Vol 46, No 1 (2018)

Chronic Kidney Disease affects approximately 10% of the world's adult population: it is within the top 20 causes of death worldwide, and its impact on patients and their families can be devastating. World Kidney Day and International Women's Day in 2018 coincide, thus offering an opportunity to reflect on the importance of women's health and specifically their kidney health, on the community, and the next generations, as well as to strive to be more curious about the unique aspects of kidney disease in women so that we may apply those learnings more broadly. Girls and women, who make up approximately 50% of the world's population, are important contributors to society and their families. Gender differences continue to exist around the world in access to education, medical care, and participation in clinical studies. Pregnancy is a unique state for women, offering an opportunity for diagnosis of kidney disease, but also a state where acute and chronic kidney diseases may manifest, and which may impact future generations with respect to kidney health. There are various autoimmune and other conditions that are more likely to impact women with profound consequences for child bearing, and on the fetus. Women have different complications on dialysis than men, and are more likely to be donors than recipients of kidney transplants. In this review, we focus on what we do and do not know about women, kidney health, and kidney disease, and what we might learn in the future to improve outcomes worldwide.

On behalf of the World Kidney Day Steering Committee.

Members of the World Kidney Day Steering Committee are: Ph. Kam Tao Li, G. Garcia-Garcia, M. Benghanem-Gharbi, K. Kalantar-Zadeh, C. Kernahan, L. Kumaraswami, G.B. Piccoli, G. Saadi, L. Fox, E. Zakharova, S. Andreoli.

The review presents the data on the prevalence of celiac disease in various world regions. The numbers of patients with celiac disease continues to rise every year. According to some authors, this is to be related not only to improvement in diagnosis, but to other extrinsic factors, as well, that require additional studies. In the 1980s the prevalence of this disease was 1.05%, and by the beginning of 2000s, it amounted to 1.99%. In particular, from 1993 to 2002 in Britain its incidence increased from 6 to 13.3 per 100,000. Both raised awareness of doctors and conduction of epidemiological studies play a decisive role in the improvement of the diagnosis of celiac disease. The information cumulated up to now makes it possible to conclude that the highest diagnostic rates of celiac disease can be found in the risk groups. They include 1st and 2nd degree relatives of patients with celiac disease, patients with autoimmune disorders (type 1 diabetes mellitus, autoimmune thyroiditis); those with clinical signs of an intestinal disorder, such as chronic diarrhea, as well as patients with anemia, osteoporosis and high transaminase levels of unknown origin. According to the Finnish epidemiological study, the prevalence of celiac disease, depending on the risk group, may vary from 6.6 to 16.3%. The guidelines by the American College of Gastroenterology, British Society of Gastroenterology, North-American Society of Pediatric Gastroenterology, and the Russian Consensus on Diagnosis and Treatment of Celiac Disease in Adults and Children all recommend thorough examination of patients from the risk groups. Active diagnosis of celiac disease (screening) has been recognized as one of the approaches to primary prevention to autoimmune disorders and cancer.

Aim: To estimate the prevalence of the rheumatic diseases in an epidemiological study.

Materials and methods: During a  questionnaire survey among 76 162 adult individuals in 12 regions of the Russian Federation, those with complaints of joint pain and swelling were selected and a group (n = 4894) was formed at random for subsequent clinical assessment by rheumatologists to identify main rheumatic diseases. In all regions, the study was conducted according to the unified protocol, with the unified strategy of the population selection and the use of unified questionnaires. Diagnoses were made based on widely adopted classification criteria for rheumatic diseases.

Results: For the first time in Russia it was found that among adult Russian population, the life-time prevalence of joint pain (mostly knee and hip) was 39.5%, whereas that of joint swelling amounted to 26%. From those, arthralgias eventually disappear in 8% and arthritis, in 7%. Among all rheumatic clinical entities, knee and/or hip osteoarthritis ranks the first, with its prevalence calculated to all adult population of Russia above 18 years of age being 13%. The prevalence of rheumatoid arthritis was 0.61%, of ankylosing spondylitis, 0.1%, psoriatic arthritis, 0.37%, reactive arthritis, 0.42%, gout, 0.3%, and of systemic connective tissue disorders (systemic lupus erythematosus, systemic sclerosis, dermatomyositis/polymyositis, Sjogren's syndrome, systemic vasculitis, etc),  0.11%.

Conclusion: The true prevalence of the rheumatic diseases found in the epidemiological study was higher than the data of the official statistics as follows: 2.5-fold for rheumatoid arthritis, 5-fold for osteoarthritis, 3.5-fold for spondyloarthritis and 3-fold for systemic connective tissue disorders. The results obtained showed an underestimation of the rheumatic diseases and incomplete data in the country statistics. This might be related to low referral levels for medical care due to significant distances of the vast regional areas from the specialized centers, as well as with insufficient qualification of primary care physicians in the field of rheumatology. All together, this leads to inevitable and serious misdiagnoses and low registration of the rheumatic diseases.

Rationale: Enteric hepatitis including hepatitis A and hepatitis E are globally prevalent diseases with large social and economic burden. In some patient categories these may be lethal. Hepatitis A (HAV) and hepatitis E (HEV) viruses are heterogeneous and have high geographic variability.

Aim: To assess current epidemiological, molecular and genetic characteristics of enteric viral hepatitis in the Russian Federation.

Materials and methods: We analyzed the data of the State statistical reports on the incidence of infectious diseases in the Russian Federation and of the analytical tables. Also, genotype identification of the HAVs isolated from 31 patients living in St. Petersburg was performed with specific primers flanking VP1/2A region.

