Hereditary angioedema: an unusual cause of genital swelling presenting to a genitourinary medicine clinic

Dhairyawan, Rageshri; Harrison, Richard A.; Buckland, Matthew; Hourihan, M

doi:10.1258/ijsa.2011.010401

Cited by 7 publications

(4 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

Section: Introductionmentioning

confidence: 99%

“…The disease commonly affects extremities and can cause temporary debilitation and disfigurement that can last for up to a week. Facial oedema may occasionally progress to laryngeal swelling, which can be life threatening, causing prolonged intensive respiratory care or even death from asphyxia [15][16][17]. Bradykinin binds to the B2 receptor and stimulates production of cGMP leading to an increase in prostaglandin-2 (PGI-2) and nitric oxide, which results in vasodilation and increase in permeability.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Update on treatment of hereditary angioedema

Buyantseva

Agarwal

et al. 2013

Clin Experimental Allergy

View full text Add to dashboard Cite

Hereditary angioedema (HAE) is a rare disease characterized by recurrent, self-limiting episodes of swelling. New research and therapies have recently emerged and are now available; however, many physicians are not aware of the new developments in HAE. To update immunologists and other health care providers on new advances in HAE therapies, a PubMed, OVID and Google literature search were used to develop this manuscript. English language peer-reviewed angioedema articles were selected. High quality clinical trials were reviewed and summarized. Acute therapy in the past often consisted of symptom relief with narcotics, hydration and fresh frozen plasma (FFP). Androgens and FFP are frequently used despite multiple, significant side-effects. Newer therapies include C1-inhibitor - both human plasma derived and recombinant - as well as contact system modulators such as ecallantide and icatibant. These newer products can be used for treatment of acute attacks of HAE, and C1-inhibitors can also be used for prophylaxis. These disease-specific therapies have proven to work by placebo-controlled studies, have minimal adverse effects and can be utilized for the treatment of HAE.

show abstract

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Update on treatment of hereditary angioedema

Buyantseva

Agarwal

et al. 2013

Clin Experimental Allergy

View full text Add to dashboard Cite

show abstract

“…In HA, subcutaneous oedema can occur in the genital area. In our literature scan, we can only find one case with genital oedema but we could not find priapism cases (Dhairyawan et al ., ).…”

Section: Discussionmentioning

confidence: 97%

A rare cause of recurrent priapism: hereditary angioedema

2014

View full text Add to dashboard Cite

show abstract

“…Regarding the reproductive system, C1INH deficiency is associated with the development of hereditary angioedema, which can include presentation of testicular swelling [ 82 , 146 ]. C4BP is synthesized in the epididymis through an androgen-dependent mechanism, but its role in male reproduction is unknown [ 88 ].…”

Section: Complement Regulationmentioning

confidence: 99%

Complementing Testicular Immune Regulation: The Relationship between Sertoli Cells, Complement, and the Immune Response

Washburn

Dufour

2023

IJMS

View full text Add to dashboard Cite

Sertoli cells within the testis are instrumental in providing an environment for spermatogenesis and protecting the developing germ cells from detrimental immune responses which could affect fertility. Though these immune responses consist of many immune processes, this review focuses on the understudied complement system. Complement consists of 50+ proteins including regulatory proteins, immune receptors, and a cascade of proteolytic cleavages resulting in target cell destruction. In the testis, Sertoli cells protect the germ cells from autoimmune destruction by creating an immunoregulatory environment. Most studies on Sertoli cells and complement have been conducted in transplantation models, which are effective in studying immune regulation during robust rejection responses. In grafts, Sertoli cells survive activated complement, have decreased deposition of complement fragments, and express many complement inhibitors. Moreover, the grafts have delayed infiltration of immune cells and contain increased infiltration of immunosuppressive regulatory T cells as compared to rejecting grafts. Additionally, anti-sperm antibodies and lymphocyte infiltration have been detected in up to 50% and 30% of infertile testes, respectively. This review seeks to provide an updated overview of the complement system, describe its relationship with immune cells, and explain how Sertoli cells may regulate complement in immunoprotection. Identifying the mechanism Sertoli cells use to protect themselves and germ cells against complement and immune destruction is relevant for male reproduction, autoimmunity, and transplantation.

show abstract

Hereditary angioedema: an unusual cause of genital swelling presenting to a genitourinary medicine clinic

Cited by 7 publications

References 10 publications

Update on treatment of hereditary angioedema

Update on treatment of hereditary angioedema

A rare cause of recurrent priapism: hereditary angioedema

Complementing Testicular Immune Regulation: The Relationship between Sertoli Cells, Complement, and the Immune Response

Contact Info

Product

Resources

About