• Kidney Research and Clinical Practice
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• v.36 no.3
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• pp.257-263
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• 2017

Background Rituximab (RTX) can be used as a rescue therapy for steroid-dependent nephrotic syndrome (SDNS). However, the efficacy and safety of long-term, repeated use of RTX are not established. This study was conducted to assess the efficacy and safety of long-term, repeated RTX treatment in children. Methods Eighteen consecutive child patients with SDNS who were treated with three or more cycles of RTX for one year or longer were recruited, and their medical records were retrospectively reviewed. Results The patients were followed for $4.7{\pm}1.9years$ and received $5.2{\pm}2.3cycles$ of RTX over $2.8{\pm}1.1years$. Approximately 70% of the additional RTX cycles were administered due to recovery of B-cells without relapse. The relapse rate decreased from $3.4{\pm}2.0per$ year initially to $0.4{\pm}0.8per$ year at the third year after RTX treatment. Approximately 10% of the RTX infusions were accompanied by mild infusion reactions. Eight patients showed sustained remission without any oral medication after the last cycle of RTX, while 10 patients had one or more episodes of relapse after the last cycle of RTX. The relapse rate in the latter group decreased from $2.8{\pm}1.5per$ year before RTX treatment to $1.3{\pm}0.8per$ year after cessation of RTX treatment. No significant differences in clinical parameters were found between the two groups. Conclusion This retrospective study showed that pre-emptive and long-term, repeated RTX treatment is relatively effective and safe in children with SDNS. However, well-designed prospective studies are needed to confirm these findings.

• The Journal of Pediatrics of Korean Medicine
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• v.37 no.1
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• pp.45-57
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• 2023

Objectives This study aimed to provide a basis for the research on the trend and safety of Korean herbal medicines among children and adolescents. Methods Patients aged <19 years who received outpatient treatment at the Korean pediatrics department of Kyung Hee University Hospital from January 1, 2017 to October 31, 2022 were included. The current state of annual visits and herbal medicine prescriptions was analyzed. A retrospective chart review was conducted by examining the medical records of subjects who received herbal medicines, including deer antler. Adverse drug reactions in patients who received herbal medicine were also investigated. Results A total of 57,457 people visited the Korean pediatrics department, and 51,768 people (90.1%) were prescribed herbal medicine. Among these, 21,826 patients (42.2%) were prescribed with deer antler. A total of 117 adverse drug reactions were reported among the patients who received herbal medicines. Conclusions This study aimed to determine the trends and safety of herbal medicine treatment, particularly deer antlers, in children and adolescents. Well-designed, long-term, multi-institutional follow-up studies are required to expand the field of Korean pediatrics.

• Clinical and Experimental Pediatrics
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• v.58 no.4
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• pp.136-141
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• 2015

Purpose: Continuous intravenous access is imperative in emergency situations. Ultrasound-guided internal jugular vein (IJV) catheterization was investigated in critically ill pediatric patients to assess the feasibility of the procedure. Methods: Patients admitted to the pediatric intensive care unit between February 2011 and September 2012 were enrolled in this study. All patients received a central venous catheter from attending house staff under ultrasound guidance. Outcome measures included successful insertion of the catheter, cannulation time, number of cannulation attempts, and number and type of resulting complications. Results: Forty-one central venous catheters (93.2%) were successfully inserted into 44 patients (21 males and 23 females; mean age, $6.54{\pm}1.06$ years). Thirty-three patients (75.0%) had neurological disorders. The right IJV was used for catheter insertion in 34 cases (82.9%). The mean number of cannulation attempts and the mean cannulation time was $1.57{\pm}0.34$ and $14.07{\pm}1.91$ minutes, respectively, the mean catheter dwell time was $14.73{\pm}2.5$ days. Accidental catheter removal was observed in 9 patients (22.0%). Six patients (13.6%) reported complications, the most serious being catheter-related sepsis, which affected 1 patient (2.3%). Other complications included 2 reported cases of catheter malposition (4.6%), and 1 case each of arterial puncture (2.3%), pneumothorax (2.3%), and skin infection (2.3%). Conclusion: The results suggest that ultrasound-guided IJV catheterization can be performed easily and without any serious complications in pediatric patients, even when performed by visiting house staff. Therefore, ultrasound-guided IJV catheterization is strongly recommended for critically ill pediatric patients.

