Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

More than a dozen primary hematologic disorders have been associated with ischemic stroke. Inherited deficiencies of antithrombin III, protein C, and protein S have been linked with stroke in case reports; optimal screening requires functional as well as antigenic assays. Antiphospholipid antibodies and lupus anticoagulants are the most frequently identified acquired states associated with ischemic stroke. Polycythemia vera, sickle cell anemia, sickle-C disease, and essential thrombocythemia are the major disorders of formed blood elements causing stroke. Special, step-wise screening for occult prothrombotic entities in stroke patients is recommended for young persons with stroke of uncertain cause, for those with prior venous thrombosis, for those with a family history of unusual thrombosis, and for those with no other explanation for recurrent stroke. Acquired, perhaps transient, abnormalities of platelets, coagulation inhibition, and fibrinolysis may contribute importantly to brain ischemia in synergy with other mechanisms, but at present these remain ill-defined. The contribution of prothrombotic diatheses to stroke is probably underrecognized and warrants further investigation.
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PMID:Hematologic disorders and ischemic stroke. A selective review. 186 63

Doppler blood velocity waveforms were analyzed from the umbilical artery as an indication of fetal well-being. Since the ratio of systolic to diastolic peak flows (A/B) reflects placental vascular resistance, an abnormality of this value may accurately predict compromised fetuses. Twenty-one studies were performed on 13 patients, using a new duplex system for real-time imaging and range-gated pulsed Doppler analysis, and compared to normal standards. Thirteen studies done in nine uneventful pregnancies were consistently normal. However, abnormal studies were found in the four patients reported as case histories, including women with sickle cell anemia, systemic lupus, diabetes, and growth retardation. The A/B ratio was felt to have predicted potential fetal compromise, including the two fetal deaths. Therefore, with certain cautions, we feel that this rapid, non-invasive technique is useful as a serially applicable reflector of the status of fetoplacental circulation.
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PMID:Doppler blood velocity waveforms in the umbilical artery as an indicator of fetal well-being. 392 Feb 77

Modern contraceptive methods are discussed, with special emphasis on oral contraceptives, which are regarded as the most effective. They are also regarded as generally safe, although there are contraindications and the drugs should only be prescribed after careful examination. The need for selecting the drug most suitable for the individual patients, mainly on the basis of the characteristics of the menstrual cycle (suggesting a predominance of estrogen or progestin, within safety limits, such as 50 mcg of estrogen), is emphasized. The examinations required include a general clinical, gynecological, and breast examination, cytology tests, evaluation of the menstrual flow pattern, measurements of arterial pressure, weight, glucose, cholesterol and triglyceride levels, and urine tests. They should be repeated at 6-month intervals, or 3-month intervals in the case of high-risk patients (varicose veins, obesity, heavy smokers, high cholesterol and triglyceride levels, history of jaundice, slight heart condition, clinical or potential diabetes, porphyria or predisposition to uterine myoma). Oral contraceptives are contraindicated in cases presenting a history of thromboembolism, phlebitis, cerebral apoplexy; sickle cell anemia, which indicates a predisposition to thromboembolic accidents; serious liver disease or recent hepatitis; serious heart disease; hormone-dependent neoplasia (breast cancer); predisposition to uterine cancer; erythematous lupus; metorrhagia of unknown origin; psychic disorders, especially of a depressive type. They should also be avoided for 3-4 years after puberty, in order to avoid interfering with the development of the hypothalamus and with growth. A carcinogenic effect of the pill and an increase in the risk of giving birth to abnormal children can be ruled out, although the incidence of abortions due to chromosome anomalies after suspending treatment is rather high (due to the previous inhibition of ovulation, a situation similar to repeated pregnancies at short intervals, which involve the same risk).
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PMID:[Current clinical problems of contraception]. 502 53

The salmonella group of bacteria consists of at least 1700 subtypes and causes widespread gastroenteric disease throughout the world, there being approximately two million cases yearly in the United States alone. An uncommon but interesting complication is the hematogenous lodgment of organisms in other sites of the body with resultant localized abscess formation, often months or years later. We present a review of 15 cases seen in our affiliated hospitals over a nine-year period. Remote salmonellosis most often occurs in patients with underlying systemic disease, such as lupus, malignancy, diabetes, and sickle cell disease, or in association with therapy that suppresses host resistance including steroids and cancer chemotherapy. The most frequent sites of involvement are bone marrow, joints, meninges, pleura, and blood vessels, or in an area of locus minoris resistentiae. The latter include scars, hematomas, fracture sites, prosthetic devices, and neoplasms. Our experience would suggest that these infections, which can remote both in location and time from the original gastrointestinal episode, are perhaps more frequent today than is generally realized. Diagnosis is often delayed because of lack of familiarity with this entity. Treatment consist of either drainage or excision of infected tissue often combined with long-term antibiotics.
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PMID:Remote salmonellosis: surgical masquerader. 707 24

