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.

Alnaghy, E., S. Taman, E. Abdelhalim, A. Abdel Razek, M. El-Shabrawi, M. Ezz El Regal, A. Megahed, S. Elzeny, and N. El Tantawi, "The added value of brain MR spectroscopy in children with Crigler-Najjar syndrome type-I: correlation with demographic, neurodevelopmental, and laboratory findings.", The British journal of radiology, vol. 96, issue 1144, pp. 20220433, 2023. Abstract

OBJECTIVE: The aim of this study is to demonstrate the role of proton magnetic resonance spectroscopy (1H-MRS) in the detection of brain microstructural changes in patients with Crigler-Najjar syndrome type-I (CNs-I), and its correlation with demographic, neurodevelopmental and laboratory findings.

METHODS: Prospective study was conducted on 25 children with CNs-I and 25 age and sex-matched children, who served as control. They underwent multivoxel 1H-MRS of basal ganglion at echo time 135-144 ms. N-acetyl aspartate/Creatine (NAA/Cr) and Choline (Ch)/Cr were calculated and correlated with demographic, clinical, and laboratory findings of patients with CNs-I.

RESULTS: There was a significant difference in NAA/Cr and Ch/Cr between patients and controls. The cut-off value for NAA/Cr and Ch/Cr used to differentiate patients from controls were 1.8 and 1.2 with an area under the curve (AUC) of 0.91 and 0.84 respectively. There was a significant difference in MRS ratios between patients with neurodevelopmental delay (NDD) and patients without NDD. The cut-off values for NAA/Cr and Ch/Cr used to differentiate between patients with NDD and patients without NDD were 1.47 and 0.99, with AUC of 0.87 and 0.8 respectively. The NAA/Cr and Ch/Cr were well correlated with family history ( = 0.006 and < 0.001) respectively, consanguinity ( < 0.001 and = 0.001), neurodevelopmental delay ( = 0.001 and = 0.004), serum bilirubin level ( = -0.77, < 0.001), ( = -0.49, = 0.014), phototherapy ( < 0.001 and = 0.32), blood transfusion ( < 0.001 and = 0.001) respectively.

CONCLUSION: 1H-MRS can be a useful tool in the detection of neurological changes in patients with CNs-I; NAA/Cr and Ch/Cr parameters are well correlated with demographic, clinical, and laboratory findings.

ADVANCES IN KNOWLEDGE: Our study is the first report on using MRS in assessing neurological manifestations in CNs. 1H-MRS can be a useful tool in the detection of neurological changes in patients with CNs-I.

Memon, I. A., A. Parkash, K. Sadiq, N. M. Kamal, and Mortada HF El-Shabrawi, Ola El-Sisi, "The Asia Pacific Pediatric Association (APPA) position statement on the MAFLD definition of fatty liver disease.", Therapeutic advances in chronic disease, vol. 14, pp. 20406223231164523, 2023.
Sayed, S., Mortada HF El-Shabrawi, Ola El-Sisi, E. Abdelmonaem, N. E. Koofy, and S. Tarek, "Value of Nutritional Screening Tools Versus Anthropometric Measurements in Evaluating Nutritional Status of Children in a Low/Middle-Income Country.", Pediatric gastroenterology, hepatology & nutrition, vol. 26, issue 4, pp. 213-223, 2023. Abstract

PURPOSE: Pediatric patients in low-income countries are at a high risk of malnutrition. Numerous screening tools have been developed to detect the risk of malnutrition, including the Subjective Global Nutritional Assessment (SGNA), Pediatric Yorkhill Malnutrition Score (PYMS), Screening Tool for the Assessment of Malnutrition in Pediatrics (STAMP), and Screening Tool for Risk of Nutritional Status and Growth (STRONGkids). However, anthropometry remains the main tool for assessing malnutrition. We aimed to identify the value of four nutritional screening tools versus anthropometry for evaluating the nutritional status of children.

METHODS: We conducted a cross-sectional study of 1,000 children aged 1-12 years who visited the outpatient clinic of Cairo University Pediatric Hospital. Each participant was evaluated using anthropometric measurements (weight, length/height, and weight for length/height) as well as the PYMS, STAMP, STRONGkids, and SGNA screening tools. The sensitivities and specificities of these four tools were assessed using anthropometry as the gold standard.

RESULTS: Of the patients, 1.7% were underweight, 10.2% were wasted, and 35% were stunted. STRONGkids demonstrated the highest sensitivity (79.4%) and a high specificity (80.2%) for detecting malnutrition compared with weight for height, followed by STAMP, which demonstrated lower sensitivity (73.5%) but higher specificity (81.4%). PYMS demonstrated the lowest sensitivity (66.7%) and the highest specificity (93.5%), whereas SAGA demonstrated higher sensitivity (77.5%) and lower specificity (85.4%) than PYMS.

CONCLUSION: The use of nutritional screening tools to evaluate the nutritional status of children is valuable and recommended as a simple and rapid method for identifying the risk of malnutrition in pediatric patients.

