• Childhood Kidney Diseases
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• v.18 no.2
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• pp.128-131
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• 2014

Histopathologic evidence of "full-house" immune complex deposits is a pathognomonic feature of lupus nephritis. This report presents the case of a 12-year-old boy with persistent microscopic hematuria and proteinuria. He was diagnosed with "full-house" nephropathy based on a renal biopsy. However, there was no other clinical or biological evidence of systemic lupus erythematosus (SLE). Although the potential for isolated "full-house" nephropathy preceding SLE is unclear, such patients should be followed for clinical signs and autoantibodies of SLE. In most cases, microscopic hematuria has a good prognosis, and follow-up usually requires only regular urinalysis. However, we should be aware of isolated "full-house" nephropathy that remains asymptomatic for a long time, as few patients with no clinical signs and negative serology ultimately develop SLE.

• 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)

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.205-213
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• 2004

Purpose: To evaluate the clinical manifestations of various glomerular diseases in children, a clinicopathological study was performed in 52 children who had renal biopsy. The type and relative incidence of the glomerular pathologies were analyzed, and the clinical predictability and usefulness of renal biopsy in glomerular diseases were assessed. Methods: Medical records of fifty two children with renal disease who had undergone percutaneous renal biopsy under ultrasonic guidance at Chungnam University Hospital from October 1995 to August 2003 were reviewed. In addition, we compared the clinical findings before renal biopsy with the pathological diagnosis. Results: The male to female ratio was 1.6:1 and they were $9.8\pm2.6$ years old on average. The chief complaints for biopsy were hematuria in 22 cases which was the most common (42.3%), proteinuria in 16 cases(30.8%), and hematuria & proteinuria(26.9%). Among the 22 cases of hematuria, there were 15 cases of gross hematuria(68.2%) and 7 cases of microscopic hematuria(31.8%). In terms of histopathologic diagnosis, most of them were primary glomerular diseases(84.6%), which included IgA nephropathy(28.8%), thin glomerular basement membrane disease(25.0%), focal segmental glomerulosclerosis(FSGS)(11.5%), membranous proliferative glomerulonephritis(7.7%), minimal change lesion(3.8%), acute poststreptococcal glomerulonephritis(3.8%) and membranous glomerulonephritis(3.8%). The clinical manifestations and pathologic diagnosis were not correlated. Conclusion: The clinical manifestations could not predict the pathological diagnosis. Therefore, renal biopsy would be inevitable in diagnosis of glomerular diseases for effective management and assessment of prognosis.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.150-156
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• 2003

Purpose : Efforts to predict the clinicopathological outcome of IgA nephropathy have been made but have yielded conflicting results and have not helped in deciding the appropriate timing of the renal biopsy. In this study, we reviewed the predictive factors of clinicopathological outcome for finding out the criteria of renal biopsy timing of IgA nephropathy. Methods : Forty children diagnosed with biopsy proven IgA nephropathy at Wonju Christian Hospital were studied retrospectively, based on medical records. Results : Among 39 patients, 2 children progressed to higher serum creatinine level. One of them reached to the end stage renal disease within 2 year 7 months. According to WHO histopathological classification, there were 15 cases of class I, 14 cases of class II, 7 cases of class III, and 3 cases of class IV. In the mild histological classes(class I, II), gross hematuria was shown in 23 out of 29 children(P=0.02). In the severe histological classes(class III, IV), gross hematuria was noted in 4 out of 10(P>0.05). The tubulointerstitial changes were grade 1 in 24 cases, grade 2 in 4 cases, grade 3 in 8 cases, and grade 4 in 3 cases. With an increase in the tubulointerstitial grade, the 24 hour urine protein/albumin ratio increased. Serum creatinine less than 0.79 mg/dL could predict the lower grade(grade 1 and 2) of tubulointerstitial changes. But serum creatinine greater than 1.13 mg/dL could predict the higher grade(grade 3 and 4) of tubulointerstitial changes. In children with gross hematuria(n=27), serum creatinine was lower(0.78 vs 1.09 mg/dL, P=0.027), serum IgA was higher(316.3 vs 198.8 mg/dL), and the cases of lower WHO classification(I and II) were more common(23 vs 4, P=0.029) than the children with microscopic hematuria. Conclusion : Serum creatinine less than 0.79 mg/dL, macroscopic hematuria, and higher 24 hour urine protein/albumin ratio would predict the lower grade glomerulo tubulointerstitial lesion in IgA nephropathy and could be used as the criteria delaying the renal biopsy.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.243-250
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• 2002

Amyloidosis comprises a diverse group of systemic and local diseases characterized by organ involvement by the extracellular deposition of fibrils composed of subunits of a variety of normal serum proteins. Secondary amyloidosis is caused by the deposition of amyloid A(AA) protein in chronic inflammatory disease. Juvenile rheumatoid arthritis(JRA) has been known to be the most common cause of secondary amyloidosis. We experienced one case of secondary renal amyloidosis in a 12-year-old girl who had suffered from JRA for several years who had visited our renal clinic to evaluate the proteinuria with microscopic hematuria which was detected by chance at school urine screening examination. Apple green birefringence was observed under polarized light with Congo red stain at)d characteristic electron microscopic findings was also noted in renal tissues which was obtained by percutaneous renal biopsy. In our knowledge, this is the first case report of secondary renal amyloidosis developed in pediatric age in Korea.

