UMLS:C0032285 - FACTA Search

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Query: UMLS:C0032285 (pneumonia)
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Churg-Strauss syndrome (CSS) is a disease characterised by hypereosinophilia and systemic vasculitis occurring in patients with asthma and allergic rhinitis. In the course of CSS three phases may be distinguished. The prodromal phase, which may persist for many years, consists of allergic disease. The second phase is characterised by peripheral blood eosinophilia and eosinophilic tissue infiltrates that produce a clinical picture diagnosed as Loeffler's syndrome, chronic eosinophilic pneumonia or eosinophilic gastroenteritis. The third phase is dominated by systemic vasculitis in which skin, cardiovascular system, gastrointestinal tract and peripheral nervous system are frequently involved. Renal disease in CSS is less common and generally less severe than that classical polyarteritis nodosa and Wegener's granulomatosis. Genitourinary tract may be involved, too. In the postvaculitic phase, allergic rhinitis and asthma usually persist and clinical picture is characterised by the consequences of the vasculitic illness, most commonly in form of neuropathy and hypertension. The pathogenesis of CSS is unknown but its association with asthma and allergic rhinitis may indicate an abnormal immune reactivity. The recently reported association with antineutrophil cytoplasmic antibodies with antimyeloperoxidase specificity may suggest a their role in the pathogenesis of the disease. An important role may be played by eosinophils, too. The main therapy is that with corticosteroids, possibly in association with immunosuppressive drugs.
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PMID:[Churg-Strauss syndrome. Personal caseload and review of the literature]. 793 57

A 33 year-old man with undiagnosed neuropathy showing mental retardation and involuntary movements has been nourished for a long period by total parenteral nutrition (TPN) because of frequent vomiting and repeated aspiration pneumonitis. After ten months' TPN, macrocytic anemia and neutropenia developed and iron preparation was administered without hematological improvement. Bone marrow examination revealed normocellular marrow without features of megaloblastosis and dysplasia. In some erythroblasts and immature myeloid cells, vacuoles were observed and mature granulocytes were reduced in the bone marrow. Both serum copper and ceruloplasmin were very low (12 micrograms/dl and 7mg/dl, respectively). Thus, oral administration of copper sulfate resulted in marked increase of reticulocytes and subsequent improvement of anemia and neutropenia within two months. Copper deficiency is a rare condition, but during an unusual nutrition such as TNP, hematological abnormality due to copper deficiency must be noticed to occur.
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PMID:[Anemia and neutropenia due to copper deficiency during long-term total parenteral nutrition]. 849 15

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

To clarify the problems of medical care of HIV-infected tuberculosis patients, we investigated clinical course of six cases admitted to our tuberculosis isolation ward. All cases were sputum smear positive for tubercle bacilli at the time of diagnosis of tuberculosis. HIV-positive was confirmed at the same time or soon after the diagnosis of tuberculosis in four cases. CD4+ cell count was on the average 21/mm3 on admission, and all cases were defined as acquired immunodeficiency syndrome (AIDS) by the criteria of AIDS surveillance committee in Japan. Two patients presented with miliary tuberculosis and five documented evidence for intrathoracic and/or cervical lymph node involvement. All cases but one responded well to antituberculosis drugs, and sputum smears and cultures became negative soon after the initiation of therapy. However, the patients were still needed to be hospitalized for the treatment and control of complications other than tuberculosis after sputum negative conversion, and they stayed in the isolation rooms of our tuberculosis ward for 110 +/- 49 days. During the treatment for tuberculosis, each patient developed 3 to 8 complications of HIV infection such as pneumocystis carinii pneumonia (PCP) (four cases), bacterial infection (four cases), neuropathy (four cases), and HIV encephalopathy (three cases). The last two complication worsened active daily life. White blood cell count was more likely to fall when sulfamethoxazole/trimethoprim mixture for the prevention of PCP and antituberculosis drugs were administered together. In three cases, ST mixture could not be continued, then two patients developed PCP after changing to an alternative pentamidine inhalation. Although three patients discharged from our tuberculosis ward, four died of AIDS related complications other than tuberculosis, one died of tuberculosis (multidrug-resistant M. tuberculosis strain was not documented initially but was detected five months later), one died of tuberculosis meningitis after the discharge, and one was lost because he returned to his own country. The survival time between the start of treatment and death ranged from 90 to 244 days in five cases. Integrated medical care system both for HIV and tuberculosis is warranted for the management of HIV-infected tuberculosis patients since they suffer many complication in addition to tuberculosis. A guideline of methods and duration of isolation for tuberculosis is needed for the most effective care of HIV-infected tuberculosis patients in Japan.
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PMID:[Clinical course of HIV-infected tuberculosis patients who admitted to the tuberculosis isolation ward: current problems of medical care]. 907 Oct 88

