Surgical Management of Marfan Syndrome in Children

Between August 1983 and January 1991, seven patients with Marfan syndrome underwent surgery for severe cardiovascular complications. The mean age at presentation was 5.7 months (range 4 to 9 months) in the infant group (n = 3), and 13.3 years (range 10 to 16 years) in a group of older children (n = 4). The primary indications for surgery in the infant group (performed at a mean of 3 years after diagnosis) were ascending aortic aneurysm with valvar regurgitation in one patient, and severe mitral valve prolapse with regurgitation in two. In the older group, surgical indications (performed at a mean of 2.8 years after diagnosis) were ascending aortic aneurysm with valvar regurgitation in three patients and acute aortic dissection in one. For aortic surgery, a composite valved conduit was used in four patients, and an aortic homograft in one. For mitral valve surgery, mechanical prostheses were used. Ail patients survived the primary operation. Over a mean follow-up of 17.5 patient-years (range 1 to 9 years), two patients in the infant Marfan group went on to further successful surgery (prosthetic mitral valve replacement and aortic root repair with aortic homograft) at a mean interval of 4.3 years after the Initial surgery. Our results suggest that the major cardiovascular risk factors of Marfan syndrome in the young, even in those diagnosed during infancy, have been favorably changed by surgery with an encouraging medium-term outlook. The correct timing of surgery is aided by echocardiography. (J Card Surg 1994;9:50–54)     

Effects of preparation techniques on the porosity of acrylic cements

We investigated four acrylic cement preparation techniques for their effects on cement porosity: hand mixing, pressurization in a pneumatic pistol, centrifugation, and vacuum mixing. All the techniques were tested on three types of cement with different viscosity characteristics. The best results were obtained with vacuum mixing using a newly designed experimental system, yielding porosity reductions of 60-80 percent relative to hand mixing. Vacuum mixing with a commercial system was also effective, but to a somewhat lesser extent.

Pressurization and centrifugation had no substantial effect on the overall porosity. Centrifugation led to considerable nonuniformity in the distribution of pores and additives.     

Short utterance recognition using a network with minimum training

A feedforward network is used to recognize short, digitized, isolated utterances. A high, multispeaker recognition rate is achieved with a small vocabulary with a single training utterance. This approach makes use of the pattern recognition property of the network architecture to classify different temporal patterns in the multidimensional feature space. The network recognizes the utterances without the need of segmentation, phoneme identification, or time alignment. We train the network with four words spoken by one single speaker. The network is then able to recognize 20 tokens spoken by 5 other speakers. We repeat the above training and testing procedure using a different speaker's utterances for training each time. The overall accuracy is 97.5%. We compare this approach to the traditional dynamic programming (DP) approach, and find that DP with slope constraints of 0 and 1 achieve 98.5% and 85% accuracies respectively. Finally we validate out statistics by training and testing the network of a four-word subset of the Texas Instruments (Tl) isolated word database. The accuracy with this vocabulary exceeds 96%. By doubling the size of the training set, the accuracy is raised to 98%. Using a suitable threshold, we are able to raise the accuracy of one network from 87% to 98.5%. Thresholding applied to all networks would then raise the overall accuracy to well over 99%.

This technique is especially promising because of the low overhead and computational requirements, which make it suitable for a low cost, portable, command recognition type of application.     

Raman and UV–vis spectroscopic study of polyaniline membranes containing a bulky cationic additive

Electrically conducting soluble polyaniline (PANI), containing different amounts of a bulky lipophilic cationic additive, tridodecylmethylammonium chloride (TDMACl), was studied by Raman (λ_exc=780 nm) and UV–vis spectroscopy. PANI was made simultaneously electrically conducting and soluble with bis[4-(1,1,3,3-tetramethylbutyl)phenyl]phosphoric acid in dichloromethane. The PANI membranes were prepared by drop casting on glassy carbon or ITO substrates. Raman and UV–vis measurements were carried out in a 0.1 M CaCl₂ solution at potentials between 400 and ?600 mV (vs. SCE) at pH 6, or alternatively at the open circuit potential at pH 10. The results of Raman, UV–vis and cyclic voltammetric measurements confirm that the incorporation of TDMACl into the PANI membrane facilitates the oxidation and reduction of PANI.     

Increased numbers of higher left intraocular pressures in 428 patients referred to the glaucoma clinic

Seventy-four per cent of 428 patients referred to the glaucoma clinic at Concord Hospital exhibited intraocular pressure (IOP) asymmetry. There was a preponderance of left eyes, in that two-thirds of the patients with asymmetrical pressures had the higher IOP in the left eye. As the degree of asymmetry increased, this trend became more pronounced. In addition, the statistically significant difference between right and left mean IOP among the glaucoma patients was not found among normal controls, even when the order of testing the eyes was reversed.     

Intracapsular tonsillar reduction (partial tonsillectomy): reviving a historical procedure for obstructive sleep disordered breathing in children.

