Iranian Journal of Pediatrics最新文献

{"title":"Lung Ultrasound Score to Predict Surfactant Administration in Premature Neonates with Respiratory Distress Syndrome: A Systematic Review and Meta-analysis","authors":"Mengke Xie, B. Deng, Song Li","doi":"10.5812/ijp-137444","DOIUrl":"https://doi.org/10.5812/ijp-137444","url":null,"abstract":"Context: The present systematic review aimed to investigate whether the lung ultrasound score (LUS) can accurately predict surfactant administration in premature neonates with respiratory distress syndrome (RDS). Methods: The systematic review was conducted according to the Cochrane collaboration or the preferred reporting items for systematic review and meta-analyses (PRISMA) guidelines. English language databases included PubMed, Ovid, Cochrane Library, Embase, Web of Science, and Scopus. The coverage date of this review was from the inception of each database to the end of May 2022. Data were extracted independently by two authors, assessed for quality using the quality assessment of diagnostic accuracy studies-2 (QUADAS-2) tool, and analyzed for heterogeneity using MetaDisc1.4 software. Results: Seven eligible articles and 653 neonates were finally selected from 176 studies for meta-analysis. Considering the LUS to predict the surfactant need in premature neonates, we extracted the variable from the studies. Also, we plotted the summary receiver operating characteristic curve with an area under the curve (AUC) of 0.94 (95% confidence interval (CI): 0.92 - 0.95). Forest plots of the LUS showed a pooled sensitivity of 0.86 (95% CI: 0.82 - 0.90) and a pooled specificity of 0.79 (95% CI: 0.75 - 0.82). The Cochrane-Q test, chi-square test, and inconsistency index confirmed the heterogeneity of the non-threshold effect (I2 > 50% or P < 0.05). The meta-regression analysis showed that the relative diagnostic odds ratio for the number of recruited cases (> 100 vs. < 100) was 0.65 (95% CI: 0.33 - 0.98, P-value < 0.05). The comparison of the cut-offs of the 4 - 6 score vs. the 8 - 12 score yielded a Z of 21.44 (P < 0.001). Conclusions: The LUS accurately predicts the onset of RDS in premature neonates and can guide surfactant administration but is subject to the cut-off effect. Variation in cut-offs is related to gestational age and disease severity.","PeriodicalId":14593,"journal":{"name":"Iranian Journal of Pediatrics","volume":null,"pages":null},"PeriodicalIF":0.5,"publicationDate":"2023-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88989168","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of Bone Mineral Density in Children with Celiac Disease","authors":"Didem Gulcu Taskin, Aysun Ata","doi":"10.5812/ijp-134470","DOIUrl":"https://doi.org/10.5812/ijp-134470","url":null,"abstract":"Background: Osteoporosis is a known entity in individuals with celiac disease (CD). Objectives: The aim of this study was to evaluate bone mineral density (BMD) measurements in children with CD. Methods: This was a retrospective design study in a tertiary hospital in Turkey. Results: Totally 106 patients were included, and the mean age was 10.2 ± 3.9 years. The mean L1-L4 Z score was 0.730 ± 0.197 (based on sex and height in Turkish children). About 20.7% (n = 22) had a history of at least one fracture and/or bone pain. Bone mineral density values obtained from lumbar (L1-L4) vertebrae were significantly lower in patients with a prior fracture and/or bone pain compared to the patient group without a fracture (0.822 ± 0.242 g/cm2 vs 0.706 ± 0.178 g/cm2, respectively; P = 0.047). L1-L4 Z score (based on sex and height in Turkish children) was lower in patients with a body mass index (BMI) SDS value below -2 (moderately or severely malnourished) than those with a BMI SDS above -2 (0.02 ± 1.19 vs 1.4 ± 1.82, respectively; P = 0.014). A moderate positive correlation (r = 0.547; P < 0.01) was found between somatomedin-C (IGF-1) level and L1-L4 BMD (g/cm2) measurement. It was remarkable that low BMD was not identified in any patients using L1-L4 Z-score by height and L1-L4 Z-score by age of Turkish children. Nine (8.5%) patients were found to have low BMD using the United States (US) age- and sex-specific L1-L4 Z-score from the GE Healthcare Lunar iDXA system. Conclusions: Bone mineral density should be screened considering risk factors for suboptimal bone health in children with celiac disease. False \"normal\" BMD interpretations can be avoided by taking into account the model of the BMD measuring device and by using the device's normal ranges in case of inconsistency with the Turkish reference values.","PeriodicalId":14593,"journal":{"name":"Iranian Journal of Pediatrics","volume":null,"pages":null},"PeriodicalIF":0.5,"publicationDate":"2023-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89693534","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comparison of Enhanced and Manual Urinalysis