Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In the spring and summer of 1988, two separate outbreaks of an illness with a rash resembling erythema infectiosum occurred among members of the nursing staff of the Children's Hospital of Philadelphia. The sources were two adolescent patients with sickle cell disease and aplastic crisis who had unsuspected parvovirus infection. Tests for IgM and IgG antibodies to parvovirus B19 were positive in both patients, and electron microscopical examination showed parvovirus-like particles in the early serum samples. Of 40 health care workers exposed to infected patients, 12 (30 percent) were infected, 2 (5 percent) were possibly infected, 8 (20 percent) had evidence of a past infection with B19, and 18 (45 percent) remained seronegative. Attack rates among the susceptible contacts were 36 percent in the first outbreak and at least 38 percent in the second. Clinical symptoms began a mean of 12.6 days after exposure and included malaise, rash, and joint pain. We conclude that hospital workers are at risk of contracting nosocomial erythema infectiosum from patients with parvovirus-associated aplastic crisis. We recommend that all patients with hereditary hemolytic anemias who are admitted with a febrile illness be evaluated for aplasia and promptly placed in respiratory and contact isolation if aplastic crisis is suspected.
 ...
PMID:Human parvovirus B19 infection among hospital staff members after contact with infected patients. 254 99

M. pneumoniae is a common cause of pneumonia. The diagnosis is suspected when the patient presents with symptoms suggesting primary atypical pneumonia including cough, fever, chills, headache, and malaise in association with a segmental or subsegmental pulmonary infiltrate(s), the white blood cell count is normal or only slightly elevated, and the Gram stain of the sputum (if any can be obtained) reveals polymorphonuclear leukocytes and few bacteria. The diagnosis is more difficult when the patient presents with symptoms not suggestive of pneumonia including lethargy, dyspnea, and a 1- to 4-week history of shortness of breath without cough or fever in association with diffuse reticulonodular or interstitial pulmonary infiltrates. The disease in the previously healthy host is usually benign and self-limiting. However, the course is shortened by the administration of tetracycline derivatives or erythromycin. M. pneumoniae pneumonia can occur in association with other diseases including sickle cell anemia, sarcoidosis, systemic lupus erythematosus, Hodgkin's disease, and various other immunodeficiency states. In these patients mycoplasma pneumonia can be very serious. Although there is no pathognomonic clinical or radiographic presentation, careful consideration of epidemiologic, clinical, laboratory, and radiographic data are usually sufficient to suggest the diagnosis in most patients.
 ...
PMID:Mycoplasma pneumonia. 676 79

In this report, we describe the case of a 28-yr-old woman with sickle cell anemia who presented with acute hepatic failure manifested by anorexia, malaise, painless jaundice, elevated aminotransferase activities, and severe coagulopathy. Liver biopsy revealed changes consistent with autoimmune hepatitis. Treatment with corticosteroids and azathioprine was followed by improvement in biochemical liver test results. The literature on sickle cell-associated liver diseases is reviewed.
 ...
PMID:Autoimmune hepatitis in a patient with sickle cell disease. 863 41

Marked variability is a keynote in the disease course of patients with hemoglobin SC (Hb SC) and hemoglobin S/beta(+)-thalassemia (Hb S/beta(+)-thal), with some patients having a frequency of complications and painful episodes similar to patients with homozygous sickle cell (Hb SS) disease. One possible explanation is that the higher hematocrit in these syndromes may contribute to an increase in blood viscosity, leading to vaso-occlusive pain episodes as well as an increased incidence of thromboembolic complications and retinopathy. We present a patient with Hb SC disease with an excellent baseline functional status who developed splenic infarction at a high altitude. Following splenectomy, the patient developed a sustained increase in hematocrit, an increase in the frequency of painful episodes, as well as new-onset dizziness and malaise. We initiated a therapeutic phlebotomy program in order to lower the hematocrit to pre-splenectomy values, as well as to induce iron deficiency. Repeated phlebotomy resulted in a dramatic decrease in symptoms. Our patient no longer requires narcotic analgesics for pain, has resolution of constitutional symptoms, and has not required further hospitalizations for vaso-occlusive pain crises. The correlation between symptoms and hematocrit levels supports the importance of blood viscosity in contributing to this patient's symptoms. A trial of phlebotomy to reduce viscosity in patients with higher hematocrit values should be considered as an intervention for symptomatic patients with sickle cell disease.
 ...
PMID:Role of phlebotomy in the management of hemoglobin SC disease: case report and review of the literature. 1274 14

Sickle cell intrahepatic cholestasis is a relatively uncommon complication of homozygous sickle cell anemia, which may lead to acute hepatic failure and death. Treatment is mainly supportive, but exchange transfusion is used as salvage therapy in life threatening situations. We describe a case of a 16-year-old female with homozygous sickle cell anemia who presented to the emergency room with fatigue, malaise, dark urine, lower back pain, scleral icterus and jaundice. She was found to have marked hyperbilirubinemia, which persisted after exchange transfusion. Because of the concomitant presence of gallstones and choledocholithiasis, the patient underwent endoscopic ultrasound and laparoscopic cholecystectomy followed by endoscopic retrograde cholangiography and sphincterotomy.
...
PMID:Acute liver function decompensation in a patient with sickle cell disease managed with exchange transfusion and endoscopic retrograde cholangiography. 2517 68