Abstract:
AIM:To analyze the incidence of primary immunodeficiencies (PIDs), to reveal the specific features of the course of this condition at the present stage, and to estimate the quality of health care to patients with PIDs. SUBJECTS AND METHODS:An open-label prospective trial was performed in 94 patients with different forms of PIDs (63 with selective immunoglobulin A (IgA) deficiency and 31 with other more severe primary immunodeficiencies) who had been permanent residents in the Perm Territory in the period 1990 to 2012. RESULTS:The registered PID cases were noted to be lower than the estimated ones. Over 22 years of follow-ups, the death rates for this group of patients with these diseases were 11%, and the disability rates were 27%. In severe PIDs (exclusive of selective IgA deficiency), these rates were as high as 35.5 and 96%, respectively. The rate of untimely diagnosis of severe PIDs was high (43%). Molecular genetic studies were conducted in only one tenth of the patents with this disease. PID treatment generally complied with the accepted medical standards. However, all patients with X-linked agammaglobulinemia were observed to have periodic irregularities of replacement therapy with intravenous immunoglobulins, which was a cause of death in 2 patients. Adult patients with common variable immune deficiency received no adequate replacement therapy. Timely diagnosis and adequate therapy could not only preserve the life of many patients with severe PIDs (64.5% survived), but could achieve its relatively satisfactory quality. CONCLUSION:As of now, PIDs ceased to be fatal diseases. To improve the quality of health care to patients with this pathology, there is a need to increase the awareness of the diagnosis and treatment of immunodeficiencies among physicians of different specialties, to extend the application of molecular genetic techniques, including those for prenatal diagnosis, and to continuously provide patients with essential drugs.
journal_name
Ter Arkhjournal_title
Terapevticheskii arkhivauthors
Troitskaya EV,Sofronova LV,Tsvetkova TYsubject
Has Abstractpub_date
2014-01-01 00:00:00pages
12-5issue
11eissn
0040-3660issn
2309-5342journal_volume
86pub_type
杂志文章abstract:AIM:To reveal the metabolic parameters of lipoproteins (L), which determine the benefits of hypolipidemic effects of simvastatin (S) and fluvastatin (F); to trace changes in the activity of lecithin-cholesterol acyltransferase (LCA) and cholesterol (C) ester transfer (CET) from high density lipoproteins to very low den...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:1998-01-01 00:00:00
abstract:AIM:to characterize the markers of endothelial dysfunction and the effect of antihypertensive drugs on them in patients with chronic urate tubulointestinal nephritis (UTIN) and articular gout. SUBJECTS AND METHODS:The study enrolled 81 patients aged 39 to 59 years with gout and urate nephropathy. All the patients were...
journal_title:Terapevticheskii arkhiv
pub_type: 临床试验,杂志文章
doi:
更新日期:2010-01-01 00:00:00
abstract:AIM:To detect endogenic intoxication syndrome (EIS) and to assess its severity depending on clinical manifestations of rheumatoid arthritis (RA) using laboratory tests. MATERIAL AND METHODS:Laboratory tests including analysis of synovial fluid, blood count with estimation of leucocytic intoxication index, urinalysis, ...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:2006-01-01 00:00:00
abstract:AIM:To assess factors of an unfavourable prognosis in a group of intermediate risk of B-cell chronic lymphoid leukemia (BCCLL). MATERIAL AND METHODS:206 BCCLL patients (mean age 55.5 years, male/female = 1.66) entered the study conducted by Hematological Research Center in 1992-2000. RESULTS:Nine patients under 35 ye...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:2003-01-01 00:00:00
abstract::The state of the renin-angiotensin system (RAS) was studied in 102 patients with chronic glomerulonephritis with the nephrotic syndrome (NS) with relation to a phase of the edematous syndrome and sodium balance. Heterogeneity of patients with NS was shown with regard to RAS activity and the circulating blood volume in...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:1988-01-01 00:00:00
abstract:AIM:To determine sensitivity and specificity of stress echocardiography (SEC) in diagnosis of stenosing atherosclerosis of coronary arteries (CA), especially in patients recovered from Q-myocardial infarction. MATERIAL AND METHODS:The above sensitivity and specificity were studied in 75 patients (70 males and 5 female...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:2004-01-01 00:00:00
abstract::In the article, we report the causes of pulmonary hemorrhage (PH) according to the literature data and own experience, with an emphasis on patients suffering from rheumatic diseases. Methods of diagnosis and modern approaches to the treatment of PH are analyzed. :В представленной статье на основе данных литературы и ...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:10.26442/00403660.2019.05.000234
更新日期:2019-05-15 00:00:00
abstract:AIM:To determine the possible boundaries of high-dose immunosuppressive therapy and autologous hematopoietic stem cell transplantation (HDIT-autoHSCT) for autoimmune diseases (AUDs), such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and multiple sclerosis (MS). SUBJECTS AND METHODS:A long-term tri...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:10.17116/terarkh201688153-59
更新日期:2016-01-01 00:00:00
abstract::Ascites and hydrothorax may be the symptoms of congestive heart failure and do not always reflects presense of the decompensated liver cirrhosis. Clinical examination of patient with chronic hepatitis C which cyanosis of the lips, cervival veins pulsation, a triple heart rhythm indicated on pathology of the heart (con...
