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Seven-Year Follow Up of Microscopic Polyangiitis Presenting with Rapidly Progressive Glomerulonephritis.

Jin Won OH ; Pyung Kil KIM ; Jae Seung LEE ; Hyeon Joo JEONG

Journal of the Korean Society of Pediatric Nephrology.2008;12(1):99-104.

Microscopic polyangiitis(MPA) is a systemic necrotizing vasculitis that involves many organ systems including the skin, joint, kidneys, and lungs. In spite of early diagnosis and intensive care, the five-year actuarial patient and kidney survival rates are 65% and 55%. We experienced a case in 7-year-old girl of microscopic polyangiitis presenting with rapidly progressive glomerulonephritis which was confirmed by renal biopsy and positive serum perinuclear antineutrophil cytoplasmic autoantibodies(p-ANCA). The diagnosis of patients first renal biopsy was MPA, p-ANCA-associated crescentic glomerulonephritis. The patients second renal biopsy was done 5 years 6 months later since first renal biopsy, and pathologic diagnosis was chronic sclerosing glomerulonephritis, advanced, due to MPA. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor, angiotensin II receptor blocker, and cyclophosphamide were used until now and the patients current age is 14 years old. On admission, the patients laboratory findings showed BUN 117 mg/dL and Cr 2.3 mg/dL, while on the hospital day BUN and Cr values fell to 20.8 mg/dL and 1.6 mg/dL. But renal function was progressed to chronic failure with latest laboratory data BUN 51.7 mg/dL and Cr 3.2 mg/dL. ACE inhibitor, angiotensin II receptor blocker and small dose of immunosuppressant with close observation is the key to maintain the patient survival.
Biopsy ; Child ; Cyclophosphamide ; Cytoplasm ; Early Diagnosis ; Follow-Up Studies ; Glomerulonephritis ; Humans ; Critical Care ; Joints ; Kidney ; Lung ; Methylprednisolone ; Microscopic Polyangiitis ; Plasmapheresis ; Receptors, Angiotensin ; Skin ; Survival Rate ; Vasculitis

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Two Cases of IgG-associated Mesangial Glomerulonephritis in Children.

Sang Hoo LEE ; Pyung Kil KIM ; Ji Sun SONG

Journal of the Korean Society of Pediatric Nephrology.2008;12(1):93-98.

Rare cases of IgG associated mesangial glomerulonephritis(IgG GN) defined by exclusive or predominant mesangial IgG deposits were reported first by Sato et al.(1993). and subsequently 10 pediatric cases were reported by Yoshikawa et al. (1994). Previous reports suggested that the prognosis of IgG GN is relatively benign course but recent report suggested that prognosis of IgG GN is highly variable. Also the recurrence of IgG GN in a renal transplant was reported by Fakhouri et al. (2002). Such a recurrence highlights the specificity of this type of glomerulonephritis. We experienced two pediatric cases of IgG GN proven by renal biopsy. Case 1. 4-year-old girl with nephrotic syndrome admitted because of general edema. The patient's urinalysis showed proteinuria and microscopic hematuria. Renal biopsy was performed because of relapsed nephritic syndrome. Light microscopic finding was nonspecific with almost normal histology. Immunofluorescent findings showed diffuse segmental IgG(+) and IgM(+) deposits in the capillary walls, and focal segmental spotty C4(trace), C1q(trace) deposits. Electron microscopic findings showed focal portion of mesangial electron dense deposits without mesangial widening. Case 2. 11-year-old girl admitted for evaluation of microsopic hematuria detected through mass school urinary screening program. Renal biopsy was performed for exact diagnosis. Immunofluorescent findings showed focal segmental IgG(+), IgM(+/-) and C3(+/-) deposits. Electron microscopic findings showed focal portion of esangial electron dense deposits without mesangial widening.
Biopsy ; Capillaries ; Child ; Edema ; Electrons ; Glomerulonephritis ; Hematuria ; Humans ; Immunoglobulin G ; Light ; Mass Screening ; Nephrotic Syndrome ; Preschool Child ; Prognosis ; Proteinuria ; Recurrence ; Sensitivity and Specificity ; Transplants ; Urinalysis

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Membranous Nephropathy Associated with Epstein-Barr Virus Infection in a Child.

