• Childhood Kidney Diseases
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• 제27권1호
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• pp.40-45
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• 2023

Tubulointerstitial nephritis and uveitis (TINU) syndrome is defined as the occurrence of tubulointerstitial nephritis and uveitis in the absence of other systemic diseases. Three pediatric cases have been reported in the Republic of Korea, and we now report a fourth case. A 15-year-old girl presented to the ophthalmology department with a 1-week history of bilateral ocular discomfort that worsened on the day of presentation with redness and pain in both eyes. She was diagnosed with bilateral uveitis, and her baseline examination revealed moderate renal dysfunction and mild proteinuria. A renal biopsy was performed and confirmed the diagnosis of TINU syndrome. She was started on steroid eye drops and a 12-week course of oral steroids at a dose of 40 mg/m²/day, which completely resolved the proteinuria and mild renal function to an estimated glomerular filtration rate of 60 mL/min/1.73 m². However, the uveitis did not improve, and despite the addition of oral methotrexate as a second-line treatment, the uveitis remains unresponsive to treatment over 21 months. Further evaluation and treatment are ongoing, and active therapeutic intervention is suggested even at a pediatric age, considering the lack of improvement in renal function and uveitis to date.

• Clinical and Experimental Pediatrics
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• 제63권12호
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• pp.491-498
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• 2020

Background: Renal size is an important indicator in the diagnosis of renal diseases and urinary tract infections in children. Purpose: The purpose of this study is twofold. First, it aimed to measure the renal length and calculate the renal volume of normal Thai children using 2-dimensional ultrasonography (2D-US) and study their correlations with somatic parameters. Second, it aimed to compare the age-specific renal size of normal Thai children with the published data of their Western and Chinese counterparts. Methods: A total of 321 children (150 boys, 171 girls; age, 6-15 years) with a normal renal profile were prospectively recruited. All subjects underwent 2D-US by an experienced pediatric radiologist and the renal length, width, and depth were measured. Renal volume was calculated using the ellipsoid formula as recommended. The data were compared between the left and right kidneys, the sexes, and various somatic parameters. The age-specific renal lengths were compared using a nomogram derived from a Western cohort that is currently referred by many Thailand hospitals, while the renal volumes were compared with the published data of a Chinese cohort. Results: No statistically significant difference (P<0.05) was found between sexes or the right and left kidneys. The renal sizes had strong correlations with height, weight, body surface area, and age but not with body mass index. The renal length of the Thai children was moderately correlated (r=0.59) with that of the Western cohort, while the age-specific renal volume was significantly smaller (P<0.05) than that of the Chinese children. Conclusion: Therefore, we concluded that the age-specific renal length and volume obtained by 2D-US would vary between children in different regions and may not be suitably used as an international standard for diagnosis, although further studies may be needed to confirm our findings.

• Childhood Kidney Diseases
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• 제27권2호
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• pp.133-138
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• 2023

Nutcracker syndrome (NCS) is a disease caused by compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. Immunoglobulin A (IgA) nephropathy (IgAN) is characterized by the predominance of IgA deposits in the glomerular mesangial area. Hematuria and proteinuria can be present in both diseases, and some patients can be concurrently diagnosed with NCS and IgAN; however, a causal relationship between the two diseases has not yet been clarified. Here, we report two pediatric cases of NCS combined with IgAN. The first patient presenting with microscopic hematuria and proteinuria was diagnosed with NCS at the initial visit, and the second patient was later diagnosed with NCS when proteinuria worsened. Both patients were diagnosed with IgAN based on kidney biopsy findings and treated with angiotensin-converting enzyme inhibitors and immunosuppressants. A high index of suspicion and timely imaging or biopsy are essential for the proper management of NCS combined with glomerulopathy.

• Childhood Kidney Diseases
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• 제19권2호
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• pp.71-78
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• 2015

The incidence of acute kidney injury (AKI) in critically ill pediatric patients has been reported as increasing to 25 %, depending on population characteristics. The etiology of AKI has changed over the last 10-20 years from primary renal disease to the renal conditions associated with systemic illness. The AKI in pediatric population is associated with increased mortality and morbidity, and prevention is needed to reduce the consequence of AKI. It is known that the most important risk factors for AKI in critically ill pediatric patients are clinical conditions to be associated with decreased renal blood flow, direct renal injury, and illness severity. Renal hypoperfusion leads to neurohormonal activation including renin-angiotensin-aldosterone system, sympathetic nervous system, antidiuretic hormone, and prostaglandins. Prolonged renal hypoperfusion can result in acute tubular necrosis. The direct renal injury can be predisposed under the condition of renal hypoperfusion, and appropriate treatment of volume depletion is important to prevent AKI. The preventable causes of AKI include contrast-induced nephropathy, hemodynamic instability, inappropriate mediation use, and multiple nephrotoxic insults. Given the evidence of preventable factors for AKI, several actions such as the use of protocol for prevention of contrast-induced nephropathy, appropriate treatment of volume depletion, vigorous treatment of sepsis, avoidance of combinations of nephrotoxic medications, and monitoring of levels of drugs should be recommended.

