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.
Child ; Adult ; Male ; Female ; Humans ; Cysts

PURPOSE: To evaluate the outcome of ductal carcinoma in situ (DCIS) patients who underwent surgery followed by radiation therapy (RT). MATERIALS AND METHODS: We retrospectively reviewed 106 DCIS patients who underwent surgery followed by postoperative RT between 1994 and 2006. Ninety-four patients underwent breast-conserving surgery, and mastectomy was performed in 12 patients due to extensive DCIS. Postoperative RT was delivered to whole breast with 50.4 Gy/28 fx. Tumor bed boost was offered to 7 patients (6.6%). Patients with hormonal receptor-positive tumors were treated with hormonal therapy. RESULTS: The median follow-up duration was 83.4 months (range, 33.4 to 191.5 months) and the median age was 47.8 years. Ten patients (9.4%) had resection margin <1 mm and high-grade and estrogen receptor-negative tumors were observed in 39 (36.8%) and 20 (18.9%) patients, respectively. The 7-year ipsilateral breast tumor recurrence (IBTR)-free survival rate was 95.3%. Resection margin (<1 or > or =1 mm) was the significant prognostic factor for IBTR in univariate and multivariate analyses (p < 0.001 and p = 0.016, respectively). CONCLUSION: Postoperative RT for DCIS can achieve favorable treatment outcome. Resection margin was the important prognostic factor for IBTR in the DCIS patients who underwent postoperative RT.
Breast ; Breast Neoplasms ; Carcinoma, Ductal* ; Carcinoma, Intraductal, Noninfiltrating* ; Estrogens ; Follow-Up Studies ; Humans ; Mastectomy ; Mastectomy, Segmental ; Multivariate Analysis ; Recurrence ; Retrospective Studies ; Survival Rate ; Treatment Outcome*

No abstract available.

Status epilepticus is commonly defined as a condition characterized by an epileptic seizure that is so frequently repeated or so prolonged as to produce a fixed and enduring epileptic condition. Common etiologies are brain tumor, CNS infection, vascular insults, trauma, withdrawal of antiepileptic drug, and metabolic disturbance such as hypoglycemia, hypocalcemia, hyponatremia and hyperosmolarity caused by hyperglycemia, hypernatremia, and uremic encephalopathy etc. Although some cases of epilepsia partialis continua in the patient with nonketotic hyperglycemia were reported in the previous literature, we could hardly find the report that generalized convulsive status epilepticus was the initial symptom of nonketotic hypergycemia. We recently experienced three eases of nonketotic hyperglycemia who manifested generalized convulsive status epilepticus as a initial clinical feature. Two cases were completely controlled within a few hours after the correction of hyperglycemia and intravenous dilantinization. Another case was needed an additional phenobarbital administration to control the status epilepticus. In all cases, afterthen no further seizure occurred under the normal serum glucose level without use of antiepileptics.
Anticonvulsants ; Blood Glucose ; Brain Neoplasms ; Epilepsia Partialis Continua ; Epilepsy ; Humans ; Hyperglycemia* ; Hypernatremia ; Hypocalcemia ; Hypoglycemia ; Hyponatremia ; Phenobarbital ; Phenytoin ; Seizures ; Status Epilepticus*

Essential thrombocytosis (ET) is a clonal myeloproliferative disorder, characterized predominantly by a markedly elevated platelet count without known cause. We report a case of essential thrombocytosis, the only clinical manifestation of which consisted of neurologic symptoms, including headache, dysarthria, right hemiparesis with a relatively benign and slowly progressive clinical course. Neuroradiologic examination (Brain CT and MRI) disclosed a multiple infarction in the both basal ganglia and left parietal region. These findings are discussed in relation to the possible pathogenetic mechanisms of the vascular occlusion due to abnormal platelet function in ET.
Basal Ganglia ; Blood Platelets ; Cerebral Infarction* ; Dysarthria ; Headache ; Infarction ; Myeloproliferative Disorders ; Neurologic Manifestations ; Paresis ; Platelet Count ; Rabeprazole ; Thrombocytosis*

