• Childhood Kidney Diseases
• /
• v.21 no.2
• /
• pp.41-46
• /
• 2017

Purpose: Thin glomerular basement membrane nephropathy (TBMN) is, along with the IgA nephropathy, the most common cause of asymptomatic hematuria in Korean children. TBMN is usually a benign renal disease not requiring treatment and is associated with a good prognosis, but some cases hematuria is indicative of a state of progressive renal insufficiency. We aimed to retrospectively evaluate clinical manifestations and renal prognosis of patients with TBMN. Methods: Among the 428 renal biopsies performed on children at Yeungnam University Hospital between January 2000 and February 2017, 167 patients were diagnosed as having TBMN. We retrospectively investigated 167 pediatric patients and identified 59 children with follow-up duration >3 years. Results: Among 59 patients, there were 33 boys and 26 girls. Mean age of onset of hematuria was $7.18{\pm}2.64$ years, and mean time from onset of disease until a renal biopsy was performed was $2.48{\pm}2.10$ years. There were no clinical features or laboratory findings among studied children to indicate decreased renal function during follow-up; however, one case progressed to chronic kidney disease (CKD) due to an unknown cause. There were seven patients among these related a positive family history of hematuria or renal insufficiency. Concluson: Although almost all patients had normal renal functions during follow-up, there were one patient who progressed to CKD and seven patients with family history of hematuria or renal insufficiency. Moreover, four among the 428 patients over 17 years underwent repeat renal biopsies, which showed results different from their earlier biopsies.Thus, large-scales studies may be required to determine long-term prognosis of TBMN in children, and further evaluation for Alport syndrome in TBMN cases is essential.

• Pediatric Infection and Vaccine
• /
• v.20 no.1
• /
• pp.41-45
• /
• 2013

Nontyphoidal Salmonella are gram negative bacilli organism, which may induce systemic infection such as febrile enteritis, bacteremia, and osteomyelitis. Main route of infection is known as food but also possible through reptile, amphibian, and fish raised as pets in the house. There is no known cases report of Salmonella infection through pets in Korea and also rare in the overseas. We report 2 patients who visited Severance children's hospital with chief complaint of fever and diagnosed as nontyphoidal salmonellosis. Each case had a history of raising turtle or tropical fish with possibility of Salmonella infection through these pets. Increasing incidence of raising pet reptile and fish lately, contact precaution and proper prevention and control of Salmonella infection of these pets especially in children under 5 years old are necessary due to higher risk of serious complications of salmonellosis.

• Clinical and Experimental Pediatrics
• /
• v.57 no.3
• /
• pp.135-139
• /
• 2014

Purpose: Adult Korean patients on chronic dialysis have a 9-year survival rate of 50%, with cardiovascular problems being the most significant cause of death. The 2011 annual report of the North American Pediatric Renal Trials and Collaborative Studies group reported 3-year survival rates of 93.4% and relatively poorer survival in younger patients. Methods: In this study, we have reviewed data from Korean Pediatric Chronic Kidney Disease Registry from 2002 to 2010 to assess survival rates and causes of death in Korean children on chronic dialysis. Results: The overall estimated patient survival rates were 98.4%, 94.4%, and 92.1% at 1, 3, and 5 years, respectively. No significant difference was observed in survival rates between patients on peritoneal dialysis and those on hemodialysis. Patients for whom dialysis was initiated before 2 years of age (n=40) had significantly lower survival rates than those for whom dialysis was initiated at 6-11 years of age (n=140). In all, 26 patients had died; the mortality rate was 19.9 per 1,000 patient years. The most common causes of death were infections and comorbidities such as malignancy and central nervous system (CNS) or liver diseases. Conclusion: The outcomes observed in this study were better than those observed in adults and comparable to those observed in pediatric studies in other countries. To improve the outcomes of children on chronic dialysis, it is necessary to prevent dialysis-related complications such as infection, congestive heart failure, or CNS hemorrhage and best control treatable comorbidities.

• Clinical and Experimental Pediatrics
• /
• v.58 no.7
• /
• pp.251-255
• /
• 2015

