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PATHOPHYSIOLOGY -The liver. Anne Aspin 2010 Bile. Thick greenish yellow fluid secreted by liver cells. Alkaline. Emulsifies fats in the intestine. It stimulates peristalsis in the intestine, acts as a natural aperient. Deodorant in faeces. During fetal life, placenta is the principal route to eliminate unconjugated bilirubin. In newborn infants hepatic cells excrete conjugated water soluble bilirubin into biliary system and gastro intestinal tract A guide to the liver. Located behind lower ribs on the right side of the abdomen. An adult- size of a rugby ball. 300 billion specialised cells- well organised intricate system of bile ducts and blood vessels. The little bile ducts drain every bile cell and join together like tributaries entering a stream to form one main duct from each lobe. The two ducts join to form the common hepatic duct, this joins the duct from the gall bladder (cystic duct), to form the common bile duct. This joins the small intestine through the Ampulla of Vater. The gall bladder and its ducts. Blood supply to and from the liver. Hepatic artery- which divides into fine branches –to the fine bile ducts. Portal vein carries nutrients from the stomach and intestine to the liver (also from spleen). Portal vein divides into fine branches- into sinusoids. Blood leaves sinusoids via hepatic vein to heart. Functions of the liver. Breaks down fat to produce energy. Amino acids broken down to form urea. Detoxicate drugs. Vit A synthesized to carotene. Liver main heat producing organ. Plasma proteins synthesized. Tissue cells broken down – uric acid and urea. Carbohydrate converted to fat for storage. Functions (cont) Prothrombin and fibrinogen synthesized from amino acids. Antibodies and antitoxins are manufactured. Heparin is manufactured. Stores: Vit A and D, anti anaemic factor, iron from diet, glucose stored as glycogen and back to glucose in the presence of insulin. Bile is formed. Guess which one was on TPN?. Extremely Low Birth Weight Infant Less than 1000g, less than 27/40 Susceptible to all complications of prematurity Thermoregulation – high surface area to body weight Hypoglycaemia – stress and low glycogen stores ELBW and electrolytes – PDA,IVH,CLD,BPD Compromised renal function – decreased GFR – decreased ability to reabsorb bicarbonate – inability to concentrate urine Fluids Nutrition High energy requirements for growth Heat loss raises energy need Trophic feeding stimulates gastro-intestinal tract and prevent mucosal atrophy Prolonged TPN may result in cholestasis and elevated triglyceride levels. Breast milk Hyperbilirubinaemia Increased production of bilirubin transfusion, infection Decreased activity of transferase enzyme due to hypoxia,infection,hypothermia or thyroid deficiency Hyperbilirubinaemia (cont) Block of transferase enzymes (drugs) Decreased enzyme- prematurity Decreased bilirubin uptake by liver cells Kernicterus Occurs when unconjugated bili crosses blood-brain barrier staining basal ganglia, pons and cerebellum Those who do not die with kernicterus are deaf, mental retardation and cerebral palsy Phototherapy Decreases Breaks unconjugated bilirubin levels down so water soluble, so can be easily excreted Hepatic complications in preterm babies on TPN Cholestasis 7.4% - 84% reported 1971 – baby 71days old, cholestasis, bile duct proliferation, early cirrhosis 1st Early feeding reduces above Increased biochemical test results of damage, function and excretion Prevention Early enteral feeding Prevention of sepsis Cycling TPN Mucus fistula re-feeding Ursodeoxycholic acid Differential diagnosis Jaundice birth-24hrs Sepsis Haemorrhage Cytomegalovirus Rubella Congenital toxoplasmosis Jaundice day 2-3 Physiological jaundice Criggler Najjar syndrome Jaundice after day 3 Septicaemia Syphillis Toxoplasmosis Cytomegalovirus Jaundice after one week Breast milk Septicaemia Congenital atresia of ducts Hepatitis Rubella Herpes Galactosaemia Hypothermia Haemolytic disease Jaundice after one month Inssipated bile syndrome TPN related cholestasis Cytomegalovirus Syphillis Toxoplasmosis Biliary atresia. What is biliary