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Clinical and Pathological Findings of Renal Biopsy in Children: Outcomes from a Single Center Over 27 Years.

Shin Ae LEE ; Min Sun KIM ; Soon Chul KIM ; Dae Yeol LEE

Childhood Kidney Diseases.2017;21(1):8-14. doi:10.3339/jkspn.2017.21.1.8

PURPOSE: To classify the results of renal biopsy in pediatric patients and to compare pathological findings with clinical features. METHODS: This study included data of 318 children who underwent renal biopsy at our hospital between December 1987 and November 2014. Biopsy specimens were examined histopathologically using light, immunofluorescence, and electron microscopy. RESULTS: Asymptomatic urinary abnormalities was the most common clinical diagnosis (35.9%), followed by nephrotic syndrome (29.3%), and acute glomerulonephritis (18.0%). Glomerular disease was identified in 98.1% of the renal biopsy specimens. The most common primary cause of glomerulonephritis was IgA nephropathy, with gross hematuria in 61.9% of the patients, hypertension in 14.2%, proteinuria >1.0 gm/24-hr in 33.3%, and impaired renal function in 3.6% patients. CONCLUSION: The most common clinical diagnosis was asymptomatic urinary abnormalities, with primary glomerular disease being the most common renal biopsy finding, and IgA nephropathy the most common histopathological lesion. This study provides a 27-year overview of pediatric renal disease at our center and underlines the importance of renal biopsy for accurate diagnosis and proper management.
Biopsy* ; Child* ; Diagnosis ; Fluorescent Antibody Technique ; Glomerulonephritis ; Glomerulonephritis, IGA ; Hematuria ; Humans ; Hypertension ; Microscopy, Electron ; Nephrotic Syndrome ; Proteinuria

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Mitogen-activated Protein Kinases in the Development of Normal and Diseased Kidneys.

Midori AWAZU

Childhood Kidney Diseases.2017;21(1):1-7. doi:10.3339/jkspn.2017.21.1.1

Mitogen-activated protein kinases (MAPKs) play important roles in various cellular functions including proliferation, differentiation, and apoptosis. We showed that MAPKs are developmentally regulated in the rat kidney. p38 MAPK (p38) and extracellular signal-regulated kinase (ERK) were strongly expressed in the fetal kidney, whereas c-Jun N-terminal kinase (JNK) was detected predominantly in the adult kidney. The inhibition of p38 or ERK in organ culture resulted in reduced nephron formation with or without reduced kidney size. On the other hand, persistent fetal expression pattern of MAPKs, i.e., upregulation of p38 and ERK and downregulation of JNK, was observed in the cyst epithelium of human renal dysplasia, ovine fetal obstructive uropathy, and pcy mice, a model of polycystic kidney disease. Furthermore, activated p38 and ERK induced by cyclic stretch mediated proliferation and TGF-β1 expression in ureteric bud cells, probably leading to cyst formation and dysplastic changes. Inhibition of ERK slowed the disease progression in pcy mice. Finally, ERK and p38 were inactivated in the early embryonic kidney subjected to maternal nutrient restriction, characterized by reduced ureteric branching and nephron number. Thus, MAPKs mediate the development of normal and diseased kidney. Their modulation may result in novel therapeutic strategies against developmental abnormalities of the kidney.
Adult ; Animals ; Apoptosis ; Disease Progression ; Down-Regulation ; Epithelium ; Hand ; Humans ; JNK Mitogen-Activated Protein Kinases ; Kidney* ; Mice ; Mitogen-Activated Protein Kinases* ; Nephrons ; Organ Culture Techniques ; p38 Mitogen-Activated Protein Kinases ; Phosphotransferases ; Polycystic Kidney Diseases ; Rats ; Up-Regulation ; Ureter

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A Patient with Henoch-Schönlein Purpura with Intussusception and intractable Nephritis.

Min Kyoung SEO ; Jeong HONG ; Hyun Ee YIM ; Ki Soo PAI

Childhood Kidney Diseases.2016;20(2):92-96. doi:10.3339/jkspn.2016.20.2.92

Henoch-Schönlein purpura (HSP) is the most common vasculitis in children, mainly affecting the small vessels of the skin, joints, gastrointestinal tract, and kidneys. Although most cases of HSP resolve spontaneously without sequelae, serious nephrological and intestinal problems may occur in some cases. We experienced a case of HSP complicated by simultaneous intussusception and nephritis in a 14-year-old boy who developed a sudden abdominal pain and gross hematuria on the 11th day after onset of the disease. Imaging studies revealed intussusception that required emergency laparotomy. Despite treatment with steroid and angiotensin-converting enzyme inhibitors, nephritis and nephrosis progressed for 4 weeks, and renal biopsy was performed to confirm the diagnosis. Cyclosporin A therapy was started, and remission of proteinuria was achieved after 5 months. However, the nephritis recurred and worsened to end-stage renal failure during 15 years of follow-up.
Abdominal Pain ; Adolescent ; Angiotensin-Converting Enzyme Inhibitors ; Biopsy ; Child ; Cyclosporine ; Diagnosis ; Emergencies ; Follow-Up Studies ; Gastrointestinal Tract ; Hematuria ; Humans ; Intussusception* ; Joints ; Kidney ; Kidney Failure, Chronic ; Laparotomy ; Male ; Nephritis* ; Nephrosis ; Proteinuria ; Purpura* ; Renal Insufficiency ; Skin ; Vasculitis

