Disorders of liver

DISORDERS OF LIVER IN CHILDREN

- Presented by,DHARA JOSHI,S.Y. M.Sc. NursingBatch:2011-2012

Anatomy and physiology of liver:

Liver is one of the vital organ of the body. In infants its weight is about one eighteenth of the birth weight (120-160gms). It usually remains palpable upto 1.5cm below the right costal margin on the midline clavicular line throught out infancy and childhood. The liver consists of lobules and cells of each are arranged in lobule radiator columns around a central vein which drains through the hepatic vein into the inferior vena cava.

Vital functions of liver

are: Metabolic functions Haemopoietic function Excretory function Detoxicating function

Major functions of the liver Regulates blood glucose, stores energy Regulates blood clotting Produces & eliminates bile Neutralises and destroys toxins, drugs Detoxifies/eliminates – hormones, endotoxins etc Resists infection Regenerates its own damaged tissue

Stores glucose, iron, vitamins, minerals, blood

Manifestations of liver diseases:

Symptoms: Jaundice with dark colored urine and pale stool. Abdominal distension; due to hepatomegaly. Bleeding tendency e.g. hematemesis, melena and epistaxis. Purities. Failure to thrive and malaise. Abdominal pain, anorexia, fever and vomiting in cases of

hepatitis. Metabolic consequence of liver diseases e.g. encephalopathy. Signs: Enlarged or shrunken liver. Signs of portal hypertension; splenomegaly, dilated abdominal

wall veins. -Ascites. -Clubbing especially in chronic liver disease.

HEPATOMEGALY Definition:- It is an

inflammatory process of the hapatocytes characterized by degeneration and regeneration with loss of hepatic architecture.  

Types:- Acute: less than six months

duration Chronic: more than six

months

Etiology: I- Infections, which may be:- Viral:

Hepatotropic viruses e.g A, B, C, D, E, F, G, H viruses

Non -hepatotropic: infect the liver in the course of other systemic illness: Cytomegalovirus (CMV), -rubella, -varicella and measles viruses.

Bacterial : As a part of generalized septicemia. liver abscess. Leptospirosis. Protozoal: e.g amoebic hepatitis.

II- Drugs and Toxins: Anti T.B. e.g Isoniozid - antimetabolites -

anticonvulsant:- valporic acid Irradiation - chloropromazin - total parenteral

nutrition III- Immunological Disorders As apart of : Systemic Lupus Erythmatous and

Juvenile Rheumatic Arthritis IV- Metabolic Causes: Galactosemia Hepatic vein thrombosis, Hepatic artery thrombosis VI- Tumors:

Primary: hepatoma or hepatoblastoma Secondary:- neuroblastoma, lymphoma,

leukemia

HEPATITIS "Hepatitis" means inflammation or swelling

of the liver. Lots of things can cause swelling of the liver such as drinking alcohol, use of certain prescription medications and viruses. Swelling can occur one time or be chronic, depending on the source.

HEPATITIS -A Mode of transmission: fecal-oral route Incubation period: 15-45 days (average 4 week). The

virus is excreted in stool during the first few weeks of infection, prior to the onset of symptoms.

Clinical manifestations: Acute Viral Hepatitis: Prodromal stage: (1-2 weeks)(pre-Icteric) Headache, anorexia, malaise. Nausea, vomiting, lethargy. This phase can be passed un-

noticed by the parents. Icteric phase (2-3 weeks): Characterized by: jaundice - dark urine. Abdominal pain – soft enlarged, and tender liver. Convalescence phase (1-2 weeks) - After which the child

become nearly normal.

Laboratory diagnosis Liver function tests shows:-

Raised serum level of direct and indirect bilirubin Urine contains both bilirubin ( dark color) and Urobilinogen.

Marked elevation of serum transaminases (ALT and AST).

Increase in serum levels of alkaline phosphatase

IgM Anti- HAV is detected at the onset of the symptoms and disappears within 4 weeks while IgG anti-HAV persists for life

Complication Acute fulminant hepatitis.

It is a rare condition with massive destruction of the liver cells.

It is presented clinically by persistent vomiting, disorientation, encephalopathy, bleeding tendency, edema and ascites.

Aplastic anemia Is a very rare complication it is transient but may be fatal. It is due to bone marrow depression.Death is usually due to

serious infection due to depressed immunity Treatment There is no specific therapy for acute viral hepatitis, Most children are managed at home except if liver cell failure

is suspected. Balanced diet with low fat intake should be given.

PREVENTION Period of infectivity: Contagious for about 7 days

before and 7 days after the onset of jaundice. Period of isolation from school: From the

appearance of dark color urine and appearance of jaundice till 7 days after disappearance of jaundice.

Careful hand washing after changing diapers and before preparation of food. Fly control.

Intramuscular immunoglobulin may be indicated in pre and post exposure.

Hepatitis A vaccine is now available to be given to children. Contacts are immunized with immunoglobulin or the vaccine.

HEPATITIS –B Mode of transmission Perinatal transmission (vertical transmission). Infection appears to be due to contact with a mother's

infected blood at the time of delivery. Transplacental transmission is rare.

