Gastro and

endocrine
disorders
Jesslei vemm Piquero, rn
 Let’s begin!

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Cleft lip / palate Esophageal Pyloric stenosis Hirschsprung's intussusception
fistula / atresia disease

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Cleft lip/ palate
Problems:
1.Feeding
2.Speech
3.Dental malformation
4.Upper respiratory tract
infection

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Methods
of feeding
Enlarge the nipple
Stimulate suck
reflex
Swallow
Rest
management
Cleft lip
• Cheiloplasty
• Rule of 10
- weeks
- pounds
- apply logan’s bow
Post-op care
- Rinsing : saline
- Burping : every 1-2 hrs
- Place on right side :
prevent aspiration
- Clear h2o
management
Cleft palate
• Palatoplasty
• Done 12-18 months to prevent
malformation
• Prone
• Apply elbow restraints
• Post- op:
- No spoon feeding
- No tongue depressor
- No oral thermometers
- No oral suctioning
- No pacifiers
- Apply elbow restraints for six to
eight weeks. remove q 2 hours with
supervision. Remove one at a time
Esophageal
fistula/ atresia
-failure of esophagus to develop as a continuous process

Fistula : abnormal opening

Atresia: blind pouch

Signs and symptoms:

- Choking - gastric reflux
- Coughing - abdominal distention
- Cyanosis - signs of respiratory distress
- Excessive drooling

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Management:
surgically ligate the
fistula and anastomose
the esophageal
segments as soon as
possible after birth.
Pyloric stenosis
• Narrowed / hypertrophy of muscles around pyloric
sphincter
• Diagnostics : barium swallow
- upon xray: “sting sign”
• Pyloromyotomy
• Post- op:
• Feeding :
- upright position
- Minimal feeding
- burp

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Evident signs:

- Dehydraytion
- Metabolic alkalosis
- Failure to thrive
Hirschsprung's
disease
- Aganglionic megacolon
- Absence of peristalsis
- Signs and symptoms:
• Failure to pass meconium
• Abdominal distention
• Ribbon-like stools
• Womits with bile and feces

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Hirschsprung's
disease
• management:
Surgery : resection
• Nursing responsibilities:
• - diet: low residue
• - stool softener
• - enema : normal saline

• pre- op
• - semi- fowler’s position
• Isotonic saline enema
• No rectal temperature taking
intussusception
Telescoping of the small intestine
Signs and symptoms:
- Colicky abdominal pain @ right upper
quadrant
- Vomiting with bile no fecalith
- Sausage – shape mass
- Currant jelly- like stools < stools with
mucus and blood >

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intussusception
Management:
• Hydrostatic reduction < barium
enema : give enema>
• intussusception is not usually
immediately life-threatening. It
can be treated with either a
water-soluble contrast enema or
an air-contrast enema, which
both confirms the diagnosis of an
intussusception, and in most
cases successfully reduces it.
 “ More than 8 million babies
worldwide are born with a
serious birth defect each year

”
Richard Branson

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Dumping
syndrome
Lie down after eating
Should be high fat
Decrease fluid intake

d
Diarrhea
Diaphoresis
dizziness

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Billroth 1 Gastrododenosto
my
billroth 2 gastrojejunostom
y
Produce hcl < hydrochloric
acid >
Rapid gastric emptying. A
condition in which food moves
too quickly from your
stomach to your duodenum.

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 Early dumping syndrome is likely to resolve on its
own within three months. In the meantime, there's a
good chance that dietary changes will ease your
symptoms. If not, your health care provider may
recommend medications or surgery.
Medications
If changes to your diet don't improve
symptoms, your health care provider may prescribe
octreotide (Sandostatin). This anti-diarrheal drug,
administered by injection under your skin, can slow
the emptying of food into the intestine. Possible side
effects include nausea, diarrhea and fatty stools
(steatorrhea).
Talk with your doctor about the proper way to self-
administer the drug.
Surgery
If conservative approaches don't help,
surgery may be recommended. Depending on your
situation, surgical procedures to treat dumping
syndrome may include reconstructing the pylorus or
surgery to reverse gastric bypass surgery.

