Search

Buckwheat is a rare causative food for food protein–induced enterocolitis syndrome (FPIES). To date, it is unknown what laboratory data patients with FPIES caused by buckwheat show. We report a 4-year-old female with FPIES caused by buckwheat and the laboratory results. Skin prick, specific IgE antibody, and basophil activation tests were negative; however, the lymphocyte stimulation test (LST) revealed a 10.2-fold increase in activation compared with the negative control. In an open-label oral food challenge (OFC) of 80 g boiled buckwheat noodles, 3 hours after ingestion, vomiting occurred four times in a 2-hour duration. Therefore, we diagnosed the patient with FPIES caused by buckwheat. Her neutrophil count, C-reactive protein, and thymus and activation-regulated chemokine were elevated after the OFC. Moreover, the patient had a positive reaction to the LST, which may theoretically be useful in diagnosing non-immunoglobulin E-mediated gastrointestinal food allergies. FPIES caused by buckwheat is rare; however, we found that the same laboratory results were observed in a comparison of FPIES cases caused by other foods.

Aim: Conventionally, the skeletal muscle area with computed tomography (CT) attenuation ranging from −29 to +150 Hounsfield unit (HU) divided by height squared (the conventional skeletal muscle index [SMI]) was used as an index of skeletal muscle mass. However, it includes fat-infiltrated skeletal muscle, which is known to have poor function. This study aims to determine whether the low-fat SMI, which uses skeletal muscle mass with CT attenuation ranging from +30 to +150 HU, or conventional SMI appropriately reflects the function of skeletal muscle. Methods: We retrospectively analyzed 120 patients with cirrhosis whose handgrip strength was measured. Among them, 48 patients underwent a physical performance assessment such as liver frailty index (LFI) and short physical performance battery (SPPB), and 80 underwent quality of life (QOL) assessment. The relationships between each SMI and handgrip strength, LFI, SPPB, and QOL were evaluated. Results: Low-fat SMI was significantly correlated with handgrip strength (males, R=0.393, p=0.002; females, R=0.423, p

A 13-year-old girl presented with fever for the past ten days without any other symptoms. Laboratory findings revealed leukopenia, low platelet count, and high lactate dehydrogenase and ferritin levels. Abdominal ultrasonography revealed mild splenomegaly and lymphadenopathy in proximity to the head of the pancreas. The patient's condition suggested macrophage activation syndrome associated with systemic juvenile idiopathic arthritis. However, her serum interleukin-18 level was 1743 pg/mL; therefore, urgent systemic corticosteroid treatment was deemed unnecessary as macrophage activation syndrome associated with systemic juvenile idiopathic arthritis was ruled out. The patient was afebrile the following day and showed improved laboratory and ultrasonographic findings without treatment. Clinical Kikuchi-Fujimoto disease was diagnosed based on transient lymphadenopathy, laboratory findings, and a self-limited clinical course. Although this patient met the early diagnostic criteria of macrophage activation syndrome with suspected systemic juvenile idiopathic arthritis, serum interleukin-18 is useful for ruling it out. Pediatricians should keep in mind that Kikuchi-Fujimoto disease may cause abdominal lymphadenopathy.

急性脳症後遺症により医療的ケアを要する重度心身障害児のコロナ病棟管理について報告する．感染初期の一般状態は，在宅管理が可能であった．第8病日に急激な酸素化不良に陥り，胸部CTで両肺野の浸潤影を認め，急性肺障害と診断した．母子同室でコロナ病棟に入院し，人工呼吸器管理下に，レムデシビルとデカドロンの加療を行い，入院17日目に退院した．スタッフは障害児の重症肺炎の管理に際し，薬の投薬時間や医療品の管理法を検討した．感染予防にも留意し，入室とケアの時間を最小限に制約した結果，二次感染者はなかった．濃厚な医療的ケアを要する障害児のコロナ病棟管理では，家族の協力と，現場に合わせた柔軟な工夫が必要であった．, We report that the management for children with severe motor and intellectual disabilities who required medical care in a specialized ward for control the COVID-19 infection. The early stage of the infection could be managed as at outpatient department. On the 8th day, she suffered from respiratory failure. Chest CT showed infiltration shadows in both lungs. Then, she was diagnosed with acute lung injury. She and her mother were admitted to a specialized ward for coronavirus infection. She was successfully treated with remdesivir and dexamethasone, with mechanical ventilation. And she was discharged on the 17th day from the admission. For managing her severe pneumonia in special hospital environment for COVID-19, the medical staff considered how to manage the time of medical care and medical supplies. As a result, we archived attention to preventive infection control and minimizing visits and care times. In the management of the COVID-19 infection in children with neurological sequelae, which requiring medical care, family cooperation and hospital room adaptations are important.

