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PEDIATRIC NURSING SEMINAR

SUBMITTED TO,
MRS RAJALEKSMI. K
ASSO PROFESSOR
GOVT COLLEGE OF NURSING, ALAPPUZHA
SUBMITTED BY,
MRS. DIVYA RAJ
II YR MSC NURSING
GOVT COLLEGE OF NURSING, ALAPPUZHA

OBJECTIVES

Central objectives
At the end of the seminar the group will gain adequate knowledge regarding Indian childhood
cirrhosis, Wilson's disease and Reye syndrome.

Specific objectives
On completion of the seminar the group will be able to,

Define the disease


Explain the etiological factors of the disease
Describe the pathophysiology of the disease
Discuss the diagnostic tests for the disease
Explain the management of the disease
Illustrate the nursing diagnosis and nursing interventions for the disease.

INDIAN CHILDHOOD CIRRHOSIS


INTRODUCTION
Indian childhood cirrhosis (ICC), a disease considered to have been endemic in and unique to India
has now been documented in children of non-Indian origin from other countries. The onset is insidious
with nonspecific complaints such as abdominal distention, irregular fever, excessive crying and altered
appetite. The disease is typical of young children (12 to 36 months of age) with hepatomegaly and even
greater splenomegaly and death within 3 to 33 months. A common killer disease in the past, Indian
Childhood Cirrhosis (ICC) which became preventable and treatable in the early 1990s is now rare.
DEFINITION
Indian childhood cirrhosis is an auto immune disorder characterized by fever, abdominal distension
and hepato-spleenomegaly and most often seen in Indian children between the age of 6 month to 4 years.
EPIDEMEOLOGY

Indian childhood cirrhosis is more common in the age group of 6 month to 4 years.
It is more affected in male child than female child (1 : 4), the first born is at greater risk.
Among twins, the member of the pair on mixed or artificial feeding in predisposed families was
reported to have developed cirrhosis of liver. If the other twin was purely breast-fed for first six
months, he or she escaped the illness.
A definite family predisposition is the hallmark of ICC. Siblings and twins are affected. An
increased prevalence of peptic ulcer, asthma, diabetes and migrane in the pedigrees affected by ICC
has been observed. .
A large majority of cases belongs to middle class families.
Majority of the patients have vegetarian dietetic background.

Recently, a significant decline in the incidence of Indian Childhood Cirrhosis has been observed in all parts
of India since the use of copper and brass utensils for boiling milk is reduced.
ETIOPATHOGENESIS
ICC continues to be a disease of obscure etiology. The following factors may predispose to the illness:

Toxic (copper intoxication): Studies reported that there is an evidence of excess of copper binding
proteins (orcein) in the liver of patient with ICC.
if the baby is weaned earlier and if the milk supplements were added to the breast milk
often before 3 months of age.
use of copper or copper alloy pots for boiling milk and cooking food. During boiling of
milk, copper is released from the copper pots and this is probably absorbed in excess from
the gut by infant.
Viral infection of the liver: It is a consequence of neonatal or infective hepatitis.

Immunologic factors: A variety of immunological disturbances were reported in patients with


Indian Childhood Cirrhosis. High levels of circulating immune complexes may indicate that an
environmental insult might alter the hepatocyte or tissue proteins and initiate an immune mediated
injury to the liver.
Nutritional factors: - it is believed that malnutrition is an important cause of cirrhosis.
The objections to this hypothesis are:
Cirrhosis is rare in children suffering from kwashiorkor.
Cirrhosis is rare in Africa, the home of kwashiorkor.
Cirrhosis is uncommon in poor in whom malnutrition is common. On the contrary, the
incidence of ICC is high in the middle class families.
Fatty changes of liver in kwashiorkor is either absent or very insignificant in ICC.
So, nutrition does not have clear role in its pathogenesis. All children are equally affected. Good
nutrition does not protect the child from the disease.
Hepatotoxic agents: Some hepatotoxic agents like Aflatoxin, produced by Aspergillus flavus that
grows on ground nuts, maize, and rice can predispose to this disease. But the actual cause effect
relationship is not established.
Familial history of the disease: A definite family predisposition is the hallmark of ICC. An
increased prevalence of peptic ulcer, asthma, diabetes and migrane in the pedigrees affected by ICC
has been observed.
Metabolic factors: A child with inborn error of tryptophan metabolism, aminoaciduria,
aminoacidemia, disturbed lactose, zinc, copper and magnesium metabolism can predispose to the
disease. So a child at risk should be put on low tryptophan diet.

To conclude, no single factor seems to be the cause of ICC. It is possible that a genetically prone child
suffers from one or more of the superadded factors (viral, toxic, metabolic and autoimmune) leading to the
overt picture of ICC.
PATHOPHYSIOLOGY
ICC manifests with jaundice, pruritus, lethargy, and hepatosplenomegaly. Histologically, it is
characterized by hepatocyte necrosis, Mallory bodies, intralobular fibrosis, and inflammation. There is an
increased hepatic copper content, usually >700microgram per gram dry weight.

CLINICAL FEATURES
The onset is generally vague and ill defined, ranging from no symptoms to an icteric onset. Some
infants show pre cirrhotic symptoms.
Two modes of presentation are known: a) Insidious which occurs in a large majority of the cases
and b) Acute which is less common.
Insidious onset: In this, the disease will last for 6 months to 3years. Symptoms are grouped under 2
headings.

