• Childhood Kidney Diseases
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• v.22 no.2
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• pp.71-74
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• 2018

Endoscopic subureteral injection for treatment of vesicoureteral reflux (VUR) is known to be safe and efficient due to its minimal invasive nature. Being non-migratory, non-antigenic, and biocompatible, Macroplastique (Polydimethylsiloxane) is likely to be stable over time. A 5-year-old boy with a past history of subureteral administration of Macroplastique for unilateral Grade V VUR 4 years ago presented with recurrent gross and microscopic hematuria, along with suprapubic pain. On computed tomography (CT) abdomen, calcified material, suspected to be a stone, was visualized in the bladder. On diagnostic cystoscopy, calcification was seen around the orifice site where Macroplastique injection had been performed. We removed the calcific material by Holmium laser. Endoscopic subureteric implantation has several advantages, but nevertheless, vigilance is needed to detect long-term complications, especially in patients with gross or microscopic hematuria.

• Childhood Kidney Diseases
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• v.22 no.2
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• pp.75-80
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• 2018

Nutcracker syndrome is a phenomenon that the left renal vein (LRV) is pressed between the superior mesenteric artery (SMA) and the aorta. Clinical characteristics include gross or microscopic hematuria, orthostatic proteinuria, abdominal pain, and back pain. It occurs due to LRV squeezing caused by narrowed aortomesenteric angle. SMA syndrome is a disease that the third part of the duodenum is prone to intestinal obstruction by narrowed angle between the SMA and the abdominal aorta. Clinical symptoms include postprandial abdominal distension, epigastric pain, nausea, and vomiting. SMA syndrome and nutcracker syndrome have common features that result from narrowed aortomesenteric angle. However, it is very rare for both syndromes to occur simultaneously, so the two syndromes are regarded as separate diseases. This is a report on a case of nutcracker syndrome with SMA syndrome in a child who presented gross hematuria, recurrent abdominal pain and vomiting. To our knowledge, nutcracker syndrome simultaneous with SMA syndrome has not been previously reported in pediatric patient, especially with an exhibition of gross hematuria. This case suggests that the simultaneous presence of SMA syndrome with the same pathogenesis needs to be considered when nutcracker syndrome is suspected in pediatric patients with hematuria.

• Childhood Kidney Diseases
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• v.16 no.2
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• pp.142-145
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• 2012

Since urachal abnormalities are uncommon and have various clinical manifestations such as umbilical discharge, periumbilical pain, recurrent urinary tract infection and abdominal mass according to its structure, it is not easy to diagnose. We report our experience of a patient with urachal remnant abscess who presented with gross hematuria initially, and improved after the management with intravenous antibiotics and percutaneous drainage of abscess.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.25-32
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• 2000

Purpose: This retrospective study of 126 children with symptomless primary hematuria was undertaken to determine the distribution of various histologic types by renal biopsy, clinical outcome according to the biopsy findings and also to find out feasibility of performing renal biopsy in these children. Patients and Methods : Study population consisted of 126 children with symptom-less primary hematuria who have been admitted to the pediatric department of Kyung-poot National University Hospital for the past 11 years from 1987 to 1998 and renal biopsy was performed percutaneously. Hematuric children with duration of less than 6 months, evidences of systemic illness such as SLE or Henoch-Schonlein purpura, urinary tract infection, and idiopathic hypercalciuria were excluded from the study. Results : Mean age of presentation was 9.2${\pm}$3.3 years (range ; 1.5-15.3 years) and male preponderance was noted with male to female ratio of 2:1. IgA nephropathy was the most common biopsy finding occuring in 60 children ($47.6\%$), followed by MsPGN in 13 ($10.3\%$), MPGN in 5 ($3.9\%$), TGBM in 6 ($4.7\%$), Alport syndrome in 2 ($1.6\%$), FSGS in 1 ($0.8\%$), and in 39 children ($30.9\%$), 'normal' glomeruli were noted. Recurrent gross hematuria was more common than persistent microscopic hematuria (84 versus 42), and especially in IgA nephropathy, recurrent gross hematuria was the most prevalent pattern of hematuria. In 58 out of 126 cases ($46.0\%$), hematuria was isolated without accompa-nying proteinuria and this was especially true In cases of MsPGN and 'normal' glomer-uli by biopsy finding. Normalization of urinalysis (disappearance of hematuria) in IgA nephropathy, MsPGN and 'normal' glomuli group were similar and it was $14\%,\;27\%\;and\;21\%$ respectively during 1-2 years of follow-up period, and $37.1\%,\;40\%\;and\;35\%$ respectively during 3-4 years of follow-up periods. However, abnormal urinalysis persi-sted in the majority of children with MPGN, TGBM. Alport syndrome and FSGS. Renal function deteriorated progressively in 6 cases (3 with IgA nephropathy, 2 with Alport syndrome and 1 with TGBM). Conclusion : In summary, present study demonstrates that in 126 children with symptomless primary hematuria, IgA nephropathy was the most common biopsy findings followed by MsPGN, MPGN, TGBM, Alport syndrome and FSGS, and 'normal glomeruli' was also seen in 39 cases ($30.9\%$). Renal histology could not be predictable on the clinical findings, so that to establish appropriate long-term planning for these children, we would recommend to obtain precise histologic diagnosis by renal biopsy.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.34-40
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• 1998

