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Objectives Hyperinsulinism refers to improper insulin secretion in the presence of low plasma glucose, causing severe and persistent hypoglycemia in infants and children. The brain's occipital lobe, which includes the visual and plays an essential role in visual perception is specifically sensitive to hypoglycemia-induced damage. The present study aims to investigate the visual perception in children suffering from hyperinsulinism and to compare it with the control group. Materials & Methods This cross-sectional control study, conducted in 2020 in Isfahan, Iran, involved 20 children aged 4-13 years with hyperinsulinism and 20 healthy children of the same age and gender for comparison. In both groups, the measuring instrument was the Test of Visual Perceptual Skills (non-motor) Third Edition. Results The mean visual perceptual quotient in the case and control groups was 80.50±26.74 and 116.50±7.56 (p-value<0.001), respectively. The results overall indicated that children suffering from hyperinsulinism were weaker than healthy children in all areas of visual perception. Conclusion Based on the obtained results, it is recommended that children suffering from hyperinsulinism be screened regarding visual perceptual disorders since this screening may be helpful in initiating different rehabilitation programs among these patients. [ABSTRACT FROM AUTHOR]

Infantile neuroaxonal dystrophy (INAD) is a rare recessive neurodegenerative disorder manifested by symptoms like hypotonia, extrapyramidal signs, spastic tetraplegia, vision problems, cerebellar ataxia, cognitive complications, and dementia before the age of three. Various reports evaluated the relationship between the incidence of INAD and different mutations in the PLA2G6 gene. We described cases of two children with INAD whose diagnoses were challenging due to misleading findings and a mutation in the C.2370 T>G (p. Y790X) in the PLA2G6 gene based on NM_001349864, which has been reported previously. [ABSTRACT FROM AUTHOR]

Objective Benign enlargement of the subarachnoid space (BESS) is the most common cause of macrocephaly in infants. This study aimed to evaluate the neurodevelopmental outcomes in infants with BESS. Materials & Methods In this follow-up study, all records of infants diagnosed with BESS in 2012-2016 were assessed. A clinical follow-up examination was carried out at 6, 12, 18, and 24 months of age to assess the macrocephaly outcomes. Denver Developmental Screening Test-II (DDST-II) was used for evaluating the psychomotor development of infants at 24 months of age. All data were entered in SPSS Version 13, and descriptive statistics were measured. Results Out of 32 infants included in this study, 28 (87.5%) were boys. Five cases of prematurity history (15.6%), and 23 cases of macrocephaly in the family (71.9%) were recorded. The mean age of BESS diagnosis was 6.8 months (SD=3.2). subdural hematoma was reported in one infant (3.1%). Also, 28 infants showed macrocephaly at 18 months of age (83.3%). Seven patients had developmental delay, according to DDST-II (22%). The mean head circumference at birth and six months of age was significantly greater in infants with developmental delay compared to those with normal development. There was a significant difference between the mean head circumference at birth (P=0.05) and the mean head circumference at six months of age (P=0.02). Conclusion Developmental delay is frequent in BESS infants, especially those with macrocephaly at birth and six months of age, and requires medical attention. [ABSTRACT FROM AUTHOR]

Introduction: Ramadan, the ninth month of the Islamic lunar calendar, has special religious importance among Muslims. During this month, Muslim adults and adolescents (who have reached the age of adulthood according to the Islamic rules) fast. Gastrointestinal (GI) disorders present with symptoms generally called GI discomforts such as heartburn, abdominal pain, dyspepsia, constipation, bloating, and irritable bowel syndrome. This study aimed to assess the effects of Ramadan fasting on GI discomfort. Methods: One hundred fasting subjects were enrolled in the study. Personal information (age, gender, education level, and occupation) was collected from participants and they were assured that their information would remain confidential. Participants had to fill out a food questionnaire before and in the third week of Ramadan. Food patterns were identified by factor analysis. The relationship between dietary food patterns and GI symptoms was assessed by logistic regression analysis. Result: The results of logistic regression analyses of the association between dietary patterns and the development of GI discomforts in Ramadan represented that adherence to high fat and protein dietary patterns significantly reduced dyspepsia and diarrhea (P<0.05) while adherence to relatively healthy dietary pattern caused a marginally significant reduction in constipation (P<0.1). Conclusion: This study showed that total energy intake during Ramadan fasting did not differ compared to before the month while the dietary patterns changed significantly. The full-fat diet reduced hunger pain and constipation but increased diarrhea while a healthy diet reduced dyspepsia and diarrhea during fasting. [ABSTRACT FROM AUTHOR]

Background: Stuttering is a kind of speech disorder that affects about 1% of total population. As the origin of this disorder is not obviously diagnosed yet, various remedies have been practiced and among them different medicines have been studied, but unfortunately no significant effective drugs have been recognized yet. As stuttering imposes a great social and mental costs to the patients and their families, finding an effective medicine will help significantly. In this study we have focused on the effects of levetiracetam (LEV) treatment on children suffering from stuttering. Methods: In this clinical trial study, 30 children aged > 3 years (median 3.8 years) with stuttering and abnormal sleep electroencephalogram (EEG) were treated by LEV and followed-up for a minimum period of 6 weeks. The starting dose of 20 mg/kg/day was increased at an interval of 1 week by 20 mg/kg/day, if necessary, up to maximum dose of 60 mg/kg/day. Results: Overall LEV was effective in 70% of patients, decreasing stuttering to at least 50%. Three children (10%) became stuttering-free and only in one (3.3%) child an increase in stuttering was observed. There were statistically significant differences for efficacy in the presence of variables such as age groups, seizure, stuttering family history, and EEG data. Conclusions: LEV is an effective drug for treatment of childhood stuttering in those that have abnormal sleep EEG. [ABSTRACT FROM AUTHOR]

