Cases reported "nephrosis, lipoid"

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1/182. thymoma associated with myasthenia gravis and minimal lesion nephrotic syndrome.

    A nephrotic syndrome has been observed rarely in association with thymoma. In most of the reported cases, it occurs when the thymoma is in remission; histological examination generally shows minimal change disease. We report a case of a 43-year-old man presenting with minimal lesion nephrotic syndrome at the time of the diagnosis of thymoma and myasthenia gravis, which persists despite remission of the thymoma. The role of a disorder of T-cell function and of circulating cytokines is discussed. ( info)

2/182. Effect of pituitary microsurgery on acromegaly complicated nephrotic syndrome with focal segmental glomerulosclerosis: report of a rare clinical case.

    A case of nephrotic syndrome complicated by acromegaly is presented. The first renal biopsy specimen showed minor glomerular abnormalities with glomerular hypertrophy, corresponding with minimal change nephrotic syndrome. Corticosteroid therapy led to a partial remission, followed by frequent relapses after reduction of the drug. A diagnosis of atypical focal segmental glomerulosclerosis (FSGS) was made based on the second renal biopsy results 6 months after the first. We combined steroid therapy with the administration of an anticoagulant, cytotoxic agents, angiotensin-converting enzyme inhibitor, and low-density lipoprotein adsorption. Except for the angiotensin-converting enzyme inhibitor, these medications were not effective in terms of allowing a reduction in the high dosage of steroid, which in turn threatened progressive osteoporosis and lumbar vertebrae fracture. Administering the steroid at a moderate dosage, treatment was focused on the complicating acromegaly from pituitary microadenoma. Subcutaneous injections of octreotide acetate, a somatostatin analogue, reduced proteinuria and increased urine volume. Subsequent transsphenoidal microsurgery of the adenoma resulted in the normalization of the elevated creatinine clearance and the further reduction in steroid dosage while maintaining a remission state. This is the first reported clinical case with acromegaly followed by FSGS, and it is suggested that hypersecretion of growth hormone participates in the development and progression of glomerular disease. ( info)

3/182. Yellow nails and minimal change nephrotic syndrome.

    We report a case of a 38-year-old man showing the yellow nail syndrome in association with minimal change nephrotic syndrome. Treatment with prednisone and vitamin e resulted in complete resolution of the nephrotic syndrome and slow improvement of the yellow nails, respectively. Although the rare yellow nail syndrome has been described in association with renal disease, this is the first report of the association of this syndrome with minimal change nephrotic syndrome. ( info)

4/182. Minimal-change nephrotic syndrome and acute renal failure in a patient with aged onset insulin-dependent diabetes mellitus and autoimmune thyroiditis.

    A 61-year-old woman with a 2-year history of insulin-dependent diabetes mellitus (IDDM) developed nephrotic syndrome. Renal biopsy showed minimal-change nephrotic syndrome (MCNS), and no evidence of diabetic glomerulosclerosis. Although steroid therapy was initiated, plasma urea and creatinine rose and hemodialysis was required. After 4 weeks, she responded to steroids and her renal function returned to normal. MCNS, which is not associated with diabetic glomerulosclerosis, has rarely been seen in IDDM patients with nephrotic syndrome. Her human leukocyte antigen typing was A24, BW52, BW61, DR2 and DR9. This typing has been reported to be associated with both IDDM and renal disease. ( info)

5/182. Minimal change nephrotic syndrome: a possible complication of ehrlichiosis.

    Ehrlichiae are rickettsial organisms recently shown to be human pathogens. Infections often cause fever, myalgia, and hematological abnormalities, and sometimes mild elevation in transaminases, creatinine, and urinary protein. We report a teenager with nephrotic syndrome from minimal change glomerulonephritis and serological evidence of ehrlichiosis. In the appropriate clinical setting, Ehrlichiae should be considered in the etiological assessment of patients with minimal change disease. ( info)

6/182. nephrotic syndrome at 5 months: no definitive treatment or complications for 12 years.

    We describe a patient who developed nephrotic syndrome at 5 months, with extensive glomerular and tubular damage on biopsy. The patient was treated with diuretics and was asymptomatic for a decade despite unremitting proteinuria. A repeat biopsy at 13 years of age showed remarkable healing with histopathological features consistent with "minimal change" nephrotic syndrome. This patient illustrates a favorable clinical outcome, without specific treatment, of nephrotic syndrome of long duration. ( info)

7/182. Minimal change glomerulonephritis associated with hydatid disease.

