Signs of liver problems in a child. Trace elements and disorders associated with vitamins. Recognize specific nosological units

This house stands on the right bank, and the largest digestive gland lives in it - the Liver.
She works day and night: she accumulates nutrients and vitamins, produces bile, which promotes the breakdown and absorption of fats, takes part in the metabolic process, in the synthesis of blood tanks, and regulates blood clotting factors. Lives, year after year becomes older, weighs more. The liver is very homely: it loves well-cooked food and does not accept drugs and alcohol, but it also does not like chemical agents very much. When the Liver gets angry and sick, then it is bad for the whole house, for all its inhabitants.

1. The child has a slight yellowness of the skin, and on palpation, a slightly enlarged edge of the liver is felt, there is slight soreness in the right side. You are upset and would not even want to think that the marked signs indicate a disease, although, alas, this is not excluded. Liver disease occupies one of the first places among diseases gastrointestinal tract in children 3-7 years old. The main reasons are irregular meals, changes in the quality of food products, the appearance of allergens in food and water, psycho-emotional overstrain.
2. You came home from work and, by moving the bundles and pots in the refrigerator, you realized that the soup was not warmed up, and the boiled condensed milk and a piece of sausage were eaten. Your child is in the 3rd grade, which means that the student has been eating dry food for the third year. You have often noticed his frequent belching. This is a sign that the liver is out of order. Obviously, you need to keep a diary of the meal report or provide for forced control measures.
3. Mom was alerted that a 3-year-old baby slept only on her left side or on her back. Mom remembered that even earlier the girl often cried when she was laid on the right side. AT medical institution functional changes in the liver were diagnosed. The reason was the early transfer of the girl to artificial and mixed feeding.
4. Returning from the kindergarten, the son was happy to eat pasties (from the kiosk). It turned out that in kindergarten the boy never ate anything. Why? Because he is not used to eating porridge, mashed potatoes, soup, pasta. At home, the “road” way of eating prevailed: what could be eaten right there was bought. In fact, all parents can be advised: while the child is small, at least cook properly for him (the first course, fruits, salads). Start keeping a food diary, find out what the baby ate today in kindergarten, at school, and what at home.
5. Keep records where you mark important little things and nuances. For example: on what date did quarantine for the virus start in kindergarten; what additional tasks the child was loaded with after kindergarten, school; how he reacted to the remark whether he had a seasonal malaise; how the intestines work. All this is very important for the timely recognition of liver diseases. The child may complain of nausea, belching, dryness and bitterness in the mouth. At the same time, during pain in the right hypochondrium, a yellow stool is noted, the head hurts, the mood is depressed, and fatigue quickly comes. These are all signs of liver disease.

Wheatgrass - A WEED OR A MEDICINE?

It heals and strengthens the liver, so it’s inconvenient to somehow call it a weed. 1 teaspoon of couch grass, wild rose and nettle is poured into a glass of water, boiled, cooled, filtered and taken half a glass half an hour before meals and at night. The decoction is useful for both children and adults, it is not only curative, but also a good prophylactic. If your child has (or has had) liver and gallbladder disorders, be sure to use this recipe.

Drink without limits

Another infusion is also useful for regulating the activity of the liver: 300 g of grass and 50 g of immortelle flowers are poured with two glasses of boiling water and left to infuse overnight. In the morning, filter and give to drink instead of water, without restrictions.

Children's fee

But the collection, which in traditional medicine has long been considered for children: 200 g of St. John's wort, knotweed, calendula flowers, 15 g of buckthorn bark and 5 g chamomile. Don't let "grams" confuse you, because the proportion of herbs taken for the infusion is important. Take 2 tablespoons of this collection, pour 0.5 liters of cold water and put it overnight. In the morning, after boiling for 5-7 minutes, insist for half an hour in a warm place, then strain. The resulting infusion should be drunk in several doses.

Symptoms of liver disease are similar to those of acute gastrointestinal diseases and an attack of appendicitis. Here, the named decoctions will not help - an urgent need is needed. health care. Therefore, it is better to refuse self-diagnosis and treatment.

BE AFRAID OF OILY DONUTS

At the first symptoms of the disease (dull It's a dull pain in the right side, heartburn, lack of appetite) a warm heating pad on the right side for 1.5-2 hours before bedtime for three days can help, as well as acupressure in the area of ​​the gallbladder (under the last rib vertically down from the right nipple). Plus cleansing enemas at night 2-3 times a week. It is desirable that the child sits quietly with his right leg tucked under him twice or thrice a day. This posture promotes the flow of bile.

WE WILL STRIKE FROM TWO FRONTS

Diseases in children associated with abnormal liver function can occur for various reasons. In any case, they pose a serious threat to the health and development of the child.

The liver is one of the main organs, the work of which affects all the others. It is responsible for blood formation, tissue nutrition with substances necessary for building cells, for the removal of toxic substances from the body.

Causes of liver disease in children

Deviation in the work of this body in children can occur for the following reasons:

The body of the child quickly reacts to any discomfort. Slight hypothermia, unusual food, stress can cause fever, indigestion, mood changes and other signs. Such symptoms should not be ignored. They can cause serious illness.

The following deviations should attract the attention of parents:

Symptoms of the disease - yellowing of the skin and whites of the eyes

• Yellowing of the skin and whites of the eyes;
• Complaints of pain in the right side;
• Temperature rise;
• Lack of appetite;
• Drowsiness and decreased activity;
• Nausea, vomiting;
• intestinal disorder;
• Chair white;
• Darkening of the urine.

Symptoms may be relieved, especially if colds. This can confuse even a doctor. As a rule, he appoints the delivery of urine and blood tests. By the color of urine, even visually, one can suspect a liver disorder in a child. General analysis blood will show the content of the dye bilirubin produced by the liver. The level of hemoglobin in healthy child is normal or slightly low. In hepatitis, the hemoglobin content is increased. Blood clotting also depends on the condition of the liver. Reduced clotting may be a sign of illness. The number of erythrocytes and platelets also changes. After examining the results of the tests, the doctor decides whether to send the child for a more complex examination. Urinalysis will show the concentration of bilirubin, the presence of toxic compounds, an increased content of leukocytes, indicating an inflammatory process.

Liver diseases possible in children

Liver disease of varying severity can occur in children of any age. Such forms of abnormalities as neonatal jaundice, moderate hepatomegaly may have physiological causes. Severe lesions can occur with the following diseases:

Due to the natural underdevelopment of the biliary tract, which is possible in the prenatal period, the liver of newborns may not immediately adapt to normal conditions. There is a so-called physiological jaundice. The cause of yellowing of the body and eyes in newborns may be breastfeeding if the mother has fatty milk. Usually, this jaundice is not dangerous until two weeks of age, it goes away on its own. If jaundice does not go away after 2 weeks, then the cause may be a disease. The child should be examined and, if the disease is confirmed, treated, paying attention to the slightest changes in the condition. A complication can be very dangerous, as it usually occurs suddenly (it is also called “nuclear jaundice”), leading to the death of the child.

Jaundice in an infant can also occur with a change in the nutrition of a nursing mother. The body can react to fruits, milk. This condition occurs with lactose intolerance or fructose intolerance. In order to exclude an anomaly in an infant, it is enough for the mother to follow a diet. Which product causes such a reaction in the child's body, tests will prompt.

A severe form of neonatal jaundice is called "nuclear jaundice"

Sometimes the cause of jaundice in a child can be a hereditary disorder of the circulation of bile in the body (familial cholestasis). Most often this disease occurs at the age of 1 month. A sign of insufficient formation of bile in this case is a white stool of a heterogeneous composition with brown patches.

