PURPOSE: Epilepsy is generally defined as a chronic condition characterized by recurrent unprovoked seizure. It is still controversial whether the long-term antiepileptic drugs(AED) treatment will be necessary in children with first unprovoked seizure. Cognitive and behavioral side effects of AEDs are especially important in developing children. Therefore the rationale of AED treatment in the children with first unprovoked seizure depends upon the seizure recurrence rate and presence of risk factors related with seizure recurrence. We are going to evaluate the risk of seizure recurrence and risk factors after a first unprovoked seizure in children. METHODS: One hundred fifty eight patients presented with a first unprovoked seizure from July, 1994 to June, 1999 were prospectively followed by regular epilepsy clinic visit or telephone interview for a mean of 27 months. We analyzed overall recurrence risk and risk factors of seizure recurrence(EEG finding, etiology of seizure, seizure type, history of prior febrile convulsions, neurodevelopmental status prior to first seizure, presence of a Todd's paresis, family history of seizures, a seizure occuring while asleep, and duration of seizure) by Cox's proportional hazards model and Kaplan-Meier survival analysis. RESULTS: In 158 patients with first unprovoked seizure, 61 (39%) patients experienced subsequent seizure. The cumulative risk of seizure recurrence was 28.5, 37.6, and 42.3% at 6, 12, and 18 months. The median time to recurrence was 3 months, with 72% of recurrence occurring within 6 months, 92% within 1 year, and 100% within 2 years. On univariate analysis, risk factors for seizure recurrence included an etiology of seizure and abnormal EEG. In idiopathic cases, the risk factor was an EEG abnormality. CONCLUSION: Many of the children with a first unprovoked seizure will not have recurrences. The recurrence risk of a first unprovoked seizure in children have statistically correlated with the etiology of seizure(cryptogenic and symptomatic) and abnormal EEG. Children with an idiopathic first seizure and a normal EEG have a particularly favorable prognosis.
Ambulatory Care ; Child ; Electroencephalography ; Epilepsy ; Humans ; Interviews as Topic ; Paresis ; Prognosis ; Proportional Hazards Models ; Prospective Studies* ; Recurrence* ; Risk Factors ; Seizures* ; Seizures, Febrile

Primary oxalosis is a rare genetic disorder caused by a deficiency of the peroxisomal enzyme alanine-glyoxylate aminotransferase(type I) and D-glyceric dehydrogenase(type II). It is characterized by the triad of radiopaque urolithiasis, nephrocalcinosis, and latered renal function. We report a case of primary oxalosis in a 10-year-old Korean girl. She presented with chronic renal failure at 9 years of age. Ultrasonographic examination revealed bilateral, multiple renal and ureteral stones. Removed stones were chemically analysed to be composed of calcium oxalate. She underwent renal transplantation after prolonged period of dialysis. Removed kidneys were firm and gritty. Cut sections showed numerous tiny yellow sandy stones and a large staghorn stone. Specimen X-ray also exhibited disseminated fine granular and often coalescent radiopaque materials throughout the cortex and medulla. Microscopically numerous varying-sized crystals were noted in the kidney in globular or rhomboid appearance. The crystals were semitranslucent and doubly refractile under the polarized light. Diffuse interstitial fibrosis and chronic inflammation as well as glomerular obsolescence were also noted. The oxalate deposit was diffuse and marked, and was thought to be intimately related to the parenchymal damage and fibrosis.

No abstract available.

Acardiac twinning affects 1 in 100 monozygotic twin pregnancies and 1 in 35,000 pregnancies overall. This condition is characterized by the absence or rudimentary development of fetal heart, and associated with various anomaly. The presence of an acardiac twin requires the normal (or "pump") twin to provide circulation for itself, as well as the acardiac sibling. The acardiac malformations are uniformly fatal in the affected twin, and mortality in the co-twin is as high as 55%. The principal perinatal problems associated with acardiac twinning are pump-twin congestive heart failure, maternal hydramnios, and preterm delivery. We recently experienced a case of acardius anceps associated with a normal male infant, so present with a brief review of the literature.
Fetal Heart ; Heart Failure ; Humans ; Infant ; Male ; Mortality ; Polyhydramnios ; Pregnancy ; Siblings ; Twins, Monozygotic

Psoriasis is characterized by disregulation of keratinocyte growth with profound epidermal hyperplasia. Keratinocyte hyperplasia in psoriasis may be expained in part by overproduction of growth factor, and by altered metabolism of the epidemal growth factor receptors (EGFR) in affected skin. The expression of epidermal growth f ictor receptor was investigated by Northern blot and slot-blot analysis of total RNA extrated from biopsies of normal skin and psoriatic lesions. In Northern blot analysis, EGFR-specific mRNA transcripts from psoriatic tissues demonstrated the specificity of hybridizarion with a EGFR mDNA probe. The size of EGFR mRNA transcript was 6.7kb in psoriasis lesions which showed no change of quality. In slot-blot analysis, the levels of EGFR mRNA in poriasis revealed a 1.2 fold to 4.1 fold elevation when compared to normal skin. EGFR were present in all epidermal layers by immunoperoxidase staining, whereas in normal skin they were primarily present in the stratum basalis. These results indicate that the increased expresion of the EGFR gene may be, in part, responsible for the hyperproliferation of the epider nis and that retained EGFR may reflect incomplet; abnormal differentiation in active porasis. This altered process of EGFR metabolism may be involved in the pathogenesis of psoriasis.
Biopsy ; Blotting, Northern ; Epidermal Growth Factor* ; Genes, erbB-1 ; Hyperplasia ; Keratinocytes ; Metabolism ; Psoriasis* ; Receptor, Epidermal Growth Factor* ; Receptors, Growth Factor ; RNA ; RNA, Messenger ; Sensitivity and Specificity ; Skin

