OBJECTIVE: Because of the limited penetration into the central nervous system after systemic administration of numerous therapeutic compounds, intratumoral chemotherapy for brain tumors has also been used. However, the efficacy of intratumoral drug administration is restricted by the poor diffusion of drug through tumor and brain interstitium. In order to enhance the diffusion of chemotherapeutic agent and increase the cytotoxicity with minimal dose, the authors report the results of convection-enhanced delivery(CED) of chemotherapeutic agent to the malignant brain tumor as a method of enhancing cerebral drug delivery. METHODS: Authors used "CADD-Micro(R) ambulatory infusion pump" from Deltec, which can be programmed for continuous infusion. Intratumoral injection of chemotherapeutic drug using the pump was applied to eight patients with glioma and one patient with lymphoma. Surgery was done and tumor was removed as much as possible. The tip of catheter was placed in the center of tumor cavity. Adriamycin (0.16~0.32mg) was put in the reservoir which was connected to the proximal catheter and fixed in the pump device. Twenty-four hours after surgery, Adriamycin was infused. RESULTS: There was no adverse reaction of CED technique. Compared with current delivery techniques, the improvement of survival rate has been observed(5 patients: alive, 3 patients: dead, 1 patient: lost(alive to 5 mo.)). CONCLUSION: CED can be useful method for distributing therapeutic molecules in the interstitial space of tumor and can be utilized for chemotherapeutic agents, immunotoxins, and gene etc..
Brain ; Brain Neoplasms ; Catheters ; Central Nervous System ; Diffusion ; Doxorubicin ; Drug Therapy ; Glioma ; Humans ; Immunotoxins ; Lymphoma ; Survival Rate

PURPOSE: To obtain basic data for development of a glioblastoma-specific immunotoxin, the expression of variable cell surface receptors on a human glioblastoma xenograft model was evaluated, using NOD/SCID mice. MATERIALS AND METHODS: We developed a xenograft model in NOD/SCID mice implanted with a human glioblastoma cell line (U-87MG). Immunohistochemical studies were performed on implanted tumor nodules (n=8) using antibodies against CD71, EGFR, IGF-IRalpha, CXCR4 and IL-4Ralpha. RESULTS: Expression of IL-4Ralpha, in implanted tumornodules, was the highest of the cell surface receptors evaluated in this study. However, the endothelial cells in, and around, the tumor nodules also revealed immunopositivity against IL-4Ralpha. The immunoreactivity of IL-4Ralpha, and other surface receptors such as CD71, IGF-IRalpha and EGFR, was prominent in tumor nodules associated with tumor necrosis. CONCLUSION: IL-4Ralpha would be a possible target for the development of glioblastoma-specific immunotoxin, although there are limitations due to its endothelial expression.
Animals ; Antibodies ; Cell Line ; Endothelial Cells ; Glioblastoma* ; Heterografts* ; Humans* ; Immunotoxins ; Mice* ; Mice, SCID ; Necrosis ; Receptors, Cell Surface*

Central neurocytoma is a rare, well-differentiated neuronal tumor and is usually located in the lateral or third ventricle of young adults. Its overall prognosis is excellent with a low proliferative index. The majority of previously reported malignant variants rarely did recurred after tumor removal and regarded as benign tumor despite of histopathological malignant feature. Nevertheless, we experienced a case of malignant variant of the central neurocytoma with high proliferative index(Ki-67 labeling index >30%), which showed recurrence immediately after surgery and died within 3 months POD. Here, we describe the case with a review of the literatures.
Humans ; Neurocytoma* ; Neurons ; Prognosis ; Recurrence ; Third Ventricle ; Young Adult