Triple-negative breast cancer (TNBC) is one of the most aggressive breast cancer subtypes, and treatment options are limited because of the lack of signature molecules and heterogeneous properties of cancer. COL8A1 expression is higher in breast cancer than in normal tissues and is strongly correlated with worse overall survival in patients with breast cancer. However, the biological function of COL8A1 on cancer progression is not fully understood. In this study, we investigated the biological function of COL8A1 on TNBC progression.COL8A1-deficient cells were generated using the CRISPR-Cas9 system. The tumor growth and metastasis of TNBC cells were evaluated using three-dimensional culture (3D) methods and xenograft mouse models. The activation of focal adhesion kinase (FAK)/Src by COL8A1 in TNBC cells was evaluated by immunoblotting.COL8A1 expression was primarily distributed into TNBC cell lines. Further, relapse-free survival in TNBC patients with the MSL subtype was strongly associated with the COL8A1 expression. MDA-MB-231 and Hs578T cells, classified as the MSL subtype, strongly express COL8A1, and COL8A1 protein expression was induced by hypoxia in both cell lines. Loss of COL8A1 expression inhibited spheroid /tumor growth and metastasis in vitro and in vivo. Further, exogenous COL8A1 promoted TNBC growth via the FAK/Src activation. Finally, the spheroid growth of MDA-MB-231 and Hs578T cells was inhibited by defactinib, a FAK inhibitor, without cytotoxicity.These results indicate that COL8A1-mediated FAK/Src activation produces a more aggressive phenotype in TNBC, and its target inhibition may be an efficacious treatment for TNBC.

Objective: In patients with liver cirrhosis, the clinical characteristics of dynapenia, a condition in which skeletal muscle mass is maintained but muscle strength is reduced, are not yet known. This study aimed to clarify the characteristics of dynapenia and its impact on quality of life (QOL) in patients with liver cirrhosis. Methods: We retrospectively analyzed 116 patients with cirrhosis. Based on grip strength and skeletal muscle mass measured by the bioelectrical impedance analysis method, patients were divided into four groups: normal muscle status, dynapenia, pre-sarcopenia (a condition involving only low muscle mass), and sarcopenia. The characteristics of dynapenia and its influence on QOL were examined. Results: Fourteen patients had dynapenia. Liver function did not differ among the four groups. In patients with dynapenia, BMI was highest and computed tomography attenuation of skeletal muscle at the third lumbar spine vertebra was lowest among the four groups. The percentage of patients with both BMI ≥25 kg/m2 and myosteatosis was significantly higher in patients with dynapenia [9/14 (64.3%)] than in those with sarcopenia [2/23 (8.7%), P = 0.004] and pre-sarcopenia [0/18 (0%), P < 0.001] and tended to be higher than those with normal muscle status [16/61 (26.2%), P = 0.065]. The physical QOL in patients with dynapenia was as low as that in those with sarcopenia and significantly lower than that in those with normal muscle status. Conclusion: Cirrhotic patients with dynapenia had high BMI and myosteatosis, and impaired physical QOL.

Bronchomotor tone is regulated by contraction and relaxation of airway smooth muscle (ASM). A weakened ASM relaxation might be a cause of airway hyperresponsiveness (AHR), a characteristic feature of bronchial asthma. Pituitary adenylyl cyclase-activating polypeptide (PACAP) is known as a mediator that causes ASM relaxation. To date, whether or not the PACAP responsiveness is changed in asthmatic ASM is unknown. The current study examined the hypothesis that relaxation induced by PACAP is reduced in bronchial smooth muscle (BSM) of allergic asthma. The ovalbumin (OA)-sensitized mice were repeatedly challenged with aerosolized OA to induce asthmatic reaction. Twenty-four hours after the last antigen challenge, the main bronchial smooth muscle (BSM) tissues were isolated. Tension study showed a BSM hyperresponsiveness to acetylcholine in the OA-challenged mice. Both quantitative RT-PCR and immunoblot analyses revealed a significant decrease in PAC1receptor expression in BSMs of the diseased mice. Accordingly, in the antigen-challenged group, the PACAP-induced PAC1receptor-mediated BSM relaxation was significantly attenuated, whereas the relaxation induced by vasoactive intestinal polypeptide was not changed. These findings suggest that the relaxation induced by PACAP is impaired in BSMs of experimental asthma due to a downregulation of its binding partner PAC1receptor. Impaired BSM responsiveness to PACAP might contribute to the AHR in asthma.