Pre-cirrhotic symptoms
Cirrhotic symptoms
Irritability
Cirrhotic symptoms are grouped under 3 stages:
Stage I
Disturbed appetite
Chalky, pasty stools and
Slight fever
Liver is enlarged to 3-5cm, edges become sharp
distension of abdomen.
Constipation or diarrhea
and giving an appearance of leafy boarder.
Often slight irregular
Children exhibits jaundice
Poor growth
fever.
Anorexia
Constipation/ diarrhea.
Clay colored stools.
Growth failure
Stage II
Diffuse hepatomegaly
Splenomegaly
Ascites
Esophageal varices
Hematemesis
Anemia
Muscle weakness
Lethargy
GI bleeding.
Stage III: It is the terminal stage of the disease
Restlessness
Confusion
Dyspnea and cyanosis on exertion.
Evidences of hepatocellular failure in the form of
palmar erythema and spider nevi 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 gastro intestinal
bleeding. Child may die at this stage either from
hepatic failure or intercurrent infections.
DIAGNOSTIC EVALUATION
1. History collection
2. Complete physical examination:
Liver can be palpable, very firm in consistency and its boarders will be sharp.

3.

4.
5.
6.

On auscultation hepatic bruit in severe cases. If there is ascites, fluid thrill test can be done.
Liver function test:
increased ALT(alanine transaminase, an enzyme present in hepatocytes.)
increased GGT( gama glutamyl transpeptidase)
Prothrombin time, clotting time and bleeding time should be assessed. PT will be prolonged
Liver biopsy: to find out the sclerosis of liver. It is a reliable method of arriving at a foolproof of
diagnosis.
Cupriuresis: testing the presence of copper in urine after administration of d-penicillamine.

TREATMENT
Until recently, ICC was dubbed as a frustrating situation for which no specific treatment was available. If
the diagnosis is made at an early stage (before the development of jaundice and ascites), ICC is potentially
treated.
1. Initial stage:
Adequate diet with enough of good quality proteins, vitamins and minerals is desirable.
Antibiotics should be given to treat the intercurrent infections / infestations.
The drug of choice is d- pencillamine (which chelate copper) in a dose of 20- 40 mg/kg/day
for 12 to 18 months, leads to marked improvement and even total reversal in the
histopathologic picture.
Symptomatic treatment should be given.
Immuno modulators such as levamisole can be used
Corticosteroids and gammaglobulins are also helpful.
administer IV fluids if there is dehydration
Prevention of infection: follow aseptic techniques
Prophylactic antibiotics can be given to prevent infection
2. Terminal stage:
If the patient has entered precoma or coma, the protein intake should be reduced.
administration of neomycin by gavage and 20% IV glucose drip are helpful.
oxygen can be administered if necessary.
exchange transfusion to remove the circulating toxins.
SURGICAL MANAGEMENT
No specific surgical correction. The only successful treatment for end stage liver disease is liver
transplantation.
If there is portal hypertension with hematemesis, Sengstaken tube may help to control the
esophageal bleed.
A portocaval anastamosis may be done to relieve the portal hypertention and complications of
hypersplenism

NURSING MANAGEMENT

Provide symptomatic treatment to the child.


Provide adequate rest and semi fowlers position.
Check and record the abdominal girth every 4th hourly.
Administer IV fluids if needed.
Provide small and frequent diet.
Provide protein rich food and massive doses of vitamin B6.
Follow aseptic techniques to prevent infection.
Intake and output chart should be maintained.
Provide parental education:
explain the cause, symptoms and management of the disease
avoid food rich in copper like dry nuts, chocolates, liver etc
provide small and frequent diet to the child.
Advise the mother to breast feed their baby for longer period and not to introduce food
supplements beyond the age of 6 months.
Milk used for infant should not be boiled and stored in copper and copper alloy pots.
reduce the use of brass and copper vessels. Use aluminium and steel utensils.
foods rich in tryptophan ( milk, eggs, meat, nuts, beans, fish, and cheese) should be reduced.
provide more vitamin B6 foods like potato, banana, spinach, soya bean, fruits and
vegetables.( vitamin B6 help to convert tryptophan to niacin)

Nursing diagnosis
1.
2.
3.
4.
5.

Hyperthermia related to inflammatory process in the liver.


Impaired 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.

WILSON DISEASE
INTRODUCTION
Wilsons disease is an inborn error of metabolism due to toxic accumulation of copper in liver,
brain, cornea and other tissues which manifest as neurological or psychiatric symptoms.
Wilson disease (WD), also known as hepatolenticular degeneration, was first described by
American neurologist, Kinnear Wilson, in 1912.

DEFINITION
Wilson disease (hepatolenticular degeneration) is an autosomal recessive disorder that can be
associated with degenerative changes in the brain, liver disease, and Kayser-Fleischer rings in the cornea.
EPIDEMIOLOGY

The incidence is 1/50,000 to 1/100,000 births. It is progressive and potentially fatal if untreated;
specific effective treatment is available.
WD may present with hepatic, hematologic, neurologic, or psychiatric symptoms. WD presents as
liver disease more commonly in children and younger adults than older adults. Its most typical
presentation is that of hepatic or hematologic symptoms in the second decade of life. Neurologic
and psychiatric presentations are more common in the third and fourth decades.

PATHOGENESIS
Copper is an essential metal which acts as a
cofactor for many proteins. In normal human copper
metabolism, the dietary intake of copper exceeds
physiologic needs and is therefore excreted. Copper is
absorbed in the stomach and proximal small intestine
relatively efficiently; therefore, to avoid copper toxicity,
the amount of copper absorbed and stored in the body
must be finely regulated. The main system for achieving
such regulation is the hepatic excretion of copper into
bile. Up to 80% of absorbed copper is actually excreted
in bile to maintain this homeostasis.

