Search published articles


Showing 26 results for Congenital
Congenital heart defects in children with oral clefts
Nahvi H, Mollaeian M, Kazemian F, Hoseinpoor M, Keiani A, Khatami F, Khorgami Z, Goodarzi M, Ebrahim Soltani A, Ahmadi J,
Volume 65, Issue 6 (9-2007)
Abstract
Background: Oral clefts are among the most common congenital anomalies. Infants with oral clefts often have other associated congenital defects, especially congenital heart defects. The reported incidences and the types of associated malformations and congenital heart defects vary between different studies. The purpose of this study was to assess the incidence of associated congenital heart defects in children with oral clefts.
Methods: All infants with cleft lip and palate referred to the Children's Medical Center and Bahramy the teaching Hospitals of the Tehran University of Medical Sciences from 1991 to 2005 were prospectively enrolled in this study group. All patients were examined and noted by an academic cleft team contain a pediatrician and a pediatric surgeon, and received cardiac consultation and echocardiography by a pediatric cardiologist. non cardiac associated anomalies, still born and patients without echocardiography were excluded from the study. Data including age, gender, exposure to contagions and high risk elements ,consanguinity and familial history of oral cleft, type of oral cleft, results of cardiac consultation and echocardiography and associated cardiac anomalies were cumulated and analyzed by SSPS version 13.5
Results: Among the 284 infants with oral clefts, 162 were male (57%) and 122 were female (43%). Seventy-nine patients (27.8%) had cleft lip, 84 (29.5%) had cleft palate and 121 (42.6%) had both cleft lip and palate. Of all the patients, 21.1% had congenital heart defects. the most common type Of these congenital heart defects(28.3%) was atrial septal defect.
Conclusions: For patients with cleft lip and palate, we recommend preoperative cardiac consultation, careful examination and routine echocardiography for associated cardiac anomalies, as well as appropriate management and prophylactic antibiotic therapy for those with associated congenital heart anomaly.
Relationship between ELA2 gene mutations, clinical and laboratory parameters in severe congenital and cyclic neutropenia
Farhoodi A, Ahangari Gh, Chavoshzadeh Z, Ramyar A, Movahedi M, Ghareghozlou M, Heydarzadeh M, Fazlolahi M, Bemanian M H, Zandieh F, Mansori M,
Volume 65, Issue 7 (10-2007)
Abstract
Background: Mutations of ELA2, the gene encoding neutrophil elastase (NE) are known to be associated with cyclic neutropenia (CN) and severe congenital neutropenia (SCN). However, high variability of these mutations has been reported. This study was designed to describe the analysis of the ELA2 gene, clinical manifestations and demographic characteristics in patients with CN and SCN.
Methods: A series of 21 patients with CN or SCN were selected, based on SCINR criteria, from the immunology ward of the Pediatric Medicine Center, Tehran, Iran, from March 2004 to August 2005. The ELA2 gene, isolated from blood samples, was analyzed using RT-PCR and automated capillary sequencing. Informed consent was obtained under the tenets of the Helsinki Declaration and the Ethical Committee of the Tehran University of Medical Sciences.
Results: Kostmann's syndrome and CN was diagnosed in three and 18 patients respectively. Of all the patients, one or two mutations were found in 18 cases (85.7%), including all three patients with SCN and 15 of the patients with CN. Exons two and four had the most mutations (eight and seven cases, respectively). Seven patients had double mutations in two distinct exons. Overall, 16 different mutations were found. At the time of presentation, the mean age of patients was 13.4 ±17.6 months, ranging from one month to seven years. Overall, 61.9% of patients had consanguineous parents. The mean absolute neutrophil count was 830.5 ±419.4 (150-2000)/mm3. On average, each patient had been admitted to the hospital 2.2 ±1.6 times. The neutrophil counts of the SCN patients were significantly higher than those of the CN patients. However, there was no significant difference in the neutrophil counts between patients with mutations and those without mutations. All patients with SCN had two or more infectious complications, although the prevalence of infectious or non-infectious complications did not correlate with ELA2 mutations or the neutropenic disorders.
Conclusion: Mutations in ELA2 appear to play an important role in the phatogenetic mechanisms of CN and SCN. Patients with CN had significantly higher neutrophil counts than SCN patients with CN. Although it possible for the gene encoding neutrophil elastase to have more than one mutation in distinct exons, we found no association between the mutations in ELA2 and their complications in CN and SCN patients.
Comparison of short-term and long-term outcomes of spinal fusion with and without posterior instrumentation in congenital scoliosis
Behtash H, Ameri E, Ganjavian M.s, Kabirian Dehkordi N, Fereshtehnejad S.m, Akbarnia B,
Volume 65, Issue 8 (11-2007)
Abstract

Background: Congenital scoliosis is a developmental disorder defined as a lateral curvature of the spine. Its progressive trend and complications, such as cosmetic problems, pain and pulmonary symptoms, have put scoliosis as an important skeletal deformity that should be corrected. One of the currently accepted methods of treatment is posterior spinal fusion (PSF) that may be performed with or without instrumentation. However, the use of implants in conjunction with PSF in congenital spine deformity has been debated over the past three decades primarily because of increased risk of neurological deficit and implant displacement. The aim of this study was to compare short-term and long-term outcomes of spinal fusion with and without posterior instrumentation in congenital scoliosis.

