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  • 1
    ISSN: 1432-1076
    Keywords: Liver transplantation ; Liver function tests ; Prognostic tests
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The pre-operative risk of paediatric liver transplantation candidates (n=41) was assessed in a prospective study by means of clinical symptoms, conventional static and liver blood flow dependent dynamic liver function tests. Nine patients died during the 365-day waiting period. The data were subjected as covariates to a survival analysis in the Cox proportional hazards model. There was a significant relationsship between the results of mono-ethylglycinexylidide (MEGX) formation and ICG test and the 365-day survival rate. In the stepwise analysis, none of the remaining parameters improved the predictive ability when added to the dynamic liver function test results. The assessment of post-transplantation liver function was studied in 27 patients during the first 28 postoperative-day period. In addition, liver function was studied in a cross-sectional study 1–7 years after successful liver transplantation in children with complete or partial rehabilitation. In the early postoperative period severe organ damage was indicated by both static and dynamic liver function tests. In the later course after transplantation no deterioration of liver function measured with MEGX formation was to be observed. These findings demonstrate the usefulness of dynamic liver function tests in the pre- and post-transplant assessment of liver function.
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  • 2
    ISSN: 1432-2277
    Keywords: FK 506, liver transplantation, plasma levels ; Liver transplantation, FK 506, plasma levels ; Plasma levels, FK 506, liver transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract FK 506 plasma levels were analyzed in 89 liver-grafted patients under FK 506-based immunosuppression. Plasma levels were found to be influenced by the patients' liver function: compared to patients without major liver dysfunction, those with cholestasis had higher plasma levels and these plasma levels were able to differentiate between rejection and toxicity. In patients with stable liver function, no clear difference was observed with regard to the plasma levels detectable during toxicity or rejection. We conclude that plasma levels can be used to determine the FK 506 dose but only in patients with cholestasis (i.e, during the early post-transplant course, or in patients with cholestatic rejection). In patients with stable liver function, plasma levels are only of limited clinical relevance.
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  • 3
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Nursing-Bottle-Syndrom ; Saugerflaschensyndrom ; 1. Dentition ; Prävention ; Kinderarzt ; Key words Nursing bottle syndrome ; Baby bottle tooth decay ; Primary dentition ; Preventive care ; Pediatrician
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Lately, toddlers suffering from ”nursing bottle syndrome” (NBS) are seen more frequently in dental and orthodontic practices. In Germany, an increase of NBS is reported since the beginning of the decade, especially in the eastern counties. The present review introduces the circumstances for the development of NBS with respect to the development for both the deciduous and permanent dentition. In most cases, the pediatrician sees the patient prior to the dentist, which explains his responsibility for the toddlers’ dental health during the first years of life. Serious consequences for the child and superfluous expenses for public health services can be avoided by •*appropriate nutrition, especially by weaning the baby from the nursing bottle at the age of 12 months, •*parent motivation to take immediate care of teeth after the first eruption, •*prevention or minimizing the early infection with cariogenic bacteria, and •*referral of the toddler to a dental practitioner at the occurance of carious lesions.
    Notes: Zusammenfassung In letzter Zeit werden Kleinkinder, die an einem Nursing-Bottle-Syndrom (NBS) erkrankt sind, in der zahnärztlichen und kieferorthopädischen Praxis wieder häufiger vorgestellt. Insbesondere aus den neuen Bundesländern wird von einer Zunahme dieser charakteristischen Gebißdestruktion berichtet. In der vorliegenden Literaturübersicht werden die Umstände für das Auftreten des NBS sowie insbesondere die gravierenden Folgen für die Gebißentwicklung dargestellt. Da die Vorstellung des Kindes bei einem Zahnarzt in der Regel in den 1. Lebensjahren unterbleibt, obliegt dem Kinderarzt als einzige medizinische Bezugsperson auch die Gesunderhaltung der Zähne in dieser Lebensphase. Durch •*eine entsprechende Ernährungslenkung, insbesondere dem Absetzen der Saugflasche nach dem 12. Lebensmonat, •*die Motivation der Eltern zur Zahnpflege mit dem Durchbruch des 1. Zahns, •*die Verhinderung bzw. Minimierung der frühzeitigen Übertragung kariogener Bakterien sowie •*die Überweisung an einen Zahnarzt beim Auftreten erster Läsionen können schwerwiegende Folgen für das Kind, aber auch unnötige Kosten für das Gesundheitssystem vermieden werden.