Objectives Considering the common use of valproate among children, we investigated the short-term side-effects of low dose valproate monotherapy in epileptic children. Methods In this prospective study, 209 epileptic children (48.3% male, mean age: 7.02 ± 3.13 yr) on low therapeutic dose of valproate monotherapy (20-30 mg/kg/d) were enrolled during 2014-2015 in Isfahan Pediatric Neurology Clinic, Isfahan University of Medical Sciences, Isfahan, Iran and side-effects were evaluated through frequent clinical visits and laboratory tests during 6 months of valproate therapy. Results Weight gain was reported in 53.1% of patients. Decreased appetite was seen in 11% of patients, more frequent in younger cases (P=0.006). Abdominal pain, nausea/vomiting, diarrhea, and constipation were reported in 16.3%, 2.4%, 1.4%, and 1% of patients, respectively. Headache, tremor, dizziness, abnormal color vision, myoclonus, and bruxism were seen in 5.7%, 1.4%, 1%, 1%, 1%, and 0.5% of patients, respectively. Enuresis, hair loss, and skin rash were reported in 8.1%, 6.7%, and 0.5% of patients, respectively. Thrombocytopenia, impaired liver function tests, and leukopenia occurred in 1%, 1%, and 0.5% of patients, respectively. Conclusion Low dose valproate monotherapy may cause numerous side-effects, mostly not life-threatening and requiring no action. Beside other reported complications, we observed decreased appetite (among younger patients), enuresis, and abnormal color vision which are only briefly discussed in the literature and need to be addressed more. [ABSTRACT FROM AUTHOR]

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Objective There are no reports about the clinical presentations and outcome of Guillain-Barré syndrome (GBS) in our region, therefore, we aimed to report some of the above mentioned finding a aforementioned findings in children diagnosed with GBS in Isfahan, central Iran. Materials & Methods In this retrospective study, pediatric children diagnosed with GBS referred to Imam Hossein Hospital, the Pediatric Referral Center of Isfahan Province, central Iran were enrolled from 2011-2014. The following datar were extracted from the medical files of the patients; age, gender, early signs and symptoms of GBS, neurological features, sensory and motor and autonomic involvements, sphincter dysfunction, bulbar muscle involvement, respiratory failure, cranial nerve paralysis, delay time from onset to definite diagnosis, management of GBS, and the outcome. Results Overall, 57 children with GBS aged 1-13 yr were evaluated. Frequency of GBS was significantly higher in boys than in girls (6.38% vs. 4.61%, P=0.01, OR=0.39). The most common clinical presentations were distal lower limb weakness (92.11%), reduced deep tendon reflex (DTR) (82.46%) and neuropathic pain (75.44%). 92.9% of patients had complete recovery. Conclusion Distal lower limb weakness, reduced deep tendon reflex, and neuropathic pain are the main clinical presentation in children with GBS but in some patients, DTR may be normal or even exaggerated in early stages of disease. Revising the diagnostic criteria for GBS may be necessary. Most of our patients had complete recovery. The only death was due to autonomic involvement. Autonomic dysfunction could be associated with catastrophic outcome and patients with this kind of clinical presentations need critical care. [ABSTRACT FROM AUTHOR]

Background & Objectives: Annually, many Muslims fast during the month of Ramadan worldwide. This practice has different favorable medical and physiological effects, such as improved serum lipid profile and blood glucose level due to changes in diet and sleep patterns. It has also been hypothesized that Ramadan fasting may affect the immune system. As reported, Ramadan fasting can influence the immunoglobulin and cytokine levels. Accordingly, tuberculin skin test or purified protein derivative (PPD) test, which is a delayed-type hypersensitivity of cellular immune response, may also be affected by Ramadan fasting. Regarding this, the present study aimed to investigate the alteration of PPD test during and after Ramadan. Materials & Methods: A total of 42 males (seminary students) who fasted during Ramadan in 2006 were included in the study; however, only 28 cases completed the study. For data collection, the participants underwent blood and tuberculin tests at the fourth week of Ramadan and three months after this month. The white blood cell (WBC) count and the tuberculin induration were recorded and compared between the two intervals to evaluate the changes. Results: According to the results of the study, the mean age of the participants was 19.21±3.83 years. Furthermore, the mean tuberculin induration was 9.3±5.4 mm (size range: 2-22 mm) on the fourth week of Ramadan, which increased to 9.79±6.8 mm (size range: 3-35 mm) three months after this month (P=0.501). The mean count of WBC decreased insignificantly from 5907±1879 mcL to 5601±1362 mcL after Ramadan (P=0.334). Additionally, the mean lymphocytes count decreased significantly from 2292±520/mcL to 2023±486/mcL after this month (P=0.003). Likewise, the lymphocyte (P=0.014) and mean neutrophil percentage also reduced significantly (P<0.001). However, there was no association between PPD test and WBC, lymphocyte, or neutrophil count (P>0.05). Conclusion: As the findings of the present study indicated, Ramadan fasting induce some changes in the immune status, including lymphocyte and neutrophil percentage and count; however, it does not affect the PPD results. [ABSTRACT FROM AUTHOR]