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 Eat smaller meals. Try eating 5 or 6 small meals a day rather than
three larger ones.
Lie down after meals. Try lying down for 30 minutes after you eat.
Drink most of your fluids between meals. At first, don't drink
anything for 30 to 60 minutes before and after meals.
Drink 6 to 8 cups (1.4 to 1.9 liters) of fluids a day. At first, limit fluid
with meals to 1/2 cup (118 milliliters). Increase fluid with meals as
you tolerate it.
Change your diet. Eat more protein, including meat, poultry, creamy
peanut butter and fish, and complex carbohydrates such as oatmeal
and other whole-grain foods high in fiber. Limit high-sugar foods, such
as candy, table sugar, syrup, sodas and juices.
The natural sugar in dairy products (lactose) might worsen your
symptoms. Try small amounts at first, or eliminate them if you think
they're causing problems. You might want to see a registered
dietitian for more advice about what to eat.
Increase fiber intake. Guar gum and pectin in food or supplements
can delay the absorption of carbohydrates in the small intestine.

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Gastro esophageal
reflux
- Backflow of gastric contents
- Relaxation of cardiac sphincter
• Signs and symptoms
- Passive regurgitation
- Poor weight gain
- Often hungry after vomiting episode
Management:
- After feeding, place your baby into
fowler’s posotian with the upper body is
elevated
- Add rice cereal to <thicken feeding>
- Burp your baby several times during bottle
feeding or breastfeeding . Your baby may
reflux more after when burping with a full
stomach

• Medical management
- Decreases esophageal pressure by relaxing
the pyloric and duodenal segments
 Part 2

Addison’s disease Cystic fibrosis Diabetes

mellitus
 Addison’s disease
Addison’s disease develops when the adrenal glands can no longer
adequately supply the body with cortisol and aldosterone. It affects men
and women equally and occurs in all age groups, but is most common
between the ages of 30 and 50.

Addison’s disease is caused by a failure of the adrenal glands. The adrenal glands are one
of several organs that comprise the endocrine system. The glands are situated atop each
kidney and are responsible for producing a variety of hormones, including cortisol,
aldosterone, and adrenaline.

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 Cortisol, often referred to as the “stress hormone,” rises when the "fight or
flight" instinct is triggered during a crisis. It also plays a central role in the
regulation of blood pressure, blood sugar, protein, fat, and carbohydrate
metabolism, bone mineralization and density, inflammation, sleep, and energy
levels. It is produced in a part of the adrenal glands known as the zona
fasciculata.

Aldosterone helps regulate the balance of sodium and potassium in the body
and, by doing so, influences blood pressure, blood volume, and the retention of
water in cells. It is produced in a different part of the gland known as the zona
glomerulosa.

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 The primary symptoms of Addison’s disease tend to be episodic and
non-specific. Common symptoms include:
•Extreme fatigue
•Abdominal pain
•Decreased appetite
•Salt craving
•Lightheadedness
•Dizziness when rising (postural hypotension)
•Blurry vision
•Shakiness or tremors
•Heart palpitations
•Nausea or vomiting
•Anxiety or irritability
•Depression
•Pins-and-needles sensations (peripheral neuropathy)
•Reduced sex drive (low libido)
•Hair loss
•Muscle or joint pain
•Weight loss
•Darkening of the skin (hyperpigmentation), particularly in areas
where there are skin creases or scars, as well as on the palms, nipples,
and inside the cheek
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 Adrenal crisis
Adrenal crisis, also known as an Addisonian crisis, occurs when cortisol levels
have dropped so rapidly that it causes serious and potentially life-threatening
symptoms.

