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Associated anomalies in children with congenital solitary functioning kidney.

Congenital solitary functioning kidney (CSFK) is a relatively common renal malformation and in children is frequently complicated by anomalies of the ipsilateral genital organs and occasionally by anomalies of other organs. The aim of this study was to determine the incidence of associated urological, cardiac, gastrointestinal, hematological, neurological, skeletal, and other congenital malformations in children with CSFK. We retrospectively reviewed 87 consecutive cases of CSFK diagnosed at our hospital between 1995 and 2003. There were 45 boys and 42 girls, whose ages at diagnosis ranged from newborn to 16 years (mean 4.67+/-4.48 years). In all patients, CSFK was diagnosed by abdominal ultrasound and confirmed by radionuclide studies. In 46 patients (53%) the left kidney was absent, and in 41 patients (47%) the right kidney was absent. Overall associated anomalies were detected in 52 of the 87 children (60%) with CSFK. Urological anomalies were most common, with an incidence of 37% (32/87). Nonurological anomalies were detected in 38 children (44%) with CSFK; these included cardiac anomalies in 13, gastrointestinal anomalies in eight, hematological anomalies in five, neurological anomalies in three, and other organ anomalies in nine. In our study, more than half of the patients with CSFK had associated anomalies. For this reason we recommend abdominal ultrasound and voiding cystourethrogram for early recognition of urological anomalies and a careful physical examination for other organ anomalies in patients with CSFK.

Abnormalities, Multiple↗

[Clinical experience of in situ extracorporeal shock wave lithotripsy using ESL-500A for bilateral ureteral stones and ureteral stones in a solitary, or a functionally solitary kidney].

Extracorporeal shock wave lithotripsy (ESWL) using ESL-500A was performed for the treatment of 18 cases of bilateral ureteral stones and 3 cases of a solitary or a functionally solitary kidney with ureteral stones from September 1991 to February 2000. Sixteen cases of the former and all 3 cases of the latter were treated by in situ ESWL, and the other 2 cases of bilateral ureteral stones were treated with auxiliary procedures. No residual stones were observed in any cases except for one of bilateral ureteral stones treated by in situ ESWL. The results show that in situ ESWL using ESL-500A seems to be useful for the treatment of bilateral ureteral stones and ureteral stones in a solitary kidney, including a functionally solitary kidney. However, auxiliary treatment will be needed in cases of urinary tract infection, undetectable stones by ultrasonography, or bilateral large stones.

Adolescent↗

Focal sclerosis of hypertrophied glomeruli in solitary functioning kidneys of humans.

Morphometric data on glomerular size are presented on three patients with solitary functioning kidneys and one with bilateral oligomeganephronic hypoplasia. Renal biopsy of each of two patients with a congenitally absent kidney (unilateral renal agenesis) and a patient with oligomeganephronie, all with proteinuria and renal insufficiency, reveal increases of mean glomerular diameters of at least 1.75X and mean glomerular volumes greater than 5X. These dimensions, which are in the range of maximal increases recorded for man, are associated in all three biopsies with focal sclerosis of the hypertrophied glomeruli. By contrast, the functionally fully-compensated solitary kidney of a patient who lost function of the contralateral kidney from acquired disease, is characterized by the absence of focal glomerulosclerosis and by glomerular enlargement of significantly lesser degree (increased mean diameter 1.24X and mean volume less than 2X). These observations correlate glomerular injury with glomerular size and suggest that in the setting of reduced nephron numbers, nephron destruction via focal glomerulosclerosis may be initiated when compensatory glomerular hypertrophy has reached its limits.

Adult↗

Acute changes in renal function following extracorporeal shock wave lithotripsy in patients with a solitary functioning kidney.

