Aboul Ella Hassanien (Abo)

Climate change is currently affecting most of the cities across the globe in the past years with its climate severe events manifested in the year 2019. This objective of this paper is to focus on the strategic renewable energy and climate action plans of two seaside cities in Egypt to strengthen clean energy capacity and implementation based on each city’s strategy in 2030. Diagnostic studies were carried out to identify the gap in each city in terms of climate change and renewable energy. Prior to providing a detailed account of the concrete measures undertaken to reduce greenhouse gas (GHG) emissions and promote the development of sustainable energy, the national and regional strategies on climate change adaptation and renewable energy capacity and future needs were assessed to draw the gap at the local government level. Climate change risks by sectors in terms of vulnerability have been assessed and presented. The adaptation scoreboard of the Adaptation Cycle-Specific Steps (ACSS) for each city, based on the European Commission Joint Research Centre (EC-JRC) guidelines, including six steps has also been utilised to assess the climate actions in the city. In addition, risk assessment and vulnerability analysis were conducted using a set of parameters and interviews with local government officials. In order to conduct the risk assessment and vulnerability analysis on the city, as a first step, the climate hazard types were identified. Both Hurghada and Luxor were selected to assess the impact of each climate hazard type, where a series of vulnerable and impacted sectors, such as population (public health), infrastructure (transport, energy, water, and social), and the built environment (building stock and materials), as well as economy (tourist and agriculture) and biodiversity (coastal zone ecosystems and green zones) were diagnosed and assessed. The vulnerability analysis, which is based on the Future Cities Adaptation Compass Tool, Governors’ (Mayors) Adapt, and the European Climate Adaptation Platform, is carried out and presented. The results of the adopted climate adaptation actions in the city of Hurghada and the city of Luxor, including impacted sectors, are presented and discussed. Finally, the renewable energy action of upgrading the solar photovoltaic (PV) capacity in both the cities from 13 MW to 35 MW is also assessed and highlighted.

Background: Inborn errors of metabolism (IEMs) commonly present with pediatric cardiomyopathy. Identification of the underlying cause is necessary as it may lead to improved outcomes.

Objectives: We aimed to investigate the diagnostic rate, the clinical, and biochemical spectra of IEMs among Egyptian pediatric patients presenting with cardiomyopathy, and their outcome measures.

Methods: We retrospectively analyzed the clinical, biochemical, and radiological data of 1512 children diagnosed with cardiomyopathy at Cairo University Children's Hospital over a 5-year duration.

Results: Two hundred twenty-nine children were clinically suspected as IEMs and underwent metabolic workup. Nineteen different IEMs were confirmed in 57 (24.4%) of the suspected children. Their median age at presentation was 2.6 years and the majority had extra-cardiac manifestations. Hypertrophic cardiomyopathy represented 43/57 (75.4%) of confirmed cases, while dilated cardiomyopathy represented 13/57 (22.8%), and one patient presented with a mixed phenotype. Twenty- six patients (45.6%) survived, while 31 patients (54%) either died or were lost to follow up and assumed deceased.

Conclusions: We developed for the first time a database and a diagnostic scheme for metabolic cardiomyopathies in Egyptian children. With the recent introduction of enzyme replacement therapy, many metabolic disorders became treatable, thus establishing an early and accurate diagnosis is extremely important.

BACKGROUND: Inborn errors of metabolism (IEMs) commonly present with pediatric cardiomyopathy. Identification of the underlying cause is necessary as it may lead to improved outcomes.

OBJECTIVES: We aimed to investigate the diagnostic rate, the clinical, and biochemical spectra of IEMs among Egyptian pediatric patients presenting with cardiomyopathy, and their outcome measures.

METHODS: We retrospectively analyzed the clinical, biochemical, and radiological data of 1512 children diagnosed with cardiomyopathy at Cairo University Children's Hospital over a 5-year duration.

RESULTS: Two hundred twenty-nine children were clinically suspected as IEMs and underwent metabolic workup. Nineteen different IEMs were confirmed in 57 (24.4%) of the suspected children. Their median age at presentation was 2.6 years and the majority had extra-cardiac manifestations. Hypertrophic cardiomyopathy represented 43/57 (75.4%) of confirmed cases, while dilated cardiomyopathy represented 13/57 (22.8%), and one patient presented with a mixed phenotype. Twenty- six patients (45.6%) survived, while 31 patients (54%) either died or were lost to follow up and assumed deceased.

CONCLUSIONS: We developed for the first time a database and a diagnostic scheme for metabolic cardiomyopathies in Egyptian children. With the recent introduction of enzyme replacement therapy, many metabolic disorders became treatable, thus establishing an early and accurate diagnosis is extremely important.

