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Disease Profile

Congenital herpes simplex

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

#N/A

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Congenital herpes simplex virus infection; Antenatal herpes simplex virus infection; Mother-to-child transmission of herpes simplex virus infection

Summary

The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
orphanet

Orpha Number: 293

Definition
Congenital herpes virus infection is a group of anomalies that an infant may present as a result of maternal infection and subsequent foetal infection with herpes virus. This virus causes recurrent cutaneous infections in adults, often involving the lips or the genitalia. Herpes infections in other organs, such as the liver or central nervous system, are less frequent.

Clinical description
Skin lesions and scars, chorioretinitis, microcephaly, hydrencephaly, and microphthalmia have been described in infants with in utero primary herpes virus infections, but a causal relationship between these manifestations and the infection could not be established. Pregnancy complications including preterm delivery, intrauterine growth retardation, and neonatal infection have been attributed to the herpes virus.

Etiology
There are two types of virus (HSV-1 and HSV-2) and both appear to have the same activity. However, type 1 infections more commonly involve the oral cavity, whereas type 2 infections more commonly involve the genital region. It has been suggested that latent HSV-2 infections occur in as many as 20 to 40% of women. Exposure of the foetus to herpes virus at the time of delivery carries a serious risk of infection for the newborn. This risk may be high during the initial infection of the mother (33 to 50%), but probably does not exceed 3% in cases of reinfection because of the protective effect of maternal antibodies.

Differential diagnosis
It remains difficult to distinguish between cases with a primary infection and those with a previously undetected recurrent infection that has become symptomatic during pregnancy.

Management and treatment
In spite of described cases of serious foetal illness, primary infection with herpes virus during pregnancy is not an indication for therapeutic abortion, and the use of antivirals in the treatment of primary herpes infections during gestation has been recommended. Some authors recommend caesarean delivery for women with first-episode genital herpes lesions at the time of delivery.

Visit the Orphanet disease page for more resources.

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
30%-79% of people have these symptoms
Intrauterine growth retardation
Prenatal growth deficiency
Prenatal growth retardation

[ more ]

0001511
Premature birth
Premature delivery of affected infants
Preterm delivery

[ more ]

0001622
5%-29% of people have these symptoms
Hydranencephaly
0002324
Microcephaly
Abnormally small skull
Decreased circumference of cranium
Decreased size of skull
Reduced head circumference
Small head circumference

[ more ]

0000252

Treatment

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.