alireza tavasoli
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Objectives
Acute Flaccid Paralysis (AFP) in children can stem from a diverse array of potential diagnoses.
Materials & MethodsThis retrospective study sought to diagnose children referred to a referral pediatric emergency unit with AFP between 2011 and 2016. The study gathered clinical observations, conducted stool and cerebrospinal fluid analyses, and assessed electrophysiological and imaging data.
ResultsThe present study enrolled 118 fully immunized children with a mean age of 6.09 ± 3.60 years. The most prevalent diagnoses included Guillain-Barré Syndrome (GBS-80 cases), acute viral myositis (20 cases), Transverse Myelitis Syndrome (TMS) (TMS-6 cases), and Vaccine-Associated Paralytic Poliomyelitis (VAPP) (VAPP-6 cases). All these six patients had primary immunodeficiency. Notably, all patients tested negative for poliovirus in stool analyses. This study encountered a unique case of a 2.5-monthold male patient who presented with acute limb motor weakness, along with fever, irritability, new-onset hypotonia, and generalized decreased deep tendon reflexes. Notably, no signs of upper motor neuron involvement were found. The Cerebrospinal Fluid (CSF) analysis was compatible with the diagnosis of viral meningitis. Moreover, among the 60 brain and spinal imaging series performed, five were indicative of GBS, six cases showed evidence of TMS, and one revealed a spinal mass. Besides, clinical investigations pointed toward acute viral myositis as a secondary etiology of AFP in 20 patients in this study.
ConclusionIn this hospital-based study, the most frequent diagnoses for children arriving at a third-level pediatric Emergency Room (ER) with acute flaccid paralysis AFP were GBS, acute viral myositis, TMS, and VAPP). These findings suggest a distinct pattern of AFP causes compared to those found in community-based epidemiological studies. Additionally, notably, unusual conditions, such as viral meningitis, can rarely present with AFP-like symptoms. Assessment for primary immune deficiency should be considered in cases of VAPP. Lastly, this research has implemented a pediatric AFP Management Protocol: A Local Practical Approach
Keywords: Acute Flaccid Paralysis, Guillain–Barre´ Syndrome (GBS), Acute Transverse Myelitis -
Background
Nowadays, it is generally accepted that focusing on in-service training can lead to organizational progress and improvements in the training process.
ObjectivesConsidering the high prevalence of epilepsy in children and the use of new technologies in education, the purpose of this study was to conduct an electronic educational course on epilepsy classification guidelines based on the guidelines of pediatric neurologists and to evaluate the effectiveness of the course using the Kirkpatrick model.
MethodsThis study was conducted in 2020. The study population comprised pediatric neurologists from universities of medical sciences throughout the country. Inclusion criteria included participation in the course during the semester, availability, and willingness to participate in the study. Participants entered the study according to their personal preference and provided informed consent, and those who were absent from two or more sessions related to these topics were excluded from the study. The study involved participation in the e-learning course on epilepsy classification according to the guidelines (ILAE 2017). The sample size was calculated using Cochran's formula, which resulted in 74 participants. Nineteen of them did not meet the inclusion criteria, and a total of 55 physicians were included in the study. Reaction and learning were the levels considered to evaluate the efficacy of the Kirkpatrick model. To analyze the data, paired t -test and linear regression were used at a significance level of 0.05, using SPSS version 22 statistical software.
ResultsThe results showed that most participants were satisfied with the e-course. The mean learning and behavior level score was 27.51. The findings indicated that the e-learning of epilepsy classification guidelines according to the guidelines (ILAE 2017) improved the performance of pediatric neurologists (P-value ˂ 0.001).
ConclusionsThe results indicate that e-learning guidelines for the classification of epilepsy according to the guidelines (ILAE 2017) can be an effective tool in improving the clinical performance of pediatric neurologists. Applying newer methods such as the Kirkpatrick model would be an effective approach in other fields of health as well. It is recommended to use up-to-date and efficient models, such as the Kirkpatrick model, in the field of health sciences.
Keywords: Effectiveness, Kirkpatrick Model, Guideline, Pediatric Neurologist, E-Learning -
Evaluation of the effectiveness of risperidone in the treatment of breath holding spells in childrenObjectives
Breath holding spells (BHS) are a type of syncope in children that is commonly seen in the first years of life. Although these attacks do not cause serious damage to the child's brain, in severe or
repeated cases, they expose the brain to hypoxia and cause a lot of stress in parents. In these cases, the clinician should consider therapy. The purpose of this study is to investigate the effectiveness of Risperidone in the treatment of BHS in children visiting the neurology clinic of the Children's Medical Center Hospital.Materials & MethodsIn this randomized clinical trial, the statistical population included patients with the history of Breath Holding spells grades 2 to 3 (after ruling out of seizure disorders) over one year old, visiting the neurology clinic of Tehran Children's Medical Center in 2019. The subjects were randomly selected from patients visiting the clinic using a table of random numbers from the admit ion list. After providing the necessary explanations and obtaining informed consent from their parents, they were treated with low-dose Risperidone (maximum 1 mg) for three months. The patients were observed for three months in terms of frequency and severity of spells monthly.
