Association between antinuclear antibodies status and preterm birth in Japanese pregnant women: a prospective cohort study from Adjunct Study of the Japan Environment and Children's Study.

Background: Antinuclear antibodies (ANA) are important biomarkers for the diagnosis of autoimmune diseases; however, the general population also tests positive at a low frequency, especially in women. Although the effects of various autoimmune diseases on pregnancy outcomes have been studied, the association of ANA with pregnancy outcomes in healthy individuals is unclear. Preterm birth (PTB), a major cause of neonatal death or long-term health problems, is a complex condition with a multifactorial etiology, and the underlying mechanism remains unclear.

Reflecting on a decade of the international consensus on ANA patterns (ICAP): Accomplishments and challenges from the perspective of the 7th ICAP workshop.

The International Consensus on ANA Patterns (ICAP) is an ongoing international initiative dedicated to harmonizing technical and interpretation aspects of the HEp-2 IFA test. Comprised of internationally recognized experts in autoimmunity and HEp-2 IFA testing, ICAP has operated for the last 10 years by promoting accurate reading, interpretation, and reporting of HEp-2 IFA images by professionals involved in various areas related to autoimmune diseases, such as clinical diagnostic laboratories, academic research, IVD industry, and patient care. ICAP operates through continuous information exchange with the international community and encourages the participation of younger experts from all over the world.

A Case of Hyperglycemia-Induced Epileptic Homonymous Hemianopsia.

Hyperglycemia sometimes initially presents with neurological manifestations, including seizures, visual hallucinations, choreoathetosis, hemiballismus, myoclonus, tremor, and consciousness disturbance. Epileptic seizures induced by hyperglycemia are reported to occur predominantly in the occipital lobe, and the epileptic form is mainly epilepsia partialis continua. Of the two patterns of hyperglycemia (ketotic or nonketotic), nonketotic hyperglycemia is more commonly associated with seizures because ketosis has an anticonvulsive effect, so hyperglycemia-induced seizures are generally seen in nonketotic patients.

A survey of the current status of neonatal disseminated intravascular coagulation in neonatal intensive care units in Kyushu, Japan.

Background: Neonatal disseminated intravascular coagulation (DIC) is a rare disease with a poor outcome. However, data on the incidence, treatment, and outcome of neonatal DIC are scarce. Thus, this study investigated the status of neonatal DIC in Japan.

Anti-SMN autoantibodies in mixed connective tissue disease are associated with a severe systemic sclerosis phenotype.

Objectives: The survival of motor neuron (SMN) complex has an essential role in the assembly of small nuclear ribonucleoproteins (RNP). Recent reports have described autoantibodies (aAbs) to the SMN complex as novel biomarkers in anti-U1RNP+ myositis patients. The aim of this study was to compare phenotypic features of anti-U1RNP+ mixed connective tissue disease (MCTD) patients with and without anti-SMN aAbs.

Update on autoantibodies and related biomarkers in autoimmune inflammatory myopathies.

Purpose Of Review: This manuscript reviews recently published advances in the identification of autoimmune inflammatory myopathies (AIM)-specific and AIM-related autoantibodies considered of value in the workup of patients suspected of having AIM. Newer autoantibodies, developments, and advances in the methodology of testing, the gaps and pitfalls in using these assays as diagnostic biomarkers, and the importance of considering overlap diseases and unique clinical AIM phenotypes are discussed.

Recent Findings: a).

Anti-survival motor neuron complex antibodies as a novel biomarker for pulmonary arterial hypertension and interstitial lung disease in mixed connective tissue disease.

Objective: The presence of anti-U1 RNP antibodies (Abs) is critical for diagnosing MCTD. The aim of this study is to evaluate the clinical relevance of anti-survival motor neuron (SMN) complex Abs, which often coexist with anti-U1 RNP Abs.

Methods: A total of 158 newly diagnosed consecutive cases of SLE, SSc or MCTD with anti-U1 RNP Abs were enrolled in this multicentre observational study between April 2014 and August 2022.

Detection of antinuclear antibodies: recommendations from EFLM, EASI and ICAP.

Objectives: Antinuclear antibodies (ANA) are important for the diagnosis of various autoimmune diseases. ANA are usually detected by indirect immunofluorescence assay (IFA) using HEp-2 cells (HEp-2 IFA). There are many variables influencing HEp-2 IFA results, such as subjective visual reading, serum screening dilution, substrate manufacturing, microscope components and conjugate.

Clinical significance of serum autoantibodies in patients with sarcoidosis.

Background: The association between sarcoidosis and autoimmunity has been reported for years. However, the significance of autoantibodies in the pathophysiology and clinical management of sarcoidosis is not well understood. No autoantibodies that can be used as serologic biomarkers to diagnose the disease, monitor the state of the disease, and predict the prognosis of patients are established.

Deciphering the Autoantibody Response to the OJ Antigenic Complex.

(1) Background: Myositis specific antibodies (MSA) are important diagnostic biomarkers. Among the rarest and most challenging MSA are anti-OJ antibodies which are associated with anti-synthetase syndrome (ASS). In contrast to the other tRNA synthetases that are targets of ASS autoantibodies (e.

Association between antinuclear antibody positivity and chemical exposure among pregnant Japanese women: A cross-sectional study based on the Japan environment and Children's study.

Antinuclear antibodies (ANAs) are detected in healthy individuals, they are more prevalent in women than in men. Pregnant women are immunologically unique, but epidemiological data on ANA positivity in them remain limited. The exposure received from the mother during the fetal period impacts the future health of the fetus and has thus received increased attention in recent years.

