Wilms's tumor is a malignant renal parenchymal neoplasm. This tumor is most frequently discovered by palpable abdominal mass, while only total gross hematuria is not common in subjective symptom. We have experienced a four year-old girl complaining total gross hematuria and could not define preoperative diagnosis by clinical, radiological and laboratory findings. After operation, Wilms's tumor was confirmed on pathology.
Diagnosis ; Female ; Hematuria ; Humans ; Pathology ; Wilms Tumor*

Ureteroarterial fistula is an extremely rare complication, but is associated with a high mortality rate. Previous pelvic surgery, long standing ureteral catheter insertion, radiation therapy, vascular surgery and vascular pathology contribute the development of this uncommon entity. Herein, a case of ureteroarterial fistula in a 69-year-old female patient, who presented with a massive hematuria, proven in a second attempt at angiography, is reported.
Aged ; Angiography ; Female ; Fistula* ; Hematuria ; Humans ; Mortality ; Pathology ; Urinary Catheters

Ureteroarterial fistula is an extremely rare complication, but is associated with a high mortality rate. Previous pelvic surgery, long standing ureteral catheter insertion, radiation therapy, vascular surgery and vascular pathology contribute the development of this uncommon entity. Herein, a case of ureteroarterial fistula in a 69-year-old female patient, who presented with a massive hematuria, proven in a second attempt at angiography, is reported.
Aged ; Angiography ; Female ; Fistula* ; Hematuria ; Humans ; Mortality ; Pathology ; Urinary Catheters

OBJECTIVESTo reveal etiologies of persistent isolated hematuria (PIH) through ultrastructural pathological examination, to disclose clinicopathological correlation in cases with PIH, and to summarize appropriate management of patients with PIH.

METHODSwe retrospectively studied 155 PIH patients receiving renal biopsy between January, 2003 and December, 2008 in Peking Union Medical College Hospital. All the clinical data and follow-up result were analyzed.

RESULTSAll subjects included 38 children and 117 adults, with mean age of 11.38±3.25 years for children and 35.17±8.44 years for adults. Thin basement membrane nephropathy (TBMN) was the most common pathology (55.3% of children and 49.6% of adults), followed by IgA nephropathy (18.4% of children and 32.5% of adults, mainly grade 2-3) and mesangial proliferative glomerulonephritis (MsPGN) without IgA deposition (13.2% of children and 12.8% of adults). Besides, Alport syndrome (2.6% of children) and membrane nephropathy (2.6% of children and 0.9% of adults) were demonstrated as other causes of PIH. Elevated mean arteral pressure or protein excretion rate, as well as episodic macrohematuria, indicated higher risk for MsPGN rather than TBMN. On the other hand, severity of microhematuria was irrelevant to pathological types of PIH. Totally, 86 patients were followed up and 37 cases therein stayed on track for long term (mean duration 41.11?28.92 months, range 8-113 months). Most cases had benign clinical course except 3 cases with TBMN, 5 cases with IgA nephropathy, 1 case with MsPGN (without IgA deposition), and 1 case with Alport syndrome, who developed hypertension or proteinuria. All of them were administered timely intervention.

CONCLUSIONSClose follow-up should be required as the primary management for PIH. Equally important is careful monitoring for early identification of undesirable predictors; while renal biopsy and other timely intervention are warranted if there is hypertension, significant proteinuria or renal impairment.

Adolescent ; Adult ; Biopsy ; Child ; Female ; Hematuria ; pathology ; Humans ; Kidney ; pathology ; Male ; Middle Aged

OBJECTIVETo compare the clinical and pathological characteristics between patients with and without glomerular hematuria.

METHODSTotally 310 patients with isolated microscopic hematuria and 24-hour urinary protein <0.5 g were enrolled in this study, who were free of renal calculi, infections, or tumors. These patients were divided into glomerular hematuria group and non-glomerular hematuria group according examination by phase-contrast microscope, and their clinicopathological data were retrospectively analyzed.

RESULTSOf the 209 patients identified to have glomerular hematuria, 46.41% had IgA nephropathy, 22.49% had small glomerular lesions, and 14.35% had non-IgA mesangioproliferative glomerulonephritis. In the patients with non-glomerular hematuria, 65.34% had IgA nephropathy, 21.78% had non-IgA mesangioproliferative glomerulonephritis, and 8.91% had small glomerular lesions. No significant differences were found in the patients' gender, age, course of disease or hypertension between the two groups (P>0.05), but renal insufficiency was more common in patients with glomerular hematuria.

