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Query: UMLS:C0016199 (flank pain)
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Nutcracker syndrome results from left renal vein compression by the abdominal aorta and the superior mesenteric artery. The consecutively increased renal venous pressure results in hematuria, proteinuria, flank pain, left-sided varicocele, pelvic congestion, and others. We report a 25-year-old man with nutcracker syndrome who underwent successful left renocaval venous bypass with autologous great saphenous vein. The patient's condition clearly improved, with no clinical relapse after treatment. Ultrasound imaging showed patency of the venous bypass and decreased venous hypertension. This technique is a feasible choice for surgical treatment of nutcracker syndrome, with a low incidence of complications and satisfactory results.
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PMID:Left renocaval venous bypass with autologous great saphenous vein for nutcracker syndrome. 2205 77

Kidney cancers account for 2-3% of all adult malignancies in the UK. Men are predominantly affected by renal cancer with an average age at diagnosis of 64 years. Renal (or clear) cell carcinoma (RCC) accounts for 90% of kidney cancers. Early diagnosis improves survival with five-year survival rates for renal cancer of 70-94% for localised tumours in the UK. RCC should be suspected in the presence of localising symptoms such as flank pain, a loin mass or haematuria; constitutional upset including weight loss, pyrexia and/or night sweats; or with unexplained laboratory tests. Smoking, obesity and hypertension are the most important and most common risk factors. Environmental exposure to asbestos, cadmium and trichloroethylene are less common risk factors. Patients on chronic dialysis and renal transplant recipients are at increased risk of RCC in their native kidneys. If kidney cancer is suspected on history, physical examination or initial screening tests then a red flag ultrasound examination of the renal tracts should be requested. Dipstick urinalysis is of great value as asymptomatic haematuria may be the only abnormal test in the presence of non-specific symptoms such as weight loss or loin pain. Visible or non-visible haematuria, in the absence of proteinuria, suggests an underlying structural abnormality is present in the kidneys, ureters or bladder. Surgical removal of RCCs, where feasible, may result in cure in up to 40-60% of cases. Individuals too frail for major surgery may benefit from thermal ablation and cryotherapy. Agents that target the VEGF and mTOR pathways are considered first line in the treatment of metastatic RCC. Sunitinib, recommended by NICE, is administered orally and acts by inhibiting the VEGF receptor.
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PMID:Early diagnosis improves survival in kidney cancer. 2249 3

Polycystic kidney disease and Alport's syndrome are the most common causes of inherited renal disease in the UK. An average GP practice is likely to have at least six patients with autosomal dominant polycystic kidney disease (ADPKD). The disorder is characterised by the formation of fluid-filled cysts in the kidneys resulting in progressive renal impairment. Mutations in two genes have been identified. The PKD1 gene abnormality is responsible for 85% of cases of ADPKD, patients with PKD2 mutations typically present later and progress more slowly. Patients with ADPKD can present with a positive family history, hypertension, flank pain, haematuria, renal insufficiency or proteinuria. The diagnosis has traditionally been based on ultrasound imaging. Screening will reduce the incidence of a late diagnosis when renal disease is advanced but a normal ultrasound scan in those under 30 years old is not conclusive. It is not recommended that children are screened. The key to minimising the rate of progressive disease is tight BP control. ACE inhibitors are recommended as the initial antihypertensive agent unless contraindicated. Alport's syndrome is a disorder characterised by abnormal type IV collagen which is found in the kidney, eyes, skin and ears. Around one in ten practices are likely to have a patient with Alport's syndrome. Eighty per cent of patients have the X-linked form of the disease. All first-degree relatives of a patient with confirmed Alport's syndrome should be offered screening. The combination of reduced hearing and urinary abnormalities in a young boy should alert GPs to consider this as a possible diagnosis and initiate referral. Diagnosis can be confirmed by renal or skin biopsy.
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PMID:Improving recognition of inherited renal disease. 2249 4

