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Introduction: Erdheim-Chester disease is an extremely rarely occuring condition and thus an optimal treatment is not known. Two new cases have been diagnosed in our centre in 2008 and 2009. Both patients had diabetes insipidus, B symptoms (subfebrile to febrile states) and pain in long bones of lower limbs., Case Studies: Imaging showed high accumulation of fluorodeoxyglucose as well as Tc-pyrophosphate in long bones of lower as well as upper limbs, aortic wall thickening with periaortic fibrosis and perirenal fibrosis. In addition, one of the patients had multiple lesions in the brain. 2-chlorodeoxyadenosine 5 mg/m2 s.c. and cyclophosphamide 150 mg/m2 administered on days 1 to 5 in 28-day cycles were selected for the treatment of both patients. Dexamethasone 24 mg/day for 5 days was added to this treatment in the second patient. Six cycles of the treatment were planned. Both patients were prescribed bisphosphonates--zoledronate and clodronate, respectively. Treatment effect was assessed with PET-CT and MR. Following treatment completion, brain infiltrates were reduced to a small residuum in the first patient who did not anymore complain of leg pain. However, there was no reduction in fluorodeoxyglucose accumulation in bone lesions and thus treatment response was assessed as partial remission. This patient is currently receiving a second line treatment and treatment follow-up is 26 months from the diagnosis. Repeated PET-CTs in the second patient showed a significant reduction in accumulation of fluorodeoxyglucose in all pathological lesions. Febrile states and pain in long bones as well as pathological fatigue ceased after the treatment. Increased CPR and fibrinogen gradually returned to their normal levels. This response is assessed as complete remission. This patient's follow-up is 16 months from the diagnosis., Conclusion: Administration of 2-chlorodeoxyadenosine (5 mg/m2 s.c.) + cyclophosphamide (150 mg/m2 intravenously) and dexamethasone (24 mg/day) led to partial remission in one patient; nearly complete remission of CNS infiltrates but persistent elevation of fluorodeoxyglucose accumulation in bone lesions. Complete remission with a significant reduction in accumulation of fluorodeoxyglucose in all disease lesions with normalization of originally increased inflammatory markers and disappearance of all symptoms of the disease was achieved in the second patient.

Schnitzler syndrome is a rare idiopathic disease characterized by chronic urtica, presence of monoclonal IgM immunoglobuline and further, less common symptoms. This case report describes another case of this disease affecting a male adult born in 1963. The first symptoms, eruptions of non-pruritic urticarial rash, appeared in this patient at the age of 43. In addition, bone pains (mainly tibias) and joint pains (mainly knees) were present. Later on however, severe attacks of fever, chills and shaking together with bone and joint pains were added to during which new urticarial eruptions appeared. Primarily, the man was followed up without any substantial therapeutic results at a department of dermatovenerology, subsequently, due to a finding of monoclonal IgM kappa immunoglobulin (serum concentration 1.9 g/l) he was referred to our department for the reason of gammopathy being a differential diagnosis. On a CT scan hyperostosis in claviculae and pelvic bones was identified. Also on the CT, an increase in cortical thickness was described in the long bones of the lower extremities, where areas of technetium pyrophosphate accumulation were identified on a bone scintigraphy. These areas were found in the chest and sacral regions as well. From the blood exams, the proinflammatory status of the organism was apparent (CRP 35.9 mg/l, erythrocyte sedimentation rate 92 mm/h, leukocytes 12.4 x 10(9)/l). After excluding other differential diagnoses, the patient was diagnosed with Schnitzler syndrome. As regards therapy, we made initial use of the effect of corticoids which abated the symptoms, however, these were causing serious adverse reactions in the form of iatrogenous Cushing's syndrome. The therapy took a turn only after biologic therapy with anakinra (interleukin-1 receptor antagonist) had started, which minimized the Schnitzler symptoms with very good drug tolerance. In the work we measured serum levels of interleukins for disease activity monitoring. The most sensitive were interleukins IL-6 and especially IL-18 the levels of which were the highest at the time of clinical exacerbation of the disease, whereas the levels of IL-1beta and TNF-alpha (tumour necrosis factor) were during all measurements below the limit of detection. Concerning the growing numbers of the reports on successful biological therapy with anakinra and our positive experience, we propose that the therapeutic response to anakinra should be included within the diagnostic criteria of Schnitzler syndrome, which is significant above all in differential diagnosis thereof.

