Myocardial performance after repair of congenital cardiac defects in infants and children. Response to volume loading

The hemodynamic response to increasing left atrial pressure by volume loading was evaluated in 70 children during the first 24 hours after repair of congenital cardiac defects. The children were grouped into four diagnostic categories: atrial septal defect or pulmonary valve stenosis (n = 8), ventricular septal defect (n = 36), complete transposition after Mustard's operation (n = 13), and tetralogy of Fallot (n = 13). Within 2 hours of bypass, both cardiac index and left ventricular stroke work index were adequate and increased appropriately with volume loading in all four diagnostic groups. The atrial septal defect group demonstrated a similar response to volume loading 4 and 24 hours after bypass. However, the other three diagnostic groups had a higher filling pressure, lower cardiac index and stroke work index, and a depressed response to increasing preload 4 hours postoperatively, which indicated a deterioration in cardiac performance. The deterioration was maximal between 4 and 12 hours after bypass, and performance tended to recover 24 hours postoperatively. The transposition group had a more profound depression in cardiac performance than the other two groups. Within the ventricular septal defect group, smaller children (body surface area less than 0.36 m2) had a more profound depression in performance than larger children. These results demonstrate a significant alteration in cardiac performance during the first 24 hours after repair of congenital cardiac defects in children. These changes should be considered when postoperative management is being planned.     

Idiopathic Thrombocytopenic Purpura Complicating Pregnancy

Summary: A patient with idiopathic thrombocytopenic purpura complicating pregnancy is presented, followed by a review of the literature and discussion of the problems of this complication.
The need for close monitoring of fetal welfare in late pregnancy and the advantages of Caesarean section are emphasized.     

Congenital isolated upper limb hypertrophy with hand abnormality--a report of 2 cases

Two cases of congenital isolated hypertrophy of the left upper limb with different hand deformities are described. A 4-year-old girl had splayed fingers and an abducted thumb due to anomalous muscles. Excision of these muscles corrected the deformity. The other, an 8-year-old boy, had severe ulnar drift of the fingers (windblown-like hand). He had corrective osteotomies of the second and third metarcarpals and reconstruction of the collateral ligaments. The deformity was corrected and at the latest follow up there was with no recurrence of the deviation. Both cases regained good hand function.     

Decoupling of normal CD40/interleukin-4 immunoglobulin heavy chain switch signal leads to genomic instability in SGH-MM5 and RPMI 8226 multiple myeloma cell lines.

The processes mediating genomic instability and clonal evolution are obscure in multiple myeloma (MM). Acquisition of new chromosomal translocations into the switch region of the immunoglobulin heavy chain (IgH) gene (chromosome 14q32) in MM, often heralds transformation to more aggressive disease. Since the combined effects of CD40 plus interleukin-4 (IL-4) mediate IgH isotype class switch recombination (CSR), and this process involves DNA double strand break repair (DSBR), we hypothesized that CD40 and/or IL-4 activation of MM cells could induce abnormal DNA DSBR and lead to genomic instability and clonal evolution. In this study, we show that MM cell lines that are optimally triggered via CD40 and/or IL-4 demonstrate abnormal decoupling of IL-4 signal transduction from CD40. Specifically, CD40 alone was sufficient to trigger maximal growth of tumor cells. We further demonstrate that CD40 triggering induced both DNA DSBs as well as newly acquired karyotypic abnormalities in MM cell lines. Importantly, these observations were accompanied by induction of activation induced cytidine deaminase expression, but not gross apoptosis. These data support the role of abnormal CD40 signal transduction in mediating genomic instability, suggesting a role for the CD40 pathway and intermediates in myelomagenesis and clonal evolution in vivo.