Pancreatic islet cell adenoma is a benign tumor of pancreatic beta-cell and a rare cause of hyperinsulinemic hypoglycemia in children. The authors experienced a case of pancreatic islet cell adenoma(Insulinoma)in a 11 year and 8 month old male who had frequent loss of consciousness and seizure. Enucleation was done after localization of tumor by selective celiac artery angiography and abdominal computed tomography. Diagnosis was confirmed by histologic findings as pancreatic-adenoma, gyriform growth pattern. A brief review of related literature was made.
Adenoma* ; Angiography ; Celiac Artery ; Child ; Diagnosis ; Humans ; Hyperinsulinism ; Hypoglycemia ; Infant ; Islets of Langerhans ; Male ; Seizures ; Unconsciousness

A case of metastatic adrenal cortical carcinoma in which partial remission was achieved with transarterial embolization is presented as probably the first reported case in the literature to date. A 29-year-old woman was admitted because of adrenal cortical carcinoma which had not responded to mitotane. A left adrenalectomy with segmentectomy of the involved liver had been done previously. Abdominal computerized tomography demonstrated multiple large metastatic tumors in the liver. Transarterial embolization with Gelfoam and 20 mCi of 131I-labeled lipiodol was performed and resulted in a decrease in tumor size and biochemical parameters. Transarterial embolization can be one of the therapeutic modalities for metastatic adrenal cortical carcinomas.
Adrenal Cortex Neoplasms/metabolism/*therapy ; Adult ; *Embolization, Therapeutic ; Female ; Gelatin Sponge, Absorbable ; Hepatic Artery ; Humans ; Hydrocortisone/metabolism ; Iodine Radioisotopes ; Iodized Oil ; Liver Neoplasms/metabolism/*secondary/*therapy

No abstract available.
DNA* ; Flow Cytometry* ; Gestational Trophoblastic Disease*

A totally implanted venous access system was inserted in 19 children with cancer. The devices were utilized for the administration of antineoplastic drugs, parenteral fluids, antibiotics, and blood products. Total duration of implantation was 4,046 days for 23 implanted system (range 7-445 days). Complications included cather infection (0.247/100 catheter days), occlusion (0.692/100 catheter days), and dislodgement of needle (0.643/100 catheter days). There were major complications that necessitated removal of catheters, including systemic infections (0.09/100 catheter days) and complete occlusions (0.09/100 catheter days). The system was thought to be safe and convenient in chemotherapy, and permitted full physical activity.
Anti-Bacterial Agents ; Antineoplastic Agents ; Catheters ; Child* ; Drug Therapy ; Humans ; Motor Activity ; Needles ; Prospective Studies*

Hereditary spastic ataxia is a familial neurological disorder which exhibit the features of a progressive combined pyramidal tract and cerebellar deficiency. The main features are progressive gait disturbance, incoordination, nystagmus, visual impairment, hyperreflexia, extensor plantar response, peripheral neuropathy, and pes cavus. A 27-year-old male patient with spastic ataxic gait was evaluated. He showed characteristic features of hereditary spastic ataxia. There were another twelve affected members in four generations of his family which may be inherited by autosomal dominant pattern. One of them is reported with review of the literature on familial spastic ataxia.
Adult ; Ataxia* ; Family Characteristics ; Foot Deformities ; Gait ; Humans ; Male ; Muscle Spasticity* ; Nervous System Diseases ; Peripheral Nervous System Diseases ; Pyramidal Tracts ; Reflex, Abnormal ; Reflex, Babinski ; Vision Disorders

The spontaneous hematomyelia is an uncommon event and its predisposing conditions are vascular malformation, syringomyelia, pregnancy and delivery, angioma, hemophilia, anticoagulant therapy, etc. We have recently experienced the patient with spontaneous onset and resolving hematomyelia in the cervical spinal cord. A 30-year-old male patient with non-traumatic spinal shock was evaluated. On MRI, a hematomyelia along cervical spi-nal cord was revealed. A suspicious AV malformation was noticed at C3-4 level. Fol-low-up MRIs showed spontaneous resolution of the hematoma.
Adult ; Hemangioma ; Hematoma ; Hemophilia A ; Humans ; Magnetic Resonance Imaging ; Male ; Pregnancy ; Shock ; Spinal Cord ; Spinal Cord Vascular Diseases* ; Syringomyelia ; Vascular Malformations

