• Childhood Kidney Diseases
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• v.18 no.1
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• pp.24-28
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• 2014

Purpose: The purpose of this study was to assess the therapeutic efficacy of methylprednisolone pulse therapy in children with IgA nephropathy and Henoch- Sch$\ddot{o}$nlein Purpura (HSP) nephritis combined with proteinuria. Methods: We retrospectively reviewed the clinical records of 21 patients who were diagnosed with IgA nephropathy and HSP nephritis based on percutaneous renal biopsy. Of the 21 patients, 15 were diagnosed with IgA nephropathy and 6 were diagnosed with HSP nephritis. They had mild to severe proteinuria at the time of diagnosis or during follow-up. Group 1 (n=7) received methylprednisolone pulse therapy three times every couple of months, and Group 2 (n=14) received oral steroid therapy. The follow-up periods for Group 1 and 2 were 14.0 (9-54) months and 26.5 (14-34) months, respectively. There was no significant difference in the follow-up duration between the two groups. Results: The average age at diagnosis and biopsy was lower in Group 1 compared to Group 2, but it was not significantly different. At admission, all patients in both groups had hematuria and 5 patients (71.4%) of Group 1 and 14 patients (100 %) of Group 2 had proteinuria. Before treatment, there was no significant difference of spot urine protein/creatinine ratio between the two groups. During followup, 7 patients of Group 1 (100%) and 10 patients of Group 2 (71.4%) showed complete improvement of proteinuria and the spot urine protein/creatinine ratio in Group 1 was significantly lower than Group 2. Conclusion: In patients with IgA nephropathy and HSP nephritis with proteinuria, methylprednisolone pulse therapy was more effective than oral steroid therapy in the reduction of proteinuria. To investigate the effects on long-term prognosis, large-scale prospective studies are needed.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.41-49
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• 1998

Treatment of $Henoch-Sch\"{o}nlein$ purpura nephritis(HSPN) accomanied by nephrotic syndrome is still controversal, even though both corticosteroids and immunosuppressants have been used for therapy. Azathioprine(AZA) is a chemical analog of the physiologic purines-adenine, guanine, and hyoxanthine and an antagonist to purine metabolism which may inhibit RNA and DNA synthesis and is mainly used for immunosuppressive agent. We studied the effects of AZA in HSPN accompanied by nephrotic syndrome and evaluating the clinical status and histopathologic changes by sequential biopsies following the treatment. Fifteen patients with nehprotic syndrome either initially or during the course of HSPN confirmed by renal biopsies were treated with AZA(2 mg/kg/day) and prednisolone (0.5-1 mg/kg/day qod) for 8months. Folow up renal biopsy was done after treatment in 11 patients. The clinical status of the patients on admission were C(12 cases) and B(3 cases). Improvement of clinical status were showed in 12 cases, but 3 cases were not improved and 1 case was aggrevated after AZA treatment. Complete remission of proteinuria were in 8 cases(53.3%), partial remission were in 4 cases(26.7%) and persistence of proteinuria and hematuria were in 3 cases(20.0%). The loss of hematuria were in 10 cases(66.7%). Histopathologically and immunopathologically, 4 cases were improved. This study suggests that, although control studies are needed, AZA could be used in the treatment of HSPN accompanied by nephrotic syndrome.

• The Korean Journal of Nuclear Medicine Technology
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• v.20 no.2
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• pp.9-13
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• 2016

Purpose PET-CT examinations using $^{18}F-FDG$ to treat urinary system cancer are limited in terms of anatomical structure and excretion route of $^{18}F-FDG$. But one of the ongoing examinations utilizing $^{11}C-Acetate$ can compensate for such defects. We would like to introduce a clinical application of $^{11}C-Acetate$ PET-CT in urinary cancer patients. Materials and Methods We conducted a clinical survey of 22 patients diagnosed with urinary cancer at our hospital, 10 prostate cancer patients, 10 renal cell carcinoma patients, and 2 bladder cancer patients. All patients were performed $^{18}F-FDG$ PET-CT examinations, $^{11}C-Acetate$ examinations were performed after two weeks on average. The equipment used to D-710 PET-CT in GE Company and we performed PET-CT procedures 15 minutes after injecting $^{11}C-Acetate$, and a medical doctor from the department of nuclear medicine appraised and compared images between $^{18}F-FDG$ and $^{11}C-Acetate$. Results According to our survey, prostate cancer patients generally had lower uptake of $^{18}F-FDG$ than other cancer patients did. In 2 out of 10 prostate cancer patients, metastasized cancer showed greater uptake in $^{11}C-Acetate$ than $^{18}F-FDG$. In renal cell carcinoma cases, 8 out of 10 patients displayed evidently greater uptake in $^{11}C-Acetate$ than $^{18}F-FDG$. We excluded bladder cancer cases in this study because uptake of $^{18}F-FDG$ in the bladder was too hot, the number of patients was insufficient, and the cases did not meet criteria such as the use of diuretics. Conclusion It is too premature to draw solid conclusions from the survey, since it involved only a small number of participants. However, there are a number of studies conducted abroad that prove the effectiveness of the $^{11}C-Acetate$ PET-CT examinations in treating urinary system cancer, and this study is still ongoing at our hospital. If the tests were to be conducted on a larger number of participants, this study could lead to numerous other potential research topics, such as the correlation between Prostatic specific antigen (PSA) values and $^{11}C-Acetate$ PET-CT, Gleason sum values from biopsy before surgery, Specificity, sensitivity, positive predictive value (PPV), negative predictive value (NPV) between $^{18}F-FDG$ PET-CT examinations and $^{11}C-Acetate$ PET-CT examinations in other urinary system cancers.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.183-192
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• 2005

