We reviewed retrospectively the medical records of 229 cases of tuberculosis, who had been diagnosed clinically and admitted to the departmet of pediatrics, Seoul National Univeristy Children's Hospital, during the period of 6 years from October, 1985, to October, 1991. Patients with tuberculosis occupied 1.2% of the hospitalized patients. Forty-three percent of the patients were under 3 years of age. Principal involvement sites were; the lungs-26.7%, the pleura-18.8%, the central nervous system-30.6%, the abdomen-7.4%, the genito-urinary tract-1.3%, the bones and joints-3.0%, the lymph nodes-3.0%. In 147 patients with extrapulmonary tuberculosis, pulmonary lesions suggestive of tuberculosis were found in 33.3%, 9.2% of the patients had miliary dissemination. In pulmonary tuberculosis and extrapulmonary tuberculosis with lung involvement, the gastric aspirates were positive for acid-fast bacilli (AFB) by Ziehl-Neelson stain in 6.1%, and grew M. tuberculosis in 43.5%. The sputa were positive for AFB in 12.9%, culture in 18,5%. In extrapulmonary tuberculosis, the specimens from involved sites were positive for staining in 5.7%, culture in 19.3%. Twenty-one isolates of M. tuberculosis were submitted to susceptibility to 11 antituberculous drugs. Resistance to one or more drugs were found in 38.1% of the isolates. INH resistance was found in 33.3%, RFP resistance in 28.6%. We conclude that bacteriologic documentation of tuberculosis is possible in significant proportion of the pediatric patients, drug resistance of M. tuberculosis isolated from the children is high in Korea, and continued monitoring of drug resistance is required.
Child ; Drug Resistance ; Humans ; Korea ; Lung ; Medical Records ; Pediatrics ; Retrospective Studies ; Seoul ; Tuberculosis* ; Tuberculosis, Pulmonary

Neurofibromatosis type 1 (von Recklinghausen disease, NF1) involves the central and peripheral nervous systems as well as the skin, bone, endocrine, gastrointestinal and vascular systems. The gastrointestinal neurofibroma associated with NF1 has been infrequently reported. We report our experience with a 15-year-old boy who had a gastric plexiform neurofibroma with upper gastrointestinal bleeding and underwent a tumorectomy because of massive upper gastrointestinal bleeding. We conclude that gastrointestinal bleeding and anemia in the setting of NF1 mandates complete endoscopic examination of the digestive tract to rule out neurofibromas. Surgical resection is the standard treatment.
Adolescent ; Anemia ; Gastrointestinal Tract ; Hemorrhage* ; Humans ; Male ; Neurofibroma* ; Neurofibroma, Plexiform ; Neurofibromatoses ; Neurofibromatosis 1* ; Peripheral Nervous System ; Skin ; Stomach

Previous reports have suggested that a high serum cyclosporine A (CsA) level could result in a lower incidence of acute-graft-versus-host disease (aGVHD). An elevated serum lactate dehydrogenase (LDH) level has been reported to be an adverse predictor of outcome in stem cell transplantation (SCT) for acute myeloid leukemia. In this study, we retrospectively analyzed the records of 24 patients who received allogeneic SCT from an HLA-matched sibling donor for acute and chronic myelogenous leukemia. Univariate analysis showed that two factors (the serum CsA level at the third week after SCT and the LDH level at the third week after SCT) were significantly associated with the incidence of aGVHD among several variables (age, sex, stem cell source, cell dose, C-reactive protein, absolute lymphocyte count, conditioning regimens, and time to engraftment). A higher serum level of CsA and lower serum LDH level at the third week after SCT were associated with a lower incidence of aGVHD (P=0.015, 0.030). In multivariate analysis, the serum CsA level (hazard ratio [HR], 0.12; 95% confidence interval [CI], 0.022-0.652, P=0.0014) and serum LDH level (HR, 6.59; 95% CI, 1.197-36.316, P=0.030) at the third week after SCT were found to be independent factors that were significantly associated with the development of aGVHD. We conclude that a high CsA level and low LDH level might predict a low cumulative incidence of aGVHD after allogeneic transplantation from a matched sibling donor.
Acute Disease ; Adult ; Cyclosporine/*blood ; Female ; Graft vs Host Disease/*epidemiology/etiology ; Humans ; L-Lactate Dehydrogenase/*blood ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive/therapy ; Leukemia, Myeloid, Acute/therapy ; Male ; Middle Aged ; Multivariate Analysis ; Predictive Value of Tests ; Retrospective Studies ; Risk Factors ; *Stem Cell Transplantation ; Transplantation, Homologous

