Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 21-year-old woman is reported with aplastic anaemia, who presented with pain in the leg. Rapid loss of sciatic nerve function followed. MRI showed irregular streaks of low intensity in the muscles of the pelvic region. A diagnosis of sciatic neuritis as initial symptom of clostridial myonecrosis was made.
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PMID:Sciatic neuritis as initial symptom of spontaneous clostridial myonecrosis. 165 97

Chemoradiotherapy-induced toxicity following unmodified allogeneic marrow grafting was studied. Patients with hematologic malignancy (n = 157) received cyclophosphamide (120 mg/kg) followed by single or fractionated total body irradiation (TBI); aplastic anemia patients (n = 41) received only cyclophosphamide (200 mg/kg). Physicians rated mucositis, pain and nausea daily as (0) none, (1) mild, (2) moderate, (3) severe, (4) life threatening. Oral mucositis pain began several days prior to transplant, peaked during the second week after transplant, and declined thereafter. Patients with hematologic malignancies (maximum mean rating of 1.6, day 11) experienced more pain than aplastic anemia patients (maximum mean rating of 0.7, day 6). Nausea peaked before transplant and gradually declined. Nausea was higher (p less than 0.001) in patients with aplastic anemia (maximum mean rating of 1.3, day -2) than with hematologic malignancies (maximum mean rating of 0.9, day -6). There was no significant difference between single dose 1000 cGy and 6-day 1200 cGy irradiation. Recipients of 1575 cGy rather than 1200 cGy TBI had significantly (p less than 0.01) higher levels of pain (mean rating 1.25 and 0.82, respectively) and nausea (mean rating 1.27 and 0.72, respectively). Additional research is needed to determine the predictors, consequences and best methods of controlling these toxicities.
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PMID:Chemoradiotherapy toxicity during bone marrow transplantation: time course and variation in pain and nausea. 265 Jul 88

Our data show that 1% of patients who required hospital treatment did so due to severe adverse reactions to analgesics. The most frequent adverse reaction was major gastrointestinal bleeding after aspirin, indomethacin, phenylbutazone or naproxen. Thrombocytopenia, second in frequency, was also mainly a complication of aspirin treatment, as was severe vertigo and tinnitus. Allergic reactions and leucopenia or agranulocytosis occurring in single cases only were associated with the use of pyrazolones. Patients with nephropathy were usually taking phenacetin or one of the close derivatives paracetamol or bucetin. Intensive monitoring for adverse reactions to drugs in 6,000 hospitalised patients in medical wards showed that analgesics, although frequently used, did not lead to life-threatening reactions. Gastrointestinal and neurological side effects were the most commonly observed reactions and these occurred more often after aspirin, indomethacin or pentazocine than after dipyrone or tilidine. Preliminary data of an international case-control-study on agranulocytosis and aplastic anaemia suggest that the incidence of agranulocytosis was in the order of 2 to 3 per million users of analgesics per year. Agranulocytosis occurred predominantly with pyrazolones, with a mortality of 1 to 2 per 10 million users per year. A cohort study on the treatment of colic pain in general practice showed that serious events most likely due to adverse reactions to analgesics were bronchospasm, shock fragments or shock. The incidence of these serious events was about 2 in 1,000 treated cases. The relative risk was not increased by treatment with pyrazolones, opioids or other drugs.
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PMID:Rare but serious risks associated with non-narcotic analgesics: clinical experience. 382 33

26 patients, 13 male and 13 female, with paroxysmal nocturnal haemoglobinuria (PNH) are described. The diagnosis was based on the finding of a positive Ham's test. PNH developed in 4 patients with aplastic anaemia, and 3 patients with established PNH developed marrow hypoplasia during the course of the disease. In 2 cases autoimmune haemolysis was also present; this association has not been described previously. The majority of patients presented with anaemia and dark urine, or with evidence of thrombosis. A high index of suspicion was needed to avoid missing the diagnosis. Haemolytic crises were usually precipitated by infection, and renal failure requiring dialysis sometimes resulted; a positive direct antiglobulin test was often found at times of increased haemolysis. Thromboses were the most frequent complication, and when intra-abdominal vessels were affected, pain was particularly troublesome. The disease had a widely variable course; 4 patients made a complete recovery and 10 died, 8 from thrombotic complications and 2 from infections associated with marrow hypoplasia. Survival ranged from 1 year to 30 years and the median survival in those who died was 3.5 years.
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PMID:Paroxysmal nocturnal haemoglobinuria. A clinicopathological study of 26 cases. 642 34

