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A Female Infant with Segmental Multicystic Dysplastic Kidney.

Journal of the Korean Society of Pediatric Nephrology.2013;17(1):25-28.

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.
Adult ; Biopsy ; Female ; Humans ; Infant ; Multicystic Dysplastic Kidney ; Natural History

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Clinical Characteristics of Community Acquired Enterococcal Urinary Tract Infections In Children.

Seong Heon KIM ; Taek Jin LIM ; Hye Young KIM ; Su Eun PARK ; Su Young KIM

Journal of the Korean Society of Pediatric Nephrology.2013;17(1):19-24.

PURPOSE: Recently, enterococcus spp. have become one of the most common nosocomial pathogens with increasing rates of multi-drug resistance. However, study on enterococcal urinary tract infections (UTIs) in children is very limited, especially community acquired UTIs. We studied the clinical characteristics of enterococcus spp. in community acquired UTIs and antibiotic resistance within our urban area. METHODS: All children with first episode of community acquired UTIs due to enterococcus spp. and Echerichia coli who were admitted in Pusan National University Children's Hospital between January 2010 and January 2013 were included in our study. We retrospectively reviewed their medical records. RESULTS: During the study period, 201 patients were identified to have first episode of community acquired UTIs. 154 cases were E.coli UTIs (76.6%) and 11 cases were enterococcal UTIs (5.5%) and all enterococcus spp. were Enterococcus feacalis. In enterococcal UTI group, voiding cystourethrogram(VCUG) was performed in 7/11 patients and demonstrated 4 vesicoureteral refluxes (VURs) with renal scar and 3 patients underwent corrective surgery. In E.coli UTI group, VCUG was performed in 121/154 patients and demonstrated 23 VURs and 11 patients underwent corrective surgery. Enterococcal group had significant high rate of underlying urinary abnormalities and surgical corrections compared with E. coli group. All enterococcus spp. were susceptible to ampicillin, vancomycin and linezolid, but all were resistant to tetracycline. They also showed 71.4% resistance to trimethoprim-sulfamethoxazole and 20% resistance to ciprofloxacin. CONCLUSION: Community acquired enterococcal UTIs in children were rare within our urban area. However, they could be indicative of severe underlying urinary tract abnormalities.
Acetamides ; Ampicillin ; Child ; Cicatrix ; Drug Resistance, Microbial ; Drug Resistance, Multiple ; Enterococcus ; Humans ; Oxazolidinones ; Retrospective Studies ; Tetracycline ; Trimethoprim, Sulfamethoxazole Drug Combination ; Urinary Tract ; Urinary Tract Infections ; Vancomycin ; Vesico-Ureteral Reflux ; Linezolid

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Podocytopathy and Morphologic Changes in Focal Segmental Glomerulosclerosis.

Hyeon Joo JEONG

Journal of the Korean Society of Pediatric Nephrology.2013;17(1):13-18.

Podocytopathy is glomerular lesions characterized by podocyte injury. It is observed in various glomerular diseases, but minimal change disease and focal segmental glomerulosclerosis (FSGS) are the prototypes. In this review, morphologic features of podocyte injury and subtypes of FSGS will be reviewed briefly. Effacement of podocyte foot processes is the most common feature of podocyte injury. As podocytic injury progresses, intracytoplasmic vacuoles, subpodocytic cyst, detachment of podocytes from the glomerular basement membrane and apoptosis develop. Glomerular capillary loops in epithelium-denuded area undergo capillary collapse. Synechia and hyalinosis may accompany this lesion. To manifest segmental sclerosis, podocyte loss above a threshold level may be required. Injured podocytes can injure neighboring intact podocytes, and thereby spread injury within the same lobule. FSGS can be categorized into five subtypes by morphologic characteristics; not otherwise specified (NOS), perihilar, cellular, tip, and collapsing types. Each subtype has been reported to show different clinical courses and associated conditions, but there are controversies on its significance. With recent progress in the discovery of genetic abnormalities causing FSGS and plasma permeability factors, we expect to unravel pathophysiology of FSGS and to understand histological sequences leading to FSGS in near future.
Apoptosis ; Capillaries ; Foot ; Glomerular Basement Membrane ; Glomerulosclerosis, Focal Segmental ; Nephrosis, Lipoid ; Permeability ; Plasma ; Podocytes ; Sclerosis ; Vacuoles

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Pathology of Thrombotic Microangiopathy.

Mee Young SOL

Journal of the Korean Society of Pediatric Nephrology.2013;17(1):6-12.

