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  • 1
    ISSN: 0930-9225
    Keywords: Schlüsselwörter Nakata-Index – hypoplastisches Pulmonalgefäßbett ; Key words Nakata-index – hypoplastic pulmonary artery bed
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Twelve non-consecutive patients aged between 5 and 19 years with univentricular heart and hypoplastic pulmonary artery bed, who early in life underwent systemic-to-pulmonary shunt for augmentation of pulmonary artery diameter, and after normalization of pulmonary artery size a Fontan procedure, were enrolled in the study. The inclusion criteria were normal pulmonary vascular resistance, competent atrio-ventricular valve(s), and globally unimpaired ventricular function. All patients were grouped according to the level of the pulmonary artery flow: restrictive QP:Qs〈1.0 (n=6), balanced QP:Qs=1.0 (n=3), and excessive Qp:Qs〉1.0 (n=3). In each patient, for assessment of the development of the proximal segments of the pulmonary arteries the Nakata-index and McGoon-ratio, and for assessment of the medial and distal segments of the pulmonary artery a selfdeveloped computer assisted planimetric area calculation – index (PPAAI; cm2/m2 b.s.a.) were analyzed in correlation to the level of the pulmonary flow index (Qpi). The cardio-pulmonary situation was assessed by following parameters: Hb, Sasat%, Qp/Qs, PAP, Rp/Rs, EDVP, FS%, and ventricular diastolic compliance (VC=EDVP/Qpi+Qsi). There was a positive correlation between the pulmonary flow index (Qpi) and the Nakata- and McGoon-values suggesting a prognostic relevance of the Qpi in the assessment of the tendency of development of the pulmonary artery diameter. There was no correlation between Qpi and PPAAI, the planimetrically assessed pulmonary vessel areas in patients with restrictive and excessive pulmonary artery flow were comparable. In conclusion, the Nakata-index is due to the shunt related distortion of the proximal segment of the pulmonary artery a non-reliable factor in the assessment of the degree of development of the pulmonary artery tree. The assessment of the medial and distal pulmonary artery segments seems to be more valid and should be considered for optimal timing for definite surgery.
    Notes: Zusammenfassung Ziel der Studie war zu untersuchen, ob der Nakata-Index bei Patienten nach Shunt-Operation als verläßlicher Parameter zur Beurteilung des Entwicklungsgrades des pulmonalarteriellen Gefäßbettes verwendet werden kann oder unterschätzt dieser Index den Entwicklungsgrad des Pulmonalgefäßbettes aufgrund der schunt-bedingten Distorsion des proximalen Pulmonalarteriensegmentes. Untersucht wurden 12 nicht-konsekutive Patienten im Alter zwischen 5 und 19 Jahren mit univentrikulärem Herz und initial hypoplastischem Pulmonalarterienbett, die im frühen Kindesalter einer Shunt-Prozedur unterzogen wurden. Die Pulmonalarteriendurchmesser wurden gemäß der Nakata-Formel gemessen und die entsprechenden Werte mit einem neukonzipierten Index zur planimetrischen Erfassung der Pulmonalgefäßareale (PPAAI; cm2/m2 K.O.) in Relation zum Pulmonalfluß (Qpi) verglichen. Zwischen pulmonalarteriellem Fluß (Qpi) und dem pulmonalarteriellen Durchmesser nach Nakata konnte eine positive Korrelation und damit eine prognostische Relevanz des Qpi in der Beurteilung der Entwicklungstendenz des Pulmonalisdurchmessers nachgewiesen werden. Eine Korrelation zwischen Qpi und PPAAI fand sich nicht, die planimetrisch erfaßten pulmonalarteriellen Areale korrelierten nicht mit den Nakata-Werten und waren bei restriktiven und bei exzessiven Qpi-Werten vergleichbar. Der Nakata-Index ist aufgrund shunt-bedingter Distorsion der proximalen Pulmonalarteriensegmente kein verläßlicher Parameter in der Beurteilung des Entwicklungsgrades des Pulmonalgefäßbettes bei Patienten nach Shunt-Operationen. Die Durchmesser der mittleren und distalen Pulmonalarteriensegmente ermöglichen eine validere Beurteilung und sollten unbedingt mitberücksichtigt werden, um ein optimales Timing für den operativen Eingriff zu garantieren.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Polyhedron 4 (1985), S. 1677-1681 
    ISSN: 0277-5387
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric cardiology 17 (1996), S. 375-381 
    ISSN: 1432-1971
