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

We report a case of renin-secreting juxtaglomerular cell tumor which developed in a hypertensive 47-yr-old Korean man. Presumptive clinical diagnosis was made before surgery based on the high level of plasma renin and the radiologic evidence of renal mass. Grossly, a round, bulging, well-encapsulated mass of 3x3 cm was located in the mid-portion of the right kidney. On microscopic examination, the tumor was composed of ovoid to polyhedral cells with bland nuclei, indistinct nucleoli and light eosinophilic cytoplasm. The immunostaining for renin showed strong positivity in the cytoplasm of tumor cells. The characteristic rhomboid shaped renin protogranules were observed in ultrastructural analysis.
Human ; Hypertension, Renal/*etiology/pathology ; Juxtaglomerular Apparatus/*pathology ; Kidney Neoplasms/*complications/*pathology/secretion ; Male ; Middle Age ; Renin/blood/secretion

OBJECTIVETo investigate the clinical and pathological characteristics of patients with clinically presumed hypertensive nephrosclerosis (HN).

METHODSClinical data and renal biopsy results were obtained in 63 patients diagnosed clinically as HN (primary hypertension plus renal injury).

RESULTSHN was confirmed by biopsy in 47 out of 63 patients (74.6%, 12 malignant nephrosclerosis and 35 benign nephrosclerosis). Primary nephritis (PN) was diagnosed by biopsy in 10 patients (7 IgA nephropathy, 2 mesangial proliferative nephritis, 1 chronic interstitial nephritis) and focal and segmental glomerulosclerosis (FSGS) in 6 patients. Blood pressure, body mass index, GFR and blood lipids were similar among groups. HN patients were related to higher age, more frequent family history of hypertension, longer hypertension duration, higher left ventricular mass index, lower serum creatinine and lower incidence of microscopic hematuria. Most patients with malignant nephrosclerosis and FSGS patients showed grades III and IV retinopathy.

CONCLUSIONOur results show that HN was misdiagnosed in nearly 25% patients in this cohort. Since the clinical features are similar between HN, PN and FSGS, renal biopsy is needed to establish the diagnosis of HN.

Adult ; Aged ; Female ; Humans ; Hypertension, Renal ; complications ; diagnosis ; pathology ; Kidney ; pathology ; Male ; Middle Aged ; Nephrosclerosis ; diagnosis ; etiology ; pathology

Angioplasty, Balloon ; methods ; Atherosclerosis ; pathology ; therapy ; Humans ; Hypertension ; complications ; drug therapy ; Renal Artery Obstruction ; etiology ; therapy ; Stents

AIMTo report two cases of the left testicular artery arching over the left renal vein (LRV) before running downward to the testis.

METHODSThe subjects were obtained from two Japanese cadavers. During the student course of gross-anatomical dissection, the anatomical relationship between the testicular vessels and the renal vein was specifically observed.

RESULTSThe arching left testicular artery arose from the aorta below the LRV and made a loop around the LRV, which appeared to be mildly compressed between the arching artery and the psoas major muscle.

CONCLUSIONClinically, compression of the LRV between the abdominal aorta and the superior mesenteric artery occasionally induces LRV hypertension, resulting in varicocele, orthostatic proteinuria and hematuria. Considering that the incidence of a left arching testicular artery is higher than that of a right one, an arching left artery could be an additional cause of LRV hypertension.

Aged, 80 and over ; Arteries ; abnormalities ; Constriction, Pathologic ; complications ; Humans ; Hypertension ; etiology ; Male ; Renal Veins ; pathology ; Testis ; blood supply

Two cases of primary intimal fibroplasia of the renal artery with renovascular hypertension are described. Case 1 was 27 year old female who was incidentally found to have blood pressure of 210/130 mmHg on routine physical examination. Renal arteriogram revealed tubular narrowing of the mid protion of the left renal artery. Both patients showed lateralization in renin activity at the involved side renal vein. Case 2 was a 10 year old girl who was first noted to have a hypertension of 180/120mmHg after a sudden attack of seizure, vomiting and altered consciousness. Renal arteriogram showed concentric narrowing of the proximal half of the right renal artery. Histopathologic examination of the affected arterial segments from both cases showed essentially same findings, i,e., diffuse fibrous thickening of the intima occluding the lumen, focal fragmentation, duplication and disappearance of the internal elastic membranes. There were no deposit of lipid and inflammatory cells. The media and adventitia remained intact. The blood pressure of both patients became normal, after the surgery and the patients are in good health up to this time.
Adult ; Arterial Occlusive Diseases/*pathology ; Child ; Female ; Fibromuscular Dysplasia/complications/epidemiology/*pathology ; Humans ; Hypertension, Renovascular/*etiology/surgery ; Korea/epidemiology ; Renal Artery/*pathology/surgery

Actins ; metabolism ; Adult ; Antigens, CD34 ; metabolism ; Carcinoma, Renal Cell ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Glomus Tumor ; metabolism ; pathology ; Hemangiopericytoma ; metabolism ; pathology ; Humans ; Hypertension ; etiology ; Juxtaglomerular Apparatus ; metabolism ; pathology ; surgery ; ultrastructure ; Kidney Neoplasms ; complications ; metabolism ; pathology ; surgery ; ultrastructure ; Nephrectomy ; Wilms Tumor ; metabolism ; pathology