急性巣状細菌性腎炎（AFBN）と可逆性脳梁膨大部病変を有する軽症脳炎・脳症（MERS）の合併例を報告する．症例1は3歳男児．発熱と異常言動で入院し，血液検査でCRP 上昇と膿尿があり抗菌薬を開始．第3病日に腎超音波で高エコー域を認めMRI拡散強調画像（DWI）でAFBNと診断．解熱後も意識障害が遷延し，脳MRI精査でMERSと診断．ステロイドパルス療法後に意識レベルは改善した．症例2は3歳男児．発熱，幻視，けいれんで入院，血液検査でCRP上昇と膿尿があり抗菌薬を開始．第2病日にMRIを施行しAFBNとMERSを診断，ステロイドパルス療法にて症状は改善した．尿路感染症に中枢神経症状を伴う小児では，AFBN とMERSを念頭に腎臓と脳のMRI-DWIを施行することを考慮すべきである．

2019年12月以降，中国湖北省武漢より広まった新型コロナウイルス（severe acute respiratory syndrome coronavirus 2；SARS-CoV-2）による感染症（coronavirus disease 2019；COVID-19）の流行により，2020年5月末時点で全世界で33万人を超える死者が報告された．致死率は高齢者や基礎疾患を有する患者に高く，現時点で有効な治療法は確立していない．また急務とされるワクチンの開発も実現していない．人々は集団感染を予防するため世界の各都市で都市封鎖や自粛生活を余儀なくされた．更なる感染拡大の防止に向けて，成人のCOVID-19においては既存の治療薬を応用した対症療法の確立が試みられている．しかし小児の治療法に関する報告は少ない．本稿では小児のCOVID-19の治療になりえる薬剤や投与方法について国内外の知見を参考に，当院小児科における治療方針（案）を定めた．, Since December 2019 , coronavirus disease 2019（COVID-19）caused by the new coronavirus（severe acute respiratory syndrome coronavirus 2；SARS-CoV-2）has been spread from Wuhan, Hubei Province, China. As of the end of May 2020, more than 330,000 deaths were reported all over the world. The mortality rate is especially high among the elderly and patients with underlying diseases. However, the effective treatment for COVD-19 has not been established. In addition, no effective vaccine has been developed against COVID-19. To prevent the COVID-19 outbreaks, people became self-restrained in daily life by lock down cities all around the world. Clinically, various therapies have been tried in adult patients of COVID-19 to prevent further outbreaks. On the other hand, there are few reports for the treatment of pediatric COVID-19 patients. In this paper, we proposed treatment strategies for pediatric patients with COVID-19 in the Department of Pediatrics, Dokkyo Medical University Hospital.

An 8-year-old boy had undergone permanent epicardial pacemaker implantation with a Y-shaped bipolar ventricular lead on day 6 after birth for treatment of congenital complete atrioventricular block. He was found to have pulmonary stenosis and mitral stenosis by follow-up echocardiography. Further studies including computed tomography and cardiac catheterization revealed that the pacemaker lead had completely encircled the cardiac silhouette and was in a state of “cardiac strangulation”. We removed the previous pacing leads and generator and implanted a new epicardial dual-chamber pacing system in the right atrium and right ventricle. Additionally, an expanded polytetrafluoroethylene sheet was placed between the new leads and the heart to prevent recurrence of cardiac strangulation.

