• Childhood Kidney Diseases
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• 제11권2호
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• pp.185-194
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• 2007

목 적 : HSP는 주로 소혈관을 침범하는 혈관염으로 대개 자연적으로 치유되지만 장기적 예후는 신장 침범의 정도에 달려 있으므로 신장 침범의 위험인자를 예측하고 예방하는 것이 중요하다. 이에 본 연구는 증상을 완화하기 위해 사용한 스테로이드가 HSP에서 신장 침범을 줄일 수 있는지를 알아보고자 하였다. 방 법 : 1993년 1월부터 2006년 12월까지 충북대학교병원 소아과에서 HSP로 진단 받은 환아를 대상으로 하여 스테로이드 사용 유무와 사용 기간, 지속성 자반의 유무에 따른 신장 침범의 유무와 정도를 분석하였다. 결 과 : 스테로이드 사용 유무와 사용 기간에 따른 신장 침범 빈도와 신장 침범 기간에는 유의한 차이가 없었다. 스테로이드의 사용 여부에 따른 신장 침범 양상을 보았을 때, 스테로이드를 사용한 경우에는 혈뇨가 많이 나타나고 사용하지 않은 경우에는 단백뇨가 많이 나타나는 경향을 보였으나 통계적으로는 유의한 차이가 없었다. 스테로이드의 사용과 자반의 지속성과는 유의한 관계가 없었다. 반면에 10일 이상의 지속성 자반이 있는 경우에 신장 침범이 유의하게 많은 것으로 나타났으며, 신장 침범의 기간도 지속성 자반이 있는 경우에 증가하는 것으로 나타났다. 결 론 : HSP로 진단받은 환아들에게 복통이나 관절통 등으로 스테로이드를 사용한 후 신장 침범의 유무를 비교해 본 결과, 스테로이드의 사용 여부와 사용 기간과는 유의한 결과를 보이지 않으므로 신장 침범에 대한 스테로이드의 예방효과는 없는 것으로 생각된다. 또한, 스테로이드 치료의 적응증이 아닌 자반의 지속성이 오히려 신장 침범의 유무와 기간과 관계있다는 사실은, 스테로이드가 신장 침범의 예방효과가 없음을 뒷받침해 주고 있다.

• Childhood Kidney Diseases
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• 제26권1호
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• pp.18-24
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• 2022

Pediatric rheumatologic diseases are rare systemic diseases that can involve various organs, including the kidneys. Each rheumatologic disease can exhibit characteristic renal involvement, which requires proper treatment and diagnosis. In this review, we discuss renal involvement in classic rheumatologic diseases, including juvenile idiopathic arthritis, Sjogren's syndrome, systemic sclerosis, and juvenile dermatomyositis. Reviews addressing lupus nephritis and antineutrophil cytoplasmic antibody-associated renal disease are complex and tend to cover a wide array of topics, and thus were excluded from this review.

• Childhood Kidney Diseases
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• 제10권2호
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• pp.125-131
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• 2006

Purpose : High interleukin-1 beta(IL-$1{\beta}$) expression in the skin biopsy specimens of patients with Henoch-$Sch\ddot{o}nlein$ Purpura(HSP) has been observed. We examined IL-$1{\beta}$ gene polymorphism in patients with HSP. The purpose of this study is to examine the relationship between IL-$1{\beta}$ gene polymorphism and renal involvement in HSP. Methods : Patients from mideast Korea with HSP were studied. All patients had at least 6 months of follow up. Patients and ethnically matched controls were genotyped for IL-$1{\beta}$ gene polymorphism by polymerase chain reaction-restriction fragment length polymorphism(PCR-RFLP). Results : Thirty-four patients(all younger than 15 years old) who had been diagnosed with HSP and 27 controls were examined. No allele or genotype differences between the HSP and control groups were observed. No significant association between the carriage of IL-$1{\beta}$(-511) T allele and renal involvement(P=0.525, OR:1.417, CI:0.545-3.686) was found. Conclusion : In unselected patients with HSP, carriage of IL-$1{\beta}$(-511) T allele does not appear to influence renal involvement.