Schematic diagram of copper


storage and excretion

WD is transmitted by autosomal recessive inheritance. It is caused by a mutation in the ATP7B


gene. The ATPB7 gene normally codes for a metal-transporting P-type adenosine triphosphatase (ATPase),
mainly expressed on hepatocytes. In healthy, this ATPase functions in the transmembrane transport of

copper within hepatocytes. In WD, the absent or reduced function of this ATPase results in the decreased
hepatocellular excretion of copper into bile and defective incorporation of copper into ceruloplasmin. This
in turn leads to the progressive accumulation of copper in the liver, and subsequent hepatic injury. Over
time, copper may be released into the bloodstream and deposited in secondary organs, such as the brain,
cornea, and kidneys. Clinical disease therefore may range over a variable spectrum including abnormal
liver function tests to fulminant hepatic failure and cirrhosis, to seizures, psychosis, and other neurologic
manifestations.
CLINICAL MANIFESTATIONS
Disease presentations are variable. The younger the patient, the more likely hepatic involvement
will be the predominant manifestation. Girls are 3 times more likely than boys to present with acute hepatic
failure. After 20 yr of age, neurologic symptoms predominate. Clinical symptoms of liver involvement in
WD typically do not occur prior to 35 years of life.
Patients less than 20 years old tend to present with liver dysfunction whereas older patients
typically present with psychiatric and neurologic manifestations.
Forms of Wilsonian hepatic disease include asymptomatic hepatomegaly (with or without splenomegaly),
subacute or chronic hepatitis, and acute hepatic failure (with or without hemolytic anemia).
From a hepatic perspective, signs and symptoms may include:

asymptomatic elevation of aminotransferases


asymptomatic hepatomegaly
isolated splenomegaly
acute hepatitis
fatty liver
portal hypertension with possible associated gastrointestinal (variceal) bleeding
ascites
edema
other effects of hepatic dysfunction (delayed puberty, amenorrhea, coagulation defect)
cirrhosis, and/or fulminant hepatic failure.

From a neurologic or psychiatric perspective, signs and symptoms may include:

changes in behavior
deterioration in school performance
movement disorders
lack of motor coordination
rigid dystonia
dysarthria
drooling
seizures

migraine
headaches
anxiety
depression
insomnia,
personality changes, and/or psychosis

Other manifestations include:

Kayser-Fleischer rings may be absent in young patients with liver disease but are always present in
patients with neurologic symptoms.
Menstrual irregularities (e.g. amenorrhea, infertility),
Coombs-negative hemolytic anemia (possibly related to the release of large amounts of copper
from damaged hepatocytes)
Copper deposition in the kidneys may lead to nephrocalcinosis, hematuria, or aminoaciduria.
Cardiac manifestations may include cardiomyopathy and arrhythmias.
Unusual manifestations include arthritis, infertility or recurrent miscarriages and endocrinopathies
(hypoparathyroidism).

DIAGNOSIS

Wilson disease should be considered in children and teenagers with unexplained acute or chronic
liver disease, neurologic symptoms of unknown cause, acute hemolysis, psychiatric illnesses,
behavioral changes, Fanconi syndrome, or unexplained bone (osteoporosis, fractures) or muscle
disease (myopathy, arthralgia).
Serum ceruloplasmin: Most patients with Wilson disease have decreased ceruloplasmin levels ( <
20 mg/dL).
Serum copper level: may be elevated in early Wilson disease
Urinary copper excretion: Urinary copper excretion (usually < 40 g/day) is increased to > 100
g/day and often up to 1,000 g or more per day.
Response of urinary copper output to chelation: Estimation of urinary copper after a dpenicillamine challenge (During the 24 hr urine collection patients are given two 500 mg oral doses
of d penicillamine 12 hr apart) help differentiate WD from other causes of raised urinary copper;
excretion >1,600 g/24 hr. is characteristic of WD.
Demonstration of Kayser-Fleischer rings, which might not be present in younger children, requires
a slit-lamp examination by an ophthalmologist.
Liver biopsy is of value for determining the extent and severity of liver disease and for measuring
the hepatic copper content (normally < 10 g/g dry weight). In Wilson disease, hepatic copper
content exceeds 250 g/g dry weight. In later stages of Wilson disease hepatic copper content can
be unreliable because cirrhosis leads to variable hepatic copper distribution and sampling error.

TREATMENT

WD is fatal without treatment. Treatment modalities for WD include diet modification, chelation,
zinc, antioxidants, tetrathiomolybdate, and liver transplantation. Choice of specific therapy is dependent
upon clinical presentation.

Dietary modification

Dietary modification with restriction of consumed copper is nearly universally recommended for
WD patients for at least their first year of therapy.
A major attempt should be made to restrict dietary copper intake to < 1 mg/day. Foods such
as liver, shellfish, nuts, mushroom and chocolate should be avoided. If the copper content of
the drinking water exceeds 0.1 mg/L, it may be necessary to demineralize the water.
Copper-chelating agents

Copper chelation is the mainstay of treatment, which leads to rapid excretion of excess deposited
copper. Currently, D-penicillamine and trientine are the typical chelation medications utilized in treating
WD.

oral administration of d -penicillamine ( , -dimethylcysteine) in a dose of 1 g/day in 2


doses before meals for adults and 20 mg/kg/day for pediatric patients
or

triethylene tetramine dihydrochloride at a dose of 0.5-2.0 g/day for adults and 20 mg/kg/day
for children.

In response to chelation, urinary copper excretion markedly increases, and with continued
administration, urinary copper levels can become normal, with marked improvement in hepatic and
neurologic function and the disappearance of Kayser-Fleischer rings.
Pyridoxine (25 mg/ day) should be co-administered with both D-penicillamine and trientine.
Severe adverse reactions can occur and have been reported to occur at a frequency of approximately 30%
of patients treated with D-penicillamine and 5% of patients treated with trientine. These reactions may
include hypersensitivity reactions, thrombocytopenia, neutropenia, proteinuria, and autoimmune diseases.
Efficacy of treatment is determined by following 24-hour urine copper measurements that is followed
every 3 months for the first 2 years and then annually. Drug toxicity needs to be followed closely with a
complete blood count, hepatic panel, creatinine, and urinalysis serially.
Ammonium tetrathiomolybdate is another alternative chelating agent under investigation for patients
with neurologic disease; initial results suggest that significantly fewer patients experience neurologic
deterioration with this drug compared to penicillamine. The initial dose is 120 mg/day (20 mg between
meals tid and 20 mg with meals tid). Side effects include anemia, leukopenia, thrombocytopenia, and mild
elevations of transaminases.