for Detecting Urinary Tract Infections in Children","authors":"G. Sarvari, S. Saadat, F. Ghane Sharbaf, M. Naseri, A. Azarfar","doi":"10.5812/ijp-131098","DOIUrl":"https://doi.org/10.5812/ijp-131098","url":null,"abstract":"Background: Urinary tract infections (UTIs) are among the most common bacterial infections in children. Urinalysis (UA) is a beneficial test for the preliminary diagnosis of UTIs. The presence of bacteriuria in UA can be determined by either an enhanced (using uncentrifuged Gram-stained specimens) or manual (using centrifuged specimens) technique. However, the diagnostic performance of enhanced UA is not well-established in childhood UTIs. Objectives: To assess the ability of enhanced and automated urinalysis to detect UTIs in children. Methods: This cross-sectional study was conducted on 191 children with the symptoms of UTI referred to Dr. Sheikh Hospital, Mashhad, Iran, from 2018 to 2019. Standard urinalysis, enhanced urinalysis, and quantitative urine culture were performed on specimens. A positive enhanced UA test was defined as ≥ 10 white blood cells per mL of urine and the presence of any bacteria per 10 high-power microscopic fields of a Gram-stained smear. A positive manual UA test was defined as ≥ 5 white blood cells per high-power field. The results of standard and enhanced UA were compared with urine culture findings to determine the accuracy of these two methods in detecting UTIs. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were determined for each test. Results: The results showed that the prevalence of UTI was 23%. Enhanced UA retrieved a sensitivity of 97.7%, specificity of 93.1%, PPV of 81.1%, and NPV of 99.3% for detecting UTIs. In standard UA, sensitivity, specificity, PPV, and NPV were 90.9%, 80.7%, 57.1%, and 96.6% for pyuria, 56.8%, 98.6%, 92.5%, and 88.4% for the nitrite test, 72.7%, 94.5%, 80%, and 92% for the leukocyte esterase test, respectively. Conclusions: Enhanced UA had higher sensitivity, specificity, PPV, and NPV than standard UA.","PeriodicalId":14593,"journal":{"name":"Iranian Journal of Pediatrics","volume":null,"pages":null},"PeriodicalIF":0.5,"publicationDate":"2023-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88936861","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of Serum Zinc Levels in Children of 6 Months to 5 Years of Age Diagnosed with Simple Febrile Seizures","authors":"Saide Busra Oguz, Alper Kaçar, O. Dikker, H. Dağ","doi":"10.5812/ijp-132778","DOIUrl":"https://doi.org/10.5812/ijp-132778","url":null,"abstract":"Background: Febrile seizures are defined as seizures associated with a febrile illness that developed without central nervous system infection or acute electrolyte imbalance, intoxication, trauma, and metabolic disorder in children aged 1 month to 5 years without previous afebrile seizures. Various studies show a relationship between zinc levels and febrile seizures. Objectives: The purpose of this study was to examine the relationship between serum zinc levels and febrile seizures. Methods: This prospective, cross-sectional, and descriptive study was conducted from 04/10/2021 to 04/02/2022. A total of 85 children aged 6 months to 5 years admitted to the pediatric emergency service of the Republic of Turkey Ministry of Health Cemil Taşcıoğlu City Hospital were included in the study. The patients who met the inclusion criteria and whose consent was obtained were included in the study. Complex and febrile status patients were not included in the study due to the small number of patients. The cases included in the study were divided into 2 groups of patients and healthy controls. The patients with fever were divided into 2 groups, including those with and without febrile seizures. The levels of zinc and complete blood count parameters were measured in the blood samples taken from all the patients. IBM SPSS 22 package software was used for the statistical examination of the data. Results: When the cases included in this study were divided according to gender, 33 (38.8%) and 52 (61.2%) patients were female and male, respectively. The mean age of the cases was 29.2 ± 15.9 months (range: 6 - 60). Of the 85 participating cases, 30 patients had febrile seizures, 30 were only febrile patients, and 25 were healthy controls. When zinc levels were compared between the groups, the plasma zinc levels of the febrile seizures and febrile groups were observed to be lower than the healthy control group (P < 0.05). Conclusions: In this study, the group consisting of patients with febrile seizures and the groups of patients who had a fever but did not have febrile seizures during the study had reduced serum zinc levels than the control group consisting of healthy patients without fever.","PeriodicalId":14593,"journal":{"name":"Iranian Journal of