The topic C1 Esterase Inhibitor Deficiency you are seeking is a synonym, or alternative name, or is closely related to the medical condition Hereditary Angioedema.

Quick Summary:

• Hereditary Angioedema (HAE) is an uncommon, genetically inherited condition. Hereditary Angioedema is a severe disorder affecting the body's response to certain triggers
• The system which controls the regulation of tissue fluids, blood vessel dilation and constriction, and itching, results in hives (urticaria) and localized deep swelling (angioedema) when triggered. Multiple biochemical agents participate in triggering up and downregulating the clinical features of hives and angioedema
• In Hereditary Angioedema, there is a decreased amount of a particular protein known as C1 esterase inhibitor. The function of this protein is to maintain the fluid that enters and exits tissues at the normal level. A defect in this protein leads to an abnormal buildup of fluid. The excessive accumulation of fluid in the tissues creates an obstruction to normal blood flow
• Too much fluid causes swelling in various areas of the body such as the face, hands, leg, feet, and throat. Depending on the severity of the condition, some individuals may be affected by an obstruction of the airway passage. Airway obstruction is an acute emergency and requires urgent treatment. If Hereditary Angioedema is not diagnosed and treated early, the condition can be fatal
• The treatment options for Hereditary Angioedema are twofold. Acute attacks are treated with supportive care with the administration of oxygen and IV fluids. In attacks, ecallantide or icatibant can be administered, as well as C1 inhibitor medications. The same C1 inhibitor medications (landelumab and berotralstat) are available for self-administration to prevent attacks and are used as soon as a potential trigger is noted by the patient. These medications are not necessarily approved in all countries

Please find comprehensive information on Hereditary Angioedema regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.