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  • 4
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Blutdruckprofile ; Lebertransplantation ; Kinder ; Key words Blood pressure profiles ; Liver transplantation ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Problem: An arterial hypertension is frequently observed in adults after organ transplantation due to the treatment with the hypertensiogenic drugs Cyclosporin A, FK 506 or prednisolone. Hardly any data exist about the blood pressure profile of children after orthotopic liver transplantation. Method: Therefore we studied ambulatory blood pressure (ABP) in 19 children aged 0.9 to 18.3 years (median value: 10.9 yrs) who had received an orthotopic liver transplant 0.28 to 12.3 yrs before ABP measurement. All patients received prednisolone (1,12–7,3 mg/m2/d); additionally, 13 patients received cyclosporine (91–45 mg/m2/d); the other 6 were given tacrolimus (FK 506) in doses from 0.6 to 4 mg/m2/d for prevention of graft rejection. For ABP recording, automatic measurements were taken every 15 minutes during the daytime (i.e., between 6.00 and 22.00 h), and every half hour in the night (i.e., between 22.00 and 6.00 h). From these measurements, mean systolic and diastolic blood pressures for the day and for the night were calculated separately. These calculated mean values were compared to 95th centiles for normotensive paediatric populations of comparable body length. Results: Eight out of the 19 liver graft recipients had pathologically elevated mean blood pressure values. Arterial hypertension had been known to exist before the ABP measurement in only three of them. Mean systolic and diastolic blood pressure values were elevated in the day and in the night in four children. In one child both mean values for the day plus the mean diastolic value at night were elevated. One child had elevated mean systolic values at day and night. In two children, only one value was above the 95th centile: in one the mean systolic pressure at day, and in the other the mean systolic pressure at night. Four of the six children with 2–4 elevated mean blood pressure values had raised values for serum creatinine and/or an abnormally low glomerular filtration rate. Conclusion: Arterial hypertension after paediatric orthotopic liver transplantation is relatively frequent although less than after renal transplantation. It is associated with a reduced glomerular filtration rate. Thus a cyclosporine-induced renal lesion might be one of the factors causing hypertension after liver transplantation.
    Notes: Zusammenfassung Fragestellung: Eine arterielle Hypertonie wird nach Organtransplantationen bei Erwachsenen als Folge der Behandlung mit den hypertensiogenen Medikamenten Cyclosporin A, Tacrolimus (FK 506) oder Prednisolon häufig beobachtet. Über das Verhalten des Blutdrucks bei Kindern nach Lebertransplantation liegen bislang kaum Untersuchungen vor. Methode: Wir haben deshalb bei 19 lebertransplantierten Kindern im Alter von 0,9–18,3 Jahren (Median: 10,9 Jahre) 24-h-Blutdruckmessungen durchgeführt. Die Lebertransplantation lag bei der Untersuchung 0,28–12,3 Jahre (Median: 3,5 Jahre) zurück. Alle Patienten erhielten Prednisolon (1,12–7,3 mg/m2 und Tag), 13 erhielten zusätzlich Cyclosporin (91–245 mg/m2 und Tag) und 6 Tacrolimus (FK 506, Dosis 0,6–4,0 mg/m2 und Tag) als Immunsuppressiva. Die Blutdruckmessung wurde zwischen 6 und 22 Uhr im 1/4stündigen und zwischen 22 und 6 Uhr im 1/2stündigen Rhythmus durchgeführt. Daraus wurden mittlere systolische und diastolische Blutdruckwerte für den Tag und die Nacht getrennt berechnet. Diese Werte wurden mit körperlängenbezogenen 95. Perzentilen aus pädiatrischen Normalkollektiven verglichen. Ergebnisse: Pathologisch erhöhte Werte zeigten 8 von 19 lebertransplantierten Kindern. Nur bei 3 dieser Kinder war vorher ein Hypertonus bekannt. Die mittleren systolischen und diastolischen Blutdruckwerte waren bei 4 Kindern am Tag und in der Nacht erhöht. Bei 1 Kind waren beide Tagesmittelwerte und der nächtliche mittlere diastolische Blutdruck erhöht, bei 1 weiteren der mittlere systolische Blutdruck am Tag und in der Nacht. Bei 2 Kindern war jeweils ein Wert erhöht: bei 1 der nächtliche mittlere systolische Blutdruck, bei dem anderen der mittlere systolische Blutdruck am Tag. Ein erhöhtes Serumkreatinin und/oder eine erniedrigte glomeruläre Filtrationsrate hatten 4 der 6 Kinder mit 2- bis 4fach erhöhten Blutdruckwerten. Schlußfolgerung: Eine arterielle Hypertonie ist nach Lebertransplantation im Kindesalter ein relativ häufiges Ereignis, jedoch ist sie seltener als nach Nierentransplantation. Sie ist mit einer Einschränkung der glomerulären Filtrationsrate assoziiert. Daher könnte ein Nierenschaden als Folge der erforderlichen Immunsuppression mit Cyclosporin für die Hypertonie mitverantwortlich sein.