Pana, Z. D., M. El-Shabrawi, M. A. Sultan, T. Murray, A. Alam, V. Yewale, D. Dharmapalan, J. D. Klein, J. Haddad, N. Thacker, et al., "Fighting the hidden pandemic of antimicrobial resistance in paediatrics: recommendations from the International Pediatric Association.", BMJ paediatrics open, vol. 7, issue 1, 2023.
El-Shabrawi, M., I. Memon, D. Attia, and N. M. El-Koofy, "The International Society of Tropical Paediatrics (ISTP) endorses the redefinition of fatty liver disease.", Journal of hepatology, vol. 76, issue 3, pp. 738-739, 2022.
El-Hodhod, M. A., Mortada HF El-Shabrawi, Ola El-Sisi, A. AlBadi, A. Hussein, A. Almehaidib, B. Nasrallah, E. M. AlBassam, H. El Feghali, H. M. Isa, K. Al Saraf, et al., "Consensus statement on the epidemiology, diagnosis, prevention, and management of cow's milk protein allergy in the Middle East: a modified Delphi-based study.", World journal of pediatrics : WJP, vol. 17, issue 6, pp. 576-589, 2021. Abstract

BACKGROUND: This study aimed to develop an expert consensus regarding the epidemiology, diagnosis, and management of cow's milk protein allergy (CMPA) in the Middle East.

METHODS: A three-step modified Delphi method was utilized to develop the consensus. Fifteen specialized pediatricians participated in the development of this consensus. Each statement was considered a consensus if it achieved an agreement level of ≥ 80%.

RESULTS: The experts agreed that the double-blind placebo-controlled oral challenge test (OCT) should be performed for 2-4 weeks using an amino acid formula (AAF) in formula-fed infants or children with suspected CMPA. Formula-fed infants with confirmed CMPA should be offered a therapeutic formula. The panel stated that an extensively hydrolyzed formula (eHF) is indicated in the absence of red flag signs. At the same time, the AAF is offered for infants with red flag signs, such as severe anaphylactic reactions. The panel agreed that infants on an eHF with resolved symptoms within 2-4 weeks should continue the eHF with particular attention to the growth and nutritional status. On the other hand, an AAF should be considered for infants with persistent symptoms; the AAF should be continued if the symptoms resolve within 2-4 weeks, with particular attention to the growth and nutritional status. In cases with no symptomatic improvements after the introduction of an AAF, other measures should be followed. The panel developed a management algorithm, which achieved an agreement level of 90.9%.

CONCLUSION: This consensus document combined the best available evidence and clinical experience to optimize the management of CMPA in the Middle East.

Razek, A. A. K. A., M. Ezz El Regal, M. El-Shabrawi, M. M. Abdeltawwab, A. Megahed, S. Elzeny, N. El Tantawi, and S. E. Taman, "Diffusion Tensor Imaging of Auditory Pathway in Patients With Crigler-Najjar Syndrome Type I: Correlation With Auditory Brainstem Response.", Journal of child neurology, vol. 37, issue 2, pp. 119-126, 2022. Abstract

AIM: To evaluate the role of diffusion tensor imaging of the auditory pathway in patients with Crigler Najjar syndrome type I and its relation to auditory brainstem response.

METHODS: Prospective study was done including 12 patients with Crigler Najjar syndrome type I and 10 age- and sex-matched controls that underwent diffusion tensor imaging of brain. Mean diffusivity and fractional anisotropy at 4 regions of the brain and brainstem on each side were measured and correlated with the results of auditory brainstem response for patients.

RESULTS: There was significantly higher mean diffusivity of cochlear nucleus, superior olivary nucleus, inferior colliculus, and auditory cortex of patients versus controls on both sides for all regions ( = .001). The fractional anisotropy of cochlear nucleus, superior olivary nucleus, inferior colliculus, and auditory cortex of patients versus controls was significantly lower, with values of, respectively, .001, .001, .003, and .001 on the right side and .001, .001, .003, and .001 on left side, respectively. Also, a negative correlation was found between the maximum bilirubin level and fractional anisotropy of the left superior olivary nucleus and inferior colliculus of both sides. A positive correlation was found between the mean diffusivity and auditory brainstem response wave latency of the right inferior colliculus and left cochlear nucleus. The fractional anisotropy and auditory brainstem response wave latency of the right superior olivary nucleus, left cochlear nucleus, and inferior colliculus of both sides were negatively correlated.

CONCLUSION: Diffusion tensor imaging can detect microstructural changes in the auditory pathway in Crigler Najjar syndrome type I that can be correlated with auditory brainstem response.

Méndez-Sánchez, N., E. Bugianesi, R. G. Gish, F. Lammert, H. Tilg, M. H. Nguyen, S. K. Sarin, N. Fabrellas, S. Zelber-Sagi, J. - G. Fan, et al., "Global multi-stakeholder endorsement of the MAFLD definition.", The lancet. Gastroenterology & hepatology, vol. 7, issue 5, pp. 388-390, 2022.
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