CONCLUSIONIgA nephropathy is still the most frequent pathological finding in patients with hematuria, and even in non-glomerular hematuria patients severe renal pathological changes can be common.

Adult ; Female ; Glomerulonephritis, IGA ; pathology ; Hematuria ; pathology ; Humans ; Kidney Glomerulus ; pathology ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

Forty five patients (46 kidneys) who were admitted to the Department of Urology, Pusan National University Hospital between Jan. 1971 and July 1989 and confirmed histopathologically as renal cell carcinoma were analyzed for 11 host and 5 tumor factors relating to prognosis. The results were as follows. 1. Overall 1 and 2 to 5 year survival rates were 66.6 % and 56.3%, respectively. 2. There was no relationship between survival rates and sex, laterality or age of patients. 3. As host factors, palpable mass, fever, weight loss, Hgb, ESR, alpha2-globulin and CRP were clinically significant parameters for survival, whereas hematuria, pain, ALP and LDH were insignificant. 4. As tumor factors, stage, grade, cell type, infiltration, and vessel invasion were clinically significant parameters for survival. Therefore, aforementioned host and tumor factors suggest to be significant parameters for the selection of appropriate therapy and prognosis for renal cell carcinoma.
Busan ; Carcinoma, Renal Cell* ; Fever ; Hematuria ; Humans ; Pathology ; Prognosis ; Survival Rate ; Urology ; Weight Loss

Alport syndrome is the most common type of hereditary nephritis, and acute poststreptococcal glomerulonephritis(APSGN) is a common disease in children. We experienced the clinical and pathologic findings of Alport syndrome and APSGN in brothers of one family. Both patients presented with heavy gross hematuria and proteinuria. ASO titer was elevated in both cases, and the C3 level was reduced in one of the cases. In renal pathology, both showed characteristics of Alport syndrome as well as the glomerular changes of APSGN with hump-like subepithelial deposits by electron microscopy. These clinical observation indicated that the patients had APSGN superimposed on Alport syndrome, and that the episode of APSGN might exacerbate the clinical course of Alport syndrome.
Child ; Glomerulonephritis* ; Hematuria ; Humans ; Microscopy, Electron ; Nephritis, Hereditary* ; Pathology ; Proteinuria ; Siblings*

PURPOSE: Henoch-Schonlein purpura(HSP) is usually a self-limited disease with a good eventual outcome. The prognosis of HSP is mainly determined by the renal involvement. In this study, We evaluated children with biopsy-proven Henoch-Schonlein purpura nephritis about the clinical outcome correlated with renal manifestation and morphologic findings. METHODS: The clinical features, initial laboratory and pathologic findings, and clinical outcome were evaluated in 60 children with biopsy-proven Henoch-Schonlein purpura nephritis at Yonsei University Severance Hospital during the period from Jan. 1990 to Dec. 2002. RESULTS: The ratio of male to female patients was 1.2:1. The interval between the onset of Henoch-Schonlein purpura and renal manifestation was less than 3 months in 81% of the patients. Initial renal manifestation was microscopic hematuria in 100% of patients, isolated hematuria in 15%, acute nephritic syndrome in 7%, nephrotic syndrome in 22% of patients. Renal manifestation correlated with clinical outcome. Grade II and III were the most common in histologic grades of ISKDC. Renal pathologic finding correlated with clinical outcome. CONCLUSION: Renal manifestation and pathologic findings correlated with the clinical outcome. It is necessary to evaluate the correlation between pathologic findings and treatment.
Child ; Female ; Hematuria ; Humans ; Male ; Nephritis* ; Nephrotic Syndrome ; Pathology ; Prognosis ; Purpura, Schoenlein-Henoch*