In 2004, diabetes insipidus was the first clinical sign of Erdheim-Chester disease in our patient. Following introduction of substitution therapy with adiuretin, the patient had no further health complaints for four years until 2008 when he gradually developed dysarthria and, consequently, movement disorder in the form of mild right hemiparesis. The first CNS CT scan (2004) did not reveal any pathology. The first pathological MRI of the brain in 2006 - thickening of pituitary stalk by pathological infiltration to 4-5 mm. During the following year, further infiltrates were detected in the CNS. The number and size of CNS infiltrates increased gradually on MRIs performed repeatedly up to 2008. Erdheim-Chester disease has become suspected based on PET-CT examination at the end of 2008. CT showed irregular structure of the skeleton with noticeable sclerotic foci in otherwise osteoporotic bone structure; changes were the most evident in the long bones of lower limbs, in the pelvic bones, skull and arms, while only one vertebra was affected from within the entire spine. Finding ofthickened aortic wall (up to 8 mm) as another pathological circumstance was consistent with the Erdheim-Chester disease-associated changes described as coated aorta. CT scan revealed clear fibrotic changes in the area of retroperitoneum. Applied fluorodeoxyglucose has accumulated in the bone foci described on CTscans as well as in the thickened wall ofthe thoracic and abdominal aorta (SUV 3.6). Tc-pyrophosphonate skeleton scintigraphy showed the same bone foci as PET-CT. Full body MRI showed pathological signal from the bone marrow of the above mentioned locations, particularly during STIR imagining, where there was clear abnormal signal corresponding to accumulated histiocytes, the higher signal of which was well-differentiated from the normal bone marrow. Measurement of bone mineral density with DEXA confirmed reduced density in lumbar vertebrae to the average value of - 2.7 SD (the lowest value was -3.1SD). The disease is associated with elevated inflammatory parameters: leucocytosis, thrombocytosis, elevated CRP and fibrinogen levels. Diagnosis was verified following histological assessment ofiliac bone marrow, where focal infiltrations with foamy histiocytes of typical immunophenotype (CD68+, CD1a-, S100-) were confirmed. Treatment was initiated with chemotherapy consisting of 2g/m2 of cyclophosphamide on day 1 and 200 mg/m2 of etoposide IV infusion on days 1-3, and followed by administration of 5 microg/kg of G-CSF and collection of haematopoietic peripheral blood stem cells (PBSC). PBSC collection was followed by 5-day administration of 5 mg/m2/day of 2-chlorodeoxyadenosine (Litac) administered to the patient at monthly intervals.

