62 research outputs found
ASSESSMENT OF THE SENSITIVITY OF NEW CRITERIA FOR SYSTEMIC SCLEROSIS IN RUSSIAN PATIENT POPULATION
Systemic sclerosis (SS) is a progressive connective tissue disease, the prognosis of which largely depends on the time ofΒ adequate therapy initiation. Low sensitivity of the 1980 American College of Rheumatology (ACR) SS classificationΒ criteria for identifying patients with early stage of the disease, and with its limited form in particular, has necessitatedΒ revision of existing SS diagnostic standards and elaboration of more sensitive criteria that allow to establish the diagnosisΒ when the first sign of the disease appear.Objective: to compare the sensitivity of the novel SS criteria of ACR and European League against RheumatismΒ (ACR/EULAR) 2013 and the 1980 ACR criteria in different stages of the disease.Subjects and methods. The investigation enrolled 302 patients who had been diagnosed by experts as having SS andΒ followed up at the V.A. Nasonova Research Institute of Rheumatology in 2007β2013. The patientsβ mean age wasΒ 49Β±13 years (18 to 80 years); male to female ratio β 1:9 (31 and 271), that of patients with diffuse and limited SS βΒ 1:2 (105 and 197); mean duration of the disease from the first non-Raynaudβs syndrome was 8.2Β±7.0 years (6Β months to 36 years). Physical examination, nailfold capillaroscopy, chest radiography or computed tomography,Β echocardiography for the determination of pulmonary artery systolic pressure and SS-specific antibodies evaluationΒ were performed.Results. 273 (90%) patients fulfilled the novel ACR/EULAR 2013 SS criteria. 76 (25%) patients had skin thickeningΒ above the metacarpophalangeal (MPC) joints in both hands; 263 (87%) β finger skin thickening [70 (23%) β fingerΒ swelling, 192 (64%) β thickening of all fingers distal to the MPC joints], 141 (47%) β digital ischemia [79 (26%) β digital pitting scars, 20 (7%) β digital ulcers, 42 (14%) β digital pitting scars and ulcers], 134 (44%) β telangiectasias, 276 (91%) β capillaroscopic changes, 225 (78%) β pulmonary hypertension (PH) or interstitial lung disease (ILD) [15 (5%) β PH 185 (61%) β ILD, 35 (12%) β ILD and PH], 301 (99%) β Raynaudβs phenomenon, and 185 (61%) β SS autoantibodies [138 (46%) β anti-Scl-70 antibodies (a-Scl-70), 42 (14%) β anti-centromere antibodies (ACA), 5 (1.7%) β ACA and a-Scl-70]. 216 (72%) patients fulfilled 1980 ACR SS criteria, and all of them met the novel criteria. With the latter, SS could be additionally diagnosed in 57 more (18%) patients.Conclusion. The 2013 ACR/EULAR SS classification criteria have much higher sensitivity than the 1980 ACR criteria. The sensitivity of the novelΒ criteria remained at the level of 90% in all, including the earliest, stages of the disease while the ACR criteria allowed to confirm diagnosis of SS inΒ only half of patients with a disease duration of less than 1 year
Π‘ΠΏΠ΅ΠΊΡΡ Π°Π½ΡΠΈΠΌΠΈΠΊΡΠΎΠ±Π½ΠΎΠ³ΠΎ Π΄Π΅ΠΉΡΡΠ²ΠΈΡ ΠΏΡΠΎΡΠΈΠ²ΠΎΠ³ΡΠΈΠ±ΠΊΠΎΠ²ΠΎΠ³ΠΎ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠ° ΠΡΡΠ»Π°Π½
