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Dear Colleagues!. We are glad to present the 6th Edition of Standards of Diabetes Care. These evidence-based guidelines were designed to standardize and facilitate diabetes care in all regions of the Russian Federation. The Standards are updated on the regular basis to incorporate new data and relevant recommendations from national and international clinical societies, including World Health Organization Guidelines (WHO, 2011), International Diabetes Federation (IDF, 2011), American Diabetes Association (ADA, 2013), American Association of Clinical Endocrinologists (AACE, 2009), International Society for Pediatric and Adolescent Diabetes (ISPAD, 2009) and Russian Association of Endocrinologists (RAE, 2011, 2012). Current edition of the ?Standards? also integrates results of completed randomized clinical trials (ADVANCE, ACCORD, VADT, UKPDS, etc.), as well as findings from the national studies of diabetes mellitus (DM), conducted in close partnership with a number of Russian hospitals. Latest data indicates that prevalence of DM increased during the last decade more than two-fold, reaching some 371 million patients by 2013. According to the current estimation by the International Diabetes Federation, every tenth inhabitant of the planet will be suffering from DM by 2030. These observations resulted in the UN Resolution 61/225 passed on 20.12.2006 that encouraged all Member States ?to develop national policies for the prevention, treatment and care of diabetes?. Like many other countries, Russian Federation experiences a sharp rise in the prevalence of DM. According to Russian State Diabetes Register, there are at least 3.799 million patients with DM in this country. However, the epidemiological survey conducted by the Federal Endocrinology Research Centre during 2002-2010 suggests that actual prevalence is 3 to 4 times greater than the officially recognized and, by this estimate, amounts to 9-10 million persons, comprising 7% of the national population. . Severe consequences of the global pandemics of DM include its vascular complications: nephropathy, retinopathy, coronary, cerebral and peripheral vascular disease. These conditions are responsible for the majority of cases of diabetes-related disability and death. . Current edition of the ?Standards? emphasizes the patient-oriented approach in making decisions on therapeutic goals, such as levels of glycaemia and blood pressure. It also features updated guidelines on the management of vascular complications and new RAE position statement on gestational diabetes, produced in collaboration with Russian Association of Obstetrics and Gynecology. . This text represents a consensus by the absolute majority of national experts, achieved through a number of fruitful discussions held at national meetings and forums. These guidelines are intended for endocrinologists and diabetologists, primary care physicians, cardiologists and other medical professionals involved in prevention and treatment of diabetes mellitus.

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338 Congenital CCR have been r e p r t e d i n over 50 p t i e n t s d i v i d e d i n t o a f amilia1 a n d de n w o c a t e g o r i e s. Between 1982 and 1986 we saw three u n r e l a t e d c h i l d r e n w i t h de n w o (p r e n t s had normal karyotyyzs) unbalanced congeni tit1 CCR. Lynpfiocyte and f i b r o h l a s t k a r y o t y ~ e s were s t u d i e d u s i n g GI Q, C, R a n d NOR banding. P a t i e n t 1. A premature (33 weeks) newborn f e n a l e (BWT 2 kg, HT 3 8 can, OEC 3 0 cm] had c l e f t l i p a n d p a l a t e , t e l e c a n t h u s , chaanal atresia and e c t r o d a c t y l y uf hands and f e e t. Tbe karyotyFe s h a r e d t (2 ; 5) (q33;@2), t (3 ; l l) (@7;p11.2) a n d del(l.3) (ql2q14). 'Ihe f i v e d e r i v a t i v e c h r a n m e s i m p l i e d s i x breaks. P a t i e n t 2. A 3 months o l d f e m a l e (IWT2.3 kg, IIT42.5 an, O K 3 1 a t) p r o d u c t of a term pregnancy w i t h oligohydrannios a n d nonnal placenta, had cleft l i p and p a l a t e and m i l d f a c i a l dysnorphy. Subsequently microceghaly a n d slav grcwth w e r e noted. ?he k a r y o t y p s h a r e d s i x d e r i v a t i v e chranosomes: t (2 ; 3) (q33.2;p22.5), t(7;18) (q32;q12.2), two i n v (2) , (p12@4) and (~$31~~33) and d e l (l 0) (p U) implying nim breaks. P a t i e n t 3. A 1 2 y e a r o l d male w i t h IQ o f 40, HTL 3 r d % tile, s c o l i o a i s and m i l d dysnor& had BWT 3 kg, HT 48.5 an and unrenarkahle n e o m t a l period. His k a r y o t y ~ e shcwed seven d e r i v a t i v e chrcrnosnnes: t (2 ; l l) (@l;p14), t(5;15) (p l 1 ; q l l) , t (6 ; l l) (p23:p14), t (6 ; 2 0) (p23;p13), d e l (6) (q26) and d e l (1 4) (a 4 1 implying e i @ t breaks. 