• Childhood Kidney Diseases
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• v.22 no.1
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• pp.12-16
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• 2018

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation syndrome that is characterized by a triad of uterine didelphys, blind hemivagina, and ipsilateral renal agenesis. There is a wide variety of phenotypic presentation which is recognized as a spectrum of disease rather than a separate entity. The exact incidence and pathogenesis of HWW syndrome are yet to be investigated. While this disease typically involves adolescent girls who present with abdominal pain or a pelvic mass that is secondary to hematocolpos, nowadays, a majority of potential patients with HWW are being prenatally screened for renal anomalies. Therefore, it is recommended to search for uterovaginal anomalies whenever a multicystic dysplastic kidney or the absence of a kidney is noted in a newborn female, and the role of pediatric nephrologists has become ever more important for early recognition of the disease.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.118-129
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• 2009

Continuous renal replacement therapy (CRRT) has been used in critically ill children for more than 20 years in the world, but in Korea, although it is becoming the preferred method of acute therapy in pediatric intensive care units, there has been a few experiences and no consensus of CRRT up to now. This review describes the basic technique of CRRT, highlights the between the adult and pediatric prescription, and elaborates on the main controversies in the application of CRRT in children.

• Journal of Veterinary Clinics
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• v.18 no.4
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• pp.459-464
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• 2001

Excretory urography is a procedure where opacification of the kidneys, renal pelvic diverticula, ureters, and urinary bladder is a result of renal excretion of an intravenously administered iodinated contrast agent providing both anatomical and functional assessment. And ultrasonography is a non-invasive modality to evaluate the important anatomic information concerning the size, shape, and internal architecture of kidney even in the presence of impaired renal function or abdominal fluid. We describe four dogs with urological signs diagnosed with excretory urography and ultrasonography. Parients showed a variety of clinical signs including vomiting, hematuria, anorexia, abdominal pain, and abdominal distension. The hydronephrosis was diagnosed in case 1, 2, and 3 that had pelvic dilation, dilation of pelvic recesses, ureteral dilation. In case 3, proximal ureteral rupture was diagnosed with evidence of contrast media leakage was seen in proximal ureter. In case 4, the rupture of urinary bladder was diagnosed with leakage of contrast media through its ventral portion.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.166-169
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• 1997

The nutcracker syndorme refers to compression of the left renal vein between the aorta and the superior mesentric artery which results in renal vein, left gonadal vein varices, hematuria and left sided flank pain. We report this experience of 11yr-11mon of girl has typical Nutcracker syndrome with persistent proteinuria and without typical hematuria. According to the renal biopsy for persistent proteinuria, biopsy shows pathologic findings similar to minimal change nephrotic syndrome. All symtpoms relieved without any specific treatments but she had no response to steroid treatment for persistent proteinuria. Now she was followed up through OPD base without symptom and consideration of surgical intervention.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.23-29
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• 2008

Kidney allograft transplantation is the most effective method of renal replacement for end stage renal disease patients. Still, it is another kind of 'disease', requiring immunosuppression to keep the allograft from rejection(allograft immune reaction). Immune system of the allograft recipient recognizes the graft as a 'pathogen (foreign or danger)', and the allograft-recognizing commanderin-chief of adaptive immune system, T cell, recruits all the components of immune system for attacking the graft. Proper activation and proliferation of T cell require signals from recognizing proper epitope(processed antigen by antigen presenting cell) via T cell receptor, costimulatory stimuli, and cytokines(IL-2). Thus, most of the immunosuppressive agents suppress the process of T cell activation and proliferation.

• Childhood Kidney Diseases
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• v.21 no.2
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• pp.136-141
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• 2017

Purpose: This study aimed to investigate clinical and radiological factors that may predict high-grade vesicoureteral reflux (VUR) in patients with febrile urinary tract infection (UTI). Methods: We retrospectively analyzed medical records of 446 patients diagnosed with febrile UTI from March 2008 to February 2017. All patients underwent renal-bladder ultrasonography (RBUS), 99mTc dimercaptosuccinic acid (DMSA) renal scan, and voiding cystourethrography (VCUG), and were divided in to 3 groups: a high-grade VUR group (n=53), a low-grade VUR group (n=28), and a group without VUR (n=365). Results: The recurrence and non-Escherichia coli infection rates in febrile UTI were significantly higher in the high-grade VUR group than in the other two groups (P<0.05). RBUS showed that hydronephrosis and ureter dilatation were more frequent in the high-grade VUR group than in the other groups (P<0.05). In the high-grade VUR group, a renal cortical defect was more likely to appear as multiple defects, and the difference in bilateral renal scan uptake between both kidneys was larger than in the other two groups (P<0.001). Conclusion: Recurrent UTI, non-E. coli UTI, abnormal findings on RBUS such as hydronephrosis and ureter dilatation, and abnormal findings in the DMSA renal scan such as multiple renal cortical defects and greater uptake difference were associated with high-grade VUR. VCUG should be selectively performed when RBUS and/or DMSA renal scan reveal significant abnormalities.