Methods: In this historical cohort study, 41 patients with congenital scoliosis were recruited. All patients underwent PSF surgery between 1977 and 1996. They were divided into two groups according to the use of instrumentation: 22 congenital scoliotic patients who were treated by PSF without any instrumentation (group A), and 19 instrumented PSF patients (group B). Instrumentation was mostly performed using the Harrington rod. The major curve angle was measured before surgery, two weeks and one year after PSF surgery and at the end of the follow-up period.

Results: The mean baseline curve angles were 66.3° and 69.1° in groups A and B, respectively. The mean Cobb angles one year after PSF were 43.1° and 38.4° in groups A and B, respectively. The mean follow-up period was 8 years (SD=3) and, at the end of this period, the final Cobb angles were 47.3° and 39.4° in groups A and B, respectively. Therefore, the final angle correction was 28.7% in patients without instrumentation and 43% in patients with instrumentation. The mean loss of correction was 5.5% and 4.3% in groups A and B, respectively. The final curve angles was significantly more corrected for those patients in whom instrumentation was used than those without instrumentation (p<0.05).

Conclusion: The treatment of congenital scoliosis can be very challenging despite the benefits of modern surgeries and instrumentation methods. The results of our study demonstrate that the application of an implant with PSF surgery may lead to increased improvement of the scoliotic curvature in the short-term and long-term periods, as well as a decrease in the loss of correction and the rate of reoperation. In addition, the low incidence of complications in our study indicates the safety of the posterior instrumentation for the treatment of congenital scoliosis.


Different presentations of congenital gastrointestinal obstructions: a report of five cases
Najafi M, Abbaslou P,
Volume 66, Issue 11 (2-2009)
Abstract

Background: Obstructive gastrointestinal (GI) malformations are one of the most important congenital problems and have different clinical manifestations, which depend on the severity and location. Although, complete obstructive lesions cause classic intestinal obstructive symptoms in infants, incomplete obstruction might be asymptomatic until adulthood and sometimes cause unusual symptoms, which need extensive work up. Moreover, this delay could produce significant complications. Near all of these lesions are surgically correctable therefore awareness of this possibility is important in order to prompt appropriate management and referral plan.

Case report: In this report, we introduce five infants who had congenital GI malformations with different presentations such as vomiting, hematemesis, growth failure and steatorrhea. Appropriate time of diagnosis caused surgical correction of lesions except in one patient with very unusual presentation who was diagnosed late. As a result, in this case, severe growth and developmental delay appeared.

Conclusion: Congenital gastrointestinal obstruction has different manifestations. Awareness can help to survive patients.


Congenital dislocation of knee: results of the treatment in 29 cases between 1970 and 2010 years at Shafa Yahyaian Hospital in Tehran
Rahbar M, Jabalameli M, Aqajani N, Shafipour R,
Volume 70, Issue 5 (8-2012)
Abstract

Background: There are not many reports regarding the treatment approaches of congenital dislocation of the knee in the literature. Therefore, the preferred method of treatment of this rare congenital disease is still controversial. Hereby, we report the treatment outcome of 29 patients admitted in Shafa Yahyaian Hospital during 40 years.

Methods: In this retrospective study done in Shafa Yahyaian Hospital in Tehran, Iran during 2010, we retrospectively reviewed the medical records of 29 patients treated conservatively or surgically for congenital dislocation of the knee. Patients had been evaluated for an average follow-up of 8.5 years.

Results: Overall, 54 knee dislocations had been treated surgically (58%) or conservatively (42%). Range of knee motion, persistent recurvatum, instability, valgus deformity after treatment and limping were the more important factors reported in the two surgically or conservatively treated groups. Knee function was satisfactory in patients with conservative treatment. Despite 80% of instability in the operated knees, patients could ambulate with or without braces and had a range of motion equal to 80 degrees. Knee function was also good after quadricepsplasty with transarticular pins in selected cases.

Conclusion: We recommend a conservative approach to the disease, regardless of the patient's age at the time of treatment and subsequent surgery in patients with dissatisfactory recovery. Quadricepsplasty with transarticular fixation is recommended as a good option in treating these patients. Posterior capsulorrhaphy for patients with CDK and ligamentous laxity is also recommended.


