• Childhood Kidney Diseases
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• 제14권1호
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• pp.32-41
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• 2010

목 적 : 사구체신염은 흔히 단백뇨를 보이며 특이 치료법이 없고, 만성 신부전으로 발전하는 경우가 많다. 몇몇 연구에서 중간엽 줄기세포(Mesenchymal stem cell, MSC)를 실험적 사구체신염에 투여하여 단백뇨가 호전된 것을 보고한 바 있으나, 이는 신염을 일으키는 약제와 중간엽 줄기세포를 함께 투여하거나 신장에 직접 투여한 것이었다. 본 연구에서는 실험적 신병증에서 단백뇨가 발현된 시점에서 정주 요법으로 MSC를 투여함으로써 MSC의 임상적인 적용 가능성을 탐색하였다. 방 법 : 실험용 생쥐에 Adriamycin을 투여하여 신병증(ADR-GN)을 유발한 후, 2주 후에 대량의 단백뇨를 확인하고 MSC를 생쥐 꼬리의 정맥에 주사하였다. MSC에 의한 질병 완화의 기전을 확인하기 위한 in vitro 실험으로 mixed lymphocyte culture(MLC)에 MSC를 투여하였을 때의 염증 관련 cytokine인 IFN-$\gamma$ and IL-10의 변화를 측정하였다. 결 과 : 실험용 생쥐에 ADR-GN를 유발하고 단백뇨가 보일 때 MSC를 정주한 군에서는 단백뇨의 소실이 더 먼저 관찰되었다. 또한 MSC를 투여받은 군에서의 생존률이 더 나은 경향이 관찰되었다. MLC 에 MSC를 투여하였을 때, 염증을 유발하는 cytokine인 IFN-$\gamma$ 는 감소하고 염증을 억제하는 cytokine인 IL-10는 증가하였다. 결 론 : 이 연구는 이전의 보고들에서 관찰되었던 사구체신염에서의 MSC의 질병완화 효과가 좀더 임상적으로 적용 가능한 방법으로 투여된 경우, 즉 단백뇨가 있을 때 정주 요법으로 투여한 경우에도 관찰됨을 확인하였다. 이러한 효과의 기전과 임상적용에 요구되는 안전성 등에 대한 확인을 위해서는 추가 연구가 필요하겠다.

• Childhood Kidney Diseases
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• 제27권2호
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• pp.97-104
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• 2023

Purpose: Angiotensin-converting enzyme inhibitors and angiotensin II receptor blockers (ARBs) are frequently employed to counteract the detrimental effects of proteinuria on glomerular diseases. However, the effects of ARBs remain poorly examined in pediatric patients with immunoglobulin A (IgA) nephropathy. Herein, we evaluated the efficacy and safety of losartan, an ARB, in pediatric IgA nephropathy with proteinuria. Methods: This prospective, single-arm, multicenter study included children with IgA nephropathy exhibiting proteinuria. Changes in proteinuria, blood pressure, and kidney function were prospectively evaluated before and 4 and 24 weeks after losartan administration. The primary endpoint was the difference in proteinuria between baseline and 24 weeks. Results: In total, 29 patients were enrolled and received losartan treatment. The full analysis set included 28 patients who received losartan at least once and had pre- and post-urinary protein to creatinine ratio measurements (n=28). The per-protocol analysis group included 22 patients who completed all scheduled visits without any serious violations during the study period. In both groups, the mean log (urine protein to creatinine ratio) value decreased significantly at 6 months. After 24 weeks, the urinary protein to creatinine ratio decreased by more than 50% in approximately 40% of the patients. The glomerular filtration rate was not significantly altered during the observation period. Conclusions: Losartan decreased proteinuria without decreasing kidney function in patients with IgA nephropathy over 24 weeks. Losartan could be safely employed to reduce proteinuria in this patient population. ClinicalTrials.gov trial registration (NCT0223277)

• Childhood Kidney Diseases
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• 제5권1호
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• pp.64-68
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• 2001

Nutcracker증후군은 좌측 신정맥이 대동맥과 상장간막 동맥에 압박되어 발생하는 드문 질환으로 혈뇨, 단백뇨, 기립성 단백뇨의 원인이 된다. 초음파와 도플러 검사로 진단이 가능하다. 저자들은 기립성 단백뇨와 특발성 만성피로를 보인 보기드문 심한 Nutcracker 증후군 환아를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• International journal of thyroidology
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• 제11권2호
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• pp.78-81
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• 2018

Lenvatinib, an oral multi-kinase inhibitor, is a valuable treatment option for advanced differentiated thyroid carcinoma. However, severe treatment-related adverse events occur up to 30% of the patients receiving lenvatinib, making it a challenge for clinicians to maintain this drug and therefore affecting the outcome of therapy. Blood vessel related events, such as hypertension or proteinuria, are among the most frequent adverse events. We present a case of 65-year-old man with radioactive iodine refractory papillary thyroid carcinoma with cervical lymph node metastasis and tracheal invasion receiving lenvatinib who developed proteinuria and worsening of hypertension. Management with repeated dose reductions and using supportive medications allowed this patient to continue lenvatinib with his disease stably controlled. Early detection of patients at risk for these adverse events and cautious administration of lenvatinib at appropriate level are crucial in managing patients receiving lenvatinib.

