical symptoms.	. Hypertension, edema (along with proteinuria indicate preeclampsia); . ascites; . jaundice; . bleeding from the upper gastrointestinal tract; . FPN with PE (60%); . OPP (50%); . infections; . pancreatitis.	. AST, ALT (300- 500 IU/l); . bilirubin (usually< 85 мкмоль/л, за счет прямой фракции); . coagulopathy (signs of DIC, mainly due to ↓ synthesis of coagulation factors): - PV; - ↓ fibrinogen; - ↓ antithrombin. . proteinuria	. Ultrasound of the OBP - echogenicity of the liver in comparison with the cortical layer of the kidney (diagnostic significance is low due to microvesicular steatosis), in some cases ascites; . CT OBP - diffuse ↓ density of the liver, in some cases ascites; . MRI OBP - ↓ liver signal intensity on T1-weighted images, in some cases ascites; . LBx - not performed and contraindicated due to coagulopathy. Picture of microvesicular steatosis with centrally located nuclei of hepatocytes (similar to Reye's syndrome and valproate-induced liver damage).
OZhPB	Swansea Criteria Six or more of the following, in the absence of other pathology: · vomit; · abdominal pain; encephalopathy; hyperbilirubinemia (>14 µmol/l); hypoglycemia (<4 ммоль/л); leukocytosis (>11x106/l); · high level of uric acid (>340 µmol/l); high ammonium levels (>42 IU/l); Ascites or reduced echo density of the liver on ultrasound; · high level of aminotransferases (>42 IU/l); Renal failure (creatinine level >150 µmol/l); · coagulopathy (prothrombin time > 14 sec. or activated partial prothrombin time > 34 sec.); microvesicular steatosis of the liver.
HELLP syndrome	. pain and defense in the right upper quadrant and epigastrium; . vomiting (sometimes with blood); . headache (30-60%); . visual impairment (17%) Malaise, flu-like symptoms.	. jaundice (5%); . AH (80%), generalized edema (most have preeclampsia); . manifestations of AKI, multiple organ failure; . DIC (hemorrhages at injection sites, bleeding, etc.); . subcapsular hematomas and liver ruptures (clinic of intra-abdominal bleeding and shock) hemorrhages in the brain, subdural hematomas (CVA clinic)	. ↓Hb; . AST > 70 IU/l; . Tts< 100 000 / мм3; . LDH > 600 IU/L; . coagulation disorders; . proteinuria (87%).	. ultrasound of the OBP; . MRI/CT OBP; . Lbx: - optional; - fibrin thrombi in sinusoids, hemorrhages, necrosis.

Diagnostic algorithm:
Diagnostic algorithm for liver diseases associated with pregnancy depending on its timing

Before pregnancy	1 trimester	2 trimester	3rd trimester	postpartum period
pre-existing	CVH, PBC, PSC, AIH, BVK, NASH, ASH (including cirrhosis in their outcome), Budd-Chiari syndrome and other vascular diseases
Acute, not related to pregnancy	AVH, symptomatic gallstone disease, drug-induced hepatitis, acute alcoholic hepatitis
pregnancy related	Uncontrollable vomiting of pregnant women	--	--	--
	--	(Pre)eclampsia -HELLP
	--	--	Acute fatty degeneration of the liver of pregnant women
--	--	Intrahepatic cholestasis of pregnancy		--

Diagnostics (hospital)

DIAGNOSTICS AT THE STATIONARY LEVEL

Diagnostic criteria at the inpatient level: see outpatient level.

Diagnostic algorithm: see ambulatory level.

List of basic and additional diagnostic measures:

Nosology	List of main events	List of additional events
NRB	. KLA + platelets; . TANK: ALT, AST, bilirubin, albumin, electrolytes (potassium, sodium, chlorine, magnesium); . KOS; . ultrasound of the OBP; . ECG	. blood/urine glucose; . BAK: pancreatic amylase; . HbA1C; . thyroid status: TSH, T4 free, Anti-TG, Anti-TSH; . SH markers: Anti-HAV IgM, Anti-HEV Ig M, Anti-HCV, HBsAg; . PCR HCV RNA (qualitative), PCR HBV DNA (qualitative); . urine diastasis; . coprogram; . fecal elastase; . feces for occult blood; . EGDS; . Ultrasound of the thyroid gland; . EEG; . MRI OBP; . MRI of the brain and cervical spine.
VHB	. KLA + platelets; . TANK: alkaline phosphatase, GGTP, albumin; . INR; . OBP ultrasound.	. reticulocytes; . OAM; . BAK: glucose, creatinine, urea, amylase, ammonia; . bile acids; . coagulogram (PV, fibrinogen); . SH markers: Anti-HAV IgM, Anti-HEV IgM, Anti-HCV, HBsAg; . ANA, ASMA, LKM, SLA, pANCA; . IgM, IgG; . AMA, AMA-M2, pANCA; . direct Coombs test; . MRI in cholangio mode (MRCP); . MRI OBP; . EGDS.
OZhPB	. KLA + platelets; . TANK: ALT, AST, bilirubin by fractions, LDH, amylase, creatinine, urea, glucose, ammonia); . KShchS; . coagulogram; . OBP ultrasound.	. IgE; . AMA, AMA-M2; . Anti-HAV IgM, Anti-HEV IgM, Anti-HCV, HBsAg; . PCR HCV RNA (qualitative); . PCR HBV DNA (qualitative); . OAM; . daily proteinuria; . Ultrasound of the pleural cavities; . Lbx.
HELLP-syndrome	. KLA + platelets, reticulocytes; . coagulogram; . OBP ultrasound.	. direct Coombs test; . OAM; . daily proteinuria; . Lbx.

Differential Diagnosis

Differential Diagnosis		Surveys	Diagnosis Criteria
Uncomplicated nausea and vomiting (in more than 50% of cases, NRP - in 0.5-1.5%)	The presence of vomiting as a common symptom	. KLA + platelets . TANK: ALT, AST, potassium, sodium, chlorine, magnesium, albumin; . urine ketones.	. vomiting less than 5 times a day; . absence of electrolyte-metabolic disorders; . absence of ketones in the urine; . lack of dehydration, weight loss
HG, YAB		. KLA + platelets; . feces for occult blood; . EGDS; . surgeon consultation.	. The presence of endoscopic / morphological changes in the gastric mucosa and duodenum
Infectious gastroenteritis		. UAC+ platelets; . acute phase indicators (CRP); . coprogram; . bacteriological examination of feces; . infectious disease consultation.	. acute onset; . signs of intoxication; . diarrhea syndrome; . detection of pathogenic microorganisms in feces.
VG		. UAC+ platelets; . VG markers: - Anti-HAV IgM; - Anti-HEV IgM; - Anti-HCV; - HBsAg. . PCR HCV RNA (qualitative); . PCR HBV DNA (qualitative); . OB ultrasound	. presence of markers and confirmation of replication of hepatitis viruses
hyperthyroidism		. thyroid status: TSH, T4 free, Anti-TG, Anti-TSH; . Ultrasound of the thyroid gland; . endocrinologist consultation	. ↓ TSH; . Antibodies to TSH; . T4 free; . on ultrasound of the thyroid gland, the presence of hypervascularization and an increase in the volume of the thyroid gland
diabetic ketoacidosis		. blood/urine glucose; . HbA1C; . KOS; . urinalysis for the determination of ketone bodies; . endocrinologist consultation.	. the presence of DM in history; . hyperglycemia; . HbA1C; . glycosuria; . ketonuria.
Migraine		. EEG; . MRI of the brain and cervical spine; . Ultrasound of the vessels of the neck and head; . consultation with an ophthalmologist, neuropathologist.	. the presence of a characteristic clinical picture (hemicrania) in the absence of organic pathology
pancreatitis		. KLA + platelets; . urine diastasis; . fecal elastase; . coprogram; . ultrasound of the OBP; . MRI OBP.	. pain syndrome; . steatorrhea, creatorrhea; . on ultrasound/MRI of ABP: enlargement of the pancreas, the presence of cysts, calcifications, GLP more than 2 cm, heterogeneity of the pancreatic parenchyma, increased echogenicity of the duct walls, unevenness contours.

