• Childhood Kidney Diseases
• /
• v.5 no.2
• /
• pp.78-86
• /
• 2001

Purpose: Rapidly progressive glomerulonephritis (RPGN) is characterized by the rapid increase in serum creatitnin and crescents formation involving more than $50\%$ of glomeruli. 10 patients who had been treated for RPGN were studied retrospectively for thier underlying diseases and clinical features Method: Cilinical review was performed on 10 children who were diagnosed with RPGN by clinical features and renal biopsy and followed up at department of pediatrics during tile last 10 years, from May 1990 to May 2000. Result: There were 6 males and 4 females between the ages of 2.1 and 14.3 years (mean $10.9{\pm}3.8$). 3 had Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis; 2, idiopathic rapidly progressive glomerulonephritis; 2, lupus nephritis; 1, hemolytic uremic syndrome; 1, membranous glomerulonephritis and 1, microscopic polyangiitis. The most common chief complaints were gross hematuria and oliguria. Initial clinical features included proteinuria, edema, hypertension, nausea and arthralgia. Mean serum BUN was $74.2{\pm}39.1\;mg/dL$ mean serum creatinin, $3.2{\pm}1.8\;mg/dL$ and mean creatinin clearance, $26.5{\pm}13.2\;mL/min/1.73m^2$. Antineutrophil cytoplasmic antibody was positive only in microscopic polyangiitis. ANA and Anti-DNA antibody were positive in two lupus nephritis patients. Serum complements were decreased in 4 patients. All patients except Hemolytic uremic syndrome received steroid pulse therapy and immunosupressive agents. 3 patients were performed acute peritoneal dialysis and 2 patients were given plasmapheresis. At the last follow up, 1 patient was dead, 4 patients had elevated serum creatinin, 2 of these 4 patients were on chronic ambulatory peritoneal dialysis and 6 patients had normal renal function. Conclusion: Rapidly progressive glomerulonephritis is a medical emergency that requires very rapid diagnosis, classification, and therapy. Appropriate therapy selected on the basis of underlying disease mechanism can substantially improve renal survival. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 78-86)

• Investigative Magnetic Resonance Imaging
• /
• v.17 no.2
• /
• pp.133-143
• /
• 2013

Purpose : To evaluate and compare the accuracy of magnetic resonance imaging (MRI) and ultrasound (US) for detection and estimation of invasion depth of colorectal carcinoma (CRC) by correlation with histopathologic findings in vitro, and to find out the best MR pulse sequence for accurate delineation of tumor from surrounding normal tissue. Materials and Methods: Resected specimens of CRC from 45 patients were examined about tumor detectability and invasion depth of US using high frequency (5-17 MHz) linear transducer in a tube filled with normal saline and MRI in a 8-channel quadrate head coil. The institutional review board approved this study and informed consent was waived. MRI with seven pulse sequences of in- and out-of-phases gradient echo T1 weighted images, fast spin echo T2 weighted image and its fat suppression image, fast imaging employing steady-state acquisition (FIESTA) and its fat suppression image, and diffusion weighted image (DWI) were performed. In each case, both imaging findings of MRI and US were evaluated independently for detection and estimation of invasion depth of tumor by consensus of two radiologists and were compared about diagnostic accuracy according to the histopathologic findings as reference standard. Seven MR pulse sequences were evaluated on the point of accurate delineation of tumor from surrounding normal tissue in each specimen. Results: In specimens of CRC, both imaging modalities of MRI (91.1%) and US (86.7%) showed relatively high diagnostic accuracy to detect tumor and evaluate invasion depth of tumor. In early CRC, diagnostic accuracy of US was 87.5% and that of MRI was 75.0%. There was no statistically significant difference between two imaging modalities (p > 0.05). The best pulse sequence among seven MR sequences for accurate delineation of tumor from surrounding normal tissue in each specimen of CRC was fast spin echo T2 weighted image. Conclusion: MRI and US show relatively high diagnostic accuracy to detect tumor and evaluate invasion depth of resected specimen of CRC. The most excellent pulse sequence of MRI for accurate delineation of tumor from surrounding normal tissue in CRC is fast spin echo T2 weighted image.

