Ask-Upmark kidney and tubulointerstitial nephritis in a woman with severe renal failure

Ask-Upmark kidney is a rare diagnosis of segmental hypoplasia in pediatric population clinically characterized by severe hypertension potentially treatable with partial to total nephrectomy. Although originally was described only as a congenital anomaly, recent data suggest to be caused by vesicoureteral reflux, either in utero or in early childhood and pyelonephritis. The case we reported indicates that Ask-Upmark kidney should be considered as potential cause of hypertension and renal failure both in children and adults. The renal biopsy is necessary for early diagnosis and may consent to normalize blood pressure with nephrectomy; however, if renal damage is severe and progressive with tubulointerstitial nephritis, surgical management is excluded and renal transplant should be considered.     

Ask-Upmark kidney with contralateral renal artery fibromuscular dysplasia

A case of Ask-Upmark kidney with coexistent contralateral renal artery fibromuscular dysplasia is presented with detailed light and electron microscopic findings. Both lesions are known to produce renin-mediated hypertension which was corrected in this patient by nephrectomy and contralateral renal artery bypass. Ask-Upmark kidney is briefly reviewed.     

Ask-Upmark kidney with bilateral pelvi-ureteric junction obstruction – A rare entity

Renal segmental hypoplasia (Ask-Upmark kidney) is a congenital disorder, first described by Eric Ask-Upmark in 1929. Habib et al. called it “segmental hypoplasia of the kidney in 1965. Ask Upmark kidney is more in females and present with hypertension or sometimes as recurrent urinary tract infections. Usually unilateral, bilaterally asymmetrical segmental hypoplasia has also been reported. The pathogenesis of Ask-Upmark kidney is controversial, attributing to vesicoureteral reflux (VUR) with intrarenal reflux or possibility of localized developmental arrest. We report a case of two years’ male child presenting as abdominal swelling with respiratory distress. On evaluation he was found to have bilateral pelvi-ureteric junction obstruction and left non-functioning kidney for which he underwent right pyeloplasty and left nephrectomy later on. Histopathology report of left kidney suggestive of segmental renal hypoplasia (Ask-Upmark kidney).     

Severe hypertension in infant with unilateral hypoplastic kidney

Unilateral renal hypoplasia unlike Ask-Upmark kidney (segmental renal hypoplasia) seldom causes hypertension. A case of an infant whose hypertension disappeared after removal of a hypoplastic kidney is reported. The removed kidney revealed no sign of Ask-Upmark lesion.     

Hypertension due to Ask-Upmark kidney

A young girl in whom severe hypertension was cured by the surgical removal of a unilateral hypoplastic kidney (Ask-Upmark kidney) is described. The clinicopathological features of the Ask-Upmark kidney are described with particular reference to the aetiology of the hypertension as well as the selection of patients for surgery. No abnormality of the renin-angiotensin system was detected.     

The association of hypertension, the Ask-Upmark kidney and other congenital abnormalities

We report 2 cases of hypertension with segmental renal hypoplasia (Ask-Upmark kidney) and other anomalies in the absence of vesicoureteral reflux. These cases support the view that the Ask-Upmark kidney is a defect of renal development rather than acquired as a consequence of vesicoureteral reflux. In 1 patient the abnormal renal vein renin ratio suggested that the renin-angiotensin system may have had a part in the pathogenesis of the hypertension.     

Living-Donor Kidney Transplantation With Laparoscopic Nephrectomy From a Donor With Horseshoe Kidney: A Case Report

BackgroundIn living-donor kidney transplantation, laparoscopic nephrectomy from a donor has become widespread. However, more careful treatment is required for nephrectomy from a donor with horseshoe kidney. This report presents an interesting surgical case of laparoscopic nephrectomy from a donor with horseshoe kidney.Case presentationA woman aged 53 years was a donor candidate for living-donor kidney transplantation for her husband. She had no medical history and had no problems on preoperative examination, but contrast-enhanced computed tomography revealed that she had horseshoe kidney. As the isthmus was thin and the contrast effect was poor, the isthmus was considered to have poor kidney parenchyma and consisted almost exclusively of fibrous tissue. Therefore, laparoscopic nephrectomy was performed for the donor. On the basis of the 99m Tc-dimercaptosuccinic acid renal scintigraphy results, the right kidney was collected. A laparoscopic nephrectomy with a retroperitoneal approach was performed using GelPort access platforms in a right abdominal incision with an accessory port. We firmly expanded the isthmus and then dissected it just above the aorta using a linear stapling device. Subsequently, we sutured a renal artery and vein with linear stapling devices. The recipient's surgery was also performed without any problems, and the postoperative course of both donor and recipient was good.ConclusionsWe suggest that even if the donor has horseshoe kidney, laparoscopic donor nephrectomy should be actively considered depending on the thickness of the isthmus of the horseshoe kidney.     

