Journal of Pathology and Translational Medicine

Case Reports

Plexogenic Pulmonary Arteriopathy in Congenital Heart Disease: A Report of Two Cases.: Seung Yeon Ha, Kook Yang Park, Hyun Yee Cho, Young Ha Oh, Jae Gul Chung, Dong Hae Chung, Chung Yeul Kim, Han Kyeom Kim; Korean J Pathol. 2002;36(6):412-415.

Abstract PDF: Hypertensive pulmonary vascular disease can develop in those cases of congenital cardiac shunt in which critical levels of pulmonary artery pressure and flow are reached and exceeded. We have experienced two cases of plexogenic arteriopathy in complex congenital heart disease and tried to evaluate of distribution of arterial lesions by total mapping of the explanted lung. Case 1 and 2 were 12-year-old boy and 36 year-old man. They were treated with combined heart-lung transplantation. Mapping of the both lungs was done, and graded according to Heath and Edward's grading scheme. The elastic pulmonary artery was tortuous, dilated and aortic configuration. Both lungs showed mostly grade 3. Plexiform lesion or veinlike branches of hypertrophied muscular arteries arosed in a lateral branch of a muscular artery that might be proximal to an area of occlusion. Comprising the right and left lung, the right was more severe than the left. By getting closer to the distal part, the grade tended to increase to 4 to 5. By analyzing the pulmonary lobe, severe pulmonary hypertension of grade 4 or 5 was comparatively disseminated throughout the right lung. On the other hand, in the left lung, the grade of the lower lobe was higher than that of the upper lobe, and within the upper lobe, there was a tendency for the grade of inferior segment to be higher than that of the corresponding apical segment.

Alagille Syndrome: A Case Report.: Hyosup Shim, Chanil Park, Soon Il Kim, Young Nyun Park; Korean J Pathol. 2004;38(1):56-59.

Abstract PDF: Alagille syndrome is a rare autosomal dominant disorder showing complicated systemic manifestations, although the hepatic manifestations are predominant in many patients. We report a case of Alagille syndrome in a male baby who presented with a heart murmur at 2 days old and his echocardiography showed aortic stenosis. He presented with acholic stool and jaundice at 3 months old and a liver biopsy revealed paucity of the interlobular bile duct in the portal tract. This progressed to cirrhosis, for which a liver transplantation was performed at 10 months old. The explanted liver showed biliary-type cirrhosis with severe cholestasis. There was an absence of the interlobular bile ducts on microscopic examination. Bile duct paucity, associated with cholestasis, a peculiar face (prominent forehead, deep-set eyes, pointed mandible and bulbous nasal tip), and cardiac anomaly were observed, which were consistent with Alagille syndrome. He died of heart failure.

Sirenomelia: An autopsy case report.: Yeong Ju Woo, Hye Kyoung Yoon, In Sook Lim; Korean J Pathol. 1994;28(1):96-98.

Abstract PDF: Sirenomelia is a severe form of caudal regression syndrome that results in a fusion of the lower extremities, which is not compatible with life. A various spectrum of anomalies affecting primarily the musculoskeletal, genitourinary and gastrointestinal systems also can occur. This rare malformation has a reported incidence rate of approximately 1 in 60,000 births, with a range of 1 to 1 percent of all malformed infants. We experienced a sirenomelic case with combined anomalies of genitourinary, cardiovascular and gastrointestinal systems. Maternal obstetric history revealed oligohydramnios and intrauterine fetal growth retardation, and the baby was spontaneously delivered at 37 weeks of gestational age, but died I hour after birth.

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