Studies of the composition of coal tar, which began in Prussia in 1834, profoundly affected the economies of Germany, Great Britain, India, and the rest of the world, as well as medicine and surgery. Such effects include the collapse of the profits of the British indigo monopoly, the growth in economic power of Germany based on coal tar chemistry, and an economic crisis in India that led to more humane tax laws and, ultimately, the independence of India and the end of the British Empire. Additional consequences were the development of antiseptic surgery and the synthesis of a wide variety of useful drugs that have eradicated infections and alleviated pain. Many of these drugs, particularly the commonly used analgesics, sulfonamides, sulfones, and local anesthetics, are derivatives of aniline, originally called "blue oil" or "kyanol." Some of these aniline derivatives, however, have also caused aplastic anemia, agranulocytosis, and methemoglobinemia (that is, "blue people"). Exposure to aniline drugs, particularly when two or three aniline drugs are taken concurrently, seems to be the commonest cause of methemoglobinemia today.
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PMID:Blue gods, blue oil, and blue people. 806 94

A 29-year-old woman with aplastic anemia who complained of visual disturbances and pain in the right eyeball was diagnosed as having cytomegalovirus (CMV) retinitis based upon the characteristic retinal changes and isolation of CMV. She received treatment with ganciclovir (GCV), and the retinitis initially responded for several months. However, the patient was found to have CMV lesions in the left eye followed by neurological symptoms. The CMV isolated just before her death was approximately 20 times more resistant to GCV than that isolated previously, suggesting that the GCV-resistant CMV had developed during the long-term treatment with GCV.
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PMID:Progressive retinitis-encephalitis due to ganciclovir-resistant cytomegalovirus associated with aplastic anemia. 921 78

A subset of 299 patients with homozygous sickle cell anaemia, enrolled in the cohort of the French Study Group on sickle cell disease (SCD), was investigated in this study. The majority of patients were children (mean age 10.1 +/- 5.8 yr) of first generation immigrants from Western and Central Africa, the others originated from the French West Indies (20.2%). We report the frequency of the main clinical events (mean follow-up 4.2 +/- 2.2 yr). The prevalence of meningitis-septicaemia and osteomyelitis was, respectively, 11.4% and 12% acute chest syndrome was observed in 134 patients (44.8%). Twenty patients (6.7%) developed stroke with peak prevalence at 10-15 yr of age. One hundred and seventy-two patients (58%) suffered from one or more painful sickle cell crises, while the others (42.5%) never suffered from pain. The overall frequency of acute anaemic episodes was 50.5%, (acute aplastic anaemia 46%; acute splenic sequestration 26%). A group of 27 patients were asymptomatic (follow-up > 3 yr). Epistatic mechanisms influencing SCD were studied. Coinherited alpha-thalassemia strongly reduced the risk of stroke (p <0.001) and increased that of painful crises (p < 0.02). There was a low prevalence of Senegal and Bantu (CAR) betas-chromosomes in patients with meningitis (p <0.04) and osteomyelitis (p < 0.03). Prevalence of Senegal betas-chromosomes was lower in the asymptomatic group of 27 patients (p < 0.02). The patients come from a population of unmixed immigrants in whom the beta-globin gene haplotype strongly reflects the geographic origin and identifies subgroups with a homogenous genetic background. Thus the observed effects might result more from differences in as yet unidentified determinants in the genetic background than from the direct linkage with differences in the beta-globin gene locus.
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PMID:Acute clinical events in 299 homozygous sickle cell patients living in France. French Study Group on Sickle Cell Disease. 1100 50

The incidence and clinical and magnetic resonance imaging features of osteonecrosis of the hip were evaluated in patients with aplastic anemia. Two hundred and forty-one patients with aplastic anemia were examined using MR imaging of bone marrow during the five years from 1994 to 1998. Osteonecrosis of the hip was observed on MR imaging in nineteen (15 males and 4 females, mean age 35 yr) of the 241 patients. It was present in both hips in 14 patients, and there were five cases with unilateral occurrence, with a total of 33 involved hips. All except for five hips with associated bone marrow edema revealed increased fatty marrow conversion in the proximal femoral metaphysis. In nine patients, osteonecrosis was detected without any pain. Five patients already had osteonecrosis before any medication was administered. Twelve patients received antilymphocyte globulin, and seven patients received a low dose of steroids before the MR diagnosis of osteonecrosis. Osteonecrosis of the hip frequently develops in patients with aplastic anemia (7.9%), associated with fatty marrow conversion of the proximal femoral metaphysis.
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PMID:Osteonecrosis of the hip in patients with aplastic anemia. 1248 6

