Cases reported "Anemia, Sickle Cell"

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771/897. gonadotropin-releasing hormone analogues in the treatment of sickle cell anemia-associated priapism.

    priapism secondary to sickle cell anemia has been treated with a variety of therapeutic regimens. Recurrent sickle cell priapism is common and in many cases results when detumescence has not been achieved reliably. We report on a patient with sickle cell disease and recurrent priapism who was treated successfully for more than a year with monthly gonadotropin-releasing hormone analogue therapy after failure of standard medical management. ( info)

772/897. acupuncture and blood studies in sickle-cell anemia.

    The author recently treated a case of sickle-cell crisis as an emergency for pain relief using acupuncture because all other conventional therapies failed. The result was so effective, dramatic and encouraging that four other cases of genotype SS/SC were also treated. These cases are reported, (though small in number) to bring attention to this easy, simple, cheap and convenient therapy and to stimulate research among practicing acupuncturists and clinicians. ( info)

773/897. death from a morphine infusion during a sickle cell crisis.

    A 15-year-old boy died during a vaso-occlusive sickle cell crisis after having received a prolonged infusion of morphine. Even in therapeutic doses, narcotics may cause significant respiratory acidosis and hypoxemia, enhancing polymerization of hemoglobin SS and thereby promoting sickling and vaso-occlusion. When narcotics are used during a sickle cell crisis, the best method is pharmacokinetically based patient-controlled analgesia. ( info)

774/897. intraoperative awareness and recall during total hip arthroplasty.

    A case of intraoperative recall and awareness in a patient with sickle cell disease who had received midazolam, 10 mg at induction of anesthesia, is reported. The patient was on opioids which may also have caused tolerance. Although the use of midazolam may have been judged to provide adequate amnesia, this was clearly not the case. Upon analysis of the reasons for recall, we predicted from a two-compartment model that the estimated blood level was 88 ng/mL-1; this level would have provided no amnesia. This case illustrates that recall can occur with the use of midazolam, especially if levels become subtherapeutic, and other measures should be taken to provide adequate anesthesia and amnesia. ( info)

775/897. Visualization of the spleen with a bone-seeking radionuclide in a child with sickle-cell anemia.

    99mTc-methylene diphosphonate, a bone-seeking radionuclide, accumulated in the spleen of a 16-year-old boy with sickle-cell anemia even though no splenic calcification was visible. This may be explained by the presence of microscopic calcium deposits in the spleen, which form at an early age in this disease. Bone-seeking radionuclides may accumulate in regions of recent splenic infarction, as has been demonstrated in cerebral and myocardial infarction. Such radionuclides may be the only means of demonstrating the spleen in such patients. ( info)

776/897. Sickle cell anemia in an American white boy of Greek ancestry.

    A 7-year-old American white boy of Greek ancestry had sickle cell anemia. The disease in this patient was not accompanied by painful episodes or recognizable hematologic complications, although he did demonstrate considerable delay in skeletal maturation. The patient sought medical attention for a dermatologic problem that was ultimately diagnosed as pityriasis rubra pilaris. To our knowledge, this is the first demonstrated example of sickle cell anemia in a white male described in the united states. ( info)

777/897. Dual-perspective analysis of vascular formations in sickle cell retinopathy.

    OBJECTIVE: To examine the sickle cell retina in dual perspective (vascular patterns en bloc and structure in serial sections) to gain new insights into sickle cell retinopathy. methods: We analyzed the retinas of two patients with sickle cell disease (a 54-year-old patient with hemoglobin SC, heterozygous for the S and C mutation in the beta chain of the globin gene, and a 20-month-old patient with sickle cell anemia [SS], homozygous for the S mutation) using the previously described adenosine diphosphatase flat-embedding technique. RESULTS: The dual-perspective analysis afforded by our technique revealed that the primary site of occlusions was located at the precapillary level. An unusual neovascular formation, the hairpin loop, was observed in both patients' retinas and appeared to result from recanalization of the wall of an occluded vessel. Many autoinfarcted pre-retinal neovascular formations were observed in the older SC patient. Two patent preretinal formations were studied in detail and their evolution appeared to be influenced by mechanical factors. The vessels appeared to have been extruded from the retina, perhaps owing to hydrostatic pressure secondary to downstream occlusions. Small pigmented lesions consisting of retinal pigment epithelial cells ensheathing channels that resembled autoinfarcted vessels were found in the eye of the SC patient. CONCLUSIONS: This study illustrates unusual morphological features of intraretinal and preretinal neovascularization and of chorioretinal lesions in sickle cell retinopathy and suggests alternative mechanisms for their formation. ( info)

778/897. osteomyelitis of the spine due to Salmonella: case report, review of clinical aspects, pathogenesis and treatment.

    osteomyelitis of the spine caused by Salmonella is rare. The authors describe a case in which the patient had fusion of the L1 and L2 vertebrae, which were affected by osteomyelitis. The infecting organism was salmonella typhimurium. The authors describe the relationship of the vascular anatomy to the development of osteomyelitis of the spine. They discuss the clinical features, diagnosis, pathogenesis and treatment of the disease. Although the association of osteomyelitis due to Salmonella and sickle cell disease has long been known, this patient did not have sickle cell disease or any other condition that would compromise the immune system. ( info)

779/897. Sickle-cell disease. Two cases in a Romanian family.

    Hemoglobin S is rarely found in Caucasians, especially in its homozygous expression. The authors report two cases of homozygotes for Hb S diagnosed in a Romanian family. Ten subjects were investigated, belonging to three generations of the same family, and the following results were obtained: two cases of homozygotes for Hb S; five cases of heterozygotes; three normal subjects. One of the two subjects homozygous for Hb S, a girl aged six, had a severe clinical evolution, typical for homozygous drepanocytosis. The second case, a sister of the first aged two, had a less severe evolution, without splenomegaly and thrombotic accidents. The heterozygote subjects showed no clinical symptoms. The two cases of homozygous Hb S reported are the first cases of sickle-cell disease described in romania. ( info)

780/897. Hemoglobin SC, SS/GPhiladelphia and SOArab diseases diagnostic importance of an integrative analysis of clinical, hematologic and electrophoretic findings.

    Hemoglobin studies are presented on two children in whom electrophoretic analysis of hemolysates at alkaline pH initially suggested the diagnosis of hemoglobin SC disease. In both patients the course of the disease was unusually severe, blood smears contained irreversibly sickled cells, and crystal formation did not occur when red cells were incubated in 3 per cent sodium chloride solution. Each of these findings is inconsistent with the interpretation of the electrophoretic patterns. family studies demonstrated that one of the patients had sickle cell anemia and was also a carrier of an alpha chain variant; citrate agar electrophoresis at pH 6.2 of the second child's hemolysate revealed that the "C-like" hemoglobin was not hemoglobin c. Tryptic peptide analyses of the nonhemoglobin S fractions showed that the first child has SS/GPhiladelphia disease and that the second child has SOArab disease. These observations emphasize the importance of integrating the clinical and hematologic data with the results of electrophoretic procedures in order to define precisely the hemoglobin abnormality in patients with sickling disorders. ( info)
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