• Childhood Kidney Diseases
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• 제26권2호
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• pp.80-85
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• 2022

Purpose: To determine the prevalence, clinical manifestations, and outcomes of renal involvements in pediatric Alagille syndrome (ALGS). Methods: A total of 21 patients diagnosed with ALGS at age under 18 years who visited Samsung Medical Center from March 1999 to March 2022 were enrolled. ALGS was diagnosed either by clinical manifestations, targeted JAG1 sequencing, and/or liver biopsy. Medical records including sex, age, renal manifestations, urinalysis, serum creatinine, JAG1 sequencing, and ultrasonography were retrospectively reviewed. Results: The male to female ratio was 9:12. The mean age of patients at confirmative diagnosis of ALGS was 18.4 months. Sanger sequencing was performed for 17 patients. Sixteen of 21 patients (76.1%) showed JAG1 mutations. Renal involvement was found in 10 patients (47.6%). The most common type of anomaly was renal dysplasia (40%). One patient having renal dysplasia was pathologically confirmed with glomerular lipid deposition. Two patients (20%) manifested nephrocalcinosis/nephrolithiasis. Among eight renal-involved patients who survived, four (50%) progressed to chronic kidney disease stage 3. Two of these chronic kidney disease patients were diagnosed with hepatorenal syndrome. The other four patients had renal functions preserved, including two without any interventions and two who underwent urological interventions. Conclusions: The current study revealed a high prevalence of renal involvement in Korean pediatric ALGS with diverse phenotypes.

• The Korean Journal of Physiology
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• 제19권2호
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• pp.173-187
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• 1985

Renal compensatory adaptation caused by ablation of a part of renal mass has long been known in the field of the compensatory renal hypertrophy or hyperplasia. Many reports were found on the chronic mechanisms on the compensatory renal hyperfunction after exclusion of the contralateral kidney. However the mechanism(s) of the acute compensatory hyperfunction after contralateral exclusion has not yet been clarified. In the present experiment, we have tried to prove the possibility of the involvement of the renin-angiotensin system and/or prostaglandin system in the control mechanism of the acute compensatory renal hyperfunction after contralateral kidney exclusion. There were found different responses of the renal hyperfunction by contralateral renal pedicle or ureteral occlusion. Contralateral renal pedicle or ureteral occlusion caused a sustained increases of the urinary volume, sodium and potassium excretion, while the magnitude of the changes was different quantitatively by the maneuvers. Blood collection affected on the acute compensatory renal responses after ureteral as well as renal pedicle occlusion. Plasma prostaglandin $E_2$ level was not changed by the contralateral renal pedicle or ureteral occlusion. Urinary excretion of Prostaglandin $E_2$, the indices of renal prostaglandin biosynthesis, was not changed by the contralateral renal pedicle occlusion, but increased without significance by the contralateral ureteral occlusion. Acute renal compensatory responses after contralateral renal pedicle occlusion were blocked by the pretreatment of indomethacin. Plasma renin activity increased after contralateral ureteral occlusion, but the pattern of the increases was the same as in the time-control group. Plasma renin activity after contralateral renal pedicle occlusion did not change by the time sequence. SQ 20,881, an angiotensin I converting enzyme inhibitor, blunted the contralateral renal responses after the renal pedicle occlusion. Bilateral renal denervation abolished the contralateral renal responses after the renal pedicle occlusion. The above data suggest that there is no direct evidence to support the involvement of the renin-angiotensin system and/or prostaglandin system for the acute compensatory renal hyperfunction after contralateral kidney exclusion, and that the functional changes of the intact kidney may be caused by a humoral substances, or other mechanisms by afferent renal nerve activity originating from the treated kidney.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제19권3호
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• pp.175-185
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• 2016

