New precision measurements of the 235U(n,γ) cross section.

The neutron capture cross section of (235)U was measured for the neutron incident energy region between 4 eV and 1 MeV at the DANCE facility at the Los Alamos Neutron Science Center with an unprecedented accuracy of 2-3% at 1 keV. The new methodology combined three independent measurements. In the main experiment, a thick actinide sample was used to determine neutron capture and neutron-induced fission rates simultaneously. In the second measurement, a fission tagging detector was used with a thin actinide sample and detailed characteristics of the prompt-fission gamma rays were obtained. In the third measurement, the neutron scattering background was characterized using a sample of (208)Pb. The relative capture cross section was obtained from the experiment with the thick (235)U sample using a ratio method after the subtraction of the fission and neutron scattering backgrounds. Our result indicates errors that are as large as 30% in the 0.5-2.5 keV region, in the current knowledge of neutron capture as embodied in major nuclear data evaluations. Future modifications of these databases using the improved precision data given herein will have significant impacts in neutronics calculations for a variety of nuclear technologies.

Abrupt change in radiation-width distribution for 147Sm neutron resonances.

We obtained the total radiation widths of s-wave resonances through an R-matrix analysis of (147)Sm(n,γ) cross sections. Distributions of these widths differ markedly for resonances below and above E(n)=300 eV, which is in stark contrast to long-established theory. We show that this change, as well as a similar change in the neutron-width distribution reported previously, is reflected in abrupt increases in both the average (147)Sm(n,γ) cross section and fluctuations about the average near 300 eV. Such effects could have important consequences for applications such as nuclear astrophysics and nuclear criticality safety.

Tumour necrosis factor alpha blockade in treatment resistant pigmented villonodular synovitis.

BACKGROUND: Pigmented villonodular synovitis (PVNS) is considered to be a neoplastic-like disorder of the synovium histologically characterised by villonodular hyperplasia, resulting in dense fibrosis and haemosiderin deposition. The pathogenesis of the disease is still unknown. CASE REPORT: A patient presented with severe treatment resistant PVNS of the right knee joint. Several conventional treatment regimens, including open surgical synovectomy and intra-articular injections of yttrium-90 ((90)Y) failed to control the disease. After finding marked tumour necrosis factor alpha (TNF alpha) expression in arthroscopic synovial tissue samples, treatment with an anti-TNF alpha monoclonal antibody (infliximab) at a dose of 5 mg/kg was started. Additional courses with the same dose given 2, 6, 14, and 20 weeks later, and bimonthly thereafter up to 54 weeks, controlled the signs and symptoms. Immunohistological analysis at follow up identified a marked reduction in macrophage numbers and TNF alpha expression in the synovium. DISCUSSION: This is probably the first case which describes treatment with TNF alpha blockade of PVNS in a patient who is refractory to conventional treatment. It provides the rationale for larger controlled studies to elucidate further the efficacy of TNFalpha blockade treatment in refractory PVNS.

Fatigue in rheumatoid arthritis: the role of self-efficacy and problematic social support.

OBJECTIVE: To examine the relationship of fatigue in people with rheumatoid arthritis (RA) with self-efficacy, positive and problematic aspects of social support, and demographic and disease-related variables. METHOD: Out-patients with at least 5 yr RA were studied. Fatigue was measured with a visual analogue scale. Other variables included were: positive social support [Social Support List-Interactions (SSL12-I)] and problematic social support; self-efficacy towards coping with RA and towards mobilizing support; health status (Dutch-AIMS2); and laboratory tests: erythrocyte sedimentation rate (ESR), haemoglobin (Hb) and rheumatoid factor (RF); and disease duration. RESULTS: A total of 229 out-patients were included. Fatigue correlated with all scales of the Dutch-AIMS2: with pain, physical function and affect (P < 0.001). There was no significant correlation with social support, but there was a highly significant correlation of fatigue with problematic social support (P < 0.001). Both forms of self-efficacy correlated strongly with fatigue: patients with high self-efficacy expectations towards coping with RA, and towards mobilizing the social network (P < 0.001), had less fatigue. In the regression analysis to explain the variation in fatigue, only pain, self-efficacy expectations towards coping with RA, and towards asking for help and problematic social support remained significant. CONCLUSIONS: Fatigue can to a large extent (37%) be explained by pain, self-efficacy towards coping with RA, and towards asking for help and problematic social support. It is known that self-efficacy can be enhanced by self-management courses and it may thus be possible to improve fatigue.

