Clinicopathological features of adult T-cell leukemia/lymphoma with HTLV-1-infected Hodgkin and Reed-Sternberg-like cells.

Hodgkin and Reed-Sternberg (HRS) cells, a hallmark of classic Hodgkin lymphoma (CHL), are occasionally detected in non-Hodgkin lymphomas, including adult T-cell leukemia/lymphoma (ATLL), a lymphoid neoplasm caused by human T-cell leukemia virus type 1 (HTLV-1). HRS-like cells associated with ATLL have been described to be of B-cell lineage and infected with Epstein-Barr virus (EBV), not HTLV-1. We herein describe clinicopathological findings in 8 cases (4 males and 4 females; median age, 73 years [range, 55-81 years]) of ATLL with HTLV-1-infected HRS-like cells identified by ultrasensitive RNA in situ hybridization for HTLV-1 basic leucine zipper factor (HBZ-ISH), a specific viral transcript of HTLV-1.

Impact of the one-carbon metabolism on oocyte maturation, fertilization, embryo quality, and subsequent pregnancy.

Purpose: To investigate impact of the one-carbon metabolism (OCM) on oocyte maturity and embryo development.

Methods: This prospective study analyzed 18 women who agreed to participate. We measured the OCM biomarkers' concentrations including Vitamin B12 (VB12), folic acid (FA), and homocysteine (Hcy) in serum and follicular fluid (FF), and assessed their correlation.

Natural History of Unruptured Visceral Artery Aneurysms Due to Segmental Arterial Mediolysis and Efficacy of Transcatheter Arterial Embolization: A Retrospective Multiinstitutional Study in Japan.

The purpose of this study was to clarify the natural history of unruptured visceral artery aneurysms due to segmental arterial mediolysis and the efficacy of transcatheter arterial embolization. Patients with a pathologic or clinical diagnosis of visceral artery aneurysms due to segmental arterial mediolysis between 2005 and 2015 were enrolled. For patients with clinical diagnoses, images were collected and assessed by central radiologic review.

Arterial switch operation for Taussig-Bing anomaly with aortic arch obstruction.

Introduction: The short-term survival rate after single-stage correction of Taussig-Bing anomaly with aortic arch obstruction remains favorable. However, some cases are encountered occasionally in which single-stage correction was not performed during the neonatal period. Accordingly, we evaluated the midterm outcomes of different surgical strategies.

Guidance regarding COVID-19 for survivors of childhood, adolescent, and young adult cancer: A statement from the International Late Effects of Childhood Cancer Guideline Harmonization Group.

Childhood, adolescent, and young adult (CAYA) cancer survivors may be at risk for a severe course of COVID-19. Little is known about the clinical course of COVID-19 in CAYA cancer survivors, or if additional preventive measures are warranted. We established a working group within the International Late Effects of Childhood Cancer Guideline Harmonization Group (IGHG) to summarize existing evidence and worldwide recommendations regarding evidence about factors/conditions associated with risk for a severe course of COVID-19 in CAYA cancer survivors, and to develop a consensus statement to provide guidance for healthcare practitioners and CAYA cancer survivors regarding COVID-19.

Co-occurrence of EBV-positive classic Hodgkin lymphoma and B-cell lymphomas of different clonal origins: A case report and literature review.

Although cases with metachronous or synchronous co-occurrence of classic Hodgkin lymphoma (CHL) and B-cell non-Hodgkin lymphoma (B-NHL) have been reported, few reports have analyzed the clonal relationship between both lesions in detail, especially in Epstein-Barr virus (EBV)-positive settings. Here, we report a case of a 38-year-old male with CHL, followed by the recurrence of EBV-positive mucocutaneous ulcers of the large intestine and EBV-positive diffuse large B-cell lymphoma in the liver. Surprisingly, polymerase chain reaction analysis for immunoglobulin heavy chain gene rearrangement revealed that all lesions were clonally distinct.

A new diagnostic algorithm using biopsy specimens in adult T-cell leukemia/lymphoma: combination of RNA in situ hybridization and quantitative PCR for HTLV-1.

Histopathological distinction between adult T-cell leukemia/lymphoma (ATLL) and other T-cell neoplasms is often challenging. The current gold standard for the accurate diagnosis of ATLL is the Southern blot hybridization (SBH) assay, which detects clonal integration of human T-cell leukemia virus type I (HTLV-1) provirus. However, SBH cannot be performed with small biopsy or formalin-fixed paraffin-embedded (FFPE) tissue samples because this assay requires a large amount of DNA without degradation.

Retrospective study of the renal function using estimated glomerular filtration rate and congenital anomalies of the kidney-urinary tract in pediatric Turner syndrome.

Although Turner syndrome (TS) is frequently associated with congenital anomalies of the kidney-urinary tract (CAKUT), which is a major cause of pediatric chronic kidney disease, renal function in TS is usually considered normal. The present study aimed to analyze the frequency of renal dysfunction and CAKUT in pediatric patients with TS. Our study included 122 patients with TS between the ages of 2 and 18 years from 30 hospitals across Japan.

A case with somatic and germline mosaicism in COL4A5 detected by multiplex ligation-dependent probe amplification in X-linked Alport syndrome.