'J3e phenanenon of c o n g e n i t a l CCR i n man a p r s r e a l a n d the p e d i a t r i c i a n should know abut it. COCKAYNE SYNDROME (CS) MASQUERADING AS SECKEL SYNDROME (SS). James M. Lewis, (Spon. by Ruth C. H a r r i s). Marshall U n i v e r s i t y School of Medicine, 339 Department of P e d i a t r i c s , Huntington, WV. Our p a t i e n t presented a t 3 months of age w i t h m i-crocephaly, nystagmus, o p t i c atrophy and development-a l delay. B i r t h weight was 2.7 kg. At 11 months he was l a b e l e d a s SS on t h e b a s i s of microcephaly, s h o r t s t a t u r e and f a c i a l feat u r e s. Over t h e next 4 y e a r s he developed marked growth f a i l u r e , mental r e t a r d a t i o n , a t a x i a , sunken eyes, p h o t o s e n s i t i v i t y , and d e n t a l c a r i e s. Kyphosis, c o n t r a c t u r e s , l e n s o p a c i t i e s and long e x t r e m i t i e s w i t h shortened t r u n k wore p r e s e n t a t t h e time of d e a t h a t 8 y e a r s of age. We i d e n t i f i e d 132 CS c a s e r e p o r t s i n t h e world l i t e r a t u r e comprised of 72 males and 56 females (4 un-d e s c r i b e d). Mean (5. D .) b i r t h weight was 2.9(%.6)kg. Mean age a t d e a t h (27 c a s e s) was 14(+8) y e a r s. The most common neurologic a b n o r m a l i t i e s noted were mental r e t a r d a t i o n (90%), mic...

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о мнению выдающегося современного кардиолога E. Braunwald, три важнейших сердечно-сосудистых эпидемии определяют судьбу человечества в XXI веке (1997 г.). Это: эпидемия застойной сердечной недостаточности, эпидемия сахарного диабета (СД) и увеличение числа больных, страдающих мерцательной аритмией (МА), приобретшее в по-следнее время характер эпидемии.Первым современным исследованием, обратившим вни-мание на МА как на один из важнейших сердечно-сосудистых показателей, определяющих результаты интенсивной терапии больных СД 2 типа (СД2), стало исследование ADVANCE (The Action in Diabetes and Vascular disease preterAx and diamicroN -MR Controlled Evalution).МА, впервые описанная в 1909 г., в настоящее время яв-ляется наиболее часто встречаемым нарушением сердечного ритма. МА характеризуется потерей способности предсердий миокарда к координированному сокращению.Значимость МА как проблемы общественного здравоохране-ния определяется ее тесной взаимосвязью с увеличенным риском возникновения нарушений мозгового кровообращения и разви-тием тяжелой сердечной недостаточности -двух наиболее тяже-лых и экономически-затратных сердечно-сосудистых осложнений, определяющих продолжительность жизни подобного рода лиц.По данным Фремингемского исследования, пациенты с МА имеют в 1,5-2 раза более высокий риск годовой смертности по сравнению с общей популяцией. Ежегодно у 5% лиц с диаг-ностированной МА «неклапанного происхождения» возникают нарушения мозгового кровообращения. Это в 2-7 раз чаще, чем у лиц без МА. В результате МА является причиной от 75 000 до 100 000 эмболических инсультов в год [1].Начиная с 2001-2004 гг., работы, посвященные изучению МА, начинают описывать эту патологию как новую сердечно-сосудистую эпидемию [2,3,4]. Указывается, что за последние 50 лет частота распространения МА возросла в 2,5 раза.В настоящее время МА страдают около 2,3 млн чело-век в США или около 1% всего населения страны. Ее частота увеличивается с возрастом, и среди лиц старше 80 лет ею стра-дают не менее 9% населения. Основываясь на данных переписи США, прогноз роста количества больных, страдающих так на-зываемыми «неклапанными» формами МА представляется сле-дующим: в 2004 г. с подобным диагнозом было зафиксировано 2,3 млн человек, к 2020 г. прогнозируется рост до 3,3 млн человек и к 2050 г. -5,6 млн человек (рис. 1) [2].Исследователи из клиники Мayo считают, правда, что этот прогноз существенно недооценивает интенсивность реального роста этого заболевания. По данным этой знаменитой клиники, расположенной в г. Рочестере штата Миннесота, реальная за-болеваемость «неклапанными» формами МА в этой местности за последние 30 лет возросла в три раза, если учитывать ее уровень в конкретных возрастных категориях.При этом отмечается непрерывное нарастание клиниче-ской тяжести этого заболевания. За последние 15 лет частота госпитализаций по поводу МА возросла примерно в 2-3 раза. Так, по поводу собственно МА госпитализация возросла с 154 086 до 376 487случаев, и с 787 750 случаев до 2 283 673 случаев по по-воду МА на фоне других заболеваний (рис 2) [5].