[Clinical thinking and decision making in clinical practice. A patient with systemic lupus erythematosus and behavioral changes]. / Klinisch denken en beslissen in de praktijk. Een patiënt met lupus erythematodes disseminatus en gedragsveranderingen.

A 48-year-old man with systemic lupus erythematosus (SLE) was admitted with fever, headache and mental change. On admission he was treated with daily doses of cyclophosphamide 100 mg and prednisone 7.5 mg. Orientation and attention were diminished and visual examination revealed right-sided homonymous hemianopia. MRI of the brain showed a ring-enhanced, space occupying lesion in the left occipital lobe. An infectious disease cause was considered, because disease activity parameters of SLE were all negative (anti-dsDNA and the complement profile). The anti-Toxoplasma titre was elevated in cerebrospinal fluid (57 IU/ml) and in serum (1140 IU/ml), both IgG. A tentative diagnosis of Toxoplasma encephalitis was made and a trial anti-toxoplasmosis treatment with pyrimethamine and sulfadiazine was administered. Two weeks later the patient was normal at neurological examination and his brain MRI had greatly improved. The anti-Toxoplasma titre had dropped significantly.

[The remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome]. / Het 'remitting seronegative symmetrical synovitis with pitting edema' (RS3PE)-syndroom.

RS3PE syndrome was diagnosed in a 83-year-old man with seronegative symmetrical polysynovitis with pitting oedema, which presented after a vehement acute beginning a remarkably favourable course. RS3PE syndrome, recently described, is characterized by a transient seronegative symmetrical synovitis with pitting oedema. The syndrome appears mainly in the aged. After a frequently acute crippling beginning, the prognosis is good. Plain and brief treatment will suffice.

[Hypomagnesemia and chondrocalcinosis]. / Hypomagnesiëmie en chondrocalcinosis.

A female patient aged 45 years is described with a rare form of chondrocalcinosis caused by hypomagnesaemia due to excessive renal loss of magnesium. The patient also had impaired renal conservation of potassium leading to a hypokalemia. She most probably had an idiopathic renal tubular dysfunction. Magnesium supplementation prevented further symptoms. Therefore in young patients with chondrocalcinosis it can be of therapeutic importance to search for an underlying treatable metabolic disorder.

Adult-onset Still's disease.

Adult onset Still's disease seems to be the adult form of Still's disease in children. The key symptoms of the disease are high spiking fever, arthritis and a macular or maculopapular, salmon-pink evanescent rash, almost always accompanied by neutrophilic leukocytosis and frequently by sore throat, intense myalgias, lymphadenopathy, splenomegaly and signs of serositis. Tests for IgM rheumatoid factor and antinuclear antibody are characteristically negative. With respect to haematologic abnormalities, the disease may give rise to several problems. First, there is a neutrophilic leukocytosis, which currently is unexplained, and often a normocytic normochromic anaemia, that may be profound. The anaemia has the characteristics of anaemia of chronic inflammatory disease. Both abnormalities disappear after effective treatment of the disease or at spontaneous remission. Secondly, there might be a problem to differentiate AOSD from malignant haematological disorders, including malignant lymphoma and leukaemia, especially when the picture is dominated by lymphadenopathy, splenomegaly, fever and leukocytosis. Although in rare cases the differential diagnosis is extremely difficult, diagnosis can mostly be made or excluded by peripheral blood smear staining, bone marrow biopsies and occasionally lymph node biopsy. Finally, like the juvenile counterpart, AOSD is occasionally complicated by sometimes life-threatening diffuse intravascular coagulation. Factors that might be important in the development of this complication include severe disease activity, liver abnormalities and particular drugs including salicylates, other NSAIDs and some slow-acting antirheumatic drugs. Prompt therapy, including withdrawal of the drug, corticosteroids and sometimes anticoagulant therapy have been successfully applied to most patients.

Adult onset Still's disease and viral infections.

Several micro-organisms, especially viruses, have been associated with juvenile and adult onset Still's disease. In the present study a search for probable triggering viral infections in five consecutive patients with early, active adult onset Still's disease has been made. In one patient echovirus 7 was identified as a probable triggering agent. Evidence of infection with this virus was acquired by virus cultures and serological tests. In two patients the illness was probably initiated by a rubella reinfection. Both had initially high stable monospecific IgG antibody titres but no IgM antibodies to this virus. In the remaining two cases no particular triggering viral infection could be designated. Evidence of a viral infection was thus found in three of these five patients. Adult onset Still's disease may represent a reaction pattern to certain infections.