• Childhood Kidney Diseases
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• 제23권1호
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• pp.48-52
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• 2019

The ketogenic diet (KD) has been used as an effective antiepileptic therapy for intractable childhood epilepsy. However, various adverse effects have been reported with use of the KD. We report a case of a child who developed acute tubular necrosis subsequent to therapy with KD. A 5-year-old girl had myoclonic epilepsy with developmental delay. She was under the treatment with antiepileptic drugs since the age of 3 months and on the KD during the past 18 months. Proteinuria persisted intermittently with the initiation of the KD and subsequently increased in the past 2 months. She was admitted with intermittent mild fever, vomiting, and lethargy for the past 3-4 weeks. At the time of admission, she presented with hypertriglyceridemia, heavy proteinuria, renal Fanconi syndrome, and acute kidney injury. Renal sonography showed a marked increase in the size and parenchymal echogenicity of both kidneys. A renal biopsy revealed acute tubular necrosis accompanied by early interstitial fibrosis. After the withdrawal of the KD and supportive therapy, without changing other anticonvulsants and their dosages, improvement of renal function was observed. Proteinuria had disappeared after 1 month and kidney size returned to normal after 8 months. It is hypothesized that the KD can induce and/or aggravate the renal tubulointerstitial injury in some patients who are under the treatment with anticonvulsants.

• Childhood Kidney Diseases
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• 제27권1호
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• pp.26-33
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• 2023

Purpose: Hematuria and proteinuria have various causes and consequential outcomes in children. Immunosuppressants are needed in some children with biopsy-proven glomerulonephropathy but have many adverse effects. Since the clinical practice patterns of Korean pediatric nephrologists are diverse, we surveyed their opinions. Methods: Using a clinical vignette, the survey was emailed to all Korean Society of Pediatric Nephrology members. The questionnaires included diagnosis, examination, medications, and dietary recommendations for patients with hematuria and proteinuria. Results: A total of 32 clinicians (5.48%, 22 pediatric certificated nephrologists) responded to the survey. Most responders (87.5%) suspected immunoglobulin A nephropathy, and 68.8% replied that kidney biopsies were a diagnostic tool. Renin-angiotensin system inhibition (62.5%) or steroids (18.8%) were selected as the treatment. Salt and protein intakes were usually encouraged as dietary reference intakes (34.4% and 65.6%, respectively). Conclusions: Children with abnormal urinalysis have various causes, treatments, and prognoses. As treatments such as immunosuppressants can have many adverse effects, it is necessary to confirm an accurate diagnosis and indications of treatments before starting the treatment. Recommendations for a diet should not hinder growth.

• Childhood Kidney Diseases
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• 제10권2호
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• pp.213-218
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• 2006

Purpose : Nutcracker syndrome refers to compression of the left renal vein(LRV) between the aorta and superior mesenteric artery(SMA) that results in elevation of pressure in the LRV and development of collateral veins. It must be considered as a possible factor when hematuria or proteinuria occurs in a healthy child. The purpose of this study is to determine the time to spontaneous resolution in childhood nutcracker syndrome, and to observe whether this is affected by sex, age, proteinuria or initial ratio of peak velocity of LRV. Methods : We investigated 26 patients who were found to have spontaneous resolution by follow-up Doppler ultrasonography among 117 patients diagnosed with nutcracker syndrome by renal Doppler ultrasonography from May 2001 to December 2005. We determined the time to spontaneous resolution in childhood nutcracker syndrome, and observed whether the duration was affected by sex, age, proteinuria or initial ratio of peak velocity. Results : 26 patients(59%) achieved spontaneous resolution by 1.2 years(mean). The time to spontaneous resolution of childhood nutcracker syndrome in 26 patients was $16.71{\pm}9.99$ months(range 6.0-49.2). The time to spontaneous resolution was not affected by sex, age, proteinuria nor initial ratio of peak velocity of LRV. Conclusion : More than half of the patients who were diagnosed by renal Doppler ultrasonography achieved spontaneous resolution. The time to spontaneous resolution was not affected by sex, age, proteinuria nor initial ratio of peak velocity of LRV.