A common cause of childhood jaundice is the presence of infectious diseases in the mother during pregnancy and childbirth. These can be infections in the urinary organs, gynecological diseases, herpes, syphilis, rubella, tuberculosis and others.

For severe illnesses infants includes biliary atresia - obstruction of the bile ducts. This disease is practically not treated, a liver transplant is required. A difficult operation can have life-threatening complications (cirrhosis and others).

Deviations can also occur if a woman uses drugs, alcohol, or smokes during pregnancy or breastfeeding. The child can get toxic hepatitis.

Diseases in older children

Before the age of 5-7 years, children may develop a mild enlargement of the liver (hepatomegaly), which is most often caused by physiological reasons, occurs due to the gradual development of the biliary system in children's body. It may not manifest itself. In some cases, jaundice and mild malaise may appear. In such cases, the child does not need treatment, he only needs diet food - low-fat, unsalted food, moderate use sweets. The diet should include fruits, vegetables, cottage cheese and dairy products. Such nutrition supports the immune system, promotes tissue repair.

A common disease in adolescents is toxic injury hepatic tissue that occurs due to alcohol intake, addiction to drugs and other bad habits.

Infection with viral hepatitis A or B can occur through dishes or hygiene items shared with a sick relative. Infection with viral hepatitis C can be the result of an accidental contact of the blood of virus carriers with medical instruments in the dentist's office, and can also occur during surgical operations, intravenous drug infusion. Acute manifestations are jaundice, skin itching, hepatomegaly, fatigue, nervous disorders, up to loss of consciousness and coma. If the child loses weight rapidly, his stomach hurts with right side, there is nausea and vomiting, then it must be shown to the doctor as soon as possible.

If you suspect a disease - an urgent examination laboratory methods

Diagnostics and treatment of liver diseases in children

If there is a suspicion of a disease, an urgent examination by laboratory methods is necessary. At the same time, it is carried out: general and biochemical analysis blood, immunological tests to detect antibodies to viruses. With their help, you can determine how affected the liver tissue is, and predict the development of the disease. Ultrasound, x-rays and other methods are used to confirm diseases. After the examination, the doctor knows exactly the nature of the disease, the stage of its development, and possible complications. Severe forms of damage to the liver tissue are cirrhosis and cancer. The cause of such ailments can be a complication of chronic viral hepatitis.

There are the following methods of treatment of childhood liver diseases:

1. Conservative - with the help of drugs and diet;
2. Surgical - bloodless operations on the liver tissue and biliary tract (laparoscopy), as well as in the most severe cases operations with an opening of the abdominal cavity (for example, a liver transplant).

All operations in children are performed under full anesthesia.

With conservative treatment, a strict diet, exemption from physical exertion, and sports activities are prescribed. Depending on the nature of the disease, an appointment is prescribed medications: antibiotics (for infectious and inflammatory processes), hepatoprotectors (for hepatitis, cirrhosis and other serious diseases), choleretic drugs, vitamins.

Timely diagnosis and treatment, even in severe cases, can restore a child to health, and sometimes save a life.

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Liver diseases in children are an extremely undesirable phenomenon at an early age, which can often cause complex violations of body functions, from a slight decrease in immunity to pronounced delays in psychophysical development.

The liver is rightly called the "laboratory" of our body, since one of its functions is to cleanse the blood of toxins and other harmful substances. In addition, the liver performs a number of important tasks for the body - it forms a supply of nutrients, participates in the production of enzymes, and provides the right amount of red blood cells in the blood.

At an early age, liver pathology manifests itself in the form of a small group of symptoms, so its timely diagnosis is often difficult. A characteristic sign of liver disease in young children is jaundice, that is, staining of certain parts of the body and eye sclera of the baby in yellow. Often, specialists do not pay due attention to this symptom, believing that this condition is temporary and will soon pass. However, a high concentration of bilirubin, a breakdown product of erythrocytes, can cause severe disorders from nervous system, which in severe cases can cause disability of the child. That is why liver disease in children should not be left to chance.

It's believed that effective tool treatment for a condition such as neonatal jaundice is to shorten the intervals between feedings. However, if the condition under consideration lasts more than two weeks, you should consult a doctor for a complex of diagnostic and medical measures.

One of the most common liver diseases in preschool and school age is viral hepatitis. In this case, hepatitis A is transmitted by the so-called through household contact, i.e. through interaction with infected common items. In most cases, if you follow the diet and follow other doctor's recommendations, liver function is fully restored. In my

In turn, such forms of hepatitis as B and C develop as a result of the virus entering the bloodstream, are characterized by a more severe course and high probability transition to a chronic form. In this case, even if there are external signs of recovery, the child should be under the constant supervision of a specialist.

How not to miss the first symptoms of such a serious liver disease in children as hepatitis? On the early stages The clinical picture of the disease is very similar to clinical picture poisoning and other disorders of the gastrointestinal tract. The child is concerned about the growing weakness, loss of appetite, nausea, vomiting. In some cases, there may be yellow staining of the skin and mucous membranes. However, the most striking sign of hepatitis, which distinguishes it from other diseases, is a noticeable darkening of the urine and discoloration of the feces. If this symptom occurs, you need to take a blood test for bilirubin as soon as possible. Based on the results of the analysis, the doctor makes a conclusion about the form of hepatitis, predicts the possible course of the disease and prescribes the necessary treatment.

The most severe liver disease in children, as well as in adults, is a malignant lesion, which, if the disease is not detected in time, can be the result of hepatitis B and C. In this regard, during the period of exacerbation of these forms of hepatitis, it is important to strictly adhere to the diet and not allow the child to experience physical exercise.

It is often recommended to follow a special dietary regimen known as medical diet № 5. This diet is sparing for the liver and contributes to the speedy restoration of its functions. Spicy, fried and smoked foods, canned food, sweet pastries, soda, concentrated juices are excluded from the diet of a child with hepatic pathology. Low-fat meat and dairy products, egg whites, vegetables, freshly squeezed juices are allowed.

One of the professional tasks of the medical team of the Gastronika clinic is the diagnosis and treatment of various liver diseases in children. The doctors of the clinic have a successful experience in treating young patients. In the clinic "Gastronika" the kids are sensitive

Close attention, and diagnostic measures are carried out on the most modern equipment. If there is something that worries you about your child and you would like to discuss it with the doctor, book an online consultation or use the online appointment form. Come to us and we will help you to save your health!

The estimated prevalence neonatal disease liver reaches 1 in 2,500 live births.

Early recognition is especially important in newborns and infants, as a delay in diagnosis can have a negative impact on prognosis.

It is clearly recognized that if biliary atresia is diagnosed after 2 months of age, the success rate of surgical correction (hepatoportoenterostomy) decreases dramatically.

Moreover, as liver dysfunction progresses, early recognition enables more successful nutritional support for the patient and potentially slows the decline in liver function. The result may be improved growth and reduced side effects.

This is of great importance because orthotopic liver transplantation is usually more successful in infants weighing more than 10 kg at the time of surgery (liver transplantation is a reality for a pediatric patient with severe liver disease).

Unfortunately, timely recognition serious illness liver in a pediatric patient remains a serious problem. One of the factors contributing to this is that liver damage in a pediatric patient has a limited number of manifestations.

Consequently, different disorders often have virtually identical initial manifestations.

For example, newborns with liver disease almost always present with jaundice. Unfortunately, the difference between "physiological hyperbilirubinemia" and hyperbilirubinemia indicating severe liver damage is often underestimated. Data from the United Kingdom documented a number of factors contributing to late referral of children with liver disease (Table 1).