Purpose : The long term disease course and prognostic factors were evaluated in childhood Henoch-$Sch{\ddot{o}}nlein$ puruura nephritis(HSPN). Methods : A total of 75 children(44 boys and 31 girls) with HSPN were included in this study. The onset age was $8.0{\pm}3.1$ years(2.3-l5.3 years), and the follow-up period was $4.3{\pm}3.6$ years(1.0-17.1 years). Kidney biopsy was done in 24 children(32$\%$). Initial clinical and laboratory findings were evaluated. In addition, polymorphisms of the renin angiotensin system(RAS) genes(insertion/deletion in intron 16 of ACE gene, M235T in AGT gene, and A1166C in AGTR gene) were analysed. The initial and last clinical states were classified into 4 groups as follows A, normal; B, minor urinary abnormalities; C, active renal disease (nephrotic-range proteinuria and/or hypertension with serum creatinine $\leq$1.5 mg/dL); D, renal insufficiency. Results : At the onset, the clinical states of the patients were B in 26(35$\%$), C in 46(61$\%$), and D, in 3(4$\%$). The distribution of the RAS gene polymorphism of HSPN were not different from that of 100 healthy control subjects. At the last follow-up, the clinical states of the patients were A in 23(31$\%$), B in 38(50$\%$), C in 9(12$\%$), and D in 5(7$\%$). A multiple logistic regression identified age at the onset and initial urine protein excretion as significant prognostic factors. Analysis of genotypes of the 3 RAS genes as prognostic values revealed no statistical significance. Conclusion : Older age at onset and severe proteinuria were identified as poor prognostic factors of childhood HSPN. Implication of the RAS gene polymorphism In HSPN could not be validated in this small-scale retrospective study. (J Korean Soc Pediatr Nephrol 2005;9:183-192)

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.22-31
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• 2010

Purpose : This study was performed to evaluate the relationship between glomerular basement membrane (GBM) alterations to epithelial cell (EpC) structure and renal function in Alport Syndrome (AS) patients. Methods : Fifteen patients diagnosed with AS (4-26yrs) were examined. The GBM in AS was categorized as : C1) normal, C2) minor alterations (widening of lamina rara interna or externa without lamina densa change), C3) nonspecific splitting of lamina densa, C4) basket-weaving pattern of lamina densa splitting. The length of each GBM portion along the epithelial side was measured on the systematically obtained electron microscopic photographs. Furthermore to obtain an objective assessment of the degree of glomerular EpC foot process change, the number of slit pores along $10\;{\mu}m$ of peripheral GBM in each category was obtained. Results : The percentage of normal GBM portion (C1) correlated inversely with daily protein excretion (g/day/$m^2$, P<0.05) and sum of the percentage of abnormal GBM portion (C2+C3+C4) had direct correlation with daily protein excretion (g/day/$m^2$, P<0.05). There were no significant relationships between the percentages of other categories of GBM alterations and creatinine clearance or protein excretion. There were no significant relationships between of creatinine clearance in relation to normal GBM(C1) portion as well as that in relation to sum of the percentage of abnormal GBM portion (C2+C3+C4). GBM abnormality did not correlate with age at biopsy. Conclusion : The extent of GBM structural abnormality is related to proteinuria in AS but the epithelial response is uniform even though the GBM ultrastructural lesions are not.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.2
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• pp.55-62
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• 2020

