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Introduction. Erdheim-Chester disease (ECD) is a rare non-Langerhans-cell histiocytosis. About 500 cases are published so far. It is multisystemic disease characterised by bilateral symmetric long bones sclerosis. Main histopathological finding is accumulation of big foamy histiocytes, immunohistochemically positive to CD68, and negative to S-100 and CD1a. There are no guidelines that reliably identify population that requires therapy, but symptomatic ECD, organ failure and central nervous system involvement re-quire treatment. Case report. We described a patient with a multisystemic form of ECD affecting long bones, the hypophysis, abdomen, and the peripheral nerves. Five years after initial symptoms ECD was suspected. Prednison was initiated, 60 mg once a day. After obtaining the diagnosis of ECD, interferon alpha 2A was introduced, but soon after stopped due to severe side effects. Considering that histiocytes were positive to platelet derived growth factor receptor alpha (PDGFR alpha) imatinib mesylate was started, but after two months stopped due to no clinical and radiological improvement. The disease was worsening and the patient died. Conclusion. We described the patient with intraperitoneal form of ECD, without cardiac and pulmonary involvement. There are several important issues: the diagnosis of ECD could be difficult to make, three treatment regimens were included and the patient died nine years after the initial symptoms due to indolent course of the disease and unsuccessful treatment.

Sister Mary Joseph's nodule is the eponym for metastatic involvement of the umbilicus. This less common entity is the sign of disseminated malignant disease, mainly of digestive and gynecologic origin, and is associated with a poor prognosis. A case of Sister Mary Joseph's nodule in a 76-year-old woman in whom the umbilical metastasis was the first sign of malignant disease in presented. The diagnosis of metastatic adenocarcinoma was established by fine needle aspiration cytology of the umbilical nodule. Radiological and ultrasonographic investigation disclosed carcinoma of the gallbladder with pancreas, stomach, and colon invasion as well as peritoneal dissemination. The diagnosis was confirmed by exploratory laparatomy and histological examination of the excised umbilical nodule.

There are 7 risk factors for NSW in gastroenterological patients, but they are not of the same importance--two primary and five secondary risk factors can be differentiated. Preventive nutritional therapy is indicated only in patients having both primary risk factors or in those presenting with one primary risk factor combined with three secondary risk factors at least.

Combining BMI with MAMC is sufficient for the assessment of the nutritional status of gastroenterological patients on admission, as well as for differentiation between severely malnourished and moderately malnourished patients.

Background / Aims. Risk factors for malnutrition of patients during hospitalization have not been precisely determined. The aim of the study was to determine these factors in hospitalized gastroenterological patients. Methods. Nutritional status (NS) of 650 gastroenterological patients was assessed on admission and at discharge by the six parameters: unintentional weight loss, lymphocyte counts, serum albumin concentration, body mass index, triceps skinfold thickness and midupper arm muscle circumference. The influence on NS at discharge was tested for ten factors: gender, age, affected organ, the nature, severity and complications of the disease, the lenght of hospitalization, mobility worsening during hospitalization, Karnofsky score and NS on admission. Primary and secondary risk factors were defined among the factors influencing significantly the malnutrition. Results. Seven factors were found to be the independent predictors for malnutrition in hospitalized gastroenterological patients. NS on admission was considered as primary risk factor (Forwald: Wald multivariate logistic regression analysis, p<0.001 for five applied assessment parameters). The other factors, obtained in the evaluation according to 1-3 parameters, were considered as secondary risk factors: severe disease activity, malignancy, the existence of complications, male gender, hospitalization >14 days, and mobility worsening during the hospitalization (Forwald:Wald multivariate logistic regression analysis, p from 0.001 to 0.027). Conclusion. There are seven risk factors for malnutrition among gastroenterological patients during hospitalization. Timely nutritional support in these patients can prevent the development of intrahospital malnutrition and its negative influence on clinical outcome.

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Introduction. Hereditary hemorrhagic telangiectasia and inherited thrombophilia are genetic disorders with quite opposite clinical manifestation. The main characteristic for hereditary hemorrhagic telangiectasia is recurrent bleeding, while the main characteristic for hereditary thrombophilia is thrombosis. The association between hereditary hemorrhagic telangiectasia and inherited thrombophilia in the same patient is rare. Case report. We presented a 32-year-old female with recurrent gastrointestinal hemorrhage and epistaxes, during a 9-year period. Hereditary hemorrhagic telangiectasia was established according to "Curaçao" criteria. Three of four criteria have been present: spontaneous recurrent epistaxis, multiple telangiectasias (nose) and visceral lesions (gastric angiodysplasias, jejunal telangiectasias, arterio-venous jejunal fistula). Pulmonary thromboembolism was the first manifestation of thrombophilia; the diagnosis was confirmed by genetic testing. Therapy of hemorrhage with tranexamic acid (anti-fibrinolytic agent; its use increases risk of thrombosis) was unsuccessful. Remission was achieved by thalidomide. The initial therapy for pulmonary thromboembolism included aspirin (that have an increased risk of bleeding), but aspirin had to be discontinued because of massive hematemesis. Unfortunately, a year later, anticoagulant therapy combined with the proton pump inhibitors, were introduced, because of a new thrombosis. One month after, the patient was still on this therapy, without new episodes of bleeding and thromboembolic events. Conclusion. Hereditary hemorrhagic telangiectasia and inherited thrombophilia could be unrecognized for years, partly due to the lower degree of clinical suspicion. Early diagnosis and the appropriate choice of therapy are essential for reducing serious consequences and to improve quality of life.