Table 1
Reasons for delayed referral for children with liver disease

Lack of follow-up for neonatal jaundice (including inability to fractionate serum bilirubin) Inadequate investigation hemorrhagic disease/ coagulopathy Misdiagnosis of cholestasis (conjugated bilirubinemia) as jaundice caused by breast milk (unconjugated bilirubinemia) False confidence caused by decreased bilirubin concentrations or the presence of pigmented stools

ETIOLOGY

The causes of liver disease in pediatric patients vary with age (Table 2).

NEWBORN AND INFANTS

Cholestatic disorders - Biliary atresia - Choledochal cyst - Insufficiency of intrahepatic bile ducts (eg Allagile syndrome) - Progressive familial intrahepatic cholestasis syndromes (Byler disease and syndrome) - Benign recurrent intrahepatic cholestasis (Caroli disease and syndrome) - Bile thickening (S/P hemolytic disease) - cholelithiasis

Idiopathic neonatal hepatitis and diseases mimicking it- Cystic fibrosis - Alpha-1 antitrypsin deficiency - Hypopituitarism/hypothyroidism - Neonatal iron storage disease

Viral hepatitis and other infectious diseases of the newborn- Cytomegalovirus - Herpes simplex /human herpes virus 6/ - Epstein - Barr virus - Parvovirus B19 - Rubella - Reovrus - type 3 - Adenovirus - Enterovirus - Syphilis - Tuberculosis - Toxoplasmosis

metabolic disease- Peroxysmal function disorders (Zellweger syndrome) - Bile acid metabolism disorders - Urea cycle disorders (arginase deficiency) - Amino acid metabolism disorders (tyrosinemia) - Lipid metabolism disorders (Niemann-Pick type C/Gaucher/Wolman) - Carbohydrate metabolism disorders (galactosemia, fructosemia, glycogen storage disease type IV)

Toxic/pharmacological injury (eg, acetaminophen (paracetamol), total parenteral nutrition, hypervitaminosis A)

Tumors (intra- and extrahepatic)

Moreover, while "neonatal hepatitis" may be caused by viruses, it is not the same entity as viral hepatitis seen in older children and adolescents.

Although the lists various reasons leading to liver disease in children are extremely long, about 10 diseases account for approximately 95% of all cases of observed cholestasis, and of these, biliary atresia and neonatal hepatitis are responsible for more than 60%.

Typically, the clinician first suspects liver disease in a neonate who presents with classic features such as persistent jaundice, hepatomegaly, coagulopathy, or underweight.
In other cases, this is an accidental finding of abnormalities in the study of serum.
Jaundice, confusion, and coma have been reported in older children and adolescents with acute hepatitis or after exposure to the toxin. Itching observed in older children may be regarded as irritability in infants.

HISTORY AND SIGNS OF LIVER DISEASE

Newborn

Although the infant may have already had jaundice at birth (physiological hyperbilirubinemia) or is breastfed, it is important not to attribute jaundice in a child older than 14 days to one of these causes.

Jaundice in any child older than 2 weeks of age should raise suspicion of liver disease and prompt appropriate evaluation.

A careful history can provide clues as to the existence and type of liver disease.

For example, the onset of liver disease associated with changes in diet may raise the suspicion of an inborn disorder of carbohydrate metabolism, such as an inability to metabolize galactose or fructose.

A positive family history for a suspected genetic disorder should focus the initial evaluation in this direction. A recurrent clinical phenotype within a family suggests an inherited disorder such as tyrosinemia or Byler syndrome (progressive familial idiopathic cholestasis).

Idiopathic neonatal hepatitis is more common among boys, especially premature and low birth weight infants.

In contrast, biliary atresia occurs more frequently among normal-weight girls, and the familial recurrence rate approaches zero. Also, the accompanying polysplenia syndrome speaks in favor of the diagnosis of biliary atresia.

Patients with biliary atresia have an earlier onset of jaundice and acholic stools than those with neonatal hepatitis.

In cholestatic disease, jaundice is almost invariably noted in the first month of life.

Acholic stools are also highly characteristic of cholestasis in infancy. In the presence of extrahepatic or intrahepatic obstruction, little or no bilirubin is excreted into the intestine, leading to a lack of color in the unformed fecal material.

Although some pigment may be present in the stool of neonates with biliary obstruction due to desquamation of cells containing the pigment into the stool.

Moreover, the disintegration of the stool into pieces usually shows that the pigment is only superficial, while its inner part has at best the color of clay.

Maternal fever or other signs of infection raise suspicion of sepsis as the underlying cause of neonatal jaundice. Gram-negative bacteria (eg, E coli) that cause infection urinary tract especially common.

older child

In older children with a history of anorexia. fever, vomiting, abdominal pain, dark urine should lead to suspicion of hepatitis A virus (HAV) infection.
HAV infection is also a likely etiology of liver disease in any child with a history of influenza-like illness who suddenly develops jaundice with elevated aminotransferases in the absence of any established exposure to hepatotoxic substances. Hepatitis A is often anicteric in children younger than 5 years of age and often goes unrecognized.

Signs of liver disease in patients who have had tattoos, who have used intravenous drugs, or in whom any disease has resulted in increased exposure to parenteral blood products (hemodialysis, hemophilia, surgery) prior to widespread screening (1992) may raise suspicion of infection hepatitis C.
Adolescents who develop jaundice should be asked confidentially about intravenous drug use, exposure to pure cocaine, intranasal use, which may be associated with hepatitis C (and possibly hepatitis B) infection.

If the course of confirmed hepatitis B infection is particularly severe, consider co-infection or superinfection with hepatitis (D) delta.

It is always important to ascertain data on exposure to potentially hepatotoxic drugs, including isonazid, nitrofurantoin, sulfonamides, and non-steroidal anti-inflammatory drugs such as acetaminophen and ibuprofen.

If overdose or intoxication is the cause of liver dysfunction, children may experience an altered mental status and even coma.

Confusion and coma raise suspicion of liver failure or metabolic disease leading to hyperammonemia, hypoglycemia, and a combination of both.
Adolescent girls who develop jaundice and have a history of acne, intermittent arthritis, and fatigue may have autoimmune hepatitis; this nosological unit is less common in boys and younger children.

Patients with immunodeficiency and jaundice may suffer from cytomegalovirus, Epstein-Barr virus, or retrovirus infections.

A history of pharyngitis in a patient who also has jaundice, splenomegaly, and lymphadenopathy suggests Epstein-Barr virus infection.

Upper right quadrant colic and a history of nausea (especially after eating a fatty meal) suggests gallbladder disease, which is more common in older children.

Consideration should be given to signs and symptoms of obesity of cardiac, endocrine, or intestinal disease, as abnormalities in serum aminotransferase may reflect secondary hepatic involvement in systemic disease (the liver as an "innocent bystander").
An increase in the concentration of aminotransferases (especially AST) can also be a manifestation of muscle disease.

Older children and adolescents with liver disease may initially complain of anorexia, fatigue, and icterus.

Cholestasis can lead to complaints such as pruritis and especially dark and frothy urine. Given color due to choluria (bile pigment in the urine); the presence of foam suggests choleuria (bile salts in the urine).

Bile salts are detergent molecules that reduce the surface tension of solutions, thus creating visible foam.

PHYSICAL MANIFESTATIONS

Common physical manifestations associated with specific liver diseases are listed in Tables 3-6.

Table 3
Diseases that cause jaundice/elevated liver enzymes

BABY
Infection- Bacterial sepsis (E coli) - Viral infections: cytomegalovirus, rubella, Coxsackievirus, echovirus, herpesvirus, adenovirus. - Metabolic disorders - Hereditary: alpha 1-antitrypsin deficiency, galactosemia, hereditary fructose intolerance, cystic fibrosis, Niemann-Pick disease, tyrosinemia - Acquired: cholestasis and liver disease caused by total parenteral nutrition, hypothyroidism, panhypopituitarism - Idiopathic disorders - Neonatal hepatitis, progressive familial intrahepatic cholestasis (eg Byler disease), Ivemark syndrome, cerebrohepatorenal (Zellweger) syndrome

Malformations of bile streams

Atresia/insufficiency of the intrahepatic bile ducts, non-syndromic and syndromic (Allagile syndrome) - Cystic malformations: choledochal cysts, cystic dilatation of the intrahepatic bile ducts (Caroli disease), congenital hepatic fibrosis, polycystic liver and kidney disease.

