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Background: Ataxia-telangiectasia (A-T) is a rare autosomal recessive DNA repair disorder, characterized by progressive cerebellar degeneration, telangiectasia, immunodeficiency, recurrent sinopulmonary infections, radiation sensitivity, premature aging and predisposition to cancer. Although the association with autoimmune and chronic inflammatory conditions such as vitiligo, thrombocytopenia and arthritis has occasionally been reported, an onset with articular involvement at presentation is rare. Case presentation: We herein report the case of a 7-year-old Caucasian girl who was admitted to the Rheumatology Department with a history of febrile chough and polyarthritis which led initially to the suspicion of an autoinflammatory disease. She had overt polyarthritis with knees deformities and presented with severe pneumonia. A chest Computed Tomography (CT) scan showed bilateral bronchiectasis, parenchymal consolidation and interstitial lung disease; rheumatoid factor and type I interferon signature resulted negative, therefore excluding COatomer Protein subunit Alpha (COPA) syndrome. A diagnosis of sarcoidosis had been suspected based on histological evidence of granulomatous liver inflammation, but ruled out after detecting normal angiotensin converting enzyme and chitotriosidase blood levels. Based on her past medical history characterized by at least six episodes of pneumonia in the previous 4 years, immunological phenotyping was performed. This showed complete IgA and IgE deficiency with defective antigen-specific antibodies to Pneumococcal, Tetanus toxin and Hemophilus Influenzae B vaccines. Additionally, low numbers of B cells and recent thymic emigrants (RTE) were found (CD4Ra 1.4%), along with a low CD4+/CD8 + T cells ratio (< 1). Finally, based on gait disturbances (wobbly wide-based walking), serum alfa-fetoprotein was dosed, which resulted increased at 276 ng/ml (normal value < 7 ng/ml). A diagnosis of Ataxia-Telangiectasia was made, strengthened by the presence of bulbar telangiectasia, and then confirmed by Whole Exome Sequencing (WES). Conclusions: Although rare, A-T should always be ruled out in case of pulmonary bronchiectasis and gait disturbances even in the absence of bulbar or skin telangiectasia. Autoimmune and granulomatous disorders must to be considered as differential diagnosis. [ABSTRACT FROM AUTHOR]

Action Induced Myoclonus in a 11-Year-Old Boy with Silver-Russell Syndrome Myoclonus-dystonia syndrome associated with Russell silver syndrome. 8 Martins J, Gabriel D, Borges T, Soares G, Temudo T. Child neurology: myoclonus-dystonia in Russell-silver syndrome: two syndromes caused by one genetic defect. [Extracted from the article]

Post-Acute COVID-19 syndrome (PACS) is considered to be one of the least understood post-infectious syndromes. We report a case of a 21-year-old female who had a history of SARS-CoV-2 infection and presented with a right atrioventricular thrombus associated with pulmonary embolism and thrombocytopenia. At the time of admission, she was not vaccinated against SARS-CoV-2, and her serological tests for IgG and IgM antibodies against SARS-CoV-2 were positive. The size of the thrombus measured approximately 6 × 8 × 4 cm, which also led to tricuspid valve insufficiency due to mechanical dilatation of the valve's ring. The right atrioventricular thrombus also extended up to the inferior vena cava, leading to mild congestive hepatomegaly. Moreover, during thrombectomy, the mass of the thrombus was attached to the interseptal right atrial wall. The histopathological assessment of the core mass revealed that it was a right atrial myxoma hidden inside that large thrombus. We suspect that the formation and propagation of the thrombus to that size occurs as a part of Post-Acute COVID-19 syndrome (PACS). This study reviews and discusses coronavirus disease 2019-relate to thrombus formation inside cardiac chambers in case of a cardiac tumor, like myxoma in the setting of post-acute phase COVID-19 syndrome. [ABSTRACT FROM AUTHOR]

Copyright of Psicoterapia Cognitiva e Comportamentale is the property of Edizioni Centro Studi Erickson SpA and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Hydatid cyst is a zoonotic endemic disease in Iraq. It carries considerable complications either related to the disease itself or following its surgical removal. Pleural and pericardial effusion is a rare postoperative complication of hydatid cyst removal. A 65year-old Iraqi female patient complained of right-sided chest heaviness and palpitation one month following surgical removal of 2 large hydatid cysts. There were no other associated symptoms. Physical examination revealed the possibility of pleural and pericardial effusions (poor air entry and stony dullness of the right posterior part of lower zone, difficulty to palpate the apex beat, increase heart dullness, and no pericardial rub). The diagnosis was confirmed by chest X-ray and echocardiogram. She received Albendazole tablet 400 mg twice daily for 6 weeks with a good response. The Early presumption of the disease in an endemic area with a good dose and appropriate course of Albendazole tablets treatment can give very good results. To our best knowledge, this is the first case in the world who presented with hydatid cyst pleural and pericardial effusions and responded completely to the Albendazole tablets. [ABSTRACT FROM AUTHOR]

Copyright of Der Chirurg is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

A case of malignant mesothelioma of the tunica vaginalis testis is reported in a 77-year-old male patient. There was no history of asbestos exposure. Recurrent right hydrocele with a papillar inguinal mass was the main clinical feature. An inguinal radical orchiectomy with en bloc resection of the surrounding tissue was performed. The therapeutic options for this rare, but aggressive neoplasm are discussed. Because of the disappointing results of antineoplastic chemotherapy or radiation therapy, the importance of initial radical surgical treatment with complete excision is emphasized. Es wird über ein malignes Mesotheliom der Tunica vaginalis testis bei einem 77 jährigen Patienten berichtet. Anamnestisch ergab sich kein Hinweis für eine Asbestexposition. Klinisch fand sich eine rezidivierende Hydrocele in Kombination mit einer knotigen Induration der rechten Leiste. Eine inguinale Orchiektomie mit En-bloc-Resektion des umliegenden Gewebes wurde durchgeführt. Die Therapiealternativen dieses seltenen, aber aggressiven Tumors und insbesondere die Bedeutung der initial radikalen chirurgischen Therapie bei enttäuschenden Ergebnissen der antineoplastischen Chemo- oder Strahlentherapie werden diskutiert. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Clinical Periodontology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Der Chirurg is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Spontaneous regression of metastatic renal cell carcinoma is a rare but well documented event, most often involving pulmonary metastases. A case of spontaneous regression of histologically verified pleural metastases after nephrectomy is reported. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Clinical Periodontology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)