Fetal tissue is the richest source of primordial stem cells and has several properties that make it particularly useful for transplantation. It is superior to adult (mature) tissue in certain respects. First, fetal cells are capable of proliferating faster and more often than mature, fully differentiated cells. This means that these donor cells are able to quickly reverse the lost function of the host. In addition, these fetal cells can often differentiate in response to the environmental cues around them. This is because of their location--they can grow, elongate, migrate, and establish functional connections with other cells around them in the host. It has been found that fetal tissue is not easily rejected by the recipient due to the low levels of histocompatibility antigens in the fetal tissue. At the same time, angiogenic and trophic factors are at high levels, enhancing their ability to grow once they are transplanted. Since early fetal hematopoietic tissue lacks lymphocytes, graft vs host reactions are minimized. Fetal cells tend to survive excision, dissection, and grafting better because they generally do not have long extensions or strong intercellular connections. Finally, fetal tissue can survive at lower oxygen levels than mature cells. This would make them more resistant to the ischemic conditions found during transplantation or in vitro situations. Studies on fetal cell/tissue transplant have been encouraging. Fetal tissue can be used in different indications, for instance, fetal liver transplants may be used in combating aplastic anemia, placental umbilical cord whole blood transfusion can serve as an emergency alternative to adult whole blood transfusion, fetal adrenal transplant has been tried in combating intractable pain in arthritis, and fetal thymic transplant in combating leucopenia in non-Hodgkin's lymhoma and other immunodeficiency conditions like DiGeorge Syndrome, only to name a few. Fetal brain tissue transplant has also been done in a heterotopic site and the proliferation of the tissue has been observed. Neurotransplantation with fetal tissue in Parkinsonism shows positive results in some globally accepted studies. There are futuristic potential uses of fetal tissue in bioengineering through coating/seedling of fetal tissue on implants, stents and other artificial surgical life-saving devices to improve their functioning, and it may also extend the life of these costly gadgets. By properly using pre-HLA fetal tissue seedling in orthopedic, thoracic and also neurosurgical appliances, there could be a reduction of long-term irritation sequelae of the implant and the host interphase, and thus, a better device, i.e., a more biofriendly interphase could be developed. This may help in the reduction of pseudomembrane formation, loss of patency and other resultant TH2 reactions of the host system.
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PMID:Fetal cell/tissue therapy in adult disease: a new horizon in regenerative medicine. 1549 Oct 58

We analyzed the records of 153 Guadeloupean children with sickle cell anemia (SCA), for whom clinical and laboratory data were prospectively collected (mean follow-up duration 8.4 +/- 4.6 yr). Prevalence and age-specific frequencies of acute clinical events were determined and correlations between complications, hematological parameters and potential modulating factors investigated. Painful crisis and acute chest syndrome (ACS) were the two most common complications, affecting 65.4% and 58.8% of the patients, respectively. The frequency of acute anemia was 49.7% (acute splenic sequestration 24.8%; acute aplastic anemia 15.0%). Prevalences of septicemia-meningitis and osteomyelitis were 15.7% and 16.3%, respectively. A higher incidence of infections, painful crises and acute anemia was detected in patients who developed ACS. The well-documented protective effect of HbF level on the overall disease expression was observed with higher HbF level in asymptomatic than in symptomatic patients (17.5% +/- 8% vs. 9.9% +/- 6.4%, P = 0.01) with similar ages and sex ratio. It was also confirmed on ACS and, for the first time, further extended to acute anemic events and septicemia. Besides its effect on hematological parameters, alpha-thalassemia seems to have little impact on the prevalence of complications, as do beta(S)-globin haplotypes. Comparison with other series suggests that the natural history of SCA in Guadeloupe is more similar to that in Jamaica with regard to those reported in Europe and the United States, suggesting a potential impact of environmental factors on the clinical course of the disease.
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PMID:Sickle cell anemia in Guadeloupean children: pattern and prevalence of acute clinical events. 1645 94


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