In the UK, fundamental changes are occurring to the organization and delivery of services to breast cancer patients, prompted by the Calman/Hine initiative and by the perception that outcomes in the UK compare unfavourably with the rest of Europe and North America. Breast cancer, being a common malignancy, has often been treated by general surgeons and oncologists rather than by site-specialized multidisciplinary teams. Outcome results for many of these surgeons and oncologists may be unknown, so it becomes difficult, in any particular centre, to quantify changes that arise from the reorganization of treatment services and adherence to new guidelines without first determining baseline results. A retrospective audit of the 350 patients with primary breast cancer who received radiotherapy in Velindre NHS Trust in 1988-1989 has been performed to provide a baseline against which future results can be measured following the adoption of agreed guidelines. The endpoints for this study were: the completeness of data; actual treatment received versus current guideline recommendations; locoregional recurrence rates; and radiation related morbidity. The adherence to agreed guidelines is likely to ensure more appropriate surgery of the primary disease and will limit radiotherapy compared with 1988-1989 in that much radiation to the axilla and Internal Mammary Chain and 'booster' treatment to the primary site will be avoided in the future. The incidence of brachial plexus neuropathy was consistent with the radiation doses used and an analysis of this data suggests that the adoption of the guidelines will probably result in the complete avoidance of this complication in the future. Likewise, it is expected that the incidence of soft tissue fibrosis, telangiectasia and pneumonitis should diminish, but this and the effect of the clinical guidelines on local recurrence rates remains to be demonstrated by future comparable audits.
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PMID:A detailed baseline audit of radiotherapy for primary breast cancer: old habits, new principles. 961 Aug 98

We analyzed response, side effects, and local control rates of a multimodal treatment consisting of intraarterial infusion chemotherapy (IAIC) and radiotherapy with or without surgery for patients with locally advanced or recurred breast cancer. Thirty-three patients, clinically diagnosed as stage IIB in 1, IIIA in 2, IIIB in 12, IV in 18, were treated from 1991 to 1998. Twenty-five were primary and eight were recurrent cases after surgery. IAIC started as initial treatment up to three times maximum. In most cases, doxorubicin 50 mg, cisplatin 50 mg, and mitomycin 10 mg were infused in the subclavian and/or internal mammary artery. After IAIC, patients in primary cases underwent radical mastectomy or breast conservation surgery, after radiotherapy at a total dose of 50 Gy/25 fractions/5 weeks with a boost of 10 Gy. In recurrent cases, a full dose of radiotherapy was delivered. Clinical objective and complete response rates were 78% and 9% after IAIC. Despite a high rate of residual positive margin (67%) or clinically residual carcinoma, local recurrence developed only in 2 patients (6%) and local control rates at 5 years were calculated as 89%. Bone marrow suppression was frequent, and skin vesiculation (15%) and ulceration (9%) were experienced after IAIC. Skin ulcer (6%), brachial plexus neuropathy (3%), and radiation pneumonitis (3%) occurred as late toxicity. IAIC was effective as an induction treatment and radiotherapy played a role of local control for patients with locally advanced or recurrent breast cancer.
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PMID:Intraarterial infusion chemotherapy and radiotherapy with or without surgery for patients with locally advanced or recurrent breast cancer. 1131 96

