• Childhood Kidney Diseases
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• v.14 no.2
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• pp.154-165
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• 2010

Purpose : We know little about the natural course of IgA nephropathy (IgAN) in association with histologic changes especially in children. We investigated clinicopathologic features with long-term follow-up biopsy to clarify the outcomes and prognostic indicators for childhood IgAN. Methods : From our patients' medical records, we retrieved 20 patients with IgAN, to whom renal biopsies had been performed for the initial diagnosis and follow-up to find out any histologic changes. Initial and follow-up biopsies were classified by Haas classification. The changes of these parameters were compared with the evolution of clinical features. Results : Patients were treated with angiotensin-converting enzyme inhibitors in combination with angiotensin receptor blockers (in subclass II or above) and short-term cyclosporine A(in patients showing nephrotic syndrome). Histologic improvement in 7 cases and deterioration in 3 cases were observed. At the time of last biopsy, 10 cases (50%) showed clinical remission and the others showed improved clinical features. These clinical outcomes did not correlate with initial Haas classifications. Hypertension at onset observed in 5 cases (25%) revealed significant correlation with clinical outcome (P =0.01) and last Haas classification (P =0.007). None of the cases showed progression to CRF or ESRD. Conclusion : During a mean follow-up of $10.8{\pm}3.4$ years, childhood IgAN showed good clinicopathologic outcome. Hypertension at onset was only a strong predictor of clinicopathologic outcomes, but initial Haas classification cannot predict outcomes in children. Histologic change of IgAN in long term follow-up period cannot be completely predicted by clinical data and vice versa. Therefore, a renal biopsy should be considered as a part of follow-up plan.

• Journal of Chest Surgery
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• v.32 no.7
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• pp.613-620
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• 1999

Background: Recently, regional or isolated organ perfusion is being studied again as a drug administration modality which is able to reduce systemic toxicity while delivering high-dose chemotherapeutic agents. This research was planned to evaluate the pharmacokinetics and long-term pathologic changes of the lung in isolated lung perfusion (ILP) with cisplatin. Material and Method: Twenty-five New Zealand white rabbits were divided into 2 groups (Group I: 10, Group II: 15). The groups were then subdivided into 2 and 3 subgroups of 5 rabbits. In group I, tissue samples of the lung and kidney, and systemic blood for platinum concentration measurement were taken 30 minutes after systemic intravenous infusion of cisplatin (5 mg/kg) and isolated lung perfusion in each 5 rabbits. In 2 subgroups of group II, lung tissues for pathologic exams were taken 30 minutes and 1 week after ILP in each 5 rabbits, which received 10% pentastarch solution only and cisplatin, respectively. In the other subgroups, lung biopsy was undertaken 4 weeks after ILP with cisplatin. Result: When cisplatin was infused via systemic vein, the platinum concentration in the lung, kidney and plasma were 1.50${\pm}$0.43 $\mu\textrm{g}$/g, 7.65${\pm}$2.49 $\mu\textrm{g}$/g, 1.19${\pm}$0.03 $\mu\textrm{g}$/ml, respectively. However, the platinum concentration in the lung was about 50 times higher (75.43${\pm}$11.47 $\mu\textrm{g}$/g) than that of intravenous infusion group, and those in the kidney and plasma were decreased (1.30${\pm}$ 0.35 $\mu\textrm{g}$/g, 0.13${\pm}$0.02 $\mu\textrm{g}$/ml) when cisplatin was introduced through ILP. Pathologic change in the treated lung with ILP was characterized by the medial hypertrophy of the pulmonary arterioles and interstitial eosinophilic infiltration, which was not dependent on cisplatin

• The Journal of Internal Korean Medicine
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• v.29 no.4
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• pp.1000-1010
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• 2008

