Fibromuscular Dysplasia/diagnostic imaging* ; Humans ; Hypertension/complications* ; Hypertension, Renovascular/etiology* ; Kidney ; Renal Artery/diagnostic imaging*

Infant, Newborn ; Humans ; Fibromuscular Dysplasia/complications* ; Renal Artery ; Hypertension, Renovascular/etiology* ; Renal Artery Obstruction/complications* ; Hypertension/complications*

A girl, aged 8 years, developed jaundice and liver dysfunction in the neonatal period, with congenital glaucoma diagnosed on day 5 after birth, hypertension and unusual facies (broad forehead, hypertelorism and deep-set eyes). Cholestasis was the main type of liver dysfunction. Cardiac macrovascular CTA showed stenosis at the abdominal aorta and the beginning of the bilateral renal arteries. Whole exon sequencing revealed a heterozygous frameshift mutation, c.1485delC (absence of cytosine), in exon 12 of the JAG1gene. The girl was diagnosed with Alagille syndrome and was given transaminase-lowering, cholagogic and antihypertensive treatment with multiple drugs. There were significant reductions in serum levels of alanine aminotransferase, aspartate aminotransferase and total bile acid, but blood pressure fluctuated between 102-140 mm Hg/53-89 mm Hg. After renal artery angiography and balloon dilatation angioplasty, the girl was given oral administration of antihypertensive drugs, and blood pressure was controlled at a level of 110-120 mm Hg/60-80 mm Hg. The rare disease Alagille syndrome should be considered when a child has refractory hypertension with the involvement of multiple systems, especially liver dysfunction with cholestasis as the main manifestation. Genetic causes should be analyzed for a early diagnosis.
Alagille Syndrome ; Blood Pressure ; Child ; Female ; Humans ; Hypertension ; etiology ; Liver Diseases ; etiology ; Renal Artery

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

Renal artery stenosis may be a cause of hypertension and a potential contributor to progressive renal insufficiency. However, the prevalence of renal artery disease in a general population is poorly defined. The purposes of this study were to evaluate the prevalence of angiographically-determined renal artery narrowing in a patient population undergoing routine cardiac catheterization, and to identify the risk factors for renal artery stenosis. After left ventriculography, abdominal aortography was performed to screen for the presence of renal artery stenosis. A total of 427 patients (274 males, 153 females) were studied and the mean age was 59 years. Renal artery narrowing was identified in 10.5% of patients. Significant (> or = 50% diameter narrowing) renal artery stenosis was found in 24 patients (5.6%) and insignificant stenosis was found in 21 patients (4.9%). Significant unilateral stenosis was present in 4.2% of patients and bilateral stenosis was present in 1.4%. The stem of the renal artery was a more common site of stenosis in 62.2% of patients than in the ostium (37.8%), but the severity of stenosis was not significantly different according to the site of stenosis. By univariate and multivariate logistic regression analysis, the association of clinical variables with renal artery stenosis was assessed. Multivariable predictors included age, hypertension and peripheral vascular disease (p < 0.05). The variables such as sex, smoking history, hyperlipidemia, renal insufficiency, as well as the presence of obesity, severity of coronary heart disease and D.M., were not associated. In conclusion, the prevalence of angiographically-determined renal artery narrowing in a patient population undergoing cardiac catheterization is 10.5%. Old age, hypertension and evidence of peripheral vascular disease represent the predictors of renal artery stenosis.
Adult ; Aged ; Female ; Heart Catheterization* ; Human ; Hypertension/etiology ; Male ; Middle Age ; Multivariate Analysis ; Prevalence ; Renal Artery Obstruction/etiology ; Renal Artery Obstruction/epidemiology* ; Risk Factors

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

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

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

A recurrent aneurysm at the anastomosis site or the remaining artery frequently occurs after the operative treatment of an aneurysm in Behcet's disease despite anti-inflammatory medication. Herein, a ruptured left renal artery stump aneurysm in a patient with Behcet's disease, who received a left nephrectomy, aorto-biiliac bypass and heterotopic autotransplantation of the right kidney for the treatment of an abdominal aortic aneurysm and renal hypertension one year prior to this admission, is reported. An aneurysm and rupture occurred despite the administration of anti-inflammatory medications while monitoring of the clinical findings, such as skin manifestations, erythrocyte segmentation rate (ESR) and C-reactive protein (CRP). Although there is no definite proven treatment modality to prevent recurrent aneurysms at the anastomosis site or a remote artery, close follow-up with anti-inflammatory medications, and surveillance with regular intervals are the only current methods for the prevention and/or to treatment of an arterial complication in patients with Behcet's disease.
Adult ; Anastomosis, Surgical/*adverse effects ; Aneurysm, Ruptured/*etiology ; Aortic Aneurysm, Abdominal/*surgery ; Behcet Syndrome/*complications/surgery ; Human ; Hypertension, Renal/surgery ; *Kidney Transplantation ; Male ; Recurrence ; *Renal Artery ; Transplantation, Autologous