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*

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

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

Heterotopic pancreas can be defined as the occurrence of pancreatic tissue at aberrant anatomic sites that lack vascular, neural and anatomic community with the pancreas. A minority of heterotopic pancreas elicit clinical signs or symptoms that can vary according to its location and size, and the involvement of the overlying mucosa. We report here on a case of heterotopic pancreas that was removed by pancreatoduodenectomy under the erroneous diagnosis of distal common bile duct cancer.
Ampulla of Vater* ; Common Bile Duct* ; Diagnosis ; Jaundice ; Mucous Membrane ; Pancreas* ; Pancreaticoduodenectomy

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

We report data on 2 members of a family affected by a dominantly inherited disorder closely resembling Roussy-Levy syndrome(RLS) Electrophysiological findings showed a marked decrease of motor and sensory conduction velocities and mild neurogenic damage. Light and electron microscopy of sural nerve biopsy showed a hypertrophic neuropathy with diffuse onion-bulb formations and marked decrease of large size fibers. Teased fiber preparations revealed reduced internodal lengths and segmental demyelination. The hypothesis that RLS is not a disease entity but a hypertrophic-type of Charcot-Marie Tooth disease with essential tremor(HMSN type 1) is strongly supported.
Biopsy ; Charcot-Marie-Tooth Disease* ; Demyelinating Diseases ; Humans ; Microscopy, Electron ; Sural Nerve ; Tooth Diseases