Posterior nutcracker syndrome with left renal vein duplication: An uncommon cause of hematuria

INTRODUCTIONPosterior Nutcracker syndrome (NCS) is a rare anomaly in which the left renal vein passes behind the aorta which compresses it against the vertebral column, restricting the venous drainage of the left kidney.PRESENTATION OF CASEA 46 year-old lady presented with intermittent painless hematuria for 6 years. Urinalysis showed microscopic hematuria. An abdominal CT scan showed left renal vein duplication with the retroaortic branch trapped between the vertebral column and the aorta at the level of the aortic bifurcation, suggestive of posterior NCS. There were multiple small cortical cysts, sand-like stones in the left kidney and duplication of both right and left renal arteries.DISCUSSIONPosterior NCS in a patient with a duplicated left renal vein may not show all the clinical features of a typical NCS as the elevated pressure due to compression is dissipated through the pre-aortic branch of the duplicated renal vein. CT Angiography can be helpful in such a patient with multiple abnormalities. Management can range from simple surveillance to nephrectomy depending on the symptoms and renocaval pressure gradient.CONCLUSIONAlthough posterior NCS is a rare anomaly of the left renal vein, it should be considered in the differential diagnosis of haematuria.     

The role of partial nephrectomy in the treatment of pediatric renal hypertension

PURPOSE: From 5% to 25% of hypertension in children is renovascular compared with only 1% in adults. Although much attention is given to renovascular disease involving the main renal arteries, renin producing renal disease may also be intrarenal, involving abnormalities of the segmental vessels or renal parenchyma. We present our results of partial nephrectomy in this unique group of pediatric patients with hypertension in whom renin dependent hypertension involved only a segment of the kidney. MATERIALS AND METHODS: Six patients 10 months to 16 years old were referred for the evaluation of hypertension. Initial evaluations included computerized tomography, ultrasound, voiding cystourethrography and radionuclide renal scan. Renal arteriography with renal vein renin sampling was performed in 5 patients, of whom 4 underwent selective segmental renal vein sampling. Diagnoses included segmental hypoplasia (Ask-Upmark kidney), reflux associated scarring and renal arteriovenous malformation. RESULTS: In patients who underwent selective segmental renal vein sampling an increased renin level was present in the area of the renal lesion (mean 24.9 ng./ml. per hour, range 9.2 to 40.6) compared to the ipsilateral renal vein (15.6, 114 to 29.8). Three patients had evidence of contralateral suppression of renin secretion. All 6 patients underwent upper, lower or mid segment partial nephrectomy. All patients became immediately normotensive and remained so at a mean 10 years of followup. CONCLUSIONS: Partial nephrectomy provides an excellent nephron sparing cure for segmental renal hypertension. In the pediatric population selective segmental renal vein renin sampling is invaluable for locating the renin producing lesion. As in adults, contralateral renin suppression is predictive of surgical cure.     

A case report of non-functional ectopic left kidney obstructing the right kidney in crossed fused kidneys: A rare entity

Introduction and importanceCrossed fused renal ectopia is a rare congenital condition that might pose some diagnostic and therapeutic challenges to clinicians. We report a patient with a non-functional crossed fused ectopic left kidney that obstructed the orthotopic kidney in a rarely observed situation.Case presentationA 68-year-old male presented a right flank pain with fever. The diagnosis of right obstructive pyelonephritis was dressed, after biological and radiological investigations. The obstacle was a crossed ectopic left kidney in its inferior variety. The ectopic kidney was non-functional as result of an obstructive ureteral calculus. The patient had right ureteral stenting with a double-J catheter. Three months later, left nephrectomy was performed by lumbotomy. Per operative difficulties were mainly the infiltration of peri renal fat, the anarchic vascularization and the multiple small pedicles of the ectopic kidney that was also malrotated with the hilum facing anteriorly. Postoperative recovery was uneventful and the patient left the hospital after three days.Clinical discussionCrossed Fused renal ectopy is rare. As shown in this case, the ectopic kidney might cause damage to the orthotopic kidney, by compression to urinary ducts. Surgery is the main treatment option. Some difficulties related to aberrant vascularization and possible malrotation is to preview.ConclusionCrossed fused renal ectopia is uncommon renal anomaly, mostly asymptomatic. However, it may be responsible of some complications, sometimes severe. Surgery can be delicate due to vascular complexity.     

