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Biomedical subjects

J B Otte

Publications and source records attributed to J B Otte.

At least 91 records · Page 5Linked to original sources

[Liver transplantation in familial amyloidosis].

A 32-year-old Portuguese with hereditary amyloidosis had been suffering from polyneuropathy for 9 years. It began insidiously with polyneuropathic complaints in the legs which gradually got worse over the years and progressively impaired walking. He also had signs of autonomic neuropathy with severe orthostatic dysregulation, abnormal micturition and impotence. His general state had deteriorated during the last 3 years with a weight loss of 18 kg, due to treatment-resistant diarrhoea. As there is so far no known cure of the amyloidosis, which usually ends fatally from cachexia after an average of 10 years, liver transplantation was performed to reduce amyloid production and thus favourably influence the course of the disease. The patient's general condition has remained stable 32 months after the transplantation.

Adult↗

Standardized quick en bloc technique for procurement of cadaveric liver grafts for pediatric liver transplantation.

This paper describes a quick procedure for cadaveric liver graft retrieval during multiple organ harvesting. The technique is based on minimal preliminary dissection, absence of in situ direct portal perfusion, and en bloc removal of the liver and pancreas, with an aortic patch encompassing the coeliac trunk and superior mesenteric artery. The results of 110 pediatric liver transplantations with 109 organs harvested using this technique are reported. There were no graft harvesting injuries. The liver graft primary nonfunction rate was 4.5% (5/110). The 3-month retransplantation rate was 10%. The actual patient survival rates were 93% at 3 months and 90% at 1 year; actual graft survival rates were 85.5% and 78%, respectively. The technique described was at least as safe as conventional procedures. A major advantage of the procedure is its flexibility, which allows for the easily combined procurement of other organs (whole pancreas and intestine).

Adolescent↗

Anaesthetic considerations in progressive familial intrahepatic cholestasis (Byler's disease).

Progressive familial intrahepatic cholestasis (PFIC) or Byler's disease is one of the most common forms of intrahepatic cholestasis of metabolic and genetic origin. Affected children progress to terminal cirrhosis before adulthood and at present the only curative treatment of PFIC is orthotopic liver transplantation (OLT). We present a retrospective review of 40 general anaesthetics administered in our hospital to 22 patients with PFIC undergoing various procedures. The clinical features of PFIC and the anaesthetic implications of chronic cholestasis in children (malnutrition, cirrhosis, portal hypertension, chronic hypoxaemia) are reviewed.

Adolescent↗

Esophageal replacement: gastric tube or whole stomach?

BACKGROUND: The stomach can be used either in its entirely or as a greater curvature tube for esophageal replacement. METHODS: The study compares the gastric tube (group A; n = 112) to the whole stomach whose lesser curvature is denuded (group B; n = 100) in terms of technical complication and alimentary comfort. The clinical results are substantiated by assessment of the eating performance of patients and control subjects at a test meal, measurement of the gastric dimensions before and after both tailoring procedures, and intraarterial staining of the gastric wall. RESULTS: Major differences between the two groups are cervical anastomosis stenoses (22.3% versus 6% [A versus B]; p = 0.008), fistulas (7.9% versus 1%; p = 0.0209), number of meals and snacks per day (4.6 versus 4; p = 0.0275), sensation of early fullness at meals (52.4% versus 17.8%; p < 0.0001), ratings given to the long-term alimentary comfort (presymptomatic condition = 10 points) (7.6 versus 8.8 out of 10 on average; p < 0.0001), and calories consumed in 1 minute at a test meal (59% [p < 0.05] versus 77% of those consumed by control subjects). The volume of the stomach is reduced by a range of 21.4% to 47.2% after tubulization (group A) whereas it increases by a range of 4.9% to 17.4% after denudation of the lesser curve (group B). Intraarterial staining of the gastric wall reveals the poor vascularity of the upper-most segment of the greater curve. CONCLUSION: Slight increase of the gastric capacity and maintenance of the submucosal vascular network account for the better results achieved with the whole stomach.

Adolescent↗

Utilization of laboratory resources: developments in knowledge-based ordering systems.

This paper describes two rule based decision support systems. The first system is used to screen incoming test requests for adequacy on the basis of signs and symptoms volunteered by the requesting GPs. The system was tested using a database of 794 requests for a TSH test. About 17% of the test requests were correctly identified as unnecessary. In total, 0.5% of the tests were incorrectly labelled as unnecessary. This concerned 4% of the patients that appeared to have hyperthyroidism and 23% of the patients that appeared to have hypothyroidism on the basis of TSH and FT4 results. The other system is a rule-based clinical decision support system for the requesting of laboratory investigations, originally designed for use at a hospital within the UK, that was implemented in a predominantly French-speaking hospital in Belgium. This involved the modification of the system to allow multilingual operation, and also the implementation of a completely new set of investigation protocols. The purpose of this study was to assess the transferability, both of the system itself, and of its benefits. The system was introduced gradually and has only recently been in full operation. However, the findings from the first months of routine use of the system indicate that the transfer of the system to a different clinical environment has been successful. Although it is too early to assess fully the impact on laboratory utilization, the clinicians believe that it is improving the appropriateness of investigations.

