Особенности синдрома Рейно у пациентов c cистемной cклеродермией

Discipline of Nephrology and Rheumatology, Medical Clinic No 5, Nicolae Testemitanu State Medical and Pharmaceutical University, Congresul III al Medicilor de Familie din Republica Moldova, 17–18 mai, 2012, Chişinău, Republica Moldova, Conferinţa Naţională „Maladii bronhoobstructive la copii”, consacrată profesorului universitar, doctor habilitat Victor Gheţeul, 27 aprilie, Chişinău, Republica MoldovaIntroducere. Fenomenul Raynaud (FR) este definit ca un atac recurent de ischemie digitală, care evoluează în trei faze de culoare – paloare, cianoză şi roşeaţă, provocate, de obicei, de expunere la frig şi emoţii. Sclerodermia sistemică (SS) este o patologie generalizată a ţesutului conjunctiv ce se caracterizează prin dezvoltarea proceselor degenerative şi inflamatorii cu o afectare obliterantă a vaselor mici şi medii, precum şi a fibrozei pielii şi organelor interne (cordul, plămânii, rinichii, tractul gastrointestinal). Particularităţile sindromului Raynaud la pacienţii cu sclerodermie sistemică Expunerea la frig şi stresul emoţional poate induce vasospasm, ceea ce cauzează episoade caracteristice de înălbire sau cianoză a degetelor. De obicei, are loc afectarea bilaterală a degetelor mâinilor, uneori şi a picioarelor. Infarctele tisulare la vârful degetelor pot duce la ulceraţii, cicatrice stelate sau chiar la o gangrenă adevărată. În sclerodermia limitată sindromul Raynaud este un fenomen universal, precedând uneori ani şi chiar decenii, apariţia altor simptome ale sclerodermiei difuze. Fenomenul Raynaud este prezent la 75-85% dintre pacienţii cu SS. Absenţa lui este asociată cu un risc crescut de dezvoltare a afectărilor renale. Mai des sunt afectate porţiunile arteriolare ale vaselor, dar pot fi afectate arterele digitale sau alte vase mai mari de calibru mediu ca a. ulnaris. Procesele similare pot fi observate microscopic în patul unghial, efectuând o capilaromicroscopie, evidenţiind micşorarea numărului de capilare, sinuozitatea şi dilatarea vaselor rămase, numite teleangiectazii. Obiectivele studiului: evaluarea frecvenţei şi variantelor de prezentare a sindromului Raynaud la bolnavii cu sclerodermie sistemică în Republica Moldova. Material şi metode În studiu au fost incluşi 80 de pacienţi care asociau sclerodermie sistemică, dintre care doar 5 au fost de sex masculin. Vârsta medie a subiecţilor, incluşi în studiu, a constituit 45,1 ± 2,2 ani. Durata medie a maladiei – 13,1 ± 1,4 ani. Dintre ei 67 (83,75%) de pacienţi au prezentat Scl forma limitată, iar 13 (16,25%) – forma difuză a bolii. Activitatea medie a maladiei a fost de 4,5 ± 0,6 (conform scorului EUSTAR). Diagnosticul a fost stabilit conform criteriilor ACR (American College of Rheumatism), propuse în anul 1980. Pacienţii au fost examinaţi clinic şi paraclinic complex, inclusiv efectuându-li-se investigaţii imunologice (anticorpi antinucleari, anticentromeri, antitopoizomerază, antiribonuclează). Rezultate obţinute La pacienţii, incluşi în studiu, boala a debutat prin semnele sindromului Raynaud în 72 (90%) de cazuri. Perioada de până la apariţia primelor semne non-Raynaud a fost în mediu de 13,4 ± 0,4 luni. Caracterul trifazic al sindromului a fost observat doar la 15 (18,75%) dintre pacienţi, bifazic – la 49 (61,25%) şi monofazic, cu paloarea tegumentelor, la 16 (20%) dintre pacienţii evaluaţi. Ca factori declanşatori ai atacurilor, pacienţii au menţionat: frigul 72 (90%), emoţiile 32 (40%), fumul de ţigară 4 (5%), aer condiţionat 2 (2,5%), vântul 3 (3,75%), ridicarea vocii 1 (1,25%). Ulcere digitale sau consecinţele acestora (cicatrice) au fost semnalate la 21% dintre bolnavii incluşi în studiu. Severitatea sindromului Raynaud (conform duratei şi frecvenţei atacurilor, prezenţei ulcerelor digitale) a corelat direct cu scorul cutanat modificat Rodnan, cu presiunea sistolică în ventriculul drept (determinat ecografic), cu cantitatea anticorpilor anticentromeri şi cu antitopoizomeraza. Concluzii 1. Sindromul Raynaud este o manifestare frecventă (90%) şi precoce a sclerodermiei sistemice. 2. Caracterul clasic trifazic s-a înregistrat doar în 18,75% dintre cazuri; s-a observat prezenţa a mai multor factori declanşatori decât frigul şi emoţiile, fapte care indică necesitatea unei elaborări mai minuţioase a anamnezei la pacienţii cu sclerodermie sitemică. 3. Severitatea sindromului Raynaud a corelat direct cu scorul cutanat modificat Rodnan, cu presiunea sistolică în ventriculul drept şi cu cantitatea anticorpilor anticentromeri şi antitopoizomerazei

