Production of chemokines in respiratory syncytial virus infection with central nervous system manifestations.

Respiratory syncytial virus (RSV) infection in children can be associated with acute encephalopathy. However, the roles of cytokines in the cerebrospinal fluid (CSF) of such patients remain unevaluated. In this study, a profile of 17 cytokines was determined for eight RSV-infected children with neurological complications.

Transaminase in rotavirus gastroenteritis.

Background: Children infected with rotavirus often show increased levels of transaminase, and symptoms are characterized by white stool, similar to biliary atresia. Rotavirus infections are also sporadically accompanied with convulsions, encephalopathy and Reye syndrome. The aim of the present study was therefore to investigate transaminase and interleukin (IL)-6 levels in rotavirus infection, in order to better understand their clinical significance.

A boy with non-herpes simplex acute limbic encephalitis and antiglutamate receptor antibodies.

This report concerns a 12-year-old male with intractable seizures over a long period. The case fulfilled the diagnostic criteria for nonherpetic acute limbic encephalitis. He had frequent convulsions starting with a partial seizure at the left angle of the mouth and progressing to secondary generalized seizures.

Myopathy and neurogenic muscular atrophy in unexpected cardiopulmonary arrest.

Background:   Neuromuscular disorders can be the cause of sudden death of infants because of their weakness and gastroesophageal reflux (GER).

Methods:   Muscle biopsy and genetic studies were performed by usual method.

Results:   In this report four cases of infants with neuromuscular disorders (two cases of congenital myopathy and two cases of spinal muscular atrophy) who had unexpected cardiopulmonary arrest on arrival (CPAOA) are presented.

Efficacy of prolonged valganciclovir therapy for congenital cytomegalovirus infection.

We report prolonged valganciclovir (VGCV) treatment of a symptomatic cytomegalovirus infection case. Automated auditory brainstem evoked response performed at 5 days of age revealed severe hearing impairment. Cranial magnetic resonance (MR) imaging at 11 days of age showed abnormal findings.

Diurnal variation in febrile convulsions.

This study sought to determine diurnal variations in febrile convulsions, and to investigate whether such variations influenced the severity of febrile convulsions. The study involved 326 children, between ages 6 months and 6 years, with simple febrile convulsions. Data were collected systematically by interviewing witnesses within the week after febrile convulsions occurred.

Association of mannose-binding lectin gene polymorphisms with Kawasaki disease in the Japanese.

Objective: Kawasaki disease (KD) is a systemic vasculitis in childhood; its etiology is unknown. The possibility that KD is an infectious disease has been discussed and investigated for decades, in light of the implication that infections are involved in the pathogenesis of KD. Young children rely on their innate immune system for protection against virus and micro-organisms.

Anti-glutamate receptor antibodies in pediatric enteroviral encephalitis.

In order to better understand enteroviral encephalitis we investigated the clinical symptoms and several disease markers. Between 2000 and 2005 eight patients aged between 9 months and 5 years were admitted to our hospital with one case having sequela. Glutamic oxaloacetic transaminase (GOT), serum IL-6, and ferritin were elevated in most of the cases.

Changes in serum lactate dehydrogenase activity in children with atopic dermatitis.

Background: In recent years an increase has been seen in the number of patients with severe atopic dermatitis (AD) accompanied with generalized typical eruptions. Some markers indicating the severity of the disease and symptom changes are very useful, and therefore the purpose of the present study was to investigate serum lactate dehydrogenase (LDH) as such a marker.

Methods: A total of 58 children with AD were enrolled.

A mutation in the IL-2 receptor gamma chain gene associated with X-linked severe combined immunodeficiency accompanying opisthotonus.

Severe combined immunodeficiency (SCID) is an inherited disease with profoundly defective T cells, B cells, and NK cells. X-linked severe combined immunodeficiency (X-SCID) is its most common form. In this report, we describe a 4-month old male with X-SCID who also showed opisthotonic posturing.

A Case of Tubulointerstitial Nephritis and Uveitis (TINU) Syndrome with High ASLO Titer.

Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare autoimmune disease and the pathogenesis is still unknown. We report a case of TINU syndrome with high ASLO titer. Uveitis improved and urine β2-MG normalized with low dose systemic predonisolone and cyclosporin A.

mRNA expression of T-helper 1, T-helper 2 cytokines in autoimmune hepatitis in childhood.

Background: In the pathology of autoimmune hepatitis the immunity mechanism of T-helper 1 (Th1) and Th2 cells was recently evaluated. The purpose of the present study was to measure the mRNA levels in peripheral mononuclear cells and serum cytokines obtained from children with autoimmune hepatitis for a better understanding of the mechanism.

Methods: Twenty-five patients with autoimmune hepatitis and seven controls were enrolled.

Viral loads of cerebrospinal fluid in infants with enterovirus meningitis.

For a better understanding of the role of the viral load, free radicals, and cytokines in viral meningitis, we surveyed cerebrospinal fluid (CSF) obtained from patients below 1 year of age who showed positive for enterovirus. In their first examinations interleukin (IL)-6 and free radicals increased whereas pleocytosis was rarely observed. IL-6 decreased within the short period.

A Case of Hyper IgD and Periodic Fever Syndrome in Japan.

We report a four-year-old Japanese girl with hyper IgD and periodic fever syndrome. There is a first report of hyper IgD syndrome (HIDS) of which the genomic study was done in Japan. In this report a girl complained of periodic fever and abdominal symptoms accompanied with high levels of mevalonic acid in urine and serum.

Increased nitric oxide production and GFAP expression in the brains of influenza A/NWS virus infected mice.

The cause of influenza to the brain was investigated using the A/NWS/33 influenza virus infected BALB/c mouse model. NOS-2 mRNA levels in the infected mouse brain was greater than in control mice in all brain regions examined, particularly in the olfactory bulb and hippocampus by 1 day p.i.

[Prevalence of chlamydia-pneumoniae-specific immunoglobulin M antibody and acute exacerbations of asthma in childhood].

Background: Chlamydia pneumoniae is a frequent causative agent of acute respiratory disease and has been recently reported as a possible cause of asthma. We investigated the prevalence of C. pneumoniae infections in childhood patients with acute exacerbations of asthma.

Extreme efficiency of anti-interleukin 1 agent (anakinra) in a Japanese case of CINCA syndrome.

We describe a case of severe CINCA syndrome in a Japanese male infant. We recently managed with anti-interleukin 1 agent (anakinra). Anakinra immediately led to improvement of both the clinical symptoms and the laboratory findings.

A case report of neonatal onset multisystemic inflammatory disease in Japan treated with continuous hemodiafiltration and steroid pulse therapy.

Neonatal onset multisystem inflammatory disease (NOMID), which is also known as chronic infantile neurological cutaneous and articular syndrome, is a rare disease that is characterized by the triad of cutaneous rash, chronic meningitis and arthropathy. The long-term prognosis is poor, with progressive deafness and visual impairment, and worsening of the central nervous system manifestations. Some fatal cases have been reported secondary to infection, vasculitis and amyloidosis.