Artificial Intelligence↗

Liver transplantation and haemophilia A.

Liver transplantation has become the standard treatment for a variety of inherited metabolic disorders. We report on two patients who underwent successful transplantation for posthepatitis viral cirrhosis, which developed following blood factor replacement for haemophilia A. The second patient was transplanted before the occurrence of major complications of either his liver or haemophilic disease. We propose early liver transplantation to achieve metabolic cure of haemophilia.

Adult↗

Role of hepatitis C virus in chronic liver disease occurring after orthotopic liver transplantation.

Paediatric orthotopic liver transplant recipients may develop chronic hepatitis after surgery. To investigate the role of hepatitis C virus in this pathology a cohort of 249 paediatric orthotopic liver transplant recipients was studied. Sixteen children (6.4%) were found to have chronic hepatitis C virus hepatitis after orthotopic liver transplantation. All but one of them had serum transaminase values which were persistently raised two to eight times the upper limit of normal. Thirteen were positive for both serology and serum hepatitis C virus RNA. Serum hepatitis C virus RNA detection occurred five to 33 months before hepatitis C virus antibodies. Liver tissue hepatitis C virus RNA and hepatitis C virus core antigen were detected in five. In one patient, tissue hepatitis C virus core antigen was detected when other tests for hepatitis C were negative. Two patients had positive human cytomegalovirus serum antibodies and RNA before transplantation. Although serum hepatitis C virus RNA was not detected after transplantation, serum enzyme immunosorbent assay and tissue core antigen were still detectable in both patients. In another child, serum hepatitis C virus RNA was positive and hepatitis C virus core antigen was found on a liver biopsy specimen but antihepatitis C virus antibodies were negative as well as liver hepatitis C virus RNA. No patient developed severe liver disease or cirrhosis during a follow up of up to 72 months. It is concluded that hepatitis C virus is a significant cause of morbidity after paediatric orthotopic liver transplantation. Diagnosis cannot rely on serological testing only. The patients remained stable on follow up, but longer prospective histological studies remain necessary to establish prognosis.

Antigens, Viral↗

Outcome following organ removal from poisoned donors in brain death status: a report of 12 cases and review of the literature.

Experience with organ procurement from poisoned donors in brain death status is still limited in comparison with other etiologies. From 1963 to 1993, 2769 grafts (heart 141, kidney 1922, liver 623, pancreas 43) were performed in our University Hospital. Since 1975, among 1174 patients admitted to the ICU for acute poisoning, 12 patients who developed brain death status were considered for organ donation. The toxics involved were: methaqualone (1), benzodiazepines (1), benzodiazepines plus tricyclic antidepressants (2), barbiturates (2), insulin (2), carbon monoxide (1), cyanide (1), methanol (1), and acetaminophen (1). Exclusion criteria for organ removal were applied according to the nature of the toxin and the general criteria used for organ donation. The organs removed were: heart 5, heart valves for graft bank 2, kidneys 22, liver 4, pancreas 2, pancrease islet cells 2. Pertinent follow-up was obtained in 23 of 32 recipients. Immediate outcome was favorable in 20/23 patients (85%). Three patients died either from stroke, heart failure or preexisting encephalopathy. Two patients died from either chronic hepatic or renal graft rejection. None of these events could be directly related to a toxic origin. The one year survival rate of 75% is similar to that observed in the population who received organs from nonpoisoned donors. Organ procurement can be considered in few selected cases of acute poisoning. The accuracy of the diagnosis of irreversible brain damage is essential in this setting.

Brain Death↗

HPLC-microparticle enzyme immunoassay specific for tacrolimus in whole blood of hepatic and renal transplant patients.