Present study was conducted to determine the frequency, clinical characteristics and long-term outcome of children with idiopathic hypercalciuria. Study patients consisted of 150 children with isolated hematuria (recurrent gross or persistent microscopic), and hypercalciuria was defined as urinary calcium excretion over 4 mg/kg/day. During follow-up period up to $6{\sim}8$ years, serial check-up of renal sonogram for stone formation and Dipstick examination for hematuria were done. Forty-four (29%) out of 150 cases were diagnosed as idiopathic hypercalciuria, and in hypercalciuric children compared to normocalciuric children boys were more common than girls (9:35) and gross hematuria was more common than microscopic hematuria (37:7) (P<0.05). Oral calcium loading test showed renal type in 29 cases, absorptive type in 8 cases and in 7 cases type could not be definable. Among 3 types no differences could be found in 24 hour urinary calcium excretion and in clinical or laboratory data. Urolithiasis developed in 4 out of 44 cases (2 at the time of initial diagnosis and 2 within $1{\sim}2$ years of follow-up periods) and these children showed lower chronologic age ($3.7{\pm}2.7\;vs\;7.2{\pm}2.9\;yr$) and more girl than boys (3:1 vs 6:34) (P<0.05) compared to the rest of the hypercalciuric children. Follow-up urinalysis showed disappearance of hematuria in 56, 50, 66 and 75% of children at $1{\sim}2,\;2{\sim}4,\;4{\sim}6$ and $6{\sim}8$ years after initial diagnosis respectively. In conclusion, present study demonstrates that idiopathic hypercalciuria is a major cause of isolated hematuria in children so that in these children 24 hour urinary calcium excretion test seems to be an essential test to be performed. And serial renal sonography should be done to detect development of nephrolithiasis. However, clinical significance of dividing hypercalciuric children into two pathophysiologically distinct subtypes by oral calcium loading test seems to be in doubt and further study is needed to solve this problem.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.97-100
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• 1997

Rupture or contrast extravasation of urinary bladder after voiding cystourethrography(VCUG) was a very rare complication which occurred in neurogenic or unused bladder. Only one case of bladder extravasation was reported in a girl with normal bladder function. Case: A 18 month-old boy presented with recurrent E. Coli urinary tract infection and was evaluated with isotope VCUG, which was failed to catheter insertion. Two days later, isotope VCUG was repeated with difficult catheter insertion. Two hours after isotope VCUG, gross hematuria and anuria developed, and abdominal distension was followed. Bladder rupture was diagnosed by abdominal sonography and computerized tomography. He was treated with simple closure and suprapubic catheter drainage.

• Childhood Kidney Diseases
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• v.24 no.2
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• pp.91-97
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• 2020

Purpose: Alport syndrome (AS) is one of the most common inherited renal diseases caused due to mutations of genes encoding specific proteins of the type IV collagen family, and its major clinical manifestations include progressive renal failure, sensorineural deafness, and ocular abnormalities. We investigated the clinical characteristics and long-term prognosis of AS in Korean pediatric and adult populations. Methods: We conducted a retrospective review of medical records of 33 children and adults who had been diagnosed or treated with AS from 1985 to 2019. Results: The mean age of the 33 patients diagnosed with AS was 16.2±13.6 years, and the male-to-female ratio was 2:1. At the first visit, recurrent gross hematuria was the most common initial symptom. In 10 of 33 patients (30.3%), sensorineural hearing loss (SNHL) was diagnosed, but none had ophthalmic problems. Moreover, 11 of 33 patients (33.3%) had advanced to end-stage renal disease (ESRD), and a significant difference was observed in the age of the patients who progressed to ESRD based on the presence or absence of SNHL (P=0.035). Conclusion: SNHL in AS can be an important prognostic factor for long-term deterioration of renal function. Further investigation is required to confirm the clinical course and the genetic characteristics of AS in Korea through prospective national cohort studies.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.153-160
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• 1999

Purpose : Present study was undertaken to find out significance of clinical presentation, initial laboratory data and renal biopsy findings on subsequent clinical course of IgA nephropathy in children. Methods : Clinical and laboratory data were analysed retrospectively from 60 children who have been admitted to the Pediatric Department of Kyungpook National University Hospital for the past 11 years and diagnosed as IgA nephropathy. Renal biopsy findings were graded according to the pathologic subclass proposed by Haas. Results : Pathologic grading according to Haas subclassification showed 10 cases in subclass I, 36 in II, 12 in IV and 2 in V and none in subclass II. Sex distribution showed male predominance (male to female ratio = 3 : 1) and mean age at onset of disease was $10.4{\pm}2.8$ years. Episodes of gross hematuria was seen in 71.7% and IgA level increased in 28.3% of children and these were not associated with pathologic grading nor clinical outcomes. With increasing subclass grading, serum protein and albumin decreased and 24 hours urinary protein excretion increased. Normalization of urinalysis (disappearance of hematuria) was seen in 14% at 1-2 years and 37.1% at 3-4 years of follow up period. In 3 cases, renal function deteriorated progressively and they belonged one each to the Haas subclass III, IV and V. Conclusion : In children with IgA nephropathy, progression to chronic renal failure appears to be quite high and pathologic grading according to Haas' subclassification seems to predict patient's outcome faily well. However, firm conclusion cannot be drawn from present study due to the small numbers of patients and short follow-up period. Therefore further multicenter study involving larger numbers of patients and longer periods of follow-up over 10 years was to be undertaken.