scholarly journals Hutchinson-Gilford Progeria Syndrome in a Young Man

2018 ◽  
Vol 5 (4) ◽  
pp. 3727-3728
Author(s):  
Maryam. Moinazad Tehrani MD ◽  
Agin Khosrow MD

Hutchinson-Gilford progeria syndrome (HGPS) is an extremely rare genetic disorder. It shows a characteristic progeria phenotype. The average life expectancy of HGPS patients is reported to be  ~ 14.6 years. A case was a 33-year-old man with progeria phenotype, severe cardiac failure, and convulsion. He had several cardiac surgeries include Mitral and Aortic valve replacement, Atrial Septum Defect (ASD) closure and Coronary Artery Bypass Graft (CABG) .

2019 ◽  
Vol 11 (4) ◽  
pp. NP244-NP246
Author(s):  
Rui Pedro Soares Cerejo ◽  
Rui A. N. Rodrigues ◽  
José D. Martins ◽  
Carolina G. E. C. Torres ◽  
Lídia M. Sousa ◽  
...  

Hutchinson-Gilford progeria syndrome is a rare genetic disorder, characterized by progressive premature aging and early death in the first or second decade of life, usually secondary to cardiovascular events (myocardial infarction and stroke). We report a case of a 14-year-old boy with progeria syndrome and cardiac arrest due to myocardial infarction, who was submitted to an immediate coronary angiography which revealed left main stem and three-vessel coronary artery disease. A prompt double bypass coronary artery grafting surgery was performed, and, despite successful coronary reperfusion, the patient remained in coma and brain death was declared on fourth day after surgery.


1987 ◽  
Vol 57 (01) ◽  
pp. 55-58 ◽  
Author(s):  
J F Martin ◽  
T D Daniel ◽  
E A Trowbridge

SummaryPatients undergoing surgery for coronary artery bypass graft or heart valve replacement had their platelet count and mean volume measured pre-operatively, immediately post-operatively and serially for up to 48 days after the surgical procedure. The mean pre-operative platelet count of 1.95 ± 0.11 × 1011/1 (n = 26) fell significantly to 1.35 ± 0.09 × 1011/1 immediately post-operatively (p <0.001) (n = 22), without a significant alteration in the mean platelet volume. The average platelet count rose to a maximum of 5.07 ± 0.66 × 1011/1 between days 14 and 17 after surgery while the average mean platelet volume fell from preparative and post-operative values of 7.25 ± 0.14 and 7.20 ± 0.14 fl respectively to a minimum of 6.16 ± 0.16 fl by day 20. Seven patients were followed for 32 days or longer after the operation. By this time they had achieved steady state thrombopoiesis and their average platelet count was 2.44 ± 0.33 × 1011/1, significantly higher than the pre-operative value (p <0.05), while their average mean platelet volume was 6.63 ± 0.21 fl, significantly lower than before surgery (p <0.001). The pre-operative values for the platelet volume and counts of these patients were significantly different from a control group of 32 young males, while the chronic post-operative values were not. These long term changes in platelet volume and count may reflect changes in the thrombopoietic control system secondary to the corrective surgery.


2006 ◽  
Vol 55 (5) ◽  
pp. 451
Author(s):  
Seung Ho Joo ◽  
Byoung Wook Choi ◽  
Jae Seung Seo ◽  
Young Jin Kim ◽  
Tae Hoon Kim ◽  
...  

2010 ◽  
Vol 13 (1) ◽  
pp. 60 ◽  
Author(s):  
Cenk Eray Yildiz ◽  
Murat Sayin ◽  
Halit Yerebakan ◽  
Suha Kucukaksu

The importance of minimally invasive cardiac operations, performed off-pump, without the support of cardiopulmonary bypass (CPB), is continuously increasing. Complete revascularization of obstructed coronary arteries is needed to obtain a better long-term outcome. Insertion into the left ventricle of an efficient microaxial pump can be useful when targeting an important coronary artery located at posterior wall of the heart in a patient with hemodynamic deficiency. The use of such a device can enable surgeons to avoid conversion from a preplanned off-pump strategy to traditional on-pump coronary bypass surgery. The Impella Microaxial Ventricular Assist Device (VAD) (Abiomed, Aachen, Germany) is a miniature pump with a 7-mm catheter and a flow rate of approximately 2.5-5 L/min. This device can enable cardiovascular surgery to be performed without damaging the left ventricle and causing serious aortic deficiency. Therefore, in patients with serious comorbidity, complete revascularization may be performed off pump, with the heart beating, because of the hemodynamic stability provided with the support of the microaxial intracardiac pump. If required, this pump can also support the heart during the early postoperative period. We report the first assisted beating-heart coronary artery bypass graft surgery performed with the Impella Microaxial VAD in our country. The surgery was performed on 2 patients considered high risk on the basis of EUROSCORE testing.


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