Plasminogen Activator Inhibitor Type 1 Gene Polymorphism in Patients with Minimal Change Nephrotic Syndrome

PURPOSE: Hypercoagulability is present in patients with nephrotic syndrome. Plasminogen activator inhibitor type 1(PAI-1) is a major inhibitor of plasminogen activators. PAI-1 inactivates both tissue plasminogen activator(tPA) and urokinase plasminogen activator(uPA) by rapid formation of inactive 1:1 stoichiometric complexes. Recently some studies showed that the enhanced PAI-1 expression may be involved in the intraglomerular fibrinogen/fibrin- related antigen deposition seen in nephrotic syndrome. METHODS: PAI-1 gene promoter -844(G/A) polymorphism was evaluated in 146 children with minimal change nephrotic syndrome(MCNS) and 230 control subjects. The patients with MCNS were subdivided into 85 infrequent-relapser(IR) group and 61 frequent relapser(FR) group. PCR of PAI-1 gene promoter region including -844(G/A) and RFLP using the restriction enzyme Xho1 were performed for each DNA samples extracted from the groups. RESULTS: The distribution of PAI-1 genotype in the control group was G/G 81(32.5%), A/A 42(16.9%), and G/A 126(50.6%). The distribution of PAI-1 genotypes in the IR group of MCNS was G/G 29(34.1%), A/A 15(17.7%), and G/A 41(48.2%). The distribution of PAI-1 genotype in the FR group of MCNS was G/G 17(27.9%), A/A 18(29.5%), and G/A 26(42.6 %). There was a significantly increased frequency of A/A genotype(P=0.0251) in the FR group of MCNS. CONCLUSION: Our results indicate that the PAI-1 gene promoter A/A genotype may be associated with the FR in MCNS.

Efficacy of Pamidronate in Nephropathic Children with Ongoing Long Term Corticosteroid Therapy

BACKGROUND: Steroid-induced osteoporosis(SIO) is one of the serious complications of long- term steroid therapy, especially in growing children. Recently bisphosphonates have been used to treat or prevent SIO in adult, which is rare in children with glomerular diseases. We studied the effect of pamidronate on SIO using dual energy X-ray absorptiometry and biochemical markers of bone turnover. METHODS: Forty four children receiving moderate-to-high doses of steroids were enrolled. They had no history of bone, liver, or endocrine disease. Patients were stratified by their baseline bone mineral density(BMD) findings. All patients received corticosteroids for 3 month and oral calcium supplementation(500 mg/day) daily. Among them, 28 patients were treated with placebo and 16 were treated with pamidronate(125 mg) for 3 months. Blood chemistry and bone mineral density(BMD) were measured at baseline, and 3months. In addition, parathyroid hormone(PTH), serum osteocalcin, and urinary dipyridinoline levels were evaluated. RESULTS:In overall population, the mean lumbar spine BMD decreased from 0.754+/-0.211 (g/cm2) to 0.728+/-0.208(g/cm2) in the placebo group(P0.05). CONCLUSION:Pamidronate appears to be effective in preventing SIO in children with glomerular diseases requiring long-term steroids therapy. Further careful observation and follow-up might be needed for children receiving bisphosphonates such as pamidronate.

Comparison of Clinical Courses According to the Existence of Patent Ductus Arteriosus in Respiratory Distress Syndrome

PURPOSE: To compare perinatal characteristics, clinical courses, and overall morbidity between respiratory distress syndrome(RDS) with patent ductus arteriosus(PDA) and RDS without PDA in neonates. METHODS: Eighty-three neonates who were diagnosed and treated for RDS in the neonatal intensive care unit(NICU) from Jan. 2000 to Dec. 2002 were included in this study. RDS was complicated with PDA(group A) in 17 patients and not complicated in 66(group B). PDA was diagnosed by echocardiogram in neonates with congestive heart failure symptom, cardiac murmur or chest X-ray findings of cardiomegaly or pulmonary edema. A retrospective study was undertaken of the perinatal charac teristics and overall morbidity in group A and group B. RESULTS: The birth weight and gestational periods of group A were less compared with group B. There was more perinatal asphyxia in group A. Incidence of overall morbidity such as bronchopul monary dysplasia, intraventricular hemorrhage and death was higher in group A. Intravenous indomethacin was administered in 17 PDA infants. CONCLUSION: The perinatal characteristics in the two groups showed a significant difference. Incidence of overall morbidity in the two groups showed significant differences, however, there is no simple conclusion to draw because we didn't do multifactorial analyses to rule out other many risk factors affecting morbidity, such as gestational weeks or birth weight.

