• Childhood Kidney Diseases
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• v.14 no.1
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• pp.94-99
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• 2010

Obesity-related glomerulopathy (ORG) is a secondary form of focal and segmental glomerulosclerosis (FSGS) manifesting as proteinuria and progressive renal dysfunction that results from maladaptive glomerular response to increasing adiposity. Reports of ORG progressing to end stage renal diseases in rare in the pediatric population. We report a 9-year-old boy with obesity (body mass index $35\;kg/m^2$) who was diagnosed with ORG presenting with proteinuria. He was diagnosed with obesity-related glomerulopathy based on the laboratory, urinary, and kidney biopsy finding. In spite of treatment with angiotensin- converting enzyme (ACE) inhibitor and/or, angiotensin-receptor blocking agent, the degree or amount of proteinuria increased and renal function declined continuously. His BMI did not decrease and eventually progressed to chronic renal failure. Consequently, obese patients should be monitored for proteinuria, which may be the first manifestation of FSGS, a lesion that may be associated with serious renal sequelae.

• Applied Microscopy
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• v.29 no.3
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• pp.331-341
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• 1999

Examination of the morphology of red blood cells in the urine has been shown to be a promising adjunct in determining whether hematuria represents glomerular or nonglomerular bleeding. This is due to distortion of RBCs as they Pass across the basement membrane of the glomerular capillaries. It is concluded that is method can greatly help the clinician in distinguishing between glomerular and nonglomerular bleeding in patients with hematuria and channeling such patients toward the most appropriate investigations. We have experimented dysmorphic red blood cells that 5 patients of the hematuria are distorted with irregular outlines and often have small blobs extruding from the red cell membrane. Tried urinary sediments were seen with phase contrast microscope and confirmed scanning electron microscope. There are seen acanthocytes, anulocytes, ghost cells and sphero-echinocytes in dysmorphic erythrocytes. Clinical diagnosis was referred from the result of the biopsy-proven. Scanning electron microscopic findings of the hematuria are good diagnostic tool that disclose in distorted red blood cells from patients with glomerular disorders.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.100-105
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• 2007

Cockayne syndrome is a rare autosomal recessive disorder characterized by cachectic dwafism, mental retardation, loss of facial subcutaneous adipose tissue, microcephaly and photosensitive dermatitis. It is associated with renal abnormalities characterized by hyalinization of glomeruli, atrophy of tubules and interstitial fibrosis. To our knowledge, this is the first report of a case of Cockayne syndrome with FSGS in Korea. A 7-year old boy was admitted for evaluation of hypertension and proteinuria, which were detected 2 month ago. He was followed for short stature(<3 percentile), mental retardation(IQ 55), strabismus and dental caries since 3 years ago. He also showed microcephaly, a bird-like face and relatively large hands and feet. Laboratory findings showed decreased creatinine clearance($C_{Cr}$ 76.1 mL/min/$1.73m^2$) and proteinuria(1,548 mg/day). Renal biopsy demonstrated focal segmental glomerulosclerosis of the hilar type with large hyaline deposits, moderate tubular atrophy and interstitial fibrosis. His cardinal features, mental retardation, and renal biopsy findings were consistent with Cockayne syndrome. We report here a very rare case of Cockayne syndrome with FSGS presenting with proteinuria and hypertension.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.31-37
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• 2005

