No abstract available.
Fingers* ; Toes*

No abstract available.
Fingers* ; Toes*

No abstract available.
Surgery, Plastic*

BACKGROUND: The survival rate of children with neuroblastoma has been improved over 20 years, excluding the metastatic disease, in which it does not exceed 20% so far. New treatment modalities have been developed to improve the outcome in metastatic disease. Preoperative chemotherapy reduce the size, the vascularity and the adhesiveness, so increase the resectability of the primary tumor. This retrospective clinical study was designed to review the survival rate in neuroblastoma and to analyze the effect of preoperative chemotherapy in the view point of neoadjuvant therapy on long-term survival in advanced disease. METHODS: One hundred and thirty-four cases were reviewed from 135 patients with neuroblastoma registered at the Department of Pediatrics in Seoul National University Children's Hospital from January, 1985 till December, 1995. The survival rate was reviewed according to the stage. The age, sex of the patients, the stage, anatomical site of the tumor, the level of serum ferritin and neuron-specific enolase were analyzed for the risk factors on survival. RESULTS: Ranges of age at diagnosis were from 1 month to 166 months with the median of 39 months. Five year survival rates and five year disease-free survival rates were 100%, 100% in stage 1(n=5), 90.9%, 90.9% in stage 2(n=13), 43.4%, 40.6% in stage 3(n=19), 27.1%, 19.8% in stage 4(n=95) and 100%, 100% in stage 4S(n=2), respectively. In stage 3, five year survival rate was 52.5% in group receiving neoadjuvant chemotherapy, 28.6% in control group(P=0.02). Five year disease-free survival rate was also noted as 48.6%, 28.6% in each group(P=0.02). In stage 4, five year and ten year survival rates were 27.6%, 23.6% in group receiving neoadjuvant chemotherapy, 26.9%, 0% in control group(P=0.02). Five year and ten year disease-free survival rates were 14.3%, 14.3% in group receiving neoadjuvant chemotherapy, 20%, 0% in control group (P=0.11). In univariate analysis, the age, the stage, and the site of primary tumor appeared to affect the long-term survival. CONCLUSION: Neoadjuvant chemotherapy and delayed primary surgery contribute for advance in survival in advanced neuroblastoma via increasing the resectability of the primary tumor.
Adhesiveness ; Child ; Diagnosis ; Disease-Free Survival ; Drug Therapy* ; Ferritins ; Humans ; Neoadjuvant Therapy ; Neuroblastoma ; Pediatrics ; Phosphopyruvate Hydratase ; Retrospective Studies ; Risk Factors ; Seoul ; Survival Rate

We report a case of recurrent choroidal neovascularization (CNV) in an eye with chorioretinal coloboma. A 36-year-old woman presented complaining of decreased visual acuity (VA) in her left eye. Best corrected visual acuity (BCVA) was 20/200 and iris coloboma was observed. Funduscopy and fluorescein angiography (FA) showed CNV in the superior extrafoveal region with chorioretinal coloboma reaching just inferior to the optic disc. No other cause for CNV was observed except for the chorioretinal coloboma. BCVA improved to 20/30 after laser photocoagulation. She revisited our clinic for deteriorating VA (20/400) in the same eye 3 years after treatment. Funduscopy and FA demonstrated recurrent CNV with subfoveal hemorrhage. Photodynamic therapy (PDT) was followed by three consecutive intravitreal bevacizumab injections (IVB) for the subfoveally-located CNV. However, the CNV persisted with the appearance of a fresh subretinal hemorrhage. Additional PDT was combined with IVB on the same day 6 months after the initial PDT. The CNV regressed 3 months after treatment and has not recurred as of 8 months after the last treatment. The patient's BCVA improved to 20/60. This case suggests that PDT combined with IVB can be an alternative treatment for the management of recurrent CNV after laser photocoagulation in eyes with chorioretinal coloboma.
Adult ; Angiogenesis Inhibitors/administration & dosage ; Antibodies, Monoclonal/administration & dosage ; Choroid Diseases/*complications/drug therapy/surgery ; Choroidal Neovascularization/diagnosis/*etiology/physiopathology ; Coloboma/*complications/drug therapy/surgery ; Female ; Fluorescein Angiography ; Fundus Oculi ; Humans ; Intravitreal Injections ; Laser Coagulation ; Photochemotherapy ; Recurrence ; Visual Acuity

PURPOSE: To examine long-term natural history and visual prognosis of idiopathic subfoveal or juxtafoveal choroidal neovascularization (CNV). METHODS: A retrospective review of fourteen eyes diagnosed as idiopathic CNV at Seoul National University Hospital was done. Patients under 40 years old whose follow-up period of more than 36 months without treatments were included. Best corrected visual acuity (BCVA) with biomicroscopic and fluorescein angiographic examinations was measured at the initial and final visit. Clinical factors correlated with final visual acuity were analyzed. RESULTS: Mean age was 34 years ranged from 20 to 40 years and male to female ratio was 4 to 10. Patients were followed for a median of 45 months (range, 36-60 months). On initial examinations, BCVA in 14 eyes was over 0.15 with eight eyes under 0.5 and with six eyes over 0.6. Subfoveal CNV were in 12 eyes and juxtafoveal were in 2. All had the size smaller than 1DD (disc diameter). On the final visit, BCVA in 14 eyes was over 0.2 with three eyes under 0.5 and 11 eyes more than 0.6. Five eyes (36%) retained visual acuity and 8 eyes (57%) improved more than two lines. BCVA in one eye deteriorated two lines. In 6 eyes (42%), CNV was found to be regressed after the follow-up period. Initial visual acuity was significantly correlated with final visual acuity (p=0.013, Pearson correlation test). CONCLUSIONS: Idiopathic CNV has favorable visual prognosis and a tendency of spontaneous involution.
Adult ; Choroid* ; Choroidal Neovascularization* ; Female ; Fluorescein ; Follow-Up Studies ; Humans ; Male ; Natural History* ; Prognosis* ; Retrospective Studies ; Seoul ; Visual Acuity