Eun Hee LEE ; Dong Hee LIM ; Hyung Eun YIM ; Kee Hwan YOO ; Nam Hee WON ; Young Sook HONG ; Joo Won LEE

Journal of the Korean Society of Pediatric Nephrology.2008;12(1):88-92.

Infection of Epstein-Barr virus(EBV) gives rise to a broad spectrum of clinical manifestations in children. Although renal involvement is rare, diverse renal manifestations are known from hematuria to acute renal failure. Secondary membranous nephropathy(MN) associated with systemic EBV infection is an uncommon renal pathology and only two cases have been reported. We are adding another case of MN associated with EBV infection in a child. An 8-year-old girl was admitted for renal biopsy. She had been followed up for microscopic hematuria and intermittent proteinuria for 5 months. There had been no specific findings in serology and radiology. Tonsil biopsy had been done due to exudative tonsillar hypertrophy and enlarged multiple cervical lymph nodes. And it showed EBV-associated lymphoproliferative findings. Serologic tests for EBV showed positive evidence of recent infection; viral capsid antigen(VCA) IgM was borderline positive, VCA IgG and early antigen IgG were positive, and EB nuclear antigen IgG was negative. In Situ Hybridization of tonsil for EBV mRNA was positive. Because her proteinuria and hematuria were aggravated at that time(protein 3+, RBC >60/HPF), renal biopsy was done. Renal biopsy showed the findings of MN, characterized by thickened capillary walls with epimembranous spikes on light microscopy and subepithelial, mesangial and subendothelial electron dense deposits on electron microscopy. On immunofluorescence microscopy, IgG, C1q, kappa and lambda chains were positive. After steroid administration, proteinuria and hematuria resolved gradually within 6 months.
Acute Kidney Injury ; Biopsy ; Capillaries ; Capsid ; Child ; Electrons ; Epstein-Barr Virus Infections ; Glomerulonephritis, Membranous ; Hematuria ; Herpesvirus 4, Human ; Humans ; Hypertrophy ; Immunoglobulin G ; Immunoglobulin M ; In Situ Hybridization ; Light ; Lymph Nodes ; Microscopy ; Microscopy, Electron ; Microscopy, Fluorescence ; Palatine Tonsil ; Proteinuria ; RNA, Messenger ; Serologic Tests

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A Survey on the Conception and Cognition about Enuresis of Primary Care Physicians in Daegu City.

Jung Youn CHOI ; Sae Yoon KIM ; Kyung Soo LEE ; Yong Hoon PARK

Journal of the Korean Society of Pediatric Nephrology.2008;12(1):78-87.

PURPOSE: Recently, the conception and cognition that enuresis was resolved spontaneously, have changed. We reviewed the attitudes of the primary care physicians who make diagnose and treat nocturnal enuresis. METHODS: From January 2006 to February 2007, a total of 293 primary care physicians in Daegu city participated in this survey. Questionnaires included questions about physicians' opinions on the appropriated age for diagnosis of enuresis, the likely causes of enuresis, etc. Physicians are grouped in two according to whether enuresis is major field of their subspecialty; the pediatrician & urologist group and the other physician group. RESULTS: 59.2% of pediatricians and urologists thought that enuresis is defined as the nightly involuntary release of urine by children of the age of 5 to 6, while 49.6% of other physicians did. For the causes of enuresis, most of clinicians checked "yes" to the question that "Under- developed bladder and nerve" and "Emotional problems". In the patient's behavioral reactions related to enuresis, "Lack of concentration in home and school" and "Frequent urination" were most responded. Attendance to the education program of enuresis in last five years and willing to participate in education program was statistically different among pediatricians-urologists and other physicians. Regarding the treatment of enuresis, most physicians used imipramin widely, but pediatricians and urologists preferred desmopressin. Alarm was the last one in treatment modality. CONCLUSIONS: This study revealed that pediatricians and urologists are attending more to the educational places and knowing much about the recent information on enuresis when compared to other primary care physicians, regarding the diagnostic age and treatment modality of enuresis. The education of enuresis for primary physicians is more needed.
Child ; Cognition ; Deamino Arginine Vasopressin ; Enuresis ; Fertilization ; Humans ; Nocturnal Enuresis ; Physicians, Primary Care ; Primary Health Care ; Surveys and Questionnaires ; Urinary Bladder

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Fever Duration and Renal Scar in Pediatric Urinary Tract Infection.

