• Childhood Kidney Diseases
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• v.3 no.2
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• pp.217-220
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• 1999

Kimura disease is a chronic benign disorder, primarily seen in asians male during the second and third decades of life, which presents itself as a tumour like lesion with a predilection for the head and neck region. There is high prevalence of associated renal disease. We report two cases of nephrotic syndrome associated with Kimura disease, and this is the first report of Kimura disease with renal involvement in Korean children.

• Childhood Kidney Diseases
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• v.23 no.1
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• pp.7-21
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• 2019

Nephrotic syndrome (NS) is a common chronic glomerular disease in children characterized by significant proteinuria with resulting hypoalbuminemia, edema, and hyperlipidemia. Renal biopsy findings of diffuse foot processes effacement on electron microscopy and minimal change disease, focal segmental glomerulosclerosis (FSGS), or diffuse mesangial proliferation on light microscopy. It has been speculated that circulating permeability factors would be implicated in the pathogenesis of NS because they have been reportedly detected in the sera of patients and in experimental models of induced proteinuria. Moreover, a substantial portion of the patients with primary FSGS recurrence shortly after transplantation. This report reviews the current knowledge regarding the role of circulating permeability factors in the pathogenesis of proteinuria in NS and suggests future targeted therapeutic approaches for NS.

• Childhood Kidney Diseases
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• v.21 no.1
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• pp.35-39
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• 2017

Azathioprine is commonly used as immunosuppressive therapy for various inflammatory diseases including chronic glomerulonephritis. Myelosuppression is a common side effect of azathioprine, resulting in the need for dose reduction. However, severe pancytopenia or alopecia is not often encountered. Here, we report a case of severe myelosuppression, and alopecia totalis that occurred after azathioprine treatment in a patient with IgA nephropathy. A 10-year-old boy with IgA nephropathy was treated with oral deflazacort and later with azathioprine. After 4 weeks, the patient complained of hair loss, and despite a dose reduction in azathioprine, he developed bone marrow suppression and alopecia totalis in two weeks. The blood indices and alopecia of the patient had returned to normal after azathioprine withdrawal and 3 consecutive doses of granulocyte colony-stimulating factor. We suggest that physicians remain vigilant to the side effects of azathioprine. Unusual hair loss after azathioprine treatment might suggest a defect in the metabolism of the drug, warranting the discontinuation of azathioprine to prevent more severe side effects.

• Childhood Kidney Diseases
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• v.25 no.1
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• pp.35-39
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• 2021

Bartter syndrome is an autosomal recessive hypokalemic salt-losing tubulopathy, and classic Bartter syndrome is associated with mutations in the CLCNKB gene. While chronic hypokalemia is known to induce renal cyst formation in different renal diseases, renal cyst formation in Bartter syndrome is rarely reported. Russian six-year-old identical male twins were referred to our hospital for the evaluation of renal cysts, which were incidentally detected on abdominal sonography due to diarrhea. Both twins had shown symptoms of polydipsia, polyuria, and nocturia since they were one year olds. Vital signs including blood pressure were normal in both twins. Renal sonography revealed nephromegaly, increased echogenicity of renal cortex, and various sized multiple cysts in both kidneys for both twins. Laboratory findings included hyponatremia, hypokalemia, hypochloremia, and metabolic alkalosis. Bartter syndrome with renal cysts were suspected. Genetic analysis for both twins confirmed a homozygous c.1614delC deletion on exon 15 of the CLCNKB gene, which was confirmed as a previously unreported variant to the best of our knowledge. They were managed with potassium chloride, nonsteroidal anti-inflammatory drugs, and angiotensin-converting-enzyme inhibitors. Metabolic alkalosis, hypokalemia, hypochloremia, and polyuria partially improved during the short course of treatment. This is the first report of a homozygous mutation in the CLCNKB gene in an identical twin, presenting with renal cysts.

• Tuberculosis and Respiratory Diseases
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• v.82 no.4
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• pp.335-340
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• 2019

Background: Snoring is the cardinal symptom of obstructive sleep apnea (OSA). Snoring and upper airway obstruction associated with major oxygen desaturation may occur in populations undergoing flexible bronchoscopy. Methods: To evaluate the prevalence of patients at a high risk of having OSA among patients undergoing bronchoscopy with sedation and to investigate whether snoring during the procedure predicts patients who are at risk of OSA, we prospectively enrolled 517 consecutive patients who underwent the procedure with moderate sedation. Patients exhibiting audible snoring for any duration during the procedure were considered snorers. The STOP-Bang (Snoring, Tiredness, Observed apnea, high blood Pressure-Body mass index, Age, Neck circumference and Gender) questionnaire was used to identify patients at high (score ${\geq}3$ out of 8) or low risk (score <3) of OSA. Results: Of the 517 patients, 165 (31.9%) snored during bronchoscopy under sedation. The prevalence of a STOP-Bang score ${\geq}3$ was 61.9% (320/517), whereas 200 of the 352 nonsnorers (56.8%) and 120 of the 165 snorers (72.7%) had a STOP-Bang score ${\geq}3$ (p=0.001). In multivariable analysis, snoring during bronchoscopy was significantly associated with a STOP-Bang score ${\geq}3$ after adjustment for the presence of diabetes mellitus, chronic obstructive pulmonary disease, chronic kidney disease, and stroke (adjusted odds ratio, 1.91; 95% confidence interval, 1.26-2.89; p=0.002). Conclusion: Two-thirds of patients undergoing bronchoscopy with moderate sedation were at risk of OSA based on the screening questionnaire. Snoring during bronchoscopy was highly predictive of patients at high risk of OSA.