ΠΡΠΎΠ±Π»Π΅ΠΌΠ°ΡΠΈΠΊΠ°. ΠΠ΅ΡΡΠ° ΠΏΠΎΠ»ΠΎΠ²ΠΈΠ½Π° XXΠ ΡΡ. Ρ
Π°ΡΠ°ΠΊΡΠ΅ΡΠΈΠ·ΡΠ²Π°Π»Π°ΡΡ ΠΏΠΎΠΌΡΡΠ½ΠΈΠΌ Π·ΡΠΎΡΡΠ°Π½Π½ΡΠΌ Π·Π°Ρ
Π²ΠΎΡΡΠ²Π°Π½ΠΎΡΡΡ Π½Π° ΠΌΡΠΊΠΎΠ·ΠΈ. ΠΠ½Π°ΡΠ½ΠΎΠ³ΠΎ ΡΠ΅ΡΠΈΡΠΎΡΡΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΠΏΠΎΡΠΈΡΠ΅Π½Π½Ρ Π½Π°Π±ΡΠ»Π° Π½ΠΈΠ·ΠΊΠ° Π³ΡΠΈΠ±ΠΊΠΎΠ²ΠΈΡ
ΡΠ½ΡΠ΅ΠΊΡΡΠΉ, Π·ΠΎΠΊΡΠ΅ΠΌΠ° Π΄Π΅ΡΠΌΠ°ΡΠΎΡΡΡΡΠΉ ΡΠ° Π²Π°Π³ΡΠ½Π°Π»ΡΠ½ΠΈΠΉ ΠΊΠ°Π½Π΄ΠΈΠ΄ΠΎΠ· (ΠΌΠΎΠ»ΠΎΡΠ½ΠΈΡΡ), ΡΠΎ ΠΌΠΎΠΆΠ½Π° ΠΏΠΎΡΡΠ½ΠΈΡΠΈ ΡΠ½ΡΠ΅Π½ΡΠΈΠ²Π½ΠΎΡ ΠΌΡΠ³ΡΠ°ΡΡΡΡ Π½Π°ΡΠ΅Π»Π΅Π½Π½Ρ ΡΠ° Π·ΠΌΡΠ½ΠΎΡ ΡΠΏΠΎΡΠΎΠ±Ρ ΠΆΠΈΡΡΡ Π² ΡΠ½Π΄ΡΡΡΡΡΠ°Π»ΡΠ½ΠΈΡ
ΠΊΡΠ°ΡΠ½Π°Ρ
. Π¦Π΅ Π·ΡΠΎΡΡΠ°Π½Π½Ρ Π½Π΅ Π²Π΄Π°Π»ΠΎΡΡ Π·ΡΠΏΠΈΠ½ΠΈΡΠΈ Ρ ΠΏΡΡΠ»Ρ Π²ΠΏΡΠΎΠ²Π°Π΄ΠΆΠ΅Π½Π½Ρ Π½ΠΎΠ²ΡΡΠ½ΡΡ
Π°Π½ΡΠΈΠΌΡΠΊΠΎΡΠΈΡΠ½ΠΈΡ
Π·Π°ΡΠΎΠ±ΡΠ², Π±ΡΠ»ΡΡΡΡΡΡ ΡΠ· ΡΠΊΠΈΡ
ΠΌΠ°ΡΡΡ ΠΏΠΎΠ±ΡΡΠ½Ρ Π΄ΡΡ ΡΠ° Ρ
Π°ΡΠ°ΠΊΡΠ΅ΡΠΈΠ·ΡΡΡΡΡΡ Π·Π½Π°ΡΠ½ΠΎΡ ΡΠΎΠΊΡΠΈΡΠ½ΡΡΡΡ. Π£ Π·Π²βΡΠ·ΠΊΡ Π· ΡΠΈΠΌ ΠΏΠ΅ΡΡΠΏΠ΅ΠΊΡΠΈΠ²Π½ΠΈΠΌ Π·Π°Π»ΠΈΡΠ°ΡΡΡΡΡ ΡΠΎΠ·ΡΠΎΠ±Π»Π΅Π½Π½Ρ Π½ΠΎΠ²ΠΈΡ
Π΅ΡΠ΅ΠΊΡΠΈΠ²Π½ΠΈΡ
Ρ Π½Π΅ΡΠΎΠΊΡΠΈΡΠ½ΠΈΡ
ΠΏΡΠΈΡΠΎΠ΄Π½ΠΈΡ
Π°Π½ΡΠΈΠΌΡΠΊΠΎΡΠΈΠΊΡΠ². ΠΠ΅ΡΠ° Π΄ΠΎΡΠ»ΡΠ΄ΠΆΠ΅Π½Π½Ρ. ΠΠΎΡΠ»ΡΠ΄ΠΆΠ΅Π½Π½Ρ Π΄Π΅ΡΠΊΠΈΡ
ΠΏΠΎΡΠ΅Π½ΡΡΠΉΠ½ΠΈΡ
ΠΌΠ΅Ρ
Π°Π½ΡΠ·ΠΌΡΠ² Π΄ΡΡ Π²ΡΡΡΠΈΠ·Π½ΡΠ½ΠΎΠ³ΠΎ Π°Π½ΡΠΈΠΌΡΠΊΠΎΡΠΈΠΊΠ° ΠΡΡΠ»Π°Π½, Π° ΡΠ°ΠΌΠ΅ ΡΠΏΠ΅ΠΊΡΡΠ° Π°Π½ΡΠΈΠΌΡΠΊΡΠΎΠ±Π½ΠΎΡ Π°ΠΊΡΠΈΠ²Π½ΠΎΡΡΡ Π²ΡΠ΄Π½ΠΎΡΠ½ΠΎ Π³ΡΠ°ΠΌΠΏΠΎΠ·ΠΈΡΠΈΠ²Π½ΠΈΡ
, Π³ΡΠ°ΠΌΠ½Π΅Π³Π°ΡΠΈΠ²Π½ΠΈΡ
Π±Π°ΠΊΡΠ΅ΡΡΠΉ, ΠΌΡΠΊΡΠΎΡΠΊΠΎΠΏΡΡΠ½ΠΈΡ
Π³ΡΠΈΠ±ΡΠ² ΡΠ° Π΄ΡΡΠΆΠ΄ΠΆΡΠ²; Π²ΠΏΠ»ΠΈΠ² Π½Π° ΠΌΠΎΡΡΠΎΠ»ΠΎΠ³ΡΡΠ½Ρ ΠΎΠ·Π½Π°ΠΊΠΈ ΡΠ° ΡΠ΅ΡΠΌΠ΅Π½ΡΠ°ΡΠΈΠ²Π½Ρ Π°ΠΊΡΠΈΠ²Π½ΡΡΡΡ Π΄ΡΡΠΆΠ΄ΠΆΡΠ². ΠΠ΅ΡΠΎΠ΄ΠΈΠΊΠ° ΡΠ΅Π°Π»ΡΠ·Π°ΡΡΡ. ΠΠΈΠ²ΡΠ΅Π½Π½Ρ Π°Π½ΡΠΈΠΌΡΠΊΡΠΎΠ±Π½ΠΎΡ Π°ΠΊΡΠΈΠ²Π½ΠΎΡΡΡ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΡ ΠΏΡΠΎΠ²ΠΎΠ΄ΠΈΠ»ΠΈ ΠΌΠ΅ΡΠΎΠ΄ΠΎΠΌ ΡΠ΅ΡΡΠΉΠ½ΠΈΡ
ΡΠΎΠ·Π²Π΅Π΄Π΅Π½Ρ. ΠΠΈΠ²ΡΠ΅Π½Π½Ρ Π΄ΠΈΠ½Π°ΠΌΡΠΊΠΈ Π·Π°Π³ΠΈΠ±Π΅Π»Ρ ΠΊΠ»ΡΡΠΈΠ½ ΠΏΡΠ΄ Π²ΠΏΠ»ΠΈΠ²ΠΎΠΌ ΠΡΡΠ»Π°Π½Ρ ΠΏΡΠΎΠ²ΠΎΠ΄ΠΈΠ»ΠΈ Π½Π° ΠΌΠΎΠ΄Π΅Π»Ρ ΠΊΡΠ»ΡΡΡΡΠΈ Candida tropicalis. ΠΠΌΡΠ½ΠΈ ΠΌΠΎΡΡΠΎΠ»ΠΎΠ³ΡΡ ΠΊΠ»ΡΡΠΈΠ½ Π²ΠΈΠ²ΡΠ°Π»ΠΈ Π·Π° Π΄ΠΎΠΏΠΎΠΌΠΎΠ³ΠΎΡ ΡΠ²ΡΡΠ»ΠΎΠ²ΠΎΠ³ΠΎ ΠΌΡΠΊΡΠΎΡΠΊΠΎΠΏΠ°. Π Π΅Π·ΡΠ»ΡΡΠ°ΡΠΈ Π΄ΠΎΡΠ»ΡΠ΄ΠΆΠ΅Π½Π½Ρ. ΠΠΎΠΊΠ°Π·Π°Π½ΠΎ, ΡΠΎ ΠΡΡΠ»Π°Π½ Ρ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠΌ ΡΠΈΡΠΎΠΊΠΎΠ³ΠΎ ΡΠΏΠ΅ΠΊΡΡΠ° Π΄ΡΡ, Π°ΠΊΡΠΈΠ²Π½ΠΈΠΌ ΠΏΡΠΎΡΠΈ Π³ΡΠ°ΠΌΠΏΠΎΠ·ΠΈΡΠΈΠ²Π½ΠΈΡ
ΡΠ° Π³ΡΠ°ΠΌΠ½Π΅Π³Π°ΡΠΈΠ²Π½ΠΈΡ
Π±Π°ΠΊΡΠ΅ΡΡΠΉ, Π΄ΡΡΠΆΠ΄ΠΆΡΠ² ΡΠ° ΠΏΠ»ΡΡΠ½ΡΠ²ΠΈΡ
Π³ΡΠΈΠ±ΡΠ². ΠΡΡΠ°Π½ΠΎΠ²Π»Π΅Π½ΠΎ ΠΌΡΠ½ΡΠΌΠ°Π»ΡΠ½Ρ ΠΏΡΠΈΠ³Π½ΡΡΡΠ²Π°Π»ΡΠ½Ρ ΠΊΠΎΠ½ΡΠ΅Π½ΡΡΠ°ΡΡΡ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΡ Π²ΡΠ΄Π½ΠΎΡΠ½ΠΎ Π΄ΡΡΠΆΠ΄ΠΆΡΠ² (250-500 ΠΌΠΊΠ³/ΠΌΠ»), Π³ΡΠΈΠ±ΡΠ² (62,5-500 ΠΌΠΊΠ³/ΠΌΠ»), Π³ΡΠ°ΠΌΠΏΠΎΠ·ΠΈΡΠΈΠ²Π½ΠΈΡ
(31,2-250 ΠΌΠΊΠ³/ΠΌΠ») ΡΠ° Π³ΡΠ°ΠΌΠ½Π΅Π³Π°ΡΠΈΠ²Π½ΠΈΡ
(62,5-250 ΠΌΠΊΠ³/ΠΌΠ») Π±Π°ΠΊΡΠ΅ΡΡΠΉ. ΠΠΎΡΠ»ΡΠ΄ΠΆΠ΅Π½Π½Ρ ΠΌΠΎΡΡΠΎΠ»ΠΎΠ³ΡΡΠ½ΠΈΡ
ΠΎΠ·Π½Π°ΠΊ ΠΊΠ»ΡΡΠΈΠ½ Candida tropicalis ΠΏΠΎΠΊΠ°Π·Π°Π»ΠΈ, ΡΠΎ Π²Π½Π΅ΡΠ΅Π½Π½Ρ ΠΡΡΠ»Π°Π½Ρ Π² ΠΆΠΈΠ²ΠΈΠ»ΡΠ½Π΅ ΡΠ΅ΡΠ΅Π΄ΠΎΠ²ΠΈΡΠ΅ Ρ ΡΡΠ½Π³ΡΡΠΈΠ΄Π½ΡΠΉ ΠΊΠΎΠ½ΡΠ΅Π½ΡΡΠ°ΡΡΡ ΠΏΡΠΈΠ·Π²ΠΎΠ΄ΠΈΠ»ΠΎ Π΄ΠΎ Π·ΠΌΡΠ½ΠΈ ΠΌΠΎΡΡΠΎΠ³Π΅Π½Π΅Π·Ρ ΠΊΠ»ΡΡΠΈΠ½ Π΄ΡΡΠΆΠ΄ΠΆΡΠ², ΡΡΡΠΏΡΠ½Ρ ΠΏΡΠΎΡΠ²Ρ ΡΠΊΠΎΠ³ΠΎ Π±ΡΠ»Π° ΠΎΠ±ΡΠΌΠΎΠ²Π»Π΅Π½Π° ΡΠ°ΡΠΎΠ²ΠΈΠΌ ΡΠ°ΠΊΡΠΎΡΠΎΠΌ. ΠΠΎΡΠ²Π° ΠΏΡΠ΄ Π²ΠΏΠ»ΠΈΠ²ΠΎΠΌ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΡ ΠΎΠΏΡΠΊΠ»ΠΎΡΡΠ΅ΠΉ ΡΠ° ΡΠΎΠ·ΡΠΈΠ²ΡΠ² Π½Π° ΠΏΠΎΠ²Π΅ΡΡ
