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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three cases of the Crow-Fukase syndrome without radiographic changes of multiple myeloma are reported, with special reference to the glomerular changes seen. Proteinuria was detected in one case, although decreased renal function was observed in all (GFR: 41.0, 62.0, 74.1 ml/min respectively) at the time of renal biopsy. Glomerular changes were similar in all three cases. The main characteristic changes were mesangial proliferation and thickening of the glomerular capillary walls. Pictures by light microscopy were therefore similar to that of MPGN. On electron microscopy, the thickened capillary walls showed circumferential mesangial interposition and the subendothelial zone was electron-lucent and contained small dense granules or flocculent deposits. By immunofluorescent microscopy, no immunoglobulins, complement components or light chain were detected in the glomeruli except in one case.
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PMID:Glomerular lesions associated with the Crow-Fukase syndrome. 308 38

We describe a patient with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) who was found to have renal involvement with particular renal pathological findings. So far, 17 other cases, most of them from Japan, of POEMS syndrome with renal involvement, have been published. Clinical features are variable: acute renal failure with anasarca or moderate chronic renal insufficiency with mild proteinuria. This latter presentation often passes unnoticed. There is no severe hypertension, no microangiopathic hemolytic anemia. Renal biopsy shows prominent glomerular changes which are unusual and distinct from membranoproliferative glomerulonephritis (MPGN) and from glomerular thrombotic microangiopathy (TMA). Mesangial proliferation and thickening of the capillary wall with double contour on light microscopy suggest an MPGN. By immunofluorescent microscopy, no immunoglobulins or complement deposits were found. The occurrence of mesangiolytic lesions has led to the term of "mesangiolytic glomerulonephritis". The presence, on electron microscopy, of lucent subendothelial spaces could indicate TMA. But there are neither thrombi nor arteriolar changes. We are inclined to consider that the microangiopathic lesions are due to chronic injury of glomerular endothelial cells, exacerbated at outbreaks of the disease. Increased production of IL 6 could support the efficacy of corticosteroid therapy, particularly in acute clinical situations.
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PMID:Renal involvement in POEMS syndrome. 763 52

The authors report four cases of POEMS Syndrome with renal involvement. Two had renal failure and mild proteinuria. The two others had hyporeninemic hypoaldosteronism with type IV renal tubular acidosis in the first and hyperkalemia alone in the second. In the two cases, renal biopsy showed mesangial proliferation. Evolution was favourable when the plasma cell proliferation could be checked. In POEMS pathophysiology of renal involvement remains unknown. Soluble factor(s) produced by plasma cells may be responsible for most signs and symptoms perhaps as a result of increased vascular permeability.
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PMID:[Renal manifestation in POEMS syndrome]. 781 63

We described a patient with a POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal grammapathy and Skin changes) who was found to have renal involvement with peculiar renal pathological findings. Hitherto, 17 other cases, most of them from Japan, of POEMS syndrome with renal involvement, have been published. Clinical features are variable: acute renal failure with anasarca or moderate chronic renal insufficiency with mild proteinuria. This latter presentation often passes unnoticed. There is no severe HTA, no microangiopathic hemolytic anemia. Renal biopsy shows prominent glomerular changes which are unusual and distinct from membranoproliferative glomerulonephritis (MPGN) and from glomerular thrombotic microangiopathy (TMA). Mesangial proliferation and thickening of the capillary wall with double contour evoke by light microscopy a MPGN. By immunofluorescent microscopy, no immunoglobulins or complement deposits are found. The finding of mesangiolytic lesions has led to the term of "mesangiolytic glomerulonephritis". The presence, on electron microscopy, of lucent subendothelial space could evoke TMA. But there is neither thrombi, nor arteriolar changes. We are inclined to presume that microangiopathic lesions are due to chronic injury of glomerular endothelial cells, exacerbated at outbreaks of the disease. An increased production of IL-6 could support the efficacity of corticosteroid therapy, particularly in acute clinical situations.
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PMID:[Renal involvement in POEMS syndrome]. 786 28

Crow-Fukase syndrome is a rare multiorgan disorder. Although renal disorders, such as proteinuria, and renal impairment, have been observed in half the cases of this syndrome, there have been few reports describing the renal lesions. We report here a case of this syndrome associated with membranoproliferative glomerulonephritis. A 43-year-old woman was referred to our hospital because of hyperglycemia. She had also been suffering from hyperpigmentation, hepatosplenomegaly, lymphadenopathy, polyneuropathy and endocrine dysfunction, including diabetes mellitus and amenorrhea. Serum electrophoresis showed M protein and immunoelectrophoresis revealed IgA (lambda). Bone marrow aspiration showed a slight increase in the number of plasma cells. Urine protein was 30 mg/dl, BUN was 17 mg/dl and creatinine 0.8 mg/dl. Light microscopic examinations showed enlargement of glomeruli with proliferation of mesangial cells and matrix, a lobular pattern of the glomeruli and thickening of the glomerular basement membrane and associated double contour. Electron microscopic examinations showed thickened capillary walls, associated mesangial interposition and subendothelial dense deposits. Moreover, fine granular deposits of IgM, C3, and fibrinogen along the basement membrane were observed on immunofluorescent studies.
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PMID:[A case of Crow-Fukase syndrome associated with membranoproliferative glomerulonephritis]. 807 25

