1 / 35

Evaluation of Liver Disease in older children and adolescents

This presentation provides an overview of liver disease in older children and adolescents, with a focus on specific illnesses and diagnostic approaches. It also includes a case presentation and discusses the functions of the liver.

rjohnson
Download Presentation

Evaluation of Liver Disease in older children and adolescents

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript


  1. Evaluation of Liver Disease in older children and adolescents Dr Juliana Muiva-Gitobu KPA ASC, Mombasa, Kenya April 2019

  2. Outline • Case presentation • Liver functions • Overview of liver disease in infancy and older children • Specific illnesses reviewed • Conclusion

  3. Patient I.A. • 15 year with history of progressive and generalized body weakness and swelling of the lower limbs and abdomen over the last 1 year • O/E: FGC. Oedema+, mild pallor, abdominal distension with ascites and hepatomegally • What are his possible differential diagnoses? • What workup would you like to do?

  4. Liver Functions Revision • Uptake and processing of nutrients from the intestinal tract • Synthesis and biotransformation of proteins, carbohydrates and lipids • Excretion of bile and hydrophobic compounds • Regulation of energy metabolism • Endocrine functions • Imunological • Drug metabolism • Fluid balance

  5. Liver Disease in Infants • Anatomic : Biliary atresia, Choledochal cyst • Infectious :TORCHes • Metabolic : galactosemia • Autoimmune : neonatal sclerosing cholangitis • Vascular : CCF, constrictive pericarditis • Toxic/Drug induced : PNALD

  6. Spectrum of Liver Disease in older children • Anatomic • Infectious • Metabolic • Autoimmune • Vascular • Toxic/Drug induced

  7. Anatomic • Intra-Hepatic Bile Duct diseases (Ductal Plate Malformation): • Congenital Hepatic Fibrosis • Caroli’s Disease and Syndrome • Autosomal Dominant Polycystic Kidney Disease • Extra-Hepatic Bile Duct Diseases • Choledochal Cysts

  8. Infectious Metabolic • Hepatitis B • Hepatitis A • Wilson’s Disease • Hemochromatosis • Non Alcoholic Liver Disease

  9. Autoimmune Liver Disease Vascular disorders • Autoimmune Hepatitis • ASC • Haemangioma • Portal Hypertension

  10. Toxic & Drug Induced Liver Disease • Toxic e.g. Aflatoxin • Drug induced • Dose Dependent • Idiosyncratic

  11. Disorders of Ductal Plate Malformation

  12. Disorders of Ductal Plate Malformation • Can present with hepatomegally or can be incidental finding • Features of portal hypertension • Liver Functions may/may not be deranged • Radiology important: Ultrasound, MRI • Management guided by management of underlying disease and associated complications e.g renal disease • Surgical intervention espfor choledochal cyst, prevent biliary cirrhosis

  13. Hepatitis B • Persistent HBsAg positive >6/12 • >360 million persons infected worldwide • Majority of childhood infections in vertical or horizontal transmission • Risk of chronicity 90% newborn 20-30% <5 years <5% adults • Majority are asymptomatic. Some with malaise, anorexia, abdominal discomfort, then jaundice. 1% of acute infections may have severe presentation

  14. Stages of Hepatitis B infection

  15. Treatment Strategies Hepatitis B

  16. Hepatitis A • RNA virus, fecal-oral transmission • Majority of infections asymptomatic, up to 1.5 million cases annually • Clinically presents with anorexia, nausea, malaise, fever, abdominal pain, diarrhoea and vomiting • 1% with severe presentation, 0.2-0.4% mortality, especially with fulminant hepatic failure. Relapses possible

  17. Hepatitis A • Diagnosis: • Clinical features • ALT> x10 ULN, or 400 • ELISA anti-HAV IgM • Ultrasound and liver biopsy not routine

  18. Wilson’s disease • Copper: essential co-factor for several proteins; useful in neurological signalling and RBC formation. • Wilson’s disease: impaired excretion of Cu →Accumulation of Cu in hepatocytes, with eventual spillover to other systems • Defective gene ATP7B →defective protein →Decreased Cu excretion & defective caeruplasmin formation

