Marphans

OBJECTIVE: We aim to present a novel surgical technique of Frozen Elephant Trunk (FET) to treat complex thoracic aortic diseases in one stage and report its short-term outcomes. PATIENTS AND METHODS: Between December 2019 and 30 April 2021, twenty-five patients underwent FET operation at Viet Duc University Hospital. The mean age of the patients was 55.9 (±9.9, range 33-72) years. Eighteen (72%) of the patients were men. Thoracic aortic aneurysm was presented in three (12%) patients...

OBJECTIVE: Penile fracture, being defined as rupture of the tunica albuginea of the corpus cavernosum, is uncommon. Here, we analyze findings on our patients during a 10-year period and evaluate the role of retrograde urethrography. METHODS: From February 2002 to April 2012, 116 patients were admitted with penile fracture at Ghaem Medical Center. Patient history and physical examination were taken at their admittance to detect probable urethral injury. Before surgery, retrograde urethrography was performed in all patients...

Acute thoracic aorta dissection is one the most dangerous pathology of aorta. If left untreated, is associated with high mortality. Early diagnosis and proper treatment improves outcomes. Miniinvasive procedures give the opportunity to plan the hybrid approach. In this way, all the advantages and opportunities of chosen methods can be used, minimising their complications. Multidisciplinary cooperation during the whole process of treatment, including out-patient care is crutial. Doing so creates the optimal conditions for the treatment by reducing operative trauma and improving the quality of life...

In development of intracranial aneurysms contribute genetic factors together with smoking, hypertension, diabetes mellitus. Epidemiology studies suggest that as many as 5% of people harbour a cerebral aneurysm by age 75. Rupture of cerebral aneurysm is the most frequent cause of spontaneous subarachnoid haemorrhage (up to 80%.) Annual incidence of SAH is 10-14/100 000, but only 15-20% of aneurysms will rupture, and that will happen probably between 40-60 years. The morbidity and mortality of aneurismal subarachnoid (SAH) continues to be high...

Retrospective analysis of the treatment results was done in 115 patients with Marphan's syndrome, operated on for the ascending aorta aneurysm. In 100 patients the complete replacement of ascending aorta and aortal valve was performed, using valvecontaining conduit with reimplantation of coronary arteries ostium into vascular prostheses (operation according to Bentalla--De Bono), in 62 of them--in our modification; in 6--the aortal valve prosthesis and aneurysmorrhaphy, in 7--the aortal valve prosthesis and aneurysmal resection with external enveloping of ascending aorta using vascular prosthesis (Robicsek operation); in 2--the valvepreserving operation on aortal valve with replacement of ascending aorta using vascular prosthesis...

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Excretion of hydroxyproline with urine was studied in 16 children (5-14 years old) with Marphan-Like syndrome and Marphan, Ehlers-Dunlos and Larson syndromes after therapy involving propranolol and a complex of vitamins (ascorbic acid, riboflavin and pyridoxine) and recommended on the basis of echocardiographic analyses. The therapeutic course appears to cause quantitative and qualitative correction of collagen and apparently of elastin fibrilles development. Depending on initial patterns of hydroxyproline excretion and the syndrome form the correction could be complete or partial, while positive effect of the treatment was stable or provisional...