Ureteral triplication

A case of ureteral triplication is presented. There was reflux of the middle ureter, which did not end in the kidney, but rather ended blindly. There was atrophic pyelonephritis of the upper pole because of an obstructed ureter, and a normal lower caleceal system and ureter. There were three ureters but only two originated in the kidney and two entered the bladder, with only one ureter originating from the kidney entering the bladder normally. The refluxing ureter and the obstructed upper pole and ureter were removed.     

Type II ureteral triplication associated with ectopic ureter

We report a case of type II ureteral triplication associated with ectopic ureter. The ureter, draining the upper renal segment, entered the vagina, and the other two ureters, draining the middle and lower renal segments, had joined and entered the bladder. The anatomic findings are compatible with those predicted by the Weigert-Meyer law. A thorough evaluation of such an anomaly is essential for effective management.     

Treatment of lithiasis in patients with one kidney by extracorporeal shock wave lithotripsy

OBJECTIVE: To describe the efficacy of extracorporeal shock wave lithotripsy in the treatment of lithiasis in patients with solitary functioning kidney. METHODS: The study comprised 56 patients with solitary kidney that had been treated by extracorporeal shock wave lithotripsy. These patients had a solitary kidney for the following reasons: 30 had undergone nephrectomy due to lithiasis, 11 had lost renal function arising from lithiasis, 9 had undergone nephrectomy for other pathologies and 6 had renal agenesis. Nine stone variables and treatment were analyzed. The same variables were analyzed and compared for statistical significance in a representative sample of 125 lithiasic patients with two kidneys who were treated by ESWL. RESULTS: Patients with solitary kidney frequently had a single calculus of < or = 1 cm located in calyces, unobstructive and did not require complementary endourological procedures. The mean number of shock waves was 8535. The psot-lithotripsy success rate was 82.1%; 98.2% had no complications. Comparison of these variables in both groups of patients showed statistically significant differences for stone location, indication for complementary endourological procedures and the number of shock waves utilized. CONCLUSIONS: In our view, ESWL is the treatment of choice in patients with lithiasis in a solitary kidney due to its high success rate and low morbidity. Stringent criteria must be employed before indicating prior endourological procedures to preserve the patency of the excretory tract and to minimize the risk of ureteral obstruction.     

Ureteric bud remnant in two patients with renal agenesis: diagnosis by MRI

The diagnosis of ureteral bud remnant associated with renal agenesis or dysplasia can be difficult. US and CT can be of limited value in these challenging congenital aberrations. When further evaluation is clinically required, MRI is recommended as a useful adjunct, as shown in the two cases discussed.     

Distal ureteral atresia associated with crossed renal ectopia with fusion: recovery of renal function after release of a 10-year ureteral obstruction

We report on a 10-year-old boy with distal ureteral atresia associated with crossed renal ectopia with fusion. He was admitted with a high fever associated with a urinary tract infection. The diagnosis was established by antegrade and retrograde pyelography. The upper hydronephrotic portion of the kidney, obstructed for 10 years, recovered its function after nephrostomy placement. To our knowledge, this is the first patient whose renal function has recovered despite an ureteral obstruction of 10-years' duration. Therefore, we recommend a transient nephrostomy placement even for far advanced pediatric hydronephrosis, to test for the possibility of functional recovery.     

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.     

Diphallus with urethral duplication: a rare case

We report on a case of glandular diphallus with incomplete urethral duplication associated with rotation anomaly in the right kidney, and a bifid pelvis and an ectopic ureteral orifice in the left kidney. The bladder was single with good sphincter control. At operation, the hipoplasic glans was resected and the urethra which opened into this glans anastomosed alongside the other urethra. Diphallus and incomplete urethral duplication are discussed.     

Masked ureteral duplication with ectopic ureter detected by magnetic resonance imaging

The usefulness of magnetic resonance imaging (MRI) in the detection of a masked duplex kidney system is described. A 9 year old girl was admitted to our hospital for urinary incontinence. She was found to possess an ectopic ureter associated with complete ureteral duplication. She had been evaluated for incontinence at several hospitals, but neither ultrasound sonography nor intravenous pyelography detected any abnormalities. Only cystoscopic examination and MRI detected the anomalies. Partial nephroureterectomy resolved the patient's incontinence. MRI is useful for the detection of ureteral duplication and should be used on cases in which this condition is suspected, prior to performing invasive procedures.     

