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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Medical microbiology and immunology 179 (1990), S. 137-144 
    ISSN: 1432-1831
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fifty-nine orthotopic liver transplant (OLT) patients were studied after transplantation to detect Epstein-Barr virus (EBV) primoinfection and reactivation. Nineteen, all children under 10 years, were EBV seronegative. Seroconversion occurred in 12 (63.3%) of the seronegative patients. Most of these patients (10/12) seroconverted 2 or 3 months after transplantation; 11 out of the 12 demonstrated clinical signs at the time of seroconversion. From 9 primoinfected patients tested for EBV excretion, 8 were found to be positive. Serological evidence of reactivation was found in 9 out 40 (22.5%) seropositive patients and EBV was isolated from 5 (56%). Eleven pediatrie OLT patients with primoinfection showed high and persistent titers of anti-EA antibodies (from 1∶32 to 〉 1∶256), when tested at least 3 months after serovonversion; however, anti-EBNA antibodies failed to develop in 5 patients and remained persistently low in 4. These patients with high EA and with negative or low EBNA titers constitute an “at risk” group for EBV-related lymphoproliferative syndrome (LpS). At presently, after a period of follow-up ranging from 3 months to 3 years, none of our 12 primoinfected patients have developed any lymphoproliferative evolution. However, in 1, during the acute phase, lymphoblasts and lymphoproliferation were observed in a tonsil biopsy.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Gastro-oesophageal reflux ; pH monitoring ; Chronic respiratory disorders
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Thirty-six infants and children presenting with recurrent respiratory disorders (RRD) as the sole clinical symptom including bronchial asthma (6), recurrent obstructive bronchitis with or without wheezing (18), chronic nocturnal cough (3), recurrent episodes of pneumonia (3), recurrent pharyngitis (3) and recurrent laryngitis (3) were investigated for associated gastro-oesophageal reflux (GER) by oesophagram, endoscopy and continuous 24 h pH monitoring of the distal oesophagus. The pH monitoring criteria were selected on the basis of a preliminary study comparing statistically measurements of 32 variables recorded in 15 patients who all had clinical, radiological and endoscopic evidence of GER and in 8 asymptomatic controls. Although patients with symptomatic GER differed significantly from the asymptomatic ones for 27 variables examined, 6 variables emerged as having the highest value for discrimination (overlap score 0–1). Among these, the Euler-Byrne index (number of reflux pH〈4+4 times the number of reflux episodes of more than 5 min), the percentage of total reflux time and the number of reflux episodes 1 h post-cibal scored 0 (no overlap). GER was considered to be present when at least five of these six parameters were abnormal. The overeall incidence of GER in children with RRD was 41% (15) when detected by oesophagram and 61% (22) when diagnosed by pH monitoring criteria. In the children with bronchial asthma or with recurrent laryngitis, the percentage of reflux time during sleep was about 40 times higher than in asymptomatic controls and 2 times higher than in those with symptomatic GER. Of the 22 patients with RRD and GER, 9 were elected to have fundoplication because of poor response to medical antireflux management. All of these, showed complete (6) or partial (3) resolution of respiratory symptoms. Of the 13 patients in whom medical therapy was continued, 9 showed satisfactory improvement with a mean follow-up time of 1 year. Silent GER might be an important contributing factor to the severity of some common chronic respiratory disorders in children.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Gastroeseophageal reflux ; pH monitoring ; Sodium alginate (Gaviscon) ; Clinical trial
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We studied the effects of an alginate compound (Gaviscon) on the frequency and the duration of gastroesophageal reflux (GOR) episodes in children. Twenty infants and children with characteristic symptoms of GOR were divided at random into two groups which were given either Gaviscon (ten patients, mean age: 21 months) or a placebo (ten patients, mean age: 35 months). A continuous pH probe monitoring of the lower oesophageal third was performed in all the patients before and after 8 days of treatment. Before the trial, sensitive pH monitoring variables of acid reflux (Euler-Byrne index, percentage of total reflux time per 24 h, mean duration and percentage of reflux time during sleep, total number of reflux episodes per 24 h and number of reflux episodes per 2 h post-cibal periods) were abnormal in all the patients tested. The oesophagram revealed a GOR in 13 of the 20 patients; none of the children who underwent an endoscopy had evidence of oesophagitis. Episodes of regurgitation reported by the parents decreased during Gaviscon therapy while no clinical improvement was noticed in the placebo group. No adverse effects were observed. After 8 days of treatment with Gaviscon, results of all the pH monitoring variables were significantly (P〈0.05) reduced between -35% and -61% of the initial values recorded. In the placebo treated group, the mean values remained little changes (-9.5 to + 8.2% of initial values). These data suggest that Gaviscon may prove useful in the medical management of GOR in infants and children.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Keywords: Type IV Glycogenosis ; Orthotopic liver transplantation ; Metabolic diseases ; Liver cirrhosis ; Inborn error metabolism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Orthotopic liver transplantation (OLT) has been proposed to treat patients with type IV glycogenosis because of early progressive cirrhosis. Reports have shown absence of disease progression in other organs after OLT and even regression of cardiac amylopectin infiltration in one case. We describe a 15-month-old child in whom a liver transplant was performed for type IV glycogenosis. There were no clinical signs of extrahepatic disease before OLT. Nine months later, the patient developed progressive cardiac insufficiency and died from cardiac failure. Because of massive amylopectin deposits, decreased myofibrils in cardiac cells, and exclusion of other causes of cardiac failure, death was attributed to amylopectinosis. Our observation contrasts with the Pittsburgh experience and suggests that cardiac amylopectionosis may progress after OLT.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1076
