Digestive system III PEDIATRIC LIVER DISEASES . Professor shahenaz M. Hussien. 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.
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Digestive system III PEDIATRIC LIVER DISEASES
Professor shahenaz M. Hussien
Investigations of liver diseases
Liver function tests
I. evidence of cellular injury: assessed by Serum transaminases:
II. Excretory function of the liver: can be assessed by:-
It is present in Obstructive and Hepatocellular jaundice
-Hepatocelluar jaundice. -Hemolytic anemia.
Imaging study of the liver:-
Definition:- It is an inflammatory process of the hapatocytes characterized by degeneration and regeneration with loss of hepatic architecture.
I- Infections, which may be:-
- Epstein-Barr virus (EBV). -Cytomegalovirus (CMV).
- Coxsakie, -ECHO, -rubella, -varicella and measles viruses.
- As a part of generalized septicemia.
- Isolated pyaemic liver abscess. - Leptospirosis.
Protozoal: e.gamoebic hepatitis.
II- Drugs and Toxins:
III- Immunological Disorders
-As apart of : S.L.E and J.R.A
- Isolated auto immune hepatitis
IV- Metabolic Causes:
V- Vascular Causes
- Hepatic vein thrombosis. - Hepatic artery thrombosis
- Primary: hepatoma or hepatoblastoma
- Secondary:- neuroblastoma, lymphoma, leukemia
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:
1- Prodromal stage: (1-2 weeks)(pre-Icteric)
2- Icteric phase (2-3 weeks): Characterized by:
3- Convalescence phase (1-2 weeks) - After which the child become nearly normal.
In endemic areas 30-80 % of children acquire subclinical or anicteric infection in the first few years of life. -Anorexia - Nausea – Vomiting -Fever - Abdominal discomfort
-Irregular bowel motions for a few days - Dark urine and mild scleral jaundice.
IgM Anti- HAV is detected at the onset of the symptoms and disappears within 4 weeks while IgG anti-HAV persists for life
Acute fulminant hepatitis.
Mode of transmission
-Infection appears to be due to contact with a mother's infected blood at the time of delivery. -Transplacental transmission is rare.
Incubation period:HBV has long incubation period (45-160 days).
Clinical manifestation: Asymptomatic carrier is more common.
Papular skin eruption - Arthralgia– Glomerulonephritis. -Aplastic anemia – Polyarthritis.
Liver function tests: The first evidence of infection is elevation of ALT. which begin to rise before the prodromal symptoms appears.
-HBsAg which indicate infection and HBeAg which indicate infectivity.
-HBcAb (IgM and IgG) is detected early in the disease and is important because it differentiates between the carrier and acute and chronic patient.
-Babies and young children. -Immunocompromised patients - Males > females.
Mode of transmission
-Cutaneous Vasculitis - Peripheral neuropathy - Cerebritis
-Membrano-prolifrative glomerulonephritis - Nephrotic syndrome
The effective therapy is under trial are:
-Monotherapy with Interferone α 2b
-Combination therapy with Interferone α 2b and Ribavirine results in higher frequency of sustained response and in histologic improvement.
Hygienic care in :-
What is Cirrhosis?
Several liver diseases can cause cirrhosis, including Hepatitis B and C, nonalcoholic fatty liver disease, biliaryatresia, Alpha-1 antitrypsin deficiency, primary sclerosingcholangitis, Wilson's disease, autoimmune hepatitis, and bile duct diseases.
Autoimmune hepatitis is one of the major etiologies of chronic hepatitis in children. Chronic hepatitis may present with numerous hepatic clinical manifestations, associated with extrahepatic disorders.
The etiology is unknown and the disease is characterized by the auto-antibody pattern and the positive response to immunosuppressive treatment.
-Cirrhosis may occur early, irrespective of the cause.
-A combination of laparoscopy and biopsy is more reliable than biopsy alone for the diagnosis of cirrhosis in children with chronic hepatitis.
-Changes in hepatic architecture in cirrhosis and chronic active hepatitis affect liver vascular haemodynamics. Portal vein velocity, arterio-portal vein ratio and hepatic artery visualisation together were reliable in diagnosis of cirrhosis in the paediatric age group.
-Blood tests, CT scans, liver biopsies, and MRI and ultrasonography studies help confirm the cause of the cirrhosis, and the extent of liver damage.
Two problems happen in patients with cirrhosis -- liver failure and portal hypertension.
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.
Even though cirrhosis can cause significant liver damage, the disease usually progresses slowly in children and most symptoms can be controlled.