• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.987-990
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• 2006

Intravenous immunoglobulin (IVIG) infusion is an effective therapy for acute Kawasaki disease (KD). Nonetheless, approximately 10 percent to 20 percent of patients have persistent or recrudescent fever despite IVIG treatment, leading to a higher risk for coronary artery aneurysms (CAA). This unresponsiveness may pose a challenge to the clinicians. Tumor necrosis $factor-{\alpha}$ levels are elevated in the acute phase of the disease, especially in patients who develop CAA. We report a 10-month-old male with KD who failed to respond to multiple doses of IVIG and methylprednisolone and who then was treated with infliximab (5 mg/kg single dose). After infliximab treatment, he became afebrile with normalization of inflammatory markers and no further progression of CAA.

• Childhood Kidney Diseases
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• v.19 no.2
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• pp.176-179
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• 2015

Rituximab (RTX), a monoclonal antibody against the B-cell marker CD20, is commonly used as a treatment for antibody-mediated diseases or B-lymphocyte-mediated diseases. Destruction of B cells may reverse the disease course in many conditions; however, patients who are treated with RTX cannot respond appropriately to de novo infection due to lack of B lymphocytes. Here, we report one such case. A 7-year-old renal allograft recipient presented with severe anemia due to parvovirus infection after RTX treatment. The patient had focal segmental glomerulosclerosis and had received cadaveric kidney transplantation 6 months previously. She was treated with high-dose steroid for acute rejection and RTX for Epstein Barr Virus infection 3 months previously. At presentation, her hemoglobin level was 5.4 g/dL and leukocyte and platelet counts were normal. She had microcytic normochromic anemia and high viral load of parvovirus B19(70,578 copies/mL). Intravenous immunoglobulin ($200mg/kg{\cdot}d$) treatment controlled the progression of anemia and parvovirus infection. De novo parvovirus infection during the B lymphocyte-depletion period may have precipitated the severe anemia in this case. Close monitoring of infection is required after RTX therapy.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.231-236
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• 2005

Juvenile rheumatoid arthritis(JRA) is the most common major connective tissue disease in children. Renal involvement in JRA is rare. Among the renal lesions that have been reported in JRA, amyloidosis and drug-induced nephropathy are the most common. Crescentic glomerulonephritis in JRA has rarely been reported. We report a case of ANCA-associated pauci-immune crescentic glomerulonephritis in JRA. The patient was a 15-year old boy with a 3-year history of JRA. He presented with gross hematuria, proteinuria, positive p-ANCA and elevation of BUN and creatinine. Pathologic findings revealed focal necrotizing and crescentic glomerulonephritis. There were no significant immunoglobulin or complement deposits. His renal function recovered after intravenous methylprednisolone pulse therapy and oral steroid use. In Korea, this is the first reported case of pauci-immune crescentic glomerulonephritis in JRA. (J Korean Soc Pediatr Nephrol 2005;9:231-236)

• Clinical and Experimental Pediatrics
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• v.61 no.2
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• pp.43-48
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• 2018

Purpose: Human adenovirus infection mimics Kawasaki disease (KD) but can be detected in KD patients. The aim of this study was to determine the clinical differences between KD with adenovirus infection and only adenoviral infection and to identify biomarkers for prediction of adenovirus-positive KD from isolated adenoviral infection. Methods: A total of 147 patients with isolated adenovirus were identified by quantitative polymerase chain reaction. In addition, 11 patients having KD with adenovirus, who were treated with intravenous immunoglobulin therapy during the acute phase of KD were also evaluated. Results: Compared with the adenoviral infection group, the KD with adenovirus group was significantly associated with frequent lip and tongue changes, skin rash and changes in the extremities. In the laboratory parameters, higher C-reactive protein (CRP) level and presence of hypoalbuminemia and sterile pyuria were significantly associated with the KD group. In the multivariate analysis, lip and tongue changes (odds ratio [OR], 1.416; 95% confidence interval [CI], 1.151-1.741; P=0.001), high CRP level (OR, 1.039; 95% CI 1.743-1.454; P= 0.021) and sterile pyuria (OR 1.052; 95% CI 0.861-1.286; P=0.041) were the significant predictive factors of KD. In addition, the cutoff CRP level related to KD with adenoviral detection was 56 mg/L, with a sensitivity of 81.8% and a specificity of 75.9%. Conclusion: Lip and tongue changes, higher serum CRP level and sterile pyuria were significantly correlated with adenovirus-positive KD.

