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LECTURE: " Neonatal jaundice " Icteric staining of the skin occurs in term infants with bilirubin levels of 80-90 mmol \l, premature - more than 120 mmol \l , in adults 35-40 mmol \l Classification causes of neonatal hyperbilirubinemia 1. Konyugatsionnye jaundice: -physiological jaundice -a prolonged jaundice in premature -family nonhemolytic neonatal hyperbilirubinemia ARIES-Lyutseya 2. Hemolytic jaundice: -hemolytic disease of the newborn -microspherocytic congenital hemolytic jaundice Minkowski Chauffard 3. Mechanical jaundice: -biliary atresia -syndrome accumulation of bile Classification causes of neonatal hyperbilirubinemia 4. Parenchymal jaundice: -congenital hepatitis -toxoplasmosis -sepsis 5. Enzymopathies: -family Congenital nonhemolytic jaundice Crigler-Najjar - Galactosemia (congenital deficiency of the enzyme-fosfogalaktozo uridemtransferazy FGVT) - Jaundice due to congenital deficiency of glucose 6-phosphate dehydrogenase (G-6-PD) Physiological jaundice - transistor observed in 86.5% of full-term newborns. Transient hyperbilirubinemia caused by rapid hemolysis newborn temporary immaturity glyukouroniltransferazy enzyme. The general condition of the child is not broken.Yellowness of the skin of the child appears at 2-3 days of age and disappears at 10-14 day. Red blood cells, the size of the liver and spleen were normal. Treatment: 5% glucose or 0.9% sodium chloride, phototherapy. Weather favorable. Konyugatsionnaya jaundice in premature infants is a prolonged duration (4-6 months), and delivers more picture. Clinical jaundice increases slowly, with hepatomegaly and splenomegaly are absent, feces and urine unpainted, anemizatsii not. At the heart of jaundice is temporary immaturity enzyme factors liver and excreted bilirubin konyugiruyuschih Neonatal hyperbilirubinemia - a disease based on the hemolysis of the fetus and newborn due to incompatibility of maternal and fetal blood by erythrocyte antigens. Etiology: HDN razvivaetsya the result of incompatible maternal and fetal blood Rh factor or group antigens. Rhesus-conflict occurs if the Rh-negative fetus has Rh-positive blood. ABO conflict develops in the presence of 0 (I) g blood of women and the fetus A (II) or B (III) g blood. Hemolysis of fetal and neonatal hyperbilirubinemia and lead to anemia. Indirect bilirubin neurotoxicity. The main source of bilirubin in the body is the hemoglobin. When a certain concentration (over 340 umol \ l) in term and (more than 200 mmol \ l) in premature infants, it can cross the blood-brain barrier and damage the structure of the brain, which leads to the development of bilirubin encephalopathy (kernicterus). Hemoglobin in the cells of the reticuloendothelial system is split into iron, bilirubin and globin. Allocated indirect bilirubin binding to albumin and globulins of blood to the liver, where konyugiruet with glucuronic acid. In the liver cells to indirect bilirubin join two molecules of glucuronic acid. In plasma indirect bilirubin albumin complex forms Indirect bilirubin - tissue poison, inhibiting oxidative processes and in various organs causing degenerative changes in the cells up to necrosis. Direct bilirubin is excreted from pechechnochnyh cells in bile capillaries. The final products of direct bilirubin is sterkobilina and urobilinogen. Infants lack putrefaction in the intestines sterkobilina not formed and released into the bilirubin neizmennenom form. Its ability to eliminate reduced because of the immaturity of the excretory system and features of the bile capillaries (narrow, a small amount). The result appears in the bile duct cholestasis, hepatitis, the formation of reactive GBN Clinic Distinguish edema (2%), icteric (88%), anemic (10%) forms. Edematous form - the heaviest, develops in utero. Massive fetal hemolysis leads to severe anemia, hypoxia, metabolic disorders, gipoprotenemii and tissue edema. Newborns are sluggish, sharply reduced muscle tone, reflexes are depressed, a cardio-pulmonary insufficiency, severe hepatosplenomegaly, belly big, barrel-shaped, Hb concentration is less than 100 g \ l. Fetus dies before birth or born in critical condition with massive edema. GBN Clinic Icteric-most common. Its main symptoms are jaundice, anemia, hepatosplenomegaly. Jaundice with orange tint appears at birth or for 1-2 hours.Than before jaundice appears, the more severe the disease. Children lethargy, drowsiness, hyporeflexia, hypotension, symptoms of kernicterus, neck stiffness, tonic convulsions, "brain scream," symptom "setting sun", big bulging fontanelle. Icteric form of HDN is accompanied by anemia. Kernicterus develops in 10% of full-term at a concentration of indirect bilirubin levels above 340 mmol \ l, 30% higher than 430 mmol \ l, 70% above 520 mmol \ l GBN Clinic anemic form adrift most benign. clinical symptoms: pale skin, anemia, enlarged liver and spleen. Indirect bilirubin concentration may be slightly raised. Antenatal diagnosis of GBN maternal history (previous abortions, stillbirths, miscarriages, birth of sick children transfusion without the Rh factor) protivorezusnyh titration of antibodies in the blood of women rezusotritsatelnyh The