scholarly journals Macroglobulinemia: An Analysis for Forty Patients

Blood ◽  
1972 ◽  
Vol 39 (6) ◽  
pp. 874-889 ◽  
Author(s):  
Malcolm R. MacKenzie ◽  
H. Hugh Fudenberg

Abstract Forty patients with Waldenström’s macroglobulinemia were evaluated for criteria of diagnosis, clinical presentation and course, response to therapy, and appropriateness of the classification primary or secondary. A wide spectrum of presentations, course, and complications were present. Thus, the major criteria for diagnosis was a monoclonal IgM serum protein abnormality present in concentrations greater than 1 g/100 ml. Clinical manifestations included weight loss, mucous membrane bleeding, presence of abnormal masses, lymphadenopathy, hepatosplenomegaly, peripheral neuropathy, and central nervous system abnormalities. A high incidence (60%) of associated malignancy was noted. The hyperviscosity syndrome was a frequent complication (33%). Alkylating drugs were successful in inducing remission in approximately 40% of the patients. Plasmapheresis was uniformly successful in relieving hyperviscosity. It was found that attempts to classify patients as primary or secondary frequently proved in error with long-term observation and as such should be discarded. Hypotheses of etiology are discussed and include the IgM proteins as: markers of a malignant lymphoid line, antibodies to microscopic or submicroscopic organisms related (or not related) to tumor induction, and antibodies to tumor-specific antigens. Firm evidence for any of these possibilities is not available.

2014 ◽  
Vol 2 (4) ◽  
pp. 61-65
Author(s):  
Vladimir Ivanovich Zavarukhin ◽  
Ekaterina Sergeevna Morenko ◽  
Sergey Ivanovich Golyana ◽  
Anton Vladimirovich Govorov

Dislocations in the carpometacarpal joints of three-phalanx fingers are rare form of injury. Their clinical manifestations are often veiled by swelling, and radiographs in standard views provide little information, which leads to difficulty in diagnosis and a high incidence of unidentified dislocations in the primary treatment. The article describes the basic provisions of the diagnosis and treatment of this type of injury, a clinical case of surgical treatment of undiagnosed dislocations of II-V metacarpal bones in the acute period, and long-term results of treatment.


2020 ◽  
Vol 16 (1) ◽  
pp. 48-51
Author(s):  
T.M. Kostiuk ◽  
A.A. Kaniura ◽  
N. Lytovchenko

Relevance. Treatment of the temporo-mandibular joint (TMJ) dysfunctions still stays an urgent issue of modern stomatology. Its significance is predisposed for its high incidence and annual increase in patients’ referrals as well as diversity of it clinical manifestations, complexity of diagnosis and treatment, variability of clinical pattern, which requires a multi-disciplinary approach. According to Ukrainian and foreign authors, the TMJ pathologies take up the third place after caries and parodontal diseases, being diagnosed in 20 % of children and almost 87 % of adults. The share of patients with the TMJ dysfunction increases every year. The 79 % are represented with the intra-capsular disorders of the temporo-mandibular joints. The study was necessitated by the fact that there is still no single agreement in the treatment methods and definite treatment protocols regarding the intracapsular TMJ disorders. Objectives: analysis of efficiency of the TMJ intracapsular disorders patients’ treatment using the pathogenetically rationalized conservative and mini-invasive surgical methods. Methods and materials. The study includes the data of 38 patients with complaints of the TMJ region pain and the TMJ dysfunction. All patients were examined according to the common protocol. According to the disorder stage, the patients were administered either the conservative treatment course using the treatment tray on the mandible or maxilla, with the subsequent orthopedic or prosthetic treatment, or the splint therapy with subsequent surgical intervention. Results. Having analyzed the dynamical pattern of the treatment outcomes within the study, it has been detected that the surgical injection correction of the TMJ disorders was effective in all examined patients. To provide for the long-term steady treatment outcome the prosthetic-orthopedic rehabilitation measures were indicated, aimed at preserving the correct occlusion relation. Conclusion. The method of hyaluronic acid preparations injection into the TMJ cavity as a stage of the TMJ dysfunction therapy has proven its effectiveness in all the examined patients. In order to achieve the long-term steady result, the patients were recommended prosthetic-orthopedic rehabilitation for producing and supporting the occlusion relation.


2021 ◽  
Vol 100 (2) ◽  
pp. 153-157
Author(s):  
A.A. Starshinova ◽  
◽  
A.M. Malkova ◽  
A.Ya. Starshinova ◽  
V.E. Karev ◽  
...  

