An autopsy case of recurrent pneumothorax and peliosis-like intrapulmonary hematoma with X-linked myotubular myopathy.

Background: The typical non-muscle complications of long-surviving X-linked myotubular myopathy (XLMTM) include scoliosis, head deformity, macrocephaly, gastroesophageal reflux disease and peliosis hepatis. Recently, pulmonary blebs and recurrent pneumothorax have also been reported as uncommon complications, whereas no reports on autopsy cases have focused on lung lesions.

Case Presentation: An 8-year-old boy with XLMTM presented recurrent pneumothorax requiring bleb resection and pleurodesis.

Pediatric procedural sedation in Japan: A single-facility study of 1,436 cases.

Background: Pediatric procedural sedation (PPS) has been established worldwide as standard practice for several decades. However, there are no comprehensive guidelines or multi-facility databases of PPS in Japan, and the current status of PPS and PPS-related adverse events is unclear. The objectives of this study were to investigate the status of PPS in Japan and clarify the adverse events and risk factors.

Vitamin D deficiency associated with dilated cardiomyopathy in early infancy caused by maternal cholestasis.

Breast feeding is known to be a major cause of vitamin D deficiency in infants because the content of vitamin D in breast milk is significantly lower than that in formula. We report a case of a 1-mo-old boy who developed hypocalcemic seizures and dilated cardiomyopathy caused by vitamin D deficiency despite being fed a sufficient amount of regular formula. The cause of vitamin D deficiency in this case was maternal vitamin deficiency due to severe hyperemesis and insufficient sunlight exposure, induced mainly by the malabsorption of fat-soluble vitamins caused by maternal cholestasis.

Reversible cerebral vasoconstriction syndrome manifesting as focal seizures without a thunderclap headache: A pediatric case report.

We report a pediatric case of reversible cerebral vasoconstriction syndrome with focal seizures without a thunderclap headache. A 7-year-old girl had a mild acute headache with nausea after swimming. She subsequently developed hemi-convulsions followed by right hemiplegia.

Severe form of encephalopathy associated with 2009 pandemic influenza A (H1N1) in Japan.

Background: Every year, an estimated 200-500 children in Japan develop influenza-associated encephalopathy (IAE), and 10-30% of these children die.

Objective: To clarify the clinical features of a severe form of acute encephalopathy seen with 2009 pandemic influenza A (H1N1).

Study Design: This retrospective survey examined 20 children with acute encephalopathy associated with the 2009 pandemic influenza A (H1N1) who died or were in a prolonged deep coma with a flat electroencephalogram tracing and loss of spontaneous respiration.

Deaths associated with pandemic (H1N1) 2009 among children, Japan, 2009-2010.

To clarify the cause of deaths associated with pandemic (H1N1) 2009 among children in Japan, we retrospectively studied 41 patients <20 years of age who had died of pandemic (H1N1) 2009 through March 31, 2010. Data were collected through interviews with attending physicians and chart reviews. Median age of patients was 59 months; one third had a preexisting condition.