Approximately 3 to 5% of patients with chronic lymphocytic leukemia (CLL) develop an aggressive large cell non Hodgkin's lymphoma (NHL) known as Richter's syndrome (RS). RS has a poor prognosis and a response rate of < 10% with fludarabine-based or other cytotoxic combination regimens. The aim of this study was to evaluate the efficacy and toxicity of the hyperCVXD regimen in RS. Twenty-nine patients, median age 61 years (36-75) 23 males, were treated. Prior diagnosis was CLL in 26 patients, NHL in 2, and Prolymphocytic leukemia in 1. Treatment consisted of fractionated cyclophosphamide, vincristine, daunoXome and dexamethasone. Six patients (20%) died while receiving study therapy, 4 (14%) during the first cycle of whom 2 had started therapy with overt pneumonia. Grade 4 granulocytopenia occurred in all 95 cycles of therapy with a median time to recovery of 14 days. Twenty three (24%) cycles were complicated by fever, and 15 (15%) by pneumonia. Sepsis was documented in 8 (8%) cycles, and neuropathy in 5 (5%) of cycles. Twenty three patients had a platelet count < 100 x 10(9)/l prior to therapy: a greater than 50% decrease in platelet count over pre-therapy level occurred in 79% of first cycles, overt bleeding occurred in 4 (4%) of all cycles. Eleven of 29 (38%) patients achieved complete remission (CR), 4 of whom have relapsed after 5, 6, 9, and 12 months of remission. Two of 11 CR patients presented with RS without any prior CLL therapy. One patient had a partial remission. Thus the overall response rate was 12/29 (41%). Overall median survival was 10 months, 19 months in patients who achieved CR, 3 months in those who did not (p = 0.0008). A landmark analysis performed at 2 months from start of therapy comparing patients alive in CR versus patients alive but not in CR showed a median survival of 19 months versus 6 months, respectively (p 0.0017). In conclusion the hyper CVXD regimen has a relatively high response rate, significant toxicity and a moderate impact on survival in RS.
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PMID:Fractionated cyclophosphamide, vincristine, liposomal daunorubicin (daunoXome), and dexamethasone (hyperCVXD) regimen in Richter's syndrome. 1169 97

The purpose of this article was to review the patterns and morbidity of regional recurrence (RR) in patients with early breast cancer, efficacy of salvage therapy for RR, and complications of regional nodal treatment. A retrospective evaluation of 1,158 patients with stage I or stage II breast cancer treated with conservative surgery and radiotherapy (RT) between 1979 and 1994 was performed. Seven hundred fifty patients underwent axillary surgery, and 229 patients received RT as their only treatment of the regional lymphatics. Regional nodal RT was given to 168 patients who also had axillary surgery. The regional lymphatics of 11 patients were not treated. The patterns and morbidity of RR, relapse management, and complications related to regional nodal treatment were reviewed from the patients' records. With a median follow-up of 88 months, a total of 31 patients (2.7%) developed a RR. Nine of 31 patients (29%) with an RR experienced significant morbidity, including pain, fungating tumor, dysphagia, dyspnoea, and/or sensory motor changes at diagnosis. Nineteen patients (61%) had symptomatic residual or progressive regional disease after salvage therapy at last follow-up or death. Six of nine patients (67%) who developed an isolated axillary recurrence and underwent salvage surgery had no further axillary recurrence. The addition of regional nodal RT to breast irradiation significantly increased the incidence of symptomatic pneumonitis (1% without regional nodal RT and 4% with regional nodal RT, p < 0.001). Combined axillary dissection and nodal irradiation resulted in a significantly higher incidence of arm edema compared with either alone (9.5% with axillary dissection, 6.1% with RT to the axilla and supraclavicular fossa, and 31% with combined modality therapy, p < 0.001). Five of 380 patients (1%) who received RT to the axilla and/or supraclavicular fossa developed a transient brachial plexus neuropathy. Although RR was uncommon in patients treated with axillary surgery and/or regional nodal irradiation, salvage therapy failed to eradicate the recurrence in approximately two thirds of the patients with a RR. Ongoing research is essential to optimize regional control with an acceptable level of risk of treatment complications. Sentinel lymph node biopsy, if validated as an accurate method of staging the axilla in patients with breast cancer, would allow selective avoidance of regional nodal treatment and hence the associated morbidity.
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PMID:Competing considerations in regional nodal treatment for early breast cancer. 1185 56