Twelve consecutive patients with a solitary functioning kidney were treated for renal stone by extracorporeal shock wave lithotripsy (ESWL*) with the modified Dornier HM3 lithotriptor and studied for 3 days after treatment. Urinary excretion of electrolytes, N-acetyl-beta-glucosaminidase (NAG), alkaline phosphatase, kallikrein, glycosaminoglycans, albumin and beta 2-microglobulin, and clearances of creatinine, inulin and para-aminohippuric acid were determined, as were serum levels of creatinine, urea, beta 2-microglobulin and aldosterone, and plasma renin activity. Urinary flow rate, free water clearance, and urinary excretion of NAG, kallikrein and beta 2-microglobulin were significantly increased 0 to 24 hours after ESWL. The urinary excretions of alkaline phosphatase, albumin and glycosaminoglycans were unchanged. Glomerular filtration rate was significantly decreased and effective renal plasma flow was unchanged. Filtration fraction was stable. Serum lactic dehydrogenase increased significantly after ESWL and remained high through the period of observation. Serum levels of creatinine, beta 2-microglobulin and aldosterone were unaltered. A decrease in plasma renin activity immediately after treatment is explained by the water immersion and the extracellular volume expansion during treatment.

Female↗

Shock wave lithotripsy in solitary functioning kidneys: is prophylactic stenting necessary?

OBJECTIVE: To assess the safety of shock wave lithotripsy (SWL) without prophylactic stents in solitary functioning kidneys. PATIENTS AND METHODS: Sixteen solitary functioning kidneys with 23 renal stones with a size of <15 mm were treated with SWL as the primary modality. All patients were counseled about the possibility of obstruction, and treatment was offered to those who consented. The safety of SWL was assessed by the need for interventions and the posttreatment renal function. RESULTS: In 14 patients lithotripsy was uneventful. The duration of treatment ranged from 5 to 35 days. One patient with a 15-mm pelvic calculus presented with anuria which resolved before intervention. In 1 patient fragmentation failed, and percutaneous nephrolithotomy was performed. CONCLUSIONS: In solitary functioning kidneys, SWL is safe without prophylactic stents in properly selected and closely monitored patients. Avoiding stents decreases costs, duration of treatment, and stent-related morbidity without unduly compromising safety.

Adult↗

[Simultaneous double malignant tumors on functional solitary kidney: a case report].

A case of simultaneous double cancer on functional solitary kidney is reported. A 72-year-old man was admitted to the Department of Internal Medicine with angina, hypertension and diabetes mellitus. After treatment involving percutaneous transluminal coronary angioplasty, he was sent to the Department of Urology to check the microhematuria. Cystoscopy showed normal interior of urinary bladder. Drip infusion pyelography and computed tomography demonstrated a tumor mass on the upper pole of the right kidney and atrophic left kidney. Preoperative diagnosis was right renal cell carcinoma. However, an additional pelvic tumor was found during surgery, and a partial nephrectomy was performed. Histologic examination confirmed the presence of 2 separate and distinct malignant entities: a renal cell carcinoma and a non-invasive transitional cell carcinoma. Postoperative recovery has been uneventful and without hemodialysis for 16 months. This is the 19th case of simultaneous occurrence of renal cell carcinoma and transitional cell carcinoma in the same kidney and the 1st case in the patient with a functional solitary kidney in the Japanese literature.

Aged↗

Renal artery stenting in patients with a solitary functioning kidney.

