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低ナトリウム（Na）血症は，小児，成人を問わず最も頻度の高い電解質異常であり，重篤な神経学的障害を起こしうる病態である．本稿では小児の低Na 血症の病態や予防，治療をめぐりこれまでに行われてきた議論の歴史的な経緯，そして小児の低Na 血症へ対応をするうえで，今後検討すべき新たな問題点や課題について述べた．, Hyponatremia is the most common electrolyte imbalance in children and adults and is a condition that can cause serious neurological disorders. In this review, we described the historical background of the debate over the pathophysiology and prevention/treatment of hyponatremia, and the issues to be considered in the future regarding the clinical practice of hyponatremia in children.

Purpose: We investigated the relationship of 25-hydroxyvitamin D (25[OH]D) level with obesity and atherosclerosis in Japanese adolescents.Methods: We examined 492 children (247 boys and 245 girls) aged 12–13 years. The serum 25(OH)D level was compared among underweight, healthy weight, and overweight children. Spearman correlation coefficient analysis was performed to examine the relationships between the 25(OH)D level and body mass index (BMI), plasma lipids, and blood pressure and to compare the latter between the normal (≥20 ng/mL) and low (

小児肥満は高率に成人肥満へ移行し動脈硬化性疾患のリスクを増加させるだけでなく，小児期から肥満に関連する健康障害を引き起こすため対策が重要である．運動は肥満の予防や改善に強く関連するが，肥満小児にとって継続が難しい側面もある．運動介入プログラムでなく，自主的な運動の効果について検討した研究は少ない．そこで運動の有効性を明らかにするため，10〜12歳の肥満男児について，自主的な運動の有無により体格指標や生化学的検査に差があるか検討した．スポーツクラブ等に所属し週1回以上継続的に運動を行う群で，ウエスト周囲長，ウエスト身長比，血圧が有意に低かった．内臓脂肪蓄積がメタボリックシンドロームの主体であり，高血圧やインスリン抵抗性等を伴って健康障害をもたらす．内臓脂肪は皮下脂肪よりも運動に鋭敏に反応することから，運動群のウエスト周囲長は少ない内臓脂肪を反映し，血圧の改善にも関与していると考えられた．肥満だと運動に対する苦手意識を持ちやすく，運動効果を実感出来ずに続かない事も多いが，本検討では，体重やBMI Zスコアに差がなくても改善しているパラメーターが認められ，運動を継続的に行うことの有効性が示された．, Childhood obesity has a high probability of transitioning to adult obesity, increasing the risk of arteriosclerosis. In addition, since childhood obesity causes health problems related to obesity from childhood, it is important to address the problem of obesity. Exercise is strongly associated with the prevention and improvement of obesity, but it is often difficult for children with obesity to continue exercising. Therefore, to clarify the effectiveness of continuous and voluntary exercise in boys with obesity aged 10 to 12, we examined whetherthere is a difference in anthropometric index and blood test depending on exercise. Waist circumference length, waist height ratio, and blood pressure were significantly lower in the group who voluntarily exercised at least once a week than that of non-exercise group. Visceral fat accumulation is the main component of metabolic syndrome and causes health problems accompanied by hypertension and insulin resistance. However, visceral fat responds more sensitively to exercise than subcutaneous fat. The waist height ratio of the exercise groupreflected low visceral fat, suggesting that exercise was also associated with improvement in blood pressure. Children with obesity tend to have a weakness in exercise. In addition, they often do not continue exercising unless they can feel the effects of exercising. However, even if there was no difference in body weight or BMI Z score, parameters that were improved by the voluntarysports activities of children with obesity were observed. Therefore, the effectiveness of continuous exercise was shown.