Parenteral : In patient receiving blood transfusion or blood products, renal dialysis, dental care, and through contaminated syringe and needles.

Child to child transmission : It may occur through biting of insects, drooling and shared chewing gums.

Although HBV was detected in breast milk of infected mother there is no role of breast milk in transmission the infection.

Incubation period: HBV has long incubation period (45-160 days).

Clinical manifestation: Asymptomatic carrier is more common.

Acute infection presented with: Jaundice, dark color urine, anorexia, nausea,

malaise. Hepatomegaly splenomegaly.Exctrahepatic manifestations as:

Papular skin eruption - Arthralgia– Glomerulonephritis. -Aplastic anemia – Polyarthritis.

Laboratory diagnosis

• Liver function tests

• HBsAg

Complications

Treatment

• Supportive treatment. • Liver transplantation

Prevention

• Hepatitis B vaccine

HEPATITIS –C Mode of transmission Post-transfusion: with repeated transfusion

of blood and blood products. Intravenous drug, needle prick exposure,

hemodialysis, organ transplant. Perinatal transmission is uncommon &

transplacental transmission not proved until now.

Incubation period The incubation period is 7-9 weeks.

Clinical picture: The clinical pattern of the acute infection is

usually similar to that of other hepatitis. Acute HCV infection is usually mild and

may be asymptomatic but fulminant liver failure may occur leading to poor prognosis.

Chronic HCV infection may be associated with extra hepatic manifestation include Cutaneous Vasculitis, Peripheral neuropathy ,Cerebritis Membrano- prolifrative glomerulonephritis, Nephrotic syndrome

Indian Childhood cirrhosis: Indian Childhood cirrhosis is a particular to

the Indian infants and children usually 1 to 4 years of age. It is characterised by progressive dissention of abdomen, marked irritability, unexplained irregular low grade pyrexia, a firm hapatosplenomegaly and progressive liver cell failure, ascites and jaundice.

Incidence of disease: More common in middle class families It is more common in Hindus of all casts in

all over India but rare in Muslims. Male suffers more than females, the first

born is at greater risk. Majority of the patients belong to

vegetarian diet families Siblings and twins are frequently affected.

It is defined as fibrosis (scarring) plus nodule formation (regeneration). Thus cirrhosis is a pattern, not a disease or a specific phenomenon.

Cirrhosis can happen after any disease that causes liver cell death. Exactly why the regenerating nodules are not able to replace the function of the liver is not clear, although it may be the scarring itself that prevents recovery.

Other organs like the kidneys, heart, and lungs are not able to regenerate. They can hypertrophy, (cells get larger) but they do not have cellular division that results in significant "regrowth" (more cells) like the liver does. This regrowth is what happens when a living donor gives part of their liver to be transplanted.

Chronic Liver Disease 2008

Liver Disease – causes in childrenMost causes are uncommon:

Chronic Biliary atresia Alagille’s syndrome A-1-antitrypsin deficiency Wilson’s Disease Autoimmune Hepatitis others…

Acute Medications Some Homeopathic remedies Poisons Toxins unknown - ?viral

PATHOGENESIS•Cell undergoes death due to any of etiological causes (Necrosis). Healing the necrotic place by fibrosis•Deficiency of blood supply to some areas

•Hypoxic necrosis due to decreased blood supply•Healing of necrotic area

•Some area retain the blood supply by anastomosis •Tries to replace the lost cell by regeneration

•Regenerated nodule , Compress•Decreased blood supply

•Continuous formation of Fibrosis and regenerated nodules •Obstruction in biliary flow•Portal hypertension

Clinical manifestations: Two problems happen in patients with cirrhosis --

liver failure and portal hypertension. The liver failure causes fatigue, jaundice (yellow

skin), and encephalopathy (a state of confusion that progresses to coma).

The portal hypertension causes bleeding and ascites. These things happen in many forms of liver disease, not just in cirrhosis.

The scarring never goes away, but the liver has "reserve," meaning it can do all the body requires even though it is not working at 100 percent.

Early GI disturbances, dull

pain in RUQ/epigastrium, fever, malaise, enlargement of liver & spleen

Late Jaundice, skin lesions

(spider angiomas, palmar erythema), hematologic problems, endocrine disturbances, peripheral neuropathy

connection.lww.com/ Products/morton/Ch41.asp

Management Rest Ascites – Sodium/fluid restriction,diuretics, Paracentesis Peritoneovenous shunt – continuous

reinfusion of ascitic fluid into the venous system

Nutrition – hi cal, hi CHO, mod/lo fat, low protein if symptomatic

Management- Drugs Hemostasis – vasopressin ↓ portal venous pressure – Propranolol Acidify stool, trap ammonia – Lactulose ↓bacterial flora – Neomycin sulfate ↓gastric acidity – Cimetidine Diuretics – Spironolactone, lasix, etc, Magnesium replacement – MgSO4 Correct clotting abnormalities – Vitamin K

