1/13. Splenic syndrome in sickle cell trait: four case presentations and a review of the literature.Four cases of splenic infarction/sequestration in sickle cell trait (SCT) patients are presented. All four patients were undergoing moderate exercise at elevations ranging from 5,500 to 12,000 feet. The patients include two African-American males, a Hispanic male, and a white female. All four presented with the acute onset of mid epigastric then left upper-quadrant pain, nausea, vomiting, and respiratory splinting. A review of the literature indicates that splenic infarction with SCT is not uncommon; however, not surprisingly, it is often initially misdiagnosed. This is the first report in the literature of a female with SCT incurring a splenic syndrome with exposure to terrestrial altitude. Although SCT is not a contraindication for moderate- or high-altitude activities, military physicians need to consider the diagnosis of splenic infarction early in any patient regardless of race or sex who presents with left upper-quadrant pain at altitudes above 5,000 feet. Prompt evacuation to sea level may hasten recovery and spare further splenic trauma. Although SCT should be considered a relatively benign entity, the literature also suggests a higher than average risk of sudden death in military recruits with SCT from exertional heat illness and rhabdomyolysis.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
2/13. Megalophallus as a sequela of priapism in sickle cell anemia: use of blood oxygen level-dependent magnetic resonance imaging.priapism is a common complication of sickle cell anemia. We report a little known sequela of priapism: painless megalophallus, with significant penile enlargement. The patient had had an intense episode of priapism 9 years previously and his penis remained enlarged. blood oxygen level-dependent magnetic resonance imaging revealed enlarged, hypoxic corpora cavernosa. Megalophallus probably resulted from permanent loss of elasticity of the tunica albuginea due to severe engorgement during the episode of priapism. This sequela needs to be recognized by physicians because no intervention is necessary and sexual function seems to remain intact.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
3/13. Sickle cell disease -- when opioids and physicians fail.An interview with a 32-year-old male with sickle cell anemia and multiple sequential admissions for vaso-occlusive crises, receiving high dose narcotic analgesics, is presented. The subsequent clinical discussion outlines psychiatric, psychosocial and treatment issues. Management of acute vaso-occlusive crisis is summarized along with a discussion of the value of comprehensive care for sickle cell disease patients. This article will be useful to physicians and consultation-liaison psychiatrists treating patients with sickle cell disease as well as policy makers developing service delivery models for this population.- - - - - - - - - - ranking = 5keywords = physician (Clic here for more details about this article) |
4/13. Fatal pulmonary artery embolism in a sickle cell patient: case report and literature review.This article reports a rare case of fatal saddle embolism to the pulmonary artery presenting as an acute chest syndrome (ACS) in a Sickle Cell patient. We present a review of the etiology, pathophysiology, clinical manifestation and management of ACS. Clinicians should be aware of the fact that a sickle cell crisis admission may represent a life-threatening process. Such awareness will help physicians to act promptly and execute proper therapeutic interventions. It is important for clinicians to be expectant of impending clinical deterioration and likewise be aware that ACS can develop in patients hospitalized for other medical or surgical conditions.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
5/13. Sickle cell vaso-occlusive pain crisis in adults: alternative strategies for management in the emergency department.The gene for sickle cell disease is carried by 8% of the African-American population in the united states. The primary care physician is often called upon to recognize and treat one of the major sequelae of sickle cell disease--vaso-occlusive pain crisis. An injectable nonsteroidal anti-inflammatory drug has recently become available and may offer some improvement in outcome of vaso-occlusive pain crises. We present five case reports reviewing various current therapeutic options, including newer pharmacologic agents, and comment on alternatives to impatient management of pain crises. The use of the emergency department short-term observation unit as an alternative to hospitalization is discussed.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
6/13. Slipped capital femoral epiphysis in a child with sickle cell disease.Slipped capital femoral epiphysis in a child with sickle cell disease has not been reported previously. The diagnostic challenges, role of imaging techniques, and the medical treatment of this patient are discussed. The presentation of acute hip or leg pain in a child with sickle cell disease should alert the treating physician to the possibility of a vaso-occlusive crisis as the likely source of the child's pain. The goal of the current case report is to emphasize the need to maintain a high index of suspicion for other potential causes of hip, thigh, or knee pain such as slipped capital femoral epiphysis in an adolescent. Preoperative and postoperative care for a child with sickle cell disease needs to be modified to minimize the risks of vaso-occlusive complications.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