Schnitzler syndrome is a rare disease characterised by chronic urticaria and the presence of monoclonal IgM immunoglobulin, and by other symptoms. We report ourexperience with 14-year treatment of a patient. The first medical examination in our workplace was at the beginning of 1995 and the patient was diagnosed with the disease in 1996 (at the age of 52). Antihistaminics, the first medication used to relieve the symptoms of urticaria, had no subjective or objective effect. After the detection of osteolytic-osteosclerotic changes in the pelvic region, in areas with intense pain, we started treatment with pamidronate (90 mg at 28-day intervals), and the pain disappeared completely within 3 months of application of the drug. When the bisphosphonate therapy was interrupted, the pain recurred and receded completely after renewal of bisphosphonate administration. After the diagnosis, we gave the patient high doses of dexametazone (40 mg day 1-4, 10-13 and 20-23, at 28-day cycles). However, the therapy suppressed urticaria only on the days dexametasone was administered and the effect did not last when the drug was discontinued. Therefore we moved to continuous daily doses of prednisone (10-30 mg, depending on the intensity of problems), which was the only therapy with a long-term effect which was relatively well tolerated at the same time. Based on the excellent effect of 2-chlordeoxyadenosine in Waldenström's macroglobulinaemia, three cycles of this therapy were administered to the patient in 1996 (0.1 mg/kg/day, 7 days, at 28-day intervals). After the first infusion, urticarious lesions disappeared, but the positive effect on skin eruptions was limited in time and lasted only 14 days after the last infusion, i.e. the medication proved ineffective from a long-term point of view. The first improvement lasting for a longer period of time (partial remission) was achieved by regular application of interferon alpha (3 QU 3 times a week). However, adverse effects of interferon alpha prevailed after two years and the therapy was discontinued. Similarly phototherapy using the PUVA method resulted in partial regression of urticarious symptoms. Subsequently tested cyclosporine A (5 mg/kg/day) brought no benefit. Thalidomide (100 mg in the evening) administered on a continuous basis relieved pruritus and improved sleep disturbed by pruritus. However, adverse effects prevailed after 4 months and the therapy had to be discontinued, too. In 2005, we were hoping to achieve positive results with the most effective treatment for multiple myeloma of the time, a combination of bortezomib (1.3 mg/m2 i.v. on day 1, 4, 8 and 11, thalidomide 100 mg daily and dexametazon 20 mg p.o. on days 1-4 and 8-11 in 21-day cycles --VTD). A total of 4 complete cycles and 4 cycles with bortezomib reduced by 50% were applied. Urticarious eruptions were reduced by at least 50% in the course of the therapy, and also the concentration of monoclonal immunoglobin decreased temporarily by more than 50%. However, after the therapy was discontinued, the symptoms returned with their original intensity, which means that VTD regime did not provide a long-term therapeutic response. In 2007, we started the anakinra (Kineret) therapy. Skin symptoms disappeared after the first injection and a dose of 100 mg/ day has kept the patient free of skin symptoms for 12 months by now. Also the CRP value which had been constantly high returned to normal, and haemoglobin values increased to achieve physiological range. In the course of 14 years, we confirmed partial therapeutic effect of glucocorticoids administered on a continuous basis, as well as a partial therapeutic effect of interferon alpha, thalidomide and PUVA, but all the therapies had to be discontinued due to adverse effects. A major turn, i.e. the complete disappearance of skin symptoms and normalisation of CRP and haemoglobin values, only came with anakinra which has become the drug of the first choice for the above syndrome.

Unlabelled: The aim of this study was investigate the appearance of multiple myeloma on flurorine--18 fluorodeoxyglucose positron emission tomography (FDG-PET). Furthermore the accuracy of FDG-PET in detecting myeloma lesions and its influence on patient's management were evaluated., Methods: Altogether 50 patients, 13 patients with newly diagnosed multiple myeloma with negative radiographs, 4 patients with solitary plasmocytoma, 27 patients in remission with suspected relapse and 6 patients with monoclonal gammopathy of unknown significance (MGUS) with suspicion for multiple myeloma or other malignancy underwent FDG-PET examination. The results of routinely performed radiographs, and MR or CT imaging modalities as well as the clinical course were used for verification of the FDG-PET results., Results: Focally increased tracer uptake was observed in 3 (23 %) of newly diagnosed myeloma patients with negative radiographs and was verified with CT or MR with followed indication for therapy. The FDG-PET was negative in two cases of newly diagnosed multiple myeloma with negative radiographs, no focal infiltration on MR imagination, but with anemia, high monoclonal imunoglobulin and bone marrow infiltration, which was indication for therapy. In all other cases FDG-PET negativity in asymptomatic myeloma had good prognostic significance; these patients are without progression after with a median follow up 14 (7-20) months. Focally increased tracer uptake was found in 5 of the 27 patients in remission. In 4 cases of them it was due to multiple myeloma relapse, in one case due to ovarial carcinoma. Only in 1 patient the PET-FDP failed to recognize extraosseal progression on the scull. 21 patients had true negative FDG-PET imagination, in 1 case disease relapsed 12 months after FDG-PET examination; the other 20 patients are still without progress of this disease with median follow up 15 (7-20) months. FDG-PET was positive in 2 from the 6 patients with MGUS. In one of them carcinoma of thyreoidea was detected, in second the FDG-PET activity was localized in gut, tumor was verified with CT and colonoscopy., Conclusion: In conclusion, FDG PET might contribute to initial staging of radiographs negative multiple myeloma and might be useful for follow up of patients in remission, especially in consecratory multiple myeloma, or in patients with large plasmocelular tumor (> 5 cm) after concomitant radiochemotherapy.