• Tuberculosis and Respiratory Diseases
• /
• v.60 no.5
• /
• pp.523-531
• /
• 2006

Background : Cervical tuberculous lymphadenopathy is a very common disease with a similar incidence to pulmonary tuberculosis. Dendritic cells play a role of initial antigen presentation of this illness. Nevertheless, the precise role of these antigen-presenting cells according to the clinical features in unclear. The aim of this study was to determine the clinical implication of dendritic cell infiltration in the cervical lymph nodes. Methods : A review of the clinical characteristics was carried out retrospectively based on the clinical records and radiography. Immunohistochemical staining was performed on the available histology specimens of 72 cases using the S-100b polyclonal antibody for dendritic cells. The number of dendritic cells with tuberculous granuloma were determined. A $X^2$ test, unpaired T test and multiple logistic regression analysis were performed. Results : Thirty percent of subjects had previous or concurrent pulmonary TB. Twenty one percent of cases showed a positive reaction on the AFB stain. Within a granuloma, the number of infiltrated dendritic cells was $113.0{\pm}7.0$. The incidence of fever and cough decreased with increasing infiltration of dendritic cells Multivariate regression analysis showed that the infiltration of dendritic cells could significantly contribute to fever. Conclusion : Overall, dendritic cells can control a Mycobacterium tuberculosis infection and modulate the immune response, as well as resolve the clinical manifestations of TB lymphadenopathy.

• Radiation Oncology Journal
• /
• v.27 no.2
• /
• pp.49-54
• /
• 2009

Purpose: To report on the clinical outcome of patients with stage I testicular seminoma by postoperative radiotherapy (PORT) or surveillance after radical inguinal orchiectomy. Materials and Methods: This study is a retrospective review of 32 stage I pure seminoma patients treated between 1996 and 2005 at the Samsung Medical Center. Twenty two of the patients were treated by PORT, which was directed at the paraaortic lymphatics with a median dose of 25.2 Gy in 14 fractions for 3 weeks. The 10 remaining patients were managed by surveillance. The median follow-up period was 96 months with a range of 24 to 155 months. Results: Clinically, most patients presented with a testicular mass or discomfort. Two of the patients had a history of undescended testes. Pathologically, 23 of the patients had intratubular germ cell neoplasia with seminoma. Both recurrence-free survival (RFS) and overall survival (OS) rates of patients treated by PORT were 100%. In the control group, 1 of the 10 patients suffered a para-aortic lymph node relapse. The RFS and OS rates of the surveillance group were 88.9% and 100%, respectively. Conclusion: No difference in survival was observed between the two groups. Moreover, symptom recurrence was only observed in 1 patient in the control group. The use of PORT may reduce the risk of relapse. With the availability of effective diagnostic and salvage modalities, surveillance monitoring may be considered for patients in good compliance.

• Journal of Chest Surgery
• /
• v.31 no.10
• /
• pp.988-994
• /
• 1998

Background: Chest wall tumors can classified into soft tissue tumors and bone tissue tumors and can be subclassified into benign and malignant tumors. Materials and methods: We report an analysis of 68 patients with primary chest wall tumors treated at the department of thoracic and cardiovascular surgery at Hanyang University Hospital from January, 1973 to September 1997. Results: Among a total of 68 patients 33(48.5%) were males and 35(51.5%) were females. The ages of the patients ranged from 10 to 79 years with a mean age of 39.3 years. According to the age distribution, 23 patients (33.8%) were from the 4th decade, 12 patients(17.6%) were from the 6th decade, and 10 patients(14.7%) were from the 5th decade. Among the primary chest wall tumors, 53 cases were benign and 15 cases were malignant. Among the benign tumors, 17 cases(32.1%) were in the 4th decade and among the malignant tumors, 6 cases(40%) were in the 4th decade. In both malignant and benign tumors the most common ages were in the 4th decade. The most common tumors were fibrous dysplasia and chondroma, each with a total of 14 cases(26.4%). Osteochondroma and lipoma each had 8 cases(15.1%). Among malignant tumors, osteosarcoma was most common with 8 cases (53.3%). According to location, 49 cases occured in both bone and cartilage tissue, 19 cases occurred in cartilage. Among the presenting symptoms, palpable mass was present in all cases. Fifty-one patients complained of tenderness and among cases with involvement of the lung, 3 patients had complained of respiratory distress. Among the malignant tumors 6 cases underwent a radical operation and 4 cases of benign tumors underwent a radical operation. Postoperativly, there was one case with recurrence from a desmoid tumor. There were no deaths postoperativly and no deaths due to complications(and their postoperative courses were uneventful). Conclusions: Most patients with primary chest wall tumors initially present with mass at admission. Resection is sufficient treatment for benign tumors but in malignant tumors wide resection of the chest wall is needed and mchest wall reconstruction.

