Acta paediatrica Taiwanica = Taiwan er ke yi xue hui za zhi最新文献

Eicosanoids belong to a diverse family of bioactive fatty acids that play important roles in regulating airway inflammation and reactivity. They are the key mediators of the pathobiology of asthma. Among the eicosanoids, lipoxins (LXs) were the first agents to be identified and recognized as potential anti-inflammatory endogenous lipid mediators. Lipoxins are biosynthesized in vivo at inflammation sites. They result mainly from the interaction between 5 and 15-lipoxygenases (LOs), which are distinct from leukotrienes (LTs) and prostaglandins (PGs) in structure and function. Leukotrienes are potent proinflammatory mediators and directly and indirectly stimulate fibroblast chemotaxis, proliferation, and collagen synthesis. Prostaglandins have both bronchoconstrictive and bronchoprotective effects and the bronchoconstriction mediated by PGD2 and PGF2alpha is only occurred in asthmatic patients but not in healthy subjects. Lipoxins counter-regulate the proinflammatory actions of LTs and activate resolution of the inflammatory response. At least two classes of receptors, CysLT1 receptors and Asprin-triggered lipoxin A4 (ALX) receptors, can interact with lipoxin A4 (LXA4) and LXA4 analogs to mediate their biologic actions. Allergen challenge initiates airway biosynthesis of LXA4 and increases expression of its receptor. In addition, LXA4 affects the release of interleukin-8 by blood mononuclear cells, and ALX affects calcium influx into epithelial cells. Therefore, the pivotal role of LXs is mediating airway homeostasis, and LXs may be part of a novel, multipronged approach for treating human asthma.

Trisomy 18 syndrome is the second most common multiple malformation syndrome. The incidence is about 0.3 per 100 newborn babies. More than 130 different abnormalities have been noted in the literature on patients with the trisomy 18 syndrome. Trisomy 18 includes a broad spectrum of anomalies and malformations, among which we found Dandy-Walker malformation (DWM) rarely mentioned. DWM is estimated to occur in approximately 1 in 25,000 to 30,000 live births. The major components of DWM are the following: partial or complete absence of the cerebellar vermis, a posterior fossa cyst continuous with the fourth ventricle, and hydrocephalus. Although DWM has been associated with many chromosomal abnormalities and genetic syndromes in live birth, trisomy 18 with DWM is a rare condition. Here we present a female newborn with intrauterine growth retardation and multiple congenital abnormalities including: craniofacial anomaly, tracheoesophageal fistula, esophageal atresia, absent radius and thumb, short ulna, clenched hands, ventricular septal defect, coarctation of the aorta, patent ductus arteriosus, Meckel's diverticulum, ectopic pancreas in the ileum, and DWM. Her chromosome karyotype was 47,XX,+18. This is a case of Trisomy 18 with multiple rare malformations.

Budd-Chiari syndrome is a rare, heterogenous and potentially lethal condition due to hepatic venous outflow obstruction. Classic triads include hepatomegaly, ascites and abdominal pain. Most cases are caused by abnormal coagulopathy, infection, inflammation or tumor invasion. Status post liver transplantation was rarely mentioned in the pediatric group. Here we report a case of 4-year-old girl with biliary atresia status post living donor liver transplantation. Marked ascites developed 2 months later and Budd-Chiari syndrome was diagnosed. Cardiac catheterization showed significant stenoses of the hepatic vein and inferior vena cava. Initial balloon angioplasty of inferior vena cava only resulted in mild improvement. After successful balloon angioplasty of the hepatic vein stenosis, the stenosis of the inferior vena cava improved significantly. The ascites resolved soon, and no more happened up to 8 months' follow-up.