Π½Ρ ΠΊΠ»ΡΡΠΈΠ½ Π΄ΡΡΠΆΠ΄ΠΆΡΠ², Π° ΡΠ°ΠΊΠΎΠΆ Π·ΠΌΡΠ½Π° ΡΡ
ΡΠΎΡΠΌΠΈ, Π²ΡΡΠΎΠ³ΡΠ΄Π½ΠΎ, Π±ΡΠ»ΠΈ Π½Π°ΡΠ»ΡΠ΄ΠΊΠΎΠΌ ΠΏΠΎΡΡΡΠ΅Π½Π½Ρ Π½ΠΎΡΠΌΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΠΎΠ±ΠΌΡΠ½Ρ ΡΠ΅ΡΠΎΠ²ΠΈΠ½ ΠΊΡΠ»ΡΡΡΡΠΈ, ΡΠΎ ΠΏΡΠ΄ΡΠ²Π΅ΡΠ΄ΠΈΠ»ΠΎΡΡ Π΅ΠΊΡΠΏΠ΅ΡΠΈΠΌΠ΅Π½ΡΠ°Π»ΡΠ½ΠΈΠΌΠΈ Π΄Π°Π½ΠΈΠΌΠΈ. ΠΠΈΡΠ½ΠΎΠ²ΠΊΠΈ. ΠΡΡΠΈΠΌΠ°Π½Ρ ΡΠ΅Π·ΡΠ»ΡΡΠ°ΡΠΈ ΡΠ²ΡΠ΄ΡΠ°ΡΡ, ΡΠΎ ΠΡΡΠ»Π°Π½ Ρ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠΌ ΡΠΈΡΠΎΠΊΠΎΠ³ΠΎ ΡΠΏΠ΅ΠΊΡΡΠ° Π΄ΡΡ, Π°ΠΊΡΠΈΠ²Π½ΠΈΠΌ Π½Π΅ Π»ΠΈΡΠ΅ Π²ΡΠ΄Π½ΠΎΡΠ½ΠΎ ΠΌΡΠΊΡΠΎΠΌΡΡΠ΅ΡΡΠ² ΡΠ° Π΄ΡΡΠΆΠ΄ΠΆΠΎΠ²ΠΈΡ
ΠΊΡΠ»ΡΡΡΡ, Π°Π»Π΅ ΠΉ Π²ΡΠ΄Π½ΠΎΡΠ½ΠΎ Π³ΡΠ°ΠΌΠΏΠΎΠ·ΠΈΡΠΈΠ²Π½ΠΈΡ
Ρ Π³ΡΠ°ΠΌΠ½Π΅Π³Π°ΡΠΈΠ²Π½ΠΈΡ
Π±Π°ΠΊΡΠ΅ΡΡΠΉ. ΠΡΡΠ°Π½ΠΎΠ²Π»Π΅Π½ΠΎ, ΡΠΎ ΠΡΡΠ»Π°Π½Π²ΠΏΠ»ΠΈΠ²Π°Ρ Π½Π° ΠΌΠ΅ΡΠ°Π±ΠΎΠ»ΡΡΠ½Ρ ΠΏΡΠΎΡΠ΅ΡΠΈ Π³ΡΠΈΠ±ΠΊΠΎΠ²ΠΎΡ ΠΊΠ»ΡΡΠΈΠ½ΠΈ.Background. The first half of the XXI-st century is characterized by increasing in the incidence of fungal infections. A wide range of spatial spreading of fungal infections, including dermatophytes and vaginal candidiasis (thrush), which can be explained by intensive migration and changing lifestyles in industrialized countries. This growth was not stopped after the introduction of new antifungal pharmaceuticals, most of which have side effects and are characterized by significant toxicity. In this regard, remains perspective development of new effective non-toxic natural antifungal drugs. Objective. The study of some potential mechanisms of domestic antimycotic Esulanum such as spectrum antimicrobial activity against gram-positive, gram-negative bacteria, microscopic fungi and yeast; influence on morphological characteristics and enzymatic activity of yeast. Methods. The study of antimicrobial activities was performed by preparation of serial dilutions. Study of the dynamics of cell death under the influence of Esulanum performed on models of culture Candida tropicalis. Cell morphology was studied using a light microscope. Results. It was shown that the Esulanum has a broad-spectrum activity against gram-positive and gram-negative bacteria, yeasts and fungies. Minimum inhibitory concentration of Esulanum against yeast (250-500 ug/ml), fungi (62,5-500 ug/ml) gram-positive (31,2-250 ug/ml) and gram-negative (62,5-250 ug/ml) bacteria was established. The study of morphological character of Candida tropicalis cells showed that the introduction of Esulanum into the culture medium in the fungicidal concentration led to changes in the morphogenesis of yeast cells, the degree of manifestation of which was due to a temporary agent. The appearance under the influence of the drug bumps and disruptions on the surface of yeast cells, as well as change their shape was probably due to a breach of the normal metabolism of culture, which was confirmed by the experimental data. Conclusions. The results indicate that Esulanum is a broad-spectrum drug, active not only in relation to micromycetes and yeast cultures, but also Gram-positive and Gram-negative bacteria. It was found that Esulanum affects the metabolic processes of the fungal cells. During the preclinical studies further research should be aimed at the study of the mechanism of action of the drug on cells of fungal cultures.ΠΡΠΎΠ±Π»Π΅ΠΌΠ°ΡΠΈΠΊΠ°. ΠΠ΅ΡΠ²Π°Ρ ΠΏΠΎΠ»ΠΎΠ²ΠΈΠ½Π° XXI Π²Π΅ΠΊΠ° Ρ
