The Spanish Pancreatic Club recommendations for the diagnosis and treatment of chronic pancreatitis: part 1 (diagnosis).

Chronic pancreatitis (CP) is a relatively uncommon, complex and heterogeneous disease. The absence of a gold standard applicable to the initial phases of CP makes its early diagnosis difficult. Some of its complications, particularly chronic pain, can be difficult to manage. There is much variability in the diagnosis and treatment of CP and its complications amongst centers and professionals. The Spanish Pancreatic Club has developed a consensus on the management of CP. Two coordinators chose a multidisciplinary panel of 24 experts on this disease. A list of questions was drafted, and two experts reviewed each question. Then, a draft was produced and shared with the entire panel of experts and discussed in a face-to-face meeting. This first part of the consensus addresses the diagnosis of CP and its complications.

The Spanish Pancreatic Club's recommendations for the diagnosis and treatment of chronic pancreatitis: part 2 (treatment).

Chronic pancreatitis (CP) is a complex disease with a wide range of clinical manifestations. This range comprises from asymptomatic patients to patients with disabling symptoms or complications. The management of CP is frequently different between geographic areas and even medical centers. This is due to the paucity of high quality studies and clinical practice guidelines regarding its diagnosis and treatment. The aim of the Spanish Pancreatic Club was to give current evidence-based recommendations for the management of CP. Two coordinators chose a multidisciplinary panel of 24 experts on this disease. These experts were selected according to clinical and research experience in CP. A list of questions was made and two experts reviewed each question. A draft was later produced and discussed with the entire panel of experts in a face-to-face meeting. The level of evidence was based on the ratings given by the Oxford Centre for Evidence-Based Medicine. In the second part of the consensus, recommendations were given regarding the management of pain, pseudocysts, duodenal and biliary stenosis, pancreatic fistula and ascites, left portal hypertension, diabetes mellitus, exocrine pancreatic insufficiency, and nutritional support in CP.

Plasma adrenomedullin levels in patients with hepatic cirrhosis.

OBJECTIVE: The role of adrenomedullin in cirrhosis is at present unknown. The aim of this study was to investigate plasma levels of adrenomedullin in cirrhosis. METHODS: We measured plasma adrenomedullin concentrations using radioimmunoassay in 43 patients with cirrhosis (17 in Child-Pugh's class A, 13 in Child-Pugh's class B, and 13 in Child-Pugh's class C) and compared these values with those in 24 age-matched healthy subjects. Renal function, plasma cyclic adenosine monophosphate (cAMP) concentration, plasma renin activity, and plasma aldosterone concentration were measured in each patient. RESULTS: Plasma adrenomedullin levels were increased significantly in patients with cirrhosis compared with healthy subjects. In addition, plasma adrenomedullin values were higher in patients with cirrhosis and ascites than in those without ascites. Moreover, plasma adrenomedullin levels increased in relation to the severity of cirrhosis. Significant positive correlations also were found between adrenomedullin and cAMP concentrations and plasma renin activity or plasma aldosterone concentrations. CONCLUSIONS: Plasma adrenomedullin is increased in patients with cirrhosis compared with healthy subjects. The increase in plasma adrenomedullin levels is related at least in part to the severity of cirrhosis and may contribute to the arteriolar vasodilation in patients with cirrhosis. These effects are related in part to a cAMP-dependent pathway.

Value of endothelin in cellular rejection after liver transplantation.

Endothelin-1 is a vasoconstrictor peptide released by the vascular endothelium by various chemical and mechanical factors. Cellular rejection is one of the most common complications following orthotopic liver transplantation, endotheliitis being its most specific and consistent histological feature. To assess the role of endothelin in cellular hepatic rejection, we studied 21 cirrhotic patients undergoing elective liver transplantation. These patients were divided in two groups: Group I comprised 10 patients without cellular rejection, and Group II comprised 11 patients with cellular rejection. Endothelin was measured on day 7 after transplantation, on the day of liver biopsy, and after treatment for rejection. We found a significant increase in the plasma endothelin levels in the early postoperative period in the patients with moderate or severe cellular rejection compared with the non-rejection group. It remained significantly elevated until the clinical diagnosis of rejection was made. This value returned to baseline with successful treatment. So, the monitorization of this peptide may be of help in the diagnosis of rejection, its severity, and the evaluation of its resolution.

