新生儿和婴儿胆汁淤积(英文版).ppt
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1、Neonatal and Infantile Cholestasis,Ying-kit Leung, MD, FAAP President, Hong Kong Society of Paediatric Gastroenterology, Hepatology and Nutrition, Yantai, Shandong, July 2006,DEFINITION,Neonatal cholestasis is defined as conjugated hyperbilirubinemia developing within the first 90 days of extrauteri
2、ne life. Conjugated bilirubin exceeds 1.5 to 2.0 mg/dl. Conjugated bilirubin generally exceeds 20% of the total bilirubin.,Bilirubin Production,Biliverdin,Bilirubin,erythrocyte hemoglobin,muscle myoglobin,cytochromes catalases,heme oxygenase,biliverdin reductase,reticulo-endothelial cell,Bilirubin,A
3、lbumin,liver,CO + Fe,Heme,GST,Bilirubin Uptake, Conjugation, Excretion,B,B,Alb,G,B,G,G,B,R,GLUCURONYL TRANSFERASE,BILE CANALICULUS,2. UPTAKE,3. CONJUGATION,4. EXCRETION,E.R.,B-G,B-G,B-,B-G,B-G,B-G,B,uBg,sB,uBg,B-G,dark urine,acholic stools,Conjugated Hyperbilirubinemia,Conjugated hyperbilirubinemia,
4、extrahepatic,intrahepatic,Neonatal Cholestasis,EXTRAHEPATIC ETIOLOGIES,Extrahepatic biliary atresia Choledochal cyst Bile duct stenosis Spontaneous perforation of the bile duct Cholelithiasis Inspissated bile/mucus plug Extrinsic compression of the bile duct,INTRAHEPATIC ETIOLOGIES,Idiopathic Toxic
5、Genetic/Chromosomal Infectious Metabolic Miscellaneous,INTRAHEPATIC ETIOLOGIES,Idiopathic Neonatal Hepatitis Toxic TPN-associated cholestasis Drug-induced cholestasis Genetic/Chromosomal Trisomy 18 Trisomy 21,INTRAHEPATIC ETIOLOGIES,Infectious Bacterial sepsis (E. coli, Listeriosis, Staph. aureus) T
6、ORCHES Hepatitis B and C Varicella Coxsackie virus Echo virus Tuberculosis,INTRAHEPATIC ETIOLOGIES,Metabolic Disorders of Carbohydrate Metabolism Galactosemia Fructosemia Glycogen Storage Disease Type IV Disorders of Amino Acid Metabolism Tyrosinemia Hypermethioninemia,INTRAHEPATIC ETIOLOGIES,Metabo
7、lic (cont.) Disorders of Lipid Metabolism Niemann-Pick disease Wolman disease Gaucher disease Cholesterol ester storage disease Disorders of Bile Acid Metabolism 3B-hydroxysteroid dehydrogenase/isomerase Trihydroxycoprostanic acidemia,INTRAHEPATIC ETIOLOGIES,Metabolic (cont.) Peroxisomal Disorders Z
8、ellweger syndrome Adrenoleukodystrophy Endocrine Disorders Hypothyroidism Idiopathic hypopituitarism,INTRAHEPATIC ETIOLOGIES,Metabolic (cont.) Miscellaneous Metabolic Disorders Alpha-1-antitrypsin deficiency Cystic fibrosis Neonatal iron storage disease North American Indian cholestasis,INTRAHEPATIC
9、 ETIOLOGIES,Miscellaneous Arteriohepatic dysplasia (Alagille syndrome) Nonsyndromic paucity of intrahepatic bile ducts Carolis disease Bylers disease, PFIC Congenital hepatic fibrosis,COMMON ETIOLOGIES,Premature infants Sepsis/Acidosis TPN-associated Drug-induced Idiopathic neonatal hepatitis Extrah
10、epatic biliary atresia Alpha-1-antitrypsin deficiency Intrahepatic cholestasis syndromes,CLINICAL PRESENTATION,Jaundice Scleral icterus Hepatomegaly Acholic stools Dark urine Other signs and symptoms depend on specific disease process,GOALS OF TIMELY EVALUATION,Diagnose and treat known medical and/o
11、r life-threatening conditions. Identify disorders amenable to surgical therapy within an appropriate time-frame. Avoid surgical intervention in intrahepatic diseases.,Bu,Bc Bu,Bu,Hemolysis Rh ABO,Breast Milk,Physiological,Hypothyroidism,Bc Bu,dark urine,acholic stools,Bc Bu,BEWARE!,dark urine,acholi
12、c stools,hepatosplenomegaly bilirubinuria conjugated bilirubin abnormal LFTs,EVALUATION,Basic evaluation History and physical examination (includes exam of stool color) CBC and reticulocyte count Electrolytes, BUN, creatinine, calcium, phosphate SGOT, SGPT, GGT, alkaline phosphatase Total and direct
