Comparison of metabolic parameters between oral and total parenteral nutrition in children with severe eating disorders.

Background: This study investigated changes of lipid parameters in children with severe eating disorders during refeeding in order to explore the optimal timing for lipid preparation administration.

Methods: We prospectively assessed the physical conditions of patients with eating disorders after the start of nutrition therapy. The assessments were performed at admission and at 2 and 4 weeks.

Comparison of metabolic parameters between oral and total parenteral nutrition in children with severe eating disorders.

Background: This study investigated changes of lipid parameters in children with severe eating disorders during refeeding in order to explore the optimal timing for lipid preparation administration.

Methods: We prospectively assessed the physical conditions of patients with eating disorders after the start of nutrition therapy. The assessments were performed at admission and at 2 and 4 weeks.

Flowchart for selecting an appropriate surgical airway in neurologically impaired pediatric intubated patients: a case series.

Objective: Medical advances have resulted in increased survival rates of neurologically impaired children who may require mechanical ventilation and subsequent tracheostomy as a surgical airway. However, at present, there is no definite consensus regarding the timing and methods for placement of a surgical airway in a neurologically impaired intubated child who needs to be cared for over a long-term period. We therefore created a flowchart for the selection of a surgical airway for Neurologically Impaired Pediatric Patients (NIPPs).

Diagnostic reference value of antibody levels measured using enzyme immunoassay for subacute sclerosing panencephalitis.

High measles-specific antibody titers in the cerebrospinal fluid (CSF) have important diagnostic significance for subacute sclerosing panencephalitis (SSPE), a progressive neurological disorder caused by measles virus variants. However, the diagnostic reference value of antibody levels and the usefulness of the CSF/serum ratio measured using enzyme immunoassays (EIAs) for SSPE diagnosis remain unclear. To facilitate SSPE diagnosis using EIAs, measles immunoglobulin G (IgG) titers in the CSF and serum of patients with and without SSPE were measured and their CSF/serum antibody ratios evaluated.

Gene expression profile and injury sites in mice treated with Shiga toxin 2 and lipopolysaccharide as a Shiga toxin-associated hemolytic uremic syndrome model.

Shiga toxin 2 (Stx2) and lipopolysaccharide (LPS) contribute to the development of hemolytic uremic syndrome (HUS). Mouse models of HUS induced by LPS/Stx2 have been used for elucidating HUS pathophysiology and for therapeutic development. However, the underlying molecular mechanisms and detailed injury sites in this model remain unknown.

Plastic bronchitis associated with influenza B virus infection: A case report.

Plastic bronchitis (PB) is a severe acute respiratory disease that develops as a result of the formation of branching mucus plugs in the bronchial tree. PB is known as a complication of influenza A virus infection, but some cases have been associated with influenza B virus infections. This patient was a 3-year-old boy with no history of allergic disease who developed PB requiring ventilator management after influenza B virus infection.

Incidence and Relapse Triggers of Childhood Idiopathic Nephrotic Syndrome between 2006 and 2016: A Population-Based Study in Fukushima, Japan.

Childhood idiopathic nephrotic syndrome (NS) is defined by proteinuria and hypoproteinemia. The incidence of childhood idiopathic NS varies with age, race, residential areas, and social conditions. In Japan, its incidence was estimated to be 6.

Valganciclovir prophylaxis for cytomegalovirus infection in pediatric kidney transplant recipients: a single-center experience.

Background: There are two approaches for treating cytomegalovirus (CMV) infection occurring after kidney transplantation (KTx). One is preemptive therapy in which treatment is started after confirming positive CMV antigenemia using periodic antigenemia assay. The other approach is prophylactic therapy in which oral valganciclovir (VGCV) is started within 10 days after KTx and continued for 200 days.

Cortical laminar necrosis in an infant with influenza A virus infection.

Cortical laminar necrosis comprises ischemic neuronal changes and glial reaction. Despite fewer reports in the pediatric population, we encountered a case of cortical laminar necrosis with influenza virus A infection in an infant.

