1/14. A 'made in one piece' skeleton in a 22-year-old man suffering from sickle cell anaemia.A 22-year-old African male with known sickle cell anaemia was referred by a Congolese medical centre with a request to improve his poor physical condition. He was unable to walk, stand or sit because his large joints and his spine were either ankylosed or very rigid. Radiographs showed joint fusion from the third to the fifth cervical vertebrae, of both hips, of the left knee, and a bilateral osteonecrosis of the humeral head. There was no scintigraphic evidence for an active osteomyelitis (99mTc-MDP (methyldiphosphonate) bone scan, Tc monoclonal antigranulocyte scan and 99mTc sulphur colloid scan). To improve his mobility the right femoral head was resected in June 1997; 14 days later the left femoral head was resected. Four months after the resection of the right hip, a right uncemented total hip prosthesis was implanted on this side. One month later the same type of hip arthroplasty was performed on the left side. During the postoperative rehabilitation period the patient regained autonomy. We have found no previous reports of such severe and multiple joint complications in a single patient suffering from sickle cell anaemia.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/14. Limb pain associated with an undiagnosed hemoglobinopathy and pseudononcyesis.Over a 2-month period, a 16-yr-old African-American female presented with recurrent episodes of severe, migratory limb pain. Sickle cell C disease was ultimately diagnosed. She adamantly denied sexual activity but was found to be 28 weeks pregnant. Her limb pain was probably triggered by the metabolic and hemodynamic demands of pregnancy. The case illustrates the importance of thoroughly examining patients with recurrent, unexplained physical complaints.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/14. Acute myocardial infarction in sickle cell anemia.Cardiovascular dysfunction consistent with ischemia has been observed during episodes of painful crisis and following periods of heavy physical exertion in individuals with sickle cell disease. Similar findings have been observed in other individuals while taking the alpha-adrenergic agonist pseudoephedrine. However, acute myocardial infarction is extremely rare. The authors describe a case of sudden death in a child with sickle cell disease due to acute myocardial infarction and suggest that heavy exertional stress and use of pseudoephedrine may have precipitated the event.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/14. safety of liver biopsy in acute sickle hepatic crisis.Hepatic dysfunction is a commonly recognized complication of sickle cell crisis. The cause of hepatic compromise is usually made evident from physical examination, clinical history, laboratory testing, and noninvasive imaging. There are occasions when liver biopsy is required to define the etiology of the hepatic dysfunction. liver biopsy during acute sickle hepatic crisis can result in hemorrhage and death. Careful consideration of alternatives must be explored prior to performing liver biopsy on a patient with acute sickle hepatic crisis. If biopsy cannot be delayed, close hemodynamic monitoring of the patient postbiopsy is essential.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/14. 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 = physical (Clic here for more details about this article) |
6/14. Primary hyperparathyroidism mimicking vaso-occlusive crises in sickle cell disease.We report a case of bone pain associated with primary hyperparathyroidism in a patient with sickle cell disease. A 17-year-old girl with sickle cell disease (SS phenotype) was seen for bilateral knee and back pain. She had had recurrent severe vaso-occlusive crises and acute chest syndrome in the course of her disease. In the last 2 years, she had frequent visits to the emergency department for severe bone pain. She complained of long-standing fatigue and lethargy. Her physical examination was normal. hydroxyurea treatment, as well as and long- and short-acting narcotics were given, with little improvement in symptoms. Poor compliance with medication, family dysfunction, and potential narcotic addiction were felt to be significant contributors to the patient's symptoms. She was incidentally found to have an extremely elevated total calcium level of 3.19 mmol/L (range: 2.25-2.76) with an ionized calcium level of 1.9 mmol/L (range: 1.15-1.35). phosphorus level was 0.82 mmol/L (range: 0.90-1.50), alkaline phosphatase level was elevated at 519 U/L (range: 10-170), and parathyroid hormone level was extremely high at 1645 pg/mL (range: 10-60). Her renal function was normal. ultrasonography of the neck and a Sestamibi scan revealed a single left inferior parathyroid adenoma adjacent to the thyroid lobe. There was no evidence of an underlying multiple endocrine neoplasia. The patient was diagnosed with primary hyperparathyroidism. Fluid hydration, hydrocortisone, calcitonin, and bisphosphonates were initiated for acute hypercalcemia management before surgical excision of the left parathyroid adenoma. On review of previous blood work, a borderline calcium level of 2.72 was present 18 months before this admission. Two years postsurgery, she has normal renal function, calcium, and parathyroid hormone levels. The weekly visits to the emergency department for pain episodes decreased to 1 every 2 months within the first few months after her surgery. The decrease in pain episodes, even if it coincided with the treatment of primary hyperparathyroidism, may still reflect the natural evolution of sickle cell disease in this