A case report of post-surgical page kidney due to extensive renal hematoma following percutaneous nephrolithotomy

Introduction and importancePost-surgical Page kidney due to large renal hematoma following percutaneous nephrolithotomy (PCNL) is a rare significant complication that may lead to loss of a kidney.Case presentationA 50-year-old lady underwent elective left side PCNL for a 3 cm renal pelvis stone, and one week later, she presented back with a massive renal hematoma with high blood pressure.Clinical discussionThe ultrasound abdomen and computed tomography diagnosed a page kidney due to massive intrarenal and perirenal hematoma as a complication of PCNL. Angioembolization and percutaneous aspiration were failed, and the antihypertensives also failed to control the blood pressure. Therefore, she underwent a left-side simple nephrectomy and had an uneventful recovery with reversal of normal blood pressure.ConclusionPost-surgical page kidney needs to identify early to facilitate the percutaneous radiological interventions that may preserve the renal parenchyma and avoid further surgeries. Nevertheless, late cases or the failed radiologically intervened cases need open renal exploration and simple nephrectomy, which may be the bailed-out procedure to reverse the consequence of page kidney.     

Trasplante renal y poliquistosis: consideraciones quirúrgicas

BackgroundThe indication and timing of nephrectomy in patients with autosomal dominant polycystic kidney disease (ADPKD) remain controversial, especially in patients who are candidates to renal transplantation (RT). The main surgical options such as unilateral vs. bilateral nephrectomy, nephrectomy before vs. after RT, or simultaneous nephrectomy and transplantation, are herein discussed.ObjectiveEvidence acquisition of the best surgical management available for ADPKD in the context of kidney transplantation.Acquisition of evidenceSystematic literature review in PubMed from 1978 to 2013 was conducted. Articles selected included:randomized controlled trials and cohort studies. Furthermore, well designed ADPKD reviews were considered for this study.Synthesis of evidenceLaparoscopic nephrectomy in ADPKD is a safe procedure with an acceptable complication rate. Unilateral nephrectomy has advantages over the bilateral one regarding theperioperative complication rate. Although the timing of nephrectomy is controversial, it seems that simultaneous nephrectomy and renal transplantation does not increase surgical morbidity neither affect graft survival.ConclusionsSimultaneous nephrectomy and RT appears to be an acceptable alternative to conventional two-stage procedure without any increased morbidity, in the context of ADPKD. Furthermore, laparoscopic nephrectomy performed in experienced centres is a safe alternative to conventional approach.     

Autotransplantation for renovascular hypertension with complete renal artery occlusion.

We report a personal series of 13 autotransplantation procedures in 12 patients who suffered from severe renovascular hypertension. In all of these cases preoperative investigation demonstrated a viable kidney, despite complete occlusion of the affected renal artery. One-half the patients had stenosis of a milder degree affecting the contralateral kidney. In eight of these patients the operation was considered successful. One patient died due to postoperative superior mesenteric artery thrombosis and two had infarction of the transplanted kidney. A fourth patient lost the autotransplant because of postoperative haemorrhage. It is suggested that if medical treatment fails then autotransplantation should be considered in place of nephrectomy for cases of renovascular hypertension with complete occlusion of the renal artery.     

Recurrent Renal Allograft Torsion After Simultaneous Kidney and Pancreas Transplantation: Is it Still Possible to Salvage the Graft? A Case Report

