The Basic Data Analysis of Lupus Nephritis in Children

Granular cell tumor is mostly benign and thought to be of Schwann cell origin. The head and neck, particularly tongue, breast, and upper respiratory tract are frequently involved. Recently, we have experienced a case of granular cell tumor of the right thigh in a 30-year old male, diagnosed by fine needle aspiration cytology which revealed distinct cytologic features. The smear revealed cellular aspirates with clear background. The tumor cells showed uniform small nuclei and abundant eosinophilic, granular cytoplasm with hazy cell border. Mitoses were not found.

Factors Associated with Renal Failure in Children with Primary Vesicoureteral Reflux

PURPOSE: The pathophysiologic mechanisms and risk factors of renal functional deterioration are still controversial. We analyzed the factors associated with renal failure in children with primary vesicoureteral reflux. METHODS: The medical records of 166 children who were diagnosed with primary vesicoureteral reflux from Jan. 1985 to Dec. 1996 in the Department of Pediatrics, Seoul National University Children's Hospital were reviewed retrospectively. The patients were divided into two groups according to renal function: patients with renal failure(RF group) and patients with normal renal function(NRF group). RESULTS: Eighteen(11%) were classified as RF group, and 16(89%) of them were male. Sixteen (89%) of RF group revealed decreased renal function at the time of diagnosis. There was no past history of documented urinary tract infection in 16(89%) of RF group. Patients of RF group had a higher grade of reflux as compared with those of NRF group. Proteinuria and hypertension were found in 16(89%) and 6(33%) of RF group, respectively, while no NRF patients had proteinuria or hypertension. Renal functional deterioration had progressed to end-stage renal disease in 6(33%) of RF group during a mean follow-up of 4.4+/-2.7(range 0.5-12) years. CONCLUSION: Reflux nephropathy and renal scar in children can be prevented, in some cases, by early diagnosis of vesicoureteral reflux and prophylaxis of urinary tract infection. Regular check up for proteinuria and hypertension is essential for early detection of renal dysfunction. However, in other patients, especially in male, renal dysfunction can develop independently on urinary tract infection.

Percutaneous transluminal coronary angioplasty in a child with Kawasaki disease

A successful attempt at percutaneous transluminanl coronary angioplasty (PTCA) to relieve stenosis of the mid-portion of the left anterior descending artery was achieved in a 6-year 9-month old boy who had multiple coronary aneurysms and stenosis due to Kawasaki disease. Despite the progression of coronary stenosis he had been well except for the perfusion defect of the anterior wall of myocardium on 99mTc-MIBI SPECT with dipyridamole infusion until PTCA was carried out after 4-year 4-months of the onset of illness. The area of stenosis was 70% before PTCA and 20% after PTCA. No restenosis at the site of PTCA was observed on follow-up angiography at 26 months after PTCA. This successful attempt may indicate that this procedure should be considered early in subclinical stenosis to prevent ischemic cardiac damage.

A Case of Successful Treatment of Pulmonary Alveolar Hemorrhage with Plasmapheresis in Child with Systemic Lupus Erythematosus

Pulmonary hemorrhage is a rare but possibly fatal complication of systemic lupus erythematosus (SLE). We report a case of massive pulmonary hemorrhage in a 14-year-old boy recently diagnosed as SLE. He developed massive pulmonary hemorrhage during the courses of i.v. methylprednisolone pulse therapy, and did not respond to i.v. cyclophosphamide. However, he rapidly improved through the use of plasmapheresis. Although various factors can precipitate pulmonary hemorrhage in SLE, our case was probably caused by an immune mediated mechanism since the hemorrhage responded promptly to plasmapheresis. This case illustrates the importance of plasmapheresis in the treatment of pulmonary hemorrhage which is not improved by methylprednisolone and cyclophosphamide. We report this case with a brief review of the related literatures.

A Case of Successful Treatment of Pulmonary Alveolar Hemorrhage with Plasmapheresis in Child with Systemic Lupus Erythematosus

Pulmonary hemorrhage is a rare but possibly fatal complication of systemic lupus erythematosus (SLE). We report a case of massive pulmonary hemorrhage in a 14-year-old boy recently diagnosed as SLE. He developed massive pulmonary hemorrhage during the courses of i.v. methylprednisolone pulse therapy, and did not respond to i.v. cyclophosphamide. However, he rapidly improved through the use of plasmapheresis. Although various factors can precipitate pulmonary hemorrhage in SLE, our case was probably caused by an immune mediated mechanism since the hemorrhage responded promptly to plasmapheresis. This case illustrates the importance of plasmapheresis in the treatment of pulmonary hemorrhage which is not improved by methylprednisolone and cyclophosphamide. We report this case with a brief review of the related literatures.

Nutcracker Syndrome Associated with Non-glomerular Hematuria in Childhood / 대한신장학회잡지

PURPOSE: This study was designed to aid the diagnosis and to predict the outcorne by understanding the clinical course of nutcracker syndrome in childhood. METHODS: The clinical, laboratory, radiological and cystoscopic data from the medical records of eleven children who were diagnosed as nutcracker syndrome by gross hematuria and pressure gradient criteria (>3mrnHg) were studied retrospectively and analyzed. RESULTS: Sex ratio of the cases was 7:4, and the median age of onset was 12.8 (3-14.3) years. Six cases showed persistent and 5 cases manifested interrnittent, exercise induced hematuria. Left flank pain (64%), abdominal pain (18%), left varicocele (9%) were associated in some of the children, but hematuria was the only symptom in 36Yo. Left renal vein entrapment was documented in 10 cases by ultrasonography. Out of the 5 cases studied by renal Doppler ultrasonography, 4 and 5 cases showed higher (>5) mean left renal vein diameter ratio (Distal/ Aortomesenteric portion) and mean peak velocity ratio respectively. Unilateral bleeding from left ureteral orifice was documented in 7 of the 9 cases at cystoscopy. The mean pressure gradient between proximal left renal vein and inferior vena cava was 4.4+/-1.6 (3-7) mmHg. Hematuria of 25% and 57% of the cases disappeared spontaneously in 3 and 5 years after onset respectively. Proteinuria disappear- ed in 3 of the 5 initial proteinuric cases. CONCLUSION: Nutcracker syndrome must be considered in the differential diagnosis of non-glomerular, especially gross hematuria in childhood, and Doppler ultrasonography can aid diagnosis non-invasively. The renal function remained stable, but 4396 of the cases continued to show hematuria still 5 years after onset.