Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0016199 (flank pain)
 2,189 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Extramedullary plasmacytoma (EMP) is a very rare disease and mainly arises in the head and neck area. We herein reported a case of EMP arising in the retroperitoneal space. A 46-year-old man was referred to our outpatient clinic in November 1989 with the complaint of flank pain on the left side. Radiological examinations showed a tumor formation in the retroperitoneal space, which involved the left kidney, spleen and pancreas. Immunoelectrophoresis showed an elevation of serum IgG level and a spike of M-protein was detected in the serum protein electrophoresis. No bone lesions were detected, and bone marrow aspiration showed no abnormal cells. US-guided needle biopsy of the tumor led to the histological diagnosis as plasmacytoma of the IgG-kappa type. Following three cycles of preoperative chemotherapy (a THP-COP regimen), which resulted in a size reduction of the tumor by 40%, extensive resection of the tumor including extirpation of the left kidney, spleen, and tail of pancreas was performed. Because of tumor extension into the posterior wall of the stomach, however, the surgery resulted in incomplete resection. A total of 11 cycles of postoperative chemotherapy (THP-COP) was performed periodically for the residual tumor in the stomach. Rapid tumor spreading in addition to re-elevation of the serum IgG level, however, developed after the 11th postoperative chemotherapy, which extensively involved the stomach and intestines. The patient died of the disease 33 months after the initiation of treatment.
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PMID:[A case of retroperitoneal extramedullary plasmacytoma]. 836 83

Retrocaval ureter is a rare disease in childhood which is due to a abnormal development of the inferior cava vein. We report a new case of retrocaval ureter in a 9 year-old girl who had consulted for macrohematuria and right flank pain. Preoperative intravenous urography and DTPA diuretic renogram suggested this entity. CT scan was no necessary for the diagnosis. We consider the diuretic renogram the best diagnostic method in the surgical decision of this disease.
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PMID:[Retrocaval ureter in children: surgical approach based on the obstructive pattern in the diuretic renogram with 99mTc DTPA]. 988 20

Thrombotic thrombocytopenic purpura (TTP) is a rare disease whose incidence is now increasing. We present a case of a 37-year-old man who presented with bilateral flank pain and hematuria, subsequently diagnosed with TTP. Thrombotic thrombocytopenic purpura has classically been characterized by the pentad of fever, microangiopathic hemolytic anemia, neurologic symptoms, renal dysfunction, and thrombocytopenia. The pathogenesis of the disease has been a mystery until recently. We review the current literature regarding the pathophysiology and management of this disorder. Our discussion focuses on the importance of understanding this disease while considering the differential diagnosis of a patient presenting with anemia and thrombocytopenia because the common pitfall of rapidly administering platelets to a patient with TTP may lead to a disastrous outcome.
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PMID:Thrombotic thrombocytopenic purpura presenting as bilateral flank pain and hematuria: a case report. 1139 82

Primary renal lymphoma is a rare disease. There is still no agreement on its development primarily in this non-lymphoid organ. Some of the reported cases are questionable because of the incomplete staging of extrarenal disease. In the present study, we describe a rare case of a 73-year-old white man presenting with right flank pain. Physical examination did not reveal any peripheral lymphadenopathy or hepatosplenomegaly. An abdominal computerized tomography (CT) showed a large tumor in the right kidney with no enlarged liver, spleen or lymph nodes. On morphological and immunohistochemical examination, the tumor fulfilled the criteria of anaplastic large cell lymphoma (ALCL). A review of the literature revealed that this is the first reported case of primary ALCL of the kidney.
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PMID:Primary anaplastic large cell lymphoma of the kidney. 1268 57

Retroperitoneal fibrosis is an inflammatory disease, which is either idiopathic or secondary to infection, neoplasm, hemorrhage, aortic aneurysm or drugs. This is a rare disease usually presenting constitutional symptoms, abdominal, back or flank pain and urinary frequency. Treatment includes surgical relief of urethral obstruction and corticosteroids. There is no clear evidence of the beneficial effect of corticosteroids treatment on the course of retroperitoneal fibrosis. We report a patient diagnosed with retroperitoneal fibrosis with an unusual presentation--uncontrolled HTN and renal failure due to renal arteries obstruction, without any abdominal symptoms. This patient responded to steroids and tamoxifen. A review of the literature is also presented.
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PMID:[Retroperitoneal fibrosis--clinical response to steroid treatment]. 1269 65

Renal artery embolism (RAE) is a rare disease. Urgent treatment is necessary, as ischaemia can cause irreversible kidney damage in 60 to 90 minutes. RAE frequently clinically manifests as a pain similar to renal colic. Source of embolus is predominantly the heart at atrial fibrillation. Laboratory findings are unspecific. Ultrasonography with color Doppler imaging is essential. Kidney perfusion is low and upper urinary tract is undilated. Renal function can be recognized by intravenous urography and at renal scintigraphy. In angiography, renal artery is closed with thromboembolus. With no delay, transcatheter clot aspiration should be performed and fibrinolytic agents (tissue plasminogen activator) should be topically administered. Continual heparinisation and later warfarinisation should follow. In spite of successful revascularisation, parameters of kidney function can almost never reach that prior the RAE and shrinkage of kidney becomes a frequent consequence. Treatment can be successful even in patients with renal occlusion lasting over 90 minutes, since occlusion is often incomplete or significant collateral blood supply exists. In conclusion, renal artery embolism must be considered in cases of flank pain in patients with certain risk actors (especially atrial fibrillation). Ultrasonography with color Doppler imaging and urgent angiography of the renal artery are necessary in these cases. Thromboembolus can be then aspirated, and kidney perfused with fibrinolytic agent.
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PMID:[Renal artery embolism]. 1275 38