  19. Clinical features

  20. Diagnosis of Wilson’s Disease

  21. Treatment of Wilson’s Disease • Reduce copper intake • Increase copper excretion: D-penicillamine, Trientine • Reduce copper absorption: Zinc acetate • Liver Transplantation

  22. Autoimmune Hepatitis Disease characterised by • Histologically:Dense mononuclear cell infiltrate in portal tracts • Serology: Hypergammaglobulinemia, Autoantibodies • Chemically: elevated transaminases 3 Types • autoimmune hepatitis • autoimmune sclerosing cholangitis  • de novo autoimmune hepatitis after liver transplantation

  23. Clinical Features Affects predominantly females Divided into 2 types based on antibody profiles • Type 1  • positive for ANA and/or anti-smooth muscle antibodies • Type 2  • positive for anti-Liver/Kidney microsomal antibody type 1 • median age of 10 years in type 1 and 7.4 years in type 2 • Can present with fulminant hepatic failure in 10%, Portal hypertension +/- GI bleeding, or can be asymptomatic • Type 2 associated with other AI disorders e.g. IDDM, Thyroiditis and IBD

  24. Diagnosis • LFTs: • raised AST and ALT • normal or mildly raised GGT and ALP • Variable bilirubin • Low Albumin • abnormal INR • Serology: • ANA, ASM, ALKM • titresof 1/20 for ANA/SMA and 1/10 for anti-LKM-1 are significant • Hypergammaglobulinemia, largely IgG

  25. Diagnosis • Histology: • dense mononuclear and plasma cell infiltration of the portal areas which expands into the liver lobule • Destruction of hepatocytes at the periphery of the lobule with erosion of the limiting plate (interface hepatitis) • Connective tissue collapse due to hepatocyte death expanding from portal area into lobule (bridging collapse) • Hepatic regeneration with 'rosette' formation • Cirrhosis • Imaging: US, MRCP (ASC)

  26. Treatment • Prednisone 2mg/kg/day (max 40mg) • Azathioprine 0.5mg/kg/day • Monitor Rx response on LFTs and TBC • Manage complications e.g. portal hypertension

  27. Portal Hypertension • Liver supplied by both the portal vein (75%) and the hepatic artery (25%) • Pressure between portal and hepatic venous systems <5mmHg • PH is defined as a portal pressure greater than 10 mmHg or a gradient greater than 4 mmHg.

  28. Portal Hypertension Aetiology can be classified as • Prehepatic • Intrahepatic (Presinusoidal, sinusoidal and postsinusoidal) • Posthepatic Peripherally other complications develop: • Peripheral and splanchnic vasodilation • Increased cardiac output • Shunting • Salt and water retention by kidneys, etc

  29. Portal Hypertension • Clinically most present with ascites, gastrointestinal hemorrhage, and splenomegally • Laboratory workup includes LFTs, blood cell and platelet count, and coagulation. Investigations targeted at underlying liver disease • Radiology • Portal vein doppler studies • Triple phase CT scan • MR angiography

  30. Portal Hypertension Management • Ascites: Diuretics, low salt diet • Variceal bleeding: endoscopy and VBL, Prophylaxis with beta blocker • Surgical procedures: TIPS, Rex shunt, etc

  31. Patient I.A. Results: 1. Thyroid profile – within normal limits 2. Alpha Fetoprotein – 2.4 iu/ml 3. Hepatitis A IgM, Hepatitis C antibody, Hepatitis B surface antigen-Negative 4. CMV IgM – Negative 5. Ferritin – 22.49 ng/mL 6. ANA - Negative 7. Anti ds DNA – Negative 8. Caeruloplasmin – 24.5 mg/dL 9. Anti-smooth muscle antibodies – Positive 10. Anti-liver kidney murosomal antibodies – Negative 11. CT Scan abdomen – Liver Cirrhosis with splenomegaly 12. Liver biopsy- not possible, elevated INR

  32. Patient I.A. Started on Prednisone nad Azathioprine with little improvement. Later results were as follows: • Urine copper excretion – 109.85ug/24hr • After Penicillamine challenge – 446.96ug/24hr I.A. started on penicillamine with significant improvement

  33. Conclusion • Liver disease in older children has a different aetiology • Many diseases may present without jaundice • Diagnosis is possible with a variety of tests, most of which can be done in the majority of facilities in this country.

More Related