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Query: UMLS:C0025362 (mental retardation)
 15,878 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

CMV is the most common cause of intrauterine infection in this country, affecting approximately 1% of newborns. As such, CMV infection is a leading cause of deafness and an important contributor to learning disabilities. The virus is transmitted through close contact with infected secretions as well as transplacentally, through sexual contact, and through blood transfusion and organ transplantation. CMV has a high prevalence in the population; antibody to the virus can be detected in 50% to 85% of individuals. Like herpes simplex, the virus can be reactivated and cause recurrent infection. Congenital disease occurs in about 30% to 40% of women with primary infection. Ten percent of infants with congenital infection will have clinical disease at birth. Of these, as many as 30% die. Ninety percent of survivors have sequelae such has deafness, mental retardation, chorioretinitis, and motor deficits. The other 90% of infants with congenital infection are asymptomatic at birth, but 5% to 17% develop long-term sequelae. Congenital disease also occurs with recurrent maternal infection in about 0.2% to 1% of immune women. Congenital infection due to recurrent disease is far less severe, and less than 10% of affected infants have long-term sequelae (Fig 3). The diagnosis of congenital infection can be established using a combination of ultrasound, amniocentesis, and cordocentesis. Viral culture of neonatal urine can confirm the diagnosis after birth. Routine screening of the pregnant population is not currently recommended because present laboratory methods limit the ability to differentiate primary from recurrent infection. Preventive efforts at this time must focus on good hygiene, limited intimate contact with infected children, and responsible sexual practices.
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PMID:Cytomegalovirus infection in pregnancy. 816 23

Short rib-polydactyly syndromes (SRPS) are a heterogeneous group of recessively inherited lethal skeletal dysplasias. Four types have been recognised. However, overlap in the clinical and radiological features of the four types has led to difficulties in distinguishing between them. The congenital infection-like syndrome is an autosomal recessive syndrome characterised by mental retardation, microcephaly, seizures, and intracranial calcifications. We report a complex consanguineous family of Baluchi origin in whom short rib-polydactyly type III and congenital infection-like syndrome are segregating. Four children inherited SRPS III, one inherited congenital infection-like syndrome, and one inherited both. Although the radiological features in all the children with SRPS in this report were typical of type III, there was overlap in the clinical features with the other types of SRP syndromes. Furthermore, the child who inherited both SRPS III and congenital infection-like syndrome had CNS malformations in addition to periventricular calcification. CNS malformations have been described in SRPS types II and IV but not type III. This report further highlights the overlap between the different types of SRP syndrome. Moreover, it draws attention to the importance of considering the possibility of two recessive syndromes in the same child in complex consanguineous families when features overlap two syndromes.
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PMID:Complex consanguinity associated with short rib-polydactyly syndrome III and congenital infection-like syndrome: a diagnostic problem in dysmorphic syndromes. 1087 34

This study was carried out to determine the prevalence of cytomegalovirus (CMV) excretion in urine among 30 deaf children and 91 mentally retarded children by cell culture and PCR. As a control, urine samples from 121 children without hearing disability or mental retardation were also tested. The study revealed that 15 of 30 (50%) deaf children and 16 of 91 (17.6%) mentally retarded children were excreting CMV in their urine. Among the control group we observed that only 2 of the 121 (1.8%) children were CMV excretors. As CMV excretion in urine is generally considered to indicate a congenital infection, it is very likely that congenital CMV is highly incriminated in mental retardation and deafness among children in Mauritius.
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PMID:Detection of cytomegalovirus in urine of hearing-impaired and mentally retarded children by PCR and cell culture. 2133 93

Cytomegalovirus (CMV), a member of the herpes virus family, is the most common cause of congenital infection in humans, affecting 0.5-3% of all newborns worldwide. Congenital cytomegalovirus infection is the leading infectious cause of deafness, learning disabilities, and mental retardation in children. The high prevalence of cytomegalovirus in the general population, unpredictability of transmission, and asymptomatic nature of the disease in otherwise healthy women challenge prevention and treatment efforts.
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PMID:Cytomegalovirus infection: perinatal implications. 1184 23