Differential diagnosis of ICP

Differential Diagnosis	Rationale for differential diagnosis	Surveys	Diagnosis Criteria
Dermatitis	Presence of pruritus as a general symptom	. TANK: ALT, AST; . alkaline phosphatase, GGTP, total bilirubin; . INR; . dermatologist consultation.	The presence of skin itching, traces of scratching in the absence of changes in FPP
VG		. VG markers: o Anti-HAV IgM; o Anti-HEV IgM; o Anti-HCV; o HBsAg. . PCR HCV RNA (qualitative); . PCR HBV DNA (qualitative); . OBP ultrasound.
cholelithiasis		. KLA + platelets; . ultrasound of the OBP; . surgeon consultation.	. the presence of ultrasound signs of calculi in the gallbladder and / or bile ducts on ultrasound of the OBP
OZhPB		. KLA + platelets; . OAM; . Coagulogram (PV, fibrinogen); . VG markers: - Anti-HAV IgM; - Anti-HEV IgM; - Anti-HCV; - HBsAg. . ultrasound of the OBP; . MRI OBP.	. symptoms of intoxication prevail with the exclusion of SH markers and other causes of FPP; . AST, ALT (300-500 IU/l); . bilirubin (usually< 85 мкмоль/л, за счет прямой фракции); . hypoglycemia (due to a decrease in gluconeogenesis against the background of liver damage); . ammonia (due to violation of detoxification processes in the liver); . PV; . ↓ fibrinogen; . amylase (due to pancreatitis); . creatinine, urea, metabolic acidosis (due to AKI); . leukocytosis, neutrophilia, TCP; . proteinuria;
YAG		. TANK: ALT, AST; . IgG;	. transaminases; . IgG;
PBC		. TANK: SHF, GGTP; . IgM; . AMA, AMA-M2.	. ALP, GGTP, Ig M; . availability of AMA/AMA-M2
PSH		. TANK: SHF, GGTP; . IgM, IgG; . pANCA; . MR enterography . (MRCP).	. ShchF, GGTP; . presence of ANCA; . the presence of strictures of the bile ducts according to MR-enterography (MRCP).
Hemolytic anemia		. KLA + platelets; . BAK: bilirubin by fractions; . reticulocytes; . Coombs test; . hematologist consultation.	. reticulocytosis; . positive Coombs test.

Differential diagnosis of AFLD

Differential Diagnosis	Rationale for differential diagnosis	Surveys	Diagnosis Criteria
LIPP (caused by Paracetamol and other drugs)	The presence of cytolysis as a general syndrome	. TANK: ALT, AST, alkaline phosphatase, GGTP, bilirubin by fractions; . IgE; . ANA, ASMA, LKM, SLA, pANCA; . AMA, AMA-M2; . Anti-HAV IgM, Anti-HEV Ig M, Anti-HCV HBsAg; . PCR HCV RNA (qualitative); . PCR HBV DNA (qualitative).	. recent use of hepatotoxic drugs in history; . exclusion of other diseases that caused cytolysis.
HELLP syndrome		. KLA + platelets; . TANK: ALT, AST, bilirubin by fractions, LDH; . coagulogram; . OAM; . daily proteinuria.	. ↓Hb; . schistocytes, echinocytes in a smear; . indirect fraction of bilirubin; . AST > 70 IU/l; . Tts< 100 000 / мм3; . LDH > 600 IU/L; . coagulation disorders; . proteinuria (87%)
Eclampsia, preeclampsia		. TANK: ALT, AST, SHF; . OAM; . daily proteinuria.	. develops after the 20th week of pregnancy; . persistent increase in blood pressure; . swelling; . proteinuria.
Reye syndrome in adults		. TANK: ALT, AST, total bilirubin, alkaline phosphatase, ammonia, glucose, albumin; . coagulogram.	. 5-6 days after the onset of a viral disease; . history of taking acetylsalicylic acid or ASA-containing drugs; . normal levels of bilirubin, alkaline phosphatase.
VG		. KLA + platelets; . VG markers: - Anti-HAV IgM; - Anti-HEV IgM; - Anti-HCV; - HBsAg. . PCR HCV RNA; (qualitative); . PCR HBV DNA (qualitative); . OBP ultrasound.	. presence of markers and confirmation of replication of hepatitis viruses
YAG		. TANK: ALT, AST; . IgG; . ANA, ASMA, LKM, SLA, pANCA.	. transaminases; . IgG; . the presence of specific autoantibodies.

Differential diagnosis of HELLP syndrome.

Differential Diagnosis	Rationale for differential diagnosis	Surveys	Diagnosis Criteria
VG	cytolysis, thrombocytopenia, hyperbilirubinemia	. KLA + platelets; . VG markers: - Anti-HAV IgM; - Anti-HEV IgM; - Anti-HCV; - HBsAg. . PCR HCV RNA (qualitative); . PCR HBV DNA (qualitative); . OBP ultrasound.	. presence of markers and confirmation of replication of hepatitis viruses
pancreatitis		. KLA + platelets; . BAK: pancreatic amylase, electrolytes; . urine diastasis; . fecal elastase; . coprogram; . ultrasound of the OBP; . MRI OBP.	. pain syndrome; . steatorrhea, creatorrhea; . on ultrasound / MRI of the OBP - enlargement of the pancreas, the presence of cysts, calcifications, GLP more than 2 cm, heterogeneity of the pancreatic parenchyma, increased echogenicity of the duct wall, unevenness contours.
I WOULD		. KLA + platelets; . feces for occult blood; . EGDS; . surgeon consultation.	. the presence of endoscopic / morphological changes in the coolant and duodenum.
Appendicitis		. KLA + platelets; . Ultrasound/CT of OBP, small pelvis; . diagnostic laparoscopy; . surgeon consultation; . consultation of an obstetrician-gynecologist; . urologist consultation.	. positive symptoms of peritoneal irritation; . acute phase indicators; . on ultrasound/CT of OBP, small pelvis, thickening of the appendix more than 6 mm, the presence of calcifications or coprolites in the lumen, in combination with signs of periappendicular inflammation.
cholelithiasis		. KLA + platelets; . TANK: ALT, AST, AP, GGTP, total bilirubin; . ultrasound of the OBP; . surgeon consultation.	. the presence of ultrasound signs of calculi in the gallbladder and / or bile ducts on ultrasound of the OBP
Hematoma		. KLA + platelets; . TANK: ALT, AST; . coagulogram; . CT/MRI OBP.	. visualization of a hematoma by ultrasound of the OBP, CT/MRI OB
OZhPB		. KLA + platelets; . OAM; . BAK: ALT, AST, bilirubin by fractions, glucose, creatinine, urea, amylase, ammonia; . coagulogram (PV, fibrinogen); . VG markers: - Anti-HAV IgM; - Anti-HEV IgM; - Anti-HCV; - HBsAg. . ultrasound of the OBP; . MRI OBP.	. symptoms of intoxication prevail with the exclusion of markers of VH and other causes of FPP. . AST, ALT (300-500 IU/l); . bilirubin (usually< 85 мкмоль/л, за счет прямой фракции); . hypoglycemia (due to a decrease in gluconeogenesis against the background of liver damage); . ammonia (due to violation of detoxification processes in the liver); . coagulopathy (signs of DIC, mainly due to ↓ synthesis of coagulation factors); . PV; . ↓ fibrinogen; . amylase (due to pancreatitis); . creatinine, urea, metabolic acidosis (due to AKI); . leukocytosis, neutrophilia, TCP; . proteinuria; . Ultrasound signs of fatty hepatosis on ultrasound of the OBP.
Immune and thrombotic TCPP		. KLA + platelets; . BAK: Total protein, albumin, ALT, AST, bilirubin by fraction, alkaline phosphatase, creatinine, urea, uric acid, LDH, glucose, C-reactive protein; . coagulogram; . direct Coombs test; . myelogram; . immunological study; . histological biopsy examination (spleen, lymph node, iliac crest); . ultrasound of the OBP; . consultation with a neurologist	. thrombocytopenia up to single at a normal or even level of megakaryocytes; . morphological changes in platelets: an increase in their size, the appearance of small-grained blue cells, as well as their poikilocytosis and a decrease in the process forms of platelets; . anemia with significant bleeding; . myelogram: quantities megakaryocytes, the absence or small number of free-lying platelets, the absence of other changes (signs of tumor growth) in the bone marrow; . signs of hypocoagulation are not characteristic; . immunological examination: detection of antiplatelet platelet-associated (TpA-IgG) antibodies in the blood; . For thrombotic TCPP: - the presence of neurological symptoms; - the formation of multiple blood clots. . articular syndrome; . kepatosplenomegaly.
Hemolytic uremic syndrome		. KLA + platelets; . BAK: Creatinine, urea, electrolytes, total protein, ALT, AST, bilirubin by fraction, C-reactive protein, LDH; . coagulogram; . KOS; . ANA, ENA, ANCA, antibodies to double-stranded DNA; . Reberg-Tareev test; . Ultrasound of the kidneys.	. hyperkalemia; . hyponatremia; . creatinine; . ↓ GFR; . urea; . LDH.
SLE		. KLA + platelets; . OAM; . BAK: Total protein, albumin, protein fractions, ALT, AST, bilirubin by fractions, C-reactive protein, cholesterol, creatinine, glucose, serum iron; . coagulogram; . ANA, Antibodies to single-stranded, double-stranded DNA; . C3-C4 components of a compliment; . lupus anticoagulant; . antibodies to phospholipids; . antibodies to cardiolipin.	. meets the major and minor diagnostic criteria according to the relevant protocol

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Treatment

Drugs (active substances) used in the treatment

Human albumin (Albumin human)
Amino acids for parenteral nutrition + Other medicines (Fat emulsions + Dextrose + Multimineral)
Hydralazine (Hydralazine)
Dexamethasone (Dexamethasone)
Dextrose (Dextrose)
Diphenhydramine (Diphenhydramine)
Doxylamine (Doxylamine)
Potassium chloride (Potassium chloride)
Colestyramine (colestyramine)
Platelet concentrate (CT)
cryoprecipitate
Magnesium sulfate (Magnesium sulfate)
Methylprednisolone (Methylprednisolone)
Metoclopramide (Metoclopramide)
Sodium chloride (Sodium chloride)
Ondansetron (Ondansetron)
Pyridoxine (Pyridoxine)
Plasma, fresh frozen
Promethazine (Promethazine)
Retinol (Retinol)
Thiamine (Thiamin)
Tocopherol (Tocopherol)
Trimethobenzamide (Trimethobenzamide)
Ursodeoxycholic acid (Ursodeoxycholic acid)

Treatment (ambulatory)

TREATMENT AT OUTPATIENT LEVEL

Treatment tactics

Liver diseases associated with pregnancy are presented.