Подагра у мужчин и сопутствующие заболевания

Discipline of Nephrology and Rheumatology, Medical Clinic No 5, Nicolae Testemitanu State Medical and Pharmaceutical University, Congresul III al Medicilor de Familie din Republica Moldova, 17–18 mai, 2012, Chişinău, Republica Moldova, Conferinţa Naţională „Maladii bronhoobstructive la copii”, consacrată profesorului universitar, doctor habilitat Victor Gheţeul, 27 aprilie, Chişinău, Republica MoldovaScopul. Studierea patologiilor concomitente ale organelor interne la bărbaţii cu gută. Material şi metode Au fost cercetaţi 130 de bărbaţi cu diagnosticul de gută (conform criteriilor ARA), cu vârsta cuprinsă între 18-72 de ani, cu durata medie a maladiei de la 2 până la 38 de ani. Au fost efectuate: analiza generală a sângelui, analiza generală a urinei, s-a apreciat în sânge nivelul colesterolului, trigliceridelor, glucozei, protrombinei, ureei, creatininei, proteinei totale, calciului seric, ECG, EcoCG, USG organelor interne, aprecierea indicelui greutăţii corporale. Rezultate În rezultatul cercetărilor au fost depistate următoarele maladii concomitente – la 87 (67%) de pacienţi a fost depistată hipertensiunea arterială, la 76 (58%) pacienţi – obezitate (gr. I-III), la 54 (41%) – creşterea trigliceridelor, la 47 (36%) bolnavi a fost depistată urolitiaza, la 39 (30%) – diabet zaharat tip II, la 34 (26%) de pacienţi – pielonefrită cronică, la 29 (22%) de bolnavi – insuficienţă renală cronică (gr. I-V după K/DOQI), la 5 (4%) bolnavi – în anamneză infarct miocardic acut, la 3 (2%) bolnavi – hemoragie gastrică în anamneză (pe fundalul tratamentului cu AINS). Concluzii Datele obţinute sunt sugestive pentru prezenta corelaţie între gută şi patologia organelor interne. Depistarea precoce a tuturor maladiilor concomitente în gută ne va permite să adaptăm corect tratamentul şi, astfel, să preîntâmpinăm apariţia complicaţiilor

Особенности хронической нефропатии у пациентов с системной склеродермией

Catedra Medicină Internă nr. 1, USMF “Nicolae Testemiţanu”, Centrul Naţional Ştiinţifico-Practic Medicină de Urgenţă, Chişinău, Conferinţa Ştiinţifico-Practică „Medicina modernă, actualităţi şi perspective”, consacrată aniversării de 40 de ani ai Spitalului Clinic al Ministerului Sănătăţii, 27-28 mai, 2010, Chişinău, Republica MoldovaImpaired renal vessels in systemic scleroderma (SS) are characterized by a specific lesion of small arteries (diameter 150-500 mm) by the proliferation of intima, media thickening and increased deposition of collagen in adventitious. The study included 70 patients with SS, of which only 3 were male. The average age of subjects included in the study was 43.4 years. The average duration of disease was 16.5 years. It was established that chronic nephropathy is diagnosed relatively frequently (21.4%) in patients with SS, especially as disease duration increases, and with an accuracy of 76.5% some factors (disease duration, spreading sclerodermic skin damage, treatment with D-penicillamine) may be considered important for its development.Поражение почечных сосудов при системной склеродермии (СС) характеризуется специфическими изменениями малых артерий (диаметром 150-500 мм): пролиферацией интимы, сгущением средней оболочки и отложением коллагена в адвентиции. В исследование были включены 70 пациентов с СС, из которых 3 были мужчины. Средний возраст больных, включенных в исследование, был 43,4 лет. Средняя продолжительность заболевания - 16,5 лет. Было установлено, что хроническая нефропатия регистрируются довольно часто, в среднем, в 21,4% случаев. Длительность заболевания, распространение склеродермических повреждений кожи и лечение D-пеницилламином могут в значительной степени способствовать развитию хронической нефропатии, увеличивая ее частоту до 76,5%

