• Childhood Kidney Diseases
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• v.12 no.2
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• pp.227-232
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• 2008

A 16-year-old girl presented with proteinuria and microscopic hematuria detected through mass urinary screening and was diagnosed as having suspected postinfectious glomerulonephritis by renal biopsy. However, heavy proteinuria did not respond to angiotensin converting enzyme inhibitor therapy. After 6 months, cervical lymphadenitis developed and a neck node biopsy showed subacute necrotizing lymphadenitis. After an additional 2 months, she developed facial erythema and thrombocytopenia. A repeat renal biopsy demonstrated lupus nephritis class IV. She was treated with pulse methylprednisolone(500 mg/day intravenously for 3 consecutive days) followed by oral deflazacort and monthly intravenous cyclophosphamide pulse(1 g/$m^2$) for 6 months. We report a case diagnosed as systemic lupus erythematosus(SLE) during medical follow-up after urinary screening.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.206-209
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• 2001

Clq nephropathy is an immune complex glomerulonephritis defined by the presence of mesangial Clq deposits in immunofluorescence microscopy and electron dense deposits on electron microscopy. It was described as a distinct disease entity in 1985 by Jennette and Hipp. Thirty four cases were reported in the literature but there has been no pediatric case reported in Korea yet. It commonly presents with steroid- resistent nephrotic syndrome in older children and young adults, and occasionally nephritic-nephrotic syndrome or rapidly progressive glomerulonephritis We report a case of Clq nephropathy in a 23-month-old girl with steroid-dependent nephrotic syndrome. (J. Korean Soc Pediatr Nephrol 2001;5 : 206-9)

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.125-135
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• 2001

Purpose : Efforts to predict long-term outcome of focal segmental glomerulosclerosis(FSCS) have been made but have yielded conflicting results. Reports are rare especially in Pediatric patients. In this study, we reviewed the predictable prognostic factors in patients of FSGS Method : Fifty children who diagnosed as biopsy-proven FSGS at department of pediatrics at Yonsei university were studied retrospectively. Based on medical records, response to treatment and pathologic slides, we compared normal renal function group and decreased renal function group, assessed the factors affecting renal survival and progression to renal failure. Results : The mean age at onset was 8 1/12 years, sex ratio was 2.3 : 1, and the mean duration of follow-up was 7 1/12 years. The overall renal survival rate was $34\%$ at 5 years, $8\%$ at 10 years Five-year survival rate was $74\%$ in normal renal function group and $27\%$ in decreased renal function group. Between the two groups, there were no significant differences in age at onset, sex ratio, amount of proteinuria, incidence of hematuria and hypertension, mesangial hypercellularity. Decreased renal function group showed higher serum creatinine level, poor response to treatment, higher percent of glomeruli with sclerosis, moderate to severe tubulointerstitial change and vascular change(P<0.05). The prognostic factors of renal survival rate were same as above and incidence of hypertension also affected renal survival( P<0.05). The progression rate to renal failure did not show statistically significant factor. Conclusion : We reviewed the factors affecting long-term outcome of FSGS. Serum creatinine level, steroid responsiveness, and the degree of glomerulosclerosis were significant prognostic factors. (J Korean Soc Pediatr Nephrol 2001 ;5 : 125-35)

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.244-249
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• 2004

Obesity-associated focal segmental glomeruloscleropis(OB-FSGS) has been known to progress into advanced renal insufficiency, and its clinicopathological features Include obesity, FSGS lesions with glornerulomegaly and, nephrotic-range proteinuria without edema. A 14 year old girl with Prader-Willi syndrome showed nephrotic-range proteinuria without hypoalbuminemia or edema. The renal biopsy revealed focal segmental glomerulosclerosis together with glomerular hypertrophy and an increased mesangial matrix. We report here a case of OB-FSGS as one of the renal problems of Pradel-Willi syndrome, and we came to the conclusion that Prader-Willi syndrome is one of the Possible disease entities that can lead to renal insufficiency through obesity.

• Childhood Kidney Diseases
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• v.14 no.2
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• pp.236-239
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• 2010

Typhoid fever is a systemic infectious disease which affects many organs. In children, few cases have been reported of acute nephritic syndrome in typhoid fever. We report an immunocompetent 9-year old girl with typhoid fever complicated by acute tubulointerstitial nephritis who presented with prolonged fever and acute renal failure.

• Journal of Yeungnam Medical Science
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• v.18 no.2
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• pp.246-252
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• 2001

We report the result of a high-dose intravenous immunoglobulin therapy in a Henoch-Schnlein purpura patient with severe abdominal pain and nephrotic syndrome who did not respond to methylprednisolone pulse therapy. Kidney biopsy showed diffuse mesangial cell proliferative glomerulonephritis with fibrocellular crescent formation in approximately 50% of glomeruli. Mesangium of all glomeruli were strong positive for IgA and C3 antibodies. High-dose intravenous immunoglobulin treatment was introduced and dramatic improvement of gastrointestinal symptom and proteinuria as well as hematuria was noted. Immunoglobulin administration should be considered in Henoch-Schnlein purpura patients with steroid-resistant intractable gastrointestinal manifestation and renal involvement.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.223-228
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• 2003

