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Background: This study aimed to determine postoperative cardiac and noncardiac complications and their association with the use of cardiopulmonary bypass (CPB), surgical outcomes (length of hospital and ICU stays), dependence on mechanical ventilation, and mortality. Methods: This retrospective cross-sectional study was conducted on pediatric patients aged 0 to 18 who underwent open or closed cardiac surgery over a 1-year period. The use of CPB support, CPB duration, cardiac surgery complexity according to the risk-adjusted classification for congenital heart surgery (RACHS-1), and demographics were examined as potential risk factors associated with an increased number of postoperative complications. The study was performed at the Children's Medical Center, a teaching hospital affiliated with Tehran University of Medical Sciences in Iran. Results: A total of 283 surgeries were included in our study. Seventy-six (26.9%) of the study population experienced at least 1 complication. Our analysis revealed that increased CPB durations were associated with higher odds of cardiac complications, with an odds ratio of 1.02 (P=0.002). Moreover, higher RACHS-1 levels were significantly associated with greater numbers of cardiac and noncardiac complications. Additionally, prolonged mechanical ventilation and open-heart surgery intensive care unit (OH-ICU) stays were significantly associated with cardiac and noncardiac complications. Our logistic regression analysis found no association between demographic and clinical risk factors, the number of complications, and mortality. Conclusion: Postoperative complications occurred in 27% of pediatric heart surgeries. Prolonged mechanical ventilation and OH-ICU stays were significantly associated with cardiac and noncardiac complications. However, no significant association was found between postoperative complications and mortality. [ABSTRACT FROM AUTHOR]

Background: CYP21A2 gene mutations are responsible for more than 95% of Congenital Adrenal Hyperplasia (CAH) disorders with autosomal recessive inheritance. Most of these pathogenic mutations originate from the CYP21A1P, a neighboring pseudogene with 98% homology, due to unequal crossing over or gene conversion events. Mutation identification of the gene could be beneficial for accurate diagnosis and outcome prediction. Methods: Twelve unrelated patients with CAH diagnosis were recruited for genetic counseling. To ensure distinct amplification of the CYP21A2 gene rather than its pseudogene, the complete sequence of the gene was amplified through two overlapping fragments by specific primers. The entire sequences were screened by direct Sanger sequencing using new sequencing primers. Results: Only two pathogenic point mutations were identified. The c.293-13C>G, also known as In2G, and the c.955C>T mutations were found in 37.5 and 33.3% of alleles, respectively. One patient showed homozygous gene deletion. We also reviewed recent reports on CYP21A2 gene mutations in Iran. Conclusion: Evaluating the ethnicity-specific gene mutation data is significant for populations with diverse ethnic groups including the Iranian population. Although several common mutations have been reported as causative mutations among CAH patients, identifying only two common point mutations in Fars province would help prioritize exon sequencing and reduce the cost and time of genotyping. [ABSTRACT FROM AUTHOR]

Background As congenital gastrointestinal diseases are pretty common and can lead to fatal consequences when combined with congenital heart diseases, we conducted a study in Iran to explore the relationship and prevalence of these two conditions. Our objective was to identify the types and frequency of congenital heart diseases in children who also have gastrointestinal anomalies. Materials and Methods: This was a cross-sectional analytical study. The research population was the patients referred to the Gastrointestinal Clinic of Hazrat Masoumeh Children's Subspecialty Hospital during the 2011-2017. In this study, the echocardiography in the patients’ files performed by a pediatric heart specialist was examined, and in the next step, the patients were divided into two groups with and without heart disorders. Gastrointestinal abnormalities were also recorded in the relevant checklist. Finally, the recorded information was entered into SPSS software version 22, and Chi-square and Fisher exact tests were used to compare the qualitative findings. The significant level for all tests was considered to be 0.05. Results: The average age of the children in this study was 7.93±7.74 months. 105 boys (54.7%) and 87 girls (45.3%) were examined. 167 children (87%) had cardiovascular abnormalities and 25 children (13%) had no abnormalities. The most common types of cardiovascular abnormalities were 69 (35.9%) septal defects, 36 (18.8%) atrial septal defects, and 22 (11.5%) pulmonary valve stenosis. The least frequent abnormalities were cardiomyopathy in three (1.6%) and ventricular inter-atrial septal defects along with atrial septal defects in one (0.5%) patient There was not a significant correlation between congenital heart diseases and frequency of closed anus, rectovaginal fistula, Hirschsprung, anal stenosis, rectal prolapse, perianal fistula, esophageal atresia, pyloric hypertrophy, intestinal obstruction, annular pancreas, biliary atresia, diaphragmatic hernia, Meckel's diverticulum.(P>0.05). A significant relationship was found only between omphalocele and pyloric hypertrophy with the presence of cardiovascular disorders (P<0.05). Conclusion: The results showed that congenital heart diseases have a high prevalence in infants with gastrointestinal anomalies, so cardiac examination is mandatory in all infants with gastrointestinal anomalies. [ABSTRACT FROM AUTHOR]