PURPOSE: To retrospectively evaluate the results of renal artery stenting in patients with renovascular disease and a solitary functioning kidney. METHODS: Palmaz stents were placed in 16 patients with a solitary functioning kidney, renal artery stenosis, hypertension and renal failure. Stenoses were evaluated with color Doppler ultrasound, MR angiography and digital subtraction angiography (DSA). Indications for stenting were: recoil after percutaneous transluminal renal angioplasty (PTRA) (63%), arterial dissection after PTRA (13%) and primary stenting (25%). Immediate results were evaluated by DSA. On follow-up (6-36 months), patients underwent periodical evaluation of clinical conditions (blood pressure and serum creatinine level) and stent patency, by means of color Doppler ultrasound. RESULTS: Stent placement was successful in all patients (100%). Cumulative primary patency rate was: 100% at 1 day, 93.75% at 6 months, 81.25% at 12 months and 75% at 24 months. A significant reduction in diastolic blood pressure occurred (mean +/- SD 104 +/- 6 vs 92 +/- 3; p < 0.05); renal function improved or stabilized in over 80% of patients. However, there was no significant difference in the creatinine values before and after treatment (mean +/- SD 200 +/- 142 micromol/l vs 197 +/- 182 micromol/l; p > 0.05). CONCLUSION: Renal artery stenting, both after PTRA and as primary stenting, represents a safe procedure, able to preserve renal function in patients with a solitary functioning kidney.

Adult↗

Percutaneous transluminal renal angioplasty in the patient with a solitary functioning kidney.

Since the introduction of percutaneous transluminal renal angioplasty, it has become established as an effective treatment for the correction of renovascular hypertension or the preservation of renal function. However, there has been a general reluctance to use it or other interventional procedures in any patient with a solitary functioning kidney. We attempted renal angioplasty in 11 patients each of whom had a solitary functioning kidney. All 11 had had either a previous contralateral nephrectomy or an autonephrectomy from chronic arterial occlusion. The patients ranged in age from 42 to 81 years. In nine of the 11 patients, a catheter was negotiated across the stenosis, and the lesion was dilated successfully. In two, the lesion could not be crossed. In the group of nine patients whose lesions were dilated successfully, clinical follow-up (2-180 weeks) revealed that hypertension was cured in four and improved in five. Of the two patients in whom angioplasty was not possible, one required emergency renal bypass surgery and the other had no change in renal status. Renal function test results after the procedure were variable and did not correlate with decreases in blood pressure. We conclude that percutaneous renal angioplasty is a satisfactory alternative to surgery for the treatment of renovascular hypertension or the preservation of renal function in a patient with a solitary functioning kidney.

Adult↗

Renal artery stenting in solitary functioning kidneys: technical and clinical results.

OBJECTIVE: To evaluate the clinical and technical results of renal artery stenting for the treatment of renovascular hypertension and renal failure in patients with solitary functioning kidney. MATERIALS AND METHODS: Fifteen patients with solitary functioning kidney underwent renal artery stenting and were followed up for 12-60 months. Before the procedures, systolic and diastolic blood pressures and serum creatinine levels were measured and the number of antihypertensive drugs was recorded and followed up after stenting. In case of restenosis, either in-stent percutaneous transluminal renal angioplasty or stent-in-stent placement was performed. RESULTS: Primary technical success rate was 100%. One lesion was nonostial while 14 were ostial. Primary patency rates were 100% for 6 months, 92.3% for 12 months, and 69.2% for 24 months. The secondary patency rate at 24 months was 100%. The differences between the baseline and postprocedural values of systolic blood pressures, diastolic blood pressures and the number of antihypertensive drug were statistically significant (P < 0.05), except the values of serum creatinine. Hypertension was cured in 1 (6.7%) patient, improved in 4 (26.6%) and stabilized in 10 (66.7%) patients. Renal function improved in 9 (60%), stabilized in 4 (26.6%), and deteriorated in 2 (13.4%) patients. Minor complication rate was 13.4% and major complication rate was 13.4%. CONCLUSION: Revascularization of renal artery stenosis using stent in solitary functioning kidneys is a safe and efficient procedure with high primary technical results, low restenosis rates and acceptable complication rates. It has an improving and controlling effect on blood pressure and renal functions.

Adolescent↗

Outcome of patients with continent urinary reconstruction and a solitary functioning kidney.