Zinc therapy

Zinc has also been used as adjuvant therapy, maintenance therapy, or primary therapy in
presymptomatic patients, owing to its unique ability to impair the gastrointestinal absorption of copper.
Zinc acetate is given in adults at a dose of 25-50 mg of elemental zinc 3 times a day, and 25 mg 3
times a day in children > 5 yr of age.
Side effects are mostly limited to gastric upset.

Liver transplantation
In hepatic failure, liver transplantation is the most appropriate therapy. Plasmapheresis or kidney
dialysis may be used as temporizing measures to bridge to transplant. It is also indicated in the absence of
liver failure in patients with neurological WD in whom chelation therapy has proved ineffective.
SCREENING FOR WD
Screening for WD in asymptomatic relatives should begin at 3 years of age. This screen should
include a history and physical examination, hepatic function panel, prothrombin time and INR, slit lamp
examination by a pediatric ophthalmologist, 24-hour urine copper, and consideration of genetic testing.

PROGNOSIS
Untreated patients with Wilson disease can die of hepatic, neurologic, renal, or hematologic
complications. The prognosis for patients receiving prompt and continuous penicillamine is variable and
depends on the time of initiation of and the individual response to chelation. Liver transplantation should
be considered for patients with fulminant liver disease, decompensated cirrhosis, or progressive neurologic
disease; the last indication remains controversial. Liver transplantation is curative, with a survival rate of
85-90%. In asymptomatic siblings of affected patients, early institution of chelation or zinc therapy can
prevent expression of the disease.

REYE SYNDROME
INTRODUCTION
Reye syndrome is characterized by acute non-inflammatory encephalopathy and fatty degenerative
liver failure. The syndrome was first described in 1963 in Australia by RDK Reye and described a few

months later in the United States by GM Johnson. Reye syndrome typically occurs after a viral illness,
particularly an upper respratory tract infection, influenza, varicella, or gastroenteritis, and is associated
with the use of aspirin during the illness. A dramatic decrease in the use of aspirin among children, in
combination with the identification of medication reactions, toxins, and inborn errors of metabolism
(IEMs) that present with Reye syndromelike manifestations, have made the diagnosis of Reye syndrome
exceedingly rare.
DEFINITION
Reye syndrome is a disorder defined as acute encephalopathy associated with other characteristic
organ involvement. It is characterized by fever, profoundly impaired consciousness, and disordered hepatic
function.
ETIOLOGY
The etiology of RS is not well understood, but most cases follow a common viral illness, typically
influenza or varicella. Etiological factors include:
Pathogens
Influenza virus types A and B and varicella-zoster virus are the pathogens most commonly
associated with Reye syndrome. Other pathogens include parainfluenza virus, adenovirus, coxsackievirus,
measles, cytomegalovirus, Epstein-Barr virus, HIV, retrovirus, hepatitis virus types A and B, mycoplasma,
chlamydia, pertussis, shigella, and salmonella.
Salicylates
The association of Reye syndrome with salicylates, particularly aspirin, was demonstrated in
several epidemiologic studies around the world. Less than 0.1% of children who took aspirin developed
Reye syndrome, but more than 80% of patients diagnosed with Reye syndrome had taken aspirin in the past
3 weeks. Recommendations by government health agencies that children not be treated with salicylates led
to an immediate and dramatic decrease in the incidence of Reye syndrome.
Other agents
Acetaminophen, outdated tetracycline, valproic acid, warfarin, zidovudine didanosine, and some
neoplastic drugs have been associated with Reye syndrome or Reye-like syndrome. Nonsteroidal antiinflammatory drugs, including sodium diclofenac and mefenamic acid, are thought to produce or worsen
Reye syndrome. An association with antiemetics, such as phenothiazines, has been postulated but not
substantiated.
Reye syndrome or Reye-like syndrome may also be associated with insecticides; herbicides;
isopropyl alcohol; paint; paint thinner; hepatotoxic mushrooms etc
Inborn errors of metabolism

IEMs that produce Reye-like syndromes include fatty-acid oxidation defects, particularly mediumchain acyl dehydrogenase (MCAD) and long-chain acyl dehydrogenase deficiency (LCAD) inherited and
acquired forms, urea-cycle defects, amino and organic acidopathies, primary carnitine deficiency, and
disorders of carbohydrate metabolism.
EPIDEMIOLOGY
Incidence peaks between age 5 and 14 years; 13.5% were younger than 1 year.
Reye syndrome rarely occurs in newborns or in children older than 18 years.
Reye syndrome is equally distributed between the sexes.
PATHOPHYSIOLOGY
RS is a condition characterized pathologically by cerebral edema and fatty changes of the liver. The
onset of RS is notable for profuse effortless vomiting and varying degrees of neurologic impairment,
including personality changes, seizures, and coma, that lead to increase ICP, herniation, and death
The pathogenesis of Reye syndrome, while not precisely elucidated, appears to involve
mitochondrial injury (induced by various viruses, drugs, exogenous toxins, and genetic factors) resulting
in dysfunction that inhibits oxidative phosphorylation and fatty-acid beta-oxidation in a virus-infected,
sensitized host. The host has usually been exposed to mitochondrial toxins, most commonly salicylates
(>80% of cases).
Histologic changes include cytoplasmic fatty vacuolization in hepatocytes, astrocyte edema and loss
of neurons in the brain, and edema and fatty degeneration of the proximal lobules in the kidneys. All cells
have pleomorphic, swollen mitochondria that are reduced in number, along with glycogen depletion and
minimal tissue inflammation. Hepatic mitochondrial dysfunction results in hyperammonemia (elevated
serum ammonia), which is thought to induce astrocyte edema, resulting in cerebral edema and increased
intracranial pressure (ICP).
CLINICAL PRESENTATION
History:
Most patients had at least 1 viral illness (viral upper respiratory illness or influenza, varicella,
gastroenteritis) in the 3 weeks preceding the onset of Reye syndrome.
Salicylates were detectable in the blood of 82% of patients.
Reye syndrome can occur after vaccination with live viral vaccines.
Physical Examination
Signs and symptoms of Reye syndrome include
protracted vomiting, with or without clinically significant
dehydration; hepatomegaly in 50%; minimal or absent
jaundice; and lethargy progressing to encephalopathy,

Cardinal Symptoms Reyes


Syndrome

Persistent vomiting
Diarrhea
Lethargy
Restlessness and irritability
Hydrocephalus
Hyperammonimia
Hyperglycemia

obtundation, coma, seizures, and paralysis. Notably,


patients are afebrile.

