Kimura's disease is a benign, uncommon, chronic inflammatory condition that usually presents with painless subcutaneous nodules or plaques. Head and neck are the most frequently involved sites in Kimura's disease. Mandible is the most commonly involved, followed by neck, cheek, scalp and forehead. Other possible sites are oral cavity, inguinal area and extremities, but there have been no reports involving the nose, especially the one that looks like a rhinophyma. We describe a case of Kimura's disease presenting like a rhinophyma.
Cheek ; Extremities ; Forehead ; Head ; Mandible ; Mouth ; Neck ; Nose ; Rhinophyma ; Scalp

Progressive renal fibrosis is considered to be the final common pathway leading to chronic renal insufficiency, however, the mechanism regarding renal fibrosis in renal injury is not well understood. Recently, several kinds of cytokines have been known to be related to fibrosis after renal injury. The interaction between elements regulating fibrogenesis would be better understood by looking at the effect of TGF-beta1 on the synthesis and accumulation of extracellular matrix, especially collagenous proteins. Crescentic glomerulonephritis (CGN) was induced in New Zealand White rabbits by administration of guinea pig anti-GBM IgG after sensitization with guinea pig IgG; and their kidneys were analyzed for the development of crescents and fibrosis through sequential renal biopsies. Serum creatinine levels in a time course progressively increased until day 15. We semi-quantitatively assayed the levels of the expression of alpha1(I) collagen mRNA and TGF-beta1 mRNA factored for GAPDH mRNA using RT-PCR. We observed a progressive interstitial fibrosis and the expression of collagen I both in the cortex and medulla. The effect of repeated renal biopsy itself on pathology and on the expression of alpha1(I) collagen mRNA and TGF-beta1 mRNA in a time course were not significant, but a very mild increase of the expression of alpha1(I) collagen mRNA was noted at day 15. Histology showed a progressive crescent formation and interstitial fibrosis in a time course that roughly paralleled the expression of alpha1(I) collagen mRNA in both cortex and medulla. TGF-beta1 mRNA was hardly expressed at day 0 in cortex as well as in medulla. It was elevated from day 1, peaked at day 7, and then decreased. In medulla, TGF-beta1 mRNA was noticeably expressed at day 1, peaked at day 4, and then decreased. The expression of alpha1(I) collagen mRNA was seen even before inducing CGN. It was gradually and continuously increased until day 15 both in cortex and medulla. These results suggest that the expression of TGF-beta1 mRNA precedes that of alpha1(I) collagen mRNA in the early stage of CGN and has a central role for provoking the accumulation the collagen I, the most representative interstitial extracellular matrix, in the rabbit model CGN induced by anti-GBM antibody. We conclude that the measurement of the expression of TGF-beta1 mRNA and/or alpha1(I) collagen mRNA in a biopsy sample can be a useful predictor for renal outcome.
Animals ; Anti-Glomerular Basement Membrane Disease ; Biopsy ; Collagen ; Creatinine ; Cytokines ; Extracellular Matrix* ; Fibrosis ; Glomerulonephritis* ; Guinea Pigs ; Immunoglobulin G ; Kidney ; Pathology ; Rabbits ; Renal Insufficiency, Chronic ; RNA, Messenger* ; Transforming Growth Factor beta ; Transforming Growth Factor beta1

No abstract available.
Niemann-Pick Disease, Type A*

No abstract available.
Spleen*

Recently it is known that Turner's syndrome is frequently associated with hypothyroidism. We report a case of Turner's syndrome associated with subclinical hypothyroidism. A 23-year-old female was admitted to the hospital with complaints of amenorrhea and short stature. She had a mosaicism of 45, X0/46, Xi(X_q) in the cell, cultured from the peripheral blood. The plasma thyroxine and triiodothyronine were normal and there was no clinical symptom of hypothyroidism. But the thyroid-stimulating hormone(TSH) concentration was unusually higher(184 uU/L). She has been treated with the cyclic therapy of conjugated estrogen and medroxyprogesterone, in addition to the thyroxine replacement therapy. After 2 months, the menstruation was restored and TSH was normalized.
Amenorrhea ; Cells, Cultured ; Estrogens ; Female ; Humans ; Hypothyroidism ; Medroxyprogesterone ; Menstruation ; Mosaicism ; Plasma ; Thyroxine ; Triiodothyronine ; Turner Syndrome ; Young Adult