Non-drug treatments for liver disease associated with pregnancy:

Nosology	Non-drug treatments
NRB	. Exclusion of triggers (aromas, perfumes, smoking, cooking, certain foods: spicy, salty, fatty) (LE A) ; . Physiological rest of the gastrointestinal tract (UD A); . Fractional diet with fat restriction (LE B); . Ginger (UD B); . Acupuncture (LE C) ; . Supplementation with multivitamins (UDC).
VHB	. Careful fetal monitoring (LE A)
OZhPB	. Careful fetal monitoring (LE A)
HELLP syndrome	. Careful fetal monitoring (LE A)

Medical methods of treatment:
When prescribing drugs during pregnancy, you should know their safety class (FDA categories).

FDA drug categories for pregnancy

Categories	Interpretation of the drug
A	. Controlled studies in animals and pregnant women showed no risk in the 1st trimester and possible harm to the fetus later.
B	. Animal studies have not identified a risk to the fetus, but controlled studies have not been conducted in pregnant women; . Animal studies have shown adverse events that have not been confirmed in controlled studies in pregnant women.
C	. Animal studies have shown adverse events and no controlled studies have been conducted in pregnant women; . Animal and human studies are lacking; . Drugs are prescribed only if the benefit outweighs the risk.
D	. Potential fetal risks identified, but benefit outweighs risk in life-threatening conditions
X	. Animal and human studies have identified fetal abnormalities; . Drugs are contraindicated

Pharmacotherapy of liver diseases associated with pregnancy.
Drug therapy of liver diseases associated with pregnancy.

Nosology	Medical methods of treatment
NRB	1 line
	2 line
VHB	. UDCA 1 g/day; . cholestyramine 10–12 g/day (less effective, use with caution, may cause neonatal coagulopathy) (LEV) ; . supplementation with fat-soluble vitamins A, D, E and K, especially when cholestyramine is prescribed (LED B).
OZhPB	. treatment is carried out in a hospital (paragraph 12.4.4 of this protocol) (LE A)
HELLP syndrome	. treatment is carried out in a hospital (paragraph 12.4.5 of this protocol) (LE A)

Indications for expert advice:

NRB	. consultation of a surgeon in order to exclude complications of ulcer; . consultation with an endocrinologist to exclude hyperthyroidism, diabetic ketoacidosis; . consultation of an infectious disease specialist in order to exclude an infectious disease; . gastroenteritis, VG; . consultation of a neuropathologist, an ophthalmologist in order to exclude migraine.
VHB	. consultation with a dermatologist to exclude dermatitis; . consultation with a hematologist to exclude hemolytic anemia; . consultation with a surgeon to exclude cholelithiasis; . consultation of an infectious disease specialist to exclude B.
OZhPB	. consultation with an infectious disease specialist to exclude VG; . consultation of an obstetrician-gynecologist in order to exclude bleeding from the vagina.
HELLP syndrome	. consultation of a surgeon in order to exclude cholelithiasis, appendicitis, complications of ulcer; . consultation with an infectious disease specialist to exclude VG; . consultation with a hematologist to exclude immune and thrombotic TCPP; . consultation with a rheumatologist to exclude SLE; . consultation of an obstetrician-gynecologist in case of suspected ectopic pregnancy, ovarian apoplexy, algomenorrhea, premature detachment of a normally located placenta; . consultation with a urologist for the purpose of differential diagnosis in case of suspected obstruction or urinary tract infection; . consultation with a neurologist to exclude neurological manifestations of thrombotic thrombocytopenic purpura.

Preventive actions:
Preventive measures for liver disease associated with pregnancy

Nosology	Preventive actions
NRB	. vitamin supplementation (LEC)
VHB	. not provided (EL B)
OZhPB	In pregnant women with risk factors for AFLD: . low-fat diet (LEC); . inadmissibility of starvation (LE H)
HELLP syndrome	. not provided (LE C)

Patient Monitoring:

NRB	. KLA + platelets at least 1 time per week; . TANK: ALT, AST, albumin, electrolytes: potassium, sodium, chlorine, magnesium at least 1 time per week;
VHB	. KLA + platelets; . TANK: ALT, AST, alkaline phosphatase, GGTP, albumin, INR at least once a week; . in the conditions of the ward / intensive care unit, monitoring these indicators daily.
OZhPB	. KLA + platelets; . OAM; . BAK: ALT, AST, bilirubin by fractions, LDH, amylase, creatinine, urea, glucose, ammonia; . KOS; . coagulogram; . daily proteinuria (according to indications).
HELLP syndrome	. Treatment in a ward/intensive care unit with daily monitoring of the following indicators: . KLA + platelets, reticulocytes; . OAM; . TANK ALT, AST, bilirubin by fractions, LDH; . coagulogram; . daily proteinuria (according to indications).

:
Indicators of the effectiveness of the treatment of liver diseases associated with pregnancy

NRB	. decrease in the multiplicity or disappearance of vomiting (normalization of FPP, electrolyte-metabolic disorders, disappearance of ketonuria);
VHB	. disappearance/reduction of pruritus, jaundice, normalization of GGTP, alkaline phosphatase, ALT, AST, bile acids; . the birth of a viable child.
OZhPB	. normalization of FPP, regression of indicators of liver failure; . the birth of a viable child.
HELLP syndrome	. relief of hemolysis, normalization of FPP, platelets; . regression/disappearance of symptoms/recovery; . the birth of a viable child.

Treatment (ambulance)

DIAGNOSTICS AND TREATMENT AT THE EMERGENCY STAGE

Diagnostic measures
Diagnosis of liver diseases associated with pregnancy at the stage of emergency care

Medical treatment:
Treatment of liver diseases associated with pregnancy in the emergency phase

Nosology	Medical treatment at the emergency stage
NRB	. Infusion therapy to correct dehydration, hypovolemia, electrolyte disturbances (LE A); . Stepwise pharmacotherapy according to paragraph 12, subparagraph 4, Table 18 of this protocol
VHB	. Not provided (LE A)
OZhPB	. Stabilization and maintenance of blood pressure< 160/105 мм рт. ст. (в/в гидралазин, лабеталол, сульфат магния 25% и другие одобренные препараты) (УД А) ; . After stabilization of blood pressure, in the absence of a threat to life during transportation - transfer to a third-level obstetric organization (LE A)
HELLP syndrome

Treatment (hospital)

TREATMENT AT THE STATIONARY LEVEL

Treatment tactics

provides regime measures, non-drug methods and pharmacotherapy.

Non-drug treatment: see ambulatory level.

Medical treatment:
Pharmacotherapy of NRB

1 line	. Pyridoxine 10–25 mg PO 3–4 times daily in combination with doxylamine 12.5 mg PO 3–4 times daily (LEL B)
2 line	. promethazine 12.5 mg orally or rectally every 4 hours or diphenhydramine 50–100 mg orally or rectally every 4–6 hours (LEV)
3 line	. in the absence of dehydration: metoclopramide 5–10 mg IM or orally every 8 hours (LE: A); Promethazine 12.5–25 mg IM orally or rectally every 4 hours (UD B) or trimethobenzamide 200 mg (after registration) rectally every 6–8 hours (UD-B); . if dehydrated: IV infusion, metoclopramide 5–10 mg IV every 8 hours (LE A) or promethazine 12.5–25 mg IV every 4 hours (LE A).
4 line	. + methylprednisolone (with caution) 16 mg IV every 8 hours for 3 days, tapered over 2 weeks or ondansetron 8 mg IV every 12 hours (UD-A);