[Purpose] Exercise is a key factor in preventing obesity and metabolic syndrome. Sumo wrestlers increase their body size from childhood for athletic advantage; however, the risk of metabolic syndrome in junior sumo wrestlers is undetermined. Preventive measures against pediatric obesity should be initiated during childhood to prevent obesity in adulthood, considering its high global incidence. We comparatively evaluated the risk factors for metabolic syndrome in junior sumo wrestlers and children with obesity.[Methods] We enrolled 70 male children (age 9–17 years [sumo group, n = 14] and 9–14 years [other sports and non-exercise groups, n = 28 each]) and evaluated their anthropometric parameters (height, weight, body mass index z-score, obesity rate, waist circumference, waist to height ratio) and hematological parameters (total, low-density, high-density, and non-high-density lipoprotein-cholesterol; triglycerides; plasma glucose, and glycated hemoglobin levels).[Results] The BMI z-score, obesity rate, waist circumference (p < 0.05, along with the non-exercise group), and systolic blood pressure were significantly higher and the high-density cholesterol level was lower in the sumo group than in the other sports group (p < 0.05). The waist to height ratio was significantly higher in the non-exercise group than in the other sports group (p < 0.05). No significant difference was found in other blood lipid, plasma glucose (significantly lower level than the reference range in the sumo group, p < 0.05), and glycated hemoglobin (within the reference range in all groups) levels among the three groups.[Conclusion] Junior sumo wrestlers had a larger body size and higher blood pressure than children with obesity who exercised regularly. This provides direction for future research into targeted preventive interventions against metabolic syndrome for junior sumo wrestlers with large body size.

Purpose: If children show inadequate infant weight gain, their growth velocity will also decrease, especially £3 years of age. We hypothesized that such children may not only lead to being underweight but also short stature during childhood, and this trend will follow them through adolescence. Methods: A total of 271 children were enrolled. The Pearson correlation coefficients were calculated to determine the relationships of the increment of weight gain £3 years, from birth to 1.6 years and from 1.6 years to 3 years of age, with height and weight at 3, 7 years and pubertal age, respectively. Results: The Pearson correlation coefficients between weight gain each of from birth to 1.6 years and from 1.6 years to 3 years of age and weight at pubertal age were 0.34 and 0.37 in boys and 0.47 and 0.48 in girls, and height at pubertal age were 0.34 and 0.24 in boys and 0.65 and 0.40 in girls, respectively. Conclusions: Insufficient weight gain in children £3 years, especially £1.6 years is not only associated being underweight, but also short stature at 3 and 7 years and at the onset of puberty and it is also suspected that adult height and weight may be affected.

Human behavioral sex differences are currently understood to result from a combination of social, cultural, cognitive, and biological mechanisms. To understand how gender identity as the sexuality of the mind is formed is important for understanding psychosexual problems of children and to consider how to manage patients with disorders of sex development（DSD）, in which the development of gonads and genitals is atypical and it is difficult to determine the gender of boys and girls. There is consistent evidence that early testosterone exposure influences childhood gender role behavior, as well as gender identity and sexual orientation. In this review, we summarize the most relevant studies on the biological basis of sexual development. In particular, we focus on the impact of sex hormones and genetic background on development of sexual differentiation and gender identity, with introduction of our research using figure drawings by pediatric patients with congenital adrenal hyperplasia, which is also a DSD.

Objectives Adiposity rebound (AR) refers to an increase in body mass index (BMI) after a nadir. Early AR, in which AR occurs earlier than five years old, is a risk factor for future obesity and metabolic syndrome, but has not been widely studied in very-low-birth-weight infants (VLBWIs). Methods The subjects were VLBWIs born in Dokkyo Medical University NICU from January 2008 to December 2010. Height and weight measured at birth and at intervals until seven years old were obtained from medical records. The lowest BMI after one year of age was used for the age of AR. The subjects were divided into those with early and normal AR ( Results There were 38 early AR cases and 62 normal AR cases, giving a prevalence of early AR similar to that in infants with normal birth weight. BMI percentile at age seven years was significantly higher in early AR cases than in normal AR cases (44.6 [IQR: 21.0–79.2] vs. 14.4 [IQR: 3.8–40.8] kg/m2). Changes in BMI were also significantly higher in early AR cases (p=0.024 by ANOVA). Conclusions Early AR in VLBWIs is a predictor of future obesity.

