Immune thrombocytopenia and Crohn's disease.
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Biomedical subjects
Publications and source records attributed to G Bordin.
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A review of the less frequent organ damage seen in the so-called essential mixed cryoglobulinemia, and distinguishing between cryodependent and cryofavouring organ involvement, is presented in this paper. In the first group, along with the well-known renal, cutaneous and articular lesions, the peripheral neuropathy and respiratory involvement, to date too little considered, are worthy of more accurate study. Gastroenteric and cardiac participations are rare. Among the cryofavouring manifestations of the disease, we have pointed out those affecting the liver. In our experience, however, as the immunohistochemical findings suggest, the histological patterns usually considered to be chronic persistent hepatitis are frequently an expression of a lymphoproliferative lymphoma-like process. A noteworthy percentage of lymphoid cells in the bone marrow has been found in 22 type II cryoglobulinemias after either needle puncture or osteomedullary biopsy. The histological pictures often resemble a lymphoproliferative disease. Clinical and laboratory (computerized tomographic scan) researches frequently show a moderate splenomegaly, but in none of the patients was superficial or deep lymph node enlargement found. The authors conclude by considering type II essential cryoimmunoglobulin to be a sign and a marker of a low-grade proliferative disease with rare evolution into overt lymphoma. At the same time, however, cryoimmunoglobulins themselves cause important injuries that affect the prognosis of the syndrome.
The aim of our paper was to investigate the short period of training offered to peritoneal dialysis patients. We focused our attention on the nutritional knowledge of patients who often gather information, and advice from external factors such as their family and the media. We organized group meetings on nutrition for 22 patients and 17 relatives. Questionnaires were completed before and after the education programme. The group meetings improved knowledge on nutrition and highlighted the influence of the media. The important role of the nurse at these meetings has also been recognised.
Every day patients on peritoneal dialysis (PD) must focus on their dialysis schedules and on nutritional and therapeutic prescriptions. Unfortunately, over time all these actions may not follow the initial protocol. In order to evaluate patient's knowledge and approach to dialysis, an alternative educational tool to hospital or home was investigated. The campus was utilised as a place for re-training and detecting and correcting discrepancies in knowledge and the management of techniques. A continuous educational programme involves some re-training of PD patients and this approach helps us to identify whether the treatment management at home is in accordance with the information given at the start of the dialysis, but there was a need to improve on this and to improve patient understanding and compliance. The campus was chosen as an alternative educational process with the aim of involving the patients themselves and their families to achieve additional benefits and long-term success of the therapy. This initiative, outside the hospital and in collaboration with professional figures not affiliated with the dialysis unit, hoped to offer patients new knowledge about: dialysis technique and procedures, prevention of infectious complications, nutrition and physical activity.
Mental anorexia (MA) is the most frequent condition of malnutrition in industrialized countries. Sudden death in MA is not infrequent. Furthermore, the role of nutritional state as an important determinant of myocardial function is known. Cardiovascular function was studied in 9 patients with MA. Blood electrolytes and thyroid function were assessed, basal and dynamic ECG, chest roentgenogram, M-mode echocardiography, ergometric test, cardiac output measurement and, in two cases, magnetic nuclear resonance (MNR) were performed. Our data confirm the ECG changes which were preeminent in the clinical context, as predictors of possible major, life-threatening arrhythmic events. The correct QT interval was normal in all patients. In 4 cases with heart rate less than 40 b/min, Holter ECG showed ventricular and atrial extrasystolic beats. In one case S-A blocks with idioventricular substitutive beats were recorded; the normal performance under maximal strain stands for a normal functional reserve. Blood electrolytes were in the normal range. The hypothalamic-pituitary axis can be considered as a cocausal factor by means of autonomic nervous system modulations. Echocardiography revealed only a mild reduction of ventricular wall thickness. Scattered degenerative myocardial involvement as shown by MNR imaging, could be the anatomical counterpart of a clinically emerging cardiomyopathy with potentially severe arrhythmias.
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OBJECTIVE: In order to investigate HCV associated thrombocytopenia, 6 patients suffering from this disease, in the absence of splenomegaly and other common causes of peripheral platelet destruction, underwent laboratory and scintigraphic tests. RESULTS: Thrombokinetic studies revealed a significant, nearly linear, delayed splenic accumulation with normal or low-normal values for the average platelet life span, low-normal recovery, and depressed platelet production. Megakariopoiesis was normal or slightly increased. HCV infection of the megakariocytes was found in two patients. Autoantibodies and liver disease were also investigated. CONCLUSIONS: A role of immunological mechanisms in HCV associated thrombocytopenia appears to be ruled out. The authors conclude that tests for HCV infection should be included in the evaluation of thrombocytopenia in adults and a possible direct involvement of HCV cannot be excluded.
OBJECTIVE: To evaluate the effect of cyclosporine in the treatment of type II mixed cryoglobulinemia, after the failure of more conventional therapies. METHODS: Two patients with type II mixed cryoglobulinemia associated with chronic HCV infection, purpura, liver disease, and sensitive/motor neuropathy were treated with cyclosporine (2.5 mg/Kg/b.w.), after their failure to respond to treatment with corticosteroid, immunosuppressive drugs, interferon, and plasmapheresis. RESULTS: In both patients an improvement in the clinical manifestations (purpura and peripheral neuropathy), laboratory results (serum transaminases and cryocrit), and liver histology was seen, as well as the disappearance of bone marrow B-cell lymphoproliferation. CONCLUSION: Cyclosporine may be useful in the treatment of type II mixed cryoglobulinemia with prominent autoimmune clinical manifestations, although further studies are needed to better define the selection of patients.
Reactivation of latent JC virus in immunodeficient subjects can lead to a demyelinating disease following lytic oligodendrocyte infection. It is known as idiopathic CD4+ lymphocytopenia and rarely occurs in immunosuppressed patients who are not infected by HIV. We describe a case of persistent idiopathic CD4+ lymphocytopenia in an HIV-negative 65-year-old woman. At autopsy, polymerase chain reaction analysis evidenced JC virus DNA in kidney, brain and liver although there were no signs of progressive multifocal leukoencephalopathy or evidence of oligodendrocyte infection. While her disease was not HIV-induced, it closely resembled AIDS in terms of the nature of the immune derangement and the clinical picture. The case also evidences the reactivation of JC virus infection in non-HIV-related immunosuppression in cerebral and/or extracerebral sites: liver infection seems to be particularly relevant since it has not yet been recognized as a common target of JC virus infection or a source of virus spreading. The absence of any sign of progressive multifocal leukoencephalopathy was remarkable: histological examination failed to disclose demyelination or other progressive multifocal leukoencephalopathy changes, and the search for JC virus DNA with in situ methods also gave negative results. The lack of lytic brain infection in this case would seem to support the hypothesis that the expression of progressive multifocal leukoencephalopathy is directly dependent on the presence of HIV infection.