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Showing 26 results for Congenital

A Seddighy , M Meamarzadeh , A Bazrafshan ,
Volume 56, Issue 4 (7-1998)
Abstract

Congenital cyst of the pancreas is an uncommon problem in the pediatric patient. The common symptoms at the time of diagnosis have been abdominal mass and pain or fullness, nausea and vomiting. Despite of various paraclinic methods for diagnosis, ultrasound is now recognized as the most effective and best noninvasive method for diagnosis of pancreatic cyts. Surgical intervention is the best mode of therapy. Upper abdominal cystic mass are seen in the fetus and newborn infants. In Amirkabir Hospital, during 12 years from 1981 to 1993, 8 pancreatic cysts have been operated on and only in this case it was congenital.
Moein A,
Volume 59, Issue 5 (9-2001)
Abstract

Congenital melanocytic nevi (CMN) are pigmented lesion which are present at birth or arise shortly there after. Some of these nevi have malignant potential, it's apparent is anxious for child and his parents. Little information is available on the prevalence of these nevi. Our purpose was to determine the frequency of CMN in primary school students in Tehran. From the students, 1902 persons were examined and 6.7%±2.2×10^-3 percent had CMN. The size of nevi was small (diameter <1.5 cm) in 4.7 percent, medium (diameter 1.5-20 cm) in 2.1 percent and large (diameter>20 cm) in 0.1 percent of cases. The location of nevi in order to prevalence was trunk, lower extremities, upper extremities and head and neck. There was not any relation between age, sex, race, hair color, eye color and prevalence of CMN. The prevalence of CMN was higher in our study than other studies perhaps reflecting different race or definition of CMN or unknown factors.
Moayeri H, Rabbani A,
Volume 60, Issue 2 (5-2002)
Abstract

Background: This study was performed in order to detect the frequency of different types of precocious puberty, predisposing factors and to show which group of patients need to treatment.

Materials and Methods: In this study, 74 patients who referred to pediatric endocrine clinic of Imam Khomeini Hospital and private office from 1993-2000 were assessed. A complete history was taken and physical examination was done in all patients by pediatrics endocrinologist. Bone age was done in all patients and specific hormonal tests were measured in some of them.

Results: Among 74 patients, 50 cases (67.6 percent) were female and 24 cases (32.4 percent) were male. The age of the patients was between 0.8-10 years old with mean 5.37 year. 75 percent of male patients had central (complete) precocious puberty and 25 percent had peripheral (incomplete) precocious puberty. 40 percent of female patients had central precocious puberty, 46 percent had premature telarche, 8 percent had premature pubarche, 2 percent had premature menarche and 4 percent had other kinds of precocious puberty (one case with hypothyroidism and one case with Mccune-Albright syndrome). 10 percent of female patients with central precocious puberty had predisposing factor and 90 percent of them were idiopathic. 38.8 percent of male patients with central precocious puberty had predisposing factor and 61.2 percent of them were idiopathic. 100 percent of male patients with peripheral precocious puberty had congenital Adrenal Hyperplasia. 38 percent of female patients and 100 percent of male patients needed to treatment.

Conclusion: According to this study and references, precocious puberty in female usually benign but in male patients it should be evaluated thoroughly because they usually have a serious disorder which needs treatment.


Akbari M, Sobhani A, Kashani I R, Amini E, Rezai Z , Shajari H, ,
Volume 61, Issue 3 (6-2003)
Abstract

