• Childhood Kidney Diseases
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• v.23 no.2
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• pp.93-99
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• 2019

C3 glomerulopathy is a renal disorder involving dysregulation of alternative pathway complement activation. In most instances, a membranoproliferative pattern of glomerular injury with a prevalence of C3 deposition is observed by immunofluorescence microscopy. Dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are subclasses of C3 glomerulopathy that are distinguishable by electron microscopy. Highly electron-dense transformation of glomerular basement membrane is characteristic of DDD. C3GN should be differentiated from post-infectious glomerulonephritis and other immune complex-mediated glomerulonephritides showing C3 deposits.

• Journal of Genetic Medicine
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• v.17 no.2
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• pp.97-101
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• 2020

The combination of central nervous system abnormalities and renal impairment is a notable characteristic of Galloway-Mowat syndrome (GAMOS), a disease which often accompanies microcephaly, developmental delay, and nephrotic syndrome. Many subtypes exist having various phenotypes and genotypes, and many genetic causes are still being identified. An 18-month-old boy first visited our clinic for seizure, delayed development, and microcephaly. During follow-up visits he developed proteinuria and nephrotic syndrome at the age of 6. Nephrotic syndrome became refractory to treatment. These phenotypes were suggestive of GAMOS. Next generation sequencing was performed for genetic analysis and revealed novel compound heterozygous variants in the WDR4 gene: c.494G>A (p.Arg165Gln) and c.540C>G (p.Ile180Met). This is the first case in Korea of GAMOS involving the WDR4 gene.

• Biomolecules & Therapeutics
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• v.29 no.4
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• pp.365-372
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• 2021

Type 2 diabetes mellitus (T2DM) leads to many health problems like diabetic nephropathy (DN). One of the key factors for chronic kidney disease and end-stage renal disease (ESRD) is T2DM. Extensive work is being done to delineate the pathogenesis of DN and to extend possible remedies. This review is intended to understand the nature of DN risk factors, progression, effects of glycemic levels, and stages of DN. We also explored the novel diagnostic and therapeutic approaches for DN such as gene therapy and stem cell treatments.

• Childhood Kidney Diseases
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• v.26 no.1
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• pp.25-30
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• 2022

Dyslipidemia in nephrotic syndrome (NS) is often characterized by marked increases in the levels of total cholesterol, triglycerides, low-density lipoprotein cholesterol, and other lipoproteins, such as very low-density lipoprotein, intermediate-density lipoprotein, and lipoprotein(a). It has been suggested that impaired catabolism of lipoproteins and cholesterol is mainly due to decreased lipoprotein lipase and hepatic lipase activity, and increased biosynthesis of lipoproteins in the liver. The management strategies for dyslipidemia in patients with NS consist of lifestyle modification, lipid-lowering agents represented by statins, second-line agents such as fibrates and bile acid sequestrants, and lipid apheresis. Compared with dyslipidemia in adult NS patients, whose risks of atherosclerotic disease and progressive renal injury are considered high, clinical data on dyslipidemia in pediatric NS patients are limited. Therefore, it is necessary to pay more attention to the evaluation and management of dyslipidemia in pediatric patients with NS in clinical practice.

• CELLMED
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• v.12 no.1
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• pp.4.1-4.13
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• 2022

The present communication attempts to evaluate the physicochemical and preliminary phytochemical studies on the fruit of Terminalia chebula Retz. Combretaceae family. Haritaki is one of the most celebrated herbs in the Indian traditional medicine system, Ayurveda. Terminalia chebula is known to exhibit different properties like anticancer, anti-inflammatory, anti-protozoal, antimicrobial, antioxidant, hepato and renal protective activities, and in the management of metabolic syndrome. As there is no detailed standardisation work reported on fruit, the physicochemical parameters, preliminary phytochemical constants, heavy metals, analysis are carried out. The phytochemical screening indicated the presence Tannin, Alkaloid, Phenol, Carbohydrate, Steroids, Protein and Resin compounds in CO₂ extract of Haritaki. The present investigation will helpful in assessing the quality and purity of a crude drug. Thus, the study provides facts that CO₂ extract of Haritaki contains medicinally important bioactive phytochemical compounds which justifies the use of plant species as conventional medicine for treatment of many diseases.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.222-230
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• 2005

Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

• The Korean Journal of Pharmacology
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• v.16 no.2 s.27
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• pp.77-83
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• 1980

