Are there phases to the vaso-occlusive painful episode in sickle cell disease?

The purpose of this study was to describe the pain experience of children with sickle cell disease who were hospitalized for vaso-occlusive painful episodes. The pain experience, and signs and symptoms prior to admission and during hospitalization, are presented in the context of whether there is evidence to support the existence of phases to a vaso-occlusive painful episode. Children were interviewed about the onset of the painful episode and were asked to describe their pain from the day of admission to the day of discharge from the hospital. They were also observed for the absence or presence of signs and symptoms associated with the painful vaso-occlusive episode. Findings from this study provide some evidence to support previous observations related to changes during the evolution of painful episodes that may be occurring in phases (e.g., evolving, inflammatory, resolving), as previously described in adults and children. These phases had different names, although the concepts were similar.     

The impact of maternal obesity on midtrimester sonographic visualization of fetal cardiac and craniospinal structures

OBJECTIVE: To examine the impact of maternal obesity on the rate of suboptimal ultrasound visualization (SUV) of fetal anatomy and determine the optimal timing of prenatal ultrasound examination for the obese gravida. METHODS: A computerized ultrasound database was used to identify ultrasound examinations for singleton gestations performed between 14(0/7) and 23(6/7) weeks at a tertiary care, university-based hospital. Patients were divided into four groups and categorized based on body mass index (BMI): nonobese (BMI <30 kg/m2), class I obesity (30< or =BMI<35 kg/m2), class II obesity (35< or =BMI<40 kg/m2), and extreme obesity (BMI > or =40 kg/m2). The rates of SUV for fetal cardiac and craniospinal structures were calculated for each group and compared. RESULTS: A total of 11,019 pregnancies were studied, of which 38.6% of the patients were obese. Overall, the rate of SUV of the fetal structures was higher for obese compared to nonobese women for both cardiac (37.3 [1723/4200] vs 18.7% [1275/6819]; P<0.0001) and craniospinal structures (42.8 [1798/4200] vs 29.5% [2012/6819]; P<0.0001). Increased severity of maternal obesity was associated with SUV rate for both the cardiac (nonobese 18.7% [1275/6819], class I 29.6% [599/2022], class II 39.0% [472/1123], and extreme obesity 49.3% [580/1055]; P<0.0001) and for the craniospinal structures: (nonobese 29.5% [2012/6819], class I 36.8% [744/2022], class II 43.3% [486/1123], and extreme obesity 53.4% [563/1055]; P<0.0001). With increasing gestational age at examination, the rate of SUV decreased for both obese and nonobese women. However, for obese women there was minimal improvement in visualization after 18-20 weeks. Even after adjustment for gestational age and the type of ultrasound machine, obese women (class I, class II, and extreme obesity) were still associated with increased odds for SUV of the fetal cardiac and craniospinal structures compared to nonobese women. CONCLUSION: Maternal obesity increases the rate of SUV for the fetal cardiac structures by 49.8% and for the craniospinal structures by 31%. The optimal gestational age for visualization of fetal cardiac and craniospinal anatomy in obese patients may be after 18-20 weeks.     

Changes in sleep, food intake, and activity levels during acute painful episodes in children with sickle cell disease

As part of a larger study that examined pain experience, pain management, and pain outcomes among children with sickle cell disease, functional status (sleep, food intake, and activity levels) was examined during hospitalization for acute painful episodes. Children were asked to rate the amount of pain they experienced as well as the amount of time they slept, the amount of food they ate, and the amount of activity they had everyday. Children reported high levels of pain, which showed only a small decrease throughout hospitalization, and had disrupted sleep and wake patterns, decreased food intake, and decreased activity levels. Nurses need to routinely monitor functional status during acute painful episodes so that strategies to promote adequate sleep, food intake, and activity may be incorporated to minimize long-term negative outcomes in children with sickle cell disease.     

Rail External Fixation for Stabilization of Closing Base Wedge Osteotomies and Lapidus Procedures: A Retrospective Analysis of Sixteen Cases

This is a retrospective study of 16 cases of proximal first-ray procedures with rail external fixation. Arthrodesis of the first metatarsal-cuneiform joint was performed on 11 feet, and a closing base-wedge osteotomy was performed on 5 feet. Heel weightbearing in a surgical shoe with ankle joint mobilization was the standard postoperative protocol. Thirteen women and one man with an average age of 41 years (range, 16-64 years) were evaluated, with an average follow-up of 15.12 months (range, 10-24 months). The mean duration of frame application among all patients was 6.75 weeks (range, 2-10 weeks; 7 weeks for the base wedge procedures; 6.64 weeks for the lapidus). Multiple complications developed. Ten patients developed pin-tract infections: 5 required isolated pin removal, whereas 2 required removal of the entire frame because of the severity of infection. Seven patients developed pin loosening. Two patients developed cellulitis requiring long-term intravenous antibiotic therapy. Two nonunions occurred, both with the lapidus procedure, and one was symptomatic. Ten of the 16 patients reported they would have surgery again. Monolateral external rail fixation in combination with heel weightbearing and ankle mobilization demonstrated a high complication rate when used for proximal first-ray procedures. The technique as described is not recommended for routine use with the lapidus and base wedge osteotomy procedures.     

Ultrasound characteristics of in utero infection

In utero infection of the fetus has become recognized as an important cause of fetal and neonatal morbidity and mortality. Since both anatomic and functional abnormalities have been described in the fetus related to various infections, ultrasonography may be a valuable diagnostic tool in this regard. A complete review of the current literature was undertaken to report available information on this topic. Common pathogens or clinical conditions were selected. The identified data were confounded by the way in which each case originally presented for study. Although certain anomalies were frequently associated with individual organisms, their incidence could not be determined, nor were most specific to that infectious agent. Representative ultrasound images are presented for common and unusual cases.     

An expanded Transactional Stress and Coping Model for siblings of children with sickle cell disease: family functioning and sibling coping, self-efficacy and perceived social support

Aim   To investigate the application of an expanded Transactional Stress and Coping Model for the psychological adjustment of non-chronically ill, African-American siblings of children with sickle cell disease (SCD).
Methods   Ninety-seven siblings (M = 11.24 years) from 65 families who care for a child with SCD participated. Primary caregivers completed the Coping Health Inventory for Parents, the Family Relations Scale and the Child Behaviour Checklist, while siblings completed the Kidcope, the Children's Self-Efficacy for Peer Interaction Scale, and the Social Support Scale for Children.
Results   Family processes were predictive of sibling adjustment, revealing that family coping, expressiveness and support improved adjustment, while family conflict predicted poor adjustment.
Conclusion   Findings suggest that family-centered interventions stressing family expressiveness and support, while minimizing conflict, will contribute to sibling psychological adjustment.