Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032285 (pneumonia)
54,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 53-year-old male was diagnosed as having ataxic polyneuropathy associated with IgM-kappa monoclonal gammopathy in January 1988. Plasmapheresis and chemotherapy with chlorambucil and Melphalan-Prednisolone were effective for his neuropathy, but hemolytic anemia appeared in February 1989. The diagnosis of low-titer cold agglutinin disease (IgM-kappa) with anti-Pr2 specificity was made. Hemolytic anemia became refractory to high-dose corticosteroids, and fever, hepatosplenomegaly and severe pancytopenia appeared in January 1990. Bone marrow involvement of malignant lymphoma (mu, kappa) was found, and he died of pneumonia and gastrointestinal bleeding after the start of chemotherapy. Postmortem examination revealed a widespread infiltration of malignant lymphoma, diffuse, large cell (B-cell) type. Erythrophagocytic histiocytes also increased in bone marrow, liver, spleen and lymph nodes, as if there were hemophagocytic syndrome associated with lymphoma present. In addition to the high thermal amplitude of cold agglutinin in this case, the systemic activation of histiocytes induced by the development of malignant lymphoma may be responsible for progressive hemolysis and severe pancytopenia.
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PMID:[Anti-Pr2 cold agglutinin disease with polyneuropathy evolving to malignant lymphoma]. 133 64

POEMS (acronym for polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) is a very rare syndrome probably related to plasma cell dyscrasia. A 43 year old man developed a progressive symmetric sensory motor polyneuropathy 2 years before admission. Hepatosplenomegaly and sclerodermatoid skin changes were present on physical examination. A sclerotic lesion of the right femur was disclosed by radiologic examination. Serum immunoelectrophoresis demonstrated a monoclonal protein IgG-lambda pattern and the bone marrow biopsy revealed an increased plasma cell count (15%). The patient died 7 months after admission from pneumonia. A review of the literature is included.
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PMID:[Poems syndrome: review of a case]. 184 55

We examined the efficacy and toxicity of recombinant interferon-alpha 2b (rIFN-alpha 2b) in 10 previously untreated patients with advanced myelodysplastic syndromes. Morphological subtypes were refractory anaemia with excess of blasts (RAEB) in 4, RAEB in transformation (RAEB/T) in 3 and chronic myelomonocytic leukaemia (CMML) in 3 cases. IFN was administered subcutaneously at increasing doses of 1 to 3 x 10(6) IU per day. The median duration of therapy was 6 months (range, 3 to 14). 2 patients, both with a diagnosis of CMML, achieved a complete and partial remission, respectively. In the complete responder, remission could be maintained for 9.5 months by daily administration of 1 x 10(6) IU IFN. The other patients were classified as failures, although in 4 cases a decrease of bone marrow blasts was noted and none of the patients progressed to overt leukaemia while being treated with IFN. During the study, all patients with RAEB and RAEB/T showed a moderate to severe reduction in peripheral leukocyte and platelet counts, requiring premature termination of IFN therapy in 5 cases. Despite adequate supportive measures, 2 patients died of pneumococcal pneumonia and gastrointestinal bleeding, respectively. In 1 patient, IFN therapy had to be stopped because of neurologic toxicity (polyneuropathy). From these data we conclude that rIFN-alpha 2b at the doses and schedule tried is not a useful treatment for advanced myelodysplastic syndromes. Patients with CMML, however, may be an exception and should further be considered as candidates for therapeutic trials with rIFN-alpha 2b.
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PMID:Treatment of advanced myelodysplastic syndromes with recombinant interferon-alpha 2b. 198 3