The effectiveness of Cefoperazone in the treatment of urinary tract infection was evaluated in the Department of Urology, St. Mary's hospital from December 1. 1979 to April 30. 1980. In this studies, the cusative organisms were divided into 2 groups; 1) Single form. E. coli (8), Staphylococcus aureus(7), Proteus vulgaris (4), P. morganii(3), S. epidermis (1), Enterococcus(1), Klebsiella (2), N. gonococcus(1). 2) Mixed from: Proteus+E. coli(4), E. coli+other(1), Pseudomonas+Enterococcus(1), Klebsiella+other(1). Effectiveness on urological diseases. 1) Neurogenic bladder: Results were excellent in 3 cases, good in 4 cases and negative effect in 4 cases. 2) Non-gonococcal urethritis: In this group, the therapeutic results were favorable in 88.9% of all cases. (Excellent in 2, Fail in1) 3) Pyelonephritis: All(4 cases) were excellent. 4) Renal stone: Among the 4 cases of renal stone, only one case was responded to cefoperazone. 5) Two cases of urethral stricture, two cases of cystitis, one case of B.P.H. and one case of gonococcal urethritis were all excellent. No serious side effects were observed except slight dizziness in one case.

• Childhood Kidney Diseases
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• v.5 no.1
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• pp.73-77
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• 2001

Acute lobar nephronia is a focal of acute bacterial nephritis, affecting one or more of the renal lobules. Nephronia is being diagnosed more frequently in accordance with the advancing imaging techniques of urinary tract. We report a case of acute nephronia in a 4 month old boy who presented persisting urinary tract infection after intial antibiotic treatment. Ultrasonography and computerized tomography showed round wedge-shaped, non-enhancing mass in right kidney. Gallium scan also revealed the lesion in the right kidney. This lesion resolved completely on one month follow up examinations. (J. Korean Soc Pediatr Nephrol 5 : 73- 7, 2001)

• Childhood Kidney Diseases
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• v.19 no.2
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• pp.56-64
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• 2015

To revise the clinical guideline for childhood urinary tract infections (UTIs) of the Korean Society of Pediatric Nephrology (2007), the recently updated guidelines and new data were reviewed. The major revisions are as follows. In diagnosis, the criterion for a positive culture of the catheterized or suprapubic aspirated urine is reduced to 50,000 colony forming uits (CFUs)/mL from 100,000 CFU/mL. Diagnosis is more confirmatory if the urinalysis is abnormal. In treating febrile UTI and pyelonephritis, oral antibiotics is considered to be as effective as parenteral antibiotics. In urologic imaging studies, the traditional aggressive approach to find primary vesicoureteral reflux (VUR) and renal scar is shifted to the targeted restrictive approach. A voiding cystourethrography is not routinely recommended and is indicated only in atypical or complex clinical conditions, abnormal ultrasonography and recurrent UTIs. $^{99m}Tc$-DMSA renal scan is valuable in diagnosing pyelonephritis in children with negative culture or normal RBUS. Although it is not routinely recommended, normal scan can safely avoid VCUG. In prevention, a more natural approach is preferred. Antimicrobial prophylaxis is not supported any more even in children with VUR. Topical steroid (2-4 weeks) to non-retractile physiologic phimosis or labial adhesion is a reasonable first-line treatment. Urogenital hygiene is important and must be adequately performed. Breast milk, probiotics and cranberries are dietary factors to prevent UTIs. Voiding dysfunction and constipation should be properly treated and prevented by initiating toilet training at an appropriate age (18-24 months). The follow-up urine test on subsequent unexplained febrile illness is strongly recommended. Changes of this revision is not exclusive and appropriate variation still may be accepted.

• Childhood Kidney Diseases
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• v.13 no.1
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• pp.1-13
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• 2009

Type 1 diabetes mellitus commonly occurs in childhood and adolescence, although the prevalence of type 2 diabetes mellitus in these age groups is now being increased in the western world and Korea. Diabetic nephropathy developing in 15-25% of subjects with type 1 diabetes mellitus and in similar or higher percentage of type 2 diabetes mellitus patients is the leading cause of end-stage renal disease worldwide. Although prepubertal diabetic duration may contribute less to the development of microvascular complications than pubertal and postpubertal duration, diabetic nephropathy in susceptible patients almost certainly begins soon after disease onset and may accelerate during adolescence, leading to microalbuminuria or incipient DN. Type 1 diabetes is commonly associated with a period of hyperfiltration followed by the development of persistent microalbuminuria after as little as 7-10 years of type 1 diabetes. Microalbuminuria is associated with pathologic lesions that are so advanced as to overlap with those seen in patients with overt proteinuria and declining kidney function, therefore, microalbuminuria currently considered the best clinical indicator of overt diabetic nephropathy risk. This review covers the natural history and renal manifestations of diabetic nephropathy in children and adolescents.