A case of primary hepatic epithelioid hemangioendothelioma with spontaneous rupture / 대한간학회지

Primary hepatic epithelioid hemangioendothelioma is a rare neoplasm of endothelial origin. The clinical manifestations are nonspecific, ranging from complete absence of symptoms to hepatic failure and death. Spontaneous rupture of a hepatic epithelioid hemangioendothelioma is an extremely rare presentation. We present a case of primary hepatic epithelioid hemangioendothelioma in a 65-year-old male patient with alcoholic liver cirrhosis. He was hospitalized due to epigastric pain and multiple liver masses on abdominal ultrasound. Dynamic liver CT imaging revealed multiple peripheral nodular enhanced mass lesions with delayed centripetal enhancement, and the adjacent collection of high-attenuation fluid along the liver capsule. Abdominal tapping revealed blood in the peritoneal cavity. Primary hepatic epithelioid hemangioendothelioma with spontaneous rupture was finally diagnosed based on a histopathologic examination revealing positive immunohistochemical staining for CD34.

A case of undifferentiated carcinoma with osteoclast-like giant cells of the pancreas / 대한내과학회지

An osteoclast-like giant cell tumor of the pancreas is a very rare neoplasm, with only three cases reported in Korea. Due to the rarity of this tumor type, few clinical data are available. We present a case of undifferentiated carcinoma with osteoclast-like giant cell tumor arising in the tail of the pancreas in a 72-year-old woman hospitalized to evaluate epigastric pain and a palpable abdominal mass. Magnetic resonance imaging revealed the presence of a large enhancing mass with septation arising from the tail of the pancreas. A distal pancreatectomy with splenectomy was performed. The pathological diagnosis was undifferentiated carcinoma with osteoclast-like giant cell tumor. Here, we describe the histopathological and immunohistochemical findings and review the clinical features of the cases reported in the Korean literature.

A Case of Surgical Treatment of Tuberculous Cholangitis and Lymphadenitis with Obstructive Jaundice due to Progressive Stricture of Bile Duct / 대한소화기내시경학회지

Obstructive jaundice is most commonly attributed to a malignancy or stones affecting the common bile duct. Biliary tuberculosis and lymphadenitis around the periportal area have also been implicated but cases are quite rare. A 24 year old man presented with jaundice and abdominal pain for 3 days. Abdominal CT and ERCP revealed a stricture of the extrahepatic bile duct with multiple enlarged lymph nodes showing necrotic foci located at the periportal area. The colonoscopic biopsy showed evidence of M. tuberculosis. The patient was treated with ERBD insertion and oral anti-tuberculosis therapy. However, the abdominal pain recurred and there was progressive stenosis of the common bile duct. A bile duct resection with choledochojejunostomy was subsequently performed. Frozen sections revealed granulomatous inflammation with caseation necrosis, which was consistent with tuberculosis. We report a case of tuberculous cholangitis and lymphadenitis with obstructive jaundice that was managed surgically due to the progressive stricture of the bile duct.

A Case of Spondylodiskitis Caused by Injection Site Infection with Methicillin-Resistant Staphylococcus aureus / 감염과화학요법

Infections of the intervertebral disc and adjacent vertebrae may present with spondylitis, discitis and spondylodiscitis and are hematogenous origin in most cases. Potential sources of hematogenous infection are skin and soft tissue infection, genitourinary tract infection, infective endocarditis, intravenous drug abuse, respiratory tract infection and infected intravenous injection site. We have experienced a case of pyogenic spondylitis caused by methicillin-resistant Staphylococcus aureus (MRSA) bacteremia with peripheral injection site infection. He was successfully treated with surgical debridement and antibiotics.

A Case of Spondylodiskitis Caused by Injection Site Infection with Methicillin-Resistant Staphylococcus aureus / 감염과화학요법

Infections of the intervertebral disc and adjacent vertebrae may present with spondylitis, discitis and spondylodiscitis and are hematogenous origin in most cases. Potential sources of hematogenous infection are skin and soft tissue infection, genitourinary tract infection, infective endocarditis, intravenous drug abuse, respiratory tract infection and infected intravenous injection site. We have experienced a case of pyogenic spondylitis caused by methicillin-resistant Staphylococcus aureus (MRSA) bacteremia with peripheral injection site infection. He was successfully treated with surgical debridement and antibiotics.

