No abstract available.
Cardiovascular Diseases* ; Estrogens*

Acromelanosis progressiva is a peculiar progressive pigmentary disorder characterized by intensely dark and sharply demarcated hyperpigmentation over the dorsa of fingers and toes, and the pigmentation rapidly spread proximally. A 19-year-old female had sharply demarcated symmetric dark aroarn macules on the dorsa of her hands and feet, forearms, lower legs, face and neck. These pigmentations at first appeared at the age of 5 on the dorsa of her fingers and toes, thereafter these pigmertatons spread progressively to the proximal portion of her body. Histologically, a proliferation of mela biocyte at the epidermal-dermal junction was seen, associated with mild hyperkeratosis. She was othervise normal on physical examination and had no family historv of consanguinity.
Consanguinity ; Female ; Fingers ; Foot ; Forearm ; Hand ; Humans ; Hyperpigmentation ; Leg ; Neck ; Physical Examination ; Pigmentation ; Toes ; Young Adult

No abstract available.
Arthroplasty* ; Arthroplasty, Replacement, Hip* ; Head* ; Hemiarthroplasty* ; Necrosis*

A polymerase chain reaction (PCR) method for detection of the pathogenic Yersinia pestis from other Yersinia spp. was developed. Five Y. pestis strains, ninety-two other Yersinia species and twenty-four Enterobacteriaceae strains were collected in Korea and from other countries. Oligonucleotide primers were designed from pathogenic gene of antiphagocytic protein capsule gene (fra 1) and plasminogen activator gene (pla). The 428 bp DNA fragment was amplified from five Y. pestis which contained the fra I gene. No product was amplified from other Yersinia species and other strains of the Enterobacteriaceae. The 439 bp DNA fragment was amplified from three K pestis which contained the pla gene. No product was amplified from two Y. pestis, other Yersinia species and other strains of the Enterobacteriaceae. These showed that the designed primers were specific for detection of Y. pestis among other Yersinia species and Enterobacteriaceae strains. Amplification was successful whether the template was derived from purified DNA or from aliquots of boiled bacterial suspension. The detection limits were 100 pg of DNA and 100 colony forming units (CFU) for fra I and 100 pg DNA and 10 CFU for pla, respectively. Our results prove that the PCR method using specific primers for Y. pestis is a rapid and convenient procedure for routine clinical detection and identification of Y. pestis.
DNA ; DNA Primers ; Enterobacteriaceae ; Korea ; Limit of Detection ; Plasminogen Activators ; Polymerase Chain Reaction* ; Stem Cells ; Yersinia pestis* ; Yersinia*

No Abstract Available.
Animals ; Cats* ; Endothelium, Corneal*

No abstract available.
Kidney Transplantation* ; Pregnancy*

Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result could prove to be unfortunately fatal. The mechanism which underlies the remote damaging effect of neural crest tumor, especially neuroblastoma, on the nervous system resulting in myoclonic encephalopathy is by no means clear. In addition the nature and the extent of the pathologic lesion are inconsistent. We experienced a case of myoclonic encephalopathy associated with an occult mediastinal ganglioneuroblastoma in a 22-month-old girl who was hospitalized for inability to walk without support and tilting of the head to the left side. She became increasingly ataxic, and during the hospitalization myoclonic jerks of upper extremities and head along with chaotic, rapidly flickering, multidirectional spontaneous eye movements, were noted. Laboratory data included normal complete blood count, urinalysis, BUN and creatinine, electrolytes and bone marrow. Chest X-ray and chest CT revealed a relatively well marginated right posterior mediastinal mass. In a 24 hours urine excretion test, VMA and catecholamines were increased. Over the next 2 weeks, a surgical exploration revealed a right posterior mediastinal mass. Microscopically the mass proved to be a ganglioneuroblastoma, extending to right innominate artery and right axillary lymph nodes. Within 2 weeks after the surgery, radiotherapy (2,400 rads) and chemotherapy (CTX, DTIC, VCR) were started, but corticosteroid was not used. She has been free of tumor and abnormal neurological systemic symptoms and signs for 1 1/2 year since the completion of chemotherapy. In the 3 1/2 years follow-up period, her neurologic symptoms has completely resolved by the completion of 2 years chemotherapy. We report a case of mycoclonic encephalopathy associated with hidden ganglioneuroblastoma in 22-month-old girl.
Blood Cell Count ; Bone Marrow ; Brachiocephalic Trunk ; Catecholamines ; Creatinine ; Dacarbazine ; Diagnosis ; Drug Therapy ; Electrolytes ; Epilepsies, Myoclonic* ; Eye Movements ; Female ; Follow-Up Studies ; Ganglioneuroblastoma* ; Head ; Hospitalization ; Humans ; Infant ; Lymph Nodes ; Myoclonus ; Nervous System ; Neural Crest ; Neuroblastoma ; Neurologic Manifestations ; Paraneoplastic Syndromes* ; Radiotherapy ; Thorax ; Tomography, X-Ray Computed ; Upper Extremity ; Urinalysis

