Clinical Utilities of Serum Ferritin as a Marker of Disease Activity and Prognostic Factor in Childhood Tumors / 소아과

PURPOSE: In order to assess the usefulness of serum ferritin as a marker of disease activity and prognostic factor in pediatric malignancy, serum ferritin levels were measured. METHODS: Peripheral blood samplings for ferritin level were made at presentation, in remission following therapy, and in relapse in 95 children with malignancy admitted to the Department of Pediatrics, Kosin University Gospel Hospital between January, 1986 and August, 1995. The patients were comprised of 35 acute lymphoblastic leukemia(ALL), 17 acute myelogenous leukemia(AML), 20 non- Hodgkin's lymphoma(NHL) and 23 neuroblastoma(NB). RESULTS: The mean values of serum ferritin at presentation were 465.3+/-53.9 ng/mL in ALL, 468.9+/-69.4 ng/mL in AML, 274.1+/-69.2 ng/mL in NHL and 337.3+/-64.4 ng/mL in NB. Those values were increased significantly compared to the mean of 20 control children(69.5+/-12.9 ng/mL). The mean values of serum ferritin concentration in remission stage(first, second, and third remission) tend to be lower compared to those in the active stage(at presentation, first relapse and second relapse). But these differences reached a statistical significance only in patients with ALL when the mean values of the active stage were compared to those checked in the remission stage over 12 months(P= 0.0002). Comparison of overall survival according to initial serum ferritin levels(below and above 200 ng/mL) did not show any significant difference in ALL, AML and NHL. However, there was a borderline relationship in NB(relative risk 3.12, P=0.06). CONCLUSION: The study showed that normalization of serum ferritin levels were found in ALL who had continuous, complete remission for more than 12 months. And patients with lower serum ferritin levels were not associated with better survival except in patients with NB in which the lower ferritin group showed borderline significance.

Seizure Types and EEG Findings of Juvenile Onset Idiopathic Generalized Epilepsy

PURPOSE: Juvenile myoclonic epilepsy, juvenile absence epilepsy, and epilepsy wth generalized tonic clonic seizure (GTCS) on awakening are the three syndromes of idiopathic generalized epilepsy of adolescent onset currently included in the classification of epilepsy syndromes of the International League Against Epilepsy (ILAE). Although they differ in their predominant seizure types, the syndromes share several seizure types. Also, there are no unique electrophysiologic or genetic markers. The question of phenotypic overlap and purity have arison. METHODS: We diagnosed 60 patients as idiopathic generalized epilepsy in Seoul National University Children's Hospital from August 1987 to June 1993 were analyzed in aspects of seizure types, electroencephalographic findings and follow up results. Their onset age of seizure was over 8 year old and the follow-up period was minimum 3 year. RESULTS: 1) seizure types : Four groups were defined by seizure type. The group with absence but not myoclonic (group A) were 19 cases (31.7%) and the group with myoclonic but not absence (group B), 12 cases (20.0%), the group with absence and myoclnic (Group C), 4cases (6.7%), and the group with GTCS only (Group D), 25 cases (41.6%). There was a tendency in that absence begins earlier and myoclonic seizure later in each group. 2) epilepsy syndromes : We could classify as 20 cases (33.3%) of jevenile absence epilepsy, 15 cases (15%) of jevenile myoclonic epilepsy, 5 cases (8.4%) of epilepsy with generalized tonic clonic on awakening, and 20 cases (33.3%) of isolated generalized tonic clonic seizure. 3) EEG characteristics by seizure type : 3-4Hz generalized bursts were most frequent in group A (p<0.05) and polyspike discharges were more frequent in group B than group A (p<0.05). The response to photic stimulation were more frequently observed in group B than group A. There was no significant differences in response to hyperventilation between group A and B. CONCLUSION: To define the combination of seizure types occurred in intervals make easy to approach the diagnosis and treatment of idiopathic generalized epilepsy syndromes. We found that the current classification does not include all patients such as isolated generalized tonic clonic seizure in this study. We can expect information from the fields of molecular genetics and neuroimaging to help to define the etiologic basis of many epilepsies and perhaps to refine the present system of classification, more etiologically oriented and disease-specifically.