Abnormal Blood Flow Between a Pericardial Mass and Right Atrium Detected by Echocardiography in a Case of a Ruptured Angiosarcoma of the Right Atrium

Spontaneous rupture of a primary cardiac angiosarcoma is a rare condition with a poor prognosis. The authors describe the case of a 48-year-old man with abnormal blood flow from a pericardial mass to the right atrium 3 months after pericardiocentesis. The flow was presumed to have occurred due to rupture of this cardiac angiosarcoma of the right atrial wall and pericardium into the right atrial cavity.

Abnormal Blood Flow Between a Pericardial Mass and Right Atrium Detected by Echocardiography in a Case of a Ruptured Angiosarcoma of the Right Atrium

Spontaneous rupture of a primary cardiac angiosarcoma is a rare condition with a poor prognosis. The authors describe the case of a 48-year-old man with abnormal blood flow from a pericardial mass to the right atrium 3 months after pericardiocentesis. The flow was presumed to have occurred due to rupture of this cardiac angiosarcoma of the right atrial wall and pericardium into the right atrial cavity.

A Case of Severe Neonatal Hypertension in Twin-Twin Transfusion Syndrome

Twin-twin transfusion syndrome is attributed to an unbalanced blood flow between the donor and the recipient twin, but the exact pathophysiology remains incompletely understood. Despite active prenatal management, fetal twin-twin transfusion syndrome is still associated with a substantial residual perinatal mortality and morbidity. The donor twin progressively becomes anemic, growth restricted, oliguric and has oligohydramnios, where as the recipient becomes plethoric, polyuric and has polyhydramnios and in the most severe cases develops cardiomegaly, congestive heart failure and hypertension. We report a case of severe systemic hypertension developed in recipient twin.

Successful Treatment of Prenatally Diagnosed Non-Chylous Primary Fetal Hydrethorax: A Case Report / 대한주산의학회잡지

Primary fetal hydrothorax presents a wide spectrum of severity ranging from small harmless effusions to life-threatening thoracic compression. Management of this condition is controversial. We experienced a case of non-chylous primary fetal hydrothorax not associated with hydrops or other malformations. A predominantly unilateral hydrothorax in a fetus was diagnosed by sonography at 31 weeks gestation. Repeated ultrasonographic examinations to detect development of hydrops or progression of intrapleural effusion were used to evaluate the need for intrauterine thoracentesis and to decide the optimal time for delivery. Two thoracenteses were performed. The baby was delivered at 34 weeks. Postnatal intubation was carried out in the baby, and then chest tube placement was performed and resulted in the collection of 805ml of serous fluid. Sustained intubation was necessary for 16 days because of developing respiratory distress as a result of prematurity and recurrent pleural fluid accumulation. The baby did well after postnatal evacuation of the intrapleural fluid. Pre and postnatal investigation to find the pathophysiological mechanism leading to hydrothorax were negative. We conclude that non-chylous primary fetal hydrothorax is generally associated with a favorable outcome.

A Study of Progression to Cerebral Palsy in Premature Infants with Neurosonographic Abnormal Findings

PURPOSE: Although neonatal intensive care and development of obstetrics play a role in improving survival rate in prematurity, cerebral palsy (CP) is still one of the neurologic sequelae. We tried to find what kinds of risk factors in the patients with abnormal neurosonographic findings who developed CP later. This study was performed to predict early enough who will develop CP later and to treat rapidly for rehabilitation. METHODS: The one hundred and forty one infants with intraventricular hemorrhage, cyst, or abnormal increased periventricular echodensity in serial neurosonographic findings were admitted to the neonatal intensive care unit of Gil general hospital from January 1992 to December 1994. Neurosonographic findings in CP group and non-CP group during postpartum 18 months to 54 months were analysed retrospectively. RESULTS: 1) 18 of one hundred and forty one cases with abnormal neurosonographic findings developed CP. The duration of mechanical ventilation of the CP group was 11.6+/-16.1 days. It is significantly shorter than that of the control group (non-CP group : 2.2+/-7.0 day). The duration of oxygen therapy in the CP group was 22.4+/-19.2 days. Which is also significantly shorter than that of the control group (non-CP group : 5.4+/-8.4 day) (P<0.01). 2) Increased periventricular echodensity and the size of the cyst in neurosonographic findings in the CP group is significantly different from those of the non-CP group P<0.01). 3) In CP group (N=18), Increased periventricular echodensity and cyst formation were located most commonly in the parietal region. 4) The grade of the intraventricular hemorrhage in the CP group was significantly different from that in the non-CP group (P<0.01). 5) The neurosonographic findings give the diagnostic accuracy for predicting CP. CONCLUSIONS: We studied the relationship of the abnormal neurosonographic findings, CP, and associated risk factors. In review, By using neurosonographic examination the risk of the development of CP could be predicted, and by early diagnosis of CP the quality of the life of CP-patient could be better.

