OBJECTIVES: To investigate the genotypes of the pathogenic gene COL4A5 and the characteristics of clinical phenotypes in children with Alport syndrome (AS). METHODS: A retrospective analysis was performed for the genetic testing results and clinical data of 19 AS children with COL4A5 gene mutations. RESULTS: Among the 19 children with AS caused by COL4A5 gene mutations, 1 (5%) carried a new mutation of the COL4A5 gene, i.e., c.3372A>G(p.P1124=) and presented with AS coexisting with IgA vasculitis nephritis; 3 children (16%) had large fragment deletion of the COL4A5 gene, among whom 2 children (case 7 had a new mutation site of loss51-53) had gross hematuria and albuminuria at the onset, and 1 child (case 13 had a new mutation site of loss3-53) only had microscopic hematuria, while the other 15 children (79%) had common clinical phenotypes of AS, among whom 7 carried new mutations of the COL4A5 gene. Among all 19 children, 3 children (16%) who carried COL4A5 gene mutations also had COL4A4 gene mutations, and 1 child (5%) had COL4A3 gene mutations. Among these children with double gene mutations, 2 had gross hematuria and proteinuria at the onset. CONCLUSIONS This study expands the genotype and phenotype spectrums of the pathogenic gene COL4A5 for AS. Children with large fragment deletion of the COL4A5 gene or double gene mutations of COL4A5 with COL4A3 or COL4A4 tend to have more serious clinical manifestations.
Humans ; Nephritis, Hereditary/pathology* ; Hematuria/complications* ; Retrospective Studies ; Collagen Type IV/genetics* ; Genotype ; Mutation

We studied 60 cases of minimal change nephrotic syndrome (MCNS) with mesangial IgA deposits occurring over a 6 year period. There were 43 adults and 17 children. Hematuria occurred in 69.0% of the adults and 88.2% of the children. Two adults and six children had gross hematuria during the course of the disease. Mesangial IgA deposits were noted in 100% of the cases, and concomitant IgG or IgM deposits were found in 78.6% of adults and 73.7% of children. The fluorescent intensity of mesangial IgA deposits was trace (+/-) to 1+ in 86.1% and 70.6% of the adults and children respectively. Most of the patients showed electron microscopic findings consistent with minimal change nephrotic syndrome. We speculate that most of our cases are variants of minimal change nephrotic syndrome but are neither IgA nephropathy nor an overlapping syndrome, and that environmental or genetic factors may be related to the deposition of IgA in these MCNS patients.
Adult ; Child ; Comparative Study ; Female ; Glomerular Mesangium/immunology/*pathology ; Hematuria/etiology ; Human ; Immunoglobulin A/*analysis ; Male ; Nephrosis, Lipoid/complications/immunology/*pathology

OBJECTIVETo assess whether primary hypertension affects the occurrence and progression of benign prostatic hyperplasia (BPH).

METHODSA total of 423 cases of BPH, undergoing transurethral resection of prostate (TURP) or open surgery due to severe low urinary tract symptoms, were reviewed and analyzed. All cases were verified to be BPH postoperatively following histopathological examination.

RESULTSOf 423 patients, 295 cases (69.7%) were simple BPH (group BPH-NT); 128 cases (30.3%) were BPH with hypertension (group BPH-HT). The mean age and the incidence of haematuria were significantly higher in group BPH-HT than those in group BPH-NT (P < 0.05). The time of BPH occurrence and surgical treatment in group BPH-HT with mean diastolic blood pressure >/= 90 mmHg was significantly earlier than those with diastolic blood pressure < 90 mmHg (P < 0.05; P < 0.01). As compared with group BPH-NT, the time of BPH occurrence was significantly earlier in group BPH-HT with more than 10 years hypertension; the rate of urinary retention and haematuria was significantly higher and prostatic volume was significantly greater in group BPH-HT with more than 20 years hypertension; (all P < 0.05). Additionally, prostatic volume was positively correlated with the years of hypertension in group BPH-HT (Rsq = 0.056, P = 0.009).

