• Childhood Kidney Diseases
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• v.14 no.2
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• pp.120-131
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• 2010

Renal tubular acidosis (RTA) is a metabolic acidosis due to impaired excretion of hydrogen ion, or reabsorption of bicarbonate, or both by the kidney. These renal tubular abnormalities can occur as an inherited disease or can result from other disorders or toxins that affect the renal tubules. Disorders of bicarbonate reclamation by the proximal tubule are classified as proximal RTA, whereas disorders resulting from a primary defect in distal tubular net hydrogen secretion or from a reduced buffer trapping in the tubular lumen are called distal RTA. Hyperkalemic RTA may occur as a result of aldosterone deficiency or tubular insensitivity to its effects. The clinical classification of renal tubular acidosis has been correlated with our current physiological model of how the nephron excretes acid, and this has facilitated genetic studies that have identified mutations in several genes encoding acid and base ion transporters. Growth retardation is a consistent feature of RTA in infants. Identification and correction of acidosis are important in preventing symptoms and guide approved genetic counseling and testing.

• Childhood Kidney Diseases
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• v.27 no.2
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• pp.117-120
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• 2023

Here, we present the case of a 2-month-old male infant with hyponatremic hypertensive syndrome resulting from stenosis of the right proximal and mid-renal arteries. The patient exhibited nephrotic-range proteinuria, low serum albumin, increased serum creatinine, and elevated renin and aldosterone levels. Doppler ultrasonography and computed tomography angiography revealed decreased vascular flow in the small right renal artery. Following a successful percutaneous balloon angioplasty, the patient experienced a decrease in blood pressure and normalization of serum electrolyte levels within a few days. However, it took 3 months for the proteinuria to resolve completely. This case is significant as it represents the first reported instance of a neonate presenting with clinical features resembling congenital nephrotic syndrome caused by renal artery stenosis that was successfully treated with percutaneous renal angioplasty.

• The Korean Journal of Physiology and Pharmacology
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• v.1 no.1
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• pp.45-53
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• 1997

In humans and many animal models with chronic progressive renal diseases, angiotensin-converting enzyme (ACE) inhibitor markedly attenuates the progression of nephropathy. Several studies have reported augmented gene expression and redistribution of renal renin in partial nephrectomized rats. Although precise mechanism(s) is not known, the renin-angiotensin system (RAS) may play an important role in the progression of renal diseases. Thus, this study was undertaken to examine the gene expression of renal renin, angiotensinogen, and $AT_1$ subtypes ($AT_{1A}$ and $AT_{1B}$) in rats with diabetic nephropathy, and the influences of lipopolysaccharide (LPS)-induced septicemia on the gene expression. Four weeks after streptozotocin (STZ) treatment (55 mg/kg, i.p.), rats were randomly divided into LPS-treated (1.6 mg/kg, i.p.) and control rats. At 6 hours after LPS treatment, the rats were killed and the kidney was removed from each rat. Northern blot and reverse transcription-polymerase chain reaction (RT-PCR)techniques were used to detect mRNA expression. STZ treatment markedly attenuated body weight gain and significantly increased blood glucose level. Renal renin content (RRC) was significantly decreased in the STZ-treated rats compared to that in control rats. The renal ACE activity between STZ-treated and control rats was not significantly different. Renal renin mRNA level was prominently increased, while angiotensinogen and $AT_{1A}$ mRNA levels were slightly decreased in STZ-treated rats compared to those in controls. $AT_1$B mRNA level did not differ in both groups. Acute LPS treatment did not show any significant changes of mRNA levels of intrarenal RAS components in both groups. These results suggest that intrarenal RAS components were differentially regulated in STZ-treated diabetic rats. Further studies are required to evaluate the relationship between intrarenal RAS and other vasomodulatory systems.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.243-250
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• 2002