OBJECTIVE: To evaluate the outcome of patients with continent urinary diversions who had a solitary functioning kidney at the time of surgery. PATIENTS AND METHODS: In all, 62 patients with continent urinary reservoirs and a solitary functioning kidney were reviewed (51 men and 11 women). The indications for surgery were bladder cancer in 54 and a contracted bladder in eight. The surgical procedures included an orthotopic ileal neobladder in 36 patients, a continent cutaneous ileal reservoir in 13 and rectal diversion in 13. Kidneys were evaluated using serum creatinine level, ultrasonography, intravenous urography and other radiological studies. RESULTS: The follow-up was 6-173 months; 44 renal units (71%) remained stable during this period. Serum creatinine was increased in four patients with an orthotopic neobladder, with no evidence of obstruction or reflux, in one with preoperative renal impairment and one with voiding dysfunction, reflux and bacteriuria. Six renal units deteriorated because of uretero-intestinal strictures; of these patients, two were treated endoscopically, two with open ureteric reimplantation, one with conversion from a rectal reservoir to an ileal loop conduit, and one was maintained on JJ stenting. Six patients with a rectal diversion had renal deterioration because of chronic pyelonephritis. CONCLUSIONS: A regular follow-up of renal function is mandatory in patients with a continent urinary diversion. Rectal diversion is associated with a higher risk of renal deterioration (54%) than are orthotopic (28%) and cutaneous reservoirs (8%).

Adolescent↗

Endovascular revascularization of renal artery stenosis in the solitary functioning kidney.

The treatment of renal artery stenosis by angioplasty and stenting is an effective and accepted alternative to surgery for the treatment of renovascular hypertension and preservation of renal function. We report the technical and clinical outcomes of renal artery stenting in patients with a solitary functioning kidney and renal artery stenosis. From October 1993 to November 1999, 30 stents were placed in the renal arteries of 27 patients (mean age 72+/-8 years) with a solitary functioning kidney and azotemia. The mean diameter renal artery stenosis was 86+/-14%. The mean preprocedure serum creatinine (Cr) level was 3.0+/-1.5 mg/dL (range 1.5-7.5 mg/dL), arterial blood pressure was 171+/-29/85+/-13 mmHg, and the number of antihypertensive drugs was 2.9+/-1.1. Indications for stenting were suboptimal balloon dilation (n = 16), intimal dissection (n = 6), and restenosis following angioplasty (n = 5). Atherosclerotic ostial lesions were present in 25 (93%) of 27 renal arteries. This represents the largest series of renal artery stenting in patients with a solitary functioning kidney, and demonstrates this treatment modality to be a relatively safe alternative to conventional surgery in this high-risk patient group. Most (74%) of the patients in this series had improved or stabilized renal function. Further efforts to define preprocedural indicators of success are necessary to identify the patients who may benefit from revascularization of their solitary kidney.

Aged↗

Proteinuria, hypertension and chronic renal failure in X-linked Kallmann's syndrome, a defined genetic cause of solitary functioning kidney.

BACKGROUND: Anosmia and hypogonadotrophic hypogonadism are the classic features of X-linked Kallmann's syndrome, a disorder caused by mutations of KAL, a gene expressed during kidney and brain development. About a third of patients have a solitary functioning kidney, but little is known about their renal morbidity. METHODS: We studied seven patients aged 22-35 years with X-linked Kallmann's syndrome and a solitary functioning kidney. RESULTS: Two patients developed significant proteinuria associated with mild to moderate arterial hypertension in the second to third decades of life. In one, proteinuria and renal impairment preceded the appearance of hypertension, and the disorder progressed to chronic renal failure. The remaining five patients had normal plasma creatinine concentrations and no significant proteinuria although four had borderline systolic and/or diastolic hypertension. In two sets of patients from the same kindreds, there was a striking discordance for the occurrence of renal morbidity. CONCLUSIONS: All patients with X-linked Kallmann's syndrome should be screened for renal malformations, and those with solitary kidneys require life-long follow-up to detect hypertension, proteinuria and renal failure.

Adult↗

Giant renal calculus in a solitary functioning kidney.