Purpose: The widespread introduction of bacterial conjugate vaccines has decreased the risk of cerebrospinal fluid (CSF) pleocytosis due to bacterial meningitis (BM) in children. However, most patients with CSF pleocytosis are hospitalized and treated with parenteral antibiotics for several days. The bacterial meningitis score (BMS) is a validated multivariate model derived from a pediatric population in the postconjugate vaccine era and has been evaluated in several studies. In the present study, we examined the usefulness of BMS in South Korean patients. Methods: This study included 1,063 patients with CSF pleocytosis aged between 2 months and 18 years. The BMS was calculated for all patients, and the sensitivity and negative predictive value (NPV) of the test were evaluated. Results: Of 1,063 patients, 1,059 (99.6%) had aseptic meningitis (AM). Only four patients (0.4%) had BM. The majority of patients (98%) had a BMS of ${\leq}1$, indicating a diagnosis of AM. The BMS was 0 in 635 patients (60%) and 1 in 405 patients (38%). All four BM patients had a BMS of ${\geq}4$. Conclusion: To our knowledge, this is the first study to investigate the diagnostic strength of the BMS in South Korea. In our study, the BMS showed 100% sensitivity and 100% NPV. Therefore, we believe that the BMS is a good clinical prediction rule to identify children with CSF pleocytosis who are at a risk of BM.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.24 no.6
• /
• pp.546-554
• /
• 2021

Purpose: Malnutrition is a significant issue for pediatric patients with cancer. We sought to evaluate the effectiveness and complication rate of percutaneous endoscopic gastrostomy (PEG) placement in pediatric oncology patients. Methods: A retrospective chart review was performed on 49 pediatric oncology patients undergoing PEG placement at Johns Hopkins All Children's Hospital between 2000 and 2016. Demographic and clinical characteristics, complications, absolute neutrophil count at time of PEG placement and at time of complications, length of stay, and mortality were identified. Weight-for-age Z-scores were evaluated at time of- and six months post-PEG placement. Results: The overall mean weight-for-age Z-score improved by 0.73 (p<0.0001) from pre- (-1.11) to post- (-0.38) PEG placement. Improvement in Z-score was seen in patients who were malnourished at time of PEG placement (1.14, p<0.0001), but not in those who were not malnourished (0.32, p=0.197). Site infections were seen in 12 (24%), buried bumper syndrome in five (10%), and tube dislodgement in one (2%) patient. One patient (2%) with fever was treated for possible peritonitis. There were no cases of other major complications, including gastric perforation, gastrocolic fistula, clinically significant bleeding, or PEG-related death documented. Conclusion: Consistent with previous studies, our data suggests a relationship between site complications (superficial wound infection, buried bumper syndrome) and neutropenia. Additionally, PEG placement appears to be an effective modality for improving nutritional status in malnourished pediatric oncology patients. However, larger prospective studies with appropriate controls and adjustment for potential confounders are warranted to confirm these findings.

• Childhood Kidney Diseases
• /
• v.21 no.2
• /
• pp.75-80
• /
• 2017

Purpose: To investigate the frequency, presentation, management, and outcome of cytomegalovirus (CMV) infection in pediatric patients who underwent renal transplantation. Methods: We performed a retrospective chart review of 70 patients under the age of 18, who underwent renal transplantation between January 1990 and November 2014. A diagnosis of CMV infection was based on serology, molecular assays, antigenemia assays, and culture. CMV infection was defined as detection of virus and CMV disease was diagnosed when clinical signs and symptoms were present. Results: The number of patients with CMV infection was 18 (25.7% of renal transplant recipients). Twelve were male (66.7%), and the $mean{\pm}standard$ deviation (SD) age at infection was $13.3{\pm}3.9$ years. Median time of infection after renal transplantation was 4 months (range 1.0-31.0 months). Pretransplantation CMV status in the infected group was as follows: donor (D)+/recipient (R)+, 11 (61.1%); D+/R-, 7 (38.9%); D-/R+, 0; and D-/R- 0. Nine patients had CMV disease with fever, leukopenia, thrombocytopenia, or organ involvement such as enteritis, hepatitis, and pneumonitis. The age of disease occurrence was $13.1{\pm}3.9$ years and the median time to disease onset after renal transplantation was 8 months (range 1.0-31.0). Immunosuppressive agents were reduced or discontinued in 14 patients (77.8%), antiviral agents were used in 11 patients (61.1%), and all patients with CMV infection were controlled. Conclusions: A quarter of the patients had CMV infection about 4 months after renal transplantation. CMV infection was successfully treated with reduction of immunosuppressants or with antiviral agents.

• Clinical and Experimental Pediatrics
• /
• v.58 no.4
• /
• pp.154-157
• /
• 2015

Plastibell is one of the three most common devices used for neonatal circumcision in the United States, with a complication rate as low as 1.8%. The Plastibell circumcision device is commonly used under local anesthesia for religious circumcision in male neonates, because of cosmetic reasons and ease of use. Occasionally, instead of falling off, the device may get buried under the skin along the shaft of the penis, thereby obstructing the normal flow of urine. Furthermore, the foreskin of neonates is highly vascularized, and hence, hemorrhage and infection are possible when the skin is cut. Necrosis of penile skin, followed by urethral obstruction and renal failure, is a serious surgical mishap requiring immediate corrective surgery and medical attention. We report a case of fulminant urosepsis, acute renal failure, and pyelonephritis in a 4-day-old male neonate secondary to impaction of a Plastibell circumcision device. Immediate medical management was initiated with fluid resuscitation and mechanical ventilation; thereby correcting life threatening complications. Pediatricians and Emergency Department physicians should be cognizant of the complications from Plastibell circumcision device in order to institute appropriate and timely management in neonates.