Adult choledochal cysts in 23 patients: a brief report
Maddah Gh, Ghamari Mj, Shabahang H,
Volume 70, Issue 8 (11-2012)
Abstract

Background: Choledochal cyst is a congenital disease of hepatobiliary system. Patients with the disease differ in terms of gender and symptoms. In this study, we aimed to evaluate the patients from clinical signs and symptoms, diagnostic methods and treatment perspectives.
Methods: In this retrospective study, we evaluated the data from the medical records of patients with choledochal cyst admitted in Ghaem and Omid hospitals affiliated to Mashhad University of Medical Sciences in Mashhad, Iran during 1994 to 2011.
Results: The medical records of 23 (18 women and 5 men) patients were evaluated. The mean age of the patients was 24.6 years. The most common type of the disease was choledochal cyst type 1. The most common type of surgery performed in the patients, respectively was resection of biliary cyst, cholecystectomy and Roux-en-Y hepaticojejunostomy. No mortality was observed during the hospital stay or follow-up period. Surgical complications were minor and patients recovered by conservative measures.
Conclusion: Regarding the favorable results of surgical treatment of choledochal cysts, timely and definite diagnosis of this congenital anomaly and proper planning for its surgery are of special importance.


Balloon atrial septostomy under echocardiographic guide: case series
Tabib A, Mortazaeian H, Meraji Sm, Birjandi H,
Volume 70, Issue 9 (12-2012)
Abstract

Background: Balloon atrial septostomy is an emergent procedure in pediatric cardiology. Nowadays, most patients in need of the procedure have acceptable outcomes after surgical repair. Thus, it is important to perform this procedure as safe as possible. By performing early arterial switch operation and prostaglandin infusion, the rate of balloon atrial septostomy has markedly decreased. However, not all centers performing early arterial switch repairs have abandoned atrial septostomy, even in patients who respond favorably to prostaglandin infusion.
Case presentation: In total, eight 1- to 15-day old term neonates admitted in Shahid Rajaee Heart Center in Tehran, Iran from October 2009 to February 2011, with congenital heart diseases were scheduled for balloon atrial septostomy. In six cases the procedure was done exclusively under echocardiographic guidance and in two cases with the help of fluoroscopy. Success was defined as the creation of an atrial septal defect with a diameter equal to or more than 5 mm and ample mobility of its margins.
Results: Male sex was predominant (87%) and the mean age of the neonates was six days. The diagnosis in all cases was simple transposition of great arteries. The procedure was successful in all patients with any cardiovascular complication.
Conclusion: Balloon atrial septostomy is an emergent procedure that can be done safely and effectively under echocardiographic guidance. According to the feasibility of this technique it could be performed fast, safe and effective at bedside, avoiding patient transportation to hemodynamic laboratory or referral center.


Evaluating the pre-, peri- and post-natal conditions of neonates affected by multiple congenital anomalies: a brief report
Rashidi-Nezhad A, Fakhri L, Hantoush Zadeh S, Amini E, Sajjadian N, Hossein Zadeh P, Niknam Oskouei F, Akrami Sm,
Volume 70, Issue 10 (1-2013)
Abstract

Background: Neonatal deaths stand for almost two-thirds of all deaths occurring in infants under one year of age. Congenital anomalies are responsible for 24.5% of these cases forming a highly important issue for health policy-makers.
Methods: We studied the pre-, peri- and post-natal conditions of 77 patients with multiple congenital anomalies (MCA) through genetic counseling at Several university Hospitals, in Tehran, Iran. The collected data were subsequently analyzed using SPSS software.
Results: The patients did not have a good prognosis, demonstrating the need for the diagnosis of such diseases early in pregnancy to be of utmost importance. We screened for trisomy and nuchal translucency, which the first showed a low risk and the second showed normal results in most cases.
Conclusion: Establishment of standards for prenatal diagnosis of congenital anomalies and monitoring their implementation seem to be necessary for the reduction of deaths due to congenital anomalies and infant mortality rate (IMR).