The patient was diagnosed with ulcerative colitis and received remission induction therapy with prednisolone. While they developed muscle pain in the lower extremities after starting prednisolone, they ultimately achieved clinical remission. After discharge, hematemesis and gastric discomfort developed. Esophagogastroduodenoscopy showed mucosal edema, redness, and oozing bleeding in the gastric body. In addition, the patient had bloody stool and was considered to have a relapse of ulcerative colitis. They therefore received remission induction therapy with tacrolimus. The patient achieved clinical remission again; however, gastric discomfort and muscle pain remained. A drug lymphocyte stimulation test revealed positivity for prednisolone; therefore, the patient was diagnosed with an allergy to prednisolone.

The incidence rate of vitamin D deficiency is increasing throughout the world. We measured the incidence rate of vitamin D deficiency and fibroblast growth factor 23 (FGF23) levels in 12- to 13-year-old adolescents in Japan. A total of 492 adolescents (247 boys and 245 girls) from Japanese community enrolled in this study. 25 hydroxyvitamin D (25(OH)D) was measured with radioimmunoassay. In the subjects with low 25(OH)D levels (≦ 20 ng/ml), intact parathyroid hormone (iPTH), calcium (Ca), phosphorus (P), albumin (Alb), alkaline phosphatase (ALP) and FGF23 were measured. 25(OH)D levels were significantly lower in girls (20.9 ± 3.1 ng/ml) than in boys (22.2 ± 3.3 ng/ml) (p

Dynamic changes in body weight have long been recognized as important indicators of risk for human health. Many population-based observational studies have shown that rapid weight gain during infancy, including a catch-up growth phenomenon or adiposity rebound in early childhood, predisposes a person to the development of obesity, type 2 diabetes, and cardiovascular diseases later in life. However, a consensus has not been established regarding which period of weight gain contributes to future risks. This review evaluates recent evidence on the relationship between early rapid growth and future obesity and cardiometabolic risk, with a focus on the differential significance of rapid weight gain in infancy and early childhood. Although there is a need for attention to childhood growth during early infancy before 1 yr of age as it may be related to future obesity, emerging evidence strongly suggests that toddlers showing an increase in body mass index (BMI) before 3 yr of age, a period normally characterized by decreased BMI, are prone to developing later cardiometabolic risk.

Aim: Children with Familial Hypercholesterolemia (FH) are widely prescribed statins, and it has been suggested that the effects of statins differ among ethnicities. We compared the efficacy and safety of pitavastatin in children and adolescents with FH in clinical trials conducted in Japan and Europe. Methods: Low-density lipoprotein cholesterol (LDL-C) reductions, adjusted for confounding factors, and safety were compared between the studies in Japan and Europe. In the Japanese study, 14 males with heterozygous FH, aged 11.8 ± 1.6 years, were randomized to 52-week double-blind treatment with 1 or 2 mg/day pitavastatin. In the European study, 106 children and adolescents with high risk hyperlipidemia (103 heterozygous FH), aged 10.6 ± 2.9 years, were randomized to 12-week double-blind treatment with 1, 2 or 4 mg/day pitavastatin or placebo; 84 of these patients and 29 new patients participated in a 52-week open-label extension study. Results: Age, body weight and baseline LDL-C were identified as factors influencing LDL-C reduction. There were no significant differences in the adjusted mean percentage reduction in LDL-C in Japanese and European children by pitavastatin (24.5% and 23.6%, respectively at 1 mg/day and 33.5% and 30.8%, respectively at 2 mg/day). Pitavastatin was well tolerated without any difference in the frequency or nature of adverse events between the treatment groups, or between the studies. Conclusion: There were no significant differences between the efficacy or safety of pitavastatin in Japanese and European children and adolescents with FH, suggesting no relevant ethnic differences in the safety or efficacy of pitavastatin.

Leukocyte-adhesion deficiency-1 is a recessively inherited disorder associated with recurrent bacterial infections, severe periodontitis, peripheral leukocytosis, and impaired wound healing. We diagnosed moderate-type leukocyte-adhesion deficiency-1 in a 7-year-old girl who developed a necrotizing ulcer after Bacillus Calmette-Guerin vaccination. The patient showed moderate expression of CD18 in neutrophils with a homozygous splice mutation with c.41_c.58+2dup20 of ITGB2 and experienced recurrent severe infections complicated with systemic lupus erythematosus. She received hematopoietic stem cell transplantation from a matched elder brother with heterozygous mutation of ITGB2, and has since remained free of infection and systemic lupus erythematosus symptoms without immunosuppression therapy.

