Serum levels of anti-SRP54 antibodies reflect disease activity of necrotizing myopathy in a child treated effectively with combinatorial methylprednisolone pulses and plasma exchanges followed by intravenous cyclophosphamide.

We describe the effectiveness of combinatorial therapy with plasma exchanges and methylprednisolone pulses followed by intravenous cyclophosphamide in a young girl with anti-signal recognition particle 54 (SRP54) antibody-associated myopathy. We also use a newly described quantitative assay to demonstrate the close association between the titers of anti-SRP54 antibodies and disease activity. This is the first report of a pediatric patient indicating that the serum levels of anti-SRP54 antibodies are also beneficial for monitoring the disease activity of progressive necrotizing myopathy.

A pediatric patient with myopathy associated with antibodies to a signal recognition particle.

We report the case of a 15-year-old Japanese girl with myopathy associated with antibodies to a signal recognition particle (anti-SRP myopathy). The patient presented with progressive symmetrical proximal muscle weakness that caused difficulty in walking within 3 months, and marked elevation of the serum creatine kinase levels. A skeletal muscle biopsy revealed active necrotic and regenerating processes, with mild inflammatory changes. Based on the above findings, the patient was diagnosed as having anti-SRP myopathy. Only a limited number of pediatric patients with anti-SRP myopathy has been reported previously, with usually a poor prognosis. Early diagnosis is important for obtaining a better prognosis in patients with anti-SRP myopathy.

[Neonatal herpes simplex type II virus infection complicated with meningitis and virus-associated hemophagocytic syndrome].

A 14-day-old neonate was transferred to our university hospital because of respiratory distress and mild disturbance of consciousness. He had no history of abnormal pregnancy or delivery, but had developed apnea at 6 days old. Thereafter, respiratory distress progressed and his condition deteriorated. On admission to our hospital, several vesicles were found on the left upper arm, and moderate hepatomegaly was also present. Herpes simplex virus (HSV) type II genome was detected from serum, spinal fluid, and bone marrow. Laboratory examinations revealed typical abnormalities of disseminated intravascular coagulation, increased levels of serum ferritin, aspartate aminotransferase, and lactate dehydrogenase. Bone marrow aspiration demonstrated activated macrophages and hemophagocytosis. Spinal tap revealed numerous mononuclear cells. Meningitis and virus-associated hemophagocytic syndrome (VAHS) due to systemic HSV type II infection were thus diagnosed. Acyclovir (60 mg/kg/day) and vidarabine were promptly administered. Dexamethasone palmitate and intravenous cyclosporine were also administered for systemic inflammation due to VAHS. Finally, these aggressive therapies rescued the patient without any sequelae. In general, neonatal systemic HSV infection is life-threatening and results in poor intact survival. Our case report suggests that not only antiviral treatment for HSV, but also anti-inflammatory treatment including steroid and cyclosporine should be considered from the early phase of neonatal systemic HSV infection.

[Actigraphy for the assessment of sleep quality in pediatric atopic dermatitis patients].

BACKGROUND: Good sleep is essential for the growth and the development of children. However, sleep is often impaired in patients with atopic dermatitis (AD). It is important to assess the sleep quality in pediatric AD patients. For that purpose, we utilized actigraphy as an objective method for the assessment of sleep quality. METHODS: Childhood patients with AD (16 cases) and 8 non-allergic volunteers were recruited. Actiwatch (AW-64) was attached to each subject's wrist for 11 days at maximum. Sleep parameters were calculated with Actiware and compared among various patient groups. RESULTS: Results demonstrate that sleep was significantly compromised in patients with AD, according to the severity. Subjective scoring of the sleep quality by parents showed limited correlation with actigraphy. CONCLUSION: Actigraphy is an objective and unobtrusive method to measure the sleep quality in childhood AD patients and can provide useful outcome in clinical trial.

Etiology and clinical study of community-acquired pneumonia in 157 hospitalized children.

We tried to verify whether the currently employed diagnosis and treatment of community-acquired pneumonia in children were appropriate. For this purpose, we created tentative criteria for the classification of pediatric community-acquired pneumonia. We classified the community-acquired pneumonia into ten categories: (1) bacterial, (2) concomitant viral-bacterial, (3) viral, (4) mycoplasmal, (5) concomitant mycoplasmal-bacterial, (6) concomitant mycoplasmal-viral, (7) chlamydial, (8) concomitant chlamydial-bacterial, (9) concomitant chlamydial-viral, and (10) unknown. Children aged 1 month to 13 years with radiographic and clinical evidence of pneumonia were enrolled. Between October 2001 and September 2002, we enrolled 165 patients. The etiologic agents were determined in 126 of the 157 (80.3%) patients who were finally diagnosed with pneumonia. Two blood cultures were positive for Haemophilus influenzae type b and Streptococcus pneumoniae. A viral infection alone was found in 28 of the 157 patients (17.8%), a bacterial (without mycoplasmal) alone infection in 42 (26.8%), a concomitant viral-bacterial infection in 28 (17.8%), and a mycoplasmal infection in 27 (17.2%) patients. RS virus was identified in 28 patients (17.8%), influenza A in 12 (7.6%), parainfluenza 3 in 8 (5.1%), adenovirus in 8 (5.1%), and influenza B and measles virus in 1 patient each. Streptococcus pneumoniae was the most common cause of bacterial pneumonia. We chose the initial treatment according to clinical and laboratory findings on admission (i.e., patients' age, clinical course, chest X-ray, and laboratory findings). In 68 of the 71 patients with bacterial (without mycoplasmal) pneumonia, an appropriate antibacterial-agent was prescribed. In 25 of the 27 patients with mycoplasmal pneumonia, clindamycin and minocycline were prescribed.