    Keywords: Endoscopic sclerotherapy ; Oesophageal varices ; Children ; Ulcer ; Orthotopic liver transplantation ; Ranitidine ; Somatostatine ; Ethoxysclerol
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Signs of portal hypertension, history of upper gastro-intestinal tract bleeding episodes and outcome of the latter were recorded in 76 cirrhotic children evaluated for liver transplantation. Fifty-three (70%) had varices and 22 (29%) had experienced upper gastro-intestinal tract bleeding. Of these 22, 19 bled from varices and 3 from ulcers. Non bleeding ulcers were also found in five patients bleeding from varices. Iterative sclerotherapy controlled acute variceal bleeding in all but one patient in whom emergency transplantation was performed. Six of the eight patients with ulcers were successfully treated by the H2 histamine receptor antagonist ranitidine. We conclude that iterative sclerotherapy is efficient to control acute variceal bleeding and prevents recurrent bleeding in children with end-stage liver diseases awaiting liver replacement. Bleeding asymptomatic ulcers are frequent and respond to H2 histamine receptor antagonists.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 140 (1983), S. 112-115 
    ISSN: 1432-1076
    Keywords: Wandering spleen ; Abdominal pain ; Splenomegaly ; Ultrasonography ; Radionuclide liver-spleen scan
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The article presents two cases of wandering spleen in the pediatric age group. Both patients were admitted for abdominal pain. In one, the onset was sudden and, on physical examination, a mass was felt in the left hypochondrium. In the other, it was the second episode and a firm mid-abdominal mass was found. The wandering spleen is a specific clinical syndrome which should be taken into account when an abdominal tumor is found. Ultrasonography and isotopic scan are very useful diagnostic tools The wandering spleen is caused by the absence of the splenorenal ligament which allows increased mobility of the splenic hilum. Both children underwent a splenectomy and made an uneventful recovery.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1076
    Keywords: Key words Cryptosporidium ; Cholangitis ; Transplant
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Three children of a series of 461 pediatric liver transplant recipients developed diffuse cholangitis associated with intestinal cryptosporidium carriage. All three received immunosuppression consisting of tacrolimus and prednisone. Cryprosporidium carriage was treated with paramomycin, while immunosuppression was decreased according to graft tolerance. No other infectious pathogens were found, and no vascular problems were detected. Bile duct anastomosis was reoperated in all three, but biliary cirrhosis developed in one patient, requiring retransplantation. All three patients are alive and well, and free of intestinal parasites on follow-up. Conclusion Cryptosporidium intestinal infection may play a role in some cases of otherwise unexplained cholangiopathies in pediatric liver transplant recipients. This may lead to significant morbidity, including need for retransplantation.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1437-9813
    Keywords: Key words Liver transplantation  ;  Living donor liver transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Between 1984 and 1996, the authors performed 499 liver transplants in 416 children less than 15 years old. The overall patient survival at 10 years was 76.5%. It was 71.3% for the 209 children grafted in 1984–1990; 78.5% for biliary atresia (n = 286), 87.3% for metabolic diseases (n = 59), and 72.7% for acute liver failure (n = 22). The 5-year survival was 73.6% for the 209 children grafted in 1984–1990 and 85% for the 206 grafted in 1991–1996. Scarcity of size-matched donors led to the development of innovative techniques: 174 children who electively received a reduced liver as a first graft in our center had a 5-year survival of 76% while 168 who received a full-size graft had a survival of 85% (NS). Results of the European Split Liver Registry showed 6-month graft survival similar to results obtained with full-size grafts collected by the European Liver Transplant Registry. Extensive use of these techniques allowed the mortality while waiting to be reduced from 16.5% in 1984–1990 to 10% in 1991–1992. It rose again to 17% in 1993, leading the authors to develop a program of living related liver transplantation (LRLT). The legal and ethical aspects are analyzed. Between July 1993 and October 1997, the authors performed 53 LRLTs with 90% survival. In elective cases, a detailed analysis was made of the 45 children listed for LRLT between July 1993 and March 1997 and the 79 registered on the cadaveric waiting list during the same period. Mortality while waiting was 2% and 14.5% for the LRLT and cadaveric lists, respectively. The retransplantation rate was 4.6% and 16.1% for LRLT and cadaveric transplants, respectively. Overall post-transplant survival was 88% and 82% for children who received a LRLT or a cadaveric graft, respectively. Overall survival from the date of registration was 86% and 70% (P 〈 0.05) for LRLT or cadaveric LT respectively. The 2-year post-transplant survival in children less than 1 year of age at transplantation was 88.8% and 80.3% with a LRLT or cadaveric graft, respectively; patient survival after 3 months post-transplant was 95.8% and 91.9% for stable children waiting at home, 93.7% and 93.7% in children hospitalized for complications of their disease, and 89.5% and 77.7% for children hospitalized in an intensive care unit at the time of transplantation for children who received a LRLT or cadaveric graft, respectively. It is concluded that LRLT seems to be justified for multidisciplinary teams having a large experience with reduced and split liver grafting.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-2323
