succimer and Renal-Insufficiency

Ask-Upmark kidney (AUK) is a scarred segment of the kidney, characterized by formation of primitive tubular and glomerular structures, and sporadically diagnosed as a cause of hypertension (HTN). A 6-year-old girl with neurofibromatosis type 1 (NF1) and moyamoya syndrome had severe HTN. Based on past history, she had HTN at the age of 1.5 years. Laboratory examination revealed slightly elevated plasma and renal venous renin activity without lateralization. No evidence of pheochromocytoma, or coarctation of the aorta was found. Contrast-enhanced computed tomography (CT) showed an area of hypoperfusion in the upper and middle poles with reduced size of the right kidney. The results of dimercaptosuccinic acid scintigraphy were in accordance with those of contrast-enhanced CT. Selected renal arteriography revealed a paucity of peripheral vascularity in the same parts of the right kidney. In the absence of a history of urinary tract infection and vesicoureteral reflux by cystography, we presumed that the severe HTN may be due to segmental hypoplasia of the kidney, AUK, with a possible contribution from NF1. Although renal artery stenosis and pheochromocytoma are well-known causes of HTN in NF1, this case demonstrates that HTN can be caused by AUK in patients with NF1.

Topics: Angiography; Antihypertensive Agents; Child; Coloboma; Contrast Media; Female; Humans; Hypertension; Kidney; Kidney Glomerulus; Moyamoya Disease; Neurofibromatosis 1; Proteinuria; Radionuclide Imaging; Renal Insufficiency; Renin; Succimer; Tomography, X-Ray Computed; Treatment Outcome; Vesico-Ureteral Reflux

To determine whether early initiation of clean intermittent catheterization is associated with increased renal preservation in children with spinal dysraphism based on dimercaptosuccinic acid (DMSA) renal scans.. A retrospective review was performed of 100 patients from a pediatric spinal defects clinic from June 2007 to October 2011 who were followed with routine studies including DMSA scans, voiding cystourethrograms, renal/bladder ultrasounds, and urodynamics. DMSA scans were reviewed for evidence of renal cortical loss as defined by presence of scarring or difference in differential function greater than 15%. Multivariate analysis was performed for risk factors for upper tract damage.. Renal cortical loss on DMSA scan was found in 43/100 (43%) of patients. CIC was started at birth in 17/100 (17%) of patients with the rest starting at a median age of 5 years (IQR 3-9). Upon multivariate regression analysis, age at DMSA scan (OR 1.21; 95% CI 1.08-1.36), history of VUR (OR 8.64; 95% CI 2.52-29.57), history of hydronephrosis (OR 3.44; 95% CI 1.12-10.5), and CIC from birth (OR 9.26; 95% CI 1.99-43.18) were statistically significant predictors of kidney damage.. Early initiation of CIC may not reduce the incidence of DMSA abnormalities in pediatric patients with spinal dysraphism.

Topics: Adolescent; Child; Child, Preschool; Cicatrix; Cohort Studies; Confidence Intervals; Cystoscopy; Female; Follow-Up Studies; Humans; Intermittent Urethral Catheterization; Kidney Function Tests; Male; Multivariate Analysis; Radiographic Image Enhancement; Regression Analysis; Renal Insufficiency; Retrospective Studies; Risk Assessment; Secondary Prevention; Severity of Illness Index; Spinal Dysraphism; Statistics, Nonparametric; Succimer; Time Factors; Tomography, X-Ray Computed; Treatment Outcome; Urodynamics; Urography

Topics: Female; Humans; Intermittent Urethral Catheterization; Male; Renal Insufficiency; Spinal Dysraphism; Succimer

Topics: Female; Humans; Intermittent Urethral Catheterization; Male; Renal Insufficiency; Spinal Dysraphism; Succimer

To assess the impact of multiple access tracts during percutaneous nephrolithotomy (PCNL) on short- and midterm renal function, and to determine risk factors predicting renal function deterioration and/or recoverability.. Patients undergoing PCNL with multiple punctures were prospectively enrolled. Preoperative evaluation included dimercaptosuccinic acid and diethylenetriaminepentaacetic acid renography. Patients were classified according to baseline renal function into patients with normal (<1.4 mg/dL) serum creatinine (group A) and patients with elevated (≥1.4 mg/dL) serum creatinine (group B). Patients were followed with serial serum creatinine evaluations and a repeated renography at 12 months. Factors evaluated for possible impact on renal function changes included preoperative renal function, number of access tracts, hypertension, and diabetes mellitus.. There were 102 patients 21 to 65 (mean 39.9) years who completed the study. Fifty patients (group A) had normal preoperative serum creatinine levels and glomerular filtration rate (GFR), which showed no statistically significant change 12 months after PCNL. Fifty-two patients had baseline renal impairment (group B), and they experienced statistically significant worsening of the serum creatinine level and GFR at 12 months postoperatively (P<0.001). Ten (19.23%) patients in group B had a significant deterioration of GFR more than 25%. Independent risk factors for this poor outcome were elevated (≥1.4 mg/dL) preoperative serum creatinine level, diabetes, and hypertension.. PCNL with multiple tracts carries a risk of adversely affecting renal function. Preoperative baseline renal impairment, diabetes, and hypertension are risk factors for significant renal function deterioration after the procedure.

Topics: Adult; Aged; Biomarkers; Creatinine; Diabetes Complications; Female; Glomerular Filtration Rate; Humans; Hypertension; Kidney Calculi; Male; Middle Aged; Nephrostomy, Percutaneous; Radioisotope Renography; Renal Insufficiency; Risk Factors; Succimer

AVF is a known complication of renal biopsy in both native and transplanted kidneys. A 20-yr-old woman with bilateral hypoplastic kidneys due to branchio-oto-renal syndrome had received living-donor renal transplantation from her father at the age of 11. She had undergone allograft renal biopsies six times and all puncture sites were at the lower pole of her kidney from the first to the fifth biopsy. AVF with aneurysmal dilation (30 mm) had developed at the puncture site after the fifth biopsy. TAE was successfully performed with 11 platinum coils in the branch of the renal artery feeding the aneurysm. According to a review of the literature, the incidence of AVF is higher in transplanted kidneys than in native kidneys (7.5% vs. 2.1%) because transplanted kidneys, as single kidneys, are likely to be punctured repeatedly at the same site. When renal biopsy of a transplanted kidney is performed, previous biopsy puncture sites should be considered and the biopsy should be performed at a different site, if possible, to prevent the development of AVF.

Topics: Arteriovenous Fistula; Biopsy, Needle; Branchio-Oto-Renal Syndrome; Dilatation; Embolization, Therapeutic; Female; Humans; Kidney; Kidney Transplantation; Nephrectomy; Renal Artery; Renal Insufficiency; Renal Veins; Succimer; Ultrasonography, Doppler; Young Adult