scholarly journals Hereditary angioedema: approaches to diagnosis and treatment, analysis of a clinical family case

2021 ◽  
pp. 34-40
Author(s):  
O. V. Skorohodkina ◽  
A. V. Luntsov ◽  
A. A. Vasileva ◽  
O. A. Bareycheva

Hereditary angioedema belongs to the group of rare, orphan, genetically determined defects that represent a significant medical and social problem due to the pronounced impact on the quality of life and potential mortality, as well as the emerging difficulties associated with timely diagnosis and the appointment of adequate treatment. The article presents data on the modern classification of hereditary angioedema, clinical manifestations of the disease, approaches to diagnosis verification and treatment principles. Therapy of hereditary angioedema is determined by the need for effective relief of acute attacks of the disease, prevention of edema before medical interventions, and, if indicated, long-term prophylaxis. The article discusses a differentiated approach to the treatment of hereditary angioedema, characterizes various options for therapeutic interventions. In a clinical case, the history of several generations of a family with manifestations of hereditary angioedema is described. A modern approach to the diagnosis of the disease based on detailed history, clinical symptoms, and laboratory research results has been demonstrated. The analysis of the effectiveness of treatment was carried out and the high efficiency of pathogenetic therapy of hereditary angioedema with human C1-esterase inhibitor was shown. 

2016 ◽  
Vol 21 (6) ◽  
pp. 329-334
Author(s):  
Sergey L. Kazakovtsev ◽  
T. V Sologub ◽  
V. V Tsvetkov

The use of immunoglobulin preparations for the postexposure prevention of tick-borne encephalitis as the main therapeutic and prophylactic agent fails to have enough high efficiency. For the postexposure prevention and treatment of tick-borne viral encephalitis the use of preparation tioloroin seems to be appropriate. Objective. To determine the expedience of the use of the preparation tiloron for the emergency prevention of tick-borne viral encephalitis and to evaluate its effectiveness in the treatment of patients with febrile and meningeal forms of this infection. Materials and Methods. The evaluation ofpost-exposure prophylaxis of tick-borne was carried out in two groups of individuals (each group was consisted of 100 cases), suffered from the suction of ticks infected by encephalitis virus. Both groups sought for medical help in the first 48 hours after the moment of the tick suction. None of the victims has not been vaccinated against the disease and had no serological markers of infection. According to existing regulations, patients in both groups received post-exposure prophylaxis antiviral tick immunoglobulin in the standard dose. Patients of the second group additionally received an interferon inducer drug tiloron. For the evaluation of the therapeutic efficacy of the drug tiloron there was executed the analysis of clinical and laboratory picture of verified tick-borne viral encephalitis in 40 patients treated at "Republican Hospital for Infectious Diseases" of the city of Syktyvkar in the period from 2010 to 2015. There were studied the dynamics of clinical symptoms, haematological and biochemical markers, CSF, certain immunological indices: CD4, CD8, CD4/CD8, IgM and IgG. For the comparative assessment of the effectiveness of treatment, all the examined patients were divided into 4 groups depending on the clinical diagnosis and ongoing taken causal treatment. Results. Among the patients received post-exposure prophylaxis with inclusion of the preparation tiloron, the disease developed significantly less often, without the formation offocal forms. The use of tiloron in combination therapy reduced the duration of main clinical manifestations in patients with febrile and meningeal forms of the disease, contributed to a more rapid rehabilitation of cerebrospinal fluid, recovery of subpopulations of T-lymphocytes. Conclusions. Immunomodulating inductor tiloron is effective in complex treatment and prevention of tick-borne viral encephalitis.


2021 ◽  
Vol 26 (4) ◽  
pp. 87-93
Author(s):  
S.V. Biletska ◽  
E.M. Dytyatkovska ◽  
M.A. Nikolaychuk

