- Volume:9 Issue: 2, Apr 2021
- تاریخ انتشار: 1400/03/23
- تعداد عناوین: 11
Phenylketonuria (PKU) is the most frequent inborn error of metabolism, in which newborns cannot metabolize phenylalanine to tyrosine. Increased phenylalanine in untreated patients with PKU can cause serious intellectual disability; its onerous financial burden also falls on societies. This review study aimed to systematically indicate the frequency of PKU worldwide. We also intended to highlight the global prevalence of PKU, which might shed light on better clinical management and screening programs.Methods
In this systematic review, two electronic databases, including PubMed and ScienceDirect were searched for the related literature using relevant keywords: “Phenylketonuria” or “PKU” and “Prevalence” or “Incidence” and “Iran” or “Middle East” or “Europe” or “America” or “Asia.” Accordingly, 4306 reports conducted on PKU from January 2007 to December 2018 were retrieved. With the removal of 44 duplicated publications, 44 reports were included in the current systematic review. Prevalence and incidence rates were categorized based on different continents in which nations used various NBS programs to report the incidence and prevalence of PKU. Non-English, non-eligible, duplicated, animal, and in vitro studies are excluded.Results
Based on the reported quantitative data, the prevalence of PKU diagnosed worldwide ranged from 0.00044% to 0.02736% in which Italy possessed the highest prevalence; however, Thailand manifested the lowest prevalence rate. However, for some countries, such as India or Finland, either the related data to the frequency of PKU was outdated or overlooked applying any newborn screening programs respecting PKU.Conclusions
The current study revealed an elevated prevalence of PKU in Iran, compared with other Asian countries; thus, it demands a more serious management program. Moreover, the high prevalence of PKU in European countries should not be underestimated.Keywords: Phenylketonuria, Prevalence, Newborn screening, Inborn Error Metabolism
Rheumatic Heart Disease (RHD) remains the prominent cause of morbidity and mortality among young individuals in developing countries. The present research aimed to estimate the prevalence of RHD in Iran.
In this review, all population-based studies with a sample size of ≥500 participants related to the prevalence of RHD were included. The search was conducted in national and international databases. The combination of medical subject headings and free-term text items concerning the incidence, prevalence, rheumatic fever, RHD, and Iran were used in this study.
In this systematic review, 490 papers were identified. A total of 296 articles were screened; of which, 9 qualified articles were selected for the final data analysis. All studies were of cross-sectional, and descriptive natures. Two authors used the data extraction form to independently extract relevant data from the included studies. Disagreements between the authors were reconciled through discussion and consensus.Results
The overall prevalence of RHD was calculated as 0.02% (20/100000 population). This prevalence rate is estimated based on studies conducted in the northern, southern, eastern, western, and central regions of the country; therefore it could be an estimation of the relevant prevalence rate in Iran. However, there were no updated and high-quality data in this regard. Thus, the reported rate might be underestimated.Conclusions
There is a need for a nationwide survey to investigate the precise prevalence of RHD in Iran. This can be achieved by creating a national database of RHD.Keywords: Prevalence, Rheumatic heart disease, Iran
Infantile Colic (IC) is an essential problem in infancy that is influenced by factors related to infants and parents. The parental factors associated with colic have not been comprehensively assessed.Objectives
The present systematic review was conducted to investigate the parental factors affecting the incidence of IC.
Data Sources: Databases, including PubMed, Web of Science, Scopus, Science Direct, Google scholar, as well as Scientific Information Database (SID), and Magiran (Iranian websites) were searched to identify all eligible papers concerning parental factors affecting infantile CI. The registration number of this study was CRD42020163518 in the PROSPERO database.
A total of 423 relevant articles published up to the end of December 2019 were assessed. The selected articles were screened based on duplicated, eligibility criteria, and quality appraisal. The main inclusion criteria were observational studies and articles in Persian and English languages.
