Journal Detail Information

1

Cite

Share

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

2

Cite

Share

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

3

Cite

Share

A Case of Azathioprine Induced Severe Myelosuppression and Alopecia Totalis in IgA Nephropathy.

Jae Choon KIM ; Ye Kyung KIM ; Hye Sun HYUN ; Eu Jin PARK ; Hee Gyung KANG ; Il Soo HA ; Hae Il CHEONG

Childhood Kidney Diseases.2017;21(1):35-39. doi:10.3339/jkspn.2017.21.1.35

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.
Alopecia* ; Azathioprine* ; Bone Marrow ; Child ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Granulocyte Colony-Stimulating Factor ; Hair ; Humans ; Immunoglobulin A* ; Male ; Metabolism ; Pancytopenia

4

Cite

Share

Spontaneous Tumor Lysis Syndrome Presenting Acute Kidney Injury with Extreme Hyperuricemia and Urinary Stone: A Rare Case of Spontaneous Tumor Lysis Syndrome.

Seong Heon KIM ; Eu Jeen YANG ; Young Tak LIM ; Su Young KIM

Childhood Kidney Diseases.2017;21(1):31-34. doi:10.3339/jkspn.2017.21.1.31

Tumor lysis syndrome is a serious complication of malignancy, resulting from the massive and rapid release of cellular components into the blood. Generally, it occurs after initiation of chemotherapy. The onset of spontaneous tumor lysis syndrome (STLS) before anti-cancer treatment is rare and occurs mostly in Burkitt lymphoma and non-Hodgkin's lymphoma. There are only a few case reports in children. Here, we report a case of STLS secondary to T-cell acute lymphoblastic leukemia (ALL), which presented with urinary stone and subsequent acute kidney injury with severe hyperuricemia. Occult malignancy should be considered in case of unexplained acute kidney injury with extreme hyperuricemia.
Acute Kidney Injury* ; Burkitt Lymphoma ; Child ; Drug Therapy ; Humans ; Hyperuricemia* ; Lymphoma, Non-Hodgkin ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; T-Lymphocytes ; Tumor Lysis Syndrome* ; Urinary Calculi*

5

Cite

Share

A Contrast Nephropathy in a Preterm Infant Following Preoperative Embolization of Giant Sacrococcygeal Teratoma.

Byong Sop LEE

Childhood Kidney Diseases.2017;21(1):26-30. doi:10.3339/jkspn.2017.21.1.26

Newborn infants with huge and highly vascular sacrococcygeal teratoma (SCT) are frequently subjected to renal hypoperfusion secondary to high-output cardiac failure. Any underlying renal dysfunction is a significant risk factor for the development of contrast-induced nephropathy (CIN). However, reports on CIN in infants are rare. I report here a case of a premature infant born at 28 weeks and 3 days of gestation with a huge SCT who survived preoperative embolization and surgical resection but presented with persistent non-oliguric renal failure that was suggestive of CIN. During radiological intervention, a contrast medium had been administered at about 10 times the manufacturer-recommended dose for pediatric patients. Despite hemodynamic stabilization and normalization of urine output immediately following surgery, the patient's serum creatinine and cystatin-C levels did not return to baseline until 4 months after birth. No signs of reflux nephropathy were observed in follow-up imaging studies. Dosing guidelines for the use of a contrast medium in radiological interventions should be provided for infants or young patients.
Acute Kidney Injury ; Creatinine ; Embolization, Therapeutic ; Follow-Up Studies ; Heart Failure ; Hemodynamics ; Humans ; Infant ; Infant, Newborn ; Infant, Premature* ; Parturition ; Pregnancy ; Renal Insufficiency ; Risk Factors ; Teratoma*

6

Cite

Share

A Case of Severe Hypercalcemia Causing Acute Kidney Injury: An Unusual Presentation of Acute Lymphoblastic Leukemia.