Human cytomegalovirus (HCMV) is the leading cause of congenital viral infection and mental retardation. HCMV infection, while causing asymptomatic infections in most immunocompetent subjects, can be transmitted during pregnancy from the mother with primary (and also recurrent) infection to the fetus. Hence, careful diagnosis of primary infection is required in the pregnant woman based on the most sensitive serologic assays (immunoglobulin M [IgM] and IgG avidity assays) and conventional virologic and molecular procedures for virus detection in blood. Maternal prognostic markers of fetal infection are still under investigation. If primary infection is diagnosed in a timely manner, prenatal diagnosis can be offered, including the search for virus and virus components in fetal blood and amniotic fluid, with fetal prognostic markers of HCMV disease still to be defined. However, the final step for definite diagnosis of congenital HCMV infection is detection of virus in the blood or urine in the first 1 to 2 weeks of life. To date, treatment of congenital infection with antiviral drugs is only palliative both prior to and after birth, whereas the only efficacious preventive measure seems to be the development of a safe and immunogenic vaccine, including recombinant, subunit, DNA, and peptide-based vaccines now under investigation. The following controversial issues are discussed in the light of the most recent advances in the field: the actual perception of the problem; universal serologic screening before pregnancy; the impact of correct counseling on decision making by the couple involved; the role of prenatal diagnosis in ascertaining transmission of virus to the fetus; the impact of preconceptional and periconceptional infections on the prevalence of congenital infection; and the prevalence of congenitally infected babies born to mothers who were immune prior to pregnancy compared to the number born to mothers undergoing primary infection during pregnancy.
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PMID:Diagnosis and management of human cytomegalovirus infection in the mother, fetus, and newborn infant. 1236 75

Rubella, also known as German measles, is usually a very mild infection that can have devastating effects in certain instances. It is a pleomorphic RNA virus in the Togaviridae family of the genus Rubivirus. It typically causes a scarletiniform rash, cervical lymphadenopathy, and mild constitutional symptoms, but in older children and adults, especially women, it may be more severe, with joint involvement and purpuric rash. Infection during the first 12 weeks of pregnancy results in congenital infection and/or miscarriage in 80-90% of cases. The congenital rubella syndrome (CRS) involves multiple organ systems and has a long period of active infection and virus shedding in the postnatal period. For these reasons, the rubella vaccine program was instituted in 1969, and the incidence of rubella infection in the United States has since declined by 99%. Rubella has been recognized as a disease for approximately 200 years, and it has since been found that humans are the only natural reservoir for the rubella virus. Virus is present in nasopharyngeal secretions, blood, feces, and urine during the clinical illness, although patients with subclinical disease are also infectious. The virus is spread via oral droplets and is shed in the nasopharynx for approximately 7 days before and after the rash is visible. CRS includes a configuration of anomalies, including nerve deafness, cataracts, cardiac anomalies (usually pulmonary artery and valvular stenosis, and patent ductus arteriosis), and mental retardation, with late complications including diabetes, thyroid disease, growth hormone deficiency, and progressive panencephalitis. In 1969, the first rubella vaccine was licensed for use, and the Centers for Disease Control and Prevention (CDC) began its National Congenital Rubella Syndrome Registry. As required under the National Childhood Injury Act, all healthcare providers in the United States who administer any vaccine shall, prior to administration of the vaccine, provide a copy of the Vaccine Information Statements (VIS) produced by the CDC to the parent or legal representative of any child to whom the provider intends to administer such vaccine, or to any adult to whom the provider intends to administer such vaccine. Despite efforts to vaccinate children, CRS continues to occur in the United States. Hispanic infants have an increased risk of CRS. HIV-1infected children with a preserved immune system and MMR immunization had a good response to rubella vaccine. In contrast, those in more advanced categories for HIV infection responded poorly. Issues of risk, choice, and chance are central to the controversy over the MMR vaccine that erupted in the UK in 1998, and has continued into the new millennium. An important contribution to the MMR controversy has come from the parents of autistic children, some of whom reject the notion that this disorder is a random genetic misfortune and insist that it is, at least in part, the result of some environmental insult, such as MMR vaccinations.
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PMID:Rubella and congenital rubella (German measles). 1602 42

