A Long-Term Survival Case of a Primary Malignant Intracerebral Nerve Sheath Tumor

We report a long-term survival case of a primary malignant intracerebral nerve sheath tumor (MINST) occurring in the right frontal lobe of a 13-year old boy. After the gross total resection (GTR), we have performed radiation therapy but it recurred 50 months after the surgery, so the second GTR was performed. Later, second tumor recurrence was found 4 months after the second surgery. Subsequently the third GTR, radiotherapy, and chemotherapy were carried out. At present, the patient has been remaining alive for 77 months without evidence of tumor recurrence. According to the previous reports, the primary MINST is very rare : there are only 8 cases reported. It is also a fast-growing, invasive tumor with poor outcome. This is the first case that had no recurrence for 50 months after the surgery among the reported cases that had been followed up for more than 5 years. It is supposed that a period of recurrence free survival after GTR and low mitotic activity are associated with the patient's prognosis. A GTR followed by adjuvant radiation therapy and chemotherapy will be recommended to patients of MINST.

Comparison with Subcutaneous Abdominal Preservation and Cryoconservation Using Autologous Bone Flap after Decompressive Craniectomy / 대한신경손상학회지

OBJECTIVE: After decompressive craniectomy was performed in patients with severe brain swelling, we were able to preserve autologous bone flap as freeze-preserved state or within abdominal subcutaneous tissue. The aim of this study was to compare the freeze-preservation with the subcutaneous abdominal preservation regarding the effectiveness and safety. METHODS: The clinical data of 53 patients who underwent decompressive craniectomy with autologous bone flap cranioplasty in our department were studied retrospectively. 43 patients underwent cranial reconstruction using autologous bone flap stored in deep freezer. In 10 patients cranioplasty was performed to repair bone defect using autologous bone flap preserved in subcutaneous abdomen. The analysis included the rates of infection, bone absorption and other complications and operation time to compare these two methods. RESULTS: Cranioplasty using deep-freezing bone flap showed a low infection rate (2.3%), low bone absorption (2.3%) and no cosmetic problem. The average time of operation is 146 minutes. Cranioplasty using a bone flap banked in the patient's abdominal wall revealed no case of complications. The average time of operation is 130 minutes. The longer period the bone flap was preserved for, the longer time the operation took in both methods. CONCLUSION: This study may be worth considering that both methods of cryoconservation and subcutaneous abdominal preservetion are feasible for the repair of skull defect although abdominal preservation seems to show better result a little. If the deep-freezer is not available, a bone flap banked in the patient's abdominal wall can be used.

The Experiences of Pump-driven Continuous Venovenous Hemofiltration Therapy in Pediatric Patients

We report the experiences of pump-driven continuous venovenous hemofiltration therapy in three children with acute renal failure. The all three patients required mechanical ventilation and needed the support of vasopressors. Renal replacement therapy was needed to meet the metabolic and fluid balance, but intermittent hemodialysis and peritoneal dialysis were not feasible because of hemodynamic instability and concurrent infection. We instituted pump-driven continuous venovenous hemofiltration(CVVH), and immediate improvement of pulmonary edema and successful removal of retained fluid were observed. Urea clearance also was satisfactory. During the filter running time, significant thromboembolic event or rapid drop of systemic blood pressure were absent. We concluded that the CVVH is an effective and safe method of renal support for critically ill pediatric patient.

Two cases of Hemolytic Uremic Syndrome Associated with Escherichia coli O114

We report two cases of hemolytic uremic syndrome (HUS) associated with Escherichia coli O114. Two cases were similar and showed the same clinical courses. After prodrome of diarrhea and vomiting lasting 1-2 days, azotemia persisted for about 10 days, and during that period, the patients were on peritoneal dialysis. They recovered without any sequelae after about 15 days. Direct multiplex PCR of stool culture revealed eae and stx2 gene and the result of ELISA done on the colony positive of eae gene confirmed Escherichia coli O114. This is the first report of HUS associated with Escherichia coli O114. We recommend, Shiga toxin producing bacterial infection must be considered and efforts should be made to scrutinize the organism in all diarrhea-prodrome HUS patients.

Analysis of Isolated Proteinuria on School Urinary Mass Screening

PURPOSE: The urinary mass screening program in school aged population has been performed since 1981, but the consensus on the follow-up schedule and the management of isolated proteinuria has not been reached yet. The aim of this study was to investigate the cause of isolated proteinuria and to propose a guideline for the treatment and follow-up afterwards. Methods: The medical records of 114 cases of isolated proteinuria detected through the analysis of urinary mass screening and evaluated at the pediatric outpatient clinic of Asan Medical Center from January 1990 to July 2001 have been reviewed. RESULTS: The classification of isolated proteinuria was as follows. Transient proteinuria 32%, orthostatic proteinuria 65%, persistent proteinuria 3%. In orthostatic proteinuria group, daytime and nighttime proteinuria were 319.2+/-89.1 mg/dL and 56.5+/-6.1 mg/dL. In persistent proteinuria group, daytime and nighttime proteinuria were 1140+/-40.5 mg/dL and 289+/-8 mg/dL. After 30 month follow-up, 2 cases of persistent proteinuria were needed renal biopsy and 1 case revealed focal segmental glomerular sclerosis. In all cases, serum creatinine, albumin and complements levels were normal. In the orthostatic proteinuria group, no significant renal diseases were detected. CONCLUSION: Since most of the isolated proteinuria detected through the school urinary mass screening were orthostatic proteinuria or transient proteinuria, initially aggressive diagnostic method such as renal biopsy is not needed and regular follow-up with quantitation of proteinuria is warranted.