• Childhood Kidney Diseases
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• v.8 no.2
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• pp.186-194
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• 2004

Purpose: Membranoproliferative glomeulonephritis(MPGN) has been diagnosed in an increasing number of asymptomatic cases. These cases have been detected by school urinary screening test even though the total cases of MPGN show a decreasing trend. We have analyzed the clinical and pathological characteristics of children with MPGN according to the clinical manifestations at the time of disease presentation. Methods: A total of 18 patients who had been diagnosed with idiopathic MPGN by percutaneous renal biopsy from January 1990 to February 2004 were involved in our study. The patients were divided into 2 groups as the school urinary screening(A) group and the symptomatic(S) group according to the clinical manifestations at the time of disease presentation. Results: Out of the total 18 patients, 8(44.4%) were in the S group and 10(55.6%) were in the A group. The mean serum total protein, albumin and $C_3$ levels in the S group were significantly lower than those levels of the A group, respectively($4.9{\pm}1.2\;g/dL,\;vs\;7.0{\pm}0.5\;g/dL\;P=0.002,\;2.8{\pm}0.9\;g/dL\;vs.\;4.1{\pm}0.3\;g/dL\;P=0.002,\;63.9{\pm}36.4\;mg/dL\;vs.\;100.8{\pm}39.5\;g/dL\;P=0.041$). The mean total protein amount of 24 hour collected urine in the S group were significantly higher than that of the A group($3684.0{\pm}2601.3\;mg/m^2\;vs.\;559.4{\pm}4.6.9\;mg/m^2$, respectively, P=0.001). Hypocomplementemia was observed in 11(61.1%) out of 18 patients at the time of disease onset, 7(87.5%) in the S group and 4(40%) in the A group. However the hypocomplementemia was decreased in 6(33.3%) out of 18 patients at the time of final follow-up, 3(37.5%) in the S group and 3(30%) in the A group. According to the pathologic type, hypocomplementemia was observed 8 patients(61.5%) with type I disease, 1 patients (100%) with type II disease, 2 patients(50%) in type III disease at the disease onset, but 4 patients(30.8%) in type I disease, 1 patient(100%) in type II disease, 1 patient(33.3%) with type III disease at the time of last follow-up. The incidence of cellular crescent formation and tubular atropy. as observed on light microscopy, were higher in the S group compared to the A group. Mean grade of capillary wall thickening and, mesangial proliferation were significantly higher in the S group. Conclusion: MPGN, as diagnosed in patients with only asymptomatic urinary abnormalities, has been increasing, it is more frequent in asymptomatic patients than in patients with presenting symptoms. Our result suggests that MPGN should be considered in the renal biopsy diagnosis regardless of serum $C_3$ level when urinary abnormalities are found by school urinary screening test.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.57-61
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• 2016

Isovaleric acidemia (IVA) is an autosomal recessively inherited organic acid disorder due to a defect of the enzyme isovaleryl-CoA dehydrogenase in the leucine metabolic pathway. Deficiency of this enzyme results in the accumulation of derivatives of isovaleryl-CoA. In acute illness in IVA, isovaleric acid and its derivatives accumulate and profound metabolic acidosis with ketosis, characteristic pungent body odor, hypoglycemia, and hyperammonemia can be developed. Additionally, recurrent vomiting, failure to thrive, developmental delay, epilepsy and mental retardation are chronic presenting symptoms and signs for IVA. On the result of newborn screening for inherited metabolic disorders, increased levels of isovalerylcarnitine (C5) are shown. However, C5 elevation can be accompanied with short/branched-chain acyl-CoA dehydrogenase (SBCAD) and therapy with certain antibiotics containing pivalic acid. Quantitative measurement of organic acids in urine and acylcarnitine profiles in plasma are necessary to differential diagnosis. Molecular genetic analysis of the IVD gene for IVA and ACADSB is also helpful to confirm IVA and SBCAD deficiency, respectively. Considering that IVA can be associated with significant morbidity and mortality at acute presentation of metabolic crisis, early diagnosis prior to the onset of symptoms by newborn screening enable to introduction of early treatment and prevention of acute and chronic complications.

