Use of mosquito repellents to protect against Zika virus infection among pregnant women in Brazil.

OBJECTIVES: To evaluate the use of repellents among pregnant women as a protective measure against infection with the Zika virus. STUDY DESIGN: Pregnant women (n = 177) were interviewed between November 2016 and February 2017 at Basic Health Units in the city of Propriá, state of Sergipe, Brazil. Two units were located in rural areas and eight in urban regions. METHODS: Data were analysed using descriptive statistical methods, the Chi-squared test or Fisher's exact test and odds ratios. The independent variables were grouped by analysis of the main components, and adherence to the use of the repellent was analysed by the logistic regression method. RESULTS: A total of 100 women reported using repellents at the time of the interview (56%). The use of repellents was greater among women with higher levels of education (83%) than those with only high school (68%) or elementary school (36%) education. Women assisted by the income transfer programme (Bolsa Família) presented a 2.27 times greater chance of not using repellents compared with pregnant women who were not receiving benefits of the programme. Regarding the logistic regression model, we observed that low economic and social conditions of pregnant women, as well as their lack of advice, had a negative effect on the use of repellents. CONCLUSIONS: Repellents were generally used as a preventive measure in pregnant women with higher levels of schooling and fewer children. The relatively high cost of repellents was the main reason for non-use.

Sustained remission of SAPHO syndrome with pamidronate: a follow-up of fourteen cases and a review of the literature.

OBJECTIVE: To evaluate the efficacy of intravenous (i.v.) pamidronate in patients with SAPHO syndrome refractory to first line treatments and to review the available literature on pamidronate for this indication. METHODS: We report 14 cases of SAPHO syndrome refractory to non-steroideal anti-inflammatory drugs (NSAIDs), glucocorticoids and disease modifying anti-rheumatic drugs (DMARDs) treated with i.v. pamidronate. All patients received i.v. 60 mg pamidronate/day for 3 consecutive days. The primary evaluation criterion was the disappearance of bone pain, considered as the reduction in the visual analogic scale for pain (VAS) greater than 50%. RESULTS: Ten patients were females and 4 were males. The mean age at onset was 40.4 years old. Ten patients presented a relapsing-remitting course, while in 4 cases the disease followed a prolonged course. In all cases anterior chest wall involvement occurred early in the disease. In 2 cases there was also a peripheral monoarthritis. Eleven patients experienced several flares of palmo-plantar pustulosis, while severe acne was present in 2. In one case there was no cutaneous involvement. Twelve of the 14 patients had a good response after 3 infusions and in 8 of these patients a sustained remission was observed. The recurrence of skin manifestations does not seem to be influenced by pamidronate. CONCLUSIONS: Pamidronate appears to be an effective treatment in the osteo-articular manifestations of SAPHO syndrome. As far as cutaneous lesions are concerned, evidence of efficacy is not so impressive.

Protein Z G79A and A-13G gene polymorphisms in Italian patients with Behçet's disease.

OBJECTIVE: To investigate potential associations between A-13G and G79A polymorphisms of the protein Z gene and venous thrombosis and other clinical manifestations in Italian patients with Behçet's disease (BD). METHODS: 176 Italian patients who satisfied the International Study Group criteria for BD and 134 healthy age- and sex- matched blood donors were genotyped for A-13G and G79A polymorphisms of the protein Z gene by molecular methods. 113 and 112 of the 176 BD patients were also genotyped for factor V Leiden and prothrombin gene G20210A polymorphisms. Serological HLA class B51 typing was performed by a standard microlymphocytotoxicity technique. The patients were subgrouped according to the presence or absence of clinical manifestations. RESULTS: The distribution of allele and genotype frequencies of A-13G and G79A polymorphisms did not differ significantly between BD patients and healthy controls.The frequencies of carriage rates of protein Z G79A and A-13G polymorphisms in BD patients with and without DVT were similar. Similarly, no associations between thrombotic events and the protein Z gene polymorphisms studied were observed in BD patients carrying factor V Leiden or prothrombin gene G20210A mutations. No significant associations were observed between protein Z polymorphisms and the occurrence of specific clinical findings. CONCLUSION: No association between DVT and A-13G or G79A polymorphisms of the protein Z gene was found in Italian BD patients. Furthermore, these protein Z polymorphisms in BD do not seem to increase the risk of DVT due to factor V Leiden or prothrombin gene G20210A mutations.