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Nonobstructive Bilateral Hydronephrosis & Hydroureter from Nephrogenic Diabetes Insipidus with a Novel Mutation of AQP2 Gene (p.A123G).

Ki Sup SONG ; Jeon Ihn SOO ; Mee Kyung NAMGOONG

Childhood Kidney Diseases.2016;20(2):88-91. doi:10.3339/jkspn.2016.20.2.88

Nephrogenic diabetes insipidus (NDI) can cause nonobstructive hydronephrosis. Congenital NDI (CNDI) is caused by a genetic mutation. This case report presents a 12-year-old girl who was incidentally diagnosed with nonobstructive hydronephrosis due to NDI caused by AQP2 gene mutation after being evaluated for microscopic hematuria found on routine health examination at school. The patient's medical and family history was unremarkable, and she complained of nocturia only at the time of the clinic visit. Bilateral hydronephrosis on abdominal ultrasonography prompted a water deprivation test, leading to diagnosis of NDI. Genetic study confirmed p.Asn (AAC)123Ser (AGC) in exon 2 of the AQP2 gene. Polyuria and hydronephrosis improved following arginine-vasopressin therapy. CNDI responsive to treatment should be considered as a possible cause of nonobstructive hydroureter.
Ambulatory Care ; Child ; Diabetes Insipidus, Nephrogenic* ; Diagnosis ; Exons ; Female ; Hematuria ; Humans ; Hydronephrosis* ; Nocturia ; Polyuria ; Ultrasonography ; Water Deprivation

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A Case of Secondary FSGS due to Chronic Chloride Diarrhea.

Byung Kwan KIM ; Hyun Soon LEE ; Hyung Eun YIM ; Hae Il CHEONG ; Kee Hwan YOO

Childhood Kidney Diseases.2016;20(2):83-87. doi:10.3339/jkspn.2016.20.2.83

Congenital chloride diarrhea (CLD) is a rare autosomal recessive disease that is difficult to diagnose. CLD requires early treatment to correct electrolyte imbalance and alkalosis and to prevent severe dehydration. Renal injury is clearly associated with defective electrolyte balance induced by CLD, particularly during the first months or years of life. A 7-year-old boy was diagnosed with CLD following detection of a homozygous mutation (c.2063-1G>T) in SLC26A3 at 6 months of age. During treatment with electrolyte supplements, mild proteinuria was detected at 8 months of age, and is still present. Renal biopsy showed the presence of focal renal dysplasia, with metaplastic cartilage and mononuclear cell infiltration, calcification, and fibrosis in the interstitium. Up to two-thirds of the glomeruli exhibited global obsolescence, mostly aggregated in the dysplastic area. In nondysplastic areas, the glomeruli were markedly increased in size and severely hypercellular, with increased mesangial matrix, and displayed segmental sclerosis. The marked glomerular hypertrophy with focal segmental glomerulosclerosis suggested a compensatory reaction to the severe nephron loss or glomerular obsolescence associated with renal dysplasia, with superimposed by CLD aggravating the tubulointerstitial damage.
Alkalosis ; Biopsy ; Cartilage ; Child ; Dehydration ; Diarrhea* ; Fibrosis ; Glomerulosclerosis, Focal Segmental ; Humans ; Hypertrophy ; Male ; Nephrons ; Proteinuria ; Sclerosis ; Water-Electrolyte Balance

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A 10-year-old Boy with Microscopic Hematuria and Renal Biopsy Findings Mimicking Fabry Disease.

