• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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• v.14 no.2
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• pp.194-197
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• 2001

Progressive pigmented purpuric dermatosis(Schamberg's disease, purpura simplex) is an uncommon eruption characterized by petechiae and patches of brownish pigmentation, particularly on the lower extremities. Lesions remain for months or years and present only a cosmetic problem. there is no hematologic disease, venous insufficiency, or associated internal disease. The most characteristic feature is orange brown, pinhead-sized "cayenne pepper" spots. It is hard to find similar disease in Oriental Medicine, however it could be though related with 瘀血. We observed and treated a 25 old female with progressive pigmented purpuric dermatosis on her lower extremities, without pain and itching sign. About 1 year after our treatment, herb-medication. acupuncture treatment, negative therapy and applied aroma oil in order to remove the 瘀血(a kind of congestion) & inner heat and promote the circulation of her blood, the area of pigmented purpuric dermatosis was decreased remarkably and the colour was lighter. She is been treated continuously now and satisfied with the efficacy of treatment.

• Clinical Psychopharmacology and Neuroscience
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• v.16 no.4
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• pp.501-504
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• 2018

Autoimmune hemolytic anemia is a disease characterized with destruction of erythrocytes as a result of antibody produce against patient's own erythrocytes and anemia. Autoimmune hemolytic anemia can be roughly stratified into two groups according to serological features and secondary causes including drugs induced hemolytic anemia. Drugs induced autoimmune hemolytic anemia is very rare in pediatric patients. Even though hematological side effects such as leucopenia, agranulocytosis, eosinophilia, thrombocytopenic purpura and aplastic anemia might occur due to psychotropic drug use; to the best of our knowledge there is no autoimmune hemolytic anemia case due to quetiapine, an atypical antipsychotics, in literature. We hereby describe the first child case of autoimmune hemolytic anemia during quetiapine treatment.We also are pointing out that one should keep in mind serious hematological side effects with atypical antipsychotic drug use with this case report.

• Journal of Yeungnam Medical Science
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• v.38 no.2
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• pp.165-168
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• 2021

We report the case of a 16-month-old patient with chronic immune thrombocytopenia (ITP) patient who experienced delayed treatment-free response (TFR) after romiplostim treatment. He received intravenous immunoglobulin every month to maintain a platelet count above 20,000/µL for 2 years. Thereafter, he received rituximab and cyclosporine as second-line therapy, with no response, followed by romiplostim. After 4 weeks of treatment, the platelet count was maintained above 50,000/µL. Following 7 months of treatment, he discontinued romiplostim, and the platelet count decreased. His platelet counts remained above 50,000/µL, without any bleeding symptoms, 2 years after romiplostim discontinuation. This is the first report of TFR after romiplostim treatment in pediatric chronic ITP.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.175-182
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• 2005

Purpose : Interleukin 1 receptor antagonist(IL-1ra) is an endogenous antiinflammatory agent that binds to IL-1 receptor and thus competitively inhibits the binding of IL-1$\alpha$ and IL-1$\beta$. Allele 2 in association with various autoimmune diseases has been reported. In order to evaluate the influence of IL-1ra gene VNTR polymorphism on the susceptibility to HSP and its possible association with disease severity, manifested by severe renal involvement and renal sequelae, we studied the incidence of carriage rate and allele frequency of the 2 repeats of IL-1ra allele 2($IL1RN^{*}2$) of the IL-1ra gene in children with HSP with and without renal involvement. Methods : The IL-1ra gene polymorphisms were determined in children with HSP with(n=40) or without nephritis(n=34) who had been diagnosed at Busan Paik Hospital and the control groups(n=163). Gene polymorphism was identified by PCR amplification of the genomic DNA. Results : The allelic frequency and carriage rate of $IL1RN^{*}1$ were found most frequently in patients with HSP and in controls. The allelic frequency of $IL1RN^{*}2$ was higher in patients with HSP compared to that of controls($4.7\%\;vs.\;2.5\%$, P=0.794). The carriage rate of $IL1RN^{*}2$ was higher In patients with HSP compared to that of controls($8.1\%\;vs.\;6.8\%$, P=0.916). The allelic frequency of $IL1RN^{*}2$ was higher in patients with HSP nephritis compared to that of HSP($5.3\%\;vs.\;2.9\%$, P=0.356). The carriage rate of $IL1RN^{*}2$ was higher in Patients with HSP nephritis compared to that of HSP($10.0\%\;vs.\;5.9\%$, P=0.523). Among 13 patients with heavy proteinuria(>1.0 g), 11 had $IL1RN^{*}1$, 1 had $IL1RN^{*}2$ and the others had $IL1RN^{*}4$. At the time of last follow up 4 patients had sustained proteinuria and their genotype was $IL1RN^{*}1$. Conclusion : The allelic frequency and carriage rate of $IL1RN^{*}1$ were found most frequently in patients with HSP and in controls. Our study suggests that the carriage rate and allele frequency of the 2-repeats of IL-1lra allele 2($IL1RN^{*}2$) of the IL-1ra gene may not be associated with susceptibility and severity of renal involvement in children with HSP (J Korean Soc Pediatr Nephrol 2005;9:175-182)