Complications – Portal HypertensionCompression

Portal vein,hepatic veins, sinusoids

Obstruction of portal blood flow

Portal Hypertension

Venous pressure, splenomegaly, collateral circulation, ascites, systemic hypertension,

esophageal varices

Complications – Esophageal Varices

To reduce portal hypertension Collateral circulation develops in lower

esophagus, anterior abdominal wall, parietal peritoneum, & rectum

Varicosities develop where collateral & systemic circulations communicate esophageal & gastric varices, caput medusae, & hemorrhoids

Complications – Peripheral Edema

↓ albumin synthesis

↓ colloidal oncotic pressure+

↑ portocaval pressure

Peripheral edema (ankle & presacral)

Complications - Ascites

Accumulation of serous fluid in peritoneumProtein from sinusoids lymph space leak

water and protein into the peritoneal cavityHypoalbuminemia ↓ colloidal oncotic

pressureHyperaldosteronism ↑ Na reabsorption

Abdominal distention,striae, weight gain Dehydration Hypokalemia

Complications – Hepatic Encephalopathy Bacterial & enzymatic deamination of amino

acids in the intestines ammonia without liver detoxification crosses blood-brain barrier toxic neuro symptoms

Euphoria, irritability, confusion, slurred speech, slow & deep respiration, hyperactive reflexes, positive Babinski’s reflex

Asterixis, fetor hepaticus, deep coma

Complications – Hepatorenal Syndrome

Portal hypertension splanchnic & systemic vasodilation ↓ arterial blood volume renal vasoconstriction renal failure

Chronic Liver Disease 2008

Psychosocial: parents

Grief Guilt Anger Helpless Anxiety & uncertainty re future Concerns re managing illness Lack of information

Loss of control Disruption of life style Social isolation Social stigma Financial hardship ↑Risk of marriage disintegration Worry about impact on siblings

Knafl Kathleen & Santacroce Sheila (2004) Chronic Conditions and the Family In: (Eds.) Allen, Patricia Jackson & Verrsy Judith A. PRIMARY CARE OF THE CHILD with a CHRONIC CONDITION (4th Ed.) Mosby St. Louis

Chronic Liver Disease 2008

Psychosocial: management - parents Partnership with parents Communication - open Education - verbal & printed Information Ready access to team Counselling Social worker – financial & other resources Introduction to other parents Parents support group

Chronic Liver Disease 2008

Psychosocial: infants & children Sadness Frustration Anger Loss of control Separation (hospital) Fear of pain Concerns re body image Social stigmatization Disruption to life style

Social isolation Developmental delay ↓Mastery ↓Confidence ↓Self Esteem ↑Dependence ↑Risk of emotional & behavioural problems

Angström-Brännström Charlotte, Norberg Astrid & Jansson Lillian. August 2008. Narratives of Children with Chronic Illness About Being Comforted. Journl of Pediatric Nursing 23, (4) 310-316

Knafl Kathleen & Santacroce Sheila (2004) Chronic Conditions and the Family In: (Eds.) Allen Patricia Jackson & Vessey Judith A. PRIMARY CARE OF THE CHILD with a CHRONIC CONDITION. 4th Ed. Mosby St. Louis

Chronic Liver Disease 2008

Psychosocial: management - infants & children

Maximal parental participation in care Facilitation of communication Facilitation of choices Facilitation of routine Play Therapist - medical play &

diversional techniques Pain management team Counselling Music therapy Liaison with school

Pediatric Liver Transplant A transplant is one of the best treatments

for fatal liver disease in children. New drugs and new surgery techniques have improved the 1-year patient survival rate. There are two main concerns about liver transplant for children. They are the cause of the liver failure in the first place, and if there are enough livers for children who need transplants.

What are the donor organ options for children?

Reduced-size liver graft. A piece of a liver is taken from a brain-dead person (deceased donor).

Split-liver transplant. A liver from a deceased donor is cut in two. A small piece is used for transplant in a child and a larger piece is used for transplant in an adult.

Living-donor transplant. A piece of a liver comes from an adult living donor and is used as a transplant for a child. Over the past 10 years, nearly 1000 of these transplants have been done in the world. They have been just as successful as transplants of whole organs. There are some risks of adult donation for transplant to a child. These risks seem to be much smaller than the risks of a living donor transplant to an adult.

Is it difficult to do a transplant on a child? Yes, for several reasons. First, the blood vessels in

children are very small. Transplants on children under one year of age may be even harder to do. And, the care after surgery must be done in ICUs that can handle small children.

Do children get the same anti-rejection drugs as adults?

In general, children get the same type of drugs that adults get, but children may have different side effects. For this reason, only doctors who specialize in this field should do the follow-up care.

What makes a transplant successful?

Three thing help make a transplant successful.

1. Good medical care2. Doing exactly what the

doctors tell you to do, 3.Good communication

between the patient, family, and medical teams.

Children who get liver transplants will need to take drugs as prescribed and see their doctors often. They may need more tests and evaluation of their growth and development than children who have not had a transplant. Children can have long, healthy lives after transplant. As a parent, make sure you stay in touch with your child’s transplant team, and follow their advice. Ask for counseling when you have any problems.