Acute pancreatitis after orthotopic liver transplantation in children.
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Biomedical subjects
Publications and source records attributed to C Chardot.
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According to French rules for cadaver organ sharing, children with biliary atresia (BA) complicated with acute necrosis (ALN) can be registered on the waiting list for liver transplantation (LT) in a special intermediate grade urgent code. Over a 7 years period, 100 children have been submitted to elective LT for BA and 15 to urgent LT. Urgent procedures accounted for 25% of LT for BA in patients aged 0-2 years and 67% (8/12) in patients under 1 year of age. Children actuarial survival at 1, 12 and 48 months was respectively 66%, 60% and 60% versus 92%, 86% and 85%, deaths occurring earlier in the urgent group. Graft actuarial survival at 1, 12 and 48 months were 60%, 53% and 53% versus 85%, 77% and 76% (p < 0.05), respectively. Outcome of children and grafts after LT is not significantly different in BA cases and in other urgent indications, excluding retransplantations. In a LT program based on cadaver organ donation, allocation of in an urgent registration code to children with BA and ALN offers them more than 50% chance to escape death and does not result in wasting of grafts.
The aim of this work is to determine the influence of age, extrahepatic biliary lesions pattern (EHBP) and association to polysplenia syndrome (PS) on 10 years outcome of 164 patients with biliary atresia (BA) treated from 1984 to 1992 by initial Kasai operation (KO) and secondary liver transplantation (LT) when necessary. Actuarial crude survival without or after LT (CS), actuarial survival with native liver (NLS) and jaundice-free actuarial survival with native liver (JFS) were calculated from 1 to 10 years versus age (under/over 45 days), EHBP (favorable/ unfavorable) and PS (no/yes). Overall 10-year CS is 70%, overall 10-year NLS and JFS are 14%. In univariate analysis, age at KO under 46 days, favorable EHBP (BA with patent gallbladder, and/or cystic dilatation of extrahepatic bile duct, or BA restricted to choledocus), and absence of PS are significant determinants of a better outcome regarding CS, NLS and JFS. EHBP is more discriminant than age. Influence of PS in this series is redundant with that of EHBP since 11/11 patients with PS had unfavorable EHBP.
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Eleven patients presenting with spontaneous perforation of the biliary tract were treated at Bicêtre Hospital between 1971 and 1993. Three groups were individualised, each with a different pattern of local presentation: generalised biliary peritonitis (n = 2), localised biliary peritonitis (n = 4), secondary biliary stenosis (n = 5). In each case, cholestatic jaundice developed after a postnatal symptom-free interval. Ten patients were operated on. Perforation was located in the cystic duct (n = 2), at the junction of the cystic and hepatic ducts (n = 4), in the common hepatic duct (n = 1) or common bile duct (n = 1). The site of perforation was no longer identifiable in two cases with stenosis. A cholecystectomy was performed in the 2 cases with cystic duct perforation; in the cases of lesions of the main duct, either simple external biliary drainage (n = 3) or biliary reconstruction (n = 5) was carried out. Postoperative complications included bile leak (n = 2), ascending cholangitis (n = 1), portal vein thrombosis (n = 2). Five patients were submitted to further surgery including biliary revision (n = 3), porto-systemic shunt (n = 1), and other procedures (n = 2). One infant died from postoperative sepsis; 2 were lost to follow-up, one of which probably did not survive; 4 are alive and well. Late sequelae are present in 4 children: portal hypertension (n = 1), mild residual bile duct dilatation without cholestasis (n = 1), and mild to moderate liver fibrosis (n = 2). Prompt diagnosis and appropriate treatment should improve the prognosis of this rare condition.
Between 1983 and 1992, 112 children underwent liver transplantation. Of 138 grafts, 60 (43.4%) were whole livers, 77 (55.6%) were reduced livers, and 1 (0.7%) was a split liver. Biliary complications (BC) were defined as any abnormality, even minor, related to the biliary tract. Results were analysed with a minimum follow-up of 9 months. Some 36 grafts (26.1%) in 34 patients (30.4%) presented with BC: bile leaks (17 grafts), biliary obstructions or dilatations (16 grafts), and other complications (3 grafts). Management was mainly surgical with biliary reconstruction via a Roux-en-Y loop. Interventional radiology had an increasing role in recent years. BC were associated with a mortality of 1.8% (2/112), a graft loss rate of 4.3% (6/138), and significant morbidity. Among the various factors whose association with BC was studied, the date of transplantation, the use of reduced grafts and the use of gallbladder conduits appeared to be the main determining factors for BC. From multivariate analysis the use of reduced grafts emerged as the most important factor in reducing BC. We therefore conclude that BC are associated with significant morbidity, but general improvements in both surgical and medical management seem to account for better results in recent years.