Woo Yeong CHUNG ; Mi Seon KANG

Childhood Kidney Diseases.2016;20(2):79-82. doi:10.3339/jkspn.2016.20.2.79

Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of the enzyme α-galactosidase A, resulting in the accumulation of glycosphingolipids within the lysosomes of various cell types. It has a wide spectrum of clinical phenotypes, and renal failure is a serious complication. Fabry disease is confirmed either by measurement of α-galactosidase A activity or by genetic testing for GLA mutations. Renal biopsy findings on light microscopy, specifically enlarged podocytes with foamy cytoplasm, and osmiophilic inclusion bodies in the cytoplasm in all types of renal cells on electron microscopy, are characteristic of this disease. The predominant differential diagnosis is iatrogenic phospholipidosis in association with certain drugs that can cause cellular injuries indistinguishable from Fabry disease. Here, we report the case of a 10-year-old boy with microscopic hematuria who underwent a renal biopsy that showed morphological findings consistent with Fabry disease, although the patient had neither a GLA mutation nor a history of drug consumption. Six years later, spontaneous regression of this renal pathology was observed in a second renal biopsy examination.
Biopsy* ; Child* ; Cytoplasm ; Diagnosis, Differential ; Fabry Disease* ; Genetic Testing ; Glycosphingolipids ; Hematuria* ; Humans ; Inclusion Bodies ; Lysosomes ; Male* ; Microscopy ; Microscopy, Electron ; Pathology ; Phenotype ; Podocytes ; Renal Insufficiency

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Prognostic Factors of Renal Scarring on Follow-up DMSA Scan in Children with Acute Pyelonephritis.

Juyeen LEE ; Byung Woo WOO ; Hae Sook KIM

Childhood Kidney Diseases.2016;20(2):74-78. doi:10.3339/jkspn.2016.20.2.74

PURPOSE: Early diagnosis and treatment of urinary tract infection have been emphasized to prevent renal scarring. If untreated, acute pyelonephritis could cause renal injury, which leads to renal scarring, hypertension, proteinuria, and chronic renal failure. The purpose of this study was to assess risk factors of renal scarring after treatment of acute pyelonephritis (APN). METHODS: The medical records of 59 patients admitted at Daegu Fatima Hospital because of APN between March 2008 and April 2015 whose renal cortical defects were confirmed by using initial technetium-99m dimercaptosuccinic acid (DMSA) scans were reviewed retrospectively. We divided 59 patients into 2 groups according to the presence of renal scar and assessed risk factors of renal scar, including sex, age at diagnosis, feeding method, hydronephrosis, bacterial species, vesicoureteral reflux, and vesicoureteral reflux grade. RESULTS: Of 59 patients (41%), 24 showed renal scar on follow-up DMSA scan. No significant differences in sex, hydronephrosis, bacterial species, and fever duration were found between the renal-scarred and non-scarred groups. As for age at diagnosis, age of >12 months had 5.8 times higher incidence rate of renal scarring. Vesicoureteral reflux (VUR) affected renal scar formation. VUR grade III or IV had 14.7 times greater influence on renal scar formation than VUR grade I or II. CONCLUSION: Our data suggest that the presence of VUR and its grade and age at diagnosis are risk factors of renal scar on follow-up DMSA scan after APN.
Child* ; Cicatrix* ; Daegu ; Diagnosis ; Early Diagnosis ; Feeding Methods ; Fever ; Follow-Up Studies* ; Humans ; Hydronephrosis ; Hypertension ; Incidence ; Kidney Failure, Chronic ; Medical Records ; Proteinuria ; Pyelonephritis* ; Retrospective Studies ; Risk Factors ; Succimer* ; Urinary Tract Infections ; Vesico-Ureteral Reflux

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Predicting Factors of Roseola Infantum Infected with Human Herpesvirus 6 from Urinary Tract Infection.

Hong Ryul KO ; Son Moon SHIN ; Sung Won PARK

Childhood Kidney Diseases.2016;20(2):69-73. doi:10.3339/jkspn.2016.20.2.69

PURPOSE: The aim of this study was to compare the clinical and laboratory features of infants with roseola infantum due to human herpesvirus 6 (HHV6) infection and those with urinary tract infection (UTI). METHODS: We retrospectively reviewed the medical records of children who were hospitalized at Cheil General Hospital and Women's Health Care Center, College of Medicine, Dankook University, and diagnosed as having HHV6 infection or UTI. RESULTS: Among the infants admitted between September 2014 and May 2016, 92 (male, 45 and female, 47) were included in the study and divided into a HHV6 infection group (n=50) and a UTI group (n=42). The relative risk of UTI compared with that of HHV6 infection increased with pyuria (P<0.001), increased with leukocytosis (mean white blood cell [WBC] count, 15,048±5,756/mm³ vs 87,916±54,056/mm³; P<0.001), increased with C-reactive protein (CRP) level (4.89±4.85 mg/dL vs 1.04±1.76 mg/dL; P<0.001), and younger age (6.3±3.2 months vs 18.3±12.6 months; P<0.001). The relative risk of HHV6 infection compared with that of UTI increased with fever duration (4.3±1.7 days vs 2.8±1.7 days; P<0.001) and decreased with platelet (PLT) count (373±94×10³/mm³ vs 229±90×10³/mm³; P<0.001). No significant differences were found between the HHV6 groups according to the presence or absence of pyuria. CONCLUSION: Pyuria, age, fever duration, WBC count, CRP level, and PLT count were the differentiating factors of HHV6 infection from UTI. However, sterile pyuria can occur in children with HHV6 infection. In the presence of pyuria, CRP level and PLT count were the strong predictors of UTI compared with HHV6.
Blood Platelets ; C-Reactive Protein ; Child ; Exanthema Subitum* ; Female ; Fever ; Herpesvirus 6, Human* ; Hospitals, General ; Humans* ; Infant ; Leukocytes ; Leukocytosis ; Medical Records ; Pyuria ; Retrospective Studies ; Urinary Tract Infections* ; Urinary Tract* ; Women's Health

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Clinical Significance of Uptake Difference on DMSA Scintigraphy in Pediatric Urinary Tract Infection.

