A Case of Renovascular Hypertension Due to Renal Artey Stenosis Related to Moyamoya Disease

Moyamoya disease is a progressive cerebrovascular disorder with stenosis or occlusion of the bilateral internal carotid arteries with abnormal vascular networks at the base of the brain. Previous reports have shown that there are extracranial vascular involvements in Moyamoya disease, especially in the renal artery. We report a 7-year-old patient with Moyamoya disease associated with renovascular hypertension, who presented in infancy with seizures and hemiparesis. Renal angiography showed multiple stenoses of the right renal artery. Although renal artery stenosis in Moyamoya disease has been effectively treated with balloon angioplasty, stent implantation, or surgery, balloon angioplasty could not be done in this patient due to multiple stenoses. His blood pressure was successfully controlled with medical treatment, and remained normotensive during the follow up period of 6 months.

The Clinical Usefulness of Cystatin C in Evaluating Renal Function in Children with Various Renal Diseases

PURPOSE: GFR(glomerular filtration rate) is a fundamental parameter in detecting renal impairment and predicts the progression of renal disease. Because serum creatinine has several disadvantages, serum cystatin C has been recently proposed as a new endogenous marker for GFR. We compared serum cystatin C with creatinine and creatinine clearance to investigate the clinical usefulness of cystatin C. METHODS: We retrospectively analyzed 46 patients(60 case numbers) who had various renal diseases and classified them into 3 groups according to creatinine clearance(Group 1 : CrCl 60 mL/min/1.73 m2). We measured serum creatinine, cystatin C and creatinine clearance and also analyzed the correlations among them. RESULTS: Serum cystatin C and creatinine showed a similar correlation to creatinine clearance (r=0.685, r=0.640, respectively) and showed similar diagnostic accuracy in detecting decreased GFR(AUC, cystatin C 0.829 vs. creatinine 0.826, P=0.848). Serum cystatin C showed a greater sensitivity for detecting a decreased GFR than creatinine in Group 2 and 3(Group 1 : 100% vs. 100%, Group 2 : 70% vs. 35%, Group 3 : 46% vs. 15%). CONCLUSIONS: Serum cystatin C could be a useful endogenous marker for GFR and would be superior to serum creatinine in early detection of renal impairment in pediatric patients with renal diseases.

The Prognosis of Focal Segmental Glomerulosclerosis Patients with Methylprednisolone Pulse Therapy Alone

PURPOSE: Since the first report by Mendoza in 1990, there have been several studies reporting that long-term intravenous methylprednisolone(MP) pulse therapy combined with cyclosporin A(CsA) or cyclophosphamide might be beneficial for the treatment of steroid resistant focal segmental glomerulosclerosis(FSGS). We investigated the therapeutic effect of long-term MP pulse therapy without CsA or cyclophosphamide on steroid resistant FSGS. METHODS: The medical records of the 10 steroid resistant FSGS patients who were treated with MP pulse therapy by the Mendoza protocol without CsA or cyclophosphamide in our hospital were retrospectively reviewed. RESULTS: The median age at onset was 2.6 years(range 1.1-10.6 years) and the median age at the initiation of therapy was 5.7 years(range 1.8-20 years). The median duration of follow-up was 35 months(range 4-132 months). At the end of therapy, 5 patients achieved complete remission(50%) and 2 partial remission(20%), one of whom relapsed after the therapy. Three patients did not respond to the therapy, two of whom progressed to end-stage renal failure during the therapy eventually requiring kidney transplantation. CONCLUSION: Intravenous long-term MP pulse therapy without CsA or cyclophosphamide by the Mendoza protocol may be effective in a subset of patients with steroid-resistant FSGS.

Outcome and Prognosis in Critically Ill Children Receiving Continuous Renal Replacement Therapy

PURPOSE: Continuous renal replacement therapy(CRRT) has been the first choice for the treatment of acute renal failure in critically ill children not only in western countries but also in Korea. However, there are very few studies that have analyzed the outcome and prognosis of this modality in Korean children. We performed this study to evaluate the factors associated with the outcome and prognosis of patients treated with CRRT. METHODS: We retrospectively reviewed the medical records of 32 children who had received CRRT at Severance hospital from 2003 to 2006. The mean age was 7.5 years(range 4 days- 16 years) and the mean body weight was 25.8 kg (range 3.2-63 kg). RESULTS: Eleven(34.4%) of the 32 patients survived. Bone marrow transplantation and malignancy were the most common causes of death and underlying disease leading to the need for CRRT. Mean patient weight, age, duration of CRRT, number of organ failures, urine output, estimated glomerular filtration rate(eGFR), C-reactive protein, and blood urea level did not differ significantly between survivors and nonsurvivors. (1) Pediatric risk of mortality(PRISM) III score at CRRT initiation(9.8+/-5.3 vs. 26.7+/-7.6, P<0.0001), (2) maximum pressor number (2.1+/-1.2 vs. 3.0+/-1.0, P=0.038), and (3) the degree of fluid overload(5.2+/-6.0 vs. 15.0+/-8.9, P=0.002) were significantly lower in survivors than in nonsurvivors. Multivariate analysis revealed that fluid overload was the only independent factor reducing survival rate. CONCLUSION: CRRT was successfully applied to the treatment of acute renal failure in a wide range of critically ill children. To improve survival, we suggest the early initiation of CRRT to prevent the systemic worsening and progression of fluid overload in critically ill children with acute renal failure.