Clarkson’s disease in children (references review)
DOI:
https://doi.org/10.15574/SP.2022.127.81Keywords:
children, syndrome of increased permeability of capillaries, immune dysregulationAbstract
Clarkson’s disease is an idiopathic systemic capillary permeability syndrome characterized by life-threatening hypotension, hypoalbuminemia, and hemoconcentration, often leading to hypovolemic shock.
Purpose - to conduct a systematic review of literature sources on the mechanisms of development, clinical, laboratory and instrumental changes, as well as methods of treatment and prevention of the syndrome of increased permeability of capillaries.
The literature review is devoted to the problem of idiopathic systemic syndrome of increased capillary permeability in pediatric practice. This syndrome can be idiopathic (Clarkson’s disease) or secondary to various conditions, such as engraftment syndrome after hematopoietic stem cell transplantation, viral and bacterial infections, taking certain medications, such as chemotherapy. The periods of its development are highlighted, as well as possible mechanisms of development and links of pathogenesis, in particular, hypotheses that indicate immune dysregulation with an increase in circulating CD25+ T-lymphocytes, which leads to the activation of proteolytic enzymes and the development of endothelial dysfunction. The available clinical, laboratory and instrumental disorders manifested in Clarkson’s disease are characterized. Special attention is paid to possible variants of the course of the Clarkson's disease, which can also have a chronic variant of the course without specific clinical signs. Possible causes of the development of secondary syndrome of increased permeability of capillaries are also identified, such as sepsis, trauma, implantation syndrome and ovarian hyperstimulation, viral hemorrhagic fevers, autoimmune and hematological diseases.
Since there are currently no clear recommendations for the prevention and treatment of the disease, it is important to become familiar with and better understand the effectiveness of the various therapeutic approaches currently in use, аnd also analyze the possibility of their use in daily practice.
No conflict of interests was declared by the authors.
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