Thrombotic microangiopathy (TMA) is a microvascular thrombotic lesion caused by endothelial injury and subsequent formation of platelet rich thrombus. TMA is first described as a classical pathologic feature of HUS/TTP. Renal biopsy finding of TMA represents kidney involvement of HUS/TTP as well as other diseases such as malignant hypertension, drug toxicity, eclampsia, pre-eclampsia, and several systemic infections. Autoimmune diseases and transplant kidney sometime also have TMA. It is needed to consider a complete autoimmune work-up of patients presenting with TMA including tests for ANA, ANCA, and ADAMTS13 inhibitory antibodies, because there are several reports of association with TMA in patients of SLE, anti-phospholipid syndrome, and ANCA-associated vasculitis.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies ; Antibodies, Antineutrophil Cytoplasmic ; Antiphospholipid Syndrome ; Autoimmune Diseases ; Biopsy ; Blood Platelets ; Drug Toxicity ; Eclampsia ; Female ; Humans ; Hypertension, Malignant ; Kidney ; Pre-Eclampsia ; Pregnancy ; Thrombosis ; Thrombotic Microangiopathies ; Transplants

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Pathology of C3 Glomerulonephritis.

Yong Jin KIM

Journal of the Korean Society of Pediatric Nephrology.2013;17(1):1-5.

C3 glomerulonephritis (C3GN) is a recently described entity that shows a glomerulonephritis on light microscopy, bright C3 staining and the absence of C1q, C4, and immunoglobulins on immunofluorescence microscopy and mesangial and/or subendothelial electron-dense deposits on electron microscopy. The term 'C3 glomerulopathy' is often used to include C3GN and dense deposit disease (DDD), CFHR5 nephropathy, those of which result from dysregulation of the alternative pathway of complement. C3GN shares some aspects of atypical hemolytic uremic syndrome, MPGN, late stage of post infectious glomerulonephritis and other glomerulonephrtis. When C3GN is considered, measurement of serum complement proteins including C3, CFH, CFI, CFB and testing for the presence of C3 nephritic factor, anti-factor H autoantibodies are necessary. To screening for mutations, genes that encode complement regulators should be evaluated. This disorder equally affected all ages, both genders, and typically presented with hematuria and proteinuria. In both the short and long term, renal function remained stable in the majority of patients.
Aminopeptidases ; Autoantibodies ; Complement C3 Nephritic Factor ; Complement Pathway, Alternative ; Complement System Proteins ; Glomerulonephritis ; Glomerulonephritis, Membranoproliferative ; Hematuria ; Hemolytic-Uremic Syndrome ; Humans ; Immunoglobulins ; Light ; Mass Screening ; Microscopy ; Microscopy, Electron ; Microscopy, Fluorescence ; Proteinuria

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Effects of Methylprednisolone on TGF-beta, Apoptosis and Renal Scarring in Experimental Acute Pyelonephritic Weaning Rats.

Soo Ja WHANG ; Soon Hee SUNG ; Seung Joo LEE

Journal of the Korean Society of Pediatric Nephrology.2002;6(1):75-84.

PURPOSE: Acute pyelonephritis of growing kidneys may result in renal scarring. TGF-beta, inflammatory cytokine, has been suggested to play an important role in promoting renal scarring through apoptosis, suppression of cellular proliferation and fibrosis. We observed the effects of a potent anti-inflammatory agent, methylprednisolone on apoptosis and renal scarring in experimentally induced acute pyelonephritic weaning rats. MATERIALS AND METHODS: To induce ascending pyelonephritis a saline solution containing Escherichia coli type ATCC No. 25922, pili-form (107 bacteria/mL) was infused into the bladder through the 16-guage silicone cannula for 48 hours to 102 three-week-old Sprague-Dawley rats (50-60g). Experimental groups were divided into three groups according to the treatment protocols, group I (ceftriaxone only, n=31), group II (methylprednisolone+ceftriaxone n=28), control group (n=43) was not treated. Histopathologic scores of inflammatory changes, fibrosis and tubular atrophy, the apoptosis index and TGF-beta1 expression score were observed at post-infection 1 and 3 week. Datas were analysed using ANOVA test and P value below 0.05 was interpreted as significant. RESULTS: The mortality rate (21.4%) of group II was not different to the control group (41.9%) and group I (32.3%). The inflammatory score of group II (0.8+/-0.87) at week 1 was significantly lower than those of the control group (2.3+/-0.87) and Group I (1.7+/-0.79) (P<0.05). Apoptosis index of group II (2.9+/-2.15) at week 1 was significantly lower than (10.0+/-0.95) and group 1 (8.3+/-2.53) (P<0.05). TGF-beta1 expression score of group II (0.8+/-0.72) at week 1 was significantly lower than those of the control group (1.9+/-0.68) and group I (1.8+/-0.60) (P<0.05). The fibrosis score of group II (1.1+/-0.10) at week 3 was significantly lower than that of the group I (1.8+/-0.83) (P<0.05). CONCLUSION: Combined treatment with methylprednisolone and ceftriaxone reduced inflammation, fibrosis, apoptosis and TGF-beta expression in acute pyelonephritic weaning rats, compared to ceftriaxone alone. Anti-inflammatory agent supplemented to antibiotics could prevent renal scarring more effectively.
Animals ; Anti-Bacterial Agents ; Apoptosis* ; Atrophy ; Catheters ; Ceftriaxone ; Cell Proliferation ; Cicatrix* ; Clinical Protocols ; Escherichia coli ; Fibrosis ; Inflammation ; Kidney ; Methylprednisolone* ; Mortality ; Pyelonephritis ; Rats* ; Rats, Sprague-Dawley ; Silicones ; Sodium Chloride ; Transforming Growth Factor beta* ; Transforming Growth Factor beta1 ; Urinary Bladder ; Weaning*

