• Kidney Research and Clinical Practice
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• v.36 no.2
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• pp.200-204
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• 2017

Administration of autologous mesenchymal stem cells (MSCs) has been shown to improve renal function and histological findings in acute kidney injury (AKI) models. However, its effects in chronic kidney disease (CKD) are unclear, particularly in the clinical setting. Here, we report our experience with a CKD patient who was treated by intravenous infusion of autologous MSCs derived from adipose tissue in an unknown clinic outside of Korea. The renal function of the patient had been stable for several years before MSC administration. One week after the autologous MSC infusion, the preexisting renal insufficiency was rapidly aggravated without any other evidence of AKI. Hemodialysis was started 3 months after MSC administration. Renal biopsy findings at dialysis showed severe interstitial fibrosis and inflammatory cell infiltration, with a few cells expressing CD34 and CD117, 2 surface markers of stem cells. This case highlights the potential nephrotoxicity of autologous MSC therapy in CKD patients.

• Childhood Kidney Diseases
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• v.23 no.1
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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.

• 대한소아신장학회:학술대회논문집
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• 2007.04a
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• pp.18-19
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• 2007

• Korean Journal of Transplantation
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• v.28 no.3
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• pp.135-143
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• 2014

Background: Kidney injury molecule-1 (KIM-1) is known as a good ancillary marker of acute kidney injury (AKI) and its expression has also been observed in acute rejection and chronic graft dysfunction. We tested usefulness of KIM-1 as an indicator of acute and chronic renal graft injury by correlating KIM-1 expression with renal graft function and histology. Methods: A total of 133 zero-time biopsies and 42 follow-up biopsies obtained within 1 year posttransplantation were selected. Renal tubular KIM-1 staining was graded semiquantitatively from 0 to 3 and the extent of staining was expressed as the ratio of KIM-1 positive/CD10 positive proximal tubules using Image J program. Results: KIM-1 was positive in 39.8% of zero-time biopsies. KIM-1 positive cases were predominantly male and had received grafts from donors with older age, deceased donors, and poor renal function at the time of donation, compared with KIM-1 negative cases. KIM-1 expression showed correlation with delayed graft function and acute tubular necrosis. In comparison of KIM-1 expression between stable grafts (n=23) and grafts with dysfunction (n=19) at the time of repeated biopsy, the intensity/extent of KIM-1 staining and renal histology at zero-time did not differ significantly between the two groups. Histologically, KIM-1 expression was significantly increased with both acute and chronic changes of glomeruli, tubules and interstitium, peritubular capillaritis, and arteriolar hyalinosis. Conclusions: KIM-1 can be used as an ancillary marker of AKI and a nonspecific indicator of acute inflammation and tubulointerstitial fibrosis. However, KIM-1 expression at zero-time is not suitable for prediction of long-term graft dysfunction.

• Clinical and Experimental Pediatrics
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• v.53 no.7
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• pp.770-773
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• 2010

A 13-year-old girl was diagnosed with non-cystic fibrosis (CF)-related multifocal bronchiectasis accompanied by nephrotic-range proteinuria of unknown cause. On renal biopsy, there were many segmental homogeneous deposits of amyloid tissue with positive Congo red staining in the glomeruli and interstitium. On electron microscopy, relatively straight, non-branching, randomly arranged amyloid fibrils were showed in the mesangium of the glomeruli. These fibrils were approximately 10 nm in diameter, compatible with secondary amyloidosis. Her level of serum amyloid A was remarkably elevated. To our knowledge, this girl is the first case of secondary renal amyloidosis induced by bronchiectasis in Korean children.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.166-175
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• 2004

Purpose: Present study has been undertaken to determine the distribution of various renal diseases causing asymptomatic hematuria in children and to evaluate the benefit of doing renal biopsy in these children. Methods: Study population consisted of 146 children with asymptomatic primary hematuria who had been admitted to the pediatric departmen of Kyungpook National University Hospital for the past 4 years from 1999 to 2002. In 122 out of 146 cases, renal biopsy was performed percutaneously and in 24 out of 146 cases, diagnosed as idiopathic hypercalciuria, oral calcium loading test was performed. Results: The age$(mean{\pm}SD)$ at onset or discovery of hematuria of the 146 children in-cluded in this study was $8.0\pm3.2$ years and the proportion of boys and girls was 54.8% and 45.2%, respectively. In 76 out of 146 cases(52%), asymptomatic hematuria was first diagnosed by school urinalysis screening. The proportion of histopathologic findings based on 122 biopsies was as follows : Thin Glomerular Basement Membrane(TGBM) 73 cases(50%): IgA nephropathy 20 cases(14%): Alport syndrome 6 cases(4%), Membranous Glomerulonephropathy(MGN) 4 cases(3%): Membranoproliferative Glomerulonephritis(MPGN) 2 cases(1%); IgA nephropathy with TGBM 3 cases(2%): 'normal' glomeruli 14 cases(10%) Twenty four cases (16%) were diagnosed as idiopathic hypercalciuria. During follow-up periods, 15% of 146 cases became hematuria-free and renal function did not deteriorate in any cases. Conclusion: Unless hematuric children manifest poor prognostic indicators for renal survival, we would recommend long term regular follow-up prior to a renal biopsy.