Stage 0
Stage 1
Stage 2

Stage 3
Stage 4
Stage 5
Stage 6

HURWITZ CLASSIFICATION
Alert, abnormal history and laboratory findings consistent with Reye
syndrome, and no clinical manifestations
Vomiting, sleepiness, and lethargy
Restlessness, irritability, combativeness, disorientation, delirium,
tachycardia, hyperventilation, dilated pupils with sluggish response,
hyperreflexia, positive Babinski sign, and appropriate response to noxious
stimuli
Obtunded, comatose, decorticate rigidity, and inappropriate response to
noxious stimuli
Deep coma, decerebrate rigidity, fixed and dilated pupils, loss of
oculovestibular reflexes, and dysconjugate gaze with caloric stimulation
Seizures, flaccid paralysis, absent deep tendon reflexes (DTRs), no pupillary
response, and respiratory arrest
Patients who cannot be classified because they have been treated with curare
or another medication that alters the level of consciousness
DIAGNOSTIC CRITERIA

Acute
non-inflammatory
encephalopathy with an altered level
of consciousness
DIAGNOSIS
Hepatic dysfunction with a liver
Workup to exclude inborn errors of metabolism (IEMs) mustbiopsy
be performed
include
showingand
fattyshould
metamorphosis
evaluation for defects of fatty-acid oxidation, amino and organic
acidurias,
urea-cycleordefects,
without
inflammation
necrosisand
or a
disorders of carbohydrate metabolism.
greater than 3-fold increase in alanine
Computed tomography (CT) of the head may reveal cerebral aminotransferase
edema, but the results
are usually
(ALT),
aspartate
normal.
aminotransferase (AST), or ammonia
Electroencephalography (EEG) may reveal slow-wave activity in the early stages and flattened
levels
waves in advanced stages.
No other explanation for cerebral
MRI characteristics of Reye syndrome are symmetric thalamic, white matter and basal ganglia
edema or hepatic abnormality
lesions, in children with recent history of salycilates or immunosuppressive
drugs
intake.
Cerebrospinal
fluid
(CSF) with a
white blood cell (WBC) count of 8
Laboratory Studies
cells/L
or
fewer
(usually
lymphocytes); note that lumbar
An ammonia level as high as 1.5 times normal 24-48 hours
puncture should not be performed in
after the onset of mental status changes is the most frequent
patients who are hemodynamically
laboratory abnormality. Ammonia tends to peak 56-60
unstable and/or those in whom
increased intracranial pressure (ICP)
is a concern
Brain biopsy with findings of
cerebral edema without inflammation

hours after the onset of symptoms. The ammonia level may


return to normal in stages 4 and 5.
Levels of alanine aminotransferase (ALT) and aspartate
aminotransferase (AST) increase to 3 times normal but
may return to normal by stages 4 or 5. Bilirubin levels are
higher than 2 mg/dL (but usually lower than 3 mg/dL) in
10-15% of patients. If the direct bilirubin level is more than
15% of total or if the total bilirubin level exceeds 3 mg/dL,
consider other diagnoses.
Prothrombin time (PT) and activated partial thromboplastin
time (aPTT) are prolonged more than 1.5-fold in more than
50% of patients. Levels of factors I (fibrinogen), II, VII,
IX, and X may be low because of the disruption of
synthetic activities in the liver. Consumption may also
contribute to low levels of coagulation factors. Platelet
counts are usually normal.
Lipase and amylase levels are elevated. The serum
bicarbonate level is decreased secondary to vomiting.
Blood urea nitrogen (BUN) and creatinine levels are
elevated.
Glucose, while usually normal, may be low, particularly during stage 5 and in children younger than 1
year.
Lactic dehydrogenase (LDH) levels may be high or low.
Levels of free fatty acids and amino acids (eg, glutamine, alanine, and lysine) may be elevated.
Determine the anion gap and venous blood gas level to evaluate for metabolic acidosis.
Urine specific gravity is increased; 80% of patients have ketonuria.
Cerebral spinal fluid WBC count, by disease definition, does not exceed 8 cells/L. Opening pressure
is usually normal but may be elevated, particularly in stages 3-5.

Invasive Procedures
The following procedures may be helpful in treatment, workup, and monitoring:
Vascular access (arterial, central venous, or both)
Lumbar puncture if the patient is hemodynamically stable and shows no signs of increased
intracranial pressure (ICP) Opening pressure may or may not be increased; the white blood cell
(WBC) count in the cerebrospinal fluid (CSF) is 8/L or fewer
Intubation to maintain airway and ventilation and to manage ICP
Nasogastric tube placement to decompress the abdomen
Bladder catheterization to monitor urine output
Percutaneous liver biopsy (if indicated) to exclude an IEM or toxic liver disease
Placement of an intracranial device for ICP monitoring in patients with increased ICP

Coagulopathy must be corrected before invasive procedures are performed.


