[Diagnosis of chronic myeloproliferative diseases].
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Biomedical subjects
Publications and source records attributed to A Kiss.
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The EcaI GGTNACC-specific DNA-adenine modification methyltransferase has been purified to apparent homogeneity. The active form of the DNA methyltransferase is a single polypeptide. The enzyme has a pH optimum at pH 8.0 and a temperature optimum at 25 degrees C. EcaI DNA methyltransferase transfers one methyl group to the adenine of the recognition site in a single binding event. The Km was 170 nM for DNA and 1.8 microM for the methyl donor S-adenosylmethionine. Methylated DNA is a competitive inhibitor with respect to DNA (Ki = 3.5 nM). The other product of the DNA-methylation reaction, S-adenosylhomocysteine was found to be a competitive inhibitor with respect to S-adenosylmethionine (Ki = 2.7 microM). The S-adenosylmethionine analog sinefungin was shown to be a very strong inhibitor (Ki = 3.5 nM) of the DNA methyltransferase reaction.
Two secondary acute leukaemias out of 166 patients with Hodgkin's disease were detected. At a young female patient treated only with chemotherapy the acute leukaemia developed 39 months following the diagnosis of Hodgkin's disease. Phenotype of leukaemic blast cells could not be determined exactly. After a short complete remission (1,5 month) the patient died because of the progression of her leukaemic process. The other young male patient treated with radio- and chemotherapy had six years disease free interval between the diagnosis of two malignant diseases. The combined cytostatic treatment of his acute lymphoblastic leukaemia resulted complete remission that lasted for one year but later the progression of leukaemia caused death. In connection with these cases the authors reviewed the current questions of treatment related acute leukaemias in Hodgkin's disease.
Unspecific complaints are one of the major reasons why doctors are consulted. The management of these patients is difficult and has been little explored. Most doctors confine their efforts to ruling out organic causes for the patient's symptoms, a policy which often results in frustration for both doctor and patient. The following guidelines for better management have proven useful in clinical practice: (1) Psychosocial history: Psychosocial data should be obtained from the start, not only when no organic reason is found to explain the symptoms. (2) Specific questions: What alleviates symptoms, what makes them worse? In the case of vague answers ("stress") it is preferable to check before giving hasty advice. (3) Disclosing the diagnosis: It is necessary to convey the idea that the symptoms are real. Phrases such as "It's nothing" or "It's all in your head" should be avoided. (4) Lay theory: The patient's view of the reasons for his symptoms should be explored. (5) Agreement on working hypotheses: Knowing the patient's lay theory, the doctor can shape his suggestions to match the patient's convictions. (6) Therapeutic goals: Establishment of a climate of trust between patient and doctor can avoid "doctor shopping" and protect the patient from unnecessary diagnostic procedures. (7) Psychotherapeutic techniques: A supportive approach and relaxation techniques of limited duration (5-10 consultations) afford long-term benefit.
A review of the last decade's literature suggests that the doctor-patient relationship could be improved in three main respects: (1) Medical, social and psychological information is obtained incompletely or incorrectly. (2) A positive emotional relationship with the doctor is reported by the patient who has been able to talk about his own ideas, fears and expectations. The doctor rarely enquires about the latter and, if he does so, pays little attention to the reply. (3) Patients are not well informed about prior test results, prognosis or etiology. Concepts in social psychology afford valuable and practical hints in improving the doctor-patient relationship: checking on what the doctor and patient have said; a structured interview (use of a list of topics; open discussion of time limits); avoidance of yes/no type questions at the beginning; perceptions of patients' emotions; partnership-based approach. As a means of approaching the doctor-patient relationship on a psychoanalytic basis, Balint groups have proved of value.
A silver staining technique for nucleolar organizer regions (NORs) has been applied to bone marrow biopsies of various types of acute and chronic leukaemias. This method could be easily evaluated on resin-embedded bone marrow obtained from acute lymphocytic leukaemia (n = 12), acute myelogenous (n = 16), chronic lymphocytic (n = 16) and chronic granulocytic (n = 20) leukaemia. A significant difference (p < or = 0.1) was only found between the AgNOR numbers in nuclei of lymphocytes from acute and chronic leukaemia (mean of 1.23 to 1.40 and 1.58) and those of cells from acute and chronic myelogenous leukaemia (from a mean of 5.00 to 9.17 per nucleus). However, no significant difference was observed among cells of various types of acute and chronic myelogenous leukaemias, despite of their markedly higher staining intensity and proliferative activity. The greatest mean of AgNOR numbers was counted in monoblasts of acute myelomonocytic leukaemia. It is suggested, that higher AgNOR counts in nuclei of more malignant leukaemic cells are in parallel with their mitotic activity and could be related to their elevated cell turn-over.
