Case report: Novel TBX5-related pathogenic mechanism of Holt-Oram syndrome.

Holt-Oram syndrome (HOS) is a rare genetic disorder characterized by upper limb abnormalities, congenital heart defects, and/or conduction abnormalities. Sequence alteration of T-box transcription factor 5 (TBX5) is correlated with the incidence of HOS. We present the case of a 24-year-old female with upper limb alterations (congenital dysplasia in the wrist and elbow joints) and an anomalous left main trunk arising from the right coronary sinus.

Anticoagulation with nafamostat mesilate during extracorporeal life support.

Nafamostat mesylate (NM) affects coagulation and fibrinolysis and impedes obesity-associated protein demethylase activity, which regulates Na/K transport properties and the NF-κB signaling pathway. NM significantly decreases macrophage, neutrophil, and T lymphocyte infiltration, thereby reducing inflammation and apoptosis after reperfusion and promoting recovery in patients with severe conditions such as near-fatal asthma and cardiac arrest. Extracorporeal life support (ECLS) devices are used for cardiac and/or pulmonary support as a bridge to recovery, decision, surgery, or transplant in patients with refractory cardio-circulatory or respiratory diseases and provide essential opportunities for organ support and patient survival.

[The use of extracorporeal membrane oxygenation in sustaining pulmonary function patients with influenza A H1N1].

Objective: To summarize the clinical method and initial experience of extracorporeal membrane oxygenation (ECMO) supportive treatment in influenza A H1N1 serious patients.

Methods: In 5 critically ill patients with influenza A H1N1, their arterial oxygen saturation was 0.70 to 0.