Bone marrow necrosis (BMN), defined morphologically by destruction of hematopoietic tissue, including the stroma, with preservation of the bone, is a rare syndrome. The conditions in which it is seen include sickle cell disease, acute leukemia, metastatic neoplasia, and bacterial infection, particularly when hypovolemia and septic shock are present. BMN is also associated with disseminated intravascular coagulation (DIC) following irradiation and antineoplastic therapy. The antiphospolipid syndrome (APS) is characterized by antibodies directed against the antiphospolipid substrate. Because this substrate is prominently involved in the coagulation cascade and widely distributed on cell walls, patients present with venous or arterial thromboses, recurrent abortion, thrombocytopenia, and Coombs' positive hemolytic anemia, typically with raised anticardiolipin antibodies or a diagnostic lupus anticoagulant test. BMN does not appear to have been previously recognized in this context. We report what we believe to be the first such case and suggest that the high titers of antibodies present may have played a central role in its pathogenesis.
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PMID:Extensive bone marrow necrosis associated with antiphospholipid antibodies. 777 73

The activated protein C resistance (APC-R) ratios in 50 patients with steady state homozygous sickle cell (SS) disease and 59 healthy AA controls was measured. There was a significant reduction in median APC-R ratio in sickle cell disease compared to controls. This reduction in APC-R ratio was not explained by (1) the presence of the factor V Leiden, found in only one of 165 patients with SS disease including those tested for APC-R, or (2) the presence of lupus anticoagulants. However, the raised levels of factor VIIIC in SS patients in this study may be contributing to increased resistance to APC, which in turn may contribute to the vaso-occlusive complications of SS disease.
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PMID:Activated protein C resistance in homozygous sickle cell disease. 907 31

The non-healing leg ulcer is examined by discussing three disease processes: peripheral vascular occlusive disease (PVOD), chronic venous insufficiency (CVI), and vasculitis. For PVOD, management decisions are based on risk factors and disease history. Comprehensive management includes the discontinuation of smoking, exercise conditioning and regulation of diabetes, hyperlipidemia, hypertension, and the appropriate application of anticoagulant/antiplatelet drugs. Methods of surgical management include bypass with autogenous or synthetic material in addition to reconstructive surgery with patch angioplasty or extra-anatomic bypass, amputation, percutaneous transluminal angioplasty/stents, thrombolytic infusion, atherectomy, intraluminal ultrasound, and angioscopy. The optimal healing environment for all ulcers prevents contamination, pain, and fluid loss. In CVI, higher venous pressure in the veins of the lower limb during exercise results in ambulatory venous hypertension and ulceration. Various theories are associated with the disease and ulceration process; the classic treatment of elevation, ambulation, and compression for venous disease remains unchallenged. Diagnosis is based on history, physical examination, invasive venography, and/or non-invasive studies. Two groups of vasculitic disorders that share varying degrees of vascular inflammation and necrosis are arteritis (lupus, erythematosus, periarteritis nodosa, dermatomyositis) and blood dyscrasias (sickle cell disease, thalassemia). Leg ulcers associated with vasculitis are due to inadequate tissue oxygenation at the local level, are typically chronic, slow to heal, and commonly recur.
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PMID:The non-healing leg ulcer: peripheral vascular disease, chronic venous insufficiency, and ischemic vasculitis. 939 80

Antiphospholipid antibodies were investigated in 37 individuals with sickle cell disease and compared to a control group of 30 healthy subjects. Sickle cell patients included 18 homozygous sickle cell patients, 8 S/beta thalassemic patients and 11 sickle cell trait subjects. In all individuals, antiphospholipid antibodies were explored by lupus anticoagulant (LA) detection and the quantification of IgG and IgM anticardiolipin (aCL) isotypes, total antiphospholipid antibodies (APA) and IgM, IgG and IgA antiphospholipid classes. In homozygous sickle cell patients, mean level of IgG aCL and total APA were significantly increased (17.02 +/- 8.88 GPL/ml, p < 0.05 and 10.64 +/- 10.58 UPL/ml, p < 0.05 respectively). The IgG aCL, total APA and LA frequencies were 22.2%, 44.4% and 62.2%, respectively. APA isotypes were mostly IgG or IgG and IgA. In S/beta thalassemic patients, mean levels of APA were significantly increased (10.81 +/- 7.82 UPL/ml, p< 0.05). Their frequency was 71.4% and they were mostly IgG or IgG and IgA. In patients with sickle cell trait, mean levels of APA were significantly increased (10.84 +/- 5.84 UPL/ml, p < 0.01). Their frequency was 72.7% and mostly of IgG isotype. Our study showed a close association between high APA levels and sickle cell syndrome, however there was no relationship between high levels of antiphospholipid antibodies and the major complications of sickle cell disease.
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PMID:Antiphospholipid antibodies: lupus anticoagulants, anticardiolipin and antiphospholipid isotypes in patients with sickle cell disease. 969 18