Primary hyperoxaluria (PH) is an autosomal recessive disorder of oxalate metabolism caused by pathogenic variants in either of three genes (AGXT, GRHPR or HOGA1). The study aimed at characterizing the clinical phenotypes as well as the genotypic spectrum of PH in Egypt. We screened 25 Egyptian patients suspected of PH for the three responsible genes by Sanger sequencing. We diagnosed 20 patients from 18 unrelated families, in which the natural history, family history, clinical features and genotypes were evaluated. PH patients were 15 males and 5 females ranging in age from 4 months to 31 years (median 8 years). Fifteen families were consanguineous (83%) and familial clustering was reported in six families (33%). Pathogenic variants in all 40 alleles were in AGXT, with none detected in GRHPR or HOGA1. We detected two novel pathogenic variants c.166-1_172dupGATCATGG (p.Asp58Glyfs*65) and c.766delC (p.Gln256fs*16) and seven previously reported variants in our cohort. This is the first study reporting the genotype of a considerable number of PH1 patients from Egypt. Our detected variants in the AGXT gene could form the basis for future genetic counseling and prenatal diagnosis in Egypt and surrounding populations.

This study analyzes the general disease characteristics, impact of enzyme replacement therapy (ERT), and overall survival (OS) of 156 Egyptian patients with Gaucher disease (GD) enrolled on hormone replacement from 1998 to 2017. The mean age at diagnosis was 32.46±12.68 months. Anemia was noted at diagnosis in 50%, thrombocytopenia in 30.7%, severe splenomegaly in 58.7%, severe hepatomegaly in 11.9%, and skeletal findings were detected in 24.3% of the patients. The most prevalent GD type was type 3 (54.5%). Twenty-two of type 3 patients had no neurological manifestations at diagnosis, and 12 developed variable central nervous system manifestations during follow-up. The most common neurological features were limited eye movements, oculomotor apraxia, and squint. Of the 60 patients for whom genotypes were obtained, homozygous L444P was the most common (n=35/60, 58.3%). Treatment with ERT (imiglucerase) revealed significant improvements in blood indices, organ volumes, and growth parameters (P<0.05). Ten (11.7%) type 3 patients did not develop any neurological manifestations under ERT over 20 years. Mortality was 16%, and the 20-year OS was 73.3%. We conclude that in Egypt, type 3 is the most prevalent phenotype of GD, and homozygous L444P is the predominant GBA genotype of GD. Early age at diagnosis and treatment with ERT over 20 years revealed significant improvements in disease manifestations, with an OS of 73.3%.

Aim: The aim is to compare the volumetric changes between pouch technique versus the modified pouch technique in pontic site development using connective tissue graft in patients that have Seibert class I ridge defects in the maxillary esthetic zone. . This randomized, controlled, double-blinded, parallel-grouped clinical trial included sixteen patients with a single pontic site in the maxillary esthetic area presenting Seibert Class I ridge defects. Patients were randomly assigned into two equal groups: test group (n: 8) received soft tissue augmentation with connective tissue graft using the modified pouch technique and control group (n: 8) received soft tissue augmentation with connective tissue graft using pouch technique. The volumetric evaluation was carried out by taking impressions at baseline, 3 and 6 months after the surgery. Keratinized tissue thickness was also measured at baseline, 3 and 6 months after the surgery. Visual analogue scale (VAS) was recorded by the patients at day 3, day 7, and day 14 after the surgery.

Results: The test group had more increase in soft tissue volume than the control group at baseline, 3 months, and 6 months. The keratinized tissue width at baseline in the test group had a higher value than that of the control group. At 3 months, both groups had the same mean value, while at 6 months, the test group had a higher value than the control group. Regarding postoperative pain, the visual analogue scale shown at day 3 in the test group had a higher value than that of the control group, while at day 7, the control group had a higher value than the test group. At day 14, both groups had the same mean value.

Conclusions: Soft tissue augmentation using both the traditional pouch technique and the modified pouch technique led to successful soft tissue volume augmentation in pontic site development in Seibert Class I ridge defects with no statistically significant difference between the two techniques.

Background: Although visual inspection is the preferred route in everyday clinical practise for detecting early caries lesions, novel technologies like light fluorescence-based devices (Vista Proof iX HD smart) have been developed to enhance early caries detection. Material and Methods: Occlusal surface of 45 molar and 49 premolar teeth from 34 adult participants who fulfilled the eligibility criteria were examined by two observers using three diagnostic methods. Examination was performed visually using the International Caries Detection and Assessment System (ICDAS-II) followed by Vista Proof. Fissurotomy was applied for histological validation. Intra- and inter-observer agreement were measured for ICDAS-II and light-induced fluorescence camera using Kappa test. The overall diagnostic accuracy parameters, area under the receiver operating characteristic curve (AUC-ROC) and 95% confidence interval (95% CI) of the (AUC) for caries detection by Vista Poof were evaluated. Results: For ICDAS-II and Vista Proof methods, there was almost perfect intra- and inter-observer agreement. Based on ICDAS-II as a reference standard 1, Vista Proof showed a low level of agreement in enamel carious lesion detection with low sensitivity value of 48%, high specificity of 100%, and AUC was 0.112, while a high level of agreement in dentin carious lesion detection with high sensitivity value of 100%, low specificity of 48% and AUC was 0.888. Based on fissurotomy as reference standard 2, Vista Proof showed a high level of agreement in dentin carious lesion detection with high sensitivity value of 95% and 0% specificity and AUC was 0.814. Conclusions: Quantitative light-induced fluorescence camera with reference to ICDAS-II is considered as an accurate diagnostic modality for detection of early occlusal caries. Histological findings validate the diagnostic accuracy of the camera in dentin.