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Query: UMLS:C0032285 (pneumonia)
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Clinical studies have long suggested the presence of a specific cardiomyopathy in sickle cell anemia secondary to intracoronary thrombosis and subsequent infarction. Fifty-two autopsy patients were studied (48 with SS hemoglobin, 4 with S-C or S-Thal hemoglobin) to ascertain the range of cardiac pathologic abnormalities associated with this disease. The average age was 17 years (range 1 month to 48 years). Renal failure and infection were the most common causes of death; the former was a more common cause in adults than in children. Right and left ventricular hypertrophy and dilatation were the most common abnormal pathologic findings. No evidence of recent or remote myocardial infarction, coronary thrombosis or arteritis was noted in any patient. Eight patients who were studied with postmortem coronary arteriograms exhibited markedly increased coronary arterial caliber with no evidence of atherosclerosis. Seventeen of the 52 patients studied had clinical evidence of congestive heart failure before death. Of these 17 patients, 7 had moderate to severe left ventricular hypertrophy associated with chronic renal failure and hypertension, 2 had right ventricular hypertrophy with organized pulmonary thrombosis, 2 had rheumatic mitral valve disease and 2 died during the second trimester of pregnancy. Two of the 17 patients thought to have pulmonary edema before death in fact had aspiration pneumonia and hemorrhagic pneumonitis, respectively. The data suggest that cardiac dysfunction in sickle cell anemia can usually be explained by the adverse effect of coexisting disease on the diminished cardiac reserve of chronic anemia. The data do not support the concept of a specific "sickle cell cardiomyopathy".
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PMID:Clinicopathologic analysis of cardiac dysfunction in 52 patients with sickle cell anemia. 15 Jul 86

In ten experiments, 53 castrated male 4- to 8-week-old weanling pigs were given adriamycin (ADR) IV at mean dosages of 0.64, 1.0, 1.6, 3.2, or 6.4 mg/kg/week at various frequencies for up to 20 weeks. Mortalities in pigs given these dosages were 0% after 112 days, 100% after 134 days (survival time was 48 to 134 days), 91% after 75 days (survival time was 5 to 75 days), 100% after 28 days (survival time was 23 to 28 days), and 100% after 14 days (survival time was 10 to 14 days), respectively. Survival time was prolonged in younger pigs and in pigs given smaller but more frequent dosages of ADR. Characteristic gross and histopathologic alterations of ADR toxicosis were observed in pigs given 1.0, 1.6, 3.2, or 6.4 mg/kg/week mean dosages. The most frequent lesions were in the alimentary tract, myeloid and lymphoid tissues, skin, and perivascular tissues at injection sites. Alimentary tract lesions were mucosal epithelial atrophy, with secondary fibrinonecrotic inflammation in the oral cavity and large intestine. Marked hypoplasia was seen in bone marrow and lymphoid tissues, with frequent terminal hemorrhagic diathesis and septicemia. Several days before death, the pigs developed severe dermatitis over the ventral portion of the abdomen and inner surfaces of the limbs. Perivascular necrosis and cellulitis produced by extravasation of ADR was a frequent complication of treatment. Terminal severe acute pneumonia occurred in most pigs. Pericarditis or cardiomyopathy (or both) developed in 14 pigs, given 0.64, 1.0, or 1.6 mg/kg each week (mean cumulative dosage 520.5 mg/m2 of body surface). Characteristic histopathologic and ultrastructural alterations in affected cardiac muscle cells were vacuolar degeneration, myocytolysis, and hyaline necrosis. Nephrotoxicosis also was in pigs with chronic ADR toxicosis. Systemic antibiotic treatment did not prolong survival of ADR-treated pigs in two experiments, but did in one other experiment.
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PMID:Pathologic features of adriamycin toxicosis in young pigs: nonskeletal lesions. 52 72

The case history of seven children aged 1 5/12 to 5 9/12 years with non tuberculous bacterial pericarditis, observed in the last 8 years at the University children's hospitals of Basle, Berne and Zurich is reported. The history showed febrile illness of 3--14 days duration, which led to an admission diagnosis of pneumonia, angina or pseudocroup. From the signs of heart failure and cardiomegaly on chest X-ray the differential diagnosis of myocardial disease or pericardial effusion was made. The ECG-changes were uncharacteristic, and a friction rub and pulsus paradoxus was encountered once only. The effusion diagnosis should preferably be substantiated by a non-invasive method (scintigram, echocardiogram) as diagnostic pericardiocentesis does often not allow to aspirate the thick pus through the needle. Diagnostic and therapeutic surgical pericardiotomy with consecutive drainage is therefore mandatory. Halothane should be avoided as an anesthetic for this procedure of hemodynamic reasons. With surgery and antibiotics the recovery rate in our series was 100%, and no pericardial constriction was observed on follow-up 1 to 8 years later.
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PMID:Pericarditis purulenta in children. 61 70

