Presentation
A synthesis of the available management methods is presented in Figure 4. Figure 4. Management of LQTS. [romanianjournalcardiology.ro]
Kaveggia in 1974,[2] its major clinical features include intellectual disability, hyperactivity, hypotonia (low muscle tone), and a characteristic facial appearance including macrocephaly (an abnormally large head).[3] Presentation[edit] FG syndrome's [en.wikipedia.org]
Skin
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Hyperkeratosis
KRT1 Striate palmoplantar keratoderma 3 Epidermolytic hyperkeratosis IHCM KRT2E (Ichthyosis bullosa of Siemens) KRT3 (Meesmann juvenile epithelial corneal dystrophy) KRT4 (White sponge nevus) KRT5 (Epidermolysis bullosa simplex) KRT8 (Familial cirrhosis [en.wikipedia.org]
Urogenital
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Kidney Failure
Bakris, G.L. (2003) Kidney failure and cardiovascular disease. Circulation, 108(16): 114e [ CR ] [ PM ] [ EČ ][ 3 ][ GS ] Goldsmith, D.J., Covic, A. (2001) Coronary artery disease in uremia: Etiology, diagnosis, and therapy. [scindeks.ceon.rs]
Workup
Blocks
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2:1 Atrioventricular Block
Timothy syndrome may include a functional 2/1 atrioventricular block, due to an exceedingly long QT interval5,19,48. [romanianjournalcardiology.ro]
Rhythm
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Torsades De Pointes
The onset of the pause-dependent torsade de pointes. VPB ventricular premature beat. Data in references 30,32. [romanianjournalcardiology.ro]
Treatment
Jardine, A. (2008) Diagnosis and treatment of coronary artery disease in patients with chronic kidney disease. [scindeks.ceon.rs]
(December 2017) Treatment[edit] This section is empty. You can help by adding to it. (December 2017) History[edit] Kim Peek (1951–2009) probably had FG syndrome. [en.wikipedia.org]
The risk stratification for a first cardiac event in life, before age of 40 and without treatment in congenital LQTS types 1, 2 and 3, depending upon QTc value and gender. [romanianjournalcardiology.ro]
Prognosis
Haase-Fielitz, A. (2009) Accuracy of neutrophil gelatinase-associated lipocalin (NGAL) in diagnosis and prognosis in acute kidney injury: A systematic review and meta-analysis. [scindeks.ceon.rs]
Etiology
Covic, A. (2001) Coronary artery disease in uremia: Etiology, diagnosis, and therapy. [scindeks.ceon.rs]
Etiology of sudden death in an apparently normal heart (7-10) Congenital LQTS inside the genetic anomalies of the ion channels So far, there is a spectrum of inherited arrhythmia syndromes arising from genetic defects in structures involved in the genesis [romanianjournalcardiology.ro]
Pathophysiology
Heart, 94(8): 1080 [ CR ] [ PM ] [ EČ ][ 10 ][ GS ] London, G.M. (2003) Cardiovascular disease in chronic renal failure: Pathophysiologic aspects. Semin Dial, 16(2): 85-94 [ CR ] [ EČ ][ 4 ][ GS ] Mccullough, P.A. (2007) Coronary artery disease. [scindeks.ceon.rs]
The role of the sympathetic nervous system in the long QT syndrome: the long road from pathophysiology to therapy. Long QT syndrome. Cardiac Electrophysiology Clinics, volume 4, number 1. Editors: Priori SG, Thakur RK, Natale A. [romanianjournalcardiology.ro]
Prevention
Clinical Journal of the American Society of Nephrology, 2(3): 611 [ GS ] Mccullough, P.A. (2010) Prevention of cardiorenal syndromes. u: Ronco C, Bellomo R, Mccullough PA [ur.] [scindeks.ceon.rs]
The ICD is recommended (class I) for the secondary prevention of sudden cardiac death after an aborted cardiac arrest, it is useful (IIa) in the secondary prevention of both syncope and TdP and it is suggested (IIb) for the asymptomatic patient carrying [romanianjournalcardiology.ro]