CONCLUSIONSThe present results demonstrate that BPH may be frequently accompanied by the disease of hypertension. A long-term hypertension, particularly the condition of high diastolic blood pressure may improve the occurrence and clinical progression of BPH.

Aged ; Aged, 80 and over ; Hematuria ; etiology ; Humans ; Hypertension ; complications ; Male ; Middle Aged ; Prostate ; pathology ; Prostatic Hyperplasia ; complications ; pathology ; Retrospective Studies ; Severity of Illness Index ; Urinary Retention ; etiology

BACKGROUND/AIMS: No definite conclusions have been reached about the natural history of patients with isolated microscopic hematuria (IMH). In this study, we observed the natural history of patients with IMH and examined factors related to a pathologic diagnosis and subsequent prognosis. METHODS: We retrospectively evaluated 156 subjects with IMH who had a renal biopsy performed. Of the 156 subjects, 33.3% were diagnosed with IgA nephropathy, 23.7% with mesangial proliferative glomerulonephritis, 15.4% with glomerular minor lesion, and 12.8% with thin basement membrane nephropathy; 6.4% had normal biopsies. RESULTS: We followed up with 100 subjects for about 31 months. During this follow-up period, two subjects who had received a pathologic diagnosis of IgA nephropathy developed chronic kidney disease. During the course of the study, one of these subjects presented with proteinuria and hypertension and the other with proteinuria. The overall incidences of proteinuria and hypertension were 6% and 5% respectively. CONCLUSIONS: The prognosis for patients with IMH was relatively favorable, but patients developing proteinuria and/or hypertension require careful observation and management during the follow-up period.
Adult ; Chronic Disease ; Female ; Hematuria/complications/*pathology ; Humans ; Kidney/*pathology ; Kidney Diseases/etiology ; Male ; Middle Aged ; Proportional Hazards Models ; Retrospective Studies

Antibodies, Antineutrophil Cytoplasmic ; blood ; Child ; Female ; Hematuria ; etiology ; Humans ; Kidney ; pathology ; physiopathology ; Kidney Function Tests ; Proteinuria ; etiology ; Renal Insufficiency ; etiology ; Vasculitis ; blood ; complications ; pathology

OBJECTIVETo understand the clinical and pathological characteristics of IgA nephropathy (IgAN) with crescentic formation in children.

METHODSClinicopathological data of 29 children with IgAN accompanied by crescents were analyzed. These patients were divided into two groups according to the percentage of glomeruli affected by crescents more or less than 50%, and their data were compared.

RESULTS(1) CLINICAL FEATURES: all the patients had hematuria and proteinuria, and macrohematuria (86%) and proteinuria were also common, protein excreted in urine was more than 1 g per day in 76% of the patients. The patients with edema, hypertension, and renal insufficiency were less than fifty percent. Nine patients in Group A (glomeruli affected by crescents > or = 50%) were crescentic IgAN. Significantly more cases in Group A had persistent macrohematuria, hypertension and renal failure than in Group B (glomeruli affected by crescents < 50%) (P < 0.05), with especially severe proteinuria (P < 0.01). It was easy to find nephritic syndrome in Group A, and asymptomatic hematuria combined with proteinuria in Group B. (2) Renal pathology: the glomeruli were affected by crescents from 5% to 85%. There were 52% to 85% in Group A, and 5% to 40% in Group B. Most crescents were cellular. All the cases had a diffuse mesangial proliferation and tubular-interstitial injury to different degree. Three cases had crescentic IgAN. Glomerulosclerosis was significantly more often seen in Group A (P < 0.05) and tuft adhesion was more frequently seen in Group B (P < 0.05). (3) Immunofluorescence: All the patients presented deposition of IgA, IgM and C3. There were 45% specimens combined with the deposition of IgG. Five cases showed 'full house' (17%), four of them were in Group A. None had IgA deposition alone.

