Prophylactic levothyroxine therapy in patients with Hashimoto's thyroiditis.
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Animals↗
Biomedical subjects
K Heller
Publications and source records attributed to K Heller.
Portion-size estimation training in second- and third-grade American Indian children.
Training in portion-size estimation is known to improve the accuracy of dietary self-reporting in adults, but there is no comparable evidence for children. To obtain this information, we studied 110 second- and third-grade American Indian schoolchildren (34 control subjects were not trained), testing the hypotheses that a 45-min portion-size estimation training session would reduce children's food quantity estimation error, and that the improvement would be dependent on food type, measurement type, or both. Training was a hands-on, 4-step estimation and measurement skill-building process. Mixed linear models (using logarithmic-transformed data) were used to evaluate within- and between-group differences from pre- to posttest. Test scores were calculated as percentage estimation errors by difference and absolute value methods. Mean within-group estimation error decreased significantly (P<0.05) from pre- to posttest for 7 of 12 foods (trained group) by both calculation methods, plus 3 additional foods by the difference method and one additional food by the absolute value method. Significant (P<0.05) between-group differences occurred for 3 foods, reflecting a greater decrease in estimation error for the trained group. Improvement was greatest for solid foods estimated by dimensions (P>0.05) or in cups (P<0.05), for liquids estimated by volume or by label reading (P<0.001), and for one amorphous food estimated in cups (P<0.01). Despite these significant improvements in estimation ability, the error for several foods remained >100% of the true quantity, indicating that more than one training session would be necessary to further increase dietary reporting accuracy.
Asian People↗
Development patterns of telomerase activity in barley and maize.
Eukaryotic chromosomes terminate with specialized structures called telomeres. Maintenance of chromosomal ends in most eukaryotes studied to date requires a specialized enzyme, telomerase. Telomerase has been shown to be developmentally regulated in man and a few other multicellular organisms, while it is constitutively expressed in unicellular eukaryotes. Recently, we demonstrated telomerase activity in plant extracts using the PCR-based TRAP (Telomeric Repeat Amplification Protocol) assay developed for human cells. Here we report telomerase activities in two grass species, barley and maize, using a modified, semi-quantitative TRAP assay. Telomerase was highly active in very young immature embryos and gradually declined during embryo development. The endosperm telomerase activity was detectable, but significantly lower than in the embryo and declined during kernel development with no detectable activity in later stages. Telomerase activity in dissected maize embryo axis was several orders of magnitude higher than in the scutellum. Telomerase activity was not detected in a range of differentiated tissues including those with active meristems such as root tips as well as the internode and leaf base. The role of telomerase repression during differentiation and the relationship between chromosome healing and telomerase activity is discussed.
Base Sequence↗
Highly attenuated modified vaccinia virus Ankara replicates in baby hamster kidney cells, a potential host for virus propagation, but not in various human transformed and primary cells.
Although desirable for safety reasons, the host range restrictions of modified vaccinia virus Ankara (MVA) make it less applicable for general use. Propagation in primary chicken embryo fibroblasts (CEF) requires particular cell culture experience and has no pre-established record of tissue culture reproducibility. We investigated a variety of established cell lines for productive virus growth and recombinant gene expression. Baby hamster kidney cells (BHK), a well-characterized, easily maintained cell line, supported MVA growth and as proficient expression of the E. coli lacZ reporter gene as the highly efficient CEF, whereas other cell lines were non-permissive or allowed only very limited MVA replication. Importantly, no virus production occurred in patient-derived infected primary human cells. These results emphasize the safety and now improved accessibility of MVA for the development of expression vectors and live recombinant vaccines.
Animals↗
[Long-term outcome after surgical treatment of gastroesophageal reflux in infancy and childhood].
In this series 99 out of 131 children have been operated upon gastroeosophageal reflux at an age of 2.5 years. They were reevaluated 4.5 years later for their longterm results in 1997. All patients have been primary free of reflux postoperatively. By questionnaire, x-ray studies and 24 h/ph-metry signs of reflux could not be found in 88/99 children. 4 patients proved to suffer from a recurrant reflux and 3 out of these had to be reoperated. Another 7 showed a mild, not pathological reflux without needing medical treatment. Looking at the recurrencies especially preoperative peptic oesophagitis and stenoses as well as brachyoesophagus after oesophageal atresia turned out to be a problem in respect to prognosis. Control studies for longterm results should be done at least 1 year postoperatively.