Bell's palsy is a relatively common, unilateral facial paralysis of unknown etiology. The purpose of this study was to evaluate the prognostic value of several electrophysiologic tests in Bell's palsy. Blink reflex (BR), side-to-side compound muscle action potential (CMAP) amplitude comparison, and side-to-side nerve excitability test (NET) threshold differ once have been studied during the first 2 weeks in 66 patients with Bell's palsy. According to the early response of BR(Rl), the patients were divided into 3 groups: Rl< or = 13ms(17cases) ; Rl>13ms(22cases), and absent BR(27cases). There was a remarkable tendency towards a satisfactory recovery from paralysis if BR occurred during the first 2 weeks from onset(p<0.001). In patients with normal BR, 88.2% had a satisfactory recovery, when the reflex was absent a bad prognosis was given in 59.3% of the patients. According to side-to-side CMAP amplitude comparison, the patients were divided into 2 groups; CMAP amplitude comparison > or = 25% (35cases), and CMAP amplitude comparison <25% (31cases). There was a tendency towards a satisfactory recovery from paralysis if side-to-side CMAP amplitude comparison was more than 25% (p < 0,003). In patients with more than 25% of CMAP amplitude comparison, 82.9% of the patients had a satisfactory recovery, but in patients with less than 25% of CMAP amplitude comparison, 51.6% of the patients had a bad prognosis. According to side-to-side NET threshold difference, the patients were divided into 2 groups, NET threshold difference < or = 3.5mA(43cases), and NET threshold difference >3.5mA (23cases). When the threshold of electrical excitability on both sides differs 3.5mA or more an unsatisfactory recovery has strongly to expected(p<0.001). In patients with less than 3.5mA of NET threshold, 81.4% had a satisfactory recovery, but in patients with more than 3.5mA of NET threshold, 60.9% had a bad prognosis. BR, side-to-side CMAP amplitude comparison, and side-to-side NET threshold difference seem to be useful independent indices for predicting the prognosis an early stage of the paralysis.
Action Potentials ; Bell Palsy* ; Blinking ; Facial Paralysis ; Humans ; Paralysis ; Prognosis ; Reflex

BACKGROUND: Several predictor of successful outcome after percutaneous mitral valvuloplasty(PMV) have been identified but the results were controversial. We analyzes the clinical and echocardiographic predictors of successful outcome after PMV. METHOD: We studied 214 patients(167 women and 47 men) undergoing PMV from October 1991 to December 1995. Echocardiographic evaluation was performed before and after PMV. RESULT: The study population had mean age of 41+/-11years, and the total echocardiographic score was 7.5+/-1.5. PMV using Inoue balloon and double balloon technique were performed in 113cases and 101 cases respectively. The successful outcome from PMV(defindedas mitral valve area > or =1.5cm2 and increase in valve area >+25% and less than grade 3 mitral regurgitation(MR)) was achieved in 178 cases(83.2%). The mean mitral valve area was increased from 0.90+/-0.21cm2 to 1.82+/-0.34cm2(p<0.01). The successful outcome group had good echocardiographic score(7.3+/-1.4 vs. 8.8+/-1.4, p<0.01) and larger valve area before procedure(0.92+/-0.21cm2 vs. o.75+/-0.17cm2, p<0.01) than suboptimal result group. The patients with total echocardiographic score <=8 had more increment in valve area after PMV(0.97+/-0.21cm2) than those with total echocardiographic score >8(0.83+/-0.22cm2, p<0.01). Significant MR(>=grade 3 MR) after PMV was developed in 10 cases(4.7%). In patients with significant MR afterPMV, echocardiographic calcification score were high(2.3+/-0.8 vs. 1.7+/-0.7, p<0.01) and mitral valve area before PMV were smaller(0.82+/-0.10cm2 vs. 0.90+/-0.22cm2, p<0.05) than in those without significant MR. CONCLUSIONS: The predictors of successful outcome after PMV are large mitral valve area and good echocardiographic score. The predictors of significant MR is severe calcified mitral valveand small mitral valve area before PMV.
Echocardiography ; Female ; Humans ; Mitral Valve