Characterization of Botswana coal from two coal fields : Mabesekwa and Mmamabula to determine its coal rank

Abstract: Coal samples from two different coal fields: Mabesekwa and Mmamabula were collected (with six seams from each coal field) to characterize using X-Ray diffractometer (XRD), Scanning Electron Microscopy (SEM) and Fourier Transform Infrared (FT-IR) spectroscopy. Proximate and ultimate analysis are also given. Clay minerals kaolinite, pyrite, dolomite, siderite and calcite, and quartz were discovered. Comparing measurements of C, VM, GCV, MC and FC obtained from the samples with literature, Botswana coal identifies as lignite grade A coal suitable for applications in coal gasification and ammonia based fertilisers due to its high volatile matter and high reactivity of char. The paper looks to use different methods to analyse Botswana coal and recommend its potential applications based on literature

Health Assessment Questionnaire-Disability Index (HAQ-DI) use in modelling disease progression in diffuse cutaneous systemic sclerosis: an analysis from the EUSTAR database

BACKGROUND: Patients with diffuse cutaneous systemic sclerosis (dcSSc) have a poor prognosis. The importance of monitoring subjective measures of functioning and disability, such as the Health Assessment Questionnaire-Disability Index (HAQ-DI), is important as dcSSc is rated by patients as worse than diabetes or hemodialysis for quality of life impairment. This European Scleroderma Trials and Research (EUSTAR) database analysis was undertaken to examine the importance of impaired functionality in dcSSc prognosis. The primary objectives were to identify predictors of death and HAQ-DI score progression over 1 year. HAQ-DI score, major advanced organ involvement, and death rate were also used to develop a comprehensive model to predict lifetime dcSSc progression. METHODS: This was an observational, longitudinal study in patients with dcSSc registered in EUSTAR. Death and HAQ-DI scores were, respectively, analyzed by Cox regression and linear regression analyses in relation to baseline covariates. A microsimulation Markov model was developed to estimate/predict natural progression of dcSSc over a patient's lifetime. RESULTS: The analysis included dcSSc patients with (N = 690) and without (N = 4132) HAQ-DI score assessments from the EUSTAR database. Baseline HAQ-DI score, corticosteroid treatment, and major advanced organ involvement were predictive of death on multivariable analysis; a 1-point increase in baseline HAQ-DI score multiplied the risk of death by 2.7 (p <  0.001) and multiple advanced major organ involvement multiplied the risk of death by 2.8 (p <  0.05). Multivariable analysis showed that baseline modified Rodnan Skin Score (mRSS) and baseline HAQ-DI score were associated with HAQ-DI score progression at 1 year (p <  0.05), but there was no association between baseline organ involvement and HAQ-DI score progression at 1 year. HAQ-DI score, major advanced organ involvement, and death were successfully used to model long-term disease progression in dcSSc. CONCLUSIONS: HAQ-DI score and major advanced organ involvement were comparable predictors of mortality risk in dcSSc. Baseline mRSS and baseline HAQ-DI score were predictive of HAQ-DI score progression at 1 year, indicating a correlation between these endpoints in monitoring disease progression. It is hoped that this EUSTAR analysis may change physician perception about the importance of the HAQ-DI score in dcSSc

Update of EULAR recommendations for the treatment of systemic sclerosis

The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc

Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis

Objective: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained. / Methods: A total of 11,318 patients were registered in the EUSTAR database, and 6,927 were included in the study. Twenty‐four clinical and serologic variables were used for clustering. / Results: Clustering analyses provided a first delineation of 2 clusters showing moderate stability. In an exploratory attempt, we further characterized 6 homogeneous groups that differed with regard to their clinical features, autoantibody profile, and mortality. Some groups resembled usual dcSSc or lcSSc prototypes, but others exhibited unique features, such as a majority of lcSSc patients with a high rate of visceral damage and antitopoisomerase antibodies. Prognosis varied among groups and the presence of organ damage markedly impacted survival regardless of cutaneous involvement. / Conclusion: Our findings suggest that restricting subsets of SSc patients to only those based on cutaneous involvement may not capture the complete heterogeneity of the disease. Organ damage and antibody profile should be taken into consideration when individuating homogeneous groups of patients with a distinct prognosis