The Mesh keywords and Boolean operators included (“risk factors” OR “causality”) AND (“parents” OR “fathers” OR “mothers”) AND (“infant”) AND (“colic”). Consequently, 18 papers were thoroughly studied and the related data were extracted. Two researchers independently performed the data extraction and quality assessment based on the STROBE checklist from the observational studies. The information of selected studies was recorded in a table, i.e. consisted of authors’ names, purpose, design, population, and main results.Results
Final articles consisted of 10 prospective, 6 cross-sectional, and 2 case-control studies. Eventually, the effective factors were placed in 6 categories, as follows: psychological factors, physical factors, taking medications, perinatal factors, family’s socioeconomic status, and maternal diet. The most important characteristics predisposing to IC were parental depression, anxiety, smoking, maternal history of migraine, young age, primiparity, low family support, high socioeconomic status, high-risk pregnancies, and delivery, taking antibiotics, as well as the consumption of celery, onions, and bananas.Conclusions
Various parental factors affect the incidence of colic. Identifying these risk factors and accurate planning can be beneficial in the prevention and treatment of IC.Keywords: Risk factors, Infant, Causality, Colic, Parent
Malnutrition is associated with a longer duration of mechanical ventilation and an increased risk of healthcare-acquired infections in critically ill children who are mechanically ventilated.Objectives
Therefore, nutritional therapy plays a critical role in the initiation and duration of mechanical ventilation and clinical outcomes in such patients.
Data Sources: This review was conducted by searching the Web of Science, Scopus, Embase, and Medline databases. A combination of related mesh terms and keywords was used to find the relevant articles. Finally, we screened search results through titles and abstracts and related articles were enrolled in the review process. We tried to address all aspects of nutritional management of mechanically ventilated critically ill children.Results
Energy demand in mechanically ventilated children is a controversial issue and Indirect Calorimetry (IC) is the recommended method to measure resting energy expenditure; however, in the absence of IC, predictive equations may be used. A minimum protein intake of 1.5 g/kg/day and a balanced diet in other macronutrients ratio, including carbohydrates and lipids could be appropriate for mechanically ventilated children. However, the administration of major substrates should be based upon the patient’s metabolism regarding the nature and phase of the illness. Moreover, individualized nutritional supplementation is among the treatment strategies in these children.Conclusions
The optimum individualized nutrition support of mechanically ventilated children is considered a major therapeutic strategy and an essential aspect of their medical management. Further large population-based studies are required to provide appropriate feeding protocols for preventing nutritional inadequacy in such patients.Keywords: Critically ill children, Mechanically ventilated children, Nutritional management
Congenital scoliosis is a difficult condition for orthopedic surgeons. There are some influencing factors to choose the best treatment option for scoliosis.Objectives
Patients with congenital scoliosis may encounter different anomalies. There exist various surgical techniques with different indications.Methods
Electronic databases, such as Google Scholar, PubMed, and Scopus were searched for congenital scoliosis. Articles published from 1928 to 2020 were searched. A narrative review was conducted by focusing on treatment options.Results
Different methods are presented in the literature that consists of operative and nonoperative approaches. Nonoperative treatment methods are seldom a final choice. They are used to postpone the final surgery. There are different methods of surgeries in the literature; the best treatment strategy concerns the patients’ condition and the surgeon’s preference.Conclusions
The critical issue in the management of congenital scoliosis is to diagnose these patients’ curves before severe progression, i.e. mandatory to achieve desirable results. Usually, a course of nonoperative treatment can be started, but only to postpone the final surgery. The preferred surgical treatment depends on the type of congenital scoliosis and the age of the patient. The treatment of congenital scoliosis should be a multidisciplinary approach due to concomitant morbidity in these patients.Keywords: Congenital scoliosis, Classifications, Diagnosis, Surgical treatment
Inborn errors of metabolism or Inherited Metabolic Disorders (IMD) are a class of genetic disorders that occur because of single-gene defects.
In this narrative review article, the authors searched Institute for Scientific Information (ISI), Web of Science, PubMed, and Google Scholar for the relevant evidence.Results
The ocular manifestations of IMDs can be distinguished in different diseases such as Albinism, Cystinosis, Homocystinuria, and Sulfite oxidize deficiency, Mannosidosis, Fucosidosis, Sialidosis, etc.Conclusions
Due to the direct toxic mechanisms of abnormal metabolites on eyes and regarding the effect of eye monitoring on the follow-up, management, and treatment, a detailed ophthalmological assessment is essential.Keywords: Inherited Metabolic Disorder (IMD), Ophthalmology, Eye
Stainless Steel Crowns (SSC) are stable and durable restorations and a valuable technique in repairing deciduous teeth with extensive caries. Nickel and chromium are the main composing elements of SSCs, i.e. released in the oral environment; they can cause allergic reactions, especially in nickel-sensitive children. The symptoms of these reactions include gingival edema and the loss of alveolar bone.