The authors present a case of a 37 year old male (proband) with a 13 year history of progressive sight impairment leading to blindness and a 4 year history of a mild hypertension. He was incidentally found to have large adrenal tumors after an ultrasound kidney examination. The tumors were confirmed with CT scan and magnetic resonance imaging. A bilateral pheochromocytoma was biochemically demonstrated and successfully removed. The eye diagnosis of angiomatosis retinae von Hippel-Lindau was ascertained after a search of the patient files in other medical departments, which led to a family screening. Proband's brother, having hypertension and a history of a cerebellar astrocytoma operation, was also diagnosed with CT scan to have a bilateral pheochromocytoma. Unfortunately, at the same time he was found to have a large irremovable neuroendocrine pancreatic carcinoma, which caused complications and his eventual death. Both proband and his brother were affected by the same CGG(Arg167)->CAG(Gln) mutation in the exon 3 of the VHL gene. Other living and examined family members were not affected, which was confirmed by negative genetic testing. One year after the pheochromocytoma operation, proband was diagnosed to have a retroperitoneal tumor left to the aorta, clinically silent, with slightly and non-constantly elevated urine norepinephrine and normetanephrine. Metaiodobenzylguanidine scintigraphy showed that it was a paraganglioma. The old CT and magnetic resonance picture review demonstrated that the tumor had already been present at the time of the operation. It was surgically removed and histologically verified. It is a pity that proband had not been sent by his ophthalmologist for an endocrine examination when the eye diagnosis was determined. Affection of the family would have been discovered earlier, and proband's brother might have possibly been saved.

Diabetes is a frequent metabolic disease which affects also the digestive tract. Functional disorders of the latter have a negative impact on the stabilization of diabetes. An important factor as regards motility is the blood sugar level. Subjective complaints of the patients frequently do not correlate with the objectively assessed functional state of the digestive tract. The authors remind of contemporary available diagnostic and therapeutic possibilities.

The authors examined retrospectively the frequency of NSAID use by patients with gastroduodenal ulcer without HP. The objective was to evaluate whether the use of NSAID is the predominating factor in the development of ulceration in subjects with HP negative ulceration. The group comprised 525 patients with confirmed presence of H, incl. 52 who used NSAID. From the results ensues that the incidence of ulcerous lesions with HP negativity and NSAID medication is significantly higher than in subjects who use NSAID and suffer from a confirmed HP infection.

24-hour ph-metry of the oesophagus is a standard examination method in patients with reflux complaints. For objective evaluation of the record the following points are most important: 1. calibration of the instrument, 2. precise placement of the pH-probe, preferably under manometric control, 3. elimination of medication (inhibitors of proton pump, H-2 blockers, antacids, prokinetic preparations), 4. cooperation of the patient. A 24-hour record makes it possible to analyze in detail the type of reflux, and when using available software, also its quantity.

