Cytomegalovirus

Herpes

Cytomegalovirus:

• Belongs to the _______ virus family

• Exposure increases with age

Saliva Urine Cervical secretions

==Transmission of CMV occurs when coming into contact with the virus through:==

• _______

• Blood

• _______

• Breast milk

• _______ _______

• Semen

36

91

In the US ~ ___% of children 6-11 years old are CMV+

By 80 years old seropositivity increases to ___%

no

mild

Most healthy children and adults infected with CMV will have ___ symptoms or might develop only a ______ mononucleosis-like illness

reactivate

mother

Once infected with CMV:

• Virus can _________, but healthy immune system prevents this in most cases

• A _________ carrier can transmit virus to fetus which causes developmental issues in infant.

  • Acute symptoms at birth

  • Permanent disabilities

  • Both

In-utero

prenatal

Diagnosis of Congenital CMV:

either _________ through amniocenteses OR in the _________ period for from direct assessment of the infant in the postnatal period.

21

Testing for congenital CMV must be completed in ____ days from birth otherwise it is IMPOSSIBLE to distinguish congenital from acquired.

amniocenteses

• A test offered during pregnancy to check if a baby has a genetic or chromosomal condition.

• It involves removing and testing a small sample of cells from amnioticfluid (the fluid that surrounds the baby in the womb)

CMV

21

___ is the most common intrauterine infection in the US with ~ __% of pregnant women being symptomatic (like mononucleosis)

universal

US

_________ = 0.64-.70% of live births

_________ = 0.2 to 2.2% (congenital) with estimated 0.5 to 1.0 newborns infected in the prenatal period

Symptoms

• Generalized petechiae

• Hyperbilirubinemia

• Hepatosplenomegaly

• Purpuric rash (3-10mm)

• Microcephaly

• Seizures

• Focal or general neurologic deficits

• Retinitis

• Intracranial calcifications

Focal/general neurologic deficits

Hyperbilirubinemia

Generalized petechiae

Seizures

Hepatosplenomegaly

Retinitis

Intracranial calficiations

Microcephaly

Purpuric rash

CMV Symptoms:

Fat Hairless Gumbo SHRIMP

(FHGSHRIMP)

90 to 95

Most aka ___ to ____% of infants w/ congenital CMV will NOT have any clinically significants apparent symptoms at birth.

No

Are newborns screened from CMV in the US?

blood spot

It is possible to ID children with CMV by obtaining a newborn _________ _________ for analysis

urine or saliva

Typically diagnosed through detection within _________ or _________ within the first 3 weeks of life (21 days)

mother exposure during pregnancy

What would lead to testing at birth?

referred NBHS

Utah, Illinois, Connecticut, Iowa, New York, and Virginia participates in a screening for cCMV following a _________ _________

Sequela

Any abnormal condition that follows and is the result of a disease, treatment, or injury, such as paralysis after poliomyelitis, deafness after treatment with an ototoxic drug, or scar formation after a laceration

Sequela

Both symptomatic and asymptomatic infants may later develop _________.

• More severe and frequent in the symptomatic infants.

CMV Sequela

• SNHL

• Retinitis

• Mental Retardation

• Microcephaly

• Seizure

• Cerebral palsy

10 to 15

SNHL is most common sequela of cCMV

__% to __%

30 to 50

HL occurs in __% to __% of children with symptoms at birth

8 to 12

HL occurs in in __% to __% of children who are asymptomatic at birth

Mild to profound

Unilateral or bilateral

fluctuating progressive

• No predictable audiometric configuration

  • _________ to _________

• Laterality: Unilateral or bilateral

• Some involve 4-8k Hz only - others all frequencies are involved

• HL may be _________ and/or _________

  • Develop within the first years for some

50

~ __% of the cases of CMV related HL are late-onset/and/or progressive.

(Not always detected at birth…. ~7% of cCMV cases were detected)

21

25

Recent estimates:

• __% of Hl at birth is cCMV related

• Late onset HL = __% of HL in children by age 4 likely CMV related.

  (Suggest that CMV is the leading non genetic cause of HL in Peds for the US)

Pathogenesis

Animal and human studies on temporal bone suggest:

• CMV may be present in the epithelium and neural cells of the inner ear.

• Cause U__amage by virus-mediated damage to neural cells and/or cause inflammatory responses resulting in damage__ to the auditory apparatus therefore causing HL

• Doesn’t explain late onset or progressive loss!!!

audiologic evaluations

changes

JCIH treatment:

• Babies ID’s early need impediment and more frequent _________ _________ to allow close monitoring

• _________ in hearing should be considered in treating the HL

  • May program HAs more often.

• Communication methods/needs to accommodate a changing HL

regular

vision

CDC recommendations:

• _________ hearing checks

• routine _________ screening

• Developmental milestone checks

Multi-disciplinary team

• Infectious disease specialists

• Otolaryngology

• Neurology

• Primary care/Pediatrician

• Audiology

• Opthamology

(Dahle et al., 2000)

Longitudinal study (24 years)

860 children with CMV were studied                   (651 asymptomatic, 209 symptomatic)

Test battery:

• ABR (chloral hydrate)

• Air and bone conduction if AC>25 dBnHL

• Tympanometry

• VRA (from 9 months to 3 years old)

Longitudinal study outcomes:

• Bilateral moderate to severe SNHL                       (equally severe in asymptomatic patients)

• Delay onset of hearing loss                                    (wide age range: 6 months to 16.4 years)

• Gradually progressive                                            (sometimes sudden decreases reaversed w/ steroids)

• Threshold fluctuations without progression of their loss (may experience improvement)

• Low frequencies (250 and 500 Hz) less stable than higher frequencies

flat

high

Audiometric pattern:

• _________ hearing loss was the predominant threshold configuration

• Initially by a sloping _________-frequency loss may gradually progress to include the lower frequencies

• Upward sloping (symptomatic)

• Downward sloping (asymptomatic)

Symptomatic audio

upward sloping

Asymptomatic audio

downward sloping

laboratory

CNS

growth and development

Medical management/evaluation:

• A general medical examination

• _________ procedures to document the diagnosis of CMV

• An examination to determine damage to the _________ (EEG)

• Assessment of the child’s _________ and _________.

antiviral treatment

Medical management

Prolonged _________ _________ for cCMV with SNHL showed improvement (58-79%) in hearing status and no deterioration in unaffected ears at baseline.

Pharmacological Treatment:

Ganciclovir

(inhibitor of viral DNA synthesis) antiviral drug may have a beneficial effect in newborns affected by severe congenital CMV infection such as preventing hearing deterioration

• Maintained normal hearing or stops the progression of hearing loss in 76% of infants when baseline compared to tests 6 months later

• Significant side effects including bone marrow suppression, therefore if severe-profound hearing loss is present at birth, the doctors will likely not administer Ganciclovir due to physical risk and limited benefit in terms of hearing restoration.

6 to 12

Hearing eval:

• Audiogram

• ABR

• OAR

• Tympanometry

If suspected a child should be routinely monitored:

• Child should be assessed every __ to __ months

Speech and language therapy

• Must be tailored to needs

Appropriate counseling for the parents!

vaccine

No approved _________ for prevention of CMV

Hand washing

_________ _________ is the #1 preventer

(esp when pregnant or trying to get pregnant)

day care

mothers

toddlers

_________ _________ providers and _________ of _________ are at the highest risk of contraction

Young children

This population tend to secrete the virus in their saliva and urine for many months following the first infection.

[typically asymptomatic]