Surgical therapy of lichen sclerosus of the vulva consists of three main operations: vulvectomy (with or without a skin graft), cryosurgery, and laser ablation. It is indicated in two conditions, either when malignant transformation is present or is likely to occur, or when medical treatment has failed. The overall risk for malignant transformation of vulvar lichen sclerosus is low, ranging between 0 and 9 percent. However, specific histological criteria, such as mixed dystrophy, have recently been associated with higher malignancy rates. Vulvectomy is indicated only when these criteria are met. Both skinning and simple vulvectomies are associated with recurrence rates as high as 50 percent. However, better sexual function and cosmetic results have been reported in the former, especially with concomitant split skin grafting. Cryosurgery also has high recurrence rates, although short-term results are favorable. Although only small series have been reported, laser therapy seems to carry better long-term results than other modes of treatment. Convalescence is complete within 6 weeks posttreatment, and remission rates are as high as 85 percent at 3 years of follow-up. The high recurrence rate of all surgical modalities makes surgical treatment suitable only for patients who failed to respond to multiple medical treatments such as topical high potent steroid ointments, testosterone, and retinoids.
Частота носительства в российской популяции мутаций в генах GJB2 и GALT, ассоциированных с развитием нейросенсорной тугоухости и галактоземии Carrier frequency of GJB2 and GALT mutations associated with sensorineural hearing loss and galactosemia in the Russian population Статья продолжает цикл работ, посвященных определению частоты носительства в российской популяции мутаций, ассоциированных с развитием распространенных моногенных заболеваний. Целью исследования было установление частоты распространенных в российской популяции мутаций в генах GJB2 и GALT у доноров первичной кроводачи. При генотипировании 1000 доноров первичной кроводачи, идентифицирующих себя как русских и постоянно проживающих на территории Российской Федерации, обнаружены 37 носителей мутаций в гене GJB2, ассоциированных с развитием нейросенсорной тугоухости (частота в выборке составила 3,7 %, или 1 : 27), и 6 носителей мутаций в гене GALT, ассоциированных с развитием галактоземии (частота в выборке -0,6 %, или 1 : 167). Выявлен 1 случай сочетанного носительства мутаций, и, таким образом, всего обнаружены 42 носителя мутаций в генах GJB2 и GALT (частота в выборке -4,2 %, или 1 : 24). This article continues a series of works estimating carrier frequencies of mutations associated with the development of common monogenic disorders in the Russian population. The study aimed to establish the frequency of GJB2 and GALT mutations in first-time blood donors. Genotyping of 1000 first-time blood donors who identify themselves as Russians and permanently reside in the Russian Federation detected 37 carriers of GJB2 mutations associated with sensorineural hearing loss (carrier frequency in the sample was 3.7 %, or 1 : 27) and 6 carriers of GALT mutations associated with galactosemia (carrier frequency in the sample was 0.6 %, or 1 : 167). In one carrier, concurrent mutations were detected; thus, in total 42 carriers of GJB2 and GALT mutations were detected (carrier frequency in the sample was 4.2 %, or 1 : 24).
The objective of the study was to define treatment strategy in cases of facial bones bisphosphonate induced osteonecrosis based on the study of the role of conditionally pathogenic oral microorganisms. Three typical clinical cases of bisphosphonate osteonecrosis of the facial bones were analyzed and 15 conditionally pathogenic oral microorganisms were identified in these patients using real-time PCR in saliva, wound and bone samples. A comparative analysis was carried out with purulent-inflammatory diseases of maxillofacial area. The study results proved an important role of conditionally pathogenic microorganisms of the oral cavity in the development of osteonecrosis of the facial bones. Wide range of bacterial species was identified in osteonecrosis of the facial bones patients. While bone tissue is most exposed to microbial communities, surgical treatment results in effective rehabilitation for a long period.
BackgroundCastleman's disease (CD) is a rare lymphoprolipherative mostly benign disorder presenting in two major forms: localized (unicentric) and systemic (multicentric). Based on pathomorphological finding it is classified as hyaline-vascular, plasma cell or mixed type. The age of onset can differ greatly from infancy to adulthood. Except for lymph node hyperplasia, patients with CD can also develop such symptoms as anemia, fatigue, hepatosplenomegaly, low-grade fevers and other systemic symptoms.ObjectivesAnalysis of clinical manifestations, pathomorphology and treatment efficiency in a group of pediatric patients.MethodsWe conducted a retrospective analysis of 12 patients (11 boys and 1 girl) with histologically confirmed cases of Castleman's disease.ResultsAge of onset varied from the moment of birth to 16 years (median 8,5 years) 8 patients had unicentric form of disease, 3 had multicentric form and one patient had two groups of lymph nodes affected, thus could not be precisely classified. The majority of the patients (9 patients) had hyaline-vascular variant of CD, one patient had plasma-cell type and two patients had the mixed pathomorphological type. The reason for seeking medical attention in three patients was visible unilateral enlargement of neck lymph nodes, four patients initially presented with anemia, fatigue, fever and laboratory signs of inflammation, and in 4 patients enlarged lymph nodes were incidentally visualized during control X-ray, and no other symptoms of CD were present. Interestingly, of 3 patients with multicentric form of CD, two had underlying primary immunodeficiency – Wiskott-Aldrich syndrome (WAS). They presented with plasma cell type and mixed type of CD, respectively. No immunodeficiency has been proven in other patients. We have conducted tests to indicate HHV VIII in affected lymph node biopsies and blood samples via PCR in half of the patients and none occurred to be positive.Treatment: The affected lymph nodes were completely excised surgically in 7 patients with unicentric form, with no relapse of the disease on follow-up. Both patients with WAS received rituximab treatment followed by bone marrow transplantation, which was originally planned as a treatment of WAS, with remission on follow-up at 3 month and 2 years, respectively. Third patient with multicentric form received several courses of anti-cytokine and immunosuppressive treatment including tocilizumab, cyclophosphamide, vincristine and prednisolone (total of 5 blocks), rituximab (6 infusions) with partial effect, then surgical excision of large conglomerates of lymph nodes followed by bortezomib and bendamustine (total of 6 blocks) with positive effect. However at the one year follow-up patients demonstrated enlarged lymph nodes again, so she was started on JAK tyrosine kinase inhibitor ruxolitinib. Follow-up results are pending.ConclusionsOur analysis of a group of pediatric patients demonstrates great variability in symptoms and severity of the disease. Castleman's disease is a disorder with poorly studied path...
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