Augmented smooth muscle contractility of the airways associated with an increased expression of RhoA, a monomeric GTPase responsible for CaCultured human BSM cells were transfected with MALAT1 antisense oligonucleotide (AS), miR-133a-3p mimic, and/or inhibitor, and then stimulated with interleukin-13 (IL-13). In animal experiments, the ovalbumin (OA)-sensitized mice were repeatedly challenged with aerosolized OA to induce asthmatic reaction.Treatment of the cells with IL-13 induced an increase in RhoA protein. Either MALAT1 AS or miR-133a-3p mimic transfection inhibited the IL-13-induced upregulation of RhoA. The inhibitory effect of MALAT1 AS was abolished by co-transfection with miR-133a-3p inhibitor. In BSMs of the murine asthma model, upregulations of Malat1 and RhoA protein were observed concomitantly with downregulation of miR-133a-3p.These findings suggest that MALAT1 positively regulates RhoA protein expression by inhibiting miR-133a-3p in BSM cells, and that its upregulation causes the RhoA upregulation, resulting in an augmented BSM contractility.

Aim Sarcopenia is associated with poor health-related quality of life (HRQOL) in the general population. However, in cirrhotic patients, as the development of sarcopenia is closely related to declined liver function, which also impairs HRQOL, whether sarcopenia deteriorates HRQOL independently from declined liver function remains unclear. The aim of this study was to clarify the impact of sarcopenia on HRQOL impairment in cirrhotic patients. Patients and methods A total of 88 cirrhotic patients [median age, 69 years; range: 31-79 years; 49 male (55.7%), 45 with hepatocellular carcinoma (51.1%)] were analyzed. We measured HRQOL using the 36-item Short-Form Health Survey version 2 questionnaire and identified factors contributing to scores lower than 50 in physical component summary (PCS), mental component summary, and role-social component summary (RCS) scores. Results Twenty-four (27.2%) patients had sarcopenia. PCS and RCS scores were significantly lower in patients with sarcopenia compared with those without sarcopenia. Patients with Child-Pugh (CP) classification B or C showed significantly lower scores in PCS and RCS than those with CP classification A. On multivariate analysis, the presence of sarcopenia was the only factor associated with low PCS scores [odds ratio (OR): 11.6; P = 0.031]. Female sex (OR: 3.34; P = 0.034), CP classification B or C (OR: 3.19; P = 0.037), and presence of sarcopenia (OR: 4.64; P = 0.016) were identified as independent factors for low RCS scores. Conclusion Sarcopenia independently impairs physical and role-social HRQOL in cirrhotic patients.

Mycotic aortic aneurysm (MAA) is rare but lethal. Detection of the causative bacteria is important for successful treatment. In some cases, however, no micro-organisms are detected by repeated blood cultures. Open surgery is the standard procedure for MAA, but endovascular intervention is also performed. An aneurysm specimen cannot be taken for culture when using an endovascular approach, decreasing the opportunity to detect the causative organism. We performed successful endovascular repair in a rare case of a Peptostreptococcus micros-induced MAA as detected by postoperative computed tomography-guided biopsy. This case may help to establish appropriate medical treatment for MAAs. Keywords: Mycotic aneurysm, Endovascular, Stent graft, Peptostreptococcus micros, Computed tomography-guided biopsy

We aim to clarify the efficacy of early palliative balloon pulmonary valvuloplasty (BPV) in neonates and young infants (

Progressive dilatation of the pulmonary autograft is one of the greatest concerns after the Ross procedure. Increased stress in the arterial wall may cause changes in the elastic properties of the pulmonary autograft, and thus lead to pathological dilatation. The present study aimed to investigate the changes in the autograft diameter and stiffness during follow-up after the Ross procedure. A total of ten patients underwent the Ross procedure at our institution between 2003 and 2011. Echocardiography was used to measure the diameters of the pulmonary autograft at the level of the annulus, sinus of Valsalva, and sinotubular junction. The stiffness index was calculated from the angiographic data, and compared with that of 16 age-matched control children. The diameters of the pulmonary autograft increased throughout the follow-up period, particularly at the level of the sinus of Valsalva and at the sinotubular junction. The aortic root was stiffer in Ross patients compared with control children (7.9 ± 1.8 vs. 3.9 ± 0.7 immediately postoperatively, p