Toll-like receptor 4 (TLR4) gene polymorphisms in Italian patients with Behçet's disease.

OBJECTIVE: To investigate potential associations between toll-like receptor 4 (TLR4) gene polymorphisms and susceptibility to, clinical features, and severity of Behçet's disease (BD). METHODS: A total of 189 Italian patients who satisfied the International Study Group criteria for BD and 210 healthy age- and sex-matched blood donors were genotyped for two coding single nucleotide polymorphisms of TLR4 (Asp299Gly and Thr399Ile) by molecular methods. The patients were subgrouped according to the presence or absence of clinical manifestations. Severity score was calculated. RESULTS: The distribution of allele and genotype frequencies did not differ significantly between the BD patients and the healthy controls. No significant associations were found when BD patients with and those without clinical manifestations were compared. No association between TLR4 polymorphisms and severity score was observed. CONCLUSION: Our data suggest that the TLR4 gene polymorphisms are not associated with susceptibility to, clinical expression of, and severity of BD in Italian patients.

[Headache in Behçet's disease: case-control study and literature review]. / La cefalea nella malattia di Behçet: contributo casistico e revisione della letteratura.

OBJECTIVE: To evaluate the prevalence of headache and its different patterns in patients with Behçet's disease (BD) with and without neurological involvement and to investigate clinical correlations. METHODS: Patients fulfilling the International Study Group criteria for Behçet disease (ISGc) were studied. Patients were invited to fill a "headache questionnaire", which consisted of two sections: the first one included demographic and anamnestic data, family history for both headache and BD, disease duration and clinical manifestations of BD; the second section included items about headache, investigated accordingly to International Headache Society diagnostic criteria (IHS, 2004). Clinical history and current comorbidities-medications were collected. Each patient underwent a neurological examination to assess neurological involvement (Neuro-BD) and, if necessary, instrumental investigations. One hundred-fifty healthy subjects matched for age and gender were used as control group for comparison. RESULTS: Of the 55 patients diagnosed as BD (ISG criteria) 41 patients adhered and were enrolled into the study. Headache occurred in 29 of BD patients (70,7%) and in 13 of Neuro-BD patients (92,8%). Migraine without aura did prove the most frequent type of headache in BD patients (with and without neurological involvement) and there were no differences in the frequency of the different pattern of headache between BD patients and controls. CONCLUSIONS: Headache is a frequent manifestation in BD and primary headache like migraine emerged as the most frequent type of headache. A careful search for headache should be included in the diagnostic work-up of BD since this manifestation may be related to the underlying disease.

[18FDG-PET and large vessel vasculitis: preliminary data on 25 patients]. / 18FDG-PET e vasculiti dei grandi vasi: studio preliminare su 25 pazienti.

OBJECTIVE: To evaluate the predictive value of clinical and biochemical features when compared to 18FDG-PET in the diagnostic work-up of large vessel vasculitis (LVV). METHODS: Twenty-five patients underwent 18FDG-PET for the clinical suspect of LVV. All of them presented history of systemic symptoms lasting >or=6 months and laboratoristic evidence of persistently high markers of inflammation. The patients were stratified according with: i) clinical manifestations, defined as presence of one or more ACR criteria for the classification of LVV; ii) laboratory investigations: Erythrocyte Sedimentation Rate (ESR) higher or lower than 50 mm/h, C-Reactive Protein (CRP) higher or lower than 2 mg/dl; iii) prednisone dose in the 4 weeks preceding PET examination. RESULTS: The total number of positive PET was higher in the group without clinical ACR criteria and in the group with inflammation markers under the established cut-off. The number of scans consistent with LVV was higher in the groups presenting one or more clinical criteria for LVV but in those with very high ESR and CRP. In all the cases differences between groups were not statistically significative. A clear cut negative correlation between steroid dose and number of scans suggestive for LVV has been observed. CONCLUSIONS: Diagnosis of LVV remains challenging, especially in patients presenting with a constellation of non-specific symptoms and laboratory findings. In this study, both clinical and biochemical features show low correlation with a vasculitic pattern of FDG uptake. In our experience 18FDG-PET represents an useful diagnostic tool in early stages of LVV and a powerful instrument to follow the treatment responses.