Response to chemotherapy and survival was retrospectively analyzed in 28 patients with bulky retroperitoneal and disseminated seminoma treated between 1977 and 1983. The median age was 41 years (range: 23-52). All patients had histological evidence of pure testicular seminoma, however, 14 patients revealed moderate increases of human beta-chorionic gonadotropin levels. Prior radiotherapy had been given to 9/28 (32%) patients. Treatment consisted of at least four courses of simultaneous or sequentially alternating therapy with cisplatin, vinblastine, bleomycin plus/minus adriamycin (PVB +/- A), administration of ifosfamide or combination therapy with ifosfamide/cisplatin (IFS/DDP) or ifosfamide/etoposide (IFS/ETP). Twenty-five of 28 patients (89%) achieved a complete (CR), and 3/28 patients a partial remission. Relapse occurred in 1/8 CR patients after adjuvant postchemotherapeutic irradiation, and in 1/11 patients without any further radiotherapy. So far, 23/28 patients (82%) are free of disease after a median follow-up of 28+ (14+----82+) months. Marked myelosuppression was observed in previously irradiated patients, mainly after PVB +/- A therapy. In two patients, transient nephrotoxicity developed after PVB and IFS/DDP, respectively. After PVB +/- A chemotherapy, three patients revealed polyneuropathy, paralytic subileus and bleomycin-induced pneumonitis, respectively. In conclusion, the present series suggests a high probability of continuous CR in even bulky retroperitoneal and widespread metastatic seminoma. So far, no definite conclusions can be made on the therapeutic superiority of one of the different chemotherapeutic regimens used. However, this preliminary experience suggests that the combination of ifosfamide and etoposide or cisplatin may prove less toxic than sequentially alternating or simultaneous PVB +/- A chemotherapy.
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PMID:Chemotherapy of metastatic seminoma. 257 65

High grade malignant non-Hodgkin's lymphoma (NHL) was the presenting manifestation of the acquired immunodeficiency syndrome (AIDS) in 3/81 reported cases of AIDS in Denmark (by April 2, 1986). Asymptomatic HIV infection, 1 and 5 yr prior to the onset of lymphoma, was documented in 2 cases. 1 patient became infected by Factor VIII treatment, 2 were male homosexuals. 2 patients had an uncommon tumour presentation in the oral cavity, 1 patient presented with an abdominal mass. The histologic subtypes were immunoblastic (2), and small noncleaved cell, Burkitt's (1). Helper/suppressor T-cell ratio was decreased at onset of lymphoma in 2 cases. All 3 patients have died, 4, 6, and 24 months after diagnosis of NHL. Only 1 patient died of NHL, 1 died of an unclassified pneumonia and the third developed progressing supranuclear HIV-associated polyneuropathy without evidence of CNS lymphoma. Thus, high grade malignant B-cell NHL is a regular initial manifestation of AIDS, and may develop after years of asymptomatic HIV infection.
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PMID:Human immunodeficiency virus (HIV) associated non-Hodgkin's lymphomas in Denmark: report of three cases. 365 75

A case of connatal polyneuropathy is described in a boy who died of pneumonia at the age of 2 years, and from whom sural nerve biopsies had been taken when he was 4 and 16 months old. Clinically, his disease was characterized by motor weakness and muscular flaccidity in the presence of normal intellectual development. The evolution of the connatal peripheral nerve lesion could be followed from the age of 4 months to death: The first biopsy evidenced the most serious pathologic changes. The findings were reminiscent of those encountered in a fetal nerve at 18 weeks of gestation. Furthermore, it showed numerous filamentous inclusions in Schwann cells. The second biopsy showed a sparsely myelinated nerve with bands of basement membrane apparently unrelated to cells arranged around the nerve's fibers. A few Schwann cells containing filamentous inclusions were still present. At autopsy, the findings were identical to those of the second biopsy. The possibility that this patient was transitionally exposed to a neurotoxic agent during pregnancy and that the biopsy findings represent a lesion that is still florid in the first and in a residual state in the second biopsy is considered.
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PMID:Connatal polyneuropathy -- a case with proliferated microfilaments in Schwann cells. 629 5

