Parenting attitude towards children with autism spectrum disorders: the Japan environment and children's study.

OBJECTIVE: The primary objective of this study was to investigate the parenting attitudes towards children with autism spectrum disorders in early childhood in Japan. DESIGN: This study was a cohort study. The participants were enrolled from January 2011 to March 2014. We obtained the prevalence of autism spectrum disorders at 3 years of age, parenting attitudes and other factors from questionnaires. We divided the participants into two groups, an autism spectrum disorders group and a non-autism spectrum disorders group, and compared the parenting attitudes. SETTING: This study used data from a Japanese birth cohort study: the Japan Environment and Children's Study, conducted across 15 regional centres in Japan. PARTICIPANTS: The full dataset of the Japan Environment and Children's Study comprised 104 059 records. We excluded 17 889 records because the answer for the autism spectrum disorders in the questionnaire was blank. As a result, we analysed the remaining 82 411 mother-child pairs. MAIN OUTCOME MEASURES: The primary outcome variable was parenting attitudes at 3.5 years of age, which was assessed using a questionnaire. We asked respondents 16 questions related to parenting attitudes, and they answered based on their behaviours. The independent variable was the prevalence of autism spectrum disorders at 3 years of age. RESULTS: Of the 82 411 participants, the children with autism spectrum disorders at 3 years of age were 372 (0.45%). In most questions about parenting attitudes, the autism spectrum disorders group had unfavourable responses. The difference was particularly noticeable when the parents taught their children social discipline. Unfavourable parenting attitudes were 16.6% in the autism spectrum disorders group and 0.8% in the non-autism spectrum disorders group in the question item with the largest difference between the two groups, a significant difference. CONCLUSIONS: Parents of children with autism spectrum disorders tended to have unfavourable attitudes, suggesting the importance of parental training.

Environments affect blood pressure in toddlers: The Japan Environment and Children's Study.

BACKGROUND: The primary objective of this study was to examine risk factors for toddler's hypertension. METHODS: Subjects of this study were children and parents participating in a national birth cohort study in Japan, the Japan Environment and Children's Study. We measured the children's blood pressure (BP) at 2 and 4 years old. We obtained children's and parents' backgrounds from the questionnaire. We investigated the factors that affect BP elevation. RESULTS: Within 4988 participants, the mean systolic BP at 2 years old was 91.2 mmHg for boys and 90.0 mmHg for girls. The mean systolic BP at 4 years old was 93.8 mmHg for boys and 93.1 mmHg for girls. Parental smoking was associated with elevated values of BP at 2 and 4 years old. Obesity, gestational hypertension, and parental lower education were associated with elevated values of BP at 4 years old. Hypertensive group had a significantly higher obesity rate. The mother's lower education and parental smoking were involved in hypertensive groups. CONCLUSION: Parental smoking had a significant effect on BP even in early toddlers. We emphasize the importance of avoiding second-hand smoking from early infancy to prevent future lifestyle-related illnesses including hypertension. IMPACT: The mean systolic BP at 2 years old was 91.2 mmHg for boys and 90.0 mmHg for girls. The mean systolic BP at 4 years old was 93.8 mmHg for boys and 93.1 mmHg for girls. Obesity, parental smoking, and lower education were associated with hypertension at 4 years old. Parental smoking was associated with hypertension at 2 and 4 years old. We emphasize the importance of avoiding second-hand smoking from early infancy.

The Utility of Limited-Montage Electroencephalography for Seizure Detection in Children.

BACKGROUND: The primary objective of this study was to investigate the utility of limited-montage electroencephalography (EEG) for seizure detection in children. We also aimed to determine whether the detection rate differed among different montage patterns. METHODS: This study was carried out between November 2019 and October 2020 at a tertiary children's hospital in Japan. The subjects were inpatients in the pediatric intensive care unit who had an epileptic seizure during EEG monitoring. Each patient's EEG record, consisting of a 15-minute recording during an epileptic seizure and a 15-minute recording in the absence of an epileptic seizure, was extracted from the medical charts. The EEG data were then analyzed using six, limited-montage coverage patterns: (1) Fp1-C3, Fp2-C4, (2) Fp1-O1, Fp2-O2, (3) Fp1-T3, Fp2-T4, (4) C3-O1, C4-O2, (5) C3-T3, C4-T4, and (6) O1-T3, O2-T4. The sensitivity and specificity of each montage for seizure detection was analyzed. RESULTS: One hundred thirty-two EEG data points from 11 patients were examined. Sensitivity and specificity were the highest for Fp1-O1 and Fp2-O2 at 73% and 91%, respectively. Overall, the montage covering the frontopolar area had the highest detection rate, followed by the montage covering the occipital, central, and temporal areas. CONCLUSION: Limited-montage EEG identified seizures in children hospitalized in the intensive care unit, but the detection rate differed by montage coverage. The detection rate was highest in the montage covering the frontopolar area.