Background: Congenital heart disease (CHD), a developmental abnormality of the heart and vessels, is encountered in the pediatric age group frequently. Brachial artery flow-mediated dilation (FMD) and carotid intima-media thickness (CIMT) are indicators of subclinical cardiovascular disease and are used as surrogate measures of subclinical atherosclerosis. The present study aimed to compare CIMT and FMD between children with acyanotic congenital heart disease (ACHD) and healthy controls. Methods: A case-control study on 50 children with ACHD and 43 healthy individuals was done in Isfahan, Iran, between 2021 and 2022. The case group was selected via non-random sampling, and healthy controls were recruited from the relatives of the patients. A checklist, including age, sex, body mass index, and blood pressure, was filled out for all the participants. Then, FMD and CIMT were measured with brachial and carotid artery ultrasonography. Results: Fifty children with ACHD and 43 healthy individuals (controls) under 18 years old participated in this study. Of these, 44 (47.3%) were girls and 49 (52.7%) were boys. The mean FMD was significantly higher in the ACHD group than in the control group (0.084±0.027 vs 0.076±0.042; P=0.021; 95% CI, 007 to 0.122;). CIMT was significantly higher in the ACHD group than in the control group (0.39±0.12 vs 0.34±0.1; P=0.037; 95% CI, 0.009 to 0.102;). However, systolic and diastolic blood pressure did not show differences between the groups. Conclusion: Based on our results, CIMT and FMD assessment may help detect early changes in peripheral vessels associated with atherosclerosis in the future in ACHD. Further studies are needed to confirm our findings. [ABSTRACT FROM AUTHOR]

Background: The prevalence of coronavirus and its health-related psychological consequences such as fear and anxiety has been one of the most important health concerns in the 21st century. Objectives: This study aimed to investigate the predictive role of fear of COVID-19 and lockdown fatigue in coronavirus anxiety among patients with Congenital Heart Disease (CHD). Methods: This descriptive-correlational study was conducted through path analysis. The statistical population included the patients with CHD referred to Shahid Rajaei Hospital and Tehran Heart Center, 105 of whom were selected using convenience sampling. The data were collected using the Coronavirus Anxiety Scale (CAS), Fear of COVID-19 Scale (FCV-19S), and Lockdown Fatigue Scale (LFS). The reliability and validity of these scales were approved in the previous studies. After all, the data were entered into the SPSS 21 software and were analyzed using Pearson's correlation test and step-wise regression analysis. Results: The study was conducted on 105 participants (6% males and 94% females) aged 20 to above 41 years. Considering marital status, 5% of the participants were single, 94% were married, and 1% were divorced. In addition, the participants' education levels ranged from diploma and lower degrees to postgraduate and higher degrees. According to the findings, coronavirus anxiety was positively correlated to the fear of COVID-19 (r = 0.509, P = 0.000) and lockdown fatigue (r = 0.466, P = 0.000) in patients with CHD. The results of step-wise regression analysis showed that based on the calculated coefficient of determination, 31% of the variability of coronavirus anxiety could be explained by the fear of COVID-19 and lockdown fatigue. Conclusions: The study findings indicated that the fear of COVID-19 and lockdown fatigue could predict coronavirus anxiety. Therefore, interventions are recommended to be designed based on the introduction of programs concerning coronavirus anxiety, which may help reduce the anxiety and fear experienced by these patients. They can also be used as prevention programs to help prevent the onset of coronavirus anxiety in patients with CHD. [ABSTRACT FROM AUTHOR]