BACKGROUND: Mechanisms of secondary injury (post-ischemic injury) in the central nervous system have cently reported in a vast of amount of experiments. Among many factors which give rise to post-ischemic neuronal damage, glial deterioration probably mediated by calcium paradox, could be another of the aggravating deleterious factars to the already ischemic neurophil. METHODS: Here we have designed experiment to investigate calcium paradox in astroglial cell line, humsn asttocytoma U1242MG. Intracellular calcium alterations in experimental cells were monitored by using calcium indicating dye fura-2 and epifluorescent photometry system. RESULTS: Intracellular free calcium changes during reperfusion phase after exposure to low calcium led to a prampt increase in intracellular calcium level after 10 and 30 minutes. The way of calcium entry during the reperfusion phase was mediated by the revase mode of Na+/Ca(2+) exchanger. Cells that had a reduction of reperfusate calcium to 10 uM increased cell viability. Also we observed an inverse relationship between major enzymatic activity in the astrocytoma cells (i.e., glutamine synthetase activity) and the duration of reperfusion in the the same protocols. CONCLUSIONS: A relatively small amount of intracellular calcium increase by the reverse mode of Na+/Ca(2+) exchanger during the reperfusion period is related to a limitation of enzyme activity and viability 24 hours later.
Anesthesia* ; Astrocytoma ; Brain ; Calcium ; Capnography* ; Cell Line ; Cell Survival ; Central Nervous System ; Fura-2 ; Glutamate-Ammonia Ligase ; Ions ; Neuroglia ; Neurons ; Photometry ; Reperfusion

Angiokeratoma circumscriptum is present at birth or early childhood and is an uncommon dermatosis characterized by papules and small nodules that may coalesce to form plaques. Histopathologically, there are varying degrees of hyperkeratosis, papillomatosis, and irregular acanthosis. In the papillary dermis, greatly dilated capillaries are observed, The acanthotic epidermis encircles the vascular spaces(blood cysts) where, occasionally, organized thrombi may be found. The use of pulsed-dye lasers to treat cutaneous vascular lesions is based on the theory of selective photothermolysis. We report a case of an angiokeratoma circumscriptum in an 48-year-old woman for whom the flashlamp pulsed tunable dye laser proved to be a highly successful means of treatment.
Angiokeratoma* ; Capillaries ; Dermis ; Epidermis ; Female ; Humans ; Lasers, Dye* ; Middle Aged ; Papilloma ; Parturition ; Skin Diseases

Hailey-Hailey disease(benign familial chronic pemphigus) is a rare autosomal dominant disorder characterized by blisters at sites of friction such as the neck, axillae and groin which are caused by suprabasal epidermal acantholysis. We report two cases of Hailey-Hailey disease in the one family. One of the two cases has asymptomatic multiple longitudinal white bands in the fingernails associated with typical skin lesions. The nail lesions have not been described until reported by Burge in 1992 and it may be a characteristic finding in Hailey-Hailey disease.
Acantholysis ; Axilla ; Blister ; Friction ; Groin ; Humans ; Nails ; Neck ; Pemphigus, Benign Familial* ; Skin

Kaposis sarcoma is a multicentric neoplastic vascular tumor involving the skin or internal organs. We report a case of classic Kaposis sarcoma in a 69-year-old male who had positive serum cytomegalovirus antibodies and disseminated multiple erythematous to purplish colored confluent papules and edema on both lower legs. The histopathological finding showed abnormally proliferated and dilated vessels, vascular spaces, spindle cells, and extravasated erythrocytes. He was treated with radiotherapy, but died due to ventricular tachycardia.
Aged ; Antibodies ; Cytomegalovirus ; Edema ; Erythrocytes ; Humans ; Leg* ; Male ; Radiotherapy ; Sarcoma, Kaposi* ; Skin ; Tachycardia, Ventricular

The monoclonal antibody Ki-1(CD30) was first described in 1982 and was reported to react selectively with Reed-Sternberg cells in Hodgkins disease, highly activated B and T cells and large cell lymphomas of both T-and B-cell origin. Lymphomatoid papulosis(LyP) is characterized by recurrent erythematous papules or nodules that undergo spontaneous healing with hyperpigmentation and scarring. Histologically, two major types of LyP can be distinguished: type A is characterized by the presence of variable numbers of Ki-1+ large, atypical lymphocytes with some Reed-Sternberg like cells and type B is characterized by Ki-1- atypical cerebriform mononuclear cells similar to those in mycosis fungoides. We report a case of Ki-1+ lymphomatoid papulosis which was histopathologically recognized as a type B lesion but represented a Ki-1 positive reaction.
B-Lymphocytes ; Cicatrix ; Hodgkin Disease ; Hyperpigmentation ; Lymphocytes ; Lymphoma ; Lymphomatoid Papulosis* ; Mycosis Fungoides ; Reed-Sternberg Cells ; T-Lymphocytes