The aim of this study was to evaluate the clinical efficacy of combined allergen-specific immunotherapy (ASIT) in patients with allergic rhinitis (AR) with combined sensitization to pollen and household allergens. To achieve this goal, 49 patients with AR of working age were examined – 35.5±1.5 years with clinical manifestations of seasonal rhinoconjunctival syndrome with a long period of 9.2±1.1 years, among which there were 25 (51.0%) males and 24 (49%) females. All patients were divided into 2 homogeneous groups by age, sex, duration of the disease, the average number of etiologically significant allergens: the main one – 31 patients who received combined ASIT with solutions of pollen and household allergens and a comparison group – 18 patients for whom only pollen allergens were used. Allergological examination included anamnesis, skin tests with pollen allergens (wormwood, ragweed, quinoa, corn, etc.) and household (house dust, mites, epidermal agents) and / or molecular research methods using the ALEX technology. The quantitative integral assessment of the intensity of AR clinical symptoms was calculated as a total score for the main symptoms. The maximum score for the severity of nasal symptoms – 12, eye - 6, total – 18. The results obtained and their analysis indicate that under the influence of ASIT pollen and household allergens in patients there is a significant and reliable decrease in the intensity of clinical manifestations of seasonal rhinoconjunctive syndrome: nasal manifestations – by 52,2%, conjunctival – by 60%, integral – by 54.3% and an increase of 2.2 times in the percentage of patients in the main group with the disappearance or minimization of clinical symptoms of the disease after treatment compared with patients from the comparison group, which convincingly proves and confirms high efficiency of the selected type of therapy in patients with AR in combination with sensitization to pollen and household allergens.


2020 ◽  
pp. 101-106
Author(s):  
V. M. Svistushkin ◽  
G. N. Nikiforova ◽  
P. S. Artamonova ◽  
E. A. Shevchik

The incidence of allergic rhinitis has been increasing rapidly worldwide in recent years. About 40% of the world’s population suffer from allergic inflammation of the nasal mucous membrane. Clinical manifestations of allergic rhinitis are absence or difficulty of nasal breathing, itching, sneezing, nasal congestion and rhinorrhoea, as well as swelling of the face, the presence of dermatitis in the region of the nasal wings, general malaise, smell disturbance. Manifestations of allergies by ENT organs in most cases do not pose a threat to life, but they can cause the development of other pathological processes, bad sleep, irritability, decreased efficiency, mood swings, which, in turn, adversely affects human health and reduces the quality of life. The pathogenesis of allergic rhinitis is based on an immediate hypersensitivity. According to the modern classification, depending on the nature of the course of the disease it is common to identify intermittent and persistent forms.At present, the drugs of choice in the treatment of patients with allergic rhinitis are intranasal glucocorticosteroids. However, the presence of a concomitant pathology of the nasal cavity in the patient, such as deviated septum, not only significantly impairs the course of nasal inflammatory process, but also creates obstacles to adequate delivery of topical drugs to all parts of the nasal cavity, which, in turn, reduces their effectiveness.For patients suffering from allergic rhinitis in combination with other pathology of the nasal cavity, combined oral medications may be a good alternative to intranasal sprays. Certain interest for doctors is caused by the appearance of a combined antihistamine drug on the Russian market, which includes a blocker of leukotrienes and H1-histamine receptors of the second generation – montelukast and levocetirizine. The drug can be used both in adults and children from 15 years old for treatment of intermittent and persistent forms of allergic rhinitis.Timely and properly performed therapy of allergic rhinitis allows to eliminate symptoms of the disease and prevent the development of complications.


2021 ◽  
pp. 20-25
Author(s):  
L. P. Evstigneeva

Background. Osteoporosis is a common pathology among older age groups. Vertebral fractures are one of the most severe complications of osteoporosis, as they lead to a decrease in the quality of life, disability and increased mortality. The risk of vertebral fractures increases with age, especially in those who have already has a fracture of this localization. To improve the prognosis in this category of patients, early detection, treatment of patients with fractures, as well as prevention of new fractures, are important. At the same time, the diagnosis of osteoporotic vertebral fractures presents certain difficulties due to the variability of their clinical manifestations, which often leads to insufficient detection, wrong diagnosis and, as a consequence, to the lack of adequate treatment.Purpose of the review. To conduct a literature search to evaluate the clinical diagnosis of osteoporotic vertebral fractures.Conclusion. There was no single highly specific and at the same time sensitive clinical symptom of osteoporotic vertebral fractures. Back pain remains a relatively nonspecific symptom in vertebral fractures. The most specific symptoms: kyphosis and height loss have a high predictive value only when they are significantly changed. Thus, vertebral fractures should be suspected if height decreased more than 2.0 cm per year or more than 4.0 cm per life, if the patient does not reach the wall with his head or if the «rib to pelvis» distance is two fingers or less. The combination of clinical symptoms with the assessment of risk factors and bone mineral density has the greatest clinical significance. If a vertebral fracture is clinically suspected, it is advisable to perform an X-ray of the thoracic and lumbar spine, since the localization of pain indicated by the patient may not correspond to the localization of the fracture.