• Childhood Kidney Diseases
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• v.14 no.2
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• pp.132-142
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• 2010

Hypokalemia usually reflects total body potassium deficiency, but less commonly results from transcellular potassium redistribution with normal body potassium stores. The differential diagnosis of hypokalemia includes pseudohypokalemia, cellular potassium redistribution, inadequate potassium intake, excessive cutaneous or gastrointestinal potassium loss, and renal potassium wasting. To discriminate excessive renal from extrarenal potassium losses as a cause for hypokalemia, urine potassium concentration or TTKG should be measured. Decreased values are indicative of extrarenal losses or inadequate intake. In contrast, excessive renal potassium losses are expected with increased values. Renal potassium wasting with normal or low blood pressure suggests hypokalemia associated with acidosis, vomiting, tubular disorders or increased renal potassium secretion. In hypokalemia associated with hypertension, plasam renin and aldosterone should be measured to differentiated among hyperreninemic hyperaldosteronism, primary hyperaldosteronism, and mineralocorticoid excess other than aldosterone or target organ activation. Hypokalemia may manifest as weakness, seizure, myalgia, rhabdomyolysis, constipation, ileus, arrhythmia, paresthesias, etc. Therapy for hypokalemia consists of treatment of underlying disease and potassium supplementation. The evaluation of hyperkalemia is also a multistep process. The differential diagnosis of hyperkalemia includes pseudohypokalemia, redistribution, and true hyperkalemia. True hyperkalemia associated with decreased glomerular filtration rate is associated with renal failure or increased body potassium contents. When glomerular filtration rate is above 15 mL/min/$1.73m^2$, plasma renin and aldosterone must be measured to differentiate hyporeninemic hypoaldosteronism, primary aldosteronism, disturbance of aldosterone action or target organ dysfunction. Hyperkalemia can cause arrhythmia, paresthesias, fatigue, etc. Therapy for hyperkalemia consists of administration of calcium gluconate, insulin, beta2 agonist, bicarbonate, furosemide, resin and dialysis. Potassium intake must be restricted and associated drugs should be withdrawn.

• 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.2
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• pp.272-279
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• 2007

Purpose : We tried to find out the clinical parameters which predict the outcome of treatment in children with enuresis. Methods : Enuresis patients who visited our hospital during 2003-2007 were included. Parameters such as age, gender, height, weight, minimal voided volume, maximal voided volume, maximum functional bladder capacity, frequency of voiding, urine S,G. before and after sleep were measured and an enuresis diary was also recorded. The reduction in wetting frequencies were classified into three groups; none(<50%), partial(50-90%) and complete(90%) response groups. We also compared the 'initial responders' who showed improvement(${\ge}50%$) during the 2 weeks of evaluation and behavioral therapy to the 'initial non-responders'. Results : Parameters mentioned above showed no significant relation to the treatment out-come. The response rate during the 2 weeks of the evaluation period was 32%(49/151) [complete in 1.3% (2/151), partial in 29.6% (47/151)]. Two-months' treatment responses were complete in 14(40%), partial in 19(54.3%) and none in 2(5.9%) responders(n=35), while they were 10(13.5%), 46(62.2%) and 18(24.3%), respectively in the non-responders(n=73) (P<0.05). Conclusion : We suggest that initial 'responsiveness' can be used as a predictor for good treatment outcome in patients with enuresis.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.11-19
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• 1999

Purpose: MCNS is found in approximately $85\%$ of the idiopathic nephrotic syndrome in children and shows good prognosis with initial steroid therapy. MCNS most commonly appears between the ages of 2 and 10 yr. But the incidence and prognosis in adolescent MCNS are different from those found in young children; the prognosis and the response to therapy is unfavorable with increasing ages. So we compared the prevalence and the clinical manifestations of adolescent MCNS with that of childhood MCNS for management of adolescent MCNS. Methods: We conducted a retrospective study with a review of histopathologic findings and clinical manifestations of the 216 cases with MCNS which were divided into children group and adolescent group by their age of onset; under 12 years(childhood) and between 12-18 years(adolescent). Results: 1) The number of childhood idiopathic nephrotic syndrome was 245 cases, and that of adolescent idiopathic nephrotic syndrome was 55 cases. 188 cases($77\%$) showed MCNS, 30 cases($12\%$) FSGS, 4 cases($1.6\%$) MSPCN in childhood idiopathic nephrotic syndrome; 28 cases($51\%$) showed MCNS, 12 cases($22\%$) FSGS in adolescent idiopathic nephrotic syndrome. 2) The mean onset age was $7.53{\pm}5.5$ years, and the male to female ratio was 3.8:1 in childhood onset and 2.5:1 in adolescent onset with male predominance. 3) Hematuria was associated with $17\%$ of childhood onset and $39.3\%$ of adolescent onset disease(P=0.005). Hypertension appeared in $0.5\%\;and\;7\%$ in each group without significant difference between the groups. 4) 24 hour urine protein, SPI, albumin, BUN, cholesterol level showed no significant difference. 5) The response of childhood onset and adolescent onset MCNS to steroid therapy showed complete remission in $11.7\%\;&\;14.7\%$, infrequent relapsing in $29.2\%\;&\;28.5\%$, frequent relapsing in $23.9\%\;&\;14.7\%$, steroid dependent in $21.8\%\;&\;28.6\%$ each. Steroid resistant showed $13.3\%\;&\;14.7\%$ with no significance. 6) Immunosuppresant therapy was performed $57\%$ in childhood onset and $65\%$ in adolescent onset. 7) Mean number of relapse and duration from onset to first relapse showed no significance between two groups. Conclusion : Our results indicate that the incidence of hematuria, the rate of steroid dependent and frequent relapsing, and the recurrence rate were higher in adolescent MCNS; showed poorer steroid responsiveness and prognosis. Our data also point to the need for a more aggressive therapy to treat and make recommendations for the adolescent population as a whole.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.24-31
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• 2007

