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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Low back pain in children and adolescents is often caused by a serious problem. The diagnostic investigation should begin with a complete patient history, complete physical examination, laboratory testing, and plain radiography. These results should be used to direct further imaging studies. Although most of the painful injuries that children sustain in recreational activities are mild, back pain that lasts for extended periods may be due to various disorders, including spondylolysis and spondylolisthesis, disk herniation, Scheuermann disease, or neoplasms. Low back pain can also be caused by diskitis and osteomyelitis, most commonly found in children younger than 10 years old. Primary osseous neoplasms of the lumbar spine are uncommon, with Ewing sarcoma, aneurysmal bone cyst, benign osteoblastoma, and osteoid osteoma being the most common followed by primary lymphoma. These lesions occur more often between the ages of 5 and 20 years. Other causes of low back pain include spinal cord tumors (eg, ependymoma), congenital disorders of the spine (eg, scoliosis), and systemic disease (eg, sickle cell disease).
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PMID:Common causes of low back pain in children. 182 29

Five cases of tumour-like extramedullary haemopoiesis (EH) causing spinal cord compression or back pain are reported. Three patients were suffering from thalassaemia major, one from sickle cell anaemia and one from thalassaemia intermedia. CT findings included soft tissue masses in the epidural space of the spinal canal, spinal cord displacement and involvement of the underlying bone. MRI studies showed masses compressing the spinal cord. On T1-weighted images there was a signal of slightly higher intensity compared to that of the adjacent marrow. CT suggested the diagnosis in four cases (in the fifth it was not performed), while MRI was positive in all five. There was good correlation between the MRI findings and the CT appearance of the EH masses. Our results suggest that MRI may eliminate the need for other diagnostic examinations in the investigation of patients with EH.
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PMID:CT and MRI of symptomatic spinal involvement by extramedullary haemopoiesis. 214 24

A case of sickle cell disease diagnosed postmortem is described. A 37-year-old black woman presented with anemia, respiratory distress, and abdominal and back pain. Death followed an intramuscular injection of iron, and anaphylaxis was clinically diagnosed. At autopsy, massive fat and necrotic bone marrow embolization of pulmonary and renal vessels was found. In the vertebral column, multifocal areas of ischemic necrosis were present, and proved to be the source of this embolization. Sickled red cells appeared in bone marrow sinusoids, and signs of disseminated intravascular coagulation were present.
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PMID:Massive fat and necrotic bone marrow embolization in a previously undiagnosed patient with sickle cell disease. 230 55

We describe two cases of sickle cell anemia who were admitted in the hospital with the complaint of backache. X-ray examination and bone scan revealed pathological fracture of vertebral column. These cases are of interest since sickle cell anemia in the Eastern part of Saudi Arabia is reported to run a relatively 'benign' course.
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PMID:Pathological fracture of vertebral column in association with sickle cell anemia in Saudi Arabia. 381 Aug 48

Blackwater fever is characterized by severe intravascular hemolysis with renal failure caused by recurrent use of quinine for prophylaxis. Once described in European patients, sporadic cases have been reported more and more often in autochthonous Africans and Asians. Newer antimalarials including aminoalchohol mefloquine, and halofantrine have also been implicated in Blackwater fever. In this report we describe two cases of blackwater fever involving patients with sickle cell anemia (HbSS). Symptoms including fever, acute hemolytic anemia, emesis, back pain, and hemoglobinuria were characteristic of blackwater fever. Both patients died. Although the underlying mechanism of blackwater fever remains unclear, a likely explanation is an immunoallergic reaction to quinine. Association with glucose-6-phosphate dehydrogenase deficiency has often been reported. Our cases suggest that blackwater fever may also be correlated with hemoglobinopathy such as HbSS.
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PMID:[Blackwater fever in adults with sickle cell anemia. Two fatal cases]. 1110 Apr 42

