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Cardiac Syndrome X
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1. Cardiac surgery unmasks latent hypoparathyroidism in a child with the 22q11.2 deletion syndrome.
Match Strength: 7.533

The 22q11.2 deletion syndrome is a developmental field defect of the third and fourth pharyngeal pouches characterized by a spectrum of thymic and parathyroid gland abnormalities and conotruncal cardiac defects. Latent hypoparathyroidism, defined as normocalcaemia at rest but reduced ability to secrete parathyroid hormone (PTH) in response to pharmacologically evoked hypocalcaemia, is found in 30-50% of people with this syndrome. Its natural history is unknown. We describe a 1.5 year-old girl with tetralogy of Fallot, normal calcium metabolism and few facial dysmorphic features who developed ... Read More »
» Published in J Pediatr Endocrinol Metab. 2006 Jul;19(7):943-6.

2. The diagnostic accuracy of 64-slice computed tomography coronary angiography compared with stress nuclear imaging in emergency department low-risk chest pain patients.
Match Strength: 6.372

STUDY OBJECTIVE: We compared the accuracy of multidetector computed tomography (CT) coronary angiography with stress nuclear imaging for the detection of an acute coronary syndrome or 30-day major adverse cardiac events in low-risk chest pain patients. METHODS: This was a prospective study of the diagnostic accuracy of myocardial perfusion imaging and multidetector CT in low-risk chest pain patients. The target condition was an acute coronary syndrome (confirmed >70% coronary stenosis on coronary artery catheterization) or major adverse cardiac events within 30 days. Patients were low risk ... Read More »
» Published in Ann Emerg Med. 2007 Feb;49(2):125-36. Epub 2006 Sep 15.

3. Epidemiology and peculiarities of pediatric multiple organ dysfunction syndrome in New Delhi, India.
Match Strength: 5.760

OBJECTIVE: Most pediatric studies on multiple organ dysfunction syndrome derive from developed countries. There is little information regarding the etiologies and outcomes of multiple organ dysfunction syndrome in critically ill children from developing countries. The objective of this study was to examine the differences in epidemiology of multiple organ dysfunction syndrome and the relationship of the Organ Failure Index (OFI) to outcomes from multiple organ dysfunction syndrome in critically ill children from a developing country. DESIGN: Prospective observational study. SETTING: A 10-bed ... Read More »
» Published in Intensive Care Med. 2006 Nov;32(11):1856-62. Epub 2006 Sep 15.

4. Cardiac arrhythmias and conduction disturbances in autoimmune rheumatic diseases.
Match Strength: 5.585

Rhythm and conduction disturbances and sudden cardiac death (SCD) are important manifestations of cardiac involvement in autoimmune rheumatic diseases (ARDs). In patients with rheumatoid arthritis (RA), a major cause of SCD is atherosclerotic coronary artery disease, leading to acute coronary syndrome and ventricular arrhythmias. In systemic lupus erythematosus (SLE), sinus tachycardia, atrial fibrillation and atrial ectopic beats are the major cardiac arrhythmias. In some cases, sinus tachycardia may be the only manifestation of cardiac involvement. The most frequent cardiac rhythm ... Read More »
» Published in Rheumatology (Oxford). 2006 Oct;45 Suppl 4:iv39-iv42.

5. Occult cardiac dysfunction in patients with obstructive sleep apnea syndrome revealed by tissue Doppler imaging.
Match Strength: 5.338

Obstructive sleep apnea syndrome has been linked to cardiovascular complications. The diagnosis of effects of sleep apnea on heart may be difficult before apparent examination findings. The aim of the present study was to evaluate changes of myocardial contractile properties in patients affected by sleep apnea showing tissue Doppler imaging, in the face of quite normal myocardial contractility on standard echocardiography. Shortly, tissue Doppler imaging may represent an early stage of myocardial abnormality despite a preserved global function ... Read More »
» Published in Int J Cardiol. 2006 Sep 21;

6. Clonidine attenuated early proinflammatory response in T-cell subsets after cardiac surgery.
Match Strength: 4.806

T-cells play a central role in the immune response to injury. Cardiac surgery is associated with significant risk of systemic inflammatory response syndrome and subsequent unbalanced induction of proinflammatory cytokines. As clonidine has immunomodulating properties via reducing sympathetic activity, this study involved the analysis of T-cell function in the early postoperative period in patients undergoing coronary artery bypass graft surgery. Forty patients undergoing cardiac surgery were randomly allocated to one of the following groups: clonidine group (n = 20) [clonidine 1 microg kg(-1) ... Read More »
» Published in Anesth Analg. 2006 Oct;103(4):809-14.