Repellency and Larvicidal Activity of Essential oils from Xylopia laevigata, Xylopia frutescens, Lippia pedunculosa, and Their Individual Compounds against Aedes aegypti Linnaeus.

In order to find new alternatives for vector control and personal protection, we evaluated the larvicidal and repellent activity of essentials oils from plants found in the Northeast of Brazil against Aedes aegypti Linnaeus mosquitoes. The plants tested include Xylopia laevigata, Xylopia frutescens, and Lippia pedunculosa and their major compounds, piperitenone oxide, and (R)-limonene. The essential oil of L. pedunculosa and its major volatile compounds were shown to be toxic for Ae. aegypti larvae with a LC50 lower than 60 ppm. The essential oil of plants from the Xylopia genus, on the other hand, showed no activity against Ae. aegypti, proving to be toxic to mosquito larvae only when concentrations were higher than 1000 ppm. All plants tested provided some degree of protection against mosquitoes landing, but only the essential oil of L. pedunculosa and the volatile compound piperitenone oxide suppressed 100% of mosquitoes landing on human skin, in concentrations lower than 1%. Among the plants studied, the essential oil of L. pedunculosa and its volatiles compounds have shown the potential for the development of safe alternative for mosquito larvae control and protection against Ae. aegypti mosquito bites.

In patients with antisynthetase syndrome the occurrence of anti-Ro/SSA antibodies causes a more severe interstitial lung disease.

We studied the clinical features and autoantibody profile in 21 patients with antisynthetase syndrome (AS) comparing to 48 patients with classical polymyositis and dermatomyositis without AS. At presentation, the AS group showed more frequently the presence of interstitial lung disease (ILD), arthritis/arthralgia, mechanic's hand and anti-Ro/SSA antibodies. Patients without AS had more frequent proximal weakness and cutaneous erythematosus rash. Interestingly, the AS patients with associated anti-Ro/SS-A antibodies seem to be predisposed to the development of a more severe ILD, expressed as HRCT total score > or = 7. During a follow up of about 3 years (range 6-110 months), the presence of anti-Jo-1 antibody alone or in association with anti-Ro/SSA did not influence survival or a more severe prognosis of ILD.

[Failure mode effect analysis applied to preparation of intravenous cytostatics]. / Análisis modal de fallos y efectos aplicado a la elaboración de citostáticos intravenosos.

OBJECTIVE: To proactively identify risks in the preparation of intravenous cytostatic drugs, and to prioritise and establish measures to improve safety procedures. MATERIAL AND METHODS: Failure Mode Effect Analysis methodology was used. A multidisciplinary team identified potential failure modes of the procedure through a brainstorming session. The impact associated with each failure mode was assessed with the Risk Priority Number (RPN), which involves three variables: occurrence, severity, and detectability. Improvement measures were established for all identified failure modes, with those with RPN>100 considered critical. The final RPN (theoretical) that would result from the proposed measures was also calculated and the process was redesigned. RESULTS: A total of 34 failure modes were identified. The initial accumulated RPN was 3022 (range: 3-252), and after recommended actions the final RPN was 1292 (range: 3-189). RPN scores >100 were obtained in 13 failure modes; only the dispensing sub-process was free of critical points (RPN>100). A final reduction of RPN>50% was achieved in 9 failure modes. CONCLUSIONS: This prospective risk analysis methodology allows the weaknesses of the procedure to be prioritised, optimize use of resources, and a substantial improvement in the safety of the preparation of cytostatic drugs through the introduction of double checking and intermediate product labelling.