Long QT in children with congenital deafness: a brief report
Naseraldin Akbari Asbagh , Parvin Akbari Asbagh , Zarrintaj Keihanidoust , Aliashraf Eghbali ,
Volume 71, Issue 5 (8-2013)
Abstract
Background: Long QT syndromes (LQT) are genetic abnormalities of ventricular repo-larization, with an estimated incidence of about one per 10000 births. It is characterized by prolongation of the QT interval in electrocardiogram (EKG) and associated with a high risk for syncope and sudden death in patients. Type of this syndrome is association with congenital deafness. Our objective was to evaluate QT interval in children with congenital deafness.
Methods: For 219 patients referred to Imam Khomeini Hospital audiometric clinic in 2011, questionnaire were completed. A total of 23 congenitally deaf children were incl-uded. All patients’ examinations were done by a pediatric cardiologist. Electrocardio-gram is conducted in all children (23 patients) with sever and deep congenital deafness. Then the QT interval was measured based on Bazett’s formula. Echocardiography was also performed in these children to assess left ventricular function and the presence of mitral valve prolapse.
Results: The overall patients were two hundred and nineteen children. A total of twenty three congenitally deaf children were included and electrocardiogram was obtained. Three children had obviously prolonged QTc (0.48±0.02) second. The median age of them was 6.1±5 year, the median weight was 18±11.3 kilogram and the median of QT interval was 0.48±0.02 second.
Conclusion: The QT interval obtained 0.48±0.02 second. In the present study we found prolonged QT in congenital deafness, thus we recommend to evaluate the electrocardio-gram of children with congenital deafness.

Prevalence of congenital Toxoplasmosis in two university hospitals: a brief report
Samileh Noorbakhsh , Majid Kalani , Ali Mohamad Aliakbari , Azardokht Tabatabaei , Fahimeh Ehsanipour , Reza Taghipour , Mohamad Reza Shokrolahi ,
Volume 71, Issue 6 (9-2013)
Abstract
Background: The incidence and clinical presentation of congenital toxoplasmosis in our newborns was not studied until yet. Goal of study is to evaluates the newborns for congenital Toxoplasma.Gondii infection and describe the clinical presentation from birth and follow up them. 
Methods: We conducted a prospective study upon 270 newborns were born in two university hospitals in Tehran (Rasoul akram & Akbar Abadi) during 2011-2012. Cord blood sample obtained from the newborns during labour. The samples centrifuged, transported and restored in -80 centigrade freezer in our Research Laboratory. Specific T.Gondii- antibodies (IGG, IGM) evaluated by ELISA methods. Neonates with positive T.Gondii- IGM diagnosed and studied as infected cases. The infected cases treated and followed for progression of disease.
Results: Gestational age of newborns was between 28-41 weeks. Positive T.Gondii -IGM and T.Gondii -IGG determined in 1.5%, 44.1% of cases respectively. The most common clinical presentation in seropositive cases was eye involvement (50%), and brain disorders (50%). Positive PCR had not found in cerebrospinal fluids of seropositive (IgM) cases.
Conclusion: One and a half percent of newborns were seropositive for T.Gondii. Wide variation of clinical presentation and early diagnosis of infected newborns in our country is so important. Adding the serologic tests (IGM) to neonatal screening test is recommended strongly.

Mitral valve Z value in Iranian patients with tetralogy of Fallot
Manouchehr Hekmat , Hamid Ghaderi , Seyedeh Adeleh Mirjafari , Shahram Rajaei Behbahani , Mehran Shahzamani , Gholamreza Masoumi ,
Volume 75, Issue 9 (12-2017)
Abstract
Background: Tetralogy of Fallot (TOF) refers to a condition in which left ventricular volume is normal or slightly less than normal. Given the differences observed in some Asian patients with TOF, the present study was conducted to investigate left heart by determining Z-scores for the mitral valve in Iranian patients with TOF.
Methods: Eligible subjects in this prospective descriptive study comprised all patients with TOF presenting to Shahid Modarres Hospital in Tehran from March 2012 to March 2015 and diagnosed as the candidates for surgery. After determining the need for surgery and the therapeutic method required, the mitral valve size and Z-scores were calculated. We analyzed sex, age, body surface area (BSA), mitral size (in 2-chamber and 4-chamber view), mitral Z-score, other cardiac anomalies, number of surgery and previous surgery.
Results: Of a total of 80 patients included in the study over 3 years, 29 (36.3%) were male and 51 (63.8%) were female. The mean age of the patients was 7.15±3.37 years and their mitral size was found to be 10-27 mm (16.2±5.99 mm) using echocardiography. Z-scores of the mitral valve were also obtained as -3.09±2.11, ranging between -7.1 SD and +1.3 SD. 58 (72.5%) patients have only TOF and 22 (27.5%) with other cardiac anomalies. 45 patients were for first time underwent surgery and 22 patients for second time, 12 patients for three time and 1 for forth. Of a total of 35 patients had previous surgery, the most common were shunt 15 (42.9%) and then tetralogy of Fallot total correction (TFTC) in 12 (34.3%). Of a total of 80 patients, 59 (73.8%) underwent TFTC, 17 (21.3%) under pulmonary valve replacement and 4 (5%) shunt.
Conclusion: Z-scores of the mitral valve were found to be significantly below the normal value, (i.e. 0±2 SD), in the study patients, suggesting the risk of hypoplastic left heart syndrome in Iranian patients with TOF, nevertheless, the type of TOF examined in these patients might have been different from those observed in other races and regions.
 