The aims of this study were to determine incidence and types of observable congenital abnormalities among births of Mirza Kochak-Khan, Imam Khomeni and Shariatti hospitals between first of Novermber 2000 to the end of September 2001.
Materials and Methods: We used neonates that survived for 24 hours after delivery. A questionnaire was specially designed to explore each of the objectives for our study. A group of experts that were thoroughly trained to completed questionnaires by interview to mothers and evaluation of neonates. Data analysis was performed and by Excel and SPSS.
Results: Results showed that congenital abnormalities were present in 205 (3.2 %) of 6424 neonates, from 3 hospitals. The most frequent abnormalities consisted of musculoskeletal defects (37.3 %), Nervous system defects (24.7 %), urogenital defects (24.3 %) head and neck defects (13.6 %).
Conclusion: The result of this study showed that incidence of visible congenital abnormalities ratio in contrast to other countries have not significant difference but the types abnormalities were significant.
Ahmadi J, Klantari M, Kahbat Zaeh A, Samadi S Mh,
Volume 61, Issue 3 (6-2003)
Abstract

Ambiguous genitalia means difference between gonads morphology and external genitalia. Gender assignment and surgical correction must be done as early as possible to assure unambiguous bonding between the parents and the child. The purpose of this study was to evaluate Ambiguous genitalia in a referral children surgical center.
Materials and Methods: We retrospectively reviewed records of 200 patients admitted to children medical center hospital in a period of 16 years for Ambiguous genitalia surgery. Data about clinical and laboratory examination collected and analyzed.
Results: The prevalence of female pseudohermaphrodism, male pseudohermaphrodism, ture hermaphrodism and Mixed gonadal digenesis was 70.5%, 20.5%, 5% and 4%, respectively. 76.5% and 23.5% of the patients was converted to female and male, respectively. 29% had congenital Adrenal hyperplasia of whom 86.5%, 10.5% and 3% had 21-hydroxylase deficiency, 11-/? hydroxylase deficiency and 3/?HSD deficiency, respectively. 70.5% had 46xx karyotype and 21.5% had 46xy karyotype and the remainder were musaic. 52.5% had relative parents. The malformation were corrected by vaginoplasty (105 patient) and clitoral resection (51 patients). A variety of anomalies was seen.
Conclusion: Age of presentation ranged from 1 day to 13 years. 151 patients were diagnosed in infancy, but only 43 patient was brought for treatment in infancy to this center by parents.
A Madani , M Shakiba , N Taei , T Esfehani , P Mohseni ,
Volume 63, Issue 1 (5-2005)
Abstract

Background: Chronic renal failure defines as progressive and irreversible dysfunction of kidneys that could eventually terminated to end stage renal disease (GFR< 10% NL). Because of therapeutic problem and high mortality and morbidity and it &aposs implication quality of life , ESRD is one of the important dilemma of pediatric medicine .

Materials and Methods: In our study 216 patients evaluated .

Results: Male to female ratio was 1.1 . The peak of the presenting age of ESRD was 10 years old (8-12 y). Congenital urological malformation (30%) , glomerulopathies (20%) , hereditary nephropathies (14.3%) , multisystem diseases (7%) and nephrolithiasis (6.2%) are the most common etiologies of ESRD . VUR in 21% and congenital obstructive disease in 8.5% are the etiology of ESRD. In patients with age five years old and lesser common causes of ESRD are congenital urologic malformation and glomerulopathies. In other age groups , urologic malformation is the most common cause of ESRD. In etiologic assessment of two separate 7 years interval , (1988-1993) and (1996-2003) , there was not any significant change in frequency of etiologies but frequency of congenital obstructive uropathy decreased from 10 % to 5.7%. Total amount of VUR (VUR ± Neuropathic bladder) has not any change but frequency of primary reflux nephropathy decreased from 14.2% to 8%. In this study , in 145 patients hemodialysis continued and 28 cases had unsuccessful renal transplant (13.8%) . 7.4 % of patients had successful renal replacement therapy (RRT) and mortality rate was 7.4% . B

Conclusion: Based on that the most common cause of ESRD is all ages in congenital urologic malformations , early diagnosis and appropriate management of these cases are effective in decreasing incidence of ESRD and with respect to few cases of renal transplant and unsuccessful results in 65% of RRT , the approach of this problem should be revised.


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.
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.
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.


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.


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.


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.


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.


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).


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.

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.

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.
 

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.

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.

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.


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