Orbital compression syndrome is caused by disorders of varying etiologies involving the orbit and presents with ocular and extraocular dysfunction. Ocular involvement of sickle cell disease is uncommon. A 17-year-old male presented with low backache and proptosis of both eyes of 5 days duration without past history of pain crisis or transfusion. Examination revealed pallor, icterus, bilateral proptosis, conjuctival chemosis, and symmetrical restriction of ocular movements with preserved visual acuity. He was drowsy with no other focal deficits. The fundus showed macular edema, venous engorgement, and no hemorrhage. His peripheral smear showed presence of sickle cells. Computed tomography (CT) scan of the orbit revealed orbital subperiosteal hematomas. CT head images showed epidural hematoma in the frontal lobe. High-performance liquid chromatography (HPLC) and mutation studies revealed sickle beta-thalassemia in the patient. He was managed with supportive care, transfusions to keep hemoglobin above 10 g/dl, and hydroxyurea. The patient recovered fully and remained well during follow-up of 12 months. Our case was unique for presenting as orbital compression syndrome without any history of vaso-occlusive crisis.
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PMID:Sickle beta-thalassemia presenting as orbital compression syndrome. 1498 68

The optimal treatment of salmonella spondylodiscitis is controversial. The cases of eight patients who had salmonella spondylitis without sickle cell disease were reviewed. Back pain (100%), fever (75%), and elevated C-reactive protein levels (100%) were common, but gastrointestinal symptoms were not (0%). Six patients had positive blood cultures, and the other two had positive tissue cultures. Group C1 salmonella was the most common serotype. Two patients with coexisting aortic mycotic aneurysms had immediate aneurysm resection. Three others responded favorably to appropriate antibiotics, and three required subsequent surgical reconstruction because of neurologic impairment or osseous instability. Clinical outcomes were significantly better than those of 46 previously reported patients. Salmonella spondylodiscitis usually responds favorably to appropriate antibiotics; consequently, a tissue diagnosis is important. Operative interventions are necessary only for patients with coexisting aneurysms or ongoing osseous instability. A ruptured aortic aneurysm with pseudoaneurysm may mimic a paravertebral abscess, and surgery at the site of an unsuspected aneurysm may precipitate life-threatening hemorrhage. Satisfactory results may be depend on early surgical intervention for a mycotic aneurysm and also are related to host immunity.
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PMID:Salmonella spondylodiscitis in patients without sickle cell disease. 1566 31

A 26-year-old female with Beta-thalassemia/sickle cell anemia was admitted to the hospital with symptoms of a painful crisis. During the next 4 days her hematocrit decreased to 13 percent, and there was reticulocytopenia. She was transfused with four units of red blood cells that were microscopically incompatible, and the hematocrit increased to 29 percent. Eight days later the patient was readmitted with back pain, hemoglobinuria, and a hematocrit of 27 percent. Anti-E, -c, -Jka, and -Yta were identified. The direct antiglobulin test was positive, and the eluate contained anti-c and -Jka. The patient's hematocrit continued to decrease to 14 percent. Transfusions were withheld and the patient recovered uneventfully. Separate 51Cr red blood cell survival studies showed significantly shortened survival of both autologous and R(1)R(1), Jk(a-), Yt(a+) erythrocytes. This case illustrates the complexity of transfusion management in hemoglobinopathy patients.
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PMID:Case report and review: alloimmunization, delayed hemolytic transfusion reaction, and clinically significant anti-Yt(a) in a patient with Beta-thalassemia/sickle cell anemia. 1594 40

The majority of data evaluating Salmonella infections in sickle cell anemia (SCD) comes from studies performed in children. We report a SCD adult who presented with ceftriaxone-resistant Salmonella bacteremia. After appropriate initial therapy, persistent back pain prompted evaluation by magnetic resonance imaging of the spine, which revealed osteomyelitis and a psoas abscess. The patient responded to percutaneous drainage and antibiotics. This report summarizes some of the findings of large SCD studies evaluating Salmonella bacteremia and osteomyelitis, focusing on adults. Our case exemplifies the need for antibiotic coverage for Salmonella species in adult SCD patients with septicemia. We argue that imaging studies looking for osteomyelitis should be done routinely in SCD patients with Salmonella bacteremia.
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PMID:Ceftriaxone-resistant Salmonella septicemia and osteomyelitis in sickle cell disease adults. 1680 Apr 21

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.
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PMID:Primary hyperparathyroidism mimicking vaso-occlusive crises in sickle cell disease. 1688 90


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