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.

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.

Eosinophilic Peritonitis ( EP ) Complicated with Continuous Ambulatory Peritoneal Dialysis ( CAPD )

A 70-year-old female who was diagnosed as myxoid chondrosarcoma by fine needle aspiration of a pleural mass is described. She presented with left chest discomfort of 4 months' duration and aggravating dyspnea and chest pain for 2 months. Chest X-ray and CT scan revealed a large lobulated low density mass invading chest wall at the left pleural cavity and massive pleural fluid. Fine needle aspiration was done under the impression of mesothelioma or metastatic cancer. The aspirates from the mass were very cellular and composed of isolated or clustered forms of large plump cells. Abundant cytoplasm was bluish opaque and the margin was rounded in the isolated cells, whereas clustered cells show ill-defined cell borders and aggregating tendency. The nuclei were eccentric, round to ovoid, and had fine chromatin pattern and multiple small nucleoli. Cellular pleomorphism or mitotic figure was not definite. These findings were consistent with cytologic features of chondrosarcoma. Final diagnosis was confirmed as myxoid chondrosarcoma by mediastinoscopic biopsy and the tumor showed strong positivity for S-100 protein.

Two Cases of Neonaal Renal Venous Thrombosis

"To evaluate whether different qualifications of a cytologic diagnosis of atypical squamous cells of undetermined significance (ASCUS) predict a greater or lesser likelihood of tissue diagnosis of uterine cervix, we compared different cytologic qualifications of ASCUS with the tissue diagnosis. One hundred twenty-two con- secutive Papanicolaou smears showing ASCUS in women who had undergone cervical biopsy within nearest 30 days were collected. The 122 smears were qualified as ""favor reactive (25%), favor low grade squamous intraepithelial lesion (LSIL) (24%), favor squamous intraepithelial lesion (SIL) (16%), favor high grade squa- mous intraepithelial lesion (HSIL) (16%), and not otherwise specified (19%). Squamous intraepithelial or invasive lesion was pathologically confirmed by cervical biopsy in 13% of the favor reactive, 27% in favor LSIL, 70% in ""favor SIL, 75% in favor HSIL, and 35% in not otherwise specified smears. There were significant associations between the favor reactive smear and the benign biopsy finding and between the favor SIL smear and the biopsy showing a squamous intraepithelial or more severe lesion. Nevertheless, rnost of favor LSIL smears exhibit reactive process in tissue biopsy. Conclusively, qualified ASCUS stratifies women into different risk groups for SIL. The cytopathologist should make the cytologic diagnosis of ASCUS, favor LSIL circumspectly."