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.

Positive Autoantibody Is Associated with Malignancies in Patients with Idiopathic Interstitial Pneumonias.

Various autoantibodies are associated with clinical outcomes in patients with idiopathic interstitial pneumonias (IIPs). We retrospectively analyzed the association between autoantibodies and malignancies in IIP patients. Comprehensive analyses of autoantibodies were performed using immunoprecipitation and enzyme-linked immunosorbent assays in 193 consecutive IIP patients.

A cell-based assay for detection of anti-fibrillarin autoantibodies with performance equivalent to immunoprecipitation.

Anti-fibrillarin autoantibodies are useful for the diagnosis and prognosis of systemic sclerosis (SSc). Anti-fibrillarin produces a clumpy nucleolar pattern in indirect immunofluorescence assay on HEp-2 cells (HEp-2 IFA). Here we develop and validate a reliable cell-based anti-fibrillarin assay (Fibrillarin/CBA) for use in clinical diagnostic laboratories.

Vitamin K Deficiency Bleeding in Infancy.

Vitamin K is essential for the synthesis of few coagulation factors. Infants can easily develop vitamin K deficiency owing to poor placental transfer, low vitamin K content in breast milk, and poor intestinal absorption due to immature gut flora and malabsorption. Vitamin K deficiency bleeding (VKDB) in infancy is classified according to the time of presentation: early (within 24 h), classic (within 1 week after birth), and late (between 2 week and 6 months of age).

Incidence and In-Hospital Mortality of Neonatal Disseminated Intravascular Coagulation in Japan: An Observational Study of a Nationwide Hospital Claims Database.

This study aimed to estimate the incidence and prognosis of neonatal disseminated intravascular coagulation (DIC) in Japan by analyzing data retrieved from a national administrative database. Clinically, the prognosis of DIC in neonates is poor, but there is little epidemiological data in Japan. This retrospective observational study identified patients diagnosed with neonatal DIC and who were registered in the Japanese diagnosis procedure combination (DPC) database between April 1, 2014 and March 31, 2016.

Inhibitor development, safety and efficacy of Advate among previously treated patients with hemophilia A in a postmarketing surveillance in Japan.

Rurioctocog alfa (recombinant factor VIII: Advate) is available for the control of bleeding in patients with hemophilia A in Japan. To evaluate the inhibitor development, safety, and efficacy of rurioctocog alfa, a non-interventional and observational postmarketing surveillance was conducted on 352 previously treated Japanese patients aged 1-76 years with ≥ 4 exposure days under the conditions of routine clinical practice. A post-hoc comparison of the mean annualized bleeding rates which required treatment with rurioctocog alfa detected a statistically significant difference (P < 0.

Perioperative safety and hemostatic efficacy of Advate in patients with hemophilia A in a postmarketing surveillance in Japan.

Rurioctocog alfa (recombinant factor VIII: Advate) is available for the control of bleeding among patients with hemophilia A in Japan. To evaluate the perioperative safety and hemostatic efficacy of Advate, a postmarketing surveillance was conducted in Japanese patients undergoing surgery in a real-world setting. A total of 74 surgical procedures performed in 58 subjects aged 0-75 years, including three females, were studied.

A phase III clinical trial of a mixture agent of plasma-derived factor VIIa and factor X (MC710) in haemophilia patients with inhibitors.

Introduction: MC710, a 1:10 protein weight ratio mixture of plasma-derived activated factor VII (FVIIa) and factor X (FX), is a novel bypassing agent for haemostasis in haemophilia patients with inhibitors. We evaluated the haemostatic efficacy and safety of one to two administrations of MC710 in 21 joint, muscle, and subcutaneous bleeding episodes in 14 male patients, in a multi-centre, open-label, non-randomized clinical trial.

Methods: Subjects were intravenously administered one or two doses of 60 or 120 μg kg MC710 (as FVIIa) once or twice (to a maximum of 180 μg kg ) over up to five bleeding episodes per subject.

A Phase II clinical trial of a mixture of plasma-derived factor VIIa and factor X (MC710) in haemophilia patients with inhibitors: haemostatic efficacy, safety and pharmacokinetics/pharmacodynamics.

MC710, a mixture of plasma-derived activated factor VII and factor X at a protein weight ratio of 1:10, is a novel bypassing agent for haemostasis in haemophilia patients with inhibitors. In a Phase II trial, we evaluated the haemostatic efficacy and safety of single doses of MC710, and investigated pharmacokinetic and pharmacodynamic parameters in nine joint bleeding episodes in six male haemophilia patients with inhibitors. This trial was a multi-centre, open-label, non-randomized study of two doses (60 and 120 μg kg(-1) as FVIIa dose), allowing the re-administration of different MC710 dosages to the same subjects.

[Crow-Fukase syndrome (POEMS syndrome)].

In 1971, the author reported an autopsy case of a 48-year-old Japanese man with polyneuropathy, skin hyperpigmentation, diabetes mellitus, and monoclonal gammopathy. Previously, a total of 2 cases of solitary myeloma accompanied by polyneuropathy and endocrinological disorders have been reported by Fukase et al in 1968 and by Shimomori and Kusumoto in 1970 in Japan. The author's case is the first reported non-myeloma case where polyneuropathy associated with dermatoendocrionological changes and dysglobulinemia was observed.