Oligmeganephronia is congenital hypoplasia of kidney with renal pathology showing very small number of nephrons with compensatory hypertrophy of the remaining glomeruli. A 7-year-old girl was referred to our nephrology clinic due to hematuria detected on school screening urinalysis and diagnosed as chronic renal failure and oligomeganephronia on renal biopsy. We are reporting the clinical and histomorphometric changes for the four years follow-up with review of literatures.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.97-101
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• 2005

Renal diseases other than diabetic nephropathy can occur In diabetic patients. Urinary abnormalities or clinical courses inconsistent with the natural progression of diabetic nephropathy are suggestive of non-diabetic renal diseases and should lead to more extensive investigations. Presence of non-diabetic renal diseases in diabetic patients can alter the treatment plan and the prognosis. We report a 9-year-old girl who had type 1 membranoproliferatiye glomerulonephritis as well as type 1 diabetes mellitus.

• Journal of Periodontal and Implant Science
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• v.36 no.1
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• pp.15-26
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• 2006

흡수성 차페막을 이용한 조직 유도 재생술시 차폐막의 견고성으로 미루어 보아 재생을 위한 공간의 유지가 어려울 수 있다. 조직 유도 재생술과 함께 골이식술을 시행함으로써 공간 확보와 함께 적절한 혈병의 유지를 도모할 수 있고 이식된 골은 선생골 형성을 위한 핵으로 작용할 수도 있다. 최근에 사람의 치아회분말과 연석고를 혼합한 골이식재가 여러 연구를 통해 좋은 골이식재로 평가되었다. 본 연구에서는 성견 하악 소구치 2급 치근이개부위에 외과적으로 형성하여 흡수성 차폐막과 치아회분말-연석고 혼합 이식재를 이용한 조직유도재생 술을 시행하여 치주 조직 재생의 양상을 조직학적으로 관찰하고자 한다. 생후 12개월에서 16개윌 된 체중 15 Kg 내외의 성견 4마리를 이용하였다. 실험 재료로 생체흡수성 차폐막 (Biogide(R), Swiss) 를 사용하였고, 골이식재로 치아회분말-연석고를 혼합매식 하였다. 양측 상악 소구치 부위에 변연 치조골하방에 4 mm ${\times}$ 4 mm ${\times}$ 4 mm, (깊이 ${\times}$ 근원심 ${\times}$ 협설폭경) 깊이로 골내낭을 형성하였다. 형성된 골내낭의 기저부위 치근 표면에 1/4 round bur로 notch를 형성하여 참고점으로 하였다. 무작위로 선택된 한 쪽의 결손부를 대조군으로 오직 생체 흡수성 차폐막을 사용하였고, 실험군으로 치아회분말-연석고와 생체 흡수성 차폐막을 결손부로부터 2 mm 이상 덮을 수 있도록 다듬어 결손부 위에 위치시킨 후 협측 판막을 덮고 봉합하였다. 4주 후 2마리 ,8주 후 2마리를 희생시키고 통상의 방법으로 고정, 탈회, 포매의 과정을 거쳐 광학 현미경으로 검경하였다. 그 결과, 1. 4주 대조군에서 Bio-gide(R)는 완전한 흡수를 보였고, 치근이개부내에는 큰 공간이 존재하였다. 2. 4주 실험군에서 역시 Bio-gide(R)는 완전한 흡수를 보였고, 골 결손부내에 더 많은 신생골 관찰되었다. 그러나 아직까진 기존골과 신생골간에 명확한 차이가 있어서 쉽게 구분할 수 있었다. 또한 골이식재 주변으로 파골세포가 다수 관찰되며 이로 미루어 보아 활발한 골흡수가 일어남을 알 수 있었다. 3. 8주 대조군에서 결손부내에서는 기존골에 인접하여 신생골 형성이 부분적으로 일어났으나 연조직 침입이 관찰되었다. 4. 8주 실험군은 신생골이 기존골과 매우 유사한 형태로 관찰되었고, 신생골 형성 부위에 신생 혈관 증식이 관찰되었다. 또한 골내낭 기저부위에서는 백악질과, 치주인대가 재생됨이 관찰되었다. 이상의 결과에서 치아회분말-연석고 혼합매식은 골재생을 위한 골전도성이 있는 재료로 사료되며, 이를 이용히여 치주조직재생술시 흡수성 차폐막과 병행하여 사용한다면 더 많은 골재생이 있을 것으로 기대된다.

• Journal of Genetic Medicine
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• v.4 no.2
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• pp.200-203
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• 2007

Niemann-Pick type C is an inborn error of metabolism that affects lipid degradation and storage, which is characterized by hepatosplenomegaly and progressive neurological symptoms. A 7-month-old girl with jaundice was presented cholestasis and hepatosplenomegaly. Laboratory study showed elevated acid phosphatase, angiotensin converting enzyme and mild decrease of cholesterol. Characteristic foamy cell and sea-blue histiocytes in bone marrow biopsy consistent with Niemann-Pick disease. Niemann-Pick type C was suspected by past medical history and findings of physical examination. Therefore, molecular analysis was performed and found mutations of NPC1 gene. We report the first Korean case of type C Niemann-Pick disease confirmed by mutation analysis.