OLDER AND GROWER CHILDREN

Acute viral hepatitis (HAV)

Hereditary diseases:

Wilson disease, cystic fibrosis, hepatic porphyrias, Dubin-Johnson syndrome, Rotor syndrome

Malignant diseases:

Leukemia, lymphoma, liver tumors

Chemical substances:

Hepatotoxic drugs, toxins (hydrocarbon insecticides, alcohol, organophosphates, hypervitaminosis A, mushrooms, acetaminophen). schistosomiasis, leptospirosis, visceral larva migrans

Idiopathic or secondary lesions: chronic hepatitis, inflammatory bowel disease (ulcerative colitis), rheumatoid arthritis, obesity.

Among this abundance of physical manifestations, the most common are hepatomegaly and jaundice.

Hepatomegaly is often the only manifestation of liver disease, although palpation of the liver margin can be misleading due to variations in the normal contour, body habitus, or displacement of the liver margin by adjacent organs or external or internal neoplasms.

Therefore, measuring the width of the liver is helpful. additional method to palpation at initial presentation and follow-up.
The width of the liver is the distance between the edge of the liver and the upper limit of dullness, determined by percussion at the right midclavicular line. The average width varies from 4.5 cm at 1 week of age to 6-7 cm in early adolescence.

Normally, the liver is round and soft, and the surface is smooth. A hard, thin margin and nodular surface may suggest fibrosis or cirrhosis.
Last state also often associated with a small liver.

Palpation of the liver in the epigastric region indicates either the presence of cirrhosis or the Riedel lobe (normal anatomical elongation of the right lobe, which can be mistaken for hepatomegaly).

Table 4
Diseases that cause hepatomegaly

INFANTS AND CHILDREN
storage diseases

Acute: Reye's syndrome (fat) - Chronic: glycogenosis, mucopolysaccharidoses, Gaucher's disease, Niemann-Pick disease, gangliodida, Wolman's disease - Nutritional problems: total parenteral nutrition (caloric overload, kwashiorkor, diabetes) - Infiltrative disorders: leukemia, lymphoma, Langerhans cell histiocytosis, granulomas (sarcoidosis, tuberculosis) Congenital hepatic fibrosis

Tumors

Primary: hepatoblastoma, hematoma, hemangioendothelioma - metastasizing: neuroblastoma, Wilms' tumor, tumors of the gonads.

Table 5
Diseases that cause liver failure

NEWBORN AND INFANTS

Infections:

Herpesviruses, echo, adenoviruses, sepsis.
Metabolic disorders: hereditary fructose intolerance, mitochondrial diseases, tyrosinemia, galactosemia, neonatal iron storage disease.
Ischemia/shock:
Drugs/toxins: valproate, acetaminophen

CHILDREN AND ADOLESCENTS
Infections: herpes viruses, echo, adenoviruses, sepsis
Drugs/toxins: valproate, acetaminophen, mushrooms (Amanita)
malignant disease
Ischemia/shock: congenital heart disease, myocarditis, severe hypotension.
Metabolic: wilson's disease, fatty infiltration liver during pregnancy.

Table 6
Various physical manifestations associated with liver disease

BABY
Microcephaly: congenital cytomegalovirus, rubella, toxoplasmosis
Typical face type: arteriohepatic dysplasia (Allagile syndrome)
Cataract: galactosemia
Retinal pigmentation: Allagille syndrome
Abnormal manifestations on auscultation of the lungs: cystic fibrosis.
Neuromuscular disorders (tremor, lethargy): lipid storage disease, Wilson's disease, oxidative phosphorylation disorders

CHILDREN
Pruritus: chronic cholestasis
Hemangiomas: liver hemangiomatosis
Kayser-Fleischer rings: Wilson's disease
Glossitis: cirrhosis

Kidney enlargement: congenital hepatic fibrosis or polycystic disease
Arthritis and erythema nodosum: liver disease with chronic inflammatory bowel disease. acne, fatigue: autoimmune hepatitis.

Abdominal palpation may also reveal the presence of an enlarged spleen, which is usually normal in size in the early stages of liver disease.

If the spleen is enlarged, one of the many causes of portal hypertension or storage disease should be suspected.
Pain on palpation with hepatomegaly may simply reflect a mild viral stroke with dilatation of the Glisson capsule due to edema, which is responsible for the felt pain localized to the liver region.

Severe hepatosplenomegaly is indicative of storage disease or malignancy, although particularly prominent hepatomegaly is itself associated with severe hepatic fibrosis

In this condition, the kidneys should be evaluated to rule out coexisting autosomal recessive or dominant polycystic kidney disease.

Auscultation of the liver may allow the clinician to detect vascular bruising due to anatomical vascular malformations or increased blood flow to the liver.

Ascites suggests increased portal venous pressure and worsening hepatic function.

Certain physical signs raise significant suspicion for specific liver disease. In newborns who suffer from congenital infection, associated manifestations often include microcephaly, chorioretinitis, purpura, low birth weight, and generalized organ failure.

Dysmorphic features may be characteristic of certain chromosomal disorders. Patients with Alagille syndrome usually have a characteristic facial pattern (beak nose, high forehead, butterfly-shaped vertebrae, and a murmur on cardiovascular auscultation due to peripheral pulmonary stenosis and posterior embryotoxin on ophthalmic examination.

Availability periodic vomiting in a newborn, especially unremitting, may indicate congenital metabolic disorder, which is usually also associated with poor nutritional status and irritability. The occurrence of symptoms (such as vomiting) after the introduction of a new product containing galactose or fructose could raise the suspicion of galactosemia or hereditary fructose intolerance.

Congenital ascites may suggest liver failure, cirrhosis, or storage disease.
Children with cholestasis often suffer from intense pruritis, which is characteristic of obstructive liver disease, which is primarily manifested by irritability.

LABORATORY EVALUATION

Types of liver damage
Laboratory manifestations of liver damage can be divided into 2 types:
1) cholestatic or obstructive lesion of the bile ducts and
2) hepatocellular damage or damage to liver cells.
However, there is often considerable overlap between the types of damage in a patient with liver disease.

Cholestasis is characterized by the accumulation of compounds that cannot be excreted due to occlusion or obstruction of the biliary tree. Hence, serum concentrations of substances (bile pigments, enzymes, bile salts) that are normally found in bile or are eliminated through bile are usually increased in cholestatic conditions. Alkaline phosphatase (AP), gamma-glutamyl transpeptidase (GGT), and conjugated bilirubin (all requiring a pure biliary tree for elimination) are usually elevated.

Conversely, necrosis of hepatocytes following viral or toxic liver stroke (eg, acetaminophen overdose or viral hepatitis) usually primarily causes an increase in hepatocyte-intrinsic enzymes such as aminotransferases (ALT and AST). In hepatocellular disease, serum levels of GGT and AP still do not rise to the same extent as aminotransferases. This distinction between the two main types of liver damage is not always clear cut.

For example, cholestasis invariably leads to some degree of hepatocellular dysfunction due to the poisonous accumulation of bile within the hepatocytes and biliary tree. In hepatocellular disease, reduced biliary blood flow (sludge) that results from necrosis of hepatocytes also causes a slight increase in serum markers of obstruction (AP, GGT).

