Sex hormones in hereditary angioneurotic oedema

Visy, Beáta; Füst, George; Varga, Lilian; Szendei, G; Takács, Eszter; Karádi, István; Fekete, B.; Harmat, G.; Farkas, Henriette

doi:10.1111/j.1365-2265.2004.02009.x

Cited by 56 publications

(40 citation statements)

References 19 publications

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“…Symptoms often worsen during puberty, particularly in females 3, 14, 31, 32. Onset of symptoms may occur with the introduction of estrogen‐containing medications for acne or birth control 17.…”

Section: Resultsmentioning

confidence: 99%

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Farkas

Martinez‐Saguer²,

Bork

et al. 2016

Allergy

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176

308

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BackgroundThe consensus documents published to date on hereditary angioedema with C1 inhibitor deficiency (C1‐INH‐HAE) have focused on adult patients. Many of the previous recommendations have not been adapted to pediatric patients. We intended to produce consensus recommendations for the diagnosis and management of pediatric patients with C1‐INH‐HAE.MethodsDuring an expert panel meeting that took place during the 9th C1 Inhibitor Deficiency Workshop in Budapest, 2015 (www.haenet.hu), pediatric data were presented and discussed and a consensus was developed by voting.ResultsThe symptoms of C1‐INH‐HAE often present in childhood. Differential diagnosis can be difficult as abdominal pain is common in pediatric C1‐INH‐HAE, but also commonly occurs in the general pediatric population. The early onset of symptoms may predict a more severe subsequent course of the disease. Before the age of 1 year, C1‐INH levels may be lower than in adults; therefore, it is advisable to confirm the diagnosis after the age of one year. All neonates/infants with an affected C1‐INH‐HAE family member should be screened for C1‐INH deficiency. Pediatric patients should always carry a C1‐INH‐HAE information card and medicine for emergency use. The regulatory approval status of the drugs for prophylaxis and for acute treatment is different in each country. Plasma‐derived C1‐INH, recombinant C1‐INH, and ecallantide are the only agents licensed for the acute treatment of pediatric patients. Clinical trials are underway with additional drugs. It is recommended to follow up patients in an HAE comprehensive care center.ConclusionsThe pediatric‐focused international consensus for the diagnosis and management of C1‐INH‐HAE patients was created.

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Section: Resultsmentioning

confidence: 99%

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Farkas

Martinez‐Saguer²,

Bork

et al. 2016

Allergy

Self Cite

176

308

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“…Visy and colleagues 11 showed that, in 44 women with HAE, the frequency of attacks correlated with estrogen and progesterone levels. The female sexual hormones act on several proteins, including the kallikrein-kinin system.…”

Section: Physiopathologymentioning

confidence: 98%

Hereditary Angioedema in Women

Bouillet

Gompel

2013

Immunology and Allergy Clinics of North America

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“…Önceden alınmış olan progesteron içeren ekzojen preparatların, endojen progesteronlara karşı duyarlılık doğuruyor olabileceği ileri sürülmüştür. Bu görüşe göre ekzojen progesteronlar antijenik özellikte olduğundan antikor oluşumuna neden olmakta, oluşan antikorlar da endojen progesteronlarla reaksiyona girip premenstrüel dönemde immün yanıta yol açmaktadır (2). Ancak bizim olgularımızın progesteron içeren ilaç kullanım öyküsü yoktu ve bu durum patogenezde başka faktörlerin de rolünün olabileceğini düşündürmektedir.…”

Section: Discussionunclassified

“…Çeşitli kutanöz ve mukokutanöz bulgular siklik bir şekilde menstrüel siklusun luteal fazında başlar, menstrüel kanamanın başlamasıyla birlikte sonlanır (1). Klinik semptomları arasında egzama, vezikül, püstül, eritema multiforme, ürtiker ve anafilaksi vardır (2). Bu yazıda 4-5 yıl sonra tanı alabilmiş, ürtikeryal lezyonlarla seyreden 2 OPD olgusu sunulmaktadır.…”

Section: Introductionunclassified

Autoimmune Progesterone Dermatitis: Two Case Reports

Benar¹,

Cemil²,

Atay³

et al. 2018

tdd

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Otoimmün progesteron dermatiti, nadir görülen ve endojen progesterona karşı gelişen bir hipersensitivite reaksiyonudur. Döngüsel olarak menstrüel siklusun luteal fazında başlayıp, menstrüel kanamanın başlamasından sonra sonlanan, kutanöz ve mukokutanöz bulgularla karakterize bir durumdur. Bu çalışmada, ürtikeryal lezyonlarla seyreden ve otoimmün progesteron dermatiti tanısı konulan 42 ve 49 yaşındaki iki olgu sunulmaktadır. Her iki hastanın da menstrüel kanamadan bir ya da iki hafta önce başlayıp, kanamayla birlikte sonlanan ürtikeryal döküntü öyküsü vardı. Parenteral progesteron preparatı ile yapılan intradermal testler her iki hastada da pozitif olarak saptandı. Hastaların öyküsü, klinik bulguları ve progesteron ile yapılan intradermal testin pozitif olması sonucunda otoimmün progesteron dermatiti tanısı kondu. Bu çalışma, nadir görülen bir hastalık olması nedeniyle tanının atlanabileceğini ve iyi bir anamnezin tanıdaki önemini vurgulamak amacıyla sunulmuştur. Öz AbstractAutoimmune progesterone dermatitis is a rare hypersensitivity reaction that develops against endogenous progesterone. This condition is characterized by cutaneous and mucocutaneous findings that begins cyclically in the luteal phase of the menstrual cycle and ends after the onset of menstrual bleeding. In this study, we present two cases aged 42 and 49 years who were had urticarial lesions and diagnosed with autoimmune progesterone dermatitis. Both patients had a history of urticaria rash that started one or two weeks before the menstruation and ended with bleeding. Intradermal tests with parenteral progesterone preparation were found to be positive in both patients. As a result of patients' history, clinical findings and the positive intradermal test with progesterone, autoimmune progesterone dermatitis diagnosed. It has been aim to emphasize the importance of anamnesis in diagnosis of this rarely seen disease.

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Sex hormones in hereditary angioneurotic oedema

Abstract: In view of these results, the monitoring of progesterone and SHBG levels can prove useful in the prediction of attacks in hereditary angioneurotic oedema.

Cited by 56 publications

References 19 publications

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Hereditary Angioedema in Women

Autoimmune Progesterone Dermatitis: Two Case Reports

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