Tuberculosis (TB) remains an important medical and social problem up to today. In recent years, great strides have been made in the fight against tuberculosis infection (TI) in reducing TB morbidity and mortality, especially in the Russian Federation. Emergence of new COVID-19 infection in the world has led to the need for long-term isolation of the population, on the one hand, and the need to resort to personal protective equipment, on the other. The spread of COVID-19 may have an impact on the increase in TB morbidity, despite the decrease in the incidence rate, which is related to objective difficulties in detecting TB and complexity of the structure of clinical manifestations of the disease against the background of coronavirus infection and severe complications after COVID-19. The use of immunosuppressive therapy in the treatment of COVID-19 may cause immunosuppression, which will affect the results of TI immunodiagnostics using skin tests. At the same time, IGRA tests can be useful in these conditions, since they are of great diagnostic value. Equally important is the reduction of patient visits to treatment facilities in the context of a pandemic or a high incidence of COVID-19.


2018 ◽  
Vol 13 (3-4) ◽  
pp. 28-37
Author(s):  
M.B. Dzhus ◽  
T.V. Marushko ◽  
H.V. Mostbauer ◽  
О.І. Ivashkivsky ◽  
I.V. Kurylchyk ◽  
...  

Relevance. It is known that the poly-articular variant of JIA is associated with significant articular and extra-articular damages with predominant lesions of small and medium joints with limitation of their function and the development of ankylosis, requiring surgical intervention in these patients. Objective. The aim of the study was to evaluate the risk factors of articular and extra-articular damages in adults with the poly-articular variant of the JIA and to work out a prognostic model for their development. Materials and methods. The study included 45 adult patients with poly-articular (RF+ and RF-) variant of the JIA. Retrospective analysis of medical records of patients with poly-articular variant of JIA was made, taking into account clinical manifestations in the onset of the disease, laboratory parameters and response to therapy. In adulthood, the integral index of articular (JADI-A) and extra-articular (JADI-E) damages was used to assess the long-term effects of JIA. Results. Unfavorable prognostic factors were revealed by method of binary logistic regression. Mathematical model for predicting the probability of long-term negative articular and extra-articular damages of poly-articular JIA was developed. Conclusions 1. The risk factors for the development of articular and extra-articular damages in adulthood in patients with a poly-articular variant of the JIA are female sex, the presence of lymphadenopathy and/or splenomegaly in childhood, the level of ESR during treatment in childhood, the activity of JADAS in childhood, the presence of symmetric arthritis in childhood, the RF positivity in the debut of the disease, the treatment with glucocorticoids, the cumulative dose of the DMARDs, and lack of therapy by DMARDs. 2. A prognostic model for the development of articular and extra-articular damages in adult patients with a poly-articular variant of the JIA is developed. This model is effective and allows determine the adverse course of the disease and can be the basis for personalized treatment for the prevention of the development of significant articular and extra-articular damages of JIA.


Author(s):  
O.M. Zнukova ◽  
◽  
A.V. Tereshchenko ◽  
I.G. Trifanenkova ◽  
M.S. Tereshchenkova ◽  
...  

Purpose. To analyze postnatal outcomes in children with spontaneous ROP regression. Material and methods. The study included 37 children (74 eyes) with spontaneous ROP regression. Gestational age was ranged from 23 to 32 weeks, body weight - from 493 to 1660 g at birth. Depending on the observation period, the children were divided into 2 age groups: 1 - from 1 month to 4 years old - 31 children (62 eyes), 2 - from 9 to 13 years old - 6 children (12 eyes). The diagnostics included standard ophthalmological examinations and additional methods. Results. In the first group, upon reaching the age of 1-year, complete retinal vascularization was observed in 26 (83%) cases (52 eyes). 5 children (10 eyes) had a narrow retinal avascular zone in the temporal segment. In the second group, in cases of postponed 3-rd stage of ROP, more severe disorders in the peripheral parts of the retina were revealed: compaction of the posterior hyaloid membrane with fixation and zones of traction retinoschisis of varying length. In the first years of life, all patients with ROP had a history of predominantly hyperopic refraction (94%). However, myopic refraction prevailed (82%) in the second group of children reaching school age. Conclusion. Considering the significant variability of the clinical manifestations of the cicatricial stage of ROP with spontaneous regression, long-term observation and a comprehensive ophthalmological examination of this group of patients are necessary for timely treatment. Key words: retinopathy of prematurity, spontaneous regression, cicatricial phase.


2016 ◽  
Vol 34 (25) ◽  
pp. 3023-3030 ◽  
Author(s):  
Sébastien Héritier ◽  
Jean-François Emile ◽  
Mohamed-Aziz Barkaoui ◽  
Caroline Thomas ◽  
Sylvie Fraitag ◽  
...  