• Journal of fish pathology
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• v.30 no.2
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• pp.115-134
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• 2017

A total number of 668 apparently healthy fish were obtained from farm to study the effect of two heavy metals (Copper and Mercury) on histopathology of liver, kidney, spleen, gills and muscles also residues in muscles. The $LC_{50}$/96 hr. of Cu and Hg were estimated and fish exposed to 1/2 $LC_{50}$ for 7 days and for 1/10 $LC_{50}$ for 8 weeks from each product separately. Histopathological findings in acute and chronic mercuric chloride toxicity revealed degeneration and necrosis in the glomeruli, interstitium tissue and epithelium lining renal tubules. The tubular epithelium became necrotic at several places. Eosinophilic hyaline droplets is exist in the cytoplasm of the necrosed cells. Degenerative changes and hyperactivity in melanomachrophage center was seen in the spleen together with some necrotic areas. Necrosis and aggregation of melanomachrophage were seen in the hepatic cells, Hepatic cells showed vacuolar degeneration in the hepatic cells. Gills showed loss in the lamellae of the filaments associated with edema, inflammatory cells infiltration and haemorrhages in the arch. The sarcoplasm of the bundles of the skeletal muscle showed granular degeneration and focal inflammatory cells infiltration between the hyalinized bundles. Mercury residues obtained from these studies in the acute toxicity were 0.22 ppm/gm in the 2nd day, 0.411 ppm/gm in the $5^{th}$ day ended with 0.96 ppm/gm in the $7^{th}$ day. In chronic toxicity it was 1.1320, 1.7140, 2.3620 and 3.5640 ppm/gm respectively from the $2^{nd}$ to the $8^{th}$ week of exposure. In acute and chronic copper toxicity, there was degenerative changes in renal tubules. Melanophores aggregation in the wall of the blood vessels of the spleen and depletion of some of the melanophores in the melanomachrophage were seen together with necrosis in some areas. Congested Mvs (Micro vessels) and vacuolation of hepatocytes were observed. Some areas of hemorrhage and melanophores vacuolar degeneration in the liver were seen. There was mitosis in some areas with displesia of hepatopancreatic cells and eosinophilic granular cells aggregation. Zymogen granules disappeared and there were dyplastic hepatocytes. Congestion in the blood vessels of the gill filaments, associated with massive number of granular eosinophilic cells infiltration were seen in the base of the filaments. There were sever vacuolization and hyalinization in the skeletal muscle bundles. Detection of residues of copper sulfate revealed increase of the amount of copper measured in ppm/gm comparing to the normal control starting from 0.60 ppm/g in the $2^{nd}$ day, 0.67 ppm/g in the $5^{th}$ day and 0.67 ppm/g in the $7^{th}$ day. Result obtained in chronic copper sulfate toxicity revealed gradual increase of the amount of copper which ranged from 0.18 ppm/g at the $2^{nd}$ week to 0.21 ppm/g in the $8^{th}$ week of exposure.

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.162-173
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• 2006

Purpose : To report the decreasing indicence of HBV(Hepatitis B virus)-associated membranous nephropathy in children after HBV vaccination and to elucidate the clinical course and treatment strategies of IMN(Idiopathic membranous nephropathy). Methods : We retrospectively reviewed the clinico-pathological findings of HBV-MN and IMN patients who underwent a renal biopsy from 1986 to 2005. We compared the HBV-MN and the IMN groups and the remission and the non-remission groups of patients with IMN. Results : Among 24 cases of MN patients, HBV-MN comprised 6 cases(25%) and IMN 18 cases(75%). Clinical masnifestations were nephrotic syndrome(3 cases, 50%), nephritic syndrome(1 case, 16.7%), asymptomatic(2 cases, 33.4%) in the HBV-MN group, asymptomatic(10 cases, 55.5%), nephrotic syndrome(5 cases, 27.8%), and gross hematuria(3 cases, 16.7%) in the IMN groups. From 1996 to 2000, there were 2 cases(28%) of HBV-MN and 5 cases(72%) of IMN. After 2001 all 10 cases were IMN. In the HBV-MN group, 4 cases(66.7%) received interferon and 1 cases received methylprednisolone pulse therapy. In the IMN group, 16 cases(88.9%) received methylprednisolone, 8 cases(44.4%) were in complete remission, 2 cases(11.1%) were in partial remission, 2 cases(11.1%) were in chronic renal failure, and 5 cases(27.8%) were lost to follow-up with sustained proteinuria, 1 case(5.6%) continued to have frequent relapse of nephrotic syndrome without renal insufficiency. In the comparison between remission and non-remission groups, nephrotic range proteinuria and hypertension were more significantly common in the non-remission group(P<0.05). Conclusion : With HBV vaccination, HBV-MN has decreased markedly. IMN is a rare glomerular disease in children. Because the prognosis for patients with nephrotic range proteinuria is poor this group needs more aggressive treatment.