No abstract available.
Angiotensin II* ; Angiotensins* ; Animals ; Hemorrhage* ; Rats*

PURPOSE: To determine the phenotypic and functional changes of circulating CD56+ T cells in ocular Behcet's disease (BD) according to the intraocular inflammatory activity METHODS: Forty patients with BD were divided into two groups according to the inflammatory activity (active; 20, inactive; 20). Twenty patients with acute anterior uveitis and 20 healthy subjects were used as controls. Populations of T lymphocytes subsets in the peripheral blood were determined by flow cytometric analysis, using monoclonal antibodies for CD3, CD4, CD8, CD56, pan alpha beta TCR, pan gamma delta TCR, and V alpha24. The frequencies of intracellular cytokine (IFN-gamma and IL-4) were measured in circulating T cells. RESULTS: CD56 expression was significantly upregulated on CD3+ T cells in active BD compared with those of inactive BD which showed similar data as acute anterior uveitis and healthy subjects (p<0.05). This upregulated expression of CD56 was prominent in CD8(high) T and gamma delta T subsets. The frequencies of IFN-gamma production on circulating T cells were increased in active BD while the frequencies of IL-4 producing cells were decreased, and these trends were prominent in the subsets of CD56+ T cells. CONCLUSIONS: Circulating T cells with CD56 expression are phenotypically and functionally upregulated according to the inflammatory activity of BD. This suggests that CD56+ T cells may play a pathogenic role in maintenance of ocular inflammation in BD.
Antibodies, Monoclonal ; Humans ; Inflammation ; Interleukin-4 ; Natural Killer T-Cells* ; T-Lymphocytes ; Uveitis* ; Uveitis, Anterior

Potassium(K+) balance is achieved by the control of urinary K+ excretion and by the control of K+ absorption from the digestive tract. It has been established that chronic potassium depletion is associated with a remarkable hypertrophy of the outer medullary collecting duct of the kidney. But, there are no morphological studies regarding the intercalated cells during the chronic changes of potassium diet. Electron microscopy was performed to observe the morphological alterations of the intercalated cell of the entire collecting duct in response to chronic changes of potassium diet in rat kidney. By electron microscopy, the characteristic features of normal type A intercalated cell of the cortical collecting duct included numerous micro-projections of the apical plasma membrane, complicated basal infolding, apical cytoplasmic tubulovesicles, evenly distributed mitochondia, and centrally located nucleus. In potasium-depleted type A intercalated cell, microprojections of the apical plasma membrane were increased in length and number, basal infoldings were uncomplicated, tubulovesicles were almostly disappeared, and mitochondria were increased in number. Type A intercalated cell of potassium-loading after restriction was found to be almost normal except longer microprojections and increased mitochondria. The characteristic features of normal intercalated cell of the outer medullarycollecting duct(OMCD) included relatively short micro-projections of the apical plasma membrane, uncomplicated basal infoldings, apical cytoplasmic tubulovesicles, and apically distributed mitochondia. In comparison with normal, potassium-depleted intercalated cell of OMCD was hypertrophy, microprojections of the apical plasma membrane were increased in length and number, basal infoldings were complicated, tubulovesicles were almost disappeared, mitochondria were increased in number, and several lysosomes were appeared. Intercalated cell of OMCD of potassium-loading after restriction was found to be almost normal except increased cell size, longer microprojections, and increased mitochondria and lysosomes compared to control. The characteristic features of normal intercalated cell of the inner medullary collecting duct (IMCD) included very short and scant microprojections of the apical plasma membrane, uncomplicated basal infoldings,apica cytoplasmic tubulovesicles, evenly distributed mitochondia, and some lysosomes. In potasium-depleted intercalated cell of IMCD, cell size was prominently increased, microprojections of the apical plasma membrane were increased in length and number, basal infoldings were complicated, tubulovesicles were almostly disappeared, and mitochondria were increased in number. Intercalated cell of IMCD of potassium-loading after restriction was found to be almost normal except increased cell size and increased microprojections in number and length compared to control. These results suggest that intercalated cells adapt through morphological changes to preserve potassium balance during chronic changes of potassium diet.
Rats ; Animals

PURPOSE: To determine the phenotypic characteristics of peripheral CD8(bright) T cells. METHODS: Forty patients with Behcet's disease (BD) with uveitis (active: 20; inactive: 20) and 20 healthy controls were recruited in this study. Phenotypic analysis of fresh PBMCs was performed using anti-CD8 mAb and anti-CD56 mAb in conjunction with a three- or four-color immunofluorescence tests for the expression levels of the following molecules: CD11b, CD27, CD45RA, CD45RO, CD62L, CD94, NKG2D, and HLA-DR. RESULTS: CD27 and CD62L were down-regulated on peripheral CD8(bright) T cells in patients with active Behcet's uveitis in contrast to the up-regulation of CD11b and HLA-DR. These phenotypic natures were remarkable in the CD56+ subsets. CD45RA(dim)CD45RO- phenotypes were expanded in peripheral CD8(bright)CD56+ T cells according to the disease activity. Interestingly, in active Behcet's uveitis, CD94 was up-regulated on peripheral CD8(bright)CD56+ T cells in BD in contrast to the down-regulation of NKG2D compared with the normal controls. CONCLUSIONS: CD8(bright)CD56+ T cells are characterized by activated effector phenotypes with functional NK receptors and they may play immunopathogenic roles in Behcet's uveitis.