7. The natural history of Noonan syndrome: a long-term follow-up study.
Match Strength: 4.693

OBJECTIVE: To define better the adult phenotype and natural history of Noonan syndrome. DESIGN: A prospective observational study of a large cohort. RESULTS: Data are presented for 112 individuals with Noonan syndrome (mean age 25.3 (range 12-71) years), who were followed up for a mean of 12.02 years. Mutations in PTPN11 were identified in 35% of probands. Ten subjects died during the study interval; three of these deaths were secondary to heart failure associated with hypertrophic cardiomyopathy. Pulmonary stenosis affected 73 (65%) subjects; 42 (58%) required no intervention, nine underwent ... Read More »
» Published in Arch Dis Child. 2007 Feb;92(2):128-32. Epub 2006 Sep 21.

8. The management of hypoplastic left heart syndrome with a right aortic arch.
Match Strength: 4.691

The combination of hypoplastic left heart syndrome and a right-sided aortic arch is extremely rare and lethal. To the best of our knowledge, no patient with this combination has previously been reported as surviving initial palliation. The anatomic variant is associated with abnormalities in the arteries branching from the aortic arch, making it difficult to construct a reliable source of flow of blood to the lungs. We present here a patient with this combination who survived an initial Damus-Kay-Stansel procedure combined with placement of a conduit from the right ventricle to the pulmonary ... Read More »
» Published in Cardiol Young. 2006 Oct;16(5):504-6.

9. First trimester Down's syndrome screening shows high detection rate for trisomy 21, but poor performance in structural abnormalities--regional outcome results.
Match Strength: 4.686

OBJECTIVE: To evaluate whether first trimester screening markers are altered in pregnancies affected both by other chromosomal defects than trisomy 21 and structural anomalies and whether it is possible to detect these pregnancies by combined ultrasound and biochemical screening test. METHODS: Altogether 4,776 singleton pregnancies underwent first trimester screening. Of them, 3,101 women were screened using a combination of maternal serum free hCG, pregnancy-associated plasma protein A and nuchal translucency and 1,361 women with first trimester biochemistry without ultrasound. Nuchal ... Read More »
» Published in Fetal Diagn Ther. 2007;22(1):45-50. Epub 2006 Sep 22.

10. Recommendations for participation in leisure-time physical activity and competitive sports of patients with arrhythmias and potentially arrhythmogenic conditions Part II: ventricular arrhythmias, channelopathies and implantable defibrillators.
Match Strength: 4.385

This consensus paper on behalf of the Study Group on Sports Cardiology of the European Society of Cardiology follows a previous one on guidelines for sports participation in competitive and recreational athletes with supraventricular arrhythmias and pacemakers. The question of imminent life-threatening arrhythmias is especially relevant when some form of ventricular rhythm disorder is documented, or when the patient is diagnosed to have inherited a pro-arrhythmogenic disorder. Frequent ventricular premature beats or nonsustained ventricular tachycardia may be a hallmark of underlying pathology ... Read More »
» Published in Eur J Cardiovasc Prev Rehabil. 2006 Oct;13(5):676-86.

11. Laser epiglottopexy for laryngomalacia: 10 years' experience in the west of Scotland.
Match Strength: 4.307

OBJECTIVES: To determine the results of laser epiglottopexy and to compare them with other surgical techniques for severe laryngomalacia. DESIGN: A retrospective 10-year case note review of laser epiglottopexy for severe laryngomalacia. SETTING: Royal Hospital for Sick Children, Glasgow, Scotland. PATIENTS: The study population comprised 52 male and 24 female children who underwent surgery for laryngomalacia between January 1, 1993, and December 31, 2002. In all children except 2, the indication for surgery was stridor associated with poor feeding and failure to thrive. The age at presentation ... Read More »
» Published in Arch Otolaryngol Head Neck Surg. 2006 Sep;132(9):978-82.