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  • 5
    ISSN: 1432-2277
    Keywords: FK 506, liver transplantation ; Conversion, FK 506, cyctosporin, liver transplantation ; Liver transplantation, conversion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Thirty-seven liver-grafted patients with steroid-resistant acute or chronic graft rejection or with cyclosporin-related complications were converted from CyA to FK 506. The clinical outcome of the patients primarily depended on the degree of liver dysfunction present at initiation of FK 506 treatment. In patients switched to FK 506 for treatment of acute or early chronic graft rejection, CyA nephrotoxicity, or CyA malabsorption, the FK 506 therapy was associated with a clear improvement in the clinical course. In contrast, in patients with advanced chronic graft rejection, a lower response rate to the conversion in immunosuppression was observed. The lower response rate was associated with a higher patient mortality. These studies demonstrate that FK 506 represents a valuable alternative immunosuppressant for liver-grafted patients. The conversion from CyA to FK 506 should take place before serious — and potentially irreversible — disturbances in liver function are observed.
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  • 6
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Primäre pulmonale Hypertension ; Leber ; Zirrhose ; Kinder ; Lebertransplantation ; Key words Primary pulmonary hypertension ; Liver ; Cirrhosis ; Children ; Liver transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary A child who suffered from primary sclerosing cholangitis presented with severe portal hypertension over years. At age eleven, symptoms of right ventricular decompensation were noted. After stabilization, orthotopic liver transplantation was performed and resulted in severe decompensation with an increase of right ventricular pressure up to 95 mmHg. At further investigation, primary pulmonary hypertension with plexiform pulmonary arteriopathy was diagnosed. After ten months under oxygen and nifedipine treatment, the patient reached a stable cardiopulmonary state comparable to the pre-transplant period (right ventricular pressure 45 mmHg) but did not improve further. Discussion: Primary pulmonary hypertension in children can occur in association with severe portal hypertension. As this complication will considerably alter prognosis and management of children with chronic liver disorders, it must be actively searched for and monitored with regular ECG, chest X-ray and echocardiography. To prove the diagnosis and evaluate treatment response, cardiac catheterization is required.
    Notes: Zusammenfassung Bei einem an einer primär sklerosierenden Cholangitis erkrankten Mädchen bestand über Jahre ein schwerer portaler Hypertonus. Mit 11 Jahren traten klinische Symptome einer Rechtsherzdekompensation auf. Die orthotope Lebertransplantation, 1 Jahr später durchgeführt, führte zu einer Dekompensation mit Anstieg des rechtsventrikulären Drucks bis 95 mmHg. Die Diagnostik ergab eine primäre pulmonale Hypertonie mit plexiformer pulmonaler Arteriopathie. Unter Sauerstofftherapie und Nifedipin kam es im Verlauf von 10 Monaten zu einer Besserung der rechtsventrikulären Belastung etwa auf den Status vor der Transplantation (rechts-ventrikulärer Druck um 45 mmHg), jedoch nicht zu einer weitergehenden Remission. Diskussion: Eine primäre pulmonale Hypertonie in Assoziation mit einer portalen Hypertension kann auch im Kindesalter auftreten und hat erhebliche Bedeutung für die Prognose und das therapeutische Vorgehen. In der Betreuung chronisch leberkranker Kinder muß bei regelmäßigen Verlaufskontrollen von EKG, Röntgenthorax und Echokardiografie bewußt auf diese oft zunächst asymptomatische Komplikation geachtet werden. Im Verdachtsfall ist eine Herzkatheteruntersuchung zur Diagnosesicherung und zum Austesten des Therapieansprechens erforderlich.