In addition, adequate rehydration, correction of electrolyte imbalance (infusion therapy), parenteral nutrition (if indicated), as well as the appointment of thiamine (vitamin B1) at a dose of 100 mg per day are necessary in order to prevent Wernicke's encephalopathy.
In the case of preterm birth between 26 and 35 weeks of gestation, intramuscular administration of desamethasone at a dose of 6 mg IM every 12 hours No. 4 before delivery is justified in order to prevent fetal respiratory distress syndrome.
· Pharmacotherapy ICP: see point 9, sub-point 4, Table 10. In case of preterm birth between 26 and 35 weeks of gestation, intramuscular administration of dexamethasone at a dose of 6 mg / m every 12 hours No. 4 before delivery is justified in order to prevent fetal respiratory distress syndrome.
· Pharmacotherapy OZHDPB:
there is no specific treatment. An urgent (minutes, hours) delivery is required (LE A). In addition, the following activities are envisaged:
- correction of hypoglycemia (infusion of 5% dextrose) under the control of glucose levels until the restoration of liver function and the possibility of enteral nutrition (LE A);
- the introduction of blood products (according to indications) (LE A);
- correction of water-electrolyte imbalance (UD A);
- treatment of complications (AKI, tubular necrosis, HRS, DIC, multiple organ failure, and others) (LE A);
- plasma exchange, hemodiafiltration, plasmapheresis, albumin dialysis (requires further study) (LEC);
- continuation of intensive care and, possibly, transfer to LT centers, especially in cases of coagulopathy, PE, hypoglycemia, including postpartum (LE B) ;
- termination of pregnancy in accordance with established indications, see paragraph 12, subparagraph 5, Table 21 of this protocol (LE A);
In case of preterm birth between 26 and 35 weeks of gestation, intramuscular administration of dexamethasone at a dose of 6 mg IM every 12 hours No. 4 before delivery is justified in order to prevent fetal respiratory distress syndrome (LEA).
· Pharmacotherapy HELLP syndrome:
Immediate delivery (LEA) is considered the only effective way. In addition, the following activities are envisaged:
The introduction of thromboconcentrate:
− at the amount of Tc< 20 000/мм3 (УД А) ;
− or< 50 000/мм3 и планируемом кесаревом сечении или любом кровотечении (УД-А) .
Choice of anesthesia method:
− there is no consensus;
− In general, epidural anesthesia is contraindicated in the amount of Tc<75000/мм3 (УД С) .
During and after childbirth:
- during and during the first 24 hours after childbirth - magnesium sulfate 25% (4 g loading dose, then 2 g / h) in order to prevent seizures (LEA);
- close monitoring, especially in the first 48 hours after delivery, for possible pulmonary edema, renal and hepatic dysfunction;
- Laboratory values begin to improve 48 hours after delivery.

Termination of pregnancy in accordance with established indications, see paragraph 12, subparagraph 5, Table 21.
In the case of preterm birth between 26 and 35 weeks of gestation, intramuscular administration of dexamethasone at a dose of 6 mg IM every 12 hours No. 4 before delivery is justified in order to prevent fetal respiratory distress syndrome.

EML, EML (http://www.fda.gov)

INN
pyridoxine	Vitamins. Other vitamins in their pure form. ATH code: A11HA02	A
thiamine	Vitamins. Other vitamins in their pure form. ATX code: A11DA01	A
doxylamine	H1 antihistamines. Sleeping drugs. ATX code: N05CM	V
promethazine	Histamine H1 receptor blockers. Antiallergic drug. ATX code: R06AD02	WITH
diphenhydramine	Antihistamines for systemic use. Aminoalkyl ethers. Diphenhydramine. ATX code: R06A A02	V
metoclopramide	Centrally acting antiemetics that block dopamine receptors. ATX Code: A03FA01	V
methylprednisolone	ATX code: H02AB04	WITH
ondansetron	Antiemetics. Serotonergic agents. ATX Code: A04AA01	V
ursodeoxycholic acid	Drugs that affect liver function ATX code A05AA02	V
cholestyramine	Bile acid sequestrants. ATX Code: C10AC01	· WITH

List of additional medicines:

INN	Pharmacotherapeutic group	FDA Pregnancy Safety Category
solution of sodium chloride	Salt solutions. Sodium chloride. ATX code: B05CB01.	V
dextrose solution	Means of carbohydrate nutrition. ATX Code: B05BA03	WITH
solution of potassium chloride	Drugs that compensate for the deficiency of potassium in the body. ATX Code: B05XA01	WITH
solution of magnesium sulfate	Vasodilators. ATX code: B05XA05	D
albumin solution	Plasma substitutes. Human albumin preparations. ATX Code: B05AA01	C
FFP	Blood components
thromboconcentrate	Blood components
cryoprecipitate	Blood components
tocopherol	Vitamins. Other vitamins in their pure form. ATX code: A11HA03	A
retinol	Vitamins. Other vitamins in their pure form. ATX code: A11CA01	A
dexamethasone	Corticosteroids for systemic use. Glucocorticosteroids. ATX code: H02AB02	C
hydralazine	Antihypertensive drugs. Myolytics are straight. Hydrazinephthalazine derivatives. ATX code: C02DB02	WITH
mixtures for parenteral nutrition	Amino acids for parenteral nutrition + Other drugs [Fat emulsions for parenteral nutrition + Dextrose + Minerals] (Aminoacids for parenteral nutrition + Other medicines). ATX code: B05BA10	-

Surgical intervention: during a delivery operation, see the clinical protocol for the diagnosis and treatment of "Caesarean section". Indications for termination of pregnancy in associated liver disease are presented in Table 21.

Nosology	Indications for termination of pregnancy
NRB	. incessant vomiting; . increasing dehydration of the body; . progressive weight loss; . progressive acetonuria within 3-4 days; . severe intractable tachycardia; . dysfunction of the nervous system (adynamia, apathy, delirium, euphoria); . bilirubinemia (over 100 µmol/l).
VHB	. there is no consensus on the term of delivery with ICP; . more evidence for the advisability of delivery after 37 weeks (in the absence of obstetric indications for an earlier delivery).
OZhPB	. immediate delivery, regardless of the gestational age; . delivery method depends on the following factors: - fetal status: In many cases, asphyxia and hypoxia develop, therefore careful monitoring is necessary; - maternal coagulation status: The patient may need replacement of clotting factors before a planned caesarean section; - Probability of successful induction of labor: If labor cannot be safely induced within 24 hours of the diagnosis of AFLD, caesarean section is optimal.
HELLP syndrome	. for > 34 weeks: - Immediate delivery. . at term< 34 недель и стабильном состоянии матери и плода: - for a more complete gestation, a course of CS (betamethasone 12 mg / m every 24 hours) is possible twice with delivery 24 hours after the last dose with careful monitoring; - in case of signs of deterioration in the condition of the mother and fetus - immediate delivery. . if the patient is already in labour: - in the absence of signs of distress, coagulopathy, multiorgan dysfunction, renal failure, ruptures, natural childbirth is continued; - in the presence of these conditions - an urgent caesarean section.

Indications for expert advice

see ambulatory level.

Nosology	Indications for transfer to the intensive care unit and resuscitation
NRB	. weight loss;
VHB	. progressive growth of signs of cholestasis and cytolysis (increased ALT, AST, alkaline phosphatase, GGTP, gallstones); . treatment failure at the outpatient level.
OZhPB	. all pregnant women with suspected AFLD or established diagnosis of AFLD should be urgently hospitalized
HELLP syndrome	. all pregnant women with suspected HELLP syndrome and an established diagnosis of HELLP syndrome should be urgently hospitalized

Treatment effectiveness indicators see ambulatory level.

Prognosis of Liver Diseases Associated with Pregnancy:

Nosology	Mother's prognosis	Prognosis for the fetus (child)
NRB	. In general, outcomes = those in the general population with minimal complications (impaired CBS, electrolyte balance); . rarely severe complications: - rupture of the esophagus; - hemorrhages in the retina; - pneumothorax; - OPP; - Wernicke encephalopathy (more often occurs with alcoholism and leads to death in 10-20%).	. with timely treatment outcomes = those in the general population; . in severe cases, reduced weight; . sometimes premature birth; . possible risk of type 2 diabetes and CVD.
VHB	. in general, good ICP resolves spontaneously after delivery; . recurs in 45-70% of subsequent pregnancies; . low risk of persistence after childbirth and manifestation as a chronic disease	. in 30-44% sl. premature birth; . each 1 µmol/l increase in BH increases the risk of complications by 1-2%; . 11-20% of newborns have an increased risk of morbidity, including the development of RDS; . increased risk of perinatal death (3.5%).
OZhPB	cases of recovery before childbirth (delivery) are not described; In most cases, improvement in liver function occurs within 48-72 hours after delivery, but may take weeks or months; Previously, mortality was up to 70%, at present, with timely treatment (delivery) - 5-18%.	· 75% of preterm births with an average term of 34%; · Previously, the mortality rate was up to 85%, currently - 9-23%.
HELLP syndrome	. risk of death 1-3.5%; . diverse complications: pulmonary edema, AKI, DIC, placental rupture, liver failure, hematomas and liver ruptures, ARDS, retinal detachment, blood transfusion complications; . History of HELLP syndrome is a risk factor for preterm birth, preeclampsia (5-22%), recurrent HELLP syndrome (3-27%) in subsequent pregnancy, especially if diagnosed before 28 weeks of gestation, arterial hypertension (33%).	. the risk of death is 7.4-20%, depending on the gestational age (the highest is at the term<28 недель) и других факторов: Gestational Age from Estimated Date of Delivery); . Complications: RDS, bronchopulmonary dysplasia, HF hemorrhage, necrotizing enterocolitis.

