Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0152025 (polyneuropathy)
 7,862 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

POEMS syndrome is an unusual multisystem disorder associated frequently with polyneuropathy, organomegaly, endocrinopathy, M-protein change and skin lesion. POEMS is the acronym the above-mentioned clinical manifestations. The patients who were diagnosed must have at least three of the manifestations; polyneuropathy and plasmagenic disorders are almost always present. We present in this paper eight patients, their clinical symptoms, laboratory tests and treatment were analysed and discussed.
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PMID:[POEMS syndrome--a report of eight cases and review of literature]. 771 29

The introduction of newer technology in the past few years, especially the use of second-generation enzyme-linked immunosorbent assays, recombinant immunoblot assays, reverse transcriptase, and DNA amplification, have clearly defined the role of hepatitis C virus as the most important etiologic factor in the development of mixed cryoglobulinemia. This has led to a better understanding of the pathogenic mechanisms involved in disease expression, particularly vasculitis, and also has provided a rationale for the use of interferon alfa and other antiviral drugs in the therapy of these disorders. The clinical manifestations of the syndrome also have been well characterized, as well as some of the risk factors. There also has been an improvement in our understanding of the pathogenic mechanisms involved in multiple myeloma and related monoclonal gammopathies, as well as several attempts to improve early recognition of bone disease with magnetic resonance imaging. The susceptibility gene for familial Mediterranean fever has been better characterized, as have risk factors for colchicine toxicity. The role of cytokines has been better delineated for both monoclonal gammopathies and POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome.
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PMID:Cryoglobulinemia and other dysproteinemias, familial Mediterranean fever, and POEMS syndrome. 771 25

We review the concept fo Monoclonal Gammopathy emphasizing that the so called benign forms, with or without polyneuropathy can have an unpredictable evolution. Polyneuropathies associated to monoclonal gammopathy-IgM, IgG, IgA, Waldestrom's disease, Multiple myeloma, Osteosclerotic myeloma (POEMS), Amyloidosis and Cryoglobulinemias are reviewed. They generally are solely sensory, sensorimotor or rarely, solely motor. The importance of neuro-physiological studies and nerve biopsy is emphasized. For progressive forms, treatment with plasmapheresis, immunotherapy, corticoids or intravenous gammaglobulin is suggested.
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PMID:[Polyneuropathies and dysglobulinemias]. 780 41

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

A Thai male, aged 36 yrs, presented with chronic progressive sensorimotor polyneuropathy and mixed osteosclerotic and lytic lesions at the lumbar vertebrae (L). Generalized hyperpigmentation, bilateral gynecomastia, small testes, hepatomegaly, lymphadenopathy and ascites were observed. Computerized tomography of the lumbosacral spines revealed osteosclerotic changes at L3,4,5 with spiculated bony proliferation at the transverse processes and posterior components of the spines. Histopathological examination of the lesion was compatible with plasmacytoma. Proliferative vasculopathy was demonstrated in plasmacytoma. Serum immunofixation showed an IgM monoclonal gammopathy. Further investigation demonstrated secondary adrenal insufficiency with low level of plasma testosterone. The patient responded well to local radiotherapy and intermittent chemotherapy with melphalan and prednisolone. The histopathological findings of proliferative vasculopathy suggested the important role of capillary leakage as a factor responsible for systemic manifestations in the POEMS syndrome. The reported cases of this syndrome among Thais are summarized.
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PMID:POEMS syndrome: a case with proliferative vasculopathy and a review of cases Thailand. 796 31

POEMS syndrome is a rare systemic multi-organ disease usually reported in Japanese patients. The clinical course is slow with a 5-year survival of 60%. Death is caused by polyneuropathy and or anasarka. We observed four cases in our ward (all males, aged 39, 57, 54 and 54 years) who all presented at least four characteristic clinical signs. Gynecomasty together with impotency was seen in all patients. All had hypogonadism and borderline hyperprolactinaemia. Hyperoestrogenism was seen in two. In three patients, rare endocrinopathies were part of the POEMS syndrome. One patient had diabetes mellitus. The clinical course was variable and a function of the effectiveness of plasmocyte dyscrasia therapy. Little work has been done on endocrinopathies in POEMS syndrome. In most cases, gonadotrope function is impaired with gynecomasty and impotency in men, amenorrhoea in women. Generally the hypogonadism is hypogonadotropic. Hyperoestrogenism is frequent and prolactin levels are normal or high with an exaggerated response to thyroid releasing hormone stimulation. The aetiology of POEMS syndrome is unknown. Current research is based on an immunologic theory based on the discovery of high levels of interleukin 6 in POEMS patients with or without Castleman's disease. The cytokine would affect the different organs and lead to clinical expression. Corticosteroids are usually effective in most patients, particularly in reducing the oedema and controlling the polyneuropathy.
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PMID:[Endocrine diseases in POEMS syndrome. Apropos of 4 cases]. 797 57

Mechanisms of peripheral neuropathies in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome are poorly understood. A peripheral nerve biopsy was performed in 22 patients. Of these 9 had histological features of Castleman's disease on lymph node biopsies, and 19 had a monoclonal lambda light chain in their serum. Certain nerve fragments were paraffin embedded, others were frozen and studied by direct immunofluorescence, and others were fixed for ultrastructural examination. Paraffin-embedded fragments did not show any amyloid deposits, and at direct immunofluorescence there was no immunoglobulin fixation. At ultrastructural examination, features of uncompacted myelin lamellae (UML) were present in 19 patients, and their frequency varied from 1% to 16% of myelinated fibres. Up to now UML have been reported only in 7 patients with POEMS syndrome in the literature. UML have also been noticed in a few cases of inflammatory demyelinating polyradiculoneuritis and inherited tendency to pressure palsy.
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PMID:Uncompacted myelin lamellae in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome. Ultrastructural study of peripheral nerve biopsy from 22 patients. 800 62

The authors report a case of a 27 year old man with severe polyneuropathy, multiple endocrine and cutaneous disorders, ascites, edema and multiple osteosclerotic lesions. Clinical and laboratory diagnosis of POEMS syndrome was made. The patient condition worsened progressively and death followed. The literature search showed that this is the first Brazilian case report about such a syndrome.
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PMID:[POEMS syndrome: report of a Brazilian case with fatal course]. 806 95

Patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome exhibit great clinical variability and often have osteosclerotic myeloma. We present a patient with an acquired demyelinating polyneuropathy who was eventually diagnosed as having POEMS syndrome. Her long period of observation permitted documentation of the natural history of a plasmacytoma, including its remarkably slow rate of growth and its transformation from a nonsclerotic to a sclerotic bone lesion. Her clinical and laboratory features emphasize the variability of this syndrome. Biopsy of benign-appearing bone lesions should be considered in patients with acquired demyelinating polyneuropathies who do not respond to standard treatment modalities. Serial x-rays may not distinguish benign lesions from plasmacytomas in this treatable condition.
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PMID:The natural history of a "benign" rib lesion in a patient with a demyelinating polyneuropathy and an unusual variant of POEMS syndrome. 806 92

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


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