Mater Dei College

Cabulijan, Tubigon, Bohol

COLLEGE OF NURSING

A Case
Presentation on
IntestinalGroup 4
Lymphangiectasia
 Group 4
LYLE MASCARIṄAS
JOHN KENT ZAMORA
ALIANA MIKE EMIE ANUNCIADO
CHRISTINE MAE BOṄON
CHRISTINE NIṄA CRUDA
JANNINE DIEZ
MERIAM GUCOR
KEREN HAPPUCH INFERIDO
RODELYN MAHUSAY
LORY RESTIFICAR
VIA RULIDA
JOSELLE MAE TORREGOSA
Contents of Case Presentation
I. PATIENT’S PROFILE

II. GORDON’S 11 FUNCTIONAL HEALTH PATTERN

III. DEFINITION

IV. DIGESTIVE SYSTEM

ANATOMY
PHYSIOLOGY

V. DRUG STUDY

VI. DIAGNOSTIC EXAM

VII. NURSING CARE PLAN (NCP)

 I. Patient’s
Profile

Name: Mary Hazel Bartolome

Age: 40 years old
Birthdate: January 16, 1983
Sex: Female
Status: Married
Address: Carmen, Bohol
Religion: Roman Catholic
Nationality: Filipino
II.

GORDON’S
11

FUNCTIONAL
HEALTH PATTERN
 HEALTH
PERCEPTION AND
HEALTH
MANAGEMENT
 NUTRITION AND
METABOLISM PATTERN
ELIMINATION
PATTERN
 ACTIVITY AND
EXERCISE PATTERN
 COGNITION AND
PERCEPTION PATTERN
SLEEP AND REST
PATTERN
SELF PERCEPTION AND
SELF-CONCEPT
PATTERN
 ROLE AND
RELATIONSHIP
PATTERN
SEXUALITY AND
REPRODUCTION
PATTERN
COPING AND STRESS
TOLERANCE
PATTERN
VALUES AND BELIEF
PATTERN
III. DEFINITION

Intestinal lymphangiectasia
is a rare disease in which the
lymph vessels supplying the
lining of the small intestine
are blocked, resulting in 
malabsorption.
ANATOMY & PHYSIOLOGY
 THE LYMPHATIC SYSTEM

The lymphatic system is a network of tissues, vessels and organs that work together
to move a colorless, watery fluid called lymph back into your circulatory system
(your bloodstream).

Some 20 liters of plasma flow through your body’s arteries and smaller arteriole
blood vessels and capillaries every day. After delivering nutrients to the body’s cells
and tissues and receiving their waste products, about 17 liters are returned to the
circulation by way of veins. The remaining three liters seep through the capillaries
and into your body’s tissues. The lymphatic system collects this excess fluid, now
called lymph, from tissues in your body and moves it along until it's ultimately
returned to your bloodstream.
 FUNCTIONS OF THE LYMPHATIC
SYSTEM

•Maintains fluid levels in your body

•Absorbs fats from the digestive tract
•Protects your body against foreign invaders
•Transports and removes waste products and abnormal cells from the lymph.
 PARTS OF THE LYMPHATIC SYSTEM

•Lymph: Lymph, also called lymphatic fluid, is a collection of the extra fluid that drains from
cells and tissues (that is not reabsorbed into the capillaries) plus other substances.

•Lymph nodes: Lymph nodes are bean-shaped glands that monitor and cleanse the lymph as
it filters through them. The nodes filter out the damaged cells and cancer cells.
•Lymphatic vessels: Lymphatic vessels are the network of capillaries (microvessels) and
a large network of tubes located throughout your body that transport lymph away from tissues.
Lymphatic vessels collect and filter lymph (at the nodes) as it continues to move toward larger
vessels called collecting ducts.

•Collecting ducts: Lymphatic vessels empty the lymph into the right lymphatic duct and
left lymphatic duct (also called the thoracic duct).

•Spleen: This largest lymphatic organ is located on your left side under your ribs and above
your stomach. The spleen filters and stores blood and produces white blood cells that fight
infection or disease.
•Thymus: This organ is located in the upper chest beneath the breast bone. It matures a
specific type of white blood cell that fights off foreign organisms.

•Tonsils and adenoid: These lymphoid organs trap pathogens from the food you eat and
the air you breathe. They are your body’s first line of defense against foreign invaders.

•Bone marrow: This is the soft, spongy tissue in the center of certain bones, such as the hip
bone and breastbone. White blood cells, red blood cells, and platelets are made in the bone
marrow.

•Peyer’s patches: These are small masses of lymphatic tissue in the mucous membrane that
lines your small intestine. These lymphoid cells monitor and destroy bacteria in the intestines.

•Appendix: Your appendix contains lymphoid tissue that can destroy bacteria before it
breaches the intestine wall during absorption. Scientists also believe the appendix plays a role
in housing “good bacteria” and repopulating our gut with good bacteria after an infection has
cleared.
 PATHOPHYSIOLOGY

Clinical manifestations of protein-losing enteropathy (PLE) arise when the rate of protein
loss into the gut exceeds the rate of protein synthesis by liver.

