Summary:Until recently, therapy for patients with severe congenital dyserythropoietic anemia (CDA) has been limited to blood transfusions and chelation therapy. Three children with transfusion-dependent CDA type I underwent allogeneic stem cell transplantation (SCT) from matched sibling donors. Conditioning was with cyclophosphamide 50 mg/kg/day for 4 days, busulphan 4 mg/kg/day for 4 days, and antithymocyte globulin (ATG) 30 mg/kg for four doses pre-SCT. All patients engrafted and are alive, and transfusion independent. To our knowledge, this is the first report of successful SCT in the management of CDA type I.
The use of chloral hydrate sedation for ophthalmic evaluation and imaging was safe and effective in this patient population with a high rate of procedure completion.
Summary:Five patients with confirmed Fanconi's anemia (FA) and myelodysplasia and/or leukemia underwent stem cell transplantation (SCT) from related donors at KFSHRC. The median age at SCT was 12.6 year (range, 6.2-15 years). Conditioning regimen consisted of cyclophosphamide (CY) 5 mg/kg/day i.v. for 4 days, total body irradiation (TBI) 450 cGy in a single dose. Graft-versus-host disease (GVHD) prophylaxis was with cyclosporine and antithymocyte globulins (ATG). The median time to engraftment (defined as ANCX0.5 Â 10 9 /l) was 16 days (range, 12-26 days). The median time to a self-sustaining platelet count of X20 Â 10 9 /l was 27 days (range, 12-40 days). All patients engrafted. Two patients developed acute GVHD; one of the gut (grade 3) and the other of the skin (grade 1), and one patient developed chronic GVHD of the liver. Four are alive and well with no evidence of the disease; one patient died of bacterial sepsis after controlling her GVHD and clearing her pulmonary aspergillosis and CMV infection. We conclude that the use of low-dose CY plus TBI in patients with FA and MDS/AML undergoing SCT is adequate; the regimen is well tolerated and may be curative for such patients.
Background:We present the incidence and determinants of endophthalmitis between July 2010 and June 2012 at King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia. On its basis, we recommended recommendations to strengthen the infection prevention and control strategies.Methods:This is a retrospective review of health records type of study. The details of cases reported having endophthalmitis among those operated in 2 years of study period were studied. The incidence of endophthalmitis was calculated for different eye surgeries and epidemiological variables. The causative organisms in vitreous tap were reviewed. The visual outcomes 6 weeks following intervention/treatment of endophthalmmitis were also studied.Results:Of the 22,554 cases operated, 17 developed endophthalmitis. The incidence was 0.08% (95% confidence interval [CI] 0.04–0.11). The incidence of endophthlamitis among cataract surgeries was 0.12% (95% CI 0.04–0.21). Five specimens did not show any bacteria or fungus. Staphylococcus epidermis (3 cases) was the main pathogen identified. In 8 (47%) eyes, vision deteriorated in spite of treatment. In 5 (29%) eyes, it became stable and in 4 (23.5%) eyes, it improved following treatment. Signs of infection were noted in 1st week, 3 weeks and 12 weeks in 4, 6 and 5 eyes respectively. Late presentation of infection (6 months postoperatively) was reported in two eyes.Conclusions:A vigilant infection control unit in a large eye hospital helps in monitoring endophthalmitis related catastrophes and suggests timely preventive measures to reduce the occurrence and appropriate measures to limit visual disabilities following eye surgery related endophthalmitis.
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