• Childhood Kidney Diseases
• /
• v.4 no.1
• /
• pp.48-56
• /
• 2000

Purpose: Alport SD., the most common herectitary rephriris, is a renal disease with rapid progression. Deafness, ocular abnormalities and a specific EM finding may be associated in addition to a family history. We have aralyged retrospectively. Methods: We observed 12 children with Alport syndrome who were diagnosed at Dept. of pediatrics in Kyunghee Univ., College of Medicine, from Apr. 1991 until Jun. 1999. We used four criteria for diagnosis: renal disease, family history, deafness or eye abnormalities, and a specific finding in electron microscopy Results: 2 of 12 patients had all features of the four diagnostic criteria. We could not trace an exact family history in 3 patients, and 6 patients did not exhibit deafness or eye abnormality. One could not have renal biopsy because offer chronic renal failure. Other three criteria were observed in her. The ratio of male to female observed was 1:2 respectively and the mean age of initial renal symptom was 5.6 years. 9 of 12 patients had a family history of renal disease. In the audiogram and ocular examination for 11 of 12 cases, sensorineural hearing loss was observed in 6 and ocular abnormality in 2 cases. In electron microscopic finding, irregular thickness of the capillary basement membranes with lamination of lamina densa and foot process obliteration was noted in 9 of 11 and thin basement membrane with splitting and foot process obliteration was noted in the other 2. The mean period of follow-up was 3 6/12 years. And one patient developed the chronic renal failure until now and had kidney transplantation. Conclusion: For the diagnosis of Alport syndrome, the following four diagnostic criteria are very important : renal disease, family history, deafness or eye abnormalities, and a specific finding on electron microscopy. We expect that more patients can be detected through the analysis of these characteristics.

• Childhood Kidney Diseases
• /
• v.8 no.1
• /
• pp.1-9
• /
• 2004

Purpose : Minimal Change Disease (MCD) is the most common primary nephrotic syndrome in children. Some suggested that tumor necrosis $factor-{\alpha}$ ($TNF-{\alpha}$) are involved in the pathogenesis of MCD. Methods : This study was done to see the changes of plasma and urinary $TNF-{\alpha}$, and its effect on the determination of permeability of the glomerular basement membrane (BM) contributed by heparan sulfate proteoglycan (HSPG). Study patients consisted of 19 biopsy-proven MCD children aged 2-15 years old. Both plasma and urinary $TNF-{\alpha}$ were measured. Employing the Millicell system, $TNF-{\alpha}$ was screened for the permeability factors. We examined whether $TNF-{\alpha}$ regulated BM HSPG gene expression and HS synthesis in the glomerular epithelial cells (GECs). Results : Urinary $TNF-{\alpha}$ during relapse was significantly increased when compared with that of during remission or controls ($364.4{\pm}51.2$ vs $155.3{\pm}20.8,\;36.0{\pm}4.5$ ng/mg cr) (P<0.05). However, negative results were obtained in the permeability assay using the Millicell system. No difference was seen in the BM HSPG gene expression and HS synthesis in the GECs. Conclusion : It seems that $TNF-{\alpha}$ may not play a disease-specific role in the pathogenesis of MCD.

• Childhood Kidney Diseases
• /
• v.9 no.1
• /
• pp.91-96
• /
• 2005

Cyclosporin A-induced central neurotoxicity has been rarely reported in patients with nephrotic syndrome. We report a pediatric patient who developed acute leukoencephalopathy diagnosed by MRI during CsA therapy for nephrotic syndrome.

• Childhood Kidney Diseases
• /
• v.6 no.1
• /
• pp.15-24
• /
• 2002

• 대한불안의학회:학술대회논문집
• /
• 2004.06a
• /
• pp.157-170
• /
• 2004

• 대한불안의학회:학술대회논문집
• /
• 2003.11a
• /
• pp.66-72
• /
• 2003

• 학교보건
• /
• s.26
• /
• pp.53-63
• /
• 2006

• 학교보건
• /
• s.26
• /
• pp.67-73
• /
• 2006

• The Journal of the Korea Contents Association
• /
• v.12 no.12
• /
• pp.304-312
• /
• 2012

In this study, for behavior clinical signs and blood tests when autumn sudden fevered disease, Tsutsugamushi Disease is Single infected with mixing hemorrhagic fever with renal syndrom.221 positive patients, who have antibody titer more than 1:80 in serologic examination of Tsutsugamushi Disease in a general hospital, Jeollanam-do province, were adopted as a Analysis. in respect of frequency. Single infection patients of Tsutsugamushi Disease were 183 and mixed infection patients with Hemorrhagic Fever with Renal Syndrome were 38. In respect of age, patients over the 70's were mostly infected. In respect of sex, male patients were 70(31.7%) and female patients were 151(66.8%) Women were more infected than men. In respect of outbreak time, the more than 70% disease occurrence of all was charged in October and November and it also occurred in spring. In the clinical signs, fever(p=0.028), urtication(p=0.000) and muscle pain(p=0.000) of mixed infection was more higher than single infection. And in the blood tests, AST(p=0.000), ALT(p=0.000), blood urea nitrogen(p=0.002), total bilirubin(p=0.000) value of mixed infection was more higher than single infection.

• Childhood Kidney Diseases
• /
• v.18 no.2
• /
• pp.123-127
• /
• 2014

Erythema multiforme (EM) is an acute mucocutaneous disorder involving the skin, mouth, eyes, and genital organs. It is classified into EM minor and EM major according to the involvement of the mucosal membrane. Stevens-Johnson syndrome (SJS) and toxic epidermal necrosis (TEN) belong to EM major. Compared to EM minor, SJS presents with more severe and progressive symptoms, and has a higher mortality rate. Corticosteroids are used in the treatment of EM. We report three cases of EM (two cases of EM minor and one case of SJS) that developed during treatment with oral corticosteroid (deflazacort; $Calcort^{(R)}$) in children with nephrotic syndrome.