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    A Case Reportby:

    PGI Joanna Rose F. Estayo

    HYALINE MEMBRANE DISEASEHYALINE MEMBRANE DISEASE

    (respiratory distress syndrome)

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    Patient Profile

    Live, preterm baby boy delivered via stat CS

    secondary to twin pregnancy to a 35 year old

    G1P0 35 4/7 weeks by LMP, 33 weeks by MT,BW = 2090 grams, AS 9,9, AGA

    Date of Birth: 10/04/10

    Time of Birth : 2125H

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    Anthropometrics

    Birth weight = 2090 grams

    Birth length = 47 cm

    Head Circumference = 33cm

    Chest Circumference = 28 cm

    Abdominal Circumference = 26 cm

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    Birth and Maternal History

    35 year old G1P0

    (+) regular prenatal check-up

    HbsAG and VDRL non-reactive CBC, UA, OGTT normal

    No maternal illness during the pregnancy

    No fetomaternal complications

    Gestation in wks: 35 4/7 weeks by LMP

    Manner of Delivery : stat CS

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    Physical Examination (on admission)

    General Apperance:

    vi

    gorous Skin: pink

    Head: normocephalic, no caput succedaneum

    Neck: supple

    Eyes/ENT: no discharge, (+) ROR, (+) alar flaring

    Lungs: equal chest expansion, clear breathsound,(+) grunting, tachypnea

    Heart: no murmurs

    Abdomen: globular, soft

    Genetalia: grossly male

    Anus: patent Extremities: no gross deformties

    Reflexes: intact

    Trunk/Spine: midline

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    Course in the Wards10 mins

    after birth

    O >(+) alar flaring

    (+) grunting(+) tachypneaO2 sat @room air 85% --> O2 @ 1lpm vianasal canula, --> 87% --> O2 @ 2 lpm --> 100%Hgt = 72 mg/dl

    P>For CXR: Hyaline Membrane DiseaseDiagnostics: CBC with PC, BCS, ABOFor isoletteHook to pulse oximeterIVF: D10 W 170cc to run @ 7cc/hr

    NPOStart antibiotics:

    Piptazo 100mg IV q12 hrs (96mkD)Amikacin 30mg IV q 24 hrs (14.6 mkD)

    WOF: respiratory distress, cyanosis, O2desaturation

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    3 hrs

    after birth

    8 hrs

    after birth

    9 hrs

    After birth

    O> (+) alar flaring, (+) grunting(+) subcostal retraction,(+) tachypnea

    (+) desaturation 80s

    P> For CPAP, FiO2 40%, PEEP = 6For ABGFor Ambubagging x 15 mins

    O> Still with persistence of signs ofrespiratory distress

    P> Increase PEEP to 7

    For surfactantvi

    a ET

    tube, thenextubate

    S/P Surfactant (4cc/kg) via ET , 8mlO2 sat = 97%

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    9.5 hrs

    after birth

    11 hrs

    after birth

    18 hrsafter birth

    O2 saturation = 83%FiO2 increased to 50%PEEP = 7

    O saturation = 94%

    Increase PEEP to 9, FiO2 decrease to 40Wean CPAP , slowly tapering FiO2, for O2saturation > 95 %If FiO2 remains >0.30 @ 1400H, give 2nd

    dose of surfactant

    O> RR= 72, O2 sat = 94-98 %

    deep subcostal retractionsgood air entry, clear BS

    P> For 2nd dose of surfactant

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    19 hrs

    after birth

    2nd dayof life

    S/P surfactantO2 sat = 95%(+) subcostal retractions

    Continue weaning CPAP

    O> active, good cry(+) subcostal retractionsgood air entry, clear BSRR = 50 70sO2 sat = 93-97%

    P> CPAP setting 25% @ 9 cmIVF (TFI : 100cc/kg/day)

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    3rd day

    of life

    4th day

    of life

    O> active, good cry(+) minimal subcostal retractionsgood air entry, clear BSRR = 50sO2 sat = 95%

    P> CPAP setting 21% @ 8 cmIVF (TFI : 110cc/kg/day)

    Revise CPAP to FiO2 =21, PEEP = 6,TFR = 6FiO2 =21, CA= 5, O2= 0,TFR = 5

    Start oral feeding, if breastmilk is notavailable, start Pre Nan 10cc q3 hrs per OGT

    Once tolerated may decrease IVF

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    6th day

    of life

    7th day

    of life

    8th day

    of life

    O> good activity, good cry(+) subcostal retractionsgood air entry, clear BS

    O2 sat = 96-97%No desaturations

    P> Discontinue CPAPIncrease feeds as toleratedContinue antibiotics to complete for

    7 days

    DC pulse oximeterDC IVFIncrease feeds

    DC isoletteIncrease feedsEncourage breast feeding

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    alient Features

    preterm baby boy (35 4/7 weeks by LMP, 33 weeks byMT)

    delivered via stat CS secondary to twin

    2nd of twin

    BW = 2090 grams, AS 9,9,

    No fetomaternal complications

    No maternal illness during pregnancy

    Alar flaring

    Grunting

    Tachypnea

    Subcostal retractions

    Desaturation

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    Differential Diagnoses

    TransientTachypnea of the Newborn

    Neonatal Pneumonia

    Hyaline Membrane Disease

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    Transient Tachypnea of the Newborn