The two main types of liver disease can be differentiated early in the disease process, but most often, the underlying type of liver disease is diagnosed by interpretation of a combination of clinical and laboratory criteria, including liver biopsy. This is especially true for neonates and infants, where there is the greatest overlap between types of liver damage. It is most important to recognize the presence of cholestasis in patients in this age group, even in premature infants in whom the presence of jaundice after 14 days of life requires evaluation. Table 7 shows the goals for the stepwise assessment of infants with jaundice.

Table 7
Objectives for stepwise assessment of infants with jaundice
Recognize cholestasis (unconjugated or physiological hyperbilirubinemia)
Assess the severity of liver damage
Separate specific nosological units (e.g. metabolic versus viral versus anatomical)
Differentiate biliary atresia from idiopathic neonatal hepatitis
Differentiate idiopathic neonatal hepatitis from progressive familial intrahepatic cholestasis and bile duct insufficiency.

Table 8 lists our recommendations for data collection when evaluating an infant with suspected cholestasis. An accelerated assessment is suggested for infants presenting at 2 months of age with cholestasis to quickly rule out biliary atresia.

Table 8
Stepwise assessment of infants with suspected cholestatic liver disease

Confirm cholestasis
- Clinical Assessment(family history, feeding history, physical examination)
- Fractionation of serum bilirubin and determination of serum bile acid levels
- Assessment of stool color
- Index of hepatic synthetic function (prothrombin and albumin time)

Recognize specific nosological units
- Viral and bacterial cultures (blood, urine, cerebrospinal fluid)
- Hepatitis B surface antigen and other viral and syphilis (VDRL) titers in selected patients at risk
- Metabolic screening (urine reducing substances, urine and serum amino acids.)
-Thyroxine and thyroid stimulating hormone
- Alpha 1 anti-spin phenotype
- Sweat chlorides
- Qualitative analysis of urine bile acid profile
- Ultrasonography

Differentiate biliary atresia from neonatal hepatitis
- Hepatobiliary scintigraphy or duodenal intubation for bilirubin content
- Liver biopsy

Liver function studies

Because the liver has a large functional reserve, abnormal laboratory values ​​are often the only manifestation of overt hepatic disease and may occur long before overt clinical manifestations. In the usual scenario, a physician who suspects liver disease will usually order "specific liver function tests" (LFTs) to evaluate liver function. Consistently monitoring these parameters can provide information on prognosis, response to therapy, and degree of dysfunction.

However, the term LFT is not entirely accurate, as only two of the commonly reported parameters are true measures of hepatic function - prothrombin time (PT) and serum albumin levels - both of which measure synthetic capacity. All of the other parameters are essentially indirect measures of liver function, and many of these measures change in situations other than liver disease. For example, elevations in aspartate aminotransferase (AST) accompany red blood cell hemolysis, muscle breakdown, and pancreatic disease.

Biochemical abnormalities associated with liver disease are not limited to LFT abnormalities. For example, nonketotic hypoglycemia suggests a defect in fatty acid beta-oxidation and ketone production. Severe ketosis, a rare finding in infants, may indicate organic acidemia, glycogen storage disease, or neurogenesis deficiency. An increase in anion shift in metabolic acidosis also suggests organic acidemia. Hypo- and hyperthyroidism may be associated with jaundice. Determination of sweat chlorides may be required to rule out cystic fibrosis. Iron and ferritin studies are useful in diagnosing neonatal iron storage disease. Determination of bile acid levels in urine and serum usually helps in ruling out the possibility of congenital disorders of bile acid metabolism. Urinary succinylacetone levels may indicate the presence of tyrosinemia. A urine sample and culture should always be obtained from any child with jaundice, as urosepsis is usually associated with conjugated hyperbilirubinemia (eg, E coli urinary tract infection). Anemia and hemolysis may indicate the presence of a hemolytic condition responsible for jaundice (usually unconjugated) and may not be associated with liver disease.

Of all laboratory investigations conducted, bilirubin fractionation is the most important.
A healthy mature liver removes unconjugated bilirubin from the blood and mediates the conjugation of unconjugated bilirubin with two molecules of glucuronic acid. Conjugation of bilirubin converts an essentially fat-soluble substance (unconjugated bilirubin) into a water-soluble substance (conjugated bilirubin) that can be excreted into the bile, aquatic environment. It is the fat-soluble nature of unconjugated bilirubin that allows it to cross the blood-brain barrier and potentially cause kernicterus.

Physiological jaundice neonatal (in which levels of unconjugated bilirubin increase) results from the immaturity of the glucuronyl transferase system responsible for bilirubin conjugation. Breastfeeding-associated jaundice (slight temporary slowdown in conjugation ability), usually the result of components found in breast milk.

In unconjugated bilirubinemia, serious liver disease is unlikely, but the child may need to be evaluated for possible hemolysis, congenital disorders of bilirubin metabolism (eg, Crigler-Najar types 1 and II), and dysfunction thyroid gland. Extreme unconjugated hyperbilirubinemia may be associated with kernicterus in the newborn.
In the presence of conjugated (direct, bound) bilirubin, the evaluation should be aggressive. Serum conjugated bilirubin above 17 mcmol/l (1 mg/dl) or above 15% of total bilirubin should be considered abnormal and evaluated immediately! Unconjugated bilirubin levels reflect excess production of bilirubin (eg, as a result of hemolysis) or a reduced ability of the liver to bind bilirubin. The conjugated fraction is associated with serious illness liver and indicates cholestasis.

Analysis of urine
Urobilinogen is formed as a result of the degradation of conjugated bilirubin by bacteria present in the intestinal lumen and is also found in the urine. Most of the urobilinogen is excreted in the stool as coprobilinogen; 20% undergoes enterohepatic recirculation. Only a small fraction enters the urine, but this is increased in the presence of hepatocellular injury due to reduced hepatic uptake and recycling. Undoubtedly, urinary urobilinogen is almost absent in the presence of an obstructive process, since less bilirubin enters the intestine and less is converted to urobilinogen. Interestingly, delta-bilirubin, due to its covalent binding to albumin, is not excreted in the urine, and therefore tends to remain elevated in serum for some time after an initial cholestatic stroke, since its disappearance depends on the breakdown of albumin. -bilirubin complex.

Aminotransferase activity
Alanine aminotransferase (ALT) (formerly known as serum glutamic pyruvate transferase - SGPT) and AST (formerly known as serum glutamic oxaloacetic transaminase -SGOT) levels are the most sensitive assays for hepatocyte necrolysis. A significant increase in these enzymes, which are released from damaged hepatocytes, indicates hepatocellular damage. Mildly disturbed levels may also be associated with cholestatic processes, as bile backflow or stasis is toxic to hepatocytes. These enzymes catabolize the reversible conversion of the alpha-amino group of the amino acids alanine and aspartic acid to the alpha-keto group of ketoglutaric acid, which leads to the formation of pyruvic acid (ALT) and oxaloacetic acid (AST).

ALT is more specific for the presence of liver disease because it is found only at low concentrations in other tissues (eg, muscle). Conversely, AST is contained in high concentrations in many tissues, including cardiac and skeletal muscles, kidneys, pancreas, and red blood cells. The coenzyme of both enzymes is vitamin B6, so it is persistently abnormal low rates AST and ALT suggest an underlying vitamin B6 deficiency.

