Indian childhood cirrhosis

PEDIATRIC NURSING

1. INDIAN CHILDHOOD CIRRHOSIS Presented by Ms Arifa T N, Second year M.Sc Nursing, MIMS CON

2. INTRODUCTION Indian child hood cirrhosis (ICC) of liver is a very serious condition affecting children in Indian subcontinent. The word Cirrhosis derived from the Greek word ‘ scirrbus ’ , in which the surface of the liver appears orange or tawny at autopsy It is one of the non- Wilsonian copper overload hepatic disorder. The first case in India was reported in the year 1880 in Kolkata.

3. Over 90% cirrhosis in pediatric age group is accounted by what are now called the Indian childhood cirrhosis and Jamaican cirrhosis or veno -occlusive disease (VOD) of liver.

4. Definition ICC is an autoimmune disorder characterized by fever, abdominal distension and hepatosplenomegaly . It is a progressive disease with abdominal distension, marked irritability, unexplained irregular low-grade fever, hepatosplenomegaly with hepatic failure, ascites and jaundice.

5. Epidemiology and incidence Prevalent all over India in some particular communities . South India—Brahmins Central India— Banias North India— Banias and Jats North Indian Hills— Rajputs Male children are 4 times more sufferer than female (4:1). First born children are at risk. Between the ages of 6months and 4 years .

6. Family predisposition for ICC, siblings and twins are affected Low middle class vegetarian family of rural area Predisposition with disorders like peptic ulcer, asthma, and migraine. Now, decrease in incidence of ICC. Use of copper and brass vessels for cooking

7. Etiology The exact etiology is not known Predisposing factors Toxic (copper intoxication) Presence of copper binding protein ( orcein ) in the liver Supplementary milk feed before 3 months Use of copper and copper alloy vessels for cooking Viral infections Infective hepatitis during neonatal period Immunological factors high levels of circulating immunocomplexes insult hepatocytes causes immune mediated injury to liver

8. Nutritional factors Malnutrition during young age Hepatotoxic agents Aflatoxin ( Aspergillus flavus ) that grows on groundnuts, maize, and rice. Family history Hall mark of ICC An increased prevalence of peptic ulcer, asthma, diabetes and migraine Metabolic factors Inborn error of tryptophan metabolism, aminoaciduria, aminoacidaemia , disturbed lactose, zinc, copper and magnesium metabolism Super added factors for genetically prone children Viral, toxic, metabolic and autoimmune

9. Pathophysiology

10. Clinical features Insidious or acute onset

11. Insidious onset Precirrhotic symptoms Irritability Disturbed appetite Chalky, pasty stools and distension of abdomen Constipation or diarrhea Often sight irregular fever

12. Cirrhotic symptoms Stage I Slight fever Liver is enlarged up to 3-5 cm, edges become sharp, giving a leafy boarder appearance Children exhibit jaundice Poor growth Anorexia Constipation or diarrhea Clay colored stools Growth failure

13. Stage II Diffuse hepatomegaly Splenomegaly Ascites Oesophageal varices Haemoptysis Anaemia Muscle weakness Lethargy GI bleeding

14. Stage III It is the terminal stage of the disease Restlessness Confusion Dyspnea and cyanosis on exertion Evidence of hepatocellular failure in the form of palmar erythema ans spider nevus appearance on the upper torso. A peculiar garlic odor is present in patients with impending liver cell failure Enlarged and hard spleen Terminally, there is jaundice and hepatic coma and is often associated with gastrointestinal bleeding The child may die at this stage either from hepatic failure or intercurrent infections.

15. Acute onset Sudden onset of disease Sometimes asymtomatic for a variable period and then shows the manifestations of insidious onset. Sudden onset of fever Jaundice Clay colored stools Hepatospenomegaly Death due to hepatic coma

16. Diagnostic evaluation Perform history collection Complete physical examination Liver can be palpable Very firm in consistency and its boarders will be sharp Hepatic bruit on auscultation In case of ascites, fluid thrill test can be done Liver function tests Increased ALT ( Alanine Transaminase ) Increased GGT ( γ - Glutamyl Transpeptidase ) Prothrombin time (PT) ( prolonded ), clotting time and bleeding time should be assessed Liver biopsy Sclerosis of liver Cupriuresis : It involves testing the presence of copper in urine after administration of D- pencillamine

17. Multidisciplinary management If diagnosed in early stage it can be treated Initial stage Diet : Good quality proteins, minerals and vitamines Antibiotics : infection/infestations D- pencillamine 20-40 mg/kg per day for 12-18 months Symptomatic treatment Immunomodulators : levamisole Corticosteroids and γ -globulins IV fluids : dehydration Strict asepsis : prevent infection

18. Terminal stage If the patient has entered the precoma or coma stage Protein intake should be reduced Administration of neomycin by gavage and 20% IV glucose drip are helpful Oxygen administration Exchange transfusion

19. Surgical management No specific surgical correction Liver transplantation (ESLD) Sengstaken tube (portal hypertension and hematemesis ) Portocaval anstomosis (portal hypertension and hyperspenism )

20. Nursing management Nursing diagnosis Hyperthermia related to the inflammatory process in the liver Ineffective breathing pattern related to pressure on diaphragm secondary to ascites Imbalanced nutrition less than body requirement related to anorexia Diarrhoea or constipation related to acute abdominal condition Parental anxiety related to management of the disease condition.

21. Nursing interventions Provide symptomatic management Adequate rest Semi-fowlers position Daily abdominal girth measurement IV fluid as per order Small and frequent diet Protein rich food and massive doses of vitamin B 6 Aseptic precautions I&O chart Parental education Disease condition and management Avoid food rich in copper eg : drycnuts , chocolates, liver etc. Breast feeding and weaning at 6 months Milk should not boiled and stored in copper or copper alloy pots Reduce use of brass and copper vessels Use aluminum and steel utensil

22. Food rich in tryptophan should be reduced Eg: milk, eggs, meat, nuts, beans, fish and cheese Provide more vitamin B 6 foods such as potato, banana, spinach, soya bean, fruits, and vegetables (vitamin B 6 helps to convert tryptophan to niacin.

23. Thank you