Liver Disease &

GI Disorders in Children

NURS 366
Alison J. Montpetit, RN, PhD
 I. Hepatic A&P – Review
study guide and especially
A. Hepatic blood flow
B. Liver lobule anatomy
C. Metabolism of bilirubin
D. Metabolism of nutrients
E. Metabolic detoxification
Hepatic Blood Flow

A. liver
B. hepatic vein
C. hepatic artery
D. portal vein
E. common bile duct
F. stomach
G. cystic duct
H. gallbladder
Hepatic arterial and venous systems
Liver Lobule
F. Tests of liver function – Table H&M 33-3

1.Alkaline phosphatase – elevates in liver damage

or biliary obstruction
2.Liver/biliary enzymes that are released from
damaged cells
a) AST
b) ALT
d) LDH
3. Bilirubin
4. Plasma proteins (esp. albumin)
5. Clotting factors made in the liver – disease
affects PTT, PT
 II. Clinical Manifestations
of Liver Disease
A. Portal Hypertension
1. High pressure in the portal
venous system
2. Caused by obstruction to
venous flow through the
portal system
 Portal Hypertension cont.
a) Obstruction from damaged
liver – scar tissue, fibrosis
b) Results in reduced blood flow
into liver
c) Causing high hepatic venous
pressure
d) Resulting in development of
collateral blood vessels
(varices – dilated veins)

Portal HTN can result in: Esophageal Varices, Splenomegaly, Ascites

3. Results in
a) Esophageal Varices
(1) Distended, tortuous, collateral veins
(2) Found in lower esophagus, stomach and
rectum
(3) Results in significant hematemesis from
rupture of varices
(4) Mortality rate is high (30-60%) with rupture
Esophageal Varices:
pushing into the lumen
of the esophagus

Sclerotherapy
Large
Esophageal
Varices
b) Splenomegaly

(1) Enlarged from increased

portal pressure - backs up
through splenic vein
c) Ascites
(1) Overview
(a) Accumulation of fluid in peritoneal cavity
(b) Reduces circulatory volume
(c) Cirrhosis most common cause, also CHF,
cancer, infections
 Ascites
(2) In liver disease, ascites is caused by
(a) Portal hypertension
(b) Decreased albumin synthesis
(c) Increased RAA due to decreased CO from hypovolemia
(d) Increased aldosterone and ADH
(3) Clinical picture
(a) Weight gain
(b) Abdominal distention (10-20 L fluid)
(c) Dyspnea, tachypnea, pleural effusion
(d) Hepatic encephalopathy
 Hepatic Encephalopathy

Accumulation of substances toxic to the brain

(a) Caused primarily by incomplete protein metabolism.
Protein normally digested as follows:
(i) Stomach:
(a) Protein – pepsin, HCL acid → proteases
(ii) Small intestine:
(a) Proteases – pancreatic enzymes: trypsin → amino
acids
(b) Amino acids – absorbed in capillaries through villi
(iii) Liver hepatocytes:
(a) a. a. used to make new proteins (tissue
proteins, plasma proteins)
(b) ammonia removed from other amino
acids – glucose, other a.a. and fatty acids
are then made
(c) ammonia converted to urea in the liver
(iv) Kidney: urea excreted
 Hepatic Encephalopathy cont.

(a) Toxic substance: Ammonia

(i) Product of protein metabolism
(ii) Also produced by bacterial action in bowel on
(a) unabsorped amino acids
(b) protein in diet
(c) blood in GI tract
(iii) Absorbed into blood, then metabolized to urea
in liver
(b) Other toxic substances contributing to encephalopathy:
fatty acids, serotonin, tryptophan
Hepatic Encephalopathy cont.
(2) Symptoms
(a) Personality changes, memory loss, irritability
(b) Lethargy, sleep disturbances
(c) Asterixis

