Childhood acute lymphoblastic leukemia in the Middle East and neighboring countries: A prospective multi-institutional international collaborative study (CALLME1) by the Middle East Childhood Cancer Alliance (MECCA)

Background: Little is known about childhood ALL in the Middle East. This study was undertaken by MECCA as initial efforts in collaborative data collection to provide clinical and demographic information on children with ALL in the Middle East. Procedure: Clinical and laboratory data for patients with ALL between January 2008 and April 2012 were prospectively collected from institutions in 14 Middle East countries and entered into a custom-built-database during induction phase. All laboratory studies including cytogenetics were done at local institutions. Results: The 1,171 voluntarily enrolled patients had a mean age of 6.1±3.9 years and 59.2 were boys. T-ALL represented 14.8 and 84.2 had B-precursor ALL. At diagnosis, 5.6 had CNS disease. The distribution of common genetic abnormalities reflected a similar percentage of hyperdiploidy (25.6), but a lower percentage of ETV6-RUNX1 translocation (14.7) compared to large series reported from Western populations. By clinical criteria, 47.1 were low/standard risk, 16.9 were intermediate risk, and 36 were high risk. Most patients received all their care at the same unit (96.9). Patients had excellent induction response to chemotherapy with an overall complete remission rate of 96. Induction toxicities were acceptable. Conclusions: This first collaborative study has established a process for prospective data collection and future multinational collaborative research in the Middle East. Despite the limitations of an incomplete population-based study, it provides the first comprehensive baseline data on clinical characteristics, laboratory evaluation, induction outcome, and toxicity. Further work is planned to uncover possible biologic differences of ALL in the region and to improve diagnosis and management. Pediatr Blood Cancer 2014; 61:1403-1410. © 2014 Wiley Periodicals, Inc

Spontaneous splenic rupture in a patient with factor XIII deficiency and a novel mutation

We report a novel mutation in factor XIIIA gene that caused severe congenital factor XIII deficiency in a 6 year and 8 month old male. The mutation is a GA deletion in the core domain leading to a premature stop at codon 502. The child had severe deficiency with two episodes of intracerebral hemorrhage. He also developed spontaneous splenic rupture, an unusual complication of this disorde

Addition of ketamine to propofol for initiation of procedural anesthesia in children reduces propofol consumption and preserves hemodynamic stability

Background: There is no ideal anesthesia protocol to perform short invasive procedures in pediatric oncology. The combination of propofol and ketamine may offer advantages over propofol alone. Methods: In a prospective, randomized, double-blind study, we analyzed 63 consecutive procedures performed in 47 oncology children. All patients received 1 mu g/kg fentanyl, followed by propofol 1 mg/kg in group P (n=33) or propofol 0.5 mg/kg and ketamine 0.5 mg/kg in group PK (n=30) for the initiation of anesthesia. The need for supplementation with propofol and/or fentanyl to maintain an adequate level of anesthesia was recorded. The hemodynamic and respiratory profile, recovery time and the occurrence of side effects were compared. Results: Significantly more children required propofol (100% vs. 83.3%) and fentanyl (75.5% vs. 43.3%) rescue doses, and developed hypotension (63.6% vs. 23.4%) and bradycardia (48.5 vs. 23.4%) in group P compared with group PK, with a comparable incidence of respiratory adverse events and recovery times. However, 40% of children in group PK were agitated following recovery compared with 6% in group P. Conclusions: The combination of propofol and ketamine for invasive procedures in pediatric oncology resulted in reduced propofol and fentanyl consumption and preserved hemodynamic stability, but more children in the combination group recovered with agitation