• Journal of Yeungnam Medical Science
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• 제31권2호
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• pp.127-130
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• 2014

Autoimmune thyroiditis is the most common cause of hypothyroidism in the world. It is characterized clinically by gradual thyroid failure, goiter formation, or both, because of the autoimmune-mediated destruction of the thyroid gland. Renal involvement presenting proteinuria in autoimmune thyroiditis is not uncommon, occurring in 10% to 30% of the cases. Glomerulonephropathy associated with autoimmune thyroiditis, however, is a rare disease. Most reports of autoimmune thyroiditis with glomerulonephropathy have demonstrated a mixed pathological morphology and have been predominantly associated with membranous glomerulopathy. The case of minimal-change disease associated with thyroiditis presenting acute kidney injury is a rare disease that has not been reported in South Korea. Reported herein is the case of a 16-year-old man diagnosed with Hashimoto's thyroiditis, with minimal-change disease presenting acute kidney injury. He revealed hypothyroidism, proteinuria, and impaired renal function. Renal biopsy showed minimal-change disease and minimal tubular atrophy. The patient was treated with thyroid hormone, and his renal function and proteinuria improved. Therefore, for patients with autoimmune thyroiditis presenting unexplained proteinuria, glomer-ulonephropathy should be ruled out. Conversely, for patients with glomerulonephropathy and persistent proteinuria despite proper treatment, thyroid function and antibody tests should be performed.

• Childhood Kidney Diseases
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• 제23권2호
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• pp.105-110
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• 2019

Purpose: We sought to determine associations of urinary neutrophil gelatinase-associated lipocalin (NGAL) and liver-type fatty acid-binding protein (L-FABP), known markers of renal injury, with hematuria in children and adolescents. Methods: A total of 112 urine samples from 72 patients aged 2 to 18 years with hematuria were enrolled in this study. Urinary concentrations of NGAL and L-FABP were measured by ELISA and compared between subjects with and without proteinuria and between subjects with and without glomerulonephritis diagnosed by renal biopsy. Results: Urinary concentrations of NGAL and L-FABP/creatinine (Cr) in subjects with proteinuria were not significantly different from those in subjects without proteinuria. They were not significant different between subjects with and without glomerulonephritis either. However, both concentrations of urinary NGAL and L-FABP/Cr were positively associated with urinary protein to creatinine ratio. Their levels had a tendency to be increased when proteinuria developed at later visits in subjects with hematuria only at initial visits. Conclusion: Monitoring urinary NGAL and L-FABP levels in addition to conventional risk factors such as proteinuria and serum creatinine might improve the prediction of renal injury in pediatric patients with hematuria.

• Childhood Kidney Diseases
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• 제3권1호
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• pp.57-63
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• 1999

목 적 : 신질환의 조기 진단을 위한 집단뇨검사 방법으로 단백뇨와 혈뇨검사를 동시에 시행하여 그 결과를 분석함으로써 집단뇨검사에서 잠혈검사 추가가 가지는 진단적 의의를 알아 보고자 하였다. 방법 및 대상 : 1차 선별검사는 전국 33개교 26,508명의 고등학교 1년생(16세군)을 대상으로 하였고 정밀검사는 서울시 1개교 875명의 고등학교 1년생(16세군)을 대상으로 실시하였다. 방법은 1997년 8월부터 12월까지 4개월간 한국 건강관리 협회와 공동으로 학교에서 무작위로 채취된 소변을 Combur-10 $N^{(R)}$ M (Boehringer Mannheim, Germany)검사지로 단백뇨, 혈뇨, 농뇨, 아질산염 등을 검사하였다. 양성자의 기준은 단백뇨, 혈뇨, 농뇨가 $1^{+}$ 이상인 경우와 아질산염에 양성반응이 나오는 경우로 정하였다. 실제 신질환의 감별을 위한 정밀검사는 1차 집단뇨검사 양성자를 대상으로 본원에서 실시하였다. 결 과 : 1) 1차 집단검사 결과에서 단백뇨 유병율은 $0.73\%$, 혈뇨 유병율은 $2.69\%$, 농뇨 유병율은 $0.23\%$ 그리고 아질산염 유병율은 $0.03\%$로 조사 되었다. 2)서울지역 1개교(875명)선별 정밀검사 결과: 1차검사 단백뇨 양성자 8명($0.91\%$)중 1명은 기립성 단백뇨, 나머지 7명은 일시적 단백뇨로 확인되었다. 1차검사 혈뇨 양성자는 41명($4.86\%$)이었고 이중 재검이 가능했던 33명중 6명만이 지속적인 혈뇨 양성자였고, 다시 시행한 정밀검사에서 한명만이 무증상 단독 혈뇨증으로 확인되었으며 나머지 5명은 정상이었다. 1차 소변검사 이상자 52명에 대한 혈액검사 및 초음파검사는 모두 정상이었다. 1차검사에서 혈뇨 양성자 33명중 1명(약$3\%$)만이 신질환이 의심되는 경우였고 나머지 32명($91\%$)은 정상이었다. 결 론 : 1) 혈뇨검사는 위양성이 높기 때문에 ($97\%$) 집단뇨검사에 포함시킬 경우 불필요한 시간과 경비의 낭비가 있을수 있다고 생각된다. 2) 집단뇨검사는 검사 기관에 따라 얻어지는 결과의 차이가 심하기 때문에 단일 기관으로 통합 관리해야 할 것이다. 3) 요로감염증의 선별검사에서 농뇨와 아질산염 검사는 양성율이 낮으므로 그 효용성에 대한 연구는 좀더 필요하다고 생각된다.