ResultsIn this study, the median (25th and 75th percentile) age of subjects was 2.3 (2.0-2.6) years. Both the number and severity of seizures in the subjects had a statistically significant decrease in the period after treatment with Risperidone (P<0.001). The studied boys and girls experienced statistically significant decrease in terms of both the frequency of spells (P-value of 0.002 and 0.039) and intensity of seizures (P=0.016) and P=0.008), respectively after treatment with Risperidone. Also, in the studied children under 2 years old and over 2 years old, both the frequency (P=0.021 and P=0.004) and intensity of spells (P=0.008) 0.016 for subjects under and over 2 years, respectively) had a statistically significant decrease after treatment with risperidone compared to pretreatment.
ConclusionAccording to the results of this study, it seems that both the frequency and the intensity of spells in children (regardless of gender and age group), experienced a statistically significant decrease after treatment with Risperidone. We suggest conducting a more comprehensive study considering a larger sample size in order to estimate this issue more correctly.
Keywords: Breath holding spells, grade of BHS, Risperidone, gender, children -
Background
Limb-girdle muscular dystrophy (LGMD) is a bothersome muscle disease associated with weakness of the shoulder and pelvic girdle.
ObjectivesThe study aimed to determine the genetic diversity and relative frequency of various forms of LGMDin Iranian children.
MethodsIn this descriptive research, 60 children referred to the neurology or emergency department of the Pediatric Medical Center were studied from April 2019 to April 2020. Additional tests (muscle biopsy and genetic testing) were performed to confirm the diagnosis of LGMDs. Quantitative data such as disease level, motor, respiratory, and cardiac functions, and molecular data underwent statistical analysis.
ResultsA total of 41 patients with a mean age of 11.1 were studied. Twenty-two patients were diagnosed with genetic tests and 19 with muscle biopsies. Also, there were 26.8% cases of alpha sarcoglycanopathy, 24.4% beta sarcoglycanopathy, 17.1% gamma sarcoglycanopathy, 7.3% calpainopathy, 7.3% dysferlinopathy, 7.3% dystroglycanopathy, 7.3% titinopathy, and one case of laminopathy. Among genetically confirmed individuals, 27.3% had SGCB mutation, and 18.2% had SGCA mutation. A significant relationship was seen between the mutation type and creatine phosphokinase (CPK) levels (P < 0.05).
ConclusionsThe prevalence of alpha and beta sarcoglycanopathy phenotypes in the study population showed that the severity of clinical involvement may be predicted by SGCB gene mutation and sarcoglycan expression.
Keywords: Clinical Characteristics, Limb-Girdle Muscular Dystrophy, LGMD, Sarcoglycanopathy -
Background
The normal values of nerve conduction studies (NCS) are different in children compared to adults. Moreover, racial and geographical factors can affect these values.
ObjectivesThe present study aimed to investigate the normal NCS values in children of different ages.
MethodsThe present cross-sectional study included children referred to the Electrodiagnosis Clinic of the Children’s Medical Center in Iran, who had normal NCS results based on the references and had no exclusion criteria. The patients were divided into 8 age groups (7 days to one month, 1 - 3 months, 3 - 6 months, 6 - 12 months, 1 - 2 years, 2 - 4 years, 4 - 6 years, and 6 - 14 years), and the NCS was performed using a Nihon Kohden electromyogram. The NCS values in the normal range were included in the analysis.
ResultsThe normal ranges of amplitude and conduction velocity of 4 motor nerves (median, ulnar, deep peroneal, and tibial) and 2 sensory nerves (median and medial plantar), as well as the F-wave latency values of 2 nerves (ulnar and tibial), were established as the reference values for the pediatric patients.
ConclusionsThe NCS parameters of Iranian children were slightly different from the normal references reported by studies in other countries. Moreover, the SNAP and CMAP amplitudes and motor conduction velocity of these children usually reached the normal values of the adult population earlier.
Keywords: Electrodiagnosis, EMG-NCV, Pediatrics Neuromuscular Disorders, Neurophysiology, Nerve Conduction Study, NormalValues -
Background
COVID-19 infection and its neurological manifestations were seen in children although less common than adults. The aim of this study was to determine the frequency of different types of neurologic findings of hospitalized children with COVID-19. ]
MethodsThis retrospective study was performed on hospitalized pediatric patients aged≤18 years with confirmed SARS-CoV-2 at Children’s Medical Center Hospital. Neurological manifestations were defined as the presence of any of the following symptoms: seizure, altered mental status, behavioral/personality change, ataxia, stroke, muscle weakness, smell and taste dysfunctions, and focal neurological disorders.
ResultsFifty-four children with COVID-19 were admitted and their mean age was 6.94±4.06 years. Thirty-four of them (63%) were male. The most frequent neurological manifestation was seizure (19 [45%]) followed by muscle weakness (11 [26%]), loss of consciousness (10 [23%]), and focal neurological disorders (10 [23%]). Other neurological manifestations consisted of headache (n=7), movement disorders (n=6), behavioral/personality change (n=5), ataxia (n=3), and stroke (n=3). Twenty-nine percent of our patients had leukocytosis. A neutrophil count above 70% was seen in 31% of participants. Among our patients, 81% had a positive reverse-transcription polymerase chain reaction (RT-PCR) test for SARS-CoV-2.
