1/13. Microsurgical free flap transfer in sickle cell disease.A patient with sickle cell anemia and recurrent ankle ulceration is presented. Multiple previous skin grafts did not resolve the painful ulcers. A radial forearm free flap was attempted. Despite preoperative transfusions to reduce her hemoglobin S levels, postoperative thrombosis occurred. The true risk of free flap failure in sickle cell anemia is unknown and there is still debate about the exact nature of the hypercoagulable state that exists in this disease. We recommend, however, that consideration is given to the use of prophylactic anticoagulation in patients undergoing complex procedures or in whom other risk factors have been identified.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/13. Mri findings in primary non-Hodgkin's lymphoma of the liver.Primary lymphoma of the liver is a very rare malignancy. Most often, these lesions consist of diffuse large B-cell non-Hodgkin's lymphoma that occurs mostly in immunodeficient patients. To prove the primary nature of a hepatic lesion, a systemic lymphoproliferative disease should be ruled out. Secondary liver involvement during Hodgkin's and non-Hodgkin's lymphoma is frequent. In advanced cases the incidence varies from 25% to 50%.The present case describes the MRI features of a primary lymphoma of the liver presenting as a solitary nodule. The primary lymphoma presents as a T2-hyperintense homogeneous nodule, with a signal intensity comparable with the signal intensity of the spleen. Signal intensity is comparable on in and out of phase imaging. The nodule is slightly T1-hypointense and doesn't show any arterial contrast uptake. In the late venous phase a slight increase in signal intensity is noted. Two and a half minutes after the administration of contrast agent, the lesion is iso-attenuating with the liver parenchyma.This case is rare because of the concomitant presence of heterozygous sickle cell anaemia and the presence of Gamna-Gandy bodies in the splenic parenchyma. It remains uncertain whether the presence of the Gamna-Gandy bodies is associated with the liver lymphoma or with the underlying sickle cell anaemia, or with a combination of both.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/13. Disseminated nontuberculous mycobacterial infections in sickle cell anemia patients.nontuberculous mycobacteria (NTM) are ubiquitous in nature and have been implicated in skin/soft-tissue, pulmonary, middle ear, bone, and surgical/traumatic wound infections. Disseminated disease occurs infrequently and almost exclusively in the immunocompromised. We describe the first 2 reported cases of disseminated mycobacterium fortuitum infection in teenagers with sickle hemoglobinopathy. Both had central venous catheters (CVCs), frequent admissions for vaso-occlusive painful episode and received hydroxyurea. diagnosis was confirmed by multiple positive blood cultures and pulmonary dissemination occurred in both. Both had successful treatment after CVC removal and combination drug therapy. Positive cultures persisted in 1 patient due to drug resistance emphasizing the need for accurate susceptibility data. NTM infection should be added to the list of pathogens in sickle cell patients with CVCs and fever. Investigation for disseminated disease should be undertaken based on clinical signs and symptoms. Although some routine blood culture systems can identify NTM, specific mycobacterial blood culture is optimal. Removal of involved CVCs is essential and treatment of NTM must be guided by susceptibilities. As dissemination almost always occurs in those with impaired cellular immunity, human immunodeficiency virus testing should be performed. hydroxyurea may be a risk factor for dissemination and needs further evaluation.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/13. cytomegalovirus in tears from patients with normal eyes and with acute cytomegalovirus chorioretinitis.cytomegalovirus (CMV) was recovered from the tears in eight of 41 (19.5%) children excreting CMV in their urine or saliva. Tear excretors were all immunosuppressed children with acute lymphocytic leukemia. Three had active CMV chorioretinitis and five did not develop retinal disease in nine to 15 months of observation. To our knowledge this was the first report of the recovery of CMV from tears and of acquired CMV chorioretinitis in children. One patient with active chorioretinitis presented with a disciform elevation of the macula. Therapy with adenine arabinoside (ara-A) or idoxuridine was ineffective in two patients while a third patient treated with ara-Apossibly had a more rapid recovery. However, the significance is uncertain due to the unusual disease presentation and lack of data regarding the nature of cytomegalic inclusion disease chorioretinitis. Areas of retinal calcification were present at autopsy in one patient.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/13. Caring for the orthopaedic patient with sickle cell disease.Sickle cell disease (SCD) is an inherited, chronic, painful condition seen primarily in blacks and populations from the Mediterranean and Caribbean areas. The crescent or sickle shaped red blood cells have a shorter lifespan causing severe anemia; they are sticky and easily clump together causing intravascular occlusions which eventually damage vital organs. Providing nursing care for patients with sickle cell disease can be very challenging because of the chronic nature of the illness. Understanding the disease phenomena can facilitate nursing assessments and help nurses individualize care for these clients.