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)

Vaso-occlusive crises are one of the most debilitating features of sickle cell disease. There appears to be no standardization of care for adults with pain crisis, and some commonly utilized regimens, such as those employing intramuscular meperidine, are pharmacologically unsound. Parenteral narcotic use may be associated with respiratory compromise acutely and with dependence over the long term, but nonopioid preparations are often unsatisfactory in relieving pain. We have recently enjoyed success with a combination of a parenteral nonsteroidal anti-inflammatory medication and an oral tricyclic antidepressant. We report four representative cases and review the salient points of the management of pain crisis in adult patients in the emergency department.
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PMID:Emergency department analgesia without narcotics for adults with acute sickle cell pain crisis: case reports and review of crisis management. 178 91

Pain control using intramuscular analgesia is often unsatisfactory in sickle cell patients. In a pilot study, 15 patients with sickle cell anemia (SS) and one patient with SB thalassemia in vaso-occlusive crisis were treated with the Patient-Controlled Analgesia (PCA) technique using a Pharmacia Deltec Programmable pump (CADD PCA). Age range was 19-50 years (median = 27); there were nine females and seven males. The protocol consisted of 3 days of therapy using a background of continuous infusion meperidine. The starting dose was 20 mg/hr and was escalated to 30 mg/hr. The average amount given was 25.8 mg/hr. One to two boluses of 2.5-5.0 mg/dose (mode = 5.0) were also allowed each hour. In addition, patients number 8 through 16 were given hydroxyzine (Vistaril) 50 mg PO q6h. The number of days in pain prior to study entry (mean +/- SD) was 3.3 +/- 1.6. The number of pain sites per patient was 3.6 +/- 1.2. Using categorical and analog pain scales, patients' pain scores decreased only about 30%. However, most patients were fairly satisfied with the treatment and rated it overall as follows: 1 poor, 1 fair, 3 good, 6 very good, 4 excellent, 1 no comment. Patients number 8 through 16 gave higher ratings probably because a more idealized dosage regimen was being used by that time in the study. There were no adverse effects or major problems noted. It is our impression that PCA, when optimized, will be a safe and effective alternative method for providing patients with sickle cell vaso-occlusive crisis pain relief.
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PMID:Patient-controlled analgesia in patients with sickle cell vaso-occlusive crisis. 229 91

Progressive lymphadenopathy in a previously healthy female adult with homozygous sickle cell disease (SCD) was found to be due to infection with the human immunodeficiency virus (HIV). Detailed questioning identified several risk factors for HIV in this apparently low-risk patient. Parenteral therapy and heterosexual relationships while abroad may place such SCD patients at risk of HIV infection and its sequelae. The additional risk due to the underlying immunological abnormalities which have been identified in SCD patients is unclear in the absence of prospective studies or reported cases.
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PMID:Unexplained lymphadenopathy in sickle cell disease. 334 28

Pain from vaso-occlusive crisis (VOC) is the major cause of hospitalization in patients with sickle cell disease (SCD). The beneficial therapeutic effects of inhaled nitric oxide (NO) on the pathophysiology of SCD have been reported. A double-blind, randomized, placebo-controlled clinical trial was conducted to determine whether NO breathing reduces acute VOC pain in adult patients and to study the safety of inhaled NO. Twenty-three patients experiencing acute VOC were enrolled. After randomization but before treatment, five were found to not meet final eligibility criteria. Nine patients were assigned to inhaled NO (80 ppm) and nine to placebo (21% O2). Primary outcome was the mean change in pain scores after 4 hr of inhalation, measured on a 10-cm visual analog scale (VAS). Both groups had similar baseline VAS pain scores but inhaled NO significantly reduced pain scores compared with placebo (P 5 0.02) at the end of NO inhalation. Secondary outcome was parenteral morphine use at baseline, 4, and 6 hr. Parenteral morphine use was lower in the inhaled NO group, but the difference was not statistically significant.Safety assessments included systolic blood pressure measurements,pulse oximetry readings, concentration of delivered nitrogen dioxide, and concentration of methemoglobin (metHb). None of these NO toxicities was observed.
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PMID:Beneficial effects of nitric oxide breathing in adult patients with sickle cell crisis. 2079 59