The Effect of Congenital Gut Obstruction on Fetal Growth

PURPOSE: The human fetus is primarily dependent on the placenta for its nutrition. However, as the fetus matures, it swallows increasing amounts of amniotic fluid, which contributes to the growth of fetus. Accordingly fetuses with congenital obstruction of the gut at high level have a reduced capacity for intestinal absorption of amniotic fluid. We undertook a study to investigate the effect of congenital gut obstruction on fetal growth. METHOD: A retrospective review of the records of all patients presenting congenital gut obstruction over 6-year period (from 1992 to 1997) in Chung-ang Gil hospital was performed. Patients with a complete proximal obstruction were included in group A; patients with incomplete or lower obstruction were included in group B. RESULT: 1) The ratio of male to female was 1.5:1. The mean birth weight and gestational age were 2.89+/-0.60kg and 38.7+/-0.20weeks. The mean birth weight and gestational age in group A were 2.68+/-0.69kg and 37.8+/-0.25weeks. The mean birth weight and gesnal age in group B were 2.980.54kg and 39.1+0.17weeks. There was significant difference between group A and B (P0.05). 3) In group A, 3 (42.8%) of 7 patients with associated anomalies had IUGR, whereas 8 (40.0%) of 20 patients without associated anomalies had IUGR (P>0.05). The corresponding figures for group B were 23.0% and 14.8%, respectively (P>0.05). CONCLUSION: Congenital gut obstruction causes IUGR by reducing intestinal absorption of amniotic fluid and the effect of IUGR is more pronounced as the obstruction is proximal to jejunum rather than distal to it.

Clinical Study of Neonatal Adrenal Lemorrhage

PURPOSE: Neonatal adrenal hemorrhage is not rare disease which can be caused by such risk factors as sepsis, large baby, birth trauma and asphyxia. The clinical manifestations include jaundice, anemia, abdominal mass and differentiation from neuroblastoma, renal vein thrombosis and adrenal abscess is needed. Through the clinical assessment of presenting features, we hope that this study be of any help to early detection and proper management of neonatal adrenal hemorrhage. METHODS: The 16 subjects out of neonates admitted to our hospital from July 1991 to June 1997 were diagnosed as neonatal adrenal hemorrhage. The risk factors, clinical manifestations, diagnostic methods and prognosis of neonatal adrenal hemorrhage were evaluated in these 16 cases. RESULTS: 1) Among 16 cases, males were 10 (62.5%) and females were 6 (37.5%). Mean birth weight was 3.601.08kg and mean gestational age was 39.82+1.08 week, and all were fullterm babies. In modes of delivery, vaginal deliveries were 12 cases (75.0%) and cesarean sections were 4 cases (25.0%). 2) 13 cases (81.2%) were involved in right side, 2 cases (12.5%) in left side and 1 case (6.3%) bilaterally. 3) The risk factors include sepsis in 6 cases (37.5%), large baby in 5 cases (31.3%), birth trauma in 5 cases<31.3%) and asphyxia in 3 cases (18.8%). 4) The clinical manifestations include jaundice in 7 cases (43.8%), anemia in 7 cases (43.8%), fever in 6 cases (37.5%) and abdominal mass in 3 cases (18.8%). 5) In the time of diagnosis, until 7 days of birth were 9 cases (56.2%), 8-14 days were 3 cases (18.8%), 15-21 days were 2 cases (12.5%) and 22-28 days were 2 cases (12.5%). Follow-up studies were done in 13 cases (81.2%), and tha lesions all decreased without any specific complications or sequelae. CONCLUSION: Neonatal adrenal hemorrhage can be diagnosed by abdominal ultrasono- gram in the presence of suggestive manifestations of jaundice, anemia, fever and abdominal mass, with relatively good prognosis. Differentiation from other conditions as well as avoidance of unnecessary explorations can be achieved by serial follow-up examinations of abdominal ultrasonogram.