Toxoplasmosis is a zoonotic disease caused by an obligatory intracellular parasite, Toxoplasma gondii. The congenital form of the disease is a significant health problem around the world. This study aimed to determine the incidence of congenital toxoplasmosis in the newborn of Shiraz, Southern Iran, between 2013 and 2018. A total of 2498 blood samples of neonates were randomly collected by a standard heel puncture technique and dried on Guthrie cards from Shiraz newborn screening center. We provided questionnaire forms for mothers according to their demographic characteristics, such as age, place of residence, history of having cats as pet, and literacy level. ELISA immunocapture and PCR assays were applied to detect anti-Toxoplasma IgM and the parasite DNA in dried blood spot samples. The anti-Toxoplasma IgM antibody was detected in two out of the 2498 infants. Moreover, borderline titers were observed in 3 samples, which were considered suspicious, so these were retested after 18 months to detect IgG against Toxoplasma. Positive IgG titer was observed in two infants who had a positive IgM level. The genome of Toxoplasma was detected in one sample out of 2498. No significant differences were seen between the epidemiological factors with congenital infection. The incidence of congenital toxoplasmosis is estimated at 0.08% of studied samples. It seems that this incidence could present to the health ministry as a logical research achievement for the national screening program of newborns in Iran. [ABSTRACT FROM AUTHOR]

Background: In 2016, a prospective registry for pediatric patients with congenital heart disease (CHD) was established in Isfahan, Iran. Data on pediatric CHD in Iran are scant; accordingly, we aimed to report the early results of the Persian Registry Of cardioVascular diseasE (PROVE/CHD) Registry in Isfahan. Methods: All patients with CHD and associated defects diagnosed by pediatric cardiologists were assessed via echocardiography for inclusion in the present study between late 2016 and August 2019. The participants' sociodemographic characteristics, maternal history, birth history, medical history, current clinical presentations in the clinic or hospital, paraclinical data, cardiac diagnoses based on the International Classification of Diseases, 10th Revision (ICD-10), disease management plans, and medications were entered into a questionnaire by the subjects 'parents/legal custodians and physicians and then transferred to the PROVE/CHD Registry. Results: The PROVE/CHD registry encompasses 1252patients with CHD (49.9% male) at a mean age of 6.50±6.36years. The most frequent cardiac diagnoses were ventricular septal defect (39.3%), atrial septal defect (29.7%), patent ductus arteriosus (25.4%), pulmonary stenosis (11.0%), tetralogy of Fallot (6.1%), coarctation of the aorta (5.4%), and aortic stenosis (5.1%), respectively. The most frequent interventions were patent ductus arteriosus closure (4.3%), atrial septal defect closure (3.6%), pulmonary valvuloplasty (2.2%), coarctation of the aorta angioplasty (1.9%), and ventricular septal defect closure (1.1%), correspondingly. The approximate corresponding rates of corrective and palliative surgeries were 32.0% and 13.1%. The corrective surgeries were mainly comprised of ventricular septal defect closure (7.8%), patent ductus arteriosus closure (7.3%), atrial septal defect closure (5.1%), and tetralogy of Fallot repair (3.8%), respectively. The palliative surgeries mainly consisted of the Glenn shunt (9.0%) and pulmonary artery banding (3.6%). Conclusion: The PROVE/CHD Registry collects data on pediatric patients with CHD. The results of this registry can provide epidemiological data and a set of homogeneously defined cases for further studies. [ABSTRACT FROM AUTHOR]

