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1.
Tohoku J Exp Med ; 262(1): 23-27, 2024 Jan 10.
Article in English | MEDLINE | ID: mdl-37793880

ABSTRACT

Hereditary angioedema is a rare, potentially life-threatening disease. There is a lack of data describing the clinical course of hereditary angioedema (HAE) in children. We aimed to evaluate the clinical characteristics of pediatric patients with hereditary angioedema: The age of disease onset, age at diagnosis, the frequency of angioedema attacks, the total number of attacks before diagnosis, the regions where angioedema attacks were observed, accompanying abdominal pain, and serum levels of C4 and C1 esterase inhibitor were obtained and recorded. In addition, the results of SERPING1 (C1INH) gene sequence analysis of the patients in this group were also collected from medical records and recorded. While none of the patients reported a skin rash as a symptom of attack, there was formication observed in the region of angioedema in 46.9% (n = 15) of the patients and pruritus in 6.2% (n = 2) of the patients. At disease onset, the complaints of the patients regarding location of edema were on the hands of 32.3% (n = 10), on the feet of 9.7% (n = 3), on the faces of 25.7% (n = 8), and abdominal attacks in 32.3% of the patients (n = 10). Four different variants, one of which was novel, were detected in the SERPING1 gene in eight different families. The results of this study suggest that hereditary angioedema is diagnosed only when the patient requests examination following recurrent angioedema. Severe laryngeal edema attacks in patients without a diagnosis of HAE are fatal at a higher rate than attacks in patients with a diagnosis. Thus, awareness of the symptoms of HAE is necessary, and correct diagnosis is essential to proper treatment.


Subject(s)
Angioedema , Angioedemas, Hereditary , Humans , Child , Angioedemas, Hereditary/diagnosis , Angioedemas, Hereditary/genetics , Angioedemas, Hereditary/drug therapy , Complement C1 Inhibitor Protein/genetics , Complement C1 Inhibitor Protein/therapeutic use , Angioedema/diagnosis , Upper Extremity
2.
Cureus ; 15(11): e48174, 2023 Nov.
Article in English | MEDLINE | ID: mdl-38046775

ABSTRACT

BACKGROUND: Atopic diseases can accompany allergic sensitization. The amount of allergen is as important to know since allergen exposure affects sensitization development. Some allergic children complain of intense sweating during the first few hours of transition to sleep especially at night. This study was carried out to investigate the relationship between the type of allergen to which children are sensitized and night sweating. METHODS: Children aged two to 18 years old with single allergic sensitization were included. A specific immunoglobulin E (IgE) skin prick test results were obtained from the medical records of the patients. Then, the patients' families were asked to evaluate the levels of sweating from 0 to 10 with the Visual Analogue Scale (VAS) questionnaire and the Sleep Disturbance Scale for Children (SDSC). RESULTS: Sensitization to mites was more prevalent in the group of patients with night sweating (p<0.001), and pollen allergy was more prevalent in the group of patients without night sweating (p<0.001).  Conclusion: The amount of mites and wetness in bed sheets might be responsible for allergic sensitization. A change in clothes and bed sheets for those with intense sweating might ensure better sleep and reduce the severity of the atopic symptoms. Such a recommendation might provide a better clinical outcome in these patients.

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