Hospitalization

Indications for planned hospitalization:

Indications for emergency hospitalization:

Nosology	Indications for emergency hospitalization
NRB	. constant vomiting and intolerance to any liquids; . weight loss of more than 5% of the initial weight and / or the appearance of ketonuria, despite ongoing therapy; . changes in vital signs, mental status; . inefficiency of treatment at the outpatient level; . diagnosed vomiting of pregnant women, regardless of severity, in the presence of a concomitant disease requiring the appointment of antibacterial drugs.
VHB	. increase in signs of hepatocellular insufficiency (hypoalbuminemia, hypocoagulation); . progressive increase in indicators of cholestasis and cytolysis (ALT, AST, alkaline phosphatase, GGTP, gallstones); . treatment failure at the outpatient level.
OZhPB	. all pregnant women with diagnosis and suspicion of AFLD should be . urgently hospitalized
HELLP syndrome	. all pregnant women with diagnosed and suspected HELLP syndrome . must be urgently hospitalized

Information

Sources and literature

Minutes of the meetings of the Joint Commission on the quality of medical services of the MHSD RK, 2016
1. 1) The Management of Nausea and Vomiting of Pregnancy and Hyperemesis Gravidarum//Green-top Guideline No. 69, June 2016//Royal College of Obstetricians and Gynaecologists, NICE accredited. 2) Elisabeth Birkeland, Guro Stokke, Randi J. Tangvik, Erik A. Torkildsen, Jane Boateng, Anne L. Wollen, Susanne Albrechtsen, Hans Flaatten, Jone Trovik. Norwegian PUQE (Pregnancy-Unique Quantification of Emesis and Nausea). Identifies Patients with Hyperemesis Gravidarum and Poor Nutritional Intake: A Prospective Cohort Validation Study. PLOS ONE | DOI:10.1371/journal.pone.0119962. April 1, 2015. 3) Schutt VA and Minuk GY (2007) Liver diseases unique to pregnancy. Best Pract Res Clin Gastroenterol 21: 771-792. 4) Khulood T. Ahmed, Ashraf A. Almashhrawi, Rubayat N. Rahman, Ghassan M. Hammoud, Jamal A. Ibdah. Liver diseases in pregnancy: Diseases unique to pregnancy. World J Gastroenterol. November 21, 2013; 19(43): 7639-7646. ISSN 1007-9327 (print). ISSN 2219-2840 (online). 5) David Friedel, Stavros Stavropoulos, Shahzad Iqbal, Mitchell S Cappell. Gastrointestinal endoscopy in the pregnant woman. World J Gastrointest Endosc. 2014 May 16; 6(5): 156-167. ISSN 1948-5190 (online). 6) Kenya Kamimura, Hiroyuki Abe, Hirokazu Kawai, Hiroteru Kamimura, Yuji Kobayashi, Minoru Nomoto, Yutaka Aoyagi, Shuji Terai. Advances in understanding and treating liver diseases during pregnancy: A review. World J Gastroenterol. 2015 May 7; 21(17): 5183-5190. ISSN 1007-9327 (print) ISSN 2219-2840 (online). 7) The management of severe pre-eclampsia/eclampsia. RCOG Guideline No. 10(A) 2010. 8) Shashank Shekhar, Gaurav Diddi. Liver disease in pregnancy. Review Article. Taiwanese Journal of Obstetrics & Gynecology, 54 (2015), 475e482. 9) Howard Ernest Herrell. Nausea and Vomiting of Pregnancy. American Family Physician, Volume 89, Number 12, June 15, 2014. 10) South Australian Perinatal Practice Guidelines "Obstetric cholestasis". South Australian Maternal & Neonatal Community of Practice. Last Revised: 19/4/2016. 11) Iris J. Grooten, Margot E. Vinke, Tessa J. Roseboom and Rebecca C. Painter. A Systematic Review and Meta-Analysis of the Utility of Corticosteroids in the Treatment of Hyperemesis Gravidarum. Nutrition and Metabolic In sights 2015:8 (S1). 12) Tram T. Tran, Joseph Ahn, Nancy S. Reau. ACG Clinical Guideline: Liver Disease and Pregnancy. Am J Gastroenterol advance online publication, 2 February 2016; doi: 10.1038/ajg.2015.430. 13) Mario Festin. All rights reserved Nausea and vomiting in early pregnancy. BMJ Publishing Group Ltd 2014. 14) Jill Ablett. Acute fatty liver of pregnancy guideline (GL780). March 2016. 15) Amelia Tan, Therese Foran, Amanda Henry. Managing nausea and vomiting in pregnancy in a primary care setting. The Royal Australian College of General Practitioners, 2016. 16) Susanna Timonen et all. Turun Yliopiston Julkaisuja – Annales Universitatis Turkuensis Sarja - ser. D osa-tom. 1215. Medica-Odontologica. Painosalama Oy - Turku, Finland 2016. 17) Royal College of Obstetricians. Obstetric cholestasis. RCOG Green-top Guideline No. April 43, 2011. NICE accredited. 18) Ghassan M. Hammoud, Jamal A. Ibdah. Preeclampsia-induced Liver Dysfunction, HELLP 19) Syndrome, and Acute Fatty Liver of Pregnancy. Clinical Liver Disease, Vol 4, No 3, September 2014. 20) ACOG (American College of Obstetrics and Gynecology) Practice Bulletin: nausea and vomiting of pregnancy. Obstet Gynecol. 2004 Apr. 103(4):803-14. ; 21) Heinrichs L. Linking olfaction with nausea and vomiting of pregnancy, recurrent abortion, hyperemesis gravidarum, and migraine headache. Am J Obstet Gynecol. May 2002 186(5 Suppl Understanding):S215-9.. 22) Jewell D, Young G. Interventions for nausea and vomiting in early pregnancy. Cochrane Database Syst Rev. 2003.(4):CD000145. . 23) Kirsten J Sasaki, BS Anand. Liver Disease and Pregnancy. Medscape, updated: Aug 2015: http://emedicine.medscape.com/article/188143-overview#a1. 24) Spruill SC, Kuller JA. Hyperemesis gravidarum complicated by Wernicke's encephalopathy. Obstet Gynecol. 2002 May. 99(5 Pt 2): 875-7. . 25) Bolin M, Akerud H, Cnattingius S, Stephansson O, Wikstrom AK. Hyperemesis gravidarum and risks of placental dysfunction disorders: a population-based cohort study BJOG 2013 Apr 120(5):541-7 26) Veenendaal MV, van Abeelen AF, Painter RC, van der Post JA, Roseboom TJ Consequences of hyperemesis gravidarum for offspring: a systematic review and meta-analysis BJOG 2011 Oct 118(11):1302-13 27) Ayyavoo A, Derraik JG, Hofman PL, Biggs J, Bloomfield FH, Cormack BE Severe hyperemesis gravidarum is associated with reduced insulin sensitivity in the offspring in childhood J Clin Endocrinol Metab 2013 Aug 98(8):3263-8 28) Guntupalli SR and Steingrub J (2005) Hepatic disease and pregnancy: an overview of diagnosis and management Crit Care Med 33 (Suppl): S332-S339.29) Glantz A, Marschall HU, Mattsson LA Intrahepatic cholestasis of pregnancy: Relationships between bile acid levels and fetal complication rates. hepatology. 2004 Aug. 40(2):467-74. . 30) Arrese M and Reyes H (2006) Intrahepatic cholestasis of pregnancy: a past and present riddle. Ann Hepatol 5: 202-205. 31) Keitel V et al. (2006) Combined mutations of canalicular transporter proteins cause severe intrahepatic cholestasis of pregnancy. Gastroenterology 131: 624-629. 32) Reyes H et al. (2006) Is a leaky gut involved in the pathogenesis of intrahepatic cholestasis of pregnancy? Hepatology 43: 715-722; Hay JE (2008) Liver disease in pregnancy. Hepatology 47: 1067-1076. 33) Heikkinen J, Mäentausta O, Ylöstalo P, Jänne O. Serum bile acid levels in intrahepatic cholestasis of pregnancy during treatment with phenobarbital or cholestyramine. Eur J Obstet Gynecol Reprod Biol. Dec. 1982 14(3):153-62. . 34) Lee NM, Brady CW. Liver disease in pregnancy. World J Gastroenterol. 2009 Feb 28.15(8):897-906.. 35) Fidelma B Rigby, Ronald M Ramus. Intrahepatic Cholestasis of Pregnancy Workup. Medscape, updated Apr 23, 2014: http://emedicine.medscape.com/article/1562288-workup. 36) Gurung V, Middleton P, Milan SJ, Hague W, Thornton JG. Interventions for treating cholestasis in pregnancy. Cochrane Database Syst Rev. 2013.6:CD000493. . 37) Ribalta J, Reyes H, Gonzalez MC, Iglesias J, Arrese M, Poniachik J, et al. S-adenosyl-L-methionine in the treatment of patients with intrahepatic cholestasis of pregnancy: a randomized, double-blind, placebo-controlled study with negative results. hepatology. 1991 Jun. 13(6):1084-9. . 38) Palma J, Reyes H, Ribalta J, et al. Ursodeoxycholic acid in the treatment of cholestasis of pregnancy: a randomized, double-blind study controlled with placebo. J Hepatol. Dec. 1997 27(6):1022-8. . 39) Kondrackiene J, Beuers U, Kupcinskas L. Efficacy and safety of ursodeoxycholic acid versus cholestyramine in intrahepatic cholestasis of pregnancy. gastroenterology. 2005 Oct. 129(3):894-901. . 40) Alsulyman OM, Ouzounian JG, Ames-Castro M, Goodwin TM. Intrahepatic cholestasis of pregnancy: perinatal outcome associated with expectant management. Am J Obstet Gynecol. 1996 Oct. 175(4 Pt 1):957-60. . 41) Jain R, Suri V, Chopra S, Chawla YK, Kohli KK. Obstetric cholestasis: outcome with active management. J Obstet Gynaecol Res. May 2013 39(5):953-9. . 42) Wikstrom Shemer E, Marschall HU, Ludvigsson JF, Stephansson O. Intrahepatic cholestasis of pregnancy and associated adverse pregnancy and fetal outcomes: a 12-year population-based cohort study. BJOG. May 2013 120(6):717-23. . 43) Rajasri AG, Srestha R, Mitchell J. Acute fatty liver of pregnancy (AFLP)--an overview. J Obstet Gynaecol. 2007 Apr. 27(3):237-40. . 44) Ahmed KT, Almashhrawi AA, Rahman RN, Hammoud GM, Ibdah JA. Liver diseases in pregnancy: diseases unique to pregnancy. World J Gastroenterol. 2013 Nov 21.19(43):7639-46. . 45) Bellig L.L. Maternal acute fatty liver of pregnancy and the associated risk for long-chain 3-hydroxyacyl-coenzyme a dehydrogenase (LCHAD) deficiency in infants. Adv Neonatal Care. Feb. 2004 4(1):26-32. . 46) Ibdah J.A. Acute fatty liver of pregnancy: an update on pathogenesis and clinical implications. World J Gastroenterol. 2006 Dec 14.12(46):7397-404. . 47) Benjaminov FS, Heathcote J. Liver disease in pregnancy. Am J Gastroenterol. Dec 2004 99(12):2479-88. 48) Michael J Barsoom, Ronald M Ramus. Acute Fatty Liver of Pregnancy. Medscape, updated Jan 15, 2015: http://emedicine.medscape.com/article/1562425-overview. 49) Vigil-de Gracia P, Montufar-Rueda C. Acute fatty liver of pregnancy: diagnosis, treatment, and outcome based on 35 consecutive cases. J Matern Fetal Neonatal Med. 2011 Oct. 24(9):1143-6. . 50) Jamjute P, Ahmad A, Ghosh T, Banfield P. Liver function test and pregnancy. J Matern Fetal Neonatal Med. March 2009 22(3):274-83. . 51) Sibai BM. Imitators of severe pre-eclampsia. Semin Perinatol. Jun. 2009 33(3):196-205. . 52) Nelson DB, Yost NP, Cunningham FG. Acute fatty liver of pregnancy: clinical outcomes and expected duration of recovery. Am J Obstet Gynecol. Nov. 2013 209(5):456.e1-7. . 53) Browning MF, Levy HL, Wilkins-Haug LE, Larson C, Shih VE. Fetal fatty acid oxidation defects and maternal liver disease in pregnancy. Obstet Gynecol. Jan. 2006 107(1):115-20. . 54) Martin JN Jr, Briery CM, Rose CH, Owens MT, Bofill JA, Files JC. Postpartum plasma exchange as adjunctive therapy for severe acute fatty liver of pregnancy. J Clin Apher. 2008.23(4):138-43. . 55) Jin F, Cao M, Bai Y, Zhang Y, Yang Y, Zhang B. Therapeutic effects of plasma exchange for the treatment of 39 patients with acute fatty liver of pregnancy. Discov Med. May 2012 13(72):369-73. . 56) Chu YF, Meng M, Zeng J, Zhou HY, Jiang JJ, Ren HS, et al. Effectiveness of combining plasma exchange with continuous hemodiafiltration on acute Fatty liver of pregnancy complicated by multiple organ dysfunction. Artif Organs. Jun. 2012 36(6):530-4. . 57) Knight M, Nelson-Piercy C, Kurinczuk JJ, Spark P, Brocklehurst P,. A prospective national study of acute fatty liver of pregnancy in the UK. gut. 2008 Jul. 57(7):951-6. ; 58) Sibai BM. Imitators of severe preeclampsia. Obstet Gynecol. 2007 Apr. 109(4):956-66. 59) Audibert F, Friedman SA, Frangieh AY, Sibai BM. Clinical utility of strict diagnostic criteria for the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. Am J Obstet Gynecol. 1996 Aug. 175(2):460-4. . 60) Martin JN Jr, Blake PG, Lowry SL, Perry KG Jr, Files JC, Morrison JC. Pregnancy complicated by preeclampsia-eclampsia with the syndrome of hemolysis, elevated liver enzymes, and low platelet count: how rapid is postpartum recovery?. Obstet Gynecol. Nov. 1990 76(5 Pt 1):737-41. . 61) Joshi D, James A, Quaglia A, Westbrook RH, Heneghan MA. Liver disease in pregnancy. Lancet. 2010 Feb. 13. 375(9714):594-605. . 62) Burwick RM, Feinberg BB. Eculizumab for the treatment of preeclampsia/HELLP syndrome. Placenta. Feb. 2013 34(2):201-3. ; Geary M. The HELLP syndrome. Br J Obstet Gynaecol. 1997 Aug. 104(8):887-91. . 63) Woudstra DM, Chandra S, Hofmeyr GJ, Dowswell T. Corticosteroids for HELLP syndrome in pregnancy. Cochrane Database Syst Rev. 2010. (9): CD008148.. 64) Habli M, Eftekhari N, Wiebracht E, et al. Long-term maternal and subsequent pregnancy outcomes 5 years after hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome. Am J Obstet Gynecol. 2009 Oct. 201(4):385.e1-5. .