In general, aminotransferase levels still do not provide information regarding specific diagnoses, but especially high levels suggest drug hepatotoxicity (eg, acetaminophen overdose), hypoxia/shock, and viral hepatitis. These levels still have no predictive value; patients with very high abnormal values ​​may do well, especially in the case of acetaminophen toxicity. However, they are useful in monitoring the patient's clinical progress, for example, progressively lower AST/ALT values ​​in a young patient who has HAV infection and is otherwise doing well is a comforting sign that liver disease is disappearing. Conversely, declining AST/ALT values ​​in the presence of a shrunken liver, increasing PT of partial thromboplastin time (PTT), and in the absence of clinical improvement are an ominous sign. This suggests a reduced functioning hepatocyte mass due to necrosis, which has reduced the amount of enzymes available for release into the circulation.

Alkaline phosphatase (AP) levels
AP is localized primarily to the tubular membrane of liver cells, therefore an elevated serum AP level usually indicates obstructive liver disease (eg, bile duct obstruction). However, AR is found in other tissues, including bone, kidney, and small intestine. High rates of AR are commonly found in children during periods of accelerated growth, such as pubertal growth spurts. Particularly high levels should lead to the suspicion of a possible bone pathology (eg rickets), especially if the increase in AP is not associated with an increase in GGT. If the levels of the latter enzyme are also elevated, bone disease is unlikely. This simple observation reduces the need to fractionate AP values ​​into individual isoenzymes to determine the exact source of their increase. For example, zinc-coenzyme-AP - persistently low levels of AP may mean low serum levels of zinc.

Serum and urine bile acids
The production and transport process that occurs in the liver (bile acid synthesis, conjugation and secretion) maintains cholesterol levels, facilitates bile flow, and provides surface active detergent molecules that promote intestinal absorption of lipids. The proper functioning of this system contributes to the balance between the absorption of bile acids from the intestine and their uptake by hepatocytes. In the absence of ileal changes (eg, short bowel, Crohn's disease), serum bile acid levels are a reliable indicator of the integrity of the enterohepatic circulation.

Although serum bile acids still do not provide specific information about the type of liver disease present, they are elevated in patients with either acute or chronic liver disease, in whom bilirubin levels may still be normal. Changes serum levels bile acids can be not only quantitative, but also qualitative. In certain diseases, "atypical" bile acids, such as lithocholic acid, accumulate instead of the normal cholic and chenodeoxycholic acids. The interpretation of elevated serum bile acid levels in newborns and infants is complicated by the presence of a relative "physiologic cholestasis" that can lead to elevated serum bile acid levels even in healthy infants. However, specific defects in bile acid metabolism are associated with cholestasis either due to insufficient production of normal trophic and choleretic bile acids, or excessive production of hepatotoxic bile acids. Accurate identification of metabolite precursors makes it possible to identify specific congenital disorders bile acid metabolism. With the help of recent technological advances, such as fast atom bombardmnet - mass spectrometry (mass spectrometry of fast atomic bombardment), it is possible to quickly analyze urine samples from individuals with suspected specific disorders of bile acids and identify specific congenital disorders of bile acid metabolism, such as insufficiency 3 -beta-hydroxysteroid dehydrogenase/isomerase and delta-4-3-oxosteriod-5-beta reductase deficiency, which manifest as severe liver disease.

Gamma-glutamyl transferase (GGT)
GGT - found in the epithelium of the small bile duct, as well as inside hepatocytes; in the pancreas, spleen, brain, mammary glands, small intestine and especially in the kidneys. Therefore, an increase in serum GGT does not specifically indicate liver disease.

Because GGT levels still do not increase in individuals with bone or intestinal abnormalities, this finding is particularly useful in identifying the origin of elevated AR levels. GGT values ​​(like AP) change with age, the laboratory should use age-appropriate reference values. For example, a seemingly high GGT level in a newborn may not be abnormal; similar rates in this age group are typically up to 8 times higher than rates observed in adults. Finally, GGT values ​​may be elevated in response to various pharmacological treatments, such as anticonvulsants, so the clinician needs to be aware of the patient's recent drug exposure.

Albumen
Decreased serum levels of albumin, which is synthesized in the irregular endoplasmic reticulum of healthy hepatocytes, may suggest decreased production due to decreased hepatic function after hepatocellular disease. However, low albumin concentration is a late manifestation in liver disease. When it is present, it raises the suspicion of a chronic disease. Undoubtedly, a sharp decrease in albumin in a patient with long-term disease is of particular concern, although such a decrease in patients with ascites may simply reflect a change in the total volume of distribution.

Ammonia
Ammonia production occurs as a result of the action of colon bacteria on proteins in the diet, and the liver plays a major role in its elimination. Undoubtedly, a poorly functioning liver still does not catabolize ammonia. Hyperammonemia and encephalopathy are classic manifestations of liver failure, and there is a labile correlation between encephalopathy and serum ammonia levels (unless the sample is obtained in a fasted state and transported quickly to the laboratory on ice, dramatically elevated ammonia levels can be obtained).

Prothrombin time (PT)
The production of coagulation factors II, VII, IX and X depends on an adequate intake of vitamin K (malnutrition is a risk group). Because it is a fat-soluble vitamin, vitamin K deficiency is common among people with obstructive liver disease, in whom the bile acids still do not reach the intestines. Therefore, PT, the time required for prothrombin (factor II) to turn into thrombin, is usually increased in the presence of biliary obstruction. The RT can also be elevated in the presence of true hepatocellular disease, a poorly functioning liver cannot properly gamma-carboxylate the aforementioned factors in the liver despite the presence of vitamin K. This is the basis for parenteral administration(non-oral) vitamin K in patients with elevated TA values. If this therapy corrects the PT value, liver function is likely to be within normal limits and the failure is most likely due to obstruction. Therefore, it is useful to re-measure the PT value after administration of vitamin K.

One of the first steps in evaluating a newborn with cholestasis is to measure PT/PTT and administer vitamin K. Untreated hypoprothrombinemia can lead to spontaneous bleeding and intracranial hemorrhage.

Micronutrients and Vitamin-Related Disorders
Copper accumulates in the liver during cholestasis because it is excreted primarily through the bile. It is possible that the interaction between copper, a pro-oxidant that generates free radicals, and a liver already affected by cholestasis (in the presence of a depletion of antioxidants such as glutathione and vitamin E) further contributes to the already existing liver damage.

Manganese is also excreted primarily through the biliary system and therefore can accumulate in the liver and cause hepatotoxicity. For this reason, we are reducing or even discontinuing manganese supplementation in total parenteral nutrition solutions used for patients with liver disease.

Aluminum is also excreted via biliary excretion and is hepatotoxic at high doses. Cholestasis can lead to its accumulation in the liver.

The fat-soluble vitamins (A, D, E, and K) are all absorbed through adequate hepatic secretion of bile acids into the intestinal lumen. When bile acids are not excreted into the intestine, malabsorption of fat-soluble vitamins occurs. Moreover, vitamin A and E esters require hydrolysis before absorption, and bile acid-dependent intestinal esterase catalyzes this reaction. Consequently, the absorption of vitamins A and E is further reduced in patients with cholestasis. The liver is also responsible for one of the hydroxylation steps required to metabolize vitamin D into its active form. Undoubtedly, impaired hepatic function usually reduces vitamin D levels, and rickets is common among children with cholestasis.

IMAGING AND HISTOPATOLOGY OF THE LIVER AND BILIAL TRACT

Abdominal ultrasonography -
relatively inexpensive, easy to administer, and can usually be undertaken without sedating the patient. It allows to measure the size of the liver, confirm changes in the structure of the liver and the presence of cystic or non-cystic parechymal lesions. Choledochal cysts and stones can be detected with an accuracy of 95%. Ultrasonography is also useful as a screening method for large lesions in the liver and dilated bile ducts. Finally, it also confirms the absence of a gallbladder, which may suggest the presence of biliary atresia.