CONCLUSIONThe main clinical feature of IgAN with crescentic formation were hematuria combined with proteinuria, especially persistent gross hematuria and severe proteinuria. All of them showed diffuse mesangial proliferation and tubular-interstitial injury in morphology of kidney. Most of them had tuft adhesion. The main type of immunofluorescence were IgA + IgM and IgA + IgM + IgG deposition. Some showed 'full house' phenomenon. The clinical manifestation and renal lesions of IgAN with diffuse crescentic formation were worse than IgAN with glomeruli affected by crescents < 50%.

Adolescent ; Biopsy ; Child ; Child, Preschool ; Female ; Glomerulonephritis, IGA ; complications ; pathology ; Hematuria ; etiology ; Humans ; Hypertension ; etiology ; Kidney ; pathology ; Kidney Function Tests ; Male ; Prognosis ; Proteinuria ; etiology ; Renal Insufficiency ; etiology

OBJECTIVE: To evaluate the clinical and pathological features of 94 children suffering from IgA nephropathy (IgAN) while estimating the prevalent situation in Hunan province. METHODS: To summarize the annual number of hospitalized children, those with kidney diseases, those accepted biopsy, and those confirmed as IgAN in both Xiangya Hospital and Second Xiangya Hospital undertaking kidney biopsy in Hunan province during 1995 and 2004. RESULTS: In the past 10 years, as the hospitalized population in both hospitals accrued to 9.98% each year. The rate of 7.5% was seen in those with kidney diseases. Among whom 56.3% accepted kidney biopsy and 94 of them were confirmed as IgAN. Hematuria was the main clinical presentation, seen in 71 cases, accounting to 76%, and even to 98% after excluding those with nephrotic syndrome and isolating proteinuria type of IgAN. Inflammation infiltration (91%), renal tubule degeneration (81%), and renal interstitial fibrosis (31%) were the major pathological features of 94 children, especially in nephrotic syndrome IgAN. CONCLUSION The number of children with IgAN synchronously accrues as hospitalized population, those with kidney diseases, and those by kidney biopsy. Hematuria is the major symptom. To routinely perform urine analysis and kidney biopsy in asymptomatic hematuria may improve the diagnosis. Inflammation infiltration, renal tubule degeneration, and renal interstitial fibrosis are the major pathological features in IgAN children, especially in nephrotic syndrome IgAN, probably relating to continuous proteinuria. Early control of proteinuria may delay or decrease renal tubule fibrosis.
Adolescent ; Biopsy, Needle ; Child ; Child, Preschool ; China ; epidemiology ; Female ; Glomerulonephritis, IGA ; complications ; epidemiology ; pathology ; Hematuria ; diagnosis ; etiology ; Hospitalization ; statistics & numerical data ; Humans ; Kidney ; pathology ; Male

OBJECTIVETo investigate the clinicopathological characteristics and the prognosis of bladder neuroendocrine carcinoma (NEC).

METHODSClinicopathological data from 17 NEC of the bladder cases were collected, and immunohistochemical staining was performed with follow-up analysis and literature review.

RESULTSThe recruited included 13 male and 4 female patients, aged from 48 to 86 years old (average 61 years; 14 patients >60 years). Gross hematuria of the whole urination course or intermittent was the initial symptom. Macroscopically, the outer surface of the tumor presented with polypoid, lobulated, fungating or ulcerous structures. Histologically, according to the criteria of WHO classification of neuroendocrine tumor of the lung, our NEC cases were divided into three histological types: 13 cases of small cell carcinoma, 3 cases of large cell neuroendocrine carcinoma and 1 case of atypical carcinoid. The urothelial carcinoma was concurrent with NEC in 6 cases, and adenocarcinoma was concurrent with NEC in 2 cases. Most tumor tissue infiltrated to the muscular layer, some infiltrated to the outer membrane. Immunohistochemically, the positive expression rates of CD56, Syn and CgA were 16/17, 16/17 and 12/17, respectively. The epithelial markers, including CK7 and CKpan, were also expressed with positive rates of 12/17 and 15/17, respectively. TTF-1 was positively expressed in 11 cases. The follow-up data were available in 14 cases, of which 9 patients died of the tumor 1-34 months after surgery (average, 11 months). Five patients lived uneventfully for 1-12 months after surgery.