Results: In the last 20 years (from 1997 to 2017), the epidemic process of hepatitis A in the Russian Federation has been changing substantially, including first of all its intensity and age distribution of the patients. Unlike hepatitis A, the official registration of hepatitis E in the Russian Federation was initiated only in 2013. During the analyzed time period, the incidence of hepatitis E varied from 0.06 to 0.08⁰⁄₀₀₀₀, with non-significant trend towards its increase in 2017. It has been found that from 2013 to 2015, two sub-genotypes of the virus (1a and 1b) circulated in St. Petersburg, with prevailing genotype 1a. The local cases of hepatitis E were caused by HEV genotype 3, whereas the imported cases, by HEV genotype 1.

Conclusion: In the Russian Federation, there has been a  long-standing and stable trend toward decreased incidence of hepatitis A. However this has led to weakening of collective immunity, mainly among the adult population. In combination with poor levels of municipal infrastructures and services in some territories, this may lead to an increase of the hepatitis A incidence. Despite that Russia is a non-endemic region for hepatitis E, local cases of the disease are increasingly identified.

Rationale: Uveal melanoma is the most common primary malignancy of the eye.

Aim: To evaluate survival in patients with uveal melanoma stratified according to the type of treatment and to identify factors significantly associated with their survival.

Materials and methods: The study was performed on the data extracted from medical files and follow-up forms of patients with uveal melanoma seen in the Ophthalmological Clinical Hospital of the Department of Healthcare, Moscow, from 1977 to 2012. Analysis of survival was used to assess the life longevity of patients with uveal melanoma. The analysis was censored at January 2013, when vital status (dead or alive) of all patients was assessed. The factors included into the study analysis, were those taken from the follow-up forms. The incidence of uveal melanoma in Moscow (2012) was 0.9 per 100,000 of the population, whereas its prevalence was 11.1 per 100,000.

Results: 698 patients with uveal melanoma were included into the study, among them 260 (37%) men (aged from 19 to 87 years, median age 60 years) and 438 (63%) women (aged from 18 to 93 years, median age 63 years); therefore, the proportion of women under the follow-up monitoring was by 26% higher than that of men. The liquidation treatment (mostly enucleation) was performed in 358 (51%) of the patients, whereas the organ preserving treatment in 340 (49%). At 5, 7, and 10 years of the follow-up, the disease-specific survival of patients with uveal melanoma after the organ preserving treatment (median survival has not been reached) and after the liquidation treatment (median, 88 months) were 89 ± 2, 83 ± 3, and 75 ± 4% versus 63 ± 3, 52 ± 4, and 47 ± 5%, respectively (р = 0.001). Overall survival and disease-specific survival of the patients after the liquidation treatment were significantly lower than in the patients after the organ-preserving treatment. According to multiple regression analysis, this was associated not with the type of treatment, but rather with higher initial grade of the disease (as the most significant factor) and with older age (> 65 years; the second significant factor). Both factors were significantly more prevalent (р = 0.05; chi-square test) among the patients who underwent the liquidation treatment.

Conclusion: The large size of the uveal melanoma, with its pre-equator location and extrascleral spread, as well as the age above 65 years can be considered as unfavorable prognostic factors.

Rationale: Lichen planus (LP) is an important problem for the healthcare due to its increasing prevalence, as well as typical, atypical and prone to malignization types of the disease that can be both severe and poorly responding to treatment. Accurate data on the prevalence of LP are lacking.

Aim: To assess the prevalence of various LP types in the patient population of the Moscow Region.

Materials and methods: We analyzed the results of examination of 338 patients with LP, who were getting standard treatment in the Department of Dermatovenereology and Dermato-oncology of the Moscow Regional Research and Clinical Institute (MONIKI) in 1999 to 2017.

Results: Among 338 LP patients, atypical LP of the skin was diagnosed in 81 (23.96%): hypertrophic type, in 31 (38.27%), pigmented, in 23 (28.4%), atrophic, in 11 (13.58%), follicular, in 8 (9.88%), lichen obtusus, in 3 (3.7%), bullous, in 1 (1.23%), erythematous, in 1 (1.23%), vulvovaginal-gingival syndrome, in 2 (2.47%), and Grinspan's syndrome, in 1 (1.23%). In 35 (43.21%) of patients, cutaneous LP was associated with atypical oral LP (OLP): in 17 (48.57%) of cases with hyperkeratotic, in 11 (31.43%), with erosive, and in 7 (20%), with exudative-hyperemic type. In 19 (23.46%) cases, atypical cutaneous LP was associated with LP of the genital mucosae, including in 12 (63.16%) cases with a typical reticular type, in 6 (31.58%), with atypical erosive, and in 1 (5.26%), with hypertrophic type. In 16 (4.73%) of the patients there was atypical OLP only; in 6 (37.5%) of them it had the exudative-hyperemic type, in 5 (31.25%), erosive, and in 5 (31.25%), hyperkeratotic. The most severe course of the dermatosis was observed for follicular, atrophic, hypertrophic types of cutaneous LP, vulvovaginal-gingival syndrome, and erosive OLP.

Conclusion: Atypical LP was diagnosed in 81 (23.96%) cases, and in 45 (55.6%) cases it was associated with the OLP. The latter in 35 (43.21%) of cases was characterized by atypical lesions of OLP, including 11 (31.43%) cases of obligate precancerous erosive LP, which necessitates that this patient category should be managed with high cancer vigilance.