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.     

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.     

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.     

Mechanisms of Tolerance Induced by Donor-Specific Transfusion and ICOS-B7h Blockade in a Model of CD4+ T-Cell-Mediated Allograft Rejection

The inducible co-stimulatory molecule (ICOS) has been shown to play a critical role in T-cell activation and differentiation, and the regulation of alloimmune responses in vivo. Using an MHC class II mismatched model of CD4(+) T-cell-mediated rejection, we found that treatment of mice with DST and ICOS-B7h blockade induced long-term skin allograft survival and donor-specific transplantation tolerance. ICOS blockade, either during antigen priming or during the effector phase, previously shown to alter the outcome of the immune response, had a similar effect on graft survival. DST and anti-B7h mAb reduced the frequency of IFN-gamma-producing allospecific cells but did not produce deviation to a T(H)2 phenotype. In an adoptive transfer model using ABM TCR transgenic mice directly reactive to I-A(bm12), DST and anti-B7h mAb reduced the number of allospecific CD4(+) T cells and increased CD4(+) T-cell apoptosis. These data demonstrate that DST and anti-B7h mAb induces transplantation tolerance to MHC class II mismatched skin grafts by a reduction of the alloreactive clone size that is, at least in part, dependent on apoptosis of host alloantigen-specific CD4(+) T cells.     

Séquelles fonctionnelles de la chirurgie carcinologique du rectum

The development of therapeutic options for rectal cancer strives to reach the ultimate goals of improved local control and overall survival. However, preserving continence, as well as genitourinary and sexual function, remains elusive in some patients. Loss of bowel control may be the most unsettling factor in terms of quality of life. Patients must be accurately informed about the surgery’s possible adverse effects, and surgeons must offer the best options (e.g. colostomy bag or sphincteroplasty) in order to minimize these complaints.     

Bench-to-bedside review: Resuscitation in the emergency department

Over the past decade the practice of acute resuscitation and its monitoring have undergone significant changes. Utilization of noninvasive mechanical ventilation, goal-directed therapy, restricted fluid volume, blood transfusion and minimally invasive technology for monitoring tissue oxygenation have changed the practice of acute resuscitation. Early diagnosis and definitive treatment of the underlying cause of shock remains the mainstay for survival after successful resuscitation. Patient-centered outcome end-points, in addition to survival, are being utilized to appraise the effectiveness of treatment. Application of medical ethics to the ever changing practice of acute resuscitation has also become a societal expectation.