[Intense neutropenia of 14 years duration as the only manifestation of a myelodysplastic syndrome]. / Neutropenia intensa de 14 años de duración como única manifestación de un síndrome mielodisplásico.

Myelodysplastic syndromes (MDS) are a group of acquired hemopathies characterized by peripheral cytopenias due to ineffective hematopoiesis and a high risk of transformation into acute non lymphoblastic leukemia (ANLL) which, in most cases, usually occurs from 6 months to 4 years after diagnosis. A patient with extreme neutropenia with intense dysgranulopoiesis as the only manifestations of MDS is described. The patient was controlled over 14 years and presented multiple infectious episodes, in various locations, throughout the evolution, some being very severe and generally caused by gram-negative germs. Likewise, during this time the patient received different treatments (oxymetholone, prednisone and lithium carbonate) with no hematologic response being observed. The leukocyte count remained around 3 x 10(9)/L with a mean proportion of neutrophils of 12% with no variations being found in the bone marrow aspirates carried out throughout the evolution (total of 9). At 14 years the diagnosis of MDS evolved to ANLL. The patient died shortly after the acute transformation due to respiratory failure secondary to bilateral pneumonia. In this case three peculiar features are of note: the almost exclusive involvement of the granulopoietic series without either anemia or thrombocytopenia, the long evolution of AREB, with acute transformation 14 years after diagnosis and the severity of the infections, among which recurrent lingual granulopenic ulcers were of note.

[Primary extralymphatic non-Hodgkin's lymphomas: the response to treatment and prognostic factors in 73 patients]. / Linfomas no hodgkinianos extraganglionares primarios: respuesta al tratamiento y factores pronósticos en 73 pacientes.

BACKGROUND: The aim of the present study was to determine the prognostic factors for treatment response, relapse-free survival (RFS) and overall survival (OS) in a series of 73 patients with primary extranodal lymphomas (PEL). METHODS: All the patients diagnosed with PEL in one center from January 1984 to December 1993 were studied. The Working Formulation was used for histologic classification and the stage was determined according to the Ann Arbor classification modified by Mushoff. Age, sex, localization of the lymphoma, the presence of B symptoms, the existence of bulky disease, human immunodeficiency virus (HIV) infection, the main hematological and biochemical parameters, the histologic grade of malignancy, the immunologic phenotype and stage were reported. Univariate and multivariate analyses of prognostic factors for complete response (CR), RFS and OS were carried out. RESULTS: The most frequent localization of PEL was the gastrointestinal tract (43 cases). Fourteen cases had HIV infection. CR was obtained in 37 out of the 55 patients (67%) of whom 8 (21%) had relapsed. Serum LDH level was the main prognostic factor for CR attainment (p = 0.01). The variables which negatively affected RFS were the presence of HIV infection (p = 0.02), advanced age (p = 0.018) and the existence of B symptoms (p = 0.02). OS was shorter in patients with high LDH levels (p = 0.004) and more aggressive histologic subtype (p = 0.0002). CONCLUSIONS: The prognostic factors observed in the patients with primary extranodal lymphomas are the same as those found in nodal non Hodgkin's lymphomas. The stage is not a factor of bad prognosis if chemotherapy is included in the treatment.

[Primary gastrointestinal lymphoma: a study of 25 cases]. / Linfoma gastrointestinal primario: estudio de 25 casos.

