Behçet's disease in Moroccan children: a report of 12 cases

We report 12 cases of Beh?et's disease (BD) in children. The mean age of symptom onset was 12.4 years. Four patients (33.3%) had a past familial history of BD. Clinical manifestations were: oral aphtosis (n = 12), genital aphtosis (n = 9), ocular involvement (n = 9), neuro-Beh?et (n = 6), venous thrombosis (n = 4), articular involvement (n = 3), and entero-Beh?et (n = 1). All patients but one were initially treated with steroids; three cases with ocular involvement were treated with chlorambucil; and three other cases of neuro-Beh?et were treated with cyclophosphamide. After a mean follow-up of 4 years, four patients with neurological involvement developed steroid-dependence with recurrence of symptoms. Four patients had optic atrophy with blindness.     

Airborne pollen calendar of Portugal: a 15-year survey (2002–2017)

IntroductionData about the occurrence of airborne pollen enables the creation of pollen calendars with an approximation of flowering periods for the most common allergenic plant species in a specific area. The aim of this work is to provide pollen calendar for each of the seven monitoring regions of Portugal based on 15 years of airborne sampling, in order to chart the seasonal behaviour of the main allergenic pollen types.Material and methodsAirborne pollen monitoring (2002–2017) was carried out by the Portuguese Aerobiology Network (RPA), using Hirst-type volumetric spore traps, following well-established guidelines.ResultsA total of 14 airborne pollen types were recorded at RPA monitoring stations, of which 64.2% belong to trees, 28.5% to herbs and 7.1% to weeds. The airborne pollen spectrum is dominated by important allergenic pollen types such as Poaceae, Quercus spp., Urticaceae and Cupressaceae. The average pollen index was 42.557 in mainland Portugal and 3.818 in the Islands. There was an increased trend in the airborne pollen levels over the years, namely in Coimbra, Évora and Porto, compared to the remaining regions.ConclusionThis report provides accessible information about the main allergenic airborne pollen types occurring in the course of the year. The pollen calendars charted for each Portuguese region showed that the occurrence of most allergenic taxa was centred from March to July. Pollen peak concentrations were detected earlier in the Centre and Lisbon and Tagus Valley regions, and later in the remaining regions.     

Polyarteritis nodosa in patients with Familial Mediterranean Fever (FMF): a concomitant disease or a feature of FMF?

BACKGROUND: Familial Mediterranean Fever (FMF) is caused by mutations in the gene encoding pyrin and is characterized by self-limited, recurrent attacks of fever and serositis. Vasculitis has been increasingly reported in FMF. A study evaluating the prognosis in FMF and polyarteritis nodosa (PAN) patients has not been reported previously. OBJECTIVES: To determine the special characteristics and the prognosis of PAN in FMF patients. METHODS: A questionnaire was used for the present survey. The setting was 7 referral centers from Turkey and Israel. Seventeen patients who were diagnosed with FMF and who developed PAN were included. PAN was diagnosed in those who met the Chapel Hill consensus criteria for microscopic polyarteritis or classic PAN. The clinical features of these 17 patients and the outcomes of their vasculitis were analyzed. RESULTS: The age at diagnosis of PAN in these FMF patients ranged from 3.5 to 37 years. All patients had constitutional symptoms, elevated acute phase reactants, and myalgia at the time PAN was diagnosed. The diagnosis of PAN was confirmed by renal angiography in 8 patients, by renal biopsy in 6 patients, and by muscle and/or nodule biopsies in 6 patients. A number of patients had definite features of both classic PAN and microscopic polyarteritis. CONCLUSIONS: When compared with other PAN patients, those with FMF tended to have a younger age at PAN onset, more frequent perirenal hematomas, and an overall better prognosis. The cases with overlapping features of microscopic and classic PAN pose a problem for the current classification of vasculitis. We suggest that the clinical representation of PAN in FMF patients has certain characteristics and may be a feature of FMF per se.     

Pyquiton in the treatment of paragonimiasis szechuanensis: a report of 18 cases.

18 cases of paragonimiasis szechuanensis (P. skrjabini) were treated with pyquitonin a dosage of 25 mg/kg tid for 2 successive days, in 1981-1982 and were followed-up for 3 months in 2 cases and for 6 months in 16 cases post-treatment. 4 patients of     

Report on trends of incidence (1970–2002) of and mortality (1952–2002) from cancer in Germany

Purpose The paper presents the statistical analysis of current and past trends of cancer mortality rates in Germany in terms of annual percent change, overall and for the major sites, and contrasts them with trends in incidence of the Cancer Registry of the Saarland, the only registry in this country with long-term completeness. It addresses also the issue of a cross-over of cancer mortality and mortality from cardiovascular diseases (CVD) in the near future, as suggested by various authors.Material and methods Analyses are based on the mortality data of the official mortality statistics as published by the Federal Statistical Office and reported annually to the WHO, and the regularly reported incidence data of the Cancer Registry of the Saarland. The data was age-standardised and analysed by piecewise regression using a freely available dedicated software package.Results The report shows a downward trend of mortality rates for all cancers combined based on declining rates for many individual sites with only few exceptions affecting mainly females (e.g. lung cancer). Recently, the long-term increase of cancer incidence also flattened out with rather heterogeneous underlying site-specific trends increasing for some sites (e.g. cancers of the intestine, breast, prostate, or some lymphoma) and decreasing for others (e.g. cancers of the stomach, gall bladder in females, larynx, and lung in males). A crossover of cancer mortality and mortality from CVD might occur—if at all—after 2,020 in males and 2,030 in females.Conclusions Depending on cancer site, primary prevention (e.g. lung cancer among males), early detection (cervical cancer), and treatment (e.g. breast and testicular cancer, lymphoma) contributed to the current decline of mortality rates. Absence of a turnaround (e.g. lung cancer among females), slower decline than in other countries (e.g. cervical cancer), or later turnaround (e.g. breast cancer) may be related to failures in promoting prevention (lung cancer among females), early detection programmes (cervical cancer), or delays in the translation of modern treatment into routine health care (breast cancer) and indicate major challenges for current and future health policy.     