Retroperitoneal fibrosis (RF) is a rare disease, typically with an insidious clinical course. The peak incidence is seen in patients 40 to 60 years of age and mostly in man. The characteristic finding in this disease is a periaortic fibrous mass that often surrounds the ureters. Although usually regarded as an obstructive uropathy, there has been growing recognition of the condition as a generalized disease. It may have a wide variety of manifestations including mediastinitis, thyroiditis and sclerosing cholangitis. The most common mode of presentation remains abdominal or flank pain with uremia, anemia and a high sedimentation rate. Although ultrasound and renal scintigraphy may contribute to the general evaluation of patients with RF, CT-scanner is the preferred imaging method. The multiplanar imaging capability of magnetic resonance may facilitate assessment of disease extent. The pathogenesis of the disease remains unknown. Steroids and, more recently tamoxifen, appear to be effective in the treatment of the RF. In most instances, RF does not lead to long-term morbidity or affect survival. The three cases of RF reported herein illustrate the varied mode of presentation and the response to the treatment.
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PMID:[Retroperitoneal fibrosis, an unrecognized inflammatory disease. Clinical observations and review of the literature]. 1289 32

Glomangiomas are a subset of glomus tumors that have a rich vascular network. Although a majority of the glomus tumors occur in the skin of the hand, they have also been reported in the deep soft tissue, bone, lungs, and gastrointestinal tract, especially the stomach. To our knowledge, only one such case has previously been reported primarily occurring in the liver. We report a case of a glomangioma primarily arising in the liver of a 57-year-old man who presented with right flank pain of several months' duration. A 3.0-cm hepatic mass was excised and consisted of numerous, small-to-medium branched vessels with the stroma containing small, round, regular cells with sharply outlined round-to-oval nuclei. Immunostains showed the tumor cells to be diffusely positive for vimentin and smooth muscle actin and to be focally positive for calponin. Collagen IV stained the pericellular matrix. The immunostain for CD34 highlighted the vascular network as well as outlined the tumor cells in many areas. Coexpression of actin and CD34 in glomus tumors, although unusual, has recently been reported in the literature. Despite its bland histology, the large tumor size and deep visceral location were suggestive of aggressive behavior; thus, a close clinical follow-up was recommended. The patient had an unremarkable postoperative course and has no evidence of metastatic disease 12 months after the procedure. An accurate diagnosis and an understanding the biology of this rare disease, especially in an unusual location, are crucial to its management.
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PMID:Primary glomangioma of the liver: a case report and review of the literature. 1498 33

Primary primitive neuroectodermal tumor (PNET) of the urinary tract is a rare disease with aggressive behavior and poor prognosis. We analyzed 851 cases of urinary tract malignancies in our hospital between 1984 and 2004. Only three (0.035%) cases with PNET of the urinary tract were identified. Presenting symptoms included flank pain and hematuria. The first case was a 44-year-old man with left renal PNET who underwent hand-assisted laparoscopic radical nephrectomy and adjuvant chemotherapy. There was no recurrent tumor at the 4-year follow-up. The second case was a 75-year-old woman with right renal PNET with inferior vena cava (IVC) thrombosis extending to the right atrium. The patient underwent right radical nephroureterectomy and IVC thrombectomy with cardiopulmonary bypass. She died of metastatic disease 7 months later. The third case was a 45-year-old man with left ureteral PNET. Left ureteral segmental resection and partial cystectomy were performed. Tumor recurrence was noted 7 years later. The patient died of disseminated disease 1 year after the discovery of recurrence. Urinary tract PNET appears to be an aggressive malignancy. Long-term survival is possible if complete resection is performed at an early stage.
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PMID:Primary primitive neuroectodermal tumor of the urinary tract. 1718 43

Retroperitoneal fibrosis (RPF) is characterised by inflammatory fibrotic processes affecting the retroperitoneal structures. Its prevalence of 1 - 2/200,000 makes it a rare disease. To date, there are no guidelines for the diagnosis of or therapy for the disease. If untreated, the disease may be fatal. In 2006, the Department of Urology of the HELIOS Klinikum Wuppertal undertook to establish a nationwide patient registry, which would facilitate prospective therapy trials and the drafting of recommendations for diagnostic procedures. The pathogenesis of the disease is still unclear. Since some RPF-patients present with associated autoimmune diseases, autoimmune processes are suspected to play a role in causing the disease. The presence of autoantibodies and histological similarities with vasculitis support this hypothesis. Following initial general symptoms, patients display localised symptoms (flank pain, leg oedema, abdominal discomfort), caused by the displacing effect of the fibrotic plaques. Laboratory tests show elevated ESR and C-reactive protein and in some cases a moderate anaemia. Histological examinations should be undertaken to rule out the presence of malignant tumours. Radiological diagnostics (excretory urography, CT, MRI) show a retroperitoneal mass which blocks, compresses and displaces, completely or in part, the large vessels and the ureter. Initial therapy aims at restoring the function of the affected hollow organs through the application of (ureteric) stents, followed by immunosuppressive therapy. If drug therapy is unsuccessful, surgical procedures will follow to protect the ureter from compression. In some cases, ureteral replacement or an autotransplant of the kidney may be necessary. Life-long observation of the patients is necessary, as the disease may be chronic and relapsing. Interdisciplinary and nationwide cooperation is of crucial importance to further investigate this disease.
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PMID:[Retroperitoneal fibrosis]. 1751 80


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