Byung Kwan KIM ; Won Jee CHOI ; Hyung Eun YIM ; Kee Hwan YOO

Childhood Kidney Diseases.2016;20(2):63-68. doi:10.3339/jkspn.2016.20.2.63

PURPOSE: Disruption of normal renal development can lead to congenital anomalies of the kidney and urinary tract, including renal hypodysplasia. We aimed to clarify whether small kidney size affects clinical manifestations in children with urinary tract infection (UTI). METHODS: One hundred fifty-four patients who had their first symptomatic UTI between January 2014 and June 2015 were enrolled in this study. Differences in kidney size were estimated based on percent uptake of (99m)Tc-dimercaptosuccinic acid (DMSA) in scintigraphy. The patients who showed more than 10% difference in kidney size on DMSA scintigraphy with none or minimal cortical defects were included in group A. (group A, n=17). Laboratory, clinical, and imaging results were compared with those of the other patients (group B, n=137). RESULTS: Group A had a relatively higher incidence of vesicoureteral reflux than group B (44% vs 20%, P<0.05). The levels of plasma neutrophil gelatinase-associated lipocalin (NGAL) and serum C-reactive protein were significantly higher in group A (193 [64-337] vs 91 [59-211] ng/mL and 4.1 [0.5-11.9] vs 2.1 [0.7-5.3] ng/mL, respectively; all P<0.05). Linear regression analysis revealed that plasma NGAL level strongly correlated with the difference in renal uptake in DMSA scintigraphy in group A (R²=0.505). CONCLUSION: The difference in kidney size could influence the clinical course and severity of pediatric UTI.
C-Reactive Protein ; Child ; Humans ; Incidence ; Kidney ; Linear Models ; Lipocalins ; Neutrophils ; Plasma ; Radionuclide Imaging* ; Succimer* ; Urinary Tract Infections* ; Urinary Tract* ; Vesico-Ureteral Reflux

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Which Factors Related to the Renal Cortical Defects in Infants Under 3 Months of Age with Urinary Tract Infections?.

Yu Kyung AN ; Myung Hyun CHO ; Kyo Sun KIM

Childhood Kidney Diseases.2016;20(2):57-62. doi:10.3339/jkspn.2016.20.2.57

PURPOSE: We used technetium-99m dimercaptosuccinic acid (DMSA) scintigraphy to identify factors predictive of renal cortical defects in infants <3 months of age with urinary tract infections (UTIs). METHODS: We retrospectively reviewed data on infants <3 months of age with culture-proven UTIs treated at a single center from March 2010 to February 2016. Blood samples were obtained for laboratory evaluation prior to commencement of antibiotic therapy. The therapeutic delay time (TDT) and therapeutic response time (TRT) were recorded. All patients were divided into two groups depending on features of their DMSA scans. We compared the demographic, clinical, and laboratory characteristics of the two groups. RESULTS: A total of 119 infants (94 males and 25 females; mean age, 56.9±21.3 days) were included. Cortical defects were evident in the DMSA scans of 47 cases (39.5%). In infants with such defects, the peak temperatures (38.9±0.57℃ vs. 38.4±0.81℃, P=0.001), the absolute neutrophil counts (8,920±4,460/mm vs. 7,290±4,090/mm, P=0.043), and the C-reactive protein (CRP) levels (6.49±4.33 mg/dL vs. 3.21±2.81 mg/dL, P=0.001) were significantly higher than those in infants without cortical defects. The TDT was also longer in those with cortical defects (P=0.037). CONCLUSION: We found that a TDT ≥8.5 hr (odds ratio [OR] 5.81), a peak temperature ≥38.3℃ (OR 6.19), and a CRP level ≥4.96 mg/dL (OR 7.26) predicted abnormal DMSA scan results in infants <3 months of age with UTIs.
C-Reactive Protein ; Female ; Humans ; Infant* ; Male ; Neutrophils ; Pyelonephritis ; Radionuclide Imaging ; Reaction Time ; Retrospective Studies ; Succimer ; Technetium Tc 99m Dimercaptosuccinic Acid ; Urinary Tract Infections* ; Urinary Tract*

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