13、 bilirubin Total protein, albumin, cholesterol, PT/PTT,EVALUATION,Tests for infectious causes Indicated cultures of blood, urine, CSF TORCH titers, VDRL Urine for CMV Hepatitis B and C serology Ophthalmologic examination,EVALUATION,Metabolic work-up Protein electrophoresis, alpha-1-antitrypsin level
14、 and phenotype Thyroid function tests Sweat chloride Urine/serum amino acids Review results of newborn metabolic screen Urine reducing substances Urine bile acids,EVALUATION,Radiological evaluation Ultrasonography Patient should be NPO to increase likelihood of visualizing the gallbladder Feeding wi
15、th exam may demonstrate a functioning gallbladder Hepatobiliary scintigraphy Premedicate with phenobarbital 5mg/kg/d for 3-5 days,EVALUATION,Invasive studies Duodenal intubation Percutaneous liver biopsy Percutaneous transhepatic cholangiography Endoscopic retrograde cholangiopancreatography (ERCP)
16、Exploratory laparotomy with intraoperative cholangiogram,ESTIMATED FREQUENCY OF VARIOUS CLINICAL FORMS OF NEONATAL CHOLESTASIS,PROPOSED SUBTYPES OF INTRAHEPATIC CHOLESTASIS,intrahepatic or extrahepatic ? treatable disorder ? liver damage ? complications of cholestasis ?,Investigation of Cholestasis,
17、Objectives,X-ray spine: butterfly vertebrae (Alagille) skull, long bones (intrauterine infection) sweat test (cystic fibrosis) ophthalmological examination cataract (galactosemia, intrauterine infection) retinopathy (intrauterine infection) posterior embryotoxon (Alagille) others bone marrow (Nieman
18、n Pick disease type C) bile acids,Investigation of Cholestasis,Special Tests,ultrasound choledochal cyst etc. post-prandial contraction of gall bladder hepatobiliary scan (99mTc - H / B / DIS / PIP / - IDA) after pre-treatment with phenobarb or cholestyramine,Investigation of Cholestasis,Special Tes
19、ts (cont),ultrasound choledochal cyst etc. post-prandial contraction of gall bladder hepatobiliary scan (99mTc - H / B / DIS / PIP / - IDA) after pre-treatment with phenobarb or cholestyramine ERCP (endoscopic retrograde cholangiopancreatography),Investigation of Cholestasis,Special Tests (cont),End
20、oscopic Retrograde Cholangio-Pancreatography,Investigation of Cholestasis,Special Tests (cont),liver histology (needle biopsy) biliary atresia: portal ductal proliferation neonatal hepatitis: giant cells specific disorder e.g a1-antitrypsin,Biliary Atresia,Definition - Progressive scarring of bile d
21、ucts outside and inside of the liver that leads to complete blockage of bile flow in the first three months of life. Bile is the yellow fluid made in the liver that helps digest food (fat) in the intestine,Anatomy in Biliary Atresia,Kasai Procedure,KASAI PROCEDURE,Performed for biliary atresia that
22、is not surgically correctable with excision of a distal atretic segment. Roux-en-Y portoenterostomy Bile flow re-established in 80-90% if performed prior to 8 weeks-old. Bile flow re-established in less than 20% if performed after 12 weeks-old,KASAI PROCEDURE,Success of the operation is dependent on
23、 the presence and size of ductal remnants, the extent of the intrahepatic disease, and the experience of the surgeon. Complications are ascending cholangitis and reobstruction as well as failure to re-establish bile flow.,LIVER TRANSPLANTATION,Survival rates approach 80% at 1 year and 70% at 5 years
24、. Biliary atresia is the most common indication for transplant and may be the initial treatment when detected late or may be used as a salvage procedure for a failed Kasai. Used early in cases of tyrosinemia.,Outcome after Kasai procedure,Short-term - bile flow dependent on age at Kasai 90 days 10-2
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