• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.1
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• pp.11-19
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• 2007

Purpose: Henoch-Sch$\"{o}$nlein purpura (HSP) is a systemic vasculitis involving the skin, joints, gastrointestinal tract, and kidney. Although the pathogenesis of HSP is still unclear, tumor necrosis factor (TNF-${\alpha}$) is regarded as an important cytokine contributing to the disease. The goal of this study was to determine the role of TNF-${\alpha}$ in the pathogenesis of HSP, and to evaluate the TNF-${\alpha}$ polymorphism for genetic susceptibility to HSP. Methods: From March 2004 to November 2005, 40 children with HSP and 32 healthy controls were included. Serum TNF-${\alpha}$ levels were measured using the ELISA method during the acute and convalescent phase of HSP. The genotypic and allelic frequencies of the TNF-${\alpha}$ gene polymorphisms at positions -308 and -238 were evaluated in patients and controls. Results: Serum TNF-${\alpha}$ levels were $23.17{\pm}11.31$ pg/mL in the acute phase of children with HSP and $10.56{\pm}5.59$ pg/mL in the convalescent phase (p=0.000). There was no significant correlation between the serum TNF-${\alpha}$ levels and the clinical scores of HSP (r=0.310, p=0.070). The genotypic frequency of the TNF-${\alpha}$ -308 polymorphism in children with HSP was not significantly different compared to healthy controls (GG 80%, GA 20% vs. GG 93.8%, GA 6.2%; p=0.094). The genotypic frequency of the TNF-${\alpha}$ -238 polymorphism in children with HSP was not significantly different (GG 97.5%, GA 2.5% vs. GG 93.8%, GA 6.3%; p=0.429). Conclusion: TNF-${\alpha}$ is assumed to be the main cytokine associated with the pathogenesis of HSP during the acute phase. However, the presence of TNF-${\alpha}$ gene polymorphisms at positions -308 and -238 did not distinguish children with HSP from normal controls.

• Childhood Kidney Diseases
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• v.8 no.1
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• pp.10-17
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• 2004

Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) nephritis has a variable range of prevalence from 25 to 50% among HSP patients and is a common cause of chronic glomerulonephritis in children. In our study, we evaluated the distribution and the association of the angioten-sinogen(AGT) M235T polymorphism with the clinical manifestations, particularly proteinuria in children with HSP with or without nephritis. Methods : The AGT M235T polymorphism was determined in children with HSP nephritis (n=33) or HSP without nephritis(n=28) who had been diagnosed at Busan Paik hospital from January 1996 to June 2001. The M235T polymorphism of the AGT gene was determined by PCR amplification of the genomic DNA. Results : The M235T polymorphism of AGT gene frequency was MM 75%, MT : 25%, TT : 0% in HSP and MM : 64%, MT : 36%, TT : 0% in HSP nephritis, there was no significant differences in the genotype and allele frequencies between the two groups. No significant differences in clinical manifestations at onset and last follow-up were seen between the two genotypes. When statistical analysis was done according to the presence of the M allele, the amount of 24-hour urinary protein excretion and the incidence of moderate to heavy proteinuria(>500 $mg/m^2/day$) at onset and at last follow-up were higher in the MT genotype than in those of in the MM genotype but these difference were not statistically significant. Conclusion : We suggest a lack of association between M235T polymorphism of the AGT gene and clinical manifestations in children with HSP nephritis. However, further follow-up studies based on sufficient number of patients and long term follow up periods are necessary to confirm the role of M235T polymorphism of AGT gene in children with HSP nephritis.

• Journal of Yeungnam Medical Science
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• v.18 no.2
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• pp.239-245
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• 2001

Background: Acute idiopathic thrombocytopenic purpura(ITF) is one of the common hematologic disorders in children. Bone marrow aspiration (BMA) is often performed in children with acute ITP to rule out leukemia, aplastic anemia or other hematologic diseases. However, whether BMA is needed in children with typical clinical and hematological features of acute ITP have been questioned. This study was performed to examine the proper indication of BMA in acute childhood ITF. Materials and Methods: The medical records and BMA reports of children with the provisional diagnosis of acute ITP were reviewed from January 1984 to December 2000. Patients were divided into two groups, one with typical and another with atypical clinical and hematological features of acute ITP. Typical acute ITP group was characterized by the history of previous viral infection, well being appearance, no hepatosplenomegaly, no lymphadenopathy, normal Hb, WEC, neutrophil count and peripheral blood smear except thrombocytopenia. A platelet count of $50{\times}l0^9/L$ or lower was the cutoff level. Results: Total 120 children with the provisional diagnosis of acute ITP were included. One hundred eighteen of them were confirmed to have acute ITP by BMAs. Of these, 66 had typical and 54 had atypical features. All of typical features and 52 of 54 with atypical features of acute ITP were confirmed to have acute ITP by BMAs. Two patients with atypical features of acute ITP were diagnosed as aplastic anemia and myelodyspalstic syndrome, respectively, by BMAs. Conclusion: This study concludes that BMA is not needed for the children with typical features of acute ITP but it is needed for the children with atypical features of acute ITP to rule out other hematologic disorders.