(3) Treatment
(a) Decrease dietary protein,
(b) Lactulose – pulls ammonia from GI tract into
stool
(c) Neomycin – antibiotic, decreases GI tract
bacteria action
 Jaundice (Icterus)
1. Caused by hyperbilirubinemia (Total > 2.5-3 mg/dL)
2. Bilirubin metabolism – in liver – Fig 33-19
a) RBC destruction → HGB → Heme
b) Heme → unconjugated bilirubin (lipid soluble)
c) Conjugated in the liver to water soluble
d) Combines with other substances to make bile
e) Leaves liver, stored in gall bladder, released
into duodenum
f) Excreted into stool (bile) and urine
 Jaundice cont.
2. Types
Obstructive & Hemolytic
a) Obstructive (intra or extrahepatic)
(1) Intrahepatic (increase in
conjugated and unconjugated
bilirubin)
(a) Hepatocyte function altered -
unable to conjugate bilirubin
(b) Obstruction of bile canaliculi
- from fibrosis
 Obstructive Jaundice types
(cont’d)
(2) Extrahepatic (increase in
conjugated bilirubin)
(a) Gall bladder disease
(b) Pancreatic disease
(c)Conjugated bilirubin
accumulates in liver and enters
bloodstream
(d) Increased bilirubin in urine
since conjugated is water soluble
(e) Accumulation of bilirubin in
tissues = jaundice
b) Hemolytic jaundice

(1) Increased levels of

unconjugated bilirubin
(2) Increased RBC hemolysis
(3) Inability to meet normal or
increased conjugation needs
(4) Physiologic jaundice of the newborn
(a) Transient in 1st week of life, healthy full-term
infants
(b) High levels (>15 mg/dL) toxic to brain
(c) Pathophysiology
(i) Increased bilirubin production
(ii) Impaired hepatic uptake
(iii) increased reabsorption from the intestine
(d) Rx - phototherapy
 III. Hepatitis
A. Pathophysiology
1. Damage caused by T cell activity
a) Hepatocyte cell necrosis
b) Scarring
c) Kupffer cell hyperplasia
d) Inflammatory process causes damage
to liver
2. Various Virus Types - H&M Table 34-8
a) Hepatitis A
(1) 30 day incubation
(2) fecal – oral – IV – sexual transmission
(3) Acute onset
(4) Mild disease
(5) Children, young adults infected
(6) Vaccine available: HAV-very effective, goal is
routine child vaccination

HEP A
b) Hepatitis B
(1) 60 – 180 day incubation
(2) IV – sexual transmission
(a) virus highest in blood, wounds
(b) then semen, vaginal fluid, saliva
(3) Insidious onset
(4) Severe disease – can be prolonged or chronic; increases
mortality of liver disease by 25%
(5) Any group affected
(6) Vaccine available – HBV routine for all infants

HEP B
c) Hepatitis D
(1) 30 – 180 day incubation
(2) Percutaneous (IV drug use)– sexual transmission
(a) not fecal – oral per CDC
(3) Insidious onset
(4) Severe disease-chronic also
(5) Any group infected
(6) Vaccine (HBV) available

HEP D
d) Hepatitis C
(1) 35 – 60 day incubation
(2) IV, sexual transmission
(a) IV drug users
(i) 4X more common than HIV
(ii) rapidly infected
(iii) 60-90% infected by 5 yrs
(3) Insidious onset
(4) Variable disease – chronic disease common (80%)
(5) Cirrhosis = 20%
(5) Any group infected; highest in males mid-30s
(6) Causes 50% of chronic liver disease

HEP C
e) Hepatitis E
(1) 15 – 60 day incubation (avg 40)
(2) fecal – oral transmission; mostly contaminated water; in
US r/t internat’l travel; person to person uncommon
(3) Acute onset
(4) Variable disease (severe in pregnancy; higher mortality)
(5) Young to middle age adults infected
(6) No evidence of chronic disease

HEP E
 Hepatitis cont.