The required data used in our review were searched from articles published until 2020 and collected from online databases, including Scopus, PubMed, Google Scholar, and Science Direct, using “Nickel, Allergy, and SSCs” keywords. Articles concerning SSCs and their application, nickel allergy released from crowns, and their symptoms were included in this study. After the abstract screening, we recalled relevant studies for full-text review.Results
We are exposed to nickel every day in various forms; some individuals present hypersensitivity reactions when exposed to the slightest amounts of this metal. Nickel is a base metal and among the elements used in SSCs. Nickel is released from SSCs during the corrosion process in the oral environment. Excessively released nickel ions cause chronic fatigue syndrome, sensitive lymph nodes, muscle aches, and headaches.Conclusions
The amount of nickel released from SSCs is less than the number of other sources; thus, the immunological reactions of a large percentage of the population to this excess amount of nickel ions in the bloodstream are normal and cause no severe problems.Keywords: Nickel, Allergy, Stainless steel crown, Children
Common arterial trunk (persistent truncus arteriosus) is a rare, congenital heart anomaly and characterized by Ventricular Septal Defect (VSD), single truncal valve, and a common ventricular outflow tract. We reported a case of truncus arteriosus type 1 in the fetus of a 28 years-old G2-P1-L1 pregnant female at 24 weeks of gestational age with large sub truncul VSD, truncal overriding, and main pulmonary artery bifurcation to the right and left pulmonary arteries.Keywords: Truncus arteriosus, Congenital heart disease, Fetal echocardiography
One of the most common nutritional deficiencies worldwide is iron deficiency. Fatigue, pallor, vertigo, dyspnea, cold intolerance, lethargy, palpitation, headache, and the pallor of the mucous membranes or nail beds are the most frequent symptoms and signs of iron deficiency. Thrombocytosis is commonly observed in iron deficiency anemia; it seems that erythropoietin plays the main role in this respect. Furthermore, thrombocytopenia and even leukopenia have been reported in iron deficiency; however, pancytopenia is a very rare condition. In this report, we presented two unusual cases of pancytopenia due to severe iron deficiency that improved after treatment with oral iron supplements. Iron deficiency anemia, if sufficiently severe, may be associated with reduced platelet and leukocyte counts. Accordingly, this condition should be considered as a differential diagnosis in all patients with pancytopenia.Keywords: Pancytopenia, Iron deficiency, Anemia
A case of intussusception may be missed on initial presentation, if the physician is not vigilant or if non-specific symptoms are present. It may progress to prolapse per anus, if not attended to. We reported a case of intussusception, i.e. missed on the initial presentation by a local practitioner and progressed to prolapse per anus and perforation peritonitis. The patient was managed well and experienced an uneventful course.Keywords: Intussusception, Trans-Anal prolapse, Gangrene, Perforation
The Relationship Between Serum Uric Acid and Cardiometabolic Risk Factors in Iranian Children and AdolescentsPages 167-174
There has been an increasing interest in epidemiological and clinical studies concerning the role of uric acid in cardiometabolic diseases, especially in children and adolescents. However, these potential relationships remain undiscovered; accordingly, its pathophysiological mechanisms remain unrecognized. This study aimed to assess the potential association between Serum Uric Acid (SUA) levels and cardiometabolic risk factors in a population-based sample of Iranian children and adolescents.Objectives
This study aimed to assess the potential association between Serum Uric Acid (SUA) levels and cardiometabolic risk factors in a population-based sample of Iranian children and adolescents.Methods
The data of 595 individuals aged 7-18 years were assessed in this research. Anthropometric measurements and laboratory tests were performed according to standardized protocols.Results
The Mean±SD age of the 595 explored students was 12.39±3.07 years. The overall Mean±SD SUA level of the study participants was measured as 4.22±1.13 mg/dL, with significant gender-wise differences (4.04±0.97 mg/dL vs 4.38±1.24 mg/dL, respectively; P<0.05). The prevalence of hyperuricemia based on the 90th percentile of SUA levels was equal to 10.6%. There was a positive association between SUA levels and abdominal obesity (waist circumference: ≥90th percentile) [Odds Ratio (OR): 1.54; 95% Confidence Interval (CI): 1.26 to 1.86] and general obesity [gender-specific Body Mass Index (BMI) for >95th percentile] (OR: 2.32; 95% CI: 1.74 to 3.11).Conclusions
This study suggested BMI and waist circumference as cardiometabolic risk factors, i.e. significantly associated with SUA levels in children and adolescents.Keywords: Uric acid, Metabolic syndrome, Obesity, Risk factors