COMPLICATIONS

Brain herniation, status epilepticus, syndrome of inappropriate secretion of antidiuretic hormone


(SIADH), and diabetes insipidus
Acute respiratory failure and aspiration pneumonia
Cardiac arrhythmias
Myocardial infarction
Cardiovascular collapse
Gastrointestinal bleeding and pancreatitis
Acute renal failure
Sepsis
Death

TREATMENT
No specific treatment exists for Reye syndrome; supportive care is based on the stage of the
syndrome.
Continue careful monitoring.
Establish and maintain the patients airway, breathing, and circulation.
Check the glucose level, particularly if the patient is younger than 1 year and/or has altered mental
status. Administer dextrose to correct hypoglycemia.
Admission to the intensive care unit (ICU) is warranted for continued monitoring and treatment.
Consider consultation with a neurologist for electroencephalography (EEG).
Consider consultation with a neurosurgeon for monitoring and treatment of increased intracranial
pressure (ICP).
Consider consultation with a gastroenterologist or surgeon for liver biopsy.
Consider consultation with a metabolic disease specialist if an inborn error of metabolism (IEM) is
a possibility.
Monitor and treat long-term neurologic sequelae. Prescribe outpatient anticonvulsants if ongoing
seizures occur.
Stage-Specific Management
Supportive care is based on the clinical stage of the syndrome, with aggressive treatment provided
to correct or prevent metabolic abnormalities, particularly hypoglycemia and hyperammonemia, and to
prevent or control cerebral edema.
Stages 0-1
Keep the patient quiet.
Frequently monitor vital signs and laboratory values.
Correct fluid and electrolyte abnormalities, hypoglycemia, and acidosis.

If the patient is hypoglycemic, administer dextrose 25% as an intravenous (IV) bolus in a


dose of 1-2 mL/kg.
The use of bicarbonate to correct acidosis is controversial because of potential paradoxical
cerebrospinal fluid (CSF) acidosis. If the initial pH is less than 7.0-7.2, consider
administering sodium bicarbonate 0.5-2 mEq/kg/h to correct it to 7.25-7.3, with the dosage
based on the deficit, calculated as follows:
Deficit in HCO3 (mEq) = weight (kg) base excess 0.3
Avoid rapid correction or overcorrection. Recognize that administration of sodium
bicarbonate results in a significant sodium load.
Maintain electrolytes, serum pH, albumin, serum osmolality, glucose, and urine output in normal
ranges.
Consider restricting fluids to two thirds of maintenance. Overhydration may precipitate cerebral
edema. Use colloids (eg, albumin) as necessary to maintain intravascular volume. Dehydration may
compromise cardiovascular volume and reduce cerebral perfusion.
Glucose should be maintained in the 100-125 mg/dL range; this will require administration of D10
or D20.
Place a Foley catheter to monitor urine output.
Consider giving ondansetron 1-2 mg IV every 8 hours to decrease vomiting.
Antacids may also be administered for gastrointestinal (GI) protection.

Stage 2
The standard of care consists of continuous cardiorespiratory monitoring, placement of central
venous lines or arterial lines to monitor hemodynamic status, urine catheters to monitor urine
output, ECG to monitor cardiac function, and EEG to monitor seizure activity.
Endotracheal intubation may be required at this stage to maintain the airway, control ventilation,
and prevent increased ICP. Use rapid-sequence agents that minimize the chance of increasing ICP.
Place a nasogastric tube to decompress the abdomen.
Hyperammonemia can contribute to cerebral edema and therefore must be corrected aggressively.
sodium phenylacetatesodium benzoate is FDA-approved for the treatment of acute
hyperammonemia and associated encephalopathy in patients with deficiencies in enzymes of the
urea cycle.
Administer ondansetron 1-2 mg IV during the first 15 minutes of the initial dose of sodium
phenylacetatesodium benzoate. If the ammonia level is higher than 500 g/dL or if the patients
condition fails to respond to the initial dose of sodium phenylacetatesodium benzoate, start
dialysis, preferably hemodialysis.
Prevent increased ICP. Elevate the head to 30, keep the head in a midline orientation, use isotonic
rather than hypotonic fluids, avoid overhydration, and administer furosemide 1 mg/kg as often as
every 4-6 hours to control fluid overload.
Stages 3-5
Continuously monitor ICP, central venous pressure, arterial pressure, or end-tidal carbon dioxide.
Perform endotracheal intubation if the patient is not already intubated.

Treat increased ICP by following standard guidelines, which, in addition to correction of


hyperammonemia, proper positioning of the head, and appropriate fluid management (see above),
include the following:
TREATMENT OF REYES SYNDROME - IN SHORT
Ventilation to maintain the partial pressure of carbon dioxide in the normal range
Aggressive
management
of Child
fever with
to prevent
the Syndrome:
increased cerebral
metabolism and
The Goal
of Treatment
for the
Reyes
Promoting
Effective increased
Cerebralcerebral
Perfusion
and
Controlling
Intracranial
Pressure
blood
flow
resulting from
hyperpyrexia
Analgesia and sedation to alleviate agitation or prepare for painful interventions
Treatment of Increased Intracranial Pressure and Impaired Cerebral
Paralytic agents to control shivering
Profusion
If other measures fail, mannitol 20% solution dosed at 0.25-0.5 g/kg IV infused over 10-20
minutes
as head
often as
6-8 by
hours,
or hypertonic saline 3% dosed at 3-5 mL/kg over 3-30
Elevate
the
of every
the bed
30 degrees
Maintain
the
head
a
neutral,
midline
position
minutes.
Limit
suctioning
interventions
to a minimum
as this may increased
Induced
barbiturate
coma and hypothermia
are controversial
pressure
intracranial
Treat seizures
with phenytoin 10-20 mg/kg IV as a loading dose, followed by 5 mg/kg/day
An intracranial pressure monitoring device may be placed
IV divided every 6 hours or fosphenytoin dosed as 10-20 mg/kg phenytoin equivalents.
Surgical management may include a craniotomy
Correct coagulopathy (prothrombin time >16 seconds). The data on treatment of coagulopathy in
Cerebrospinal fluid may be drained
Reye syndrome, like those on most etiologies of coagulopathy in children, are limited. Options
Treating
Renal
Failure
in(FFP),
Reyes
Syndromeplatelets, vitamin K, and exchange transfusion.
include fresh
frozen
plasma
cryoprecipitate,
FFP 10-15 mL/kg every 12-24 hours provides rapid correction and volume expansion and should be
Dialysis may be indicated to reduce ammonia levels or residual
administered, particularly if active bleeding is present or if invasive procedures (eg, ICP monitoring
salicylate
device placement or liver biopsy) are required. If the fibrinogen level is lower than 100 mg/dL,
Exchange transfusions may be necessary
cryoprecipitate 10 mL/kg every 6 hours should be considered instead of FFP because
Treating
cryoprecipitate
Coagulopathies
has a higher concentration
in Reyes Syndrome
of fibrinogen.
If invasive procedures are to be performed, platelets should also be given as needed to restore the
Coagulation therapies: Vitamin K
platelet count to a value higher than 50,000/L. Vitamin K 1-10 mg IV may be administered instead
Blood transfusions: fresh frozen plasma and platelets
of FFP or cryoprecipitate if the need for correction is not an emergency. Exchange transfusion is
rarely required.
Managing
Hypoglycemia in Reyes Syndrome
Reye syndrome has been successfully treated with liver transplantation