A Case of Aplasia Cutis Congenita Group 5

Aplasia cutis congenita is a rare disorder characterized by localized or generalized absence of skin at birth. The various hypothesis and classification are proposed. Type V in Frieden's classification, which is associated with fetus papyraceus or placental infarcts, occurs as a large cutaneous defect on the trunk and extremities. We experienced the patient had skin defect affecting the lower abdomen symmetrically, with no family history of the any disorders or chromosomal anomalies and associated anomalies. A twin fetus was died in utero. We report a case of aplasia cutis congenita associated with fetus papyraceus with the review of the associated literatures.

A Study of Auditory Brainstem Responses in Neonates with Hyperbilirubinemia and Their Neurodevelopmental Outcome

Neonatal hyperbilirubinemia is a significant risk factor for the developemtn of otoneurologic disorder. Hyperbilirubinemia resulting in kernicterus produces widespread neuronal damage with the most common sites of staining and destruction involving the hippocampus, basal ganglia and the brainstem nuclei in the floor of the fourth ventricle, including the dorsal cochlear nucleus. ABR may be a useful tool for the monitoring early bilirubin toxicity and postcteric sequelae in infants. This study attempts to evaluate the clinical neurodevelopmental outcome in hyperbilirubnemic infants requiring exchange transfusion through the assessment of ABR. Eight hyperbilirubinemic neonates with severely abnormal ABR findings and twelve hyperbilirubinemic neonates with normal ABR findings were studied to assess their neurodevelopemental outcome. The results were as follows; 1) There were 8 severely abnormal ABR cases, including 5 cases of bilateral flat wave and 3 cases of unilateraly elevated hearing throeshold. 2) The major cause of hyperbilirubinemia was ABO incompatibility(65%) 3) Significant clinical finding associated with severely abnormal ABR was kernicterus(p<0.05) 4) Significant laboratory findings associated with severely abnormal ABR were lower levels of hemoglobin and hematocrit(p<0.05) 5) 2 cases of bilateraly flat ABR and 3 cases of unilaterally elevated hearing threshold could be classified into sensorineural type hearing defect by latency-intensity function curve. 6) At the follow up tests of 3 cases of bilaterally flat ABR, 2 cases showed no change and 1 case showed mild improvement. 7) Among 5 follow up cases of severely abnormal BR, only 1 case showed normal neurodevelopmental outcome, 3 cases showed major neurodevelopmental defect and 1 case showed minor neurodeveoplemental defect. Among them, 1 case has had definite hearing disability.

A Study of Auditory Brainstem Responses in Neonates with Hyperbilirubinemia and Their Neurodevelopmental Outcome

Neonatal hyperbilirubinemia is a significant risk factor for the developemtn of otoneurologic disorder. Hyperbilirubinemia resulting in kernicterus produces widespread neuronal damage with the most common sites of staining and destruction involving the hippocampus, basal ganglia and the brainstem nuclei in the floor of the fourth ventricle, including the dorsal cochlear nucleus. ABR may be a useful tool for the monitoring early bilirubin toxicity and postcteric sequelae in infants. This study attempts to evaluate the clinical neurodevelopmental outcome in hyperbilirubnemic infants requiring exchange transfusion through the assessment of ABR. Eight hyperbilirubinemic neonates with severely abnormal ABR findings and twelve hyperbilirubinemic neonates with normal ABR findings were studied to assess their neurodevelopemental outcome. The results were as follows; 1) There were 8 severely abnormal ABR cases, including 5 cases of bilateral flat wave and 3 cases of unilateraly elevated hearing throeshold. 2) The major cause of hyperbilirubinemia was ABO incompatibility(65%) 3) Significant clinical finding associated with severely abnormal ABR was kernicterus(p<0.05) 4) Significant laboratory findings associated with severely abnormal ABR were lower levels of hemoglobin and hematocrit(p<0.05) 5) 2 cases of bilateraly flat ABR and 3 cases of unilaterally elevated hearing threshold could be classified into sensorineural type hearing defect by latency-intensity function curve. 6) At the follow up tests of 3 cases of bilaterally flat ABR, 2 cases showed no change and 1 case showed mild improvement. 7) Among 5 follow up cases of severely abnormal BR, only 1 case showed normal neurodevelopmental outcome, 3 cases showed major neurodevelopmental defect and 1 case showed minor neurodeveoplemental defect. Among them, 1 case has had definite hearing disability.