Information

Abbreviations used in the protocol

AG	arterial hypertension
HELL	arterial pressure
YAG	autoimmune hepatitis
ALT	alanine aminotransferase
Anti-TG	antibodies to thyroglobulin
Anti-TSH	thyroid-stimulating hormone receptor antibodies
ASG	alimentary steatohepatitis
AST	aspartate aminotransferase
TANK	blood chemistry
BVK	Wilson-Konovalov disease
VG	viral hepatitis
VHB	intrahepatic cholestasis of pregnancy
HF	intracranial
GGTP	gamma-glutamyl transpeptidase
GM	brain
GSP	main pancreatic duct
GDS	hepatorenal syndrome
MS	hypersensitivity
ICE	disseminated intravascular coagulation
DNA	Deoxyribonucleic acid
DPK	duodenum
LCD	fatty acid
cholelithiasis	cholelithiasis
gastrointestinal tract	gastrointestinal tract
JCHK	bile acids
BMI	body mass index
KOS	acid-base state
KS	corticosteroids
CT	CT scan
LDH	lactate dehydrogenase
LIPP	drug-induced liver injury
INR	international normalized ratio
MRI	Magnetic resonance imaging
MRCP	magnetic resonance cholangiography
NASH	non-alcoholic steatohepatitis
NSAIDs	non-steroidal anti-inflammatory drugs
NRB	uncontrollable vomiting of pregnant women
UAC	general blood analysis
OAM	general urine analysis
OBP	abdominal organs
OVG	acute viral hepatitis
OZhPB	acute fatty degeneration of the liver of pregnant women
ONMK	acute cerebrovascular accident
OPP	acute kidney injury
ARDS	acute respiratory distress syndrome
BCC	circulating blood volume
PBC(X)	primary biliary cirrhosis (cholangitis)
PV	prothrombin time
pancreas	pancreas
PSH	primary sclerosing cholangitis
PCR	polymerase chain reaction
Pch	hepatic
PE	hepatic encephalopathy
RDS	respiratory distress syndrome
RCT	randomized controlled trials
Solution	solution
SD	diabetes
FFP	fresh frozen plasma
SLE	systemic lupus erythematosus
coolant	mucous membrane of the stomach
SRP	C-reactive protein
CVD	cardiovascular diseases
T4	thyroxine
TP	liver transplant
TSH	thyroid-stimulating hormone
Tts	platelets
TCP	thrombocytopenia
TCPP	thrombocytopenic purpura
UDCA	ursodeoxycholic acid
UZDG	doppler ultrasound
ultrasound	ultrasonography
FPN	fulminant liver failure
FPP	liver function tests
CVH	chronic viral hepatitis
CG	chronic gastritis
CPU	cirrhosis of the liver
thyroid	thyroid
AP	alkaline phosphatase
EGDS	esophagogastroduodenoscopy
ECG	electrocardiogram
EEG	electroencephalography
I WOULD	peptic ulcer
ANA	antinuclear antibodies
ANCA	antineutrophil cytoplasmic antibodies
Anti-HAV IgM	IgM antibodies to hepatitis A virus
Anti-HCV	antibodies to hepatitis C virus antigens
Anti-HEV Ig M	IgM antibodies to hepatitis E virus
ASMA	anti-smooth muscle antibodies
FDA	Food and Drug Administration (Food and Drug Administration)
Hb	hemoglobin
HbA1C	glycated hemoglobin
HBsAg	hepatitis B surface antigen
HCVRNA	hepatitis C virus ribonucleic acid
HELLP	Hemolysis (hemolysis), Elevatedliver enzymes (increased activity of liver enzymes), Low platelet count (decrease in platelet count)
ht	hematocrit
HBV DNA	hepatitis B virus deoxyribonucleic acid
Lbx	liver biopsy
LKM	antibodies to liver microsomes
MRCP	magnetic resonance cholangiopancreatography
pANCA	perinuclear antineutrophil cytoplasmic antibodies
SLA	antibodies to soluble liver antigen
ULN	upper limit of normal
AMA/AMA-M2	antimitochondrial antibodies antineutrophil cytoplasmic antibodies antineutrophil cytoplasmic antibodies
HP	Helicobacter pylori
	rise, increase
↓	decrease, decrease