• Clinical and Experimental Pediatrics
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• v.59 no.8
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• pp.335-340
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• 2016

Purpose: This study aimed to evaluate the clinical course of childhood immune thrombocytopenia (ITP) and to assess the risk factors for developing chronic ITP Methods: The records of 64 children diagnosed with ITP from November 2005 and December 2014 at single center were retrospectively analyzed. Results: The median age at diagnosis and the median platelet count were 1 year (range, 1 month to 15 years) and $9{\times}10^9/L$ (range, $0-84{\times}10^9/L$), respectively. No patient experienced severe bleeding. Nineteen children (29.7%) spontaneously recovered their platelet count to ${\geq}100{\times}10^9/L$ at a median of 10 days. In total 45 patients (70.3%) received intravenous immunoglobulin (IVIG) as first-line therapy, and showed platelet recovery at 1 week. The final diagnosis of 55 (85.9%) and 9 patients (14.1%) was acute and chronic ITP, respectively. Older age, absence of prior infection and insidious onset of symptoms were significantly associated with the development of chronic ITP. Among the patients who received IVIG, those with platelet count <$45{\times}10^9/L$ at 1 month after IVIG showed a significantly higher incidence of chronic ITP compared to those with platelet count ${\geq}45{\times}10^9/L$ (88.8% vs. 44.4%, P<0.01). Conclusion: In most patients, ITP runs a benign course and approximately 86% of them recover within 1 year of their initial diagnosis. The potential impact of the risk factors of chronic ITP on clinical practice needs to be explored and further studies are warranted to determine whether IVIG influences the course of ITP.

• Archives of Plastic Surgery
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• v.38 no.5
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• pp.687-690
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• 2011

Purpose: Pemphigus vulgaris, a rare autoimmune blistering disease of the skin and mucous membranes remains a challenging disease to treat. Management is focused on immunotherapy against autoimmune antibodies that target keratinocyte cell adhesion molecules, and antibiotics preventing secondary infections. There is no established dressing protocol and skin is usually manipulated the least amount possible in order to minimize irritation. The authors suggest that early initiation of aggressive bathing and debridement of skin lesions, with nutritional support, is essential in accelerating resolution. Methods: A 40 year-old male previously diagnosed with pemphigus vulgaris was admitted due to exacerbation of mucocutaneous lesions involving the epidermis and mucosa of the whole body. Steroids, immunosuppressants, intravenous immunoglobulin and antibiotics were administrated, but infection and de-epithelialization progressed, while his general condition deteriorated with a weight loss of over 20 kilograms. The plastic surgery department intervened with daily bathing, debridement of unhealthy debris and non-traumatizing coverage of growing epithelium. Total parenteral nutrition and mobilization with rehabilitation therapy was initiated as early as possible. Results: After bathing, healthy epithelium gradually covered the patient's entire body, while his general condition improved with a corresponding weight gain of 14 kgs. Conclusion: Treatment of pemphigus vulgaris focuses on immunotherapy and infection control. However, an equal amount of attention should be laid on early intervention with daily dressings including bathing and irrigation, nutritional support, and exercise as this accelerates resolution of existing infections, promotes healthy epithelialization and leads to faster recovery.

• Clinical and Experimental Pediatrics
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• v.64 no.2
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• pp.68-75
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• 2021

The novel coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has been spreading worldwide since December 2019. Hundreds of cases of children and adolescents with Kawasaki disease (KD)-like hyperinflammatory illness have been reported in Europe and the United States during the peak of the COVID-19 pandemic with or without shock and cardiac dysfunction. These patients tested positive for the polymerase chain reaction or antibody test for SARS-CoV-2 or had a history of recent exposure to COVID-19. Clinicians managing such patients coined new terms for this new illness, such as COVID-19-associated hyperinflammatory response syndrome, pediatric inflammatory multisystem syndrome temporally associated with COVID-19, or COVID-19-associated multisystem inflammatory syndrome in children (MIS-C). The pathogenesis of MIS-C is unclear; however, it appears similar to that of cytokine storm syndrome. MIS-C shows clinical features similar to KD, but differences between them exist with respect to age, sex, and racial distributions and proportions of patients with shock or cardiac dysfunction. Recommended treatments for MIS-C include intravenous immunoglobulin, corticosteroids, and inotropic or vasopressor support. For refractory patients, monoclonal antibody to interleukin-6 receptor (tocilizumab), interleukin-1 receptor antagonist (anakinra), or monoclonal antibody to tumor necrosis factor (infliximab) may be recommended. Patients with coronary aneurysms require aspirin or anticoagulant therapy. The prognosis of MIS-C seemed favorable without sequelae in most patients despite a reported mortality rate of approximately 1.5%.