Purpose: To investigate the epidemiology, clinical manifestations, investigations and management, and prognosis of patients with Henoch-Schonlein purpura (HSP). Methods: We performed a retrospective review of 212 HSP patients under the age of 18 years who were admitted to Inje University Sanggye Paik Hospital between 2004 and 2015. Results: The mean age of the HSP patients was 6.93 years, and the ratio of boys to girls was 1.23:1. HSP occurred most frequently in the winter (33.0%) and least frequently in the summer (11.3%). Palpable purpura spots were found in 208 patients (98.1%), and gastrointestinal (GI) and joint symptoms were observed in 159 (75.0%) and 148 (69.8%) patients, respectively. There were 57 patients (26.9%) with renal involvement and 10 patients (4.7%) with nephrotic syndrome. The incidence of renal involvement and nephrotic syndrome was significantly higher in patients with severe GI symptoms and in those over 7 years old. The majority of patients (88.7%) were treated with steroids. There was no significant difference in the incidence of renal involvement or nephrotic syndrome among patients receiving different doses of steroids. Conclusion: In this study, the epidemiologic features of HSP in children were similar to those described in previous studies, but GI and joint symptoms manifested more frequently. It is essential to carefully monitor renal involvement and progression to chronic renal disease in patients ${\geq}7$ years old and in patients affected by severe GI symptoms. It can be assumed that there is no direct association between early doses of steroids and prognosis.

• Childhood Kidney Diseases
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• 제4권1호
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• pp.40-47
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• 2000

목 적 : Henoch-$Sch\ddot{o}nlein$(HS)자반증에서 신증상은 가장 심각한 증상이고 HS자반증의 예후와 관련되어 중요성이 강조되고 있다. 이에 대하여 immunoglobulins, 보체계, interleukin(IL)-1, interleukin(IL)-6 또는 tumor necrosis factor(TNF)-$\alpha$등과 같은 pro-inflammatory cytokines 등의 병인론적 연구가 진행되고 있다. 본 연구의 목적은 HS자반증의 임상 증상들을 신침범 유무에 따라 비교하여 신침범의 임상적 위험인자를 알아보고자 하였고 급성기와 회복기 혈청 및 뇨중 TNF-$\alpha$의 농도를 각각 측정하여 신침범 환아에서 병인론적 역할을 검증해 보고자 하였다. 대상 및 방법 : 1998년 3월부터 1999년 4월까지 충북대학교병원 소아과를 방문한 HS자반증 환아 12명, HS 신염 환아 7명, 연령별 대조군 5명을 대상으로 환아의 연령, 성별, 전구증상, 복통, 관절통, 자반의 정도와 지속기간, 스테로이드의 사용유무 등에 따른 신증상 발현의 상관관계를 조사하였다. 또한 대상 환아들의 혈청은 내원 당시 채혈하여 3,000g, 5분 동안 원심분리하여 $-20^{\circ}C$에 보관하였으며 소변은 내원 당시 채취하여 $-20^{\circ}C$에 보관 후,R & D system(Mineapolis, USA)의 $Quantikine^{TM}$ human TNF-$\alpha$ immunoassay kit를 이용하여 TNF-$\alpha$의 농도를 측정하였다. 결 과 :임상인자 중 자반이 4주 이상 지속되는 지속성 자반증 환아에서 신염 발생율이 의미있게 높았으며 (P=0.0018), 복통이나 관절통 등으로 인하여 급성기에 사용한 스테로이드는 신염의 발생율과는 연관성이 없었고 자반의 지속기간에도 영향을 주지 않았다. 급성기 혈청 TNF-$\alpha$는 신염을 동반한 HS자반증 환아에서 연령별 대조군이나 신염이 없는 HS자반증 환아보다 의미있게 증가하였으나(P=0.027, P=0.012) 뇨중 TNF-$\alpha$농도는 연광성을 발견할 수 없었다. 또한 혈청 TNF-$\alpha$의 상승과 임상적 위험인자와의 연관성을 조사해 보았을 때 지속적 자반군에서 통계적으로 유의한 증가를 보였다(P=0.038). 결 론 :혈청 TNF-$\alpha$농도는 HS자반증에서 신염의 발생과 관련이 있으므로 임상적 위험인자로 자반의 지속기간과 함께 HS자반증 환아의 신침범에 대한 예측인자로 활용할 수 있을 것이며 따라서 HS신염의 예방과 치료에 있어서 TNF-$\alpha$에 대한 생성억제제나 특이항체 등의 이용가능성에 대한 연구가 필요할 것이다.