A Japanese man with a variety of neurological complications, had drunk Japanese rice wine (sake) daily for about 25 years. There was a progressive development of parkinsonism, cerebellar ataxia, and mental deterioration by the time he was 32. He died of pneumonia at age 50 and the autopsy revealed Marchiafava-Bignami disease (MBD), striatal degeneration, pseudolaminar sclerosis of Morel, atrophy of the corpus mamillare and pons, cortical cerebellar atrophy, pseudopellagra, and polyneuropathy. This is the first case of MBD in a Japanese related to the ingestion of Japanese "sake", and it is also a rare case in that almost all of the neurological complications seen with chronic alcoholism were apparent. Striatal degeneration seems to be a rare complication of chronic alcoholism.
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PMID:Marchiafava-Bignami disease, striatal degeneration, and other neurological complications of chronic alcoholism in a Japanese. 721 Nov 93

Immunosuppressive treatments of neuro-immunologic diseases: myasthenia gravis, polymyositis, chronic inflammatory demyelinating polyneuropathy and multiple sclerosis were reviewed. The treatments need to be planned in terms of 2-5 years. Cautions must be taken for adverse effects of short and long terms. Corticosteroids were the most well used and were studied medication of the first choice among immunosuppressants in these diseases except for CIDP in which large amounts of IV-Ig or plasmapheresis are the first choice. Pulse treatment of very high doses of steroids are used in refractory or severe cases. As for immunosuppressants, in polymyositis iv MTX is the choice since its response is quicker than AZ. CsA is used in cases with pneumonitis. In MS, pulse treatments of steroids followed by gradual decreasing doses of steroids are used for 2-3 months in each relapse. Recently, IFN-alpha 2a and IFN-beta significantly reduced the number of relapses and improved MRI findings. Chronic applications of AZ, CY, Cs or MTX have possibilities of reducing relapses, and new drugs like mizoribine and mitoxantrone etc. are in trials.
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PMID:[Treatment of neuro-immunologic diseases by immunosuppressants]. 799 8

Thinner which contains aromatic hydrocarbons such as xylene, toluene and N-hexane is widely used in industrial plants manufacturing dyes, plastic, varnishes and glues. Chronic intoxication due to abuse of solvents, including thinner, by workers who inhale the solvent vapor is frequently encountered. Acute intoxication with ingestion of excessive amounts is relatively rare and usually fatal. It is reported that 45-50 ml of orally ingested thinner is enough to cause severe complications. The case reported here was forced to drink 200 ml of thinner by an older friend, and presented with severe complications such as rhabdomyolysis, polyneuropathy, chemical pneumonia and coma. To the best of our knowledge this is the first case reported in the literature to survive acute thinner intoxication with such complications.
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PMID:Severe acute thinner intoxication. 870 88

Onion bulb formations involving cranial nerves are an unusual pathologic feature. We report the postmortem neuropathologic findings in a 69-year-old man with a longstanding neuropathy characterized by progressive muscle weakness, sensory ataxia and multiple cranial nerve abnormalities. Electrodiagnostic testing disclosed features of an acquired demyelinating polyneuropathy. Treatment with corticosteroids and plasmapheresis resulted in no change in his neurologic status, and the patient died after repeated episodes of pneumonia and sepsis. Autopsy showed widespread onion bulb formation in cranial nerves III, IV, V, VI, X, XI and XII, anterior and posterior spinal nerve roots, dorsal root ganglia and multiple peripheral nerves, some of which also had foci of epineurial perivascular inflammation. Muscle sections revealed severe neurogenic atrophy. This case demonstrates that, in longstanding acquired demyelinating neuropathy, the cranial nerves also undergo repetitive cycles of demyelination and remyelination resulting in severe weakness of the bulbar musculature and histologic features of hypertrophic neuropathy.
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PMID:Severe cranial nerve involvement in longstanding demyelinating polyneuropathy: a clinicopathologic correlation. 883 44


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