If not treated aggressively with intravenous fluids and steroids, death may
ensue, most often as a result of hypotensive shock or respiratory failure
. According to research, an adrenal crisis results in death in 1 of every 16 cases,
most often due to delayed or inadequate treatment

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 Severe vomiting and/or diarrhea leading to dehydration
Pain in the lower back, legs, or abdomen
Fainting (syncope)
Muscle spasms
Confusion and disorientation
Extreme thirst and the inability to urinate
Abnormal heartbeats (arrhythmia)
Intermittent muscle paralysis

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Cystic
fibrosis
is a genetic condition that affects a
protein in the body. People who
have cystic fibrosis have a faulty
protein that affects the body's
cells, tissues, and the glands that
make mucus and sweat
 Cystic Fibrosis usually affects the exocrine
glands of the human body. The exocrine
glands produce secretions such as mucus,
sweat, tears and digestive juices. The
secretions are normally thin and slippery;
they are essential for normal functioning of
the body. In children with cystic fibrosis the
mucus is thick and sticky, which creates
problems with normal body functioning.
What is the genetic problem in Cystic Fibrosis?
Humans have a gene called as cystic fibrosis transmembrane conductance regulator or CFTR gene on the
chromosome 7. The CFTR gene is responsible for making a protein called the cystic fibrosis
transmembrane conductance regulator protein. This protein functions as a channel across the membrane
of cells that produce mucus, sweat, saliva, tears, and digestive enzymes.
In children with cystic fibrosis there is a defect or mutation in the CFTR gene. When the protein is
dysfunctional the movement of the chloride ions into and out the cells is impaired. The chloride ions are
required for the movement of water in tissues. In children with cystic fibrosis without the chloride ions
and water the mucus in various organs becomes thick and sticky.

What happens to the lungs in Cystic Fibrosis?

In children with cystic fibrosis, the thick and sticky mucus plug-up the small airways of the lungs and
traps germs, like bacteria, leading to repeated infections. These repeated infections in children with
cystic fibrosis leads to inflamed and dilated airways, a condition called as bronchiectasis. This progresses
over time and lead to chronic lung damage, respiratory failure, and other complications.
Children with cystic fibrosis have a chronic wet or a productive cough which does not go with usual
medicines, recurrent episodes of chest infection, chest congestion and pneumonias.

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What happens to the digestive system in children with Cystic Fibrosis?
In children with cystic fibrosis, the thick and sticky mucus blocks the small ducts in
the pancreas thus preventing the pancreatic enzymes from reaching the small
intestines. This would lead to incomplete digestion of food and poor absorption of
nutrients. Hence these children would have large, bulky and frothy stools. In addition
children with cystic fibrosis have a poor weight gain.

What other organs are affected in children with Cystic Fibrosis?

The other parts of the body affected by cystic fibrosis are the sweat glands, digestive
system and reproductive system. In children with cystic fibrosis, excessive salt is lost
in the sweat, leading to problems of dehydration especially in hot weather. In patients
with cystic fibrosis there can be involvement of the reproductive system leading to
fertility problems.

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Keeping airways clear
- You can help to keep your airways clear if you
have cystic fibrosis in a number of ways:
* You can learn special ways of coughing and
breathing.
* You can use devices that fit into your mouth
or therapy vests that rely on vibrations to
loosen mucus.
* You can learn something called chest physical
therapy, also known as postural drainage and
percussion to loosen mucus. With this method,
you move into certain positions so that your
lungs can drain. Another person claps their
hand on your chest and/or your back to help
loosen the mucus. You might combine this with
coughing.
 What is the medical management of cystic
fibrosis in child?
The main goals of treatment for someone with
cystic fibrosis are to prevent and treat infections, keep
the airways and lungs as clear as possible, and maintain
adequate calories and nutrition. To accomplish these
objectives, treatments for cystic fibrosis in children may
include: Childhood immunizations.

What are 3 treatments for cystic fibrosis?