BackgroundKidney allograft torsion (KAT) is defined as a rotation of the renal allograft around its vascular pedicle. It is a rare complication with high rate of graft loss. The nonspecific presentation and inability to provide a definitive diagnosis by imaging, mainly in cases of partial torsion, often delay the diagnosis and treatment. We report a case of recurrent complete torsion of the renal allograft after simultaneous kidney and pancreas transplantation, requiring 2 emergency exploratory laparotomies.Case reportA 38-year-old woman with a history of intraperitoneal simultaneous kidney and pancreas transplantation underwent 2 separate emergency exploratory laparotomies secondary to complete renal allograft torsion, respectively, 7 and 11 months after the transplant. In both episodes, no adhesions were encountered. During the first operation, nephropexy was performed. During the second operation, an abdominal wall mesh was placed and fixed to the abdominal wall. Acute kidney injury related to KAT recovered in both occasions with a creatinine of 1.3 mg/dL at 4 months follow-up.ConclusionsRenal torsion always should be suspected in intraperitoneally placed kidneys presenting with nonspecific symptoms, abdominal pain, oliguria, and worsening kidney function. Surgical exploration should be considered to salvage the renal graft. This case illustrates the reversibility of a severe injury related to this vascular complication with an adequate return to baseline kidney function even when diagnosis and surgical treatment of KAT might be delayed secondary to its misleading clinical presentation.     

Compensatory Changes in the Retained Kidney After Nephrectomy in a Living Related Donor

ObjectiveHerein, we investigate the compensatory changes in morphology, function, and hemodynamic indices of the retained kidney after nephrectomy among living related donors.MethodsThe 136 living related kidney donors underwent assessments before surgery as well as at 1 and 12 months, postoperatively, including length, width, short diameter, glomerular filtration rate (GFR), effective renal plasma flow (ERPF), peak systolic blood flow velocity (Vsmax), resistance index (RI), as well as pulsatility indices (PI) of main renal artery (MRA), segmental renal artery (SRA), and interlobar renal artery (IRA).ResultsAll subjects were followed up for 12 to 62 months, with no observed hypertension or kidney failure. The length, width, and short diameters of the retained kidney increased significantly (P < .01) at 1 and 12 months postoperatively. The renal sizes were similar (P > .05). GFR and ERPF increased significantly compared with preoperative values (P < .01) with similar values at postoperative month 1 and 12 (P > .05). The Vsmax of MRA, SRA, and IRA in the retained kidney increased significantly (P < .01); the RI and PI also increased compared with the preoperative values (P < .05), albeit these indicators were similar at postoperative months 1 and 12 (P > .05).ConclusionsFor all subjects studied after unilateral nephrectomy in a living related donor, the diameter of the retained kidney as well as the GFR and ERPF showed compensatory increases. Various arterial hemodynamic parameters also showed compensatory changes. Under strict donor inclusion criteria, living related kidney donor procedures should be safe.     

Bilateral native nephrectomy for refractory hypertension in kidney transplant and kidney pancreas transplant patients

Hypertension is common in renal transplant patients and sometimes very difficult to control. Refractory hypertension can adversely affect renal graft and patient survival. Many antihypertensive medications are not well tolerated or can have important drug interactions with immunosuppressive medications. These drugs can cause significant side effects including fluid depletion, azotemia, electrolyte imbalance, and anemia. Bilateral native nephrectomy in renal transplant patients has been reported to be beneficial in controlling severe hypertension.We report five patients with severe hypertension despite as many as 9 different antihypertensive medications. All patients had previous kidney or simultaneous kidney pancreas transplantation. Each of our patients underwent laparoscopic bilateral native nephrectomy.Renal function varied from creatinine of 1.4–2.4, and the number of antihypertensive medications from 3 to 9 at the time of nephrectomy surgery. Mean arterial blood pressure improved in all five patients at 3–6 months post nephrectomy, the number of antihypertensive medications decreased in 4, but renal function remained stable at 3–6 months in only 3 patients.We found laparoscopic bilateral native nephrectomy to be beneficial in renal and simultaneous kidney pancreas transplant patients with severe and refractory hypertension. Our patients with better baseline renal allograft function at time of nephrectomy received the most benefit. No decrease in allograft function could be attributed to acute rejection.     

Xanthogranulomatous pyelonephritis in an eight year old male child: A case report and review of the literature