• Clinical and Experimental Reproductive Medicine
• /
• v.41 no.2
• /
• pp.75-79
• /
• 2014

Objective: To study the effect of body composition on reproduction in women with unexplained infertility treated with a controlled ovarian hyperstimulation and intrauterine insemination programme. Methods: This prospective observational study was conducted on 308 unexplained infertile women who were scheduled for a controlled ovarian hyperstimulation and intrauterine insemination programme and were grouped as pregnant and non-pregnant. Anthropometric measurements were performed using TANITA-420MA before the treatment cycle. Body composition was determined using a bioelectrical impedance analysis system. Results: Body fat mass was significantly lower in pregnant women than in non-pregnant women ($15.61{\pm}3.65$ vs. $18.78{\pm}5.97$, respectively) (p=0.01). In a multiple regression analysis, body fat mass proved to have a stronger association with fecundity than the percentage of body fat, body mass index, or the waist/hip ratio (standardized regression coefficient${\geq}0.277$, t-value ${\geq}2.537$; p<0.05). The cut-off value of fat mass, which was evaluated using the receiver operating characteristics curve, was 16.65 with a sensitivity of 61.8% and a specificity of 70.2%. Below this cut-off value, the odds of the pregnancy occurrence was found to be 2.5 times more likely. Conclusion: Body fat mass can be predictive for pregnancy in patients with unexplained infertility scheduled for a controlled ovarian hyperstimulation and intrauterine insemination programme.

• Advances in pediatric surgery
• /
• v.11 no.1
• /
• pp.19-26
• /
• 2005

Choledochal cysts have been associated with complications such as cholangitis, pancreatitis, and malignancy of the biliary tract. Recently, the incidence of choledochal cyst in neonate and young infant is increasing due to advances in diagnostic imaging. The aim of this study is to investigate the rationale of excision of choledochal cyst during the neonatal period. The clinical outcome and correlation between age at surgery and the degree of liver fibrosis were reviewed retrospectively. A total of 198 patients with choledochal cyst who were managed surgically between January 1985 and December 2000 at the Department of Surgery, Seoul National University Children's Hospital were included in this study. The overall outcome and the outcome of patients who were managed surgically during the neonatal period were compared. Correlation between age and the degree of liver fibrosis was evaluated by chi-square test and Pearson exact test. The mean age of the patients was 2 years 7 months (ranged from 5 days to 15 years). Mean postoperative follow-up period was 7 years 1 month (7 months to 20 years). The results are as follows. Twelve patients (6%) had postoperative complications, cholangitis (7), bleeding (4) and ileus (1). Eleven patients operated during the neonatal period had no postoperative complications. The positive correlation between age group and degree of liver fibrosis was statistically significant (chi-square: p=0.0165, Pearson exact test: p=0.019). The results support the rationale that excision of choledochal cyst can be performed safely without increasing morbidity in neonates.

• Advances in pediatric surgery
• /
• v.19 no.2
• /
• pp.122-129
• /
• 2013

Immature ganglion cell (IGC) is known for its relationship with intestinal motility and its impact on postoperative functional outcomes of Hirschsprung's disease (HD). There are few studies on the relationship between intestinal dysmotility and IGC in HD patients. 67 patients pathologically diagnosed with HD and who received definitive operation in Seoul National University Children's Hospital from 2010 to 2011 were included. 10 patients were excluded due to inadequate immunohistochemical staining results. The proximal end of resected ganglionic segment was evaluated with immunohistochemistry examination with MAP-2, a marker of ganglionic cells and bcl-2, a marker of IGCs The median age at operation was 155 (15-4678) day-old. 55 (96.5%) patients positive for bcl-2, were regarded as having IGC, and 2 (3.5%) patients positive for MAP-2 but negative for bcl-2, were regarded as having only mature ganglion cells. In the bcl-2 positive group, there were 7 patients (12.7%) with constipation, 15 patients (27.3%) with soiling, 3 patients (5.5%) with perianal excoriation and 6 patients (10.9%) with medication use. In bcl-2 negative group, intestinal dysmotility was not seen. There was no statistical significance in the two groups. Considering that HD is diagnosed at a young age, the rate of IGC present is very high and it might be inappropriate to relate IGC to functional outcome at young ages.