OBJECTIVE: We sought to reintroduce a historical procedure-intracapsular tonsillar reduction (partial tonsillectomy or tonsillotomy)-for tonsillar hypertrophy causing obstructive sleep disordered breathing (OSDB) in children, as well as to determine whether partial tonsillectomy, compared with conventional (total) tonsillectomy when performed by more than one surgeon, is equally effective for the relief of OSDB while resulting in less pain and more rapid recovery. STUDY DESIGN: We conducted a retrospective case series at a tertiary children's hospital. The charts of children who underwent partial tonsillectomy and total tonsillectomy (1998 through 2002) for postoperative complications were reviewed. The caregivers were surveyed to assess postoperative pain, rapidity of recovery, and effectiveness of surgery for relieving symptoms of OSDB. RESULTS: Two hundred forty-three children underwent partial tonsillectomy and 107 children underwent total tonsillectomy. There were no significant differences in immediate and delayed complications between the groups. Both operations were equally effective in relieving OSDB. Children who had partial tonsillectomy had significantly less postoperative pain and significantly more rapid recovery. CONCLUSION: Intracapsular tonsillar reduction with an endoscopic microdebrider relieves OSDB as effectively as conventional tonsillectomy, but results in less postoperative pain and a more rapid recovery.     

Working for patients: further implications for nurse education.

The National Health Service and Community Care Act (1990) has focused attention on all aspects of health care. This paper explores the wider implications of the White Paper on nurse education. It examines the issues of student numbers; rationalisation of courses; the impact of contracts; quality control and clinical placements. It further considers the issues of capital assets; educational premises; funding and income generation. The response of schools and colleges of nursing to these challenges has yet to be evaluated. Nurse education is steering an uncertain course in turbulent and uncharted waters. If colleges of nursing are to capitalise on the changes demanded by the White Paper, the prime concern must be the establishment of an advantageous position from which to market viable courses. To this end there must be adequate resources, and control over high quality learning environments. The courses must be educationally led and respond to the learning needs of all students.     

Postprandial acid reflux is reduced by delayed gastric emptying.

The aim of this study was to investigate influence of delayed gastric emptying on postprandial reflux in esophageal pH. Sixty-nine consecutive patients underwent 24 hour (h) esophageal pH monitoring and gastric emptying. In 24 h esophageal pH monitoring, % postprandial reflux pH<4 for 2 h after each meal (% PRT) was extracted from the 24 h pH profile. After solid test meal (1 mCi, Tc99m) was given, gastric emptying was measured with a gamma detector placed transnasally 5 cm below lower esophageal sphincter. % PRT was similar among the 34 normal, 26 delayed and 9 rapid gastric emptying rate patients. Thirty-five with a positive pH study and 34 with a negative had a similar prevalence of gastric emptying disorder. In the positive pH study group, patients with normal gastric emptying had significantly higher % PRT than those with delayed gastric emptying (22.0 vs 12.1%, P<0.05). In the same population, patients with a normal %PRT had a significantly higher prevalence of delayed gastric emptying compared with those with a positive % PRT (6/8 vs 9/27, P<0.05). In patient with abnormal acid exposure but normal % PRT on 24 h esophageal pH monitoring, gastric emptying may be delayed.     

The primary motor and premotor areas of the human cerebral cortex.

Brodmann's cytoarchitectonic map of the human cortex designates area 4 as cortex in the anterior bank of the precentral sulcus and area 6 as cortex encompassing the precentral gyrus and the posterior portion of the superior frontal gyrus on both the lateral and medial surfaces of the brain. More than 70 years ago, Fulton proposed a functional distinction between these two areas, coining the terms primary motor area for cortex in Brodmann area 4 and premotor area for cortex in Brodmann area 6. The parcellation of the cortical motor system has subsequently become more complex. Several nonprimary motor areas have been identified in the brain of the macaque monkey, and associations between anatomy and function in the human brain are being tested continuously using brain mapping techniques. In the present review, the authors discuss the unique properties of the primary motor area (M1), the dorsal portion of the premotor cortex (PMd), and the ventral portion of the premotor cortex (PMv). They end this review by discussing how the premotor areas influence M1.     

Novel pore mutation in SCN5A manifests as a spectrum of phenotypes ranging from atrial flutter, conduction disease, and Brugada syndrome to sudden cardiac death

OBJECTIVES: The purpose of this study was to determine the clinical and biophysical characteristics of a novel SCN5A mutation. BACKGROUND: Brugada syndrome and isolated cardiac conduction defect have been linked to SCN5A mutations. METHODS: Eleven members of a western European family underwent electrophysiologic investigations and mutation analysis of the SCN5A gene. Wild-type and mutant SCN5A channels were expressed in HEK293 cells, and whole cell currents were studied using patch clamp procedures. RESULTS: A novel mutation, R376H, in the first pore segment of SCN5A variably causes Brugada syndrome and/or conduction disease in a single family. Biophysical analysis demonstrated a significant current reduction for the mutant, a pathophysiologic profile consistent with Brugada syndrome and isolated cardiac conduction defect. Among 11 family members, 9 were carriers of the mutation. The proband's initial presentation was a saddleback Brugada ECG, atrial flutter, and diffuse conduction disturbances. He had no inducible ventricular arrhythmias but experienced sudden cardiac death. His brother was affected by atrial flutter and had a clear conduction disorder, but he did not display baseline or evocable ECG signs of Brugada syndrome. He received an implantable cardioverter-defibrillator that delivered one appropriate shock after 1 year of follow-up. The phenotype in the family members was highly variable and ranged from noninducible and inducible asymptomatic carriers of the mutations to isolated conduction disease and to symptomatic Brugada syndrome. CONCLUSIONS: We describe the functional characterization of a novel SCN5A pore mutation, R376H, with variable clinical expression in the same family. Differentiating between electrophysiologic entities (Brugada syndrome-isolated cardiac conduction defect) is more challenging. Recognition of factors modifying the clinical presentation may be important for clinical decision making.