Objective. To describe perinatal and neonatal outcomes in newborns exposed to SARS-CoV-2. Methods. A systematic review was conducted by searching PubMed Central, LILACS, and Google Scholar using the keywords 'covid ' AND 'newborn' OR 'child' OR 'infant,' on 18 March 2020, and again on 17 April 2020. One researcher conducted the search and extracted data on demographics, maternal outcomes, diagnostic tests, imaging, and neonatal outcomes. Results. Of 256 publications identified, 20 met inclusion criteria and comprised neonatal outcome data for 222 newborns whose mothers were suspected or confirmed to be SARS-CoV-2 positive perinatally (17 studies) or of newborns referred to hospital with infection/pneumonia (3 studies). Most (12 studies) were case-series reports; all were from China, except three (Australia, Iran, and Spain). Of the 222 newborns, 13 were reported as positive for SARS-CoV-2; most of the studies reported no or mild symptoms and no adverse perinatal outcomes. Two papers among those from newborns who tested positive reported moderate or severe clinical characteristics. Five studies using data on umbilical cord blood, placenta, and/or amniotic fluid reported no positive results. Nine studies reported radiographic imaging, including 5 with images of pneumonia, increased lung marking, thickened texture, or high-density nodular shadow. Minor, non-specific changes in biochemical variables were reported. Studies that tested breast milk reported negative SARS-CoV-2 results. Conclusions. Given the paucity of studies at this time, vertical transmission cannot be confirmed or denied. Current literature does not support abstaining from breastfeeding nor separating mothers and newborns. Further evidence and data collection networks, particularly in the Americas, are needed for establishing definitive guidelines and recommendations. [ABSTRACT FROM AUTHOR]

Background: This is a case report regarding a 2051-gram female newborn affected by right-sided congenital diaphragmatic hernia (CDH) presenting with encephalocele in the occipital region. Case report: The newborn was delivered by a 38-year-old mother from Darmian city, a rural district located in South Khorasan province, Iran. Conclusion: The CDH is an abnormality that rarely occurs with an approximate ratio of one in 3000 live births. It has been reported that 85% of the infants affected by this condition are left-sided and the classic posterolateral or Bochdalek hernia is the most common form. An incidence of 40%-50% has been reported regarding the other malformations associated with CDH, the most common of which are those involving the central nervous system. Some studies reported other rare associated abnormalities, including hepatopulmonary fusion, hypoplastic left heart syndrome, left heart hypoplasia, duodenal atresia, malrotation, and anorectal malformation. This case report aimed to mention encephalocele as another complication, which has not been reported for the CDH. [ABSTRACT FROM AUTHOR]

This study aimed to investigate clinical symptoms in patients with congenital factor V (FV) deficiency and the relationship between phenotype and factor activity level. Thirteen patients with congenital FV deficiency were investigated and the factor activity level and first clinical presentations were studied for each patient. The most common first signs and symptoms were post-surgery, post-partum, post-circumcision, and post-traumatic bleeding (30.76%), followed by easy bruising in 23.10% of the patients. The median age at the onset of clinical signs was 18 (range: 1-53) years. Patients were categorized into two groups of major and minor bleeding based on their first clinical bleeding symptoms. There was not a significant difference between the two groups with regard to factor activity level, age at diagnosis, prothrombin time, partial thromboplastin time, and international normalized ratio (p>0.05). There is a discrepancy between plasma FV activity level and the severity of clinical presentations. [ABSTRACT FROM AUTHOR]