Puruose : Thin glomerular basement membrane disease(TGBMD) is found in patients with family history of hematuria. TGBMD is autosomal dominant and is known to be one of the commonest causes of asymptomatic hematuria. This study was conducted to evaluate the histological and clinical features of patients with TGBMD. Methods : 150 cases diagnosed with TGBMD by renal biopsy while admitted in the department of pediatrics, Kyungpook National University Hospital between January 1999 and December 2003 comprised the study group. The following parameters were retrospectively anaIyzed age of onset, hematuria pattern, existence of proteinuria, process of diagnosis, laboratory findings, thickness and character of basement membrane and family history. Results : The mean age at the time of diagnosis was 7.9 years. The male to female ratio was 65:77. 94 patients or 66% visited the hospital with a chief complaint of persistent microscopic hematuria. Gross hematuria accounted for 13 cases or 9%. 78 cases(55%) were found to have hematuria for the first time from a routine school urinalysis screening. The renal biopsy showed the thickness of basement membrane to be 186$\pm$36 nm. Focal lamellation of the basement membrane was found in eight cases. In the family history, hematuria was shown in 10 cases on the Paternal side, 13 on The maternal side and none on both sides. In seven cases, hematuria was shown among siblings. No significant differences were found among the laboratory test results which were conducted at an average interval of fifteen months. Conclusion : TGBMD is one of the major causes of asymptomatic hematuria in children, which was diagnosed in increasing numbers since school urinary mass screening test started in 1998. In cases with familial progressive renal disease or focal duplication in the basement membrane Alport syndrome should be considered.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.147-155
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• 2001

Purpose : IgA nephropathy(IgAN) and thin glomerular basement membrane disease(TGBMD) are common glomerular diseases that cause hematuria in childhood. IgAN has characteristics of IgA deposit as the sole or predominantly localized to the mesangium Recently, it has been reported that thinning of glomerular basement membrane(GBM) is commonly accompanied with precipitation of electron dense deposits in IgAN. We performed this study to examine the frequency of thinning of GBM among children with IgAN and to analysis tile correlation between urinary abnormalities and GBM thickness and furthermore to conduct comparative analysis of the clinical and pathological features of IgAN and TGBMD. Methods : This study summarizes data collected from Department of Pediatrics, Busan Paik Hospital, Inje Medical College. Data include 51 cases who were diagnosed as IgAN from 1995 to 2000, and 26 cases who were diagnosed as TGBMD from 1990 to 2000 by percutaneous renal biopsy. Results : Males accounted for 29/51($56.9\%$) patients with IgAN and 8/26($30.8\%$) of those with TGBMD. The clinical and laboratory features between IgAN and TGBMD were significantly different regarding the incidence of proteinuria(IgAN vs TGBMD: $43.1\%\;vs\;3.8\%$, p=0.001), the incidence of co-appearance of proteinuria with hematuria ($41.2\%\;vs\;3.8\%$, p=0.001), total amount of protein in 24 hours collected urine ($808{\pm}\;mg\;vs\;251{\pm}200.7\;mg$, p=0.001) and the incidence of proteinuria more than 1 gm in 24 hours collected urine ($23.5\%\;vs\;3.8\%$, p=0.01). On the contrary, there were no significant differences in the levels of serum albumin, creatinine, BUN, and Ccr between two groups. The mean thickness of GBM in patients with IgAN was $293.0{\pm}79.2\;nm$(139.7-461.9 nm) and $180.9{\pm}35.8\;nm$(110.5-229.5 nm) in patients with TGBMD. The mean GBM thickness revealed significantly thinner in TGBMD compared than those with IgAN (P=0.0001). The frequency of thickness being less than 250 nm was $37.4{\pm}34.4\%$ in IgAN and $93.0{\pm}7.0\%$ in TGBMD (P=0.0001). But there were no correlations between urinary abnormalities and GBM thickness in patients with IgAN. Conclusion : The thinning of GBM would be one of the common pathological findings in IgAN Moreover, there is no significant correlations between urinary abnormalities and GBM thickness in patients with IgAN, However, patients with IgAN tend to have significantly higher possibilities of proteinuria, co-appearance of proteinuria with hematuria and higher total amount of protein in 24 hours collected urine compared those with TGBMD. These differences might be play all important role as progressive prognostic indicators in patients with IgAN. (J Korean Soc Pediatr Nephrol 2001;5 : 136-46)

• The Korean Journal of Nuclear Medicine Technology
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• v.24 no.1
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• pp.27-32
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• 2020

