This is a 1.5Kg female baby delivered in the department of Obstetrics and Gynecology of Severance Hospital with normal spontaneous vaginal delivery at 32 weeks gestation period. In delivery room they noticed eviceration of the entire liver, small intestine and stomach through an abdominal wall defect above umbilicus without a covering membrane. Umbilicus was normally inserted at the inferior margin of the abdominal wall defect. This anomaly was diagnosed as Gastroschisis after reviewing the literature. This is the first case report of Gastroschisis in Korea.
Abdominal Muscles/*abnormalities ; Female ; Human ; Infant, Newborn ; Intestine, Large/*abnormalities ; Intestine, Small/*abnormalities ; Liver/*abnormalities ; Stomach/*abnormalities

Focal fatty change of the liver is a nodular lesion which is a rarely described and poorly characterized entity. The hepatic nodule measured 1.4cm at its maximum diameter, was subcapsular in location and occurred adjacent to the falciform ligament. Microscopically it was composed of hepatic tissue with a preserved lobular architecture. The central venous structures and portal tracts with their triads were regularly placed. The cytoplasm of almost all of the hepatocytes within the nodule was replaced by macrovesicular fat vacuoles with the nuclei displaced. Several large abnormal vessels were found at the margin of the nodule. The nodule was discovered incidentally on postmortem examination of a female infant who proved, at autopsy, to have multiple cardiac anomalies and bronchopneumonia. The possible inadequate local tissue perfusion due to abnormal intrahepatic vessels at this particular location could be augmented by multiple cardiac anomalies culminating in focal ischemia and focal fatty change. When encountered in surgery or on gross examination, it could be confused with other space occupying lesions such as liver cell adenoma, abscess and metastatic lesions.
Fatty Liver/complications ; Fatty Liver/pathology* ; Female ; Heart Defects, Congenital/complications ; Hepatic Artery/abnormalities ; Human ; Infant

Humans ; Liver ; abnormalities ; anatomy & histology ; Liver Transplantation ; methods ; Male ; Middle Aged ; Situs Inversus ; complications ; Tissue Donors

PURPOSE: The purpose of this study was to examine the clinical courses and long-term outcomes of children with Allagille syndrome in Korea, and to evaluate the prognostic potentials of identified variables. METHODS: We reviewed the clinical manifestations and outcomes of 30 children with Alagille syndrome, investigated from 1984 to 2006 until the end of this study (defined as death or last visit; mean follow-up : 5 years). RESULTS: Cholestasis occurred in 100 percent, cardiovascular abnormalities in 83.3 percent, butterfly vertebrae in 30.0 percent, posterior embryotoxon in 43.3 percent, and a characteristic facial appearance in 100 percent. At study conclusion, of these 30 patients, eight had died (26.7 percent); six related to Alagille syndrome. Five patients died of a liver disease complication. Liver transplantation was carried out in five of the 30 patients (16.7 percent) and one of these died due to hyperacute rejection. At age two, cholestasis improved in 17 of the 30 patients. Those who had severe cholestasis at 2 years of age tended to have a complication, such as liver cirrhosis or liver transplantation, or to have died. CONCLUSION: Hepatic complications account for the most mortalities in patients with Alagille syndrome. Careful and complete assessments should be made in children who have cholestasis at 2 years of age. Further investigations of more cases are required.
Alagille Syndrome* ; Butterflies ; Cardiovascular Abnormalities ; Child ; Cholestasis ; Follow-Up Studies ; Humans ; Korea* ; Liver Cirrhosis ; Liver Diseases ; Liver Transplantation ; Mortality ; Spine

Cavernous transformation of the portal vein that is resulted as a collateral vessel formation from a portal hypoplasia is very rarely to be observed in human being. Such an abnormal transformation can be caused by congenital defect of vein formation in the early embryonal development or congenital hematologic hypercoagulability which gives rise to the cavernous development of cavernous collateral branches. Acute thrombosis of portal vein can also be the cause of acquired cavernous transformation. In most cases it extends into the parenchyme of liver, and often has porto-systemic shunt (with left gastropiploic vein and varices around spleen) or porto-portal shunt (with varices around gallbladder). A 48 years old male patient underwent splenectomy at the age of 24 owing to splenomegaly. He had liver cirrhosis without hepatitis and alcohol history. Intrahepatic stone in left lateral lobe of liver was diagnosed in the course of medical treatment. The cavernous transformation of portal vein with right lobe hypoplasia was confrimed during the operation.
Congenital Abnormalities ; Hepatitis ; Humans ; Liver Cirrhosis ; Liver* ; Male ; Middle Aged ; Portal Vein* ; Splenectomy ; Splenomegaly ; Thrombophilia ; Thrombosis ; Varicose Veins ; Veins