Classification of pediatric headache cases referred to a neurology department.

BACKGROUND: Headaches are very common in children. The patients often have mild symptoms, but on occasion may have severe, secondary headaches. The present study aimed to clarify the details of children with headaches seen at the outpatient clinic of a pediatric neurological department. METHODS: The present, retrospective observational study was conducted at a tertiary pediatric hospital in Japan and enrolled children referred to the neurology department outpatient clinic for headache between April 2018 and March 2021. RESULTS: In total, 113 cases of headache were examined; of these, 99 (87.6%) were primary headaches, one case (0.9%) was a secondary headache, and 13 (11.5%) were unclassified or unspecified. There were 46 cases (40.7%) of tension-type headache (TTH), both confirmed and suspected, 30 cases (26.5%) of migraine, and 23 cases (20.4%) of a combination of the TTH and migraine. One case of secondary headache was attributed to an infection. Arachnoid cysts were found in seven patients (7.8%). Acute drug treatments were administered to 93 patients (82.3%), with acetaminophen being the most common drug, followed by ibuprofen. Prophylactic drug treatments were administered to 39 patients (34.5%), with goreisan (a Chinese herbal medicine containing Alisma orientale, Poria cocos, Polyporus umbellatus, Atractylodes lancea, and Cinnamomum cassia) being the most common (41%). CONCLUSIONS: Few cases of secondary headache and none of emergency headache were diagnosed. The prevalence of arachnoid cysts was higher than in the general pediatric population, suggesting that arachnoid cysts might be associated with headache.

Tocilizumab for juvenile Takayasu arteritis complicated with acute heart failure at onset.

Chronic heart failure caused by aortic valve regurgitation is a common complication of Takayasu arteritis (TA). However, fewer patients develop acute heart failure (AHF), and no specific treatment for AHF in TA has been established. We encountered a 12-year-old girl with TA who developed AHF at the onset. We successfully treated her with intravenous methylprednisolone and tocilizumab. She developed palpitations and shortness of breath 3 weeks before admission. Her symptoms exacerbated rapidly and she finally entered the intensive care unit due to respiratory distress and tachycardia. Blood pressure measurements on the left arm and bilateral legs were paradoxically lower than that on the right arm. Chest X-ray revealed a severely enlarged heart. Contrast computed tomography showed an expanded aorta, aortic aneurysm, meandering, and irregular diameter of the aorta. The left ventricular ejection fraction was 20% on cardiac ultrasound. Her medical condition was finally diagnosed as TA with AHF. Along with inotropes and diuretics, methylprednisolone pulse therapy was administered on hospital days 2-4 and hospital days 12-14, followed by oral prednisolone. However, cardiac function was not notably improved. As intravenous cyclophosphamide therapy requires hydration and may exacerbate AHF, we initiated weekly subcutaneous tocilizumab treatment (162 mg/week) from hospital day 20. Inotropes were discontinued on hospital day 51 and her left ventricular ejection fraction had gradually improved to 37.5% at discharge (day 63). As AHF in TA is presumed to be due to inflammation of the myocardium, tocilizumab could be a treatment option for TA with AHF.

Neurological complications after living-donor liver transplantation in children.

AIM: Liver transplantation (LT) has been used as a definitive management for children with end-stage liver disease or acute liver failure. Living-donor LT (LDLT) has been a common type of LT performed in Asian countries, including Japan, where deceased donors are rarely available. However, the neurological complications (NCs) associated with LDLT remain unknown. The purpose of this study was to clarify the characteristics of NCs in children after LDLT. METHODS: This study is a retrospective observational study carried out at a tertiary children's hospital in Japan. We studied children who had undergone LDLT between January 2001 and January 2020. RESULTS: We examined 602 cases of LT, of which 559 were LDLT cases (92.9%). NCs after LT were present in 21 cases (3.8%). The most common neurological symptoms were seizure (n = 17), whereas disturbance of consciousness without seizure was observed in four cases. The frequency of NCs for each of the indications was 12.2% for fulminant hepatic failure, 6.5% for metabolic liver disease, and 0.7% for cholestatic liver disease. INTERPRETATION: We report the characteristics of NCs after LDLT in children. The frequency of NCs after LT was high in cases of fulminant hepatic failure and metabolic diseases, who might have had neurological symptoms or impaired consciousness before LT.

