While the incidence of nephrotic syndrome (NS) is decreasing in Korea, the morbidity of difficult-to-treat NS is significant. Efforts to minimize treatment toxicity showed that prolonged treatment after an initial treatment for 2-3 months with glucocorticosteroids was not effective in reducing frequent relapses. For steroid-dependent NS, rituximab, a monoclonal antibody against the CD20 antigen on B cells, was proven to... |
PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome is one of the causes of periodic fever in pediatrics with unknown etiology. It is characterized by abrupt onset of fever, malaise, aphthous stomatitis, pharyngitis and cervical adenitis without long-term sequelae. Laboratory findings of this sporadic and nonhereditary syndrome are so non-specific that the diagnosis is based on clinical findings. Oral... |
The major pathogenesis of acute respiratory distress syndrome (ARDS) is an inflammatory process that results from a diversity of injuries to the body. Due to the various cytokines and vasoactive peptides released from the endothelium, the vascular permeability is increased; the migration of inflammatory cells and the leakage of plasma proteins then occur and edema develops in the alveolus. There... |
Purpose : Kawasaki disease is the most common cause of systemic vasculitis in children less than 5 years of age. Recent immunohistochemistry findings suggest that many vascular growth factors play a role in the formation of the coronary artery lesions. Active remodeling of the coronary artery lesions in Kawasaki disease continues in the form of intimal proliferation and neoangiogenesis for... |
Castleman's disease is a rare non-neoplastic lymphoproliferative disorder of unknown etiology. It is divided into three histologic subtypes; hyaline-vascular(HV), plasma cell(PC) type and mixed type (HV-PC). It has two clinical expressions. The localized form, which presents as a slow growing mass, has a relatively benign clinical course. The multicentric form is multilocated and holds significant morbidity. The mainstay of treatment... |
Purpose : Kawasaki disease(KD) manifests a systemic vasculitis of unknown etiology in young children. Vascular endothelial growth factor(VEGF), vascular cell adhesion molecule-1(VCAM-1) and interleukin-1 beta(IL-1β) may play important roles in the pathogenesis of KD. Intravenous immunoglobulin(IVIG) and methylprednisolone(MP) are therapeutically effective for KD, however, the precise mechanisms of the two drugs are still unknown. We investigated the therapeutic efficacy of... |
Purpose : Interstitial lung disease(ILD) is a rare and poorly characterized disorder in children with poor prognosis. To understand the ILD in children, we reviewed our experience with 21 patients who were diagnosed interstitial lung disease during 9-year period at Asan Medical Center retrospectively. Methods : Severity-of-illness score was measured by the Denver protocol. We evaluated underlying diseases, clinical manifestations, high resolution computed tomography findings,... |
Protein-losing enteropathy can result from primary or secondary intestinal lymphangiectasia. Secondary intestinal lymphangiectasia develops as a result of lymphatic obstruction or elevated lymphatic pressure. Cardiac lesions such as constrictive pericarditis, post-Fontan procedure state, and chronic congestive heart failure increase lymphatic pressure and may cause intestinal lymphangiectasia and protein-losing enteropathy. An 11-year-old girl who underwent corrective surgery for Tetralogy of Fallot... |
Transverse myelitis is a rare complication of systemic lupus erythematosus(SLE) and its prognosis is very poor including death or severe neurologic sequelae. We report a 14-year-old girl with transverse myelitis who was not exactly diagnosed as SLE before the onset of neurologic symptoms. Transverse myelitis was diagnosed based on the clinical presentations, cerebrospinal fluid analysis and MRI findings. We employed aggressive treatment with pulse... |
Purpose : To evaluate the efficacy of steroid therapy on prevention of development and progression of coronary artery aneurysm in intravenous immune globulin(IVIG)-resistant Kawasaki disease, we treated three children with high dose of intravenous methylprednisolone followed by low dose oral prednisolone. Methods : We selected three children with Kawasaki disease who did not repond or who initially responded but soon developed recrudescent fever after retreatment... |
