Cases reported "Kidney Diseases"

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1/21. Nephron-sparing surgery in a case of giant renal hydatid cyst.

    We report a case of a centrally located giant renal hydatid cyst managed successfully by excision of the cyst alone and preserving the renal parenchyma after clamping the main renal artery. Follow-up IVP showed good function.
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2/21. Pediatric acute leukemia presenting as bilateral renal enlargement. Report of a case with fine needle aspiration cytologic features suggestive of megakaryocytic differentiation.

    BACKGROUND: Fine needle aspiration (FNA) cytologic diagnosis of acute myeloid leukemia involving the kidney has rarely been reported, but acute leukemia with cytologic features suggestive of megakaryocytic differentiation has not been described before. CASE: An 8-month-old male presented with an abdominal swelling, bilateral cervical and inguinal lymphadenopathy and enlarged left epididymis. ultrasonography of the abdomen revealed a space-occupying lesion in the liver and bilateral enlargement of the kidneys. FNA smears from the right kidney and right submandibular lymph node showed numerous blast cells. Since rare blast cells were positive for myeloperoxidase, a cytodiagnosis of involvement by acute myeloid leukemia (AML) was made. However, following the hematologic diagnosis of acute megakaryoblastic leukemia (M7) from peripheral blood and bone marrow smear examination, FNA smears were reviewed. There were cytoplasmic blebs or protrusions in the blast cells and cytologic features suggestive of their differentiation toward micro-megakaryocytes and megakaryocytes. There was also evidence of shedding of platelets, including numerous giant platelets. The reviewed FNA cytodiagnosis was suggestive of AML (M7). CONCLUSION: Extramedullary involvement by acute megakaryoblastic leukemia (M7) can be suspected based on cytomorphologic features in FNA smears.
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3/21. giant cell arteritis "causing" AA-amyloidosis with rapid renal failure.

    giant cell arteritis (GCA) is a systemic vasculitic disease, which in very rare cases causes inflammatory complications such as secondary amyloidosis. We describe a well-documented case, with a clinically mild course, of biopsyproven giant cell arteritis as the only apparent cause of systemic AA-amyloidosis. The deterioration in renal function due to amyloid deposition occurred rapidly and only a few months after manifestation of giant cell arteritis and was not reversible by steroid treatment. The renal arteries were normal and there was no glomerulonephritis due to giant cell arteritis. This unique case demonstrates that giant cell arteritis with a mild clinical course is closely associated with early-onset severe secondary amyloidosis.
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4/21. giant cell arteritis and renal amyloidosis: report of a case.

    polymyalgia rheumatica and giant cell arteritis are rarely associated with renal diseases such as amyloidosis. Here is a case of a 72-year-old man, who developed some renal failure and a nephrotic syndrome while presenting clinical symptoms of giant cell arteritis. Percutaneous renal biopsy showed secondary amyloidosis, and temporal artery biopsy revealed some lesions which suggested temporal arteritis. In spite of aggressive steroid treatment, chlorambucil, and colchicine, the patient's renal function continued to deteriorate and the patient is now being given hemodyalisis.
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5/21. Angiocentric immunoproliferative lesions of the lung associated with diffuse renal involvement.

    A 62-year-old Japanese man presented with high fever, cough, and sputa. Computed tomography (CT) scan of the chest revealed lung infiltrates with air bronchogram of the right middle lobe and mediastinal lymphadenopathy. Bronchoscopic examination was performed, and mycobacterium avium complex was detected from bronchoalveolar lavage fluid. Although the administration of clarithromycin and levofloxacin improved the patient's symptoms, the lung infiltrates on chest CT scan gradually worsened. lung biopsy of segments 4 and 8 by video-assisted thorachoscopy revealed angiocentric and angiodestructive massive lymphoplasmocytic infiltrations with multinucleated giant cells, which were compatible with grade II angiocentric immunoproliferative lesions. The patient was found to have deterioration of renal function, and glomerular filtration rate was 32.6 mL/min. His kidneys were enlarged and showed prominent and diffuse uptake of gallium-67 citrate. Percutaneous renal biopsy revealed massive infiltration of CD4 mononuclear cells, plasma cells, and eosinophils in the interstitium and destruction of normal structure of tubules. blood vessels were destroyed and replaced by inflammatory cells. The combination chemotherapy achieved a remission, and the patient has remained free of disease at 2 years after onset of the illness. We recommend the imaging of kidneys for diagnosis and following renal biopsy to evaluate the renal involvement of angiocentric immunoproliferative lesions.
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6/21. Giant renal hydatid cyst resembling a simple cyst: an intraoperative diagnosis.

