Recombinant activated factor VII for intractable bleeding post splenectomy in a patient with myeloproliferative disorder.

Recombinant activated factor VII has been Food and Drug Administration approved to treat hemorrhages in hemophiliac patients with inhibitors and in acquired hemophilia patients. Recombinant activated factor VII use has also been considered for the management of uncontrolled bleeding in a number of congenital and acquired hemostatic abnormalities. The myeloproliferative disorders are a group of clonal hematologic diseases where, frequently, abnormal platelet function is considered a hallmark. This is the first case report addressing the clinical benefit of off-label use of recombinant activated factor VII in an attempt to control intractable bleeding in a patient with a myeloproliferative disorder after splenectomy.     

Mental stress-induced myocardial ischemia in coronary artery disease patients with left ventricular dysfunction

Background  Reduced left ventricular ejection fraction (LVEF) is a risk factor for poor outcomes in patients with coronary artery disease (CAD). Mental stress-induced myocardial ischemia (MSIMI) also identifies a subset of CAD patients at increased risk for future cardiovascular events. Susceptibility to MSIMI in patients with CAD and reduced LVEF is unknown. Methods and Results  We enrolled 182 patients (67 women) with a mean age of 64 years and a documented history of CAD in this study. Baseline resting ejection fraction was determined by use of technetium 99m sestamibi gated single photon emission computed tomography. Abnormal LVEF was defined as less than 45% for men and less than 50% for women (based on published norms for our software [Cedars-Sinai Medical Center]). All participants underwent mental stress testing with a public speaking task. Rest/stress myocardial perfusion single photon emission computed tomography was performed via conventional methodology. Images were visually compared for number and severity of perfusion defects by use of a scoring method from 0 to 4. A summed difference score was calculated as the difference between summed stress and rest scores. A score of greater than 3 was considered abnormal. MSIMI developed in 19% of patients with normal LVEF and 31% of those with reduced LVEF. There is no statistically significant difference between the two groups (P=.11). Conclusions  CAD patients with left ventricular dysfunction are equally susceptible to MSIMI as those with normal LVEF. This study was supported by grants HL 070265 and HL 072059 from the National Heart. Lung, and Blood Institute. This material is also the result of work supported by resources and with the use of facilities at the Department of Veterans. Affairs Medical Center, Gainesville, Fla.     

Laparoscopic flip-flap technique versus conventional inguinal hernia repair in children.

BACKGROUND AND OBJECTIVES: Inguinal hernia repair is one of the most frequently performed pediatric surgical operations. Several pediatric laparoscopic inguinal hernia repair techniques have been introduced. But debate is unresolved regarding the feasibility of laparoscopy for treating pediatric inguinal hernias. METHODS: A retrospective cohort study enrolled 33 patients who underwent congenital inguinal hernia repair by either the new laparoscopic flip-flap technique or conventional open repair. Patients were divided into 2 groups according to the type of surgery: Group A included those who underwent the new laparoscopic technique, and Group B included those who underwent conventional open repair. RESULTS: Group A comprised 15 patients (mean age, 39 months), and group B comprised 18 (mean age, 44 months). Mean operative time was 47.5 minutes for Group A versus 27.5 minutes for Group B. Intraoperative complications for Group A included 1 case (7%) of vas deferens injury, and 3 cases (20%) in which the flaps were torn during suturing. In Group B, no intraoperative complications were encountered. In both groups, the mean postoperative hospital stay was 5.5 hours. Postoperative follow-up of 3 months revealed recurrence in 4 patients in Group A (27%), while there were no recurrences in Group B. CONCLUSION: Our preliminary experience shows unsatisfactory outcomes with laparoscopic flip-flap hernia repair in children. In spite of advancement in the application of laparoscopy in pediatric surgery, conventional open hernia repair is still the gold standard for children, in our experience. Future studies with more numbers and longterm follow-up should be conducted.     

The effect of total arterial grafting on medium-term outcomes following coronary artery bypass grafting

Background  

While it is believed that total arterial grafting (TAG) for coronary artery bypass grafting (CABG) confers improved long-term outcomes when compared to conventional grafting with left internal mammary artery and saphenous vein grafts (LIMA+SVG), to date, this has not become the standard of care. In this study, we assessed the impact of TAG on medium-term outcomes after CABG.     

Haploinsufficiency of Pkd2 is associated with increased tubular cell proliferation and interstitial fibrosis in two murine Pkd2 models.

