A Child

With
Chronic
Liver
Disease
Secondary
to Biliary
Atresia
Group 2
Objectives
To gain knowledge about Biliary Atresia.
To be able to apply a nursing theory in the care for the
patient.
To know the appropriate nursing management for a
patient diagnosed with Chronic Liver Disease Secondary to
Biliary Atresia.
To formulate an appropriate nursing diagnosis for the
patient.
To create a realistic, individualized, and effective Nursing
Care Plan that involves the patient in the process.
 Introducing, Patient A.L., a 5 month old
female with a chief complaint of fever
and jaundice and a discharged
diagnosis of:

 Sepsis Unspecified;
 Ascending Cholangitis;
 Chronic Liver Disease Secondary to
Biliary Atresia;
 S/P Portoenterostomy (KASAI
Procedure)

https://ph.theasianparent.com/baby-with-biliary-atresia
Definition of the
case
Jandonero
  Biliary atresia is a gastrointestinal
disorder in which the biliary
What is Biliarysystem is closed or absent.
 This congenital disorder begins
Atresia? to progress very soon after birth.
In its most common form,
extrahepatic biliary atresia, ducts
outside the liver are affected
first. The cause of biliary atresia
is not known
 The first line of treatment is a Kasai
portoenterostomy (PE) following which
patients may develop cholangitis. The early
effect of early cholangitis on the outcome of
portoenterostomy (PE), namely jaundice
clearance and early native liver survival (NLS).
However, more than half of the children
require liver transplantation (LT) even after
this procedure.
 Cholangitis is the most common complication
that occurs after a PE. It has been postulated
that cholangitis causes rapid progression of
liver injury leading to cirrhosis. Recurrent
attacks of cholangitis have been shown to be a
prognostic marker for hastened liver failure
leading to the early requirement of
transplantation.
 Symptoms of biliary atresia usually
begin to appear between two and
six weeks after birth, and include:

Symptoms
 Jaundice (a yellow appearance of
the skin and whites of the eyes)
that does not improve within
one to two weeks
 Dark yellow or brown urine, due
to excessive bilirubin in the
bloodstream that passes to the
kidneys
 Pale or clay-colored
(acholic) stools, an
indication that very little or
no bile (which gives bowel
movements their normal
color) is reaching the
intestine
 Enlarged liver that feels
harder than normal,
enlarged spleen
 Poor weight gain

Acholic, discolored stool of a baby with biliary atresia.

Wildhaber, Barbara. (2012). Biliary Atresia: 50 Years after the First Kasai. ISRN surgery. 2012. 132089. 10.5402/2012/132089.
 Blood tests that measure liver function
and blood clotting factors, as well as a
CBC (complete blood count)
 Liver biopsy
Diagnosis
 Ultrasound to identify any clear
abnormalities in the liver or in other
organs
 A nuclear scan, which tracks bile flow
from the liver
 Sometimes, exploratory surgery is
performed to examine the liver and bile
ducts
  Some complications of biliary
atresia can be treated temporarily
with medications and special diets;
Treatment
liquid vitamin supplements can be
given orally to help lessen
deficiencies.
 The preferred biliary atresia
treatment is the Kasai procedure
 Liver transplantation is the only cure
for biliary atresia.
Current Jandonero

trends
Although it is relatively rare (occurring in 1 out of
every 10,000 live births), biliary atresia is the
most common liver disease that requires
International
transplantation. On average, there is one case of
biliary atresia out of every 15,000 live births. It
Trends
occurs slightly more often in females than in
males (1.4:1), and affects children of all races. In
the United States, approximately 300 new cases
of biliary atresia in infants are diagnosed each
year.
 In approximately 80% of infants who undergo a biliary atresia Kasai
procedure, bile flow is re-established. Between 25-30% percent of these
infants will have good or complete bile flow after surgery, with normal
levels of bilirubin. The other 50% will have some bile flow. The remaining
20–25% are not helped by the Kasai procedure; they gain little or no bile
flow. In these cases, the infants will require liver transplantation.

Source: UPCM ∣ Children’s Hospital of Pittsburrgh ∣ January, 2019

Link: https://www.chp.edu/our-services/transplant/liver/education/liver-disease-states/biliary-atresia
 The Philippines,
ONE however,
in every 5,000 babieshas
inrecorded
East Asia,the
most rapidtoincrease
according in incidence
the National of biliary
Biotechnology
atresia among children, according to Dr.
Information of Maryland, has been
Subhash Gupta, chairman of the liver and
diagnosed with biliary atresia. The
Local Trendsbiliary sciences department of Max Super
NIDDKD adds: “Doctors treat biliary
Specialty Hospital in Saket, New Delhi,
atresia with a surgery called the Kasai
India.
procedure and, eventually, in most cases,
a“Limited awarenessThanks
liver transplant. amongto theadvances
population,
in
lack of dedicated
treatment, more thantreatment
80% to centers,
90% of as
well aswith
infants financial constraints,
biliary often to
atresia survive impact
access to quality health care,” Gupta said in
adulthood.”
a prepared statement at the EDSA Shangri-
La during a recent visit.
 A liver transplant in
the Philippines
reportedly costs P5
million.
At Max Healthcare in
India, the expenses
could go down to P1.2
million. Source: Business Mirror ∣ Max Healthcare ∣ May 05, 2018
Link:
https://businessmirror.com.ph/2018/05/16/max-healthcare-offers-more-affor
dable-liver-transplant-services-to-filipino-children/
Lopez

