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1、Neonatology: Neonatal Jaundice,Contents,Billirubin metabolism in normal neonates Special problems in neonates The diseases in relation with Neonatal Jaundice Dangerous of the Hyperbillirubinemia,Billirubin +plasm albumin-in blood,Capillary bile duct,breakdown of hemoglobin 80%,ineffective erythropoi
2、esis,Reticulo- endothelial system,liver,Bilirubin+Protein Y、Z,conjugative,conjugated Bi,conjugated Bi,Reabsoption:90%,Hepatic cells,Glucuronic acid 90% Glucose conjugates Xylose conjugates,intestine lumen,Bacteria,urobilinogen 80% excrete,Kidney 10%,Portal vein,Urine :urobilinogen (0-4mg/d),Fecal:ur
3、obilinogen(40-280mg/d),breakdown of hemoproteins,Normal Billirubin Metabolism in neonates,Intrahepatic hemachrome Tissue hemachrome,Smooth endoplasmic reticulum,Hepato- enteral circulation,Specificity of Billirubin Metabolism in newborns,Increased bilirubin formation Excesive RBCs after birth Shorte
4、r half life of fetal hemoglobin Heme oxygenase Increased bilirubin originated from bypass,Specificity of Billirubin Metabolism in newborns,Insufficient in transiting bilirubin albumin linking/transporting bilirubin ,Specificity of Billirubin Metabolism in newborns,Insufficient in treating bilirubin
5、by liver Y. Z protein Under developed of hepatic enzyme Activity of hepatic enzyme easily being undermined Serums glucose rapidly ,Specificity of Billirubin Metabolism in newborns,Load of hepatoenteral circulation Intestine flora Activity of-Glucuronidase within mesocaval High quantity of bilirubin
6、in meconium,Physiological Jaundice,Pathological Jaundice,Presenting earlier, 12mg/dl increasing rapidly, 5mg/dl/d Longer duration: Term:2 w preterm:4 w Persistently progressing, or re-presence after disappearing,Etiology of Pathological Jaundice,Unconjugated Hemolytic Disease of the Newborn alloimmu
7、ne hemolytic (ABO and Rh types) deformity of RBC RBC Enzyme deficiency Extravascular Hemolysis Breastfeeding Jaundice Erythrocytosis,Etiology of Pathological Jaundice,Conjugated Neonatal Hepatic diseases Congenital bile duct disorders Congenital metabolic disorders,Etiology of Pathological Jaundice,
8、Mixed by Unconjugated with Conjugated Infections,Hemolytic disease of newborn,ABO type incompatability alloimmune hemolytic disease 50% happened in first pregnancy 20 ABO type incompatability,incidence 10 majority maternal type O and infant type A or B RH type incompatability alloimmune hemolytic di
9、sease No Occurrence in first pregnancy, except the one had history of abortion and transfusion Antigenic: DECce RH D most commonly seen,Hemolytic disease of newborn,Clinical manifestation Jaundice Present at different time: ABO 2-3d. RH24h Level of TBi: ABO: slightly elevated RH: severely elevated a
10、nd progressing Anemia: severe in RH and may complicated with heat failure Hepatosplenomegaly Hydrops fetalis (universal edema of the fetus),Hemolytic disease of newborn,Laboratory investigation Prompt hemolysis indicators RBC/Hb Reticulocyte Nuclear RBC 10% blood type incompatibility Maternal: O, ne
11、wborn: A,B Maternal: Rh-, newborn: Rh+,Hemolytic disease of newborn,Laboratory investigation Coombs test Rh: Direct: positive Indirect: positive (Ab. present and the type) ABO: Direct: positive Free antibody present Ab. Release test: positive,Hemolytic disease of newborn,Diagnosis History: Past prod
12、uction history of the mother Newborn with hyperbilirubinemia, anemia Stillbirth Hydrops fetalis Clinical manifestation Laboratory evidences,Hemolytic disease of newborn,Prenatal diagnosis Mother with Rh-negative: measure Rh Ab in GA 28,32,36 w Examining amniotic fluid: bilirubine level,Hemolytic dis
13、ease of newborn,Prevention Mother with Rh-negative delivering the first fetus given anti-D Ab.,Neonatal Hepatitis,Intrauterine, intrapartum and during delivering infected Virus: common: CROTCHS/CMV Pathogenesis Cholestasis: Capillary bile duct and hepatic duct obstruction Liver cells and mesenchymal
14、 inflammatory change portal area hyperplasia,Neonatal hepatitis,Clinical manifestation Obstructive jaundice Jaundice represent following the fading of physiological jaundice Dark yellow urine and grey-white stool Gastrointestinal symptoms: anorexia, hyperphagia, vomiting, diarrhea Anemia hepatosplen
15、omegaly,Neonatal hepatitis,Laboratory investigation TBi , VDB: direct and indirect positive Hepatic function: ALT , serum proteins , albumin Serous virology Positive: CMV IgG/M, TOXO IgG/M HBV: antigens and antibodies,Biliary atresia cholestasis,Severe obstructive jaundice with high Conjugated Bi Pe
16、rsistent grey-white stool Severe hepatosplenomegaly Progressive worsening in hepatic function Diagnosis based on ultrasound, CT and MRI Isotope ECT may helpful,Jaundice with Infection,When there are no find in particular General manifestation of the infection Blood stream infection or organ infection Both conjugated and unconjugated Bi Jaundice subsided when infection controled,Breastfeeding Jaundice,Babies with exclusive breast feed