Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A search of the records at the New York City Department of Health and the charts of patients at Columbia Presbyterian Hospital identified 37 cases of bone infection and nine cases of joint infection due to Salmonella between 1964 and 1978. Factors that apparently contributed to the development of either osteomyelitis or septic arthritis in 23 of the patients included hemoglobinopathy, previous trauma or surgery, connective tissue disorder, and lymphoma. Salmonella typhimurium and Salmonella enteritidis were the most common serotypes involved with bone infections, whereas members of the C1 serogroup were the most common cause of septic joint infections. Isolates of C1 serogroup Salmonella were represented in both bone and joint infections with frequencies (24% and 67%, respectively) disproportionate to the numbers of Salmonella isolated from other sources during this period. Therapy for joint infections was usually successful, with minimal residual damage. Therapy for acute osteomyelitis was unaccountably inadequate, with many patients (47%) developing chronic infections. Use of inappropriate therapy or an insufficient period of therapy were the most important factors contributing to poor outcome.
 ...
PMID:Bone and joint infections due to Salmonella. 36 64

Sickle-cell disease is a well-recognized clinical entity. The pathophysiology of this hemoglobinopathy has been described in detail by numerous investigators since the first case report appeared in 1910. Orthopaedic manifestations of sickle-cell disease account for much of the morbidity associated with this disorder, including pain, osteonecrosis, arthritis, and sepsis. Effective management of these bone and joint sequelae reflect accurate diagnosis, understanding of this disorder's pathophysiology, and knowledge of available medical and surgical treatment alternatives. In this review, the authors summarize the major orthopaedic manifestations of sickle-cell disease with special emphasis placed upon osteonecrosis and osteomyelitis, since these conditions are the most disabling and serious complications in patients with sickle-cell disease.
 ...
PMID:Orthopaedic manifestations of sickle-cell disease. 223 15

A 12-year-old girl with sickle cell hemoglobinopathy presented with a Salmonella osteomyelitis of her right humerus requiring six weeks of parenteral antibiotic therapy. Home therapy was evaluated. Due to the frequency of the medication administration (every six hours) and the apprehension of the family members, a Pharmacia-Deltec CADD-VT Infusion Pump was chosen for drug administration. Based on the stability of ampicillin, 1.3 g q6h was administered to provide a minimum of 1 g for the last dose of a 24-hour cycle. Ampicillin 6 g contained in 100 mL of sterile water for injection provided a 60 mg/mL solution with an osmolarity of 347 mOsmol. The pump was programmed to deliver 22 mL of solution over one hour, every six hours. A keep-vein-open rate of 0.2 mL/h maintained line patency. A 100 mL cassette of solution prepared daily was replaced on the pump by a home therapy nurse each morning. At the end of six weeks of therapy, the osteomyelitis was eradicated. We found the use of an ambulatory infusion pump an effective, convenient, and cost-saving method of treatment for our patient.
 ...
PMID:Use of an ambulatory infusion pump in a 12-year-old with Salmonella osteomyelitis. 272 25

The clinical records and scintigrams of patients with sickle hemoglobinopathy who underwent combined Tc-99m bone marrow imaging and Ga-67 imaging to differentiate osteomyelitis from bony infarction were reviewed. Of 18 paired examinations in 15 patients, osteomyelitis was diagnosed correctly in six cases; in all six, gallium uptake at the symptomatic site was incongruently increased relative to the bone marrow activity. Of the 12 episodes of infarction, 11 showed congruent activity on both Tc-99m and Ga-67 images. The remaining study was interpreted incorrectly as osteomyelitis due to incongruent Tc-99m and Ga-67 uptake. The use of sequential Tc-99m bone marrow and Ga-67 imaging is an effective means of distinguishing osteomyelitis from bony infarction in patients with sickle hemoglobinopathy.
 ...
PMID:Combined bone marrow and gallium imaging. Differentiation of osteomyelitis and infarction in sickle hemoglobinopathy. 316 13

Splenic dysfunction measured by pitted red cells (pit) was studied in hemoglobinopathies (SS-, SC-, and S beta-type thalassemias and CC-type hemoglobinopathy) in relation to age, in steady state, and during certain significant events. Our experience revealed that the pit count rose with age during steady state in most children with SS disease. A marked increase in pit count was noted in patients with CC disease. The pit count in four patients with S beta+ thalassemia remained normal (i.e., less than 3.5%) at all ages. In children with homozygous SS disease tested at the time of pneumococcal sepsis, the pit count was universally elevated. The pit count was in the normal range in one child with SS disease and osteomyelitis but was elevated in all others. All children had normal pit counts (less than 3.5%) at the onset of acute splenic sequestration crisis, and the counts remained normal during transfusion therapy. No correlation was detected between the pit count and the size of the spleen in patients under 1 year of age.
 ...
PMID:Spleen dysfunction in hemoglobinopathies determined by pitted red cells. 323 12