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Suprapubic Bladder Aspiration Assisted by Ultrasound.

Jung Won LEE ; Soeun PARK ; Su Jin CHO ; Eun Sun YOO ; Hae Soon KIM ; Seoung Joo LEE

Journal of the Korean Society of Pediatric Nephrology.2002;6(1):68-74.

PURPOSE: Suprapubic bladder aspiration(SBA) of urine is the most reliable method to obtain urine avoiding contamination in non-toilet trained infants. Ultrasonography is a useful tool for guiding the anatomic location as well as for direct visualization during procedure. We evaluated the success rate and complication of ultrasound(US) assisted SBA. METHODS: Sixty infants who visited Ewha Womans University Mokdong Hospital, with suspected urinary tract infection were randomly divided into the US assisted (n=32) and blind SBA(control, n=28) group. In US assisted SBA group, the anteroposterior(AP), transverse, and sagittal diameters and the volume of the bladder were measured. In the blind SBA group, urine was blindly aspirated when the urinary bladder was palpated at the suprapubic area. The rate of successful urine aspiration, the number of attempts until successful aspiration, aspirated urine volume were compared between the two groups. RESULTS: The success rate was 100%(32/32) in the US assisted group, which was significantly higher than 85.7%(24/28) of the control group (P<0.05). The aspirated urine volume in the US assisted group was 7.4+/-3.7 mL, which was significantly higher than 4.5+/-3.4 mL of the control group (P<0.05). The diameters and volume of bladder in successful aspiration were 2.1+/-0.7 cm in AP diameter, 3.1+/-0.6 cm in transverse diameter, 4.2+/-1.0 cm in sagittal diameter and 15.2+/-10.4 mL in volume, which were significantly higher than those (1.7+/-0.3 cm, 1.8+/-0.7 cm, 2.4+/-0.6 cm, 3.9+/-2.5) of the control group (P<0.05). The correlations between the AP(r=0.78), transverse (r=0.72), sagittal(r=0.91) diameter and bladder volume were significant (P<0.05). SBA was 100% successful in the AP diameter >3 cm, transverse diameter >4 cm, depth >4 cm and bladder volume >5 mL. CONCLUSION: US assistance can significantly improve the success rate of SBA in infant with suspected urinary tract infection.
Female ; Humans ; Infant ; Ultrasonography* ; Urinary Bladder* ; Urinary Tract Infections

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Analysis of Isolated Proteinuria on School Urinary Mass Screening.

Cheol Min KIM ; Hye Won HAHN ; Byung Sun LEE ; Young Seo PARK

Journal of the Korean Society of Pediatric Nephrology.2002;6(1):61-67.

PURPOSE: The urinary mass screening program in school aged population has been performed since 1981, but the consensus on the follow-up schedule and the management of isolated proteinuria has not been reached yet. The aim of this study was to investigate the cause of isolated proteinuria and to propose a guideline for the treatment and follow-up afterwards. Methods: The medical records of 114 cases of isolated proteinuria detected through the analysis of urinary mass screening and evaluated at the pediatric outpatient clinic of Asan Medical Center from January 1990 to July 2001 have been reviewed. RESULTS: The classification of isolated proteinuria was as follows. Transient proteinuria 32%, orthostatic proteinuria 65%, persistent proteinuria 3%. In orthostatic proteinuria group, daytime and nighttime proteinuria were 319.2+/-89.1 mg/dL and 56.5+/-6.1 mg/dL. In persistent proteinuria group, daytime and nighttime proteinuria were 1140+/-40.5 mg/dL and 289+/-8 mg/dL. After 30 month follow-up, 2 cases of persistent proteinuria were needed renal biopsy and 1 case revealed focal segmental glomerular sclerosis. In all cases, serum creatinine, albumin and complements levels were normal. In the orthostatic proteinuria group, no significant renal diseases were detected. CONCLUSION: Since most of the isolated proteinuria detected through the school urinary mass screening were orthostatic proteinuria or transient proteinuria, initially aggressive diagnostic method such as renal biopsy is not needed and regular follow-up with quantitation of proteinuria is warranted.
Ambulatory Care Facilities ; Appointments and Schedules ; Biopsy ; Chungcheongnam-do ; Classification ; Complement System Proteins ; Consensus ; Creatinine ; Follow-Up Studies ; Humans ; Mass Screening* ; Medical Records ; Proteinuria* ; Sclerosis

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Efficacy of 23-valent Pneumococcal Polysaccharide Vaccine in Steroid Responsive Nephrotic Syndrome.