ConclusionDuring the current pandemic outbreak, hospitalized children with COVID-19 should be evaluated for neurological signs because it is common among them and should not be under-estimated.
Keywords: Children, COVID-19, Neurological manifestations, SARS-CoV-2 -
Tumor necrosis factor-alpha-blockers in rheumatology have revolutionized the treatment of rheumatoid arthritis. However, they can increase the risk of latent tuberculosis reactivation. This risk is evaluated by tuberculosis skin tests and chest radiography in many nations such as Iran. The interferon-gamma release assay test is a secondary test that is used to rule out the potential risk of latent tuberculosis reactivation but is not mandatory. In this report, we aim to present a 67-year-old rheumatoid arthritis female patient who was on Tumor necrosis factor-alpha-blockers (Adalimumab) and manifested severe symptoms of tuberculosis infection in our service 8 months after taking the initial treatment. The tuberculosis skin test and chest radiography were negative at the initiation of remedy in this case. The patient was cured after a timely diagnosis and receiving treatment for tuberculosis. Mandatory double-checking with tuberculosis skin test and interferon-gamma release assays test is recommended before tumor necrosis factor-alpha-blocker prescription in rheumatoid arthritis patients to prevent the risk of fatal tuberculosis reactivation.
Keywords: Rheumatoid arthritis, anti-tumor necrosis factor-alpha blocker, tuberculosis, interferon-gamma -
زمینه و هدف
بخش عمدهی زندگی هر فرد، صرف اشتغال به کار میشود. فرسودگی شغلی یک نشانهی روان شناختی، پاسخ ممتد به فشارهای مزمن فردی و عاطفی موجود در محیط کار است. رزیدنتها به علل متعدد در معرض فرسودگی شغلی قرار دارند که سبب کاهش در کیفیت ارایه خدمات میشود.
روش بررسیاین مطالعه توصیفی- تحلیلی مقطعی، بر روی 57 نفر از رزیدنتهای کودکان در دانشگاه تهران انجام شد. پرسشنامهی فارسی شدهی ماسالاچ جهت تعیین میزان و شدت فرسودگی شغلی مورد استفاده قرار گرفت. جهت تجزیه و تحلیل دادهها ابتدا با استفاده از روشهای مبتنی بر آمار توصیفی شامل میانگین ± انحراف معیار مقادیر نمرات فرسودگی شغلی استفاده شد.
یافته هامیانگین شدت فرسودگی شغلی رزیدنتهای کودکان 55.45 هم چنین میانگین فراوانی فرسودگی شغلی رزیدنتهای کودکان 62.1 میباشد. در این مطالعه میان شدت و فراوانی فرسودگی شغلی با سن، وضعیت تاهل، سابقهی کاری ارتباط معنیداری یافت نشد. ولی بین شدت فرسودگی عاطفی با تعداد روز کار در هفته ارتباط معنیداری یافت شد. بین فراوانی فرسودگی عاطفی با محل سکونت خانواده ارتباط معنیدار یافت شد. بین شدت تهی شدن از ویژگیهای شخصیتی و داشتن فرزند نیز ارتباط معنیدار یافت شد.
نتیجه گیریبا توجه به نتایج این مطالعه، فرسودگی شغلی در رزیدنتهای کودکان در دانشگاه تهران در عدم موفقیت فردی از لحاظ فراوانی شدید و از لحاظ شدت میزان متوسط را نشان داده، در حالی که این متغیر در ابعاد فرسودگی عاطفی شدت کم و فراوانی متوسط و مسخ شخصیت شدت کم و فراوانی کم را گزارش کرده است. پیشنهاد میشود که تعدیل روزهای کاری هفتگی رزیدنتها با افزایش جذب رزیدنت ها و انتخاب دانشگاه محل تحصیل رزیدنت نزدیک خانواده خود در اولویت انتخاب رشته-محل قرار گیرد.
کلید واژگان: رزیدنت کودکان, فرسودگی شغلی, رضایت فردیBackground and ObjectiveMost of a person's life is spent in employment. Today, we see a trend and special attention to the issue of job burnout all over the world. Burnout is a psychological symptom, an ongoing response to chronic personal and emotional stress in the workplace. Pediatric resident physicians are exposed to job burnout for various reasons, which reduces the quality of service delivery. Therefore, due to the importance of paying attention to the issue of job burnout and also the lack of a studies in this field in the community of pediatric resident physicians of Tehran University of Medical Sciences, this study was conducted with the aim of investigating the job burnout of pediatric resident physicians of Tehran University of Medical Sciences based on Maslach questionnaire in 2020-2021.
Materials and MethodsThis descriptive-analytical cross-sectional study was conducted on 57 active in Pediatric resident physicians of Tehran University of Medical Sciences. To determine the level and intensity of job burnout, the Persianized Maslach questionnaire was used. To analyze the data, first descriptive statistics were used, including the mean standard deviation of burnout scores and their severity, and for comparison, a two-sample t-test or Mann-Whitney test was used. SPSS 26 software was used for data analysis.