Angiotensin induces the urinary peristaltic machinery during the perinatal period

The embryonic development of mammalian kidneys is completed during the perinatal period with a dramatic increase in urine production, as the burden of eliminating nitrogenous metabolic waste shifts from the placenta to the kidney. This urine is normally removed by peristaltic contraction of the renal pelvis, a smooth muscle structure unique to placental mammals. Mutant mice completely lacking angiotensin type 1 receptor genes do not develop a renal pelvis, resulting in the buildup of urine and progressive kidney damage. In mutants the ureteral smooth muscle layer is hypoplastic and lacks peristaltic movements. We show that angiotensin can induce the ureteral smooth muscles in organ cultures of wild-type, but not mutant, ureteral tissues and that, in wild-type mice, expression of both renal angiotensin and the receptor are transiently upregulated at the renal outlet at birth. These results reveal a new role for angiotensin in the unique cellular adaptations of the mammalian kidney to the physiological stresses of postnatal life.     

Optimal method of urgent decompression of the collecting system for obstruction and infection due to ureteral calculi

PURPOSE: We compare the efficacy of percutaneous nephrostomy with retrograde ureteral catheterization for renal drainage in cases of obstruction and infection associated with ureteral calculi. MATERIALS AND METHODS: We randomized 42 consecutive patients presenting with obstructing ureteral calculi and clinical signs of infection (temperature greater than 38 C and/or white blood count greater than 17,000/mm.3) to drainage with percutaneous nephrostomy or retrograde ureteral catheterization. Preoperative patient and stone characteristics, procedural parameters, clinical outcomes and costs were assessed for each group. RESULTS: Urine cultures obtained at drainage were positive in 62.9% of percutaneous nephrostomy and 19.1% of retrograde ureteral catheterization patients. There was no significant difference in the time to treatment between the 2 groups. Procedural and fluoroscopy times were significantly shorter in the retrograde ureteral catheterization (32.7 and 5.1 minutes, respectively) compared with the percutaneous nephrostomy (49.2 and 7.7 minutes, respectively) group. One treatment failure occurred in the percutaneous nephrostomy group, which was successfully salvaged with retrograde ureteral catheterization. Time to normal temperature was 2.3 days in the percutaneous nephrostomy and 2.6 in the retrograde ureteral catheterization group, and time to normal white blood count was 2 days in the percutaneous nephrostomy and 1.7 days in the retrograde ureteral catheterization group (p not significant). Length of stay was 4.5 days in the percutaneous nephrostomy group compared with 3.2 days in the retrograde ureteral catheterization group (p not significant). Cost analysis revealed that retrograde ureteral catheterization was twice as costly as percutaneous nephrostomy. CONCLUSIONS: Retrograde ureteral catheterization and percutaneous nephrostomy effectively relieve obstruction and infection due to ureteral calculi. Neither modality demonstrated superiority in promoting a more rapid recovery after drainage. Percutaneous nephrostomy is less costly than retrograde ureteral catheterization. The decision of which mode of drainage to use may be based on logistical factors, surgeon preference and stone characteristics.     

An immunochemical study of the antigens from Toxoplasma gondii tachyzoites obtained in different cultivation systems

Ureteral obstruction in a renal allograft, due to a variety of etiologies, is both a challenging diagnostic and therapeutic disorder. Since ureteral obstruction in a renal transplant recipient usually presents as azotemia, it must also be distinguished from acute rejection. Although ultrasound is non-invasive and readily available, the most definitive diagnostic tool is percutaneous nephrostomy tube placement with antegrade nephrostogram. A variety of therapeutic approaches are available to treat ureteral obstruction in a renal allograft. These procedures can be open (e.g., repeat ureteroneocystotomy) or utilize an endourological approach (e.g., transluminal ureteral dilatation). From an experimental standpoint, recent data in rodent models of experimental hydronephrosis demonstrate similar pathobiologic events in both the obstructed kidney and an allograft undergoing the chronic rejection process. To this end, investigation needs to be conducted to assess whether partial, unrecognized ureteral obstruction in an allograft hastens the development of chronic rejection. This would further underscore the importance of ureteral obstruction as a cause for not only acute azotemia in an allograft, but also chronic deterioration in renal transplant function.     