• Childhood Kidney Diseases
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• 제1권2호
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• pp.183-188
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• 1997

Familial juvenile hyperuricemic nephropathy is an autosomal dominant disease characterized by progressive renal disease and hyperuricemia or gout, affecting young people of either sex equally. There are two biochemical markers of this disorder. The first is hyperuricemia disproportionate to the degree of renal dysfunction; the second is a grossly reduced clearance of uric acid relative to creatinine, dispropotionate to age, sex and degree of renal failure. We experienced 2 family members with hyperuricemia. One family member, a 13-year-old girl who had suffered from tophaceous gout and chronic renal failure. Her younger brother also had hyperuricemia and moderately reduced renal function. Their urinary excretion fractions of uric acid($FE_{uric\;acid}$) were reduced and renal biopsy specimens showed interstitial fibrosis with tubular atrophy and interstitial urate crystal deposition. We have treated these two patients with allopurinol but we have done renal transplantation because she progressed to end stage renal disease at 16 year old age.

• Childhood Kidney Diseases
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• 제1권2호
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• pp.161-165
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• 1997

Renal venous thrombosis (RVT) in neonatal period is a rare disease and usually complicated to clinical situations with reduced renal blood flow and hypercoagulability ; like acute blood loss, sepsis, shock, and birth asphyxia. RVT should be suspected in sick babies with hematuria, anemia, thrombocytopenia, enlarged kidney and acute renal failure. And the diagnosis can be confirmed by renal ultrasonography. We report two cases of neonatal renal venous thrombosis with review of literatures. One case, associated with E. coli sepsis, recovered completely, and the other, follwed respiratory distress in the neonate, revealed permanent renal functional impairment.

• Childhood Kidney Diseases
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• 제9권1호
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• pp.102-107
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• 2005

With the advent of hemodialysis, the success of renal transplants in the 1960s and the wide use of continuous ambulatory peritoneal dialysis at the end of the 1970s, children with renal failure now enjoy an extended life span. As a result, several children experience renal osteodystrophy and growth retardation. Renal osteodystrophy is induced by phosphorus retention, hypocalcemia, low vitamin D levels and hyperparathyroidism. The pharmacologic interventions are used to prevent bone deformities and to normalize growth velocity. But surgical intervention is required sometimes whorl osteodystrophy is severe and poorly controlled. We report an eight-year-old boy with ctironic renal failure who developed severe bone deformities and needed osteotomy.

• Childhood Kidney Diseases
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• 제10권1호
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• pp.77-82
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• 2006

Renal abscess in childhood is a rare disease, and generally treatment of renal abscesses in childhood follows the guidelines in adults. The guidelines of treatment of renal abscesses in adults include the following : renal abscesses smaller than 3 cm in size can be managed by antibiotics administration, while renal abscesses above 3 cm in size must be considered for percutaneous abscess drainage or open drainage. We experienced a case of a 2 year-old girl with multiple renal abscesses greater than 4 cm in size which resolved by oral antibiotics administration after 2 weeks of intravenous administration. We report this case with literature review.

• Childhood Kidney Diseases
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• 제25권1호
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• pp.44-48
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• 2021

Idiopathic renal hypouricemia (iRHUC) is a rare hereditary disease caused by a defect in urate handling of renal tubules. Type 1 renal hypouricemia (RHUC1) is diagnosed with confirmation of a mutation in SLC22A12 gene which encodes a renal urate-anion exchanger (URAT1). The majority of iRHUC patients are asymptomatic, especially during childhood, and thus many cases go undiagnosed or they are diagnosed late in older age with complications of hematuria, renal stones, or acute kidney injury (AKI). We report a case of a 7-year-old boy with subtle symptoms such as general weakness and dizziness and revealed hypouricemia and incidental nephrolithiasis. Homozygous mutations were detected in the SLC22A12 (c.774G>A) by molecular analysis. The present case suggests that fractional excretion of uric acid (FEUA) screening could be better followed by the coincidental discovery of hypouricemia, to prevent conflicting complications of iRHUC, even with normal urine uric acid to creatinine ratio (U_UA/U_Cr), and sequential genetic analysis if needed.

• Journal of Veterinary Science
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• 제24권6호
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• pp.75.1-75.8
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• 2023

Background: In veterinary medicine, previous studies regarding the diagnostic performance of shear wave elastography (SWE) in chronic kidney disease (CKD) are not consistent with each other. Moreover, there has been no study evaluating the relationship between symmetric dimethylarginine (SDMA) concentration and renal shear wave velocity (SWV) using two-dimensional SWE (2D SWE) in dogs with CKD. Objectives: This study aimed to evaluate the diagnostic capability of 2D SWE in dogs with CKD and to assess the relationship between renal SWV and SDMA concentration. Methods: Dogs with healthy kidneys and dogs with CKD underwent 2D SWE and SDMA assay. Renal stiffness was estimated as renal SWV in m/s. Results: SDMA concentration had a weak positive correlation with the left (r = 0.338, p = 0.022) and right renal SWV (r = 0.337, p = 0.044). Renal SWV was not significantly different between healthy kidney and CKD groups in the left (p = 0.085) and right (p = 0.171) kidneys. Conclusions: 2D SWE may could not distinguish between dogs with healthy kidney and dogs with early stage of CKD, but it would be useful for assessing the serial change of renal function in dogs.