• Childhood Kidney Diseases
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• v.19 no.2
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• pp.98-104
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• 2015

Objective: To find out clinical features and long-term outcomes of idiopathic childhood nephrotic syndrome(NS) patients with late steroid resistance(LSR)/late steroid sensitiveness(LSS). Patients and Methods: A retrospective chart review was performed on 480 patients diagnosed with idiopathic childhood NS at Asan Medical Center Children's Hospital from 1990 to 2013. Twenty-four patients whose responsiveness to steroids changed over a minimum 2 year follow-up period (2-17.5 years) were investigated. All patients had undergone a renal biopsy. Results: Among 480 nephrotic children, 428 (89%) were sensitive to the first steroid course. Of those who initially responded, 11 (2.5%) developed resistance to steroid therapy after relapses. LSR mostly developed between 1 month and 1 year after the initial episode. Six patients showed a minimal change and five showed focal segmental glomerulosclerosis (FSGS). Nine (82%) responded to cyclosporine or methylprednisolone pulse therapy. Of these, two had no further relapse, whereas the other seven experienced several relapses that ranged in length from 1.1 to 13.9 years. Three of the nine who initially responded to immunosuppression went on to experience several changes in steroid responsiveness. Two (18%) with resistance to immunosuppressants, including steroids, eventually progressed to end stage renal disease. Among the 52 patients (11%) who were initially steroid resistant, 13 (23%) were converted to steroid sensitive at relapses. Among these, 9 showed minimal change and 4 showed FSGS. Two had no further relapse and the other 11 responded to steroids on subsequent relapses ranging in length from 1.3 to 9.4 years. All these patients have had no further changes in steroid responsiveness with normal renal function. Conclusions: In this study, 2.5% of initial steroid responders and 25% of initial steroid non-responders changed their responsiveness to steroids at subsequent relapses. Eighteen percent of LSR patients developed end stage renal disease. All of the LSS patients showed preserved normal renal function. Responsiveness to immunosuppressants seemed to be the most important factor determining longterm outcomes in LSR/LSS patients.

• Advances in pediatric surgery
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• v.10 no.1
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• pp.52-55
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• 2004

A newborn male baby was transferred to our hospital with a left inguinal mass. The mass was huge measuring $10{\times}10cm$, engorged, and dark-blue colored as a result of internal hemorrhage. Unstable vital signs were combined with DIC and acute renal failure. Emergency operation was performed because of the suspicion of bowel perforation. The peritoneal cavity was full of ascitis and the distal jejunum had a 0.5 cm perforation. Segmental resection of the jejunum and incisional biopsy of the inguinal mass were performed. On pelvic and thigh MRI, the mass protruded into pelvic cavity and encircled large vessels and nerves of the thigh. Pathologic diagnosis was congenital infantile fibrosarcoma. Fifteen days after operation, primary tumor excision was undertaken. The second look operation, performed after 6 times VAC chemotherapy, revealed no remained malignant cell on microscopic section. The baby has been followed closely for the last eight months.

• Clinical and Experimental Pediatrics
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• v.58 no.8
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• pp.275-282
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• 2015

While the incidence of nephrotic syndrome (NS) is decreasing in Korea, the morbidity of difficult-to-treat NS is significant. Efforts to minimize treatment toxicity showed that prolonged treatment after an initial treatment for 2-3 months with glucocorticosteroids was not effective in reducing frequent relapses. For steroid-dependent NS, rituximab, a monoclonal antibody against the CD20 antigen on B cells, was proven to be as effective, and short-term daily low-dose steroids during upper respiratory infections reduced relapses. Steroid resistance or congenital NS are indications for genetic study and renal biopsy, since the list of genes involved in NS is lengthening.

• Journal of Korean Neurosurgical Society
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• v.37 no.4
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• pp.296-299
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• 2005

We report a case of primary central nervous system(CNS) lymphoma in an organ recipient. A 33-years-old man who underwent a renal transplantation 3years previously presented with headache and vomiting. In Brain computed tomography scans and magnetic resonance images showed multiple periventricular cystic rim enhancing masses. Pathologic diagnosis by stereotactic biopsy revealed malignant non-Hodgkins B-cell lymphoma. After pathologic confirmation, methotrexate chemotherapy and whole brain radiation therapy were done. Having experienced such a case, the authors strongly recommend to add primary CNS lymphoma as one of the differential diagnoses to brain abscess, metastatic brain tumor and glioblastoma multiforme in cases of multiple ring enhancing periventricular lesions of immunocompromised patient or organ recipient.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.192-195
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• 1998