PLE can be divided into three categories according to its mechanism:

• mucosal injury,
• increased permeability,
• and lymphatic obstruction.

All factors contributing to these lymphatic changes lead to dilatation, rupture of the
lymphatic vessels, and opening into intestinal lumen. Intestinal lymphangiectasia is a rare
disease causing PLE. Intestinal lymphangiectasia is classified into primary or secondary
types.
Primary intestinal lymphangiectasia, also called idiopathic lymphangiectasia,
occurs congenitally in the absence of causative factors.

Secondary intestinal lymphangiectasia is induced by risk factors including heart

surgery, chemotherapy, infection, or toxic materials known to trigger lymphatic
changes  
SIGNS AND
SYMPTOMS
Other symptoms include:
- Hypoproteinemia (low protein levels
in the blood)

- Lyphopenia (low lymphocyte counts

in the blood)
 DRUG STUDY
Drug Name
Sirolimus
2mg/day
Mammalian Target of Rapamycin (mTOR) Kinase Inhibitors
Immunosuppressants

Pregnancy category C
Mechanisms of Action:

Sirolimus inhibits T-lymphocyte activation and proliferation that occurs in response to antigenic and
cytokine (Interleukin (IL-2, IL-4, and IL-15) stimulation by a mechanism that is distinct from that of other
immunosuppressants. Sirolimus also inhibits antibody production.
Sirolimus forms an immunosuppressive complex with intracellular protein, FKBP12. This complex
blocks the activation of the cell-cycle-specific kinase, TOR. The downstream events that follow the
inactivation of TOR result in the blockage of cell-cycle progression at the juncture of G1 and S phase.
Indications
-Sirolimus is has been used to treat lymphangiectasia.
-Treatment of patients with primary intestinal lymphangiectasia involves control of symptoms with the
use of dietary, pharmaceutical, and behavioral modifications.
-Sirolimus is used together with other medicines to prevent the body from rejecting a transplanted
kidney.
-Patients at low- to moderate-immunologic risk, it is recommended that Rapamune be used initially in a
regimen with cyclosporine and corticosteroids; cyclosporine should be withdrawn 2 to 4 months after
transplantation.

Contraindications:

-Rapamune is contraindicated in patients with a hypersensitivity to Rapamune;Lung transplant use,

Liver transplant use.
-Immunosuppressants, such as sirolimus, may affect the bodies' response to vaccines, making
vaccination less effective. 
-Impaired or delayed wound healing, lymphocele, and wound dehiscence have been reported in
sirolimus recipients.
Adverse Reactions/Side Effects:

The most common (≥ 30%) adverse reactions observed with Rapamune in clinical studies are:
peripheral edema, hypertriglyceridemia, hypertension, hypercholesterolemia, creatinine increased,
constipation, abdominal pain, diarrhea, headache, fever, urinary tract infection, anemia, nausea,
arthralgia, pain, and thrombocytopenia
 Nursing Responsibilities:
•-All patients receiving sirolimus should be monitored for sirolimus-induced hypercholesterolemia and
hypertriglyceridemia.
-Check blood sugar level.
-Monitor vital signs.
-Avoid grapefruit juice within 2 h of taking sirolimus.
-Limit exposure to sunlight (UV exposure).
-Avoid injury.
-Talk with the doctor before getting any vaccine.
-Report any signs of high blood sugar, confusions, sleepy, more thirst, more hungry, fast breathing
-Use or add barrier contraceptive before, during, and for 12 wk after discontinuing therapy.
-Do not breast feed while taking this drug
 DRUG STUDY
Drug Name
Generic Name: Octreotide Acetate
Brand Name: Sandostatin
Dosage: 1-10 mcg
Route: Subcutaneous
Frequency: TID
Classification: Antidiarrheals

Mechanisms of Action:

Octreotide binds to somatostatin receptors coupled to phospholipase C through G proteins and leads to
smooth muscle contraction in the blood vessels. Downstream effects that stimulate phospholipase C,
the production of 1, 4,5-inositol triphosphate, and action on the L-type calcium channels lead to the
inhibition of growth hormone, treating the various growth-hormone and metabolic effects of acromegaly.
Octreotide’s suppression of luteinizing hormone (LH), reduction in splanchnic blood flow, and inhibition
of serotonin, gastrin, vasoactive intestinal peptide, secretin, motilin, and pancreatic polypeptide provide
relief for the gastrointestinal and flushing symptoms of carcinoid and/or VIPoma tumors.
Indications:
Octreotide by injection is used for the treatment of acromegaly and the reduction of flushing and
diarrhea symptoms related to carcinoid tumors and/or vasoactive intestinal peptide (VIPoma) tumors.
The delayed-release oral formulation is used for the long-term treatment of acromegaly in patients who
tolerate and respond adequately to injectable octreotide and lanreotide.

