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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cytomegalovirus (CMV) infection is the most common cause of congenital infection in the developed world, occurring in approximately 1% of all liveborns. Symptomatic disease occurs in 10% of all congenitally infected infants, resulting in a spectrum of clinical manifestations that include microcephaly, chorioretinitis, hepatosplenomegaly and sensorineural hearing loss, among others. Even those children who are asymptomatic at birth have a risk of hearing loss, with approximately 8% experiencing this sequela. Overall, congenital CMV infection accounts for one-third of all cases of sensorineural hearing loss. The economic burden of disease exceeds $2 billion annually in the USA. Therefore, this infection has been the target for antiviral therapy. Studies performed by the National Institute of Allergy and Infectious Diseases Collaborative Antiviral Study Group (CASG) have evaluated ganciclovir for the treatment of symptomatic congenital CMV infection with central nervous system involvement. In a randomized, controlled clinical trial of ganciclovir treatment (6 mg/kg iv every 12 h for 6 weeks) brainstem-evoked responses were utilized as the primary endpoint and demonstrated stabilization of hearing both at 6 months and >1 year. Treatment was associated with neutropenia in over 60% of treated patients. Since ganciclovir must be given intravenously, studies with its prodrug, valganciclovir, have been performed to assess pharmacokinetics and pharmacodynamics. Currently, a clinical trial of 6 weeks versus 6 months of valganciclovir is being performed by the CASG. Notably, only intravenous ganciclovir and orally administered valganciclovir have been used to treat congenital CMV infection. Hopefully, other drugs such as maribavir will be available for evaluation in this population.
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PMID:Treatment of congenital cytomegalovirus infection: implications for future therapeutic strategies. 1928 11

A preterm infant with very low birth weight was born with fetal onset familial hemophagocytic lymphohistiocytosis. Known gene abnormalities responsible for the disease were not identified in the patient. The infant died at 13 months of age owing to complications from cord blood stem cell transplantation. We found selectively elevated expression of interleukin-6 and chemokines in the cord blood of the patient. We also reviewed 7 other preterm cases of congenital hemophagocytic lymphohistiocytosis to highlight the significance of this condition, as it can cause ascites and hepatosplenomegaly in utero and be mistaken for congenital infection in the fetus.
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PMID:Congenital hemophagocytic lymphohistiocytosis in a preterm infant: cytokine profile and a review of the disease. 2161 64

Cytomegalovirus (CMV) is currently the most common cause of congenital infection and the leading infectious cause of brain damage and hearing loss in children. Perinatal CMV infection rarely causes clinical manifestations in normal individuals and usually follows a benign course in immunocompetent infants. However, ~15-25% of infected preterm infants may develop pneumonia, hepatitis or sepsis-like illness, bradycardia, hepatosplenomegaly, distended bowel, anemia, or thrombocytopenia. Bronchiolitis obliterans (BO) is a rare, fibrosing form of chronic obstructive lung disease that follows severe insults to the lower respiratory tract and results in narrowing and/or complete obliteration of the small airways. In non-transplant children, the most common form of BO is a severe lower respiratory tract infection, especially of adenovirus. We experienced a case of a 37-day-old male who was diagnosed as BO on chest computed tomography (CT) after CMV pneumonia. To our best knowledge, this is the first case of BO caused by CMV pneumonia in a healthy infant.
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PMID:Bronchiolitis obliterans caused by CMV in a previously healthy Asian infant. 2534 9

TORCH refers to the most common congenitally acquired infections: toxoplasma, rubella, cytomegalovirus, and herpes simplex virus. Neonatal cytomegalovirus infection remains a common cause of congenital infection worldwide with effects ranging from hearing impairment to significant neurological morbidity. We report a case of a term neonate with ventriculomegaly on prenatal ultrasound who presented with low birth weight, small head circumference, hepatosplenomegaly, and purpuric rash on physical exam. Central nervous system cytomegalovirus infection typically shows periventricular calcifications and associated deep white matter damage and ventriculomegaly. Ultrasound, computed tomography, and magnetic resonance imaging have different roles in the diagnosis of congenital central nervous system cytomegalovirus infection. Many imaging features of congenital cytomegalovirus are distinctive, and can spur a diagnostic work-up as well as help provide a prognosis.
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PMID:Diffuse periventricular calcification and brain atrophy: A case of neonatal central nervous system cytomegalovirus infection. 2753 61