atresia?. Inflammation develops within bile ducts around time of birth, either within ducts inside or outside of the liver. Bile ducts outside liver – irreversible damage preventing bile flow. Signs of biliary atresia. Seem well, but white of the eyes are yellow, yellowing of the skin. Yellow coloured urine. Pale stools. Bleeding –prolonged. Investigations. Haematological and liver function tests. Screening for infection and metabolic causes. Ultrasound examination. Radionuclide studies. Percutaneous liver biopsy. Always look for yellow pooh!!! Treatment. Surgical operation called a Kasai procedure. Major surgery, very sick babies. Extrahepatic biliary atresia. The blocked duct is removed. A single open duct is then joined to a loop of intestine. Hepaticojejunostomy. Post operation. IV fluids. Pain relief. Antibiotics. Parents. Medications regime. Medications. antibiotics –to reduce risk of cholangitis. Vitamins A,D,E,K - due to poor bile flow which reduces absorption of dietary fat soluble vits. Phenobarbitone – given to increase bile flow. Questran – improves liver function and removes substances which cause the skin to itch. IV Medications (cont) – excretion of excess fluid which otherwise collects in the abdomen. Ursodeoxycholic acid – promotes the flow of bile. Ranitidine – reduces stress induced stomach irritation. Spironalactone Some complications may occur. Cholangitis Ascites Low albumins Portal hypertension Itching Cholangitis. An infection of the bile ducts resulting in inflammation. Pyrexia Increasing jaundice Further liver damage IV antibiotics. Ascites An abnormal collection of fluid in the abdomen around the organs. May be associated with general oedema. Protruding abdomen Shiny, tense Prominent veins Rapid weight gain Shortness of breath Reduced appetite. Complications of ascites Failure to thrive and vomiting Infection Restricted movement Breathing difficulties. Albumins. If ascites persists, disease process has affected the livers ability to make albumin. Diet will be assessed to reduce fluid and salt intake. 20% Albumin infusion. Portal hypertension. High blood pressure in the portal vein, the main vein carrying blood from the gut to the liver. Occurs due to scarring in the liver which causes back pressure in the portal vein. This causes veins like varicose veins to develop in the lining of the oesophagus, stomach or intestine. Portal hypertension Blockage of portal vein –thrombus. Scarring in the liver Increased resistance to blood leaving the liver, due to obstruction or heart disease. Portal hypertension (cont) spleen – from back pressure, effects breakdown of RBC as new are made, when spleen enlarged it removes more than it should – effects platelets needed for blood clotting. Ascites –from back pressure on blood vessels –forces fluid to leak out to around surrounding organs. Prominent veins over abdomen. Enlarged Portal hypertension (cont) Varices, also known as collaterals. Thin walled and can bleed. Bleeding causing tiredness, breathlessness, pale appearance. Present in vomit or stools. Diarrhoea, poor weight gain –the blood vessels in the lining of the intestine swell as blood flows under pressure, reduces absorption of digested food. Pruritis/itching. Occurs with poor bile flow. Excessive amounts of bile acids. From mild , intermittent or severe. Palms, soles of feet, extremeties and trunk. Medications for treatment. Pruritis/itching (cont) Questran/ cholestyramine. Combines with bile acids in the small intestine and reduces reabsorption. Phenobarbitone –helps liver to excrete the substances thought to induce itching. Rifampicin –significant relief from itching by reducing harmful bile acids but increases the amount of protective acids. Sedatives –Vallergan. Ursodeoxycholic acid –increases watery bile salts to aid secretion. Choledochal cyst. – relating to bile Dochal – containing or receiving Cyst – fluid collection Chole Affects ducts outside liver. Bile collects inside dilated ducts – flow of bile impaired. Caused by ?malformation of bile duct inutero. Types of choledochal cyst. 1:50,000 3 x girls than boys These two types cause pancreatitis. Other types