Congenital cystic adenomatoid malformation of the lung is a rare form of congenital cystic disease of the lung and associated with developmental arrest of bronchioles in embryonic life. We report 3 cases of congenital cystic adenomatoid malformation which are classified as type I, II and III according to the morphological classification by Stocker et al. The first case was a 6-year-old female with a mass in the lower lobe of the right lung. The mass was composed of several cysts which were filled with inflammatory exudate and lined by ciliated pseudostratified columnar epithelium (type I + II). The second case was a 4-year-old female with a mass in the middle lobe of the right lung. It was composed of numerous small cysts which were uniform sized and contained inflammatory exudate. These were lined by ciliated columnar and pseudostratified columnar epithelium (type II). The third case was a stillborn female at 27 weeks of gestation. There was generalized edema and severe ascites. The left lower lobe consisted of an ill-defined solid area and small cysts. The solid lesion was composed of bronchiole-sized cysts lined by non-ciliated cuboidal epithelium (type II + III).
Female ; Humans ; Cysts

A case of papillary eccrine adenoma on the right hand of a 84-year-old woman is reported. The tumor was 1 cm in diameter, occupying the whole thickness of the dermis. Histologically, the tumor was composed of dilated tubules of various sizes with intraluminal papillary projections, and was surrounded by a fibrous stroma. An immunohistochemical study revealed that the proliferating tubules were composed of the outermost layer of alpha-smooth muscle actin-positive and the inner layer of keratin 14-negative cells. This antigen expression pattern was comparable to that of the normal eccrine secretory coil, which indicates that the tumor differentiated toward the secretory coil of an eccrine sweat gland.
Adenoma* ; Aged, 80 and over ; Dermis ; Female ; Hand ; Humans ; Sweat Glands

No abstract available.
Cytomegalovirus Infections*

No abstract available.
Autopsy* ; Down Syndrome* ; Leukemia* ; Megakaryocyte Progenitor Cells*

No abstract available.
Autopsy* ; Down Syndrome* ; Leukemia* ; Megakaryocyte Progenitor Cells*

PURPOSE: Spinocerebellar ataxia (SCA) is a genetically heterogeneous disease for which more than 30 subtypes have been identified. However, 5 subtypes, SCA1, SCA2, SCA3, SCA6, and SCA7, account for more than 60% of cases. In this study, we report the distribution of these 5 subtypes in Korean patients. MATERIALS AND METHODS: Six hundred and thirty-eight unrelated patients with a presumptive diagnosis of SCA were included in this study. Trinucleotide (CAG) repeat number (TNR) repeat number was determined using fluorescently labeled primers and fragment analysis. RESULTS: A total of 128 unrelated patients (20.1% of all individuals tested) tested positive for SCA subtypes, including SCA1 (5 patients, 3.9% of those testing positive), SCA2 (38 patients, 29.7%), SCA3 (30 patients, 23.4%), SCA6 (39 patients, 30.5%), and SCA7 (16 patients, 12.5%). The mean copy number of pathogenic TNR alleles was 45+/-8.5 for SCA1, 42+/-3.1 for SCA2, 72+/-5.4 for SCA3, 23+/-1.5 for SCA6, and 50+/-11.4 for SCA7. TNR copy number was inversely correlated with onset age in SCA2, SCA6, and SCA7. CONCLUSION: SCA2, SCA3, and SCA6 are common SCA subtypes in Korean patients and could be screened as a first-line test. Expanded pathogenic allele size was associated with early onset age.
Age of Onset ; Alleles ; Diagnosis ; Humans ; Spinocerebellar Ataxias* ; Trinucleotide Repeats

No abstract available.
Animals ; Cryopreservation* ; Embryonic Structures* ; Mice* ; Ovum*