Meckel-Gruber syndrome is a congenital disorder characterized by occipital encephalocele, bilateral multicystic kidneys with fibrotic changes of the liver and postaxial polydactyly. Other anomalies are frequently associated. This syndrome is a rare autosomal recessive disorder often resulting in neonatal death within a few hours of life and has the high recurrence(one in four) in future pregnancies. We present the case of Meckel-Gruber syndrome diagnosed by ultrasonography at 18 weeks` gestation with a brief literature review.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Encephalocele ; Liver ; Multicystic Dysplastic Kidney ; Polydactyly ; Pregnancy* ; Prenatal Diagnosis ; Ultrasonography*

A 4-month-old female Holstein Friesian calf was referred to the Veterinary Teaching Hospital, University of Berne, Switzerland for evaluation of ataxia, weakness, apathy and stunted growth. Clinical examination revealed generalized ataxia, propioceptive deficits, decreased menace response and sensibility. Postmortem examination did not reveal macroscopic changes of major organs. Histologically, the brain and the spinal cord lesions were characterized by polymicrocavitation, preferentially affecting the white matter fibers at the junction of grey and white matter and by the presence of Alzheimer type II cells. The liver revealed lesions consistent with a congenital portosystemic shunt, characterized by increased numbers of arteriolar profiles and hypoplasia to absence of portal veins. The pathological investigations along with the animal history and clinical signs indicated a hepatic encephalomyelopathy due to a congenital portosystemic shunt.
Animals ; Cattle ; Cattle Diseases/*congenital ; Cerebrum/pathology ; Female ; Hepatic Encephalopathy/*veterinary ; Liver/pathology ; Portal System/*abnormalities

No abstract available.
Hepatic Encephalopathy/*congenital ; Humans ; Liver Neoplasms/*congenital ; Male ; Portal Vein/*abnormalities ; *Septal Occluder Device

OBJECTIVE: The maintenance of the correction of kyphotic deformity is one of the difficult problem in tuberculous spondylitis after anterior debriment and fusion with tricortical bone graft. The goal of this study is to find out the efficacy of titanium mesh cage impacted with autogenous bone chip in tuberculous spondylitis treated with anterior intervertebral fusion. MATERIALS AND METHODS: Twelve patients were treated with anterior intervertebral fusion using titanium mesh cage for tuberculous spondylitis from January 1996 to June 1999. We analized the changes in the correction of kyphotic deformity, changes of ESR and CRP, fusion state and recurrence after anterior intervertebral fusion with titanium mesh cage. RESULTS: Clinical symptoms were improved in all twelve patients without any neurologic complications. The mean kyphotic angle corrected was 7.3 degrees immediately after operation, but the loss of correction of kyphotic angle was 2.2 degrees after 3 months and 2.6 degrees after 6 months. We found that the loss of correction of kyphotic deformity occurred mainly within the first 3 months after surgery. Only one patient, suffered from acute hepatic failure after first operation and had an insufficient anti-tuberculous medication therapy, showed recurrence of tuberculous spondylitis after 6 months. The patient underwent a second operation with posterior fixation procedure with good outcome. The changes of ESR and CRP were not specifically important factor to reveal recurrence of tuberculosis of the spine in our series. CONCLUSION: The surgical procedure of tuberculous spondylitis using titanium mesh cage with bone chip seems to be an effective procedure to minimize loss of the correction of kyphotic deformity without any aggravating inflammatory change and recurrence with titanium mesh cage, when sufficient debridement and anti-tuberculous chemotherapy are achieved.
Congenital Abnormalities ; Debridement ; Drug Therapy ; Humans ; Liver Failure, Acute ; Recurrence ; Spine ; Spondylitis* ; Titanium* ; Transplants ; Tuberculosis

Primary biliary cirrhosis is a chronic, progressive liver disease characterized by inflammatory destruction of septal and intralobular bile ducts which results in intrahepatic cholestasis. Although the cause remains obscure, it is frequently associated with a variety of disorders presumed to be autoimmune in nature. We report a case of early primary biliary cirrhosis which was anteceded by rheumatoid arthritis. The patient was a 54-year-old female who was admitted due to arthralgia and joint deformity. She had been diagnosed as having rheumatoid arthritis 10 years before. On admission, she had elevated serum ALT, AST, alkaline phosphatase, gamma-GTP and positive serum antimitochondrial antibody test. Microscopic findings of the liver were consistent with the early stage of primary bi]iary cirrhosis.
Alkaline Phosphatase ; Arthralgia ; Arthritis, Rheumatoid* ; Bile Ducts ; Cholestasis, Intrahepatic ; Congenital Abnormalities ; Female ; Fibrosis ; Humans ; Joints ; Liver ; Liver Cirrhosis, Biliary* ; Liver Diseases ; Middle Aged