• Korean Journal of Radiology
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• 제22권8호
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• pp.1323-1331
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• 2021

Objective: To identify the association between renal tumor complexity and pathologic renal sinus invasion (RSI) and evaluate the usefulness of computed tomography tumor features for predicting RSI in patients with renal cell carcinoma (RCC). Materials and Methods: This retrospective study included 276 consecutive patients who underwent radical nephrectomy for RCC with a size of ≤ 7 cm between January 2014 and October 2017. Tumor complexity and anatomical renal sinus involvement were evaluated using two standardized scoring systems: the radius (R), exophytic or endophytic (E), nearness to collecting system or sinus (N), anterior or posterior (A), and location relative to polar lines (RENAL) nephrometry and preoperative aspects and dimensions used for anatomical classification (PADUA) system. CT-based tumor features, including shape, enhancement pattern, margin at the interface of the renal sinus (smooth vs. non-smooth), and finger-like projection of the mass, were also assessed by two independent radiologists. Univariable and multivariable logistic regression analyses were performed to identify significant predictors of RSI. The positive predictive value, negative predictive value (NPV), accuracy of anatomical renal sinus involvement, and tumor features were evaluated. Results: Eighty-one of 276 patients (29.3%) demonstrated RSI. Among highly complex tumors (RENAL or PADUA score ≥ 10), the frequencies of RSI were 42.4% (39/92) and 38.0% (71/187) using RENAL and PADUA scores, respectively. Multivariable analysis showed that a non-smooth margin and the presence of a finger-like projection were significant predictors of RSI. Anatomical renal sinus involvement showed high NPVs (91.7% and 95.2%) but low accuracy (40.2% and 43.1%) for RSI, whereas the presence of a non-smooth margin or finger-like projection demonstrated comparably high NPVs (90.0% and 91.3% for both readers) and improved accuracy (67.0% and 73.9%, respectively). Conclusion: A non-smooth margin or the presence of a finger-like projection can be used as a preoperative CT-based tumor feature for predicting RSI in patients with RCC.

• Childhood Kidney Diseases
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• 제21권2호
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• pp.61-68
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• 2017

Purpose: To investigate differences in clinical features, blood/urinary findings, and prognosis in different age groups of patients with Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP). Methods: A total of 469 patients with HSP were analyzed retrospectively from June 2003 to February 2016. We classified patients into child or adult groups based on their age. Results: The adult group had more patients with anemia (child vs. adult; 7.5% vs. 16.4%), and higher immunoglobulin A (IgA) (30.0% vs. 50.0%) levels, C-reactive protein (34.2% vs. 54.0%) and uric acid (3.1% vs. 12.1%) levels than the child group. The child group was highly positive for Mycoplasma pneumoniae immunoglobulin M (IgM) (34.4%). More patients in the child group presented with high levels of antistreptolysin O (24.7% vs. 2.9%) and high C4 (11.5% vs. 4.2%). Low C3 (1.1% vs. 10.2%) levels, and renal involvement with gross hematuria (8.6% vs. 21.5 %), nonnephrotic proteinuria (1.1% vs. 11.2%), and nephrotic syndrome (1.1% vs. 6.0%) were common in the adult group. Adults also had poorer renal outcomes [persistent hematuria/proteinuria (10.5% vs. 32.8%), and chronic kidney disease (0% vs. 11.2%)] than the child group. Risk factors for renal involvement such as older age and higher level of uric acid were only found in the child group. The risk factors for poor renal outcome were nephrotic syndrome in the child group and gross hematuria in the adult group. Conclusion: In this study, child and adult groups presented with different clinical manifestations of HSP. We found that risk factors for renal involvement included age and high uric acid level in the child group. Moreover, nephrotic syndrome in the child group and gross hematuria in the adult group increased the risk of poor renal outcome.

• Childhood Kidney Diseases
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• 제21권2호
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• pp.156-159
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• 2017

Systemic lupus erythematosus (SLE) is characterized by various symptoms and multi-organ involvement. Hypophosphatemia has been described in several diseases accompanied with systemic inflammation. However, hypophosphatemia has rarely been described in SLE patients, especially in those without nephritis. We report the case of a 13-year-old girl with SLE who developed hypophosphatemia without renal involvement. Her hypophosphatemia was caused by renal loss of phosphorus and persisted for 7 months. It improved as her complement levels increased. Therefore, hypophosphatemia may be related to disease activity in SLE patients.