engleski

SPECIFICITY OF ABDOMINAL FORM OF HEREDITARY ANGIOEDEMA IN PREGNANT WOMEN

Introduction: Hereditary angioedema (HAE) is rare for life-threatening illness due to the onset of seizures of painless, limited, recurrent submucosal, subcutaneal and interstitial tissues swelling. Attack lasts for several hours to several days. Incidence is 1:50 000, and in our country there is about 100 patients in register. Abdominal form is a particular entity of this disease due to the ability of mimicry and presentation as any other form of acute abdominal events. Methods: The abdominal type of HAE is manifested with abrupt abdominal pain, followed by abdominal distension, nausea.This symptoms are occurring in approximately 70-90% of the patients (depending on the authors). As the disease is presented as an acute abdomen, physicians often reach for an urgent surgical procedure, expecting an acute appendicitis, small bowel obstruction, diverticulitis, billiary attack etc. Diagnostic procedure is complicated by the unusual nature of this disease-the ability to self-relieve, so the time to set up the correct diagnosis will often prolong until 10 years. Diagnostic procedure includes assessment basic C3 and C4 components, and abdominal ultrasound to prove or exclude the free fluid in the abdominal cavity. Abdominal MSCT will show relaxed and thickened bowels with the release of free liquid without signs of obstruction. By excluding the obstruction of any vascular or other etiology, acute surgery will be avoided because HAE is a partially or completely “self-relieving” disease. Case report: pregnant woman 35 years old treated for HAE from childhood, now by Fyrazir (icatibant) as needed. During pregnancy attacks of HAE almost 2 x a week, which are somewhat controlled by injection of CINRYZE (INN-C1 inhibitor) and with BERINERT (INN-C1 inhibitor). Frequent attacks of HAE in the form of swelling of the extremities, severe abdominal pain accompanied by diarrhea, and occasional swelling of one side of the face and half of the lips. . In early March, 2021. cesarean delivery was done. The Commission for Medicines of General Hospital Zadar, approved the injection of Fyrazir for home use.

hereditary angioedema ; pregnancy

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