Ji In JUNG ; Dong Hee LIM ; Hyung Eun YIM ; Man Sik PARK ; Kee Hwan YOO ; Young Sook HONG ; Joo Won LEE

Journal of the Korean Society of Pediatric Nephrology.2008;12(1):70-77.

PURPOSE: Urinary tract infections(UTIs) are not uncommon findings in febrile pediatric patients and approximately one third of patients with UTI may have renal scars. This research was intended to establish the relationship between duration of fever and renal scars. METHODS: The medical records of 143 patients were reviewed retrospectively. Inclusion criteria were as follows: 1) fever as defined by an axillary temperature > or =37.5degrees C, 2) accurate history of fever duration and the use of antibiotics 3) no previous history of UTI and 4) positive urine culture. We observed whether the longer fever duration could be associated with the development of initial renal defects and subsequent renal scars, increased C-reactive protein(CRP), leukocytosis and the presence of vesicoureteral reflux(VUR). RESULTS: 1) Patients with longer fever duration after antibiotics showed more frequent initial renal defects(P=0.014). However, fever duration before antibiotic use was not associated with the development of initial renal defects(P=0.244). 2)Incidence of renal scar increased with fever duration before antibiotic use(P=0.006) and fever duration after antibiotic use(P=0.015). 3) CRP correlated with the fever duration after antibiotic use(r=0.287, P=0.003). 4) There was no relationships between fever duration and VUR(P>0.05). CONCLUSIONS: Our data suggest that fever duration before/after antibiotic use is significantly associated with the increased development of renal scars in pediatric UTI.
Anti-Bacterial Agents ; C-Reactive Protein ; Cicatrix ; Fever ; Humans ; Leukocytes ; Leukocytosis ; Medical Records ; Retrospective Studies ; Urinary Tract ; Urinary Tract Infections ; Vesico-Ureteral Reflux

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Predictive Value for Vesicoureteral Reflux in Children with Urinary Tract Infection.

Seung Hyun LEE ; Sung Hoon NOH ; Jeung Eun OH ; Min Sun KIM ; Dae Yeol LEE

Journal of the Korean Society of Pediatric Nephrology.2008;12(1):62-69.

PURPOSE: The most concerning issue in children with urinary tract infection(UTI) is the probability of underlying genitourinary anomalies and vesicoureteral reflux(VUR), which is frequently associated with renal scarring and eventually end-stage renal disease. Therefore, voiding cystourethrography(VCUG) is usually recommended at the earliest convenient time for children with UTI. However, VCUG is an invasive procedure that requires catheterization and exposure to X-ray. In this study, we aimed to determine the predictability of clinical, laboratory and imaging parameters for VUR in children with UTI. METHODS: Data of children with bacteriologically proven UTI who underwent VCUG were evaluated retrospectively for clinical(age, gender, fever), laboratory(leukocytosis, ESR, CRP, pyuria, blood urea nitrogen, serum creatinine) and imaging(renal ultrasound and DMSA renal scan) findings. First, children with UTI were divided into two groups according to the presence of VUR as non-VUR group and with VUR group, and clinical, laboratory variables were compared between these groups. Second, patients who had VUR were reclassified as low- grade VUR(grade I-II) group and high-grade(grade III-V) VUR group according to grading of VUR, and clinical, laboratory and imaging variables were compared between these groups. RESULTS: Among 410 children with UTI, 137 had VUR and 78 high-grade VUR. Fever, leukocytosis, ESR, CRP, pyuria were associated with VUR. In addition, abnormal findings of ultrasonography and DMSA renal scan were closely related to VUR. However, these clinical and laboratory variable in patients with high grade VUR were not different significantly, compared to those with low-grade VUR group. CONCLUSIONS: Fever, leukocytosis, ESR, CRP seems to be potentially useful predictors of VUR in pediatric patients with UTI. In addition, renal ultrasonography and DMSA renal scan findings supported the presence of VUR. Further study of these findings could limit unnecessary VCUG in patients with UTI.
Blood Urea Nitrogen ; Catheterization ; Catheters ; Child ; Cicatrix ; Fever ; Humans ; Kidney Failure, Chronic ; Leukocytosis ; Pyuria ; Retrospective Studies ; Succimer ; Urinary Tract ; Vesico-Ureteral Reflux

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Should Voiding Cystourethrography be Performed for Infants with Urinary Tract Infection?.

