ABO-incompatible blood transfusion and invasive therapeutic approaches during pediatric cardiopulmonary bypass

Human error has been identified as a major source of ABO-incompatible blood transfusion which most often results from blood being given to the wrong patient. We present a case of inadvertent administration of ABO-incompatible blood to a 6-mo-old child who underwent congenital heart surgery and discuss the use of invasive therapeutic approaches. Invasive techniques included total circulatory arrest and large-volume exchange transfusion, along with conventional ultrafiltration and plasmapheresis, which could all be performed rapidly and effectively. The combination of standard pharmacologic therapies and alternative invasive techniques after a massive ABO-incompatible blood transfusion led to a favorable outcome in our patient.     

Polyarteritis nodosa in a case of familial Mediterranean fever

We describe a 7-year-old boy with familial Mediterranean fever (FMF) complicated by polyarteritis nodosa (PAN) with distinct angiographic findings. On admission, he had abdominal pain, arthralgia, and severe fibromyalgia. During hospitalization, he displayed maculopapular eruptions, high blood pressure, gastrointestinal bleeding, and persistent constitutional symptoms mimicking a vasculitic process, most probably PAN. Renal angiography showed a perfusion defect compatible with a renal infarction secondary to a vasculitic process. He responded well to pulse methylprednisolone therapy with colchicine. We emphasize the rare association of FMF and PAN and the non-aneurysmal angiographic signs of PAN.     

Endovascular treatment of obstructive iliac artery dissections

Purpose: To report our results from a study of the endovascular treatment of flow restricting chronic atherosclerotic or catheter-induced segmental iliac artery dissections with bare stents.

Material and Methods: Thirty symptomatic patients with 32 lesions, including chronic atherosclerotic (n = 21) and catheter-induced (n = 11) segmental arterial dissections, were treated with primary stenting. The common iliac artery was involved in 19 lesions and the external iliac artery in the remaining 13. Two patients had two lesions in the same vessel. Technical success was defined as restoration of the smooth contoured luminal patency with no more than 20% residual stenosis in diameter in atherosclerotic dissections associated with plaque formation or total obliteration of the false lumen in catheter-induced dissections. Complete relief of, or marked improvements in, presenting symptoms, or at least single category improvement, was assessed for clinical success.

Results: Technical success rate was 100%. No procedure-related complications such as distal emboli or early occlusions were observed. Complete symptom relief was achieved in all patients with catheter-induced dissection and in all but three cases with chronic spontaneous atherosclerotic dissection. In two cases, occlusion of the stents occurred during the follow-up period. Clinical and radiological mean follow-up for 24 months (range 3-55) revealed patency of all other stented segments. Cumulative primary patency rate was 97% over 12 months and 90% over 24 months.

Conclusion: Endovascular treatment of chronic atherosclerotic and catheter-induced short obstructive iliac arterial dissections with bare stents is safe and effective. Patency of the diseased arterial segment with a smooth lumen can be sustained for an extensive period.     

Difficult intubation in pediatric cardiac anesthesia

OBJECTIVE: The aim of this study was to investigate cases of difficult intubation in pediatric cardiac surgical patients and to evaluate the importance of associated congenital abnormalities. DESIGN: Retrospective analysis. SETTING: Departments of Anesthesiology and Pediatric Cardiovascular Surgery of a tertiary university hospital. PARTICIPANTS: All children undergoing congenital heart surgery. INTERVENTIONS: Patients who had difficult intubations according to their anesthetic charts were further evaluated from hospital files for demographic characteristics, associated congenital abnormalities, and perioperative airway and/or respiratory complications. MEASUREMENTS AND MAIN RESULTS: A total of 1,278 pediatric patients with congenital heart disease were operated on from January 1999 to July 2002. Difficult intubation was encountered in 16 cases (1.25%). Two of these were newborns, 11 were infants, and 3 were in the pediatric age group. Anterior larynx was the most common reason for difficult intubation (7 cases, 43.7%). There were associated syndromes and/or other congenital abnormalities in 8 children (50%). CONCLUSION: The likelihood of difficult intubation during pediatric cardiac surgery, especially in cases with other congenital pathologies should be kept in mind, and the anesthetic approach must be planned accordingly.     

Performance of a new clinical grading system for chronic graft-versus-host disease: a multicenter study

We recently reported 3 risk factors (RFs) at diagnosis of chronic graft-versus-host disease (cGVHD) that were significantly associated with increased nonrelapse mortality. These included extensive skin involvement (ESI), thrombocytopenia (TP), and progressive type of onset (PTO). The hazard ratio (HR) for mortality of the patients with prognostic score (PS) between 0 and 2 (intermediate-risk; 1 RF) compared to those with PS 0 (favorable-risk; 0 RF) was 3.7 (95% CI, 1.4, 9.3); the HR for patients with PS equal to or more than 2 (high-risk; > 1 RF) compared with intermediate-risk group was 6.9 (3.8, 12.4). A rare presentation of TP and PTO without ESI yielded a PS of 1.8 (intermediate-risk). This paper reports the performance of the prognostic model and the individual RFs using data from an additional 1105 patients from University of Nebraska (n = 60), International Bone Marrow Transplantation Registry (n = 708), Fred Hutchinson Cancer Research Center (n = 188), and University of Minnesota (n = 149). The extent of skin involvement was quantified in 3 cohorts using the available data collected in different formats before the analysis. Although the HR for mortality of the patients in the intermediate-risk group versus those in the favorable-risk group ranged from 2.3 to 8.9 across the centers, it was between 1.6 to 6.9 for patients in the high-risk group versus those in the intermediate-risk group. Although TP itself was uniformly associated with increased risk of mortality across all test samples, ESI and PTO showed statistically significant associations with mortality in 1 and 2 cohorts, respectively. In conclusion, the model was predictive of cGVHD-specific survival, but the mortality hazard associated with ESI was lower in each of these test samples compared with the learning sample. Although the new clinical grading based on the model is promising because of its utility across multiple independent data sets, prospective validation is needed.