Purpose : Nitric oxide(NO) is a very potent vasodilator synthesized from L-arginine by endothelial cells. We investigated whether urinary NO excretion was altered in various renal diseases in children and whether urinary NO excretion could be used in predicting pathologic causes and fibrosis in renal diseases in children. Methods : We recruited 48 patients(32 minimal change nephrotic syndrome[MCNS] and 16 vesicoureteral reflux[VUR] patients from the pediatric renal clinic in Korea University Guro Hospital. We measured the concentration of nitrite$(NO_2)$ and nitrate$(NO_3)$ by Griess reaction and that of creatinine(Cr) by Jaffe method in randomized spot urines. We then analyzed the urinary$(NO_2+NO_3)/Cr$ ratios and compared the values between each patient group. Urinary $(NO_2+NO_3)/Cr$ ratios were also evaluated according to the recurrence and the degree of proteinuria at sampling in the MCNS group and compared according to the presence of renal scarring and the grade of reflux in the VUR group. Results : The ratios of urinary$(NO_2+NO_3)/Cr$ were significantly increased in the VUR and MCNS groups, as compared to the control group. In the MCNS group, a higher level of urine $(NO_2+NO_3)/Cr$ was observed In frequent relapse patients(relapse over four times within one year after first diagnosis) and the patients with severe proteinuria at sampling, respectively. The VUR group with renal scars also showed a higher level of urinary$(NO_2+NO_3)/Cr$ compared to that without scars. Conclusions : In summary, VUR may play a role in the pathogenesis of VUR and MCNS. NO also seems to affect proteinuria and renal scar formation. (J Korean Soc Pediatr Nephrol 2007;11:24-31)

• The Korean Journal of Nuclear Medicine
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• v.37 no.4
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• pp.245-253
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• 2003

Puorpose: The purpose of this study was to evaluate the usefulness of $^{99m}Tc$ DMSA scintigraphy on the dignosis of a renal scar in children with urinary tract infections. Materials and Methods: Eighty three patients were included in this study, who were diagnosed as the urinary tract infection on the basis of symptom, urinalysis and urine culture. $^{99m}Tc$ DMSA scintigraphy and voiding cystoureterography were peformed within 7days before the treatment in all patients. We classified the scintigraphic findings as follow s : 1 ; a large hypoactive upper or lower pole. 2 ; a small hypoactive area. 3 ; single defect resulting in localized deformity of the outlines. 4 ; deformed outlines in a small or normal sized kidney. 5 ; multiple defects. 6 ; diffuse hypoactive kidney without regional impairment. Follow-up scintigraphy was done at least 6 months after the initial study. When the abnormality on the initial scintigraphy was not completely resolved on the follow-up scan, the lesion was defined as containing a scar. Results: One hundred and fifteen renal units of 166 units(69.3%) showed abnormal findings on the DMSA scintigraphy. 65 units(56.5%) was diagnosed as containing renal scars on follow-up scintigraphies. Incidences of renal scar among renal units showing pattern 3, 4 and 5 on the initial scan was 75%, 78% and 78%, respectively. Whereas many of renal units showing 1, 2 and 6 pattern were recovered(65%, 76%, 50%). Sensitivity, specificity and accuracy of pattern-based DMSA scintigraphic findings on the diagnosis of renal scar was 76.9%, 85.1% and 81.9%, respectively. VUR was significantly associated with the renal scar when the initial DMSA shows unrecoverable findings(pattern 3, 4, 5). Odds ratio of the renal scar in a kidney showing unrecoverable initial scintigraphic findings was 19.1. Odds ratio in a kidney with mild or moderate-to-severe VUR was 3.5 and 14.4 respectively. Conclusion: In the urinary tract infection, renal scar was significantly developed in a kidney showing unrecoverable findings on the initial DMSA scan and VUR on voiding cystoureterography.