Partial nephrectomy for renal tumour: 21 cases

Partial nephrectomy has been performed in 21 cases of kidney tumours. It can be used for well-limited parenchymatous tumours and obligatory in patients with a solitary kidney, but is not advisable for tumours originating in the urothelium. These results are compared with the parallel experience with radical surgery and followed during 46 years in the same centre, they give a greater value to this study and encourage the reasoned practice of partial nephrectomy for tumours especially in the solitary kidney.     

Hypernephroma and associated ureteral involvement

The propensity of a hypernephroma to invade the renal vein and even the inferior vena cava is widely known. However, in contrast to papillary carcinoma of the kidney, hypernephromas rarely involve the ureter. Review of the English literature reveals 42 reported cases of ureteral involvement. Most represented metastases as opposed to direct, contiguous extension which is extremely rare. C case of hypernephroma with contiguous castlike extension into the ureter without mucosal violation is presented. Mechanisms of ureteral involvement and implications regarding treatment of hypernephromas are discussed.     

The ureteral "J" sign: radiographic demonstration of iatrogenic distal ureteral obstruction after ureteral reimplantation

Progressive caliceal and ureteral dilatation in postoperative patients is assumed to be secondary to either postoperative distal ureteral stricture or ureteral atony. Any study used to differentiate obstruction from atony must be done with an awareness of the effect of a full bladder on the upper tracts, since it can by itself produce varying degrees of ureteral dilatation. We describe 2 patients who had virtually complete distal ureteral obstruction on anterograde pyelography with a "J" shaped distal ureteral segment. The striking observation was that the deformity and associated distal ureteral obstruction could be made to appear and disappear at will, merely by distending or emptying the bladder. We believe that this is an important factor in the progressive dilatation, as well as in the observed deterioration of renal function, and it is properly regarded as an iatrogenic complication.     

Soft tissue infections with S. Lugdunensis. Presentation of 2 cases and general review]

When perfusion pressure to the kidney falls, e.g., as a result of dehydration or mechanical hindrance to the renal arterial blood flow, the release of renin, hence angiotensin (Ang), surges. This feedback regulation is geared to preservation of renal hemodynamic environment by raising systemic blood pressure. We are aware that a surge of renin-angiotensin release also occurs when there is a mechanical hindrance to urine outflow. This phenomenon of ureteral pressure-sensitive activation of renin-angiotensin has been heretofore viewed as an error of nature. We have obtained evidence which challenges this traditional view when we examined strains of mutant mice which are completely devoid of either angiotensin type 1 (AT1) receptor gene (Agtr1-) or angiotensin type 2 (AT2) receptor gene (Agtr2-) as a result of genetic manipulation of these animals. These strains of mice display varying degrees of urinary tract obstruction. In Agtr2- mice obstructions develop during early kidney ontogenesis in ureto, and, in Agtr1- mice, during late ontogenesis ex utero. One may recall that, throughout its normal ontogenesis, the kidney is twice at risk for obstruction of urine outflow. Thus, in utero the ureter is transiently obliterated. This transient obliteration is believed to protect the kidney from the high pressure from the cloaca when urine is not yet formed. During this period, the ureter is surrounded by dense layers of undifferentiated mesenchymal cells. Subsequent expansive growth that the ureter must achieve, therefore, in concert with a timely disappearance of the surrounding mesenchymal cells. The study in Agtr2- embryos indicated that Ang, through the Agtr2 receptor, promotes disappearance of these mesenchymal cells, and that inactivation of this receptor results in congenital obstructive nephropathy. Our additional studies in human specimens indeed indicate that many infants with congenital anomalies of the kidney and urinary tract have a significant mutation within the AT2 gene. Once animals are born, the kidney comes to be of primary importance for preservation of body fluid homeostasis, and urinary output increases dramatically. The large volume of urine predisposes the kidney to obstructive nephropathy due to the high resistance offered to the urine by the downstream ureter. Normally, a special device develops within the urinary tract in a timely fashion, which enables the kidney to collect a bulk of urine, and then to expel it downward periodically without imposing positive pressure upon the renal parenchyma. This special device is the renal pelvis. In the studies on Agtr1 null mutant mice, we learned that Ang, through the AT1 receptor, promotes development of the pelvis shortly after birth, so that inactivation of this receptor in Agtr1- mice leads to absence of development of the pelvis, hence to obstructive nephropathy. Collectively, Agtr1 or Agtr2 null mutant mice suffer from urinary tract obstruction. Given that urinary tract obstruction per se is a potent stimulus for Ang generation, Ang is essential for the kidney to escape from obstructive injury.     

