Diagnostic and Therapeutic Challenges of Hereditary Tyrosinemia Type 1 in Lebanon: A 12-Year Retrospective Review.

Background: Hereditary tyrosinemia type 1 is a rare genetic disorder leading to liver cirrhosis and hepatocellular carcinoma. Few decades ago, dietary measures and ultimately liver transplant constituted the only treatment modalities. Nowadays, early diagnosis and therapy with nitisinone can reverse the clinical picture. In developing countries, diagnostic and therapeutic challenges may affect the outcome of this disease. The choice of the treatment modality may depend on the economic status of each country. Few reports on the long-term outcome of hereditary tyrosinemia type 1 are available from developing and Arab countries. Methods: A retrospective study of charts of Lebanese patients diagnosed with tyrosinemia type 1 and followed, at the American University of Beirut, during a 12-year period was performed. Clinical presentation and liver biochemical profile at diagnosis were analyzed, along with therapeutic modalities and long-term outcome. Results: Twenty-two children were diagnosed and followed during the study period. Median age at diagnosis was 7 months (range: one day to 35 months). Most of the patients presented with hepatomegaly and jaundice. Four patients were referred for atypical presentations with developmental delay and seizures, secondary to undiagnosed hypoglycemia episodes. Around half of the patients presented with failure to thrive. Transaminitis, cholestasis and increased α-fetoprotein level were variably present at diagnosis (36% to 50%). All patients had elevated plasma tyrosine and urinary succinylacetone levels. Genetic testing was performed in 9%. Only one third could be treated with nitisinone. Liver transplant was electively performed in 9% of cases, to overcome the long-term cost of nitisinone. One third of the patients died between the age of 1 month and 11 years. Surviving patients are still candidates for liver transplant. Conclusion: Our experience reflects the challenges of diagnosis and treatment of hereditary tyrosinemia type 1 in a developing country. In the absence of specific neonatal screening, early diagnosis relies mostly on the clinical awareness of the physician. Long-term nitisinone use may be deterred by its high cost and liver transplantation carries risks of surgical complications. New, effective, and less expensive treatments are needed, especially for developing countries.

Acute Kidney Injury Post-cardiac Surgery in Infants and Children: A Single-Center Experience in a Developing Country.

Introduction: The incidence of acute kidney injury (AKI) in pediatric patients following cardiac surgery varies between 15 and 64%, with a mortality rate of 10-89% among those requiring dialysis. This variation in the incidence and mortality of AKI across studies is probably due to the inconsistent definitions used for AKI. The purpose of this study is to present our experience with AKI post-cardiac surgery with emphasis on predisposing or aggravating factors. Patients and Methods: We evaluated the incidence of AKI using the KDIGO criteria in 150 infants and children undergoing cardiac surgeries between 2015 and 2017. Post-operatively, all patients were admitted to the pediatric intensive care unit (PICU) at a tertiary care center in a developing country. This is a retrospective chart review in which data collected included age, gender, type of heart disease, prior cardiac surgeries, RACHS-1 category, and pre- and post-operative creatinine levels. Neonates were not included in this study. Results: Six percent of the studied patients were below 1 year of age, 84% 1-10 years, and 10% 10-18 years. Fourteen patients (9.3%) developed AKI. Patients with cyanotic heart disease were more prone to develop AKI (78%) compared to those with non-cyanotic heart disease (44%). Children with AKI had a higher length of stay in PICU, 2.56 ± 1.44 vs. 4 ± 2.66 (p- 0.02). Serum lactic acid was higher in patients who developed AKI with a mean value of 6.8 ± 6.9 vs. 2.85 ± 1.55 mmol/l in the non-AKI group (p- 0.03). Lower hemoglobin levels and hyperlactic acidemia were significantly more prevalent in the AKI group. There were five deaths in this series (3.3%), and four of those (80%) were in the AKI group. Conclusion: Using the KDIGO criteria, the incidence of AKI in infants and children following cardiac surgery was 9.3%. This is slightly lower than in previously published studies where the range was between 15 and 64%. Children with cyanotic cardiac disease, hyperlactic acidemia, and anemia were more prone to developing AKI. Identifying patients at risk might help decrease the risk of post-operative AKI.

Cardiac involvement in a child post COVID-19: a case from Lebanon.

We report on an 8-year-old boy, who presented to the emergency department at our institution with fever, generalised oedema and hypotension. Investigations revealed anaemia, thrombocytopenia in addition to elevated serum inflammatory markers, a negative COVID-19 PCR test and a positive COVID-19 IgG. His echocardiography was consistent with carditis in otherwise morphologically normal heart with depressed cardiac function, moderate-to-severe mitral valve regurgitation, moderate tricuspid regurgitation with an estimated right ventricular systolic pressure half systemic, trace aortic regurgitation, bilateral small pleural effusions, distended inferior vena cava and normal coronaries. He was started on inotropic support, intravenous immunoglobulin and methylprednisolone, and was transferred to the paediatric intensive care unit. To the best of our knowledge, this was the first case of multisystem inflammatory syndrome in children encountered in Lebanon. The presentation and management were thoroughly described in this article aiming to share our experience and to contribute to the rapidly emerging literature on this syndrome.

Health Care Workers in the setting of the "Arab Spring": a scoping review for the Lancet-AUB Commission on Syria.