journal_title:Terapevticheskii arkhiv
pub_type:
doi:10.26442/terarkh201890874-80
更新日期:2018-08-27 00:00:00
abstract::Arterial hypertension (AH) is one of the basic components of metabolic syndrome that is caused by four factors: autonomic sympathetic dysfunction; activation of the hypothalamic-pituitary-adrenal axis; that of the renin-angiotensin-aldosterone system; and endothelial dysfunction (ED). AH is a slowly progressive hemody...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章,评审
doi:
更新日期:2014-01-01 00:00:00
abstract:AIM:To compare the impact of cardiac resynchronization therapy (CRT) on survival in patients with ischemic and non-ischemic cardiomyopathy (CMP) in clinical practice. SUBJECTS AND METHODS:The study enrolled 206 patients with NYHA Functional Class II-IV chronic heart failure (CHF) and a left ventricular ejection fracti...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:2012-01-01 00:00:00
abstract:AIM:To quantify interleukin-8 (IL-8), interleukin-2 (IL-2) and soluble receptor of IL-2 (sIL-2r) in blood serum of patients with various forms of ischemic heart disease (IHD). MATERIAL AND METHODS:Levels of IL-8, IL-2 and sIL-2r were measured with enzyme immunoassay (EIA) in the serum of 75 patients with IHD: angina o...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:2001-01-01 00:00:00
abstract::The efficacy and tolerability of high-dose neurotrophic drug cerebrolysin was studied in 20 patients with vascular dementia diagnosed by criteria NINDS-AIREN and DSM-IV. The drug was well tolerated, affected positively cognitive function, especially neurodynamic processes. EEG featured after treatment intensification ...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:1996-01-01 00:00:00
abstract:AIM:To study epidemiological characteristics of cryptosporidosis (CS) in conditions of arid climate which contributes to suppression of the immune status of the organism. MATERIAL AND METHODS:1935 patients were treated for acute intestinal infection with watery diarrhea in Ashkhabad hospital in 1994-1999. Monocryptosp...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:2002-01-01 00:00:00
abstract:AIM:To evaluate the efficiency and safety of using statins in combination with ursodeoxycholic acid (UDCA) in patients with this or another liver disease at high risk for cardiovascular events (CVE). SUBJECTS AND METHODS:A register of 262 patients at high risk for CCE who needed statin therapy and have concomitant chr...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:10.17116/terarkh2014861248-52
更新日期:2014-01-01 00:00:00
abstract::A study of 186 coronary patients with rhythm disturbance has shown that early incorporation of the elements of physical rehabilitation in combined treatment improves functioning of all the links of the blood circulation system, helps to raise cardiac rhythm stability and reduces the period of patients' stay in hospita...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:1986-01-01 00:00:00
abstract:AIM:to determine the perspectives for the use of drugs with combined antiviral, anti - inflammatory and immunomodulatory activity on the basis of medical studies of existing antiviral drugs for the treatment of influenza and acute respiratory viral infections in Russia. MATERIALS AND METHODS:A brief review of the anti...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:10.26442/00403660.2019.11.000454
更新日期:2019-11-15 00:00:00
abstract:AIM:To evaluate the presence and the severity of the complaints (headache, dizziness, memory loss, concentration of attention, sleep disturbances, decreased mood, increased anxiety), the state of cognitive functions, emotional status and quality of night sleep in treatment-naïve middle-aged patients with mild to modera...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:10.26442/terarkh201890915-26
更新日期:2018-09-20 00:00:00
abstract::The examination covered 44 patients (16 males and 28 females) with chronic bronchitis (CB) associated with diseases of the nose and paranasal sinuses. Most of the patients (63.6%) had allergic rhinosinusopathy (ARSP). The kind of nasal and sinus affection varied with CB form (catarrhal or purulent). Aggravation of ENT...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:1996-01-01 00:00:00