Congenital leukemia cutis: an unusual manifestation of a rare disease

This paper discusses a case of congenital leukemia cutis of lymphoblastic type presenting as a solitary frontonasal tumor. The presentation is unusual when compared with other reported cases of neonatal leukemia and represents the only reported case with comparable presenting features to the authors' knowledge. The differential diagnosis with other frontonasal tumors is discussed.     

Hypernephroma in the solitary kidney: experience with 20 cases and review of the literature

Twenty patients with renal cell carcinoma in anatomically or functionally solitary kidneys were treated and followed for up to 18 years. Factors pertinent to management and survival of these patients and 66 other well documented similar patients reported in the literature are analyzed. Most of the patients were unusually young and a significant number had had nephrectomy for contralateral renal cell carcinoma. Survival was closely related to the earlier presence of malignant disease in the other kidney, the duration of the interval between detection of the 2 neoplasms and the stage of the lesion in the solitary kidney. Partial nephrectomy has been the most successful treatment. The mean survival in the Mayo Clinic series has been 6 years for patients still alive and 2.4 years for those dead at the time of this analysis. Results in this and other series emphasize the importance of thorough long-term followup after nephrectomy for hypernephroma and of aggressive therapy when the remaining kidney becomes involved. It is foolhardy to abandon hope merely because there is a malignant tumor in a solitary kidney.     

The human natural killer lymphocyte receptors for MHC class I molecules

OBJECTIVES: Angiomyolipoma (AML) is a benign renal tumor that may require treatment because of associated local complications. The present study was undertaken to evaluate the efficacy of nephron-sparing surgery (NSS) in the management of renal AML, with respect to the long-term preservation of renal function and absence of tumor recurrence. METHODS: From 1980 to 1997, 27 patients underwent NSS for treatment of renal AML. The clinical presentation, surgical approach, and outcome in these patients were analyzed. Surgical treatment for renal AML was indicated because of associated symptoms, size of 4.5 cm or greater, and/or suspicion of renal malignancy. RESULTS: In symptomatic patients (52%) the most common presenting signs or symptoms were pain (50%), retroperitoneal hemorrhage or shock (43%), hematuria (36%), hypertension (7%), palpable mass (7%), and anemia (7%). Two patients had tuberous sclerosis. Fifteen patients had a solitary functioning kidney (group I), 6 patients had an impaired contralateral kidney (group II), and 6 patients had a normal contralateral kidney (group III). All operations were performed in situ. There were no operative deaths. All operated kidneys functioned postoperatively, and no patient required dialysis. The mean postoperative serum creatinine level in groups I, II, and III was 1.81, 0.98, and 0.97 mg/dL, respectively. No patients have developed recurrent AML, related symptoms, or required dialysis with follow-up to 177 months (median 39). CONCLUSIONS: When surgical treatment for renal AML is indicated, NSS can be performed with a high success rate even in patients with a very large tumor involving a solitary kidney.     

APIC position paper: responsibility for interpretation of the PPD tuberculin skin test. Association for Professionals in Infection Control and Epidemiology, Inc

Although the deletion of one of the 9-bp repeats in region V of mitochondrial DNA is very common in Asians, Asian-derived populations and Africans, the triplication of the 9-bp segment was described only a few times, mostly on individuals from Asian origin. Here, we report for the first time the presence of the 9-bp triplication in Europeans. The triplication was initially found in one Brazilian individual. Sequencing of the hypervariable segments I (HVSI) and II (HVS2) of the control region and RFLP analysis of the coding region classified the mtDNA as belonging to the European haplogroup H. Since white Brazilians are predominantly of Portuguese descent, we screened 96 unrelated Northern Portuguese for the 9-bp triplication and found its presence in two of them (2.1%). One of these had an mtDNA haplotype identical to that of the Brazilian individual, while the other differed in a single base change in HVS2. The fact that the 9-bp triplication has reached polymorphic frequencies in Northern Portugal and that it has apparently differentiated into at least two lineages defined by the mutuation in HVS2 suggests that it probably occurred a long time ago.