Autor: |
Abass MK; Department of Pediatrics, Sheikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates., Dabosy A; Department of Pediatrics, Sheikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates.; Pediatric Rheumatology Pediatric Division, Sheikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates., Walid Khawaja K; Pediatrics Rheumatology, Sheikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates., Fischer PR; Department of Pediatrics, Sheikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates.; Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN, USA.; Department of Pediatric, Khalifa University College of Medicine and Health Sciences, Abu Dhabi, United Arab Emirates. |
Abstrakt: |
A 16-year-old female presented to an outpatient clinic with a 13-year history of recurrent episodes of abdominal pain, vomiting and mild cutaneous swelling, either spontaneously or following minor trauma. The episodes occurred every 1-2 months. There was no family history of a similar complaint or hereditary angio-oedema (HAE). At the age of 16, evaluation confirmed the diagnosis of HAE type II, characterised by low C4 levels and reduced C1 esterase inhibitor function. The patient was prescribed tranexamic acid 1 g twice daily as well as C1 esterase inhibitor used as rescue medication during symptomatic episodes. This case report emphasises the importance of considering a diagnosis of HAE in patients with recurrent, unexplained abdominal pain, even in the absence of a positive family history of HAE. Abbreviations: ANA Antinuclear antibodies; C1-INH C1-inhibitor; CBC Complete blood count; FMF Familial Mediterranean fever; HAE Hereditary angioedema; IBD Inflammatory bowel diseases; SDP Solvent detergent-treated plasma; SLE Lupus erythematosus. |