Fouad, Y., M. Alboraie, M. El-Shabrawi, and M. - H. Zheng, "Letter to the Editor: How F to S turned the premature to be mature?", Hepatology (Baltimore, Md.), vol. 79, issue 6, pp. E157-E158, 2024.
El-Anwar, N., M. El-Shabrawi, O. O. Shahin, R. Abdel Kareem, A. M. Salama, and sherif Baroudy, "Health-related quality of life and cognitive function in children with Crigler-Najjar syndrome type 1.", Paediatrics and international child health, vol. 44, issue 1, pp. 18-23, 2024. Abstract

BACKGROUND: The aim of the study was to assess the health-related quality of life (HRQOL) and cognitive function in patients with Crigler-Najjar syndrome (CNS) type I and its impact on their lives.

METHODS: Twenty-one patients diagnosed with CNS type I aged 1 month to 18 years in the Paediatric Hepatology Unit of Cairo University Children's Hospital were enrolled in this cross-sectional observational study. The patients' health-related quality of life (HRQOL) was assessed using the World Health Organization Quality Of Life BREF questionnaire (WHOQOL-BREF) and the Short Form 36 Health Survey Questionnaire (SF-36). Cognitive function was assessed using the Stanford-Binet Intelligence Scale: Fifth Edition (SB5).

RESULTS: All patients had a history of admission to a neonatal intensive care unit, 17 were managed by phototherapy only and 5 also underwent exchange transfusion. According to the WHOQOL questionnaire, 11 cases (52.4%) had a low QOL score, and 7 of 13 patients had an average score for their total IQ test. Cases with poor compliance to phototherapy had statistically significantly lower QOL scores (=0.001), while, according to the SF36 survey, cases who received exchange transfusion had statistically significantly higher cognitive function (=0.03). There was a positive correlation between the neurological effect as a complication of the disease and poor physical QOL.

CONCLUSION: Paediatric patients with CNS have significantly lower HRQOL, especially physically, psychologically and environmentally. It is recommended that assessment of HRQOL should be a routine part of follow-up in CNS patients. Patients whose HRQOL is affected receive regular psychiatric counselling, social support and rehabilitation. CNS: Crigler-Najjar syndrome; HRQOL: health-related quality of life; IQ: intelligence quotient; NICU: neonatal intensive care unit; QOL: quality of life; SB5: Stanford-Binet intelligence scale: 5th edition; SF-36: Short Form 36 Health Survey Questionnaire; UDGT: uridine diphosphate glucuronosyl transferase; UGT1A1: uridine 5'-diphosphate glucuronosyltransferase; WHOQOL-BREF: World Health Organization Quality of Life Brief Version.

Fouad, Y., H. Ghazinyan, M. Alboraie, M. Alkhatry, H. Desalegn, F. Al-Ali, Mortada HF El-Shabrawi, Ola El-Sisi, P. Ocama, M. Derbala, S. Barakat, et al., "Joint position statement from the Middle East and North Africa and sub-Saharan Africa on continuing to endorse the MAFLD definition.", Journal of hepatology, vol. 80, issue 5, pp. e194-e197, 2024.
El-Koofy, N., E. Mahmoud, F. E. Mougy, E. Nasr, Sawsan Okasha, Mona Isa, H. El-Karaksy, G. Anwar, M. H. El-Shabrawi, N. E. Badawi, and N. Arafa, "Effect of medium chain triglycerides enriched formula on growth of biliary atresia patients after Kasai portoenterostomy.", Arab journal of gastroenterology : the official publication of the Pan-Arab Association of Gastroenterology, vol. 25, issue 2, pp. 188-193, 2024. Abstract

BACKGROUND AND STUDY AIMS: Biliary atresia (BA) is the most common cause of neonatal cholestasis, negatively affecting nutritional status, growth, and development. It is the most frequent paediatric indication for liver transplantation. The Kasai portoenterostomy (KPE) operation is an effective procedure with favourable outcomes when performed before two months of age. The present study aimed to assess the nutritional status of patients with biliary atresia who underwent the Kasai operation and to evaluate the effectiveness of nutritional counselling using medium-chain triglyceride (MCT) formulas and proper supplementation on their nutritional status, growth, and vitamin D levels.

PATIENTS AND METHODS: This prospective observational study included 36 infants with biliary atresia who underwent Kasai portoenterostomy. All patients underwent clinical assessment, anthropometric evaluation, nutritional counselling, and an evaluation of vitamin D levels. Only compliant patients (22/36) were followed up after 3 and 6 months of nutritional counselling.

RESULTS: Z-scores for weight, triceps skinfold thickness, and mid-upper arm circumference improved significantly after three months, and the height velocity Z-score improved after six months of nutritional counselling using an MCT-containing formula and supplementations. Patients who showed an improvement in cholestasis had better responses. The initial assessment revealed low serum levels of 25-hydroxyvitamin D in 77.8 %, which increased significantly (p = 0.012).