    Keywords: Key words: Down syndrome — Pulmonary artery banding — Pulmonary hypertension — Pulmonary vascular obstructive disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. The hemodynamic response to pulmonary artery banding (PAB) in relation to the preoperative pulmonary/systemic vascular resistance (Rp/Rs) ratio and to the timing of surgery, with special regard to Down syndrome, was investigated in 56 nonconsecutive pediatric patients aged 3 days to 6 months (mean 2.5 months) with simple and complex congenital shunt-related cardiac malformations. Among the non-Down patient group (39 patients; mean age 6.9 weeks) there was a good hemodynamic response in all but three cases, irrespective of the preoperative Rp/Rs ratio; these three poor responders had preoperatively normal or nearly normal Rp/Rs ratios (Rp/Rs 〈 0.3) and were affected postoperatively by lung complications. In the Down patient group (17 patients; mean age 8.2 weeks) the mean preoperative as well as the mean postoperative Rp/Rs ratio was higher than in the non-Down patient group (preoperative Rp/Rs 0.49 versus 0.32; postoperative Rp/Rs 0.31 versus 0.18). There was a good hemodynamic response in all five patients with Down syndrome who had preoperative normal or nearly normal pulmonary vascular resistance ratios (Rp/Rs 〈 0.3). Among 12 patients with Down syndrome and preoperative increased resistance ratios (Rp/Rs 〉 0.3) PAB did not cause a reduction in pulmonary vascular resistance (PVR) in five patients (postoperative Rp/Rs 0.49–1.00), all operated on at more than 6 weeks of age. PAB resulted in effective reduction of postoperative Rp/Rs ratios (range 0.10–0.27) in seven patients, six of them younger and one older than 6 weeks at the time of the banding procedure. In conclusion, patients with Down syndrome and shunt-related cardiac malformations (predominantly total atrioventricular canal cases) in general have higher pre- and postoperative Rp/Rs ratios than non-Down children and also have a higher potential for developing pulmonary vascular obstructive disease despite hemodynamically effective PAB. Especially in children with Down syndrome and pathologically high resistance ratios, PAB, if indicated, should be performed as early as possible.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1569-8041
    Keywords: cytogenetics ; fluorescence in situ hybridization ; mantle-cell lymphoma ; translocation t(11;14)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background: The chromosomal translocation t(11;14)(q13;q32) is thehallmark of mantle cell lymphoma (MCL) in which it can be detectedcytogenetically in about 75% of cases. The t(11;14) translocationjuxtaposes the bcl-1 locus in chromosome band 11q13 next to the IgH locus inchromosome band 14q32 and, thus, leads to deregulation of the cell cycleregulatory protein cyclin D1, which is encoded by the CCND1 gene localizedat the telomeric border of the bcl-1-locus. MCL has the worst prognosis ofall low-grade non-Hodgkin‘s lymphomas (NHL). In some instances, however,histopathologic differentiation between MCL and other low-grade B-cell NHLis difficult. Therefore, detection of the t(11;14) translocation is ofessential diagnostic value for the risk-adjusted management of patients withMCL. Unfortunately, chromosome analyses are frequently hampered by the lowyield and quality of tumor metaphases. As the 11q13 breakpoints arescattered over a region of more than 120 kb the application of moleculargenetic techniques is also limited. Patients and methods: We established an interphase fluorescence in situhybridization (FISH) approach for the detection of the t(11;14)translocation by use of a cosmid probe hybridizing to the IgH constantregion and a YAC spanning the bcl-1 region. Cells containing a t(11;14)translocation show a co-localisation of the signals for IgH and bcl-1. Eightcontrol samples and 15 MCL specimens were investigated. Results: According to our control studies, samples containing more than10% of cells with this signal constellation can be diagnosed ascarrying a clonal t(11;14) translocation. All eleven MCL found to carry thet(11;14) translocation by chromosome analysis were positive in our FISHassay. Additionally, two of four MCL lacking a clonal t(11;14) translocationby chromosome analysis were shown to carry this aberration in 14% and37% of interphase nuclei. Southern blot data indicate that our FISHassay reliably detects the t(11;14) translocation irrespective of thelocation of the breakpoints within the bcl-1 region. Conclusions: The described interphase FISH assay provides a reliable androutinely applicable tool for diagnosis of the t(11;14) translocation.
    Type of Medium: Electronic Resource
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