Biodata
Biodata
Name: A.L.
Age: 5 months old
Date of Birth: November 28, 2020
Marriage Status: Single
Religion: INC
Gender: Female
Weight: 5.2 Kg
Character High Grade Fever
Onset 3 days prior to admission

Location N/A
Duration 5 days
Severity high grade
Pattern N/A
Associated Factor Jaundice
Character Jaundice
Onset 3 days prior to admission

Location all extermities

Duration N/A
Severity N/A
Pattern N/A
Associated Factor Fever
Physical Exam
Findings

Lopez
Review finding Findings IMPLICATIONS
General Survey Irritable and crying abnormal finding
Vital signs:    
Temperature: 37.7 °C Temperature is elevated
Respiration: 34 breaths per minute
Pulse Rate: 140 beats per minute
Blood Pressure: 116/82

HEENT dry mucous membrane, icteric abnormal finding

sclerae

Chest and Lungs essentially normal normal

CVS tachycardia, regular rhythm tachycardic, abnormal breathing
abdomen slightly distended, soft abnormal finding

GU/EI Normal Normal

Skin Jaundice abnormal finding yellow-
discoloration on all
extremities

Neuro Exam Normal Normal

GCS 15 Normal
History of Present Illness

The patient is a known case of Chronic Liver Disease secondary

to biliary atresia. Status post Hepatic Portoenterostomy last
March 2021. 3 days prior to admission the patient started to
experience high grade fever and was later noted to have deepening
of the jaundice and with episode of acholic stools. She was brought
to a hospital in their town & was treated as a case of viral illness but
the persistence of high grade fever & development of diarrhea
prompted them to transfer to our institution.
 Past medical History

Chronic Liver Disease sec to Biliary Atresia; S/P Biliary

Exploration, Intraoperative cholangiogram, KASAI procedure;
Liver Biopsy, JP Drain
Nursing
Theory
De Guzman
Desengaño
 “Anne Casey” is an English
nurse who developed a nursing
theory known as Casey’s
Model of Nursing. The model
was developed in 1988 while
she was working in pediatric
oncology at the Great Ormond
Street Hospital in London.

Anne Casey
The five aspects of this nursing theory are child, family, health,
environment, and the nurse
 Course in
the ward
Matel
Anatomy and
Physiology
Enriquez
Biliary System Anatomy and
Functions
Biliary System Anatomy and
Functions

The biliary system consists

of the organs and ducts
(bile ducts, gallbladder, and
associated structures) that
are involved in the
production and
transportation of bile.
The transportation of bile follows this
sequence:
 When the liver cells secrete bile, it is collected
by a system of ducts that flow from the liver
through the right and left hepatic ducts.
 These ducts ultimately drain into the common
hepatic duct.
 The common hepatic duct then joins with the
cystic duct from the gallbladder to form the
common bile duct. This runs from the liver to
the duodenum (the first section of the small
intestine).
However, not all bile runs directly
into the duodenum. About 50% of
the bile produced by the liver is first
stored in the gallbladder. This is a
pear-shaped organ located directly
below the liver.
Then, when food is eaten, the
gallbladder contracts and releases
stored bile into the duodenum to
help break down the fats.
 Functions of the biliary
system

The biliary system's main function includes the

following:
 To drain waste products from the liver into the
duodenum
 To help in digestion with the controlled release of
bile
 Bile is the greenish-yellow fluid (consisting of waste
products, cholesterol, and bile salts) that is secreted
by the liver cells to perform 2 primary functions:
 Functions of the biliary
system

The biliary system's main function includes

the following:
 To carry away waste
 To break down fats during digestion
 Bile salt is the actual component that helps
break down and absorb fats. Bile, which is
excreted from the body in the form of feces,
is what gives feces its dark brown color.
Terms

• Duodenum: This is the first of three sections of the small intestine, and
receives food from the stomach and digestive juices from the liver,
gallbladder, and pancreas via the biliary tract. This is the part of the
small intestine that is primarily involved in breaking down food so that
nutrients can later be absorbed in the jejunum (middle section of the
small intestine).
• Liver: A large glandular organ that performs many vital metabolic
functions, such as the digestion of fats to make energy in the body. The
liver cells make bile.
Terms

• Bile duct: This is a small, hollow tube that functions to transport bile.
The biliary system is comprised of a system of these ducts, which flow
from the liver to the gallbladder for storage and then into the small
intestine (duodenum).
• Gallbladder: A pear-shaped organ located in front of the duodenum,
just underneath the liver, the gallbladder's main function is to store bile.
It connects to the cystic duct.
• Pancreas: A large gland located behind the stomach, the pancreas
secretes pancreatic enzymes (such as lipase, which breaks down fats)
into the biliary system via the pancreatic duct.
Pathophysiology
De Ocampo
Guleng
 Nursing
Care Plan
Hyperthermia
Risk for complications of hyperthermia
related to inflammatory process

Imbalanced Nutrition: Less Than Body

Requirements related to impaired
absorption of fat and nutrients
 Laboratory
Results
Deña
Espina
Drug Dinglasan
Janoras

study
Discharge plan

Gumapac
Thankyou
for
staying
with us!