2020 ◽  
Vol 65 (5-6) ◽  
pp. 30-34
Author(s):  
L. L. Logvina ◽  
D. N. Bayram ◽  
Z. A. Kambachokova ◽  
F. V. Shavaeva ◽  
Z. S. Krymshokalova ◽  
...  

Evaluation of the effectiveness of aminophthalhydroside in the complex treatment of patients with recurrent genital herpes was carried out. The efficacy of the drug was assessed by clinical, biochemical, and immunological criteria. The inclusion of aminophthalhydrazide in the complex therapy of patients with recurrent herpesvirus infection led to an earlier relief of clinical manifestations of the disease, an increase in the relapse period, a decrease in lipid peroxidation products in the blood plasma, an increase in the content of antioxidant defense components, and correction of immunological disorders.


2018 ◽  
Vol 18 (3) ◽  
pp. 182-191 ◽  
Author(s):  
Linda L. Eastham ◽  
Candace M. Howard ◽  
Premalatha Balachandran ◽  
David S. Pasco ◽  
Pier Paolo Claudio

Enthusiasm for the use of dietary bioactive compounds as chemopreventive agents and adjuvants for current therapies has increased laboratory research conducted on several types of cancers including Head and Neck Squamous Cell Carcinoma (HNSCC). The green chemoprevention movement is a modern approach to highlight healthy lifestyle changes that aim to decrease the incidence of HNSCC. A healthy diet can be an effective way to prevent the development of oral cancers. Discovery of the naturally occurring plant based compounds called phytochemicals has facilitated the development of new treatment strategies for patients that are at risk for, or have developed HNSCC. Many of these compounds have been shown to elicit very potent anti-carcinogenic properties. While there are many compounds that have been studied, the compounds from two specific categories of phytochemicals, phenolics (resveratrol, EGCG, curcumin, quercetin, and honokiol) and glucosinolates (sulforaphane, PEITC and BITC), are emerging as potent and effective inhibitors of oral carcinogenesis. These compounds have been shown to inhibit HNSCC growth through a variety of mechanisms. Research has demonstrated that these compounds can regulate cancer cell proliferation through the regulation of multiple cell signaling pathways. They can impede cell cycle progression, induce differentiation and apoptosis, prevent angiogenesis, and inhibit cancer cell invasive and metastatic properties. They can protect normal cells during treatment and reduce the damage caused by chemotherapy and radiotherapy. This review aims to provide an overview of some of the most effective phytochemicals that have the potential to successfully prevent and treat head and neck squamous cell carcinoma.


Author(s):  
Sara Abolghasemi ◽  
Mohammad Alizadeh ◽  
Ali Hashemi ◽  
Shabnam Tehrani

Introduction: Epididymo-orchitis is a common urological disease among men. Little is known about the clinical and epidemiological aspects of the disease in Iran. Thus, the present study was aimed to investigate the etiology, clinical sequelae and risk factors of patients with epididymo-orchitis in Tehran, Iran. Materials and Methods: Patients presenting with epididymo-orchitis were prospectively analyzed in order to study the etiology and pattern of the disease. Bacteriological, molecular and serological tests were undertaken to look for Chlamydia trachomatis, Neisseria gonorrhoeae, Brucella spp., Mycoplasma spp, and other bacteria. Results: Fifty patients with epididymo-orchitis were evaluated according to their clinical symptoms, duration of symptoms, physical examination, and laboratory studies. The mean age of the patients was 53 years. Fever, dysuria, pain in the flanks, urinary frequency and discharges occurred in 58.0%, 50.0%, 50.0%, 28.0% and 6.0%, respectively. Bacterial pathogen was identified in 26% (13/50) of patients by urine culture. Escherichia coli was the etiological agent in 11/13 patients (84.6%). Two out of 50 patients (4.0%) were also positive for Chlamydia trachomatis. Two samples were serologically positive for Brucella spp. High Mean age, fever, urinary frequency, history of the underlying disease and history of urinary tract infections were found to have a significant association with the positive bacteriologic urine culture (P<0.05). Conclusions: The most common clinical manifestations were fever, dysuria, and abdominal pain. E. coli and C. trachomatis were the major causative agents. Use of a set of diagnostic approaches including clinical symptoms, urine culture and more precise techniques such as PCR should be taken into consideration for the definitive diagnosis.