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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.
The Assembly of French Professors in Cancerology, consisting of medical and surgical oncologists as well as radiotherapists, has introduced a new diploma for specialized studies in medical oncology. This diploma is entitled oncology, medical option; the pre-existing diploma for radiotherapy is entitled oncology, radiotherapeutic option. Both diplomas are awarded after a common training period followed by specific studies for each one. A third diploma requiring a shorter study period (2 years) has been in existence for a few years and is awarded after complementary pluridisciplinary studies in cancerology. It provides medical oncologists and radiotherapists, as well as surgical and medical specialists in organs with the possibility, if they wish, to acquire global knowledge in cancerology over and above that connected with their specific area of expertise. It familiarizes them with a pluridisciplinary cancerological approach to setting up diagnostic and treatment protocols with subsequent applications to clinical and therapeutic trials. The recent reforms in post-graduate studies have now made available teaching models and state diplomas for all areas of clinical cancerology including an optional pluridisciplinary course--and should constitute an invaluable contribution to both public and private practice. They should also facilitate the work of the state bodies and those in charge of health expenditure.
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The records of 429 patients seen at the Centre Alexis-Vautrin in 1979 and 1980 with bony metastases were retrospectively reviewed to assess their natural history and survival. Breast was the location of primary lesion in 140 patients (32.6%), lung in 95 patients (22.1%) and prostate in 33 patients (7.7%). The primary tumor was of unknown origin in 42 cases (10.9%). The median survival from time of diagnosis was 5 months. Two and 5-year survival rates were 17.5 and 5.1% respectively. According to the nature of the primary tumor, 2 and 5-year survival periods for bony metastases were 36.4 and 7.9% in breast cancer, 33.3 and 15.2% in prostatic cancer, 4.3 and 2.1% in metastases of unknown origin, 2.1 and 0% in pulmonary carcinoma. Multivariate analysis revealed four factors to be of prognostic significance for survival: the nature of the primary tumor, the absence of local relapse, the disease-free interval and the absence of metastases in other sites. Patients with hormone-sensitive lesions or slowly-growing tumors had a better prognosis.
Surgical treatment of metastasis disease of the humerus was applied for 46 lesions among 42 patients, i-e 22% of overall osteosyntheses for skeletal metastasis. Two patients only are still alive at 3 and 4 years. The mean survival time is 8.1 months and at 3 months 50% died. Metastasis disease of the humerus becomes evident late during the evolution of the primary cancer, but 26% are revealing lesions. For 9.5% the primary tumor remains unknown. After review of the surgical methods generally used, indirect osteosynthesis by intra-medullary pinning according to Hackethal, without bone cement, is alleged. So the arm is immediately mobilized and a complementary irradiation is applied in post-operative. Prophylactic fixation of impending fractures is encouraged in order to suppress pain, and for better function. This paper demonstrates the changing concept of internal fixation of pathologic fractures and recommends intra-medullary nailing or pinning.
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During the past 10 years a number of screening tests aimed at an earlier diagnosis of breast cancer have been introduced, and the time has come for their value to be assessed. In this paper, thermography, senography, xerography, echography and needle aspiration or biopsy are reviewed, and their insufficiencies and drawbacks are shown. Periodical self-examination of the breasts, more detailed examination by the practitioner, systemic senography once every 5 years in women older than 35 and earlier in high risk women, needle aspiration for cysts and needle biopsy for solid tumours are at present the only efficient means of early detection of breast cancer.
The authors present 143 cases of breast carcinoma T1 and T2, N0, N1, N2 and N3 without clinical signs of bone metastasis. They study the value of bone scintigraphy with technetium 99m labelled diphosphonates. One hundred and twelve patients had normal bone scans; 31 patiets showed abnormal fixation sites: in 23 (16%) benign bone lesions explained the increase of the bone radioactivity, in 8 cases, scintigraphy was considered to be consistent with diagnosis of bone metastasis. Metastasis has been proved in only 3 cases within a period of 2 years. The detection rate cannot be better than 5,6 per cent. Considering 50 per cent at least of these patients will eventually develop bone metastasis, the false negative rate will be about 45 per cent. Routine bone scan does not seem to be useful for patients with T1 and T2 breast cancer.
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