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    Microscopicpolyangiitis (MPA) is a

    systemic pauci-immune

    necrotizing vasculitisthat affects mainly

    small vessels.

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    Facts:

    MPA is a rare type of

    autoimmune disease.

    Incidence rate is

    approximately 1:100,000 with

    the slight predominance inmen.

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    The average age onset is

    50 years, and is very rare

    in children.

    The most common

    antineutrophil cytoplasmic

    autoantibodies (ANCA)-associated small vessel

    vasculitis

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    What is ANCA?

    Antineutrophil cytoplasmic

    autoantibodies are antibodies

    directed against the bodys

    neutrophils and monocytes.

    Two types of these

    autoantibodies are identified

    that are present in patients

    with this disease.

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    P-ANCA(perinuclear staining)

    -70% of patients with

    MPA.

    C-ANCA

    (cytoplasmic

    staining) -30% of the

    cases.

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    Clients name: D.B.

    14-year old, Female

    Filipino, RomanCatholic

    3rd year High Schoolstudent

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    She had an active high

    school social life withno history of sexual.

    activity or drug abuse.

    She was born and is

    residing at Las PinasCity with her mother

    and her sister.

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    Patient DB had skin

    asthma when she wasfive years old and was

    ruled out before she

    reached twelve.

    No other past illnesses

    were cited.

    Immunization status:

    complete immunization

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    General Appearance

    Upon assessment, the patient isweak looking but with no signs

    of cardio-respiratory distress.

    Admission Vital Signs

    BP: 130/80 mmHg PR: 80 bpm

    RR: 20cpm Temp: 36.5 C

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    Withedematous

    facial feature

    and

    periorbital

    edema.

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    With presence ofdandruff and

    falling of hair

    uponobservation.

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    The patient

    reported episodeof red spots

    before her eyes

    prior to her

    admission to San

    Juan De Dios

    Hospital.

    With pale

    conjunctiva.

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    Lips appeared

    dry and scaly.

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    Skin

    Dry and slightly

    pale.

    With presence

    of ecchymosis in

    both arms.

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    NAILS

    With visible

    splinter

    hemorrhage

    .

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    NECK

    With right

    Intrajugularcatheter

    access.

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    Cardiovascular

    Hypertensive.

    Noted of taking anti-

    hypertensive drugs.

    Upon assessment,

    the patients blood

    pressure: 130/80

    mmHg.

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    With

    ecchymosis onboth arms.

    Visible striae

    noted on

    upper andlower limbs.

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    (+) bipedal edema

    upon assessment.

    Upon palpation there

    is no complain of painand discomfort.

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    Breast/Axilla

    with striae in both side

    of the axilla.

    Genitals

    Appeared to be

    edematous.

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    HEMATOLOGY09-March-2011

    Results

    12-March-2011

    Results

    13-March-2011

    Results

    PT patient

    PT control

    PT INR

    PT activity

    APTT

    APTT control

    10 - 14 sec.

    11 - 14 sec.

    0 - 1.4

    70 - 130%

    25 - 39

    25 - 39

    14.1 sec.

    12.5 sec.

    1.16

    83%

    High 54.6

    34.6

    13.3 sec.

    12.5 sec.

    1.08 sec.

    91%

    34.6

    10.6 sec.

    12.5 sec.

    0.81 sec.

    130%

    27.3

    34.6

    HEMATOLOGY Normal Range

    09-March-2011

    Results

    12-March-2011

    Results

    13-March-2011

    Results

    PT patient 10 - 14 sec. 14.1 sec. 13.3 sec. 10.6 sec.

    PT control 11 - 14 sec. 12.5 sec. 12.5 sec. 12.5 sec.

    PT INR 0 - 1.4 sec. 1.16 sec. 1.08 sec. 0.81 sec.