Refractory cerebral infarction in a child with an ACTA2 mutation.

INTRODUCTIONS: A specific mutation in the ACTA2 gene is known to cause multisystemic smooth muscle dysfunction syndrome, which is associated with cerebrovascular diseases and various organ disorders. Cerebral infarctions resulting from severe vasculopathy can be refractory; however, there are no previous reports describing the detailed clinical course of recurrent cerebral infarctions due to an ACTA2 mutation. Herein, we report a patient with an ACTA2 mutation who experienced multiple refractory cerebral infarctions in early childhood. PATIENT DESCRIPTION: The patient was aged 1 year and 5 months at her first episode of cerebral infarction. Arteriopathy due to an ACTA2 mutation was diagnosed based on the characteristic cerebrovascular findings and abnormal physical findings, such as bilateral dilated pupils. Bilateral encephaloduroarteriosynangiosis and encephalogaleosynangiosis were performed after the first episode. Because the cerebral infarctions recurred postoperatively, administration of cilostazol followed by bosentan was started. However, despite these treatments she experienced seven cerebral infarctions by age 2 years and 6 months. INTERPRETATION: Cerebral infarctions in patients with a specific ACTA2 mutation can occur even in early childhood, recur frequently, and cause severe motor and cognitive impairment. Physicians should be highly aware of this disease and be ready to provide the medical and surgical interventions necessary to minimize the disabling sequelae.

Complications of herpes zoster in children.

BACKGROUND: Herpes zoster (HZ) may cause complications; however, information regarding complications of HZ in children is relatively sparse. We reviewed cases of HZ in children retrospectively and investigated the risk factors contributing to the development of complications. METHODS: A retrospective observational study was conducted at a tertiary care children's hospital in Japan. Children receiving a diagnosis of HZ between January 2010 and October 2016 were identified from electronic medical records. In this study, the following diseases were recognized as complications of HZ: bacterial skin infection, facial paralysis, meningitis, uveitis and keratitis, postherpetic neuralgia, acute retinal necrosis, pneumonia, and otitis interna. Details regarding clinical information of HZ patients with complications were described. RESULTS: We found 138 cases with HZ. Among these, 58 (42%) occurred in immunocompetent children and 80 cases (58%) occurred in immunocompromised children. Complications were observed in 10 cases of immunocompromised children and in eight cases of immunocompetent children. Although secondary bacterial skin infection was the most common complication (n = 6, 33.3%), severe complications such as facial paralysis (n = 3, 16.7%), meningitis (n = 2, 11.1%), uveitis and keratitis (n= 2, 11.1%), acute retinal necrosis (n = 1, 5.6%), pneumonia (n = 1, 5.6%), and otitis interna (n = 1, 5.6%) were observed. Patients with complications tended to have a rash on the head or neck, compared with those without complications (n = 9, 50% vs n = 18, 15%; P = 0.001). CONCLUSIONS: Severe HZ complications may occur in immunocompetent children. Development of a rash on the head or neck might be a risk factor for complications.

Prolonged mild disturbance of consciousness and acute encephalopathy.

BACKGROUND: Acute encephalopathy (AE) is defined by the Japanese guidelines as the acute disturbance of consciousness (Glasgow coma scale [GCS] score ≤11) that persists for >24 h. We have often encountered, however, cases of prolonged mild disturbance of consciousness (PMDC) with GCS score >11, meaning that they do not fit the guideline definition of AE. The reports of these cases have been relatively sparse, and the nosological position, prognosis, and other characteristics remain unknown. To clarify the characteristics of PMDC we compared cases of PMDC with cases of AE. METHODS: This study was a retrospective observational study at a tertiary children's hospital in Japan. We studied children with a diagnosis of AE or PMDC between January 2011 and August 2016. RESULTS: Thirteen cases of PMDC and 19 cases of AE were identified during the study period. PMDC patients more frequently had hyponatremia (P < 0.01), paradoxical arousal response on electroencephalogram (P = 0.010), normal computed tomography (CT; P = 0.025), and normal magnetic resonance imaging (MRI; P < 0.01) than the AE patients. Sequelae were more frequently observed in AE than in PMDC patients (P = 0.011). CONCLUSIONS: PMDC has different characteristics to AE with regard to hyponatremia, paradoxical arousal response, CT or MRI findings, and prognosis. Despite the differences, PMDC might also be regarded as being a milder member of the wide variety of AE and related diseases.