ResultsThe average intensity of job burnout in pediatric resident physicians of Tehran University of Medical Sciences is 55.45 and also the mean frequency of job burnout in pediatric resident physicians of Tehran University of Medical Sciences is 62.1. In this study, there was no significant difference between the severity and frequency of job burnout with age, marital status, and work experience. However, a significant relationship was observed between the intensity of emotional burnout and the number of working days per week. A significant relationship was observed between the frequency of emotional burnout and living in a family. A significant relationship was observed between the severity of personality disorder and having children.
ConclusionBased on the results of this study, job burnout among pediatric resident physicians of Tehran University of Medical Sciences showed the level of severe personal failure in terms of frequency and in average level, while this variable in the dimensions of emotional exhaustion has low intensity and moderate frequency, and depersonalization has low intensity and frequency.
Keywords: Pediatric resident, Burnout professional, personal satisfaction -
Objectives
Infantile spasm is an epileptic disorder of early childhood and infancy and is characterized by cluster epileptic spasms and abnormal EEG findings. Developmental delay is prevalent. Some studies have indicated the significant effect of the Ketogenic Diet (KD) on intractable spasms in children who are unresponsive to first-line treatments. It has been used successfully as a first-line treatment with fewer side effects than ACTH.
Materials & MethodsThis was an interventional study in which the effectiveness of KD over a six-month period was evaluated in patients with infantile spasms. Those who fulfilled the inclusion criteria and were willing to use the diet received free cans of the 4:1 ketogenic formula. The diet was prescribed based on the Johns Hopkins protocol in the outpatient setting. All patients used a full formula diet for one month. After a month, the patients were examined by a neurologist and a dietitian, and an EEG was obtained to compare pre- and post-KD findings. In order to compare pre- and post-KD seizures, the maximum number of seizures was multiplied by the longest duration of seizures
ResultsTen patients were assessed for one month. Using the KD led to significant changes in seizures/clusters and EEG findings. Nine parents reported improvement in their children’s social interactionsafter using the KD.
ConclusionBased on the findings of this study, the KD can control seizures in patients suffering from infantile spasms by reducing seizure frequency & duration and improving EEG findings
Keywords: infantile spasm, ketogenic diet, refractory seizure -
Background and objectives
Neuro-developmental delay (NDD) is a chronic neurologic problem that adversely affects the quality of life of affected individuated. The aim of this study was to compare magnetic resonance imaging (MRI) findings in patients with global developmental delay (GDD) and Isolated NDD and evaluate their efficacy in diagnostic approach to children with NDD.
MethodsA cross-sectional study was designed to enroll all patients with static developmental delay who had been referred to outpatient neurology clinic at Bahrami hospital, Tehran, Iran, between Feb 2012-Feb 2013. Neurologic examination results and the presence of GDD or Isolated NDD in patients were recorded using of a pre-prepared questionnaire, Bayley Scales for Infants and Toddler-4th edition and Denver Developmental Screening Test.
ResultsTotally 140 patients with developmental delay were enrolled during the study. 70 patients (50%) had global developmental delay (GDD), and 70 cases (50%) had Isolated NDD. In addition, abnormal associated neurological symptoms were present in 67.9% of patients. Patients whit GDD had significantly probability of abnormal MRI compare to patients with isolated NDD (P<0.001). Furthermore, a great proportion of positive MRI findings was reported from patients with Isolated motor NDD (P=0.007). Microcephaly was the most abnormal neurologic finding in patients with GDD (P=0.04).
ConclusionA significant proportion of abnormal MRI findings in patients with global developmental delay, confirms the necessity of imaging diagnostic tool, especially brain MRI, in diagnostic approach to patients with GDD. In addition, the benefit of brain MRI in diagnostic approach to children with isolated NDD seems to be low.
Keywords: Global Developmental Delay, Isolated developmental delay, Magnetic Resonance Imaging, Pediatric -
Background
Although the relationship between childhood trauma and fear of happiness (FOH) is well-documented, little attention has been paid to the mediating factors of this relationship.
ObjectivesThis research aimed to investigate the mediating role of cognitive emotion regulation in the relationship between childhood trauma and FOH.
MethodsThree hundred and seventy-eight students participated in this descriptive correlational study. The participants were selected by the convenience sampling method among all the students of the Islamic Azad University of Kashmar during 2017 - 2018. Data were collected by the Cognitive Emotion Regulation questionnaire (CERQ), the Childhood Trauma questionnaire (CTQ), and Fear of Happiness scale (FHS). All data were analyzed using Structural equation modeling (SEM).
ResultsThe relationship between childhood trauma and FOH is mediated by cognitive emotion regulation. The fitness rates of the indices of GFI, CFI, NFI, IFI, NNFI, AGFI, RFI, and RMSEA were equal to 0.90, 0.96, 0.94, 0.96, 0.95, 0.87, 0.93, and 0.074, respectively.
