Liver fibrosis in an extremely small infant for gestational age.
Premature infants with intrauterine growth restriction (IUGR) are at greater risk for an adverse perinatal outcome. IUGR affects hepatocyte function, but the histopathological changes in the postnatal liver are not well known. We report a male infant with severe IUGR. His mother was transferred to our hospital at 26 weeks of gestation because of preterm labor and severe IUGR. An emergency cesarean section was carried out because of a non-reassuring fetal status. The birth weight of the infant was 332 g. He showed congestive heart failure and marked hepatomegaly from birth. After 1 week, blood examinations showed hyperbilirubinemia with high direct bilirubin. Because of liver dysfunction, he received the minimal total parenteral nutrition for 7 days. After 1 month, he progressively developed ascites and coagulopathy, and died 3 months after birth. Liver autopsy showed diffuse perisinusoidal fibrosis. Fibrosis was also prominent around the central vein. Immunohistochemical study revealed many alpha-smooth muscle actin-positive cells, which represent activated hepatic stellate cells, and a few transforming growth factor-beta1-positive cells in the perisinusoidal fibrotic area. These results indicate that the infant developed chronic (end stage) liver failure by 3 months of age. We excluded congenital infection, metabolic syndrome and citrin deficiency. It is therefore conceivable that intrauterine cardiac failure may be responsible for liver fibrosis. Early detection of liver dysfunction soon after birth is a key to predict the prognosis of severe IUGR in preterm infants.
Department of Pediatrics, Akita University Graduate School of Medicine, Akita, Japan. arahiro@med.akita-u.ac.jp
Hirokazu
H
Arai
Atsuko
A
Noguchi
Ryoji
R
Goto
Takefumi
T
Matsuda
Hatsushi
H
Nakajima
Tsutomu
T
Takahashi
D000328:Adult; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006932:Hyperbilirubinemia / Q000097:blood; D007150:Immunohistochemistry; D052577:Infant, Extremely Low Birth Weight / Q000097:blood; D007231:Infant, Newborn; D007235:Infant, Premature, Diseases / Q000097:blood / Q000000981:diagnostic imaging / Q000503:physiopathology*; D007236:Infant, Small for Gestational Age / Q000097:blood; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000097:blood / Q000151:congenital* / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008297:Male; D011247:Pregnancy; D016216:Ultrasonography, Prenatal