12. Skipping of Exon 1 in the KCNQ1 gene causes Jervell and Lange-Nielsen syndrome.
Match Strength: 4.143

The Jervell and Lange-Nielsen syndrome (JLNS) is a rare autosomal recessive form of the long QT syndrome linked with a profound hearing loss caused by mutations affecting both alleles of either the KCNQ1 or the KCNE1 gene. We carried out a mutant screening of the KCNQ1 and KCNE1 genes in a clinical diagnosed German family with JLNS. Family members were examined by single strand conformation polymorphism analysis and PCR and amplified products were characterized by DNA sequence analysis. We identified a splice donor mutation of exon 1 in the KCNQ1 gene (G477+1A). Analysis of lymphocyte RNA by ... Read More »
» Published in J Biol Chem. 2006 Nov 17;281(46):35397-403. Epub 2006 Sep 19.

13. Maternal hemodynamic changes following treatment by laser coagulation of placental vascular anastomoses and amnioreduction in twin-to-twin transfusion syndrome.
Match Strength: 3.721

OBJECTIVES: To investigate maternal hemodynamic changes following laser therapy and amnioreduction in twin-to-twin transfusion syndrome (TTTS). METHODS: Thirty-nine cases with severe TTTS were treated at 16-26 weeks of gestation. Maternal hemodynamic evaluation was performed, including heart rate (HR), arterial pressure and echocardiography with calculation of shortening fraction (SF), left atrial dimensions, stroke volume (SV), cardiac output (CO) and total vascular resistance (TVR), before and 6 h and 24 h after placental surgery. Hemoglobin (Hb), hematocrit (Ht) and protein levels were also ... Read More »
» Published in Ultrasound Obstet Gynecol. 2006 Oct;28(5):670-3.

14. The impact of trauma activations on the care of emergency department patients with potential acute coronary syndromes.
Match Strength: 3.559

STUDY OBJECTIVE: Trauma systems improve the care of trauma patients; however, it is possible that prioritizing the emergency care of trauma patients might adversely affect other potentially ill patients requiring the same resources. We seek to determine whether the presence of a concurrent trauma activation negatively affects processes of care and outcomes for patients with potential acute coronary syndromes. METHODS: Patients who presented to the emergency department (ED) with a potential acute coronary syndrome from July 2003 to June 2004 were stratified according to whether they presented ... Read More »
» Published in Ann Emerg Med. 2006 Oct;48(4):347-53. Epub 2006 Apr 21. Comment in: Ann Emerg Med. 2006 Oct;48(4):355-7.

15. Pharmacogenetics and anti-arrhythmic drug therapy: a theoretical investigation.
Match Strength: 3.532

Pharmacological management of cardiac arrhythmias has been a long and widely sought goal. One of the difficulties in treating arrhythmia stems, in part, from incomplete understanding of the mechanisms of drug block and how intrinsic properties of channel gating affect drug access, binding affinity, and unblock. In the last decade, a plethora of genetic information has revealed that genetics may play a critical role in determining arrhythmia susceptibility and in efficacy of pharmacological therapy. In this context, we present a theoretical approach for investigating effects of drug-channel ... Read More »
» Published in Am J Physiol Heart Circ Physiol. 2007 Jan;292(1):H66-75. Epub 2006 Sep 22.

16. A Large-Scale Analysis of Ion Channel Gene Expression in the Mouse Heart during Perinatal Development.
Match Strength: 2.756

The immature and mature heart differs from each other in terms of its excitability, action potential properties, contractility and relaxation. This includes upregulation of repolarizing K(+) currents, an enhanced inward rectifier K(+) current and changes in Ca(2+), Na(+) and Cl(-) currents. At the molecular level, the developmental regulation of ion channels is scantily described. Using a large-scale real-time quantitative reverse transcriptase polymerase chain reaction (qRT-PCR) assay, we performed a comprehensive analysis of ion channel transcript expression during perinatal development in ... Read More »
» Published in Physiol Genomics. 2006 Sep 19;

17. Relief of acute pain in chronic idiopathic gastroparesis with intravenous phentolamine.
Match Strength: 2.504

OBJECTIVE: To report a case in which complete relief of pain associated with gastroparesis, with promotion of gastric emptying, was achieved with administration of phentolamine. CASE SUMMARY: A 37-year-old opiate-tolerant female with a history of recurrent abdominal pain, gastroparesis, cyclic vomiting syndrome, and migraine headaches was admitted to the emergency department (ED) with severe acute abdominal pain, nausea, and vomiting. The patient had been previously implanted with a permanent gastric electrical stimulator and she was adherent to her prokinetic, antiemetic, analgesic, and ... Read More »
» Published in Ann Pharmacother. 2006 Nov;40(11):2032-6. Epub 2006 Sep 26.

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