Mi Kyeong WOO ; Mun Sub KIM ; Ja Wook KOO

Journal of the Korean Society of Pediatric Nephrology.2008;12(1):54-61.

PURPOSE: This study was performed to assess necessity of voiding cystourethrography (VCUG) for infants with urinary tract infection(UTI) who had both normal renal sonography and normal DMSA renal scans. METHODS: We reviewed 117 infants hospitalized for UTI between February 2002 and July 2007 at Sanggye Paik Hospital. The diagnosis of UTI was made by culture from a urine specimen obtained by suprapubic puncture(n=57), catheterization(n=58), or collection bag method (n=2, twice positive culture of the same organism). All patients had undergone renal sonography, DMSA renal scan and VCUG. Children with both normal renal sonography and normal DMSA renal scans were evaluated for the presence or severity of vesicoureteral reflux (VUR). RESULTS: Of the 117 patients, 96 were boys and 21 were girls. 28 patients(23.9%) had VUR. 59(50.4%) showed both normal renal sonography and normal DMSA renal scans. Among these 59 patients, 7(11.9%) showed VUR. Three of them had grade I-II reflux, two grade III reflux, and the other two grade IV reflux. One of them showed bilateral VUR, grade IV reflux on the right and grade III on the left. CONCLUSIONS: Although the negative predictive value of both normal renal sonography and normal DMSA renal scan for VUR was 88.1%, 7 patients had VUR and two of them had high grade reflux(grade IV). So, we suggest that VCUG should be performed in infants with UTI despite both normal renal sonography and normal DMSA renal scans.
Child ; Humans ; Infant ; Succimer ; Urinary Tract ; Urinary Tract Infections ; Vesico-Ureteral Reflux

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The Investigation of Current Information Regarding Renal Diseases in Mass Media.

Dong Hee LIM ; Ji In JUNG ; Hyung Eun YIM ; Baik Lin EUN ; Kee Hwan YOO ; Young Sook HONG ; Joo Won LEE

Journal of the Korean Society of Pediatric Nephrology.2008;12(1):47-53.

PURPOSE: We often have patients who acquired incorrect medical information from the mass media. The purpose of this study was to evaluate credibility of articles in newspapers and medical counseling on websites about renal diseases. METHODS: Kidney information was searched in 6 newspapers for the past 10 years, and 4 portal websites and 17 internet health counseling sites for the past 5 years. We classified them according to information providers and evaluated credibility by giving points 3, 2, 1 to correct, mostly correct but ambiguous, and incorrect contents, respectively. We compared the credibility of the groups with each other. RESULTS: Sixty four articles from newspapers, and 789 and 506 medical counselings from portal websites and internet health counseling sites were selected, respectively. The kidney information providers in newspapers were medical journalists(doctors)(31.2%), kidney specialists(doctors) (23.4%) and so on. The consultants in the portal sites were doctors(49.1%)and anonymous reporters(49.9%). In internet health counseling sites, 91% of the consultants were doctors. All articles in the newspapers were credible. Doctors' answers were more credible than nonphysicians'(P=0.005) and anonymous contributors(P<0.001) in portal sites. In health counseling sites, doctors answered more reliably than nonphysicians. CONCLUSIONS: The kidney information in newspapers was credible. It is important for questioners to confirm the type of consultants in websites. We suggest that doctors, especially kidney specialists need to increase their roles in offering information to mass media.
Anonyms and Pseudonyms ; Consultants ; Counseling ; Humans ; Internet ; Kidney ; Mass Media ; Periodicals ; Specialization

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The Effect of Growth Hormone and the Factors Influencing Growth in Pediatric Chronic Peritoneal Dialysis Patients.

Su Jin KIM ; Sung Won PARK ; Young Bae SOHN ; Dong Kyu JIN ; Kyung Hoon PAIK

Journal of the Korean Society of Pediatric Nephrology.2008;12(1):38-46.