patient. However, the high morbidity associated with primary hyperparathyroidism was successfully prevented in this patient. Primary hyperparathyroidism is rare in childhood. In a recent study, it occurred more commonly in female adolescents and was because of a single adenoma, as in our patient. Significant morbidity, mainly secondary to renal dysfunction, was because of the delay in diagnosis after the onset of symptoms (2.0-4.2 years), emphasizing the need for a rapid diagnosis. Sickle cell disease affects approximately 1 of every 600 blacks in north america. Acute episodes of severe vaso-occlusive crisis account for > 90% of sickle cell-related hospitalizations and are a significant cause of morbidity in patients. There is no known association between sickle cell disease and primary hyperparathyroidism, and this case is most probably a random occurrence. However, as emphasized by this case report, pain may also be a harbinger of other disease processes in sickle cell disease. Because management may vary, we suggest that care providers consider the diagnosis of vaso-occlusive crisis as the diagnosis of exclusion and that other etiologies for pain be envisaged in this patient population, especially in the presence of prolonged pain or unusual clinical, radiologic, or biological findings.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/14. Aplastic crisis in sickle cell disorders: bone marrow necrosis and human parvovirus infection.Aplastic crisis in patients with sickle cell disease who develop a parvovirus infection may be associated with extensive bone marrow necrosis as well as acute selective erythroblastopenia. This illness may be manifested by pyrexia, lymphadenopathy, bone tenderness and significant hypoxemia with minimal roentgenographic findings in the lungs. It is uncertain whether the hypoxemia is caused by the effects of the viral infection on the lungs or is secondary to sickling of red blood cells in the pulmonary vasculature or both. The hypoxia may be sufficiently severe to require treatment with both oxygen and transfusion. The physical damage to the bone marrow associated with bone marrow necrosis may be more important than selective acute erythroblastopenia in inducing aplastic crisis in patients with sickle cell disorders. Studies of bone marrow biopsy specimens collected during parvovirus-associated aplastic crisis in patients with nonsickle cell hemolytic disorders would be helpful in determining the pathophysiology of parvovirus-associated disorders.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
8/14. Roentgen rounds #94. osteomyelitis of the left ilium--left iliacus muscle abscess.A left iliac osteomyelitis and left iliacus muscle abscess occurred in a patient who underwent bipolar hemiarthroplasty of the left hip six months prior for avascular necrosis of the left femoral head, secondary to sickle-cell disease. This illness followed an upper respiratory tract infection, and her physical examination was suggestive of a septic process involving the left hip. An aspirate of the hip was not confirmative for septic arthritis. The plain roentgenograms demonstrated that the prosthesis was in an acceptable position, but had limited value in the remainder of the differential diagnosis. In this case, the bone scan contributed significant information distinguishing osteomyelitis from osteonecrosis. The CT scan allowed rapid localization of an occult abscess and destructive changes in the left ilium secondary to osteomyelitis and guided surgical treatment.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
9/14. Exacerbation of sickle cell disease by obstructive sleep apnea.It is difficult to recognize and treat the factors that exacerbate the vascular crises of sickle cell disease. We describe a 12-year-old black girl with sickle cell anemia who, since the age of 9 years, has been repeatedly hospitalized for pain associated with vaso-occlusive disease. A diagnosis of obstructive sleep apnea was suggested by the history and physical examination and was confirmed by polysomnography. Following preoperative transfusion, the patient underwent elective tonsillectomy and adenoidectomy. She has been free of vaso-occlusive pain and crises for over two years and has not been hospitalized since her surgery. Post-operative polysomnography has shown no evidence of obstructive sleep apnea. It is our hypothesis that repeated oxygen desaturation during periods of obstructive sleep apnea was the cause of this patient's frequent vaso-occlusive crises. She not only obtained complete relief of her symptoms, but objective sleep study measurements were normal following surgery. Obstructive sleep apnea is a recent diagnosis, and its pathologic effects are only beginning to be known. physicians taking care of patients with hemoglobinopathies need to be aware of possible contributing factors to their patients' disease, and an aggressive approach to their diagnosis and possible relief should be sought.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
10/14. Cryptococcal pneumonia in a patient with sickle cell disease.We present the findings in a patient having sickle cell disease who developed multilobar pneumonia. Cultures of bronchial aspirates and histologic specimens grew cryptococcus neoformans. There was neither spontaneous clearing of the infection nor a response to bactericidal antibiotics. The patient had no underlying malignant neoplasm or immunodeficiency as indicated by history, physical examination, and specialized tests of humoral and cell-mediated immunity.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
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