BACKGROUND The aim of this study was to verify whether lipid emulsion treatment aggravates infection and inflammation in very low-birthweight (VLBW) infants. STUDY DESIGN Very low-birthweight (

Trisomy 18 syndrome is a common autosomal aneuploidy chromosomal abnormality caused by the presence of extra chromosome 18 that leads to malformations of various parts of the body. In this study, we retrospectively investigated the effect of the medical progression and prognosis of 44 cases of trisomy 18, admitted to our neonatal intensive care unit between 1992 and 2013. The patients were divided into group A (n=20, 1992–2002) and group B (n=24, 2003–2012). Following delivery, karyotype, gender, gestational weeks, birth place, cesarean section, Apgar score and birth weight were analyzed using the Fisher's exact test, unpaired t-test and Mann-Whitney U test. Based on the statistical results, a comparison was made of the two groups and no significant differences were observed. Clinical data of major complications, mechanical ventilation, discharge from hospital and survival days were reviewed for the cases of trisomy 18. Of the 44 patients, 42 had cardiac anomaly, 16 had esophageal atresia, and 3 patients had brain anomaly. Ventilation treatment was performed in 29 cases (65.9%) and an increased percentage was identified in group B patients. The percentage survival was estimated using Kaplan-Meier curves and the two groups were analyzed using the generalized Wilcoxon test. Improvement in life prognosis was observed in group B as compared to group A. The log-rank test was used to assess survey periods of 180 days, 1 year, and the entire observation period. Although significant differences were observed for the prognosis of trisomy 18 at 180 days after birth, after 1 year and the entire survey period after birth, the significant differences were not confirmed. In conclusion, results of the present study provide information concerning genetic counseling for parents/guardians and life prognosis, prior to applying intensive management to newborns with trisomy 18.

Fetal bone development is a complex process that is regulated and maintained by minerals, hormones, and growth factors delivered from the mother via the placenta. Various biochemical markers of fetal bone development have been identified. However, many aspects of this process remain unclear. The aim of the study was to measure the activities of serum tartrate-resistant acid phosphatase type 5b (TRACP 5b) as a bone resorption marker and bone alkaline phosphatase (BAP) as a bone formation marker in preterm and term neonates, and to investigate fetal bone development in middle and late pregnancy. The study included 111 neonates (87 preterm and 24 term) born at Dokkyo Medical University Hospital. Neonates with illnesses and maternal diseases were excluded. Serum samples were collected within 3 hours after birth and stored at -80°C. Univariate and multivariate linear regression analyses were performed. The 111 neonates (median birth weight, 1,510 g) were born at a median of 31.3 weeks of gestation, and had TRACP 5b and BAP activities of 10.9 ± 4.0 U/L and 127.5 ± 49.2 U/L, respectively. TRACP 5b activity showed a tendency to be higher in term neonates, while BAP activity tended to be lower in term neonates. Importantly, TRACP 5b activity was positively correlated with gestational age and birth weight, and BAP activity was negatively correlated with gestational age, rate of born small-for-gestational-age neonates, and birth weight. These results suggest that bone formation during fetal growth is gradually decreased from middle pregnancy to birth, whereas bone resorption is gradually increased.