A questionnaire was sent to 1,041 owners of fiberoptic bronchoscopes requesting data about complications of bronchoscopic examination; 323 (31 percent) of the questionnaires were returned. From approximately 48,000 procedures, ten deaths were reported and are described. Information about two additional deaths not obtained from data on the questionnaires is also included. All patients who died had either myocardial disease, severe chronic pulmonary disease, serious pneumonia, or cancer. Two deaths were associated with evidence on necropsy of fresh myocardial infarctions that had been unsuspected prior to the procedure. Two deaths occurred after administration of local anesthesia prior to bronchoscopic examination. Two were patients who previously had slowly hemorrhaging tumors that hemorrhaged massively following, respectively, forceps biopsy and saline lavage. Four brushes broke off in bronchi. Ten cardiac arrests and 41 life-threatening reactions to anesthesia also occurred.
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PMID:Deaths and complications associated with fiberoptic bronchoscopy. 127 93

This work reviews the most frequent late effects seen in long-term survivors and how they relate to individual therapeutic modalities: a) Growth: severe growth retardation is seen in patients treated by radiation therapy, related to dose, anatomical site and age of patient, along with bony abnormalities (scoliosis, atrophy or hypoplasia, osteoporosis). b) Fertility: chemotherapy, in particular alkylating agents and the methylhydrazine procarbazine, can interfere with gonadal function, especially when administered with abdomen and pelvic irradiation. This effect is often seen in Hodgkin disease. c) Cardiovascular function: the anthracyclines cardiotoxicity is well known and most commonly presents with cardiomyopathy, pericarditis or both. d) Pulmonary function: pulmonary fibrosis and recurring pneumonitis are the most common effects when more than a total dose 3000 cGy has been delivered to more than 50% of the lung. Chemotherapeutic agents (bleomycin, busulfan and many others) appear to be dose-related responsible for pulmonary disease in long-term survivors. e) Gastrointestinal function: fibrosis and enteritis are the most common pathologic abnormalities of the gastrointestinal tract, particularly after radiation therapy. The hepatotoxicity of anticancer therapy is well known: fibrosis-cirrhosis is seen after radiation therapy when a total dose between 1200 and 5800 cGy is administered, but abnormal liver function is also found after chemotherapy, being methotrexate implicated as cause of chronic hepatopathy. f) Urinary tract: hemorrhagic cystitis has been associated with cyclophosphamide and iphosfamide, but today this complication has been reduced by the use of prophylactic measures such as vigorous hydration and diuresis. Radiation in dose exceeding 2000 cGy is a well-defined cause of renal failure.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Late data in pediatric oncology]. 207 95

Two Pediatric Intergroup Ewing's Sarcoma studies of patients with metastatic disease (IESS-MD) have used multimodal therapy consisting of intensive combination chemotherapy and radiation therapy (XRT) to areas of gross disease detected at the time of diagnosis. In IESS-MD-I, conducted from 1975 to 1977, 53 eligible patients were entered and received the chemotherapeutic agents vincristine, Adriamycin (doxorubicin; Adria Laboratories, Columbus, OH), cyclophosphamide, and dactinomycin with concomitant XRT (VACA + XRT). In IESS-MD-II, conducted from 1980 to 1983, 69 eligible patients were entered and received 5-fluorouracil (5FU) in addition to the chemotherapeutic agents of IESS-MD-I; initial intensive chemotherapy was given and XRT was delayed until week 10 (VACA + 5FU, delayed XRT). The best response rate (complete and partial remissions combined) was 73% in IESS-MD-I and 70% in IESS-MD-II, so there was no statistical evidence of a difference in response rates (P = 0.62). The length of best response also was similar between studies (P = 0.79), with approximately 30% of the patients on both studies remaining in remission at 3 years. The percentage of patients surviving 5 years or more was 30 on the first study and 28 on the second study (P = 0.49). The major sites of relapse after a response were lung and bone, each occurring with nearly equal frequency. The age of the patient was related to both best response rate and survival: patients 10 years of age or younger had substantially higher response and survival rates than patients 11 years of age or older. The favorable prognosis for younger patients might be explained by a more favorable distribution of primary sites at diagnosis; 39% of patients 10 years of age or younger had rib primary sites, compared with only 16% for patients older than 10 years of age (P = 0.05). The frequency of life-threatening toxicity was substantially higher in IESS-MD-I (30%) than in IESS-MD-II (9%), but the frequency of fatal toxicity was similar (6% to 7%). Fatal complications included Adriamycin-induced cardiomyopathy, Pneumocystis carinii pneumonia, unspecified pneumonitis, and sepsis. The most common toxicity and complications were leukopenia and infections.
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PMID:Ewing's sarcoma metastatic at diagnosis. Results and comparisons of two intergroup Ewing's sarcoma studies. 220 33

Over a 10-year period 110 patients over the age of 65 years were admitted to the Burn Center, Rui Jin Hospital and 36 (32.7 per cent) died. Significant differences between the survivors and non-survivors were related to the total burn surface area and full skin thickness burn size. Among the causes of death, pre-existing cardiopulmonary diseases and associated inhalation injury were particularly important since pneumonia was considered as a primary cause of death in 13 patients, myocardial disease in two, cor-pulmonale and heart failure in two. Care of the early fluid resuscitation, early excision of deep burn wounds and grafting, prevention or treatment of a variety of life-threatening complications, and nutritional supplementation appeared to decrease the mortality of aged burn patients.
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PMID:Causes of death in aged burn patients: analysis of 36 cases. 238 62

