Cases reported "Hydronephrosis"

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1/34. The egg-shell sign: a possible indicator of raised intrarenal pressure.

    prenatal diagnosis of pelvicalyceal dilatation has produced clinical material that we are continually reinterpreting with the help of improving ultrasound equipment. However, the ability to predict the outcome for any one patient with marked dilatation remains poor. We describe a new sign that may help identify those fetuses who have high intrarenal pressure and therefore justify more aggressive management, while obviating the need for intervention for those in whom it is not present. The egg-shell sign consists of a thin crescent of increased echogenicity over a distended calyx and, in this case, was documented to be associated with other features of raised intrarenal pressure.
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2/34. Silent hydronephrosis/pyonephrosis due to upper urinary tract calculi in spinal cord injury patients.

    STUDY DESIGN: A study of four patients with spinal cord injury (SCI) in whom a diagnosis of hydronephrosis or pyonephrosis was delayed since these patients did not manifest the traditional signs and symptoms. OBJECTIVES: To learn from these cases as to what steps should be taken to prevent any delay in the diagnosis and treatment of hydronephrosis/pyonephrosis in SCI patients. SETTING: Regional spinal injuries Centre, Southport, UK. methods: A retrospective review of cases of hydronephrosis or pyonephrosis due to renal/ ureteric calculus in SCI patients between 1994 and 1999, in whom there was a delay in diagnosis. RESULTS: A T-5 paraplegic patient had two episodes of urinary tract infection (UTI) which were successfully treated with antibiotics. When he developed UTI again, an intravenous urography (IVU) was performed. The IVU revealed a non-visualised kidney and a renal pelvic calculus. In a T-6 paraplegic patient, the classical symptom of flank pain was absent, and the symptoms of sweating and increased spasms were attributed to a syrinx. A routine IVU showed non-visualisation of the left kidney with a stone impacted in the pelviureteric junction. In two tetraplegic patients, an obstructed kidney became infected, and there was a delay in the diagnosis of pyonephrosis. The clinician's attention was focused on a co-existent, serious, infective pathology elsewhere. The primary focus of sepsis was chest infection in one patient and a deep pressure sore in the other. The former patient succumbed to chest infection and autopsy revealed pyonephrosis with an abscess between the left kidney and left hemidiaphragm and xanthogranulomatous inflammation of perinephric fatty tissue. In the latter patient, an abdominal X-ray did not reveal any calculus but computerised axial tomography showed the presence of renal and ureteric calculi. CONCLUSIONS: The symptoms of hydronephrosis may be bizarre and non-specific in SCI patients. The symptoms include feeling unwell, abdominal discomfort, increased spasms, and autonomic dysreflexia. physicians should be aware of the serious import of these symptoms in SCI patients.
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3/34. Laparoscopic repair of acquired lumbar hernia.

    Lumbar hernias are rare; approximately 300 cases have been described in the literature since their first description. They are typically subdivided by categories such as congenital or acquired and by their location. Acquired lumbar hernias may follow trauma, poliomyelitis, loin incision, and the use of iliac crest as a donor site for bone grafting. Although they tend to grow in size and have a 25% risk of incarceration and 8% risk of strangulation, surgery is indicated once the lesion is confirmed. Many techniques have been described for surgical repair of lumbar hernias, including primary repair, local tissue flaps, and conventional mesh repair. All these open techniques require a large incision plus extensive dissection to expose the area. The first laparoscopic repair of lumbar hernia was described in 1996. The laparoscopic approach for lumbar hernia has significant advantages: it enables exact localization of the anatomic defect, the mesh can be placed deep into the defect allowing intraabdominal pressure to hold it in position, and it also has all the well-known advantages of the laparoscopic approach. We present two cases of laparoscopically repaired acquired lumbar hernias.
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4/34. Presence of circulating antibodies against brush border antigens (Fx1A) in a patient with membranous nephropathy and bilateral pyeloureteral stenosis. Comparison with idiopathic membranous nephropathy.

    In a patient with membranous nephropathy and bilateral pyeloureteral stenosis with hydronephrosis, we examined the possibility that an increase in the intratubular pressure could facilitate the passage of the Fx1A antigens to the circulation. Elevated serum anti-Fx1A antibodies were detected in this particular patient by ELISA on three occasions during the disease follow-up, even though he was in clinical remission. These antibodies reacted in vitro with the tubular brush border of a normal human kidney. The anti-Fx1A antibodies isolated from the patient's sera by affinity chromatography competed with the rabbit anti-Fx1A antisera binding to plates coated with human Fx1A antigen. In immunoblotting studies the isolated specific IgG antibodies from that patient reacted with a 180 kDa antigen of the human Fx1A and with less intensity with 75 kDa and 50-55 kDa polypeptides. In none of 12 patients with idiopathic membranous nephropathy could the circulating anti-Fx1A antibodies be demonstrated. On the whole, this particular case suggests that on some occasions increased intratubular pressure could cause the release of Fx1A antigens, facilitating an autologous immunocomplex nephritis. These antigens, by contrast, do not seem to play any role in most cases of membranous nephropathy in man.
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5/34. Presentation of idiopathic retroperitoneal fibrosis in the pediatric population.