Background Bronchial asthma is a common but important chronic disease in children in all over the world. To take measures against prevalence of childhood asthma, many researchers have surveyed the actual statuses of childhood asthma in developed countries, but in most Asia–Pacific developing countries including Mongolia such surveys have never been sufficiently conducted until now. We have thought that this survey, though performed in 2009, will give important and meaningful information even now in taking measures to prevent prevailing bronchial asthma in children in Mongolia or the countries under similar statuses. Methods The asthma prevalence and patient background information in Mongolian children aged 6–7 living in Ulaanbaatar were examined using a written questionnaire modified for their parents from that prepared by the International Study of Asthma and Allergies in Childhood (ISAAC). Results The estimated prevalence of asthma in Mongolian children was 20.9%. The following 3 risk factors were found to be related to asthma: (1) having allergic rhinitis symptoms, (2) mothers' smoking, and (3) history of severe respiratory infection before 1-year-old. Conclusions The asthma prevalence in Mongolian children was higher than that in the world and Asia–Pacific countries reported by ISAAC. The higher prevalence was probably attributable to households' (especially mothers) smoking in draft-free houses designed for the cold area and severe air-pollution due to rapid industrialization and urbanization in Mongolia. Smoking prohibition in the mother (including family members) and a reduction of exposure to air pollutants are urgently needed to prevent developing childhood asthma.

Background: If asthmatic children cannot obtain sufficient control of their disease, not only do they suffer from asthma symptoms, but the daily life activities of their caregivers are also disrupted. We investigated the effectiveness of an inhaled corticosteroid (ICS) for symptom control in previously ICS-untreated school-aged asthmatic children as well as caregiver treatment satisfaction (CTS). Methods: A multicenter, open-label, single-arm study on 12-week ICS (budesonide Turbuhaler®) monotherapy was undertaken in subjects aged 5–15 years with bronchial asthma not treated with ICS during the previous 3 months. At 0, 4, 8, and 12 weeks after start of ICS administration, Japanese Pediatric Asthma Control Program (JPAC) scores, and CTS scores were summated and lung function measured. At weeks 0 and 12, questionnaires on caregiver anxiety were also assessed. Results: Seventy-five patients were enrolled, and 69 assessed. Ninety percent of subjects had been treated with asthma controller medication except ICS before study enrollment. JPAC score and CTS score were improved significantly at weeks 4, 8, and 12 (p

確定診断前に凝固第VIII因子(F VIII)製剤の緊急投与を要した重症型血友病A の2 乳児例を報告する.症例1 は10 か月男児.反復する嘔吐を主訴に入院した.左上肢に腫張を伴う筋肉内出血と硬膜外血腫を認めた.症例2 は10 か月男児.止血困難な口唇裂傷後の出血を主訴に来院し,重度の貧血を認めた.2 例ともプロトロンビン時間(PT)正常,活性化プロトロンビン時間(APTT)の著明な延長より,血友病を強く疑った.いずれも重篤な出血を認めたため,凝固因子活性値による確定診断を待たず,緊急でF VIII製剤の投与を試みた.投与後2 例ともAPTT は改善し,出血症状も改善した.後日2 例ともにF VIII活性が1%未満と判明し,重症型血友病A と確定診断された., The cause of hemophilia A and B involves loss of factor VIII( FVIII) and factor IX( FIX), respectively. The hereditary form of this hemorrhagic disease is X-linked recessive. It is well established that the critical region for hemophilia A is localized on Xq28 and for hemophilia B on Xq27.1- 27.2. The initial symptom is bleeding in the mucous membranes often accompanied by intramuscular and intraarticular hemorrhage. The hemorrhages in the joints cause joint contracture as a sequela. The diagnosis of hemophilia is based on a normal bleeding and prothrombin time(PT)and prolonged activated partial thromboplastin time (APTT). We report here two infants of the severe typical from of hemophilia A who were treated with sufficient needed dosage of recombinant factor VIII before a final diagnosis was made. Case 1 was a 10-month-old boy. He was hospitalized for recurrent vomiting. He had intramuscular bleeding with swelling of the left shoulder and upper extremity. A head CT showed multiple epidural hematomas. Case 2 was a 10-month-old boy. He was sent to our hospital because of a lip laceration that did not stop bleeding, and he had severe anemia. We made the diagnosis of hemophilia A based on both their normal laboratory finding of PT and on the finding of extended APTT. Before confirming the decision diagnosis of hemophilia, we intravenously injected recombinant FVIII immediately, because of the severe hemorrhagic symptoms. After the therapy, both the APTT and hemorrhagic symptoms improved. These two cases were later confirmed as a severe infantile form of hemophilia A with less than 1 % factor VIII activity.

