Safety and efficacy of blood exchange transfusion for priapism complicating sickle cell disease.

BACKGROUND: Priapism is unwanted painful penile erection that affects about 36% of boys and men with sickle cell disease (SCD) most of whom have sickle cell anemia. Clinically, priapism could be stuttering, minor, or major. The first two types are mild, last \u3c 4 h, are usually treated at home, have good prognosis with normal sexual function. The major type of priapism lasts \u3e4 h, associated with severe pain, requires hospitalization; often does not respond to medical treatment and may require shunt surgery. Untreated major priapism and surgical intervention often cause impotence. In this study, we report our 15-year experience in treating adult patients with SCD and major priapism with blood exchange transfusion after being refractory to other medical therapies. METHODS: Adult male African Americans patients with SCD and major priapism were enrolled in this study and followed for 15 years. A Haemonitics V-50 machine was initially used for whole blood exchange and was later replaced with Cobe Spectra machine for RBC exchange. RESULTS: We used 239 blood exchanges requiring 1,136 RBC units. We maintained a post-exchange hemoglobin level of about 10 g/dL and hemoglobin S level \u3c 30%. None of the patients had any neurological complications such as headache, seizures, neurological deficits, or obtundation post-exchange. CONCLUSION: Together, the data indicate that blood exchange transfusion for the treatment of patients with SCD and major priapism is efficacious and safe

Acridinium 3-carb­oxy­pyrazine-2-carboxyl­ate

The title ion pair, C13H10N+·C6H3N2O4 −, contains a protonated acridine cation and a 3-carb­oxy­pyrazine-2-carboxyl­ate monoanion, which are linked together through O—H⋯O, N—H⋯O and weak C—H⋯O hydrogen bonds. These hydrogen bonds generate a C(10) chain graph-set motif. The crystal structure is further stabilized by extensive π–π stacking inter­actions between nearly parallel [dihedral angle = 1.21(2)°] acridine systems. The shortest distance between the centroids of the six-membered rings within the cations is 3.6315 (8) Å. In addition, C—H⋯π edge-to-face inter­actions are present

β-globin haplotypes in normal and hemoglobinopathic individuals from Reconcavo Baiano, State of Bahia, Brazil

Five restriction site polymorphisms in the β-globin gene cluster (HincII-5‘ ε, HindIII-G γ, HindIII-A γ, HincII- ψβ1 and HincII-3‘ ψβ1) were analyzed in three populations (n = 114) from Reconcavo Baiano, State of Bahia, Brazil. The groups included two urban populations from the towns of Cachoeira and Maragojipe and one rural Afro-descendant population, known as the “quilombo community”, from Cachoeira municipality. The number of haplotypes found in the populations ranged from 10 to 13, which indicated higher diversity than in the parental populations. The haplotypes 2 (+ - - - -), 3 (- - - - +), 4 (- + - - +) and 6 (- + + - +) on the βA chromosomes were the most common, and two haplotypes, 9 (- + + + +) and 14 (+ + - - +), were found exclusively in the Maragojipe population. The other haplotypes (1, 5, 9, 11, 12, 13, 14 and 16) had lower frequencies. Restriction site analysis and the derived haplotypes indicated homogeneity among the populations. Thirty-two individuals with hemoglobinopathies (17 sickle cell disease, 12 HbSC disease and 3 HbCC disease) were also analyzed. The haplotype frequencies of these patients differed significantly from those of the general population. In the sickle cell disease subgroup, the predominant haplotypes were BEN (Benin) and CAR (Central African Republic), with frequencies of 52.9% and 32.4%, respectively. The high frequency of the BEN haplotype agreed with the historical origin of the afro-descendant population in the state of Bahia. However, this frequency differed from that of Salvador, the state capital, where the CAR and BEN haplotypes have similar frequencies, probably as a consequence of domestic slave trade and subsequent internal migrations to other regions of Brazil