Swiprosin-1 exhibits the highest expression in CD8+ T cells and immature B cells and has been proposed to play a role in lymphocyte biology through actin remodeling. However, regulation of swiprosin-1 gene expression is poorly understood. Here we report that swiprosin-1 is up-regulated in T cells by PKC pathway. Targeted inhibition of the specific protein kinase C (PKC) isotypes by siRNA revealed that PKC-theta is involved in the expression of swiprosin-1 in the human T cells. In contrast, down-regulation of swiprosin-1 by A23187 or ionomycin suggests that calcium-signaling plays a negative role. Interestingly, swiprosin-1 expression is only reduced by treatment with NF-kappaB inhibitors but not by NF-AT inhibitor, suggesting that the NF-kappaB pathway is critical for regulation of swiprosin-1 expression. Collectively, these results suggest that swiprosin-1 is a PKC-theta-inducible gene and that it may modulate the late phase of T cell activation after antigen challenge.
Actins ; Biology ; Calcimycin ; Down-Regulation ; Gene Expression ; Humans ; Ionomycin ; Lymphocytes ; NF-kappa B ; Precursor Cells, B-Lymphoid ; Protein Kinase C ; Protein Kinases ; RNA, Small Interfering ; T-Lymphocytes

Krabbe disease is a rare autosomal recessive disorder clinically characterized by retardation in motor development, prominent spasticity, seizures, and optic atrophy. Pathologically, there are many globoid cells in the white matter, in addition to the lack of myelin and the presence of severe gliosis. Hence Krabbe disease is known as globoid cell leukodystrophy. Biochemically, the primary enzymatic deficiency in Krabbe disease is galactocerebroside beta-galactosidase. Patients with Krabbe disease can be subdivided into the early-onset type and late-onset type, according to the onset of clinical manifestations. Most patients with early-onset type die before their second birthday. We describe a girl with Krabbe disease associated with uncontrolled seizures, which was confirmed with biochemical study and MRI. The clinical findings of this patient included hyperirritability, scissoring of the legs, flexion of arm, and clenching of the fists, and generalized tonic seizures. EEG showed hypsarrhythmia, and MRI demonstrated degenerative white matter changes in bilateral periventricular white matter, posterior rim of internal capsule, basal ganglia and brain stem on T2W1 and FLAIR image. The diagnosis was based on clinical features of progressive neurologic deterioration in conjunction with low galactocerebroside beta-galactosidase activity.
Infant ; Male ; Female ; Humans

Many interventional cardiologists rely upon percutaneous coronary intervention (PCI) with drug-eluting stents (DES), but DES may provoke serious complications, such as stent thrombosis and delayed restenosis. Previous studies of bare metal stent placement showed regression of neointimal proliferation after 6 months. Here, we report the case of a 50-year-old man demonstrating spontaneous regression of neointimal hyperplasia after undergoing PCI with a bare metal stent to treat a middle left anterior descending (LAD) artery lesion. Coronary angiography showed 90% diffuse restenosis at 6 months after stent placement, but the patient refused PCI due to monetary concerns. After 29 months, follow-up angiography revealed spontaneous regression of restenosis in the middle LAD.
Angiography ; Arteries ; Coronary Angiography ; Coronary Restenosis ; Drug-Eluting Stents ; Follow-Up Studies ; Humans ; Hyperplasia ; Middle Aged ; Percutaneous Coronary Intervention ; Stents ; Thrombosis

Granulocytic sarcoma is a localized tumor that's composed of immature granulocytic cells and this is more common in patient with 8;21 translocation. We present here a case in a 64-year-old man who was diagnosed with acute myelogenous leukemia (erythroleukemia) that had a complex hyperdiploid karyotype. While he underwent chemotherapy, he developed nausea, vomiting, headache and dysarthria. After several diagnostic work-ups, granulocytic sarcoma in the cerebellum and leptomeningeal metastasis of his leukemia were found on the magnetic resonance imaging and the cerebrospinal fluid cytology.
Brain ; Cerebellum ; Dysarthria ; Headache ; Humans ; Karyotype ; Leukemia ; Leukemia, Erythroblastic, Acute ; Leukemia, Myeloid, Acute ; Magnetic Resonance Imaging ; Middle Aged ; Nausea ; Neoplasm Metastasis ; Sarcoma ; Sarcoma, Myeloid ; Vomiting

