ABNORMAL NAILFOLD VIDEOCAPILLAROSCOPY IN  PATIENTS WITH ANTISYNTHETASE SYNDROME: CASE REPORTS AND REVIEW OF THE LITERATURE

Classified as a distinct subset of idiopathic inflammatory myopathies, anti-synthetase syndrome is an orphan autoimmune disease characterized by the presence of anti-aminoacyl-tRNA synthetase antibodies associated with a spectrum of clinical features including interstitial lung disease, myositis or dermatomyositis, chronic polyarthritis, fever, mechanic‘s hands and Raynaud‘s phenomenon. The main objective of our study was to evaluate nailfold capillary abnormalities in a series of cases diagnosed with anti-synthetase syndrome, aiming to describe capillaroscopy findings and to identify potential relation with clinical and functional outcomes. Irrespective of clinical and serological disease subtype, significant capillaroscopic changes may help diagnosis and identify negative prognostic factors, advancing the role of routine capillaroscopy in the decisional algorithm of anti-synthetase syndrome. Nailfold videocapillaroscopy is able to provide valuable information in patients with anti-tRNA synthetase myositis, suggesting that diffuse microangiopathy represents an early pathobiologic event.

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Embryonal tumor with abundant neuropil and true rosettes in the brainstem: case report

Journal of Neurosurgery Pediatrics ◽

10.3171/2015.3.peds14727 ◽

2015 ◽

Vol 16 (3) ◽

pp. 291-295 ◽

Cited By ~ 3

Author(s):

Hidetoshi Sato ◽

Yuzo Terakawa ◽

Naohiro Tsuyuguchi ◽

Yuko Kuwae ◽

Masahiko Ohsawa ◽

...

Keyword(s):

Case Report ◽

Surgical Treatment ◽

Case Reports ◽

Subtotal Resection ◽

Surgical Removal ◽

Rare Entity ◽

Review Of The Literature ◽

Embryonal Tumor

Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is rarely seen in the brainstem, and there are few case reports of brainstem ETANTR in the literature. Accordingly, the characteristics and the role of surgical treatment of this rare entity remain unclear. The authors present a case of brainstem ETANTR involving a 33-month-old boy along with a review of the literature and discuss the role of surgical removal in the treatment of this entity. In the authors’ case, the tumor was surgically treated with subtotal resection, which resulted in improvement of the patient’s preoperative symptoms. Chemotherapy was initiated but did not appear to be effective, radiotherapy was declined, and the boy died 6 months after the operation. Based on their analysis of 10 previously reported cases and their own case, the authors conclude that, with respect to survival, surgery may be beneficial even in cases of ETANTR in the brainstem. They note, however, that further studies with a large number of cases are needed to validate the role of surgical treatment in brainstem ETANTR.

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Correction of aminoacylation errors: Evidence for a non significant role of the aminoacyl-tRNA synthetase catalysed deacylation of aminoacyl-tRNAs

FEBS Letters ◽

10.1016/0014-5793(74)80125-0 ◽

1974 ◽

Vol 39 (3) ◽

pp. 259-262 ◽

Cited By ~ 19

Author(s):

J. Bonnet ◽

J.P. Ebel

Keyword(s):

Significant Role ◽

Trna Synthetase ◽

Aminoacyl Trna Synthetase

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Role of two aminoacyl-tRNA synthetase associated proteins (Endothelial Monocyte Activating Polypeptides 1 and 2) of Leishmania donovani in chemotaxis of human monocytes.

Acta Tropica ◽

10.1016/j.actatropica.2021.106128 ◽

2021 ◽

Vol 224 ◽

pp. 106128

Author(s):

Smriti Tandon ◽

Rohini Muthuswami ◽

Rentala Madhubala

Keyword(s):

Leishmania Donovani ◽

Trna Synthetase ◽

Human Monocytes ◽

Aminoacyl Trna Synthetase ◽

Associated Proteins

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Thrombotic microangiopathy in the course of catastrophic antiphospholipid syndrome successfully treated with eculizumab: case report and systematic review of the literature

Lupus ◽

10.1177/0961203320917460 ◽

2020 ◽

Vol 29 (6) ◽

pp. 631-639 ◽

Cited By ~ 1

Author(s):

Marta Skoczynska ◽

Mark A Crowther ◽

Malgorzata Chowaniec ◽

Monika Ponikowska ◽

Shruti Chaturvedi ◽

...

Keyword(s):

Systematic Review ◽

Case Report ◽

Antiphospholipid Syndrome ◽

Lupus Erythematosus ◽

Single Case ◽

Case Reports ◽

Review Of The Literature ◽

Catastrophic Antiphospholipid Syndrome ◽

Total Remission

Objective The purpose of this study was to characterize the role of eculizumab, a monoclonal antibody against the terminal complement component C5, in patients with catastrophic antiphospholipid syndrome (CAPS). Methods We present a case report of a patient with systemic lupus erythematosus (SLE) and CAPS treated with eculizumab, as well as results of a systematic review of the literature. Results Including our patient, we identified 11 case reports of patients with CAPS treated with eculizumab. All of them had partial or total remission of symptoms. Conclusion Data on eculizumab efficacy in CAPS are promising but are limited to single case reports. More studies are needed to develop evidence-based recommendations for eculizumab use in CAPS.

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