Objective: Cervical saccular limited dorsal myeloschisis (LDM), previously so-called "cervical myelomeningocele," is a rare spinal dysraphism. Although the pathogenesis of true myelomeningocele is primary neurulation failure, LDM results from a delayed abnormality during the final stages of neurulation. The aim of the study was to evaluate the outcome of these patients and to assess the correlation of outcomes with the level and type of lesion. Also, pooled data from the literature on similar lesions were systematically reviewed., Methods: A retrospective study was conducted at Children's Medical Center (CMC), Tehran, Iran. Information of patients who underwent surgery between 2004 and 2020 (i.e., the recent series) was extracted and combined with data from a previously published series from the same center that were obtained between 2000 and 2003 (CMC series). The literature was reviewed for all published cases, to be combined with the CMC series for further analyses., Results: Twenty-two patients were included in the recent series. Combined with 16 previously published cases, 38 patients with a mean ± SD age at surgery of 11.75 ± 28.64 months were included in the CMC series. The rates of neurological deficit, hydrocephalus, and Chiari malformation type II in the CMC series were 26.32%, 39.47%, and 28.95%, respectively. The lesions were at the upper levels in 17 (44.7%) and lower cervical levels in 21 (55.3%) patients, with 31 cases (81.58%) diagnosed with stalk-type lesions and 7 cases (18.42%) with myelocystocele-type lesions. At final follow-up, 31 patients (81.57%) achieved sphincter continence, and all 36 accessible patients were ambulated, consisting of 28 (73.68%) independent and 8 (21.05%) dependent ambulation patients. The rates of Chiari malformation type II and hydrocephalus were insignificantly higher in patients with upper-level lesions, but those of neurological deficit, ambulation, and sphincter continence were not associated with level. The rates of hydrocephalus (p < 0.01), Chiari type II malformation (p < 0.01), and neurological deficit (p = 0.04) were significantly higher in the myelocystocele group. In the systematic review, 24.77% of patients had neurological deficit. Binary logistic regression showed that older age at surgery (p = 0.03) and associated spinal anomalies (p = 0.04) were significant predictors of deficits. Chiari type II malformation was significantly (p < 0.001) and hydrocephalus was marginally (p = 0.06) more common in patients with myelocystocele-type lesions. The rate of Chiari malformation type II was higher in patients with upper-level lesions (p = 0.02)., Conclusions: Patients with cervical saccular LDM had better outcome compared with those patients with true myelomeningocele in more distal areas. According to the current series, most patients obtained ambulation and voiding continence, regardless of the level or type of lesion. Hydrocephalus, Chiari type II malformation, and neurological deficit were more common in patients with myelocystocele-type lesions.

Background. Prevalence of congenital hypothyroidism is about one per 414 live births in Kurdistan Province, a western province in Iran. Recently, a surveillance system (diagnosis, report and treatment) was developed to control the disease in Iran. Aim. This study aimed to compare the IQ of children with congenital hypothyroidism with normal children in this province, where the disease is highly prevalent. Subjects and Methods. This retrospective cohort study was conducted on 100 children with congenital hypothyroidism under continuous treatment with thyroxine and a group of 100 healthy infants. Two tests of Goodenough draw-a-man test and Proteus Mazes were used to measure IQ. Statistical analysis was done by SPSS 16 and multiple regression. Results. The average IQ of the patient group treated for 42 months with thyroxine and healthy group were 103.4 (±16.9) and 103.4 (±15.4), respectively (p=0.989). There was no statistically significant difference between the IQ of children with transient and permanent hypothyroidism. In multivariate analysis, the only effective factor on IQ was mothers' education. Conclusion. There was no statistically significant difference between the IQ of children with congenital hypothyroidism who have been treated with thyroxine and the IQ of healthy children. So, there is appropriate quality of care for patients who are living in this province where the disease is highly prevalent. [ABSTRACT FROM AUTHOR]