Forth-nine patients with clinically nonfunctioning pituitary adenomas were evaluated clinically, endocrinologically and morphologically in this study.The results obtained were as follows.1) The mean age was 47.1 years(range 23 to 76 years), and 22 were male(44.9%) and 27(55.1%) female.2) The major clinical manifestations of male patients were visual disturbance(72.7%), headache(54.5%), loss of libido(45.5%), but those of female visual disturbance(59.6%), headache(48.1%), amenorrhea(48.1%), loss of body hair(25.9%), and galactorrhea(22.2%).3) All were macroadenomas evaluated by CT scan, and in the male patients 16(72.7%) were grade III and 6(27.3%) grade IV by Hardy classification, and in the female patients 6(22.2%) were grade II, 12(44.4%) grade III, and 9(33.3%) grade IV.4) The elevation of serum prolactin were observed 7(31.8%) out of male, and 24(88.9%) of female.5) Combined stimulation test revealed that GH insufficiency was 89.6%, ACTH 58.9%, LH 58.7%, FSH 51.1 %, and TSH 50.0% and hormone insufficiency more than 4 pituitary hormone was 54.2%.6) Prolactin response to TRH decreased in 12(70.6%) of 17 patients with normal basal prolactin, and 19(76.0%) of 25 with elevated prolactin.7) Immunohistochemistry revealed that null cell adenoma was 57.1%, gonadotrope adenoma 26.5%, plurihormonal adenoma 8.0%, silent corticotrope adenoma 4.0%, thyrotrope adenoma(2.0%), and lactotrope adenoma(2.0%).8) The ultrastructural characteristics examined by electron-microscopy were similar despite of immunohistochemical differences.In summary, the prevalance of clinically nonfunctioning pituitary adenoma was middle aged men and women, and their main symptoms were visual disturbance and headache. Hyperprolactinemia and pituitary hormone insufficiency more than 4 hormone were observed commonly. Most of them were null cell adenoma and gonadotrope adenoma examined by immunohistochemistry. Further study using modern techniques: cell culture, subunit-immunostaining. And Northern blot analysis of mRNA for pituitary hormone or subunit, will be needed to clarify null cell adenomas.
Adenoma ; Adrenocorticotropic Hormone ; Blotting, Northern ; Cell Culture Techniques ; Classification ; Female ; Headache ; Humans ; Hyperprolactinemia ; Immunohistochemistry ; Lymphocytes, Null ; Male ; Middle Aged ; Pituitary Neoplasms ; Prolactin ; RNA, Messenger ; Tomography, X-Ray Computed

BACKGROUND: The automated urine cell analyzer UF-100 (Syxmex co., Japan), flow cytometer-based instrument, has enabled to perform rapid and efficient work. We evaluated the UF-100 by comparing performance in urine sediment testing with counting chamber, standardized method, and traditional manual microscopy widely used in laboratories, and established reference ranges in our hospital. METHODS: Urine samples were obtained from patients in their 20s to 60s who visited hospital for regular check-up between March and April 2007 at Ewha Womans University Mokdong Hospital. We selected randomly a total of 261 samples (male 130, female 131) and evaluated correlations of red blood cell (RBC) and white blood cell (WBC) counts of UF-100 with counting chamber, and manual microscopy. Moreover, we established reference ranges of UF-100 and counting chamber according to CLSI guideline, using 156 urine samples (male 93, female 63) with normal dipstick (strip) test results. RESULTS: The RBC correlation coefficients between UF-100 and counting chamber, UF-100 and manual microscopy, counting chamber and manual microscopy were 0.538, 0.873, and 0.619, respectively. The WBC correlation coefficients between UF-100 and counting chamber, UF-100 and manual microscopy, counting chamber and manual microscopy were 0.992, 0.902, and 0.893, respectively and showed good correlations. The results of UF-100 were higher than counting chamber and manual microscopy. The RBC reference ranges of UF-100 nd counting chamber were 0.5-24.9/microliter (male 0.4-12.2/microliter, female 0.9-38.8/microliter) and 0-4/microliter (male 0-4/microliter, female 0-5/microliter), and the WBC reference ranges of those were 0.9-21.8/microliter (male 0.8-12.6/microliter, female 2.0-23.4/microliter) and 0-7/microliter (male 0-7/microliter, female 0-9/microliter). CONCLUSIONS: The fully automated analyzer UF-100 could be useful to enhance efficiency by labor-saving, turnaround time reduction and improving throughput and to enable standardization. But it is needed for further study including clinical evaluation, because the results and reference ranges between UF-100 and counting chamber or manual microscopy showed considerable differences.
Erythrocytes ; Female ; Humans ; Leukocytes ; Microscopy ; Reference Values