Adult-onset Still's disease; clinical and laboratory features, treatment and progress of 45 cases.

The clinical and laboratory features, treatment, prognosis, complications and disability of 45 patients with adult-onset Still's disease were studied. Sixty per cent of the patients were female. Median age at onset was 25 years and median observation period after onset was 41 months. For 28 patients detailed data were available of the first month of illness. In only 43 per cent of these did the disease present with the classical triad of fever, arthritis and rash, although in the whole group eventually, fever with temperatures of 40 degrees C or more occurred in 84 per cent, arthritis in 98 per cent, and the typical rash in 82 per cent. In 32 per cent of the patients with rash, this was pruritic. Other features seen frequently were: lymphadenopathy (71 per cent), splenomegaly (36 per cent), pleuritis and/or pneumonitis (31 per cent), pericarditis (22 per cent), leucocytosis (98 per cent) and hepatic abnormalities (84 per cent). The high spiking fever was reduced to normal in six of 21 cases (29 per cent) by aspirin, in 19 of 27 cases (70 per cent) by indomethacin and in six of seven cases (86 per cent) by naproxen. In 16 of 21 cases (76 per cent) glucocorticoids reduced the systemic and/or joint symptoms. In three patients who remained febrile on glucocorticoids, indomethacin reduced temperature to normal. Eight patients whose joint disease improved on steroids later developed severe joint destructions. Thirteen patients received one or several slow-acting antirheumatic drugs. Only in eight of 18 trials (44 per cent) with one of these drugs did symptoms and signs improve. Fifty-one per cent of the 45 patients had self-limiting disease and 49 per cent had persistent disease with continuous activity for at least one year. At the time of evaluation 47 per cent of the 45 patients were in remission without medication, 33 per cent were in remission while on medication and 20 per cent had active disease. Three life-threatening complications occurred: two patients developed signs of cardiac tamponade and one almost died from diffuse intravascular coagulation. Disability was determined primarily by the course of the arthritis. At the time of review 43 per cent of the 45 patients had no joint destruction (Group 1), 24 per cent had destruction of at least one joint, but had no evidence of disease of the root joints (Group 2) and 33 per cent had destruction in at least one root joint (Group 3).(ABSTRACT TRUNCATED AT 400 WORDS)

Adult-onset Still's disease. Disease course and HLA associations.

Disease course and HLA antigens were determined in 29 patients with definite and 13 patients with probable adult-onset Still's disease (AOSD). Twenty-six patients had persistent disease with continuous disease activity for at least 1 year. Radiographic examination revealed evidence of joint destruction in 26 patients. In 15 patients, at least 1 root joint was impaired. The frequency of DR4 was increased in the total group of patients (35%, P = 0.03) and in the 29 patients with definite AOSD (41%, P = 0.02) compared with the frequency in normal controls (20%). None of the patients was positive for DR1, and only 1 was positive for Bw35. The frequency of DRw6 was increased in the patients with involvement of at least 1 root joint (40%, P = 0.03) compared with the patients without involvement of these joints (11%).

Adult onset Still's disease in the elderly: a report of two cases.

Two patients with the first symptoms of adult onset Still's disease in their 7th decade are reported. In both cases their advanced age was a main reason for a prolonged delay in diagnosis. Besides the characteristic fever pattern, rash and arthritis, our patients showed other typical features of Still's disease such as splenomegaly, pleuritis, pneumonitis, carpal ankylosis and leukocytosis. In addition both showed marked hepatic abnormalities which seemed to be due to the disease itself in one case and at least in part related to exposure to phenylbutazone and diclofenac in the other.

Adult-onset Still's disease complicated by hypercalcaemia: possible relationship with rapidly destructive polyarthritis.

A 34-year-old man with adult-onset Still's disease developed a striking hypercalcaemia during a rapidly destructive polyarthritis with extensive osteoporosis. The hypercalcaemia seemed to be primarily caused by inflammation-induced bone resorption. On prednisone the polyarthritis went into remission and the plasma calcium levels became normal. Other remarkable features in this case were a subluxation of the atlantoaxial joint, a brain-stem haemorrhage, transient hyperuricaemia and hyperuricosuria, and urolithiasis.