Glucose may be administered intravenously


Emergency hypoglycemic treatments

Airway Patency for Reyes Syndrome

Intubation may be required for some patients due to neurological


deficits or lung infiltration of fatty deposits
Endotracheal tube placement
Mechanical ventilation
A paralytic agent may be used

Pharmaceutical Therapies for Reyes Syndrome

Diuretics such as mannitol and furosemide


Decompression craniotomy
Hypothermia measures
Exchange transfusion
Gluconate

Maintain a Quiet and Relaxed Environment

Minimize stimuli and visitors


Spread out nursing interventions

as

possible

to

eliminate

PREVENTION
Salicylates should be avoided in children, except in those who have conditions for which salicylates
are a mainstay of therapy (eg, Kawasaki disease).
It is critical to be alert for and recognize early symptoms of Reye syndrome. It is also important to
be mindful of the possibility that an IEM may be the actual cause of the symptoms and, if this is the
case, to be prepared to treat the IEM. Appropriate management of IEMs dramatically decreases
morbidity and mortality.
Influenza vaccine is recommended by the Centers for Disease Control and Prevention (CDC) for
everyone older than 6 months.
PROGNOSIS

Recovery from RS is rapid and usually without sequelae if the diagnosis is determined early and
therapy is initiated promptly. Patients who survive have full liver function recovery; however,
approximately one third may have subtle neuropsychological deficits.
NURSING CARE MANAGEMENT
Care and observations are implemented as for any child with an altered state of consciousness and
increasing ICP.
Potential Nursing Diagnoses

Ineffective breathing pattern


Impaired gas exchange
Impaired physical mobility
Risk for ineffective cerebral tissue perfusion
Risk of injury from convulsions
Risk for impaired skin integrity and infection related to impaired mobility (possibly from
coma)

Possible Nursing Interventions

Maintain hydration by administering fluids intravenously


Institute and maintain seizure precautions
Preventing skin breakdown from immobility: pad bony prominences, range of motion
exercises, turning and position changes
Monitor intake and output, as the patient may be taking diuretics and may experience renal
impairment from the condition
Maintain nothing-by-mouth status of ordered
Provide care for endotracheal tube and mechanical ventilator; ensure patency
Initiate intravenous access and ensure patency
Administer medications as ordered; such as glucose, vitamin K, diuretics, and paralytics
Care for intracranial pressure monitoring device
Provide a quiet environment to reduce risks of increasing the intracranial pressure
Position the head at midline and raise the head of the bed to 30 degrees
Administer blood products as ordered Administer vitamin K, fresh frozen plasma, and
platelets as necessary to reduce the risk of bleeding; institute bleeding precautions
Because of related liver dysfunction, laboratory studies to determine impaired coagulation,
such as prolonged bleeding time, should be monitored.
Support the patient and family and provide teaching for coping mechanisms related to
situational crises.
Parents of children with RS need to be kept informed of the childs progress, to have
diagnostic procedures and therapeutic management explained, and to be given concerned
and sympathetic support.

Families need to be aware that salicylate, the alleged offending ingredient in aspirin, is
contained in other products. They should refrain from administering any product for
influenza-like symptoms without first checking the label for hidden salicylates.

Nursing Outcomes

Maintenance of adequate ventilation and oxygenation


Maintenance of range of motion and joint mobility
Maintenance of respiration rate
Remain oriented to environment
Maintenance of skin integrity without any signs of skin breakdown

RESEARCH STUDIES
Role of copper in Indian childhood cirrhosis.
Tanner MS1.
Author information

Department of Paediatrics, University of Sheffield, United Kingdom. m.s.tanner@sheffield.ac.uk

Abstract
Of the cirrhoses that affect Indian children, Indian childhood cirrhosis (ICC) is a discrete clinical and
histologic entity in which large amounts of copper are deposited in the liver. The evidence linking copper
deposition to increased dietary copper intake in infancy was reviewed. Prevention of this feeding pattern
prevents ICC, and the disease has now largely disappeared from many parts of India. Penicillamine, if
given before the terminal clinical stage of ICC, reduces mortality from 92% to 53%. Long-term survivors
show a sequence of histologic resolution, resulting either in inactive micronodular cirrhosis or in virtually
normal histologic appearance. Twenty-nine treated ICC patients reexamined at 8.8 y of age (range: 6.3-13
y), 5-12 y after diagnosis, were well and had normal results from liver function tests. Clinical and
epidemiologic evidence show that there must be excessive copper ingestion for ICC to develop, but the
lack of an animal model, the inconstant relation between liver copper concentrations and liver damage, and
the rarity of liver disease in adults suggests that other etiologic factors contribute. Two mechanisms are
discussed: 1) that copper may be acting in synergy with a hepatotoxin, or 2) that there may be a genetic
predisposition to copper-associated liver damage, as suggested recently for Tyrollean childhood cirrhosis.
Although ICC is now rare, sporadic cases of an ICC-like disorder in infants continue to occur. There should
be a greater awareness among pediatricians of this disease to enable early diagnosis. Penicillamine should
be used early and adverse prognostic factors recognized as indications for early transplantation and
unregulated water supplies should not be used to prepare infant feeds

