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Biliary atresia lulu


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biliary atrsia

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Biliary atresia lulu

  1. 1. Mrs lulu Fasna k k 2 nd yr msc nursing MIMS CON
  2. 2. • Congenital absence orclosure of a normal body opening ortubularstructure.
  3. 3. Definition Biliary atresia is a condition in which the normal hepatic biliary system is disrupted. Progressive damage of extrahepatic and intrahepatic bile ducts which occur secondary to inflammation, leading to fibrosis, biliary cirrhosis, and eventual liver failure. Incidence • Biliary atresia affects approximately 1 in 10,000- 15,000 births • Biliary atresia seems to affect girls more than boys. • There does not appear to be any link to medications taken during pregnancy.
  4. 4. BASED ON THE PRESUMED TIMING OF THE OBLITERATION OF THE LUMEN OF THE EXTRAHEPATIC BILE DUCT Types The fetal-embryonic form  appears in the first 2 weeks of life 10-20% of affected neonates have associated congenital defects. . The fetal form, which occurs in up to 20% of cases of biliary atresia . Associated congenital anomalies include malrotation of abdominal viscera,  interrupted inferior vena cava, midline liver preduodenal portal vein, polysplenia,  situs inversus, and congenital heart anomalies
  5. 5. • The postnatal form of biliary atresia is typically found in neonates and infants aged 2-8 weeks. • Progressive inflammation and obliteration of the extrahepatic bile ducts occur after birth. • This form is not associated with congenital anomalies, and infants may have a short jaundice-free interval
  6. 6. Classification: Three main types of biliary atresia are surgicaly defined: 1 type I,  the common bile duct is obliterated while the proximal bile ducts are patent. 2 -type II  atresia of the hepatic duct is seen.  In type IIa, the cystic and common bile ducts are patent,  In type IIb, the cystic, common bile duct and hepatic ducts are all obliterated. 3-Type III  atresia refers to discontinuity of both right and left hepatic ducts to the level of the porta hepatis.  Unfortunately, type III biliary atresia is common, accounting for >90% of cases.
  7. 7. • Infectious agent Reovirus  rotavirus retrovirus cytomegalovirus Human papilloma virus , other agent
  8. 8. • AUTOIMMUNE MECHANISM • There is clearly a predominance of lymphocytes in BA liver tissue. • 10 of 11 patients with BA were positive for serum IgG and IgM antineutrophil cytoplasmic antibodies (ANCA in BA patients compared with children and adults with other liver diseases
  9. 9. • It has been proposed that the embryonic form of BA is caused by defective development of the biliary tree. • The association of anomalies of visceral organ symmetry with BA (the polysplenia or BA splenic malformation syndrome) • genes that control normal situs development are also key regulators of normal extrahepatic bile duct development.
  10. 10. • Abnormal remodeling of the ductal plate leads to the ductal plate malformation that is present in congenital hepatic fibrosis and other bile duct dysplasias
  11. 11. • Vascularlesion/arteriopathy • Inherited mutations • polysplenia • asplenia syndromes • Othergenes • Somatic mutations • Toxin exposure
  12. 12. • The biliary system is the network of tiny tubular structures and ducts that drain bile from the liver to the small intestine, where it helps the digestive process • .
  13. 13. • Bile is a liquid secreted by liver cells, made up of cholesterol, bile salts and waste products (including bilirubin] • Biliary atresia progressively destroys the bile ducts that carry bile from the liver to the intestine, beginning outside the liver and later affecting bile ducts inside the liver.
  14. 14. • The damaged ducts prevent the draining of bile from the liver; as a result, bile trapped inside the liver causes damage and scarring that can lead to cirrhosis
  15. 15. • As the liverbecomes scarred, it presses against the walls of the veins. This constricts the veins and blood cannot pass through themproperly. The result is portal hypertension (high blood pressure in the portal vein). • This congenital disorderbegins to progress very soon afterbirth. In its most common form, extrahepatic biliary atresia, ducts outside the liverare affected first.
  16. 16. • Jaundiced infants with • Acholic stool • Darkurine • Enlarged firmliver • Late manifestations • LiverCirrhosis • Varices fromPortal Hypertension
  17. 17. What are the symptoms of biliary atresia?  Babies with biliary atresia usually appear healthy when they are born. Symptoms of the disease typically appear within the first two weeks to two months of life. Those symptoms include:  -A baby with biliary atresia usually develops jaundice at two or three weeks after birth.  Jaundice − a yellow coloring of the skin and eyes due to a very high level of bilirubin (bile pigment) in the bloodstream.  -Dark urine - The bilirubin is filtered by the kidney and removed in the urine. -