Metabolic acidosis (MA) is associated with increased proteolysis, increased osteoclast activity and blunted cardiac muscle response, but the effect of MA on various clinic al parameters in CAPD patients is not well known. To evaluate the effects of MA on serum albumin level and other nutritional parameters in CAPD patients, we studied 106 CAPD patients retrospectively who have had monthly biochemical measurement and urea kinetic studies every 6 months for more than 2 years. The patients were divided into three groups according to their mean total CO2 (tCO2) level of the 2-year follow-up (Group l; mean tCO2 < 22mM/L, Group ll; 22mM/L < or = mean tCO2 < 26mM/L, and Group lll; mean tCO2 > or = 26mM/L), and the clinical, biochemical, and urea kinetic data were compared among the three groups. 1)The mean age of the subjects was 46.9+/-12.2 years with a sex ratio of 1.2:1, the mean CAPD duration 28.3+/-21.8 months, mean body weight (Bwt) 59.0+/-8.9kg, %Bwt/IBW 104.1+/-11.5%, %LBM/Bwt 75.5+/-11.1% and well-nourished patients by subjective global assessment (SGA) were 65%. 2)The mean BUN, creatinine, total protein and albumin of all patients were 55.6+/-13.6mg/dL, 12.3+/-3.5mg/dL, 6.6+/-0.7g/dL and 4.0+/-0.4g/dL, respectively. In urea kinetic study, the mean NPCR, weekly Kt/ Vurea, SCCr and RRF were 0.96+/-0.16g/kg/day, 2.02+/-0.37, 63.7+/-18.4L/week/1.73m2, and 0.99+/-1.32ml/ min, respectively. 3)The mean age was significantly higher in group l (51.0+/-10.8) than those of group ll (47.0+/-12.4) and lll (42.6+/-11.4) (P<0.05). %Bwt/IBW of group l (114.4+/-15.8%) was also significantly higher than those of group ll (104.6+/-12.6%) and lll (103.5+/-13.7 %) (P<0.05), but there were no significant differences in sex ratio, CAPD duration, %LBM/Bwt, and SGA among the three groups. 4)The mean tCO2 in group l, group ll, and group lll were 20.6+/-1.2mM/L, 23.9+/-1.1mM/L, and 27.3+/-0.8mM/L, respectively. Compared to group lll, group l had significantly higher BUN (61.1+/-14.3 vs. 46.1+/-7.2mg/dL, P<0.05) and serum albumin (4.04+/-0.31 vs. 3.75+/-0.39g/dL, P<0.05), in spite of comparable dialysis dose and albumin loss into dialysate. 5)NPCR (1.02+/-0.21g/kg/day vs. 0.88+/-0.14g/kg/ day, P<0.05) and ultrafiltration volume (1.4+/-0.4 vs. 1.0+/-0.3, P<0.05) were significantly higher in group l than those of group lll. But there were no significant differences in Kt/Vurea, SCCr, RRF, and 24-hour dialysate loss of protein/albumin among the three groups. 6)No differences were observed among the three groups in the changes of body weight, %Bwt/IBW, %LBM/Bwt, BUN, albumin, NPCR, and RRF from the baseline values after the 2-year follow-up. 7)There were significant inverse correlations between the mean tCO2 level and NPCR (r=-0.33, P<0.001), %Bwt/IBW (r=-0.32, P<0.001), RRF (r=-0.29, P<0.005), and serum albumin level (r=-0.24, P<0.05). But, creatinine, %LBM/Bwt, and Kt/Vurea did not show any correlation with the mean tCO2 level. 8)Using stepwise multiple regression analysis, NPCR (beta=-0.3491, P<0.001), %Bwt/IBW (beta=-0.046, P<0.001), and ultrafiltration volume (beta=-0.0012, P< 0.005) were independent factors affecting the mean tCO2 level. In conclusion, low total CO2 level in long-term CAPD patients may reflect increased protein intake and mild to moderate degree of metabolic acidosis may not affect the nutritional status of well-dialyzed CAPD patients.
Acidosis* ; Body Weight ; Creatinine ; Dialysis ; Follow-Up Studies ; Humans ; Myocardium ; Nutritional Status ; Osteoclasts ; Peritoneal Dialysis, Continuous Ambulatory* ; Proteolysis ; Retrospective Studies ; Serum Albumin* ; Sex Ratio ; Ultrafiltration ; Urea

PURPOSE: In the emergency department (ED), there are many cases of routine emergent endoscopic evaluation for patients who ingest liquid household bleach containing sodium hypochlorite. Many researchers have recommended endoscopic evaluation for caustic injury, but there is much controversy over the indication for emergent endoscopy. Because the clinical course of patients who ingest sodium hypochlorite is commonly mild, we investigated the necessity for routine emergent endoscopy. METHODS: We included patients who visited the ED due to ingestion of liquid bleach containing sodium hypochlorite during the 4 years from June 2001 to May 2005. We retrospectively reviewed the demographic characteristics, ingested amount, symptoms and signs, disposition, and endoscopic results. We also tried to find relationships between the variables and the final endoscopic results. RESULTS: Of the 63 patients included in this study, 47 underwent emergent endoscopy in the ED. The endoscopic results were Grade 1 in 31 patients, Grade 2a in 13, Grade 2b in 2, and Grade 3a in 1. Clinically significant results were observed in only 3 (6.4%) cases. The symptoms and signs was not related to the endoscopic results, but diluted ingestion and the amount of ingestion (<100ml) were significantly related to the endoscopic severity. CONCLUSION: The indications for emergent EGD (esophagogastroduodenoscopy) for caustic injury were discussed. In almost all cases, however, the clinical course of the patients ingesting the sodium hypochlorite was not significant. If the patient ingests a small amount of diluted liquid bleach, < 100 ml, and has few symptoms and signs, we recommend follow up in the outpatient department, which will reduce unnecessary emergent endoscopy.
Caustics ; Eating ; Emergency Service, Hospital ; Endoscopy* ; Family Characteristics* ; Follow-Up Studies ; Humans ; Outpatients ; Retrospective Studies ; Sodium Hypochlorite* ; Sodium*

Churg-Strauss syndrome is a rare primary vasculitis called allergic granulomatosis angiitis. There is a substantial overlap of symptoms or signs between different vasculitides, so that a diagnosis of any vasculitis can be determined by a combination of clinical symptoms and laboratory tests. We report a case of a 54-year-old woman who had bronchial asthma, eosinophilia, peripheral polyneuropathy, pulmonary infiltrate, maxillary sinusitis, and extravascular eosinophilic infiltration on skin biopsy. So she was diagnosed as having Churg-Strauss syndrome, but she was antineutrophil cytoplasmic antibodies-negative on immunologic study. She had fever and showed cardiac involvement that was presented as cardiomegaly and pulmonary edema at chest radiography and left ventricular hypertrophy on electrocardiography. After steroid therapy, general condition and skin manifestation were rapidly improved.
Antibodies, Antineutrophil Cytoplasmic ; Asthma ; Biopsy ; Cardiomegaly ; Churg-Strauss Syndrome ; Cytoplasm ; Electrocardiography ; Eosinophilia ; Eosinophils ; Female ; Fever ; Humans ; Hypertrophy, Left Ventricular ; Maxillary Sinus ; Maxillary Sinusitis ; Middle Aged ; Polyneuropathies ; Pulmonary Edema ; Skin ; Skin Manifestations ; Thorax ; Vasculitis