A 22-month-old girl who had taken iron supplements due to iron deficiency anemia, presented bloody mucoid stool for one month. She had a bruise at the right periorbital area due to minor trauma and hepatosplenomegaly. Laboratory studies showed anemia, thrombocytopenia, elevated alkaline phosphatase (ALP), hypophosphatemia, decreased haptoglobin, hypocomplementemia, negative direct/indirect Coomb's test, normal vitamin D3 level and high PTHi. Wrist x-ray showed no signs of rickets. The abdominal ultrasound showed only accessory spleen. Tandem mass spectrometry was normal. During follow up, bloody stool regressed after seven days of withdrawal of iron supplement and cow milk, and the total CO2 level had been within 15-20 mEq/L with normal anion gap. NGS (next generation sequencing) panel test for evaluation of renal tubular acidosis showed negative results. After low dose steroid and vitamin D supplements under the impression of hypocomplementemic vasculitis, thrombocytopenia, C3/C4, decreased haptoglobin, and elevated ALP level became normal. At 57 months of age, laboratory findings showed elevated liver enzyme, ALP and gamma-glutamyl transferase again. And liver cirrhosis with splenomegaly and diffuse renal disease were reported with abdomen CT scan. Liver biopsy reported macro- and micronodular cirrhosis. Urine organic acid profile showed elevated succinylacetone level. Whole exome sequencing revealed novel compound heterozygous mutations (NM_00137.2:c.107T>C, NM_00137, 2:c.614T>C) in FAH gene and confirmed by Sanger sequencing. Consequently, the patient was diagnosed as chronic hereditary tyrosinemia type I. She started low phenylalanine/tyrosine diet and nitisinone treatment. Our case had presented symptoms very slowly, which is the first case of chronic tyrosinemia type I in South Korea.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.120-126
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• 2000

Purpose : This retrospective study has been undertaken to find out the clinical outcome of children with HS nephntis and its relationship with initial clinical presentation and/or renal pathologic finding. Patients and methods : Study population consisted of 59 children with HS nephritis who have been admitted to the Pediatric department of Kyungpook University Hospital from 1987 to 1999, and biopsy was done with indications of heavy proteinuria (> 1 g/m2/day) lasting over 1 month, nephrotic syndrome, and persistent hematuria and/or proteinuria over 1 year. Patients were divided clinically into 3 groups ; isolated hematuria, hematuria with proteinuria and heavy proteinuria (including nephrotic syndrome). Biopsy findings ore graded from I-V according to International Study of Kidney Disease in Children (ISKDC). Results : Mean age of presentation was $8.1{\pm}3.0$ years and slight male preponderance m noted (33 boys md 26 girls). Histopathologic grading showed Grade I ; 2, Grade II ; 44, and Grade III ; 13 cases. Clinical outcome at the follow-up period of 1-2 year (49 cases) and 3-4 years (30 cases) shooed normal urinalysis in 75 (30.6$\%$) and 18 cases (60.0$\%$), persistent isolated hematuria in 20 (40.8$\%$) and 2 cases (6.7$\%$), hematuria with proteinuria in 11 (22.5$\%$) and 8 cases (26.6$\%$), and persistent heavy proteinuria in 3 (6.1$\%$) and 2 cases (6.7$\%$) respectively. Clinical outcome according to histopathologic grading showed the frequency of normalization of urinalysis being lower in Grade III compared to grade I or II. Clinical outcome according to initial clinical presentation showed no relationship to the normalization or urinalysis at follow-up periods. However, 15-20$\%$ of children with initial heavy proteinuria showed persistent heavy proteinuria (3 out of 20 cases at 1-2 years, and 2 out of 10 case at 3-4 years of follow-up periods). Conclusion : The majority of children with HS nephritis (histopathologic grade I, II, III) improved within 3-4 years and persistent heavy proteinuria was seen only in a kw of children with initial clinical presentation of heavy proteinuria.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.2
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• pp.181-190
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• 2001

