Xeroderma pigmentosum is a hereditary disorder characterized by the early development of pigmentary chnges, atropht, keratoses, and carcinoma, predominantly light exposed area skin.Two cases of xeroderma pigmentosum complicated by basal cell Ca. were presented. The one was 15 years old male who had been suffered from pea to walnut sized multiple tumors and ulcers on the face with scattered brownish macuIes on the eun exposed area since about 9 years old of his age. The other was 22 years old maIe who had been suffered from the same skin lesions as the former on the face, neck and dorsum of hands. In both cases skin lesions aggravated during each summer seasons and there were on history of convulsions and other neurological symptoms Histopathological findings of tumors on both cases revealed typical findings of basal cell Ca.
Adolescent ; Child ; Hand ; Humans ; Ichthyosis* ; Juglans ; Keratosis ; Male ; Neck ; Peas ; Seasons ; Seizures ; Skin ; Ulcer ; Xeroderma Pigmentosum* ; Young Adult

No abstract available.
Myxoma*

No abstract available.
Leukemia*

No abstract available.
Biomarkers* ; Leukemia, Myeloid, Acute*

We experienced a case of postoperative quadriparesis secondary to operative position in a patient with pre-existing, asymtomatic,cervical neurilemmoma. The patient was admitted with chronic otitis media for mastoidectomy on right side. There was no specific event throughout operation and anesthesia but developed quadriparesis one hour after arrival on ward. With magnetic resonance imaging, the patient was dignosed to have cervical neurilemmoma. On seventh postoperative day the patient had second operation,cervical laminectomy and tumor removal. Ten days after second operation he was discharged without any complication. Eight months later he had mastoidectomy on the left side and was discharged without any sequale.
Anesthesia ; Anesthesia, General* ; Humans ; Laminectomy ; Magnetic Resonance Imaging ; Neurilemmoma ; Otitis Media ; Quadriplegia*

BACKGROUND: Hepatitis B virus (HBV)-associated glomerulonephritis (HBGN) occurs with high prevalence in Asia, and accounts for over 30% of secondary glomerulonephritis in Korea. However, the association between HLA and HBGN has been hardly reported upon in the literature. METHODS: A total of 50 Korean patients with HBGN, 100 HBsAg (-) healthy controls and 89 HBsAg (+) controls (subjects with chronic HBV infection, HBsAg positive at least for 6 months) were included. HLA-DR typing was done using a reverse sequence specific oligonucleotide typing kit and HLA-DRB1 genotyping was done for HLA-DR2 positive samples by PCR-single strand conformational polymorphism method. RESULTS: In the HBGN patients, HLA-DR2 was highly significantly increased compared with HBsAg (-) controls (p=0.0002, corrected p=0.002, OR=4.0) and also compared with HBsAg (+) controls (p= 0.0005, corrected p=0.006, OR=3.7). Different HLA- DR2 alleles were strongly associated with different pathologic subtypes of HBGN: DRB1*1502 was associated with membranoproliferative glomerulonephritis (MPGN) (p=0.0003, corrected p=0.004, OR=14.5), and DRB1*1501 with membranous nephropathy (MN) (p= 0.05, OR=3.8). These associations were also found to be significant compared with HBsAg (+) controls (HBV-MPGN, p=0.002; HBV-MN, p=0.04). DR13 was found to have some protective effect in HBV infection (p=0.01, OR=0.3) and DR11 was found to be weakly associated with HBV infection (p=0.01, OR= 4.6), however these HLA alleles were not associated with disease susceptibility to HBGN. CONCLUSION: These results suggest that HLA- DR2 or a closely associated genetic factor is associated with disease susceptibility to HBGN, and different HLA-DR2 subtypes are associated with different pathologic subtypes of HBGN in Koreans.
Alleles ; Asia ; Disease Susceptibility ; Fibrinogen ; Glomerulonephritis* ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Hepatitis B Surface Antigens ; Hepatitis B virus ; Hepatitis B* ; Hepatitis* ; HLA-DR Antigens* ; HLA-DR2 Antigen ; HLA-DRB1 Chains ; Humans ; Korea ; Prevalence

Ganglioglioma comprises about 2% of all intracranial neoplasm, however, it is rarely originated from the brain stem. We report a case of ganglioglioma arising from the brain stem. A 15-year-old girl presented with gait disturbance and dysarthria. Magnetic resonance image revealed a high signal intensity mass with cystic cavities in the right cerebellum and the brain stem. The patient underwent subtotal resection and microscopic examination revealed a ganglioglioma. It is suggested that ganglioglioma should be included in the differential diagnosis of posterior fossa tumors, even located in the brain stem.
Adolescent ; Brain Neoplasms ; Brain Stem* ; Brain* ; Cerebellum ; Diagnosis, Differential ; Dysarthria ; Female ; Gait ; Ganglioglioma* ; Humans ; Infratentorial Neoplasms ; Magnetic Resonance Imaging