PURPOSE: An experimental study was carried out to evaluate the morphological changes in the blood brain barrier and neighbouring brain tissue caused by Gd-DTPA in uremia-induced rabbits. MATERIALS AND METHODS: Bilateral renal arteries and veins of ten rabbits were ligated. Gd-DTPA(0.2 mmol/kg) was intravenously injectedinto seven rabbits immediately after ligation. After MRI, they were sacrificed 2 or 3 days after ligation in orderto observe light and electron microscopic changes in the blood brain barrier and brain tissue. RESULTS: MRIfindings were normal, except for enhancement of the superior and inferior sagittal sinuses on T1 weighted imagesin uremia-induced rabbits injected with Gd-DTPA. On light microscopic examination, these rabbits showed perivascular edema and glial fibrillary acidic protein expression ; electron microscopic examination showed separation of tight junctions of endothelial cells, duplication/rarefaction of basal lamina, increased lysosomes of neurons with neuronal death, demyelination of myelin, and extravasation of red blood cells. Uremia-induced rabbits injected with Gd-DTPA showed more severe changes than those without Gd-DTPA injection. CONCLUSION: Injuries to the blood brain barrier and neighbouring brain tissue were aggravated by Gd-DTPA administration inuremia-induced rabbits. These findings appear to be associated with the neurotoxicity of Gd-DTPA.
Animals ; Basement Membrane ; Blood-Brain Barrier* ; Brain Injuries ; Brain* ; Demyelinating Diseases ; Edema ; Endothelial Cells ; Erythrocytes ; Gadolinium DTPA* ; Glial Fibrillary Acidic Protein ; Ligation ; Magnetic Resonance Imaging ; Myelin Sheath ; Neurons ; Rabbits* ; Renal Artery ; Tight Junctions ; Veins

PURPOSE: This study was aimed to investigate the relationship between the mandibular asymmetry and the internal derangement of temporomandibular joint. MATERIALS AND METHODS: One hundred and sixty eight patients had been assessed through clinical examinations, panoramic radi-ographs and magnetic resonance imagings (MRIs), were selected. The samples were classified into three subgroups according to the severity of the mandibular asymmetries in the panoramic radiographs and the status of TMJ discs on the MRI were compared among each groups. RESULTS: In an apparent asymmetry group, there was a significant difference in the number of temporomandibular disk displacement without reduction between the long and short side (66.7%, 18/27 joints on the short side) when the ratio of condylar process and coro-noid process was used (P<0.05), but there was no statistically significant difference when the ratio of condyle and ramus was used. CONCLUSION: The probability of the disc displacement without reduction was higher at the side with relatively shorter condylar process on the panoramic radiograph, and also it might be more effective to use ratio of condylar process and coronoid process in the assessment of mandibular asymmetry. Therefore, a careful assessment on the temporomandibular disorders is necessary to diagnose and establish the treatment plans for the patients with a mandibular asymmetry and the panoramic radiograph can be used effectively on that way.
Humans ; Joints ; Magnetic Resonance Imaging* ; Temporomandibular Joint Disc* ; Temporomandibular Joint Disorders ; Temporomandibular Joint*

Wolf-Hirschhorn syndrome (WHS) is a congenital anomaly associated with partial deletion of distal short arm of chromosome 4, characterized by distinct facial anomalies ("Greek-Helmet"), mental and growth retardation and midline defects. The deletion of 2.2Mb area of 4p16.3 is thought to be the critical for WHS. It is reported that the sizes of 4p deletions are variable and the clinical findings are different from classic WHS according to the deleted portion. Recently proximal interstitial deletions have been infrequently reported. On fetal ultrasonography, a fetus of this case was found to have intrauterine growth retardation and multiple somatic anomalies including cleft palate, cleft lip, club foot, and hypoplastic thoracic cage with cardiomegaly, hypoplasia of right kidney and small stomach. At 36th week of gestation, the female baby was born by normal vaginal delivery, and her body weight was only 2.1kg. In spite of vigorous resuscitative measures, she died of respiratory failure on the second day of life. Karyotype was 46,XX,del (4) (p15.2). It is supposed that the size of deleted 4p in patients with WHS doesn't have substantial influence on the phenotype of the disease if the critical region in 4p16.3 is also deleted.
Arm ; Body Weight ; Cardiomegaly ; Chromosomes, Human, Pair 4 ; Cleft Lip ; Cleft Palate ; Female ; Fetal Growth Retardation ; Fetus ; Foot ; Humans ; Karyotype ; Kidney ; Phenotype ; Pregnancy ; Respiratory Insufficiency ; Stomach ; Ultrasonography, Prenatal ; Wolf-Hirschhorn Syndrome*

