Biomedical subjects
Publications and source records attributed to J Rahier.
The authors report their experience of liver transplantation for metastatic tumor in 6 patients. Although good palliation can be offered with prolonged survival in some patients, secondaries of the liver remain the poorest indication for liver transplantation. A prospective multicentric study would be needed to evaluate the usefulness of post-transplantation chemotherapy.
We report the case of a 72-year-old woman with well-controlled chronic lymphocytic leukemia (CLL) and splenomegaly who developed portal hypertension with bleeding oesophageal varices in the absence of liver fibrosis or regenerative nodular hyperplasia at surgical wedge liver biopsy. The hepatic venous pressure gradient (HVPG) was elevated and splenectomy resulted in both its normalisation and the regression of oesophageal varices. This case shows the potential for an increased spleno-poral flow to generate severe portal hypertension likely through a "protective" sinusoidal vasoconstriction.
The Paediatric Liver Transplant Program at Saint-Luc University Clinics constitutes a substantial single centre experience, including 667 transplantations performed between March 1984 and April 2003, and the history of this program reflects the tremendous progress in this field since twenty years. Liver transplantation in children constitutes a considerable undertaking and its results depend on multiple, intermingled risk factors. An analysis of the respective impact of several surgical and immunological parameters on patient/graft outcome and allograft rejection after paediatric liver transplantation showed a significant learning curve effect as well as the respective impact of pre-transplant diagnosis on survival and of primary immunosuppression on the rejection incidence. The introduction of living related liver transplantation in 1993 not only permitted to provide access to liver replacement in as many as 74% more candidate recipients, but also resulted in better graft survival and reduced retransplantation rate. The results of a recent pilot study suggest that steroid avoidance is not harmful, and could even be beneficial for paediatric liver recipients, particularly regarding growth, and that combining tacrolimus with basiliximab (anti-CD25 chimeric monoclonal antibody) for steroid substitution appears to constitute a safe alternative in this context. The long-term issues represent the main future challenges in the field, including the possibility of a full rehabilitation through immunosuppression withdrawal and tolerance induction, the development of adolescence transplant medicine, and the risk of early atherogenesis in the adulthood.
The authors present the results of a single centre study of 587 liver transplants performed in 522 adults during the period 1984-2002. Results have improved significantly over time due to better pre-, peri- and post-transplant care. One, five, ten and fifteen year actuarial survivals for the whole patient group are 81.2; 69.8; 58.9 and 51.2%. The high incidence of de novo tumors (12.3%), of cardiovascular diseases (7.5%) and of end-stage renal function (3.6%) should be further incentives to tailor the immunosuppression to the individual patient and to direct the attention of the transplant physician to the long-term quality of life of the liver recipient.
Somatostatin blocks the release of numerous growth factors and is therefore a potent inhibitor of cell division and/or secretion. It exerts its effects through binding to somatostatin receptors. Five different subtypes of such receptors are identified (SSTR1 to SSTR5), having various tissue expression. The detection of their presence in tumours can be performed on histological sections and has potential therapeutic implications.
Liver biopsy is considered to be the most specific analysis to assess the nature and severity of liver disease. In case of chronic hepatitis, scoring liver biopsies is an established part of the pathologist's work. Four different scores are most often used: the Scheuer, Ludwig and French METAVIR systems, which are fairly simple, and the Ischak score, which is more complex. All systems generate scores, which are based upon inflammatory activity (the grade) and fibrosis (the stage), with splitting of these two components. To be valid in routine analyses, a scoring system must be clinically relevant, reproducible and simple to understand and to apply. Scoring will then be helpful to study series of patients and to evaluate the efficacy of new therapeutic strategies. However, a score does not replace the study of a liver biopsy and the generated numbers does not correspond to true measurements. Furthermore, its accuracy will always depend on adequate sampling.