• The Korean Journal of Blood Transfusion
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• v.24 no.3
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• pp.233-240
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• 2013

Background: A previous history of transfusion has been known to be associated with production of anti-HLA class I antibodies. However, platelet glycoproteins are the main target of idiopathic thrombocytopenic purpura (ITP). The mechanism of antibody production is known to differ significantly between glycoproteins and anti-HLA class I. The aim of this study was to evaluate the clinical significance of anti-HLA class I antibodies in childhood ITP. Methods: Enrollment for the normal control group targeted 48 people who visited Gyeongsang National University Hospital from 1990 to 2010, and 48 young children with ITP. Anti-glycoproteins and anti-HLA class I antibodies were tested using the Modified Antigen Capture Enzyme-linked immunosorbent assay (MACE) kit. Results: The positive rate of anti-HLA antibodies was significantly different [36/39 (92.3%) vs 29/46 (63%)] [ITP group vs normal control group] (P=0.002). The mean positive S/C ratio of anti-HLA antibodies was also significantly different (3.55 vs 1.51) [ITP group vs normal control group] (P=0.0000). The positive rate of anti-HLA did not differ significantly between the transfused group and the non-transfused group [12/12 (100%) vs 24/27 (88%)] [transfused ITP vs non-transfused ITP]. The mean positive S/C ratio of anti-HLA antibodies did not differ significantly between the transfused ITP group and the non-transfused ITP group (4.30 vs 3.25) [transfused ITP vs non-transfused ITP]. Consecutive testing showed that positive rate and positive S/C ratio of anti-HLA antibodies did not change significantly between sampling times in both groups [transfused ITP vs non-transfused ITP] (P=1.00 and P=0.15). Conclusion: Anti-HLA class I antibodies may be involved in childhood ITP. Transfusion did not affect the course of childhood ITP.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.12 no.2
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• pp.177-182
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• 2009

Purpose: Colitis is a condition associated with a spectrum of altered morphologic changes and cellular adhesion. E-cadherin plays a key role in the establishment and maintenance of epithelial tissue structure and cell-cell adhesion. The purpose of this study is to evaluate E-cadherin expression in colonic epithelium of various colitis in children. Methods: The expressions of E-cadherin were examined in 39 cases of colonic mucosal biopsy specimen using immunohistochemical staining. When more than 50 percent of cells exhibited uniformly the same intensity and pattern of immunostaining as the adjacent normal mucosa, the antigen expression was considered normal. Abnormal expression was defined when less than 50 percent of cells stained, when cells showed a heterogeneously weak or altered distribution, or when complete absence of staining was observed. Results: Fifteen cases with non-specific colitis (38.5%), 7 cases of with Crohn's disease (17.9%), 5 cases of infectious colitis and milk protein sensitive proctocolitis (12.8%), 3 cases of ulcerative colitis (7.7%), 2 cases of Henoch-Schonlein purpura colitis (5.1%), one case of Behcet's disease and ischemic colitis (2.6%) were included in this study. E-cadherin expression was decreased in all kinds of colitis. Reduced expression of E-cadherin was observed in 77 percent of cases. E-cadherin was weaker or no expression in reparative epithelium and "ulcer associated cell lineage". Conclusion: Altered expression of E-cadherin occurs during mucosal inflammation in any kinds of colitis. These changes may be involved in promoting cell migration during epithelial restitution of the gastrointestinal mucosa.

• Pediatric Infection and Vaccine
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• v.11 no.1
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• pp.126-130
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• 2004

