A fatal case of neonatal onset multiple acyl-CoA dehydrogenase deficiency caused by novel mutation of ETFDH gene: case report.

Background: Multiple acyl-CoA dehydrogenase deficiency (MADD) or glutaric aciduria type II is an extremely rare autosomal recessive inborn error of fatty acid beta oxidation and branched-chain amino acids, secondary to mutations in the genes encoding the electron transfer flavoproteins A and B (ETFs; ETFA or ETFB) or ETF dehydrogenase (ETFDH). The clinical manifestation of MADD are heterogeneous, from severe neonatal forms to mild late-onset forms.

Case Presentation: We report the case of a preterm newborn who died a few days after birth for a severe picture of untreatable metabolic acidosis.

Economic results of a palivizumab seasonal prophylaxis using a cohorting software and vial sharing.

Background: Respiratory syncytial virus is the most important pathogen in lower respiratory tract infection in infants and young children. In high-risk populations it may develop severe, sometimes fatal, lower respiratory tract infections. A proportion of these infants require admission to intensive care units due to the severity of the condition and the level of care needed.

Rosiglitazone, a ligand of the peroxisome proliferator-activated receptor-gamma, reduces the development of nonseptic shock induced by zymosan in mice.

Objective: Peroxisome proliferator-activated receptors (PPARs) are members of the nuclear hormone receptor superfamily of ligand-activated transcription factors that are related to retinoid, steroid, and thyroid hormone receptors. The PPAR-gamma receptor subtype appears to play a pivotal role in the regulation of cellular proliferation and inflammation. Rosiglitazone (Avandia) is a PPAR-gamma agonist (the most potent PPAR-gamma agonist of the thiazolidinedione antidiabetics).

Protective effects of M40401, a selective superoxide dismutase mimetic, on zymosan-induced nonseptic shock.

Objective: Zymosan enhances formation of reactive oxygen species, which contributes to the pathophysiology of organ failure during nonseptic shock. Here we have investigated the effects of M40401, a new superoxide dismutase mimetic, on the organ failure associated with nonseptic shock caused by zymosan in rats.

Design: Experimental study.

Pyrrolidine dithiocarbamate attenuates the development of organ failure induced by zymosan in mice.

Objective: Nuclear factor (NF) kappaB is a transcription factor which plays a pivotal role in the induction of genes involved in physiological processes as well as in the response to injury and inflammation. Dithiocarbamates are anti-oxidants which are potent inhibitors of NF-kappaB. We postulated that pyrrolidine dithiocarbamate (PDTC) would attenuate multiple-organ failure (MOF).

Effects of calpain inhibitor I on multiple organ failure induced by zymosan in the rat.

Objective: Zymosan enhances the formation of reactive oxygen species, which contributes to the pathophysiology of multiple organ failure. We investigated the effects of calpain inhibitor I (5, 10, or 20 mg/kg) on the multiple organ failure caused by zymosan (500 mg/kg, administered intraperitoneally as a suspension in saline) in rats.

Setting: University research laboratory.