The treatment of childhood acute lymphoblastic leukemia in Guatemala: Biologic features, treatment hurdles, and results.

BACKGROUND: The National Pediatric Oncology Unit (UNOP) is the only pediatric hemato-oncology center in Guatemala. METHODS: Patients ages 1 to 17 years with acute lymphoblastic leukemia (ALL) were treated according to modified ALL Intercontinental Berlin-Frankfurt-Münster (IC-BFM) 2002 protocol. Risk classification was based on age, white blood cell count, immunophenotype, genetics (when available), and early response to therapy. RESULTS: From July 2007 to June 2014, 787 patients were treated, including 160 who had standard-risk ALL, 450 who had intermediate-risk ALL, and 177 who had high-risk ALL. The induction death rate was 6.6%, and the remission rate was 92.9%. The rates of death and treatment abandonment during first complete remission were 4.8% and 2.5%, respectively. At a median observation time of 3.6 years, and with abandonment considered an event, the 5-year event-free survival and overall survival estimates ( ± standard error) were 56.2% ± 2.1% and 64.1% ± 2.1%, respectively, with a 5-year cumulative incidence of relapse of 28.9% ± 2.0%. Twenty-one of 281 patients (7.5%) investigated were positive for the ets variant 6/runt-related transcription factor 1 (ETV6/RUNX1) fusion. CONCLUSIONS: A well organized center in a low-middle-income country can overcome the disadvantages of malnutrition and reduce abandonment. Outcomes remain suboptimal because of late diagnosis, early death, and a high relapse rate, which may have a partly genetic basis. Earlier diagnosis, better management of complications, and better knowledge of ALL will improve outcomes. Cancer 2017;123:436-448. © 2016 American Cancer Society.

Pediatric cardiac surgery: a challenge and outcome analysis of the Guatemala effort.

A large underserved population of children with congenital cardiac malformation (CCM) exists in many developing countries. In recent years, several strategies have been implemented to supplement this need. These strategies include transferring children to first-world countries for surgical care or the creation of local pediatric cardiovascular surgical programs. In 1997, an effort was made to create a comprehensive pediatric cardiac care program in Guatemala. The objective of this study is to examine the outcome analysis of the Guatemala effort. The goals of our new and first pediatric cardiac care program were to: 1) provide diagnosis and treatment to all children with a CCM in Guatemala; 2) train of local staff surgeons, 3) established a foundation locally and in the United States in 1997 to serve as a fundraising instrument to acquire equipment and remodeling of the pediatric cardiac unit and also to raise funds to pay the hospital for the almost exclusively poor pediatric cardiac patients. The staff now includes 3 surgeons from Guatemala, trained by the senior surgeon (A.R.C.), seven pediatric cardiologists, 3 intensivists, and 2 anesthesiologists, as well as intensive care and ward nurses, respiratory therapists, echocardiography technicians, and support personnel. The cardiovascular program expanded in 2005 to 2 cardiac operating rooms, 1 cardiac catheterization laboratory, 1 cardiac echo lab, 4 outpatients clinics a 6-bed intensive care unit and a 4-bed stepdown unit, a 20 bed general ward (2 beds/room) and a genetics laboratory. Our center has become a referral center for children from Central America. A total of 2,630 surgical procedures were performed between February 1997 and December 2007, increasing the number of operations each year. Postoperative complication occurred in 523 of 2,630 procedures (20%). A late follow-up study was conducted of all the patients operated from 1997 to 2005. Late mortality was 2.7%. Development of a sustainable pediatric cardiac program in emerging countries presents many difficult challenges. Hard work, perseverance, adaptability, and tolerance are useful aptitudes to develop a viable PCP in an "emerging" country. We are not in favor of Medical-Surgical Safari efforts, unless these efforts include training of a local team and eventual unit independence. It helps if an experienced (+/- senior/retired!) surgeon leads this effort on a full-time, pro bono basis. Local and international fund raising is essential to complement vastly insufficient government subsidies.