• Childhood Kidney Diseases
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• v.21 no.2
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• pp.75-80
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• 2017

Purpose: To investigate the frequency, presentation, management, and outcome of cytomegalovirus (CMV) infection in pediatric patients who underwent renal transplantation. Methods: We performed a retrospective chart review of 70 patients under the age of 18, who underwent renal transplantation between January 1990 and November 2014. A diagnosis of CMV infection was based on serology, molecular assays, antigenemia assays, and culture. CMV infection was defined as detection of virus and CMV disease was diagnosed when clinical signs and symptoms were present. Results: The number of patients with CMV infection was 18 (25.7% of renal transplant recipients). Twelve were male (66.7%), and the $mean{\pm}standard$ deviation (SD) age at infection was $13.3{\pm}3.9$ years. Median time of infection after renal transplantation was 4 months (range 1.0-31.0 months). Pretransplantation CMV status in the infected group was as follows: donor (D)+/recipient (R)+, 11 (61.1%); D+/R-, 7 (38.9%); D-/R+, 0; and D-/R- 0. Nine patients had CMV disease with fever, leukopenia, thrombocytopenia, or organ involvement such as enteritis, hepatitis, and pneumonitis. The age of disease occurrence was $13.1{\pm}3.9$ years and the median time to disease onset after renal transplantation was 8 months (range 1.0-31.0). Immunosuppressive agents were reduced or discontinued in 14 patients (77.8%), antiviral agents were used in 11 patients (61.1%), and all patients with CMV infection were controlled. Conclusions: A quarter of the patients had CMV infection about 4 months after renal transplantation. CMV infection was successfully treated with reduction of immunosuppressants or with antiviral agents.

• Asian Pacific Journal of Cancer Prevention
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• v.14 no.3
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• pp.1597-1599
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• 2013

Since renal failure causes decrease in tumor marker excretion, use of these markers in cancer care and treatment in patients with renal insufficiency or hemodialysis is controversial. The aim of this study was to investigate differences of serum levels of tumor markers CA15-3, AFP, CA19-9 and CEA in patients with impaired renal function. A total of 100 patients referred to the Tabriz Immam Reza and Amiralmomenin hospital from June 2010 to November 2011 were selected for study. Subjects were divided to 3 groups of healthy, dialysis and renal failure but non hemodialysis cases, the last category being re-grouped based on creatinine clearance. No significant relationship between different groups in serum levels of CEA (P=0.99) and CA19-9 (P=0.29) tumor markers was found. A significant correlation was observed between serum levels of AFP (P<0.001) and CA15-3 (P<0.001) and also a tendency between creatinine clearance and CEA (r=0.05, P=0.625). Creatinine clearance significantly correlated with AFP (P<0.001, r=0.53) and CA15-3 (p=0.00, r=-0.412), but not CA19-9 (P=0.089, r=-0.171). According to results of this study it appears that use of tumor markers in patients with impaired renal function should be performed with special precautions.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.262-266
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• 2008

Crossed renal ectopia is a congenital malformation in which both kidneys lie on the same side of the spine, usually side by side longitudinally. More often on the right side. Fusion of the two renal units is eight times more common than nonfusion. Although crossed renal ectopia is uncommon, this unusual entity must be considered in an infant when cystic mass in the abdomen or pelvis paticularly if no kidney can be found on the opposite side. In many cases of crossed fused ectopia with multicystic dysplastic kidney(MCDK), the diagnosis can be strongly suspected from the sonogram, and no other studies may be necessary. However, both intravenous urography and isotope renography is useful to assess the function of the crossed kidney. Crossed renal ectopia and MCDKs are associated with a greater incidence of ureteropelvic junction obstruction and reflux. So, screening voiding cystourethrography should be performed. Very few studies of MCDK in the setting of crossed fused ectopia have been reported. We have experienced a 3-year-old boy with crossed fused renal ectopia with multicystic dysplasia.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.65-71
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• 2006

Idiopathic renal hypouricemia is a disorder characterized by impaired urate handling in the renal tubules. Most patients with hypouricemia are asymptomatic and are found incidentally, but the condition is known to be at high risk for exercise-induced acute renal failure or urolithiasis. URAT1 protein encoded by SLC22A12 gene has been identified recently as a urate/anion exchanger in the human kidney. Inactivation mutations in SLC22A12 gene have been shown to cause renal idiopathic hypouricemia. We experienced a 3-year-old boy who presented with persistent orange-colored urine since infancy. His urine contained many uric acid crystals, while the serum showed hypouricemia(0.7 mg/dL). The fractional excretion of uric acid was increased to 41.7%. SLC22a12 gene analysis revealed W258X homozygote alleles. Renal hypouricemia must be included in the differential diagnosis of red-urine and SLC22A12 gene analysis is recommended in idiopathic renal hypouricemia.