Antibiotics to treat and prevent lung
infections. Anti-inflammatory medications to lessen
swelling in the airways in your lungs. Mucus-thinning
drugs, such as hypertonic saline, to help you cough up
the mucus, which can improve lung function

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Diabetes
mellitus
Diabetes mellitus (DM) is a
disease of inadequate control of
blood levels of glucose. It has
many subclassifications,
including type 1, type 2,
maturity-onset diabetes of the
young (MODY), gestational
diabetes, neonatal diabetes, and
steroid-induced diabetes.
Type 1
diabetes mellitus
Type 1 diabetes in children is a
condition in which your child's body no
longer produces an important hormone
(insulin). Your child needs insulin to
survive, so the missing insulin needs to
be replaced with injections or with an
insulin pump. Type 1 diabetes in
children used to be known as juvenile
diabetes or insulin-dependent diabetes.
Signs and Symptoms
The signs and symptoms of type
1 diabetes in children usually
develop quickly, and may
include:
•Increased thirst
•Frequent urination, possibly
bed-wetting in a toilet-trained
child
•Extreme hunger
•Unintentional weight loss
•Fatigue
•Irritability or behavior changes
•Fruity-smelling breath
 Risk factors
Type 1 diabetes most often
occurs in children but can
occur at any age. Risk factors
for type 1 diabetes in children
include:
•Family history. Anyone with a
parent or siblings with type 1
diabetes has a slightly
increased risk of developing the
condition.
•Genetics. Certain genes
indicate an increased risk of
type 1 diabetes.
•Race. In the United States,
type 1 diabetes is more
common among white children
of non-Hispanic descent than
among children of other races.
•Certain viruses. Exposure to
management
Individuals with T1D require life-
long insulin replacement with
multiple daily insulin injections
daily, insulin pump therapy, or the
use of an automated insulin delivery
system. Without insulin, diabetic
ketoacidosis (DKA) develops and is
life-threatening.
Cellular
aberrations
Chromosomal Aberrations
What Are They, Causes, and More
Chromosomal aberrations, or
abnormalities, are changes to the
structure or number of chromosomes,
which are strands of condensed genetic
material. Humans typically have 23 pairs
of chromosomes, of which 22 pairs are
autosomal, numbered 1 through 22. The
last pair of chromosomes are sex
chromosomes, which determine an
individual’s sex assignment. At birth, most
people with XY sex chromosomes are
assigned male, and most individuals with
XX are assigned female. In general, each
parent contributes one set of
chromosomes to their offspring, which
collectively make up the 23 pairs of
chromosomes. A change to any of the
chromosomes, in number or structure,
creates a chromosomal aberration and
may cause medical disorders
What causes chromosomal aberrations?

Chromosomal aberrations are most often

caused by errors during cell division. Cell
division in humans occurs via mitosis or, only
in sex chromosomes, meiosis. In mitosis,
cells duplicate their chromosomes and
produce daughter cells with an identical
number of chromosomes as the original cell.
In other words, a cell with 46 chromosomes
will produce two cells, each with 46 identical
chromosomes. Meanwhile, cell division by
meiosis involves two rounds of cell division
that allow for the recombination of genetic
material, resulting in four sex cells with only
half of the number of chromosomes. For
example, a cell with 46 chromosomes
undergoing meiosis will produce four unique
daughter cells, each with 23 chromosomes.
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Carcinoma: cancer that begins in the skin or in
tissues
that line or cover internal organs
Sarcoma: begins in bone, cartilage, fat, muscle,
blood
vessels, or connective/supportive tissues.
Leukemia: begins in blood forming tissue, such as
bone marrow, and causes large numbers of
abnormal
blood cells to be produced and enter the blood.
Lymphoma and myeloma: begins in cells of the
immune system, bone marrow
CNS cancers: begin in tissues of the brain and
spinal
cord;44ex: nerve cells: neuroblastoma Presentation title 20XX
Trisomy 21 Trisomy 18 Trisomy 13
Down syndrome Edward’s syndnrome Patau’s syndrome

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What is down
syndrome?
is a condition in which a person
has an extra chromosome.
Chromosomes are small
“packages” of genes in the body.
They determine how a baby’s body
forms and functions as it grows
during pregnancy and after birth.
Typically, a baby is born with 46
chromosomes. Babies with Down
syndrome have an extra copy of
one of these chromosomes,
chromosome 21
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Some common physical features of Down
syndrome include:
•A flattened face, especially the bridge of
the nose
•Almond-shaped eyes that slant up
•A short neck
•Small ears
•A tongue that tends to stick out of the
mouth
•Tiny white spots on the iris (colored part)
of the eye
•Small hands and feet
•A single line across the palm of the hand
(palmar crease)
•Small pinky fingers that sometimes curve
toward the thumb
•Poor muscle tone or loose joints
•Shorter in height as children and adults