This article reviews several different articles which have contributed to an understanding of the harmful or beneficial effects of oral contraceptives (OCs) on various diseases. The Royal College of General Practitioners study found that current OC users compared to women who had never used OCs had relative risks of .52 for menorrhagia, .37 for dysmenorrhea, .65 for irregular cycles, .72 for intermenstrual bleeding, and .71 for premenstrual syndrome. Several studies found combined OCs to offer protection against ovarian cysts. Microdose progestin only pills did not ameliorate most menstrual problems and aggravated ovarian cysts. Despite some theoretical grounds for suspecting an association between pituitary prolactinomas and OC use, recent studies have failed to find an increased relative risk for prolactinomas in women using OCs for contraceptive purposes, although 1 study found an increased risk in women using OCs for cycle control. 1 study reported 11 pregnancies in 30 diabetic women in 15 months of IUD use; the high rate was attributed to abnormal patterns of mineral deposit on the IUD surface. The 11 pregnancies occurred with 5 Gravigardes, 5 Saf-T-Coils, and 1 Dalkon Shield. Other studies on the contrary have noted no difference in pregnancy rates among 103 diabetic women using Copper Ts or 118 diabetic women using Lippes loops. Combined OCs appear to reduce the incidence of rheumatoid arthritis by 1/2 among current OC users and to protect former users as well. Combined OCs aggravate lupus erythmatous but synthetic progestins alone are effective without aggravating the condition. It has recently been argued that low dose OCs are not contraindicated in cases of sickle cell disease and may even offer protection against thromboembolic vascular accidents for women with sickle cell anemia. Estimates of relative risk of pelvic infection among IUD users vary from 1.5 to 6.5, with the risk apparently greatest for women under 25. Recent studies have indicated that copper IUDs do not have the bactericidal power formerly attributed to them. Numerous in vitro studies and statistical comparisons of the effect of spermicides in vivo have demonstrated that local methods provide protection against sexually transmitted diseases. OCs may favor vaginal infection, but some recent studies have indicated that they offer protection against pelvic infections. The protective effect of the condom against sexually transmitted diseases is well known. It has been estimated that, relative to non-users of OCs, each 100,000 users will have 235 fewer cases of benign breast disease, 35 fewer of ovarian cysts, 320 fewer of iron deficiency anemia, 600 fewer of pelivc infection, 117 fewer of extrauterine pregnancy, 32 fewer of rheumatoid arthritis, 1 fewer of endometrial cancer, and 3 fewer of ovarian cancer.
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PMID:[Disease and contraception. Recent aspects]. 1228 Feb 11

All 302 children treated at the rheumatology clinic of a children's hospital in Santo Domingo between September 1985 and September 1986 were included in a prospective study of the causes of joint pain in children. The 137 girls and 165 boys were grouped in five categories according to the underlying condition. The largest group, reactive arthritic conditions, affected 78 patients (25.9%). 72 of the 78 had rheumatic fever. The second largest category, hematological processes, affected 75 patients (22.6%); 40 of the 75 patients had sickle cell disease, 25 had hemophilia or other conditions, and 8 had neoplasias. 63 patients (20.8%) had infectious processes, including 33 with septic arthritis, 17 with abscesses and cellulitis, 7 with arthritis and osteomyelitis, and 6 with osteomyelitis. Prostration and pain on movement were more pronounced in patients with septic arthritis. 42 children (12.6%) had collagen vascular disease. 32 had juvenile rheumatoid arthritis, 4 had lupus, 3 vasculitis, 2 dermatomyositis, and 1 each had scleroderma and erythum nodosum. 25 patients (8.2%) had a miscellaneous array of other conditions. Sickle cell disease or neoplasia were most likely when anemia was also present. Extreme prostration and signs of inflammation suggested infectious processes. The small joints were primarily affected in juvenile rheumatoid arthritis and sickle cell disease.
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PMID:[Causes of joint pain in children]. 1229 May 50


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