The Xe-133 ventilation pattern in congestive heart failure (CHF) was assessed using 24 inpatient ventilation/perfusion studies performed to rule out pulmonary embolism. Patients with histories of CHF, myocardial infarction (MI), and cardiomyopathy were included in the study. Frank pulmonary edema, pulmonary embolism, and other known lung diseases such as chronic obstructive lung disease, tumor, and pneumonia were excluded. Fifteen of the 24 patients had abnormal ventilation scans. Twelve of the 15 showed bilateral basal Xe-133 retention on washout; the remaining 3 showed diffuse, posterior regional retention. On perfusion scans, 14 of the 15 abnormal ventilation patients showed evidence of CHF such as inverted perfusion gradient, enlarged cardiac silhouette, or patchy perfusion, and all of them had a history of CHF or cardiac disease. Nine of the 24 patients had normal ventilation scans, including normal washout patterns. Seven of the nine had normal perfusion (p less than 0.01). Four of the nine normal ventilation patients had a history of cardiac disease or CHF but no recent acute MI. Bilateral basal regional Xe-133 retention, coupled with perfusion scan evidence of CHF such as inverted perfusion gradient, enlarged cardiac silhouette, and patchy perfusion pattern, appears to be a sensitive and characteristic ventilation/perfusion finding in mild or subclinical CHF.
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PMID:Bilateral basal Xe-133 retention and ventilation/perfusion patterns in mild and subclinical congestive heart failure. 260 44

Interferon-alpha (IFN-a) or 2'-deoxycoformycin (pentostatin; DCF) have each been shown to be highly active in hairy-cell leukemia (HCL). In this phase II study of the Leukemia Cooperative Group of the European Organization for Research and Treatment of Cancer (EORTC), the efficacy and toxicity of DCF were investigated in patients who were resistant to IFN-a treatment. Resistance was defined as: (1) progressive disease (PD) under IFN-a therapy for more than 2 months; (2) stable disease (SD) after more than 6 months of IFN-a treatment; (3) relapse within 3 months of discontinuing IFN-a; and (4) intolerance to IFN-a because of World Health Organization (WHO) grade 3 or 4 toxicity. DCF was applied at a dosage of 4 mg/m2 weekly x 3, then 4 mg/m2 every other week x 3. Responders were given a maintenance therapy once per month for a maximum of 6 months. At the time of report, 33 patients with resistant disease were evaluable for response and toxicity. Median duration of IFN-a therapy before DCF administration was 14.7 months (range, 1 to 41 months). Complete remissions (CRs) were achieved in 11 patients and partial remissions (PRs) in 15, resulting in a total response rate of 78.8%. Median interval between beginning of DCF therapy to best response was 3.9 months with a range from 2.0 to 7.0 months. Two patients who achieved PR have relapsed 7 and 14 months after cessation of DCF therapy. The median duration of response was over 11.5 months (range, over 3.0 to over 24.0 months). Three patients died within the first 6 weeks of DCF treatment: one of drug-unrelated cardiomyopathy and two of fungal pneumonia. The patients with early death (n = 3) and nonresponsive disease (n = 4) received IFN-a treatment for a longer period (median, 18.0 months) than did the 26 responsive patients (median, 10.0 months). Major side effects included nausea, skin rash, and infections and were otherwise mild. Thus, DCF is highly active in patients with HCL resistant to IFN-a.
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PMID:Response to pentostatin in hairy-cell leukemia refractory to interferon-alpha. The European Organization for Research and Treatment of Cancer Leukemia Cooperative Group. 278 73

Forty-four heart and five heart-lung transplant recipients with cytomegalovirus (CMV) infection were investigated for risk factors associated with symptomatic CMV infection (17 patients) and CMV pneumonia (eight patients). Symptomatic infection was associated with primary rather than reactivated infection (P less than .005), younger age (P less than .005), heart-lung transplantation (P less than .001), and significant rises in titer of antibody to the early antigen of Epstein-Barr virus (P less than .001). Among recipients of heart transplants, patients with cardiomyopathy more often had symptomatic disease due to CMV (P less than .05). CMV pneumonia was associated with heart-lung transplantation and, in patients with primary CMV infection, earlier positive cultures for CMV after transplantation (P less than .02). CMV viremia was found in all patients with symptomatic infection, including the eight patients with CMV pneumonia, and the frequency of positive buffy coat cultures for CMV was significantly higher in patients with symptoms than in patients without symptoms (P less than .001). Neither symptomatic CMV infection nor CMV pneumonia was significantly associated with the use of antithymocyte globulin, restricted to therapy for rejection, and the use of high doses of acyclovir in 11 patients had no demonstrable impact on CMV culture positivity.
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PMID:Morbidity of cytomegalovirus infection in recipients of heart or heart-lung transplants who received cyclosporine. 299 55


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