Cytomegalovirus (CMV) is the most common cause of congenital infection in humans. Some congenitally infected infants will develop sequelae later in life, especially sensorineural hearing loss (SNHL) and mental retardation. There is no generally accepted antiviral therapy for the treatment of symptomatic congenital CMV infections yet. We present a neonate with symptomatic congenital CMV infection, who was treated with intravenous (iv) ganciclovir (GCV) during 18 days and subsequently with oral valganciclovir (VGCV) for 5.5 months, in an attempt to prevent development of SNHL. GCV was given intravenously 10 mg/kg/day in two doses and VGCV doses ranged from 280-850 mg/m2 bidaily (bid). Our experience shows that it is not possible to give a fixed dosing regime for VGCV in neonates and that continuous adaptation of dose is necessary to achieve stable target levels of GCV and to keep the viral load in urine at undetectable level. At 18 months of age no hearing deterioration has occurred. While the current findings are encouraging, the limitations of a single case report with a relatively short follow-up emphasizes the need for further prospective randomized studies to evaluate pharmacokinetics, efficacy and safety of VGCV therapy in neonates with congenital CMV infection.
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PMID:Treatment of symptomatic congenital cytomegalovirus infection with valganciclovir. 1621 80

Cytomegalovirus (CMV) is the most common viral congenital infection, producing both sensorineural hearing loss and mental retardation. Our objective was to assess the population pharmacokinetics of a research-grade oral valganciclovir solution in neonates with symptomatic congenital CMV disease. Twenty-four neonates received 6 weeks of antiviral therapy. Ganciclovir and valganciclovir were measured by liquid chromatography/tandem mass spectroscopy. NONMEM version VI beta was used for population analyses. All profiles were consistent with a one-compartment model. Postnatal age, body surface area, and gender did not improve the model fit after body weight was taken into account. The typical value of clearance (l/h), distribution volume (l), and bioavailability of ganciclovir were 0.146 x body weight (WT)(1.68), 1.15 x WT, and 53.6%, respectively. Although these results cannot be extrapolated to extemporaneously compounded valganciclovir preparations, they provide the foundation on which a commercial-grade valganciclovir oral solution may be a viable option for administration to neonates.
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PMID:Ganciclovir population pharmacokinetics in neonates following intravenous administration of ganciclovir and oral administration of a liquid valganciclovir formulation. 1739 28

Human Cytomegalovirus (CMV) is the main cause of mental retardation and sensorineural hearing loss related to congenital infections. Justification of systematic screening for fetal CMV infection is still controversial and is not recommended in most developed countries. This is mainly justified by the paucity of antenatal prognostic factors and the lack of established intrauterine treatment when fetal infection has been diagnosed. Our aim was to review the current state of the knowledge about the CMV congenital infection and to highlight recent advances in the diagnosis as well as in the identification of prognostic factors.
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PMID:[Cytomegalovirus (CMV) congenital infection]. 1833 44

Cytomegalovirus (CMV) is the most significant infectious cause of brain disorders in humans. Although the brain is the principal target organ for CMV infection in infants with congenital infection and in immunocompromised patients, little has been known about cellular events in pathogenesis of the brain disorders. Mouse models have been developed by the authors for studying the cell tropism, infectious dynamics of CMV infection and the effects of CMV infection on proliferation, regeneration and differentiation of neural cells. It has been shown, using brain slice cultures and neurospheres, that neural stem progenitor (NSP) cells are the most susceptible to CMV infection in developing brains. The NSP cells are also susceptible to CMV infection in adult and aged brains. The susceptibility can be enhanced by stimulation of neurogenesis. It was shown that latent murine CMV infection occurs in NSP cells by demonstrating the reactivation in brain slice culture or neurospheres. It is hypothesized that CMV brain disorder such as microcephaly is caused by disturbance of cellular events in the ventricular regions, including proliferation and differentiation of the neural stem cells, whereas neurons are also targets in persistent CMV infection, presumably resulting in functional disorders such as mental retardation.
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PMID:Roles of neural stem progenitor cells in cytomegalovirus infection of the brain in mouse models. 1842 23


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