[Transfusion complications. A comparison between Fenwal bags and glass flasks].
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Biomedical subjects
Publications and source records attributed to C H Gips.
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From March, 1979 to March, 1983, 26 orthotopic liver transplantations and 1 retransplantation were performed in our center. Sixteen patients are alive, 5 beyond 2 years and 1 longer than 4 years after transplantation. The actuarial 1- and 2-year survival is 60%. Factors contributing to this result are patient selection and biliary anastomosis. In cirrhotic patients, the degree of deterioration of liver function correlated with survival; in patients with hepatocellular carcinoma, extrahepatic spread of tumor was the most frequent limiting factor. Choledochocholedochostomy with a widening plasty had a low complication rate providing that the arterial blood supply was undisturbed. Until now, HLA-matching has not contributed to the result of liver transplantation, although the effect for long-term survival is still unknown. The high survival rate and excellent quality of life of long-term survivors justify liver transplantation as treatment for otherwise noncurable liver diseases.
Trained specialists in hepatology, liver and biliary surgery, anesthesiology and pathology must form the core of a team for liver transplantation. The hospital must be a center for organ transplantation to provide the essential infrastructure to manage specific aspects of transplantation. A well-organized blood bank is essential to cope with acute requests for large amounts of donor blood. Because of the extremely high costs, liver transplantation has to be approved medically and politically before it can be considered as a generally accepted modality of treatment.
Antimitochondrial antibodies are markers for primary biliary cirrhosis and probably reflect a specific defect in immunoregulation underlying this disease. Antimitochondrial antibodies and their primary biliary cirrhosis-specific subtypes were tested before and up to 6 years after orthotopic liver transplantation. Sera from 31 consecutive patients were tested, 15 patients had primary biliary cirrhosis and 16 non-primary biliary cirrhosis. Antimitochondrial antibodies were investigated under code by immunofluorescence, and primary biliary cirrhosis-specific subtypes were determined by radioimmunoassay (anti-p62, anti-p48) and complement fixation test (anti-M2, anti-M4, anti-M8). Before orthotopic liver transplantation, antimitochondrial antibodies were detected by immunofluorescence in 13 of 15 patients with primary biliary cirrhosis. Of these patients, 12 were positive for anti-p62 and 8 for anti-p48. Ten patients were positive for anti-M2, 4 patients for anti-M4 and 7 patients for anti-M8. Two primary biliary cirrhosis patients and all non-primary biliary cirrhosis patients were negative with all tests. One month after orthotopic liver transplantation, antimitochondrial antibodies titers declined or became negative by antimitochondrial antibodies immunofluorescence, 3 patients became negative by radioimmunoassay for anti-p62 and 1 for anti-p48. With complement fixation test, 4 patients became negative for anti-M2, 2 for anti-M4 and 4 for anti-M8. Antimitochondrial antibody titer reduction observed 1 month after orthotopic liver transplantation remained unchanged in most sera during the following years. A rise was observed in two patients after 4 and 5 years.(ABSTRACT TRUNCATED AT 250 WORDS)
We have determined ammonia in cerebrospinal fluid (CSF) with the indophenol direct method. The results were compared with an enzymatic method. The method is very simple, and precision (coefficient of variation 1.6%) and linearity (r = 0.9999, p < 0.001) of the method are excellent. The recoveries of the method are very good (within-sample recovery: range 88-93, median 93%; between-sample recovery: 88-93, median 91%). In a population of 23 neurological patients not suffering from liver disease, the reference values ranged from 8 to 26, median 18 microM. Males and females did not differ (p = 0.5). The values obtained with the indophenol method were equal to the enzymatic method (range 9-28, median 18 microM, p = 0.6). On storage in the deep freeze (-20 degrees C), there was no change in CSF ammonia concentration for at least 1 mo. When stored at 4 degrees C (refrigerator), ammonia determinations have to be performed within 2 d. CSF storage at room temperature results in artificially elevated ammonia levels and should be avoided.