• The Journal of Pediatrics of Korean Medicine
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• v.14 no.1
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• pp.117-126
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• 2000

Background: In recent years, pediatric chronic sinusitis patients who didn't respond with antibiotics were increasing, but there were a few reports on recurrence rate of the herbal medicine therapy treating pediatric chronic sinusitis, so this report was studied. Objective: To know the recurrence rate of herbal medicine therapy in the chronic sinusitis using follow-up investigation. Materials and methods: Thirty four patients( 16 male and 18 female) treated in our hospital between December 1998 and August 1999 were studied. Ages ranged from 4 to 13 years (mean age :7.7 years). Twenty five patients(66%) had a underlying family history (allergy or sinusitis of family). In the past history, 32 patients (86%) had asthma, allergy of milk, atopic dermatitis, bronchiolitis and irritable bowel syndrome. Duration of treatment were from eleven to eighteen month (mean:14.3 month). Results: Of the 34 patients, 31 patients were improvement, 3 patients not. Of the 31 patients, thirty patients showed not recurrent (91%). Two patients showed no significant interval change(6%) and one patient was aggrevated (3%). The degree of the Health , thinking of protector for the herb medicine therapy and improvement of sinusitis were significant statistically. (significant <0.005) Conclusion: We know that herbal medicine therapy is the effective treatment of pediatric chronic sinusitis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.18 no.4
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• pp.268-275
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• 2015

Purpose: Primary sclerosing cholangitis (PSC) is a rare condition that can be associated with inflammatory bowel disease (IBD). The aim of this study was to evaluate PSC and its association with IBD in children. Methods: We retrospectively enrolled 13 pediatric patients (<18 years) with PSC treated at Asan Medical Center between June 1989 and December 2013. Clinical findings and long-term outcomes were investigated. During the same period, the incidence of PSC among IBD patients was evaluated among 600 Crohn disease (CD) and 210 ulcerative colitis (UC) patients. Results: All 13 study patients diagnosed with PSC also presented with IBD. Eleven boys and two girls with a median age of 15.0 years old (9.0-17.8 years) were included. The cumulative incidence of PSC for UC was 5.7% (12 of 210) and 0.2% for CD (1 of 600), respectively. PSC occurred during follow-up for IBD for five patients (38.5%) whereas, IBD developed during follow-up for PSC for two patients (15.4%), and was diagnosed during the initial work-up for PSC for 6 patients (46.2%). For the 77.3 month median follow-up period, 9/13 patients (69.2%), neither the clinical symptoms nor blood test results worsened. Two cases (15.4%) developed liver cirrhosis and underwent liver transplantation. Among 13 PSC patients with IBD, two (15.4%) developed colorectal cancer, and no one developed cholangiocarcinoma. Conclusion: All patients with PSC in this study had associated IBD. The incidence of PSC was not rare compared to reports in adults. PSC should be considered during the management of IBD and vice versa in children.

• The Journal of Pediatrics of Korean Medicine
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• v.15 no.1
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• pp.59-70
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• 2001

Background : In recent years, pediatric chronic sinusitis patients who didn't respond with antibiotics were increasing, but there were a few reports on recurrence rate of the herbal medicine therapy treating pediatric chronic sinusitis, so this report was studied. Objective : To know the recurrence rate of herbal medicine therapy in the chronic sinusitis using follow-up investigation. Materials and methods : Thirty four patients(16 male and 18 female) treated in our hospital between December 1998 and August 1999 were studied. Ages ranged from 4 to 13 years (mean age :7.7 years). Twenty five patients(66%) had a underlying family history (allergy or sinusitis of family). In the past history, 32 patients (86%) had asthma, allergy of milk, atopic dermatitis, bronchiolitis and irritable bowel syndrome. Duration of treatment were from eleven to eighteen month (mean:14.3 month). Results : Of the 34 patients, 31 patients were improvement, 3 patients not. Of the 31 patients, thirty patients showed not recurrent (91%). Two patients showed no significant interval change(6%) and one patient was aggrevated(3%). The degree of the Health, thinking of protector for the herb medicine therapy and improvement of sinusitis were significant statistically. (significant <0.005) Conclusion : We know that herbal medicine therapy is the effective treatment of pediatric chronic sinusitis.

• Clinical and Experimental Pediatrics
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• v.62 no.2
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• pp.62-67
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• 2019

Purpose: We aimed to determine the prognostic significance of lymphocyte counts and the lymphocytemonocyte ratio (LMR) in pediatric patients with osteosarcoma. Methods: We retrospectively reviewed the medical records of 27 pediatric patients with localized extremity osteosarcoma, treated at the Korea Cancer Center Hospital between May 2002 and March 2016. Leukocyte counts and LMR before treatment and on day 14 (LMR14) of the first cisplatin-doxorubicin chemotherapy round were evaluated. Patients were dichotomized according to the median value of these parameters, and survival rates were compared. Results: The median age of the 27 patients was 9.9 years (range, 3.2-14.1 years) and tumor sites were: distal femur (n=14), proximal humerus (n=7), proximal tibia (n=2), proximal fibula (n=2), and elsewhere (n=2). Patients were followed up on for a median of 76.4 months (range, 4.5-174.7 months), and 5-year overall (OS) and event-free survival (EFS) rates were $66.0%{\pm}9.8%$ and $60.9%{\pm}9.7%$, respectively. Patients with a higher pretreatment lymphocyte count (${\geq}2,320/{\mu}L$) had better OS (90.9% vs. 46.2%, P=0.04) and EFS (83.9% vs. 38.5%, P=0.02). However, the day 14 lymphocyte count was not associated with survival. While no survival difference was observed between patients grouped according to pretreatment LMR (median value, 6.3), patients with a higher LMR14 (${\geq}5$) fared better than those with lower LMR14 (5-year OS: 83.3% vs. 46.3%, P=0.04). Conclusion: Pretreatment lymphocyte count and LMR during chemotherapy had prognostic significance in pediatric osteosarcoma patients. Further studies involving larger cohorts are necessary to validate our findings.