Cerebral aneurysms associated with arteriovenous malformation (AVM) have been reported with a variable incidence, averaged 10% of total AVM cases. It has been AVM. We report four cases of the intracranial aneurysm associated with AVM. Three cases of aneurysm were located in distal portion of feeding artery, and one case was proximal on major feeding artery. And bleeding resulted from rupture of aneurysm. Three of them located in the posterior circulation. Removal of aneurysm and AVM was successfully accomplished in 3 cases.
Aneurysm ; Arteries ; Arteriovenous Malformations* ; Hemorrhage ; Incidence ; Intracranial Aneurysm ; Rupture

BACKGROUND AND OBJECTIVES: The symptoms of salivary diseasess are often nonspecific, and a variety of investigative methods can be employed. Conventional sialography, which is still widely used for diagnosis of salivary ductal pathologies, has the withdrawback of invasiveness and radiation exposure, and thus ultrasound and magnetic resonance (MR) sialography can replace the conventional tools. This study was performed to evaluate the usefulness of MR sialography for the diagnosis of idiopathic chronic sialadenitis. SUBJECTS AND METHOD: From November 2013 to June 2014, we have retrospectively analyzed 26 patients who have had swelling and pain of salivary glands and undergone MR sialography for further diagnosis of the idiopathic salivary obstructive symptom. We analyzed the symptom scores, salivary flow rate (SFR) and parameters of salivary gland scintigraphy. Then we evaluated correlation among MR sialography findings (duct visualization, grade of stenosis at main duct, degree of sialectasis and glandular volume size). RESULTS: Among the 26 patients, stenosis of salivary duct was observed in 14 patients (53.8%), chronic sialadenitis without stenosis in 6 patients (23.1%), Sjogren's syndrome in 3 patients (11.5%), Juvenile reccutent parotitis in 1 patient (3.8%), and 2 patients were norma (7.7%). The degree of sialectasis was significantly correlated with Tmin (time interval, in minutes, from stimulation to minimum count), maximum secretion (p<0.05), and glandular volume size was also significantly correlated with unstimulated SFR (p<0.05). But others did not show any significant correlations. From these findings, we report three cases that were useful to diagnose the gland disease using MR sialography. CONCLUSION: Resutls show that MR sialogarphy indirectly reflects the salivary gland function. Therefore MR sialography can be helpful when the differential diagnosis of idiopathic chronic sialadenitis is difficult with conventional tools.
Constriction, Pathologic ; Diagnosis* ; Diagnosis, Differential ; Humans ; Parotitis ; Pathology ; Radionuclide Imaging ; Retrospective Studies ; Salivary Ducts ; Salivary Glands ; Sialadenitis* ; Sialography* ; Sjogren's Syndrome ; Ultrasonography

No abstract available.
Plasma* ; Prostate* ; Prostatic Neoplasms* ; Stomach Neoplasms*

BACKGROUND: To analyze hemodynamic changes during single catheter technique of hepatic venous isolation and extracorporeal charcoal hemoperfusion for malignant liver tumor. METHODS: Drugs for chemotherapy were infused to the liver through hepatic artery. With 4-lumen- 2-balloon (4L-2B) catheter, hepatic venous blood was circulated to the extracorporeal charcoal system. During extracorporeal charcoal system circulation, drugs were eliminated and the blood was reinfused to supra-hepatic vein-IVC. At the same time, IVC was clamped. Systemic vascular resistance index (SVRI), cardiac index (CI), stroke volume index (SVI), mean arterial pressure (MAP), heart rate (HR) and arterial blood gas were measured after 4L-2B catheter insertion (T1), during test circulation (T2), after 20min chemotherapy (T3) and after 10min reperfusion (T4). RESULTS: MAP was decreased at T3 compared to T1 and increased at T4 compared to T3. CI was decreased at T3 and increased at T4 compared to T1. SVRI was decreased at T4 compared to T1. HR was increased at T2 and T3 compared to T1. SVI was decreased at T2 and T3 compared to T1. CONCLUSIONS: During clamping of IVC, MAP is decreased by decreased SVI in spite of increased HR. After IVC is released and the stagnated blood of lower extremity is recirculated, the MAP is returned to the value of after catheter insertion by increased SVI in spite of decreased SVRI.
Arterial Pressure ; Blood Pressure ; Carcinoma, Hepatocellular* ; Catheters ; Charcoal ; Constriction ; Drug Therapy ; Heart Rate ; Hemodynamics* ; Hemoperfusion* ; Hepatic Artery ; Liver* ; Lower Extremity ; Reperfusion ; Stroke Volume ; Vascular Resistance