Introduction and importanceXanthogranulomatous pyelonephritis is an extremely rare but known form of chronic pyelonephritis resulting from prolonged suppuration of the kidney. Pre-operatively, it may mimic renal tuberculosis or neoplastic lesions including renal cell carcinoma due to its vague clinical presentation, equivocal laboratory and radiological investigations. Due to its rarity and academic interest, herein we report such a rare case we recently encountered in our clinical practice.Case presentationAn-eight-year old male child patient resented to our hospital with three months history of abdominal distension associated with progressive left flank pain. Preoperative investigations including CT-scan were suggestive of nephroblastoma with differential diagnosis of clear cell sarcoma. Radical nephrectomy was performed and histopathology of the specimen confirmed the diagnosis of Xanthogranulomatous pyelonephritis. The patient fared well postoperatively and he had no symptoms in the subsequent follow up visits.Clinical discussionXanthogranulomatous pyelonephritis is a rare, severe and atypical form of chronic pyelonephritis due to infection (E. coli, Proteus) or stones. The disease may resemble renal cell carcinoma preoperatively. Thus, high index of suspicion is necessary for preoperative diagnosis.ConclusionPreoperative diagnosis of Xanthogranulomatous pyelonephritis may be a daunting task related to the rarity of its presentation. Thus, scrupulous histopathological evaluation is essential for the definitive diagnosis. Radical nephrectomy is the mainstay treatment of choice especially in diffuse cases.     

闭合性肾损伤的诊断与治疗

目的 总结闭合性肾损伤的诊治体会，提高闭合性肾损伤的诊治水平，减少肾切除率。方法 189例闭合性肾损伤患者，男146例，女43例。平均年龄38岁。综合应用尿常规、B超、IVU、CT等检查，诊断闭合性肾损伤，其中合并腹腔内脏器损伤27例，合并颅脑外伤5例，合并四肢脊柱骨折13例，3例为腹腔、颅脑与腹腔、脊柱复合伤伴休克。行保守治疗156例，其中有脾破裂3例，颅脑外伤2例，四肢脊柱骨折7例；行手术治疗27例，其中行肾修补术15例，肾部分切除术5例，肾切除7例；3例行肾动脉栓塞治疗。结果3例重度闭合性肾损伤因复合伤、顽固性休克者入院不久死亡，186例痊愈出院。102例随访3个月～8年，保守治疗病例中发生肾性高血压3例，肾周尿囊肿1例，肾萎缩1例，局限性肾积水1例，手术治疗与肾动脉栓塞治疗者均无并发症。结论 CT检查是闭合性肾损伤准确诊断与选择治疗方案的重要依据，Ⅲ级以上闭合性肾损伤伤后2～4周复查CT有利于肾周尿囊肿的早期发现与治疗，Ⅳ级以下闭合性肾损伤尽可能采用非手术治疗。     

Renal artery revascularisation can restore kidney function with absent radiotracer uptake

Background

Children with renovascular hypertension often present with severe hypertension. Some children have severe obstruction of their renal arteries resulting in <10% relative function on [^99mTc]dimercaptosuccinic acid (DMSA) scan. Conventional treatment of these children has been nephrectomy of the poorly functioning kidney to normalise their blood pressure (BP).

Case-Diagnosis/treatment

We describe three children aged 20 months to 9 years with severe renal artery stenosis and severe hypertension who had radionucleotide uptake of 0% in one kidney. In one case, no renal perfusion was demonstrated by duplex ultrasound scan. Significant recovery of relative renal function of 18 to 52% was achieved after revascularisation by percutaneous angioplasty or open surgery of the obstructed renal artery.

Conclusion

These cases illustrate that scintigraphy alone cannot be used to predict salvageable function in children with renovascular disease.     

Juxtaglomerular cell tumor of the kidney—a new classification scheme

ObjectiveTo introduce a new classification scheme of juxtaglomerular cell tumor (JCT) of the kidney for differential diagnosis of hypertension and renal cell carcinoma.MethodsFive cases of JCT have been diagnosed and treated surgically in our hospital during the last 4 years. Through a search in PubMed, we incorporated 7 large series of case reports of JCT into a review of 71 cases previously published in the literature. Clinical presentations (blood pressure), laboratory examinations [serum potassium, plasma renin activity (PRA), aldosterone (ALD), and renal venous sampling for renin assay], and imaging examinations [ultrasonography, computerized tomography (CT), excretory urography, and selective renal angiography] were summarized.ResultsThe 71 cases of JCTs can be classified into 3 types, which are typical type, atypical type, and non-functioning type. The 57 typical cases had the typical characteristics of hypertension, hypokalemia, hyperaldosteronism, and high renin. The 12 atypical cases had hypertension with normal potassium levels, and the 2 non-functioning cases had normal blood pressure and normal potassium levels.ConclusionsThe classification of typical, atypical, and non-functioning JCTs depends on blood pressure and serum potassium. JCT of the kidney should be considered in patients with hypertension and renal tumor, and nephron-sparing surgery is the first choice.