Tacrolimus is a relatively new immunosuppressant used in organ transplantation to prevent graft rejection. However, its use is not devoid of side effects, making it important to maintain blood concentrations within therapeutic ranges. Several analytical methods are currently available for routine drug monitoring. However, these methods are based on use of the same monoclonal antibody, which also cross-reacts with some metabolites, resulting in overestimation of some blood concentrations. Even though this antibody appears appropriate for therapeutic drug monitoring, no reference method measures only the parent drug, mainly because of the poor absorptivity of tacrolimus in ultraviolet light. We have developed a method displaying an increased specificity towards the unchanged drug, using conventional equipment available in most clinical laboratories. After chromatographic separation of the blood extract, the tacrolimus fraction is analyzed by an automated microparticle enzyme immunoassay (MEIA) performed on the IMx analyzer (Abbott Labs.). This method is linear from 0 to 40 micrograms/L, yields CVs from 8.5% to 18.2%, and has a detection limit of 5 micrograms/L. Tacrolimus concentrations obtained by HPLC-MEIA in hepatic and renal transplant patients are from 47.5% to 18.8% lower than those obtained by MEIA, according to liver function tests and metabolite accumulation, even though no significant differences were observed between the methods for drug-free blood samples supplemented with known amounts of tacrolimus.

Antibodies, Monoclonal↗

Mitochondrial respiratory chain defect: a new etiology for neonatal cholestasis and early liver insufficiency.

Two siblings presented with neonatal cholestasis and early liver insufficiency. The older was admitted for end-stage cirrhosis with severe hypoglycemia and had long-term successful liver transplant at the age of 15 months. The second child presented a similar neonatal history of cholestasis, hypoglycemia, hyperlactacidemia, liver insufficiency and progressive cirrhosis. Extensive work-up excluded all known causes of neonatal cholestasis. Gluconeogenesis was found normal following alanine and fructose infusion. Repeated hypoglycemia with early post-prandial hyperlactacidemia led us to investigate the mitochondrial respiratory chain enzyme activities. Selective defects of complexes I, III and IV, coded by mitochondrial DNA, were detected in liver tissue of this patient and on preserved frozen tissue from his sibling, whilst normal activities were found in liver tissue samples from control patients with end-stage liver diseases. No extrahepatic manifestations were found. We conclude that liver deficiency of mitochondrial respiratory chain enzymes may cause liver disease in neonates, associated with hypoglycemia and post-prandial hyperlactacidemia. The disease is cured by liver transplantation.

Acidosis, Lactic↗

Liver procurement without in situ portal perfusion. A safe procedure for more flexible multiple organ harvesting.

The outcome after liver transplantation when grafts were retrieved from the donor by the classical aortic and portal cooling (APC) method was compared with the outcome when exclusively aortic in situ perfusion (AC) was used. Retrospectively, 163 donor hepatectomies performed over a 20-month period were reviewed to analyze overall graft (APC n = 78, AC n = 85) and patient outcome. The global graft and patient survival rates were not significantly lower in the APC group, except for 3-month graft survival (APC 72%, AC 87%; P = 0.015). However, this could be unrelated to the technique. In a subgroup of 140 cases (APC n = 64, AC n = 76), a more detailed analysis was performed. Populations of donors and recipients were similar. The graft injury and the immediate graft function were not significantly different between both groups. A multivariate analysis shows that low donor weight (P = 0.007), donor hypernatremia (P = 0.014), and in situ portal perfusion (P = 0.045) were significant determinants of a higher postoperative peak of glutamic pyruvic transaminase. In summary, in this series, routine human liver procurement using exclusive aortic perfusion seemed to be at least as safe as using a combined aortic and portal perfusion technique. This simplified method may also represent some advantages for combined pancreas and intestinal harvesting in the future.

Adenosine↗

Technical problems in shipped hepatic allografts: the UCL experience.

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.

Belgium↗

Percutaneous transjugular intrahepatic stent shunt for treatment of intractable varicose bleeding in paediatric patients.

UNLABELLED: 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.

Child↗

Sequential treatment of biliary atresia with Kasai portoenterostomy and liver transplantation: a review.

Biliary atresia is the most frequent cause of chronic cholestasis in infants. When left untreated, this condition leads to death from liver insufficiency within the first 2 yr of life. The modern therapeutic approach consists of a sequential strategy with Kasai portoenterostomy as a first step and, in case of failure, liver transplantation. After portoenterostomy, no more than 20% to 30% of patients will live jaundice-free into adulthood. Illness in another third will be palliated, and these patients have extended survival, delaying liver transplantation to later childhood (2 to 15 yr). The remaining 30% to 40% will not benefit from the Kasai operation and will die of liver failure in infancy. The annual need of liver transplantation for biliary atresia is one case per million people. This indication represents 35% to 67% of the reported series of pediatric liver transplantation and between 5% and 10% of the indications for liver transplantation, all ages included. Approximately four of five children transplanted for biliary atresia will become long-term survivors with good physical and mental development; recurrence of the disease after transplantation has not been observed. Because most candidates are young children (< 3 yr) of small size (< 10 kg), there is a shortage of size-matched donors (which has been alleviated by the use of innovative techniques such as reduced and split livers). The resulting redistribution of the adult donor liver pool is ethically justified by the equal quality of the results after transplantation of a full-size or partial graft.

Adolescent↗