"Familial Multiple Coagulation Factor Deficiencies of FXI and FXII in an Asymptomatic Saudi Woman".

Factor XI deficiency (FXI) is the third most common coagulation factor deficiency after hemophilia A and B, ie, in the hierarchy after factors VIII and IX, taking into account von Willebrand's factor deficiency, as bleeding disorders are higher than in hemophilia C. Factor XII deficiency (FXII) is a congenital condition, inherited in the vast majority of cases in an autosomal recessive manner, more often associated with thromboembolic complications. A combination of both factor deficiencies has been found very rarely, and it can be familial multiple coagulation factor deficiency (FMCFD). This study reports the case of a 39-year-old woman from Saudi Arabia who had the combination of FXI and FXII deficiencies, known to be on treatment for hypothyroidism and was referred to a hematology clinic with an incidental finding of prolonged activated partial thromboplastin time (aPTT). Although there was no history of bleeding tendency, her siblings had a family history of an unknown type of bleeding disorder. On physical examination, the patient did not show any bruising, petechiae, or ecchymosis. The aPTT was 69 seconds (27-38) with normal use of other hemostatic agents and was corrected after a 50:50 mixing study. Intrinsic coagulation factors were evaluated, and they revealed severe FXI and moderate FXII deficiencies. Due to a strong family history, the patient was diagnosed with FMCFD. In conclusion, familial combined multiple clotting factor deficiency (FCMFD) is a rare condition that requires attention and reporting. The management strategy in such cases has not been well studied, especially in the long-term symptomatic patient with severe but asymptomatic combined FXI and FXII deficiencies, which is an area for review and further study.

Integration of a Patient-Centered Medical Home Into Ambulatory Health Care Services Centers in Abu Dhabi.

This study describes the transformation of Abu Dhabi Ambulatory Healthcare Services into patient-centered medical homes (PCMH) in 3 pilot sites by applying the National Commission for Quality Assurance (NCQA) PCMH standards. The intervention was system redesign, population management, team building, and optimizing electronic medical records toward patient-centered care. The pilot centers outperformed non-pilot centers in clinical key performance indicators. Based on the NCQA 2011 PCMH criteria, the pilot achieved 84% compared with 42% at the start of the project. In conclusion this study shed light on a firm path to operationalizing PCMH to enhance the quality and access to care.

Effect of extended application time on the efficacy of an in-office hydrogen peroxide bleaching agent: an in vitro study.

PURPOSE: To measure the influence of light activation, color pigments and extended application time on the in vitro bleaching efficacy of a hydrogen peroxide bleaching agent. METHODS: Ninety-six 2.5 mm thick bovine enamel and dentin samples were selected for the study. Samples were stained in a tea solution for three days and then randomly divided into three groups. Bleaching was performed with a 35% hydrogen peroxide gel containing different color pigments. Red pigments were used in group one, black pigments were used in group two, and no pigments (transparent gel) in group three. In these three groups the bleaching agent was applied once every 15 min (4 x 15 min). Then each group was divided into subgroups: with or without light activation. In addition, a fourth group with red pigment was used to test the efficacy of an extended application time (2 x 30 min). All samples were measured with a spectrophotometer to determine the L*a* b* values before and after bleaching. The ΔE value was then calculated. Analysis of variance and Duncan post-hoc test were used to identify differences between groups at a preset a of 0.05. RESULTS: When black and red pigments were used on enamel, light activation of the bleaching gel had a significant effect on the ΔE values. On dentin without light activation, transparent gel was significantly different from the red and black colored gel. There was no significant difference in the ΔE values between 4 applications of 15 min each and 2 applications of 30 min each. CONCLUSIONS: Within the limitations of this in vitro study, no difference in the bleaching effect could be observed between applying the product 2 times of 30 min each and applying the product 4 times of 15 min each. The effect of light activation on colored gel was limited to enamel surface.

The value of renal ultrasound in children with a first episode of urinary tract infection.

BACKGROUND: Urinary tract infection (UTI) is a common pediatric problem. Guidelines recommend obtaining a renal ultrasonogram (RUS) for young children after a first UTI. Our aim was to assess the value of routine RUS in the management of children hospitalized with a first episode of UTI. METHODS: We conducted a retrospective review of the medical records of 130 children 12 years of age or younger admitted with a first UTI. Children were excluded if they had a urinary tract abnormality before admission and/or had been treated with an antibacterial agent within 7 days before admission. The yield of RUS was measured by ability to detect renal abnormalities, by the sensitivity and specificity for detecting vesicoureteral reflux (VUR) using voiding cystourethrogram (VCUG) as a gold standard, and by its influence on UTI management. RESULTS: RUS was conducted in 130 children, but only 118 returned for a VCUG and were included in the study. The findings were positive for VUR in 20 of 40 patients (50%) with a confirmed VUR on VCUG and positive in 18 of 78 patients (23.1%) without VUR on VCUG. Of the 20 patients with a normal RUS who showed VUR, 2 had grade I reflux, 8 had grade II reflux, 7 had grade III reflux and 3 had grade IV reflux. The sensitivity, specificity, positive and negative predictive value of ultrasound in suggesting VUR was 50% and 76.9%, 52.6% and 75%, respectively. Except for one, the result of an abnormal RUS did not alter the management of our patients. CONCLUSION: The results of our study show that the RUS has a little value in the management of children with a first UTI.