• Pediatric Infection and Vaccine
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• v.17 no.1
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• pp.23-29
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• 2010

Purpose : Urinary tract infection (UTI) in children is the most common disease during the infantile period, therefore early diagnosis and treatment are important. Pyuria is a useful clinical parameter for the initial diagnosis of a UTI. In this study we aimed to compare the clinical, laboratory, and imaging findings in relation to the duration of pyuria in infants with UTIs. Methods : Three hundred seventy-four infants <12 months of age who were admitted between January 1995 and December 2005 for the first episode of a febrile UTI were retrospectively reviewed. Patients were divided into two groups according to the duration of pyuria as follows: group 1, pyuria resolved <3 days after initial treatment; and group 2, pyuria lasted at least 3 days after initial treatment. Results : There were no significant differences between the two groups in relation to gender, age, total duration of fever, and organisms in the urine. Group 2 had a significantly higher peripheral blood leukocyte count ($14,360.86{\pm}5,526.16cells/mm^3$ vs. $11,822.55{\pm}5,687.26cells/mm^3$, P <0.001), erythrocyte sedimentation rate ($32.81{\pm}19.34mm/hr$ vs. $23.74{\pm}20.43mm/hr$, P <0.001), and C-reactive protein ($6.84{\pm}5.68mg/dL$ vs. $3.78{\pm}3.99mg/dL$, P <0.001) than group 1. There was a significantly higher incidence of hydronephrosis and a higher grade of vesicoureteral reflux (VUR) in group 2 compared to group 1. Conclusion : In infants with UTI, pyuria of longer duration is related to severe UTI and higher grade VUR, therefore aggressive radiologic studies may be necessary.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.136-146
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• 2001

Purpose : One of the most difficult problems in the care of children with nephrotic syndrome remains the occurrence of relapses, despite initial response to steroids. Constantinescu reported that rapidity of initial response to steroid therapy could predict fewer relapses in the first year. So we evaluated the changes in serum lipid abnormalities in children with corticosensitive nephrotic syndrome before steroid treatment and the correlation between serum lipid levels and renal function, days to remission. Methods . We analyzed the Medical records of children who were managed by us between October 1994 and August 2000. In 33 patients with corticosensitive nephrotic syndrome, we evaluated the correlation between serum lipid levels and renal function [Creatinine clearance(Ccr)] and proteinuria before steroid treatment, and days to remission defined as the third day when the patient's urine becomes protein free. Results : There were 21 males and 12 females. Median age at presentation was 6.4 years (range: 1.8-17.3 years). Median days to remission were 15.4 days (range 4-42 days) on Prednisolone $60mg/m^2$ daily. The increased levels of triglyceride, total cholesterol, LDL cholesterol, apolipoprotein B, total cholesterol/HDL cholesterol, Lipoprotein(a) were observed. But the level of HDL cholesterol was not increased. Serum albumin was decreased a]id proteinuria was increased before steroid treatment. But Ccr was not decreased. There were negative correlation between serum albumin and total cholesterol (r = -0.5157, P<0.005), LDL cholesterol (r = -0.5543, P<0.005), total cholesterol/HDL cholesterol (r = -0.4506, P<0.01), lipoprotein(a) (r = -0.4570, P<0.025), apolipoprotein B (r = -0.5297, P<0.025), apolipoprotein B/apolipoprotein Al (r = -0.5851, P<0.01), apolipoprotein B/HDL cholesterol (r = -0.4961, P<0.05) before steroid treatment. There was no correlation between proteinuria and serum lipid profiles. Also Ccr and serum lipid profiles were not correlated. There was positive correlation between days to remission and HDL cholesterol (r = +0.4511, P<0.05), apolipoprotein B (r = +0.5190, P<0.05), apolipoprotein B/HDL cholesterol (r = +0.7169, P<0.005). Conclusions : This results reveal that HDL cholesterol, apolipoprotein B and apolipoprotein B/HDL cholesterol can be used as a predictive factor in corticosensitive nephrotic syndrome. We could not determine the significant level of these lipids for insufficient patients number, but these level may predict future relapses of corticosensitive nephrotic syndrome patients and thus may allow to better management and treatment protocols. More data and long term follow up studies should be needed. (J Korean Soc Pediatr Nephrol 2001;5 : 136-46)