ConclusionsThe findings of this study supported the proposed conceptual model in which the relationship between childhood trauma and FOH was mediated through positive and negative cognitive emotion regulation strategies. Therefore, it is necessary for psychologists and other specialists to pay attention to these variables.
Keywords: Childhood Trauma, Emotional Regulation, Happiness -
زمینه و هدف
بروملین ماده ای است که از آناناس به دست می آید و دارای اثرات آنتی اکسیدانی، ضد التهابی و ضد دردی می باشد. این مطالعه با هدف بررسی تاثیر بروملین بر روند ترمیم حفره دندان کشیده شده در مدل حیوانی صورت گرفت.
روش تحقیقاین مطالعه تجربی بر روی 24 سر موش صحرایی نر انجام شد. پس از بیهوشی، با سوزن گیر، دندان مولر اول بالای یک طرف همه موش ها با حداقل آسیب خارج شد. موش ها به صورت تصادفی به دو گروه کنترل و بروملین تقسیم شدند و سپس در گروه بروملین، بروملین به میزان 500 میلی گرم که با آب به صورت محلول درآمده بود، دو بار در روز به مدت دو روز به موش ها گاواژ شد. در گروه کنترل دارویی به موش ها داده نشد. در هر یک از روزهای سوم و دهم پس از جراحی شش موش از هر گروه کشته شد. سپس فک بالای موش ها جدا شد و از حفره دندانی آن ها لام تهیه گردید. لام ها توسط میکروسکوپ نوری از نظر متغیرهای هیستوپاتولوژی (میانگین ماکروفاژها، فیبروبلاست ها، لنفوسیت ها، نوتروفیل ها؛ وسعت بافت گرانولاسیون و میزان آنژیوژنز و سلول های استخوانی) مورد بررسی قرار گرفتند.
یافته هانتایج نشان داد، در روز سوم و دهم میزان آنژیوژنز (05/0<P) و وسعت بافت گرانولاسیون (05/0<P) در گروه بروملین به طور معناداری (05/0<P) نسبت به گروه کنترل افزایش داشت. همچنین تجویز بروملین باعث افزایش معنی دار تعداد فیبروبلاست ها (01/0<P)، وسعت بافت گرانولاسیون (05/0<P) و میزان آنژیوژنز (05/0<P) روز دهم نسبت به روز سوم مطالعه می شود.
نتیجه گیرینتایج مطالعه حاضر این احتمال را نشان داد که بروملین روند ترمیم زخم بعد از کشیدن دندان را تسریع می بخشد.
کلید واژگان: بروملین, حفره دندان, کشیدن دندانBackground and AimsBromelain is a substance derived from pineapple and has antioxidant, anti-inflammatory, and analgesic effects. This study aimed to investigate the effect of bromelain on the healing of tooth extraction sockets in an animal model.
Materials and MethodsThis experimental study was performed on 24 male rats. After anesthesia, the first maxillary molar tooth was extracted with minimal damage using a hemostat. The rats were randomly divided into two groups, namely control and bromelain. In the bromelain group, the rats were orally administered by gavage with a 500 mg dose of bromelain that was dissolved in water twice a day for 2 days, while the control group received no medications. On the 3rd and 10th days after the surgery, 6 rats were killed per group each day. Afterward, the maxillae of rats were removed and slides were prepared from their dental sockets. The slides were examined by light microscope for histopathological variables (mean of macrophages, fibroblasts, lymphocytes, neutrophils; granulation tissue extent and angiogenesis and bone cells).
ResultsThe results showed that the angiogenesis and granulation tissue extent increased significantly in the bromelain group, compared to the control group, on the 3rd day (P<0.05). Also, bromelain administration significantly increase the the number of fibroblasts (P<0.01), granulation tissue extent (P<0.05) and angiogenesis (P<0.05) in 3rd day of the experiment in comparison with the 10th day.
ConclusionThe results of the present study showed that bromelain accelerates the process of wound healing after tooth extraction.
Keywords: Bromelain, Extraction, Socket -
Background
Despite the great progress in the surgery of complex congenital heart diseases, there is still concern regarding adverse neurological events. We aimed to determine the pre- and on-pump modifiable factors that could predict the neurocomplications after pediatric cardiac surgery.
MethodsIn a prospective study, modifiable factors such as arterial blood gas, serum lactate, serum glucose, mean arterial pressure, and nasopharyngeal temperature were measured before and during cardiopulmonary bypass (CPB). Moreover, the CPB time, the aortic cross-clamp time, and the deep hypothermic circulatory arrest time were recorded. If there were adverse neurological complications, brain computed tomography scan was done.
Results435 patients with congenital heart diseases that underwent cardiac surgery, 364 patients at a mean age of 22 months were enrolled in the study. Thirty-three (9%) patients had adverse early-onset neurological events. Seizure and hemorrhage were the most common clinical and neuroimaging findings, respectively. Although the pre-pump oxygen saturation (P = 0.03), the blood CO2 level (P = 0.04), and the serum glucose level (P = 0.03) showed statistical significance in the univariate analysis, the only predictive variables of neurocomplications in the multivariate analysis of logistic regression were the on-pump serum glucose level (P = 0.001) and the nasopharyngeal temperature (P = 0.004).
