Familial exudative vitreoretinopathy with mutation without signs of Loeys-Dietz syndrome.

: Familial exudative vitreoretinopathy (FEVR) is an inherited retinal disorder with high genetic heterogeneity, and it is characterized by a defect in the development of the retinal vascular system. Loeys-Dietz syndrome (LDS) is an autosomal dominant systemic connective tissue disorder that is caused by mutations in the genes related to transforming growth factor signaling systems including the gene. Two earlier studies reported that patients with LDS from mutations in the gene were associated with FEVR-like retinal phenotype.

Possible Involvement of Sphingosine-1-Phosphate/G(i)/RhoA pathways in adherence of eosinophils to pulmonary endothelium.

Background: Sphingosine-1-phosphate (S1P), a lysophospholipid released from inflammatory cells, causes cell migration by increasing cytokines and chemokines. This study was designed to determine whether S1P causes adherence of eosinophils to pulmonary endothelial cells via enhancement of adhesion molecule expression.

Methods: Expression of VCAM-1 and ICAM-1 was assessed by RT-PCR and Western blot analysis in human pulmonary microvasucular endothelial cells (HPMVECs).

Thalidomide attenuates airway hyperresponsiveness and eosinophilic inflammation in a murine model of allergic asthma.

Asthma is characterized by chronic eosinophilic inflammation and hyperresponsiveness of the airways. We hypothesized that thalidomide, which has numerous immunomodulatory properties, may have anti-inflammatory effects in allergic asthma. BALB/c mice sensitized and challenged with ovalbumin (OVA) were treated orally with thalidomide (30, 100, or 300 mg/kg) or a vehicle.

[A case of pulmonary inflammatory pseudotumor accompanied with increased serum immunoglobulin G levels and autoimmune pancreatitis].

A 77-year-old man with increased serum immunoglobulin G levels and autoimmune pancreatitis was found to have a chest X-ray abnormality. The chest X-ray and CT films showed a mass shadow in the right lower lobe and lymphadenopathy. Since transbronchial tumor biopsy did not obtain diagnostic material, CT-guided cutting needle biopsy was performed.

[Prevalence of myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA) in patients with interstitial pneumonia].

Myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA) is an autoantibody that is frequently found in patients with vasculitides. We encountered some MPO-ANCA positive patients with interstitial pneumonia who lacked vasculitides, but its meaning remains unclear. We measured MPO-ANCA titers in 69 patients with interstitial pneumonia (IP) who did not have collagen vascular diseases and observed their outcome.

[A case of primary pulmonary MALT lymphoma with diffuse micronodules and anemia].

A 52-year-old woman presented with low grade fever and fatigue. She had diffuse micronodules in both lung fields on chest X-ray. Chest CT showed diffuse multiple small nodules.