Wagener's granulomatosis (WG) is a rheumatologic disease with unknown etiology which renal and pulmonary involvement is commonly seen. Renal involvement in Wagener's granulomatosis represents as a segmental necrotizing glomerulonephritis which is not visible with imaging techniques and usually presents with proteinuria, microhematuria, and hypertension. A rare presentation of the disease is a renal mass which can be mistaken as renal tumors, abscess, or lymphoma. We report a 22-year-old female with flank pain and fever who was admitted in our hospital. The patient underwent renal tumor biopsy and diagnosed with Wagener's granulomatosis in pathologic staining. The aim of this work is introduction of Wagener's granulomatosis as a differential diagnosis of renal tumors, to prevent unnecessary interventions and delayed treatment.
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PMID:Renal Solid Mass as a Rare Presentation of Wagener's Granulomatosis: A Case Report. 2291 35

The clinical manifestations of hyperuricosuria (HU) are usually underestimated by the clinician. The aim of this study was to review the clinical spectrum of symptomatology of HU and to evaluate the presence of associated hypercalciuria (HC) and hyperoxaluria (HX). A retrospective review was done on 64 children with HU seen between January 2004 and December 2008. The patients were divided into HU 19, HU + HC 4, HU + HX 21 and HU + HC + HX 20. The mean age at diagnosis was 80 months (range six to 156 months). Duration of follow-up ranged was from six to 66 months. There were 228 symptomatic episodes for 64 patients (males 31, females 33). The relationship of symptomatology to age and gender were not significant. The most common symptoms were abdominal pain 67.2% (in 7/44 it was localized to the right lower quadrant, mimicking appendicitis), flank pain 59.4%, increased urinary frequency 43.4%, urgency 39%, enuresis 31.25%, oliguria 29.7%, dysuria 25%, red urine 20.35%, vaginal itching 15.21%, dribbling 14.06%, orange urine 12.5%, hesitancy 12.5% and penile pain 7.81%. To our knowledge, the vaginal itching and penile pain were not previously described. Family history was positive for stones and/or gout in 62.5%. The presence of a positive family history and red urine were significant (P-value <0.05) for the presence of an underlying HU. In the presence of recurrent abdominal/flank pain, hematuria without proteinuria or edema and urological symptomatology, especially in the presence of red urine, and a positive family history of gout or stones, a search for HU is in order. This will avoid unnecessary and invasive investigations.
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PMID:The clinical spectrum of idiopathic hyperuricosuria in children: isolated and associated with hypercalciuria/hyperoxaluria. 2298 10

A young 23-year-old male patient presented with a two-day history of right flank pain. He had no history of any significant illnesses in the past. His investigations showed nephrotic range proteinuria with hypoalbuminemia. The patient developed cough and shortness of breath after having a left kidney biopsy. He did not respond to regular respiratory tract infection treatment. The kidney biopsy revealed membranoproliferative glomerulonephritis. Further investigations for the cough showed thromboembolism of the posterior and lateral basal segments of the right lower lobe. Moreover he was found to have thrombosis of the right upper pole renal vein. The patient was started on full anticoagulation along with three days pulse steroid, followed by 1 mg/kg oral steroid. Clinical improvement was noticed within 48 h. After eight weeks the proteinuria decreased from 8.5 gm/day to 1.1 gm/day. The kidney function was normal with eGFR 145 mL/min through the course of the disease. This case represent one of the unusual presentation of nephrotic syndrome with pulmonary and renal vascular thromboembolic events. The response to the combination of anticoagulation and steroid was remarkable.
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PMID:Unusual presentation of renal vein thrombosis with pulmonary artery embolism. 2364 Jun 33

Nutcracker syndrome (NCS), which is caused by compression of the left renal vein between the abdominal aorta and the superior mesenteric artery, leads to a series of clinical symptoms including hematuria, proteinuria, flank pain, and varicocele. The diagnosis of NCS is difficult due to variations in normal anatomy. Treatment, which ranges from observation to nephrectomy, remains controversial. We conducted a review based on the related literature and our experience with hundreds of cases. We summarize the characteristics of NCS, the different measurements used in diagnosis, and the current treatment options. We present our diagnostic criteria and recommend endovascular stenting as the primary option for NCS.
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PMID:Nutcracker syndrome--how well do we know it? 2413 44