Objective : Hypothyroidism is a syndrome characterized by symptoms such as cold intolerance, or weight gain. Because of side effects of Western medicine treatment, interest in oriental medicine has been increasing. In this study, we examined the therapeutic effects of Evodiae fructus and histological modification in hypothyroidism rat model induced by PTU(6-propyl, 2-thiouracil). Methods : After inducing hypothyroidism in the rats by PTU injections, we divided the rats into four groups, the Evodiae fructus 100 500, control, and $LT_4$ (levothyroxine) groups. After 2 weeks Evodiae fructus and $LT_4$ were administered, respectively. The weight was measured once a week. After sampling the blood for biochemical analysis to check the levels of $T_3$, $T_4$ and TSH, the thyroid tissue was removed, stained with H&E, and an image was obtained using an optical microscope. Results : Compared with normals, controls showed low $T_4$ and high TSH. The Evodiae fructus group showed a statistically meaningful $T_3$ increase to be dose related. But the levels of TSH showed no meaningful difference between the Evodiae fructus group and normals. About the biochemical tests(liver, kidney etc) and weight change, there was no meaningful difference between the Evodiae fructus group and controls. In the biopsy, the Evodiae fructus group showed thyroid tissue improvement compared to controls. Conclusions : These results show that Evodiae fructus stimulates the decreased functions of the thyroid, and also has thyroid cell protecting effects. The lab results also showed that Evodiae fructus is a safe substance that doesn't cause hepatotoxicity or nephrotoxicity. Therefore it is an effective substance in the treatment of hypothyroidism.

• 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.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.170-175
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• 2009

Purpose : FSGS do not respond well to any kind of therapy and gradually progress to end-stage renal disease. This study was conducted to investigate the difference of protein expression between MCNS and FSGS as a preliminary study for understanding the pathophysiology of FSGS. Methods : Renal biopsy samples of MCNS and FSGS were obtained, which was diagnosed by one pathologist. They were solubilized with a conventional extraction buffer for protein extraction. The solution was applied on immobilized linear gradient strip gel (pH 4-7) using IPGphor system. Silver staining was carried out according to standard method. Protein identification was done by searching NCBI database using MASCOT Peptide Mass Fingerprint software. Results : The differences in protein expressions between MCNS and FSGS were shown by increased or decreased protein spots. Most prominently expressed spot among several spots in FSGS was isolated and analyzed, one of which was glutathione S-transferase (GST) P1-1, whereas it was not found in MCNS. So GSTP1-1 was considered as the one of the key biomarkers in pathogenesis of FSGS. Conclusion : This result would be helpful in diagnosing FSGS and researching FSGS. Further studies for glutathione S-transferase P1-1 might be necessary to elucidate the mechanisms regarding FSGS.

• Clinical and Experimental Pediatrics
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• v.48 no.6
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• pp.606-613
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• 2005

Purpose : Acute poststreptococcal glomerulonephritis(APSGN) is a common form of glomerulonephritis in children. Most patients recover completely after the acute phase but a few patients have acute complications or progress to chronic renal disease. In recent years, the frequency of APSGN has been was decreasing but is still common in children. So we studied the clinical characteristics of APSGN from 1994 to 2003 and compared it with past studies. Methods : We studied 105 patients who were diagnosed with APSGN in the Department of Pediatrics, Asan Medical Center between January 1994 and December 2003, with a retrospective chart review. Results : The mean age was $8.5{\pm}2.6$ years. The male to female ratio was 2 : 1. Average annual incidence was $10.5{\pm}4.9$ most patients(60.0 percent) occurred from October to January. Edema was seen in 82 cases(78.1 percent), gross hematuria in 70 cases(66.7 percent), hypertension in 50 cases (47.6 percent) and oliguria in 22 cases(20.9 percent). Microscopic hematuria was seen in 105 cases (100 percent), positive ASO in 99 cases(94.2 percent), proteinuria in 67 cases(63.8 percent) and azotemia in 38 cases(36.2 percent). Serum complement 3(C3) level decreased in 96 cases and returned to normal within eight weeks in 70 patients(75.3 percent). Kidney biopsy was carried out in 22 cases. Most acute symptoms subsided within 2 weeks of onset. Conclusion : We concluded that there was no significant difference between clinical features of recent and past APSGN in children, and short term prognoses were excellent.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.31 no.4
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• pp.343-348
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• 2009

Kaposi's sarcoma was first descrided by Kaposi in 1872 as an idiopathic multiple hemorrhagic sarcoma. Its clinical features revealed to be erythematous red or purple macule started out, and developing into palpable dome-shaped nodules. Etiology is not defined to detail at present. Kaposi's sarcoma is classified to 4 categories; Classical, African, Epidemic and Immunosuppressive type. Epidemic categories is found approximately 20% of all AIDS patients and has strong predilection for head and neck region. The first case of immunosuppresive type Kaposi's sarcoma in patients with kidney transplants was reported in 1969. Kaposi's sarcoma accounts for 5% of all tumors associated with transplanted patients. The most common site of Kaposi's sarcoma in immunosuppressed patients are extremities, but rare in head and neck area. A 42 years old woman who had systemic lupus erythematosus visited to our clinic because of gingival hyperplasia, and excisional biopsy revealed Kaposi's sarcoma. We experienced a case of favorable results using excision and chemotherapy, so we report with review of literatures.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.106-111
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• 2007