Antiviral Effect of Favipiravir (T-705) against Measles and Subacute Sclerosing Panencephalitis Viruses.

Subacute sclerosing panencephalitis (SSPE) is a late-onset, intractable, and fatal viral disease caused by persistent infection of the central nervous system with a measles virus mutant (SSPE virus). In Japan, interferon-α and ribavirin are administered intracerebroventricularly to patients with SSPE. However, as the therapeutic effect is insufficient, more effective drugs are needed.

Comprehensive Targeted Sequencing Identifies Monogenic Disorders in Patients With Early-onset Refractory Diarrhea.

Objectives: Causes of early-onset refractory diarrhea include exudative diarrhea associated with very early-onset inflammatory bowel diseases, osmotic or secretory diarrhea, and protein-losing enteropathy. Monogenic disorders are included in these diseases, yet a comprehensive genetic analysis has not been fully established.

Methods: We established targeted gene panels covering all responsible genes for early-onset diarrhea.

Utility of enzyme immunoassays for diagnosis of subacute sclerosing panencephalitis.

Background: Subacute sclerosing panencephalitis (SSPE) is a progressive neurologic disorder caused by the measles virus (MV) and is identified by positive MV-specific antibody titers, detected mainly by hemagglutination inhibition (HI) tests in the cerebrospinal fluid (CSF). However, an alternative method, the enzyme immunoassay (EIA), has increasingly become a preferred method for detecting MV antibodies. To establish the index for SSPE diagnosis using EIA, we investigated the correlation between HI and EIA titers of MV antibodies in SSPE patients.

Pneumorrhachis in children: A report of two cases and review of the literature.

Pneumorrhachis refers to the clinical presentation of air within the spinal canal, and it is rarely associated with pneumomediastinum, particularly in young children. Pneumorrhachis associated with pneumomediastinum is generally asymptomatic. Here we report 2 unusual cases involving very young children with pneumorrhachis secondary to pneumomediastinum and present a review of the relevant literature.

Involvement of high mobility group box 1 in the pathogenesis of severe hemolytic uremic syndrome in a murine model.

Typical hemolytic uremic syndrome is caused by Shiga toxin (Stx2) and lipopolysaccharide (LPS) of and leads to acute kidney injury. The role of innate immunity in this pathogenesis is unclear. We analyzed the role of high mobility group box 1 (HMGB1) at the onset of disease in a murine model.

Clinical manifestations of respiratory syncytial virus-associated encephalopathy in Fukushima, Japan.

Background: We investigated the epidemiology and clinical course of children with respiratory syncytial virus (RSV)-associated encephalopathy.

Methods: We retrospectively collected data for 280 patients from a questionnaire survey of acute encephalitis/encephalopathy (AE/E) in Fukushima Prefecture. We enrolled six patients diagnosed with RSV-associated encephalopathy from these 280 patients, and retrospectively investigated the clinical features and prognosis.

Ectopic expression of CLDN2 in podocytes is associated with childhood onset nephrotic syndrome.

Background: Animal models of nephrotic syndrome (NS) revealed that tight junction (TJ)-like structures are generated together with a concomitant decrease in slit diaphragms (SDs). Claudins (CLDNs) are capable of forming TJ strands and thereby the backbone of TJs. We showed the ectopic expression of CLDN2 in podocytes in pediatric NS, and detected its localization.

Haloperidol-Induced Dystonia due to Sedation for Upper Gastrointestinal Endoscopy: A Pediatric Case Report.

Dystonia is a movement disorder characterized by sustained muscle tone. Antipsychotic agents sometimes cause acute dystonia that can rapidly worsen within a few hours or days. Because healthy children rarely receive antipsychotic agents, it is unusual to see antipsychotic agent-induced dystonia in pediatric emergency departments.

Predictive factors for poor outcome in pediatric C3 glomerulonephritis.

Background: To clarify the predictive factors for poor outcome in pediatric C3 glomerulonephritis (C3GN), we retrospectively evaluated the relationship between the clinico-pathological findings and prognosis in cases of pediatric C3GN.