As commonly used, adjuvant paclitaxel after doxorubicin in high-risk breast cancer patients results in a prolonged delay of the onset of radiation therapy after breast-conserving surgery. Concurrent delivery of breast irradiation with paclitaxel would allow for earlier initiation of radiation. We report on the toxicity of concurrent paclitaxel and breast irradiation after doxorubicin and cyclophosphamide. Twenty-four patients were treated with concurrent breast radiation and paclitaxel. All patients received four cycles of doxorubicin and cyclophosphamide followed by four cycles of paclitaxel, 175 mg/m2 every 3 weeks. The radiation therapy started after the first cycle in 3 patients, after the second cycle in 16, and after the third in 5. The breast received 4680-5040 cGy external beam irradiation, followed by a boost of 1000-2000 cGy. Fifteen patients received supraclavicular irradiation, and a posterior axillary supplement was used in five patients. Median follow-up after completion of irradiation was 11.5 months (range 2-29 months) with 21 patients followed >or=6 months, 12 followed >or=12 months, and 7 followed >or=18 months. Using Radiation Therapy Oncology Group (RTOG) acute toxicity scoring criteria, 7 patients experienced grade 1 skin and/or soft tissue reactions and 17 patients had grade 2 reactions. The average total duration of radiation treatment was 49 days (range 41-57 days). Only eight patients had radiation therapy interruptions for a median of 3.5 days (range 2-8 days): two more than 5 days. None had a chemotherapy dose reduction. One patient discontinued paclitaxel after the third cycle due to bilateral upper extremity neuropathy. No cases of pneumonitis or brachial plexopathy were seen. Concurrent treatment with every 3-week paclitaxel and breast irradiation was well tolerated. Additional study is needed to determine optimal timing, long-term toxicity, and potential benefits of concurrent radiation therapy and paclitaxel.
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PMID:Breast-conserving therapy with adjuvant paclitaxel and radiation therapy: feasibility of concurrent treatment. 1260 78

Pontocerebellar hypoplasia type 1 is a rare disease characterized by pontocerebellar hypoplasia and anterior horn cell degeneration. The oldest reported child died at the age of 26 months. Two siblings were diagnosed with pontocerebellar hypoplasia type 1 after the death of the second sibling at 40 months of age from respiratory failure and the unexpected finding of anterior horn cell degeneration on her autopsy. The older sibling was a boy who was labeled as having cerebral palsy. He died at 14 months of age from pneumonia following a clinical course similar to his sister's, who was born 5 years after his death. Both siblings had significant global developmental delay with axial and peripheral hypotonia initially. Peripheral hypertonia with brisk reflexes developed later but were absent prior to death. Extensive investigations in the second sibling ruled out known metabolic (including congenital disorders of glycosylation) and mitochondrial diseases using skin fibroblast cultures and enzyme analysis. Genetic testing for Friedreich's ataxia; neuropathy, ataxia, and retinitis pigmentosa (NARP); spinal muscular atrophy; and spinocerebellar ataxia type 1, 2, 3, 6, 7, and 8 gene abnormalities was negative. The elecroretinogram showed a previously unreported finding of abnormal and progressive rod/cone response. Our cases provide clinical and previously unreported electroretinographic evidence for neurodegeneration in pontocerebellar hypoplasia type 1 and call for the expansion of the disease phenotype.
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PMID:Pontocerebellar hypoplasia type 1: new leads for an earlier diagnosis. 1273 47

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