ConclusionsAmong several modifiable factors exerting an influence on the neurological outcome of children undergoing cardiac surgery, special attention should be paid to the control of the intraoperative serum glucose level and the provision of the optimal cooling temperature. (Iranian Heart Journal 2020; 21(2): 48-56)
Keywords: Neurocomplications, Cardiac surgery, Cardiopulmonary bypass, Deep hypothermic circulatory arrest -
ObjectiveOne of the difficulties for conduct electroencephalography (EEG) in pediatric patient population is that they are not always cooperative during the procedure. Different medications have been used to induce sedation during EEG recording. In order to find a medication with least adverse effects and high efficacy, we aimed to compare clonidine and chloral hydrate as a premedication prior EEG performing in pediatric population. Materials & MethodsA prospective, randomized, single-blinded, controlled trial was carried out over 198 children (9 to 156 months) to investigate the sedative and adverse effects of clonidine and chloral hydrate. Patients, partially sleep-deprived the night before, were randomly divided in two groups of clonidine (100 patients) and chloral hydrate (98 patients), on an alternative day basis.Results The average sleep onset latency was significantly longer in the clonidine group than chloral hydrate group (Mann-Whitney test, p < 0.0001). Sleep duration ranged between 15-150 minutes and it was not significantly different between two groups (Mann-Whitney test p = 0.2). Drowsiness with chloral hydrate terminated faster than with clonidine. Drowsiness after arousal was seen in 58% and 26.1% of patients in the clonidine and chloral hydrate groups respectively that was significant (Mann-Whitney test, p = 0.058). EEG results were reported normal in 77 subjects in the chloral hydrate group (77%) and in 69 subjects (69%) in the clonidine group (p = 0.161). Generalized epileptiform discharges reported significantly in the clonidine group (Mann-Whitney test , p = 0.006).ConclusionThe results of this study showed that both chloral hydrate 5% (one ml/kg)and clonidine (4 μg/kg)could be administered as a pre medication agent for EEG recording in children , although drowsiness after arousal of clonidine is greater than chloral hydrate . However, the yield of generalized epileptiform discharges in the clonidine group was more than the chloral hydrate group.
Keywords: Premedication, Chloral hydrate, Clonidine, Electroencephalography (EEG), Sedation, Sleep -
Background
The purpose of treatment of children with autism is to help them to acquire functional skills in routine life and to control behaviors that are disruptive.
ObjectivesIn this study, the safety and efficiency of cerebrolysin was determined by childhood autism rating scale (CARS) on behavioral, verbal and nonverbal developments of children with autism referred to children’s medical hospital clinic of neurology.
MethodsIn a quasi-experimental study, 36 children with autism were enrolled and the effect of cerebrolysin on their behavioral, nonverbal and verbal development was determined by CARS questionnaire fulfilled before and one month after complete intervention.
ResultsAll evaluated items except level and consistency of intellectual response had favorable reduction in scores of CARS questionnaire (P = 0.001). The total score decreased from 40.6 to 36.1 showing 11.1% improvement. Despite the efficiency of cerebrolysin observed in this study, its partial use may be limited by the large number of intramuscular injections, i.e. nine per months. Sustained release formulation may alleviate this limitation, if this therapy proves to be beneficial in future studies.
ConclusionsAccording to the obtained results, the safety and feasibility of cerebrolysin administration could be considered and be effective on behavioral, nonverbal and verbal development in children with autism.
Keywords: Cerebrolysin, Behavioral, Nonverbal Verbal Development, Autism, Children -
Megalencephalic leukoencephalopathy with subcortical cysts (MLC), a demyelination leukodystrophy, is usually characterized by early-onset macrocephaly and gradually progressive motor and mild mental deterioration. Variable clinical expression has been reported in MLC especially in adult cases. In this study, a multi affected family with variable phenotypes including an asymptomatic adult individual is reported to expand the clinical spectrum of MLC. A seven-year old boy was referred to our hospital due to macrocephaly and gait disturbance. According to brain magnetic resonance imaging (MRI) findings, molecular studies were done to confirm the probable diagnosis of MLC in index case followed by family segregation analysis. A homozygous splice site variant, c.177+1G>T in MLC1 gene was found in proband, his mother and two aunts. Aunts were clinically affected but his mother had no clinical symptoms despite bi-allelic mutation in MILC1. The clinical data and available MRI findings were reviewed for these cases. A comprehensive searchwasconductedonclinical variations of MLC. Phenotypic variability and/or reduced penetrance are important phenomena in MLC.We extended phenotypic variation in MLC by reporting an asymptomatic adult case with a known pathogenic mutation in MLC1 gene.Keywords: MLC, Megalencephalic Leukoencephalopathy, Subcortical Cysts, Splice-Site Mutation