• Journal of Chest Surgery
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• v.44 no.6
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• pp.448-451
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• 2011

Microscopic polyangiitis (MPA) is a necrotizing vasculitis involving the small vessels without granulomatous inflammation. Most MPA initially presents with renal involvement without pulmonary involvement. Isolated and initially presenting alveolar hemorrhage is very rare. The patient was a 39-year-old female with a progressive cough, dyspnea, and blood-tinged sputum for the previous 5 days. We determined that her condition was MPA though VATS lung biopsy and renal biopsy. After 2 months of steroid therapy, the chest lesions had improved. We report here a rare case of MPA with isolated and initial involvement of the lung with a review of the literature.

• Childhood Kidney Diseases
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• v.17 no.1
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• pp.6-12
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• 2013

Thrombotic microangiopathy (TMA) is a microvascular thrombotic lesion caused by endothelial injury and subsequent formation of platelet rich thrombus. TMA is first described as a classical pathologic feature of HUS/TTP. Renal biopsy finding of TMA represents kidney involvement of HUS/TTP as well as other diseases such as malignant hypertension, drug toxicity, eclampsia, pre-eclampsia, and several systemic infections. Autoimmune diseases and transplant kidney sometime also have TMA. It is needed to consider a complete autoimmune work-up of patients presenting with TMA including tests for ANA, ANCA, and ADAMTS13 inhibitory antibodies, because there are several reports of association with TMA in patients of SLE, anti-phospholipid syndrome, and ANCA-associated vasculitis.

• Tuberculosis and Respiratory Diseases
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• v.53 no.2
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• pp.202-208
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• 2002

A diffuse alveolar hemorrhage is a rare manifestation in microscopic polyangiitis. Recently we experienced a case of diffuse alveolar hemorrhage associated with microscopic polyangiitis, which was diagnosed with the typical clinical manifestations, ANCA and a renal biopsy. A 71 year old female was admitted complaining of coughing and dyspnea. A chest X-ray, HRCT and BAL revealed a diffuse alveolar hemorrhage. A diffuse alveolar hemorrhage was noted during a bronchoscopy. She also had proteinuria, microscopic hematuria and mild azotemia. The renal biopsy showed necrotic glomerulonephritis without immune complex deposits or granuloma. Under the diagnosis of microscopic polyangiitis, she was treated with steroid pulse therapy, and prednisolone with cyclophosphamide subsequently. She showed marked improvement in the clinical manifestations.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.93-98
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• 2008

Rare cases of IgG associated mesangial glomerulonephritis(IgG GN) defined by exclusive or predominant mesangial IgG deposits were reported first by Sato et al.(1993). and subsequently 10 pediatric cases were reported by Yoshikawa et al.(1994). Previous reports suggested that the prognosis of IgG GN is relatively benign course but recent report suggested that prognosis of IgG GN is highly variable. Also the recurrence of IgG GN in a renal transplant was reported by Fakhouri et al. (2002). Such a recurrence highlights the specificity of this type of glomerulonephritis. We experienced two pediatric cases of IgG GN proven by renal biopsy. Case 1. 4-year-old girl with nephrotic syndrome admitted because of general edema. The patient's urinalysis showed proteinuria and microscopic hematuria. Renal biopsy was performed because of relapsed nephritic syndrome. Light microscopic finding was nonspecific with almost normal histology. Immunofluorescent findings showed diffuse segmental IgG(+) and IgM(+) deposits in the capillary walls, and focal segmental spotty C4(trace), C1q(trace) deposits. Electron microscopic findings showed focal portion of mesangial electron dense deposits without mesangial widening. Case 2. 11-year-old girl admitted for evaluation of microsopic hematuria detected through mass school urinary screening program. Renal biopsy was performed for exact diagnosis. Immunofluorescent findings showed focal segmental IgG(+), IgM(+/-) and C3(+/-) deposits. Electron microscopic findings showed focal portion of mesangial electron dense deposits without mesangial widening.