Methylprednisolone(MP) is administered by means of intravenous pulse therapy especially in the patients with focal segmental glomerulosclerosis(FSGS). There have been reports of its complications in a few increasing frequency. We experienced three cases of cardiaccomplications during intravenous pulsetherapy.Therewere onecaseof2¡ÆA-V block(Mobitztype I) and two cases of bradycardia and hypotension. In the former,2¡ÆA-V block in the first case deveoloped 45 hours after... |
Purpose : Infantile spasms are considered malignant epilepsy of infancy. Primary objectives of treatment are complete control of seizure attack and prevention of further brain damage. The aim of this study is to assess prednisolone(PDL) efficacy in infantile spasms. Methods : From June 1985 to July 1994, 20 children with infantile spasms who were diagnosed at Kyung Hee University Hospital and were... |
We experienced a case of idiopathic crescentic glomerulonephritis in a 10-year-old girl who was admitted to our hospital due to gross hematuria and oliguria for 2 months. The diagnosis was based on the rapidly progressive clinical course to chronic renal failure, positive p-ANCA test and light microscopic findings of diffuse crescents formation in about 80% glomeruli of renal tissue obtained... |
Purpose : It is well known that use of intranenous methylprednisolone(IVMP)for the patients with idiopathic thrombocytopenic purpura(ITP) is not only as effective as use of intravenous gammaglobulin to mincrease platelets quickly, but also the former is less expensive than the latter. As the adequate dosage of IVMP has not been fully elucidated, we have tried to determine it. Methods : The... |
The major goal in the treatment of acute idiopathic thrombocytopenic purpura (ITP) is to increase the platelet count above the critical level of 20,000/mm3 to avoid intracranial hemorrhage. We have treated eighteen children with acute ITP whose platelet count were below 20,000/mm3 at initial diagnosis. Thirteen of 18 patients (group A) received IV methylprednisolone (IVMP) 10〜20 mg/kg/day for 5 days from the day of... |
We experienced a case of aplastic anemia following hepatitis in a 4-year-old girl. Pancytopenia was developed during the convalescent period of hepatitis, and it was getting worse, while serial data of liver function test showed improvement. We tried a methylprednisolone pulse therapy, and about 7 months after treatment the CBC findings returned completely normal. She has enjoyed her healthy life during follow-up of over 2... |
Methylprednisolone “pulse” therapy was performed on 22 patients of childhood nephropathies who showed resistance to conventional therapy. The diseases included steroid-resistant or frequent relaps- ing nephrotic syndrome including minimal change nephrotic syndrome, focal segmental glomer- ulosclerosis, membranous nephropathy, mesangioproliferative glomerulonephritis, Henoch-Schonlein purpura nephritis and SLE nephritis. Methylprednisolone 30 mg/kg/day was administered intravenously over one hour, which was repeated 6 times on every other day. Sixteen of... |
We experienced 17 pediatric patients of glomerulonephritis with crescents in 25% or more of glomeruli, who were admitted to Department of Pediatrics, Seoul National University Hospital, from Jan. ’79 to Dec. ’84. A review of clinical and pathologic findings revealed as follows; 1)The 17 patients were 10 cases of Henoch-Schdnlein nephritis, 3 cases of idiopathic nephrotic syndrome which were due... |
The authors presented a 17 months old female with malignant osteopetrosis, who was admitted to the Pediatric Department of Kyung Hee University Hospital on May, 1981 because of epistaxis and growth retardation. Diagnosis was made by typical clinical manifestations, hematologic and radiologic findings, such as frontal bossing, opened anterior fontanel, exophalmoses, strabismus, optic atropy, marked hepatosplenomegaly, severe anemia, thrombocytopenia, reticulocytosis... |
Chronic active hepatitis (CAH) is defined as ongoing inflammation within the liver sustained beyond the expected time of resolution. The etiology of CAH is not completely understood, but hepatitis B infection, drugs such as oxyphenisatin, isoniazid, and alpha-methyldopa, and altered immunity are considered. The most common findings are jaundice, hepatosplenomegaly, elevated serum transaminase activities, hypergammaglobulinemia, and positive "autoimmune" serologic tests,... |