• Childhood Kidney Diseases
• /
• v.13 no.2
• /
• pp.161-169
• /
• 2009

Purpose : This study was performed to report the diagnosis and treatment of nephrotic syndrome manifesting in the first year of life. Methods : We retrospectively reviewed the clinical data with chart review in 7 patients who were diagnosed as nephrotic syndrome manifesting in the first year of life from 1996 to 2007. Results : Three patients had congenital nephrotic syndrome, the other 4 patients had infantile nephrotic syndrome. Their ages ranged from birth to 11 months and male to female ratio was 1 to 6. Renal biopsies were done in 6 patients. One patient had Finnish type congenital nephrotic syndrome, 2 patients had diffuse mesangial sclerosis, 2 patients had focal segmental glomerulosclerosis and 1 patient had minimal change disease. Genetic analyses of NPHS2, PLCE1, and WT1 were done in 4 patients and 2 of them had WT1 mutation. Among 3 patients with congenital nephrotic syndrome, 1 patient was diagnosed as congenital nephrotic syndrome of Finnish type and the other 2 patients were diagnosed as Denys-Drash syndrome. All of the patients with congenital nephrotic syndrome died due to sepsis. Among 4 patients with infantile nephrotic syndrome, 2 patients died and 1 had remission, another patient progressed to end stage renal disease. Conclusion : Most of nephrotic syndrome manifesting in the first year was hereditary renal disease. Patients with nephrotic syndrome manifesting in the 3 month of life had poorer prognosis and needed more aggressive management including early dialysis and renal transplantation might be considered compared with infantile nephrotic syndrome. Further genotype-phenotype correlation studies are needed.

• Journal of Chest Surgery
• /
• v.30 no.3
• /
• pp.308-314
• /
• 1997

A retrospective review of the histopathology and clinical information of primary lung cancer was performed to investigate the trends in the histologic type related to sex, age, and smoking history. During January 1988 and July 1995, 541 pateints were diagnosed as primary lung cancer at the Korea Univeristy Anam Hospital. Male (423) to female(118) ratio was 3. 6:1. The most frequent histologic type of lung cancer in male patients was squamous cell carcinoma (223 patients, 52.7%) followed by adenocarcinoma (86, 20.3%) and small cell carcinoma (85, 20.1 %). In female patients, adenocarcinoma (64, 54.2%) wa most common, which was followed by squamous cell carcinoma (22, 18.6%) and small cell carcinoma (22, 18. 6%). The incidence of adenocarnimoma had an increased tendency recently (14.3% in 1988, 33.3% in 1995)(P=0.019). The predominant type in smokers was squamous cell carcinoma; whereas adenocarcinoma was the most frequent type in non-smokers. The proportion of patients aged less than 40 years (younger group) was 4. 0% (n=22). Of them, adenocarcinoma (7) and small cell carcinoma (7) were most common. In patients older than 40 years (older group, n=519), 243 (46.8%) patients had squamous cell carcinoma, and 143 (27.6%) adenocarcinoma. As age increased, the prevalence of squamous cell carcinoma was increased (P=0.0005), adenocarcinoma decreased (P=NS), and small cell carcinoma remained unchanged. We suggest above data as a clinical guidance for management of primary lung cancer.

• Clinical and Experimental Pediatrics
• /
• v.49 no.8
• /
• pp.875-881
• /
• 2006

Purpose : The aims of this study were to analyze the clinical characteristics of children with Kikuchi's disease(KD) at a medical center and to investigate the etiologic role of human herpesvirus 8(HHV 8) or Epstein-Barr virus(EBV) in children with KD. Methods : Twenty six children who were diagnosed as KD between Jan. 1998 and Dec. 2005 were included. Medical records were reviewed on the clinical characteristics of children with KD. Follow up data were collected by chart review and telephone contact. Polymerase chain reaction(PCR) was performed in order to detect HHV 8 DNA, and in situ hybridization(ISH) was perfomed in order to detect EBV RNA from 20 lymph node tissues. Results : There were 15 girls and 11 boys with a mean age of 13 years. Posterior cervical lymph nodes were involved in 72 percent(18/25) of the patients. Extracervical lymphadenopathy was associated in one patient. Fever was an associated symptom in 31 percent(8/26) of the patients. Leukopenia was observed in six (46 percent) patients. The cervical lymphadenopathy usually resolved spontaneously within 6 months. Only one patient had a recurrence of lymphadenopathy with fever during follow-up. No children with KD in our series developed systemic lupus erythematosus. HHV 8 DNA was not amplified by nested PCR in any of the cases, and all cases were negative for EBV RNA by ISH. Conclusion : KD should be differentiated as a cause of cervical lymphadenopathy in children. HHV 8 and EBV may not play major causative roles in KD in children.