BACKGROUND: "Health Care Workers in Conflict Areas" emerged as one of the priority themes for a Lancet Commission addressing health in conflict. The objective of our study was to conduct a scoping review on health workers in the setting of the Syrian conflict, addressing four topics of interest: violence against health care workers, education, practicing in conflict setting, and migration. METHODS: Considering the likelihood of scarcity of data, we broadened the scope of the scoping review to include indirect evidence on health care workers from other countries affected by the "Arab Spring". We electronically searched six electronic databases. We conducted descriptive analysis of the general characteristics of the included papers. We also used the results of this scoping review to build an evidence gap map. RESULTS: Out of the 11 165 identified citations, 136 met our eligibility criteria. The majority of the articles tackled the issue of violence against health care workers (63%) followed by practicing in conflict setting (19%), migration (17%) and education (10%). Countries in focus of most articles were: Syria (35%), Iraq (33%), and Bahrain (29%). News, editorials, commentaries and opinion pieces made up 81% of all included papers, while primary studies made up only 9%. All the primary studies identified in this review were conducted on Iraq. Most of the articles about violence against health care workers were on Bahrain, followed by Syria and Iraq. The first and corresponding authors were most frequently affiliated with institutions from non-Arab countries (79% and 79% respectively). CONCLUSIONS: Research evidence on health care workers in the setting of the "Arab Spring" is scarce. This review and the gap map can inform the research agendas of funders and researchers working in the field of health care workers in conflict setting. More well-designed primary studies are needed to inform the decisions of policymakers and other interested parties.

Public health journals' requirements for authors to disclose funding and conflicts of interest: a cross-sectional study.

BACKGROUND: Public health journals need to have clear policies for reporting the funding of studies and authors' personal financial and non-financial conflicts of interest (COI) disclosures. This study aims to assess the policies of public health journals on reporting of study funding and the disclosure of authors' COIs. METHODS: This is a cross-sectional study of "Public, Environmental & Occupational Health" journals. Teams of two researchers abstracted data in duplicate and independently using REDCap software. RESULTS: Of 173 public health journals, 155 (90%) had a policy for reporting study funding information. Out of these, a majority did not require reporting of the phase of the study for which funding was received (88%), nor the types of funding sources (87%). Of the 173 journals, 163 (94%) had a policy requiring disclosure of authors' COI. However, the majority of these journals did not require financial conflicts of interest disclosures relating to institutions (75%) nor to the author's family members (90%) while 56% required the disclosure of at least one form of non-financial COI. CONCLUSIONS: The policies of the majority of public health journals do not require the reporting of important details such as the role of the funder, and non-financial COI. Journals and publishers should consider revising their editorial policies to ensure complete and transparent reporting of funding and COI.

Authorship in reports of clinical practice guidelines: A systematic cross-sectional analysis.

BACKGROUND: A transparent and explicit reporting on authors' contributions to the development of clinical practice guidelines and on panelists' characteristics is essential for their credibility and trustworthiness. We did not find published studies on authorship or panel involvement in clinical practice guidelines. OBJECTIVE: To describe the approach to authorship in reports of clinical practice guidelines, and the characteristics of individual authors. METHODS: We conducted a cross-sectional survey of guidelines listed in the National Guideline Clearing House (NGC) in 2016. We abstracted data on the general characteristics of the guidelines, report approach to authorship, and individual authors characteristics. Data abstraction was in duplicate and independent manner using standardised form. Data analyses were both descriptive and regression analyses. RESULTS: Overall, 139 eligible guidelines with published papers were identified. Of these, 48 (35%) included a group authorship statement in the author byline. A third of these guidelines (n = 45; 32%) reported on authors' contributions, while about half of the guidelines (n = 74; 53%) reported who of the authors served as panel members. Around one-fifth of the guidelines (n = 30; 22%) reported group membership (eg, content expert, patient representative) for at least 1 author. Less than one-seventh of the eligible guidelines indicated who selected the panel members (n = 18; 13%), reported the types of panel members (n = 18; 13%) or the selection criteria (n = 12; 9%). Higher journal impact factor was associated with both "reporting of the author contributions" (OR = 1.07) and "the inclusion of a panel membership section in the guideline report" (OR = 1.21). CONCLUSION: Low percentages of clinical practice guidelines report information on important aspects of authorship and characteristics of individual authors. Better reporting of some of these criteria was associated with journal impact factor.

Factors associated with waterpipe tobacco smoking among Lebanese women.

Waterpipe tobacco smoking (WTS) has become a serious public health threat in the Middle East and beyond. To estimate the prevalence rates of cigarette and WTS among Lebanese women and describe characteristics associated with each behavior. Secondary analysis of women's health data obtained in a national survey in 2010. Of 2,255 selected women, 78 percent reported no or past long-term WTS. Among the 12 percent of regular waterpipe smokers, 40 percent were light users (mean three waterpipe heads weekly), while 60 percent were heavy users (mean of 11 heads per week). About 70 percent were never or past long-term cigarette smokers. Younger age, location within Greater Beirut (GB) and having professional employment were significantly associated with use of WTS. Older age, GB location, lower education, and ever-married were significantly associated with cigarette smoking. WTS is becoming a socially normative behavior among empowered professional women, who can spare the time and expense to engage in this behavior in easily accessible cafés, which prohibit cigarette smoking. Government, media, and NGO campaigns against smoking should target waterpipe use, not only in Lebanon but also across the Arab world and among Arab communities in the Diaspora with messages different from anti-cigarette campaigns.