We analyzed 178 episodes of bacteremia that occurred during 13,771 patient-years of follow-up of 3451 patients with sickle hemoglobinopathies. Age-specific incidence rates of bacteremia were calculated for patients with sickle cell anemia (SS) and sickle cell-hemoglobin C (SC) disease. The incidence rate was highest among children with SS and SC younger than age 2 years. Children with SC showed an abrupt decrease after age 2 years, whereas children with SS had a gradual decline in rate from 2 to 6 years of age. The predominant pathogen in patients younger than 6 years was Streptococcus pneumoniae (66%); gram-negative organisms were responsible for 50% of bacteremias in patients 6 years and older. Urinary tract infection was present during 73% of Escherichia coli bacteremias, and 77% of Salmonella bacteremias were associated with osteomyelitis. In contrast, no focus of infection was present in 52% of pneumococcal bacteremias. The incidence of pneumococcal bacteremia in children with SS younger than age 3 years was 6.1 events/100 patient-years; the case fatality rate for pneumococcal sepsis in this age group was 24%. No hematologic or demographic variables were associated with occurrence of pneumococcal bacteremia in young children. Retrospective analysis of pneumococcal bacteremia suggests that the prophylactic use of penicillin may decrease the incidence in children younger than 3 years of age.
 ...
PMID:Bacteremia in sickle hemoglobinopathies. 353 49

The increased incidence of Salmonella osteomyelitis in patients with sickle cell disease has never been entirely explained. Problems such as cholelithiasis, intestinal infarction, and frequent antibiotic use in this population could possibly result in prolonged or chronic intestinal carriage of Salmonella after acute gastroenteritis. If prolonged carriage were a factor in the pathogenesis of osteomyelitis, attempts to eliminate the Salmonella with antibiotics would be indicated. We did a stool culture survey of 71 patients attending our pediatric sickle cell clinic to determine the incidence of asymptomatic Salmonella carriage. At least two rectal swab cultures were obtained from each patient; 69% of patients also mailed in a stool sample for culture. No Salmonella was isolated. It therefore appears unlikely that prolonged intestinal Salmonella carriage is an important mechanism in the development of Salmonella osteomyelitis in patients with major sickle hemoglobinopathies.
 ...
PMID:Intestinal Salmonella carriage in patients with major sickle cell hemoglobinopathies. 397 46

A case of septicemia owing to Salmonella choleraesuis with localization in the lumbar spine and left knee is described. The spinal lesion is dominated by tuberculoid granulomas with or without central necrosis. The necrotic foci within some granulomas show heavy polymorph infiltration, whereas in others they simulate caseous necrosis and are indistinguishable histologically from tuberculosis, brucellosis, and fungal infections. As Salmonella osteomyelitis has a strong tendency to chronicity if antimicrobial treatment is delayed, inappropriate, or inadequate, this diagnosis should be considered in all cases of granulomatous osteomyelitis, especially when the patient is immunosuppressed or has hemoglobinopathy.
 ...
PMID:Salmonella osteomyelitis. An important differential diagnosis of granulomatous osteomyelitis. 409 Nov 85

Clinical records and scintigrams were reviewed of 18 patients with sickle cell hemoglobinopathies who had undergone combined technetium and gallium scintigraphy during 22 separate episodes of suspected osseous infection. The combined scintigrams were correctly interpreted as indicating osteomyelitis in four studies. Ga-67 localization was abnormally increased in all of these studies, including one where the Tc-99m MDP bone scan showed decreased activity, another where the bone scan was normal at the site of Ga-67 uptake, and two where the distribution of Tc-99m MDP and Ga-67 were incongruent. Of 18 studies in patients with infarction, the combined scintigrams were correctly interpreted in 16 and showed either no local accumulation of Ga-67 or less accumulation than that of Tc-99m MDP at symptomatic sites. In the other two studies, the scintigrams were falsely interpreted as indicating osteomyelitis and showed congruent, increased accumulation of both Tc-99m MDP and Ga-67 (with the latter of similar or greater intensity). This pattern must be considered indeterminate. Overall, the results indicate that the combination of technetium and gallium scintigraphy is an effective means to distinguish osteomyelitis from infarction in patients with sickle cell hemoglobinopathies.
 ...
PMID:Osteomyelitis and infarction in sickle cell hemoglobinopathies: differentiation by combined technetium and gallium scintigraphy. 649 77

The clinical features of long bone infarction in patients with sickle cell disease have not been well defined, and differentiation of bone infarct from osteomyelitis has accordingly been difficult. We reviewed records from 192 children with sickle hemoglobinopathies and identified 41 episodes of acute long bone infarction in 21 patients. The most commonly affected bones were the humerus (38%), tibia (23%), and femur (19%). The distal segment was more commonly involved. Tenderness and prominent swelling occurred in all cases; other findings included impaired joint motion (68%), local heat (65%), and erythema (145). Fever was usually absent or low grade, and patients did not appear ill. Laboratory studies included negative bacterial cultures in all cases, absence of left shift in WBC count in most, and variable erythrocyte sedimentation rate. Plain roentgenographs were unremarkable. Contrary to previous reports, radionuclide bone and bone marrow scans were not helpful in differentiation of bone infarction from osteomyelitis. Patients received supportive therapy and improved within several days. Long-term sequelae were not evident. The rarity of osteomyelitis in our sickle cell population (five cases in 22 years) precluded direct comparison of most of its clinical features with those of bone infarction. Acute long bone infarction is at least 50 times more common than bacterial osteomyelitis in sickle cell disease.
 ...
PMID:Acute infarction of long bones in children with sickle cell anemia. 709 7


1 2 3 Next >>