Congenital hepatic fibrosis (CHF) is a developmental disorder of the biliary system, characterized by defective remodeling of the ductal plate. Herein a family of three children, from consanguineous parents, with minor thalassemia is presented who suffered from congenital hepatic fibrosis (CHF). Prompt diagnosis and appropriate treatment are necessary to avoid further complications in the affec...

This study describes a 34-year-old pregnant woman who underwent prenatal echocardiography at 24 weeks of gestation and found fetal heart abnormalities. She in our hospital. Echocardiography showed complex malformation heart, thickening calcification left ventricular myocardium tendon, weakening or even flattening wall movement, very severe mitral stenosis, regurgitation, aortic narrowing the ar...

In 2014, a published study based on dissections and skeletal examinations noted that 19:50 Thoroughbred horses had a congenital malformation of the 6th cervical vertebra (C6). In addition, it was found that in those 19 Thoroughbred horses expressing a congenital malformation of C6, 9 displayed a concurrent congenital malformation of the 7th cervical vertebra (C7). In this study, 3 Thoroughbred ...

some limitations and it is unable to detect small abnormalities beyond the imaging resolution of the apparatus. Ventricular septal defects (VSDs) can only be detected if they are sufficiently large or are associated with certain anatomical malformation (Magherini et al., 1983). Accurate demonstration of a patent ductus arteriosus (PDA) is limited due to echo dropout which makes visualisation of...

Medullary sponge kidney (MSK) is associated with recurrent stone formation, but the clinical phenotype is unclear because patients with other disorders may be incorrectly labeled MSK. We studied 12 patients with histologic findings pathognomonic of MSK. All patients had an endoscopically recognizable pattern of papillary malformation, which may be segmental or diffuse. Affected papillae are enl...

CT and MR revealed a case of nasopharyngeal atresia, a malformation in which the soft palate is not formed, and the hard palate extends posteriorly to fuse with the anterior surface of the clivus, resulting in complete isolation of the nasal and oral cavities and the absence of a nasopharynx. We believe this rare anomaly results from abnormal persistence of the embryologic bucconasal plate and/...

UNLABELLED Background and rationale for the study. The aim of the study was to determine the prognostic value of histopathological findings with special care to the severity of liver fibrosis at the moment of hepatoportoenterostomy (HPE) in children with biliary atresia (BA). We performed analysis of 142 wedge liver biopsies taken at the time of HPE. All patients were operated by the same surgi...

A 1-year-old girl had pancreaticobiliary maljunction, a choledochal cyst, and polycystic kidney. At the age of 4 years, she was treated by resection of the choledochal cyst and Roux-en-Y reconstruction because of the cyst's risk of cancer. She was diagnosed as having congenital hepatic fibrosis based on the histological findings. Postoperatively, she suffered recurrent fever of unknown origin, ...