List of protocol developers indicatingqualification data:

1) Nersesov Alexander Vitalievich - Doctor of Medical Sciences, Professor, Head of the Department of Gastroenterology and Hepatology of the Research Institute of Cardiology and Internal Diseases of the Ministry of Health and Social Development of the Republic of Kazakhstan, Chairman of the Kazakh Association for the Study of the Liver, member of EASL, AASLD, APASL, WHO Expert Committee, Almaty, Kazakhstan;
2) Kaliaskarova Kulpash Sagyndykovna - Doctor of Medical Sciences, Head of the Center for Hepatology and Gastroenterology JSC "National Scientific Center of Oncology and Transplantology" CF "UMC", Chief Freelance Gastroenterologist-Hepatologist of the MHSD RK, Deputy Chairman of the Kazakh Association for the Study of the Liver, member of EASL , Astana, Kazakhstan;
3) Doskozhaeva Saule Temirbulatovna - Doctor of Medical Sciences, Professor, Vice-Rector, Head of the Department of Infectious Diseases of the Kazakh Medical University of Continuing Education, Almaty, Kazakhstan;
4) Bazylbekova Zeynep Omirzakovna - Doctor of Medical Sciences, Head of the Pathology of Pregnancy Department of the Scientific Center for Obstetrics and Gynecology of the Ministry of Health and Social Development of the Republic of Kazakhstan, Almaty, Kazakhstan;
5) Bedelbayeva Gulnara Gabdualievna - Doctor of Medical Sciences, Professor, Head of the Department of Therapy of the Institute of Postgraduate Education of the Kazakh National Medical University. S.D. Asfendiyarov, Almaty, Kazakhstan;
6) Jumabayeva Almagul Yerkenovna - Master of Medicine, Assistant of the Department of Gastroenterology and Hepatology of the Research Institute of Cardiology and Internal Diseases of the Ministry of Health and Social Development of the Republic of Kazakhstan, Secretary of the Kazakh Association for the Study of the Liver, member of EASL, Almaty, Kazakhstan
7) Kaliyeva Mira Maratovna - Candidate of Medical Sciences, Head of the Department of Clinical Pharmacology and Pharmacotherapy, KazNMU named after. S.D. Asfendiyarov.

Indication of no conflict of interest: no.

List of reviewers:
Palgova L. K. - Doctor of Medical Sciences, Professor of the Department of Propaedeutics of Internal Diseases, Gastroenterology and Dietetics, North-Western State Medical University. I.I. Mechnikova, member of EASL, APASL, St. Petersburg, Russia;

Revision terms: revision of the protocol 3 years after its publication and from the date of its entry into force or in the presence of new methods of diagnosis and treatment with a level of evidence.

Attached files

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The period of pregnancy is marked for a woman not only by the joy of bearing a child, but also by the risk of various diseases. Organ systems do not cope with their functions due to the increasing weight of the expectant mother and the constant consumption of nutrients by the fetus. The most significant problems occur with the liver, and one of the most common among them is hepatosis. This article will discuss the causes of this disease, its symptoms, the principles of treatment, diagnosis and the basics of prevention.

What is this disease

Hepatosis is the accumulation of fatty inclusions in hepatocytes - liver cells and, in the absence of timely treatment, is accompanied by degradation of hepatocytes. Inflammatory processes do not occur. There are several types of this pathology, such as fatty and cholestatic. Each species has its own code in the International Classification of Diseases. Hepatosis code according to ICD 10 - K70.

What is dangerous during pregnancy: consequences for the child

The impact of this disease on both the expectant mother and the developing fetus cannot be underestimated. Most often, hepatosis leads to such complications:

oxygen and placental starvation of the fetus, accompanied by delays in its development;
birth suffocation of the baby, pulmonary insufficiency in the postpartum period;
stimulation of premature birth, most often at the beginning of the third trimester and, as a result, prematurity of the baby;
the appearance of hematomas, vascular network on the skin of the fetus;
intrauterine death. Most characteristic of the fatty form of the disease.

Important! In the acute form of fatty hepatosis, natural childbirth is fraught with intense bleeding. Even if you managed to stabilize your condition and bring the pregnancy to the end of the ninth month, agree to a caesarean section to avoid possible complications.

Reasons for development

The main cause of hepatosis is pregnancy itself, or rather the processes that accompany it:

hormonal changes. An increase in the concentration of estrogens in the blood provokes rapid weight gain, thickening of bile and deterioration in the patency of the biliary tract;
increase in fetal weight. The amniotic sac with water puts pressure on the liver, it is partially deformed, the outflow of bile worsens and the rate of cell recovery decreases;
chronic liver or kidney disease, in particular - liver failure;
inflammatory processes in the abdominal cavity. Occur due to injuries or poor-quality surgical interventions;
haemorrhoids. Since this pathology is accompanied by a decrease in the tone of the intestinal blood vessels, the blood supply to the liver also worsens;
genetic predisposition. Hepatosis often occurs in women whose close relatives suffer from this disease;
wrong diet. An abundance of fatty, fried foods, meat dishes and animal fats is bad for the health of liver cells;
hypervitaminosis. Many women during pregnancy begin uncontrolled intake of vitamin complexes, thereby disrupting the functioning of the liver.

Symptoms

Different types of this pathology are characterized by different symptoms.

cholestatic

This form of hepatosis is characterized by the flow of a large amount of bile into the bloodstream. Her symptoms:

intense itching. The bile pigment bilirubin irritates nerve receptors, resulting in an itchy sensation. It tends to intensify at night. In some pregnant women, scratching becomes uncontrollable and they scratch themselves until they bleed;
jaundice. Appears three to four weeks after the onset of itching. It occurs on average in 20% of pregnant women. It is characterized by yellowing of the whites of the eyes and skin;
dry skin of the face, the appearance of acne and fine wrinkles;
weakness, sleep disturbance, lethargy, which is accompanied by a violation of appetite and nausea;
light stools. The color is disturbed due to insufficient flow of bile into the digestive tract;
dark color of urine. Occurs due to the utilization of excess bile pigment through the urinary system;
change in blood composition. It is determined using a general analysis, shows an increase in the amount of bilirubin, cholesterol, cholestasis.

Did you know? The liver has a unique ability to regenerate. If a person decides to become a donor and donate 75% of this organ to someone, then the liver restores its natural size in two weeks after the operation! The regeneration rate is amazing, considering that this is the heaviest internal organ. On average, its weight is 1.2 kg- exactly the same weight as a large chihuahua.

Fatty liver disease

It proceeds in four stages almost asymptomatically, in the absence of treatment it transforms into cirrhosis. The zero stage is not expressed, it is treated by changing the lifestyle. Symptoms:

heaviness in the right hypochondrium, radiating to the lower back, shoulder, shoulder blade and left half of the body;
bitter taste on the root of the tongue, yellow coating on the tongue and tonsils;
bowel disorders such as excessive gas formation, disorders, nausea;
an increase in the liver with its protrusion from the hypochondrium, swelling of neighboring soft tissues;
an increase in adipose tissue on the internal organs of the abdominal cavity.

