scholarly journals Surgical treatment of Jeune syndrome in a child (rare clinical case and review)

2021 ◽  
Vol 28 (1) ◽  
pp. 45-52
Author(s):  
Igor A. Komolkin ◽  
Aleksandr Yu. Mushkin ◽  
Olga E. Agranovich ◽  
Ardan P. Afanasiev
Keyword(s):  
Respiratory Failure ◽  
Surgical Treatment ◽  
Clinical Case ◽  
Multidisciplinary Approach ◽  
Severe Disease ◽  
Positive Outcome ◽  
Case Review ◽  
Jeune Syndrome ◽  
Chest Deformity ◽  
Syndrome Analysis

Objective: possibility of surgical treatment of chest deformity in children with Jeune syndrome (analysis of the clinical case), review of literature on Jeune syndrome in children. Methods: clinical, radiological, laboratory, instrumental, statistical. Results: Positive outcome of surgical treatment of a 3-year-old patient with severe chest deformity and severe respiratory failure. Conclusions: Jeune syndrome is a severe disease with a high mortality rate. A multidisciplinary approach is required in the treatment of patients with this pathology. Surgical intervention aimed at reducing the compression of the chest organs and increasing its volume, even in conditions of decompensation, allows you to stop the phenomenon of respiratory failure.

scholarly journals The case of detection and differential diagnosis of Hypell-Lindau disease in a patient as a result of a comprehensive examination of a patient in a chronic critical condition

2020 ◽  
Author(s):  
A Yakovleva
Keyword(s):  
Differential Diagnosis ◽  
Surgical Treatment ◽  
Family History ◽  
Critical Condition ◽  
Clinical Case ◽  
Multidisciplinary Approach ◽  
Von Hippel Lindau ◽  
Live Births ◽  
Comprehensive Examination ◽  
Von Hippel Lindau Syndrome

According to evidence in the literature occurrence of the Hippel Lindau disease changes from 1: 33000 - 1: 36000 (population frequency) to 1 in 39000 live births. Up to 20% of them have not family history. Hereditary predisposition of most patients with systemic angioreticulomatosis has patterns and attributes. Diversity of forms and infrequent cases of the Hippel Lindau disease complicate timely diagnosis. Untimely control of condition of patient with the Hippel Lindau disease increases the probability of complications in surgical treatment. It can be the reason of early disability too. The presented clinical case reflects the need for a multidisciplinary approach to the management of patients with von Hippel-Lindau syndrome.

Experience of surgical treatment of false popliteal artery aneurysm and tibioperoneal stem. Clinical case

2019 ◽  
Vol 17 (6 (part 2)) ◽  
pp. 84-85
Author(s):  
M. V. Plotnikov ◽  
◽  
E. A. Gaysina ◽  
R. M. Nuretdinov ◽  
L. M. Muhametdinova ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Popliteal Artery ◽  
Clinical Case ◽  
Artery Aneurysm ◽  
Popliteal Artery Aneurysm

Thyroidectomy in a patient with a giant multinodular nontoxic cervical retrosternal goiter

2020 ◽  
pp. 97-99
Author(s):  
U. V. Kukhtenko ◽  
O. A. Kosivtsov ◽  
L. A. Ryaskov ◽  
E. I. Abramian
Keyword(s):  
Surgical Treatment ◽  
Clinical Case ◽  
Clinical Signs ◽  
Disease Relapse ◽  
Cardiac Pathology ◽  
Nontoxic Goiter ◽  
Retrosternal Goiter

A clinical case of successful surgical treatment of a patient with a giant cervical retrosternal nontoxic goiter with severe cardiac pathology is presented. Thyroidectomy from cervical access without sternotomy was performed. At the follow-up examination 5 months after the operation, instrumental and clinical signs of disease relapse were not detected.

AB0980 CASE-REVIEW ON FAMILIAL MEDITERRANEAN FEVER IN A SPECIALIZED CENTRE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1784.1-1784
Author(s):  
R. Dos Santos Sobrín ◽  
M. Martí Masanet ◽  
B. Lopez-Montesinos ◽  
L. Lacruz Pérez ◽  
I. Calvo
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Familial Mediterranean Fever ◽  
Periodic Fever ◽  
Severe Disease ◽  
Strong Association ◽  
Mefv Gene ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Case Series ◽  
Case Review ◽  
Mediterranean Fever