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.12 no.2
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• pp.156-162
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• 2009

Purpose: The aim of this study was to investigate the clinical usefulness of upper gastrointestinal (GI) endoscopy in children with Henoch-Sch$\"{o}$nlein purpura (HSP). Methods: We retrospectively analyzed the clinical, endoscopic, and histopathologic records of children with HSP who had been admitted to the Department of Pediatrics of Gil Hospital and underwent upper GI endoscopy between January 2002 and June 2009. Patients were classified into the following two groups for statistical analysis: duodenal involvement (+) and duodenal involvement (-). Results: Fifty-one children with HSP underwent upper GI endoscopy; the mean age was 7.2${\pm}$2.9 years. The upper GI endoscopy showed abnormalities of the duodenum in 38 cases (74.5%), 22 of which had duodenal ulcers. Among the biopsy specimens obtained from the duodenum of 37 cases, 13 cases (35.1%) had leukocytoclastic vasculitis, neutrophil debri, and/or extravasation of RBCs. Steroid use was more frequent in the duodenal involvement (+) group (86.8%) than the duodenal involvement (-) group (53.8%; p=0.02). The mean length of hospitalization was 13.9${\pm}$8.43 days in the duodenal involvement (+) group and 8.1${\pm}$4.62 days in the duodenal involvement (-) group (p=0.003). The recurrence rate was significantly higher in the duodenal involvement (-) group than the duodenal involvement (+) group (p=0.027), whereas none of the other study parameters, such as the age of onset, renal involvement, and steroid use, led to significantly higher or lower recurrence rates. Conclusion: These results suggest that duodenal involvement can influence the clinical course and prognosis of HSP in children.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.14 no.2
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• pp.141-147
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• 2011

Purpose: Ultrasonography (US) is widely used as a screening test in patients with abdominal pain (AP). We investigated the usefulness of US by a pediatrician in children with AP. Methods: We retrospectively analysed the medical records of children with AP who undertook US from December, 2008 to July, 2010. Results: A total of 628 patients (325 male, 303 female) were enrolled in this study. The mean age of patients was $8.08{\pm}4.61$ years. Duration of AP was acute in 427 and chronic in 201 patients. Localization of AP was diffuse (36.9%), periumbilical (24.4%), epigastric (21.0%), and right lower quadrant (8.1%). On the examination, there were no abnormal findings in 327 patients (52.1%). Abnormal ultrasonographic findings were mesenteric lymphadenitis (27.1%), intestinal mural thickening (10.0%), intussusception (3.0%), appendicitis (2.6%), choledochal cyst (1.6%), and pancreatitis (0.3%). We performed additional imaging studies such as computed tomography (CT) or magnetic resonance imaging (MRI) in 39 patients who showed obscure findings on the US. In 33 patients (84.6%), the same results were obtained from CT or MRI. Two cases of appendicitis, one case of pancreatitis and one case of Henoch-Sh$\ddot{o}$nlein purpura were diagnosed by the CT examination. However, there were two cases of appendicitis diagnosed by US thathad no evidence of appendicitis on the CT. Diagnostic accuracy of initial US in children with abdominal pain was 99.4%. Conclusion: US by a pediatrician as a screening test in children with AP provides a rapid and accurate diagnostic indication and has non-invasive and radiation-free advantages.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.146-152
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• 2009

Purpose : We performed this study to evaluate the incidence and clinical significance of antiphospholipid antibodies (aPL Ab) in Korean children with Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP). Methods : The medical records of 62 patients (31 boys and 31 girls) aged $46.0{\pm}3.1$ (1-16) years with a clinical diagnosis of HSP based on the EULAR/PReS criteria were reviewed retrospectively. From the years 2007 to 2009, the sera from children with acute HSP were tested for aPL Ab such as LA, anti-cardiolipin antibody and anti-${\beta}_2$ glycoprotein I antibody. Results : LA was positive in 18 (29%) of the 62 patients with HSP and We divided the patients into the two groups LA positive group (N=18) and LA negative group (N=44). There were no significant differences between the two groups with regard to abdominal pain, arthralgia and renal involvement, but LA positive group had significantly higher C-reactive protein ($4.3{\pm}7.2$ mg/dL vs. $1.3{\pm}1.8$ mg/dL, P=0.035), erythrocyte sedimentation rate ($37.5{\pm}26.2$ mm/hr vs. $25.1{\pm}22.6$ mm/hr, P= 0.039), IgM ($148.1{\pm}48.4$ mg/dL vs. $114.9{\pm}41.5$ mg/dL, P=0.024), C3 ($143.1{\pm}21.9$ mg/dL vs. $129.7{\pm}24.5$ mg/dL, P=0.048) and C4 levels ($30.9{\pm}6.3$ mg/dL vs. $24.9{\pm}7.8$ mg/dL, P=0.002) compared with LA negative group. Conclusion : We found that the incidence of positive aPL Ab tests was relatively higher in Korean children with HSP and the presence of aPL Ab was associated with acute inflammatory process of HSP. These results suggest that the aPL Ab are involved in the pathogenesis of HSP in children.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.505-511
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• 2002