    A 63-year-old man presented to our department with dyspnea and peripheral edema. A cystic mass in the right upper abdomen, consistent with echinococcal disease was discovered. proteinuria was also present, and a nephrotic syndrome was diagnosed. The kidney biopsy revealed minimal change glomerulonephritis. Treatment with the antiechinococcal drug albendazole induced complete remission of the nephrotic syndrome, suggesting an etiopathogenic role for a hydatid antigen in the development of an immune-mediated glomerulonephritis. ( info)

8/182. T-cell lineage acute lymphoblastic leukemia with chromosome 5 abnormality in a patient with Crohn's disease and lipoid nephrosis.

    We describe a 17-year-old patient with a documented history of Crohn's disease (CD) and of minimal-change nephrotic syndrome (MCNS) in whom a diagnosis of T-cell acute lymphoblastic leukemia (ALL) was made. The diagnosis of ALL was established by morphological examination of bone-marrow aspirates and confirmed by means of immunophenotypic analysis showing the involvement of T-cell lineage leukemic cells. The lymphoid clone showed a karyotypic abnormality involving the long arm of chromosome 5 in a translocation (5;6). Few cases of CD complicated by ALL have been previously reported. The present one is the first case combining CD and ALL in a patient with a past history of MCNS. This raises the possibility of a relationship among those diseases. The possible mechanisms for such a relationship are discussed here. ( info)

9/182. nephrotic syndrome in Hodgkin's disease. Evidence for pathogenesis alternative to immune complex deposition.

    The nephrotic syndrome has been reported to occur in patients with Hodgkin's disease even in the absence of amyloidosis, tumor infiltration of renal vein thrombosis. Three patients are presented with Hodgkin's disease and the nephrotic syndrome whose renal biopsy specimens studied with light, immunofluorescence and electron microscopy were compatible with "lipoid nephrosis" (minimal change disease). A review of the literature reveals 35 patients with Hodgkin's disease and the nephrotic syndrome. Renal tissue was available for examination in only 27 patients. The majority of patients apparently had glomerular alterations consistent with lipoid nephrosis. The nephrotic syndrome in most of these patients remitted with a variety of methods of therapy (including excision, irradiation, prednisone and cyclophosphamide) and tended to relapse with a recurrence of Hodgkin's disease. In three-fourths of the patients with Hodgkin's disease and the nephrotic syndrome, the Hodgkin's disease was of a mixed cellularity type. The etiology of lipoid nephrosis, although unclear, may be a consequence of altered lymphocyte function. Hodgkin's disease is a malignancy involving T lymphocytes, and the nephrotic syndrome occurring in the course of Hodgkin's disease may be a result of an adverse effect of glomeruli by products of tumor lymphocytes rather than of glomerular deposition of immune complexes. ( info)

10/182. Simultaneous occurrence of minimal change glomerular disease, sarcoidosis and Hashimoto's thyroiditis.

    We herein report a very rare case of a patient suffering from simultaneous occurrence of three immune disorders, i.e. Hashimoto's thyroiditis, sarcoidosis and minimal change glomerular disease. A 66-year-old man was admitted to our hospital for evaluation of nephrotic syndrome. Six months before admission, he was pointed out as having positive proteinuria, hypoalbuminemia and associated pretibial pitting edema. Initial laboratory data showed high gammaglobulinemia, high titers of both antimicrosomal and antithyroglobulin antibodies with normal thyroid function. Chest X-ray and CT scan revealed bilateral hilar lymphadenopathy with interstitial shadow. Ga-citrate scan disclosed positive accumulation in the thyroid glands, the mediastinum, the lungs and the kidneys. The diagnosis of minimal change nephritic syndrome and pulmonary sarcoidosis was made, based on the findings of transbronchial lung biopsy and kidney biopsy. After one and a half months of admission, thyroid function had gradually deteriorated. The histological findings of the thyroid were consistent with the features of Hashimoto's thyroiditis. Treatment with prednisolone and cyclophosphamide resulted in a decrease in urinary protein excretion, reduction in the size of mediastinal lymphadenopathy and disappearance of positive findings of Ga-citrate scan in the thyroid glands and the kidneys. Simultaneous occurrence of minimal change-glomerular disease, sarcoidosis and Hashimoto's thyroiditis in our case suggests that similar immunological abnormalities may be involved in the pathogenesis of the diseases. ( info)
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