Threr cases of Hypertensive Encephalopathy in a cute Post-streptococcal Glomerulonephritis: MRI Findings

Two cases of giant cell tumor of bone diagnosed by fine needle aspiration cytology are described. Case 1 was a 28-year-old male who had pain sense for one year at the right distal thigh. His radiologic finding revealed a destructive cortical lesion with soft tissue extension at medial side of epiphysis of the distal femur. Case 2 was a 21-year-old female complaining pain at left distal forearm for eight months and showed a well-demarcated expansile osteolytic lesion with multiseptation, and cortical destruction at epiphysis and metaphysis of the left distal radius on the X-ray. Fine needle aspiration of each lesion was performed. The aspirate of the case 1 revealed moderate cellularity, which was composed of scattered giant cells of osteoclastic type and small round to oval monotonous stromal cells in large areas. Giant cells were evenly distributed in single or small groups and had irregular but abundant cytoplasms with 10 to 20 nuclei in the center. The nuclei showed ovoid shape, fine granular chromatin, and a small but conspicuous nucleolus. Stromal cells were dispersed in isolated pattern or sometimes aggregated in clusters and showed the same nuclei as those of giant cells and scanty cytoplasms. Comparing to case 1, case 2 had a more translucent abundant cytoplasm in the giant cells and more spindled stromal cells. All two cases revealed neither nuclear atypism nor increased abnormal mitoses in both giant and stromal cells, suggesting no evidence of malignancy. Thereafter the lesions were treated with excision and curettage, and histologically confirmed as giant cell tumors of the bone.

Intractable Temporal Lobe Epilepsy in Children : Presurgical Evaluation and Surgical Treatment / 대한소아신경학회지

BACKGROUD: The purpose of our study was to evaluate the usefulness of the ictal semiology, interictal and ictal electro-encephalography(EEG), brain magnetic resonance imaging(MRI), interictal and ictal single photon emission computed tomography(SPECT), positron emission tomography(PET) and neuropsychometry for localization or lateralization of the ictal onset zone, and analyze the result of surgical treatment. METHODS: We evaluated 13 children with intractable temporal lobe epilepsy, who are diagnosed at children's hospital of Seoul National University, from January 1995 to August 1996. The ictal onset zone was determined by ictal semiology, electrophysiologic study, structural and functional neuroimaging, and neuropsychometry. RESULTS: 1) Ictal semiology : Sixty-nine% of the studied children had change of consciousness, 62% had automatism, 31% had aura, 23% had contralateral versive movement, and 23% had contralateral dystonic posturing. Secondarily generalized seizure occured in 38% of them. 2) Interictal EEG showed localized or lateralized value in 8 cases(62%). In interictal EEG, 12 cases(92%) showed epileptiform discharges on the ipsilateral temporal lobe. 3) Magnetic resonance imaging revealed ipsilateral temporal lobe lesion in 8 cases(62%). Interictal SPECT was performed in 6 cases, only one of them had decreased cerebral blood flow on the ipsilateral temporal lobe. Ictal SECT was done in all, 8 cases(67%) had localized or lateralized value. PET was performed in 11 cases, 8 cases(73%) of them had localized or lateralized value. 4) Neuropsychometry was performed in 7 cases, 6 cases(86%) of them had localized or lateralized value. 5) Nine patients underwent epileptic surgery, 6 of them had class I outcome and each one of the rest had class II, class III and class IV. CONCLUSIONS: Electroencephalography, neuropsychometry, MRI, ictal SPECT, and PET are valuable for localization and lateralization of the ictal onset zone, but interictal SPECT is valuless. The outcome after surgery is relatively good, but more extensive study including patient's social, cognitive and emotional status will be necessary.