PURPOSE: Percutaneous renal biopsy in children with primary nephrotic syndrome(NS) contributed to establish the renal pathology and clinicopathological correlation. The most common minimal change lesion(MCL) was steroid sensitive and could be predicted by clinical and laboratory findings. It was uniformly agreed that most nephrotic children who were predicted as MCL, should receive an 8 week course of prednisolone before considering renal biopsy. Early indications of renal biopsy has been those nephrotic children with the age below 1 year and above 8 years, hypertension, hematuria, low serum C3, renal insufficiency which are not compatible with MCL. Late indications has been frequent relapser(FR), steroid dependent(SDNS) and steroid resistant nephrotic syndrome(SRNS). The indications have been challenging recently and we tried to evaluate the commonly recommended indications. METHODS: Clinical, laboratory, pathologic findings and therapeutic responses were compared in 81 children with primary nephrotic syndrome who had renal biopses beteen 1984 and 1996. RESULTS: 1) Among 11 children with the age indication, MCLs were diagosed in 9(81.8%), mesangial proliferative glomerulonephritis(MsPGN) in 1(9.1%) and membranous nephropathy(MGN) in 1(9.1%). 2) Among 4 children with microscopic hematuria, MCLs were diagosed in 3(75.0%), MsPGN in 1(25.01%). In 13 children with hypertension, macrohematuria, azotemia and low serum C3, focal segmental glomerulosclerosis(FSGS), membrano-proliferative glomerulonephritis(MPGN) and IgA nephropathy(IgA) were frequently diagnosed instead of MCL. 3) All 5(100%) frequent relapsers were diagnosed as MCLS Among 30 children with the indication of SDNS, MCLs were diagosed in 28(93.3%), MsPGN in 2(6.7%) 4) Among 18 children with the indication of SRNS, MCLs were diagosed in 6(33.3%), MsPGN in 6(33.3%), FSGS in 6(33.3%) 5) The probability to diagnose MCL was 81.8%(9/11) in age indication, 75.0%(3/4) in microscopic hematuria, 100%(5/5) in FR and 93.3%(28/30) in SDNS. 6) The response rate to standard steroid treatment were 81.8%(9/11) in age indication 75.0%(3/4) in microscopic hematuria, 100%(5/5) in FR and 96.7%(29/30) in SDNS. CONCLUSIONS: Among the commmon indications of renal biopsy in children with primary nephrotic syndrome, age, microscopic hematuria, frequent relapser and steroid dependant should be reevauated to reduce the unnecessary renal biopsy.
Azotemia ; Biopsy* ; Child* ; Hematuria ; Humans ; Hypertension ; Immunoglobulin A ; Nephrotic Syndrome* ; Pathology ; Prednisolone ; Renal Insufficiency

OBJECTIVETo analyze the clinical features and gene mutation of Chinese children with Alport syndrome(AS).

METHODFrom May 2011 to May 2014, clinical and pathological information gathered from 25 patients was retrospectively analyzed. COL4A5, COL4A4 and COL4A3 genes were analyzed using next-generation sequencing in these patients, and gene mutations of related family members were identified by Sanger method.

RESULTOf these 25 cases, 19(76%) had X-linked Alport syndromes (XL-AS), 6 had autosomal recessive Alport syndromes (AR-AS). Twenty five patients had an onset of hematuria and proteinuria and in 8 cases the disease was induced by upper respiratory tract infections. Hearing loss was present in 2 of 25 (8%) cases and ocular lesions in 1 of 25 (4%). Renal pathology showed that 16 of them had minimal change disease (MCD), 8 mesangial proliferative glomerulonephritis (MsPNG), 1 focal segmental glomerulo-sclerosis (FSGS). Extensive lamination and split of glomerular basement membrane (GBM) dense layers were found in 2 (8%) of 25 patients. Twenty one of 25 patients (84%) showed abnormal renal α-chain distribution. COL4A5, COL4A4 and COL4A3 genes of 25 patients (23 families) were analyzed and 24 pathogenic mutations were identified: 18 in COL4A5, 1 in COL4A3 and 5 in COL4A4. It was observed that 13 patients inherited the mutation from the mother, 3 patients inherited from the father, 2 patients inherited 1 mutation from the mother and another mutation from the father, and 7 patients carried the novel mutations.

CONCLUSIONXL is the main inherited type in AS. Most of patients showed MCD and MsPNG in renal biopsy. This research examined 24 mutations and 16 mutations were not reported previously.

Child ; Deafness ; Genes, Recessive ; Genotype ; Hematuria ; Humans ; Kidney ; Mutation ; Nephritis, Hereditary ; genetics ; pathology ; Pedigree ; Phenotype