Pediatrics","volume":null,"pages":null},"PeriodicalIF":0.5,"publicationDate":"2023-08-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83981882","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prevalence of Sleep Bruxism and Respiratory Disorders in Schoolchildren Aged 8 - 11 Years in Kerman, Iran: Pre-COVID Pandemic Phase","authors":"Elham Farokh Gisoure, Athareh Zare Emamzadeh, Amir Nekouei","doi":"10.5812/ijp-127527","DOIUrl":"https://doi.org/10.5812/ijp-127527","url":null,"abstract":"Background: Bruxism is a parafunctional oral activity defined as excessive teeth grinding or jaw clenching. This disorder causes damage to the teeth and deforms them. Objectives: This is the first phase of the sleep bruxism and respiratory disorders assessment study in Kerman, Iran, which evaluated respiratory disorders and sleep bruxism in schoolchildren aged 8 - 11 years between 2018 - 2019. Methods: A total of 573 primary school students aged 8 - 11 years were recruited for this cross-sectional study in Kerman between 2018 - 2019. The subjects were chosen randomly from 20 schools located throughout the city. Following permission from officials, 30 students from each school were admitted to the study randomly. The parents were invited to fill out a checklist that included a history of respiratory and sleep problems and signs of bruxism or abnormal jaw movements. Additionally, an examination was performed, and the symptoms of bruxism, such as tooth wear and restoration fractures, were documented. Results: The parents of 573 children were recruited to the study. The prevalence of bruxism and respiratory diseases was observed to be 20.6% (n = 118) and 26.5% (n = 152), respectively. The children with sound production had 2.3 times higher odds of bruxism prevalence than those without sound production (P = 0.004). However, children with temporomandibular joint and paranasal sinus sensitivity had 4.5 (P = 0.001) and 3.8 (P = 0.001) times higher odds, respectively. Additionally, the odds of bruxism prevalence were 1.4 times higher in children with respiratory disorders than those without (P = 0.001). Conclusions: Bruxism was common in children who had a respiratory disorder. Given the potential impact of bruxism on children’s dental health, it is important to pay special attention to the health status of children with respiratory disorders.","PeriodicalId":14593,"journal":{"name":"Iranian Journal of Pediatrics","volume":null,"pages":null},"PeriodicalIF":0.5,"publicationDate":"2023-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83396768","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Improvement with Therapeutic Plasma Exchange in Neuroimmunological Children: A Single Center Experience","authors":"A. Nikkhah, M. Nasehi, N. Momtazmanesh, Kourosh Etemad, Somaye Hajatnia","doi":"10.5812/ijp-137105","DOIUrl":"https://doi.org/10.5812/ijp-137105","url":null,"abstract":"Background: Neuroimmunological diseases in children encompass a range of disorders that lead to neurological complications in patients due to immune responses and systemic circulating antibodies. Limited research has been conducted on therapeutic plasma exchange’s efficacy and potential side effects in children with neuroimmunological diseases. Objectives: This study aimed to investigate this procedure’s effectiveness and potential side effects in children afflicted by these diseases. Methods: This cross-sectional study examined a cohort of 18 children with neuroimmunological diseases who were admitted to the neurology department of Mofid Hospital over one year from March 2021 and underwent therapeutic plasma exchange. Results: The study included 18 patients, with an equal distribution of 9 females and 9 males. A total of 121 procedures were performed across 6 different disease groups: Multiple Sclerosis (22%, n = 4), Autoimmune Encephalitis (22%, n = 4), Neuromyelitis Optica Spectrum Disorder (22%, n = 4), Guillain-Barré syndrome (22%, n = 4), Acute Disseminated Encephalomyelitis (6%, n = 1), and Optic Neuritis (6%, n = 1). Following the plasma exchange, 17 patients (95%) showed immediate clinical improvement, while one patient diagnosed with optic neuritis did not respond to the treatment. During the follow-up period, 14 patients (78%) demonstrated significant improvement, one patient (6%) showed moderate improvement, and two patients (11%) exhibited mild improvement compared to their pre-plasmapheresis condition. Laboratory examinations revealed that only one patient experienced thrombocytopenia, which resolved without requiring treatment. No complications were observed during the follow-up visits for any of the patients. Conclusions: Plasma exchange is a safe procedure for children with neuroimmunological diseases and yields favorable clinical responses.","PeriodicalId":14593,"journal":{"name":"Iranian Journal of Pediatrics","volume":null,"pages":null},"PeriodicalIF":0.5,"publicationDate":"2023-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78792142","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk Factors of Mortality in the Intensive Care Unit Following Cardiac Surgery for Congenital Heart Diseases in Children","authors":"M. Sharifi, M. Mirzaaghayan, Sara Memarian, Hamid Sharifi, B. Gharib","doi":"10.5812/ijp-132744","DOIUrl":"https://doi.org/10.5812/ijp-132744","url":null,"abstract":"Background: Children with congenital heart diseases (CHDs) often require cardiac surgery, whose outcome depends on many preoperative, intraoperative, and postoperative factors. Objectives: We aimed to investigate the factors associated with in-hospital mortality among patients undergoing pediatric heart surgery for CHDs. Methods: This retrospective cross-sectional study included patients younger than 18 who underwent cardiac surgery due to CHDs at the Children's Medical Center of Tehran University of Medical Sciences and were admitted to the open-heart surgery intensive care unit (OH-ICU) between March 2018 and March 2019. Patients with incomplete records were excluded. The collected data included age (months), weight (kg), type of congenital defect, duration of intensive care unit (ICU) stay, cardiopulmonary bypass (CPB) time, aortic cross-clamping (XCT) time, postoperative (day 1) platelet count and neutrophil-to-lymphocyte ratio (NLR), and mortality. Results: This study included 275 CHD patients who underwent cardiac surgery. The mean age and weight were 32.54 ± 37.4 months and 11.01 ± 8.43 kg, respectively. Those who died were younger (P = 0.05) and had lower weights (P = 0.001). An inverse relationship existed between thrombocytopenia and mortality. The NLR was higher in those who survived (mean 4.08 vs. 2.87), while the deceased patients had an extended duration of hospitalization, longer CPB time, and longer XCT time. Conclusions: Younger age, lower body weight, lower NLR ratio, lower platelet count, longer intraoperative CPB time, and XCT time were not associated with increased in-hospital mortality following cardiac surgery for CHDs. The Risk Adjustment for Congenital Heart Surgery (RACHS-1) score was not significantly related to mortality. Also, CPB and XCT times were significantly longer in cyanotic than in non-cyanotic patients. Cyanotic patients also had significantly lower platelets than non-cyanotic patients after the operation. Large, multicenter studies are needed to further investigate the predictors of mortality following surgery for CHDs.","PeriodicalId":14593,"journal":{"name":"Iranian Journal of Pediatrics","volume":null,"pages":null},"PeriodicalIF":0.5,"publicationDate":"2023-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74511674","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of Swallowing Coordination in Infants with a History of Mechanical Ventilation Longer Than 7 Days in the Neonatal Period and Assessment of Response to Oral Stimulation Maneuver","authors":"M. Asgarshirazi, M. Shariat, Raheleh Moradi, Z. Farahani, Farzaneh Ziaei","doi":"10.5812/ijp-135552","DOIUrl":"https://doi.org/10.5812/ijp-135552","url":null,"abstract":"Background: Dysphagia is a frequent complication defined as difficulty in any stage of swallowing. Infants with a history of mechanical ventilation might show difficulty in coordinating pharyngeal muscles resulting in dysphagia. Objectives: The present study aimed to show the prevalence of swallowing disorders and associated symptoms among high-risk infants with a history of prolonged mechanical ventilation. Methods: A quasi-experimental study was conducted at the Breastfeeding Research Center, Tehran, Iran. All the neonates with a history of prolonged mechanical ventilation entered the study. Parents were asked to respond to a provided questionnaire regarding their infants’ swallowing disorders. Accordingly, the infants with any symptoms were considered candidates for intervention. The parents also received simple training to perform oral sensorimotor stimulation protocol for their children. Through a follow-up visit, the questionnaire was filled up for every infant. Finally, all the data related to the responses before and after interventions were compared to show the possible effects of the interventions. Results: A total of 25 infants entered the study. The mean mechanical ventilation period was 15.95 ± 6.644 days. Concerning the frequency of swallowing complications, 24 infants (96%) had different degrees of dysphagia. After 3 - 6 months of interventions, the number of cases with symptoms of coughing (P = 0.016), spitting food out by mouth (P = 0.0001), choking (P = 0.016), humid breath (P = 0.031), poor weight gain (P = 0.002), and the need to cut food into small pieces (P = 0.004) was significantly lower than the number of cases suffering from such complications before the intervention. The results also showed that after 3 - 6 months of interventions, dysphagia symptoms in 10 out of 24 infants (41.66%) entirely and in others (38.44%) partially improved. Conclusions: The results of the present study delineated that infants with a history of prolonged mechanical ventilation were at greater risk of swallowing complications. The early diagnosis and implementation of oral sensorimotor interventions could improve different symptoms of dysphagia.","PeriodicalId":14593,"journal":{"name":"Iranian Journal of Pediatrics","volume":null,"pages":null},"PeriodicalIF":0.5,"publicationDate":"2023-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77942913","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Efficacy of Intrapleural Fibrinolytic Agents Following Surgical Intervention for Empyema Thoracis: A Prospective Cross-Sectional Study in a Pediatric Population","authors":"M. Sarafi, L. Mohajerzadeh, M. Ebrahimian, Mahdokht Siavashi, Gholamreza Ebrahimi, N. Farahbakhsh, M. Hajipour, N. Mahdavi, Behnaz Niroomand","doi":"10.5812/ijp-136509","DOIUrl":"https://doi.org/10.5812/ijp-136509","url":null,"abstract":"Background: Intrapleural instillation of fibrinolytic agents is novel in treating empyema. Although the efficacy of this method for treating empyema remains a topic of debate among experts, several studies have shown that the results of this technique are almost equivalent to surgical operations in the lower stages of the disease. However, in more advanced stages of empyema, surgery may be required. Despite the worldwide use of fibrinolytic agents instead of surgical procedures, the benefits of using these agents postoperatively still need to be defined. Objectives: In this prospective study, we aimed to compare the effects of streptokinase and alteplase in managing empyema in patients who underwent any surgical operations. Methods: We prospectively compared the outcomes of using alteplase and streptokinase in children who underwent surgical operations for empyema between 2015 and 2022. Following the surgery, fibrinolytic agents were instilled through thoracostomy tubes according to a specific protocol. The length of stay, frequency of complications, need for another surgery, and mortality were measured in each group. Results: Among 53 patients who met the inclusion criteria, 31 (58.5%) patients received streptokinase postoperatively, while the others were treated using alteplase. The length of stay after the surgery, wound infection, and mortality rate did not differ significantly between the groups (P-value = 0.394, 0.080, 0.767, respectively). However, the need for another surgery due to unexpanded lungs was significantly lower in the alteplase group (0% vs. 19.4%, P = 0.028). Conclusions: Alteplase as an intrapleural fibrinolytic is more effective than streptokinase in patients who underwent surgical operations due to empyema. The need for another surgery following the instillation of alteplase through chest tubes postoperatively may be decreased.","PeriodicalId":14593,"journal":{"name":"Iranian Journal of Pediatrics","volume":null,"pages":null},"PeriodicalIF":0.5,"publicationDate":"2023-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77220883","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Inborn Errors of Metabolism in Iran: First Report from Iran Metabolic Registry","authors":"F. Abbasi, Saideh Abdolahpour, Reihaneh Mohsenipour, A. Rabbani, Ali Talea, A. Mohebbi, M. Khazdouz","doi":"10.5812/ijp-130939","DOIUrl":"https://doi.org/10.5812/ijp-130939","url":null,"abstract":"Background: Inborn errors of metabolism (IEMs) comprise over 1,000 disorders. Wide geographical and racial variations in the global birth prevalence and their inheritance patterns have been reported by epidemiological studies. Methods: Iran's Metabolic Registry was launched in March 2017. All patients diagnosed with IEM registered in the Iran Metabolic Registry from 2017 to July 2022 were included in this study. Results: During the five years, 1,233 patients were recorded in the database. Due to missing data, 320 patients were excluded from the study. Of the 913 registered patients, 402 were female, and 511 were male. The median age of registered patients was 10.3 years (one week to 48.3 years). The most prevalent group of IEMs was amino acid disorders, with 596 (66.6%) patients. The mortality rate of the patients was 36.5%. Conclusions: This study represents the first report from the IEM registry. A comprehensive registry of IEMs for collecting patient data will help clinicians with more accurate diagnoses of the disorders, monitoring, and follow-up. Furthermore, by estimating the burden of IEMs, the healthcare system and government could accurately assess the medication and equipment needed.","PeriodicalId":14593,"journal":{"name":"Iranian Journal of Pediatrics","volume":null,"pages":null},"PeriodicalIF":0.5,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79746459","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}