The effect of short-term preoperative nutritional intervention for cleft surgery eligibility.

BACKGROUND: Children with orofacial clefts are highly susceptible to malnutrition, with severe malnutrition restricting their eligibility to receive safe surgery. Ready-to-use therapeutic foods (RUTF) are an effective treatment for malnutrition; however, the effectiveness has not been demonstrated in this patient population prior to surgery. We studied the effectiveness of short-term RUTF use in transitioning children with malnutrition, who were initially ineligible for surgery, into surgical candidates. METHODS: A cohort of patients from Ghana, Honduras, Malawi, Madagascar, Nicaragua, and Venezuela enrolled in a nutrition program were followed by Operation Smile from June 2017 to January 2020. Age, weight, and length/height were tracked at each visit. Patients were included until they were sufficiently nourished (Z > = -1) with a secondary outcome of receiving surgery. The study was part of a collaborative program between Operation Smile (NGO), Birdsong Peanuts (peanut shellers and distributors), and MANA Nutrition (RUTF producer). RESULTS: A total of 556 patients were recruited between June 2017 and January 2020. At baseline 28.2% (n = 157) of patients were diagnosed with severe, 21.0% (n = 117) moderate, and 50.7% (n = 282) mild malnutrition. 324 (58.3%) presented for at least one return visit. Of those, 207 (63.7%) reached optimal nutrition status. By visit two, the mean z-score increased from -2.5 (moderate) to -1.7 (mild) (p < 0·001). The mean time to attain optimal nutrition was 6 weeks. There was a significant difference in the proportion of patients who improved by country(p < 0.001). CONCLUSION: Malnutrition prevents many children with orofacial clefts in low- and middle-income countries from receiving surgical care even when provided for free. This creates an even larger disparity in access to surgery. In an average of 6 weeks with an approximate cost of $25 USD per patient, RUTF transitioned over 60% of patients into nutritionally eligible surgical candidates, making it an effective, short-term preoperative nutritional intervention. Through unique partnerships, the expansion of cost-effective, large-scale nutrition programs can play a pivotal role in ensuring those at the highest risk of living with unrepaired orofacial clefts receive timely and safe surgical care.

The Personal Impact of Involvement in International Global Health Outreach: A National Survey of Former Operation Smile Student Volunteers.

BACKGROUND: Humanitarian surgical organizations such as Operation Smile provide global health opportunities for students and medical trainees. Prior studies have shown a positive benefit for medical trainees. This study aimed to determine if the international global health experiences of young student volunteers impact their career choices as adults. METHODS: A survey was sent to adults who were involved with Operation Smile as students. The survey elicited information about their mission trip experience, education, career, and current volunteer and leadership activities. Data were summarized with descriptive statistics and qualitative analysis. RESULTS: 114 prior volunteers responded. The majority participated in leadership conferences (n = 110), mission trips (n = 109), and student clubs (n = 101) while in high school. Many graduated from college (n = 113, 99%) and completed post-graduate degrees (n = 47, 41%). The most highly represented occupational industry was healthcare (n = 30, 26%), including physicians and medical trainees (n = 9), dentists (n = 5), and other healthcare providers (n = 5). Three-fourths reported that their volunteer experience impacted their career choice, and half reported that their experience allowed them to connect with career mentors. Their experience was associated with the development of leadership skills, including public speaking, self-confidence, and empathy, and increased awareness of cleft conditions, health disparities, and other cultures. Ninety-six percent continued to volunteer. Narrative responses revealed that the volunteer experiences impacted their inter- and intrapersonal development into adulthood. CONCLUSIONS: Participation in a global health organization as a student may encourage a long-term commitment to leadership and volunteerism and foster interest in a healthcare career. These opportunities also encourage development of cultural competency and interpersonal skills. LEVEL OF EVIDENCE: III, Cross-Sectional Study.

High prevalence of deleterious mutations in concomitant nonsyndromic cleft and outflow tract heart defects.

Our previous work demonstrating enrichment of outflow tract (OFT) congenital heart disease (CHD) in children with cleft lip and/or palate (CL/P) suggests derangements in common underlying developmental pathways. The current pilot study examines the underlying genetics of concomitant nonsyndromic CL/P and OFT CHD phenotype. Of 575 patients who underwent CL/P surgery at Children's Hospital Los Angeles, seven with OFT CHD, negative chromosomal microarray analysis, and no recognizable syndromic association were recruited with their parents (as available). Whole genome sequencing of blood samples paired with whole-blood-based RNA sequencing for probands was performed. A pathogenic or potentially pathogenic variant was identified in 6/7 (85.7%) probands. A total of seven candidate genes were mutated (CHD7, SMARCA4, MED12, APOB, RNF213, SETX, and JAG1). Gene ontology analysis of variants predicted involvement in binding (100%), regulation of transcription (42.9%), and helicase activity (42.9%). Four patients (57.1%) expressed gene variants (CHD7, SMARCA4, MED12, and RNF213) previously involved in the Wnt signaling pathway. Our pilot analysis of a small cohort of patients with combined CL/P and OFT CHD phenotype suggests a potentially significant prevalence of deleterious mutations. In our cohort, an overrepresentation of mutations in molecules associated with Wnt-signaling was found. These variants may represent an expanded phenotypic heterogeneity within known monogenic disease genes or provide novel evidence of shared developmental pathways. The mechanistic implications of these mutations and subsequent developmental derangements resulting in the CL/P and OFT CHD phenotype require further analysis in a larger cohort of patients.

Cleft Lip and Palate Research in Low- and Middle-income Countries: A Scientometric Analysis.

Cleft lip and palate (CLP) comprise over 90% of the world's congenital anomalies and cause significant disability worldwide, while disproportionally burdening low- and middle-income countries (LMICs). Research can help inform strategies that reduce disparities in accessing CLP care. We performed a scientometric analysis of CLP research in LMICs to identify influential contributors and themes. METHODS: The authors searched seven citation databases accessed via Web of Science, from inception to March 2, 2021. Social network analysis was done using VOSviewer. The Kruskal-Wallis test and linear regression were used. RESULTS: In total, 1561 articles authored by 6414 researchers affiliated with 2113 organizations in 119 countries were included. Most authors (n = 6387, 99.6%) had published two or more articles. The USA (454 articles), Brazil (211 articles), China (175 articles), and India (127 articles) published the most. The most prolific institutions were the University of Sao Paulo (94 articles), the University of Pittsburgh (57 articles), and the University of Iowa (55 articles). Marazita ML (33 articles), Shi B (27 articles), and Murray JC (22 articles) had the highest number of publications. An estimated 510 articles (32.7%) were focused on epidemiology, 240 (15.4%) on management, and 54 (3.5%) on global plastic surgery for CLP. CONCLUSIONS: LMICs are disproportionally burdened by CLP, but research is limited and often produced by high-income countries. This study elucidates partnership and health system strengthening opportunities to improve LMIC research capacity and ultimately informs the management and outcomes for patients with CLP.