Cytomegalovirus infection in twin pregnancy and coincidence of type i tyrosinemia in one of twins

Cytomegalovirus [CMV] is the most common cause of congenital infection. Although most of the involved neonates are asymptomatic but virus can cause a range of problems from mild to severe illness with involvement of different organs like central nervous system, gastrointestinal and liver. Proneness to CMV is very high [up to 1% of neonates]. In the other hand trosinemia type I is a rare metabolic disorder with involvement of liver, neurologic, bone and other organs. A 3-month-old infant, product of twin pregnancy was hospitalized because of jaundice, FTT, hepatomegaly and sepsis. The other twin showed normal growth with no problems. Work up for cholestasis and FTT was suggestive of two different entities. Polymerase chain reaction for CMV in liver tissue and serum was positive. Meanwhile laboratory findings for metabolic disorder led to the diagnosis of type 1 tyrosinemia. The other twin was infected with CMV too. Although coexistence of two causes for cholestasis is very rare, it is sometimes necessary to study more to rule out other entities like metabolic disease including tyrosinemia [if any symptoms exist]

Embryonal biliary atresia with levocardia and situs inversus: a case report

Biliary atresia [BA] is a rare disease and the end result of a destructive inflammatory process in bile ducts, leading to fibrosis and liver cirrhosis. It has two forms: 1] syndromic or fetal or embryonic [10-35%] with various congenital anomalies, 2] non syndromic [70-90%], in which BA is an isolated anomaly. In this article we report on an infant with the first form of BA in which diagnosis and operation was not based on routine liver biopsy but on associated malformations and clinical features that were highly suggestive of embryonal form of biliary atresia. A 70-day old infant with syndromic BA, levocardia, situs inversus and polysplenia. He developed jaundice in 4th day of life, liver was not palpable. Kasai operation was not effective. He developed liver cirrhosis at 3 months of age. Syndromic type of EHBA is a very rare disease with a worse outcome than non syndromic type of BA. Early diagnosis is important and may be difficult as the liver sometimes could not be palpable because of its malposition