BACKGROUND: The digestive tract constitutes the most frequent localization of the extranodular non Hodgkin's lymphoma. The chief clinical and histological characteristics were analyzed as were the evolution and response to treatment of 25 patients diagnosed with primary gastrointestinal lymphoma (PGIL) in one center over a period of eight years. METHODS: To establish the diagnosis of PGIL the Dawson criteria were used and the state was determined by the Ann-Arbor classification modified by Musshoff. To histologically classify of the PGIL the Working Formulation was followed. The influence of the clinical, histological characteristics and the state of the obtention of complete remission (CR), the survival free period of the disease (SFD) and global survival (GS) were analyzed. RESULTS: The mean age of the series was 56 years (standard deviation 15 years) (12 males). The most frequent localization was gastric followed by the small intestine and the large intestine. Abdominal pain and weight loss were the most frequent clinical manifestations in the PGIL, regardless of its site. In 12 patients the PGIL was of an intermediate grade of malignancy, 8 were of low grade and 5 of high grade. The state was IE in 11 patients, IE1 in 9 and IIE2 in 5. The most used treatment was radical surgery followed by polychemotherapy. Complete remission was obtained in 15 patients and 2 recurred. The foreseen SFP at 7 years was 69% of the cases and GS was 53%. In IE state patients was most frequently obtained and GS was also more prolonged in these patients. CONCLUSIONS: In the present series the gastric localization of patients with primary gastrointestinal lymphoma was more frequent than intestinal localization. The HNL of intermediate and high grades of malignancy predominated those of low grade. The prognosis of patients with primary gastrointestinal lymphoma in a IE state was more favorable than the remaining patients. In turn, surgery constituted a good option in the initial treatment of patients with primary gastrointestinal lymphoma.

[Plasma cell leukemia. Study of 6 patients]. / Leucemia de células plasmáticas. Estudio de seis pacientes.

PURPOSE: To analyse the clinico-biological characteristics, the clinical course and the response to therapy in a group of patients with plasma cell leukaemia (PCL). MATERIAL AND METHODS: Out of a total number of 107 patients diagnosed of multiple myeloma (MM) between 1983 and 1991, 6 were found to meet the criteria for PCL (prevalence: 5.6%). This was primary in 2 cases and secondary in the remaining 4. The M/F ratio was 2/1 and the median age was 63 years (range: 57-69 years). RESULTS: Two patients had bone pain and two others weight loss at the onset of PCL. The outstanding haematological findings were increased ESR, normocytic-normochromic anaemia and thrombocytopenia, which were present in all cases. The percentage of peripheral blood plasma cells was between 29 and 70, and the bone marrow aspirate showed plasma cell infiltration over 40% in all cases. Serum M component was found in 5 patients, with decreased values of the polyclonal immunoglobulins; the remaining patient had non-secretory MM. Renal insufficiency was present in 3 patients at diagnosis. Three of the 4 patients with secondary PCL had been previously given combination chemotherapy and the remainder had received melphalan and prednisone. The period between the diagnosis of MM and the development of PCL ranged between 1 and 21 months (median 15 months). Three patients were treated with the M-2 protocol and the others received only supportive therapy. Transient partial response could be achieved in only one case with chemotherapy. All the patients have died, the actuarial survival median being 1 month (range, 1-7 months). Three patients died of infection, 2 of renal insufficiency and one of heart failure after acute myocardial infarction. CONCLUSION: The poor prognosis of PCL was confirmed, along with the scarce response to therapy of these patients.

Cardiac tamponade as the initial manifestation of acute leukemia: report of a case and review of the literature.

An 18-year-old woman with acute monoblastic leukemia presenting with cardiac tamponade is reported. Subxiphoid pericardiotomy was performed, and blast cells were observed in the pericardial fluid. The leukemia was refractory to chemotherapy and the patient died at three months from diagnosis, without recurrence of the pericardial effusion. In the review of the literature, only ten cases of cardiac tamponade as the first manifestation of acute leukemia have been found.