A 26-year-old woman presented to the emergency department complaining of left flank pain, and proteinuria and hematuria were detected during urinalysis. A renal ultrasound did not reveal any disorder, and after performing a computed tomography angiography scan, compression of the left renal vein between the superior mesenteric artery and the aorta was seen. This compression is known as Nutcracker syndrome. From among the different treatment options available, it was decided, with patient consensus, to use open surgical management, performing a transposition of the left renal vein to a more distal level in the inferior vena cava. The immediate postoperative care progressed without complications and the symptoms resolved; after 1 year of surveillance, the patient continues to be asymptomatic. Nutcracker syndrome is a rare phenomenon, with few cases described. There are different therapeutic options for the treatment of Nutcracker syndrome, such as open surgery, endovascular treatment, or conservative treatment; because of the low prevalence of this syndrome, there are no sufficiently large series at present or with the necessary long-term surveillance to decide on the most suitable treatment. Distal transposition of the left renal vein in the inferior cava vein has proved to offer good long-term results, and this option offers a higher chance of resolution without the need for as many postsurgery controls as would be required with endovascular treatment.
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PMID:Recurrent hematuria caused by nutcracker syndrome. 2432 Dec 67

Nutcracker syndrome (NCS) is a rare pathology, caused by compression of the left renal vein (LRV) between the abdominal aorta (AA) and the superior mesenteric artery (SMA), due to reduction of the angle between AA and SMA. This leads to LRV varices, left gonadal vein varices and therefore, the pelvic congestion syndrome. For this reason, coexistence of NCS and pelvic congestion syndrome has been described. It manifests by hematuria, proteinuria, and nonspecific pelvic pain secondary to pelvic congestion, dyspareunia and persistent genital arousal. We report a 27-year-old woman who experienced hematuria and left flank pain. The diagnosis of NCS accompanied by pelvic congestion syndrome was missed initially, but later on the diagnosis was made by color Doppler ultrasound, abdominal computed tomography (CT) and CT angiography that were later performed. She refused interventional and surgical treatments, and was lost to follow up.
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PMID:Nutcracker Syndrome Accompanying Pelvic Congestion Syndrome; Color Doppler Sonography and Multislice CT Findings: A Case Report. 2503 94

Genetic disorders of the kidney include cystic diseases, metabolic diseases and immune glomerulonephritis. Cystic diseases include autosomal dominant and recessive polycystic kidney disease (ADPKD, ARPKD, respectively). Neonates with enlarged, cystic kidneys should be evaluated for PKD. Patients with ADPKD have cysts and renal enlargement. Most patients present with hypertension, hematuria or flank pain; the most common extrarenal manifestation is polycystic liver disease. Oligohydramnios, bilaterally enlarged kidneys and decreased urine are featured in utero in ARPKD. Medullary sponge kidney is uncommon and features nephrocalcinosis, recurrent calcium stones and a history of polyuria/nocturia and/or urinary tract infections. Alport syndrome (AS) is an inherited disease of the glomerular basement membrane that is usually inherited as an X-linked dominant trait. Most patients with AS present in the first two decades of life with persistent microscopic or gross hematuria. Later, proteinuria is seen and its presence portends disease progression. Other findings may include sensorineural hearing loss and ocular abnormalities. There are various inherited tubulopathies, including Bartter syndrome, a group of renal tubular disorders that consist of two phenotypes with four genotypes. Patients usually present early in life with salt wasting, hypokalemia and metabolic alkalosis. Other features, depending on genotype, may include polyhydramnios and premature birth. Gitelman syndrome is also a salt-losing tubulopathy characterized by hypokalemic alkalosis. The majority of patients with Gitelman syndrome present during adolescence or early adulthood.
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PMID:Inherited renal diseases. 2508 62


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