Hye Won HAHN ; Il Soo HA ; Hae Il CHEONG ; Hoan Jong LEE ; Yong CHOI

Journal of the Korean Society of Pediatric Nephrology.2002;6(1):56-60.

PURPOSE: Streptococcus pneumoniae is a major pathogen in both adults and children, causing significant morbidity and mortality. In patients with nephrotic syndrome, Streptococcus pneumoniae is a major cause of spontaneous peritonitis, and the increasing incidence of penicillin-resistance strain facilitates the development of effective vaccine. The limitation of current pneumococcal polysaccharide vaccine prompted development of polysaccharide-protein conjugate vaccine. METHODS: We reviewed the medical record of total 225 steroid responsive nephrotic patients to ascertain the effectiveness of 23-valent pneumococcal polysaccharide vaccine. RESULTS: Twenty-eight patients have developed peritonitis during the courses, and 7 of those have recurrent peritonitis. Fifty-five patients were vaccinated and followed-up for 1-108 months (mean 38.5 months), and during the follow-up period, pneumococcus related peritonitis was not detected. Vaccine-related relapse of nephrotic syndrome was absent. CONCLUSION: In spite of the non-consensus about the efficacy of PPV23, clinically it benefits, and until the clinical trial of PCV7 is completed, PPV23 will be recommended.
Adult ; Child ; Follow-Up Studies ; Humans ; Incidence ; Medical Records ; Mortality ; Nephrotic Syndrome* ; Peritonitis ; Pneumococcal Vaccines* ; Recurrence ; Streptococcus pneumoniae

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A Study on the Association of Thin Glomerular Basement Membrane Abnormality with Minimal Change Nephrotic Syndrome.

Chang Woo KIM ; Min Hyun CHO ; Cheol Woo KO ; Ja Hoon KOO ; Jung Sik KWAK

Journal of the Korean Society of Pediatric Nephrology.2002;6(1):48-55.

PURPOSE: Thin glomerular basement membrane nephropathy (TGBMN) is recognized as the leading cause of microscopic hematuria in both children and adults. However thinning of glomerular basement membrane (TGBM) has been found in healthy adult and also is known to be associated with various renal diseases such as Alport syndrome, IgA nephropathy and mesangial proliferative glomerulonephritis. The association of TGBM with minimal change nephrotic syndrome (MCNS) has been very rare so that the present study was undertaken to determine the relationship between TGBM and MCNS. METHODS: The study population consisted of 49 children with biopsy-proven MCNS who have been admitted to the pediatric department of Kyungpook University Hospital during the past 5 years from 1997 to 2001. Group I consisted of 8 children associated with TGBM and Group II 41 children without TGBM. Various parameters such as age of illness, duration from discovery of illness to the time of biopsy, family history of hematuria and other laboratory tests were compared between these two groups and the following results were obtained. RESULTS: Age distribution showed slightly older age in Group I (7.1+/-3.5 years) compared to Group II (4.8+/-2.9 years). However this was not statistically different (P=0.056). Family history of hematuria was noted in 2 cases in Group II. Though statistically not significant, hematuria was seen in 2 out of 8 cases (25%) in MCNS children with TGBM, compared to 7 out of 41 cases (17%) with MCNS children without TGBM. Other parameters such as BUN, creatinine, 24 hours urine protein excretion, serum protein, albumin, cholesterol, and T4/T8 ratio, showed no difference. Also renal biopsy finding showed no significant difference and the thickness of glomerular basement membrane in Group I was 188 30 nm. CONCLUSION: TGBM was found in 8 out of 49 children with MCNS (16.3%). And this high frequency of occurrence indicates that these association is not an incidental findings. Typical clinical findings of TGBMN was not noted in all of the 8 children with MCNS associated with TGBM, suggesting that thinning of glomerular basement membrane (TGBN) is secondary to rather than the cause of MCNS.
Adult ; Age Distribution ; Biopsy ; Child ; Cholesterol ; Creatinine ; Glomerular Basement Membrane* ; Glomerulonephritis ; Glomerulonephritis, IGA ; Gyeongsangbuk-do ; Hematuria ; Humans ; Incidental Findings ; Nephritis, Hereditary ; Nephrosis, Lipoid*

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