    PT activity 70 - 130% 83% 91% 130%

    APTT 25 - 39High 54.6

    27.3

    APTT

    control 25 - 39 34.6 34.6 34.6

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    HEMATOLOGYNORMAL

    RANGE

    11-March-2011

    Results

    12-March-2011

    Results

    16-March-2011

    Results

    Leukocytes

    5.0 - 10.0 x

    109/L High 11.95 High 10.10 High 11.10

    Erythrocytes 4.6 - 6.2 x1012/L

    Low 2.80 Low 3.22 Low 3.95

    Hemoglobin 12 - 17 g/dL Low 9.1 g/dL Low 8.9 g/dL 12.8 g/dL

    Hematocrit37 - 47 %

    Low 27.39% Low 27% 37.7%

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    Thrombocytes 150 450 x109/L

    160 x 109/L 175 x 109/L Low 146 x

    109/L

    Neutrophils 50 70 % High 84.3% High 78.1%

    Lymphocytes 20 40 % Low 9.3% Low 10% Low 12%

    Monocytes0 7 %

    4.9% 5.9% High 8.4%

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    Eosinophils 0 5 % 1% 1%

    Basophils 0 1 % 0.5% 0.5%

    Granulocyte 0 1 % High 83.5%

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    CLINICAL

    CHEMISTRY

    NORMAL

    RANGE

    12-March-2011

    Results

    15-March-2011

    Results

    Blood Urea

    Nitrogen 7 17 mg/dL High 95 High 69

    Creatinine 0.52 1.04

    mg/dLHigh 4.2 High 2.25

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    UrinalysisMarch 11, 2011

    PHYSICAL CHEMICAL

    Color: YELLOWAlbumin: +++

    Sugar: TRACE

    Reaction: 7.5

    Transparency: SLIGHTLY TURBID

    Quantity: 25 mL

    Specific Gravity: 1.010

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    Manifestation from the book Actual ManifestationCONSTITUTIONAL SYMPTOMS

    Fever & myalgiaNo

    Malaise, fatigue Yes

    Weight loss No (weight gain due to generalizededema)

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    Manifestation from the book Actual Manifestation

    Skin manifestations

    SplinterHemorrhage

    Yes

    Skin rash, palpable

    purpura, skin ulcerationsNecrosis and

    gangrene/Necrotizing nodules

    No

    Pulmonary Manifestation

    Hemoptysis, Dysnea, cough,

    pulmonary rales,respiratoryory distress

    No

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    Hypertension Yes

    Signs of cardiac failure,

    myocardial infarction No

    Gastrointestinal Involvement

    GI bleeding, melena Yes

    Bowel

    ischemia/perforation/pancrea

    titis

    No

    Cardiovascular

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    Nervous System Manifestation

    Seizures/ /brain swelling Yes

    tingling sensation No

    Renal Involvement

    Uremia, generalized edema,

    proteinuria, red blood cells in the

    urine

    Yes

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    CAUSE:

    IdiopathicPredisposing Factor

    Gender/Sex: Men

    Autoimmune Infection Malignancy Drug Therapy

    Triggers production on p-ANCA

    (antineutrophil cytoplasmic

    antibodies)

    Large antibodies bind to

    neutrophil

    Activate immune complexes

    Deposits in small vessel walls

    Increase Vascular permeability

    Release of vasoactive amines and mast cells

    Neutrophil

    Releases

    toxins

    Causes a

    release of

    lytic

    enzyme

    FromWBC

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    Endothelial Injury

    INFLAMMATION

    Of small blood vessels

    Damage

    peripheralnerves Nerve

    InfarctionNerves are

    deprived of

    nutrients

    Damage to vessels

    of glumeruli

    Impairs renal function:

    PROTEINURIAHEMATURIA

    FATIGUE

    SWELLINGOF LEGS

    EDEMA

    ANEMIA

    UREMIA

    Constitution

    al

    Symptoms:

    FEVER

    WEIGHT

    LOSS

    MALAISE

    Skin Lesions:

    PALPABLE

    PURPURA

    NECROSIS

    GANGRENE

    Cardiovascular

    Signs/symptoms

    HYPERTENSION

    CHEST PAINCARDIAC

    FAILURE

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    DRUG NAME

    CYCLOPHOSPHAMIDE

    50mg/tab 1 tab POODCLASSIFICATION

    Antineoplastic or cytotoxic

    DRUG ACT

    IO

    NCyclophosphamide first is converted by the liver into

    two chemicals, acrolein and phosphoramide.

    Acrolein and phosphoramide are the active

    compounds, and they slow the growth of cancer cellsby interfering with the actions of deoxyribonucleic

    acid (DNA) within the cancerous cells. It is, therefore,

    referred to as a cytotoxic drug.