新生児遷延性肺高血圧症(persistent pulmonary hypertension of the newborn:PPHN)は,出生早期に肺高血圧症を発症し高度の低酸素血症を来す疾患である.正期産児のPPHNに対しては,NO吸入療法(inhaled nitric oxide:iNO)が有効とされているが,早産児における有効性は確立していない.今回,我々は在胎30週未満の早産児に対するiNOの有効性および安全性について検討した.対象は,出生後3日以内にiNOを施行した在胎30週未満の早産児39例である.肺高血圧の原因疾患は肺炎・敗血症が多かった.基礎疾患によって有効率が異なったが,iNO開始1時間の時点で全体の64%の症例はPPHNが改善した.PPHN改善後は速やかにiNOを減量・離脱できた症例が多かった.無効症例は週数・出生体重がより小さい児で多かった.有害事象としてはメトヘモグロビン血症を2例認めた.iNOは早産児のPPHNに対して,短期間の加療で出生早期の循環動態を改善させる有用な治療法であると考えられた., Persistent pulmonary hypertension of the newborn (PPHN) is a disease in which newborns develop pulmonary hypertension soon after birth, causing severe hypoxemia. Inhaled nitric oxide (iNO) therapy is effective in treating PPHN in term infants;however, the efficacy of iNO in preterm infants has not been established. We investigated the efficacy and safety of iNO as a treatment for preterm infants born at

Objective To investigate whether increments of weight gain in early infancy are related to the timing of adiposity rebound (AR). Study design A total of 271 children (147 boys and 124 girls) in 1 community were enrolled in the study. Serial measurements of body mass index were carried out at the ages of 4, 8, and 12 months and 1.5, 2, 3, 4, 5, 6, 7, 8, 9, and 10 years, based on which the age of AR was determined. We also calculated body weight increments in 3 separate periods: birth to 4 months, 4-8 months, and 8-12 months. Results There was no significant relationship between weight gain in any period of infancy and the age of AR. Weight gain between birth and 4 months was positively correlated only with body mass index at 7 years of age. Conclusions We could not find an association between body weight gain during infancy and the timing of AR. This suggests that infantile weight gain is not related to childhood obesity through AR.

Male hypogonadotropic hypogonadism (MHH), a disorder associated with infertility, is treated with testosterone replacement therapy (TRT) and/or gonadotropins replacement therapy (GRT) (TRT and GRT, together with HRT hormone replacement therapy). In Japan, guidelines have been set for treatment during adolescence. Due to the risk of rapid maturation of bone age, low doses of testosterone or gonadotropins have been used. However, the optimal timing and methods of therapeutic intervention have not yet been established. The objective of this study was to investigate the current situation of treatment for children with MHH in Japan and to review a primary survey involving councilors of the Japanese Society for Pediatric Endocrinology and a secondary survey obtained from 26 facilities conducting HRT. The subjects were 55 patients with MHH who reached their adult height after HRT. The breakdown of the patients is as follows: 7 patients with Kallmann syndrome, 6 patients with isolated gonadotropin deficiency, 18 patients with acquired hypopituitarism due to intracranial and pituitary tumor, 22 patients with classical idiopathic hypopituitarism due to breech delivery, and 2 patients with CHARGE syndrome. The mean age at the start of HRT was 15.7 yrs and mean height was 157.2 cm. The mean age at reaching adult height was 19.4 yrs, and the mean adult height was 171.0 cm. The starting age of HRT was later than the normal pubertal age and showed a significant negative correlation with pubertal height gain, but it showed no correlation with adult height. As for spermatogenesis, 76% of the above patients treated with hCG-rFSH combined therapy showed positive results, though ranging in levels; impaired spermatogenesis was observed in some with congenital MHH, and favorable spermatogenesis was observed in all with acquired MHH. From the above, we propose the establishment of a treatment protocol for the start low-dose testosterone or low-dose gonadotropins by dividing subjects into two groups to determine different treatment protocols, acquired and congenital MHH, and to conduct them at a timing closer to the onset of puberty, namely, at a timing near entrance to junior high school. We also propose a new HRT protocol using preemptive FSH therapy prior to GRT aimed at achieving future fertility in patients with congenital MHH.

