Journal of Pathology and Translational Medicine

The Effects of Immunosuppressant and Immunostimulant on the Splenic Cell Subset of Rats Having Undergone Experimentally Induced Septal Fibrosis of Liver.: Mee Young Sol, Joon Yeon Kim, Sun Kyoung Lee; Korean J Pathol. 1995;29(5):572-583.

Abstract: Although there have been many reports about the importance of the spleen's role in hepatic fibrogenesis, the exact mechanism is still uncertain. The author designed this study to evaluate splenic function on hepatic fibrogenesis. The degree of hepatic fibrosis and the population of splenocyte subsets were studied in the experimental animal model with fibrosis produced by injecting normal swine serum intra-peritoneally into Sprague-Dawley rats. The animals were divided into three groups; group A was subjected to injection of swine serum only, group B swine serum and complete Freund's adjuvant and group C swine serum and cyclosporin A. The experimental hepatic fibrogenesis by swine serum was augumented by coinjection with the adjuvant and inhibited by cyclosporin A. The study of the splenocyte subset revealed increased percentages of spienic B cell and CD4+ cell and a decreased percentage of CD8+ cell, and these changes of splenocyte subset were also augumented by the adjuvant and inhibited by cyclosporin A. The percent of monocytes was not significantly altered, although a tendancy of early decrease by the adjuvant was noted.

Congenital Hepatic Fibrosis with Caroli's Disease.: Yoon Jung Kim, Soon Ae Oak, In Chul Lee; Korean J Pathol. 1997;31(3):275-279.

Abstract PDF: Congenital hepatic fibrosis is an inherited, congenital disorder of the liver, and is occasionally associated with cystic disease of the liver and kidney. We present a case of congenital hepatic fibrosis with Caroli's disease. A 21-year-old woman had suffered from an episodic fever with headaches for 3 years. In laboratory examination, the liver function test was within the normal limits. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and multiple dilated bile ducts were seen by abdominal CT scanning. An orthotopic whole liver transplantation was done. The liver was fibrotic and enlarged. Multiple cystically dilated intrahepatic ducts were noted. Microscopically, diffuse portal fibrosis and widening with proliferation of bile ductules were seen. Intrahepatic bile ducts were markedly dilated and tortuous. The liver cell cords were well preserved.

Meckel-Gruber Syndrome: An autopsy case report.: Kyung Ha Kang, Duck Hwan Kim, Hee Jin Chang, In Sook Kim, Jin Hee Sohn, Jung Il Suh; Korean J Pathol. 1994;28(2):200-202.

Abstract PDF: Meckel-Gruber syndrome is a quite rare congenital disorder, characterized by posterior encepalocele, cleft palate and lip, polycystic kidneys, hepatic fibrosis with bile duct proliferation, and postaxial polydactyly and syndactyly. We experienced an autopsy case of M eckel-Gruber syndrome in a second baby of 28 year-old woman. At 26 weeks of gestation, congenital anomaly was detected on ultrasonographic examination and the pregnancy was terminated. Familial history was not noted.

Congenital Hepatic Fibrosis: A case report.: Weon Young Choi, Sun Hee Yoon, In Sook Lim, Ha Jin Choi; Korean J Pathol. 1991;25(1):50-53.

Abstract PDF: Congenital hepatic fibrosis is an uncommon disease of children and young adults with two major risks: gastrointestinal hemorrhage caused by portal hypertension, and cholangitis related to bacterial infection of dilated intrahepatic bile ducts. It is characterizeed by stony hard hepatomegaly and portal hypertension with rather well preserved hepatic function and architecture, and frequent association of the renal lesions. We have recently experienced a case of congenital hepatic fibrosis in a 24 year-old Korean male. The chief complaint was hematemesis from esophageal varices. There were marked hepatosplenomegaly, mild pancytopenia and the liver function test was within normal engorgement and dilatation of portal and splenic veins and multiple cysts of both kidneys.

Meckel-Gruber Syndrome: An autopsy case.: Soong Deok Lee, Chul Woo Kim, Je Geun Chi; Korean J Pathol. 1988;22(4):505-509.

Abstract PDF: We report an autopsy of a male fetus that showed multiple congenital anomalies that could best be designated as Meckel-Gruber syndrome. The fetus was born dead at the gestational age of 38 weeks. His parents denied any history of congenital malformation. And the parity of the mother was 0-0-0-0, but she had the past history of receiving herb medication for common cold. The congenital anomalies found in this case consited of occipital meningoencephalocele, midline cleft palate, bifid epiglottis, hepatic fibrosis, choledochal cyst, bilateral polycystic kidneys, postaxial polydactyly of both hands and feet, aplasia of the left testis, secundum type atrial septal defect and patent ductus arterious. This malformation syndrome is rare and lethal. The prenatal diagnosis should be made by ultrasound study or analysis of the amniotic fluid for alpha-feto protein during intrauterine period. The kidneys showed Potter type III cystic change and there was a characteristic hepatic fibrosis.

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