Purpose : Mean platelet volume(MPV) and platelet distribution width(PDW) are useful parameters in evaluating disorders of platelets. In cases with idiopathic thrombocytopenic purpura(ITP), they change as platelet count increases. In this study, we compared the values of MPV and PDW in ITP patients at diagnosis with those of normal children. We also studied whether the early changes in MPV may predict the clinical course. Methods : From December 1995 to May 2001, 71 patients with ITP were admitted to Ajou University Hospital. They were treated with IVIg 400 mg/kg for five days and MPV, PDW, platelet count were analysed. Normal control group(n=38) was compared. The study group was divided into acute and chronic forms, and also divided into group A, good early responders whose platelets increased more than $100,000/{\mu}L$ within 5 days and group B who did not. Results : Mean value of MPV at diagnosis in ITP patients was lower than the normal control group(P<0.05). In group A, MPV was abruptly increased on the first day after IVIg and then started to decrease. But in group B, MPV was steadily increased until the fourth day after IVIg. In the normal control group, there were inverse correlations between platelet count and MPV(r=-0.415, P<0.05), but in ITP patients, there were positive relationships between platelet count and MPV(r=0.646, P<0.05) at diagnosis. Conclusions : MPV at diagnosis of ITP was lower than the normal control. MPV and PDW could not predict the course of ITP patients, but MPV could distinguish good early responders. More research is needed to find out the reasons of decreased MPV at diagnosis of ITP.

• Clinical and Experimental Pediatrics
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• v.49 no.7
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• pp.784-789
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• 2006

Purpose : $Henoch-Sch{\ddot{o}}nlein$ purpura(HSP) is a systemic vasculitis involving small vessels of skin, gastrointestinal(GI) tract and kidney. Digestive involvement of HSP can be serious with massive GI bleeding, perforation, and intussusception. However, some patients do not respond to conventional corticosteroid therapy. In this study, we investigated the efficacy of intravenous immunoglobulin (IVIG) for serious digestive manifestations not responding to steroid. Methods : From April 1999 to January 2005, 22 children diagnosed as HSP with severe GI symptoms were included. Initially, all patients were treated with intravenous methylprednisolone. IVIG 2 g/kg of body weight was infused in children refractory to steroid therapy. Clinical data were reviewed retrospectively. Results : Among 22 children, 12 children underwent IVIG therapy. The mean duration of corticosteroid therapy was $5.61{\pm}4.9$ days before IVIG therapy, and 11 of 12 patients experienced disappearance of GI manifestations after the initiation of IVIG infusion. In one patient, IVIG was ineffective in relieving abdominal pain, but melena subsided. Comparison of the duration of hospitalization between IVIG group and corticosteroid group revealed no significant difference($12.8{\pm}7.6$ days vs. $13.2{\pm}7.8$ days, P=0.777). But, the total duration of abdominal pain decreased in IVIG group although the difference between two groups was not significant($8.8{\pm}8.1$ days vs. $14.8{\pm}16.9$ days, P=0.306). Among 10 children treated with steroid only, 2 children were operated for bowel perforation and intussusception. In contrast, there was no perforation in 12 children who underwent IVIG therapy. Conclusion : IVIG could be the alternative therapy to corticosteroids in children with severe digestive manifestations of HSP.

• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1217-1221
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• 2008

Purpose : An association between idiopathic thrombocytopenic purpura (ITP) and systemic lupus erythematosus (SLE) has been recognized for decades because thrombocytopenia is the first manifestation in some patients with SLE. However, the risk of later development of SLE in childhood ITP is currently unknown. We retrospectively evaluated the incidence and clinical significance of the positive antinuclear antibody (ANA) in children with acute ITP. Methods : This study was retrospectively performed to review the clinical and laboratory characteristics in 77 children diagnosed to have acute ITP and admitted to the Pusan National University Hospital between January 2003 and December 2006. Patients tested positive for ANA were regularly followed-up for at least 12 months for symptoms indicative of SLE. Results : Seventy-seven children were included in the study; 38 males (49.4%) and 39 females (50.5%), the mean age was 4.5 years. Sixteen (20.8%) ITP patients had a positive ANA, with a median titer of 1:320. The mean age of the patients with positive ANA was 9.3 years, which is much older than 3.3 years for patients with negative ANA (P<0.05). The positive ANA group was predominantly female (81.3%) compared to the negative ANA group (P<0.05). There was no statistically significant difference in mean platelet counts between both groups. No statistically significant difference was found in ANA positivity and progression to chronic ITP or SLE. After the median follow-up of 32 months, SLE was diagnosed only in one ITP patient with positive ANA. Conclusion : Our data demonstrated that ANA positivity is often found in children with acute ITP. Large-scale studies should be considered to determine the significance of ANA positivity in childhood ITP for the later development of SLE.