    Reasons for Ruling In Reasons for RulingOut

    (+) alar flaring

    (+) tachypnea (+) retractions

    (+) grunting

    CS delivery

    Tachypnea not relieved by

    O2 support alone Patient still have minimal

    retractions on the 6th dayof life

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    Neonatal Pneumonia

    Reasons for Ruling In Reasons for RulingOut

    (+) alar flaring

    (+) tachypnea (+) retractions

    (+) grunting

    No maternal illness /

    unremarkable PMHx ofmother

    No prolonged rupture ofmembranes

    No other s

    igns of seps

    is /

    infection

    Blood CS : NEGATIVE

    CBC: normal

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    Hyaline Membrane Disease

    Reasons for Ruling InReasons for RulingOut

    (+) alar flaring

    (+) tachypnea

    (+) retractions

    (+) grunting

    Prematurity

    Male sex

    CS delivery

    Multiple gestation

    CXR result: reticulogranularpattern, ground glassappearance, (+) air bronchogram,

    flattened diaphragm

    Marked improvement aftersurfactant therapy

    ---------

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    Assessment

    Hyaline Membrane Disease

    (Respiratory Distress Syndrome)

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    Hyaline Membrane Disease

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    Definition

    Clinical diagnosis ofHyaline Membrane Disease iswarranted in a preterm newborn with the ff:

    Respiratory difficulty

    Tachypnea (>60 cpm) Chest retractions Cyanosis

    *** progresses over the first 48-96 of life

    CXR: uniform reticulogranular pattern andperipheral air bronchogram

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    Incidence

    HMD occurs in 44% ofinfants with

    birthweight between 501 and 1500 grams

    Incidence is inversely proportional to the

    gestational age and birthweight

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    Pathophysiology

    Primary cause : SURFAC

    TAN

    TDEFICIENCY

    SURFACTANT

    produced by type II alveolar cells at 28 to 34 wks

    Appears in amniotic fluid between 28 and 32 wks Mature levels present after 35 wks reduces surface tension within the alveoli help to maintain alveolar stabilitybypreventing

    collapse of alveoliat end expiration

    Major constituents of surfactant: dipalmitoylphosphatidylcholine (lecithin) phosphatidylglycerol apoproteins (surfactant proteins SP A,B,C,D) cholesterol

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    Overly compliant chest wall

    Decreased intrathoracic pressure

    Shunting

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    Risk Factors

    Increased Risk Decreased Risk

    Prematurity

    Male sex Familial predisposition

    Cesarean delivery withoutlabor

    Peri

    natal asphyxi

    a Chorioamnionitis

    Multiple gestation

    Maternal diabetes

    Chronic intrauterine stress

    Prolonged rupture ofmembranes

    Maternal hypertension

    Narcotic/cocaine use

    IUGR or SGA Corticosteroids

    Thyroid hormone

    Tocolytic agents

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    Clinical Presentation

    History Physical Examination

    Preterm (by dates orgestational examination)

    Hx of asphyxia in theperinatal period

    Px with respiratory difficultyat birth, which becomesprogressively severe

    Worsening of atelectasis onthe CXR

    Increasing oxygenrequirement

    Tachypnea

    Grunting Nasal flaring

    Retractions of the chest

    wall

    Cyanosis

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    MANAGEMENTOF

    HMD

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    Diagnostics

    Chest Radiography

    - uniform reticulogranular pattern (ground-glass

    appearance) and peripheral air bronchogram Blood gas sampling

    Sepsis work-up (CBC and BCS)

    Serum glucose levels Serum electrolytes

    Echocardiography

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    Prevention

    Antenatal corticosteroid

    enhances fetal lung maturity Betamethasone - two 12mg doses via IM 24 hours apart

    Dexamethasone - increased risk for cystic periventricularleukomalacia

    Antenatal ultrasonography and continuous fetalmonitoring

    Tocolytic agents

    Assessment of fetal lung maturity before delivery lecithin-to-sphingomyelin ratio phosphatidylglycerol

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    urfactant Replacement

    now considered a standard of care in the

    treatment ofintubated infants with HMD.

    Surfactant prophylaxis (within 15 mins ofbirth) is recommended to almost all infants

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    Respiratory Support

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    Other Additional Management

    Fluid and Nutritional Support

    AntibioticTherapy

    - Usually wide spectrum antibiotics are used Sedation

    - Might be indicated for infants who fight the

    vent

    ilator and exhale dur

    ing the

    insp

    iratorycycle of mechanical ventilation

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    Prognosis

    Survival ofinfants with HMD has improved

    greatly during the past years

    Prognosis for survival with or without

    respiratory or neurologic sequelae is highly

    dependent on birthweight and gestational

    age .

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    References:

    Kliegman, R. e. (2007).Nelson's textbook of Pediatrics (18th

    ed.). Philadephia: Saunders Elsevier.

    Philippine Pediatric Society: Standard Care of the Newborn

    Thank you!