Twenty-one patients with a medium severe form of acute pancreatitis were divided into two groups. Eleven were treated with octreotide, 200 micrograms s.c. every 8 hours, combined with standard conservative treatment, 10 patients had the same treatment but without octreotide. In the octreotide-treated group the level of free oxygen radicals and important antioxidants--vitamins A, E and C--was assessed. In the octreotide-treated group the authors found after five days normal levels of originally elevated free oxygen radicals and a significant increase of the vitamin C level. As compared with the control group, in the octreotide group the period of hospitalization was significantly shorter (9.8 +/- 3.2 vs. 13.7 +/- 4.7). The number of complications was not affected.

Eradication regimes with the blocking agent of the proton pump and without it do not influence the activity of cell division after treatment of Helicobacter pylori (H.p.) when using cytoflowmetric evaluation. The non-significant difference in proliferation activity of the gastric mucosa after treatment of H.p. can be also a sign of more rapid repair of the gastric mucosa after elimination of the inflammatory elements.

Colonization of the gastric mucosa with Helicobacter pylori H.p. reduces the vitamin C concentration of gastric juice. Eradication of H.p. within four weeks after completed treatment does not exert a significant effect on changes in the concentration of vitamins A, E and C in gastric juice or serum. Despite this after eradication a rising trend of vitamin E in gastric juice was recorded. Substitution of vitamin C and E in gastritis associated with colonization with H.p. has a favourable effect and may reduce the risk of malignization.

Extremely effective drugs used in the therapy of diseases etiologically associated with excessive HCl secretion by the gastric mucosa include proton pump blockers. Their obvious advantage, as compared with commonly used drugs, is that they cause a marked drop of HCl production by the gastric mucosa regardless of the type of stimulus, i.e. whether parietal cell receptors are influenced. Their therapeutic action is extremely effective, while side-effects are minimal. The publication gives the basic indications of administration of proton pump blockers, compares their therapeutic efficiency with H2 blockers and draws attention to possible pitfalls of therapy.

Impaired motility of the oesophagus is relatively frequent in patients with functional blocks of the cervical spine. The patient does not always realize oesophageal dysmotility. Auricular acupuncture focused on reflex relations seems to be a useful therapeutic procedure.

The authors give an account of functional disorders of the digestive tract. They deal briefly with possibilities of their medicamentous treatment with regard to the pharmacological action of prokinetics. Finally they review indications for Prepulside.

Atenolol and bisoprolol, two beta-1-selective blockers, were compared in patients with mild and medium severe hypertension. Forty patients with a diastolic blood pressure of 95-115 mm Hg were two weeks after discontinuation of treatment divided at random into two groups treated with 50 mg atenolol and 5 mg bisoprolol. If the blood pressure was not affected by this dosage, the dose was raised to 100 mg atenolol and 10 mg bisoprolol. The blood pressure readings, heart rate, side effects of the drugs, laboratory and echocardiographic values were followed up for a period of three months. The authors recorded a marked decline of pressure and heart rate after the first week of therapy, the pressure readings in an upright position did not differ statistically from values in a sitting position, orthostatic hypotension was not recorded. The rate of success of bisoprolol was 85%, of atenolol 75%. During treatment no serious side-effects were observed. The biochemical parameters and echocardiographic values were not altered significantly by treatment. Atenolol and bisoprolol are two effective beta-1-selective blockers with a comparable effect in the treatment of mild and medium severe hypertension. They are useful in monotherapy as well as in combination with other antihypertensive drugs. In hypertension associated with diabetes mellitus, incipient bronchopulmonary disease and pregnancy they are also indicated. They are well tolerated also by elderly patients, an advantage of bisoprolol may be that it has a smaller impact on pressure and heart rate during the night.

A total of nine patients with diabetes type I and II and with claudications due to macro- and microangiopathies of the lower extremities took sulodexide (Vessel Due F) by the i.m. route for a period of 20 days and then as capsules for 90 days. In all a favourable therapeutic effect was recorded, in particular as regards delay of claudications. The authors recorded lower blood sugar values, lower levels of cholesterol, triglycerides, fibrinogen and plasma viscosity. In the authors' view sulodexide is an important advance in the treatment of diabetics with claudications.