Background:Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder caused mostly by mutations in MEFV gene. Its inheritance is autosomal recessive and is the most frequent periodic fever syndrome. First-line treatment is based in colchicine use, so biologics (anti-IL-1) are reserved for refractory cases1, 2.Objectives:To account for clinic and treatment features of patients with FMF in a specialized center as opposed to non-referent centers.Methods:This study was developed in the Pediatric Rheumatology Service in Hospital Universitario y Politécnico La Fe de Valencia. Demographic, clinic and treatment data were collected from patients diagnosed of FMF since January 2004 to September 2019.Results:106 patients met last FMF criteria3. 55% had a pathogenic mutation in genetic analysis. 52% were female. Before 10 years old, 71% of patients had the diagnosis (51% before 4 years old). Arthralgia/myalgia (73%), periodic fever (62%) and abdominal pain (54%) were the most common symptoms. Systemic Juvenile Idiopathic Arthritis (JIA, 6), other forms of JIA (9) and vasculitis (10) were the most prevalent comorbidities. When talking about treatment, 76,4% received Colchicine (60,5% with good response), 22,6% needed a classical disease modifying antirheumatic drug (mostly Methotrexate) and 22 patients got biologic treatment (73% anti-IL-1).Conclusion:When analyzing this case-review, JIA has a strong association with our patients, so it could explain severe disease activity and more articular involvement. This could be an illustration to the higher use of Methotrexate. Also, the most relevant symptom was arthralgia while fever is the most frequent in literature. Likewise, age of diagnosis has been earlier than other case-series (this would be more frequent in other autoinflammatory syndromes, as literature relates)1, 2, 4.References:[1]Ozdogan H, Ugurlu S. Familial Mediterranean Fever. Presse Med. (2019).[2]Ozen S, Demirkaya E, Erer B, et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis 2016;75:644-651.[3]Sag E, Demirel D, Demir S, et al. Performance of the new “Eurofever/PRINTO classification criteria” in FMF patients. Semin Arthritis Rheum. 2019;19:30369-5.[4]Rozenbaum M, Rosner I. Severe outcome of juvenile idiopathic arthritis (JIA) associated with familial Mediterranean fever (FMF). Clin Exp Rheumatol. 2004;22:S75-8.Disclosure of Interests:Raquel Dos Santos Sobrín: None declared, Miguel Martí Masanet: None declared, B Lopez-Montesinos: None declared, Lucía Lacruz Pérez: None declared, Inmaculada Calvo Grant/research support from: Bristol-Myers Squibb, Clementia, GlaxoSmithKline, Hoffman-La Roche, Merck Sharpe & Dohme, Novartis, Pfizer, Sanofi, Speakers bureau: AbbVie, GlaxoSmithKline, Hoffman-La Roche, Novartis

scholarly journals SARS-CoV-2 and Obesity: “CoVesity”—a Pandemic Within a Pandemic

2021 ◽  
Author(s):  
Kimberley Zakka ◽  
◽  
Swathikan Chidambaram ◽  
Sami Mansour ◽  
Kamal Mahawar ◽  
...  
Keyword(s):  
Systematic Review ◽  
Risk Factor ◽  
Viral Load ◽  
Respiratory Failure ◽  
Health Outcomes ◽  
Severe Disease ◽  
Vulnerable Population ◽  
Low Grade ◽  
Obesity Management ◽  
Low Grade Inflammation

AbstractIndividuals who are overweight or suffering from obesity are in a chronic state of low-grade inflammation, making them particularly susceptible to developing severe forms of respiratory failure. Studies conducted in past pandemics link obesity with worse health outcomes. This population is thus of particular concern within the context of the COVID-19 pandemic, considering the cessation of obesity management services. This systematic review highlights [1] the reciprocal link between the obesity and COVID-19 pandemics, [2] obesity as a risk factor for more severe disease in past pandemics, [3] potential mechanisms that make individual’s suffering from obesity more susceptible to severe disease and higher viral load, and [4] the need to safely resume bariatric services as recommended by expert guidelines, in order to mitigate the health outcomes of an already vulnerable population.

scholarly journals Thromboembolism after COVID-19 vaccine in patients with preexisting thrombocytopenia

2021 ◽  
Vol 12 (8) ◽  
Author(s):  
Alessandro Mauriello ◽  
Manuel Scimeca ◽  
Ivano Amelio ◽  
Renato Massoud ◽  
Antonio Novelli ◽  
...  
Keyword(s):  
Adverse Drug Reaction ◽  
Drug Reaction ◽  
Exome Sequencing ◽  
Clinical Case ◽  
Severe Disease ◽  
Low Frequency ◽  
Postmortem Examination ◽  
Capillary Leak ◽  
Platelet Factor ◽  
Whole Exome

AbstractWhile vaccination is the single most effective intervention to drastically reduce severe disease and death following SARS-CoV-2 infection, as shown in UK and Israel, some serious concerns have been raised for an unusual adverse drug reaction (ADR), including vaccine-induced immune thrombotic thrombocytopenia (VITT) with concurrent low platelets as well as capillary leak syndrome. In fact, the overall safety of the vaccine is highlighted by the low frequency of ADR considering that in UK, by the early June, 40 million first doses and 29 million second doses have been injected; nonetheless, 390 thrombotic events, including 71 fatal events have been reported. Interestingly, the cases reported low platelet counts with the presence of anti-platelet factor-4 (PF4) antibodies, indicating an abnormal clotting reaction. Here, out of three referred cases, we report a post-vaccine clinical case of fatal thrombosis with postmortem examination and whole exome sequencing (WES) analysis, whose pathogenesis appeared associated to a preexisting condition of thrombocytopenia due to myelodysplasia.

Surgical treatment as option in treatment of metastatic hormone-sensitive prostate cancer after prior drug treatment

2022 ◽  
pp. 41-45
Author(s):  
Sh. G. Khakimova ◽  
G. G. Khakimova ◽  
G. A. Khakimov ◽  
J. B. Sadullaev
Keyword(s):  
Prostate Cancer ◽  
Surgical Treatment ◽  
Drug Treatment ◽  
Bone Metastases ◽  
Clinical Result ◽  
Metastatic Prostate Cancer ◽  
Clinical Case ◽  
Complex Treatment ◽  
Good Clinical Result ◽  
Hormone Sensitive

Currently, there is no consensus on the place of prostatectomy in the complex treatment of patients with metastatic prostate cancer. A description of a clinical case of complex treatment and observation of a patient with prostate cancer with an unfavorable baseline prognosis and the presence of bone metastases with a good clinical result is presented.

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