Leukotriene receptor antagonists (LTRAs) are identified as a monotherapy for asthma and allergic rhinitis; however, their use in children for treatment of these diseases has not been examined. Accordingly, the present study investigated the efficacy of pranlukast dry syrup for children with both pollinosis and asthma. The subjects were children receiving treatment for asthma who were also diagnosed with cedar pollen allergy. Patients were divided into a group that received continuous treatment with pranlukast (group A; n=20) and a group that commenced add-on treatment for pollinosis following the onset of symptoms (group B; n=20). Patients in group B were randomly allocated to subgroup B1 (add-on treatment with pranlukast dry syrup) or subgroup B2 (add-on treatment with a second-generation antihistamine). In both groups, nasal and ocular symptoms were evaluated every day and recorded in a diary. Exacerbation of nasal obstruction was demonstrated in group B; however, not in group A. There was a significant difference in symptoms observed between the two groups during the late peak pollen period (P

Octreotide, a long-acting somatostatin analog, has been used for treating hypoglycemia caused by congenital hyperinsulinism (CHI). However, octreotide has not been evaluated in clinical trials and has not been approved in any developed country. We aimed to test the efficacy and safety of octreotide for diazoxide-unresponsive CHI through a combination of a single-arm, open-label clinical trial (SCORCH study) and an observational study to collect data on the clinical course of patients treated off-label in Japan (SCORCH registry). In the SCORCH study, 5 patients were stabilized (blood glucose > 45 mg/dL) by hypertonic glucose infusion, and treated by continuous subcutaneous octreotide infusion at a dose of 5-25 μg/kg/day. Continuous blood glucose monitoring was performed between -24 and +48 hours. In 3 patients, a clinically meaningful rise in blood glucose was achieved and therapy was continued. The glucose infusion was gradually decreased and stopped after 5, 11, and 174 days, respectively. In one case, remission of CHI was reached after 606 days and octreotide was discontinued. The SCORCH registry included 19 diazoxide-unresponsive patients treated by subcutaneous octreotide, by continuous infusion or multiple daily injections. Of the 17 patients treated with hypertonic glucose infusion, the infusion rate was reduced after 4 weeks to less than 50% in 11 patients (64.7%) and stopped in 9 (52.9%). During the combined observation period of 695.4 patient-months in both studies, no severe adverse events related to octreotide were observed. In conclusion, subcutaneous octreotide injection was effective and well tolerated in the majority of patients with diazoxide-unresponsive CHI.

症例1は6歳女児.インフルエンザ感染症初日に発熱しオセルタミビルを開始.第3病日,右下腹部に限局した圧痛が出現.腹部造影CTで糞石を認め急性虫垂炎と診断.保存的に加療し炎症反応と腹痛は改善した.症例2は5歳女児.第1病日に発熱と腹痛を認め,第3病日に鼻咽腔迅速検査でインフルエンザB型と診断しザナミビル吸入を開始.触診で右下腹部に反跳痛を認め,腹部単純CTで虫垂壁の肥厚と糞石を確認.急性虫垂炎の併発と診断し,第4病日に虫垂切除術を施行.切除虫垂に膿瘍を認め腹腔ドレーンを留置.第5病日に解熱し経過は順調であった.インフルエンザに伴う腹痛では感染に付随する腹痛と断定せず急性虫垂炎の可能性も考慮し腹部CTなどの画像検査を行うことが肝要である., We report herein two pediatric cases of influenza virus infection associated with acute appendicitis. Patient 1 was a 6-year-old girl. She presented to hospital with a high fever and received oral administration of oseltamivir phosphate. On the third day from the onset, tenderness developed in the right lower area at McBurney's point. Abdominal enhanced computed tomography( CT) showed intestinal fecalith, and acute appendicitis was subsequently diagnosed. Antibiotic therapy was performed, and abdominal pain and inflammation data improved. Patient 2 was a 5-year-old girl. On the first day, abdominal pain developed. On the third day from the onset, influenza type B was diagnosed using rapid antigen testing of the nasal mucosa, and inhalation therapy was started with zanamivir. Rebound tenderness was seen in the lower right abdomen. Abdominal CT showed both hypertrophy of the appendix wall and intestinal fecalith, and influenza infection associated with acute appendicitis was subsequently diagnosed. On the fourth day, appendectomy was performed. Drainage treatment was required to prevent peri-appendicitis abscess. On the fifth day, the patient defervesced and, as for the progress, was favorable. When encountering influenza infection associated with abdominal pain, performing abdominal CT is important.