PURPOSE: Growth failure is a common problem in chronic renal failure(CRF). We studied the effect of growth hormone(GH) treatment and the factors influencing growth on chronic peritoneal dialysis patients. METHODS: Seventeen patients who were treated with peritoneal dialysis and GH for more than one year were enrolled. Factors influencing growth such as age, height at start of GH treatment, total Kt/Vurea, residual renal Kt/Vurea, hemoglobin, albumin, BUN, creatinine, total CO2, calcium, phosphate and iPTH during GH treatment were compared between the growth group(increase in height-standard deviation score(Ht-SDS) after one year of GH treatment, n=11) and poor growth group(no increase in Ht-SDS after one year of GH treatment, n=6). RESULTS: The mean age at the start of dialysis was 7.7+/-5.2 years and the mean age at the start of GH treatment was 8.5+/-4.8 years. In the growth group, Ht-SDS at start of GH treatment was smaller(-1.72+/-1.00 vs. -0.77+/-0.88, P=0.048) and residual renal Kt/Vurea was better (1.54+/-0.51 vs. 0.15+/-0.26, P=0.02) than the poor growth group. After three years of GH treatment, Ht-SDS of the growth group was better than the poor growth group. CONCLUSIONS: GH treatment in children with peritoneal dialysis was more effective on patients who had more severe growth retardation. The reservation of residual renal function was important for improvement of effect of GH treatment. And the growth response during the first year of GH treatment may be predicted as the indicator for long-term response.
Calcium ; Child ; Creatinine ; Dialysis ; Growth Hormone ; Hemoglobins ; Humans ; Kidney Failure, Chronic ; Peritoneal Dialysis

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Clinical Analysis of Acute Intrinsic Renal Failure in Neonates and Children.

Eunji KWON ; Ji Mi JUNG ; Chung Woo YEUNG

Journal of the Korean Society of Pediatric Nephrology.2008;12(1):30-37.

PURPOSE: The present study is an investigation of the progression and prognosis of acute intrinsic renal failure in neonates and children with a diagnosis of acute renal failure or other diseases on admission. METHODS: This research is based on a retrospective analysis conducted on 59 patients(male: female=2.2:1) diagnosed with acute intrinsic renal failure between January 2000 and June 2006 at Busan Paik Hospital. The clinical diagnostic criteria of acute renal failure used was serum creatinine <1.2 mg/dL, oliguria with urine output< or =0.5 mL/kg/hr and anuria with urine output <50 mL per day. RESULTS: Among those placed under investigation, 7 patients were neonates, 10 patients were 2 months-2 years old, 12 patients were 3-6 years old, 21 patients were 7-12 years old and 9 patients were 13-16 years old. It took 3.1+/-2.8 days on average until the diagnosis was made. The urine output distribution was 21 persons for the oliguria group, and 36 persons for the non-oliguria group, and 2 persons for the anuria group. For the underlying causes, 30 persons were classified in the primary renal disease group, 14 persons in the infection group, 9 persons in the malignancy group, and 6 persons were categorized in another group. As for age distribution, the infected group was predominantly neonates, whereas the dominant age ranges for the primary renal disease and infection categories were 2 months to 2 years old. Also, the primary renal disease was dominant among older children, aged 3 and up. No difference was detected according to seasonal prevalence. However, there was a high morbidity rate among hemolytic uremic syndrome diagnosed in the summer. Peritoneal dialysis was used to treat 4 patients. It took 10.0+/-6.7 days until the patients improved. 18 patients died. The non-oliguria group's mortality rate was lower than other groups. There was a high mortality rate in the neonates and malignancy group. CONCLUSIONS: Acute renal failure in childhood seems to take a better clinical course than in adulthood when there is an early diagnosis and proper treatment of underlying diseases.
Acute Kidney Injury ; Age Distribution ; Aged ; Anuria ; Child ; Creatinine ; Early Diagnosis ; Hemolytic-Uremic Syndrome ; Humans ; Infant, Newborn ; Oliguria ; Peritoneal Dialysis ; Prevalence ; Prognosis ; Renal Insufficiency ; Retrospective Studies ; Seasons

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