X-linked Alport syndrome (XLAS) is a progressive hereditary kidney disease caused by mutations in the COL4A5 gene encoding the type IV collagen α5 chain. To date, 11 cases having somatic mosaic variants in COL4A5 have been reported; however, all of them involved single-nucleotide variations (SNVs). Here, we report a female XLAS patient with somatic mosaicism identified by copy number variation (CNV) in COL4A5.

Risk factors of recurrence after postoperative electron beam radiation therapy for keloid: Comparison of long-term local control rate.

Aim: To investigate the new risk factors for keloid recurrence after postoperative electron beam radiotherapy (RT) and evaluate the effectiveness of tranilast in combination with electron beam RT by comparing the local control rate.

Background: Identifying patients at high risk of recurrence after postoperative RT for keloids remains a challenge. Besides, no study examined the effectiveness of tranilast in combination with RT after surgery for the prevention of keloids recurrence.

Evaluation of an Integrated Device Diagnostics Algorithm to Risk Stratify Heart Failure Patients - Results From the SCAN-HF Study.

Background: Integrated device diagnostics, Triage-HF, is useful in risk stratifying patients with heart failure (HF), but its performance for Japanese patients remains unknown. This is a prospective study of Japanese patients treated with a cardiac resynchronization therapy defibrillator (CRT-D), with a Medtronic OptiVol 2.0 feature.

A novel homozygous missense mutation in three CACT-deficient patients: clinical and autopsy data.

Carnitine-acylcarnitine translocase (CACT) deficiency is a fatty acid ß-oxidation disorder of the carnitine shuttle in mitochondria, with a high mortality rate in childhood. We evaluated three patients, including two siblings, with neonatal-onset CACT deficiency and revealed identical homozygous missense mutations of p.Arg275Gln within the gene.

Complete Remission of Multiple Brain Metastases in a Patient with -Mutated Non-Small-Cell Lung Cancer Treated with First-Line Osimertinib without Radiotherapy.

Osimertinib has demonstrated efficacy against stable or asymptomatic central nervous system (CNS) metastases of epidermal growth factor receptor () mutation-positive non-small-cell lung cancer (NSCLC) in phase 2 and 3 clinical trials that allowed prior CNS radiotherapy. However, the efficacy of osimertinib only or the optimal treatment combination or sequence of radiotherapy has not been investigated. A 74-year-old woman diagnosed with T4N1M1c Stage IVB lung adenocarcinoma with mutation presented with a left upper lobe mass and multiple bilateral lung metastases.

Dermatopathic reaction of lymph nodes in HTLV-1 carriers: a spectrum of reactive and neoplastic lesions.

Aims: Dermatopathic reaction is a histopathological finding of lymph nodes that usually occurs in patients with inflammatory pruritic cutaneous lesions. However, it is sometimes seen in patients with cutaneous T cell lymphoma. Adult T cell leukaemia/lymphoma (ATLL) is a T cell malignancy caused by infection with human T cell leukaemia virus type I (HTLV-1), which is frequently accompanied by cutaneous lesions.

A significance of school screening electrocardiogram in the patients with ventricular noncompaction.

Left ventricular noncompaction (LVNC) is a hereditary cardiomyopathy and is associated with high morbidity and mortality. However, the role and significance of school screening for LVNC have not been fully elucidated. In this multicenter, retrospective cohort study, a total of 105 children with LVNC were included from 2000 to 2017.

Beating heart thoracic aortic surgery under selective myocardial perfusion for patients with congenital aortic anomalies.

Objective: We evaluated beating heart thoracic aortic surgery (BHTAS) using selective myocardial perfusion (SMP) in patients with aortic anomalies with complex surgical needs.

Methods: Between 2012 and 2018, 27 infants with aortic anomalies underwent BHTAS using SMP.

Results: Median body weight was 3.

Successful Treatment of Anti-Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome.

Anti-complement factor H (CFH) autoantibody (Ab)-associated atypical hemolytic uremic syndrome (aHUS) has a poor prognosis in terms of frequent relapses. Although eculizumab is an effective treatment for this type of aHUS, the method of eculizumab discontinuation is not yet established. Herein, we report a case of anti-CFH Ab-associated aHUS in a 6-year-old boy.

Supplementation of Colostrum and Breast Milk Enhances Weight Gain and Metabolic Responses Associated with Microbiota Establishment in Very-Preterm Infants.

Background: Postnatal growth restriction in very-preterm infants (VPIs) may have long-lasting effects. Recent evidence suggests that developmental problems in VPIs are related to abnormalities in intestinal microbial communities.

Objective: To investigate the effect on growth outcomes in VPIs of supplementation with along with mother's colostrum and breast milk.

Stanford type IV venous collateral blood flow following complete chronic occlusion of the superior vena cava in a patient with lung cancer.

In superior vena cava occlusion, multiple collateral pathways develop to maintain venous drainage. Major patterns and pathways of venous collateral blood flow are well described, but rarely in complete chronic superior vena cava occlusion secondary to malignancy. A 59-year-old man with facial and upper extremity edema had a severely compressed superior vena cava at the initial diagnosis of stage IV mediastinal lung adenocarcinoma.

A case of pheochromocytoma presenting with cardiopulmonary arrest.

A 33-year-old woman complained of sudden chest pain and intense headache. She was unconscious and underwent defibrillation for ventricular fibrillation in the ambulance. In the emergency room, she was placed on an artificial respirator.