Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019270 (hernia)
 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Congenital diaphragmatic hernia (CDH) is a rare disease, which affects 1 in 2,500 newborns. Congenital diaphragmatic hernia can interfere with the normal development of the pulmonary parenchyma and vascular bed, and in severe cases, it can lead to the development of severe pulmonary arterial hypertension (PAH) and right ventricular failure. We present a neonate with CDH who developed severe PAH and right ventricular dysfunction and was managed with a unique strategy combining venoarterial extracorporeal membrane oxygenation, prostaglandin E1, and a variety of PAH therapies.
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PMID:Combination of PGE1 and Pulmonary Vasodilator Therapy in Managing a Challenging Case of Severe PAH Secondary to CDH. 3264 88

Cerebral venous sinus thrombosis (CVST) is a rare disease associated with high disability and mortality rates. A subset of patients do not respond to standard anticoagulation therapy, leading to the progression of CVST with hemorrhagic stroke, which represents a major challenge for its treatment. Severe hemorrhagic (SH)-CVST is life-threatening due to large hematoma, edema and/or cerebral hernia. Anticoagulation or thrombolytic therapy alone may lead to further aggravation of the hematoma. Stent retriever thrombectomy combined with long-term local thrombolysis (SRT-LLT) has been used in certain centers for those refractory cases or patients with new intracranial hemorrhage. However, to date, no studies on SRT-LLT treatment specifically for SH-CVST have been performed. The aim of the present retrospective study was to specifically evaluate the effectiveness of SRT-LLT in SH-CVST. Between December 2013 and November 2018, SRT-LLT was performed at our center in 8 patients with hemorrhagic CVST who did not respond to intravenous anticoagulation. The clinical characteristics, results of the radiological evaluation, details on the surgical procedure and clinical outcomes were assessed. The patients were administered systemic intravenous anticoagulation as the initial treatment following admission. SRT-LLT was performed when their condition deteriorated with a high risk of a fatal outcome within a short time period. SRT-LLT was performed in 8 patients, with successful recanalization confirmed by angiography. In 4 of the patients, complete recanalization was achieved, whereas in the remaining 4, recanalization was partial. There were no intraoperative complications. Two patients developed rebleeding after surgery, but they all gradually recovered. There were no treatment-associated fatalities. Therefore, SRT-LLT appears to be a feasible, safe and effective option for SH-CVST and it may be used as rescue therapy for carefully selected patients with SH-CVST.
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PMID:Stent retriever thrombectomy combined with long-term local thrombolysis for severe hemorrhagic cerebral venous sinus thrombosis. 3296 96


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