Prophylaxis and treatment of NSAID-induced gastroduodenal disorders.

A significant percentage of patients taking nonsteroidal anti-inflammatory drugs (NSAIDs) experience some type of adverse gastrointestinal symptoms, lesions of the gastroduodenal tract being clinically the most relevant. NSAIDs cause gastrointestinal damage by 2 independent mechanisms: a topical effect, which is pH and pKa related, and a systemic effect mediated by cyclooxygenase (COX) inhibition with a reduction in prostaglandin synthesis. Using endoscopy, gastroduodenal lesions identified include subepithelial haemorrhages, erosions and ulcers. The prevalence of ulceration in NSAID users has been reported as being between 14 and 31% with a 2-fold higher frequency of gastric ulcers compared with duodenal ulcers. Among the strategies used to decrease the risk of ulcer development are: (i) the use of analgesics other than NSAIDs; (ii) use of the lowest possible dosage of NSAID; (iii) the use of a COX-2 selective NSAID; (iv) the use of low doses of corticosteroids instead of NSAIDs; (v) avoidance of concomitant use of NSAIDs and corticosteroids; and (vi) use of preventive therapy. In an attempt to reduce the incidence of NSAID-induced gastrointestinal lesions, the following approaches have been proposed: (i) use of the prostaglandin analogue misoprostol, which is an antiulcer drug which has been proven to be as effective in the prevention of NSAID-induced gastric and duodenal ulcers as in the reduction of serious upper gastrointestinal complications; (ii) histamine H2 receptor antagonists (H2 antagonists), e.g. ranitidine, cimetidine and famotidine, which are useful in the prevention of NSAID-induced duodenal ulcers during long term treatment, but not in the prevention of NSAID-induced gastric ulcers; (iii) proton pump inhibitors, e.g omeprazole, and pantoprazole, whose efficacy in preventing NSAID-associated ulcers has been recently demonstrated; and (iv) barrier agents, e.g. sucralfate, which cannot be recommended as prophylactic agents to prevent NSAID-induced gastropathy. The first step in the treatment of NSAID-associated ulcers lies in a reduction in the dosage of the NSAID or discontinuation of the drug. If NSAID treatment cannot be withdrawn, a proton pump inhibitor appears to be the most effective treatment in healing ulcers, accelerating the slow healing observed with H2 antagonists.

Hyperostosis and multifocal osteitis: a purely rheumatological subset of the SAPHO syndrome.

SAPHO has recently been proposed as an acronym to identify a syndrome characterized by synovitis, acne, pustulosis, hyperostosis and osteomyelitis. Several authors have, however, found that this rare condition may even occur without cutaneous manifestations. The work reported here presents the case of a 43-year-old male with skeletal involvement alone. An in-depth study of the patient revealed the arthro-osteitic pattern typical of the SAPHO syndrome. In agreement with previous reports, these data confirm that the present clinical picture of hyperostosis and osteitis, even without cutaneous involvement, is nonetheless related to the SAPHO syndrome.

Increased bronchial responsiveness in primary Sjögren's syndrome. A sign of tracheobronchial involvement.

Thirty-six patients with primary Sjögren's syndrome (pSS) and 60 healthy volunteers underwent provocative bronchial testing with aerosolized dosed methacholine. On the average, pulmonary functions tests performed before bronchial testing were normal. However, 18/36 patients (50%) had bronchial hyper-responsiveness (BH), an incidence higher that that found in our control population (6.6%). No difference between BH and normally responsive patients was found in the duration of disease, immunological abnormalities or symptoms, and only the FEF50 was significantly lower in the BH group. It is therefore hypothesized that in pSS bronchial hyper-responsiveness may be due to lymphocytic inflammation and an alteration in secretion secondary to gland damage.

Elderly onset rheumatoid arthritis: clinical aspects.