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Résumé L'analyse rétrospective de 22 cas d'insulinomes pancréatiques colligés par les auteurs dans leur service leur a permis d'apprécier la valeur diagnostique des différentes méthodes d'exploration pré-opératoires et per opératoires. Dans 18 malades, les insulinomes uniques ont été localisés dans 55.5% des cas par l'artériographie sélective, dans 64% des cas par le cathétérisme transhépatique avec prélèvement étagé de sang veineux, et seulement dans 11% des cas par l'ultfasonographie et la tomodensitométrie. La combinaison de l'artériographie sélective et du cathétérisme transhepatique permit de définir le siège de la tumeur dans 83% des cas. La palpation chirurgicale, l'ultrasonographie au cours de l'intervention, le dosage peropératoire du glucose sanguin permirent dans tous les cas de découvrir une tumeur unique. Lorsque les insulinomes sont multiples les diverses méthodes pré-opératoires d'exploration ne sont pas fiables. Dans 4 cas d'insulinomes multiples les différentes explorations preoperatoires permirent de découvrir seulement 8 (28%) tumeurs sur un total de 28 existantes. On ne peut se fier à la palpation pour les decouvrir. Seuls l'échographie peropératoire et le dosage peropératoire du glucose sanguin décèlèrenttoutes les tumeurs multiples (le diamétre de la plus petite tumeur était de 4 mm). Ces 2 méthodes d'exploration opératoire constituent actuellement les procédés de choix pour mettre en évidence les petits insulinomes pancréatiques.
    Abstract: Resumen Se ha hecho la determinación del valor diagnóstico de diversos procedimientos de localización pre- e intraoperatoria mediante el anâlisis retrospectivo de 22 casos de insulinoma pancreáticos operados en nuestro centra médico. En los 18 pacientes, los insulinomas solitarios pudieron ser localizados por arteriografía selectiva (AS) en 55.5% de los casos, por cateterismo transhepático para muestreo venoso esplénico (MVET) en 64% de los casos, pero por ultrasonografía (US) y tomografía computadorizada (TC) apenas en 11% de los casos. La combinación de AS y MVET logró la localización preoperatoria del tumor en 83% de los casos. La palpación intraoperatoria, la ultrasonografía, y la monitoría de la glicemia logró la localización del tumor en la totalidad de los otros casos que poseían un tumor único. Cuando los insulinomas eran multiples, los diversos exámenes preoperatorios demostraron no ser confiables. En los 4 casos con insulinomas múltiples, varios exámenes (AS, US, TC, MVET) lograron localizar solo 8 (28%) tumores entre 28. La palpación intraoperatoria fue igualmente inadecuada. Sólo la US intraoperatoria y la monitoría continuada de la glicemia lograron la localización de latotalidad de los tumores multiples, siendo 4 mm el diámetro del más pequeño de los tumores. Estos 2 tipos de examen intraoperatorio representan actualmente los procedimientos de elección para detectar pequenos insulinomas pancreáticos.
    Notes: Abstract From a retrospective analysis of 22 cases of pancreatic insulinoma operated in our center, we have determined the predictive value of various pre- and intraoperative localization procedures. In 18 patients, solitary insulinomas were localized by selective arteriography (SA) in 55.5% of cases, by transhepatic catheterization with pancreatic venous sampling (THVS) in 64% of cases, but by ultrasonography (US) and computed tomography (CT) in only 11% of cases. The combination of SA and THVS allowed the preoperative localization of the tumor in 83% of cases. Intraoperative palpation, ultrasonography, and blood glucose monitoring localized a single tumor in all cases. When the insulinomas were multiple, the various preoperative investigations were not reliable. In the 4 cases of multiple insulinoma, various investigations (SA, US, CT, THVS) localized only 8 (28%) tumors of 28. Intraoperative palpation was also unreliable. Only intraoperative ultrasonography and continuous blood glucose monitoring localizeall multiple tumors (the diameter of the smallest tumor was 4 mm). These 2 intraoperative investigations are now the procedures of choice for the detection of small pancreatic insulinomas.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1238
    Keywords: Organ transplantation ; Organ procurement in children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To cover the need in paediatric organ transplantation, every potential donor should be considered as a multi-organ donor. Successful transplantation may be performed with kidneys retrieved from very young infants, even anencephalic neonates if the en-bloc technique using both kidneys is used. Regarding the liver, paediatric donors can be accepted from one month of age while livers harvested from older children and even young adults can be transplanted into small children after ex-vivo reduction of the size of the graft. Multi-organ procurement from the same donor provides valuable organs if the anaesthetic management of the donor is appropriate. Active transplant programs needs international cooperation which is made possible by the organ exchange organizations.
    Type of Medium: Electronic Resource
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