Purpose Glomerular Filtration Rate(GFR) is an important indicator for evaluating renal function and monitoring the progress of renal disease. Currently, the method of measuring GFR in clinical trials by using serum creatinine value and ^99mTc-DTPA(diethylenetriamine pentaacetic acid) renal dynamic scan is still useful. After the Gates method of formula was announced, when ^99mTc-DTPA Renal dynamic scan is taken, it is applied the GFR is measured using a gamma camera. The purpose of this paper is to measure the GFR by applying the Gates method of formula. It is according to effect activity and matrix size that is related in the GFR. Materials and Methods Data from 5 adult patients (patient age = 62 ± 5, 3 males, 2 females) who had been examined ^99mTc-DTPA Renal dynamic scan were analyzed. A dynamic image was obtained for 21 minutes after instantaneous injection of ^99mTc-DTPA 15 mCi into the patient's vein. To evaluate the glomerular filtration rate according to changes in activity and matrix size, total counts were measured after setting regions of interest in both kidneys and tissues in 2-3 minutes. The distance from detector to the table was maintained at 30cm, and the capacity of the pre-syringe (PR) was set to 15, 20, 25, 30 mCi, and each the capacity of post-syringe (PO) was 1, 5, 10, 15 mCi is set to evaluate the activity change. And then, each matrix size was changed to 32 × 32, 64 × 64, 128 × 128, 256 × 256, 512 × 512, and 1024 × 1024 to compare and to evaluate the values. Results As the activity increased in matrix size, the difference in GFR gradually decreased from 52.95% at the maximum to 16.67% at the minimum. The GFR value according to the change of matrix size was similar to 2.4%, 0.2%, 0.2% of difference when changing from 128 to 256, 256 to 512, and 512 to 1024, but 54.3% of difference when changing from 32 to 64 and 39.43% of difference when changing from 64 to 128. Finally, based on the presently used protocol, 256 × 256, PR 15 mCi and PO 1 mCi, the GFR value was the largest difference with 82% in PR 15 mCi and PO 1 mCi. conditions, and at the least difference is 0.2% in the conditions of PR 30 mCi and PO 15 mCi. Conclusion Through this paper, it was confirmed that when measuring the GFR using the gate method in the ^99mTc-DTPA renal dynamic scan. The GFR was affected by activity and matrix size changes. Therefore, it is considered that when taking the ^99mTc-DTPA renal dynamic scan, is should be careful by applying appropriate parameters when calculating GFR in the every hospital.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.188-195
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• 2001

Type II membranoproliferative glomerulonephritis (Dense deposit disease) is an acquired primary glomerular disease characterized by electron microscopic evidence of a continuous dense membrane deposition replacing the lamina densa. It is a subtype of idiopathic membra- noproliferative glomerulonephritis, and was described as a separate entity by Berger and Galle in 1963. It frequently occurs in older chilren and young adults and the clinical course is variable, but is generally progressive. The presenting feature is nephrotic syndrome in many patients, and proteinuria and hematuria are also seen frequently. The purpose of this paper is to present a case of DDD (Dense deposit disease) from a 10 year old boy who was diagnosed as a acute poststreptococcal glomurulonephritis with protenuria, hematuria, and facial edema by renal biopsy 4 years ago. (J, Korean Soc Pediatr Nephrol 2001 ; 5 : 188-95)

• Journal of Yeungnam Medical Science
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• v.11 no.1
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• pp.101-108
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• 1994