• Clinical and Experimental Pediatrics
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• v.58 no.5
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• pp.194-198
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• 2015

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at Severance Children's Hospital, Seoul, Korea, were enrolled in this retrospective study. We diagnosed each patient on the basis of the CIDP diagnostic criteria developed in 2010 by the European Federation of Neurological Societies/Peripheral Nerve Society Guidelines. We present the cases of four pediatric patients diagnosed with CIDP to understand the variable clinical course of the disease in children. Our four patients were all between 8 and 12 years of age. Patients 1 and 2 were diagnosed with acute cerebellar ataxia or Guillain-$Barr{\acute{e}}$ syndrome as initial symptoms. While patients 1 and 4 were given only intravenous dexamethasone (0.3 mg/kg/day) for 5 days at the first episode, Patients 2 and 3 were given a combination of intravenous immunoglobulin (2 g/kg) and dexamethasone (0.3 mg/kg/day). All patients were maintained with oral prednisolone at 30 mg/day, but their clinical courses were variable in both relapse intervals and severity. We experienced variable clinical courses of CIDP in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval.

• Pediatric Infection and Vaccine
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• v.30 no.3
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• pp.129-138
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• 2023

Purpose: Cancer incidence is known to be higher in patients with inborn errors of immunity (IEI) compared to the general population in addition to traditionally well-known infection susceptibility. We aimed to investigate cancer occurrence in patients with IEI in a single center. Methods: Medical records of IEI patients treated at Samsung Medical Center, Seoul, Korea were retrospectively reviewed from November 1994 to September 2023. Patients with IEI and cancer were identified. Results: Among 194 patients with IEI, seven patients (3.6%) were diagnosed with cancer. Five cases were lymphomas, 4 of which were Epstein-Barr virus (EBV)-associated lymphomas. The remaining cases included gastric cancer and multiple myeloma. The median age at cancer diagnosis was 18 years (range, 1-75 years). Among patients with cancer, underlying IEIs included X-linked lymphoproliferative disease-1 (XLP-1, n=3), activated phosphoinositide 3-kinase delta syndrome (APDS, n=2), and cytotoxic T-lymphocyte antigen 4 (CTLA-4) haploinsufficiency (n=2). Seventy-five percent (3/4) of XLP-1 patients, 40.0% (2/5) of APDS patients, and 50.0% (2/4) of CTLA-4 haplo-insufficiency patients developed cancer. Patients with XLP-1 developed cancer at earlier age (median age 5 years) compared to those with APDS and CTLA-4 (P<0.001). One patient with APDS died during hematopoietic cell transplantation. Conclusions: Cancer occurred in 3.6% of IEI patients at a single center in Korea. In addition to infectious complications and inflammation, physicians caring for IEI patients should be aware of the potential risk of cancer, especially in association with EBV infection.

• Clinical and Experimental Pediatrics
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• v.60 no.7
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• pp.216-220
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• 2017

Purpose: Vitamin D is associated with various pathological conditions such as cardiovascular diseases and cancer. We investigated the relationship between vitamin D and Kawasaki disease (KD). Methods: We performed a retrospective review of the medical records of patients with KD between February 2013 and March 2016 in Daegu Fatima Hospital. Study participants were grouped according to vitamin D serum concentration. Group 1 included patients with 25(OH)-vitamin D ${\geq}20ng/mL$. Group 2 included patients with 25(OH)-vitamin D <20 ng/mL. We analyzed the clinical characteristics and laboratory data of the 2 groups. Results: Of the 91 patients, 52 were included in group 1, and 39 in group 2. Group 1 patients had significantly higher levels of calcium, phosphate, albumin and sodium than group 2 patients did. There were no differences in clinical characteristics, but the proportion of patients with polymorphic rash was significantly higher in group 2. Resistance to intravenous immunoglobulin was more frequent in group 2 (P=0.023). No significant difference in the incidence of coronary artery complications was observed. Conclusion: Low vitamin D levels are associated with resistance to intravenous immunoglobulin therapy in KD. Vitamin D deficiency might be a risk factor for immunoglobulin resistance in KD.