Tolosa-Hunt症候群(THS)は先行する片側眼窩部痛と眼球運動障害を生じ,病態は海綿静脈洞の非特異的炎症性肉芽腫病変と推測されている.発症は年間100万人あたり1人前後で,40歳台の成人に多く小児例は稀である.今回,1カ月続く右眼をえぐられる様な頭痛を主訴とした15歳のTHSを報告する.発症後,各種頭痛薬で改善がなく,各種検査を施行し異常がないため心身症に伴う反復する片頭痛と診断された.当院で脳MRIを施行し右内頸動脈の狭窄を認めTHSと確定診断した.プレドニゾロン(PSL)1?mg/kg/dayを朝1回開始し,翌日頭痛は改善した.以降,半年かけてPSLを漸減し再発はない., Tolosa-Hunt syndrome(THS)is characterized by periorbital pain accompanying opthalmoplegia. The pathogenesis is considered to involve non-specific granulomatous inflammation in the cavernous sinus, and the frequency is around one case per year per million people. Symptoms usually develop in adulthood, and pediatric cases are rare. We report herein a case of THS in a 15-year-old girl whose headache was diagnosed as psychosomatic disease in the early stage of the clinical course. Her chief compliant was headache with strong pain in the right eye, continuing for 1 month. Although several medications were trialed to alleviate headaches, no improvement was achieved. Various physical examinations proved uninformative. Headache was therefore tentatively diagnosed as psychosomatic disease associated with migraine. Brain magnetic resonance imaging in our university hospital revealed strangulation of the internal carotid artery, and headache was diagnosed as confirmed THS. Oral administration of prednisolone was started at 1 mg/kg/day, given once in the morning. Headache improved from the next day. Oral therapy with prednisolone was tapered over the course of 6 months and headache did not recur.

Although previous studies have reported on the effectiveness of brain hypothermia therapy in childhood acute encephalopathy, additional studies in this field are necessary. In this review, we discussed brain hypothermia therapy methods for two clinical conditions for which sufficient evidences are currently available in the literature. The first condition is known as hypoxic-ischemic encephalopathy and occurs in newborns and the second condition is acute encephalopathy which occurs in adults following cardiopulmonary resuscitation associated with out-of-hospital cardiac arrest state resulting from ventricular arrhythmia. Furthermore, we assessed the prospects of applying these therapies to acute encephalopathy in children.

Acute disseminated encephalomyelitis confined to the brainstem is associated with poor prognosis. We describe a case of a 10-year-old boy with acute disseminated encephalomyelitis in the brainstem that developed after influenza A infection. A 10-year-old boy presented with fever and prolonged disturbance of consciousness and was admitted to our hospital. Magnetic resonance imaging (MRI) of the midbrain, with T2-weighted and fluid-attenuated inversion recovery images, suggested acute disseminated encephalomyelitis accompanied by a brainstem lesion. Lumbar puncture showed pleocytosis and increased protein content, including myelin basic protein, interleukin-6, and immunoglobulin G, all suggestive of acute disseminated encephalomyelitis. Treatments such as methylprednisolone pulse therapy, intravenous immunoglobulin, and therapeutic hypothermia were performed. Although the patient presented with anisocoria with increased intracranial pressure monitoring during hypothermia, prompt therapy with d-mannitol and dopamine was effective. Our case results suggest that hypothermia could be included in the choice of therapy for acute disseminated encephalomyelitis with brainstem lesions.