Clinical study of intussusception in infancy and childhood: comparing the result of barium enema with that of air enema

Intussusception is the most common cause of acquired intestinal obstruction during infancy and early childhood and requires early diagnosis and treatment. We observed 431 cases of intussusception who admitted at Chungang Gil Hospital from January 1987 to June 1991, and compared the result of treatment with Barium Enema in 302 cases with that of Air Enema in 129 cases. The results were as follows; 1) In sex distribution, males were more affected than females as a ratio of 2:1. 2) In age incidence, 78.2% of the cases were between 3~10 months. 3) Slight seasonal prevalence was noted in spring and autumn. 4) The common symptoms and signs were cyclic irritability (89.1%), vomiting (78.9%), bloody stool (84.9%) and abdominal mass(43.1%). 5) The common diseases accompanied were URI (39.7%), AGE (13.0%) and UTI(1.2%). 6) 5.8% of cases were visited after the 48 hours of symptom onset, in these cases the operation rate was 39.1%. So the longer duration of symptoms persists, the lower reduction rate follows. 7) The most common type of intussusception was ileocolic type, and the lowest reduction rate was noted in ileoileocolic type. 8) In the reduction rate, 87.4% with Barium Enema and 89.1% with Air Enema. 9) After 24 hours of symptom onset, the reduction rate, 74.4% with Barium Enema and 78.6% with Air Enema. 10) Recurrence rate was 9.3% with Barium Enema and 7.8% with Air Enema. 11) In Barium Enema, 1 case of bowel perforation was observed, and he expired with barium peritonitis, in Air Enema, 2 cases of bowel perforation were observed, and cured after operation. 12) We observed some advantages in Air Enema such as simplicity, high reduction rate and absence of fatal complications. And we think that Air Enema is a useful diagnstic and treatment method of intussusception.

Periventricular leukomalacia: Ultrasonographic findings, risk factors and neurological outcome

The thirty eight newborn infants with periventricular leukomalacia who were admitted to the neonatal intensive care unit of Gil General Hospital from March 1, 1988 to June 30, 1991, were investigated for ultrasonographic findings, risk factors and neurological outcome. The results were as follows: 1) There were 38 cases of PVL including 21 echogenic flarings and 17 cystic PVL's. 2) Mean birth weight was 2,250 gm and mean gestational age was 35 week. 3) Mean detection timing was 4th day in echogenic flarings and 18th day in cystic PVL's. 4) PVL's were located in the parietal region in 1 case and fronto-parieto-occipital in 3 cases. 5) Mean cyst size was 6 mm. 6) Multiple logistic regression analysis for the risk factors of PVL showed that low birth weight, apnea and seizure were the most significant contributing factors (p<0.05). 7) In the follow-up study of cystic PVL's, 7 cases showed improvement, 7 cases developed into multicystic encephalomalacia and 3 cases developed into atrophy. 8) Neurodevelopmental outcome of cystic PVL's showed nomal; 6.2%, minor neurodevelopmental defect; 43.8%, major neurodevelopmental defect; 31.2% and death; 18.8%. 9) Neurosonographic predictability for neurodevelopemental sequelae by cystic PVL's showed sensitivity; 63.6%%, specificity; 98.0%, positive predictive value; 92.8% and accuracy; 88.2%. 10) Major neurodevelopmental defect was more frequent, cyst size being larger and location being more extensive (p<0.05).

Primary endodermal sinus tumor of the common bile duct

No abstract available.

Congenital Cytomegalovirus infection

No abstract available.

A case of Hypokalemic Familial Periodic Paralysis

No abstract available.