7/13. Discriminate biopower and everyday biopolitics: views on sickle cell testing in Dakar.Many physicians in senegal and france, where most Senegalese sickle cell specialists are partially trained, assume that genetic testing that could imply selective abortion for people with sickle cell would run counter to the religious and cultural ethics of people living in Dakar. Senegalese affected by this genetic disease, however, often cite "traditional" rationales to indicate why such testing, if offered, might appeal to them. The reluctance of medical practitioners to entertain such testing technologies for their patients evinces a protectionist attitude toward care--an attitude that emerges within a context in which family planning and a blind concentration on hiv/AIDS have created a public health system that completely overlooks sickle cell anemia. This discriminate biopower leaves everyday biopolitics largely in the hands of families faced with this disease. It falls to them to pragmatically calculate the value that genetic testing may, or may not, hold for their own lives.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
8/13. Outpatient management of sickle cell pain with chronic opioid pharmacotherapy.We report our experience of providing chronic opioid pharmacotherapy on an outpatient basis to selected patients with frequent episodes of moderate-to-severe pain from sickle cell disease (SCD). Three cases illustrate our clinical experience in approximately 40 patients with sickle cell pain. patients were seen at our sickle cell pain clinic at Beth israel Hospital once each month for a three-hour visit. Visits included group music therapy and individual medical care, including comprehensive blood work and scheduling of medical tests when appropriate. Between visits, the pain and palliative care physicians followed patients on an as-needed basis. The SCD pain opioid pharmacotherapy protocol was modeled on a regimen used to treat malignant pain-typically a long-acting opioid in combination with a short-acting opioid, such as oral transmucosal fentanyl citrate (OTFC; Actiq) for breakthrough pain (BTP). Emergency department (ED) visits and hospital admissions were dramatically reduced in the three patients whose pain was managed by adapting the cancer pain model. During the year before their first visit to our pain clinic, the patients each had between six and 18 ED visits, which resulted in six- to 13 hospital admissions amounting to 32-182 inpatient days per patient. Each of the patients was prescribed a long-acting opioid (methadone, control-release oxycodone, or transdermal fentanyl) with a short-acting opioid for BTP from crises (oral transmucosal fentanyl citrate for two patients; short-acting oxycodone for one patient). Pain was well controlled. For each patient, hospital admissions were reduced to < or = 1 visit per year. These reduced levels of ED visits and hospital admissions have remained constant for more than three years.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
9/13. Acute hepatic sequestration in sickle cell disease.Sickle cell anemia is a disease that affects one out of every 600 african americans. It is often debilitating and can cause many physical restrictions to individuals with the disease. The disease has many complications which can be vexing for patients and their physicians. The hepatic complications attributed to vascular occlusion encompass a variety of clinical syndromes of which the relationship among clinical presentation, biochemical findings, and histologic features remains unclear. The conditions range from the self-limiting hepatic right upper quadrant syndrome (hepatic crisis) to the potentially lethal intrahepatic cholestasis and acute hepatic sequestration syndromes. Few cases have been documented, and there have not been many sizable studies on acute hepatic sequestration in sickle cell disease. This case is useful for clinicians who are not familiar with the intrahepatic vaso-occlusive syndromes in sickle cell disease. It provides insight into the presentation, diagnosis, and management of these syndromes.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
10/13. Splenic uptake of both technetium-99m diphosphonate and technetium-99m sulfur colloid in sickle cell beta (0) thalassemia.A 19-year-old black woman with sickle cell beta(0) thalassemia had experienced more than 100 hospital admissions for sickle cell crisis and aseptic necrosis of both femoral heads. Her spleen was enlarged threefold and accumulated both radiocolloid and bone-seeking agent on two occasions, demonstrating an exception to the rule in sickle cell anemia that spleens that take up bone-seeking agents demonstrate functional asplenia. In the context of fever, left upper quadrant pain, and splenomegaly, the pattern of calcification in the patient's spleen as revealed in ultrasound and CT studies suggested possible abscess and led to unnecessary splenectomy. The nuclear medicine studies did not support this diagnosis. nuclear medicine physicians should not be misled by splenic findings of sickle cell thalassemia (and possibly of other heterozygous sickle cell disorders) that differ from those of the more familiar homozygous sickle cell anemia.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
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