f) Signs and symptoms

(1) Elevated AST, ALT
(2) Febrile illness
(3) Malaise, lethargy
(4) Jaundice
 Stages of Hepatitis
g) Stages
(1) Prodromal
(a) 2 weeks after exposure
(b) gen’l sx – fatigue, anorexia, N&V, HA, fever
(c) lasts about 2 weeks
(2) Icteric (jaundice)
(a) lasts 2-6 weeks
(b) dark urine, clay-colored stools
(c) enlarged, tender liver
(3) Recovery
(a) jaundice gone
(b) liver remains enlarged
(c) normal function up to 12 weeks from start of jaundice
h) Fulminant hepatitis
(1) Very rapid course – 6-8 wks after 1st sx
(2) Usually related to Hep B or C
(2) Critical illness, high mortality, transplant early
 IV. Cirrhosis
A. Overview
1. Irreversible inflammatory disease
2. Disrupts liver structure and function
3. Leading cause of death
a) Women more likely to get severe disease
with alcohol abuse
b) 25% of alcoholics have cirrhosis
 Cirrhosis cont.
4. Alcohol effect - stages
a) Fatty liver
(1) Mild, reversible
(2) May be asymptomatic
b) Alcoholic hepatitis
(1) Inflammation and immune response
(2) Necrosis of hepatocytes
(3) Precursor to cirrhosis
c) Cirrhosis
(1) Alcohol changed to acetaldehyde – toxic
(2) Affects all liver functions
Normal Liver
Cirrhosis - scarring and fatty necrosis
 Cirrhosis cont.
B. Manifestations of Cirrhosis
1. Damage to all cell processes
2. Altered metabolism of nutrients
3. Liver Inflammation
a) Pain
b) Fever
c) N & V, anorexia
d) fatigue
 Cirrhosis cont.
4. Liver fibrosis and scarring
a) Portal hypertension
(1) Ascites
(2) Edema
(3) Splenomegaly
(a) Anemia
(b) Thrombocytopenia
(4) Varices
(a) Esophogeal varices
(b) Hemorrhoids
(c) Superficial abd veins
(i) Caput medusae
Caput Medusae - vessel engorgement due to
liver blood flow obstruction
 Cirrhosis cont.
5. Fatty infiltrates
6. Liver necrosis
a) Decreased bilirubin metabolism
(1) Hyperbilirubinemia
(2) Jaundice
b) Decreased bile in GI tract
(1) Clay colored stools
c) Decreased vit K absorption
(1) Bleeding
d) Increased urobilinogen
(1)Dark urine
e) Decreased metabolism of proteins, fats, CHO
(1) Hypoglycemia
f) Decreased plasma protein production
(1) Ascites and edema
 Cirrhosis cont.
g) Decreased hormone metabolism
(1) Gynecomastia
(2) Loss of body hair
(3) Menstrual dysfunction
(4) Spider angiomas
(5) Palmer erythema
(6) Increased ADH and aldosterone
Spider angioma
Palmer erythema - from cirrhosis
 Cirrhosis cont.
C. Types
1. Alcoholic
2. Biliary
a) Damage begins in canaliculi and bile ducts
b) Not in hepatocytes
c) Types
(1) Primary biliary cirrhosis: 2-5%
(a) most likely viral and autoimmune
(b) anti-mitochondrial antibodies
(c) most often women > 30
(d) transplant candidate
(2) Secondary
(a) obstruction of bile ducts
(b) inflammation of port ducts, scar
(c) gall stones, tumor, strictures, pancreatitis
 V. Disorders of the
Gallbladder - Review
A. Cholelithiasis

B. Cholecystitis

Gall stones
 VI. Pancreatitis
A. General

1. Pancreas secretes enzymes that break down

protein (trypsins), CHO (amylase) and fats
(lipase)

2. Normally proteolytic enzymes (trypsins) are

secreted in inactive form – into duodenum

3. Pancreatitis = inflammation of pancreas

 Pancreatitis cont.

B. Acute Pancreatitis
1. Causes – activation of
trypsins while still in pancreas
a) Biliary obstruction
b) Alcohol

2. Pathophysiology
a) Obstruction to outflow of pancreatic enzymes
(1) Alcohol induced edema of sphincter of Odi
(2) Gall stones
b) Activated proteases (trypsins)
c) Auto – digestion
 Pancreatitis cont.
3. S / S
a) Pain
b) Fever, N & V
c) Abdominal distention
d) 3rd spacing of fluid – peritoneal edema
e) Acute renal failure
f) Increased WBC – neutrophils
g) Increased amylase, lipase levels
C. Chronic pancreatitis