Π°ΡΠ°ΠΊΡΠ΅ΡΠΈΠ·ΠΎΠ²Π°Π»Π°ΡΡ Π·Π°ΠΌΠ΅ΡΠ½ΡΠΌ ΡΠΎΡΡΠΎΠΌ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π΅ΠΌΠΎΡΡΠΈ ΠΌΠΈΠΊΠΎΠ·Π°ΠΌΠΈ. Π¨ΠΈΡΠΎΠΊΠΎΠ΅ ΡΠ΅ΡΡΠΈΡΠΎΡΠΈΠ°Π»ΡΠ½ΠΎΠ΅ ΡΠ°ΡΠΏΡΠΎΡΡΡΠ°Π½Π΅Π½ΠΈΠ΅ ΠΏΠΎΠ»ΡΡΠΈΠ» ΡΡΠ΄ Π³ΡΠΈΠ±ΠΊΠΎΠ²ΡΡ
ΠΈΠ½ΡΠ΅ΠΊΡΠΈΠΉ, Π² ΡΠΎΠΌ ΡΠΈΡΠ»Π΅ Π΄Π΅ΡΠΌΠ°ΡΠΎΡΠΈΡΠΈΠΉ ΠΈ Π²Π°Π³ΠΈΠ½Π°Π»ΡΠ½ΡΠΉ ΠΊΠ°Π½Π΄ΠΈΠ΄ΠΎΠ· (ΠΌΠΎΠ»ΠΎΡΠ½ΠΈΡΠ°), ΡΡΠΎ ΠΌΠΎΠΆΠ½ΠΎ ΠΎΠ±ΡΡΡΠ½ΠΈΡΡ ΠΈΠ½ΡΠ΅Π½ΡΠΈΠ²Π½ΠΎΠΉ ΠΌΠΈΠ³ΡΠ°ΡΠΈΠ΅ΠΉ Π½Π°ΡΠ΅Π»Π΅Π½ΠΈΡ ΠΈ ΠΈΠ·ΠΌΠ΅Π½Π΅Π½ΠΈΠ΅ΠΌ ΠΎΠ±ΡΠ°Π·Π° ΠΆΠΈΠ·Π½ΠΈ Π² ΠΈΠ½Π΄ΡΡΡΡΠΈΠ°Π»ΡΠ½ΡΡ
ΡΡΡΠ°Π½Π°Ρ
. ΠΡΠΎΡ ΡΠΎΡΡ Π½Π΅ ΡΠ΄Π°Π»ΠΎΡΡ ΠΎΡΡΠ°Π½ΠΎΠ²ΠΈΡΡ ΠΈ ΠΏΠΎΡΠ»Π΅ Π²Π½Π΅Π΄ΡΠ΅Π½ΠΈΡ Π½ΠΎΠ²Π΅ΠΉΡΠΈΡ
Π°Π½ΡΠΈΠΌΠΈΠΊΠΎΡΠΈΡΠ΅ΡΠΊΠΈΡ
ΡΡΠ΅Π΄ΡΡΠ², Π±ΠΎΠ»ΡΡΠΈΠ½ΡΡΠ²ΠΎ ΠΈΠ· ΠΊΠΎΡΠΎΡΡΡ
ΠΈΠΌΠ΅ΡΡ ΠΏΠΎΠ±ΠΎΡΠ½ΠΎΠ΅ Π΄Π΅ΠΉΡΡΠ²ΠΈΠ΅ ΠΈ Ρ
Π°ΡΠ°ΠΊΡΠ΅ΡΠΈΠ·ΡΡΡΡΡ Π·Π½Π°ΡΠΈΡΠ΅Π»ΡΠ½ΠΎΠΉ ΡΠΎΠΊΡΠΈΡΠ½ΠΎΡΡΡΡ. Π ΡΠ²ΡΠ·ΠΈ Ρ ΡΡΠΈΠΌ ΠΏΠ΅ΡΡΠΏΠ΅ΠΊΡΠΈΠ²Π½ΠΎΠΉ ΠΎΡΡΠ°Π΅ΡΡΡ ΡΠ°Π·ΡΠ°Π±ΠΎΡΠΊΠ° Π½ΠΎΠ²ΡΡ
ΡΡΡΠ΅ΠΊΡΠΈΠ²Π½ΡΡ
ΠΈ Π½Π΅ΡΠΎΠΊΡΠΈΡΠ½ΡΡ
ΠΏΡΠΈΡΠΎΠ΄Π½ΡΡ
Π°Π½ΡΠΈΠΌΠΈΠΊΠΎΡΠΈΠΊΠΎΠ². Π¦Π΅Π»Ρ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ. ΠΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠ΅ Π½Π΅ΠΊΠΎΡΠΎΡΡΡ
ΠΏΠΎΡΠ΅Π½ΡΠΈΠ°Π»ΡΠ½ΡΡ
ΠΌΠ΅Ρ
Π°Π½ΠΈΠ·ΠΌΠΎΠ² Π΄Π΅ΠΉΡΡΠ²ΠΈΡ ΠΎΡΠ΅ΡΠ΅ΡΡΠ²Π΅Π½Π½ΠΎΠ³ΠΎ Π°Π½ΡΠΈΠΌΠΈΠΊΠΎΡΠΈΠΊΠ° ΠΡΡΠ»Π°Π½Π°, Π° ΠΈΠΌΠ΅Π½Π½ΠΎ ΡΠΏΠ΅ΠΊΡΡΠ° Π°Π½ΡΠΈΠΌΠΈΠΊΡΠΎΠ±Π½ΠΎΠΉ Π°ΠΊΡΠΈΠ²Π½ΠΎΡΡΠΈ Π² ΠΎΡΠ½ΠΎΡΠ΅Π½ΠΈΠΈ Π³ΡΠ°ΠΌΠΏΠΎΠ»ΠΎΠΆΠΈΡΠ΅Π»ΡΠ½ΡΡ
, Π³ΡΠ°ΠΌΠΎΡΡΠΈΡΠ°ΡΠ΅Π»ΡΠ½ΡΡ
Π±Π°ΠΊΡΠ΅ΡΠΈΠΉ, ΠΌΠΈΠΊΡΠΎΡΠΊΠΎΠΏΠΈΡΠ΅ΡΠΊΠΈΡ
Π³ΡΠΈΠ±ΠΎΠ² ΠΈ Π΄ΡΠΎΠΆΠΆΠ΅ΠΉ; Π²Π»ΠΈΡΠ½ΠΈΠ΅ Π½Π° ΠΌΠΎΡΡΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΈΠ΅ ΠΏΡΠΈΠ·Π½Π°ΠΊΠΈ ΠΈ ΡΠ΅ΡΠΌΠ΅Π½ΡΠ°ΡΠΈΠ²Π½ΡΡ Π°ΠΊΡΠΈΠ²Π½ΠΎΡΡΡ Π΄ΡΠΎΠΆΠΆΠ΅ΠΉ. ΠΠ΅ΡΠΎΠ΄ΠΈΠΊΠ° ΡΠ΅Π°Π»ΠΈΠ·Π°ΡΠΈΠΈ. ΠΠ·ΡΡΠ΅Π½ΠΈΠ΅ Π°Π½ΡΠΈΠΌΠΈΠΊΡΠΎΠ±Π½ΠΎΠΉ Π°ΠΊΡΠΈΠ²Π½ΠΎΡΡΠΈ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠ° ΠΏΡΠΎΠ²ΠΎΠ΄ΠΈΠ»ΠΈ ΠΌΠ΅ΡΠΎΠ΄ΠΎΠΌ ΡΠ΅ΡΠΈΠΉΠ½ΡΡ
ΡΠ°Π·Π²Π΅Π΄Π΅Π½ΠΈΠΉ. ΠΠ·ΡΡΠ΅Π½ΠΈΠ΅ Π΄ΠΈΠ½Π°ΠΌΠΈΠΊΠΈ Π³ΠΈΠ±Π΅Π»ΠΈ ΠΊΠ»Π΅ΡΠΎΠΊ ΠΏΠΎΠ΄ Π²Π»ΠΈΡΠ½ΠΈΠ΅ΠΌ ΠΡΡΠ»Π°Π½Π° ΠΏΡΠΎΠ²ΠΎΠ΄ΠΈΠ»ΠΈ Π½Π° ΠΌΠΎΠ΄Π΅Π»ΠΈ ΠΊΡΠ»ΡΡΡΡΡ Candida tropicalis. ΠΠ·ΠΌΠ΅Π½Π΅Π½ΠΈΡ Π² ΠΌΠΎΡΡΠΎΠ»ΠΎΠ³ΠΈΠΈ ΠΊΠ»Π΅ΡΠΎΠΊ ΠΈΠ·ΡΡΠ°Π»ΠΈ Ρ ΠΏΠΎΠΌΠΎΡΡΡ ΡΠ²Π΅ΡΠΎΠ²ΠΎΠ³ΠΎ ΠΌΠΈΠΊΡΠΎΡΠΊΠΎΠΏΠ°. Π Π΅Π·ΡΠ»ΡΡΠ°ΡΡ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ. ΠΠΎΠΊΠ°Π·Π°Π½ΠΎ, ΡΡΠΎ ΠΡΡΠ»Π°Π½ ΡΠ²Π»ΡΠ΅ΡΡΡ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠΌ ΡΠΈΡΠΎΠΊΠΎΠ³ΠΎ ΡΠΏΠ΅ΠΊΡΡΠ° Π΄Π΅ΠΉΡΡΠ²ΠΈΡ, Π°ΠΊΡΠΈΠ²Π½ΡΠΌ ΠΎΡΠ½ΠΎΡΠΈΡΠ΅Π»ΡΠ½ΠΎ Π³ΡΠ°ΠΌΠΏΠΎΠ»ΠΎΠΆΠΈΡΠ΅Π»ΡΠ½ΡΡ
ΠΈ Π³ΡΠ°ΠΌΠΎΡΡΠΈΡΠ°ΡΠ΅Π»ΡΠ½ΡΡ
Π±Π°ΠΊΡΠ΅ΡΠΈΠΉ, Π΄ΡΠΎΠΆΠΆΠ΅ΠΉ ΠΈ ΠΏΠ»Π΅ΡΠ½Π΅Π²ΡΡ
Π³ΡΠΈΠ±ΠΎΠ². Π£ΡΡΠ°Π½ΠΎΠ²Π»Π΅Π½Ρ ΠΌΠΈΠ½ΠΈΠΌΠ°Π»ΡΠ½ΡΠ΅ ΠΏΠΎΠ΄Π°Π²Π»ΡΡΡΠΈΠ΅ ΠΊΠΎΠ½ΡΠ΅Π½ΡΡΠ°ΡΠΈΠΈ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠ° ΠΎΡΠ½ΠΎΡΠΈΡΠ΅Π»ΡΠ½ΠΎ Π΄ΡΠΎΠΆΠΆΠ΅ΠΉ (250-500 ΠΌΠΊΠ³/ΠΌΠ»), Π³ΡΠΈΠ±ΠΎΠ² (62,5-500 ΠΌΠΊΠ³/ΠΌΠ»), Π³ΡΠ°ΠΌΠΏΠΎΠ»ΠΎΠΆΠΈΡΠ΅Π»ΡΠ½ΡΡ
(31,2-250 ΠΌΠΊΠ³/ΠΌΠ») ΠΈ Π³ΡΠ°ΠΌΠΎΡΡΠΈΡΠ°ΡΠ΅Π»ΡΠ½ΡΡ
(62,5-250 ΠΌΠΊΠ³/ΠΌΠ») Π±Π°ΠΊΡΠ΅ΡΠΈΠΉ. ΠΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ ΠΌΠΎΡΡΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΈΡ
