• Childhood Kidney Diseases
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• v.27 no.2
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• pp.55-63
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• 2023

In 2021, a new chapter on the general management of glomerulonephritis (GN) was added to the Kidney Disease: Improving Global Outcomes (KDIGO). It emphasizes the importance of early general management of GN for improving long-term kidney outcomes and prognosis. The chapter introduces the management of glomerular diseases in 18 subchapters. Here, kidney biopsy for the diagnosis and evaluation of kidney function and the management of complications, such as hypertension, infection, and thrombosis, are presented. Moreover, the adverse effects of glucocorticoids and immunosuppressive therapy, which are commonly used drugs for glomerular disease, are mentioned, and a guideline for drug selection is presented. Each subtheme focused on items reflecting the interpretation of the "practice points" of the expert working group are introduced. In this review of the general treatment for GN in the KDIGO guidelines, excluding pregnancy and reproductive health, we focused on and compared various references pertaining to pediatric GN management.

• Childhood Kidney Diseases
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• v.19 no.1
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• pp.23-30
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• 2015

NPHP is the most common monogenic cause of CKD in children or adolescents. Extra-renal symptoms often accompany, therefore examination of retina, hearing, and skeleton is necessary in patients with CKD with insidious onset. Genes involved in NPHP-RC are mostly related in primary cilia. While genetic diagnosis is necessary for definitive diagnosis, there is no curative treatment.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.225-230
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• 2001

Multilocular cystic nephroma is a rare disease, noninherited benign renal neoplasm occurring in both children and adults. It is necessary to make a differential diagnosis from all renal diseases with a cystic component, such as Wilms tumor, harmatoma or polycystic dysplastic kidney in childhood. There are about only 200 case reports in the world since Walter Edmunds had described it first. We report a case of multilocular cystic nephroma presented with painless abdominal mass, treated with nephrectomy and confirmed with pathology. (J. Korean Soc Pediatr Nephrol 2001 ;5 : 219-24)

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.144-150
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• 1997

Purpose : The kidney is one of the most common sites of cyst formation. Cystic diseases of the kidney are a diverse group of clinicopathologic entities and variable prognosis. They span a wide range of both age of presentation and severity of the renal disease. And many of them are systemic disorders, sharing similar process of cyst formation in other organs. Recently, development of imaging studies has been contributing widely to the diagnosis of the diseases. Treatment, however, is not established satisfactorily. We performed this study to evaluate the occurrence and treatment of cystic diseases of the kidney. Methods : We reviewed retrospectively the medical records of 44 patients with cystic diseases of the kidney in the Department of Pediatrics, during last 11 years. Results : In the 44 patients with cystic diseases of the kidney, 31 patients(71%) had multicystic dysplastic kidney and 11(35%) of them received nephrectomy due to differentiation from neoplasms or severe abdominal distension. Seven patients(16%) had polycystic kidney disease, and all of them were infantile type. Five patients(11%) were diagnosed as having a simple renal cysts. Progression to renal failure was noted in none of the cases. In 14(32%) out of total 44 patients, the diagnosis was made in neonatal or infantile pelted. Conclusion : The incidence of cystic diseases of the kidney appeared very low, but further investigation on their pathogenesis, classification, and indication of treatment is needed.

• Childhood Kidney Diseases
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• v.25 no.1
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• pp.14-21
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• 2021

Chronic kidney disease, the presence of structural and functional abnormalities in the kidneys, is associated with a lower quality of life and increased morbidity and mortality in children. Genetic etiologies account for a substantial proportion of pediatric chronic kidney disease. With recent advances in genetic testing techniques, an increasing number of genetic causes of kidney disease continue to be found. Genetic testing is recommended in children with steroid-resistant nephrotic syndrome, congenital malformations of the kidney and urinary tract, cystic disease, or kidney disease with extrarenal manifestations. Diagnostic yields differ according to the category of clinical diagnosis and the choice of test. Here, we review the characteristics of genetic testing modalities and the implications of genetic testing in clinical genetic diagnostics.