A review on Zika virus, a re-emerging arbovirus: review article
Farshad Khodakhah , Talat Mokhtari Azad ,
Volume 75, Issue 11 (2-2018)
Abstract
Before the recent outbreaks of Zika virus, few people have ever heard of its name. Even virologists had paid little attention to this member of the Flaviviridae family. Hence, up to January 2016, only 269 articles about Zika virus had been indexed in PubMed compared to the 9187 articles related to dengue virus. However, declaration of the World health organization (WHO) about the global Zika virus spreading, which has been associated with birth defects and some neurological problems, diverted more attention to this forgotten virus. Afterwards, the virus hit the headlines and became a research interest. Since then, up to 9 August 2017, the number of Zika related articles indexed in PubMed reached to 3214. Zika virus is a re-emerging arbovirus. The First detection of Zika virus was in Uganda in 1947. It belongs to the Flavivirus genus in the Flaviviridae family. Zika can typically cause a mild and self-limiting disease in a healthy person. However, in pregnant women, it might cause birth defects and occasionally it can be associated with peripheral neuropathy such as Guillain-Barre syndrome. Although many research have been conducted to find out the casual link between this virus and these disorders but this relationship is still dim and controversial. Considering its recent epidemics in 2015 and 2016 the geographical distribution of Zika virus seems to expand all over the world progressively. Interaction between virus and vector is dynamic. Variety of competent vectors and adaptability of virus to new arthropod vectors are the two major factors for this process. According to the last report published by WHO, 84 countries/territories in five continents have reported the circulation of Zika virus in their area. In the recent outbreak, WHO regional office in our region (EMRO) have reported no case of Zika virus transmission from this region. Nonetheless, because specific and competent vectors exist in some countries, this region has a potential of epidemic risk. Until now we have neither autochthonous nor imported case of Zika virus in our country but we should prepare for any unexpected situation. In this review, we will discuss new findings about the history, virological features, vectors, transmission routes and epidemiological aspects as well as laboratory diagnosis of Zika virus. In addition, the epidemiology of this virus in Iran will be discussed.

An assessment of urinary Iodine in pregnant women: brief report
Manijeh Jamshidi , Ahmad Naghibzadeh-Tahami, Elham Maleki, Vahidreza Borhaninejad, Hosniyeh Alizadeh , Mehrdad Farokhnia , Salman Daneshi,
Volume 76, Issue 3 (6-2018)
Abstract
Background: According to the direct connection between congenital hypothyroidism and iodine deficiency in pregnant women, also relatively high incidence of congenital hypothyroidism in some areas of Kerman province, especially Raver district located in North of Kerman province, this study was performed to determine and compare the urinary iodine concentration (UIC) in pregnant women referring to health centers.
Methods: This cross-sectional study was done during March 2014 and May 2015. Inclusion and exclusion criteria to be considered and UIC were measured by spectrophotometry in 384 and 374 pregnant women in Ravar and Kerman cities, Iran. Sampling method for this study was all of pregnant women in Ravar and random stratified sampling in Kerman. data were collected using a structured questionnaire. All statistical analyses were performed using SPSS Software, version 20.0 (IBM SPSS, Armonk, NY, USA). Chi-square test, Pearson's correlation coefficient and Logistic regression were used for associations and differences.
Results: The mean UIC was 200.21 µg/L in pregnant women of Ravar and 238.79 µg/L in pregnant women of Kerman. 22.7% of pregnant women were with low concentrations of iodine, 57.8% within the normal range and 19.5 percent were with high iodine concentrations in Ravar. While 5.3 percent of pregnant women were with low concentrations of iodine, 54.5% were within the normal range and 40.1% were with high UIC in Kerman. There were no significant differences between demographic variables and UIC in the two regions (P> 05/0). Multivariate regression models showed significant connections between the residence and UIC pregnant women (P< 0.001).
Conclusion: The results of this study showed that UIC in pregnant women of Ravar was significantly lower than Kerman and the place of living can be considered as a predictor of UIC in pregnant women.