The presentation, severity and prognosis of rheumatoid arthritis (RA) differ depending on the age of disease onset. Elderly onset RA (EORA: age of onset > 60 years) has been reported to differ from younger-onset RA (YORA) by a more balanced gender distribution, a higher frequency of acute onset often associated with systemic features, more frequent involvement of the shoulder girdle and higher disease activity. To add to our knowledge of this disease, 101 EORA and 88 YORA patients, not previously treated with DMDARs or steroids, were studied and compared, paying particular attention to the onset. The female to male ratio was higher in the YORA group (4.4:1 vs 1.6:1; p < 0.05). The disease duration was similar: 5.6 +/- 3.3 months in EORA and 7.9 +/- 3.8 months in YORA. EORA presented a more frequent acute onset (33.6% vs 13.6%; p < 0.05) especially if rheumatoid factor was absent. This subset also showed more frequent polymyalgic onset. Constitutional symptoms (fever, weight loss, fatigue) were more frequent in EORA patients without differences between seropositive and seronegative patients. The distribution of involved joints showed a significantly higher frequency of shoulder involvement in EORA (64% vs 38%; p < 0.05) and of feet involvement in YORA (25% vs 52%; p < 0.05). Hands and wrists were the most frequently involved joints in all patients.

Clinical manifestations of Behçet's disease in 137 Italian patients: results of a multicenter study.

OBJECTIVE: To determine the type and frequency of clinical features of Behçet's disease in a population of Italian patients. METHODS: We retrospectively studied 137 Italian patients (76 males and 61 females, age at onset 29.6 +/- 12.2 [mean +/- SD] years) seen consecutively in nine different referral centers. The duration of follow-up at study entry was 10.9 +/- 8.2 years. Virtually all patients fulfilled the classification criteria developed by the International Study Group for Behçet's disease. The clinical manifestations of the patients were recorded by the attending physicians using specifically designed forms. RESULTS: The most frequent manifestations at disease onset were oral (78.3%) and genital aphthae (29.2%) followed by inflammatory ocular involvement (20%) and arthritis (14.2%). The commonest (>50% of cases) manifestations observed throughout the disease course were oral aphthae (99.3%), genital aphthae (62.8%), various cutaneous lesions including erythema nodosum (81.8%), and inflammatory ocular disease (60.6%). Panuveitis and posterior uveitis/retinitis occurred more frequently in males compared with females (28.9% versus 11.5% and 57.9% versus 36.1%, respectively; p < 0.05). 61.6% of our patients were HLA-B51 positive. CONCLUSION: Behçet's disease in Italian patients is characterized by a variety of clinical manifestations in agreement with the medical literature. Panuveitis and posterior uveitis/retinitis occur more frequently in male patients.

Colon involvement in systemic sclerosis: clinical-radiological correlations.

About one third of all patients with systemic sclerosis (SS) presents colon abnormalities, although these may be underestimated because they frequently remain asymptomatic for a long time. Thirty-five patients (33 women and 2 men; mean age 56.5 years; mean disease duration 11.9 years) affected by SS (25 with limited and 10 with diffuse pattern of skin involvement) were investigated using barium enema to detect radiological changes in the colon, and to correlate them with other visceral involvement, autoantibody profile, abdominal symptoms and duration of the disease. Ten patients (28.6%) showed X-rays abnormalities (excluding isolated diverticula), wide-mouthed sacculations being the most frequent finding. Our data confirm that the colon is frequently involved in SS, even in the limited form of the disease. The most relevant finding was the dissociation between clinical symptoms and radiological features which proved to be more evident among the patients with limited SS. No correlations were found between the radiological picture and any other parameter, thus suggesting that careful evaluation of the colon should be performed in any patient suffering from the disease.

[Reynolds syndrome associated with Sjögren's syndrome]. / Associazione sindrome di Reynolds-sindrome di Sjögren.

Observation of a case of CREST syndrome associated with autoimmune chronic hepatopathy (so-called Reynolds syndrome) and Sjögren's syndrome brings to the fore the importance of symptomatological superimpositions within the autoimmune diseases. A number of diagnostic and pathogenetic aspects of this rare disease association are discussed.