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Multiple sclerosis (MS) is a neurodegenerative disease arising from interactions of both environmental and genetic factors. The SNP rs6897932 is located in IL‐7Ra gene associated with MS susceptibility in some population. In this study, we investigated the possible association of SNP rs6897932 with MS susceptibility in 157 Iranian MS patients and 152 healthy controls. We also studied genotype-dependent severity and response to IFN-β in MS. Unlike some previous studies, our results clearly demonstrate that there are no significant differences in distribution of the SNP rs6897932 in our chosen Iranian MS patients and controls. Furthermore our results show, Interferon beta (IFN-β) therapy over a period of two years demonstrated an IL‐7Ra genotype-dependent therapeutic effect in MS population. Patients carrying TT or TC genotypes gave a better response to IFN-β treatment, whereas patients carrying the homozygous CC genotype were the worst responders to IFN-β treatment. In other words, despite the lack of linkage the therapeutic response to the severity and progression of MS was related to the genotype of SNP rs6897932 presented in our patient. Therefore, in light of our findings reported here, to reach higher certainties, further studies are needed to associate IL-7Ra gene with the pathogenesis of MS.Keywords: Multiple sclerosis, IL-7Ra, rs689793
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WITHDRAWN: Megalencephalic Leukoencephalopathy with Subcortical Cysts: Presentation of an Asymptomatic Patient and Review of Literature
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IntroductionVanishing white matter disease (VWM) is considered as one of the most frequent types of inherited childhood leukoencephalopathies. Various neurological and non-neurological manifestations have been reported in this type of leukodystrophy; however, seizures are rarely described in infantile type of VWM.Case PresentationTo patient is a 12 months old boy who experienced frequent seizures at 4th month of age. The seizures were resistant to anti-epileptic drugs and caused 3 periods of hospitalization. Magnetic resonance imaging (MRI) demonstrated demyelinating pattern and whole exome sequencing (WES) reported homozygous mutation (c.922G > A) in EIF2B2 gene in exon 8 leading to an amino-acid substitution (p.Val308Met).ConclusionsInfantile onset of vanishing white matter disease can be considered as one of few childhood leukodystrophies that are associated with early onset seizures.Keywords: Leukodystrophy, Vanishing White Matter, Seizure, Epilepsy
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BackgroundCerebral Palsy (CP) constitutes a heterogeneous group of developmental disorders resulting from damage to the brain which affects motor system. Cerebrolysin, as a neurotrophic peptide has been used for improving symptoms of patients suffering from neurodegenerative disorders.ObjectivesDue to the presence of limited information about the usefulness of Cerebrolysin in CP, this study was performed for the evaluation of Cerebrolysin in the management of spasticity in children with CP.Methods26 Spastic CP patients aged between 2 and 6 years entered this study. At the first visit, Modified Ashworth scale for assessment of spasticity was used. Then Cerebrolysin was administered at a dose of 0.1 cc/kg intramuscularly (IM) for 3 months. In the first month, the medication was injected 5 times per week. In the second month of therapy, injections were performed as follows: 4 injections in the first week, 3 injections in the second week, 2 injections in the third week and a single one in the fourth week. In the third month of therapy, Cerebrolysin was continued as weekly injections. The spasticity of the patients was evaluated at the end of the first and third month of therapy.ResultsModified Ashworth score at baseline was 20.62 ± 9.65 which reached 15.19 ± 7.30 after 1 month of treatment. This figure was 14.81 ± 3.77 at month 3. Total decline in Modified Ashworth Scale was 5.42 ± 5.33 at the first month and 5.81 ± 2.74 at the third month in comparison to baseline. The difference between Modified Ashworth Scale at baseline and 1 month after therapy was significant (P = 0.000) but there was no significant difference between first month and third month of therapy. (P = 0.755). Also analysis indicates that there was an association between absolute reduction of Modified Ashworth score and the age at beginning of treatment (P = 0.037).ConclusionsUsing Cerebrolysin as a neurotrophic peptide may improve spasticity of children with CP and should be considered as a possible useful treatment in CP cases.Keywords: Cerebrolysin, Cerebral Palsy, Spasticity, Modified Ashworth-Score
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A tumefactive lesion of central nervous system (CNS) is defined as a mass-like lesion with a size greater than 2 cm in brain detected by magnetic resonance imaging (MRI). Neuroimaging may help to distinguish the nature of a tumefactive lesion and therefore, can prevent an unnecessary brain biopsy. Here we emphasized on determining the nature of a CNS tumefactive lesions with the help of MRI and more explanations about demyelinating lesions with focus on Schilder and Balo diseases as two multiple sclerosis variants. We have reported here two boys of 10 and 8 years of age respectively of multiple sclerosis (MS) variants who presented with acute neurologic complications to our hospital as one of the two referral children hospital in Tehran, Iran. Tumefactive demyelinating lesions can be considered a separate entity that itself can contain Schilder disease, Balo disease, some cases of acute disseminated encephalomyelitis (ADEM) or classic MS. MRI can help to establish a diagnosis of a tumefactive lesion and to differentiate among different underlying etiologies.Keywords: Central Nervous System, Demyelination, Tumefactive Lesion, Multiple Sclerosis Variants, Schilder Disease, Balo Disease
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We present a 7 year old boy afflicted with super-refractory seizure that responded poorly to antiepileptic drugs and sustained a long course of hospitalization and complications of high doses of medications as well as longstanding stay in hospital. The differential diagnoses were, fever-induced refractory epileptic encephalopathy (FIRES), and infectious and autoimmune encephalitis.