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/13. sickle cell trait and hematuria associated with von Willebrand syndromes.A von Willebrand syndrome was present in four patients with sickle cell trait and hematuria. The first two patients had severe anemia and active bleeding and received cryoprecipitate, with prompt cessation of hemorrhage. All of the patients had repetitive laboratory and clinical features; that is, reduced, but detectable, factor viii-related antigen, heterogeneity of, and incongruities within, the coagulation studies performed by consistently defective platelet aggregation to ristocetin correctable only with normal plasma. Bleeding outside the genitourinary tract never was observed. Because of the focal nature of the hemorrhage, the hematuria may not have been perceived as part of a general hemorrhagic disorder and the diagnosis not pursued. These observations suggest that when sickle cell trait and hematuria occur together, a von Willebrand syndrome should be a major diagnostic consideration that ultimately may point toward a rational, effective, easily administered, and clinicially acceptable form of treatment with cryoprecipitate.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/13. salmonella vertebral osteomyelitis and epidural abscess in a child with sickle cell anemia.A case of salmonella vertebral osteomyelitis with epidural abscess in a child with sickle cell anemia is presented. Spinal osteomyelitis is a rare event in children. Although osteomyelitis in sickle cell anemia may occur in any bone, it has most often been documented as beginning in the medullary cavity of the long and tubular bones. This is in contrast to the clinical presentation of osteomyelitis in the normal individual, who is likely to have infection beginning in and restricted to the metaphyseal regions of bones. Nonspecific or constitutional symptomatology may obscure the diagnosis of vertebral infection with ensuing cord compression. This case stresses the rapidity of development of paralysis or other neurologic complications, as well as the difficulty and emergent nature of the diagnosis of epidural abscess in this situation.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/13. Renal papillary necrosis: an update.The clinical and diagnostic features of renal papillary necrosis (RPN) of 27 patients were studied. diabetes mellitus was the most frequent (56%) condition associated with RPN. Analgesic abuse, sickle hemoglobinopathy and urinary tract obstruction were present in 4 patients each; in 6 of these 12 patients these conditions were present as a coexistent disease with diabetes mellitus. There was evidence of an acute or chronic infection of the urinary tract in 18 patients, as a coexistent condition with another underlying disease that itself can cause RPN in 14 patients and as the only cause of RPN in another 4. Thus, the presence of more than one diagnostic condition which might be implicated in the causation of RPN was present in 15 patients or 55% of the cases in this series. When infection was excluded, six patients or 22% of the cases had two coexisting diseases, each of which has been implicated as a cause of RPN. This observation underlines the multifactorial nature of this entity and might explain why RPN is not encountered more frequently in each of the various primary diseases with which it has been associated. The average age of the patients at the time of diagnosis was 53 years for women and 56 years for men. Only six of the patients were younger than 40 years, and three of these had sickle hemoglobinopathy. The diagnosis of RPN was based on x-ray findings in eight patients, on the histologic examination of papillary tissue in urine in one, and on autopsy findings in the rest. Papillary necrosis was bilateral in three-fourths of the cases. The clinical picture varied. Most of the patients (67%) presented with chills and fever. flank pain and dysuria were present in 11 patients (41%). As a rule oliguria was rare and progressive uremia was uncommon. In cases diagnosed at post-mortem, the patients had succumbed to infection or to a primary severe extrarenal disorder with the possibility of RPN having been entertained clinically in only half these cases prior to autopsy.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/13. Splenic compression of the left kidney simulating pathologic unilateral renal enlargement.A case in which pathologic enlargement of the left kidney was simulated by splenic compression in a 17-year-old boy with hemoglobin sc disease and recurrent left flank pain is presented. The true nature of the apparent enlargement was demonstrated by computed tomography.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/13. Sickle myonecrosis revisited.This review focuses on the classification of sickle myonecrosis in the context of sickle cell vaso-occlusive crisis. Further, the potential and novel use of magnetic resonance imaging to further clarify the nature of sickle crisis is discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
| | Next -> |