Methods: We enrolled 18 patients diagnosed with C3GN. These patients were divided into two groups, four patients in the end-stage renal disease (ESRD) group and 14 patients in non-ESRD group, based on clinical status at the last examination.

Maintaining Concentration of Ribavirin in Cerebrospinal Fluid by a New Dosage Method; 3 Cases of Subacute Sclerosing Panencephalitis Treated Using a Subcutaneous Continuous Infusion Pump.

Background: Subacute sclerosing panencephalitis (SSPE) is a slow virus infectious disease resulting from persistent infection with mutant measles virus. At present, there is no effective treatment for SSPE. Interferon-α and inosine pranobex have both been used for the treatment of SSPE, and partial success has been reported for the antiviral drug, ribavirin (RBV).

Serum sickness with refractory nephrotic syndrome following treatment with rituximab.

Rituximab (RTX) is effective for treating childhood refractory nephrotic syndrome (NS), such as steroid-dependent (SD), frequently relapsing (FR), and steroid-resistant (SR) NS. While RTX has been proven to be effective in treating SDNS, FRNS, and SRNS, it may cause serum sickness, a rare illness characterized by fever, rash, and arthralgia, 10-14 days after primary antigen exposure or within a few days after secondary antigen exposure, by producing human anti-chimeric antibodies (HACAs). A 17-year-old girl with refractory SDNS treated with RTX and oral cyclosporine A was admitted with fever and arthralgia 10 days after the fifth RTX dose was administered.

Comparison of long-term follow-up outcomes between multiple-drugs combination therapy and tonsillectomy pulse therapy for pediatric IgA nephropathy.

Background: To clarify the long-term efficacy of multiple-drugs combination therapy (PWDM) and tonsillectomy pulse therapy (TPT) for pediatric IgA nephropathy (IgAN), we retrospectively evaluated the clinical and laboratory findings as well as the prognosis for IgAN patients treated with each treatment at long-term follow-up.

Methods: We collected data on 61 children who had been diagnosed with severe IgAN. The children were retrospectively divided into two groups.

Serum IgA/C3 and glomerular C3 staining predict severity of IgA nephropathy.

Background: The aim of this study was to determine whether serum immunoglobulin A/complement factor 3 (IgA/C3) ratio and glomerular C3 staining predict outcome in IgA nephropathy.

Methods: We collected data for 44 IgA nephropathy children treated with multi-drug combination therapy. The children were retrospectively divided into four groups based on serum IgA/C3 ratio and glomerular C3 staining: group A, IgA/C3 ratio >2.

Relapse of nephrotic syndrome triggered by Kawasaki disease.

Minor infections, allergies, insect bites, and bee stings are commonly reported causes of nephrotic syndrome (NS). Herein, we report, to the best of our knowledge, the first case of NS relapse due to Kawasaki disease (KD). An 8-year-old boy presented with high fever of 4-day duration.

The incidence and severity of IgA vasculitis with nephritis over a 10-year period in our hospital.

Background: To evaluate the recent frequency of onset and severity of IgA vasculitis with nephritis (IgAVN) in Fukushima Prefecture, we examined the epidemiology and clinico-pathological manifestations of IgAVN in our hospital over a 10-year period.

Methods: We enrolled 18 patients with IgAVN treated between 2004 and 2013 in the Department of Pediatrics, Fukushima Medical University School of Medicine. These patients were divided into two groups; Group 1 consisted of 12 patients with IgAVN hospitalized between 2004 and 2008 and Group 2 consisted of 6 patients with IgAVN hospitalized between 2009 and 2013.

Serum myeloid-related protein 8/14 in minimal change- and glomerulonephritis-related nephrotic syndrome.

Background: Myeloid-related protein 8/14 (MRP8/14) forms stable heterodimers and is the major calcium-binding protein secreted by activated granulocytes and monocytes. We evaluated whether serum MRP8/14 level is a useful indicator for a differential diagnosis of glomerulonephritis (GN)- and minimal change disease (MC)- related nephrotic syndrome (NS).

Methods: Serum MRP8/14 complex was evaluated in 37 NS patients with MC or GN.