Child↗
Mortality benefit of implantable cardioverter-defibrillator therapy in patients with persistent malignant ventricular arrhythmias despite amiodarone treatment.
Implantable cardioverter defibrillators (ICDs) are very effective in preventing sudden cardiac death. However, debate continues as to whether ICD implantation is superior to amiodarone in prolonging survival in patients with life-threatening ventricular arrhythmias. Of 442 consecutive patients treated with amiodarone, we identified 48 patients with symptomatic ventricular arrhythmias who met all of the following inclusion criteria: (1) had inducible sustained ventricular tachycardia at baseline electrophysiologic study, (2) had an oral amiodarone load of at least 10 g over 10 to 14 days, (3) remained inducible with a hemodynamically unstable ventricular arrhythmia at follow-up electrophysiologic study, and (4) were advised to continue amiodarone therapy and undergo ICD implantation. Patients who agreed to undergo ICD implantation (n = 28) had a lower ejection fraction (29 +/- 9% vs 40 +/- 12% p <0.005) and were younger (61.0 +/- 10 vs 69 +/- 7 years, p <0.01) than patients who refused device implantation (n = 20). Using a Cox proportional-hazards model, defibrillator therapy was the strongest independent predictor of improved survival in patients with an ejection fraction < or =40% (RR = 0.42; 95% confidence interval 0.22 to 0.79). Thus, patients with depressed ejection fraction and continued inducibility of sustained ventricular tachycardia despite oral amiodarone loading have a poor prognosis. In such patients, ICDs are associated with a 58% reduction in total cardiac mortality.
Aged↗
Congenital microgastria, growth hormone deficiency and diabetes insipidus.
UNLABELLED: Microgastria is a rare malformation of the stomach always associated with variable patterns of malformations of the lung, heart, aortic arch, skeleton, and central nervous system. Many cases present with asplenia and hepatic symmetry as well as intestinal malrotation. We report a first case of a 4.5-year-old girl with congenital microgastria in association with growth hormone deficiency, diabetes insipidus, brachyoesophagus, hernia of the diaphragm, gastro-oesophageal reflux, intestinal malrotation, enlarged symmetrical liver, asplenia, as well as mental and statomotor retardation. CONCLUSION: Congenital microgastria is always associated with malformations of other organs. Patients at any age presenting one of the symptoms: failure to thrive, vomiting, asplenia, midline defects and parts of the VACTERL association should be carefully examined to exclude microgastria.
Abnormalities, Multiple↗
The treatment of atrial fibrillation: pharmacologic and nonpharmacologic strategies.
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Anti-Arrhythmia Agents↗
[Simultaneous use of different nuclear medical examinations in Klippel-Trenaunay syndrome--vs. Proteus syndrome].
A three-year-old male patient presented already at his birth a disproportion macrosomia of the left foot and a large, nodular nevus flammeus in the left hip region, which led to the tentative diagnosis of a Klippel-Trenaunay syndrome. In the following years, both changes showed a continuous progression, with distinct soft-tissue swelling as well as papillomatous and verruciform vegetations of the nevus. Additionally, large, plain subcutaneous masses developed under the right shoulder, and a macrodactyly of the first and second left toe could be observed. Although several examinations had been performed in the meantime, the tentative diagnosis could not be confirmed up to that time. On the occasion of a severe local infection in the hip region, which led to the consideration of a surgical therapy, a radionuclide lymphography, a blood pool scintigraphy including dynamic phlebography and ventriculography as well as a bone scintigraphy were performed. These examinations were done simultaneously at one day in order to avoid a longer period of immobilization. The findings led to the diagnosis of a large lymphangioma, which could be confirmed histologically after surgery. In consideration of all results, the basic disorder seems to be the rare proteus syndrome rather than a Klippel-Trenaunay syndrome.
Bone and Bones↗
Mode of onset of torsade de pointes in congenital long QT syndrome.