    Idiopathic fibrosis of the retroperitoneum is rare in childhood. The authors describe an 11-year-old boy who presented with progressive renal failure, bilateral hydronephrosis, hypertension, and elevated erythrocyte sedimentation rate (ESR) owing to retroperitoneal fibrosis. Ureterolysis was performed with improvement in his creatinine level and blood pressure. The soft tissue mass consisted of dense collagenous fibers consistent with retroperitoneal fibrosis. Postoperatively, he received steroids and azathioprine. retroperitoneal fibrosis in the pediatric population is rare with only 23 cases reported in the English-language literature. Treatment includes pulsed steroid regimens, ureteral catheterization, and retroperitoneal exploration with ureterolysis. If allowed to progress, renal failure can result and lead to death. The etiology of retroperitoneal fibrosis in the pediatric patient may include autoimmune diseases, infection, and neoplasm, but most cases are idiopathic. retroperitoneal fibrosis should be considered in patients with an elevated ESR, hypertension, renal failure, and hydronephrosis. Evaluation also should include a search for autoimmune diseases and malignancy.
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6/34. A case of a novel mutant vasopressin receptor-dependent nephrogenic diabetes insipidus with bilateral non-obstructive hydronephrosis in a middle aged man: differentiation from aquaporin-dependent nephrogenic diabetes insipidus by response of factor VII and von willebrand factor to 1-diamino-8-arginine vasopressin administration.

    We describe a case of a novel mutant vasopressin 2 receptor (V2R)-dependent nephrogenic diabetes insipidus (NDI) with bilateral non-obstructive hydronephrosis in a middle aged man. This could be distinguished from aquaporin 2 (AQP2)-dependent NDI by the response of factor viii and von willebrand factor (vWF) to 1-deamino-8-D-arginine vasopressin (DDAVP) administration. A 47-year-old man was admitted to hospital because of polyuria, which had been present from infancy and was suspected of causing non-obstructive hydronephrosis. His mother's father, the older brother of his mother and his second daughter also all had polyuria. sodium concentration, osmolality and vasopressin in blood were high, while sodium concentration and osmolality in urine were low. There were no changes in urine osmolality, factor viii and vWF in response to DDAVP infusion. Neither was heart rate, diastolic blood pressure nor facial flushing affected. These findings suggested this case was V2R-dependent NDI rather than AQP2-dependent NDI. Molecular genetic analysis demonstrated that the patient had a V2R missense mutation involving a substitution of cysteine for arginine at position 104 (R104C) located in the first extracellular loop of the V2R. It was also found that the patient's mother and his second daughter were heterozygous for this R104C mutation.
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7/34. Malignant hypertension in a child with solitary functioning hydronephrotic kidney.

    A child with solitary functioning hydronephrotic kidney and hypertensive encephalopathy is described. Removal of the contralateral dysplastic kidney failed to normalize his blood pressure.
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8/34. Management of gestational nephrolithiasis in the presence of a bicornuate uterus and pelvic kidney.

    A 39-year-old para 0( 1) woman with known nephrolithiasis within a left-sided pelvic kidney presented with left-sided renal colic at 7 weeks gestation. She had a previous miscarriage due to a bicornuate uterus. Ultrasound and magnetic resonance urography confirmed an incomplete obstruction of the left upper renal tract which was relieved by percutaneous nephrostomy. She presented again at 14 weeks with renal colic and minimal output. An ultrasound confirmed recurrent hydronephrosis and a nephrostogram showed that the catheter had retracted almost completely from the collecting system. This was considered to be due to the upward pressure of the enlarging uterus on the catheter, which had been fixed externally to the skin. This problem was obviated by not securing the replacement nephrostomy tube to the skin. She developed pre-eclamptic toxaemia and gave birth at 35 weeks gestation by caesarean section. The calculus was later dissolved using extra-corporeal shockwave lithotripsy.
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9/34. Bladder rupture in premature infant during voiding cystourethrography.

    We report what we believe to be the youngest patient with bladder rupture during voiding cystourethrography. If voiding cystourethrography needs to be done in a premature infant, the filling pressures should be minimized, multiple filling cycles should be avoided, and the voiding pressures should be minimized by using a small, balloon-less catheter.
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10/34. Unilateral hydroureteronephrosis caused by abdominoscrotal hydrocele.

    Abdominoscrotal hydrocele is extremely rare in children. A case is presented of an unusually large abdominoscrotal hydrocele in a child, with secondary right hydroureteronephrosis due to contiguous pressure and right cryptorchidism. This entity, although unusual, should be considered in the differential diagnosis of a lower abdominal mass in children, as well as a cause of hydroureteronephrosis. Diagnosis is made with ultrasound, computerized tomography and occasionally excretory urography. Complete surgical excision through a groin incision is recommended, but optional inguinoabdominal or abdominal incisions have been reported. After surgical removal, healing is usually complete, including regression of the hydroureteronephrosis.
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