Hye Sun HYUN ; Peong Gang PARK ; Jae Choon KIM ; Kyun Taek HONG ; Hyoung Jin KANG ; Kyung Duk PARK ; Hee Young SHIN ; Hee Gyung KANG ; Il Soo HA ; Hae Il CHEONG

Childhood Kidney Diseases.2017;21(1):21-25. doi:10.3339/jkspn.2017.21.1.21

Severe hypercalcemia is rarely encountered in children, even though serum calcium concentrations above 15-16 mg/dL could be life-threatening. We present a patient having severe hypercalcemia and azotemia. A 14-year-old boy with no significant past medical history was referred to our hospital with hypercalcemia and azotemia. Laboratory and imaging studies excluded hyperparathyroidism and solid tumor. Other laboratory findings including a peripheral blood profile were unremarkable. His hypercalcemia was not improved with massive hydration, diuretics, or even hemodialysis, but noticeably reversed with administration of calcitonin. A bone marrow biopsy performed to rule out the possibility of hematological malignancy revealed acute lymphoblastic leukemia. His hypercalcemia and azotemia resolved shortly after initiation of induction chemotherapy. Results in this patient indicate that a hematological malignancy could present with severe hypercalcemia even though blast cells have not appeared in the peripheral blood. Therefore, extensive evaluation to determine the cause of hypercalcemia is necessary. Additionally, appropriate treatment, viz., hydration or administration of calcitonin is important to prevent complications of severe hypercalcemia, including renal failure and nephrocalcinosis.
Acute Kidney Injury* ; Adolescent ; Azotemia ; Biopsy ; Bone Marrow ; Calcitonin ; Calcium ; Child ; Diuretics ; Hematologic Neoplasms ; Humans ; Hypercalcemia* ; Hyperparathyroidism ; Induction Chemotherapy ; Leukemia ; Male ; Nephrocalcinosis ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Renal Dialysis ; Renal Insufficiency

7

Cite

Share

Clinical Characteristics of Febrile UTI First Developed Over 5 Years of Age.

Da Eun ROH ; Hyo Rim SUH ; So Yoon MIN ; Tae Kyoung JO ; Hee Sun BAEK ; Min Hyun CHO

Childhood Kidney Diseases.2017;21(1):15-20. doi:10.3339/jkspn.2017.21.1.15

PURPOSE: Febrile urinary tract infection (UTI) is one of the commonest bacterial infections in children. The purpose of this study is to investigate the clinical characteristics of the first episode of febrile UTI occurring in children over 5 years compared to those in infants younger than a year. METHODS: We retrospectively reviewed the medical records of 10 patients over 5 years, having febrile UTI, and 25 controls under 1 year. Clinical characteristics including symptoms at admission, the time interval between symptom onset and hospital visit and/or diagnosis, duration of fever, urinalysis, and other laboratory and imaging test results were compared between the two groups. RESULTS: Most patients in the control group showed only high fever at the time of presentation to the hospital. However, 60% of the case group had fever along with gastrointestinal (GI) symptoms such as abdominal and flank pain, vomiting, as well as relatively mild pyuria. The case group showed a longer duration between symptom onset and hospital visit and/or diagnosis. CONCLUSIONS: Delay in diagnosis and initiation of treatment of UTI increases the risk of permanent renal scarring and associated complications. Therefore, early diagnosis and treatment of febrile UTI is vital for very young infants, as well as children considering that febrile UTI could be an important cause of febrile illness in children over 5 years.
Bacterial Infections ; Child ; Cicatrix ; Diagnosis ; Early Diagnosis ; Fever ; Flank Pain ; Humans ; Infant ; Medical Records ; Pyuria ; Retrospective Studies ; Urinalysis ; Urinary Tract Infections ; Vomiting

8

Cite

Share

Effect of Diet and Water Intake on Aquaporin 2 Function.