Neisseria meningitidis is a leading cause of meningitis and septicemia in children and young adults. Meningococcemia, the most severe form of infection, represents a part of the various spectrum of the illness, and its clinical manifestations varied from mild fever and occult bacteriemia to fulminant catastrophic events(e.g., sepsis, shock, and death) within a few hours after onset of symptoms. Complement deficiencies, either congenital or acqired, increase the risk for invasive meningococcal disease. Since C3 plays a critical role in the complement cascade, congenital C3 deficiency or conditions that decrease C3(e.g., systemic lupus erythematosus, cirrhosis, nephritis, C3 nephritic factor) increase the risk for invasive disease due to pyogenic bacteria including N. meningitidis. We had experienced a case of meningococcemia and meningococcal meningitis presenting with mild fever, petechiae, and purpura. The patient was a 7-year old male. He had mild fever, vomiting, headache, nuchal rigidity, petechiae and purpura on both lower extremities, which spreaded to the whole body. His blood culture grew Neisseria meningitidis. The diagnosis of meningococcal meningitis was confirmed by examination of cerebrospinal fluid. The clinical symptoms of the illness were improved after treatment of antibiotics(cefotaxime) for 12 days. However the patient had developed generalized edema, microscopic hematuria & proteinuria on the third day after admission. High ASO titer and low serum C3 level were also noted. The microscopic hematuria returned to normal about 2 months after discharge. The C3 level returned to normal range about 3~4 months after discharge. we report a case of combined acute poststreptococcal glomerulonephritis and acute invasive meningococcal disease with reference review.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.149-153
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• 2013

We report the case of a 14-year-old girl, diagnosed with atypical thrombotic microangiopathy (TMA). The patient presented with persistent fever, nausea, and newly developed peripheral edema. Her laboratory findings indicated chronic anemia with no evidence of hemolysis, thrombocytopenia, or elevated serum creatinine level. A few days after hospitalization, acute renal failure and fever worsened, and proteinuria developed. On day 40 of hospitalization, she experienced a generalized tonic seizure for 5 min, accompanied by renal hypertension. Brain magnetic resonance imaging revealed posterior reversible leukoencephalopathy syndrome. After steroid pulse therapy, a renal biopsy was performed because of delayed recovery from thrombocytopenia. The biopsy findings showed features of thrombotic microangiopathic hemolysis with fibrinoid change restricted. Current diagnostic criteria for TMA have focused on thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, and diagnosis is based on the clinical presentation and etiology, with the consequence that idiopathic and atypical forms of TMA can be overlooked. Developing effective tools to diagnose TMA, such as studying levels of ADAMTS13 or testing for abnormalities in the complement system, will be the first step to improving patient outcomes.

• Journal of Yeungnam Medical Science
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• v.4 no.2
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• pp.165-172
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• 1987

The term histiocytic medullary reticulosis first was introduced by Scott and Robb-Smith. It is a clinicopathologic symdrome characterized by wasting, fever, lymphadenopathy, hepatosplenomegaly, pancytopenia, and is often accompanied by jaundice, purpura. Cardinal pathologic features are systemized proliferation of atypical, neoplastic, erytherophagocytic histiocyte. We are here reporting one case which considered compatible for HMR, with a few elementary reviewed literatures.

• Journal of the korean academy of Pediatric Dentistry
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• v.24 no.1
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• pp.293-299
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• 1997

Wiskott-Aldrich syndrome is a rare, hereditary disease occurring in males and was first described in 1937. It is characterized by cutaneous eczema, thrombocytopenic purpura and an increased susceptibility to infection due to an immunologic defect. Patients with Wiskott-Aldrich syndrome have a poor antibody response to polysaccharide antigens, low levels of IgM and high levels of IgA and IgE in serum. Oral manifestations of Wiskott-Aldrich syndrome was observed a spontaneous gingival bleeding, palatal petechiae, ulcer and gingival hyperplasia. We report on dental treatment of a 5 years old boy with severe spontaneous gingival bleeding and ulcer suffered from Wiskott-Aldrich syndrome. Prophylactic antibiotics to prevent infection and all potential measures including platelet concentrate therapy to prevent postoperative bleeding should be undertaken. Good oral hygiene should be maintained for prevention of infection by oral normal flora.

• Advances in pediatric surgery
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• v.5 no.1
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• pp.53-57
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• 1999

Pediatric laparoscopic splenectomy has been gradually accepted as the surgical management of a various splenic disorders, particularly in hematologic diseases. We report our experience with 16 patients who underwent this procedure because of hematologic disorders during the past 3 and a half years at the Department of Surgery, St. Mary's Hospital, the Catholic University Medical College. The mean age was 10 years(range 6-16 years) and the mean spleen weight was 210 gm(range 85-500 g). The indication for splenectomy were hereditary spherocytosis(6 cases), idiopathic thrombocytopenic purpura(8 cases), autoimmune hemolytic anemia(1 case), and idiopathic splenomegaly(1 case). All splenectomies were performed safely with mean estimated blood loss of 233 ml. Mean operative time and mean postoperative hospital stay were 157 min and 4.5 days, respectively. Postoperative pain, medication was needed in 3 cases, just one injection in immediate postoperative period. Diet was started on the second or third postoperative day. In conclusion, laparoscopic splenectomy in pediatric patients is a safe procedure, offering a small of abdominal scar, much less pain, a shorter hospital stay and car the lower postoperative morbidity.