Purpose: The aim of this study is to investigate the usefulness of intestinal ultrasonography (US) and upper gastrointestinal endoscopy in the early diagnosis of Henoch-Sch$\"{o}$nlein purpura (HSP) with the gastrointestinal (GI) symptoms preceding the emergence of the skin lesion. Methods: The clinical, intestinal US and upper gastrointestinal endoscopic records of 85 patients (88 cases) with GI symptoms relating to HSP presenting between January 1999 and April 2001 were reviewed. Results: 1) GI symptoms were observed in 52 cases (59%) and skin, joint, renal and scrotal manifestations were observed in 88 (100%), 64 (73%), 15 (17%), 3 cases (3%) respectively. 2) Out of 52 cases with GI symptoms, abdominal pain was observed in all cases (100%). Positive stool occult blood, nausea and vomiting, abdominal tenderness, melena or tarry stool, diarrhea, hematemesis, rebound tenderness and rigidity were observed in 28 (50%), 17 (33%), 17 (33%), 12 (23%), 6 (12%), 4 (8%), 1 (2%) and 1 case (2%) respectively in order of frequency. 3) Intestinal US examination was performed in 27 cases with HSP and GI symptoms (52 cases). Out of 27 sonographic examinations 22 showed abnormal findings. Thickening of the duodeno-jejunal wall was observed in 16 cases (73%). Free peritoneal fluid, enlarged mesenteric lymph node, ileus and abnormal gall bladder were seen in 8 (36%), 8 (36%), 4 (18%) and 1 case (5%) respectively. In three cases of HSP without GI symptoms, those changes were absent. 4) In all of five cases with HSP and GI symptoms, endoscopic study showed mucosal edema and multiple hemorrhagic erosions especially at the second portion of the duodenum. Biopsy specimens from the duodenum of 2 cases out of 5 endoscopic examinations showed acute inflammatory infiltrates in the mucosa with hemorrhage. 5) Both intestinal US and endoscopic studies were performed in 4 cases with HSP and GI symptoms simultaneously. Out of 4 those cases, 3 cases showed the thickened duodeno-jejunal wall on the intestinal US, which suggested erosive hemorrhagic duodenitis by endoscopic findings. Conclusion: The typical but nonpathognomonic intestinal US findings including the thickening of the duodeno-jejunal wall and upper gastrointestinal endoscopic findings including hemorrhagicerosive duodenitis, in children with GI symptoms, should be considered a manifestation of HSP, even in the absence of skin lesion.

• The Journal of the Korean bone and joint tumor society
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• v.9 no.1
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• pp.12-17
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• 2003

Purpose: In this report we are going to discuss about the functional evaluation and the outcome of treatment of metastatic tumor in the lower extremities treated with tumor prosthetic arthroplasty. Materials and Methods: This report is based on nine patients diagnosed as a metastatic tumor and treated by tumor prosthetic arthroplasty, from June 1998 to December 2001. Age of the patients ranged from 49 to 63 with the average of 56.3. The average follow up period was 23.4 months. Two patients had lung cancer, three had breast cancer, two had renal cancer, one colon cancer, and one had multiple myeloma. All these were primary cancers. The site of metastasis were six in proximal femur, two in distal femur, and one in proximal tibia. Tumor excision was performed after biopsy in following the principle of primary tumor management. Excision with wide surgical margin was tried as possible could. Six cases were treated with tumor prosthesis, and the other three cases were reconstructed with bone cement and arthroplasty. Results: The functional evaluation in the extremities at the last follow up was performed on Enneking evaluation score with 6 categories. The highest scored 26, and the lowest scored 10, with an average of 19.5. A case in which the patient died 15 days after the operation was excluded from the evaluation. Among the categories, emotional acceptance to postoperative function and pain relief were highly scored. At the final follow up, seven patients survived, and one colon cancer patient died 68 days after operation. Conclusion: Metastatic tumor occurring in joints of lower extremities could be treated in accordance to the treatment principle of primary tumor. By insertion of tumor prosthesis, we can get satisfactory results of function in the lower extremity and pain relief especially. So, this aspect of medical favor must be considered in treating patients.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.167-174
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• 2005

Purpose : Relapses are a major problem in children with steroid responsive nephrotic syndrome(SRNS). This study has been performed to determine the predictive factors for relapse in children with SRNS. Methods : The study group consisted of 7,3 children with SRNS who had been admitted to the Department of Pediatrics, Kyungpook National University Hospital, over 6 years from 1996 to 2001. The medical records were reviewed retrospectively and analyzed to determine significant relationships between selected variables[age at onset, sex, laboratory data, the rapidity of response(days to remission), interval to first relapse] and the frequency of relapse. Results : The age($mean{\pm}SD$) of patients was $4.53{\pm}2.53$ years old. The male to female ratio was 52:21. In 95$\%$, 39 out of the 41 children had a renal biopsy, and the final diagnosis was minimal change nephrotic syndrome. There was no significant correlation between the frequency of relapse and the following variables age at onset, sex, and presence of hematuria. However, the rapidity of response correlated well with the frequency of relapse, especially during the first year after the onset of the disease(P=0.005). Conclusion : The rapidity of response is expected to be one of the predictive (actors for relapse in children with SRNS. (J Korean Soc Pediatr Nephrol 2005;9:167-174)