  18. 18. Acholic stools (clay-colored stools) -- because no bile or bilirubin coloring is being emptied into the intestine.  -Also, the abdomen may become swollen from a firm, enlarged liver.  -Weight loss and irritability -- develop when the level of jaundice increases.
  19. 19. • Small proportion of children will present with vit k dependent coagulopathy and bleeding. • Features of cirrhosis o Ascites o Splenomegaly o Hard liver
  20. 20. How is biliary atresia diagnosed? • Blood tests for liver function abnormalities. • X-rays of the abdomen- • look for an enlarged liver and spleen. • abdominal ultrasound- to find out whether there is a small gall bladder or none at all. • A nuclear test-HIDA scan- • determines the flow of bile. • In this scan, a radioactive dye is injected into the infant's vein. The dye acts like bilirubin. If the baby has biliary atresia, the liver will take up the dye but it will not be able to flow through the damaged biliary system into the small intestine. • liver biopsy.
  21. 21. Investigations continue---- Cholangiography .This is done by injecting contrast material through the gallbladder. To find the communication between the biliary tree and the gastrointestinal tract, Duodenal intubation To performthis study, a nasogastric tube is placed in the distal duodenum. The absence of bilirubin in aspirated fluid suggests obstruction. Liverbiopsy • Percutaneous liverbiopsy is useful in evaluating neonatal cholestasis. • Histologic findings, including bile-duct proliferation and obstruction,
  22. 22. Associated abnormalities? Te n to 1 5 pe rce nt o f infants with biliary atre sia m ay be bo rn with o the r pro ble m s: • Heart defects. • Spleen (polysplenia). • Blood vessels (inferiorvena caval anomalies, pre- duodenal portal vein). • Intestine (situs-inversus ormalrotation).
  23. 23. HOWIS BILIARY ATRESIA TREATED? -TheKasaiprocedureis anoperationto createanopenduct so bilecandrainfromtheliver. -Thesurgeonremoves thedamagedducts outsideof theliver (extrahepatic ducts) andreplaces themwithapieceof the baby's ownintestine. This new duct allows biletopass fromtheliverinto theintestine. -TheKasaiprocedureis not acureforbiliaryatresia, but it does allow babies to grow andhavefairlygoodhealthfor severalyears. -Whenthis proceduredoes not work, livertransplantation cancorrect this problem.
  24. 24. • Age. Surgery is most successful in infants youngerthan two to three months of age. • Extent of liverdamage (cirrhosis) at the time of surgery. • The numberand size of microscopic ducts in the scarred tissue that can drain bile. • The experience of the surgical and medical team.
  25. 25. Guidelines fornutrition fora child with BA Childre n with live r dise ase have a faste r m e tabo lism than he althy childre n. This m e ans that childre n with biliary atre sia m ay re q uire m o re calo rie s. • Achild with biliary atre sia canno t pro pe rly dig e st fats. This is be cause no t e no ug h bile g e ts to the inte stine . Due to live r dam ag e , the re m ay also be a lo ss o f vitam ins and pro te in. • A well-balanced diet, consisting of three meals a day plus small snacks in between meals.
  26. 26. • Vitamin supplements. • Adding medium-chain triglyceride (MCT) oil to foods and liquids orinfant formulas. MCT adds extra calories that will help yourchild grow. • High-calorie liquid feedings may be recommended if yourchild is too ill to eat normally. Feedings are given through naso- gastric tube. • Altho ug h dig e stio n m ay re turn to no rm alafte r surg e ry, e xtra vitam ins o r MCT o ilm ay be ne e de d.
  27. 27. What are the complications of biliary atresia and what can be done forthem? • Infection in the bile ducts. This is usually treated using intravenous antibiotics. • Jaundice oritching may occur. • These can often be treated successfully with phenobarbital (forjaundice), • cholestyramine- to relive pruritus • ursodeoxycholic acid (foritching). been shown to enhance bile flow
  28. 28. • Many patients with cirrhosis have changes in blood flow through the liverand intestines. These changes may produce problems such as easy bruising of the skin, nasal bleeding, retention of body fluid and varices in the stomach and esophagus. • If retention of body fluid occurs, it can be treated with diuretics and potassium replacement
  29. 29. LiverTransplant • If thereis still not enoughbileflow withtheKasai procedure, livertransplantationis afinal option. Aliver transplant operationremoves thedamagedliverand replaces it withanew liverfromadonor. • Aftertransplant surgery, thechild's healthmayimprove quitequickly. However, thechild's bodymight reject the new organ. To prevent rejection, astrict scheduleof anti- rejectionmedications must betaken. • Afteratransplant, ongoinglifelongcareis required. Frequent contact withphysicians andothermembers of the transplant teamis also necessary.
  30. 30. Monitor • stool color •ascites, peripheral edema, • hepato/splenomegaly • anorexia • urine color •lethargy •jaundice •bleeding •pruritus, vital signs, signs of dehydration, and weight