In thirty patients with acute leukemia and severe aplastic anemia receiving random single donor platelet transfusions, the development of refractoriness by consecutive platelet transfusions with cytapheresis and its relationship to the appearance of anti-platelet antibodies were investigated. The median number of platelet transfusions inducing refractoriness was 13 times, and 20% of the patients remained unrefractory despite of the repeated multiple platelet transfusions up to 20 to 25 times. The results of anti-platelet antibody tasts by the enzyme-linked immunosorbent assay(ELISA) and immunofluorescent techniques(IFT) showed no statistically significant relationship with the refractoriness (p greater than 0.1). Although there was significant correlation between the results of ELISA and IFT, both tests were insufficient to find out refractoriness even with the use of pooled platelets from multiple donors as target cells. This study shows that 13 single donor platelet transfusions result in refractoriness, that both ELISA and IFT are insufficient to detect refractoriness despite of their significant correlation, and that other methods than these are needed in order to detect alloimmunization.
Adolescent ; Adult ; Aged ; Anemia, Aplastic/therapy ; Anemia, Refractory/*etiology ; Antibodies/metabolism ; *Blood Platelets/immunology ; *Blood Transfusion ; Female ; Humans ; Leukemia/therapy ; Male ; Middle Aged

PURPOSE: To compare Icare rebound tonometer (IRT) and Goldmann applanation tonometer (GAT) and investigate the clinical usefulness of IRT. METHODS: In a retrospective study of 131 eyes with glaucoma and glaucoma suspect, intraocular pressure (IOP) was measured with IRT and GAT. The correlation between IRT and GAT and the influence of refractive error (spherical equivalent; SE) and central corneal thickness (CCT) were analyzed. RESULTS: A distinct correlation between IRT and GAT was found. IOP measured with GAT was 2.23 +/- 3.16 mm Hg higher than with IRT (p < 0.001). As CCT increased, IOP was measured higher with both GAT and IRT. IOP in myopic eyes (SE > or = -5 diopter) was measured relatively lower than in hyperopic eyes (SE < -5 diopter) with GAT (+1.50 +/- 0.68 mm Hg) and IRT (+1.88 +/- 0.75 mm Hg). CONCLUSIONS: IRT showed strong correlation with GAT, but IOP measured approximately 2 mm Hg lower. The IOP tends to measure lower at low CCT and in myopic eyes.
Eye ; Glaucoma ; Intraocular Pressure ; Iron-Dextran Complex ; Manometry ; Peptides ; Refractive Errors ; Retrospective Studies

Over a two-year period, immunophenotypic patterns of 266 acute leukemia cases were analyzed using a panel of tests including TdT, SmIg and 9 surface antigens by the immunofluorescence stains for the assessment of the incidence and grade of phenotypic ambiguity (lineage infidelity) and the possible clinical significance of unusual immunophenotypes. Immunophenotypes were classified into four groups according to the degree of ectopic antigen expression. We classified as Group A (91.7%, 244 of 266 cases) those expressing conventional pattern without ectopic antigen. Group B (3.0%, 8 of 266 cases) was defined to have at least two lineage specific markers and single ectopic antigen. Such a "low grade deviation" did not prevent a definite immunodiagnosis. Group C (4.2%, 11 of 266 cases) revealed a promiscuous coexpression of markers related to different lineages, including two cases (0.8%, 2 cases) of biphenotypic leukemia. Group D (1.1%, 3 cases) included unclassifiable immunophenotypes with no antigen or HLA-DR only expression. Both patients with biphenotypic leukemia and one patient with unclassifiable immunophenotypes failed to respond to induction chemotherapy, suggesting a poor prognosis in these patients. The incidence of acute myelogenous leukemia (AML) cases with one or more ectopic surface antigens was 10 (8.1%) of the 124 AML cases. Ectopic antigen expression was seen in 5 (4%) of the 125 B-lineage acute lymphoblastic leukemia (ALL) cases and 3 (25%) of the 12 T-ALL cases. It is concluded that nearly 95% of cases of acute leukemia cases can be diagnosed accurately with immunophenotyping alone including patients with a mild degree of deviation from expected antigenic patterns.(ABSTRACT TRUNCATED AT 250 WORDS)
Acute Disease ; Antigens, Differentiation/blood ; Burkitt Lymphoma/immunology ; Humans ; Immunophenotyping ; Leukemia/*immunology ; Leukemia, Myeloid/immunology ; Leukemia-Lymphoma, Adult T-Cell/immunology ; Retrospective Studies