A Case of X-Linked Agammaglobulinemia and COVID-19 in a Japanese Infant.

An infant was diagnosed as having X-linked agammaglobulinemia (XLA) at age 3 months and was receiving immunoglobulin replacement therapy. He developed SARS-CoV-2 infection at age 7 months and was treated with intravenous immunoglobulin, remdesivir, and dexamethasone. His respiratory symptoms improved quickly, and the infection resolved.

Pediatric Retroperitoneal Paraganglioma Invading the Inferior Vena Cava: A Case Report and Literature Review.

Paraganglioma, a catecholamine-producing tumor originating in extra-adrenal paraganglion cells, is rare in children. Although diagnosis of paraganglioma is based on the presence of catecholamine symptoms, some patients lack such symptoms. Delayed diagnosis is associated with tumor growth and invasion of surrounding vessels.

Immunological role of zinc in preterm neonates.

Zinc (Zn), an essential trace element, plays a significant role in fetal development and biological defense during the embryonic and neonatal periods. Therefore, exploring the kinetics of Zn related to immune disturbances in preterm neonates is important. We here performed the measurement of Zn concentration along with immunological analysis of neonates and investigated the role of Zn in the neonatal period.

Use of tocilizumab to treat arthritis associated with mixed connective tissue disease complicated by ovarian teratoma: a case report.

Mixed connective tissue disease (MCTD) is characterized by mixed features of systemic lupus erythematosus, systemic sclerosis, and polymyositis/dermatomyositis and is rare in children. Here, we report a case of MCTD in a 10-year-old girl who presented at our hospital with arthralgia, Raynaud's phenomenon, and fatigue. Blood tests were positive for anti-U1-ribonucleoprotein (RNP) antibodies and for rheumatoid factors (RFs) IgG-RF and anti-galactose-deficient IgG.

Intake levels and main sources of nutrients for Japanese children with motor or intellectual disabilities.

Proper nutritional management is important for the growth and development of children with motor or intellectual disabilities; however, few studies have investigated the nutrient intake of children with disabilities. This study aimed to investigate the nutrient intake and food groups that are the main sources of nutrients for children with disabilities. This cross-sectional observational study included twenty-five children (mean age, 11⋅8 years) from five hospitals in Japan.

Diagnosis and clinical management of pneumonia in a patient with anorexia nervosa: A case report.

We report a patient with anorexia nervosa without bronchiectasis and cystic fibrosis who developed acute pneumonia caused by (). The black fungus found in multiple sputum cultures was determined to be using mass spectrometry and identified using genetic analysis. Although the initiation of antifungal therapy was late, the pneumonia gradually improved with long-term treatment.

Proctoring System of Pediatric Laparoscopic Surgery for Choledochal Cyst.

Endoscopic surgery also has been becoming widespread in the field of pediatric surgery. However, most disease treated by pediatric surgery in a single institution are small number of cases. Besides, the variety of operative procedures that need to be performed in this field is quite wide.

Plasma-derived factor VIIa and factor X mixture agent (MC710) prophylaxis in haemophilia B patients with inhibitors.

Introduction: Haemophilia B patients with factor IX inhibitors have particularly unmet needs for conventional therapy.

Aim: Phase II/III clinical trial, multicentre, open-label, prospective, self-controlled study was conducted to assess MC710 prophylaxis in haemophilia B patients with inhibitors.

Methods: We enrolled haemophilia patients who had received episodic or prophylactic treatment with bypassing agents up to that time.

Incidence of testicular microlithiasis after orchidopexy for cryptorchidism and its risk factors: A retrospective cohort study.

Introduction: Testicular microlithiasis (TML) is detected using scrotal ultrasound (US) and is characterized by hyperechoic non-shadowing foci 1-3 mm in diameter within the testicular parenchyma. Although a history of orchidopexy is a known risk factor for TML, and TML is a relatively common condition in postoperative cryptorchidism patients, no previous report has focused on risk factors for TML after orchidopexy.

Objective: The aims of this study were to evaluate the time-dependent incidence of TML after orchidopexy for cryptorchidism and to identify risk factors for TML.

Role of a Fetal Ultrasound Clinic in Promoting Multidisciplinary and Inter-Facility Perinatal Care.

