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INDIAN CHILDHOOD CIRRHOSIS

Belbimol E 2nd Year MSc Nursing

Indian Childhood Cirrhosis

Peculiar to the Indian infants and children Usually 6 months to 4 years of age First described by Sen in 1887 from Calcutta. Occasionally in some other countries Designation was suggested by prof. D B Jelliffe in early 1950s. Synonyms: infantile cirrhosis, hypertrophic biliary cirrhosis, sub-acute toxic cirrhosis

EPIDEMIOLOGY
prevalent all over India communities predominantly affected
South India Brahmins Central India- Banias North India- Banias and Jats North Indian hills- Rajputs

Continued Males > females Vegetarian dietetic background Family predisposition ,siblings and twins are equally affected. An increased prevalence of peptic ulcer, asthma, diabetes and migraine in the pedigrees incidence in 1980 -1:4100 to 1:9400 Over the last more than two decades, a steady decline -appears to be related to declining practice of employing copper/brass utensils for boiling milk.

ETIOLOGY
Unknown hypotheses Nutritional viral Hepatotoxic Genetic Autoimmune Metabolic

PATHOLOGY
The diffuse liver cell damage by way of degeneration going on to necrosis and replacement fibrosis. In established case, there is combination of;

Complete disorganization of liver architecture=>macronodules and micronodules. Absence of regenerative activity indicated by regenerating nodules which are encircled by bands of fibrous tissue. Gross pericircular (creeping) fibrosis in the hepatic lobules. Predominant neutrophilic exudate in the lobules.)

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Intracellular hyaline that is identical to the so-called mallory hyaline seen in the alcoholic cirrhosis of adults; no primary disease of intrahepatic biliary system. Fatty change is either absent /minimal. Gross excess of copper and/ or copper associated protein (orcein). Degenerative changes in hepatocytes, scattered fibrosis, electronulecent cytoplasmic areas with loose arrangements organelles, distinct outline of mitochondria, fine fibrillar mallory hyaline and irregular dense bodies (cuprosomes)

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CLINICAL FEATURES

ACUTE

INSIDIOUS

INSIDIOUS

3 stages

Vague manifestations Liver is enlarged by 3-5 cm and has a firm feel with sharp leafy border. Abdominal distension Anorexia or voracious appetite Lassitude Irritability Slight fever Constipation or diarrhea with clay colored, sticky, formed stools Growth failure

First stage

Second stage
Characterized by further exaggeration of the manifestations of the first stage. Liver size increases and is more firm Definite jaundice is evident Domination of manifestations of portal hypertension

Splenomegaly Ascites Hematemesis Anemia Prominent superficial abdominal veins Thrombocytopenia Intravascular hemolysis

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Apathetic Emaciated Having deep jaundice Protuberant abdomen Prominent superficial veins Ascites Liver may be grossly enlarged or shrunk Splenomegaly

Third stage

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Hepatocellular failure Restlessness and confusion Hepatic coma Flapping tremors Dies at this stage either from hepatic failure per se or intercurrent infections.

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Duration of illness :varies between 6 months to 3 years Significant retardation of growth due to alteration in the ratio of collagen synthesis in skeletal tissue as indicated by low hydroxyproline index

ACUTE

Sudden onset Jaundice Clay-colored stools Hepatomegaly Rapid downhill course, the child may finally dying in hepatic coma: called fulminant or malignant hepatitis

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DIAGNOSIS
Liver biopsy (CI:ICC with prolonged PT) Cupriuresis test:

TREATMENT
In initial stage, Adequate diet Treat intercurrent infections / infestations Corticosteroids, gammaglobulins, d-pencillamine (20-40 mg/kg/day for 12 to 18 months)or levamisole

In terminal stage Reduced protein intake Neomycin 20% IV glucose drip Oxygen Exchange transfusion Sengstaken tube

PREVENTION
Encourage the use of aluminium and steel Promotion of breast feeding Demineralize water if copper content of drinking water over 0.1mg/L Avoid/minimize consumption of foods rich in copper content(liver, nuts, chocolates)

N.C. Nayak & A.R. Chitale published in Indian Journal of Medical Research( 137, June 2013, pp 1029-1042) More recently available findings from a large multicentre study sponsored by the Indian Council of Medical Research (ICMR) A primary role of exogenous copper in causing the disease was earlier debated on the basis of studies in India but investigators abroad studying some sporadic cases and a series of endemic ICC-like diseases supported a hepatotoxic injury by ingested copper in genetically susceptible infants and children in ICC- like disease and in ICC. Epidemiologic and morphologic findings in the well-controlled ICMR study based on 225 cases of ICC and 426 controls, all confirmed on liver biopsy, have however, convincingly refuted this concept. Additionally, this study revealed that unlike what has been believed earlier, older children more than 3 yr age can get the disease and that in its natural course the hepatic histology can transform between the characteristic one considered diagnostic and some other patterns, any one of which can be the morphologic manifestation at first presentation of the patient. Older children and cases with milder morphologic changes at presentation had longer survival. The overall inference from critical analysis of all available data is that ICC and ICC-like diseases clinically manifest in a child of any age though common in younger ones, and a clinical diagnosis must be made in any child with socalled cryptogenic cirrhosis. Exposure to exogenous copper in food, milk and water should not be a prerequisite for this consideration. A liver biopsy whenever feasible should be mandatory for confirmation with the understanding that the morphologic changes in liver can present a few other patterns besides the characteristic one currently taken to be diagnostic. The ascribed current decline in encountering ICC is likely to be due partly to missing a diagnosis and partly to a true reduction in incidence consequent on time related economic and socio-cultural changes.

Related research

NURSING MANAGEMENT

Nutrition: imbalanced, less than body requirements Fluid volume excess Activity intolerance [specify level]/fatigue Anxiety/fear; ineffective coping Interrupted family processes/impaired parenting Risk for delayed growth and development Risk for ineffective health maintenance