• Genomics & Informatics
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• v.11 no.4
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• pp.224-229
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• 2013

Receptor for advanced glycation endproducts (RAGE) is a multi-ligand receptor that is able to bind several different ligands, including advanced glycation endproducts, high-mobility group protein (B)1 (HMGB1), S-100 calcium-binding protein, amyloid-${\beta}$-protein, Mac-1, and phosphatidylserine. Its interaction is engaged in critical cellular processes, such as inflammation, proliferation, apoptosis, autophagy, and migration, and dysregulation of RAGE and its ligands leads to the development of numerous human diseases. In this review, we summarize the signaling pathways regulated by RAGE and its ligands identified up to date and demonstrate the effects of hyper-activation of RAGE signals on human diseases, focused mainly on renal disorders. Finally, we propose that RAGE and its ligands are the potential targets for the diagnosis, monitoring, and treatment of numerous renal diseases.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.31-37
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• 1997

The DMSA scan is a useful radiologic study in diagnosis of morphologic and functional diseases of kidney. We evaluated the distribution of sex and age, clinical manifestations, diagnosis, combined diseases, treatment and prognosis of the 61 patients with non-functioning kidney(no isotope uptake or uptake below 5% in DMSA scan) who admitted in our hospital from 1980 to 1995. The proportion of patients under 1 year old age was 46%. Sex ratio was 1.4:1 with male predominance. Most diagnosis of non-functioning kidneys were congenital such as multicystic dysplastic kidney, hydronephrosis due to ureteropelvic junction obstruction, renal agenesis and renal hypoplasia. In order of frequency thirty one percent of them were previously detected on antenatal ultrasonogram. Treatment consisted of operation in 47.5%, mostly nephrectomy and 32.8% of patients were followed up at OPD base without definite treatment. The most common combined diseases was hydronephrosis, in 4patients who had both kidneys inveloved progressed to chronic renal failure, but the prognosis in most cases were good. It is important to evaluate renal diseases in perinatal periods, and we believe that highly sensitive diagnostic study contribute to early treatment plan and thus to good prognosis.

• Journal of Yeungnam Medical Science
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• v.32 no.2
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• pp.155-158
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• 2015

Lymphangioma is a congenital abnormality of the lymphatic system detected primarily in early childhood. There are rare reports of mediastinal lymphangioma in older adults. We hereby report on a 66-year-old female patient who underwent kidney transplantation 20 years previously and who developed pathologically confirmed solitary mediastinal lymphangioma 1 year ago. Chest radiography showed a mediastinal nodule, which was not observed 2 year previously, therefore she was referred to the pulmonary division. She had no symptoms, and chest computed tomography demonstrated a 25-mm, well-defined, low-density nodule located at the anterior mediastinum. The size of the nodule had increased from 25 mm to 34 mm 1 year later, and it was completely resected via video-assisted thoracic surgery. The histological diagnosis was cystic lymphangioma. Therefore, we recommend that clinicians consider cystic lymphangioma as a possible diagnosis even in older patients with a mediastinal cystic mass that shows progressive enlargement.

• The Journal of Korean Institute of Communications and Information Sciences
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• v.35 no.7B
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• pp.1066-1072
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• 2010

Kidney diseases rates in modern Korean society, which is entering an aging society, are growing. These diseases diagnosed at early stage can be cured with the drug or dietary treatment. If, however, at worsening stage, kidney dialysis or transplant is needed. In this context, we analyzed the relationship between kidney and voice by measuring and analyzing the changing rate of the vocal chord and intensity according to hemodialysis. For this, we collected voice samples before and after hemodialysis, and compared and analyzed them through application of vocal analytical element. Namely, based on oriental medical diagnosis theory that voice is quieted down when kidney is abnormal, we collected voice samples before and after hemodialysis, and applied trembling and intensity analysis of vocal chord.

• Childhood Kidney Diseases
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• v.17 no.1
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• pp.25-28
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• 2013

There have only been 35 pediatric cases and one adult case reported on segmental multicystic dysplastic kidney (MCDK) from our search in PubMed, including 19 cases detected antenatally. There is little documentation of segmental MCDK, particularly concerning its natural history. Segmental MCDK can be presented atypically, making diagnosis more difficult. We report an another case with segmental MCDK. Multicystic abdominal mass detected on antenatal sonogram in this infant was diagnosed as segmental MCDK by renal ultrasonography and computed tomography. If a definitive diagnosis of segmental MCDK can be made on imaging, surgery is not required for a diagnostic biopsy.