BACKGROUND: The automated urine cell analyzer UF-100 (Syxmex co., Japan), flow cytometer-based instrument, has enabled to perform rapid and efficient work. We evaluated the UF-100 by comparing performance in urine sediment testing with counting chamber, standardized method, and traditional manual microscopy widely used in laboratories, and established reference ranges in our hospital. METHODS: Urine samples were obtained from patients in their 20s to 60s who visited hospital for regular check-up between March and April 2007 at Ewha Womans University Mokdong Hospital. We selected randomly a total of 261 samples (male 130, female 131) and evaluated correlations of red blood cell (RBC) and white blood cell (WBC) counts of UF-100 with counting chamber, and manual microscopy. Moreover, we established reference ranges of UF-100 and counting chamber according to CLSI guideline, using 156 urine samples (male 93, female 63) with normal dipstick (strip) test results. RESULTS: The RBC correlation coefficients between UF-100 and counting chamber, UF-100 and manual microscopy, counting chamber and manual microscopy were 0.538, 0.873, and 0.619, respectively. The WBC correlation coefficients between UF-100 and counting chamber, UF-100 and manual microscopy, counting chamber and manual microscopy were 0.992, 0.902, and 0.893, respectively and showed good correlations. The results of UF-100 were higher than counting chamber and manual microscopy. The RBC reference ranges of UF-100 nd counting chamber were 0.5-24.9/microliter (male 0.4-12.2/microliter, female 0.9-38.8/microliter) and 0-4/microliter (male 0-4/microliter, female 0-5/microliter), and the WBC reference ranges of those were 0.9-21.8/microliter (male 0.8-12.6/microliter, female 2.0-23.4/microliter) and 0-7/microliter (male 0-7/microliter, female 0-9/microliter). CONCLUSIONS: The fully automated analyzer UF-100 could be useful to enhance efficiency by labor-saving, turnaround time reduction and improving throughput and to enable standardization. But it is needed for further study including clinical evaluation, because the results and reference ranges between UF-100 and counting chamber or manual microscopy showed considerable differences.
Erythrocytes ; Female ; Humans ; Leukocytes ; Microscopy ; Reference Values

The object of this study was to investigate the influences of hypoglycemia and hypothermia on the direct current(DC) pontetial changes during cortical spreading depression(CSD) in rats. The induction of CSD was achieved by the application of KCI solution on the cortex of the frontal lobe. Hypoglycemia and hypothermia were induced respectively by insulin injection and the application of an ice pack. The DC potential changes during progressive hypoglycemia and hypothermia were measured with microelectrodes from the cortex of the parietal lobe of rats. Under contril condition, the rate of CSD was one per 5-10 min and the negative shift of DC potential was about 30 mV. The recovery time from negative shift to base line of DC potential was about 40 sec. In rats treated with insulin, the amplitude of DC potential shift was unaffected by hypoglycemia. The recovery time of DC shift was 40+/-2.26 sec at normoglycemia and it was delayed progressively as the blood glucose level lowered. The mean of it was 63+/-8.02 sec at 30 mg/dl and 77.1+/-22.0 sec with the blood glucose falling below 20 mg/dl. The same delay in the recovery time as seen in the hypogylcemia group was observed in rats treated with hypothermia. The recovery time of DC shift was 39.4+/-3.02 sec in normothermia(36.5degrees C), but it was delayed to 61.15+/-4.15 sec at 30degrees C and 96.67+/-14.92 sec at 26degrees C body temperature. This study suggested that each condition of profound hypoglycemia below 30 mg/dl and hypothermia below 30degrees C was to be harmful to the ion homeostasis and the integrity of the cell membrane and it may lead neurons to death.
Animals ; Blood Glucose ; Body Temperature ; Cell Membrane ; Cortical Spreading Depression* ; Frontal Lobe ; Homeostasis ; Hypoglycemia* ; Hypothermia* ; Ice ; Insulin ; Microelectrodes ; Neurons ; Parietal Lobe ; Rats*

BACKGROUND: Despite the diagnostic utility of immunophenotyping for myelodysplastic syndromes (MDS), it has not been widely performed, and reports on this are absent in Korea. We aimed to evaluate the immunophenotypic features of non-blastic granulocytes, monocytes, and blasts in patients with MDS and non-clonal disorders using routine flow cytometry (FCM). Moreover, we evaluated the phenotypic abnormalities of mature cells in leukemic patients. METHODS: Marrow aspirates from 60 patients, including 18 with MDS, 18 with leukemia, and 24 with non-clonal disorders (control group), were analyzed using FCM. Blasts, non-blast myeloid cells, and monocytes were gated based on CD45 expression and side scatter (SSC). The phenotypes were then compared among the 3 groups. RESULTS: Compared to non-clonal disorders, the granulocytic lineages of MDS showed decreased SSC (P=0.005), increased CD45 intensity (P=0.020), decreased CD10-positive granulocytes (P= 0.030), and a higher CD56-positive rate (P=0.005). It is noteworthy that similar results were obtained in the leukemia group, and these findings were not related to the phenotypes of the leukemic cells. Using blast and monocytic gating, useful parameters for generating a differential diagnosis were not found. CONCLUSIONS: Gating the granulocytic region is a relatively easy method for MDS immunophenotyping. Among the parameters studied, SSC, CD10, and CD56 were the most useful for differentiating MDS from non-clonal disorders. While immunophenotypic changes in MDS appear to be useful for differentiating MDS from non-clonal disorders, these changes were also noted in the mature cells of leukemic patients.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Antigens, CD45/metabolism ; Antigens, CD56/metabolism ; Bone Marrow Cells/cytology ; Cell Lineage ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Flow Cytometry ; Granulocytes/*classification ; Humans ; *Immunophenotyping ; Leukemia/diagnosis/pathology ; Male ; Middle Aged ; Monocytes/*classification ; Myelodysplastic Syndromes/*diagnosis ; Neprilysin/metabolism ; Phenotype