Jun Mo KIM ; Tae Hee KIM ; Tong WANG

Childhood Kidney Diseases.2016;20(1):11-17. doi:10.3339/jkspn.2016.20.1.11

Appropriate control of diet and water intake is important for maintaining normal blood pressure, fluid and electrolyte homeostasis in the body. It is relatively understood that the amount of sodium and potassium intake directly affects blood pressure and regulates ion transporters; Na and K channel functions in the kidney. However, little is known about whether diet and water intake regulates Aquaporin (AQP) function. AQPs, a family of aquaporin proteins with different types being expressed in different tissues, are important for water absorption by the cell. Water reabsorption is a passive process driven by osmotic gradient and water permeability is critical for this process. In most of the nephron, however, water reabsorption is unregulated and coupled to solute reabsorption, such as AQP1 mediated water absorption in the proximal tubule. AQP2 is the only water channel founded so far that can be regulated by hormones in the kidney. AQP2 expressed in the apical membrane of the principal cells in the collecting tubule can be regulated by vasopressin (antidiuretic hormone) controlling the final volume of urine excretion. When vasopressin binds to its receptor on the collecting duct cells, it stimulates the translocation of AQP2 to the membrane, leading to increased water absorption via this AQP2 water channel. However, some studies also indicated that the AQP2 is also been regulated by vasopressin independent mechanism. This review is focused on the regulation of AQP2 by diet and the amount of water intake on salt and water homeostasis.
Absorption ; Aquaporin 2* ; Arginine Vasopressin ; Blood Pressure ; Diet* ; Drinking* ; Homeostasis ; Humans ; Ion Transport ; Kidney ; Membranes ; Nephrons ; Osmolar Concentration ; Permeability ; Potassium ; Sodium ; Vasopressins ; Water*

9

Cite

Share

Clinical Manifestation Patterns and Trends in Poststreptococcal Glomerulonephritis.

Kee Hyuck KIM

Childhood Kidney Diseases.2016;20(1):6-10. doi:10.3339/jkspn.2016.20.1.6

Poststreptococcal glomerulonephritis (PSGN) is one of the most recognized diseases in pediatric nephrology. Typical clinical features include rapid onset of gross hematuria, edema, and hypertension, and cases are typically preceded by an episode of group A β-hemolytic streptococcus pharyngitis or pyoderma. The most common presenting symptoms of PSGN are the classic triad of glomerulonephritis: gross hematuria, edema, and hypertension . However, patients with PSGN sometimes present with unusual or atypical clinical symptoms that often lead to delayed diagnosis or misdiagnosis of the disease and increased morbidity. Additionally, the epidemiology of postinfectious glomerulonephritis (PIGN), including PSGN, has changed over the past few decades. This paper reviews atypical clinical manifestations of PSGN and discusses the changing demographics of PIGN with a focus on PSGN.
Delayed Diagnosis ; Demography ; Diagnostic Errors ; Edema ; Epidemiology ; Glomerulonephritis* ; Hematuria ; Humans ; Hypertension ; Nephrology ; Pharyngitis ; Pyoderma ; Streptococcus

10

Cite

Share

Antibiotics and Probiotics Prophylaxis for Recurrent Urinary Tract Infection in Children.

Jung Won LEE

Childhood Kidney Diseases.2016;20(1):1-5. doi:10.3339/jkspn.2016.20.1.1

Since many years, continuous low dose antibiotic prophylaxis (CAP) has been used for children at a risk for recurrent urinary tract infection (UTI), especially those with vesicoureteral reflux (VUR). The incidence of recurrent UTI has been shown to be higher in children with VUR with bladder and bowel dysfunction (BBD) than in those with VUR without BBD. Therefore, CAP has been recommended for children with BBD and VUR because of the increased risk of UTI. However, the use of CAP has become highly controversial because of bacterial resistance developed due to antibiotic over-usage. The preventive effects of probiotics have been proved in various adult urogenital infections, and the antimicrobial activities of lactobacilli against uropathogens have been demonstrated in previous in vitro studies. However, a critical assessment of their efficacy in children with UTI is lacking. The importance of the use of urogenital probiotics is that it is a natural approach that replenishes the depleted normal flora to create a better environment to fight off uropathogens. Probiotics have a great potential, particularly today with the increasing threat of antibiotic-resistant microorganisms.
Adult ; Anti-Bacterial Agents* ; Antibiotic Prophylaxis ; Child* ; Humans ; Incidence ; Probiotics* ; Urinary Bladder ; Urinary Tract Infections* ; Urinary Tract* ; Vesico-Ureteral Reflux

DISPLAY OPTIONS

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share