Nutritional status of Down syndromic children with congenital heart disease undergoing surgical correction
Ehsan Aghaei Moghadam , Mohammad Reza Mirzaaghayan, Azadeh Sayarifard , Marjan Kouhnavard , Azin Ghamari ,
Volume 77, Issue 7 (10-2019)
Abstract
Background: Growth disturbance is a common phenomenon in children with congenital heart diseases (CHD). Malnutrition and nutritional disturbances have a higher prevalence among children with down syndrome, especially children with Down syndrome; on the other hand, the prevalence of CHD is higher among syndromic children, which needs surgical repair as the definitive treatment. The nutritional status plays an important role in determining the postoperative complications and recovery. The purpose of this study was to investigate the growth status of children with Down syndrome and congenital heart disease before cardiac surgery.
Methods: This study was conducted as a retrospective study by evaluating the records of all syndromic patients undergoing cardiac surgery at Children’s Medical Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran, from March 2011 to March 2017. Age, weight, height, weight-for-age z-score (WAZ), weight-for-height z-score (WHZ), height-for-age z-score (HAZ), mortality and hospitalization rate in an intensive care unit (ICU) were recorded in these patients. The z-scores more than -1 were considered as normal, between -1 and -2 as mild malnutrition, between -2 and -3 as moderate malnutrition and below -3 as severe malnutrition.
Results: 35 (51.5%) patients were female and 33 (48.5%) were male. The mean age, weight, and height of these children were 26.9±24.9 months, 9.1±4.95 kg, and 79.55±17.95 cm, respectively. The mean of WHZ, WAZ, and HAZ in these children was -2.18+1.65, -1.95+2.25 and -1.22+3.11, respectively. Based on the values of WAZ, WHZ, and HAZ, 85.3%, 77.9% and 75% of patients have malnutrition (mild to severe forms, z-score less than -1). The most common cardiac defect was ventricular septal defect (VSD) accompanied by pulmonary arterial hypertension.
Conclusion: Considering the high prevalence of impaired nutritional status in these children and considering the effect of preoperative malnutrition on surgical outcomes, including mortality, assessing the nutritional status is much important. The adequate nutritional support in these patients leads to a reduction of the mortality, postoperative complications and morbidities.

Assessment of the frequency and causes of heart consulting in patients admitted to the neonatal intensive care unit
Amir Hosein Movahedian , Mohammad Jahangiri , Mona Nabovati, Mohammad Reza Sharif , Raheleh Moradi , Ziba Mosayebi ,
Volume 77, Issue 9 (12-2019)
Abstract
Background: Congenital heart diseases are the second group of congenital anomalies in infants. These disorders are a major cause of death in the first year of a child's life. Early detection helps to treat these diseases better. In this study cardiology consultations of hospitalized infants in the neonatal intensive care unit were evaluated.
Methods: In this cross-sectional study, two hundred and fifty pediatric cardiology consultations conducted in Shahid Beheshti Hospital in the year 2012 were reviewed. Information such as the cause of consulting, delivery type, age of parents, relative couples, family history of congenital heart disease, maternal medications, maternal background diseases, the final diagnosis, and prognosis follow-up of the patients were recorded in a designed questionnaire. Finally, the data were entered into the SPSS software, version 16 (IBM SPSS, Armonk, NY, USA) and analyzed using descriptive statistics and chi-square test. P-value of less than 0.05 was considered significant.
Results: The mean age of the consulted neonates was 4.845±5.14 days with a gestational age of 33.933±3.65 weeks. Male sex and cesarean section were the most frequent. Fifty-six percent of consulted infants were male. The present study revealed that prematurity (76%), murmurs (30.8%), respiratory distress syndrome (14.4%) and cyanosis (13.2%) were the most common causes of the cardiac consultation seeking among infants. Seventy-six percent of infants were consulted due to prematurity. Eighty-four percent of infants had a normal conditions. Septal defects (ventricular or atrial septal defect) and patent ductus arteriosus were the most common disease diagnosed with the prevalence of 27.5 and 17.5%, respectively. There was a significant relationship between preterm labor and congenital heart disease (P<0.001). Additionally, prematurity associated with respiratory distress syndrome and using assisted reproductive techniques.
Conclusion: The higher prevalence of congenital heart disease in the present study, compared with other studies, reflects the fact that cardiology consultation based on clinical suspicion leads to the more identification of congenital heart disease that means the right referral of newborns for consultation was accompanied with a higher incidence of heart failure.