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    Indication

    Treatment of vasculitis

    DRUG OF CHOICE for the treatment of MPA.

    Cyclophosphamide has a significant

    ability to suppress the immune system.

    Thus, the medicine is very effective in thetreatment of immunologicallymediated

    diseases, including some forms of vasculitis.

    i C id i

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    Nursing Consideration

    1. Check for allergic history2. Periodically check renal function because the drug

    is toxic for the kidneys

    3. Teach patient to drink plenty of fluids because thisdrug can damage kidneys and bladder

    4. The drug is immunosuppressive, monitor patientfor signs of infection

    5. Causes depletion of number of blood cells, monitor

    patients blood test

    DRUG NAME

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    DRUG NAME

    PREDNISONE30mg 1 tab, 2 tabs post breakfast

    CLASSIFICATION:

    Corticosteroid (Glucocorticoid)

    DRUG ACTIONSuppresses the immune system and

    inflammation. This synthetic corticosteroid

    mimic the action of cortisol (hydrocortisone),the naturally-occurring corticosteroid

    produced in the body by the adrenal glands.

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    IndicationDRUG OF CHOICE for treatment of vasculitis in combination

    with cyclophosphamide.

    Nursing Consideration

    1. Check and record BP during dose stabilization

    period at least 2 times daily. Report an

    ascending pattern.2. Be alert to possibility of masked infection and delayed

    healing.3. Obtain fasting blood glucose, serum electrolytes, and

    routine laboratory studies at regular intervals during

    long-term steroid therapy.

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    4. Be alert to signs of hypocalcemia.

    5. Monitor bone density.

    6. Be aware that long-term

    corticosteroid therapy is notimmediately interrupted. Tapering is

    done.

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    DRUG NAME

    METHYLPREDNISOLONE1g in 50mL D5W IV infusion

    Single orderCLASSIFICATION:

    Corticosteroid

    (Glucocorticoid)

    DRUG ACTIONMethylprednisolone is a synthetic (man-made)

    corticosteroid. It mimics the action of cortisol

    (hydrocortisone), the naturally-occurringcorticosteroid produced in the body by the adrenal

    glands. Corticosteroids block inflammation and are

    used in a wide variety of inflammatory diseases.

    I di ti

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    Indication

    For treating inflammatory conditions (vacultitis).

    Nursing Consideration

    1. Watch for allergic reactions

    2. Check the electrolyte and glucose reports

    for early indications of electrolyte imbalanceor hyperglycemia

    3. Keep accurate records of intake and output,

    daily weights, vital signs4. Ask patients about any signs of infection

    (sore throat, fever, malaise, nausea and

    vomiting)

    Oth D

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    FUROSEMIDE 60mg 1 1/2tab q8 hrs

    Loop diuretics Treatment ofedema

    CLONIDINE 150mg /tab

    1 tab BID

    Central acting alpha

    adrenergic

    antihypertensivedrug

    Hypertension

    AMLODIPINE

    10mg/tab

    OD

    Calcium Channel

    Blocker

    Hypertension

    Used to prevent

    angina

    TELMISARTAN

    40mg/tab 1

    tab OD

    Angiotensin II

    receptor

    antagonist

    Hypertension

    Other Dugs

    NAME OF DRUG DOSAGE/FREQUENCY

    CLASSIFICATION INDICATION

    NAMEOF DRUG DOSAGE/FREQUENCY CLASSIFICATION INDICATION

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    ENALAPRIL

    (renital)

    10mg /tab,

    1/2 tab OD

    Angiotensin

    converting

    enzyme (ACE)inhibitors.