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We report the case of a VIPoma diagnosed in a 15-year-old teenager who experienced profuse secretory diarrhea associated with hypokalemia, metabolic acidosis and high plasma levels of vasoactive intestinal peptide (VIP) and pancreatic polypeptide (PP). Angiography showed an abnormal mass in the head of the pancreas. Before surgery, subcutaneous injections (100 micrograms every 8 hours) of the long-acting somatostatin analogue octreotide or SMS 201-995, were administered in order to stabilize the clinical status of the patient and to reduce the intravenous administration of fluid and electrolytes. This treatment resulted in prompt relief of the symptoms and in a partial decrease of the plasma levels of VIP and PP. At subsequent laparotomy, there was a tumour localized in the head of the pancreas, which was completely removed by Whipple resection. The immunohistochemical staining revealed the presence of VIP and PP inside the tumour cells. Two years after surgical resection, the patient is healthy without clinical or laboratory evidence of recurrence.
The authors review the indications and outcome of liver transplantation for hepatic cirrhosis of various aetiologies in adults, on the basis of their experience and that of the literature. Up to 1989, they performed 107 liver transplantations in 93 adults, including 70 patients with cirrhosis: primary biliary cirrhosis (PBC) in 20, primary sclerosing cholangitis (PSC) in 2, secondary biliary cirrhosis in 1, post-necrotic cirrhosis in 35, alcoholic cirrhosis in 5, metabolic cirrhosis in 7. The best indications are PBC with rising bilirubinemia (greater than 5 mg/dl) or portal hypertension and PSC with severe and diffuse lesions. The outcome of transplantation was satisfactory in patients with PBC: 16 survived with a mean follow-up of 23 months. In patients transplanted for postnecrotic cirrhosis the outcome might be better if HBS is associated preoperatively with delta antigen: 6 patients of this group survived with a mean follow-up of 12.6 months while in the group of 8 patients with HBS not associated preoperatively with delta antigen, 6 patients survived with a mean follow-up of 12.8 months. There was a trend toward a higher hospital mortality after transplantation in cirrhotic patients with pulmonary arterio-venous shunts in comparison with cirrhotic patients without significant pulmonary arterio-venous shunts preoperatively.
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The artificial endocrine pancreas is generally used to control blood glucose in brittle diabetic patients. In this study, it was applied to the diagnosis and surgical management of a pancreatic insulinoma. Several tests were performed without the risks inherent to severe hypoglycemia. It was also used during surgery, permitting an optimal blood glucose control.
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The authors review the recent development of methods aimed at characterizing endocrine tumours. With new immunocytochemical methods, not only specific hormonal peptides can be identified but also hormonal precursors and their sites of processing. Immunodensitometry allows to quantify the hormonal cellular load and to assess hormonal synthesis via the precursors. Finally hormonal receptors can be identified by various techniques of autoradiography.
The authors report their experience with octreotide in 20 patients (median age 57 years, 10 M, 10 F) from 1984 to 1991; 16 had metastatic APUDoma: 1 PPoma with VIPoma, 1 glucagonoma, 5 gastrinoma including 1 associated to PP-oma, 9 mid-gut carcinoid; 3 patients had multiple-endocrine neoplasia type I (MEN-I) with Zollinger-Ellison syndrome (ZES) and 1 patient a non-metastatic VIPoma. Octreotide (200-750 micrograms/day) was administered bid or tid with regular laboratory controls and morphological assessment. There was a striking improvement of symptoms, particularly in the carcinoid group (reduction of flushing in all patients and of diarrhoea in 3/5), in the patient with gastrinoma + acromegaly (regression of congestive heart failure) and in the patient with non-metastatic VIPoma. The hormonal markers were markedly reduced, particularly gastrin, PP (except in the patient with PPoma + VIPoma), VIP, GH and Somatomedin-C and urinary 5HIAA in 4/9 patients with carcinoid. There was only one partial regression of metastases (gastrinoma) and 4 apparent stabilizations of tumour growth, in the 16 metastatic cases. Among them, 4 patients died: 1 glucagonoma, 1 PPoma + VIPoma, 2 mid-gut carcinoids after a treatment of 5, 16, 30, 36 months, respectively. The patient with acromegaly + ZES died after 6 years of treatment at age 81. A patient with prolactinoma, resected insulinoma, hyperparathyroidism and ZES was not improved by a short course of octreotide (hypoglycemia); he died later of recurrent insulinoma. In conclusion, octreotide is a useful drug to control most of the symptoms related to gut endocrine tumours; it may inhibit tumour growth.
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