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited human kidney disease and is caused by germline mutations in PKD1 (85%) or PKD2 (15%). It has been estimated that around 1% of tubular cells give rise to cysts, and cell hyperproliferation has been noted to be a cardinal feature of cystic epithelium. Nevertheless, it is uncertain whether the increase in proliferative index observed is an early or late feature of the cystic ADPKD kidney. METHODS: Two Pkd2 mouse mutants (WS25 and WS183) have been recently generated as orthologous models of PKD2. To determine the effect of Pkd2 dosage on cell proliferation, cyst formation and renal fibrosis, we studied renal tissue from Pkd2(WS25/WS25) and Pkd2(+/-) mice by histological analysis. We also examined the proliferative index in archival nephrectomy tissue obtained from patients with ADPKD and normal controls. RESULTS: The proliferative index of non-cystic tubules in Pkd2 mutant mice as assessed by proliferating cell nuclear antigen and Ki67-positive nuclei was between 1-2%, values 5-10 times higher than control tissue. Similarly, the proliferative index of non-cystic tubules in human ADPKD kidneys was 40 times higher than corresponding controls. In Pkd2 mutant mice, significant correlations were found between the fibrosis score and the mean cyst area as well as with the proliferative index. Of significance, proliferating tubular cells were uniformly positive for polycystin-2 expression in Pkd2(+/-) kidney. CONCLUSION: These results suggest that an increase in cell proliferation is an early event preceding cyst formation and can result from haploinsufficiency at Pkd2. The possible pathogenic link between tubular cell proliferation, interstitial fibrosis and cyst formation is discussed.     

L'atteinte du pied au cours du rhumatisme psoriasique. À propos de 26 cas

Objective. – To describe the clinical and radiological features of foot involvement in patients with psoriatic arthritis.Methods. – We retrospectively reviewed the medical records of patients admitted between 1972 and 1999 for psoriatic arthritis with involvement of the foot. We included all patients who had peripheral and/or axial, asymmetric, chronic inflammatory joint disease meeting or not Avila's radiological criteria for psoriatic arthritis, with or without other imaging findings suggestive of psoriatic arthritis and with or without psoriasis.Results. – Twenty-six patients were included. Inflammatory heel pain was reported by 14 patients, whereas forefoot involvement was found in only seven patients. Sausage toe was present in two patients. None of the patients had Bauer's toe (combining arthritis and psoriatic skin and/or nail changes) or psoriatic onychopachydermoperiostitis of the great toe. Radiological abnormalities were found in 20 patients. Half the patients had calcaneal changes. Osteoperiostitis of the great toe was noted in two patients and mushrooming in five.Discussion. – The features of psoriatic arthritis in Morocco seem similar to those in other countries. Hindfoot involvement was present in 53% of patients. Involvement of the forefoot was rarely recorded in the charts, suggesting missed cases because of insufficient attention to the forefoot during the physical examination and availability of anteroposterior radiographs only. Oblique views (Hirtz and Chaumet) should be obtained because they give a clearer image of the distal part of the toes, which is often difficult to analyze on anteroposterior films.     

Prednisone-Free Maintenance Immunosuppression—A 5-Year Experience

Concern persists that prednisone-free maintenance immunosuppression in kidney transplant recipients will be associated with an increase in late allograft dysfunction and graft loss. We herein report 5-year follow-up of a trial of prednisone-free maintenance immunosuppression. From October 1, 1999, through January 31, 2005, at our center, 589 kidney transplant recipients were treated with a protocol incorporating discontinuation of their prednisone on postoperative day 6. At 5 years, actuarial patient survival was 91%; graft survival, 84%; death-censored graft survival, 92%; acute rejection-free graft survival, 84% and chronic rejection-free graft survival, 87%. The mean serum creatinine level (+/-SD) at 1 year was 1.6 +/- 0.6; at 5 years, 1.7 +/- 0.8. In all, 86% of kidney recipients with functioning grafts remain prednisone-free as of April 30, 2005. As compared with historical controls, recipients on prednisone-free maintenance immunosuppression had a significantly lower rate of a number of complications, including cataracts (p < 0.001), posttransplant diabetes mellitus (p < 0.001), avascular necrosis (p = 0.001), and fractures (p = 0.004). We conclude that prednisone-related side effects can be minimized in a protocol incorporating prednisone-free maintenance immunosuppression. Five-year graft outcome remains good.