• Childhood Kidney Diseases
• /
• v.1 no.1
• /
• pp.4-12
• /
• 1997

Purpose: To evaluate the prevalence and clinical manifestations of various glomerulonephritis (GN) in children, a clinicopathological anlysis of 310 biopsied cases were performed. Method: We conducted retrospective study with review of histopathologic findings and clinical manifestations of the 310 cases diagnosed as glomerulonephritis by percutaneous renal biopsy which were done between January 1986 and December 1996 at department of pediatrics, Pusan Paik hospital. Results: 1) Male to female ratio was 1.54:1 and the range of age was from 13 months to 15 years 10 months. 2) Among these, 217 (70.0%) patients were belong to primary GN and 93 (30.0%) patients were belong to secondary GN. As a whole, the most common pathologic diagnosis was minimal change lesion (MC, 32.6%), which was followed by IgA nephropathy (IgAN, 15.8%), $Henoch-Sch\"{o}nlein$ purpura nephritis (HSPN, 13.5%), Poststreptococcal glomerulonephritis (PSAGN, 8.1%). 3) Clinical manifestations of patients were asymptomatic urinary abnormality (43.2%), nephrotic syndrome (41.0%), acute glomerulonephritis (14.2%), chronic glomerulonephritis (1.0%), rapidly progressive glomerulonephritis (0.6%). 4) In primary GN, the most common pathologic diagnosis was MC (46.5%), IgAN (22.6%), thin glomerular basement membrane (GBM) disease (7.8%), membranoproliferative glomerulonephritis (MPGN, 5.5%), mesangial proliferative glomerulonephritis (MesPGN,4.6%), focal segmental glomerulosclerosis (FSGS, 4.6%), membranous nephropathy (MN, 0.9%), sclerosing glomerulonephritis (SCGN, 0.9%), crescentic glomerulonephritis (CreGN, 0.5%) and non-specific glomerulonephritis (NonspGN, 6.0%). 5) Major causes of secondary GN were HSPN (45.2%), PSAGN (26.9%), hepatitis B associated glomerulonephritis (HBGN, 17.2%), lupus nephritis (LN, 6.5%), Alport syndrome (2.2%), hemolytic uremic syndrome (1.0%), fibrillary glomerulonephritis (1.0%) in descending order. Conclusions: There are some differences of the results of clinicopathological stuidies of glomerulonephritis in children because of its different indications of renal biopsy, pathologic classification of renal disease and methods of analysis among investigators. In order to establish more reliable data of incidence and classification of childhood glomerulonephritis in Korea, multicenter cooperative study were necessary.

• Tuberculosis and Respiratory Diseases
• /
• v.52 no.5
• /
• pp.519-528
• /
• 2002

Background : Steroid therapy has been shown to improve the clinical outcome in acute respiratory distress syndrome (ARDS) patients with histological evidence of fibroproliferation in the lung tissue and no identifiable source of infection. Because the histopathological features of acute interstitial pneumonia(AIP) are identical with that of ARDS, early steroid therapy was used in AIP patients who had histological evidence of fibroproliferation in the lung tissue and no identifiable source of infection. We analyzed seven years of our experience to evaluate the efficacy of early steroid therapy in AIP. Materials and Methods : A retrospective review was performed on AIP patients who received steroid therapy within 7 days of mechanical ventilatory support in Dankook university Hospital between May 1995 and May 2002. AIP was diagnosed clinically by ARDS without a known cause of the etiology and pathologically by a lung biopsy showing a fibroproliferative stage of diffuse alveolar damage. The clinical response and physiologic parameters were evaluated during steroid therapy. Results : Five AIP patients received intravenous methylprednisolone (1-2 mg/kg every 6 hours) after $0.6{\pm}1.7$ days of mechanical ventilatory support. Lung biopsies were performed after $1.8{\pm}1.4$ days of mechanical ventilatory support. Four patients(80%) survived and were extubated after $2.8{\pm}0.4$ days of steroid therapy with improvement in the $PaO_2/FiO_2$ ratio ($127.4{\pm}10.0$ at day 0 to $223.8{\pm}37.6$ at day 7) by steroid therapy. However, one patient(20%) died of respiratory failure after 15 days of steroid therapy. Conclusion : Early steroid therapy sppears to be beneficial in AIP patients without evidence of infection. However, as our study group was too small, further large scale studies to define the effectiveness of steroids are required.