Comparison of neurological manifestation in children with and without coronavirus 2019 experiencing seizures with fever.

Whether neurologic symptoms due to SARS-CoV-2 differ from those of non-SARS-CoV-2 viral infection is unclear. We aimed to describe these neurological manifestations and compare the clinical characteristics and treatments in children with seizures and fever with or without COVID-19. We retrospectively analyzed data from 105 hospitalized children (<18 years) with clinical seizures and fever between September 2021 and August 2022.

Annual patterns in the outcomes and post-arrest care for pediatric out-of-hospital cardiac arrest: A nationwide multicenter prospective registry in Japan.

Aim: Out-of-hospital cardiac arrest (OHCA) has a poor prognosis in children; however, the annual patterns of prognosis and treatment have not been fully investigated.

Methods: From the Japanese Association for Acute Medicine OHCA registry, a multicenter prospective observational registry in Japan, we identified pediatric patients (zero to 17 years old) between June 2014 and December 2019. The primary outcome was one-month survival.

Long-term outcome of combination therapy with corticosteroids, mizoribine and RAS inhibitors as initial therapy for severe childhood IgA vasculitis with nephritis.

Background: Patients with severe IgA vasculitis with nephritis (IgAVN) typically receive aggressive therapy as an initial approach. We have consistently performed combination therapy including corticosteroids and immunosuppressants as initial therapy for severe IgAVN over a 20-year-plus period, with only minor changes to the treatment protocol. This study seeks to reveal the efficacy of combination therapy for severe IgAVN.

Clinical and laboratory characteristics of complex febrile seizures in the acute phase: a case-series study in Japan.

Background: Patients with complex febrile seizures (CFS) often display abnormal laboratory results, unexpectedly prolonged seizures, and/or altered consciousness after admission. However, no standardized values have been established for the clinical and laboratory characteristics of CFS in the acute phase, making the management of CFS challenging. This study aimed to determine the clinical and laboratory characteristics of children with CFS during the acute phase.

Application of adult prehospital resuscitation rules to pediatric out of hospital cardiac arrest.

Background: Prehospital termination of resuscitation (TOR) rules can be recommended for adults with out-of-hospital cardiac arrests (OHCAs). This study aimed to investigate whether adult basic life support (BLS) and advanced life support (ALS) TOR rules can predict neurologically unfavorable one-month outcome for pediatric OHCA patients.

Methods: From a nationwide population-based observational cohort study, we extracted data of consecutive pediatric OHCA patients (0-17 years old) from January 1, 2005, to December 31, 2011.

Prognostic effects of treatment protocols for febrile convulsive status epilepticus in children.

Background: Febrile status epilepticus is the most common form of status epilepticus in children. No previous reports compare the effectiveness of treatment strategies using fosphenytoin (fPHT) or phenobarbital (PB) and those using anesthetics as second-line anti-seizure medication for benzodiazepine-resistant convulsive status epilepticus (CSE). We aimed to examine the outcomes of various treatment strategies for febrile convulsive status epilepticus (FCSE) in a real-world setting while comparing the effects of different treatment protocols and their presence or absence.

Mycophenolate Mofetil after Rituximab for Childhood-Onset Complicated Frequently-Relapsing or Steroid-Dependent Nephrotic Syndrome.

Background: Rituximab is the standard therapy for childhood-onset complicated frequently relapsing or steroid-dependent nephrotic syndrome (FRNS/SDNS). However, most patients redevelop FRNS/SDNS after peripheral B cell recovery.

Methods: We conducted a multicenter, randomized, double-blind, placebo-controlled trial to examine whether mycophenolate mofetil (MMF) administration after rituximab can prevent treatment failure (FRNS, SDNS, steroid resistance, or use of immunosuppressive agents or rituximab).

Identification of novel OCRL isoforms associated with phenotypic differences between Dent disease-2 and Lowe syndrome.

Background: Although Lowe syndrome and Dent disease-2 are caused by Oculocerebrorenal syndrome of Lowe (OCRL) mutations, their clinical severities differ substantially and their molecular mechanisms remain unclear. Truncating mutations in OCRL exons 1-7 lead to Dent disease-2, whereas those in exons 8-24 lead to Lowe syndrome. Herein we identified the mechanism underlying the action of novel OCRL protein isoforms.

Early steroid pulse therapy for children with suspected acute encephalopathy: An observational study.

Steroid pulse therapy is widely used to treat virus-associated acute encephalopathy, especially the cytokine storm type; however, its effectiveness remains unknown. We sought to investigate the effectiveness of early steroid pulse therapy for suspected acute encephalopathy in the presence of elevated aspartate aminotransferase (AST) levels.We enrolled children admitted to Hyogo Children's Hospital between 2003 and 2017 with convulsions or impaired consciousness accompanied by fever (temperature >38°C).

Moyamoya disease with refractory hypertension associated with peripheral arterial stenosis in the renal parenchyma.

Moyamoya disease (MMD) has long been known to be associated with hypertension. While renal artery stenosis (RAS) is considered one of the causes of hypertension with MMD, most hypertension causes remain unexplained. A boy with MMD was diagnosed with renovascular hypertension (RVH) due to left-sided RAS by angiography.

Target Temperature Management and Survival with Favorable Neurological Outcome After Out-of-Hospital Cardiac Arrest in Children: A Nationwide Multicenter Prospective Study in Japan.

To assess whether target temperature management (TTM) is effective for 1-month survival with favorable neurological outcome among pediatric patients who achieved return of spontaneous circulation (ROSC) after out-of-hospital cardiac arrest (OHCA). The Japanese Association for Acute Medicine-out-of-hospital cardiac arrest (JAAM-OHCA) Registry, a multicenter prospective observational registry in Japan, included OHCA patients aged ≤17 years who achieved ROSC between June 2014 and December 2017. The primary outcome was 1-month survival with favorable neurological outcomes, defined as pediatric cerebral performance category 1 or 2.

Clinicopathological significance of glomerular capillary IgA deposition in childhood IgA nephropathy.

Background: IgA nephropathy (IgAN) is characterized by predominant mesangial IgA deposition. Some patients with IgAN demonstrate IgA deposition in glomerular peripheral capillaries (cap-IgA). The clinicopathological significance of cap-IgA remains incompletely investigated in children.