CONCLUSION: Dietary intervention and supplementation with MCT and micronutrients can improve the nutritional status of children with BA following KPE.

Zein, M. M., N. Arafa, Mortada HF El-Shabrawi, Ola El-Sisi, and N. M. El-Koofy, "Effect of nutrition-related infodemics and social media on maternal experience: A nationwide survey in a low/middle income country.", World journal of clinical pediatrics, vol. 13, issue 1, pp. 89139, 2024. Abstract

BACKGROUND: Undernutrition is a crucial cause of morbidity and mortality among children in low- or middle-income countries (LMICs). A better understanding of maternal general healthy nutrition knowledge, as well as misbeliefs, is highly essential, especially in such settings. In the current era of infodemics, it is very strenuous for mothers to select not only the right source for maternal nutrition information but the correct information as well.

AIM: To assess maternal healthy nutritional knowledge and nutrition-related misbeliefs and misinformation in an LMIC, and to determine the sources of such information and their assessment methods.

METHODS: This cross-sectional analytical observational study enrolled 5148 randomly selected Egyptian mothers who had one or more children less than 15 years old. The data were collected through online questionnaire forms: One was for the general nutrition knowledge assessment, and the other was for the nutritional myth score. Sources of information and ways of evaluating internet sources using the Currency, Relevance, Authority, Accuracy, and Purpose test were additionally analyzed.

RESULTS: The mean general nutrition knowledge score was 29 ± 9, with a percent score of 70.8% ± 12.1% (total score: 41). The median myth score was 9 (interquartile range: 6, 12; total score: 18). The primary sources of nutrition knowledge for the enrolled mothers were social media platforms (55%). Half of the mothers managed information for currency and authority, except for considering the author's contact information. More than 60% regularly checked information for accuracy and purpose. The mothers with significant nutrition knowledge checked periodically for the author's contact information ( = 0.012). The nutrition myth score was significantly lower among mothers who periodically checked the evidence of the information ( = 0.016). Mothers dependent on their healthcare providers as the primary source of their general nutritional knowledge were less likely to hold myths by 13% ( = 0.044). However, using social media increased the likelihood of having myths among mothers by approximately 1.2 ( = 0.001).

CONCLUSION: Social media platforms were found to be the primary source of maternal nutrition information in the current era of infodemics. However, healthcare providers were the only source for decreasing the incidence of maternal myths among the surveyed mothers.

Zhang, L., M. El-Shabrawi, L. A. Baur, C. D. Byrne, G. Targher, M. Kehar, G. Porta, W. S. Lee, S. Lefere, S. Turan, et al., "An international multidisciplinary consensus on pediatric metabolic dysfunction-associated fatty liver disease.", Med (New York, N.Y.), 2024. Abstract

BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) is highly prevalent in children and adolescents, particularly those with obesity. NAFLD is considered a hepatic manifestation of the metabolic syndrome due to its close associations with abdominal obesity, insulin resistance, and atherogenic dyslipidemia. Experts have proposed an alternative terminology, metabolic dysfunction-associated fatty liver disease (MAFLD), to better reflect its pathophysiology. This study aimed to develop consensus statements and recommendations for pediatric MAFLD through collaboration among international experts.

METHODS: A group of 65 experts from 35 countries and six continents, including pediatricians, hepatologists, and endocrinologists, participated in a consensus development process. The process encompassed various aspects of pediatric MAFLD, including epidemiology, mechanisms, screening, and management.

FINDINGS: In round 1, we received 65 surveys from 35 countries and analyzed these results, which informed us that 73.3% of respondents agreed with 20 draft statements while 23.8% agreed somewhat. The mean percentage of agreement or somewhat agreement increased to 80.85% and 15.75%, respectively, in round 2. The final statements covered a wide range of topics related to epidemiology, pathophysiology, and strategies for screening and managing pediatric MAFLD.

CONCLUSIONS: The consensus statements and recommendations developed by an international expert panel serve to optimize clinical outcomes and improve the quality of life for children and adolescents with MAFLD. These findings emphasize the need for standardized approaches in diagnosing and treating pediatric MAFLD.

FUNDING: This work was funded by the National Natural Science Foundation of China (82070588, 82370577), the National Key R&D Program of China (2023YFA1800801), National High Level Hospital Clinical Research Funding (2022-PUMCH-C-014), the Wuxi Taihu Talent Plan (DJTD202106), and the Medical Key Discipline Program of Wuxi Health Commission (ZDXK2021007).

Zhang, H., G. Targher, C. D. Byrne, S. U. Kim, V. W. - S. Wong, L. Valenti, M. Glickman, J. Ponce, C. S. Mantzoros, J. Crespo, et al., "A global survey on the use of the international classification of diseases codes for metabolic dysfunction-associated fatty liver disease.", Hepatology international, 2024. Abstract

BACKGROUND: With the implementation of the 11th edition of the International Classification of Diseases (ICD-11) and the publication of the metabolic dysfunction-associated fatty liver disease (MAFLD) nomenclature in 2020, it is important to establish consensus for the coding of MAFLD in ICD-11. This will inform subsequent revisions of ICD-11.