The role of alpha 1-adrenergic receptors in the secretion of corticotropin-releasing hormone (CRH) during stress was studied by immunohistochemical analysis of the CRH content of the median eminence (ME) after intracerebroventricular (icv) administration of the alpha 1-adrenergic agonist, methoxamine, or the antagonist, prazosin, in rats pretreated with colchicine. Immunohistochemical staining was performed by the peroxidase technique on 40 microns free-floating sections using a polyclonal antibody specific for CRH. In the first experimental model, rats were implanted with icv cannulae and adapted to the experimental conditions by daily handling and icv injection of artificial CSF. Colchicine (75 micrograms) was administered through the cannulae 6 h before the experiment, conditions in which axonal transport was blocked with little change in basal immunostaining. Two hours after immobilization stress or a single injection of methoxamine (100 micrograms, icv), there was a marked decrease in CRH immunoreactivity throughout the ME, reflecting release of the neuropeptide into the portal circulation. The decrease in CRH immunostaining following immobilization was largely prevented by icv injection of the alpha 1-adrenergic antagonist, prazosin. In the second experimental model, rats were sacrificed 48 h after icv colchicine injection, conditions in which colchicine acts as a stressor and causes marked depletion of irCRH from the ME. This chronic effect of colchicine was also partially prevented by administration of prazosin, 400-ng injection 5 min prior to colchicine, followed by a continuous icv mini-pump infusion of prazosin, indicating that alpha 1-adrenergic stimulation contributes to the action of colchicine.(ABSTRACT TRUNCATED AT 250 WORDS)
DNA sequences distantly related to the proviral DNA of HTLV-I were found in the leukemic cells of a Hungarian patient suffering from Sézary syndrome. Serum samples from the patient contained antibodies reactive with the internal core polypeptides of HTLV-I and HTLV-II, but not with the env gene encoded type-specific HTLV antigens. The husband and daughter of the patient also had antibodies of the same specificity. These findings suggest the presence of a virus distantly related to HTLV-I and HTLV-II.
The Authors propose the use of intraoperative colon irrigation in emergency surgery of left-sided lesions of the colon. This technique allows the colon to be decompressed and cleaned at the same time with the possibility of performing a resection or hemicolectomy without having to resort to protective colostomy and its related problems of morbidity.
We investigated infants with Kawasaki disease, congenital coronary artery fistulas and anomalous origin of the left coronary artery from the pulmonary trunc (Bland-White-Garland syndrome) by 2-dimensional and Doppler echocardiography. We describe the systematic approach for visualizing in detail coronary anatomy by two-dimensional echocardiography. Our results suggest that this echocardiographic technique is useful for detecting coronary anomalies and has a great rolbefore angiocardiography especially in sick babies. We recommend performing 2-dimensional echocardiography in the acute and chronic stage of Kawasaki disease for evaluating coronary arterial aneurysms.
Cyclitol derivatives have been synthesized and screened for growth inhibitory effect upon prokaryotic and eukaryotic organisms. One derivative, (2S,3R,5R)-3-azido-2-benzoyloxy-5-hydroxycyclohexanone, was studied in detail: it has no effect upon bacteria, but it is inhibitory to Neurospora crassa. In Neurospora crassa it increased the amount of myo-inositol-1-phosphate synthase and inhibited the activity of myo-inositol-monophosphatase. The enhanced synthesis of myo-inositol-1-phosphate synthase was the consequence of lowering the intracellular inositol concentration. Li+ treatment of Neurospora crassa has effects similar to those of P.I.-658.
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Clinical data of 70 patients, treated and observed with myelodysplastic syndrome between 1977 and 1989 were analysed. Two-thirds of the patients belonged to the elder age-group and a mild female predominance was registered. With the application of complex cytochemical-histological and cytogenetical methods, correct diagnosis could be established. The clinical material included patients from different morphologic subtypes: 19 with refractory anaemia (with a longer course of the illness). 20 with sideroblastic anaemia, 26 with chronic myelomonocytic leukaemia and the remaining 5 with refractory anaemia with excess of blasts (a more progressive type of the myelodysplastic syndrome, with a short duration). The mean survival of all patients were 42 months. 45 (69%) died during this period and 12 (18.5%) among them in acute myelogenous leukaemia (mean survival: 16 month). Megakaryoblastic leukaemic transformation was observed in three patients with sideroblastic refractory anaemia. Haemorrhage and infection-sepsis, due to thrombocytopenia and/or granulocytopenia, was fatal in 30 cases. In the treatment of the myelodysplastic syndrome an appropriate supportive therapy (blood transfusion, antibiotics) has a decisive importance. A more aggressive treatment with cytostatic drugs is suggested in the progressive form of the disease of younger patients and in patients with overt acute leukaemia.
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In view of the proteiform symptoms of duodenal diverticula, the Authors underline the diagnostic precision and surgical value of e.g.d. scopy. Indications for surgery in cases of symptomatic or complicated diverticula are also stressed, and the value of mechanical suturing is confirmed.
Only few clinical reports must suffice at present to assume that in patients with Crohn's disease like in those with ulcerative colitis psychotherapy--regardless how intensive it might be--has not been proven to cure the underlying biologic disturbance. Therefore the hope of complete cure by psychotherapy is not warranted. While improvement and even freedom of all symptoms may take place, psychotherapy cannot prevent stress-related relapse.
Authors report the case of a 42 years old female with primary Sjögren's syndrome in whom nine years after the beginning of her disease generalised swollen lymphatic nodes and enlargement of liver as well as a spleen has appeared. The simultaneous appearance of fever, loss of body weight and worsening of her general condition raised the possibility of an associated malignant lymphoproliferate disease. The histology of a lymph node proved the presence of a highly malignant form of B-cell lymphoma (CB NHL). Attention is drawn to the necessitate of regular control of patients with Sjögren's syndrome. Lymphadenopathy, the decrease of the immunoglobulin levels and autoantibody titer in the serum, as well as the predominance of monoclonal immunoglobulins point to the possibility of malignant transformation. With reference to the presented case a short review of the Sjögren's syndrome is given by the authors.
The connective tissue content of four different human liver tumors (Fibrolamellar carcinoma, FLC; hepatocellular carcinoma, HCC; focal nodular hyerplasia, FNH and hepatocellular adenoma, HCA) was investigated by computer aided morphometry. A significantly higher connective tissue content was found in FNH and FLC as compared to HCA and HCC. The distribution of the connective tissue was homogeneous in the cases of FNH, HCA and HCC, while a highly unhomogeneous distribution was observed in FLC cases.