Many previously described nuclear medicine procedures to assess glomerular filtration rate have some problems because numerous blood sample is to be taken and they don't measure each separate renal function. Gates described isotopic method for the measurement of global and unilateral GFR based on the fractional renal uptake of $^{99m}Tc$-DTPA 2 to 3 minutes after its intravenous injection. We evaluated GFR using $^{99m}Tc$-DTPA in 57 people according to Gates method and compared with creatinine clearance. A good correlation was observed between creatinine clearance and GFR calculated by Gates' formula with an r value of 0.9(P<0.05). And also the relationship between parameters of $^{99m}Tc$-DTPA renal scan images and GFR was taken. They were significantly correlated with GFR calculated by Gates' formula : r value 0.66 between relative intensity of peak renal to peak aortic activity(pK/pA) and GFR, -0.42 between time between aortic and kidney peak(A-K) and GFR and -0.48 between parenchymal renal activity at 25 min compared to peak kidney activity(25K/pK) and GFR. In conclusion, the determination of GFR according to the Gates' formula shows good and reproducible of GFR with rapidity and simplicity. And the parameters from the renal scan images can use to estimate the renal function.

• The Korean Journal of Nuclear Medicine Technology
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• v.22 no.2
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• pp.97-100
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• 2018

Purpose The glomerular filtration rate (GFR) test is an important indicator of glomerular filtration and has been used to test renal function and the extent of its function. The GFR test is performed by intravenous injection of radioactive medicines made of $^{51}Cr$-EDTA, and blood concentration is measured by taking blood according to the elapsed time. also, PET-CT, bone scan, transfusion and so on will affect the outcome. Therefore, we will improve the quality of the test by providing guidelines for the GFR test for more accurate testing. Materials and Methods 5 mL of physiological saline solution and 2 mL of $^{51}Cr$-EDTA solution are used to make 5 mL of the radiopharmaceutical solution to be injected into the patient. First, the syringe weight is measured before the injection, and then the radioactive medicine is injected into the patient's vein and the syringe weight is measured after the injection. Blood sampling is performed twice in total. In adults, blood is collected 3 hours / 5 hours after injection and in children 2 hours / 5 hours after injection. The blood sample is centrifuged at 3300 rpm for 5 minutes. Standard solution is prepared by filling diluent water up to the scale indicated in the 200-mL volumetric flask, discarding $500{\mu}L$, injecting $500{\mu}L$ of GFR reagent and mixing well. $500{\mu}L$ each of the standard solution is dispensed into two test tubes, and $500{\mu}L$ of each of the plasma samples collected in time is dispensed into two test tubes and measured with a Cobra Counter. Results At present, the reference range applied in this study is $119.5{\pm}30.3ml/min/1.73m2$ for males and $125.2{\pm}28.2ml/min/1.73m^2$ for females. Conclusion The GFR test is conducted using radioactive medical products. GFR testing is performed as a scheduled test, but PET-CT, dialysis and transfusion, which may affect GFR testing, may be scheduled during GFR testing. Therefore, we could get accurate GFR test results by notifying the ward and department beforehand when booking.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.154-160
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• 2000

The pathogenesis of IgA nephropathy and acute poststreptococcal glomerulonephritis is not fully understood. In the past, acute poststreptococcal glumerulonephritis was the most common cause of gross hematuria in children, but now IgA nephropathy is the most common one. We experienced two cases of acute poststreptococcal glomerulonephritis superimposing to IgA nephropathy in boys Case 1 had upper respiratory infection before elevation of anti-streptolysin O, generalized edema, gross hematuria and proteinuria. The complement levels were normal. Electron microscopic findings of renal biopsy at ten days after onset showed a few big subepithelial 'humps' and localized heavy subendothelial and mesangial deposits. Immunofluoroscopic findings revealed predominant IgA deposition in the mesangium. The electron microscopic findings were diagnostic of acute poststreptococcal glomerulonephritis On the other hand, immunoflorescence microscopic findings were compatible to IgA nephropathy. In case 2, the renal biopsy which was done 2 years after onset showed only finding of IgA nephropathy. To our knowledges, there has been kw reports of acute poststreptococcal glomerulonephritis superimposing to IgA nephropathy which was confirmed by renal biopsy. We report two cases of acute poststreptococcal glomerulonephritis superimposing: to IgA nephropathy with a brief review of the literatures.