ΠΏΡΠΈΠ·Π½Π°ΠΊΠΎΠ² ΠΊΠ»Π΅ΡΠΎΠΊ Candida tropicalis ΠΏΠΎΠΊΠ°Π·Π°Π»ΠΈ, ΡΡΠΎ Π²Π½Π΅ΡΠ΅Π½ΠΈΠ΅ ΠΡΡΠ»Π°Π½Π° Π² ΠΏΠΈΡΠ°ΡΠ΅Π»ΡΠ½ΡΡ ΡΡΠ΅Π΄Ρ Π² ΡΡΠ½Π³ΠΈΡΠΈΠ΄Π½ΠΎΠΉ ΠΊΠΎΠ½ΡΠ΅Π½ΡΡΠ°ΡΠΈΠΈ ΠΏΡΠΈΠ²ΠΎΠ΄ΠΈΠ»ΠΎ ΠΊ ΠΈΠ·ΠΌΠ΅Π½Π΅Π½ΠΈΡ ΠΌΠΎΡΡΠΎΠ³Π΅Π½Π΅Π·Π° ΠΊΠ»Π΅ΡΠΎΠΊ Π΄ΡΠΎΠΆΠΆΠ΅ΠΉ, ΡΡΠ΅ΠΏΠ΅Π½Ρ ΠΏΡΠΎΡΠ²Π»Π΅Π½ΠΈΡ ΠΊΠΎΡΠΎΡΠΎΠ³ΠΎ Π±ΡΠ»Π° ΠΎΠ±ΡΡΠ»ΠΎΠ²Π»Π΅Π½Π° Π²ΡΠ΅ΠΌΠ΅Π½Π½ΡΠΌ ΡΠ°ΠΊΡΠΎΡΠΎΠΌ. ΠΠΎΡΠ²Π»Π΅Π½ΠΈΠ΅ ΠΏΠΎΠ΄ Π²Π»ΠΈΡΠ½ΠΈΠ΅ΠΌ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠ° Π²ΡΠΏΡΠΊΠ»ΠΎΡΡΠ΅ΠΉ ΠΈ ΡΠ°Π·ΡΡΠ²ΠΎΠ² Π½Π° ΠΏΠΎΠ²Π΅ΡΡ
Π½ΠΎΡΡΠΈ ΠΊΠ»Π΅ΡΠΎΠΊ Π΄ΡΠΎΠΆΠΆΠ΅ΠΉ, Π° ΡΠ°ΠΊΠΆΠ΅ ΠΈΠ·ΠΌΠ΅Π½Π΅Π½ΠΈΠ΅ ΠΈΡ
ΡΠΎΡΠΌΡ, Π²Π΅ΡΠΎΡΡΠ½ΠΎ, Π±ΡΠ»ΠΈ ΡΠ»Π΅Π΄ΡΡΠ²ΠΈΠ΅ΠΌ Π½Π°ΡΡΡΠ΅Π½ΠΈΡ Π½ΠΎΡΠΌΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΠΎΠ±ΠΌΠ΅Π½Π° Π²Π΅ΡΠ΅ΡΡΠ² ΠΊΡΠ»ΡΡΡΡΡ, ΡΡΠΎ ΠΏΠΎΠ΄ΡΠ²Π΅ΡΠ΄ΠΈΠ»ΠΎΡΡ ΡΠΊΡΠΏΠ΅ΡΠΈΠΌΠ΅Π½ΡΠ°Π»ΡΠ½ΡΠΌΠΈ Π΄Π°Π½Π½ΡΠΌΠΈ. ΠΡΠ²ΠΎΠ΄Ρ. ΠΠΎΠ»ΡΡΠ΅Π½Π½ΡΠ΅ ΡΠ΅Π·ΡΠ»ΡΡΠ°ΡΡ ΡΠ²ΠΈΠ΄Π΅ΡΠ΅Π»ΡΡΡΠ²ΡΡΡ, ΡΡΠΎ ΠΡΡΠ»Π°Π½ ΡΠ²Π»ΡΠ΅ΡΡΡ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠΌ ΡΠΈΡΠΎΠΊΠΎΠ³ΠΎ ΡΠΏΠ΅ΠΊΡΡΠ° Π΄Π΅ΠΉΡΡΠ²ΠΈΡ, Π°ΠΊΡΠΈΠ²Π½ΡΠΌ Π½Π΅ ΡΠΎΠ»ΡΠΊΠΎ ΠΏΠΎ ΠΎΡΠ½ΠΎΡΠ΅Π½ΠΈΡ ΠΊ ΠΌΠΈΠΊΡΠΎΠΌΠΈΡΠ΅ΡΠ°ΠΌ ΠΈ Π΄ΡΠΎΠΆΠΆΠ΅Π²ΡΠΌ ΠΊΡΠ»ΡΡΡΡΠ°ΠΌ, Π½ΠΎ ΠΈ ΠΊ Π³ΡΠ°ΠΌΠΏΠΎΠ»ΠΎΠΆΠΈΡΠ΅Π»ΡΠ½ΡΠΌ ΠΈ Π³ΡΠ°ΠΌΠΎΡΡΠΈΡΠ°ΡΠ΅Π»ΡΠ½ΡΠΌ Π±Π°ΠΊΡΠ΅ΡΠΈΡΠΌ. Π£ΡΡΠ°Π½ΠΎΠ²Π»Π΅Π½ΠΎ, ΡΡΠΎ ΠΡΡΠ»Π°Π½ Π²Π»ΠΈΡΠ΅Ρ Π½Π° ΠΌΠ΅ΡΠ°Π±ΠΎΠ»ΠΈΡΠ΅ΡΠΊΠΈΠ΅ ΠΏΡΠΎΡΠ΅ΡΡΡ Π³ΡΠΈΠ±Π½ΠΎΠΉ ΠΊΠ»Π΅ΡΠΊΠΈ. ΠΡΠΈ ΠΏΡΠΎΠ²Π΅Π΄Π΅Π½ΠΈΠΈ Π΄ΠΎΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΈΡ
ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠΉ Π΄Π°Π»ΡΠ½Π΅ΠΉΡΠ°Ρ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°ΡΠ΅Π»ΡΡΠΊΠ°Ρ ΡΠ°Π±ΠΎΡΠ° Π΄ΠΎΠ»ΠΆΠ½Π° Π±ΡΡΡ Π½Π°ΠΏΡΠ°Π²Π»Π΅Π½Π° Π½Π° ΠΈΠ·ΡΡΠ΅Π½ΠΈΠ΅ ΠΌΠ΅Ρ
Π°Π½ΠΈΠ·ΠΌΠ° Π΄Π΅ΠΉΡΡΠ²ΠΈΡ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠ° Π½Π° ΠΊΠ»Π΅ΡΠΊΠΈ Π³ΡΠΈΠ±Π½ΡΡ
ΠΊΡΠ»ΡΡΡΡ
Π€ΠΈΠ±ΡΠΎΠ·ΠΈΡΡΡΡΠ°Ρ Π°ΡΡΡΠΎΠΏΠ°ΡΠΈΡ ΠΏΡΠΈ ΡΠ²Π΅Π½ΠΈΠ»ΡΠ½ΠΎΠΉ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠΈ
The group of scleroderma diseases includes a number of clinical entities, the main symptom of which is skin tightening. Scleroderma is a prominent example of these diseases, characterized by excessive synthesis and deposition of collagen in organs and tissues. A patient with juvenile systemic scleroderma with induration of the skin and underlying tissues, and persistent contractures of large joints since childhood, is described. This clinical example illustrates disease course peculiarities and differential diagnosis of systemic and limited (focal) scleroderma and scleroderma-like conditions in pediatric patients. The feasibility of pathogenetic therapy aimed at improving patient's the quality of life with formed disease phenotype is shown.Π Π³ΡΡΠΏΠΏΠ΅ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΡΠ΅ΡΠΊΠΈΡ
Π±ΠΎΠ»Π΅Π·Π½Π΅ΠΉ ΠΎΡΠ½ΠΎΡΠΈΡΡΡ ΡΡΠ΄ Π½ΠΎΠ·ΠΎΠ»ΠΎΠ³ΠΈΠΉ, ΠΎΡΠ½ΠΎΠ²Π½ΡΠΌ ΠΏΡΠΈΠ·Π½Π°ΠΊΠΎΠΌ ΠΊΠΎΡΠΎΡΡΡ
ΡΠ²Π»ΡΠ΅ΡΡΡ ΡΠΏΠ»ΠΎΡΠ½Π΅Π½ΠΈΠ΅ ΠΊΠΎΠΆΠΈ. Π‘ΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΡ β ΡΡΠΊΠΈΠΉ ΠΏΡΠ΅Π΄ΡΡΠ°Π²ΠΈΡΠ΅Π»Ρ ΡΡΠΈΡ
Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠΉ, Ρ