abstract::Altogether 3875 women aged 30 to 50 engaged in the textile industry, were examined. Primary prevalence of arterial hypertension was 7.0-7.2%. The implementation of a special program of active prophylaxis resulted in a significant rise of the number of effectively treated patients as compared to the control group. ...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:1985-01-01 00:00:00
abstract::Data are presented on external respiration at rest and during exercise (bicycle ergometry) in 20 patients with chronic circulatory insufficiency due to a variety of heart diseases, as well as variation in the above in the course of treatment with digoxin and calcium antagonist corinfar. External respiration parameters...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:1986-01-01 00:00:00
abstract:AIM:To determine the effect of plasma homocysteine (Hc) on the structure and function of the left ventricle (LV) in patients treated by programmed hemodialysis. SUBJECTS AND METHODS:109 patients on programmed hemodialysis were examined. Blood Hc levels were determined and echocardiography was performed in all the pati...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:2013-01-01 00:00:00
abstract::ORT effectiveness for AII cases of different etiology was studied in two groups of patients: 153 patients as ORT received glucosalan (group 1), 75 patients underwent ORT with sodium citrate in solution from 2.5 to 51 within 6 hours. Both group 1 and group 2 patients benefited from the above solutions as following 6 ho...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:1994-01-01 00:00:00
abstract:AIM:To investigate potentialities of a novel biochemical test assessing oxidative resistance of plasma as a marker of ischemic damage to the myocardium and to study this marker correlation with high-performance ECG parameters reflecting electrophysiological remodeling of the myocardium in patients with ischemic heart d...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:2006-01-01 00:00:00
abstract::The influence of the preliminary fat intake on the rate of contrast capsules administered with semolina porridge and morning breakfast evacuation from the gastric stump and small intestine was studied in 39 patients with gastric resection according to Billroth II by means of the x-ray method suggested for quantitation...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:1989-01-01 00:00:00
abstract::In up-to-date industrial conditions the new clinical forms of occupational pulmonary diseases occur more and more frequently in addition to the most prevalent dust pathology of the lungs. They occur in workers exposed to industrial aerosols of complex composition, including toxic substances and allergens along with fi...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:1990-01-01 00:00:00
abstract::A study was made of exercise tolerance and indices of central hemodynamics in 151 patients with "isolated" angina and its combination with cardialgia, arterial hypertension and postinfarctional cardiosclerosis. Daily attacks of angina of effort (III functional class) were noted in all the patients. In patients with "i...
journal_title:Terapevticheskii arkhiv
pub_type: 临床试验,杂志文章
doi:
更新日期:1986-01-01 00:00:00
abstract:AIM:To assess an association of uric acid level with preclinical target organ damage in patients with hypertensive disease (HD). SUBJECTS AND METHODS:The trial enrolled 100 patients (63 men and 37 women) with Stage I-II HD at moderate and high risk for cardiovascular events (CVEs). The mean age of the patients was 44....
journal_title:Terapevticheskii arkhiv
pub_type: 临床试验,杂志文章
doi:
更新日期:2013-01-01 00:00:00
abstract:AIM:To evaluate the impact of hyperglycemia concurrent with obstructive sleep apnea syndrome (OSAS) on endothelial function (EF) and vessel remodeling in patients with Grade 1-2 hypertension. MATERIAL AND METHODS:A total of 101 patients with Grade 1-2 hypertension were examined. A study group (SG) consisted of 74 hype...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:10.17116/terarkh2015871026-30
更新日期:2015-01-01 00:00:00
abstract::The rate of Na-Li countertransport was studied in inpatients with essential hypertension (n = 59), chronic diffuse glomerulonephritis (n = 30), chronic pyelonephritis (n = 26), renovascular hypertension (n = 15) and in those with associated renovascular hypertension and essential hypertension (n = 4). Multiple regress...
journal_title:Terapevticheskii arkhiv
pub_type: 杂志文章
doi:
更新日期:1990-01-01 00:00:00