[ $\beta$ ]-streptococci have been reported as an etiology of Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP), but the role of $\beta$-streptococci in HSP is not yet confirmed. Our report of two HSP patients is as follows : Although the two patients' clinical symptoms resembled those symptoms of HSP, whereas serum $C_3$ decreased in one case, ASO titer increased in both cases and a histological examination of renal biopsy specimens revealed the fetures commonly described in poststreptococcal glomerulonephritis.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.82-85
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• 1997

The incidence of nephritis associated with Henoch-Sch nlein purpura varies, but glomerulonephritis consistently accounts for most of the associated morbidity and mortality. A very small number of Henoch-Sch nlein purpura develop rapidly progressive glomerulonephritis. A three-year old male patient who showed acute nephritic nephrotic syndrome developed abdominal pain, arthralgia and multiple purpurae on lower extremities later. Peritoneal dialysis was done at the 6th hospital day and continued for 7 months. Renal biopsy disclosed crescentic glomerulonephritis (with 81% crescent formation) and methylprednisolone pulse therapy was done. These days, his general condition is good, but serum creatinine levels are 1.2-1.3 mg/dL. This case was reviewed briefly with the literatures.

• Parasites, Hosts and Diseases
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• v.50 no.4
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• pp.349-352
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• 2012

A 94-year-old female with end-stage renal disease presents with fever, fatigue, and hematochezia. She had previously resided in Hunan Province, China, and Myanmar, and she immigrated to Taiwan 30 years ago. Colonoscopy revealed a colonic ulcer. Biopsy of the colonic ulcer showed ulceration of the colonic mucosa, and many Paragonimus westermani-like eggs were noted. Serum IgG antibody levels showed strong reactivity with P. westermani excretory-secretory antigens by ELISA. Intestinal paragonimiasis was thus diagnosed according to the morphology of the eggs and serologic finding. After treatment with praziquantel, hematochezia resolved. The present case illustrates the extreme manifestations encountered in severe intestinal paragonimiasis.

• Korean Journal of Bronchoesophagology
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• v.6 no.2
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• pp.204-208
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• 2000

Endobronchial metastases from extrathoracic primary malignancies are uncommon. Breast, renal, and colonic carcinomas are primary sites most likely to give rise to endobronchial metastases. A number of other tumours have been reported as being complicated by endobronchial metastasis, including ovarian, thyroid, uterine, adrenal, testicular and prostatic carcinomas. The incidence of endobronchial metastasis has been estimated at 2% in patients who died of metastatic disease. Lung parenchymal metastases are common manifestations in patients with rectal cancer, however spread to the major airway is extremely rare. We herein report a case of endobronchial metastasis from rectal adenocarcinoma. A 69-year-old male patient who had been previously treated with surgical resection with rectal cancer presented with a 8-month history of gradually increasing dyspnea and non-productive cough. Clinical and radiological investigations revealed endobronchial metastasis involving, and penetrating, the lower carina and the left main bronchus. We confirmed endobronchial metastasis from the rectal carcinoma by bronchoscopic biopsy.

• Childhood Kidney Diseases
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• v.23 no.1
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• pp.7-21
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• 2019

Nephrotic syndrome (NS) is a common chronic glomerular disease in children characterized by significant proteinuria with resulting hypoalbuminemia, edema, and hyperlipidemia. Renal biopsy findings of diffuse foot processes effacement on electron microscopy and minimal change disease, focal segmental glomerulosclerosis (FSGS), or diffuse mesangial proliferation on light microscopy. It has been speculated that circulating permeability factors would be implicated in the pathogenesis of NS because they have been reportedly detected in the sera of patients and in experimental models of induced proteinuria. Moreover, a substantial portion of the patients with primary FSGS recurrence shortly after transplantation. This report reviews the current knowledge regarding the role of circulating permeability factors in the pathogenesis of proteinuria in NS and suggests future targeted therapeutic approaches for NS.

• Journal of Medicine and Life Science
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• v.20 no.2
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• pp.94-98
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• 2023

This report presents the case of 75-year-old men with spindle cell neoplasm. The patient underwent percutaneous nephrolithotomy and transurethral resection of the prostate (TURP) for renal stones and benign prostatic hyperplasia. One month postoperatively, the patient was able to void without any difficulty. Five months later, the patient experienced difficulty voiding and presented to the emergency room with severe pelvic pain. Computed tomography (CT) showed regrowth of the prostate mass into the posterior bladder and penile root. The prostate-specific antigen level remained constant at 1.14 ng/mL during the pre-and postoperative periods. Five months before the TURP operation, the patient's CT scan showed a soft and mildly enlarged prostate with no protrusion into the bladder. Biopsy of the prostate, however, showed a protruding mass, indicative of a spindle cell neoplasm. The patient was subsequently treated with the chemotherapeutic drug adriamycin. Unfortunately, treatment was unsuccessful, and the patient died 18 months later.