Cholescintigraphy -
reveals abnormalities in hepatic uptake, as well as parenchymal concentrating and excreting abilities. Imaging with cholephilic radiolabeled N-substituted imino-diacetate (IDA), radiolabelled technetium-99 atoms such as diisopropyl-IDA (DISIDA) or para-isopropyl-IDA (PIPIDA) are used in the examination of children with liver disease. These radiolabeled atoms are concentrated within the bile, thus giving an image of bile flow, even in the presence of severe cholestasis. The appearance of a labeled atom within the intestinal region after 24 hours virtually rules out biliary atresia, but the reverse is not true. In fact, the absence of a tagged atom in the gut may not represent an obstructive defect, but rather a parenchymal disease process in which tagged atom uptake or concentration is poor. To facilitate the flow of bile, patients often receive phenobarbital (5 mg/kg per day, divided into 2 daily doses) for 3-5 days before having a scan. Phenobarbital facilitates bile flow by inducing specific liver enzymes in infants with immature hepatic enzyme systems but no other abnormalities.

In general, radionuclide excretory scans are not essential for assessing neonatal cholestasis. This time-consuming process has the potential to delay diagnosis. Moreover, it has high false-positive (i.e., no excretion) and false-negative (i.e., clear tagged atom excretion) rates (at least 10%) and is not very effective when serum bilirubin levels are elevated. . Percutaneous liver biopsy should never be delayed in favor of a radionuclide scan.

Computed tomography (CT) and magnetic resonance imaging (MRI)
These two methods are rarely necessary as first-order methods in the diagnosis of liver disease in children. However, they may be useful in specific situations. CT scanning of the liver can reveal deposits of glycogen, iron, and lipids within the hepatic parenchyma and can clearly identify and characterize neoplasms in the liver. However, CT is more expensive and technically more difficult in infants than ultrasonography, and the patient is exposed to ionizing radiation. MRI has comparable sensitivity to CT in recognizing chemical differences in tissues and detecting tumors and infiltrates without injecting a contrast agent or exposing the patient to ionizing radiation. However, MRI is more expensive and cannot be used to detect calcium deposits or in patients with implanted metal devices.

Percutaneous liver biopsy -
cardinal method for quickly arriving at a diagnosis of the underlying liver disease. The technique is fast, safe and effective and usually does not require the patient to stay in the operating room or stay overnight in the hospital. The histological picture of the liver can be examined; glycogen, copper, iron and other components in liver tissue can be quantified. Pathologists can determine if there is any storage disorder: to confirm the presence of Wilson's disease (copper storage), glycogen storage disease, or neonatal iron storage disease, as well as many other pathological conditions. The histological picture of the tissue provides useful information about the degree of fibrosis or the presence of cirrhosis and allows the diagnosis of biliary atresia, neonatal hepatitis, congenital hepatic fibrosis, and alpha-1 antitrypsin deficiency. It has been reported that percutaneous liver biopsy can provide an accurate diagnosis of biliary atresia in 94-97% of all cases, thus eliminating the risk of exposing a child with intrahepatic bile duct insufficiency cholestasis to unnecessary surgery.

ABBREVIATIONS
AIH - autoimmune hepatitis
ALT - alanine aminotransferase
AR - alkaline phosphatase
AST - aspartate aminotransferase
GGT - Gamma glutamyl transpeptidase
HAV - hepatitis A virus
HVB - hepatitis B virus
HCV - hepatitis C virus
HDV - hepatitis D virus
HEV - hepatitis E virus
LFT - Liver Function Test
RT - prothrombin time
PTT - partial thromboplastin time

LIVER DISEASES IN A NEWBORN

neonatal hepatitis

This relatively specific syndrome has been characterized both clinically and histologically, but its pathophysiological basis is unknown.

A small percentage of cases may be associated with viral hepatitis acquired in utero or postnatally.

In most cases, "idiopathic" neonatal hepatitis is a very general category that includes all cases of neonatal liver dysfunction for which an etiology has not been identified.

For example, homozygous alpha-1 antitrypsin deficiency, which is now recognized as specific reason neonatal liver disease, was formerly included in the category of idiopathic neonatal hepatitis.

As bile acid metabolism is more clearly defined and specific defects are identified, it is now believed that many children previously thought to have "neonatal hepatitis" have a defect in bile acid metabolism.

The hepatobiliary systems of infants and newborns are susceptible to damage due to metabolic immaturity, insufficient protein transport, or altered organelle function. These characteristics lead to altered permeability or inefficient transport within the system, which contributes to the onset or chronicity of cholestasis.

primary goal in the management of children with neonatal hepatitis is to conduct an exhaustive search for detectable and treatable causes of this clinical condition. Jaundice may occur from birth or appear during the first 3 months of life.

The classic manifestations of cholestasis are not always present during the first few weeks of life in patients with bile duct insufficiency, alpha1-antitrypsin deficiency, or even biliary atresia. These children often, but not always, have poor appetite, vomiting, and a sick appearance.

Cholestasis is manifested by the passage of acholic stools and dark urine. Serum bilirubin is elevated and more than 50% is due to the conjugated fraction. Aminotransferase levels are almost always elevated, but to varying degrees. AP and GGT levels, if elevated, only moderately.

Serum albumin and RT may be abnormal, and the degree of abnormality correlates with disease severity at presentation. Ultrasonography usually shows the presence of a gallbladder and the absence of cystic malformations of the biliary tree, explaining cholestasis. Hepatobiliary scintigraphy reveals delayed transport of the radionuclide and eventually shows an open extrahepatic biliary tree.

A severely affected liver may have difficulty absorbing the radionuclide, reducing the usefulness of this study in seriously ill children. Liver biopsy usually provides a definitive diagnosis by demonstrating giant cell transformation with portal inflammatory infiltrates and no bile duct proliferation.

The management of these children involves supportive measures and the avoidance of the consequences of malabsorption by providing adequate nutrition and receipts fat soluble vitamins and medium chain triglycerides until recovery (which occurs in most). Liver transplantation is an alternative for those who do not benefit from conservative treatment.

Biliary atresia

Biliary atresia is the result of a destructive idiopathic inflammatory process that affects the intra- and extrahepatic bile ducts. This leads to fibrosis and obliteration of the biliary tract with the development of biliary cirrhosis. It occurs worldwide, affecting approximately 1 in 12,000 live births, and is the most frequent indication to liver transplantation in infants and children.

Early differentiation of biliary atresia from idiopathic neonatal hepatitis allows rapid surgical correction (Kasai portoenterostomy) that allows improved nutrition and maximum growth.

Because cirrhosis develops rapidly, surgical repair of biliary atresia is most effective if performed before 3 months of age, emphasizing the need for rapid and accurate diagnosis causes of liver dysfunction observed in the infant. As with idiopathic neonatal hepatitis, children with biliary atresia present with signs of cholestasis. Once these signs are recognized, abdominal ultrasonography can rule out the presence of a choledochal cyst.

Cholescintigraphy usually shows good capture of the labeled atom and no excretion into the intestine even after 24 hours. A liver biopsy confirms the diagnosis - reveals proliferation of interlobular bile ducts, periportal fibrosis, and bile plugs in the tubules.

Liver transplantation may become necessary if there is progressive hepatic decompensation, refractory growth retardation, impaired synthetic dysfunction, and the development of coagulopathy or intractable portal hypertension with recurrent gastrointestinal bleeding or hypersplenism.

Other reasons

Other entities causing hepatic dysfunction in the newborn and infant can be diagnosed by a combination general research.

Structural abnormalities of the biliary tree, such as congenital hepatic fibrosis or choledochal cysts, are usually detected by ultrasonography. Liver biopsy shows the characteristic "ductal plate defect" in infants with congenital hepatic fibrosis.