METHODS: Using the Qualtrics XM and WJX platforms, questionnaires were sent online to MAFLD-ICD-11 coding collaborators, authors of papers, and relevant association members.

RESULTS: A total of 890 international experts in various fields from 61 countries responded to the survey. We also achieved full coverage of provincial-level administrative regions in China. 77.1% of respondents agreed that MAFLD should be represented in ICD-11 by updating NAFLD, with no significant regional differences (77.3% in Asia and 76.6% in non-Asia, p = 0.819). Over 80% of respondents agreed or somewhat agreed with the need to assign specific codes for progressive stages of MAFLD (i.e. steatohepatitis) (92.2%), MAFLD combined with comorbidities (84.1%), or MAFLD subtypes (i.e., lean, overweight/obese, and diabetic) (86.1%).

CONCLUSIONS: This global survey by a collaborative panel of clinical, coding, health management and policy experts, indicates agreement that MAFLD should be coded in ICD-11. The data serves as a foundation for corresponding adjustments in the ICD-11 revision.

Mortada HF El-Shabrawi, Ola El-Sisi, A. Elamin, and N. M. Kamal, "Letter to the Editor: MAFLD versus MASLD criteria debate- Certainly not for children!", Hepatology (Baltimore, Md.), 2024.
El-Shabrawi, M. H. F., N. M. Kamal, H. R. El-Khayat, E. M. Kamal, M. M. A. H. AbdElgawad, and M. Yakoot, "A pilot single arm observational study of sofosbuvir/ledipasvir (200 + 45 mg) in 6- to 12- year old children.", Alimentary pharmacology & therapeutics, vol. 47, issue 12, pp. 1699-1704, 2018. Abstract

BACKGROUND: No available data on the use of sofosbuvir/ledipasvir combination in treatment of hepatitis C virus (HCV) infection in children 6- to 12- year old.

AIM: To assess the safety and efficacy of sofosbuvir plus ledipasvir in children 6- to 12- year old with chronic HCV genotype 4 infection.

METHODS: This is a pilot prospective single arm observational open-label multicentre study. A total of 20 consecutive eligible chronic HCV infected children, aged from 6- to 12- years were included in this study and treated with a fixed sofosbuvir/ledipasvir combination in half the adult dose (200/45 mg) once daily for 12 weeks. Laboratory tests including virological markers were measured at baseline, 2, 4, 8 and 12 weeks (end of treatment [EOT]), and 12 weeks after end of treatment for sustained virological response 12 (SVR12).

RESULTS: The intention-to-treat (ITT) SVR12 rate was 19/20 (95%; 95% CI: 76.4%-99.1%). SVR12 was not assessed in one patient who was lost to follow-up after showing viral negativity at the EOT12. All the remaining 19 patients (100%, 95% CI: 83.18%-100%) who completed the full protocol and follow-up visits achieved SVR12 with normal liver, haematological, and renal function tests and no side effects or fatalities.

CONCLUSIONS: This pilot study demonstrated that the fixed dose sofosbuvir/ledipasvir combination could be safe and effective treatment in children 6- to 12- years with chronic hepatitis C genotype 4 infection. Our pilot results might encourage larger and multicentre studies in this age group.

Kamal, N. M., O. Saadah, H. Alghamdi, A. Algarni, Mortada HF El-Shabrawi, Ola El-Sisi, L. M. Sherief, and S. A. Abosabie, "Case Report: Dubin-Johnson Syndrome Presenting With Infantile Cholestasis: An Overlooked Diagnosis in an Extended Family.", Frontiers in pediatrics, vol. 10, pp. 855210, 2022. Abstract

Dubin-Johnson syndrome (DJS) is an often-missed diagnosis of neonatal cholestasis. We report two patients with DJS, who presented with neonatal cholestasis. The first patient underwent extensive investigations for infantile cholestasis with no definitive etiology reached; the diagnosis of DJS was missed until the age of 14 years old. The diagnosis was confirmed genetically with c.2273G > T, p.G758V mutation in exon 18 of the ABCC2 gene. The 2nd patient is a 7-day-old baby, the son of the 1st patient who gave birth to him at the age of 21 years old. He was diagnosed with DJS at the age of 2 weeks based on normal clinical and laboratory workup apart from direct hyperbilirubinemia. He had the same mutation as his mother in homozygous status. The husband was heterozygous for the same mutation. DJS is one of the often-missed differential diagnoses of neonatal cholestasis. It should be suspected in patients of infantile cholestasis, who have an, otherwise, normal physical examination, and laboratory investigations to avoid unnecessary lengthy, invasive, and expensive workups.