BACKGROUND: Several studies have been conducted on the role of the p63 gene family in non-small cell lung carcinoma (NSCLC). Nevertheless, the role of these genes in the development and progression of NSCLC remains controversial. This study was designed to examine the expression and clinicopathologic significance of the p63 family in NSCLC. METHODS: Immunohistochemical staining was performed on 92 cases of NSCLC (47 squamous cell carcinomas [SqCCs] and 45 adenocarcinomas [ACs]) using tissue microarray blocks. The results were analyzed and correlated with clinicopathologic data. RESULTS: The expression of delta Np63 (Delta Np63) was elevated in SqCC (39/47), but not in AC (2/45; p<0.01). Both p63 and Delta Np63 had high expression in 39 SqCCs; p63 and Delta Np63 also had a similar geomorphologic distribution in most positive tumors. The expression of Delta Np63 was correlated with histologic type, gender, pT stage, p53 expression, and p63 expression. pT and pN stages were independent factors in survival (p<0.05, respectively). CONCLUSIONS: The major p63 isoform in NSCLC, Delta Np63, had a strong correlation with p53 and p63, and was exclusively expressed in SqCC. However, our findings suggest that Delta Np63 was not an independent prognostic factor for NSCLC.
Adenocarcinoma ; Carcinoma, Non-Small-Cell Lung ; Carcinoma, Squamous Cell ; Humans ; Immunohistochemistry ; Lung

Background: Acinetobacter baumannii infections cause high morbidity and mortality in intensive care unit (ICU) patients. However, there are limited data on the changes of longterm epidemiology of imipenem resistance in A. baumannii bacteremia among pediatric ICU (PICU) patients. Methods: A retrospective review was performed on patients with A. baumannii bacteremia in PICU of a tertiary teaching hospital from 2000 to 2016. Antimicrobial susceptibility tests, multilocus sequence typing (MLST), and polymerase chain reaction for antimicrobial resistance genes were performed for available isolates. Results: A. baumannii bacteremia occurred in 27 patients; imipenem-sensitive A. baumannii (ISAB, n = 10, 37%) and imipenem-resistant A. baumannii (IRAB, n = 17, 63%). There was a clear shift in the antibiogram of A. baumannii during the study period. From 2000 to 2003, all isolates were ISAB (n = 6). From 2005 to 2008, both IRAB (n = 5) and ISAB (n = 4) were isolated. However, from 2009, all isolates were IRAB (n = 12). Ten isolates were available for additional test and confirmed as IRAB. MLST analysis showed that among 10 isolates, sequence type 138 was predominant (n = 7). All 10 isolates were positive for OXA-23-like and OXA-51-like carbapenemase. Of 27 bacteremia patients, 11 were male (41%), the median age at bacteremia onset was 5.2 years (range, 0–18.6 years). In 33% (9/27) of patients, A. baumannii was isolated from tracheal aspirate prior to development of bacteremia (median, 8 days; range, 5–124 days). The overall case-fatality rate was 63% (17/27) within 28 days. There was no statistical difference in the case fatality rate between ISAB and IRAB groups (50% vs. 71%; P = 0.422). Conclusion IRAB bacteremia causes serious threat in patients in PICU. Proactive infection control measures and antimicrobial stewardship are crucial for managing IRAB infection in PICU.

Approximately 15% of patients with frontotemporal dementia (FTD) have co-occurring motor neuron disease (MND). FTD-MND cases have frontotemporal lobar degeneration (FTLD)-transactive response DNA-binding protein (TDP) pathology, which is divided into four subtypes (types A, B, C, and D) based on the morphological appearance, cellular location, and distribution of the abnormal TDP inclusions and dystrophic neurites. We report a patient with FTD-MND whose pathological diagnosis was FTLD-TDP type B. This is the first documented autopsy-confirmed case of FTD-MND in Korea.
Autopsy* ; Diagnosis ; Frontotemporal Dementia* ; Frontotemporal Lobar Degeneration ; Humans ; Korea ; Motor Neuron Disease* ; Motor Neurons* ; Neurites ; Pathology

Approximately 15% of patients with frontotemporal dementia (FTD) have co-occurring motor neuron disease (MND). FTD-MND cases have frontotemporal lobar degeneration (FTLD)-transactive response DNA-binding protein (TDP) pathology, which is divided into four subtypes (types A, B, C, and D) based on the morphological appearance, cellular location, and distribution of the abnormal TDP inclusions and dystrophic neurites. We report a patient with FTD-MND whose pathological diagnosis was FTLD-TDP type B. This is the first documented autopsy-confirmed case of FTD-MND in Korea.
Autopsy* ; Diagnosis ; Frontotemporal Dementia* ; Frontotemporal Lobar Degeneration ; Humans ; Korea ; Motor Neuron Disease* ; Motor Neurons* ; Neurites ; Pathology