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Table 3 Characteristics of this case compared with other Human Cytomegalovirus (CMV) Infections

From: Death from human cytomegalovirus infection in a girl with congenital thymic dysplasia

CMV infection type

Population

Age at onset

Symptoms and signs

Treatment

Outcome

This case

Congenital thymic dysplasia

3 months old

Refractory CMV disseminated disease (retinitis, pneumonia, and hepatitis) and central nervous system involvement

GCV, V-GCV, and FOS

Death

Congenital cytomegalovirus( cCMV) Infection

Infants born to CMV-infected mothers

Pregnancy period

a. Asymptomatic; b. Asymptomatic during the newborn period with isolated SNHL; c. Symptomatic cCMV disease (approximately 10%), with manifestations including jaundice, petechiae, purpura, hepatosplenomegaly, microcephaly, intracerebral (typically periventricular) calcifications, SNHL, retinitis, and developmental delays

a. No antiviral therapy for asymptomatic; b. Not routinely receive antiviral treatment for mild symptomatic disease or with isolated SNHL (Uncertain); c. Six months of V-GCV therapy, only most severely affected symptomatic infants who are > 32 weeks gestation and less than 1 month of age; c. Monitoring of hearing and development

Death in 3% to 10% of symptomatic infants associated with primary maternal infections and occurs earlier during gestation; SNHL in approximately 10% of asymptomatic

Acquired CMV infection

Infants

Perinatal period

a. Asymptomatic and mild infections are the most common; b. Severe symptoms, end-organ disease (e.g., hepatitis, interstitial pneumonia, hematologic abnormalities including thrombocytopenia and leukopenia), and a viral sepsis syndrome

a. Mild cases should not receive antiviral treatment; b. Two weeks of GCV therapy, an additional 1 to 2 weeks can be considered if symptoms and signs have not resolved

The majority of them are curable, and few have a poor prognosis

 

Secondary Immunodeficiencies a

Highly variable

Severe symptoms are the same as above

a. Antiviral Pharmacotherapy (GCV, V-GCV, FOS, Cidofovir, Brincidofovir, Letermovir); b. Immune Globulin Intravenous (IGIV) or CMV-IGIV; c. CMV-Specific T-Cells Virus-specific T-cells

Vary with the age and immunocompetence of the host, life-threatening in resistant and refractory CMV infection

 

Primary Immunodeficiencies

Usually during early childhood

a. Severe symptoms are the same as above; b. Haemophagocytic ymphohistiocytosis induces sustained systemic inflammatory responses and immune dysregulation, and autoimmune phenomena; persistence of infection with T-cell exhaustion

  
  1. CMV cytomegalovirus, cCMV congenital cytomegalovirus, SNHL sensorineural hearing loss, GCV ganciclovir, V-GCV valganciclovir, FOS foscarnet
  2. a. Secondary Immune Deficiencies, including people receiving treatment for malignant neoplasms, people infected with human immunodeficiency virus (HIV), and people receiving immunosuppressive therapy for solid organ or hematopoietic stem cell transplantation