Enterocytozoon bieneusi is a microsporidian pathogen. Recently, the equestrian population is increasing in Korea. The horse-related zoonotic pathogens, including E. bieneusi, are concerns of public health. A total of 1,200 horse fecal samples were collected from riding centers and breeding farms in Jeju Island and inland areas. Of the fecal samples 15 (1.3%) were PCR positive for E. bieneusi. Interestingly, all positive samples came from Jeju Island. Diarrhea and infection in foals were related. Two genotypes (horse1, horse2) were identified as possible zoonotic groups requiring continuous monitoring.

Although the prognosis of papillary thyroid cancer (PTC) is extremely good, locoregional recurrences after initial treatment occur. Thyroglobulin (Tg) is a reliable tumor marker to detect recurrence or persistence of PTC. However, occasionally serum Tg may miss the detection of a recurrence. We report a 54-year-old female presented with hoarseness due to cervical recurrence without concomitant elevation of serum Tg and anti-Tg antibody, in contrast to extremely increased needle-washout Tg, who had undergone a total thyroidectomy and radioiodine ablation as initial therapies for PTC. Several factors causing such discrepancy between needle-washout Tg and serum Tg can be suggested including site of recurrence, volume of tumor, interference by some kind of plasma antibodies other than anti-Tg antibody, and any conformational defect of Tg protein. Among them, the most convincing explanation is that any conformational defect of Tg may lead to impaired secretion of Tg to blood. We suggest that more studies are needed to find the cause for potential mechanisms involved in PTC recurrences without increased serum Tg.
Antibodies ; Female ; Hoarseness ; Humans ; Middle Aged ; Plasma ; Prognosis ; Recurrence ; Thyroglobulin* ; Thyroid Neoplasms* ; Thyroidectomy

Cholangiocarcinoma (CC) is an intrahepatic bile duct carcinoma with a high mortality rate and a poor prognosis. Sarcomatous change/epithelial mesenchymal transition (EMT) of CC frequently leads to aggressive intrahepatic spread and metastasis. The aim of this study was to identify the genetic alterations and gene expression pattern that might be associated with the sarcomatous change in CC. Previously, we established 4 human CC cell lines (SCK, JCK1, Cho-CK, and Choi-CK). In the present study, we characterized a typical sarcomatoid phenotype of SCK, and classified the other cell lines according to tumor cell differentiation (a poorly differentiated JCK, a moderately differentiated Cho-CK, and a well differentiated Choi-CK cells), both morphologically and immunocytologically. We further analyzed the genetic alterations of two tumor suppressor genes (p53 and FHIT) and the expression of Fas/FasL gene, well known CC-related receptor and its ligand, in these four CC cell lines. The deletion mutation of p53 was found in the sarcomatoid SCK cells. These cells expressed much less Fas/FasL mRNAs than did the other ordinary CC cells. We further characterize the gene expression pattern that is involved in the sarcomatous progression of CC, using cDNA microarrays that contained 18,688 genes. Comparison of the expression patterns between the sarcomatoid SCK cells and the differentiated Choi-CK cells enabled us to identify 260 genes and 247 genes that were significantly over-expressed and under-expressed, respectively. Northern blotting of the 14 randomly selected genes verified the microarray data, including the differential expressions of the LGALS1, TGFBI, CES1, LDHB, UCHL1, ASPH, VDAC1, VIL2, CCND2, S100P, CALB1, MAL2, GPX1, and ANXA8 mRNAs. Immunohistochemistry also revealed in part the differential expressions of these gene proteins. These results suggest that those genetic and gene expression alterations may be relevant to the sarcomatous change/EMT in CC cells.
Acid Anhydride Hydrolases/genetics ; Animals ; Cell Line, Tumor ; Cholangiocarcinoma/*genetics ; Female ; *Gene Expression Profiling ; Humans ; Mice ; Mice, Inbred BALB C ; Mutation ; Neoplasm Proteins/genetics ; Oligonucleotide Array Sequence Analysis ; Sarcoma/*genetics ; Tumor Suppressor Protein p53/genetics