Increased Frequency of Mutations in the Gene Responsible for Familial Mediterranean Fever (MEFV) in a Cohort of Patients with Ulcerative Colitis: Evidence for a Potential Disease-Modifying Effect?

The MEFV gene, responsible for familial Mediterranean fever (FMF), is involved in inflammatory reactions through altered leukocyte apoptosis, secretion of interleukin (IL)-1β, and activation of the NF-κ B pathway. Ulcerative Colitis (UC) and FMF are both characterized by a recurrent pattern of presentation with periods of remission and flares associated with neutrophilic infiltration at the site of injury. The aim of this study was to investigate the possible correlation between UC and MEFV gene alterations. Twenty-five consecutive, first-diagnosed and untreated UC patients, 28 control patients with rheumatoid arthritis, and 65 normal individuals were analyzed. Nonisotopic RNase Cleavage Assay (NIRCA) was applied as a first-step mutational screening method of exons 10 and 2 of MEFV gene; direct sequencing was subsequently performed to confirm the results. MEFVmutations were identified in 7 (3 M694V/0, 2 M680I/0, 1 E148Q/E148Q, and 1 A744S/0) out of 25 UC patients versus 1 (M694V/0) out of 28 rheumatoid arthritis patients (P = .0199) and 1 (M694V/0) out of 65 healthy controls (P = .0004). Four out of 7 patients with MEFVmutations had inflammatory arthritis, a clinical finding that was not observed in the 18 UC patients with unmutated MEFV (P = .0028). Patients with UC almost universally carried the T A C G MEFV exon 2 haplotype in contrast with normal individuals (P < .0001) and FMF patients (P = .0310). In conclusion the increased frequency of mutations of MEFV in UC patients, especially in those with episodic arthritis, suggests a possible modifying effect of MEFV in the disease process and its localization within the joint. The difference in distribution of MEFV exon 2 haplotypes between UC patients and both FMF patients and normal individuals, suggests that UC patients constitute a genetically distinct population. Larger, longitudinal studies are needed to confirm these initial findings.     

Coronavirus disease 2019 in patients with Behcet’s disease: a report of 59 cases in Iran

Clinical Rheumatology - To present the clinical characteristics, disease course, management, and outcomes of COVID-19 infection in patients with Behcet’s disease (BD). In this retrospective...     

Rheumatologists’ practice during the Coronavirus disease 2019 (COVID-19) pandemic: a survey in Egypt

Rheumatology International - The aim of this work is to trace how rheumatologists all over Egypt are approaching the COVID-19 pandemic and what changes it has brought about in the patients’...     

Management of antithrombotic therapy in adults with immune thrombocytopenia (ITP): a survey of ITP specialists and general hematologist–oncologists

While patients with immune thrombocytopenia (ITP) and low platelet counts are at risk for bleeding, they are not protected against arterial and venous thrombotic events. Frequently, hematologists are asked to consult on a patient with ITP requiring an antiplatelet (AP) agent or anticoagulant (AC). No direct evidence exists to guide hematologists in weighing the risk of thrombosis against the risk of bleeding in patients with ITP. Therefore, we performed a survey to determine the preferred management of AP/AC therapy in ITP patients. The survey described hypothetical patient scenarios and asked respondents to recommend a minimum platelet count for initiation of AP/AC therapy. We surveyed both hematologists with an international reputation in treatment of ITP (n?=?48) and also general hematologist–oncologists in Oklahoma (n?=?97). Response rates were 38/48 (79%) for the ITP specialists and 46/97 (47%) for general hematologist–oncologists. Overall, recommended platelet thresholds for antithrombotic therapy were similar between ITP specialists and general hematologist–oncologists. Although both groups recommended a minimum platelet count of 50?×?10⁹/L for AP and AC therapy in most scenarios, there was great variability in individual practice patterns among respondents. This study highlights the need for studies of patients with ITP who require AP/AC therapy to provide high-quality evidence for establishing optimal management strategies.     

A survey of traditional Chinese medicine use among rheumatoid arthritis patients: a claims data–based cohort study

Clinical Rheumatology - Traditional Chinese medicine (TCM) is commonly used for symptom relief in patients with chronic diseases. Nevertheless, large-scale surveys focusing on the utilization of...     

Burden of herpes zoster requiring hospitalization in Spain during a seven-year period (1998–2004)

Background  

A thorough epidemiological surveillance and a good understanding of the burden of diseases associated to VZV are crucial to asses any potential impact of a prevention strategy. A population-based retrospective epidemiological study to estimate the burden of herpes zoster requiring hospitalization in Spain was conducted.