Background: Immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is a rare autosomal recessive disorder. ICF1 is caused by bi-allelic mutations in the gene encoding deoxyribonucleic acid methyltransferase-3B (DNMT3B). Herein, we report a novel homozygous DNMT3B mutation in a patient with ICF1., Case Presentation: An eight-month-old Iranian Caucasian infant of consanguineous 1st-degree cousins presented to our clinic for evaluation of neutropenia. Physical examination was unremarkable except for low-set ears and a systolic cardiac murmur. He had a history of recurrent respiratory infections and oral thrush. Moreover, a collateral artery between the bronchial and pulmonary arteries was observed on the angiogram, mimicking a patent ductus arteriosus on the echocardiogram. Growth percentiles were normal; however, he had a neurodevelopmental delay. Family history was significant for a sibling who deceased at nine months of age after recurrent respiratory infections. Laboratory evaluation revealed a normal white blood cell count with neutropenia and normal bone marrow studies. He had hypogammaglobinemia with normal flow cytometric studies and was treated with prophylactic trimethoprim-sulfamethoxazole and itraconazole. After that, he was re-admitted three times due to recurrent episodes of pneumonia and an episode of pseudomonas aeruginosa meningitis. Currently, he is five years old and doing well on monthly intravenous immunoglobulin. Due to recurrent infections, hypogammaglobulinemia, and neutropenia, as well as a family history of consanguinity and a sibling who deceased during infancy, a primary immune deficiency was suspected. Genetic studies utilizing whole-exome sequencing demonstrated a homozygous missense mutation in DNMT3B (LRG&#95;56t1:c.2008C>T; p.Arg670Trp) in the patient studied. The mutation has not been previously reported., Conclusion: We describe a novel homozygous DNMT3B mutation in an Iranian boy with ICF1. It is associated with recurrent infections, hypogammaglobinemia, neutropenia, mild facial anomalies, and a bronchopulmonary collateral artery., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Objective: Unrecognized congenital hypothyroidism (CH) leads to mental retardation. Newborn screening and thyroid therapy started within 2 weeks of age can normalize cognitive development. In this systematic review, the local results of the national CH screening program in different provinces in Iran are reviewed and evaluated. Methods: Literature on the CH screening, the national databases including SID, Medlib, Iran Medex, Magiran as well as international databases including PubMed/Medline, ISI Web of Knowledge and web of science, EMBASE, SCOPUS and Google Scholar. Appraisal was guided by a checklist assessing clarity of aims and research questions. The 95% confidence intervals were calculated by I-square models. Meta regression was introduced to explore the heterogeneity between studies. Findings: We identified 25 samples including 1425124 neonates in our country. Data were Meta analyzed using random-effects models, and we found a TSH levels of 19633 babies in the first sampling were greater than the cut-off level (TSH ≥5mIU/L). The pooled recall rate was 0.014 (95 % CI: 0.013 - 0.015). According to Meta analysis the overall incidence of CH was 2/1000 (95% CI: .002 - .002). The incidence of CH did not appear to be increasing over time (P=0.08). Conclusion: Considering TSH ≥5mIU/L as a cut-off point for recalling neonates and low positive predictive value (14%) of this point shows that more investigation and research is needed for establishing accurate level of TSH as a criterion for recalling patients. [ABSTRACT FROM AUTHOR]

Background: Chylothorax is a rare but serious postoperative condition with a high rate of morbidity and may lead to the mortality of children undergoing congenital heart disease (CHD) surgery. This study evaluated the specific surgical procedures associated with the higher risk of postoperative chylothorax. Methods: We assessed 435 cases undergoing CHD surgery between April 2003 and May 2006. We detected postoperative chylothorax in 6 patients. The diagnosis of chylothorax was established based on the presence of an odorless fluid with the characteristic milky appearance of the fluid (except when the patients were fasting in the immediate postoperative period), a triglyceride level greater than 110 mg/dL or between 50 and 110 mg/dL with a pleural fluid white cell count greater than 1000, and more than 80% lymphocytes on differential when the pleural fluid was not chylous. Results: Over a 37-month period, 435 (mean age = 51.6 months; 232 males) patients underwent various types of surgical procedures for CHD; 6 patients developed chylothorax after the Fontan operation; one patient died due to severe chylothorax; 3 patients were managed by nutritional modifications, diuretics, and thoracocentesis; and 2 patients required thoracic duct ligation. The Fisher exact test analysis showed a significant association between the Fontan operation and postoperative chylothorax (p value < 0.0001). Conclusion: Our study showed a significant association between the Fontan surgery and chylothorax. [ABSTRACT FROM AUTHOR]