BACKGROUND/AIMS: One of the prognostic methods for survival in primary biliary cirrhosis (PBC) is the Mayo model, with a time-scale limited to 7 years. The aim of our study was to assess how major clinical events, signs, several severity assessment methods and Mayo survival probabilities fit in with actual patient survival, by using yearly observations until 0.5 years before patient death from PBC. METHODOLOGY: Data of 32 patients dying from PBC were collected prior to death at -0.5, -1, -2 etc. years (median: -5 years, range: -16 to -0.5 years). Major events registered were: first occurrence of ascites, upper gastrointestinal bleeding or manifest hepatic encephalopathy and signs, first observation of spider naevi or purpura. Severity assessment methods applied (all with scores and classes) were: Mayo (M), Child-Campbell (C), Pugh-Child (P), Pugh-Child-PBC (PP), 'Child-Pugh' (CP), and Ascites Nutritional State-Child (ANS). Fifty percent survival estimates were calculated from Mayo scores. Severity assessment method variables were: ascites (C, P, PP, CP, ANS), encephalopathy (C, P, PP, CP), nutritional state (C, ANS), edema (M), age (M), serum albumin (M, C, P, PP, CP), bilirubin (C, M, P, PP, CP), and prothrombin time (M, P, PP, CP). RESULTS: In 27 out of 32 patients a major event occurred, always between -6 and -0.5 years (median: -1 year) and, never between -16 and -7 years (p < 0.0001). A sign was first observed in 30/32 between -14 and -0.5 years (median: -2 years). Compared to the total population, a sign, and even more so, an event indicated a shorter survival (p = 0.004 and p = 0.0002, respectively). The median 50% estimated survival (predicted by the Mayo model) fitted the actual survival from -6 to -0.5 years (r = -0.7, p < 0.0001), but not from -16 to -7 years (r = -0.1, p = 0.4). All -6 to -0.5-year severity scores correlated (p < 0.0001) both with actual survival (M, C, P, PP, and CP r = 0.7; ANS r = 0.5) and with estimated M 50% survival (C, P, PP, CP r = -0.9; ANS r = -0.6; M score: -0.99), but none with actual survival from -16 to -7 years, except for M, slightly (r = -0.3, p = 0.04). A nomogram for mean C, CP, M and ANS scores related to actual survival was constructed for the -6 to -0.5-year period. The C and CP classes A, B, and C did not appear to distinguish sufficiently into actual survival, whereas the M classes did. CONCLUSIONS: The occurrence of a major event appeared to exclude survival over 6 years. In these final 6 years, Child-Campbell, Mayo and Pugh scores correlated equally well with actual survival and better than Ascites/Nutritional State score. In our PBC patients, Campbell was an excellent alternative for Pugh; for Pugh, the original Child-Turcotte variable limits were fully sufficient.
BACKGROUND/AIMS: In 1979, separate liver transplantation (LT) and primary biliary cirrhosis (PBC) death rate registration became available in The Netherlands (15 million inhabitants). The objective of this study was to investigate death rates from 1979-1992 and analyse the impact of LT. PATIENTS AND METHODS: PBC was either a primary or secondary cause of death. Rates were expressed as absolute numbers or per million inhabitants in the corresponding age category. Age classes of 5 years were used. The Netherlands was divided in four regions, North, South, East and West. Standardized mortality ratios (SMR) were used for calculation of regional differences. RESULTS: In the 14 year period between 1979-1992, 417 persons died from and 179 persons died with PBC, totaling 596 PBC patients (6.3 per million inhabitants > or = 35 years). No person younger than 35 died. Eighty-two percent were female, with a corresponding female/male ratio of 4.2 per million females/males inhabitants. In region South there were significantly fewer deaths (SMR 66%, p < 0.001) and in region North significantly more (SMR 141%, p < 0.05). The median age class at death was 70-74 (males and females alike). At age 35-59, death from PBC in 1992 per million was 1.2, and for > or = 65 years 15.7. In age class 80-84, the highest death rate from or with PBC was found with 28 deaths per million inhabitants and with a female/male ratio of 3.6. In 1992, with two deaths only, LT appeared to have nearly eliminated death from PBC in the age category 35-59 years. CONCLUSION: Death from PBC mainly occurs in the old and very old, who may never seek a specialized center. This indicates a more specific management and therapy for this particular group is needed.
