Epidemiology of Phenylketonuria Disease in Jordan: Medical and Nutritional Challenges.

BACKGROUND: Phenylketonuria (PKU) is the most frequent inborn error in amino acid metabolism caused by a deficiency of the phenylalanine hydroxylase enzyme (PAH). If PKU is left untreated, high concentrations of phenylalanine (Phe) accumulate in the blood, leading to severe brain dysfunction, neurodevelopmental, behavioral and psychological problems. Data concerning the epidemiology of PKU in Jordan are limited. The main objectives of our study were to determine the prevalence of PKU in Jordan, analyze the PKU phenotypes, and identify major challenges in providing dietary management to PKU patients. METHODS: Data were collected utilizing the medical records of PKU patients attending the PKU clinic at the Ministry of Health in Amman, Jordan, between 2008 and 2021. RESULTS: The total number of patients diagnosed with PKU was 294. The prevalence of PKU was estimated to be 1/5263. Most patients were Jordanians (90.8%), and 9.2% were non-Jordanians. More than half of the patients (56%) were diagnosed through the national newborn screening (NBS) program. Regarding the phenotypes of PKU, 46.6% had moderate PKU, whereas 42.9% had the classic type of PKU and only 8 (2.7%) had cofactor Tetrahydrobiopterin (BH4) deficiency (atypical PKU). According to the age of diagnosis, 66% of patients were diagnosed more than 30 days post-birth. Consanguinity was found in 87.4% of patients, and the majority of patients, 218 (74.2%), had first-degree consanguinity. The most common complication was mental retardation (31%). Most patients were committed to dietary management (83%) and developed fewer complications. CONCLUSION: In our study, we demonstrated the importance of the NBS program in the early identification and diagnosis of new PKU cases which allows the initiation of treatment and dietary management to prevent severe complications of PKU in Jordan.

The capacity of primary health care centers in Jordan to manage hypertension: areas for improvement.

The assessment of the capacity of Primary Health Care (PHC) settings for the management of hypertension is essential to identify areas for improving management outcomes. This study aimed to assess the capacity of PHC centers in Jordan to manage hypertension including the assessment of human resources, equipment, and infrastructure. All comprehensive PHC centers located in Irbid and Mafraq governorates in north of Jordan (n = 23) were assessed. An assessment tool from HEARTS Technical Package was adapted and used for assessment. All centers have general practitioners and half of the centers (n = 11, 47.8%) had at least one family doctor working full time. In only one center, all doctors, nurses, and other health workers were trained on the management of hypertension. All centers, except one, had at least one functional automatic blood pressure measuring devices (BPMDs). Almost two thirds of centers (43.5%) had no measuring tapes. ECG machines were present in all centers except two. One third (n = 8, 34.8%) of centers had no functional glucometers. The majority of health centres carry out the laboratory investigations. Educational materials on physical activity, hypertension, and diabetes were seen in four (17.4%) centers only. Necessary medications were always available in the majority of health centers. In conclusion, this assessment revealed many areas for improvement in human resources, equipment, infrastructures, and other resources, such as developing an updated guideline/protocol of hypertension management, training the PHC staff on these guidelines, providing PHC centers with the necessary equipment, and establishing e-registry to improve documentation of data.

Meta-Analysis and Systematic Review of Micro- and Macro-Nutrient Intakes and Trajectories of Macro-Nutrient Supply in the Eastern Mediterranean Region.

The Eastern Mediterranean Region (EMR) is experiencing a nutrition transition, characterized by the emergence of overnutrition and micro-nutrient deficiencies. No previous study has comparatively examined nutrient intake in adults across countries in the EMR. This review examined the adequacy of nutrients in adults living in the EMR. Moreover, it analyzed the food balance sheets (FBS) for 1961-2018 to identify the trajectory of energy supply from macro-nutrients in the EMR. A systematic search was conducted from January 2012 to September 2020. Only observational studies were retained with a random sampling design. An assessment of the methodological quality was conducted. Levels of nutrient daily intake and their adequacy compared to the daily reference intake of the Institute of Medicine were reported across the region. No studies were identified for half of the region's countries. Although nutrient energy intake was satisfactory overall, fat and carbohydrate intake were high. Intake of vitamin D, calcium, potassium, zinc, and magnesium were below that recommended. The analysis of the FBS data allowed for the identification of four linear patterns of trajectories, with countries in the EMR best fitting the 'high-energy-supply from carbohydrate' group. This systematic review warrants multi-sectorial commitment to optimize nutrient intake.