Is Indian childhood cirrhosis an extinct disease now?--An


observational study.
Patra S1, Vij M, Kancherala R, Samal SC.
Indian J Pediatr. 2013 Aug;80(8):651-4. doi: 10.1007/s12098-012-0935-1. Epub 2012 Dec 22.
Author information

Department of Pathology, Global Health City, Chennai, Tamil Nadu 600100, India.
wususama@gmail.com

Abstract
OBJECTIVE:
Indian childhood cirrhosis (ICC), an unique liver disease that has been endemic in most parts of India and
advocated by some to be caused by hepatotoxic effect of excess dietary copper is generally believed now to
have virtually disappeared from the country. In the face of this the authors report here five cases of ICC
encountered in one children hospital over the last 10 y period.
METHODS:
Cases histologically categorized as ICC were initially picked up from the records of the department of
Pathology. Their clinical, investigational and follow up information retrieved from hospital data base along
with pathologic features of liver biopsies were reviewed in detail.
RESULTS:
The age range of the three male and two female children were from 1 to 12 y and on clinical and
investigational features all 5 cases were labeled as non-Wilsonian liver disease of uncertain etiology.
Histopathologic findings in each case however, was characteristic of accepted established phase of ICC.
Three of the five children died in hospital while the other two left the hospital and were lost to follow up.
None of the children had exposure to excess dietary copper.
CONCLUSIONS:
Cases of ICC continue to occur in Andhra Pradesh and very likely in other parts of India. Established and
non-typical cases are possibly being missed because of no histologic confirmation and unawareness of the
protean manifestation and natural history of this disease. Dietary copper overload is unlikely to play a
causal role in ICC.

Simultaneous Presentation of Wilson's Disease and Autoimmune


Hepatitis; A Case Report and Review of Literature.

Dara N1, Imanzadeh F1, Sayyari AA1, Nasri P1, Hosseini AH1.
Author information

Department of Pediatric Gastroenterology, Mofid Children Hospital, Shahid Beheshti University of


Medical Sciences, Tehran, IR Iran.

Abstract
INTRODUCTION:
Coexistence of Wilson's disease and autoimmune hepatitis has been rarely reported in English literature. In
this group of patients, there exist features of both diseases and laboratory and histopathological studies may
be misleading. Medical treatment for any of these entities, per se, may result in poor response. Therefore,
by considering the acute hepatitis resembling Wilson's disease and autoimmune hepatitis, simultaneous
therapy with immunosuppressive and penicillamine may have a superior benefit.
CASE PRESENTATION:
We present the case of a 10-year-old boy with nausea, vomiting, yellowish discoloration of skin and sclera,
abdominal pain and tea-color urine. Physical examination showed mild hepatomegaly and right upper
quadrant tenderness. Laboratory and histochemical studies and atomic absorption test were done and the
results were highly suggestive of both Wilson's disease and autoimmune hepatitis, in him.
CONCLUSIONS:
This case study highlights, although rare, the coexistence of Wilson's disease and autoimmune hepatitis and
the need to maintain a high level of awareness of this problem. Therefore, it is reasonable to consider this
type of hepatitis in rare patients, with dominant features of both diseases at the same time.

Aspirin and Reye syndrome: a review of the evidence.


Schrr K1.
Author information

Institut fr Pharmakologie und Klinische Pharmakologie, Heinrich-Heine-Universitt Dsseldorf,


Dsseldorf, Germany. kschroer@uni-duesseldorf.de

Abstract
Reye syndrome is an extremely rare but severe and often fatal disease. Death occurs in about 30-40% of
cases from brainstem dysfunction. The disease typically is preceded by a viral infection with an

intermediate disease-free interval of 3-5 days. The biochemical explanation for Reye-like symptoms is a
generalized disturbance in mitochondrial metabolism, eventually resulting in metabolic failure in the liver
and other tissues. The etiology of 'classical' Reye syndrome is unknown. Hypothetically, the syndrome may
result from an unusual response to the preceding viral infection, which is determined by host genetic
factors but can be modified by a variety of exogenous agents. Thus, several infections and diseases might
present clinically with Reye-like symptoms. Exogenous agents involve a number of toxins, drugs
(including aspirin [acetylsalicylic acid]), and other chemicals. The 'rise and fall' in the incidence of Reye
syndrome is still poorly understood and unexplained. With a few exceptions, there were probably no new
Reye-like diseases reported during the last 10 years that could not be explained by an inherited disorder of
metabolism or a misdiagnosis. This may reflect scientific progress in the better understanding of cellular
and molecular dysfunctions as disease-determining factors. Alternatively, the immune response to and the
virulence of a virus might have changed by alteration of its genetic code. The suggestion of a defined
cause-effect relationship between aspirin intake and Reye syndrome in children is not supported by
sufficient facts. Clearly, no drug treatment is without side effects. Thus, a balanced view of whether
treatment with a certain drug is justified in terms of the benefit/risk ratio is always necessary. Aspirin is no
exception.

BIBLIOGRAPHY
1. Robert M. Kliegman, Stanton, NELSON TEXTBOOK OF PEDIATRICS, 19 th edn,2011, Elsevier
publishers, Philadelphia, page no: 1390-1395
2. John F. Pohl, Christopher Jolley, Pediatric Gastroenterology A Color Handbook,2014, CRC Press,
USA, Page no 342-350
3. Marilyn J Hockenberry, David Wilson. Wongs Nursing Care of infants and children. 9th edition.
Elsevier publishers.USA.2011
4. www.medscape.com
5. N.C. Nayak & A.R. Chitale, Indian childhood cirrhosis (ICC) & ICC-like diseases: The changing
scenario of facts versus notions, Indian J Med Res 137, June 2013, pp 1029-1042

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