study of amniotic fluid obtained by amniocentesis ultrasound study Postnatal diagnosis of GBN The clinical symptoms (jaundice , anemia , hepatosplenomegaly ) Increase in blood indirect bilirubin fraction erythroblastosis , reticulocytosis , positive Coombs in rhesus conflict. Conservative treatment Phototherapy - Phototherapy for photo-oxidation of indirect bilirubin is found in the skin to form a water-soluble isomers that are excreted in urine and feces inducers of microsomal liver enzymes, phenobarbital Vitamin E, B1, B2, B6, C and cocarboxylase improves liver metabolism and stabilizes in order to inhibition of absorption of bilirubin in the gut cleansing enemas administered activated charcoal accumulation of bile syndrome cropped appointment cholagogue (magnesium sulfate, drotaverine, noshpy) Surgeries : replace blood transfusions hemosorbtion , plasmapheresis Absolute indications for exchange transfusion of blood: -indirect bilirubin concentration in the umbilical cord blood of more than 60 mmol\ l -hour increase in indirect bilirubin concentrations over 6.10 mmol\ l -indirect bilirubin concentration in the peripheral blood of more than 340 mmol\ l -severe anemia (hemoglobin less than 100g\ l) For use exchange transfusion of blood Rh (-) of the same blood group as the baby's blood Currently not whole blood transfusions and Rh (-) red cell mass, mixed with fresh frozen plasma. If HDN due to incompatibility group, then use the red cell mass 0 (I) group, plasma or AB (IV) group. The volume of transfused blood is from 170 to 180 ml \ kg body weight of the child. Prophylaxis Nonspecific: -carrying blood transfusions only given Rh-factor -prevention of abortion -range of social protection measures for women's health Specific: -introduction of Rh (antidepressant) Lg in the first 24 hours after the birth of a healthy Rh (+) child, and abortion, which facilitates rapid elimination of red blood cells from the blood of mother of the child, preventing the synthesis of Rh antibodies mother -high titer of Rh antibodies in the blood during pregnancy make the replanting of the skin on her husband 16-32 weeks pregnant 2-3 times at intervals of 4-6-8 weeks -delivery to spend 37-39 weeks of gestation by cesarean section Congenital hemolytic jaundice Minkowski Chauffard newborns The disease runs hard with severe jaundice. Reinforced hemolysis of red blood cells due to a defect in the maturation of the violation in these carbohydrate metabolism, lack of ATP in the stroma of erythrocytes. Clinic: jaundice, hyperbilirubinemia, anemia, enlargement of the liver, especially the spleen, decreasing the osmotic resistance of red blood cells, red blood cells microspherocytic grit, reticulocytosis and normablastozom Treatment: The treatment of the same, as in the HDN Biliary atresia - a birth defect Clinic: jaundice appearing in the first days of life and gradually increases to 1-1.5 months, discolored stools, bloating, enlarged liver and spleen, dark urine, (+) response to bile pigments (-) reaction to urobilinogen, the level of direct fraction increases, signs of intoxication increases, signs of portal hypertension, blood clotting Treatment: Surgery Accumulation of bile syndrome , due to total or partial blockage of the bile duct bile plugs Clinic: jaundice, discolored stools, dark urine, (+) response to bile pigments, enlarged liver, blood appears in indirect bilirubin fraction Treatment: 25% magnesium sulfate solution inside of 5ml 3 times, a heating pad or diathermy to the liver Jaundice due to congenital enzymopathies Congenital nonhemolytic jaundice family - CriglerNajjar syndrome, is a rare enzimopaty caused by congenital deficiency of the enzyme glucuronyl transferase in the liver cells, which leads to disruption of binding and release of bilirubin Clinic: jaundice at birth, continues throughout the child's life, the amount of indirect bilirubin from 256 to 765 mmol \ l, urine and feces normally painted, there is no hepatosplenomegaly Treatment: symptomatic, in severe cases, Plant. Most of the children die before their first birthday due to CNS. Jaundice due to congenital enzymopathies Galactosemia - due to congenital deficiency of the enzyme fosfogalaktozauridil transferase, which regulates the exchange of galactose Clinic: jaundice with one day of life, an increase in the direct fraction of bilirubin, hepatomegaly, vomiting, general intosikatsiya, glycosuria The diagnosis is the detection of high content of galactose in the blood and the detection galactosuria. Treatment: elimination of milk from the diet, replacing the artificial mixture containing galactose Jaundice due to congenital deficiency of the enzyme glucose -6- phosphate dehydrogenase The lack of this enzyme leads to a violation of exchange in red blood cells and to their breakdown. Children with deficiency of this enzyme are basically healthy Clinic: severe jaundice in the first days, with anizopoykilotsitozom anemia, reticulocytosis, normablastoz, indirect hyperbilirubinemia Diagnosis: Determination of glucose-6-phosphate dehydrogenase deficiency in red blood cells Treatment: symptomatic, with high hyperbilirubinemia shown exchange transfusion of blood THANK YOU FOR ATTENTION !