Π°ΡΠ°ΠΊΡΠ΅ΡΠΈΠ·ΡΡΡΠΈΡ
ΡΡ ΠΈΠ·Π±ΡΡΠΎΡΠ½ΡΠΌ ΡΠΈΠ½ΡΠ΅Π·ΠΎΠΌ ΠΈ ΠΎΡΠ»ΠΎΠΆΠ΅Π½ΠΈΠ΅ΠΌ ΠΊΠΎΠ»Π»Π°Π³Π΅Π½Π° Π² ΠΎΡΠ³Π°Π½Π°Ρ
ΠΈ ΡΠΊΠ°Π½ΡΡ
. ΠΠΏΠΈΡΠ°Π½Π° Π±ΠΎΠ»ΡΠ½Π°Ρ ΡΠ²Π΅Π½ΠΈΠ»ΡΠ½ΠΎΠΉ ΡΠΈΡΡΠ΅ΠΌΠ½ΠΎΠΉ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠ΅ΠΉ Ρ ΠΈΠ½Π΄ΡΡΠ°ΡΠΈΠ΅ΠΉ ΠΊΠΎΠΆΠΈ ΠΈ ΠΏΠΎΠ΄Π»Π΅ΠΆΠ°ΡΠΈΡ
ΡΠΊΠ°Π½Π΅ΠΉ, Π° ΡΠ°ΠΊΠΆΠ΅ ΡΡΠΎΠΉΠΊΠΈΠΌΠΈ ΠΊΠΎΠ½ΡΡΠ°ΠΊΡΡΡΠ°ΠΌΠΈ ΠΊΡΡΠΏΠ½ΡΡ
ΡΡΡΡΠ°Π²ΠΎΠ² Ρ Π΄Π΅ΡΡΠΊΠΎΠ³ΠΎ Π²ΠΎΠ·ΡΠ°ΡΡΠ°. ΠΠ° ΡΡΠΎΠΌ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΎΠΌ ΠΏΡΠΈΠΌΠ΅ΡΠ΅ ΡΠ°ΡΡΠΌΠ°ΡΡΠΈΠ²Π°ΡΡΡΡ ΠΎΡΠΎΠ±Π΅Π½Π½ΠΎΡΡΠΈ ΡΠ΅ΡΠ΅Π½ΠΈΡ ΠΈ Π΄ΠΈΡΡΠ΅ΡΠ΅Π½ΡΠΈΠ°Π»ΡΠ½Π°Ρ Π΄ΠΈΠ°Π³Π½ΠΎΡΡΠΈΠΊΠ° ΡΠΈΡΡΠ΅ΠΌΠ½ΠΎΠΉ ΠΈ ΠΎΠ³ΡΠ°Π½ΠΈΡΠ΅Π½Π½ΠΎΠΉ (ΠΎΡΠ°Π³ΠΎΠ²ΠΎΠΉ) ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠΈ ΠΈ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΎΠΏΠΎΠ΄ΠΎΠ±Π½ΡΡ
ΡΠΎΡΡΠΎΡΠ½ΠΈΠΉ Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Π΄Π΅ΡΡΠΊΠΎΠ³ΠΎ Π²ΠΎΠ·ΡΠ°ΡΡΠ°. ΠΠΎΠΊΠ°Π·Π°Π½Ρ Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎΡΡΠΈ ΠΏΠΎΠ΄Π±ΠΎΡΠ° ΠΏΠ°ΡΠΎΠ³Π΅Π½Π΅ΡΠΈΡΠ΅ΡΠΊΠΎΠΉ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ, Π½Π°ΠΏΡΠ°Π²Π»Π΅Π½Π½ΠΎΠΉ Π½Π° ΡΠ»ΡΡΡΠ΅Π½ΠΈΠ΅ ΠΊΠ°ΡΠ΅ΡΡΠ²Π° ΠΆΠΈΠ·Π½ΠΈ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ ΡΠΆΠ΅ ΡΡΠΎΡΠΌΠΈΡΠΎΠ²Π°Π½Π½ΡΠΌ ΡΠ΅Π½ΠΎΡΠΈΠΏΠΎΠΌ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡ
ΠΠΎΠΌΠΏΠ»Π΅ΠΊΡΠ½Π°Ρ ΡΠ΅ΡΠ°ΠΏΠΈΡ ΡΠΎΡΡΠ΄ΠΈΡΡΡΡ Π½Π°ΡΡΡΠ΅Π½ΠΈΠΉ Ρ Π±ΠΎΠ»ΡΠ½ΡΡ ΡΠΈΡΡΠ΅ΠΌΠ½ΠΎΠΉ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠ΅ΠΉ
Scleroderma systematica (SDS) is a disease in which vascular diseases underlie the pathogenesis and presented by diverse clinical manifestations. Raynaud's syndrome and digital ulceration are the most common symptom of the diseases, which influences the quality of life in patients and requires continuous drug therapy. The paper discusses the recent European guidelines for the management of this manifestation of SDS. The proposed recommendations cannot unfortunately be realized in full measure now due to the unavailability of some drugs. The authors give their clinical experience with therapy for the vascular manifestations of SDS.Π‘ΠΈΡΡΠ΅ΠΌΠ½Π°Ρ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΡ (Π‘Π‘Π) - Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠ΅, ΠΏΡΠΈ ΠΊΠΎΡΠΎΡΠΎΠΌ ΡΠΎΡΡΠ΄ΠΈΡΡΡΠ΅ Π½Π°ΡΡΡΠ΅Π½ΠΈΡ Π»Π΅ΠΆΠ°Ρ Π² ΠΎΡΠ½ΠΎΠ²Π΅ ΠΏΠ°ΡΠΎΠ³Π΅Π½Π΅Π·Π° ΠΈ ΠΏΡΠ΅Π΄ΡΡΠ°Π²Π»Π΅Π½Ρ ΡΠ°Π·Π½ΠΎΠΎΠ±ΡΠ°Π·Π½ΡΠΌΠΈ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΈΠΌΠΈ ΠΏΡΠΎΡΠ²Π»Π΅Π½ΠΈΡΠΌΠΈ. Π‘ΠΈΠ½Π΄ΡΠΎΠΌ Π Π΅ΠΉΠ½ΠΎ ΠΈ ΠΎΠ±ΡΠ°Π·ΠΎΠ²Π°Π½ΠΈΠ΅ Π΄ΠΈΠ³ΠΈΡΠ°Π»ΡΠ½ΡΡ
ΡΠ·Π² - Π½Π°ΠΈΠ±ΠΎΠ»Π΅Π΅ ΡΠ°ΡΡΡΠΉ ΡΠΈΠΌΠΏΡΠΎΠΌ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡ, Π²Π»ΠΈΡΡΡΠΈΠΉ Π½Π° ΠΊΠ°ΡΠ΅ΡΡΠ²ΠΎ ΠΆΠΈΠ·Π½ΠΈ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² ΠΈ ΡΡΠ΅Π±ΡΡΡΠΈΠΉ ΠΏΠΎΡΡΠΎΡΠ½Π½ΠΎΠΉ Π»Π΅ΠΊΠ°ΡΡΡΠ²Π΅Π½Π½ΠΎΠΉ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ. Π ΡΡΠ°ΡΡΠ΅ ΠΎΠ±ΡΡΠΆΠ΄Π°ΡΡΡΡ Π½Π΅Π΄Π°Π²Π½ΠΎ ΠΎΠΏΡΠ±Π»ΠΈΠΊΠΎΠ²Π°Π½Π½ΡΠ΅ Π΅Π²ΡΠΎΠΏΠ΅ΠΉΡΠΊΠΈΠ΅ ΡΠ΅ΠΊΠΎΠΌΠ΅Π½Π΄Π°ΡΠΈΠΈ ΠΏΠΎ Π»Π΅ΡΠ΅Π½ΠΈΡ ΡΡΠΎΠ³ΠΎ ΠΏΡΠΎΡΠ²Π»Π΅Π½ΠΈΡ Π‘Π‘Π. ΠΡΠΏΠΎΠ»Π½Π΅Π½ΠΈΠ΅ ΠΏΡΠ΅Π΄Π»ΠΎΠΆΠ΅Π½Π½ΡΡ