Amyloidosis comprises a diverse group of systemic and local diseases characterized by organ involvement by the extracellular deposition of fibrils composed of subunits of a variety of normal serum proteins. Secondary amyloidosis is caused by the deposition of amyloid A(AA) protein in chronic inflammatory disease. Juvenile rheumatoid arthritis(JRA) has been known to be the most common cause of secondary amyloidosis. We experienced one case of secondary renal amyloidosis in a 12-year-old girl who had suffered from JRA for several years who had visited our renal clinic to evaluate the proteinuria with microscopic hematuria which was detected by chance at school urine screening examination. Apple green birefringence was observed under polarized light with Congo red stain at)d characteristic electron microscopic findings was also noted in renal tissues which was obtained by percutaneous renal biopsy. In our knowledge, this is the first case report of secondary renal amyloidosis developed in pediatric age in Korea.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.94-99
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• 2010

Obesity-related glomerulopathy (ORG) is a secondary form of focal and segmental glomerulosclerosis (FSGS) manifesting as proteinuria and progressive renal dysfunction that results from maladaptive glomerular response to increasing adiposity. Reports of ORG progressing to end stage renal diseases in rare in the pediatric population. We report a 9-year-old boy with obesity (body mass index $35\;kg/m^2$) who was diagnosed with ORG presenting with proteinuria. He was diagnosed with obesity-related glomerulopathy based on the laboratory, urinary, and kidney biopsy finding. In spite of treatment with angiotensin- converting enzyme (ACE) inhibitor and/or, angiotensin-receptor blocking agent, the degree or amount of proteinuria increased and renal function declined continuously. His BMI did not decrease and eventually progressed to chronic renal failure. Consequently, obese patients should be monitored for proteinuria, which may be the first manifestation of FSGS, a lesion that may be associated with serious renal sequelae.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.1-7
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• 2006

Osteopontin (OPN) is a glycosylated phosphoprotein which mediates cell adhesion and migration, and is produced by bone, macrophages, endothelial cells, and epithelial cells. The many regulatory functions of OPN include bone remodeling, tumor invasion, wound repair, and promotion of cell survival. It is produced by renal tubular epithelial cells, and expression is upregulated in glomerulonephritis, hypertension, ischemic acute renal failure, renal ablation, and UUO. In this review, we discuss about osteopontin in general aspect, expression, role on the development and pathologic condition of neonatal kidney.

• Childhood Kidney Diseases
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• v.21 no.2
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• pp.47-52
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• 2017

Purpose: Renal ultrasonography has been widely used in children with renal disease. However, the relationship of renal echogenicity with renal pathology and function in children is not well known. Method: Ultrasound examination was performed in 75 patients undergoing renal biopsy for suspected renal disease in Konkuk University Medical Center from August 2005 to November 2015. We compared renal echogenicity to pathologic findings and renal function. Renal echogenicity was scored as 0 to 2 by comparing adjacent liver echogenicity. Three histologic characteristics were evaluated: glomerular changes, interstitial infiltration or fibrosis, and tubular atrophy. These were graded as 0 to 3, according to increasing severity. Laboratory results included urine albumin excretion and estimated glomerular filtration rate (eGFR). Results: Among pathologic findings, renal echogenicity revealed a positive correlation with interstitial infiltration or fibrosis (r=0.259, P=0.025), and with tubular atrophy (r=0.268, P=0.02). Renal echogenicity and glomerular changes were not correlated. Renal echogenicity showed a positive correlation with microalbuminuria (r=0.283, P=0.014), but a negative correlation with eGFR (r=-0.352, P=0.002). Conclusion: Increased renal echogenicity suggested severe interstitial infiltration or fibrosis and tubular atrophy among the pathologic findings. Moreover, increased echogenicity is correlated with increased urine albumin excretion and decreased eGFR. Echogenicity on ultrasonography is useful for determining the status of renal pathology and function.