POEMS syndrome is a multisystem disorder with signs such as peripheral neuropathy, organomegaly, endocrinopathy, monoclonal protein, skin lesions, papilledema, and increased cerebrospinal fluid proteins, which can also evolve with renal and cardiac affection. It is considered a result of a plasma cell cyscrasia with the production of a monoclonal protein. A 46-year old man was seen as an outpatient referring progressive weakness of legs and arms, fever, impotence, inguinal and cervical lymphadenopathies, peripheral edema, hepatomegaly and skin hyperpigmentation. In laboratory test, platelet count was between 528 x 10(9)/L and 599 x 10(9)/L, creatinine clearance 27.2 ml/min, proteinuria 0.8 g/dl, IgA 455 mg/dl, T3 30 ng/100 ml, T4 2.6 vg/dl, T4F 0.5 ng/dl, TSH 12.4 vU/ml; testosterone 1.56 ng/ml. The electromyography showed a mixed sensitive-motor pattern. On the pelvis radiography, an osteosclerotic lesion on the left sacroiliac joint was identified. Bone biopsy of the site of the sclerotic lesion revealed plasma cell dyscrasia. The patient was treated with diuretics, digitalis and prednisone. Diagnosis of this disorder is difficult because of the multipathology it is necessary to establish differential diagnosis.
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PMID:[POEMS syndrome. Report of a case]. 1042 26

Monoclonal immunoglobulin (Ig) deposition diseases are characterized by deposition in tissues of excessive amounts of the Ig, compromising organ functions. Light chain deposition disease (LCDD) and AL amyloidosis are the commonest [Buxbaum 1992]. LCDD is usually characterized by rapidly progressive renal failure with glomerular and tubular deposits of Ig fragments mostly composed by kappa light chain. Monoclonal Ig production can also be observed associated with various symptoms, that, taken together, have been described as the Crow-Fukase syndrome or POEMS syndrome. It associates polyneuropathy, organomegaly, endocrinopathy, monoclonal Ig, and skin changes. In POEMS syndrome, renal abnormalities are rare and are reported as a moderate renal insufficiency with mild proteinuria or acute functional renal insufficiency leading in some cases to end-stage renal failure [Fukatsu et al. 1991]. Although a monoclonal Ig is produced, no Ig deposit disease had been described in POEMS syndrome except a case of AL amyloidosis [Toyokuni et al. 1992]. Here, to our knowledge, we report the first case of an LCDD associated with a POEMS syndrome. Although an autologous bone marrow graft was realized, the monoclonal component reappeared and was responsible for end-stage renal disease, cachexia and death.
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PMID:Association of a POEMS syndrome and light chain deposit disease: first case report. 1143 61

POEMS syndrome is a rare plasma cell dyscrasia which is characterized by small amounts of monoclonal protein, and a multisystem complex manifested by various combinations of polyneuropathy, organomegaly, endocrinopathy and skin changes. Here, we presented an atypical case of POEMS syndrome with IgG kappa monoclonal protein, chronic demyelinating polyneuropathy, hepatosplenomegaly, hypothyroidism, gynecomastia and severe renal impairment. The finding of IgG kappa type of monoclonal protein in our patient was interesting because the majority of cases were reported to have lambda light chain. Also, the absence of typical skin and bone lesions were atypical. Though speculative, these atypical features may account for the unusual presentation of this case. Our patient rapidly progressed to end-stage renal failure and died of cachexia. Renal involvement in POEMS syndrome is rare but may show substantial clinical and pathological variations. Proteinuria, hematuria, renal dysfunction and renal failure requiring hemodialysis can be seen. The pathogenesis of renal dysfunction is unclear. As a conclusion, POEMS syndrome may present with diverse clinicopathologic manifestations. In this syndrome, renal involvement may lead to end stage renal failure and the course may be fatal due to severe polyneuropathy and wasting.
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PMID:An atypical case of POEMS syndrome with IgG kappa M protein and end stage renal failure. 1630 46

Glomeruloid haemangioma is considered a specific marker of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome and it is usually but not always associated with multicentric Castleman's disease. We report a 78-year-old man who presented with a single, red-violet soft nodule with superficial telangiectases on the scalp. Histopathologically, the lesion consisted of lobules of coiled aggregated capillaries that involved the lumina of dilated vascular structures, mimicking renal glomeruli. A collagenous stroma separated the capillary lobules, and eosinophilic, periodic-acid-Schiff positive globules of varying sizes and shapes were seen within the cytoplasm of endothelial cells. Immunohistochemical studies with antibodies against IgA and IgG, and against the kappa and lambda light chains of immunoglobulins showed immunoreactivity within the eosinophilic globules. Results of complete blood count, liver, renal and thyroid function tests, fasting blood sugar measurement, serum levels of oestradiol, testosterone, prolactin and cortisol, serum protein electrophoresis, immunoelectrophoresis and immunofixation yielded normal or negative results. No Bence-Jones proteinuria was found in a sample from a 24-h urine collection. To our knowledge, only two cases of glomeruloid haemangioma have been previously reported in patients without POEMS syndrome. We describe the third case of glomeruloid haemangioma in a patient without features of POEMS syndrome.
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PMID:Glomeruloid haemangioma is not always associated with POEMS syndrome. 1907 91

A 43-year-old female patient admitted with a 2.5-year history of lower extremity symmetrical sensorimotor polyneuropathy, hypertrichosis, sweating, diarrhea, weight loss, and hyperpigmentation. The clinical evaluation met the criteria for the diagnosis of POEMS syndrome. The patient was initially treated with prednisolone and responded well. We planned high-dose chemotherapy with autologous stem cell rescue and introduced a more immunosuppressive regimen containing cyclophosphamide and dexamethasone. We present a case differing from the other cases with her 2 g/day proteinuria and hypertrophic osteoarthropathy.
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PMID:POEMS syndrome: a case report. 1911 89


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