Objective: Permanent congenital hearing loss, a common congenital anomaly, may affect speech and language acquisition, academic achievement and social development. Current standards emphasize early recognition of congenital hearing loss. This study was conducted to find the prevalence of hearing impairment in term newborns in Yazd, Iran. Methods: This was a descriptive-analytic study conducted in Yazd on 7250 term newborns. Otoacoustic emissions (OAEs) test was performed in all newborns during the first 24 hours after birth. Those who failed to respond at the first step were retested 15 days later. Those who failed to respond at the second step too, were tested by acoustic brainstem responses (ABR) test. Chi square test was used for data analysis. Findings: From 7250 newborns in the first step 598 (8.2%) and 682 (9.4%) ears (right and left, respectively) failed. In the second step 51 (0.7%) and 58 (0.8%) ears (right and left, respectively) failed. Consanguinity and route of delivery had significant effect on the frequency of hearing loss. Conclusion: The overall frequency of congenital hearing loss in this study was found high. [ABSTRACT FROM AUTHOR]

Introduction. Congenital nephrotic syndrome (CNS), an uncommon form of kidney disease, presents during the first year of life and is a diagnostic and therapeutic challenge for the pediatricians as well as pediatric nephrologists. Our study is the first study of Iranian children with CNS in two pediatric nephrology centers in Tehran, Iran. Materials and Methods. We reviewed medical charts of 30 infants diagnosed with CNS from 1990 to 2005. Results. There were 15 boys and 15 girls with CNS (mean age, 1.7 months). The presentation of the disease was nephrotic syndrome in 96.6% of the patients. Eighty percent of the patients presented within 3 months of life and 16 in the neonatal period. The Finnish type of CNS was seen in 43.3% and diffuse mesangial sclerosis in 50%. Preterm labor and low birth weight was seen in 20%. A family history of nephrotic syndrome in infancy was noted for 8 children (26.7%). Numerous complications of nephrotic syndrome occurred in 73.3%. Seventy percent of the patients had 27 episodes of infections. Sepsis was seen in 43.3% of the children, of which 61.5% were caused by gram-negative bacteria and 38.6% were caused by Staphylococcus aureus. Thrombotic complications and hypertension developed in 6.6% and 23.3% of the patients, respectively. The mortality rate of patients was 86.6%. Conclusions. Diffuse mesangial sclerosis is an important cause of CNS. The outcome of our patients was poor and most of our patients died before reaching the age of 5 years old. [ABSTRACT FROM AUTHOR]

Background & objectives: Measles and Rubella Control Campaign was conducted in Iran in December 2003 targeting both males and females 5 to 25 yr old using measles-rubella vaccine. During the campaign, some pregnant women received vaccine during the first trimester of pregnancy or some others became pregnant shortly thereafter. The goal of this study was to evaluate the risk of congenital rubella syndrome (CRS) among the infants born to the vaccinated mothers. Methods: A total of 106 pregnant women, who had received vaccine during the first trimester of pregnancy or become pregnant less than three months after vaccination were included in the study for comparison 40 pregnant women without rubella vaccine were also included. The mothers' blood samples at the time of delivery, infants' cord blood and blood samples at the end of the second month of birth of sixty, children whose parents agreed about blood sampling, were tested for rubella IgM and IgG antibodies using ELISA method. Results: There were 107 live births in the exposed group and 42 in the control group. Serological study showed no IgM rubella antibody in the maternal and infant cord blood; it was not found in the second blood specimens of 60 infants tested at 8 wk of age. IgG rubella antibody was positive in all infants' cord blood but it decreased in the second blood specimens of the infants. None of the children exhibited signs of congenital rubella syndrome. Conclusions: Finding of our study showed that none of the infants born to mothers vaccinated by MR vaccine during the first trimester of pregnancy or had become pregnant within three months after vaccination, had CRS. [ABSTRACT FROM AUTHOR]