OBJECTIVES: We sought to describe the mode of onset of spontaneous torsade de pointes in the congenital long QT syndrome. BACKGROUND: Contemporary classifications of the long QT syndrome (LQTS) refer to the congenital LQTS as "adrenergic dependent" and to the acquired LQTS as "pause dependent." Overlap between these two categories has been recognized, and a subgroup of patients with "idiopathic pause-dependent torsade" has been described. However, it is not known how commonly torsade is preceded by pauses in the congenital LQTS. METHODS: We reviewed the electrocardiograms (ECGs) of all our patients with congenital LQTS evaluated for syncope or sudden death (30 patients). Documentation of the onset of torsade de pointes was available for 15 patients. All these patients had "definitive LQTS" by accepted clinical and ECG criteria. RESULTS: Pause-dependent torsade de pointes was clearly documented in 14 of the 15 patients (95% confidence interval 68% to 100%). The cycle length of the pause leading to torsade was 1.3 +/- 0.2 times longer than the basic cycle length, and most pauses leading to torsade were unequivocally longer than the preceding basic cycle length (80% of pauses were > 80 ms longer than the preceding cycle length). CONCLUSIONS: The "long-short" sequence, which has been recognized as a hallmark of torsade de pointes in the acquired LQTS, plays a major role in the genesis of torsade in the congenital LQTS as well. Our findings have important therapeutic implications regarding the use of pacemakers for prevention of torsade in the congenital LQTS.
Adult↗
Telomerase activity in plant extracts.
Telomeres of most eucaryotes terminate in long stretches of short, guanine-rich repeats. Telomerase, a specialized enzyme with reverse transcriptase-like activity, has been shown to synthesize these repeats in many lower eucaryotes and several animal species. Although a sequence (TTTAGGG)n that matches the eucaryotic consensus sequence Tx(A)Gy is present in several plant species, the activity and expression patterns of plant telomerase have not been reported. Here we document the presence of telomerase activity in plant tissues using a modification of the human Telomeric Repeat Amplification Protocol (TRAP) assay. Telomerase activity was detected in barley embryo, anther and carpel tissues and in immature seeds of Arabidopsis thaliana, but not in barley leaf tissue.
Arabidopsis↗
Current therapy for Crigler-Najjar syndrome type 1: report of a world registry.
This study represents a multicenter survey on the management of patients with Crigler-Najjar syndrome (CNS) type 1. The aim of the survey was to find guiding principles for physicians in the care of these patients. Fifty-seven patients were included. At the time of inclusion, 21 patients had received a liver transplant (37%). The average age at transplantation was 9.1 +/- 6.9 years (range, 1-23 years); the age of the patients who had not been transplanted at the time of inclusion was 6.9 +/- 6.0 years (range, 0-23 years). Brain damage had developed in 15 patients (26%). Five patients died, and 10 are alive with some degree of mental or physical handicap. In 2 patients, ages 22 and 23 years, early signs of bilirubin encephalopathy could be reversed, in 1 by prompt medical intervention followed by liver transplantation and in the other by prompt liver transplantation. Seven patients underwent transplantation with some degree of brain damage at the time of the surgery; 1 of these died after retransplantation, 2 improved neurologically, and 4 remained neurologically impaired. The age of 8 patients with and 13 without brain damage at or before transplantation was 14.3 +/- 5.9 and 5.9 +/- 5.4 years (P < .01), respectively. Therapy of CNS type 1 consists of phototherapy (12 h/d), followed by liver transplantation. Phototherapy, although initially very effective, is socially inconvenient and becomes less efficient in the older age group, thus also decreasing compliance. Currently, liver transplantation is the only effective therapy. This survey shows that, in a significant number of patients, liver transplantation is performed after some form of brain damage has already occurred. From this, one must conclude that liver transplantation should be performed at a young age, particularly in situations in which reliable administration of phototherapy cannot be guaranteed.
Adolescent↗
Postextrasystolic U wave augmentation, a new marker of increased arrhythmic risk in patients without the long QT syndrome.