A Case of Rhodotorula Mucilaginosa Peritonitis undergoing Continuous Ambulatory Peritoneal Dialysis in a Neonate with Acute Renal Failure

Fungal peritonitis is a serious complication of continuous ambulatory peritoneal dialysis (CAPD). It has been reported with increasing frequency and has been estimated that 1- 10% of peritonitis associated with CAPD is reported to be caused by the fungus. Most cases of fungal peritonitis involve candida species, however, other oppotunistic pathogens including encapsulated yeast named Rhodotorula species have been reported. Reports of infection due to Rhodotorula species, rarely being a pathogenic organism. include septicemia, endocarditis, meningitis, ventriculitis and peritonitis. We report a case of serious fungal peritonitis caused by Rhodotorula mucilaginosa in a neonate with history of neonatal asphyxia undergoing CAPD.

The Significance of Perioperative Serum Carcinoembryonic Antigen Measurement in Patients with Curative Colorectal Cancer

PURPOSE: Regular monitoring of serum carcinoembryonic antigen (CEA) has been used as a tool to detect recurrence of colorectal cancer postoperatively. This study aimed to evaluate the significance of perioperative serum CEA level in patients with curative colorectal cancer. METHODS: We analyzed the data obtained from the 420 patients with colorectal cancer who underwent curative resection. Preoperative serum CEA level (ng/ml) was divided into 3 groups, i.e. groups I: or=20. Each group of preoperative serum CEA level was analyzed in accordance with location, histologic differentiation, stage of tumor, recurrence and survival. Postoperative serum CEA level was analyzed in accordance with preoperative serum CEA level and recurrence. RESULTS: Preoperative serum CEA level correlated with tumor stage (P=0.009). Ninety six patients among 420 patients showed recurred and recurrences were more common in patients with high preoperative serum CEA level (P =0.002). Systemic recurrences were more common in patients with high preoperative serum CEA levels than normal levels (P=0.029). In recurrence cases, 75 patients (78.1%) had elevated serum CEA levels and 55 patients had high preoperative serum CEA levels (P=0.008). The disease free 5-year survival rate in preoperative serum CEA group I, II, and III were 91.4%, 70.5%, and 58.3% respectively (P= 0.000) CONCLUSION: Preoperative serum CEA levels seemed to be closely correlated with distant metastasis and survival. Meticulous follow-up evaluation and generous use of adjuvant therapy are recommanded in patients with high preoperative CEA level.

Clinical Characteristics of Inguinal Hernia Following Rectal Cancer Surgery

PURPOSE: The anatomic and physiologic changes after rectal cancer surgery was suggested to be a cause of inguinal hernia. But, there are only few reported cases about the inguinal hernia following rectal cancer surgery. The aim of this study is to verify risk factors and surgical techniques to prevent inguinal hernia following rectal cancer surgery. METHODS: Out of 1226 patients who underwent operations at the Department of Surgery, Asan Medical Center, between the period from June 1989 to July 1999, 12 patients who had developed inguinal hernia and their medical records were reviewed retrospectively regarding the clinical characteristics. RESULTS: The median duration of hernia following rectal cancer surgery was 12 (3-36) months. The median age was 63 (36-74) years with eight of them more than seventh decades of their life. All patients had male sex. Three of them were overweighted. Five cases had hernias on the left side, six on the right, and one case on both side. All patients had indirect inguinal hernia. Three cases had preoperative symptoms of bowel obstruction. The operations for rectal cancer were low anterior resection in eight cases, ultralow anterior resection in three cases, and abdominoperineal resection in one case. The complications in rectal cancer surgery were found in six cases. Six cases had the advanced stage. A postoperative radiation therapy was applied in five cases. Five cases had the coexisting diseases. CONCLUSIONS: Male sex and old age would be related with occuring inguinal hernia after rectal cancer surgery and, it would be necessary to give a special attention to the patients who have these factors, although the present study could not show the definite correlation between inguinal hernia and rectal cancer surgery due to a small number of cases.