[Autologous stem cell transplantation in a patient with diffuse systemic sclerosis]. / Trapianto autologo di cellule staminali in paziente affetta da sclerosi sistemica a varietà diffusa.

Systemic Sclerosis (SSc) is a systemic disease of unknown etiology presenting with disseminated skin thickening and fibrotic impairment of various organs including lung and kidney. According to the rate and degree of skin involvement, SSc can be classified in a limited and a diffuse form, the latter showing a severe and progressive lung involvement, which is responsible for its high related morbidity and mortality along with resistance to standard therapeutic protocols. High dose chemotherapy, followed by autologous stem cell transplantation, is a standard therapeutic regimen for haematological diseases: re-infusion of mobilised peripheral blood progenitor cells overcomes the myeloablative effect of super-maximal eradicative doses of chemotherapeutic agents. Recently, this therapeutic approach has been applied in some cases of resistant SSc and, albeit the low number of cases, it has been proven effective in early diagnosed and rapidly progressive forms of the disease showing a clinical improvement and an instrumentally detectable decrease of fibrosis extent. We report the case of a young woman affected by diffuse SSc with a rapid progression of clinical signs and instrumentally detectable lesions who underwent a conditioning regimen with fludarabine, cyclophosphamide and anti-thymoglobulines followed by re-infusion of autologous peripheral blood stem cells. Two years after transplantation a clinical and instrumental evidence of treatment was observed, with good control of disease evolution. The only sign of disease resumption was a slow worsening of skin involvement.

[Functional reserve of the exocrine pancreas in Sjögren's syndrome]. / La riserva funzionale del pancreas esocrino in corso di sindrome di Sjögren.

Exocrine pancreatic function was studied in a homogeneous group of 33 female patients aged 42-67 years. Of these patients, 11 were classified as rheumatoid arthritis (RA), 11 as Sjögren's syndrome I (SSI) and 11 as Sjögren's syndrome associated with RA (SSII). Clinical features, laboratory tests and special instrumental techniques excluded gastroenteric-hepatobiliary causes of pancreatic diseases. These patients were subjected to direct pancreatic stimulation with secretin and caerulein (S. Cae test). Test results, compared to control-group (10 voluntary healthy females) showed, in the last 30 m of stimulation, a statistically significant decrease (p < 0.05) in duodenal juice volume, bicarbonates and trypsin in 6 cases (54.5%) of SSI and in 3 cases (27.2%) of RA. SSII S. Cae test showed a decrease of volume and bicarbonates in 6 patients (54.5%) and in only 4 of these (36.4%) it was associated with a concomitant decrease in trypsin levels. Authors discuss the subclinical exocrine pancreatic function in relationship to sicca-syndrome, possible immunological factors and primary disease of pancreatic ducts.

[Pancreatic duct antibodies and subclinical insufficiency of the exocrine pancreas in Sjögren's syndrome]. / Gli anticorpi anti-duttuli e l'insufficienza subclinica del pancreas esocrino nella sindrome di Sjögren.

In previous studies we reported evidence of subclinical exocrine pancreatic insufficiency in primary or secondary Sjögren's syndrome (SSI, SSII) and rheumatoid arthritis (RA). In present study we evaluated the occurrence of pancreatic duct antibodies (PDA), and their relationship to exocrine pancreatic function in 36 women. Of these patients, 12 were classified as SSI, 12 as SSII, and 12 as RA. Exocrine pancreatic function was evaluated using the Secretin-Caerulein test (S.Cae test). The indirect immunofluorescent technique was used to evaluate patient sera for PDA. S.Cae test results, compared to controls, showed a statistically significant decrease in duodenal juice volumes, bicarbonates and enzymes in 58.3% of SSI, in 58.3% of SSI and in 30% of RA, according to our previous trials. PDA were found in two patients, the former with SSI and the latter with SSII, both asymptomatic with regard to pancreatic diseases but with impaired exocrine pancreatic function as evaluated by S.Cae test. We discuss the possible causes of these results.