Autoantibodies to nuclear valosin-containing protein-like protein: systemic sclerosis-specific antibodies revealed by in vitro human proteome.

OBJECTIVES: To identify and characterize undescribed systemic sclerosis (SSc)-specific autoantibodies targeting nucleolar antigens and to assess their clinical significance. METHODS: We conducted proteome-wide autoantibody screening (PWAS) against serum samples from SSc patients with nucleolar patterned anti-nuclear antibodies (NUC-ANAs) of specific antibodies (Abs) unknown, utilizing wet protein arrays fabricated from in vitro human proteome. Controls included SSc patients with already-known SSc-specific autoantibodies, patients with other connective tissue diseases, and healthy subjects. The selection of nucleolar antigens was performed by database search in the Human Protein Atlas. The Presence of autoantibodies was certified by immunoblots and immunoprecipitations. Indirect immunofluorescence assays on HEp-2 cells were also conducted. Clinical assessment was conducted by retrospective review of electric medical records. RESULTS: PWAS identified three candidate autoantibodies, including anti-nuclear valosin-containing protein-like (NVL) Ab. Additional measurements in disease controls revealed that only anti-NVL Abs are exclusively detected in SSc. Detection of anti-NVL Abs was reproduced by conventional assays such as immunoblotting and immunoprecipitation. Indirect immunofluorescence assays demonstrated homogeneous nucleolar patterns. Anti-NVL Ab-positive cases were characterized by significantly low prevalence of diffuse skin sclerosis and interstitial lung disease, compared with SSc cases with NUC-ANAs other than anti-NVL Abs, such as anti-U3-RNP and anti-Th/To Abs. CONCLUSION: Anti-NVL Ab is an SSc-specific autoantibody associated with a unique combination of clinical features, including limited skin sclerosis and lack of lung involvement.

Causa inusitada de insuficiencia aórtica aguda / Unusual cause of acute aortic insufficiency

CASO: Paciente, pós-implante de marcapasso (MP) definitivo, com piora da classe funcional. O ecocardiograma mostra insuficiência aórtica (IAo) aguda. É incorreto afirmar: a. A imagem é sugestiva de dissecção tipo I com acometimento valvar. b. A IAo não é secundária a alteração da anatomia das válvulas nem do anel valvar aórtico. c. O ECG de 12 derivações e a radiografia de tórax em perfil oferecem importantes informações que podem levar à suspeita diagnóstica. d. A anticoagulação oral (ACO) pode ser necessária para prevenção de eventos embólicos até a resolução definitiva da causa da IAo. RESPOSTA: a. A imagem é sugestiva de eletrodo de MP impactando na valva aórtica, não é característica de linha de disseção e não altera a anatomia das valvas nem do anel aórtico. Até a retirada e reposicionamento do eletrodo, a ACO ajuda diminuir a incidência de eventos tromboembólicos secundários à localização do eletrodo em circulação sistémica. link do youtube: https://youtu.be/BEAwtn7yPjA

Ecocardiografia transesofágica tridimensional no auxílio diagnóstico da disfunção de prótese valvar aórtica / Three-dimensional transesophageal echocardiography in the diagnosis of prosthetic aortic valve dysfunction

CASO: Paciente com troca de valva aórtica há cinco anos e dispneia aos esforços. Sobre o diagnóstico, é incorreto afirmar: a. Métodos quantitativos não influenciados pela presença da prótese também devem ser usados na avaliação da gravidade do refluxo. b. O diagnóstico é ruptura de prótese, afasta a possibilidade de endocardite infecciosa. c. Usando eco 3D color podemos identificar o plano perpendicular ao jato e planimetrar a vena contracta. d. A complementação esofágica é indicada em estudos tecnicamente difíceis e na avaliação da etiologia da IAo e de complicações associadas. Alternativa Correta: d. A imagem sugere ruptura de folheto causando insuficiência importante, no entanto devido a características como: pouco tempo de prótese, ausência de imagem sugestiva de degeneração dos folhetos não fraturados e grande espessamento do folheto fraturado, a possibilidade de endocardite como causa inicial da disfunção deve ser considerada entre os possíveis diagnósticos etiológicos. Link do youtube: https://www.youtube.com/watch?v=oLF_VYxX_Ho

The Effective of Keishibukuryogan on Uterine Subinvolution after Childbirth: NRCT / 日本東洋医学雑誌

The uterus starts shrinking after giving birth and returns to its size prior to pregnancy one month postpartum. However, absence of this natural shrinking is called uterine subinvolution. There are two types of subinvolution : organic and functional. Removal of the cause is the first option for organic subinvolution. Conversely, most cases of functional subinvolution require drug treatment. This study included patients with subinvolution caused by accumulation of lochia with a uterine cavity width of 15 mm or more during a 14-day postpartum checkup. These patients were categorized into the control, keishibukuryogan, and ergometrine maleate groups ; therapeutic intervention was performed. A comparative study was conducted to determine the presence of subinvolution during the 1-month medical screening. There was no difference in the rate of uterine cavity shrinkage between the ergometrine maleate group and the control group. However, the keishibukuryogan group had a significantly higher reduction rate than the ergometrine maleate group. Furthermore, the keishibukuryogan group showed a tendency of higher shrinking rate when compared with the control group (76.1 ± 17.1% vs 65.8 ± 25.4%, 68.3 ± 22.9%, p = 0.0101, p = 0.0709). Additionally, no difference in the reduction rate of the fundal height was noted among the groups. These results suggest that although keishibukuryogan has little effect on reducing the uterine size, however, it has the effect of lochia accumulated within the uterine cavity.

Effects of Topical N-Acetylcysteine on Skin Hydration/Transepidermal Water Loss in Healthy Volunteers and Atopic Dermatitis Patients

No abstract available.