Repeated invasive electrophysiological studies in a 7-year-old boy with the classic Jervell and Lange-Nielsen syndrome disclosed increased ventricular refractoriness, unusual late diastolic endocardial waveforms, and the inability to induce ventricular fibrillation. Despite apparently beneficial electrophysiological responses to left stellate block, surgical left cervical sympathectomy was foll...

Recessive mutations in the ion channel-encoding KCNQ1 gene may cause Jervell and Lange-Nielsen syndrome (JLNS), a fatal cardiac disease leading to arrhythmia and sudden cardiac death in young patients. Mutations in KCNQ1 may also cause a milder and dominantly inherited form of the disease, long QT syndrome 1 (LQT1). However, why some mutations cause LQT1 and others cause JLNS can often not be u...

Patients with hereditary long QT disorders--Romano-Ward Syndrome and Jervell Lange-Nielsen Syndrome--sometimes present with seizures due to cardiac tachyarrhythmias. Two such patients are presented, emphasizing diagnostic clues--syncope, seizures with onset in early life, precipitation of attacks by emotional or physical stress, positive family history of sudden death and/or seizures, normal EE...

TAYLOR, C. RICHARD, KNUT SCHMIDT-NIELSEN, AND JACOB L. RAAB, Scaling of energetic cost of running to body size in mammals. Am. J. Physiol. 219(4) : 1104-l 107. 1970.-The purpose of this study was to see if a convenient relationship could be found between the energetic cost of running and the body weight of mammals. We found that steady-state oxygen consumption of seven groups of mammals (21 g-1...

Each year during Experimental Biology (EB), an informal class reunion takes place at the President’s Reception that is traditionally documented by a group picture of the current, past, and elected presidents in attendance. Marty Frank, APS Executive Director, shared the most recent one a few days after EB. This year’s presidents’ picture – with a distinctive and impressive group of women front ...

TAYLOR, C. RICHARD, RAZI DMI'EL, MICHAEL FEDAK, AND KNUT SCHMIDT-NIELSEN. Energetic cost of running and heat balance in a large bird, the rhea. Am. J. Physiol. 221(Z): 597-601. 1971.-The energetic cost of running in the rhea (Rhea americana), determined as oxygen consumption, increased linearly with increasing speed. Oxygen consumption (M, ml 02 g-1 hr-I) relative to running velocity (v, km hr-...

Strain 2362 sphaericus B.

Congenital long QT syndrome is a rare hereditary disease that is related to the dysfunction of ion channels in cardiac cells. We report on a very rare case of its autosomal recessive form--the Jervell-Lange Nielsen syndrome--in a Pakistani family, which was diagnosed after the incidental finding of bradycardia in a newborn baby girl. We discuss the range of presentations in neonates; the import...

Introduction Long QT syndrome (LQTS) is a group of genetically distinct arrhythmogenic disorders resulting in abnormal cardiac sodium and potassium ion channels causing delayed repolarization of the heart. This syndrome can present clinically as syncope, seizures, or sudden cardiac death secondary to its characteristic ventricular arrhythmia of torsades de pointes. The clinical and electrocardi...

Each of us as a scientist has an academic legacy that consists of our mentors and their mentors continuing back for many generations. Here, I describe two genealogies of my own: one through my PhD advisor, H. T. (Ted) Hammel, and the other through my postdoctoral mentor, Knut Schmidt-Nielsen. Each of these pathways includes distingished scientists who were all major figures in their day. The st...