Contraindications:

Hypersensitivity to drug or its components

Adverse Reactions/Side Effects:

Central nervous system

• Dizziness
• Drowsiness
• Fatigue
• Headache
• Weakness

Ear, Eye, Nose, Throat

• Visual disturbances

Cardiovascular
• Bradycardia
• Edema
• Orthostatic hypotension
• Palpitations
 Nursing Responsibilities
• Assess frequency and consistency of stools and bowel sounds throughout therapy.
• Monitor pulse and BP prior to and periodically during therapy.
• Assess patient’s fluid and electrolyte balance and skin turgor for dehydration.
• Monitor diabetic patients for signs of hypoglycemia. May require reduction in requirements for insulin
and sulfonylureas and treatment with diazoxide.
• Assess for gallbladder disease; assess for pain and monitor ultrasound examinations of gallbladder
and bile ducts prior to and periodically during prolonged therapy.
Patient/Family Teaching
• May cause dizziness, drowsiness, or visual disturbances. Caution patient to avoid driving or other
activities requiring alertness until response to medication is known.
• Advise patient to change positions slowly to minimize orthostatic hypotension.
• As appropriate, review all other significant and life-threatening adverse reactions and interactions,
especially those related to the drugs, tests, and foods.
• Home Care Issues: Instruct patients administering octreotide at home on correct technique for
injection, storage, and disposal of equipment.
- Instruct patient to administer octreotide exactly as directed. If a dose is missed, administer as soon
as possible, then return to regular schedule. Do not double doses.
 DRUG STUDY
Drug Name
Generic Name: Everolimus

Brand Name: Afinitor, Zortress

Dosage/ Route / Frequency : Everolimus is taken by mouth, in pill form. The medication comes in
2.5mg, 5 mg, and 10 mg oral tablets. Take your dose around the same time every day.

Classification: Everolimus is in a class of medications called kinase inhibitors.

Mechanisms of Action:

It blocks growth-driven transduction signals in the T-cell response to alloantigen and thus acts at a later
stage than the calcineurin inhibitors ciclosporin and tacrolimus. Everolimus also inhibits the expression
of hypoxia-inducible factor, leading to a decrease in the expression of vascular endothelial growth
factor. The result of everolimus inhibition of mTOR is a reduction in cell proliferation, angiogenesis, and
glucose uptake.
Indications:

Indicated for the patients who are refractory to dietary therapy and also adjunctive treatment of adult
and pediatric patients aged 2 years and older with tuberous sclerosis complex (TSC)-associated partial-
onset seizures.

Contraindications:

Hypersensitivity to everolimus and other rapamycin derivatives. Lactation. Concomitant use with live
vaccines.
Adverse Reactions/Side Effects:

Significant: Renal arterial and venous graft thrombosis, hepatic artery thrombosis, bone marrow
suppression, malignancy (e.g. lymphoma, skin cancer). Gastrointestinal disorders: Nausea, diarrhoea,
vomiting, dry mouth, constipation, abdominal or oral pain, dyspepsia, dysphagia, dysgeusia.
Nursing Responsibilities

• Instruct patient to guard against infection (frequent hand washing, etc.), and to avoid crowds and
contact with persons with contagious diseases.
• Consider wearing gloves or pour the pills directly from their container into the cap, a small cup, or
directly into your hand. Avoid touching the pills.
• Always wash their hands before and after giving you the medication.
• Monitor CBCs, renal function, lipids, blood glucose, and for pneumonitis and infections.
VI. DIAGNOSTIC
FLOW
 To rule out initial Once the intestinal Confirmative tool for Diagnosis
clinical symptoms: lymphangiectasia is
suspected, it is
recommended to distinguish
whether the disease is of
primary or secondary origin.
Tests involves:

1. Urinalysis 1. Enodscopy 1. Endoscopy 

2. Blood Tests 2. Echocardiography and 2. Esophagogastroduodenography 
3. Stool Analysis abdominal imaging  3. Capsule endoscopy
VII.
NURSING
CARE PLAN
 Mater Dei College
Cabulijan, Tubigon, Bohol
COLLEGE OF NURSING
NURSING CARE PLAN

Name: Mary Hzel Bartolome Gender: Female

Age: 40 years old Chief Complaint: abdominal pain
Civil status: Married Diagnosis: intestinal lymphagiectasia
 Resources:
1. Kwon, Y., & Kim, M. (2021). The Update of Treatment for Primary Intestinal
Lymphangiectasia. Pediatric Gastroenterology, Hepatology & Nutrition, 24(5), 413.
https://doi.org/10.5223/pghn.2021.24.5.413
2. Huber R et al. Primary intestinal lymphangiectasia. Primary intestinal
lymphangiectasia in an adult patient: A case report and review of literature
3. Kwon, Y., & Kim, M. (2021b). The Update of Treatment for Primary Intestinal
Lymphangiectasia. Pediatric Gastroenterology, Hepatology & Nutrition, 24(5),
413. https://doi.org/10.5223/pghn.2021.24.5.413
Thank
You!