Epidural hematoma is relatively rare in newborn infants and frequently associated with instrumental deliveries or other complications during labor and delivery. Although surgical evacuation has been the most common therapy, many other procedures have been described. Although many epidural hematomas require surgical evacuation rather than nonsurgical management, conservative or aspiration of hematoma have been attempted. In the case of EDH associated with cephalhematoma, aspiration of cephalhematoma could be attempted because frequent features of these combination were communication between these hematoma. We report a case of successful nonsurgical management of epidural hematoma through the aspiration of accompanying cephalhematoma in a 5-day-old newborn infant.
Hematoma* ; Humans ; Infant, Newborn*

The distribution of pemphigus subclass autoantibodies in a patient with pemphigus vulgaris (PV) has been investigated by semiquantitative indirect immunofluorescence (IIF), using the HP series monoclonal antibodies specific for four human IgG subclasses on human foreskins. IgG1 and IgG4 intercellular substance-specific autoantibodies were detected in the serum of the patient, whereas IgG2 and IgG3 autoantibodies were absent. In addition to foreskins, human tonsillar epithelia were used as substrates of IIF for detecting the PV autoantibodies and it was one of satisfactory substitutes for monkey esophagus.
Antibodies, Monoclonal ; Autoantibodies* ; Esophagus ; Fluorescent Antibody Technique, Indirect ; Foreskin ; Haplorhini ; Humans ; Immunoglobulin G* ; Pemphigus*

PURPOSE: Refractory status epilepticus refers to patients who do not respond properly to adequate first-line drug treatment such as diazepam, phenytoin, phenobaribital, lorazepam and show longer than 60 minute seizure. This study was designed to determine the efficacy and safety of midazolam given as a continuous infusion in the treatment of status epilepticus in children. METHODS: Midazolam was infused to twelve children with seizures, for whom two time repeated doses of 0.3mg/kg of diazepam, 20mg/kg of phenobarbital, and 20mg/kg of phenytoin failed to bring the episode under control. All patients received a bolus of midazolam(0.15mg/kg) followed by a continuous infusion at 1microgram/kg/min. The dose was increased every 15 min until the episode of seizure was brought under control. Time required to control seizures, infusion rate, and side-effects were monitored. RESULTS: The mean age of the patient population was 6.06 yrs(range 2 months to 16 yrs; 6 females and 6 males). In 11 patients, seizures were controlled in a mean time of 60.1 min(range 15-180 min). The mean infusion rate was 8.5microgram/kg/min(range 1-20). The total treatment duration was 17.6 days(range 1-54 days). One patient who was confirmed to have cortical dysplasia failed to respond. Two of the patients showed respiratory depression and bradycardia. CONCLUSION: Midazolam is an effective and safe drug to control refractory seizures in children with status epilepticus.
Bradycardia ; Child* ; Diazepam ; Female ; Humans ; Lorazepam ; Malformations of Cortical Development ; Midazolam* ; Phenobarbital ; Phenytoin ; Respiratory Insufficiency ; Seizures ; Status Epilepticus*