• Journal of Acupuncture Research
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• v.38 no.2
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• pp.170-174
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• 2021

A patient diagnosed with Guillain-Barre syndrome (GBS) suffering from numbness in the toes and fingers, paralysis in the face, and back pain received intravenous immunoglobulin on December 28, 2019. Without an improvement in symptoms the patient was admitted to a Korean medicine hospital on January 1, 2020 to receive combined Korean medicine treatment including herbal medicine (Sipjeondaebo-tanggami and Hyangbujasunkipalmul-tang), acupuncture treatment, moxibustion, cupping treatment, and physical therapy. Treatment was administered over 51 days, of which 17 days were during hospitalization. Paralysis of facial muscle, and numbness in fingers and toes were evaluated using the Yanagihara score and numeric rating scale (NRS) score, respectively. Facial paralysis improved (21 to 40 based on Yanagihara score) and paresthesia of the hands and feet disappeared (NRS score 6-7 to NRS score 0). There were no side effects or sequela. This study suggested combined Korean medicine treatment may be effective for the treatment of GBS.

• Pediatric Infection and Vaccine
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• v.23 no.2
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• pp.149-154
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• 2016

Although an association of Kawasaki disease (KD) with infectious agents has been suggested, none have been proven to cause KD. In this case study, we present a case of KD with concurrent onset of influenza and Mycoplasma pneumoniae (MP) infections. A 27-month-old boy presented with prolonged fever, cough, and rhinorrhea. During the initial testing, influenza A infection was identified, and he was treated with oseltamivir. Despite the antiviral therapy, the fever persisted, and he had cervical lymph node enlargement, bilateral conjunctival injection, fissured red lips, strawberry tongue, and erythematous skin lesions on the Bacillus Calmette-$Gu{\acute{e}}rin$ vaccination site. Thus, the patient was diagnosed with KD and was treated with intravenous immunoglobulin (IVIG). The result of the initial antimycoplasma immunoglobulin M (IgM) antibody testing and was positive, and an increased IgM titer from baseline was found in a repeat test. We reviewed the hypotheses on pathogens known to be associated with KD and the etiology of KD. Based on our findings, we suspect that symptoms of KD and coronary artery lesions can occur from various infections besides those caused by Mycoplasma species and influenza viruses.

• Clinical and Experimental Pediatrics
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• v.50 no.3
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• pp.292-297
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• 2007

Purpose : Kawasaki disease (KD) rarely occurs in school-aged children. We clarified the characteristics of KD in this age group to provide tips for a high index of suspicion. Methods : Features of 38 patients with KD who were 7 years of age or older were retrospectively reviewed. Results : The incidence of the KD patients ${\geq}7years$ was 4.9 percent. The ratio of male to female was 2.5:1. Of the 38 patients, nine patients (24.0 percent) were diagnosed with typical KD and 29 patients (76.0 percent) with incomplete KD. In incomplete KD patients, cervical lymphadenopathy (69.0 percent) occurred most frequently, followed by conjunctival injection (62.0 percent) and polymorphous rash (45.0 percent). These patients occasionally presented with other additional symptoms including abdominal pain, headache, vomiting and arthralgia. Incomplete KD was initially diagnosed as cervical lymphadenitis (34.0 percent), viral infection (14.0 percent), scarlet fever (7.0 percent), meningitis (7.0 percent), and Kikuchi disease (7.0 percent). Coronary complications were noted in 15 patients (39.0 percent). Of the 37 patients treated with intravenous immunoglobulin, five (14.0 percent) were resistant to the therapy and all had coronary abnormalities. Conclusion : Most patients with KD ${\geq}7years$ of age have incomplete presentations. They tend to have a higher incidence of initial presentations of unilateral neck mass and coronary artery involvement. In school-aged children, fever and cervical lymphadenitis or suspected neck infection unresponsive to intravenous antibiotics should signal the possibility of KD. A high index of suspicion and prompt treatment is essential in this age group of patients.