BACKGROUND: From a primary clinical database, we wanted to obtain insight in disease distribution and clinical presentation of adult jaundiced patients in a Western country. MATERIALS AND METHODS: As part of the Euricterus project, 24 Dutch general and academic hospitals in a period of 2 years gathered prospectively 702 patients on a standard proforma. Patient aged 16 years or more (median 61) and with a serum bilirubin of 20 mmol/l or more (median 83) were included. The final diagnosis was established within 3 months. RESULTS: Pancreatic or biliary carcinoma (20%), gallstone disease (13%) and alcoholic liver cirrhosis (10%) were the 3 most frequent diagnoses. Imaging (79%), clinical course (63%) and chemistry/serology (57%) were the most used ascertaining methods. Pancreatic or biliary carcinoma and gallstone disease were more common and age higher in general hospitals (p = 0.0001), and 'immunological' liver disease, non-alcoholic cirrhosis and hepatocellular carcinoma (HCC) more common in academic hospitals (p = 0.001). Patients aged 90 years or older (13%) had pancreatic or biliary carcinoma, liver metastases or heart failure and patients with age less than 20 (0.9%) had acute viral hepatitis, nonalcoholic active liver disease or HCC. Risk factors were more apparent (p < 0.02) in those aged less than 61 years. Feeling unwell (78%), dark urine (67%) and anorexia (57%) were the 3 most frequent symptoms; the 3 most frequent signs were liver enlarged (39%), looking ill (29%) and appearing wasted (23%). CONCLUSIONS: Through Euricterus, fresh clinical knowledge has emerged of symptomatology, age stratification and hospital preponderance of (sub)clinical jaundice in this country. This is important both for teaching and in preparing clinical studies.
BACKGROUND/AIMS: A retrospective study of primary biliary cirrhosis (PBC) was performed to study the Original Mayo Model for predicting survival by a Dutch data-set of patients, presentation of disease progression; assessment of liver transplantation, prediction of post-transplantation survival; and the addition of two laboratory variables to the Original Mayo Model. MATERIALS AND METHODS: Survival of 83 patients, 37 of whom underwent transplantation, were studied. Mean follow-up was 6.0 +/- 0.45 SEM years. Risk score at diagnosis, platelet count, and serum sodium were analyzed in a Cox model. RESULTS: The Original Mayo Model estimated survival for low-, medium-, and high-risk groups accurately and it also presented disease progression. Baseline Mayo risk score in a Cox model had a regression coefficient of 1.01, indicating an excellent predictor p < 0.0001. Platelet count was a predictor of survival (p < 0.002), whereas serum sodium did not (p = 0.67). A new model combined of the Original Mayo risk score and platelet count predicted survival in high-risk patients somewhat better compared to the Original Mayo Model. With both models, liver transplantation had a significant beneficial effect on survival (p < 0.001). The scores revealed no significant influence (p = 0.47) for overall post-transplantation survival. CONCLUSIONS: The Original Mayo Model remains the model of choice for patients with PBC for prognostication from 3-8 years, is a useful tool in the assessment of liver transplantation but not an indicator of post-transplantation survival. Platelet count showed to have additional prognostic value. A new model combined of platelet count and the Original Mayo risk score did predict survival in high-risk groups slightly better compared to the Original Mayo Model.
BACKGROUND/AIMS: Estimation of prognosis becomes increasingly important in primary biliary cirrhosis (PBC) with advancing disease and also with regard to patient management. The ubiquitous used Pugh scoring for severity of disease is simple while the Mayo model which has been validated for survival estimates is more sophisticated. We wanted to investigate whether Pugh and Mayo scores correlate (they have 3 of 5 variables in common) and if so whether a survival probability based on Mayo data could be affixed on Pugh classes and scores obtained in the same patients. METHODOLOGY: All variables used for Mayo Clinic Prognostic Model (Mayo) scoring and Pugh-Child-PBC (Pugh) scoring were available in 143 PBC patients of the Pan European database Euricterus. Pugh scores P5-P15 and has classes A (P5-6), B (P7-9) and C (P10-15). We subdivided P5 in P5A (patients with albumin > 40 g/l plus prothrombin time < or = 12 secs) and P5B (the other patients in P5). We designed a category Pugh Early (PE) for patients with P5A characteristics and bilirubin < 17 mmol/l. Mayo scores R0-R15-with 1-7 years survival probabilities S-and has risk classes Low (L), Intermediate (Int), High (H) and Very High (VH). RESULTS: The estimated survival probabilities of the 143 patients ranged from 88% at 7 years to 0% at 1 year, median 14% at 5 years. The Pugh and Mayo scores correlated r = 0.87 (p < 0.0001) and except age with P, all Mayo and Pugh variables correlated with both R and P at p < 0.0001. Survival in Pugh class A was median 43% at 7 years and was not different from survival in Mayo L+Int (p 0.58). In Pugh class B 7 years survival was 2%, not different from Mayo H (p 0.25). Survival in Pugh C was median 24% at 1 years and better than Mayo VH (p 0.02). Between P5A (survival 78% at 7 yr) and R 3-4; P5B-6 (40% at 7 yr) and R5; P7 (22% at 7 yr) and R6; P8-11 (12% at 5 yr) and R7-8; and P12-14 (5% at 1 yr) and R9-10 no significant differences were found. From P8 upward there was a steep increase in death rate. PE has a 7 year survival of at least 89%. Charts of projected survival estimates for Pugh scores and classes are presented. CONCLUSION: It was possible (affixing Mayo to Pugh) to define 1-7 years survival probabilities to Pugh classes and scores for the last 7 years of the disease, i.e. the most important period for therapeutic decisions. These results need to be validated in other PBC populations.