Development of clinical guideline for early detection of critical congenital heart diseases at birth by pulse oximetry
Alireza Ahmadi , Mohammad Reza Sabri , Zohreh Sadat Navabi, Mehdi Ghaderian , Bahar Dehghan,
Volume 80, Issue 5 (8-2022)
Abstract
Background: Congenital heart defects (CHD) are the most common type of birth defects. The main screening tests used to identify babies with congenital heart defects include prenatal echocardiography and postnatal clinical assessment. Routine pulse oximetry has been reported as an additional screening test that can potentially improve the early diagnosis of critical congenital heart disease (CCHD).
Methods: This study is a multi-stage evolutionary study that was conducted for 12 months from March 2021 to March 2022 at Pediatric Cardiovascular Research Center in Cardiovascular Institute, Isfahan, Iran. In the first phase, after identifying the topic and aims of the present study, questions were designed through the PICO method including (population, intervention, control, and outcomes). Then conducting systematic searches, the quality of all existing clinical guidelines (CG) of this field were evaluated using the Appraisal of Guidelines for Research and Evaluation (AGREE) instrument and a committee consisting of pediatric cardiologists and neonatologists in the field. Next, based on the opinions of experts, the quality of 1 out of 6 clinical guidelines for early detection of critical congenital heart disease at birth by pulse oximetry was assessed as optimal. Finally, the clinical guidelines draft was evaluated using the Delphi method and the panel of experts. After expert panel meetings and consensus between members, the final version of the guideline for early detection of critical congenital heart diseases at birth by pulse oximetry was developed.
Results: In the present study, criteria of clinical practice regarding the stages of newborn screening by pulse oximetry at birth were determined based on the evidence and health conditions and were presented in twenty-eight recommendations and six sections. The final recommendations were presented in the results section.
Conclusion: The finding of the present study showed that physicians, nurses, and midwives can apply the recommendations of this clinical guideline for the early detection and referral of neonates with critical congenital heart disease.

Evaluation of sensitivity and specificity of filter paper in screening of congenital hypothyroidism
Maryam Razzaghi-Azar , Mina Sepehran, Bahareh Lesani-Gouya ,
Volume 81, Issue 3 (6-2023)
Abstract
Background: Congenital hypothyroidism (CH) is one of the most preventable causes of mental retardation throughout the world. The aim of this study was to evaluate the sensitivity and specificity of filter paper in screening for congenital hypothyroidism and to determine an appropriate cut-off point for filter TSH for recalling screened neonates.
Methods: In this cross-sectional study (descriptive analytic), the records of 2739 neonates who had been recalled during the CH screening program in the east of Tehran, Iran (health care centers of the Iran University of Medical Sciences), from March 2011 to March 2015, were studied. According to the CH screening program in Iran, the TSH on filter paper>5 is the current cut-off point for recalling the neonates. The data was analyzed with SPSS software, version 21. The ROC curve was used to determine the TSH cut-off point and the sensitivity and specificity of the filter test. The relationship between the filter and serum TSH was also determined. The study was approved by the Ethics Committee of the Iran University of Medical Sciences.
Results: The recall rate was 5.4%. The prevalence of hypothyroidism was 3/1000 neonates (164). The prevalence in males and females was 51.1% and 48.9%, respectively, with no significant difference. The number of recalled neonates diagnosed as patients with congenital hypothyroidism based on the filter TSH level was 5
Conclusion: Based on the findings of the study, considering the kits available in the country and their sensitivity and specificity, the importance of diagnosing infants with congenital hypothyroidism, and the lower cost of filter paper than missing a hypothyroid infant, the current cut-off point is acceptable. However, more studies are needed in different regions of Iran to obtain more accurate results.

Prevalence of congenital cytomegalovirus infection in newborns admitted to the intensive care units in Tehran, Iran
Samileh Noorbakhsh, Mohammad Farhadi , Sara Minaeian, Morteza Haghighi Hasanabad ,
Volume 81, Issue 3 (6-2023)
Abstract
Background: Cytomegalovirus (CMV) is the most common cause of congenital infections in newborns which can lead to long-term complications in more than half of the cases with symptomatic infection at birth time. Unfortunately, neonates with congenital CMV infection will mostly remain undiagnosed because the golden time for detection is limited to the first 3 weeks of infants' life. This study aimed to determine the prevalence of congenital CMV infection in newborns admitted to intensive care units of hospitals in Tehran, Iran and assess related risk factors associated with the infection.
Methods: In this cross-sectional study from April to October 2017, newborns within the first three weeks of life who were admitted to the neonatal intensive care units (NICUs) of university-affiliated hospitals in Tehran, Iran, were eligible for enrollment. CMV infection in neonates was diagnosed through testing infants' Guthrie cards and detection of viral DNA via an in-house nested-PCR assay. Congenital CMV infection in neonates with positive results was confirmed by testing urine specimens as a sensitive and gold standard sample. Related data (demographic and maternal factors) were collected by questionnaires and analyzed.
Results: Congenital cytomegalovirus infection was diagnosed in 8 of 63 newborns (12.7%). Hearing loss was seen in 2 infected infants. The mean of head circumferences among infected neonates was significantly lower than that observed in uninfected cases. Infants with CMV related symptoms had statistically more chance to have infection (P=0.02). We also found Guthrie cards as a reliable sample with high sensitivity for CMV detection assays.
Conclusion: The current study showed a high rate of symptomatic congenital CMV infection among neonates attending on NICU sections of hospitals in Tehran, Iran. It is of crucial importance to note that based on evidence, diagnosis of infants with congenital CMV infection at early stages could help to decrease the burden of long-term diseases if associated with prompt interventions and reduce the costs of late-ineffective treatment. Therefore, routine screening of newborns for congenital CMV infection via Guthrie cards is suggested.