Purpose Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia with a broad spectrum of clinical manifestations and outcomes in children. The somatic BRAFV600E mutation occurs frequently, but clinical significance remains to be determined. Patients and Methods BRAFV600E mutation was investigated in a French LCH cohort. We analyzed associations between mutation status and clinical presentation, extent of disease, reactivation rate, response to therapy, and long-term permanent sequelae. Results Among 315 patients with successfully determined BRAF status, 173 (54.6%) carried a BRAFV600E mutation. Patients with BRAFV600E manifested more severe disease than did those with wild-type BRAF. Patients with BRAFV600E comprised 87.8% of patients (43 of 49) with multisystem LCH with risk organ involvement (liver, spleen, hematology), 68.6% of patients (35 of 51) with multisystem LCH without risk organ involvement, 43.9% of patients (86 of 196) with single-system LCH, and 42.1% of patients (8 of 19) with lung-involved LCH (P < .001). BRAFV600E mutation was also associated with organ involvement that could lead to permanent, irreversible damage, such as neurologic (75%) and pituitary (72.9%) injuries. Compared with patients with wild-type BRAF, patients with BRAFV600E more commonly displayed resistance to combined vinblastine and corticosteroid therapy (21.9% v 3.3%; P = .001), showed a higher reactivation rate (5-year reactivation rate, 42.8% v 28.1%; P = .006), and had more permanent, long-term consequences from disease or treatment (27.9% v 12.6%; P = .001). Conclusion In children with LCH, BRAFV600E mutation was associated with high-risk features, permanent injury, and poor short-term response to chemotherapy. Further population-based studies should be undertaken to confirm our observations and to assess the impact of BRAF inhibitors for this subgroup of patients who may benefit from targeted therapy.


Author(s):  
V.G. Lutsyshyn ◽  
A.V. Kalashnikov ◽  
O.V. Maiko ◽  
V.M. Maiko

The course of coxarthrosis, its short-term and long-term prognosis remains sufficiently unknown, and the influence of one or another method of treatment on the course of the disease and the time of onset of irreversible changes is still the subject of scientific research. The aim of the study is to evaluate the dynamics of clinical data in patients with the initial stages of coxarthrosis in the observation interval of 1–5 years with various treatment regimens that include both medical therapy and arthroscopic intervention. The analysis of the dynamics of clinical parameters (using the modified Harris scale) in the course of various treatment methods (non-steroidal anti-inflammatory drugs with / without chondroprotector or hyaluronic acid) with a group of arthroscopically operated patients was carried out after 1, 3 and 5 years from the diagnosis of 225 patients with initial stages coxarthrosis. All quantitative data obtained are processed by variational statistics methods (Wilkoxon test for 2 groups of dependent populations and Mann-Whitney U-test for 2 groups of independent populations). It has been established that the combination of NSAID therapy with symptom-modifying delayed-action drugs contributes to the solution of individual therapeutic problems in patients with coxarthrosis during the short and medium term observation period. Reception of chondroprotectors improves the effectiveness of pain control, intra-articular administration of hyaluronic acid contributes to a longer preservation of the motor function of the affected joint. The most effective method in overcoming the progression of loss of motor function of the hip joint and increasing the intensity of the pain syndrome is arthroscopic treatment. The obtained data allow us to state that arthroscopy has a pathogenetic and preventive orientation, slows the progression of the disease and, as a consequence, improves the patient's quality of life.


2020 ◽  
Vol 2 (1) ◽  
pp. 13-22
Author(s):  
Muhammad Rizki Akbar ◽  
Putu Ristyaning Ayu Sangging

Hypersensitive pneumonitis (HP), also known as extrinsic allergic alveolitis, is a complex pulmonary syndrome mediated by the immune system and caused by inhalation of various antigens that have previously been sensitized by the individual. The pathobiology of this disease is not fully understood, but in addition to the triggers that initiate the disease, genetic factors tend to be necessary, because only a small proportion of people are exposed to HP. Because of the lack of standard diagnostic standards, the diagnosis of HP is not directly established and depends on several factors, including the history of exposure, antibodies present in specific antigens, clinical features, bronchoalveolar lavage (LAB), and radiological and pathological features. However, in proper management, high suspicion is significant and can negate the need for more invasive tests. Clinical manifestations and allergic history vary greatly. Corticosteroids may be useful in acute episodes to relieve symptoms or chronic progressive disease, but their long-term effects have never been validated in prospective clinical trials. We conduct a systematic review of published journals about HP. The search uses the Pubmed database using the medical term (MeSH) until January 2020. A total of 13 journals and one textbook were identified.


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