BACKGROUND/AIMS: Primary Biliary Cirrhosis (PBC) is a relatively rare chronic progressive disease in which a working diagnosis of PBC easily leads to a final diagnosis by testing for anti-mitochondrial antibodies. Liver transplantation is the only effective treatment. The aim of this study was to test an electronic diagnostic tool (tool) for it's ability to include PBC in the working differential diagnosis. METHODOLOGY: In the European Union Euricterus project a large number of (sub)icteric patients in 17 discrete disease categories, PBC being one of them, were gathered prospectively. A tool was developed-using Bayes (B) and Trial Algorithm (TA) pattern-recognition and based on items related to the history, symptoms and signs of all Euricterus patients. We have tested the diagnostic tool on 143 PBC Euricterus patients. RESULTS: PBC was mentioned by the tool in 86% (B) and 91% (TA) of the 143 patients. These figures were higher for patients under 60 and (TA only) females. Females under 60 (n = 89) scored 92% B and 96% TA. A sole diagnosis of PBC was made in 31% (B) and 66% (TA). In the other patients with a PBC probability, 7 other (first) diagnoses were presented by the tool of which non-alcoholic active liver disease and pancreatic or biliary carcinoma were the leaders. These 7 diseases appeared evenly distributed along the percentual probabilities of PBC given by the tool (B) and also along Pugh and Mayo scores (B and TA). PBC was mentioned by the tool in all patients with a Pugh score 10 or higher (advanced disease, class C). In the patients in whom the tool did not mention PBC, the primary diagnoses came from 9 other disease categories. CONCLUSION: This electronic tool has been able to identify PBC as one of the differential diagnostic modalities in the large majority of a present population of PBC patients.
BACKGROUND/AIMS: In the European Union Euricterus Project on (sub)Icterus proforma, the history and physical examination items were to be used for the physician's working diagnosis (PWD) and 'among others, for the development of the real life data electronic diagnostic tool, Trial. Trial delivers diagnosis probabilities based on Bayes' Theorem (B), completed by Trial Algorithm (TA). We wanted to compare the diagnostic accuracies (PWD and Trial probabilities as a percentage of the final diagnosis (FD) in a patient population) in 3 Dutch databases. METHODOLOGY: The inclusion criteria for both Euricterus and Trial were age > or = 16 and bilirubin > or = 20 mmol/l. Euricterus data gathering took place at the bedside on a proforma with (among other questions) 79 questions on history and physical examination as well as the diagnosis levels for the PWD (1 alternative possible) and FD (17 disease categories, dc). Trial was developed on the data of 7,104 Euricterus patients and its data-entry Demo has the same questions. It calculates the probability of each diagnosis of the 17 dc as a percentage, as each significant finding is encountered (BO, Bayesian Overall). It can simultaneously calculate the resemblance of the patient's signs and symptoms to each disease concomitantly (BV, Bayesian Vertical), and to any subset of a disease. Any probability is further tested for compatibility using TA, a subset of BV, delivering TA-PWD, TA-BO and TA-BV. The Trial test patients came from 3 databases: a Euricterus Dutch Patients Random Sample EDRS (n = 184, internal database) and 2 independent databases: prospective P (n = 80) and retrospective R (n = 152), totalling 416 patients. RESULTS: The accuracies of PWD and Trial showed no differences between the databases, and the results are therefore pooled (n = 416). With testing on the highest probability found, the PWD accuracy was 78%, TA-PWD 81%, TA-BO 74% and TA-BV 72%. The true FD's were mentioned (at any probability) in the PWD in 86%, TA-PWD in 92%, TA-BO in 94% and TA-BV in 91% of the patients. Testing only patients whose FD was "certain" or whose data were without omissions did not improve accuracy. Testing on probability > 95% improved BO and BV accuracy, but not TA-BO or TA-BV. CONCLUSIONS: The Physician's Working Diagnosis accuracy was approximately 80% and did not greatly improve after TA. The Trial TA-BO and TA-BV accuracies were only slightly less than the PWD. For well-trained physicians, Trial strengthens the physician's judgment, and for those less trained (or those to be trained), it delivers a (sub)icterus diagnostic disease probability at nearly consultant level.
