Cholestasis newborn - Causes, Symptoms, Diagnosis and Treatment

Cholestasis newborn - A pathological condition which is characterized by impaired bile secretion, leading to hyperbilirubinemia and jaundice. Clinical manifestations include subikterichnost sclera, yellowing of the mucous membranes and skin, hepatomegaly, delay in physical development, hypovitaminosis A, D, K, E. Diagnosis of cholestasis newborn is to identify the underlying disease, laboratory evaluation of liver ultrasound, hepatobiliary scan and liver biopsy. Treatment depends on the etiologic factors and includes a balanced diet, the introduction of the diet of easily digestible fats, vitamin therapy, or surgery.

  • Causes of neonatal cholestasis
  • Symptoms and diagnosis of neonatal cholestasis
  • Treatment of neonatal cholestasis
  • Cholestasis newborn - Treatment

  • Cholestasis newborn


    Cholestasis newborn - is a heterogeneous condition in pediatrics and neonatology, which manifests itself in violation excretion of bile to the further development of hyperbilirubinemia. The exact prevalence of pathology difficult to establish, because this condition often included in the structure of the underlying disease. The therapeutic strategy and the prognosis for the child's life depends on the etiology of cholestasis newborn. As a rule, the elimination of the leading pathology leads to the relief of cholestatic syndrome. In severe cases may require a liver transplant. The outcome varies from benign course long before the rapid development of cirrhosis, liver failure and fatal in the first year of life.

    toxoplasmosis, rubella, CMV, herpes virus, syphilis, hepatitis B and others. Provoke cholestasis neonatal metabolic disorders are such as galactosemia, cystic fibrosis, tyrosinemia, α1-antitrypsin deficiency. Genetic factors are Alagille syndrome, cystic fibrosis, progressive familial intrahepatic cholestasis, and others. Gestational alloimmune liver damage develops when transplacental passage of maternal IgG in the child's body. This causes activation of the complement system, which is capable of damage hepatocytes. Long-term parenteral nutrition in severely premature babies and the availability of short bowel syndrome related to toxic causes of cholestasis newborn.

    The list of factors extrahepatic cholestasis newborn includes all disorders that impair the flow of bile from the liver. The most common reason - Biliary atresia. Most often seen in premature babies. Sometimes, in addition to the extrahepatic biliary tract, affects and intrahepatic bile ducts. The exact etiology of this condition is unknown. Presumably, development of biliary atresia cause infectious diseases of the mother during pregnancy.

    Trigger the onset of clinical symptoms in the newborn cholestasis is a violation of the excretion of direct bilirubin, provoking an increase in its level in the blood - hyperbilirubinemia. This leads to a deficiency of bile acid in the systemic circulation. Since they are responsible for the transport and absorption of fats and fat-soluble vitamins (A, D, K, E) in the body of the child occurs deficiency. The result is a lack of weight gain, hypovitaminosis, delayed physical and psychomotor development.

    portal hypertension. She, in turn, becomes the cause of ascites and bleeding from esophageal varices.

    Diagnosis of neonatal cholestasis involves the collection of anamnesis, physical examination of the child, laboratory and instrumental methods of research. In a survey of parents pediatrician or neonatologist pay attention to all possible etiological factors: diseases of the mother during pregnancy, genetic disease, congenital anomalies development of the child. On physical examination, it is determined by body weight deficit and growth, yellowness of the sclera, mucous membranes and skin, hepatomegaly. In the biochemical analysis of blood revealed hyperbilirubinemia due to direct fraction, Dysproteinemia. Perhaps the increase in transaminases - ALT and AST. Characteristic signs of cholestasis newborn - acholic, rich in fats, calories and saturated with urine urobilinom. In order to avoid TORCH-infections carried ELISA and bacterial urine culture. Tests are also used for metabolic disorders, or genetic abnormalities that may cause the development of neonatal cholestasis - assays NaCl content in sweat, urine galactose, α1-antitrypsin levels in the blood, etc.

    Diagnostics for suspected neonatal cholestasis includes ultrasound, hepatobiliary scan and liver biopsy. The first stage - ultrasound of the abdominal cavity. It reveals hepatomegaly, structural abnormalities of the gallbladder and bile ducts. Further, scanning is performed to assess liver bile excretion at all levels of the biliary tract - from intrahepatic duct to the duodenum. If unable to confirm the diagnosis of these methods is a liver biopsy. When neonatal cholestasis histological analysis of biopsy may reveal enlarged portal triad, proliferation and fibrosis of the bile ducts with Biliary atresia, polynuclear giant cells in congenital hepatitis and others. If you suspect that the origin of alloimmune neonatal cholestasis is defined by a large amount of iron in hepatocytes or tissues of the lips.

    triglycerides in the body. Ursodeoxycholic acid is hepatoprotector and cholagogue, stimulating the liver and biliary tract, and relieves hyperbilirubinemia.

    If confirmed Biliary atresia is already at 1-2 months of age is held portoenterostomiya Kasai method. This operation significantly improves survival, but in the future leads to frequent recurrent cholangitis, cholestasis and persistent delays in psychophysical development. If you suspect that the origin of alloimmune neonatal cholestasis (even unconfirmed diagnosis) shows the introduction of intravenous immunoglobulin, or exchange transfusion. Against the background of rapidly developing liver cirrhosis need an organ transplant.

    Prediction and prevention of neonatal cholestasis



    The prognosis of neonatal cholestasis is directly dependent on the underlying disease and the effectiveness of therapeutic interventions. When Biliary atresia and absence of early surgery is rapidly developing liver failure. Further, this state is transformed into cirrhosis with increasing hypertension in the portal vein system, already in the first months of a child's life. Death occurs typically at age 10-12 months. Infectious and metabolic causes of cholestasis newborn may have a good prognosis and rapidly progressive course. Alloimmune liver damage in patients without early treatment in most cases ends in death.

    Prevention of neonatal cholestasis includes antenatal protection of the fetus, medical-genetic counseling and couples planning pregnancy, regular attendance at antenatal clinics, the full completion of all necessary tests and studies. These measures make it possible to assess the risk of genetic disease before the pregnancy and identify dangerous diseases already in the early stages.