Trend of Iodine Status in the Eastern Mediterranean Region and Impact of the Universal Salt Iodization Programs: a Narrative Review.

Recognizing the importance of iodine deficiency as a single most important preventable cause of brain damage, World Health Organization (WHO) urged all countries to scale up salt iodization since 1991 where iodine deficiency was declared as a global public health problem. All countries committed to report on the national iodine status of school-aged children (6-12 years), every 3 years. This paper aims to evaluate the progress of countries in Eastern Mediterranean Region (EMR) toward the eradication of iodine deficiency disorders (IDD) and recommend operational action to support implementation of the WHO Regional Nutrition Strategy (2020-2030). Salt iodization in EMR has been scaled up with significant progress in increasing the proportion of dietary salt which is adequately iodized. As a result, many countries such as Qatar, Kuwait, Bahrain, Jordan, Oman, and Iran have achieved, or are now on the threshold of achieving IDD elimination. The WHO in coordination with United Nations Children's Fund is working with member states to ensure suitability of these achievements. Hence, efficient monitoring will help member states to measure the magnitude of IDD as a public health problem and to monitor the effects of the intervention on the iodine status of a population. Policy makers should take proactive steps to enforce mandatory regulatory measures for USI, through a multi-sectoral coordination, and careful assessments and estimates of iodine nutritional status. Coordination between the two national programs of USI and salt reduction intake is necessary.

Fat Intake Reduction Strategies among Children and Adults to Eliminate Obesity and Non-Communicable Diseases in the Eastern Mediterranean Region.

Non-communicable diseases (NCDs) are the leading cause of mortality globally with an estimated 39.5 million deaths per year (72% of total death) in 2016, due to the four major NCDs: cardiovascular diseases, cancers, chronic respiratory diseases and diabetes. In the Eastern Mediterranean Region (EMR), almost 68% of all deaths are attributed to NCDs commonly known as chronic or lifestyle-related diseases. Two-thirds of NCD premature deaths are linked to 4 shared modifiable behavioral risk factors: tobacco use, unhealthy diet, physical inactivity and harmful use of alcohol. These unhealthy behaviours lead to 4 key metabolic/biological changes; raised blood pressure, overweight/obesity, high blood glucose levels/diabetes, and hyperlipidemia (high levels of fat in the blood), that increase the risk of NCDs. Globally, countries are already working towards agreed global goals on maternal and infant nutrition and on the prevention of NCDs. In both fields the goals include halting the increase in overweight and obesity and reducing NCD diet-related risk factors including reducing saturated fatty acids (SFAs) and trans fatty acids (TFAs) intake. The objective of this review is to present an up-to-date overview of the current fat (SFAs and TFAs) intake reduction initiatives in countries of the Eastern Mediterranean Region (EMR) by highlighting national and regional programs, strategies and activities aiming at decreasing the intakes of dietary fat (SFA and TFA). The literature review shows that the average intake of SFA is estimated to be 10.3% of the total energy intake (EI), exceeding the WHO (World Health Organization) upper limit of 10%. The average TFA intake is estimated at 1.9% EI, which also exceeds the WHO upper limit of 1% EI. The highest SFAs intake was reported from Djibouti, Kuwait, Saudi-Arabia, Lebanon and Yemen, while the highest TFAs intakes were reported from Egypt and Pakistan. If countries of the EMR receive immediate public health attention, that toll of NCD-related morbidity and mortality would be considerably decreased through the implantation of evidence-based preventive interventions. In this context, reductions in SFAs and TFAs intakes have been highlighted as cost-effectives strategies that may hamper the growth of the NCD epidemic.