However, work-ups had not revealed any evidence of any specific diagnosis, so we assumed that he was afflicted by viral infectious encephalitis as he had, fever, vomiting, and prodromal symptoms of infectious (most probably viral) disease prior to onset of the seizure attacks.Keywords: Refractory seizure, Infectious encephalitis, Lateralized seizure, Autoimmune encephalitis -
مقدمهبا توجه به ماهیت بیماری های قلبی و استفاده از بای پس قلبی -ریوی، ممکن است عوارض نورولوژیکی گذرا یا دایمی در طول یا پس از جراحی قلب کودکان بوجود آید. هدف از این مطالعه ارزیابی بروز عوارض نورولوژیکی حاد در دوره پس از عمل جراحی قلب در کودکان با بیماری های مادرزادی قلب تحت عمل جراحی قلب بود.مواد و روش هادر این مطالعه، تمام بیماران با بیماری های قلبی -عروقی (از جمله بیماری های مادرزادی قلب و دیگر بیماری های قلبی -عروقی) که عمل انجام داده اند (باز یا بسته) در مرکز پزشکی کودکان، تهران، ایران، برای یک سال بین تیر 1393 و تیر 1394 مورد ارزیابی قرار گرفت. کسانی که در طی اقامت در بیمارستان یا عمل جراحی مرده بودند، بیماران با اطلاعات ناقص در پرونده های پزشکی و کودکانی که به دلیل بیماری مادرزادی قلب عمل نشده بودند از مطالعه حذف شدند. از 435 بیمار مورد بررسی، 364 بیمار در این مطالعه وارد شدند. عوارض نورولوژیکی حاد و یافته های مرتبط در سی تی اسکن مغز مورد بررسی قرار گرفت.یافته هامحدوده سنی بیماران بین 5 روز تا 15 سال سن بود، که 64 (17/6٪) بیمار کمتر از 2 ماه ، 131 (36٪) بیمار بین 2 ماه تا 1 سال و 169 (46/6٪) بیمار بالای یک سال بودند. سی و سه بیمار (9/06٪) با عوارض نورولوژیکی حاد پس از عمل جراحی قلب، از جمله تشنج، اختلالات حرکتی، از دست دادن هوشیاری، اختلالات بینایی، سردرد، و هیدروسفالی مشخص شدند.نتیجه گیریبهبود اجرای پروتکل بای پس قلبی -ریوی و استفاده از مانیتورینگ های عصبی برای ایسکمی و خونریزی و همچنین فیلتر شریانی در حین عمل جراحی می تواند بروز عوارض نورولوژیکی بعد از عمل جراحی قلب کودکان را کاهش دهد.کلید واژگان: تظاهرات نورولوژیک, جراحی قفسه سینه, نقایص مادر زادی قلبIntroductionAccording to the nature of heart diseases and the use of cardiopulmonary bypass, transient or permanent neurologic complications may arise during or after pediatric cardiac surgery. The aim of this study was to evaluate the incidence of acute neurologic complications in postoperative cardiac surgery period in children with congenital heart diseases (CHD) undergoing cardiac surgery.Materials And MethodsIn this study, all patients with cardiovascular disease (including CHD and other cardiovascular diseases) had been operated (open or closed) in the Childrens Medical Center, Tehran, Iran, were evaluated for a year between July 2014 and July 2015. Those who had died during hospital stay or surgery, patients with incomplete information in their medical records, and children who had not been operated because of CHD were excluded. Of the 435 patients surveyed, 364 patients were enrolled in this study. Acute neurologic complications and related findings in brain CT scan were investigated.ResultsThe age range for patients was between 5 days to 15 years old, of whom 64 (17.6%) patients were under 2 months, 131 (36%) patients were between 2 months to 1 year, and 169 (46.6%) patients were over one year. Thirty-three patients (9.06%) were identified with acute neurological complications after heart surgery, including seizures, movement disorders, loss of consciousness, visual disturbances, headache, and hydrocephalus.ConclusionImproving the implementation of the cardiopulmonary bypass protocols and using neural monitoring for ischemia and bleeding as well as arterial filter during surgery can reduce the incidence of neurological complications after pediatric cardiac surgery.Keywords: Neurologic Manifestations, Thoracic Surgery, Heart Defects, Congenital
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ObjectiveCanavan disease (CD) is a type of vacuolating leukodystrophy with autosomal recessive inheritance. Aspartoacylase deficiency results in decrease of myelin biosynthesis, dysmyelination and brain edema. Although CD is a very common in Ashkenazi Jews patients, several cases have been reported from non- Jewish population. This report is based on a homozygous C.202G>A mutation in the ASPA gene identified from an Iranian patient. To our knowledge, this type of mutation has not been reported in non-Jewish population in the literature.Keywords: Canavan disease, Aspartoacylase deficiency, Aspartoacylase enzyme, ASPA gene
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