• The Korean Journal of Nuclear Medicine
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• v.25 no.2
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• pp.227-236
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• 1991

Background: The evaluation of individual renal function is important to diagnosis and follow-up of various diseases. Ureteral catheterization of each kidney has been widely used for this purpose, but this method had some technical difficulty, frequent complications and much restriction in reapplication. Therefore we tried to applicate radiopharmaceuticals for the evaluation of individual renal function. Methods: We measured 2 hour, 4 hour and 24 hour relative renal uptake of $^{99m}Tc-DMSA$ and relative glomerular filteration rate of $^{99m}Tc-DTPA$ with 59 patients with various renal diseases to determine their usefulness for assessment of individual renal function and to compare correlations between every renal uptake of $^{99m}Tc-DMSA$ and relative glomerular filteration rate. Results: The correlations between 2 hour-, 4 hour- and 24 hour- relative renal uptake of $^{99m}Tc-DMSA$ and relative glomerular filteration rate of $^{99m}Tc-DTPA$ were R=0.9190 (p < 0.001), R: 0.9229 (p<0.001) and R=0.9917 (p<0.001). In acute obstructive uropathy, the correlations at 2 hour and 4 houre were poor as R=0.1812 (p<0.05) and R=0.4923 (p < 0.05), but the correlation at 24 hour was good as R=0.9942 (p<0.001). Conclusions: We concluded that relative renal uptake at 2 hour and 4 hour had good correlation with relative DTPA uptake ratio in the cases without chronic renal failure and obstructive uropathy. Delayed image with 24 hour relative renal uptake $^{99m}Tc-DTPA$ had the best correlation with relative glomerular filteration rate of $^{99m}Tc-DTPA$ and that might be useful in evaluation of chronic renal disease in which showed increased beckground activity or acute obstructive uropathy.

• The Journal of Korean Society of Virology
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• v.28 no.4
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• pp.377-382
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• 1998

Hemorrhagic fever with renal syndrome (HFRS), scrub typhus, murine typhus and leptospirosis have been the principal acute febrile diseases in Korea. To evaluate the seroepidemiologic patterns of acute febrile illness, sera collected from 2,423 patients in 1996 were examined for antibodies against Hantaan virus, Orientia tsutsugamushi, Rickettsia typhi, and Borrelia burgdorferi by indirect immunofluorescent antibody technique (IFA) and macroscopic agglutination test for Leptospira interogans. Seropositive cases against Otsutsugamushi, Rickettsia typhi, Leptospira interogans and Hantaan virus were 192 (7.9%), 193 (8.0%), 12 (0.5%) and 324 (13.4%), respectively. Male was more affected in HFRS and murine typhus contrasting to scrub typhus and leptospirosis in female. Most positive cases occurred during October and November for scrub typhus, and during November and December for HFRS. These results showed similar patterns with previous epidemical data for recent couple of years, and possibly implied no significant changes occurred in ecologic situations for acute febrile diseases in Korea.

• Journal of Preventive Medicine and Public Health
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• v.22 no.1 s.25
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• pp.45-50
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• 1989

The purpose of this study was to select the manageable diseases which are prevalent in Korea for periodic health examinations and to evaluate the sensitivity and specificity of periodic health examination in detecting these diseases. The data was derived from reimbursement data of the Korea Medical Insurance Corporation over a two year period (April 15, 1986 to April 14,1988) and interviews with Yonsei Medical School professors. This study demonstrated that: 1. The manageable diseases which were selected for periodic health examinations are pulmonary tuberculosis, viral hepatitis, diabetes mellitus, syphilis groups, essential hypertension, renal disease groups, and iron deficiency anemia. 2. The sensitivity of the health screening for each disease was as follows: pulmonary tuberculosis 51.5%, viral hepatitis 60.3%, diabetes mellitus 64.7%, syphilis groups 63.3%, essential hypertension 49.9%, renal disease groups 44.0%, and iron deficiency anemia 80.9%. We conclude that peiodic health esaminations should focus on the manageable diseases that we have defined. The number of items in periodic health screening tests and hospital quality control should be increased for the diseases with a demonstrated low sensitivity.