Prenatal diagnosis and perinatal clinical course of isolated left-sided innominate artery.

Isolated left-sided innominate artery, a rare congenital anomaly in which the left-sided innominate artery arises from the main pulmonary trunk, is usually diagnosed incidentally in children and adults. Limited reports exist on its prenatal diagnosis, with none comprehensively describing the associated perinatal haemodynamic changes. We report a case of prenatally diagnosed isolated left-sided innominate artery, describing the postnatal clinical course.

Electrocardiographic Changes with Age in Japanese Patients with Noonan Syndrome.

Little information is available on age-related electrocardiographic changes in patients with Noonan syndrome. This single-center study evaluated the electrocardiograms of patients with Noonan syndrome. We divided the patients ( = 112; electrocardiograms, 256) into four groups according to age: G1 (1 month-1 year), G2 (1-6 years), G3 (6-12 years), and G4 (>12 years).

Cardiac features of Noonan syndrome in Japanese patients.

Background: Cardiovascular disease is one of the most important problems in long-term follow-up for Noonan syndrome. We examined cardiovascular issues and clinical manifestations, with a focus on the cardiovascular disease and prognosis of patients with Noonan syndrome.

Methods: This single-centre study evaluated patients who were clinically and genetically diagnosed with Noonan syndrome.

Cardiac Resynchronization Therapy Using Single Site Left Ventricular Pacing in a Tricuspid Atresia Patient With Left Bundle Branch Block.

Cardiac resynchronization therapy (CRT) is typically achieved by pacing both ventricles. However, left ventricular-only pacing has been shown to be noninferior to biventricular pacing in patients with left bundle branch block and normal atrioventricular conduction. However, there is no evidence in favour of CRT with single-site pacing for patients with single-ventricle physiology.

A retrospective study of perioperative clinical seizures and epilepsy in children after operation for CHD.

This study investigated the incidence and risk factors of perioperative clinical seizure and epilepsy in children after operation for CHD. We included 777 consecutive children who underwent operation from January 2013 to December 2016 at Kanagawa Children's Medical Center, Kanagawa, Japan. Perinatal, perioperative, and follow-up medical data were collected.

RAG1 deficiency may present clinically as selective IgA deficiency.

Background: Recombination-activating gene (RAG) 1 and 2 deficiency is seen in patients with severe combined immunodeficiency (SCID) and Omenn syndrome. However, the spectrum of the disease has recently expanded to include a milder phenotype.

Objective: We analyzed a 4-year-old boy who was initially given the diagnosis of selective immunoglobulin A deficiency (SIgAD) based on immunoglobulin serum levels without any opportunistic infections, rashes, hepatosplenomegaly, autoimmunity or granulomas.

[A case of severe systemic juvenile idiopathic arthritis introduced tocilizumab in early phase of the disease].

A 14-year-old boy was admitted in the former hospital with remittent fever, erythematous rash, joint pain, and muscle pain. Antibiotics were ineffectively administered and then, methylprednisolone (mPSL) pulse therapy with methotrexate was introduced under the diagnosis of suspected systemic juvenile idiopathic arthritis (JIA). However, he still had clinical symptoms and signs, and was transferred to our hospital.