CONCLUSIONSNEC is a rare malignant tumor of the bladder. Immunohistochemical markers such as CD56, Syn, CgA and CKpan could be helpful in determining the diagnosis and differential diagnosis of the tumor. NEC is a highly invasive malignant tumor with poor prognosis. Based on its biological behavior, radical cystectomy is the preferred method of treatment for the tumor.

Adenocarcinoma ; pathology ; Aged ; Aged, 80 and over ; Biomarkers, Tumor ; Carcinoma, Large Cell ; pathology ; Carcinoma, Neuroendocrine ; classification ; complications ; pathology ; Carcinoma, Small Cell ; pathology ; Carcinoma, Transitional Cell ; classification ; complications ; pathology ; Cystectomy ; Female ; Hematuria ; etiology ; Humans ; Male ; Middle Aged ; Neuroendocrine Tumors ; classification ; pathology ; Prognosis ; Urinary Bladder Neoplasms ; classification ; complications ; pathology

BACKGROUNDBoth benign prostatic hyperplasia (BPH) and primary hypertension are common in the elderly men. The purpose of this study was to investigate the possible effect of primary hypertension on the hematuria in patients with BPH.

METHODSAll patients who underwent transurethral resection of prostate or opening operation had confirmed diagnoses of BPH histologically. comparative analysis of packet was used to analyze the incidence of hematuria in 423 BPH patients with or without hypertension. Immunostaining of CD34 and vascular endothelial growth factor (VEGF) was carried out in tissues of 50 cases of simple BPH and 50 cases of BPH accompanied with hypertension.

RESULTSThe incidence of hematuria in the BPH with hypertension was significantly higher than that in the simple BPH (P < 0.01). Furthermore, the incidence of hematuria in patients who had hypertension for more than 10 years was clearly higher than that in the patients who had hypertension for less than 10 years (P < 0.01). Both microvessel density (MVD) based on CD34 immunostaining and VEGF expression were significantly higher in the BPH tissues of patients with hypertension than that in the simple BPH (P < 0.01, P < 0.05).

CONCLUSIONSLong-term hypertension may significantly increase the incidence of hematuria in patients with both BPH and hypertension. Increased MVD level and VEGF expression may account for the higher incidence of hematuria in these patients.

Aged ; Aged, 80 and over ; Hematuria ; metabolism ; pathology ; Humans ; Hypertension ; metabolism ; physiopathology ; Immunohistochemistry ; Male ; Microvessels ; pathology ; Middle Aged ; Neovascularization, Pathologic ; metabolism ; pathology ; Prostatic Hyperplasia ; complications ; metabolism ; pathology ; Vascular Endothelial Growth Factor A ; metabolism

We treated a 54-year-old woman who was suffering from membranoproliferative glomerulonephritis associated with a complete type of hydatidiform mole. The renal manifestations were proteinuria and hematuria. A renal biopsy, performed before gynecologic management, disclosed focal and segmental subendothelial deposits with a proliferation of the mesangial cell and showed irregularly thickened capillary loops by light and electronmicroscoy. Genralized edema, proteinuria and hematuria were completely recovered by suction and curettage of the hydatidiform mole with prophylactic chemotherapy. The clinical manifestation of earlier presented 3 cases have been the nephrotic syndrome. The common feature of them was a complete remission of the nephropathy after the removal of the hydatidiform mole. The relationship between the hydatidiform mole and glomerulonephritis remains unresolved at present. But we concluded that the hydatidiform mole might be a cause of glomerulonephritis in this case.
Case Report ; Diagnosis, Differential ; Edema/etiology ; Female ; Glomerulonephritis, Membranoproliferative/pathology ; Glomerulonephritis, Membranoproliferative/etiology* ; Hematuria/etiology ; Human ; Hydatidiform Mole/therapy ; Hydatidiform Mole/diagnosis* ; Hydatidiform Mole/complications* ; Middle Age ; Pregnancy ; Proteinuria/etiology ; Uterine Neoplasms/therapy ; Uterine Neoplasms/diagnosis* ; Uterine Neoplasms/complications*