2016 ◽  
Vol 5 (09) ◽  
pp. 4896
Author(s):  
Sripriya C.S.* ◽  
Shanthi B. ◽  
Arockia Doss S. ◽  
Antonie Raj I. ◽  
Mohana Priya

Scrub typhus (Orientia tsutsugamushi), is a strict intracellular bacterium which is reported to be a recent threat to parts of southern India. There is re-emergence of scrub typhus during the past few years in Chennai. Scrub typhus is an acute febrile illness which generally causes non-specific symptoms and signs. The clinical manifestations of this disease range from sub-clinical disease to organ failure to fatal disease. This study documents our laboratory experience in diagnosis of scrub typhus in patients with fever and suspected clinical symptoms of scrub typhus infection for a period of two years from April 2014 to April 2016 using immunochromatography and IgM ELISA methods. The study was conducted on 648 patients out of whom 188 patients were found to be positive for scrub typhus. Results also showed that pediatric (0 -12 years) and young adults (20 – 39 years) were more exposed to scrub typhus infection and female patients were more infected compared to male. The study also showed that the rate of infection was higher between September to February which also suggested that the infection rate is proportional to the climatic condition. Statistical analysis showed that the mean age of the patients in this study was 37.6, standard deviation was 18.97, CV % was 50.45. 


Hematology ◽  
2017 ◽  
Vol 2017 (1) ◽  
pp. 242-250 ◽  
Author(s):  
Lisa J. McReynolds ◽  
Sharon A. Savage

Abstract The clinical manifestations of inherited susceptibility to leukemia encompass a wide phenotypic range, including patients with certain congenital anomalies or early-onset myelodysplastic syndrome (MDS) and some with no obvious medical problems until they develop leukemia. Leukemia susceptibility syndromes occur as a result of autosomal dominant, autosomal recessive, or X-linked recessive inheritance, or de novo occurrence, of germline pathogenic variants in DNA repair, ribosome biogenesis, telomere biology, hematopoietic transcription factors, tumor suppressors, and other critical cellular processes. Children and adults with cytopenias, MDS, dysmorphic features, notable infectious histories, immunodeficiency, certain dermatologic findings, lymphedema, unusual sensitivity to radiation or chemotherapy, or acute leukemia with a family history of early-onset cancer, pulmonary fibrosis, or alveolar proteinosis should be thoroughly evaluated for a leukemia susceptibility syndrome. Genetic testing and other diagnostic modalities have improved our ability to identify these patients and to counsel them and their family members for subsequent disease risk, cancer surveillance, and therapeutic interventions. Herein, the leukemia susceptibility syndromes are divided into 3 groups: (1) those associated with an underlying inherited bone marrow failure syndrome, (2) disorders in which MDS precedes leukemia development, and (3) those with a risk primarily of leukemia. Although children are the focus of this review, it is important for clinicians to recognize that inherited susceptibility to cancer can present at any age, even in older adults; genetic counseling is essential and prompt referral to experts in each syndrome is strongly recommended.


2020 ◽  
Vol 10 (01) ◽  
pp. e137-e140
Author(s):  
Mosaad Abdel-Aziz ◽  
Nada M. Abdel-Aziz ◽  
Dina M. Abdel-Aziz ◽  
Noha Azab

AbstractThe clinical manifestations of novel coronavirus disease 2019 (COVID-19) vary from mild flu-like symptoms to severe fatal pneumonia. However, children with COVID-19 may be asymptomatic or may have mild clinical symptoms. The aim of this study was to investigate clinical features of pediatric COVID-19 and to search for the factors that may mitigate the disease course. We reviewed the literature to realize the clinical features, laboratory, and radiographic data that may be diagnostic for COVID-19 among children. Also, we studied the factors that may affect the clinical course of the disease. Fever, dry cough, and fatigue are the main symptoms of pediatric COVID-19, sometimes flu-like symptoms and/or gastrointestinal symptoms may be present. Although some infected children may be asymptomatic, a recent unusual hyperinflammatory reaction with overlapping features of Kawasaki's disease and toxic shock syndrome in pediatric COVID-19 has been occasionally reported. Severe acute respiratory syndrome-coronvirus-2 (SARS-CoV-2) nucleic acid testing is the corner-stone method for the diagnosis of COVID-19. Lymphocyte count and other inflammatory markers are not essentially diagnostic; however, chest computed tomography is highly specific. Factors that may mitigate the severity of pediatric COVID-19 are home confinement with limited children activity, trained immunity caused by compulsory vaccination, the response of the angiotensin-converting enzyme 2 receptors in children is not the same as in adults, and that children are less likely to have comorbidities. As infected children may be asymptomatic or may have only mild respiratory and/or gastrointestinal symptoms that might be missed, all children for families who have a member diagnosed with COVID-19 should be investigated.


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