Cardiovascular diseases (CVD) occupy a leading place in the structure of non-infectious pathology in adults and are the main cause of early disability and premature death in most economically developed countries. According to recent epidemiological studies, there has been a significant increase in both high normal blood pressure (BP) and arterial hypertension (AH) among children and adolescents. This applies to the greatest extent to adolescent children, while AH is detected from 4 to 22% of those surveyed, depending on the age of the surveyed and the selected criteria.

Aim. To identify profibrogenic mediators, markers of endothelial dysfunction and hemostasis in patients with diabetes mellitus (DM) and chronickidney disease (CKD). Materials and methods. The study included 120 patients with DM and 20 age-matched normotensive subjects without DM showing the glomerularfiltration rate (GFR) > 60 ml/min/1.73 m3. Four groups of patients were distinguished: 1 - DM2 patients without renal pathology (n=33), 2 - DM2 patients with diabetic nephropathy (n=24), 3 - DM2 patients with ischemic nephropathy (IN) (n=33) verified by contrast visualization techniques(multispiral CM of abdominal aorta and renal arteries, abdominal angiography of renal arteries or MR angiography of renal arteries and abdominal aorta), 4 - DM1 patients with DN (n=30). Clinical examination included assessment of complaints, analysis of medical history of the main diseaseand concomitant disorders, determination of the main clinical and biochemical characteristics of blood and urine, measurement of НbА1с and 24-hralbuminuria (AU) by standard methods, estimation of GFR by the MDRD formula, ECG, echocardiography, 24-hr AP monitoring, counseling bycardiologist and ophthalmologist (fundal examination by ophthalmoscopy). Standard kits were used to detect profibrogenic mediators and markersof endothelial dysfunction including transforming growth factor-beta (TGF-b), angiotensin II (AT II), monocyte chemoattractant protein (MCP-1),regulated on activation normal T cell expressed and secreted (RANTES), adhesion factors (intracellular adhesion molecule (ICAM-1), vascular celladhesion molecule (VCAM-1) vascular endothelial growth factor (VEGF), interleukin-6 (IL-6), asymmetric dimethylargnine (ADMA), homocysteine(HCYST), metalloproteinases (MMP), von Willebrand factor (vWF), plasminogen activator inhibitor (PAI-I). Results. DM patients with CKD had elevated blood profibrogenic cytokine (MCP-1, TGF-1b, IL-6) and extracellular matrix degradation factor(MMP-9) levels compared with patients without CKD and healthy subjects. These changes were unrelated to the type of diabetes or the cause ofnephropathy, which suggests their contribution to renal pathology through the universal mechanism of tubulointerstitial fibrosis. Activation of profibrogeniccytokines in DM patients with CKD was closely associated with endothelial dysfunction manifest as enhanced production of blood adhesive angiogenic, thrombogenic factors (FW, PAI, VICAM, sICAM, VEGF), and endothelium-affecting factors (ADMA, homocysteine). Mediators of inflammationand fibrogenesis in these patients negatively correlated with GFR and positively with AU, the main markers of renal dysfunction. Hyperuricemia,TGF-1b, ADMA, and MCP-1 are considered to be the risk factors of impaired renal filtration function. Conclusion. The level of profibrogenic cytokines and ndothelial dysfunction factors in DM patients with different renal lesions reflects severity of tubulointerstitialfibrosis. It may be used for the purpose of prognostication and substantiation of intensification of secondary prophylaxis of renal insufficiency.