[ $Henoch-Sch\ddot{o}nlein$ ] Purpura(HSP) is a form of vasculitis that typically affects small arteries in the skin, joints, intestinal tract and kidneys. It usually resolves spontaneously but sometimes can cause serious problems in the kidneys and intestinal tract. A 6-year-old girl with purpura, arthralgia and abdominal pain for 2 weeks was admitted. She also showed gross hematuria, generalized edema and decreased urine output. Blood pressure was in the upper normal range. Initial laboratory findings showed hypoalbuminemia, hyperlipidemia, microhematuria and nephrotic-range proteinuria(27.2 g/day). Initially, she was treated with pulse methylprednisolone, azathioprine, albumin and furosemide. Her renal biopsy revealed diffuse mesangial proliferation with strong IgA deposition. There were no crescents. On the third hospital day, she complained of severe abdominal pain and free peritoneal air was seen on abdominal X-ray. Primary repair of small bowel was performed and two pin-point sized holes were found. One week later, she still showed heavy proteinuria. Therefore, we added an ACE inhibitor and dipyridamole, and changed azathioprine to cyclosporine. One month later, the urine protein/creatinine ratio was decreased to 17.8 from 57, but heavy proteinuria has been still persisted. Here we report a rare case of a patient with HSP who had both severe nephrritc-nephrotic syndrome and small bowel perforation.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.1-8
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• 2003

Purpose : Focal segmental glomerulosclerosis(FSGS) is known to have a poor prognosis and a poor response to treatment. We performed a combination therapy of plasmapheresis, steroid pulse and immunoglobulin in 4 patients with relapsed steroid dependent(SD) or steroid resistant(SR) FSGS after steroid pulse therapy. Materials and Methods : 8 cycles of plasmapheresis were performed in 4 patients with biopsy proven FSGS who had relapsed after steroid pulse therapy from March 1988 to July 2002. Clinical findings and treatment courses were reviewed retrospectively. Results : Among the 4 patients, there were 3 males and 1 female. After 8 cycles of plasmapheresis, clinical remissions were obtained. Two of the four patients had two relapses and received 2 more cycles of plasmapheresis which resulted in remissions. One of these patients had two further relapses and was treated with oral steroid resulting in clinical remission. Three patients have maintained normal serum creatinine level and glomerular filtration rates during the follow-up period of 10 years, and the other 1 patient for 5 months. Conclusion : A combination therapy of plasmapheresis, steroid pulse and immunoglobulin led to a complete remission in patients with FSGS who were SD or SR and was effective in maintaining normal renal function.

• Childhood Kidney Diseases
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• v.8 no.1
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• pp.1-9
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• 2004

Purpose : Minimal Change Disease (MCD) is the most common primary nephrotic syndrome in children. Some suggested that tumor necrosis $factor-{\alpha}$ ($TNF-{\alpha}$) are involved in the pathogenesis of MCD. Methods : This study was done to see the changes of plasma and urinary $TNF-{\alpha}$, and its effect on the determination of permeability of the glomerular basement membrane (BM) contributed by heparan sulfate proteoglycan (HSPG). Study patients consisted of 19 biopsy-proven MCD children aged 2-15 years old. Both plasma and urinary $TNF-{\alpha}$ were measured. Employing the Millicell system, $TNF-{\alpha}$ was screened for the permeability factors. We examined whether $TNF-{\alpha}$ regulated BM HSPG gene expression and HS synthesis in the glomerular epithelial cells (GECs). Results : Urinary $TNF-{\alpha}$ during relapse was significantly increased when compared with that of during remission or controls ($364.4{\pm}51.2$ vs $155.3{\pm}20.8,\;36.0{\pm}4.5$ ng/mg cr) (P<0.05). However, negative results were obtained in the permeability assay using the Millicell system. No difference was seen in the BM HSPG gene expression and HS synthesis in the GECs. Conclusion : It seems that $TNF-{\alpha}$ may not play a disease-specific role in the pathogenesis of MCD.