We report a case of giant renal calculus 230 x 140 mm in size and weighing 1,350 g in a solitary functioning kidney treated by nephrolithotomy. A 47-year-old man presented with right lumbar pain, abdominal mass and microscopic hematuria. Physical examination revealed a stony hard mass on the right side of the abdomen, extending from the subcostal region to the iliac crest. A giant renal calculus in his right kidney and atrophic nonfunctioning left kidney was diagnosed by ultrasonography, IVP and CT scan. Right nephrolithotomy was performed. Analysis revealed a calcium phosphate stone. This case is the largest and the heaviest stone reported in the literature in a solitary functioning kidney.

Diagnosis, Differential↗

Transluminal angioplasty in patients with bilateral renal artery stenosis or renal artery stenosis in a solitary functioning kidney.

Renal angioplasty in patients with bilateral renal artery stenosis or with renal artery stenosis in a solitary functioning kidney has been thought to be relatively contraindicated. We report the results of renal artery angioplasty in 18 patients, 10 with severe bilateral renal artery stenosis and eight with severe renal artery stenosis in a solitary kidney. Twenty-five (89%) of 28 angioplasties were successful, with a mean improvement of the degree of stenosis from 85% to 18% after angioplasty and a restoration of renal blood flow. A significant drop in mean blood pressure at admission of 187/101 mm Hg to 154/87 mm Hg at discharge, 152/86 mm Hg at 3-month, and 146/82 mm Hg at 1-year follow-up was seen. Because of the decrease in blood pressure, 11 of the patients decreased or stopped taking diuretics and 15 decreased or stopped taking antihypertensive medications. Although no significant change in renal function was found by measuring mean serum creatinine levels over time, no patient had an elevated serum creatinine level at follow-up, indicating preservation of renal function. One major complication, cholesterol embolization to the bowel, was seen. Our results suggest that angioplasty is an acceptable treatment of hypertension in patients with bilateral renal artery stenosis or renal artery stenosis in a single kidney.

Aged↗

Treatment of severe renovascular hypertension by percutaneous transluminal renal angioplasty in patients with solitary functioning kidney. Effects on blood pressure and renal function.

In this study the effects of percutaneous transluminal renal angioplasty on blood pressure and renal function were studied in 9 hypertensive patients with stenosis of the main artery of a solitary functioning kidney. The outcome of the percutaneous dilatation of the renal artery stenosis, and the effects on blood pressure and renal function were evaluated for a mean period of 16.4 +/- 2.13 (SE) months (ranging from 3 to 65 months). A successful dilatation of the renal artery stenosis was shown in all the patients by the aortography performed 1 h after the procedure. At the discharge (7.8 +/- 0.9 days after dilatation), blood pressure was 'cured' in 2 patients and 'improved' in the remaining patients; renal function was improved in all patients who had reduced renal function. At the last follow-up, no restenosis was found in patients who repeated the follow-up angiography; blood pressure was 'cured' in 3 patients and 'improved' in 6 patients, and renal function appeared steadly improved. In contrast with other reports, our results demonstrate that percutaneous renal angioplasty is a safe and effective procedure and should be attempted before considering surgical intervention in patients with artery stenosis of a solitary functioning kidney.

Adult↗

Treatment of renal stones in a solitary functioning kidney with extracorporeal shock wave lithotripsy.

Forty extracorporeal shock wave lithotripsy (ESWL) sessions were performed in the treatment of 36 stones in 22 solitary functioning kidneys between October 1990 and October 1991. We inserted a double-J catheter in all patients preoperatively and no serious obstruction such as stone street formation was observed after treatments. Following ESWL therapy, at the end of 3 months follow-up, 14 patients (63.6%) were completely stone free while in 5 patients ESWL therapy was unsuccessful. ESWL became the treatment of choice for stones in solitary kidneys as a result of its highly effective, safe and encouraging outcome.

Adolescent↗