• Parasites, Hosts and Diseases
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• v.48 no.1
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• pp.57-59
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• 2010

A 59-year-old Korean man complained of a painless scrotal hard nodule and weak urine stream. The ultrasound scan revealed a 2.2-cm sized round heteroechogenic nodule located in the extratesticular area. Microscopic hematuria was detected in routine laboratory examinations. On scrotal exploration, multiple spargana were incidentally found in the mass and along the left spermatic cord. On cystoscopy, a 10-mm sized mucosal elevation was found in the right side of the bladder dome. After transurethral resection of the covered mucosa, larval tapeworms were removed from inside of the nodule by forceps. Plerocercoids of Spirometra erinacei was confirmed morphologically and also by PCR-sequencing analysis from the extracted tissue of the urinary bladder. So far as the literature is concerned, this is the first worm (PCR)-proven case of sparganosis in the urinary bladder.

• The Korean Journal of Cytopathology
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• v.10 no.1
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• pp.67-71
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• 1999

We report a case of 53-year-old man with plasmacytold transitional cell carcinoma of the urinary bladder, which may be confused with plasmacytoma. The patient initially presented with gross hematuria and dysuria for two months. Cystoscopy and radiologic studios revealed multiple intraluminal protruding masses on the urinary bladder invading perivesical fat tissue. After urinary cytologic examination and cystoscopic biopsy, radical cystectomy and pelvic lymph node dissections were done. Urine cytology showed single cells and poorly cohesive cells with round eccentric nuclei, bi-or multi-nucleation, indistinct nucleoli, coarse chromatin, and abundant basophilic cytoplasm within relatively clear background. The cytologic findings of tumor cells were similar to the plasma cells seen in plasmacytoma. The tumor of the bladder was composed on discohesive, individual cancer cells with diffuse pattern that simulated lymphoma or plasmacytoma. Immunohistochemical and electron microscopic studies clearly established the epithelial nature of the neoplasm. Recognition of this plasmacytoid type of transitional cell carcinoma of the urinary bladder can avoid the misdiagnosis.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.259-265
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• 2002

Bartter syndrome is a rare disorder characterized by the association of hypokalemic hypochloremic metabolic alkalosis, hyperreninemia, hyperaldosteronemia, short stature and nephrocalcinosis. This disorder presents with hyperplasia of juxtaglomerular apparatus on renal biopsy. We experienced a case of late-onset Bartter syndrome with nephrocalcinosis in a 9-year-old boy, whose chief pictures were muscle weakness, short stature, persistent sterile pyuria and microscopic hematuria. We report this case with a brief review of related literatures.

• Childhood Kidney Diseases
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• v.25 no.2
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• pp.128-132
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• 2021

Morning glory syndrome (MGS) is a rare congenital optic disc anomaly with a characteristic fundal finding with severe visual impairment. It may occur in association with various systemic manifestations, even though most of the reported cases were isolated. A 6-year-old male visited the nephrology clinic with a history of microscopic hematuria and at the age of 12 years, he was diagnosed thin glomerular basement membrane nephropathy by kidney biopsy. After the following years, the patient had progressive deterioration of visual acuity, and diagnosed as MGS. Whole Exome Sequencing of this patient and his mother revealed heterozygous COL4A4 mutations [c.81_86del (p.Ile29_Leu30del)]. It is more reasonable to consider MGS seen in this patient as a coincidental finding of autosomal dominant Alport syndrome. To our knowledge, this case represents the first case report of autosomal dominant Alport syndrome associated with MGS.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.168-177
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• 2007

Purpose : The purpose of this study was to investigate whether hypercalciuria patients with hematuria show different renal indices compared to non-hypercalciuria patients with hematuria. Methods : We retrospectively reviewed the medical records of patients with gross or microscopic hematuria whose blood chemistry and 24 hour urine chemistry were examined. After excluding the patients with more than $4 mg/m^2/day$ proteinuria or the patients with urinary calcium excretion between 3 and 4 mg/kg/day, we divided the patients into two groups: a hypercalciuria group whose calcium excretion was more than 4 mg/kg/day(n=30) and a non hypercalciuria group whose calcium excretion was less than 3 mg/kg/day(n=41). The urinary excretion, clearance, and fractional excretion(FE) of Na, K, Cl, Ca, P, urea, and creatinine were calculated and compared between the two groups. Results : The hypercalciuria group had more calcium excretion($6.1{\pm}2.9$ vs $1.5{\pm}0.9 mg/kg/day$), more urea excretion($341{\pm}102$ vs $233{\pm}123 mg/kg/day$), greater glomerular filtration rate(GFR) ($93.7{\pm}31.1$ vs $79.5{\pm}32.0 mL/min$) but lower FENa($1.0{\pm}0.4%$ vs $1.3{\pm}0.6%$) than the nonhyper-calciuria group, although the urinary sodium excretion was similar between the two groups. Conclusion : The greater urea excretion and GFR in hypercalciuric patients suggest that they might be on a higher protein diet than the non-hypercalciuria group. The increased glomerular filtration of sodium and calcium induced by the higher GFR in hypercalciuria would have increased their delivery to the distal tubule, where sodium is effectively reabsorbed but calcium is not, which is suggested by the lower FENa but higher FECa in hyercalciuria. It is recommended that the diet of hematuria patients be reviewed in detail at initial presentation and during treatment.