1. Chronic alcohol abuse

2. Chronic pain

3. Pancreatic enzyme deficiency

 VII. Cleft lip and cleft
palate - Review

A. Incidence
B. Pathophysiology
C. Signs and symptoms
 VIII. Esophageal
malformations - Review
A. Type
1. Esophageal atresia
2. Tracheoesophageal fistula
B. S / S
1. Bubbling, drooling
2. Aspiration
3. Abdominal distention
4. Coughing with feeding
 VIII. Esophageal Malformation
Esophageal atresia (EA) and tracheoesphageal fistula (TF)
Esophageal atresia and tracheoesphageal fistula
 IX. Pyloric stenosis
A. Incidence
1. Common in early infancy
2. Affects infants between 1-2 weeks and 3-4 months
3. Males > females
4. More in Caucasians
5. Full term > premature
 Pyloric Stenosis cont.
B. Causes
1. Increased maternal gastrin secretion
2. May be stress related
3. Family history
C. Pathophysiology
1. Hypertrophy of sphincter muscles
2. Stomach hypertrophies
3. S / S
a) New, unexplained forceful vomiting
b) May be bloody vomitus
c) Prolonged retention of food
d) Constipation
e) Wt Loss
 X. Cystic Fibrosis
A. Incidence
1. Most frequent cause of chronic lung disease in children
2. Most common life threatening inherited disease in Caucasian
population
B. Pathophysiology
1. Dysfunction of exocrine glands
2. These secrete digestive enzymes, mucus, sweat and tears
3. Triad
a) Pancreatic enzyme deficiency
b) Mucus overproduction
c) Elevated NaCl in sweat
4. All abnormal secretions are thick, difficult to remove,
obstructing
5. 85% have pancreatic insufficiency (more severe)
Thick, dry mucus that obstructs
both airways and pancreatic ducts
 Cystic Fibrosis cont.
C. Results
1. Severe maldigestion of PRO, CHO, fats
a) Steatorhea
b) Failure to thrive
c) Short stature, edema, anemia
d) Purpura (vit K malabsorption)
2. Obstruction of pancreatic ducts
3. Secondary bile cirrhosis r/t obstruction of bile channels
4. Pulmonary obstruction from mucus plugs
Purpura
from poor
Vit k
absorption
 X. Cystic Fibrosis
D. Dx
1. 72 hour stool for fat
2. Absence of pancreatic enzymes
E. Rx
1. Pancreatic enzyme replacement
2. Aggressive pulmonary hygiene
XI. Necrotizing Enterocolitis
A. Incidence
1. Potentially fatal
2. 3 in 1000
3. primarily in premature infants
a) mean age 31 weeks
b) weigh less than 1500 gms
c) risk decreases as GI tract matures
Necrotizing Enterocolitis
 NEC cont.
B. Cause
1. Hypoxic injury to intestinal mucosa - probably
2. Associated with infection, immune response,
stress, meds
C. S / S
1. Abdominal distention
2. Sepsis
3. Blood in stools
4. Gastric retention
 XII. Biliary Atresia
A. Definition
1. Absence or obstruction of intrahepatic or extrahepatic
bile ducts
B. Causes
1. Chromosomal disorder
2. Infection – immune response - viral
C. Pathophysiology
1. Inflammation, plugging
2. Fibrosis of bile canniculi and ducts
3. Continuum – process of bile duct destruction
4. 80% die by 3 yrs if not treated (transplant)
Biliary Atresia
 Biliary Atresia
D. Signs and Symptoms
1. Jaundice
2. Hepatomegaly
3. Decreased fat absorption
4. Clay colored stools
5. Wt loss
6. Increased conjugated bilirubin
7. Increased AST
 I. Hepatic A&P – Review
study guide and especially
A. Hepatic blood flow
B. Liver lobule anatomy
C. Metabolism of bilirubin
D. Metabolism of nutrients
E. Metabolic detoxification
F. Tests of liver function – Table H&M 33-3

1.Alkaline phosphatase – elevates in liver damage

or biliary obstruction
2.Liver/biliary enzymes that are released from
damaged cells
a) AST
b) ALT
d) LDH
3. Bilirubin
4. Plasma proteins (esp. albumin)
5. Clotting factors made in the liver – disease
affects PTT, PT