Bacterial infections causing cholestasis can be identified by a positive blood or urine culture. Maternal exposure to or previous infection (toxoplasmosis, syphilis, cytomegalovirus, herpes simplex virus, varicella, and other viruses) should prompt screening for these infections. In case of suspected viral infection antibodies to both serum immunoglobulin M (IgM) and IgG should be determined. Urine is often positive for cytomegalovirus.

Hereditary tyrosinemia, a defect in tyrosine metabolism, is indicated by disproportionate impairment of hepatic synthetic function, mild abnormalities in bilirubin, transaminase levels, and rickets.

The presence of succinyl acetone in the urine and the detection of insufficient activity of fumarylacetoacetate hydrolase (which catalyzes the final step in the oxidative catabolism of tyrosine) confirms the diagnosis. Screening for alpha 1-antitrypsin deficiency can be done by typing for the ZZ inhibitor protein (PiZZ) and is usually confirmed by characteristic periodic Schiff acid-positive alpha 1-antitrypsin granules deposited within the hepatic parenchyma on liver biopsy.

Excess iron deposits are seen on biopsy in infants with neonatal iron storage disease. Congenital hypothyroidism, which may present with vague hyperbilirubinemia, is ruled out by evaluation of thyroid function.

LIVER DISEASES IN AN OLDER CHILD

The main causes of liver disease seen after infancy are toxic, infectious, metabolic, autoimmune, vascular, and infiltrative. The relative frequency of each disorder varies with the age of the patient. Viral hepatitis occurs in patients of all ages, as does liver disease caused by cardiac or collagen vascular disease.

Fat metabolism disorders are noted in older infancy and early childhood. Wilson's disease is usually detected in older childhood or adolescence.

Acute hepatitis

The clinical picture of viral hepatitis varies depending on the pathogen. HAV infection presents most often as a flu-like illness (malaise and signs of an upper respiratory tract infection). Patients often have a fever and may have very severe jaundice.

The pathogens spread primarily through the fecal-oral route. In children, the disease is usually self-limiting and often has no clinical manifestations. No chronic carrier state has been identified. Diagnosis of acute infection is based on the presence of anti-HAV IgM antibodies in serum.

Hepatitis B virus (HAV) infection can cause both acute and chronic hepatitis and may develop cirrhosis and hepatocellular carcinoma. Diagnosis is based on detection of hepatitis B surface antigen (HBsAg) or anti-HBV core (anti-HBc) IgM antibody. Chronic HBV infection is associated with the persistence of HBsAg and HBV DNA.

Hepatitis C virus (HCV) causes acute hepatitis that progresses to chronic disease in more than 70% of affected people. End-stage disease (liver cirrhosis, hepatocellular carcinoma) may occur in 10% of patients. Fulminant hepatitis has rarely been described. The diagnosis is based on the detection of anti-HCV antibodies and is confirmed chain reaction polymerase on HCV RNA.

Hepatitis D virus (HDV) infection usually complicates liver disease in a patient with hepatitis B and should always be considered in patients with particularly aggressive HBV disease. HDV is prevalent in the Mediterranean basin and less common in North America, where it is primarily associated with intravenous drug use.

Hepatitis E virus (HEV) occurs as an epidemic in parts of the world with poor sanitation. The cases diagnosed in North America were in patients who brought the virus from abroad. It can be especially devastating in pregnant women.

chronic hepatitis

It is traditionally defined as an inflammatory liver disease in which biochemical and histological abnormalities persist for more than 6 months. However, irreversible changes can occur in children and within the given 6 months.

The most acute hepatitis disappears in children within 3 months.

The etiology of the observed liver disease should be actively identified, since many of the diseases that cause chronic hepatitis in children are amenable to specific conservative therapy. Chronic hepatitis in children is the result of a viral infection, an autoimmune process, exposure to hepatotoxic drugs, or cardiac metabolic or systemic disorders.

Autoimmune Hepatitis (AIH)

AIH is inflammation of the liver associated with the presence of circulating autoantibodies in the absence of other recognized causes of liver disease. At least two types have been described: type I AIH is more common among women and is characterized by a positive antinuclear antibody, and type II AIH involves anti-liver-kidney-microsomal antibodies.

Other autoimmune diseases may coexist with this type of hepatitis, including thyroiditis, diabetes, hemolytic anemia and erythema nodosum. Laboratory evaluation reveals an increase in aminotransferases (often very high) and varying degrees of hyperbilirubinemia, most of which is conjugated.

Serum gammaglobulin concentrations are elevated in almost all patients. AR and GGT values ​​are usually normal or only slightly elevated. Liver biopsy reveals inflammatory infiltrates limiting plate and intralobular zones with partial necrosis, distortion of the lobular structure and even fibrosis in severe cases.

Other liver diseases

Sclerosing cholangitis is characterized by chronic inflammation of the intra- or extrahepatic biliary tree. It may be primary (without evidence of underlying disease and unclear etiology) and is commonly associated with inflammatory bowel disease. Secondary sclerosing cholangitis results from stones, postoperative strictures, or tumors.

Children may present with jaundice, pruritis (itching), or fatigue upon presentation. Elevated AR levels are very characteristic, but there is no specific disease marker. Biopsy shows typical concentric fibrosis around the interlobular bile ducts (an "onion skin" appearance). Cholangiography or endoscopic retrograde cholangiopancreatography is required to make the diagnosis.

Treatment involves the administration of fat-soluble vitamins and ursodeoxycholic acid, a natural choleretic bile acid, which appears to reduce pruritus in affected individuals.

Unfortunately, liver disease progresses in many patients and cirrhosis develops. Liver transplantation is ultimately required for long-term survival.

Wilson's disease is an autosomal recessive disorder caused by a defect in biliary copper excretion, in which excess copper deposits lead to cirrhosis. Excess copper is deposited in the cornea, kidneys and brain, leading to extrahepatic manifestations of the disease.

Although clinicopathological manifestations can be extremely variable, Wilson's disease should be included in the differential diagnosis of any child presenting with liver disease, neurological abnormalities, behavioral changes, or Kayser-Fleischer rings.

Serum ceruloplasmin, the carrier of copper, is usually, but not always, reduced. Definitive diagnosis requires assessment of 24-hour urinary copper excretion and copper quantification in liver tissue obtained by biopsy.

Wilson's disease may present as fulminant liver failure, usually in association with a hemolytic crisis due to copper toxicity to red blood cells.

The therapy is penicillamine, which allows the excretion of copper into the urine. Since the forecast depends on early treatment and individual reaction for therapy, it is important to consider this diagnosis in every child with signs of chronic liver disease.

Ischemic hepatitis occurs as a result of congestive heart failure, shock (eg, dehydration), asphyxia, cardiac and respiratory arrest, or convulsions. Usually, aminotransferases are elevated in the absence of other markers of severe liver disease. This disorder is caused by hypotension/hypoperfusion of the liver during one of the above events, the liver is affected as an "innocent bystander" of the initial process.

Ischemic hepatitis may resemble infectious hepatitis but is easily distinguished by a rapid decrease in transaminase levels during the days following the initial stroke without increasing coagulopathy or hyperbilirubinemia.

Infiltrative disorders of the liver are observed with leukemia, lymphoma and neuroblastoma , but these nosological units are usually quickly identified. Similarly, primary liver tumors are easily identified in a child who presents with hepatomegaly or abdominal distention. Less commonly, children initially present with jaundice and lag in the masses.

Hepatoblastoma, hepatocarcinoma, and hemangioendothelioma account for almost two-thirds of all childhood liver tumors. Hepatocellular carcinoma tends to occur over later dates of life than hepatoblastoma and is more common among children with a history of liver disease (eg, chronic hepatitis B).

Serum levels of alpha-fetoprotein are usually elevated. CT scan usually reveals