    An interesting case of a giant renal hydatid cyst is presented. The big cystic mass detected at ultrasonography (US) and computerized tomography (CT) in a ten-year-old girl looked like a simple cyst. No germinative membrane or any other radiological sign of a hydatid cyst was present. The operation was planned for a simple cyst; but was altered, after an intraoperative diagnostic needle sampling brought out the characteristic fluid of hydatid cyst. We came to the conclusion that hydatid cystic masses may not always present with their characteristic radiological findings; and that extreme caution should be practiced by the radiologist and the surgeon in order to prevent iatrogenic echinococcal dissemination.
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7/21. erdheim-chester disease. Case report with autopsy findings.

    erdheim-chester disease is a rare pathologic entity characterized by symmetrical radiodensities in the metaphyseal and the diaphyseal portions of the long bones. fibrosis, osteoblastic cortical bone deposition, and fibroxanthomatous granulomas with lipid-laden macrophages and multinucleated giant cells, which have a particular tropism for connective and adipose tissues, are the pathologic hallmarks. To our knowledge, 27 cases have been reported in the literature since the entity was first described in 1930. Protean clinical features range from a focal and asymptomatic process to a multisystemic infiltrative disease. We describe the clinical course of a new case and review the extensive pathologic findings at autopsy, including those demonstrated by light and electron microscopy and cytochemical and immunocytochemical studies.
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8/21. A chronic granulomatous syndrome of unknown origin.

    A small clinically distinct group of patients with widespread tissue granulomata are described. The principal presenting symptoms are malaise, fever, and weight loss, although a wide variety of complaints are documented. Pulmonary involvement is uncommon. The granulomata are noncaseating with a few multinucleate giant cells and some surrounding chronic inflammatory infiltrate. There is no evidence of an associated arteritis. The disease has a relapsing and remitting course and although it may require treatment with immunosuppressive drugs, particularly if the kidneys are involved, the prognosis is relatively good. We propose that this entity be called granulomatous syndrome of unknown origin. The characteristics that set this syndrome apart from the other granulomatous vasculitides are discussed. The current limited understanding of granuloma formation does not allow us to propose a definite etiology for this condition. It is emphasized that it is not helpful to encompass it within a label of sarcoidosis. First, it may only serve to confuse the doctor in assessing and treating this very particular group of patients. Secondly, it may hinder future attempts to understand the different pathogenetic mechanisms underlying the various conditions in which granulomata may arise.
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9/21. Combined sarcoidosis and disseminated visceral giant cell vasculitis.

    A middle-aged man with only slight symptoms of disease of short duration died suddenly. On autopsy he was found to have not only disseminated visceral giant cell vasculitis with involvement of large and medium-sized arteries and veins, but also sarcoid granulomas in many organs, especially in enlarged mediastinal lymph nodes as well as in several vessel walls. death was caused by myocardial infarction due to granulomatous vasculitis. This combination of diseases has not previously been described, and the question is whether it represents giant cell arteritis and sarcoidosis, separately, or whether it is a manifestation of a broad spectrum of the same disease.
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10/21. Percutaneous drainage of a giant pyelocaliceal diverticular abscess in pregnancy.

    A large renal pyelocaliceal diverticular abscess during pregnancy has been successfully managed by a percutaneous approach. After a normal vaginal delivery, an excision of the diverticulum was done with an uneventful postoperative course.
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