    Hypertension

    CITICOLINE

    500mg 1 cap, 1

    capOD

    ODCentral nervous

    system stimulantPatient is noted to have

    hypertensive

    encepalopathy

    CALCITRIOL1 tab 0.25mg

    ODSynthetic

    Vitamin D

    Use to treat and

    prevent low levels of

    calcium in the bloodof patients with

    damaged kidneys or

    with patients in

    hemodialysis

    NAMEOF DRUG / CLASSIFICATION INDICATION

    NAMEOF DRUG DOSAGE/FREQUENCY CLASSIFICATION INDICATION

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    CALCIUM

    CARBONATE1 tab 500mg

    TID

    Mineral electrolyte

    replacement

    supplements

    Hyperphosphataemia in

    chronic renal failure

    FERROUS

    SULFATE

    325mg 1 tab post

    lunch, 2 tabs

    post dinner

    Iron preparationUsed to treat and prevent

    iron deficiency anemia

    COTRIMOXAZOLE800mg /100mg

    tab/tablet forte,

    1 tab (every

    Sunday, Tuesday

    and Thursday)

    Sulfamethoxazol

    e and

    trimethoprim

    Prophylaxis for

    infection

    OMEPRAZOLE40 mg IV OD

    Proton pump

    inhibitor

    Short-term

    treatment of

    active, benign

    gastric ulcer

    CLASSIFICATION INDICATION

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    Problem/ SignificantI t ti E l ti

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    / g

    NotationsInterventions Evaluation

    Objective Data:

    broken skin,traumatized tissue

    Problem/ Significant

    NotationsInterventions Evaluation

    Objective Data:

    broken skin,traumatizedtissue

    presence of right

    intrajugular

    catheter access

    needle pricks onthe arms from

    invasive

    procedures

    Intrajugular access

    cleaned and dressed

    aseptically

    Proper use of PPE

    for staff and visitors

    Problem/ SignificantI t ti E l ti

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    / g

    NotationsInterventions Evaluation

    Problem/ Significant

    NotationsInterventions Evaluation

    Sterile technique

    for all invasive

    procedure. Suggested to place

    in protective

    isolation.

    Maintained ortaught asepsis for

    dressing changes

    and wound care.

    Problem/ Significant

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    Problem/ Significant

    NotationsInterventions Evaluation

    Immunosupressionrelated to

    immunosuppressive

    drugs

    Cyclophosphamide

    Prednisone

    Proper handhygiene by all care

    givers

    Offer mask or

    tissue to

    client/visitors who

    are

    coughing,sneezing

    to limit exposure

    Follows medication

    regimen

    No noted signs of

    infection (feveror pus on lesions)

    Problem/ Significant

    Maintained IV flow rate

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    Problem/ Significant

    NotationsInterventions Evaluation

    Objective Data:y Hematemesis

    (fresh blood,

    scanty amount)

    y Melena

    (semi-formed,

    moderate in

    amountTarry stool)

    y Auscultate BP,calculate

    pulse pressure every 4

    hours

    y O

    bserve for color of allexcretion to evaluate

    for bleeding

    Have a written record

    of fluid intake, urine

    output and daily weight.

    Maintained IV flow rate

    No bleeding

    episodes noted

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    Problem/ Significant

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    Problem/ Significant

    NotationsInterventions Evaluation

    Objective Data:

    y Blood Pressure: 150/90

    mmHg

    y

    Generalized edema

    y Pitting edema

    grade 2

    y Facial edema

    Monitoring of vital signs q1

    especially blood pressure

    y Record an accurate intake and

    output

    Administer medication

    as prescribed ( Diuretics,

    antihypertensive)

    Protected edematous

    skin from injury

    Fluid restriction of 1L

    per day as ordered

    Blood pressure :

    110/70 mmHg

    Patient still

    edematous

    Cautious in eatingand drinking

    Problem/ Significant

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    Problem/ Significant

    NotationsInterventions Evaluation

    Objective Data:

    (+) Weakness(+) Fatigue

    (+) Muscle

    weakness

    (+) Fatigue whenmoving

    Assessed level of

    activity intoleranceand degree of fatigue

    Encouraged

    alternating periods ofrest and ambulation

    Encouraged and

    assisted with gradualincreasing of periods

    of exercises

    Patient reported

    no signs of

    decrease in fatigue

    and body malaise

    Still requires full

    assistance inmoving

    Exhibited

    increased interestin activities and

    event

    Problem/ Significanti l i

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    / g

    NotationsInterventions Evaluation

    Turned side to side

    every 2 hours

    Health teachings about

    the importance of

    ambulation

    ROM exercises done

    Collaborated withphysical therapist for

    treatment plans and

    interventions

    Increases exercises

    within physical

    limits

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