The family of staphylococcal superantigen-like proteins (SSLs) have a structure similar to bacterial superantigens but exhibit no superantigenic activity. These exoproteins have recently been shown to disturb the host immune defense system. One family member, SSL5, was reported to bind to human leukocyte P-selectin glycoprotein ligand-1 (PSGL-1) and matrix metalloproteinase-9 (MMP-9) and to interfere with leukocyte trafficking. In the present study, we explored human plasma proteins bound by glutathione S-transferase (GST)-tagged recombinant SSL5 (GST-SSL5) and identified plasma protease C1 inhibitor (C1Inh) as a major SSL5-binding protein based on the results of peptide mass fingerprinting analysis with MALDI-TOFMS. GST-SSL5 was found to attenuate the inhibitory activity of recombinant histidine-tagged C1Inh (C1Inh-His) toward complement C1s. We also observed that the treatment of C1Inh-His with neuraminidase markedly decreased its binding to GST-SSL5. Moreover, C1Inh-His produced by Lec2 mutant cells (deficient in sialic acid biosynthesis) showed much lower binding affinity for SSL5 than that produced by the wild-type CHO-K1 cells, as assessed by pull-down assay. These results suggest that SSL5 binds to C1Inh in a sialic acid-dependent fashion and modulates the host immune defense through perturbation of the complement system in association with S. aureus infection.

The current study aimed to determine the role of a microRNA (miRNA), miR-140-3p, in the control of RhoA expression in bronchial smooth muscle cells (BSMCs). In cultured human BSMCs, incubation with interleukin-13 (IL-13) caused an up-regulation of RhoA protein concurrently with a down-regulation of miR-140-3p. Transfection of the cells with a miR-140-3p inhibitor caused an increase in basal RhoA protein level. Although a mimic of miR-140-3p had little effect on the basal RhoA level, its treatment inhibited the IL-13-induced up-regulation of RhoA. These findings suggest that RhoA expression is negatively regulated by miR-140-3p, and that the negative regulation is inhibited by IL-13 to cause an up-regulation of RhoA protein in BSMCs.

Left pulmonary artery stenosis is a well-recognized complication following the Norwood procedure. We herein report two cases in which ascending aortic extension was performed to enlarge the retroaortic space in children with left pulmonary artery stenosis after the Norwood procedure. We used graft interposition in the ascending aorta to increase the retroaortic apace and concomitantly performed extended left pulmonary artery reconstruction. This procedure obtains a more balanced distribution of the pulmonary blood flow, which is crucial to achieve good Fontan circulation.

Five species of decapod crustaceans, including Calliax nishiki sp. nov. (Axiidea, Eucalliacidae) and Carcinoplax longimana (de Haan), are described from the upper part of the Moeshima Silt Bed (latest Pleistocene to Holocene: ca 13,000 to 8,000 cal BP). Ten decapods, including Laticallichirus grandis (Karasawa & Goda) and Ebalia tuberculosa (A. Milne-Edwards) are described from the Moeshima Shell Bed (Holocene: ca 8,000 to 2,000 cal BP). Calliax nishiki is abundant and Carcinoplax longimana is common in the decapod assemblage of Moeshima Silt Bed. The new species of Calliax seems to have dispersed around the deep marine setting with a reducing environment. The decapod assemblage of the Moeshima Shell Bed is characterized by a predominance of L. grandis. The present record suggests that L. grandis is abundant under sandy and gravelly bottoms mixed with shells of the lower sublittoral to subtidal zones during the Quaternary.

Histopathological assessment of the pulmonary arteries is crucial to determine the surgical indications in patients with congenital heart disease (CHD) and intractable pulmonary vascular disease (PVD). We aimed to clarify whether pulmonary hemodynamic parameters can predict PVD in patients with CHD and pulmonary arterial hypertension (PAH) We performed histopathological evaluations of lung specimens and cardiac catheterizations in 27 patients with CHD–PAH. We divided these patients into the patients with and without PVD, and compared pulmonary hemodynamic parameters including pulmonary arterial compliance (Cp) between two groups. Age at lung biopsy was 4 (2–7) months. There were 16 patients with trisomy 21. Cardiac diagnosis included ventricular septal defect in 16, atrial septal defect in 5, atrioventricular septal defect in 4, and others in 2. There were 11 patients with histopathologically proven PVD (Heath–Edwards classification grade ≥ 3 in 5; the index of PVD ≥ 1.1 in 3; extremely thickened media in 6; hypoplasia of the pulmonary arteries in 3). Cp in the patients with PVD was significantly lower than that in patients without PVD (0.99 [0.74–1.42] vs 1.56 [1.45–1.88], p = 0.0047), although there was no significant difference in the ratio of systemic to pulmonary blood flow, pulmonary arterial pressure, and resistance between two groups. A Cp cutoff value of