Tremor is a continuous, repetitive twitching of skeletal muscles, which is usually visible and palpable. Repeated birth of lambs with neurological disorders in a herd of the region in Northwest of Kerman province of Iran was the reason for careful examination one of the patients. The 1-day-old lamb with slowly progressive neurologic disturbances consisting of difficulties in moving and coordination was transferred to the Veterinary Clinic of Shahid Bahonar University of Kerman. The patient suffered from ataxia and severe tremor. In clinical examination, significant findings were restricted to the nervous system. The animal was normal in other respects. At necropsy the meningeal blood vessels were congested and occasional small foci of hemorrhage was seen. Microscopic findings in the brain included ischemic cell change, laminar necrosis, demyelination, diffuse and focal gliosis, microcavitation, and hemorrhage. Based on the history, clinical, hematological, biochemical, and histopathologic findings, a severe degenerative process caused the tremor. This is the first report of congenital tremor in an Iranian Fat-Tailed lamb. [ABSTRACT FROM AUTHOR]

A 4-day-old female Iranian cross-breed calf with a history of weakness, ataxia, difficulty in moving, and congenital abnormal position of forelimbs was referred to the Veterinary Clinic of Shahid Bahonar University of Kerman. In clinical examination weakness, ataxia, difficulty in moving, and carpus valgus deformity were observed. Vital signs, hematological indices, and serum concentrations of electrolytes, minerals, total protein, albumin, and trace elements were normal, but serum concentration of manganese was low. Because of carpus valgus deformity and low level of serum concentration of manganese, congenital manganese deficiency was suspected. Manganese sulfate was administered orally at a dose rate of 50 mg/day for 2 weeks. After the treatment, all clinical signs subsided. Radiography of forelimbs after treatment revealed no abnormality. Carpus valgus due to manganese deficiency has not been reported previously, and this is first report in veterinary literature. In the present report, low serum concentration of manganese and response to manganese supplementation confirmed that the cause of carpus valgus was manganese deficiency. The pregnant cow (the mother of the present calf) had low serum concentration of manganese. She was fed on corn silage, which was low in manganese during pregnancy. The cause of carpus valgus is believed to be maternal manganese deficiency. [ABSTRACT FROM AUTHOR]

Objective: Surgical repair and closure of myelomeningocele (MMC) defects are important and vital, as the mortality rate is as high as 65%-70% in untreated patients. Closure of large MMC defects is challenging for pediatric neurosurgeons and plastic surgeons. The aim of the current study is to report the operative characteristics and outcome of a series of Iranian patients with large MMC defects utilizing the V-Y flap and with latissimus dorsi or gluteal muscle advancement., Methods: This comparative study was conducted during a 4-year period from September 2013 to October 2017 in the pediatric neurosurgery department of Shiraz Namazi Hospital, Southern Iran. The authors included 24 patients with large MMC defects who underwent surgery utilizing the bilateral V-Y flap and latissimus dorsi and gluteal muscle advancement. They also retrospectively included 19 patients with similar age, sex, and defect size who underwent surgery using the primary or delayed closure techniques at their center. At least 2 years of follow-up was conducted. The frequency of leakage, necrosis, dehiscence, systemic infection (sepsis, pneumonia), need for ventriculoperitoneal shunt insertion, and mortality was compared between the 2 groups., Results: The bilateral V-Y flap with muscle advancement was associated with a significantly longer operative duration (p < 0.001) than the primary closure group. Those undergoing bilateral V-Y flaps with muscle advancement had significantly lower rates of surgical site infection (p = 0.038), wound dehiscence (p = 0.013), and postoperative CSF leakage (p = 0.030) than those undergoing primary repair. The bilateral V-Y flap with muscle advancement was also associated with a lower mortality rate (p = 0.038; OR 5.09 [95% CI 1.12-23.1]) than primary closure. In patients undergoing bilateral V-Y flap and muscle advancement, a longer operative duration was significantly associated with mortality (p = 0.008). In addition, surgical site infection (p = 0.032), wound dehiscence (p = 0.011), and postoperative leakage (p = 0.011) were predictors of mortality. Neonatal sepsis (p = 0.002) and postoperative NEC (p = 0.011) were among other predictors of mortality in this group., Conclusions: The bilateral V-Y flap with latissimus dorsi or gluteal advancement is a safe and effective surgical approach for covering large MMC defects and is associated with lower rates of surgical site infection, dehiscence, CSF leakage, and mortality. Further studies are required to elucidate the long-term outcomes.