BACKGROUND: Restenosis remains as the major limitation of percutaneous translumainal coronary balloon angioplasty (PTCA). Although its mechanism remains incompletely understood, proliferative action of arterial smooth muscle cells has been found to play an important role on restenosis by neointimal formation after PTCA. Glycosaminoglycan-containing compounds, including Sulodexide (Vessel Due , ALFA, Wasserman, S.p.A, Italy), inhibit the proliferation and maigration of vascular smooth muscle cells in vitro. OBJECTIVES: This study was performed to assess the efficacy of Sulodexide, a glycosaminoglycan compound with antithrombotic and antiproliferative properties, in preventing restenosis after PTCA. METHOD: Two hundred eighty-four patients with ischemic heart disease were randomized to receive either the standard PTCA without Sulodexide in 144 patients (control group, M : F = 99 : 45, Age = 58 +9 or -9), 160 lesions or the standard PTCA with Sulodexide in 140 patients (treated group, M : F = 89 : 51, age = 58 +10 or -10), 158 lesions. Successful angioplasties were performed in 258 atheromatous coronary lesions in 224 patients for whom follow-up angiographic data were obtained 6 month later. Quantitative coronary angiographic analysis (QCA) was performed before , immediate after PTCA and 6-month later. Angiographic restenosis (>50% diameter stenosis at follow-up) was the primary end point : miniamal luminal diameter at follow-up angiogram was the secondary end point. RESULT: Successful PTCA was 97.6% and 97.5% in the standard PTCA with Sulodexide and the standard PTCA without Sulodexide, respectively. Although reference vessel size and minimal luminal diamater after PTCA were larger in the control group than in the Sulodexide group(2.94+0.11 or-0.11 vs 2.83+0.13 or -0.13 mm and 2.26+0.12 or -0.12 vs 2.18+0.08 or -0.08 mm, respectively, p=NS), there was a increased tendency of minimal lumen diameter at 6 months angiogram in the Sulidexide group than in the control group (1.12+0.50 or -0.50 vs 1.07 + 0.53 or -0.53 mm, respectively, p=NS). Angiographic restenosis occured in 42% of lesions in the Sulodexide group and 52% of the control group (p=NS). CONCLUSIONS: Sulodexide treatment had a tendency to reduce restenosis rate in 6 months after coronary angioplasty. However, further study is necessary to verify the antiproliferative effect of Sulodexide with much larger number of patients.
Angioplasty ; Angioplasty, Balloon, Coronary* ; Constriction, Pathologic ; Follow-Up Studies ; Humans ; Muscle, Smooth, Vascular ; Myocardial Ischemia ; Myocytes, Smooth Muscle ; Phenobarbital ; Prospective Studies*

Chronic active Epstein-Barr virus infection(CAEBV) is a nonfamilial syndrome that shows a specific immunodeficiency for the Epstein-Barr virus(EBV). CAEBV is characterized by fever, lymphadenopathy, splenomegaly, hepatitis, interstitial pneumonitis, interstitial nephritis, and uveitis. Cardiovascular complications are rare in EBV infection. Patients with CAEBV show characteristically high titers of anti-viral capsid antigen(VCA) IgG antibody and anti-early antigen(EA) antibody, as well as relatively low titer of anti-EB nuclear antigen(EBNA) antibody. We experienced a case of CAEBV with giant coronary aneurysms, who was a 6-year-old boy. He had 5 episodes of high fever and cervical lymphadenopathy and hepatosplenomegaly. The 6 mm sized bilateral coronary aneurysms were detected by echocardiography at second admission. IgG antibodies to EBV was positive, whereas negative for IgM antibody. Antibodies to EA and EBNA were also positive. The EBV was detected in lymph node tissue by in situ hybridization, and in the peripheral blood and bone marrow by the PCR. Treatment has been done with ganciclovir and interferon (IFN)-alpa for 5 weeks. The EBV-PCR of peripheral blood was converted to negative from 12th day of treatment. At present he has followed well for two years without fever and enlargements of lymphnode, except large coronary aneurysm.
Antibodies ; Bone Marrow ; Capsid ; Child ; Coronary Aneurysm* ; Echocardiography ; Epstein-Barr Virus Infections ; Fever ; Ganciclovir ; Hepatitis ; Herpesvirus 4, Human* ; Humans ; Immunoglobulin G ; Immunoglobulin M ; In Situ Hybridization ; Interferons ; Lung Diseases, Interstitial ; Lymph Nodes ; Lymphatic Diseases ; Male ; Nephritis, Interstitial ; Polymerase Chain Reaction ; Splenomegaly ; Uveitis