· Systemic autoinflammatory disorders (SAID) are disorders caused by dysregulation of the innate immunity with genetic background, leading to recurrent episodes of systemic inflammation. · SAID is characterized by recurrent acute inflammatory responses including fever or skin manifestations, unrelated with infection or malignancy. · Diagnosis is based on family and long-term history with detailed clinical and laboratory manifestations during febrile periods. |
Recurrent macrophage activation syndrome (MAS) is very rare. We present the case of an adolescent boy with human leukocyte antigen (HLA) B27-positive ankylosing spondylitis (AS), who experienced episodes of recurrent MAS since he was a toddler. A 16-year-old boy was admitted because of remittent fever with pancytopenia and splenomegaly after surgical intervention for an intractable perianal abscess. He had been... |
Varicella-zoster virus infection can lead to severe illness in immunocompromised patients. Further the mortality rate of disseminated varicella infection is extremely high particularly in immunocompromised children. We report a case of disseminated varicella infection in a child with acute lymphoblastic leukemia who was receiving chemotherapy, but was initially admitted with only for acute abdominal pain. The patient rapidly developed severe... |
Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosuppressive agents, and immunoglobulins for managing the disease and controlling hemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia and... |
Pyogenic liver abscess (PLA) is rare in healthy children. We report a case of PLA in an immunocompetent 12-year-old boy. Percutaneous catheter drainage was performed for the abscess. In addition, parenteral antibiotics were administered for 3 weeks. |
Wiskott-Aldrich syndrome (WAS) is an inherited X-linked disorder. The |
Enterovirus 71, one of the enteroviruses that are responsible for both hand-foot-and-mouth disease and herpangina, can cause neural injury. During periods of endemic spread of hand-foot-andmouth disease caused by enterovirus 71, CNS infections are also frequently diagnosed and may lead to increased complications from neural injury, as well as death. We present the results of our epidemiologic research on the... |
Idiopathic pulmonary hemosiderosis (IPH) is a rare disease affecting mostly children. This disorder is characterized by recurrent episodes of hemoptysis, bilateral diffuse pulmonary infiltrates, and iron-deficiency anemia. An acute fulminant alveolar hemorrhage can be fatal due to respiratory failure, while chronic hemorrhage leads to hemosiderin-laden macrophages and pulmonary fibrosis. Genetic, autoimmune, allergic, environmental, and metabolic mechanisms of pathogenesis have been... |
Purpose : This study aimed to determine the frequencies of CD4+CD25+ T cells in donor graft and peripheral blood CD4+CD25+ T cells in recipients after hematopoietic stem cell transplantation (HSCT) and their association with graft-versus-host disease (GVHD). Methods : Seventeen children who underwent HSCT were investigated. CD4+CD25+ T cells in samples from donor grafts and recipient peripheral blood were assessed... |
Purpose : To investigate the discriminative ability of pediatric index of mortality 2 (PIM2) and pediatric risk of mortality III (PRISM III) in predicting mortality in children admitted into the intensive care unit (ICU). Methods : We retrospectively analyzed variables of PIM2 and PRISM III based on medical records with children cared for in a single hospital ICU from January 2003... |
Purpose : In this study, we retrospectively analyzed the clinical outcomes of patients who underwent allogeneic hematopoietic stem cell transplantation (HSCT) grafted from HLA-matched parents. Methods : Seven children with acute leukemia (4 acute lymphoblastic leukemia, 3 acute myeloid leukemia) in first complete remission received allogeneic HSCT from their respective parents at the St. Marys Hospital between April, 1999 and October,... |
Purpose : Due to its high potency against leukemic blasts, our institution has opted for the use of dexamethasone during acute lymphoblastic leukemia (ALL) remission induction, but in our most recent treatment protocol, CMCPL-2005, we shortened the length of steroid treatment from 4 to 3 weeks. We compared both the rates of remission induction and significant complications observed during induction... |
Purpose : While pediatric observation units (POU) have become a common practice in hospitals throughout developed countries, there has been no report about POUs in Korea so far. The aims of this study were to analyze our one-year's experience of the POU and to decide which disease entities are suitable for the POU. Methods : All children admitted from... |
Allogeneic organ or hematopoietic stem cell transplantation(HSCT) is the treatment of choice for end-stage organ diseases or various hematologic disorders. The induction of alloantigen specific T cell tolerance and its maintenance are critical for preventing immune responses, including graft rejection or graft-versus-host disease(GVHD) in allogeneic transplantation. CD4+ T cells are classified as immune functions : Th1 CD4+ cells for cellular... |
Purpose : Colonic polyps are among the most common causes of rectal bleeding in children. We studied the clinical spectrum, histology, malignant potential and treatment of colonic polyps in our cases. Methods : We reviewed hospital charts of all patients with colonic polyps, seen over a 15-year period from January 1988 to November 2002. Colonoscopy and/or barium enema were done for... |
Tuberous sclerosis(TS) is known to be associated with multi-organ involvements, such as neurologic, renal and cardiac abnormalities. Abdominal aortic aneurysms in children and young adults are uncommon, however, some have been observed in patients with TS. We present an 8 month-old female patient with TS who developed aortic aneurysm and underwent successful surgical management. Since an aortic aneurysm is potentially... |
Median cleft face syndrome is diagnosed by two or more of the following anomalies : ocular hypertelorism, cranium bifidum occultum, median cleft nose, median cleft lip and median cleft premaxilla. The most consistent and prominent ocular finding associated with this syndrome is hypertelorism. We experienced a case of median cleft face syndrome which had orbital hypertelorism, cranium bifidum occultum, bifid... |
Purpose : We reviewed 100 cases of HLA-matched sibling allogeneic bone marrow transplantation( allo-BMT) in children and wish to share these results. Methods : One hundred children had undergone allo-BMT from HLA-identical siblings between Nov. 1983 and May 1998. There were 50 males and 50 females with a median age of 10 years and a median follow-up of 38 months. Out of 100 cases,... |
Wolf-Hirschhorn syndrome is caused by a partial loss of the distal short arm of chromosome 4. Characteristic clinical features are severe growth retardation, mental retardation, seizures, congenital cardiac defects, urogenital abnormalities, microcephaly, hypertelorism, prominent glabella, cleft lip and palate and micrognathia. In 87% of cases, chromosome 4 deletion arises as a de novo event, whereas in the remaining cases it... |
Twenty, six children younger than 15 years with severe aplastic anemia underwent either bone marrow transplantation or immunomodulation therapy (antilymphocyte globulin with cyclosporin A therapy). Six patients who had an HLA-identical sibling donor underwent bone marrow transplantation after conditioning with cyclophosphamide, procarbazine and antilymphocyte globulin. Twenty children who did not have an HLA-identical donor recieved antilymphocyte globulin and cyclosporin A. The results were as follows; 1) Hematologic responses:... |