Background: With the increasing rate of high-risk pregnancies, there is an increased need for early evaluation of at-risk fetuses. Fetal ultrasound imaging has become a pivotal part of this evaluation.

Methods: To evaluate the role played by a fetal ultrasound clinic in promoting comprehensive perinatal care of patients with high-risk pregnancies, we retrospectively analyzed the indications and findings of fetal scans and the outcomes of the examined fetuses collected over the past 7 years (2014-2020) by our institute, which is reorganized as a perinatal medical center.

Paroxysmal sympathetic hyperactivity and the later development of epilepsy in a chemotherapy-associated brain damage.

Background: Chemotherapy in childhood leukemia potentially induces brain lesions and neurological sequelae. Paroxysmal sympathetic hyperactivity (PSH) is known as a treatment-associated complication; however, the full clinical spectra of PSH remain to be elusive.

Case Report: A 5-year-old girl was diagnosed of acute myeloid leukemia (AML) M5.

Hepatoblastoma with multiple tumors in a school-aged child.

Clinicians need to consider hepatoblastoma in the differential even in school-aged children or adolescents presenting with multiple liver tumors.

Statistical Evaluation of the First Year of a Neonatal Intensive Care Unit Established in a Medical School Hospital.

Background: There has been significant progress in reducing perinatal mortality in Japan. However, due to changes in social conditions, the total fertility rate and the number of births are decreasing, whereas the number of low birth weight infants is increasing along with the number of newborn babies that require intensive care. Further, although the number of high-level perinatal medical centers has increased, so has that of infants who need long-term hospitalization.

Mind as a Behavioral Inhibition Network.

This study aimed to propose to add a new perspective on what may create the impression of "mind" in other beings. The conventional is perspective is that when we observe mental activities in animals, this creates in us the impression that they have a mind. On the other hand, the authors' proposal is that when we observe unpredictable activities in living beings, this creates in us the impression of mind.

Impact of immunophenotypic characteristics on genetic subgrouping in childhood acute lymphoblastic leukemia: Tokyo Children's Cancer Study Group (TCCSG) study L04-16.

Immunophenotyping was performed in 1044 consecutive childhood acute lymphoblastic leukemia (ALL) patients enrolled in the Tokyo Children's Cancer Study Group L04-16 trial, revealing novel findings associated with genetic abnormalities. In addition to TCF3-PBX1 and MEF2D fusions, the CD10 subtype of KMT2A-MLLT3-positive ALL frequently exhibited the cytoplasmic-μ pre-B ALL immunophenotype. Although ETV6-RUNX1 was significantly correlated with myeloid antigen expression, more than half of patients expressed neither CD33 nor CD13, while the CD27 /CD44 immunophenotype was maintained.

Efficacy and safety of intravenous thiamylal in pediatric procedural sedation for magnetic resonance imaging.

Objective: To evaluate the efficacy and safety of intravenous (i.v.) thiamylal in pediatric magnetic resonance imaging (MRI) sedation.

Interfacility Neonatal Transport for Convalescent Care: Improving Regionalized Care.

Background: Transfer of infants who no longer need intensive or specialized care from tertiary to community hospitals or clinics contributes to efficient bed utilization in neonatal intensive care units (NICUs).

Methods: We retrospectively analyzed the records of all 1,503 infants admitted to our NICU during the 6-year period from April 2013 through March 2019 to evaluate the impact of interfacility neonatal transport for convalescent care.

Results: During the study period, our NICU accepted 33 infants from other tertiary NICUs and transferred 103 infants to other hospitals or clinics before their home discharge for convalescent care.

Changes in Cytokine Profile during Initial Treatment of Pediatric Hemophagocytic Lymphohistiocytosis Associated with Epstein-Barr Virus.

Hemophagocytic lymphohistiocytosis (HLH) associated with Epstein-Barr virus (EBV) infection can be self-limiting, severe/aggressive, or fatal. We report a case of EBV-HLH with persistent fever, severe pancytopenia, hypertriglyceridemia, and hypofibrinogenemia in a 4-year-old boy. Levels of plasma cytokines and chemokines were measured with a Bio-Plex system at 1, 2, 3, 4, 5, and 8 days after hospital admission.