of choledochal cysts. Signs and symptoms Jaundice, Persistant or intermittent. Pale stools and dark urine Intermittent abdominal pain. Cholangitis, causing rigors. Peritonitis, if the cyst bursts or leaks. An abdominal swelling Roux loop. Complications Bleeding Infection Leakage where ducts join to the bowel. Cholangitis Pancreatitis. Cancer of bile ducts later in life –rare. Cystic fibrosis and liver disease. Liver disease more likely to develop with those babies who had meconium ileus as an infant. Damage starts in the small bile ducts, bile stickier than normal –blockage of ducts. Surrounding tissue then becomes damaged and scarred –biliary fibrosis. Liver becomes hard and the blood flow through more difficult. examination – hepatomegaly, splenomegaly. Portal hypertension Liver function tests Abdominal ultrasound Physical Treatments Medications Nutrition Endoscopy for varices Sclerotherapy of oesophageal varices Banding TIPS –radiological procedure which reduces blood flow through varices directing it through a channel created in the liver. Alagille Syndrome Biliary hypoplasia in association with at least two other anomalies in the body. 1:100,000, boys = to girls. Severity differs with each baby Biliary hypoplasia Ducts not formed properly so the bile flow is poor causing liver dysfunction and sometimes jaundice. Typical facial features include prominent forehead, deep set eyes and a small chin. Cardiovascular – pulmonary artery stenosis, from mild to severe symptoms. Posterior Embrytoxon –accumulation of a substance on inner aspect of cornea. Butterfly notches of the spine. Autoimmune liver diseases Caused by body’s defence mechanism, immune system malfunctioning and attacking part of itself. Two types: autoimmune hepatitis and autoimmune sclerosing cholangitis. Continuous cell destruction results in scarring of the liver leading to cirrhosis. Symptoms Tiredness, loss of appetite,spider-naevi, nausea and vomiting, weight loss, itching, fever, nose bleeds, jaundice, abdominal pain, ascites,lower limb oedema, irritability. Treatment with immunosuppresive drugs. Gilbert’s disease. A liver condition which is mild in nature. Intermittent jaundice, no abnormality of liver function. Males more than females. Abdominal discomfort, lethargy, malaise. May be triggered by infection, overexertion and fasting. Feeding baby’s with liver disease. Nutrition The liver plays an important role in good nutrition and growth. It produces bile which helps to absorb fat in the diet It converts the nutrients in the diet into energy and substances needed for growth and normal body functions. Babies drink large quantities of milk, as they do not digest nutrients they still feel hungry. Poor nutrition leads to poor growth, lack of energy and increased risk of infection. Need to drink special formula. Not normal formula which has long chained triglycerides and needs a good bile flow for absorption. Milks Medium chain triglycerides found in Pregestimil, Peptijunior and Neocate. These milks can be taken and fully absorbed as they do not need bile for absorption of the fat. Weaning between 4-6 months. Supplementary vitamins. Tube feeding This is not unusual. Pressure to get nutrition into a baby that will not feed. Need to get full requirements as mentioned before. Continuous overnight feeding, small bolus’ by day Gastrostomy –only if absolutely necessary. Thank you Time to go home. References Bassett M, Murray K (2008). Biliary atresia: recent progress. Journal of Clinical Gastroenterology. 42 (6) : 720 – 729 Cincinnati Childrens Hospital Medical Center (2009). Liver diseases and treatments. Biliary atresia. www.cincinnatichildrens.org/suc/alpha/1/liver Manning D, Todd P, Maxwell M et al (2007). Prospective surveillance study of severe hyperbilirubinaemia in the newborn in the UK and Ireland. Archives of Disease in Childhood. Fetal Neonatal Edition. 92 : F342 - F346 Schwarz S (2009). Biliary atresia: eMedicine Pediatrics. Emedicine.medscape.com Smitherman H, Stark A, Bhutani V (2006). Early recognition of neonatal hyperbilirubinaemia and its emergent management. Seminars in Fetal Neonatal Medicine. 11 : 214 - 224