BACKGROUND/AIMS: The assessment of disease stage in cirrhosis is important for the individual patient (prognosis, timing and risk for requiring surgical intervention) and also for population comparisons and trials. There are several established methods, and we have aimed at comparison of the methods within a large cirrhosis population. METHODOLOGY: In the European Union Euricterus database, there are 1015 patients with a "certain" diagnosis of cirrhosis, each of whom in one session had a protocol work-up of history, physical examination and all laboratory investigations needed for this study. The Child-Turcotte (CT), Campbell-Child (C) and Pugh-Child (P) classifications, as well as ascites/no ascites, ascites 1, 2, 3 (no, therapy responsive, nonresponsive) and ascites/nutritional state (ANS, 1-9) scores were used. CT and C have the same 5 variables, P has prothrombin time instead of nutritional state. CT, C and P variables score 1-3 each. C and P furthermore have variable range scores of 5-15. CT, C and P have classes A-C. The variables used were ascites, nutritional state, encephalopathy, bilirubin, albumin and prothrombin time. RESULTS: Only 53 patients (5%) fit within the CT criteria. C and P variable range scores (5-15) correlated strongly (r = 0.84). Cross-over calculation showed slightly different results in the P and C choice of variables, while the variable ranges (1-3) did not matter. Different selection of score ranges for the A-C classes in C and P resulted in 69% class C in P (35% in C) and 3% A in P (19% in C). The patients with ascites (70%) had worse bilirubin, albumin, nutritional states and C and P 5-15 scores (p < 0.0001). Patients with ascites 3 had all variables and also C, P 5-15 scores worse than those with ascites 2 (p < 0.02). ANS scoring showed wasting in 33% of the patients without ascites (ANS 3), 50% of the patients with ascites 2 (ANS 6) and 60% with ascites 3 (ANS 9) (p < 0.0003), and C and P scores were higher in the 3 ANS scores with wasting. CONCLUSIONS: Campbell and Pugh 5-15 scores correlated closely and can be used interachangeably. As C does not contain the more elaborate prothrombin time determination, it probably can be used anywhere in the world. Ascites (degree) and Ascites/Nutritional State (ANS) scoring only use history and physical examination and are, or remain, although less refined, clinically relevant.
13N-ammonia, applied rectally, after absorption and transportation visualises the liver. After release of 13N-aminoacids and 13N-urea by the liver, 13N-activity can be measured over other organs. In patients with porta-systemic shunts, 13N-ammonia will appear in the systemic circulation as well. In 16 controls and 26 patients with cirrhosis, activities were measured for 20 minutes over liver, spleen, heart, lungs and forearm. In all subjects, the liver was visualised within a minute, in some patients with cirrhosis faintly, however. Release by the liver of 13N-ammonia metabolites started within a few minutes. Liver/heart activity ratios proved to be more discriminating between the control- and the cirrhosis group than liver/lung and liver/spleen ratios. In the control group, the 20 minutes' liver/heart ratio was most suitable for determining the normal range. The lower normal level was found to be 2.25. Fourteen of the 26 patients with cirrhosis had a normal, 12 an abnormally low 20 minutes' liver/heart ratio.
The 20 minutes' liver/heart activity ratio after rectal administration of 13N-ammonia was abnormally low (less than 2.25) in 12 of 26 patients with cirrhosis of the liver. An abnormal conventional rectal arterial ammonia test (porta-systemic shunts), an abnormally low urea index (prevailing hepatofugal portal venous flow direction), marked portal hypertension (hepatic sinusoidal pressure greater than or equal to 8 mm Hg), ascites and extreme enlargement of the spleen occurred significantly more often in the patients with an abnormally low 13N-liver/heart ratio than in those with a ratio greater than or equal to 2.25. There was no correlation between the 13N-liver/heart ratio and absence or presence of oesophageal varices. The non-invasive rectal 13N-ammonia test appears to be an easy to perform, informative test in cirrhosis of the liver.