• Pediatric Infection and Vaccine
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• v.28 no.3
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• pp.181-188
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• 2021

Anemia commonly occurs in kidney transplantation (KT) recipients. Many factors such as viral infection, bleeding, erythropoietin deficiency, and immunosuppressants are the causes of chronic anemia in KT recipients. We report 2 cases who developed severe anemia caused by parvovirus B19 infection and were successfully treated with intravenous immunoglobulin in pediatric KT recipients.

• Biomolecules & Therapeutics
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• v.31 no.6
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• pp.619-628
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• 2023

In the modern era, chronic kidney failure due to diabetes has spread across the globe. Prunetin (PRU), a component of herbal medicines, has a broad variety of pharmacological activities; these may help to slow the onset of diabetic kidney disease. The anti-nephropathic effects of PRU have not yet been reported. The present study explored the potential nephroprotective actions of PRU in diabetic rats. For 28 days, nephropathic rats were given oral doses of PRU (20, 40, and 80 mg/kg). Body weight, blood urea, creatinine, total protein, lipid profile, liver marker enzymes, carbohydrate metabolic enzymes, C-reactive protein, antioxidants, lipid peroxidative indicators, and the expression of insulin receptor substrate 1 (IRS-1) and glucose transporter 2 (GLUT-2) mRNA genes were all examined. Histological examinations of the kidneys, liver, and pancreas were also performed. The oral treatment of PRU drastically lowered the blood glucose, HbA1c, blood urea, creatinine, serum glutamic-oxaloacetic transaminase, serum glutamic pyruvic transaminase, alkaline phosphatase, lipid profile, and hexokinase. Meanwhile, the levels of fructose 1,6-bisphosphatase, glucose-6-phosphatase, and phosphoenol pyruvate carboxykinase were all elevated, but glucose-6-phosphate dehydrogenase dropped significantly. Inflammatory marker antioxidants and lipid peroxidative markers were also less persistent due to this administration. PRU upregulated the IRS-1 and GLUT-2 gene expression in the nephropathic group. The possible renoprotective properties of PRU were validated by histopathology of the liver, kidney, and pancreatic tissues. It is therefore proposed that PRU (80 mg/kg) has considerable renoprotective benefits in diabetic nephropathy in rats.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.41-50
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• 2007

Purpose : Dialysis in children with chronic renal failure presents with many difficulies. The purpose of this study is to find an improved method in chronic dialysis in infants and children less than 2 years of age by analyzing the experience with 10 cases. Methods : A retrospective review of the medical records of 10 patients(6 boys and 4 girls) was conducted. The patients had chronic renal failure and underwent chronic dialysis at Samsung medical center from March 1999 to February 2007. Results : At Initiation of dialysis, the median age was 3 months old(22 days-20 months), the median body weight was 3.75 kg(2.2-10.3 kg), and the median serum creatinine level was 4.3 mg/dL(2.0-11.4 mg/dL). The median duration of dialysis was 29.5 months(3-62 months). Dysplastic kidney disease was the most common underlying renal disease. Two patients were treated with hemodialysis, 4 patients with peritoneal dialysis, and 4 patients eventually switched dialysis modality. Nine of the 10 patients took erythropoietin and anti-hypertensive drugs. At the end of the follow up period, 1 patient received kidney transplantation, 2 patients died due to sepsis, and 5 patients were treated with peritoneal dialysis. Two patients were lost to follow up. The most common complication of dialysis was infection. Achieving vascular access and maintaining proper catheter function were the most important factors in treating patients with hemodialysis. The growth status of patients was aggravated after 6 month of dialysis but improved after 1 year of dialysis. Patients showed better growth on peritoneal dialysis than hemodialysis. Conclusion : Chronic dialysis can be performed successfully in infants and children under 2 years of age. Vascular access was the main limitation of hemodialysis, and infection was the common problem in both hemodialysis and peritoneal dialysis. To improve the patients survival rate and quality of life, major efforts should be directed toward the prevention of infection and preservation of catheter function. (J Korean Soc Pediatr Nephrol 2007;11:41-50)