OBJECTIVES: We attempted to determine the correlation between the presence of postextrasystolic changes in the STU segment and a history of sustained ventricular arrhythmias. BACKGROUND: Postextrasystolic U wave augmentation (a marked increment in U wave amplitude after premature ventricular complexes [PVCs]) is an adverse prognostic sign in the "pause-dependent long QT syndrome." However, the prevalence of postextrasystolic changes in patients without the long QT syndrome is unknown. METHODS: We compared the configuration of the STU segment of the postextrasystolic beat (the sinus beat after a PVC) with the STU configuration during sinus rhythm in three patient groups: 1) 41 patients with spontaneous ventricular tachycardia/fibrillation (VT/VF) (VT/VF group), 2) 63 patients with heart disease and high grade ventricular arrhythmias (control group), and 3) 29 patients with high grade ventricular arrhythmias but no heart disease (reference group). RESULTS: Postextrasystolic T wave changes did not correlate with a history of ventricular tachyarrhythmias. However, postextrasystolic U wave changes were more common among the patients with VT/VF than among control subjects (39% vs. 8.7%, p < 0.001). By logistic multiple regression analysis, a low left ventricular ejection fraction (p < 0.001) and postextrasystolic U wave changes (p < 0.005) were independent predictors of ventricular tachyarrhythmias. CONCLUSIONS: Postextrasystolic T wave changes are common and lack predictive value. Postextrasystolic U wave changes may be a specific marker of a tendency to the development of spontaneous ventricular arrhythmias. Prospective studies should be performed to confirm this association.
Aged↗
Coming of age of prevention science. Comments on the 1994 National Institute of Mental Health-Institute of Medicine Prevention reports.
The impressive results of prevention research in the last decade should be seen as only an initial demonstration phase of a research agenda that will require sustained support at national, state, and local levels. Future research will need to address problems of long-term follow-up, attention to mediators of program effectiveness, resources to adequately administer programs, and current neglect of state and local prevention infrastructures. Research on factors to facilitate program adoption and dissemination should receive greater emphasis and should appear earlier in the prevention intervention research cycle.
Community Mental Health Services↗
Distribution of marker-Y chromosome containing cells in different tissues of a Turner mosaic patient with mixed gonadal dysgenesis.
We here describe a 12-year-old girl with numerous Turner stigmata and virilized external genitalia. Chromosome analysis of PHA stimulated lymphocytes using different banding techniques revealed a 45,X/46,X,+mar Turner mosaicism with the prominent marker present in about 90% of the blood cells. A PCR-based analysis using a set of 9 STS from different regions of the human Y chromosome indicated the presence of Y chromosomal material with a deletion breakpoint most likely within deletion interval 6. Because of the risk of gonadoblastoma for Turner patients carrying Y chromosomal material, and clinical indications of functional testicular tissue, a gonadectomy in addition to surgical correction of the external genitalia was performed. The histological analysis of the gonads showed a mixture of testicular tissue and ovarian stroma, thus indicating mixed gonadal dysgenesis. Fibroblasts from skin and different parts of the gonads were cytogenetically analyzed and showed a variable distribution of the Y-derived marker between 4% in skin, 11-31% in gonadal tissue and up to 90% in peripheral lymphocytes.
Child↗
[Ovarian tumors in childhood].
Over 16 years 60 girls underwent surgery for ovarian disease. A total of 23 girls suffered from ovarian tumors, as was confirmed by surgery and histological examination in 18 cases. In five girls ultrasound, CT, and MRT examinations showed tumors, which later turned out to be ovarian cysts; in two cases a tumor was found despite the preoperative diagnosis of an ovarian cyst. A total of 15 patients underwent total resection of the ovary, two partial tumor resections and one exploratory laparotomy. Histological findings: eight teratomas, three dysgerminomas, two endodermal sinus tumors, one cystadenoma, one thecalutein cell tumor, one Sertoli cell tumor, one germinal cell tumor, and one angiofibroma. Five tumors were malignant. Surgery and histological examinations are necessary to exclude ovarian tumors.
Adolescent↗
Dissemination of genetic risk information to relatives in the fragile X syndrome: guidelines for genetic counselors.
Fragile X Syndrome, which affects 1 in 1,250 males, is the most common inherited condition causing mental retardation. Although carrier detection for the fragile X syndrome utilizing DNA has now been simplified, genetic counseling and the process of informing at-risk family members remains complex. The purpose of this paper is to offer practical guidelines to health professionals providing genetic counseling to fragile X families in order to facilitate the dissemination of genetic risk information to relatives. This paper was developed from a workshop held at the 4th International Fragile X Conference. The guidelines presented here represent a beginning in the development of an approach to informing relatives in fragile X families about genetic risk, and the identification of mechanisms to reduce the burden to families.
Family↗
Attitudes of attending physicians to the incidental diagnosis of hypercholesterolemia in hospitalized patients.
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Aged↗