ΡΠ΅ΠΊΠΎΠΌΠ΅Π½Π΄Π°ΡΠΈΠΉ Π² ΠΏΠΎΠ»Π½ΠΎΠΌ ΠΎΠ±ΡΠ΅ΠΌΠ΅, ΠΊ ΡΠΎΠΆΠ°Π»Π΅Π½ΠΈΡ, Π² Π½Π°ΡΡΠΎΡΡΠ΅Π΅ Π²ΡΠ΅ΠΌΡ Π½Π΅Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎ ΠΈΠ·-Π·Π° Π½Π΅Π΄ΠΎΡΡΡΠΏΠ½ΠΎΡΡΠΈ Π½Π΅ΠΊΠΎΡΠΎΡΡΡ
ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠ². ΠΠ²ΡΠΎΡΠ°ΠΌΠΈ ΠΏΡΠΈΠ²ΠΎΠ΄ΠΈΡΡΡ ΡΠΎΠ±ΡΡΠ²Π΅Π½Π½ΡΠΉ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΈΠΉ ΠΎΠΏΡΡ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ ΡΠΎΡΡΠ΄ΠΈΡΡΡΡ
ΠΏΡΠΎΡΠ²Π»Π΅Π½ΠΈΠΉ Π‘Π‘Π
ΠΠΏΡΡ ΠΏΡΠΈΠΌΠ΅Π½Π΅Π½ΠΈΡ Π°ΠΊΡΠΎΠ²Π΅Π³ΠΈΠ½Π° ΠΏΡΠΈ ΠΎΡΠ°Π³ΠΎΠ²ΠΎΠΉ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠΈ
SCLERODERMA SYSTEMATICA WITH INTERSTITIAL LUNG LESION: COMPARATIVE CLINICAL CHARACTERISTICSWITH PATIENTS WITHOUT LUNG LESION
Objective. To compare disease history data and clinical and laboratory parameters in patients with scleroderma systematica (SDS) with high-resolution computed tomography (HRCT)-verified interstitial lung lesion (ILL) versus those without lung involvement. Subjects and methods. An examination was made in 138 patients with SDS who had been consecutively admitted in 2006-2008, female/male ratio, 124 : 14; limited : diffuse : mixed forms, 78 : 40 : 20; mean age, 47Β±13 years; median disease duration, 6 (2.5 11) years. The history data (occupational hazards, smoking, respiratory diseases) and clinical manifestations of SDS and laboratory data were studied. The diagnosis of ILL was established on the basis of chest HRCT. Results. According to HRCT data, the signs of varying ILL were found in 82% of the patients with SDS. The duration of SDS was similar in the patients with and without lung involvement; but the latter were younger at the time of disease onset. There were no significant differences between the groups compared in history data, clinical forms of SDS, the frequency of involvement of visceral organs and systems. Crepitation was heard only in the patients with ILL. The frequency of respiratory manifestations increased with a larger number of the involved lung segments. The prevalence of ILL was found to be positively correlated with age at the onset of SDS (r=0.29;
STUDY OF THE EFFICIENCY AND SAFETY OF MYCOPHENOLATE MOFETIL THERAPY IN PATIENTSWITH SYSTEMIC SCLERODERMA
Interstitial lung disease (ILD) is one of the major causes of death in systemic scleroderma (SSD). Treatment of these patients remains difficult and controversial. Mycophenolate mofetil (MPM) has been in vitro shown to inhibit overproduction of type I collagen and hence may be effective against SSD. Objective: to study the efficiency and safety of MPM therapy in patients with SSD and clinically relevant ILD in an open-label prospective study. Subjects and methods. Ten patients with SSD (7 and 3 with its diffuse and limited forms, respectively) and ILD were given MPM in combination with glucocorticoids (mean daily dose was 10+4 mg). The mean MPM therapy duration was 11.4+1.3 months. The Rodnan total skin thickness score, flexion index, forced vital capacity (FVC), diffusing capacity of the lung for carbon monoxide (DLCO), and European