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  • 7
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Epitheloides Hämangioendotheliom ; Leber ; Kindesalter ; Proliferation ; Apoptose ; Key words Epithelioid hemangioendothelioma ; Liver ; Childhood ; Proliferation ; Apoptosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary We report on a 12-year old boy suffering from malignant epithelioid hemangioendothelioma of the liver, which is a very rare tumor in childhood. The tumor was detected by ultrasound examination at the age of 10 and appeared at that time as a solitary intrahepatic nodular lesion. During the following 2 years multiple nodular lesions developed in both hepatic lobes. There were neither any suspect anamnestic findings nor abnormal clinical or laboratory data. The tumor showed the typical histomorphological, immunohistochemical, and ultrastructural features of this entity, which is usually seen in older patients. We investigated proliferative activity, apoptotic regulation, and expression of VEGF and VEGF-receptor flk-1 by means of immunohistochemical techniques. According to the known slow growth activity of these tumors we found only a few Ki-67 positive tumor cells. We did not detect any apoptotic cells using TUNEL technique. The positive immunoreaction of the tumor cells with antibodies against VEGF and VEGF-receptor flk-1 may indicate the regulation of tumor growth by angiogenetic factors. We present our findings together with a summary of the most important publications of recent years concerning these tumors.
    Notes: Zusammenfassung Bei einem 12 Jahre alten Jungen wurde ein im Kindesalter sehr seltenes malignes epitheloides Hämangioendotheliom der Leber diagnostiziert. Im Alter von 10 Jahren fiel erstmals sonografisch ein solitärer Leberrundherd auf, im Verlauf der nächsten zwei Jahre entwickelten sich multiple Rundherde in beiden Leberlappen. Die Anamnese des Patienten war hinsichtlich möglicher prädisponierender Faktoren unauffällig. Die klinischen und laborchemischen Parameter befanden sich im Normbereich. Der Tumor wies die für diese, üblicherweise bei Erwachsenen auftretenden Entität typischen histomorphologischen, immunhistochemischen und ultrastrukturellen Merkmale auf. Mittels immunhistochemischer Untersuchungen wurde das Tumorgewebe hinsichtlich Proliferationsaktivität, Apoptoseregulation und Expression angiogenetischer Faktoren (VEGF und VEGF-Rezeptor flk-1) untersucht. Bei bekanntermaßen langsamer Wachstumstendenz dieser Tumoren fand sich ein geringer Anteil Ki-67-positiver Tumorzellen. Mittels TUNEL-Technik wurden keine Apoptosen gefunden. Die positive Immunreaktion der Tumorzellen mit Antikörpern gegen VEGF und den VEGF-Rezeptor flk-1 deutet auf eine Regulation des Tumorwachstums durch angiogenetische Faktoren hin. Die Ergebnisse werden in Verbindung mit einer Zusammenstellung der wichtigsten Publikationen der letzten Jahre über diese seltenen Tumoren diskutiert.
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  • 8
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Among the worldwide accepted indications for liver transplantation, inherited metabolic disorders play an increasing role. In some paediatric centres this indication runs second after extrahepatic biliary atresia. The aim of liver transplantation in inherited metabolic disorders is twofold: the first is to save a patient's life, the second is to accomplish phenotypic and functional cure of his disease. These aims may be achieved in disorders presenting with cirrhosis, hepatoma, life-threatening progression or failure of other organs with preserved liver function. The timing of liver transplantation has become easier with development of surgical techniques of reduced-size donor livers. These techniques enable the performance of liver transplantation with ABO blood group compatible organs of almost any size if indicated either by deterioration of liver function or impending complications such as hepatoma or life-threatening progression. In comparison with other indications such as extrahepatic biliary atresia, postnecrotic liver cirrhosis or acute liver failure, the results of transplantation in patients with inherited metabolic disorders seem to be better, reaching up to 78–95% actuarial 1-year survival rates. However, lifelong immunosuppressive therapy is necessary. This seems to be acceptable even in disorders with only partial liver function defects.
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