Vancomycin-resistant Enterococcus faecium endocarditis in a premature infant successfully treated with linezolid

A 4 1/2-month-old, 26-week premature infant with multiple complications of prematurity required a central venous catheter for venous access and antibiotic treatment of bacterial nosocomial infections. He developed tricuspid valve endocarditis with vegetation caused by Enterococcus faecium resistant to ampicillin, vancomycin and quinupristin-dalfopristin but susceptible to linezolid. He was successfully treated with linezolid intravenously (7 weeks) and then orally (2 weeks).     

Pancytopenia in a patient with sickle cell anemia.

An 11-year-old black boy with sickle cell anemia developed profound pancytopenia during the course of his disease, but fully recovered therefrom. The patient was receiving anticonvulsant drugs for a seizure disorder secondary to a "stroke," and, therefore, a drug-related marrow aplasia cannot be ruled out.     

Malignant hyperthermia in a patient with sickle cell anemia

A sixteen-year-old male with sickle cell anemia and congenital strabismus developed malignant hyperthermia a few minutes after the administration of succinylcholine, used as the general anesthetic for corrective eye surgery. The patient's hemoglobin S level was reduced to fifteen percent before the operation. He recovered uneventfully within a few hours. Increased serum creatinine phosphokinase activity and pathological changes observed in the muscle biopsy along with strabismus suggest that the patient had an inherited susceptibility to malignant hyperthermia.     

Blood transfusion rate in congolese patients with sickle cell anemia

Objective The main objective of this study was to evaluate the rate of blood transfusion in African Sickle Cell Patients and the risks related to the use of total blood. Methods 186 sickle cell patients (95 males and 91 females) aged 0–21 years were regularly followed over a 3 years period in Katanga province, DR Congo. Indications for blood transfusion were mainly based on clinical criteria and Hb level (less than 5g% ml or a drop of 2g% under the steady state value). All the subjects, who were transfused, wer screened for hepatitis B surface antigen (HBs Ag) and Human Immune deficit Virus (HIV). Results Of 186 patients, 150 (80.6%) were transfused, and the average blood transfusion requirement was 0.4 units per patient-year. According to the age of first transfusion, 75.3% (113/150) of them were transfused before the 6^th year of life; but the frequency of transfusions seemed to decline in children aged more than 13 years. The risk of HIV infection from blood transfusion was estimated at 1 per 37.1 units or 26 per 1000 blood units. The hepatitis B surface antigen was detected in 15 cases (10%) and HIV serology was positive in 17 patients (11.3%). Conclusion Because of the complications related to blood transfusions in Africa, efforts are needed in order to reduce the frequency of transfusions, by preventive measures (early diagnosis, malarial and penicillin-prophylaxis) and to use more rational indications.     

Asymmetrical closure of epiphyses in a patient with sickle cell anemia

There is a high incidence of delayed sexual development and short stature during childhood in children with sickle cell anemia (SCA). We report a 15 year-old male with SCA who presented with significant short stature after a near death event (involving seizures and prolonged hypoxia). His evaluation showed growth hormone (GH) deficiency with low insulin-like growth factor-I (IGF-I), low IGF binding protein-3, and low GH response to stimulation. He was started on GH replacement with poor response in height gain although with normal response in terms of elongation of his arm span. Further studies showed premature closure of the epiphyses of the femora and tibiae bilaterally. This report demonstrates that children with SCA may present with growth failure not only due to nutritional and GH abnormalities but also due to abnormal growth plates, probably due to local anoxic events. Children with SCA should always have their arm span measured carefully.     

Planning an exchange transfusion in patients with sickle cell syndromes

Partial exchange transfusions are performed in sickle cell patients for a variety of reasons. An algorithm to plan a nonautomated exchange in patients with sickle cell syndromes was developed and validated by a study of 40 such procedures. Formulas that can be used to explore alternatives, by assessing at any point during the exchange the current concentration of sickleable cells and the hematocrit, were devised: a computer program in BASIC is available for maximum versatility. The two most important determinants of the exchange are the patient's initial hematocrit and the desired final concentration of sickleable cells; the rate and type of exchange (continuous or discontinuous) are not important. The final hematocrit depends on the type of blood product used. An exchange can be performed with packed red blood cells (PRBC), whole blood (or its equivalent, PRBC reconstituted with albumin), or it can be started with PRBC and continued with whole blood. Whole blood decreases the concentration of sickeleable cells rapidly and increases the hematocrit slowly; it does not markedly increase the viscosity. PRBCs decrease the concentration of sickleable cells more slowly and increase the hematocrit faster; thus, they may increase the blood viscosity to dangerous levels.     

Reversibility of anatomical and functional asplenia by chronic transfusion in a child with sickle cell anemia

We describe a 13-year-old black girl with sickle cell disease in whom spleen regrew after autosplenectomy during the course of a transfusion program for a cerebrovascular accident. This case illustrates the remarkable regrowth potential of the shrunken and fibrotic spleen when sickled red cells are replaced with normal cells.     

Reversibility of splenic function by transfusion in two young adults with sickle cell anemia

A level of circulating "pitted" or vesiculated red blood cells higher than 3.5% was recently reported in studies in the Cooperative Study of Sickle Cell Disease to correlate with splenic dysfunction as shown by spleen scans. Reversal of splenic dysfunction by transfusion in children with sickle cell anemia (SS disease) is known to occur in the young child. We report two older patients with homozygous sickle cell disease, aged 17 and 21 years, whose spleen function, as measured by pit count, was restored to normal range after transfusion.