Scleroderma Study Group (EScSG) activity index were estimated and a 6-minute walk test (6MWT) was carried out before and after MPM therapy. Results. After therapy, the whole group showed a significant reduction in skin scores from 12.9+9.8 to 5.6+3.2 (p=0.036) and EScSG from 3.9+1.4 to 2.25+1.03 (p=0.015) and an increase in exercise tolerance from 446+155 to 535+78 m (p=0.03) as evidenced by 6MWT. The degree of flexion contractures decreased from 15+21 to 3.7+11.3 mm (p>0.05). FVC (77.8+18.7% versus 73.8+11.3%) and DLCO (45+14.4% versus 42+16.4%) were significantly unchanged. A 10% or more clinically significant fall was noted in FVC and DLCO in 3 and 1 patients, respectively. In the remaining patients, the lung functional test results remained stable. MPM tolerability was satisfactory. All the patients completed their course of treatment. Conclusion. Stabilization of lung function with higher exercise tolerance and significantly reduced skin density allow therapy with MPM in combination with low-dose glucocorticoids to be regarded as an effective and well-tolerated treatment in patients with ILD in the presence of SS
Comparative Analysis of Blood and Marrow Cellular Structure of White Mice Infected With Bacillus Anthracis Different Genotypes
Background. Detection of the pathogenicity factors is one of the actual directions in studying of the anthrax causative agent. First of all, it includes the presence of tripartite toxin, capsule and the related structural and regulatory genes. Their presence or absence is defined by structural changes in cellular structure of blood and immunocomplex organs of a macroorganism.Objective: To conduct a comparative analysis of subpopulational structure of marrow and peripheral blood cells at anthrax infection in experimental animals and to trace dynamics of the infectious process caused by Bacillus anthracis of different genotypes.Methods. Comparative examination of subpopulational structure of red marrow and peripheral blood cells of white mice in dynamics of the infectious process caused by B. anthracis of different genotypes was conducted. Also, neutrophil maturing index and leucoerythrocytic correlation were calculated in red marrow. Degree of manifestation of neutrophil pathological granularity was revealed in peripheral blood smears stained by the Freifeldβs method. Statistical processing of the received data was conducted using the computer program βStatisticsβ, version 6 (Novosibirsk). Authentic results were considered if the error probability was less than 0.05 (Ρ < 0.05) in relation to the control.Results. It is revealed that alterations in white mice infected by B. anthracis strains with the same set of plasmids are significantly various.Conclusion. The resulted data permit to assume that the distinction of pathological alterations in peripheral blood and red marrow is probably connected with the other factors of the pathogen invasiveness like enzymatic activity, ability to adhesion and many others
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