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  • 1
    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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  • 2
    ISSN: 1432-1076
    Keywords: Liver cirrhosis ; Varicose bleeding ; Transjugular intrahepatic portosystemic stent shunt ; Children ; Orthotopic liver transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two 10-year and 11-year-old children with oesophageal and gastric varicose haemorrhage unresponsive to medical treatment and repeated endoscopic sclerotherapy underwent percutaneous transjugular intrahepatic portosystemic shunting (TIPSS). A newly developed introducing system was used. The procedure was performed to avoid the increased risk of emergency liver transplantation in children with hepatic failure. Immediately after the procedure bleeding stopped and the patient's condition improved. Ascites disappeared and liver function improved. The stent shunt was shown to be patent by angiography and Doppler ultrasound for a follow up period of more than 1 year. Conclusion TIPSS may be of benefit in children with severe portal hypertension. It allows control of intractable bleeding, and stabilizes the patients preparing them for subsequent elective orthotopic liver transplantation.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1238
    Keywords: Abdominal trauma ; Children ; Ultrasound ; CT scan ; Laparotomy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Objective To evaluate the strategy of a combined diagnostic and therapeutic approach in children with intra-abdominal organ injury following blunt abdominal trauma. Design Retrospective clinical study. Setting Pediatric intensive care unit of an university hospital. Patients 38 children with documented intra-abdominal injury. Intervention Initial non-surgical treatment by a team of pediatric intensivists, radiologists and surgeons. Measurements and results Physical examination, oriented blood and urine tests, plain abdominal film, abdominal ultrasound (US) and computed tomography (CT) with contrast. US documented intra-abdominal fluid in 30 and initial organ lesion in 14 out of 31 patients evaluated. Abdominal CT demonstrated the precise organ lesion in 34 out of 36 patients examined with solid organ lesion. Early laparotomy was needed in 7 because of severe shock, pneumoperitoneum and ruptured diaphragm, and delayed surgery in 6 patients. All 38 patients regained a normal life. Conclusions The stepped diagnostic approach combined with initial non-surgical treatment by a team provided accurate diagnosis and appropriate treatment. Abdominal US, by demonstrating free intra-abdominal fluid is very sensitive to detect patients with intra-abdominal organ injury, CT scan with contrast is needed to give precise information of specific organ lesions.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Transplant international 7 (1994), S. 297-301 
    ISSN: 1432-2277
    Keywords: Liver transplantation, shipped ; Shipped livers, complications
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Due to developments in surgical techniques and organ preservation, the shipping of renal and extrarenal organs is becoming increasingly more frequent. During the period from 1 January 1991 to 31 December 1992, 39 of 180 (21%) implanted liver allografts were shipped to our center by local harvesting teams. The fact that each of nine livers (23.1%) presented with minor and major (vascular and parenchymatous) problems stresses the need for better surgical training and standardization in procurement techniques. The introduction of a liver allograft feedback report could be an easy way to perform quality control.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-2277
    Keywords: Key words Pediatric liver transplantation ; abdominal wall ; prosthesis ; Abdominal wall ; pediatric liver transplantation ; prosthesis ; Prosthesis ; pediatric liver transplantation ; abdominal wall
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Due to the shortage of size-matched liver donors, relatively oversized liver grafts (even after ex situ volume reduction) are frequently used for liver transplantation in children. This was recently observed when livers from large, living related donors were procured for transplantation in very small recipients. Given that abdominal hyperpressure can compromise vascular flow in the new graft, primary closure of the abdomen was delayed by temporary Silastic prosthetic closure in selected cases. The new technique was original in that the skin was closed, avoiding fluid loss and reducing the risk of infections reported with other techniques, and in that reoperation allowed for a delayed, but primary-type, closure (fascia and skin) that resulted in an esthetically correct aspect. Over a period of 7 years, 330 pediatric liver transplantations were performed, and delayed prosthetic closure was achieved successfully and safely in 47 cases. The present report outlines this clinical experience.
    Type of Medium: Electronic Resource
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