Neonatal hepatitis and extrahepatic biliary atresia associated with cytomegalovirus in twins

Article Abstract:

Interwoven among liver cells are canals that carry bile from the liver, where the bile is produced, to the bile ducts and gallbladder where the bile is stored. Atresia (the absence or closure) of bile canals and ducts may occur within the liver or extrahepatically, outside liver. Extrahepatic biliary atresia (EHBA) occurs in 1 of 10,000 live births, has several causes, and is part of a disease continuum that includes neonatal hepatitis. Cytomegalovirus (CMV) infection is a common congenital (at birth) viral infection, which is usually asymptomatic. This article describes twins infected with CMV in utero, one of whom developed neonatal hepatitis, while the other developed EHBA following birth. The twins were not identical, and differences in immune-related cells likely contributed to the different expression of liver disease in the two infants. The article supports an association between CMV infection and liver disease, but does not prove that the former caused the latter. The first twin had hyperbilirubinemia, high levels of bilirubin resulting in yellow skin color (jaundice), the second day after birth, and studies confirmed hepatitis with inflammation and fibrosis of some liver cells. The patient was normal at one year of age. The second twin required surgical repair of birth defects on the first day of life, and developed hyperbilirubinemia on the second day. Studies showed a normal gall bladder and normal bile ducts, but inflammation of liver cells. However, by day 27, there was poor bile excretion and the extrahepatic bile structures were found to be obliterated. Cholestasis, halted bile flow, resulted in accumulation of bile within liver cells. The patient continued to have high blood bilirubin levels at two months, and at four months had a CMV-related severe lung inflammation. The patient received a liver transplant at two years due to end-stage liver disease. Findings from these cases are discussed. (Consumer Summary produced by Reliance Medical Information, Inc.)

Care and treatment, Causes of, Biliary atresia, Hepatitis, Neonatal, Neonatal hepatitis

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Asymptomatic congenital cytomegalovirus infection: audiologic, neuroradiologic, and neurodevelopmental abnormalities during the first year

Article Abstract:

Cytomegalovirus (CMV) is a common cause of infections. It is the most frequent cause of congenital infections (those present at birth), but most infected newborns do not show any clinical signs of infection, and are identified through screening programs for the virus. Sensorineural hearing loss (defect in the inner ear that causes sound distortion) has been found in older children with CMV infection who are otherwise asymptomatic (without symptoms). A study was undertaken to determine the incidence of congenital sensorineural hearing loss in 28 babies with asymptomatic congenital CMV infection, as compared with 13 uninfected normal babies. The usefulness of computed tomographic (CT) scanning in identification of asymptomatic infants at risk for delayed neurologic development was also investigated. Sensorineural hearing loss was able to be documented (by auditory brain-stem responses) in four CMV-infected babies, but in none of the controls. Four infected babies had CT scans showing diffuse periventricular radiolucencies. No differences between the two groups were noted on neurological examination or on an index of mental development. One infected infant demonstrated an index score indicative of delayed psychomotor development. The average mental development index score of the four babies with CT abnormalities was significantly below that of the other CMV-infected babies. These findings suggest that asymptomatic congenital CMV infection may be associated with a variety of audiologic and neurologic differences in infancy. (Consumer Summary produced by Reliance Medical Information, Inc.)

Infection, Risk factors, Pediatric diseases, Hearing disorders, Infection in children

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The pattern of growth failure in Rett syndrome

Article Abstract:

One of the first indicators that a girl has Rett syndrome (RS) is a deceleration of head growth. This is often followed by substandard weight gain and growth. Rett syndrome occurs only in females, and the children often appear normal at birth. The children eventually develop mental disabilities, communication problems and loss of purposeful hand movements. A longitudinal study of 96 patients diagnosed with RS tracked their weight, height and fronto-occipital head circumference. The findings were plotted against a normal growth curve. Changes in some children's growth patterns started as early as three months of age. The absence of pre- or post-natal trauma combined with deviation from normal growth patterns should alert clinicians to the possibility of Rett syndrome in their female patients.

Diagnosis, Rett syndrome

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