Maternofoetal infection with Cytomegalovirus (CMV) is the most common congenital infection and a leading cause of mental retardation and sensori-neural hearing loss. Population-based studies indicate that at least 0.5% of all infants born alive have CMV of whom approximately 10% have clinically evident symptomsat birth. The Justification of systematic screening for foetal 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 foetal infection has been diagnosed. In case of congenital CMV infection, infants can be symptomatic or asymptomatic at birth. Mortality for such infants can reach 30%, and survivors can have mental retardation, sensorineural hearing loss, chorioretinitis, and other significant medical problems. A newborn symptomatic is defined by the existence of clinical and / or biological signs and / or neonatal imaging, the most frequent clinical signs are: hepatosplenomegaly (60%), microcephaly (53%), jaundice (67%), petechiae (76%), at least one neurological abnormality (68%). The frequency of biological abnormalities is as follows: increase in transaminases (83%), thrombocytopenia (77%), hyperbilirubinemia (69%), haemolysis (51%), hyperproteinorrachy (46%). The abnormalities of neonatal imaging are present in 70% of symptomatic newborns; intracerebral calcifications are the most frequent abnormalities. We report a case of newborn who presented a congenital infection by CMV, evoked on the intrauterine growth retardation, organs of the reticulo endothelial and haematological system were reached while nervous system was spared, and CMV PCR was very positive. indicating an antiviral treatment for 6weeks based on ganciclovir.
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PMID:Severe neonatal cytomegalovirus infection: about a case. 2890 89

Among all other viruses, human cytomegalovirus (HCMV) is the most frequent cause of congenital infection worldwide. Strain variation in HCMV may predict severity or outcome of congenital HCMV disease. Previous studies have associated a particular genotype with specific sequelae or more severe illness, but the results were contradictory. There are no previous studies addressing the genotype of HCMV in Iraq. Therefore, the present study is aimed at molecular detection and genotyping of HCMV isolated from symptomatic congenitally/perinatally infected neonates. This prospective study comprised 24 serum samples from symptomatic neonates with congenital/perinatal infection. Viral DNA was extracted from these serum samples; nested polymerase chain reaction was used to amplify the HCMV gB (UL55) gene. Polymerase chain reaction products of the second round of amplification were subjected to direct Sanger sequencing. Bioedit and MEGA5 software (EMBL-EBI, Hinxton, Cambridgeshire, UK) were used for alignment and construction of a phylogenetic tree. Human cytomegalovirus DNA was detected in 23 of 24 samples (95.8%). According to the phylogenetic analysis, three genotypes of the virus were identified; gB1, gB2, and gB3 genotypes. However, the gB4 genotype was not detected. Human cytomegalovirus gB3 was the most frequent genotype: 14 of 24 (58.33%) among symptomatic infected infants, followed by gB1 (6/24; 25%) and gB2 (4/24; 16.67%). A mixed HCMV infection with gB3/gB1 was detected in only one case. Human cytomegalovirus gB3 was the most predominant genotype among symptomatic congenitally/perinatally HCMV-infected neonates. No association was found between B3 genotype and specific clinical presentation. Jaundice was the most common clinical feature among symptomatically infected neonates, followed by hepatosplenomegaly.
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PMID:Genotyping of Cytomegalovirus from Symptomatic Infected Neonates in Iraq. 3081 Jan 4

Congenital infections refer to a group of perinatal infections that may have similar clinical presentations, including rash and ocular findings. TORCH is the acronym that covers these infections (toxoplasmosis, other [syphilis], rubella, cytomegalovirus, herpes simplex virus). There are, however, other important causes of intrauterine/perinatal infections, including enteroviruses, varicella zoster virus, Zika virus, and parvovirus B19. Intrauterine and perinatal infections are significant causes of fetal and neonatal mortality and important contributors to childhood morbidity. A high index of suspicion for congenital infections and awareness of the prominent features of the most common congenital infections can help to facilitate early diagnosis, tailor appropriate diagnostic evaluation, and if appropriate, initiate early treatments. In the absence of maternal laboratory results diagnostic of intrauterine infections, congenital infections should be suspected in newborns with certain clinical features or combinations of clinical features, including hydrops fetalis, microcephaly, seizures, cataract, hearing loss, congenital heart disease, hepatosplenomegaly, jaundice, or rash. Primary prevention of maternal infections during pregnancy is the cornerstone of prevention of congenital infection. Available resources should focus on the promotion of public health.
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PMID:Congenital infections in Hong Kong: an overview of TORCH. 3253 15

Congenital infections refer to a group of perinatal infections that are caused by pathogens transmitted from mother to child during pregnancy (transplacentally) or delivery (peripartum) which may have similar clinical presentations, including rash and ocular findings. TORCH is the acronym that covers these infections (toxoplasmosis, other [syphilis], rubella, cytomegalovirus, herpes simplex virus). Other important causes of intrauterine/perinatal infection include human immunodeficiency virus, varicella-zoster virus, Treponema pallidum, Zika virus, and parvovirus B19. This overview aims to describe various congenital infections beyond TORCH with a Hong Kong perspective. Intrauterine and perinatal infections are a major cause of in utero death and neonatal mortality, and an important contributor to childhood morbidity. A high index of suspicion for congenital infections and awareness of the prominent features of the most common congenital infections can help to facilitate early diagnosis, tailor appropriate diagnostic evaluation, and initiate appropriate early treatment. Intrauterine infections should be suspected in newborns with clinical features including microcephaly, seizures, cataract, hearing loss, congenital heart disease, hepatosplenomegaly, small for gestational age, and/or rash. Primary prevention of maternal infections during pregnancy is key to the prevention of congenital infection, and resources (if available) should focus on public health promotion and pre-marital counselling.
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PMID:Congenital infections in Hong Kong: beyond TORCH. 3270 96


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