Urologic implications of von Hippel-Lindau syndrome

A patient with von Hippel-Lindau syndrome is presented. Bilateral simultaneous radical nephrectomy was performed for bilateral renal cell carcinoma without evidence of metastasis. The literature is reviewed and the urologic implications of this syndrome are emphasized.     

Urologic implications of anorectal malformations

The urologist has assumed a major role in the management of the child with imperforate anus. Fistulas from the rectum to the urinary tract, as well as associated genitourinary anomalies such as vesicoureteral reflux, make the urologist an important member of the team managing imperforate anus. Special spheres of urologic interest occur immediately (when accurate imaging is important), during the first year (when urinary tract infections and acidosis may occur), and in later childhood and adolescence (when the management of neurogenic bladder or complications of previous repairs of the anorectal anomaly are important).     

Urologic implications of imperforate anus

Recent clinical studies have documented the high association of genitourinary and lumbosacral spine anomalies with imperforate anus. Many of these associated anomalies may go undetected unless special diagnostic studies are performed, and these genitourinary anomalies can be the cause of significant clinical morbidity in later life if not managed properly initially. All neonates with imperforate anus should therefore undergo radiographic examination with lower spine films, intravenous pyelography and voiding cystourethrography, and cystoscopy should be reserved for those with cloaca deformity or those whose x-ray films demonstrate urinary anomalies requiring further workup. Significant morbidity involving the genitourinary tract may be encountered after surgical treatment of the anorectal anomaly, particularly if total anorectal reconstructions are performed in the neonatal period.     

Urologic manifestations of the iliacus hematoma syndrome.

Anticoagulated patients may have a characteristic syndrome of femoral neuropathy from an iliacus muscle hematoma. They may present with urologic signs and symptoms, including groin, flank and thigh pain, groin tenderness, an iliac fossa mass and hematuria. Urography may reveal an enlarged psoas shadow and hydronephrosis from ureteral obstruction. Prompt diagnosis is essential so that early operative decompression of the femoral nerve can be done. The urologist has an important role in the diagnosis and treatment of this syndrome.     

Urologic manifestations of laurence-moon-biedl syndrome

Although uremia is the major cause of death in more than 30 per cent of patients with Laurence-Moon-Biedl syndrome, little attention has been paid to this rare syndrome in the urologic literature. We herein report on 3 patients with this syndrome.     

Urologic manifestations of postpolio syndrome

To determine the character and prevalence of bladder dysfunction among persons with a history of polio, we conducted a survey of randomly selected polio survivors. The survey solicited information on the acute episode of polio and the nature of late-onset neurologic symptoms that could be attributed to postpolio syndrome. There were 242 female and 88 male respondents to the study. Symptoms attributable to postpolio syndrome were present in 87.2% of female subjects and 73.9% of male subjects. Respondents with postpolio syndrome had a significantly greater prevalence of urologic symptoms than seen among respondents without postpolio syndrome, although no dominant pattern of voiding dysfunction was noted. The early onset of erectile dysfunction was more common among male subjects with postpolio syndrome than among male subjects without postpolio syndrome. Genuine stress incontinence was seen in 36.3% of the survey population. Sixteen women with postpolio syndrome underwent surgical repair for urinary incontinence, with a success rate of 60.5%. Bladder disorders are common among persons with PPS, but further clinical and urodynamic data are necessary to define the nature and magnitude of this dysfunction.     

Urologic evaluation of megacystis-microcolonintestinal hypoperistalsis syndrome

The seventh case of megacystis-microcolon-intestinal hypoperistalsis syndrome is presented with its urologic aspects. In contrast to the bowel conditions, the urologic problems were tractable.     

5例Wolfram综合征患者的护理

介绍5例Wolfram综合征患者的临床表现、家族史及预后。提出做好各种检查配合的健康教育,给予对症处理,如控制血糖、改善多尿、佩戴助听器改善耳聋等,制定个性化的饮食治疗计划,预防酮症酸中毒,并做好并发症(肾衰竭、脑部病变)护理和安全护理是本病护理要点。     

5例Wolfram综合征患者的护理

介绍5例Wolfram综合征患者的临床表现、家族史及预后。提出做好各种检查配合的健康教育,给予对症处理,如控制血糖、改善多尿、佩戴助听器改善耳聋等,制定个性化的饮食治疗计划,预防酮症酸中毒,并做好并发症（肾衰竭、脑部病变）护理和安全护理是本病护理要点。     

Kidney transplantation unraveling Wolfram syndrome: a case report

BACKGROUND: In Wolfram syndrome insulin-dependent diabetes is associated with a multisystem neurodegenerative disorder. There are no prior reports of kidney transplantation in patients with Wolfram syndrome. METHODS: Kidney transplantation was undertaken in a child with dysplastic kidneys, sensorineural hearing impairment and bilateral optic atrophy-a combination of features insufficient to define Wolfram syndrome. RESULTS: After the procedure diabetes mellitus, diabetes insipidus and urinary bladder dysfunction emerged, thereby revealing Wolfram syndrome. CONCLUSIONS: We discuss the etiology of our patient's postoperative events, and conclude that kidney transplantation may expose dormant manifestations-or aggravate existing manifestations-of Wolfram syndrome.     

Urologic aspects of kidney-pancreas transplantation.

The population of pancreas transplant recipients is growing steadily, and urologists most likely will be confronted with their unique anatomy and metabolic complications. The principles of diagnosis and management of these patients can be applied to other transplant recipients (e.g., heart, lung, and liver) who also are maintained on life-long immunosuppression and in whom urologic pathology develops commensurate with the incidence in the general population.     

Urologic complication of laparoscopic appendectomy.

Abscess formation after a ruptured appendix is a well-known phenomenon. Extra-abdominal complications are somewhat rare. Here we present an unusual urologic complication in a case of a perforated appendix and abdominal sepsis.