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What is
Edward’s
syndrome?
Edwards syndrome is a genetic
condition in babies that causes
severe disability. It is caused by
an extra copy of chromosome 18
and babies born with the
condition usually do not survive
for much longer than a week.
 Children with Edwards syndrome have 3
copies of part or all of chromosome 18,
instead of the usual 2 copies. It is also
called Trisomy 18.
This can be caused by a mistake in the
formation of the egg or sperm, or the
problem can arise while the baby is
developing in the womb.
Babies with Edwards syndrome are
either miscarried, stillborn or born with
severe physical abnormalities. It is very
rare for a baby with Edwards syndrome
to survive their first year of life, and most
die within a week of birth.
Edwards syndrome is much more
common in girls.

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What is patau’s
syndrome?
Patau's syndrome is a serious
rare genetic disorder caused by having an
additional copy of chromosome 13 in some
or all of the body's cells. It's also called
trisomy 13.

This severely disrupts normal

development and, in many cases, results
in miscarriage, stillbirth or the baby dying
shortly after birth.
Babies with Patau's syndrome grow slowly
in the womb and have a low birthweight,
along with a number of other serious
medical problems.
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Musculoskeletal and trauma

Congenital hip Club scoliosis

dysplasia
foot
Congenital hip
dysplasia
is a condition where the "ball
and socket" joint of the hip
does not properly form in
babies and young children.
It's sometimes called
congenital dislocation of the
hip, or hip dysplasia. The hip
joint attaches the thigh bone
(femur) to the pelvis.
Pavlik harness
Babies diagnosed with DDH early in life are
usually treated with a fabric splint called a
Pavlik harness.
This secures both of your baby's hips in a
stable position and allows them to develop
normally.

The harness needs to be worn constantly for

6 to 12 weeks and should not be removed by
anyone except a health professional.

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Hip spica cast
is a type of plaster cast that covers one or both legs
from the ankles up to the belly button. An area
around the groin is cut out for toileting. This type of
cast is used to prevent movement for hip dysplasia,
after hip surgery or for fractures of the femur (thigh
bone), allowing the area to heal.

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 Goal of management:
*flexed and abducted : external rotation

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Club foot
(also called talipes) is where a baby
is born with a foot or feet that turn in
and under. Early treatment should
correct it. In club foot, 1 foot or both
feet point down and inwards with the
sole of the foot facing backwards.

Is plantar flexed with an

inverted heel and abducted
forefoot
Club foot happens because the
Achilles tendon (the large tendon at
the back of the ankle) is too short.
Club foot can affect 1 or both feet.
It's not painful for babies, but it can
become painful and make it difficult
to walk if it's not treated.
 Treatment for club foot usually starts within
1 to 2 weeks of your baby being born.
The main treatment, called the Ponseti
method, involves gently manipulating and
stretching your baby's foot into a better
position. It's then put into a cast.
This is repeated every week for about 5 to 8
weeks.
After the last cast comes off, most babies
need a minor operation to loosen the
Achilles tendon at the back of their ankle.
This is done using a local anaesthetic. It
helps to release their foot into a more
natural position.

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scoliosis
is a sideways curvature of the spine
that most often is diagnosed in
adolescents. While scoliosis can
occur in people with conditions such
as cerebral palsy and muscular
dystrophy, the cause of most
childhood scoliosis is unknown. Most
cases of scoliosis are mild, but some
curves worsen as children grow.v
 Most cases of scoliosis are mild,
but some curves worsen as
children grow. Severe scoliosis can
be disabling. An especially severe
spinal curve can reduce the
amount of space within the chest,
making it difficult for the lungs to
function properly.. - lateral curvature
- Spiral rotation
- S- shaped appearance
- Unequal leg length
- C-shaped

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Halo
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Care:

-extensive
radiographs
-flat on bed when
lying
-log-rolling to the
side
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