Approach and management of congenital cataract: A review article
Parisa Abdi, Zakieh Vahedian, Atefeh Dehghani, Mona Safizadeh, Elias Khalilipour ,
Volume 81, Issue 5 (8-2023)
Abstract
Congenital cataract refers to the presence of any opacity in the lens at birth or during early childhood. Cataracts in children can manifest as unilateral or bilateral, congenital or acquired, with specific inheritance patterns or occurring sporadically. Additionally, the condition can be stable or progressive. Congenital cataracts can be associated with certain systemic diseases, or they may occur in the eye of an otherwise healthy child in isolation. Congenital cataract is one of the main causes of blindness in childhood. According to the report of the World Health Organization in 2001, congenital cataract is responsible for about 5 to 20 percent of cases of childhood blindness in the world based on different geographical regions. Untreated cataract in children leads to a tremendous social, economic and emotional burden for the child, family and society. Common complaints associated with congenital cataracts often include leukocoria (white pupil), reduced vision, a child's inability to track objects up close, light sensitivity leading to eye closure, squinting, the presence of either small eyes (microphthalmos) or large eyes (buphthalmos), strabismus (eye deviation), and abnormal eye movements such as nystagmus. Childhood cataract-related blindness can be cured with early detection and appropriate management. Managing pediatric cataracts is a challenge. Increased intraoperative complications compared to adults, tendency to increase postoperative inflammation, associated with complications such as aphakic glaucoma and persistent fetal vasculature, change in refractive status of the eye, and tendency to develop amblyopia, all complicate the achievement of good vision. Congenital cataract has a good prognosis if it is diagnosed early and surgery is performed before 6 weeks. Factors that may adversely affect the outcome, include the presence of unilateral cataract, presence of nystagmus, strabismus, or any ocular defect such as microphthalmos and PFV. Pediatric cataract surgery has evolved over the years, and with improved knowledge about myopic shift and axial growth, outcomes for these patients have become more predictable. Optimal results depend not only on effective surgery, but also on careful postoperative care and visual rehabilitation. Hence, it is the combined effort of parents, surgeons, anesthesiologists, pediatricians and optometrists that can make the difference.

Prevalence of congenital heart disease among term infants with respiratory distress: a case study in Kerman
Mohammad Mehdi Bagheri , Fahimeh Sadeghi Zarandi ,
Volume 81, Issue 8 (11-2023)
Abstract

                                                            
Background: Congenital heart diseases (CHD) as one of the most common disorders in newborns are leading cause of perinatal mortality, also one of respiratory distress syndrome causes in newborns. This study aimed to determine the prevalence of congenital heart diseases in infants with respiratory distress syndrome.
Methods: This cross-sectional study is a retrospective study that surveyed congenital heart diseases in 145 term infants who were hospitalized in neonatal intensive care unit (NICU), Afzalipour hospital, Kerman. These infants were admitted to the neonatal intensive care unit from March 20, 2012 to the end of March 20, 2015 due to respiratory distress syndrome. Information was extracted from the medical records and then were entered in the checklist. Descriptive statistics (frequency, percentage), analytical (chi-square test) and SPSS version 20 software were used to analyze the data.
Results: Out of 145 infants with respiratory distress syndrome, 123 infants had congenital heart diseases. Therefore, the prevalence of congenital heart diseases in patients with respiratory distress syndrome was 84.83%. The most infants with congenital heart diseases were male (66.67%) and weighed between 3-4 kg (47.97%). More than 69% of newborns with congenital heart diseases were born by cesarean section and most of them were receiving medical treatment (85.37%). There was a significant difference between patient outcome and congenital heart diseases (P=0.018). The difference between sex, weight and type of delivery with congenital heart diseases was not significant. In terms of the prevalence of congenital heart diseases, atrial septal defect (ASD) was the most prevalent with 14.48%. After that, patent ductus arteriosus (PDA) was the most prevalent with 11.72%.
Conclusion: Due to the high prevalence of congenital heart diseases in term children with respiratory distress syndrome, all term infants with symptoms of respiratory distress syndrome need cardiac examination, especially echocardiography to diagnose the cause. In addition, due to the high mortality of infants with congenital heart diseases with symptoms of respiratory distress syndrome, the need for more, more accurate and complete care of these infants is suggested.


Page 1 from 2    
First
Previous
1
2
Next
Last
 

© 2024 , Tehran University of Medical Sciences, CC BY-NC 4.0

Designed & Developed by : Yektaweb