Diagnostics

It is extremely difficult to determine this disease during pregnancy due to the enlarged uterus and the inability to perform procedures such as palpation and elastometry. Most often, the diagnosis is based on the indicators of the general blood test and the patient's complaints. Sometimes an ultrasound of the liver, a general urinalysis and a study of the condition of the fundus are additionally prescribed. The cholestatic form is easier to diagnose than the fatty form.

Treatment

During pregnancy, treatment is carried out by adjusting the diet, herbal preparations, and less often - pharmacy medicines.

Medicines

Treatment is carried out in three directions - symptoms are eliminated, placental blood supply to the fetus is stimulated, and abortion is prevented.

At an early stage of the disease, sorbents, antioxidants, hepatoprotectors, drugs with a choleretic effect, and drugs to reduce the acidity of gastric juice are prescribed.
If hepatosis has developed into a more severe form, detoxification of the body is additionally carried out, folic acid, antipruritic drugs and cholestyramine are prescribed. In extreme cases, the patient is prescribed plasmapheresis - hardware blood purification.

Important! Aching pain in the right hypochondrium can be a sign not only of hepatosis, but also of a malfunction of the gallbladder, such as cholecystitis. Duodenal sounding is the only possible way to distinguish one disease from another, it is rarely performed due to the possible stimulation of preterm labor.

They include herbal decoctions with choleretic, antioxidant and restorative effects. For the manufacture of decoctions, herbal preparations are used, so before buying a collection, check the composition for strong allergens. If you plan to cook the collection yourself, then use the following recipe.
Ingredients:

licorice - 20 g;
sequence leaves - 15 g;
birch leaves - 15 g;
sage leaves - 10 g;
wormwood - 10 g;
chamomile - 5 g;
linden - 5 g;
calamus root - 5 g.

Cooking method:

Mix and grind all the ingredients of the collection.
Bring 1 liter of water to a boil in an enamel saucepan.
Add 15 g of the collection to the saucepan. Cover the lid with a saucepan and simmer the collection over low heat for twenty minutes.
Remove the saucepan from the heat, wrap it in a blanket and leave to infuse for an hour and a half.
Strain the finished broth through gauze, pour into a ceramic container. Take a decoction of 50 ml twice a day for three weeks, then take a week-long break and repeat the course if necessary.

Did you know? At the eighth week of intrauterine development, the liver makes up 50-60% of the body weight of the fetus. For the entire period from its formation to the birth of a baby, it pumps over 20,000 liters of blood through itself. If this organ did not exist, the baby would get poisoned from every product drunk and eaten by his mother.. It is not for nothing that the Lezgins and some peoples of Africa believe that it is in the liver that the soul of a person is enclosed, and they never eat the liver of animals for food.

Another collection with pronounced cleansing properties is prepared as follows.

Ingredients:

licorice root - 20 g;
birch leaves - 20 g;
rosehip - 15 g;
hawthorn - 15 g;
rowan - 15 g;
lingonberries - 10 g;
nettle leaves - 10 g;
marshmallow root - 10 g;
St. John's wort - 5 g.

Cooking method:

Mix and grind all ingredients. Boil 1.5 liters of water, add 30 g of the collection to boiling water.
Cover the container with water with a lid and put it in a warm place for two hours.
Strain the finished infusion through a strainer into a glass container and close it hermetically for the period of storage.
Use the infusion twice a day for 100 ml for two weeks or until the symptoms are completely eliminated.

Diet

The therapeutic diet called table number 5 provides for the complete exclusion of products containing animal fats, fatty meat, fast food. To reduce stress on the liver, avoid:

smoked meats;
conservation;
salty foods;
and other store-bought sweets;
alcoholic drinks;
tea and tea drinks.

Eat as little as possible foods that provoke gas formation - white bread, legumes, muffins, mushrooms.

Important! Hepatosis has a negative impact mainly on the baby. A young mother gets rid of this disease in one to one and a half weeks after childbirth, and the child may suffer from its consequences for many years. To prevent this from happening, be attentive to yourself and seek medical help at the first deviations from normal health.

The diet can include:

Prevention measures

To prevent the development of this disease, follow these recommendations:

First of all, control your diet. Eat foods rich in fiber and low in fat. Do not get carried away with sweets, canned food, smoked meats;
lead an active lifestyle. Sign up for a pool or yoga for pregnant women, attend classes regularly. If this is not possible, walk more often in the fresh air;
get rid of bad habits. Stick to a daily routine so that the body can prepare for sleep and produce enzymes to digest food at the right time;
avoid stressful situations conflict as little as possible. If you are worried about the upcoming birth, talk to someone close to you or make an appointment with a psychologist.

Hepatosis in any of its forms begins to develop in pregnant women in the third trimester of gestation. This pathology affects the liver cells and interferes with the normal functioning of the organ. There are symptoms specific to each type of disease.

Did you know? The world's first liver transplant was performed in the 1960s at an American university in Colorado. The operation was successful and the patient recovered. Unfortunately, medicine at that time was still underdeveloped. Due to improperly prescribed immunosuppressive drugs, which were supposed to protect the transplanted organ from rejection, the patient died a few weeks after the operation. Nowadays, more than 8 thousand such operations are performed annually.

Timely diagnosis and competent medical intervention will help stop the course of the disease and reduce its impact on the baby. If your liver is functioning normally, keep it working with proper nutrition and moderate physical activity - this way you will avoid the occurrence of hepatosis.

Hundreds of suppliers bring hepatitis C medicines from India to Russia, but only M-PHARMA will help you buy sofosbuvir and daclatasvir, while professional consultants will answer any of your questions throughout the therapy.

Cholestasis is a violation of the flow or formation of bile due to a pathological process localized in any area from the sinusoidal membrane of the hepatocyte to the nipple of Vater.

ICD-10 K71.0

general information

In this case, there is a decrease in hepatic excretion of water, bilirubin, bile acids and the accumulation of bile in hepatocytes and bile ducts; retention and accumulation in the blood of components excreted in bile. The terms "cholestasis" and "mechanical jaundice" are not synonymous: in many cases of cholestasis, there is no mechanical blockage of the biliary tract.

Clinical picture

The disease is characterized by: pruritus (not always); steatorrhea and diarrhea (due to a decrease in the level of bile acids in the intestine and impaired digestion of fats), “night blindness”, osteomalacia, osteoporosis and bone fractures, petechiae, spontaneous hemorrhages, increased thrombin time, muscle weakness (impaired absorption of fat-soluble vitamins A, D, K , E), skin xanthomas and xanthelasma, increased blood levels of bilirubin, alkaline phosphatase, more than 3 times higher than normal, GGTP, total cholesterol, phospholipids, LDL, TG; in urine - conjugated bilirubin, urobilinogen.
Prolonged cholestasis leads to the formation of primary biliary cirrhosis of the liver.

Diagnostics

Questioning - an indication in the anamnesis of signs of diseases that can cause cholestasis (cholelithiasis, tumor formations, inflammation of the biliary system, medication).
examination - petechial rashes of xanthoma and xanthelase; yellowness of the skin is possible.
Laboratory research
Mandatory:
complete blood count - the appearance of target erythrocytes, an increase in the surface area of erythrocytes; anemia, neutrophilic leukocytosis;
urinalysis - conjugated bilirubin, urobilinogen;
blood bilirubin - increase;
blood enzymes - ASAT, ALT, GGTP, alkaline phosphatase;
total cholesterol and its fractions;
TG;
albumins and globulins of blood;
prothrombin time.
If there are indications:
bacteriological examination of a blood culture (if sepsis is suspected);
coagulogram;
markers of hepatitis viruses A, B, C, D.
Instrumental research methods
Mandatory:
Ultrasound of the abdominal organs (determination of the state of the bile ducts, the size and condition of the parenchyma of the liver and spleen; size, shape, wall thickness; the presence of stones in the gallbladder and bile ducts).
If there are indications:
ERCP (CHCHG);
targeted percutaneous puncture biopsy of the liver (determination of the morphological substrate of the disease).
Expert advice
Mandatory:
Not shown.
If there are indications:
infectious disease specialist - if markers of the hepatitis virus are detected;
surgeon - with extrahepatic cholestasis;
oncologist.

Treatment

Pharmacotherapy
Standard:
As an addition to the treatment of the underlying disease that caused the development of cholestasis:
cholestyramine - 4 g 2-3 times a day;
urosodeoxycholic acid - 13-15 mg / kg per day;
ondasetron - 1 tab. 2 times or parenterally 1 ml (reduction of itching).
If there are indications:
ademetionine - in / m or / in 400-800 mg / day;
fat-soluble vitamins (orally): vitamin K - 10 mg / day; A - 25000 IU / day; D 400-4000 IU / day;
calcium in the form of skimmed milk or dietary supplements.
Surgery
With obstruction of the bile ducts - endoscopic sphincterotomy, nasobiliary drainage, stenting, surgical treatment.