BACKGROUND: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders (PID), with a broad spectrum of clinical features ranging from severe and recurrent infections to asymptomatic disease. OBJECTIVES: The current study was performed to evaluate and compare demographic and clinical data in the most common types of PAD. MATERIALS AND METHODS: We performed a retrospective review of the medical records of all PAD patients with a confirmed diagnosis of common variable immunodeficiency (CVID), hyper IgM syndrome (HIgM), selective IgA deficiency (SIgAD), and X-linked agammaglobulinemia (XLA) who were diagnosed during the last 30 years at the Children's Medical Center, Tehran, Iran. RESULTS: A total number of 280 cases of PAD (125 CVID, 32 HIgM, 63 SIgAD, and 60 XLA) were enrolled in the study. The median (range) age at the onset of disease in CVID, HIgM, SIgAD, and XLA was 2 (0-46), 0.91 (0-9), 1 (0-26), and 1 (0-10) years, respectively. Gastrointestinal infections were more prevalent in CVID patients, as were central nervous system infections in XLA patients. Autoimmune complications were more prevalent in HIgM patients, malignancies in CVID patients, and allergies in SIgAD patients. The mortality rate for CVID, HIgM, and XLA was 27.2%, 28.1%, and 25%, respectively. No deaths were reported in SIgAD patients. CONCLUSIONS: SIgAD patients had the best prognosis. While all PAD patients should be monitored for infectious complications, special attention should be paid to the finding of malignancy and autoimmune disorders in CVID and HIgM patients, respectively.
BACKGROUND:Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders (PID), with a broad spectrum of clinical features ranging from severe and recurrent infections to asymptomatic disease. OBJECTIVES: The current study was performed to evaluate and compare demographic and clinical data in the most common types of PAD. MATERIALS AND METHODS: We performed a retrospective review of the medical records of all PAD patients with a confirmed diagnosis of common variable immunodeficiency (CVID), hyper IgM syndrome (HIgM), selective IgA deficiency (SIgAD), and X-linked agammaglobulinemia (XLA) who were diagnosed during the last 30 years at the Children's Medical Center, Tehran, Iran. RESULTS: A total number of 280 cases of PAD (125 CVID, 32 HIgM, 63 SIgAD, and 60 XLA) were enrolled in the study. The median (range) age at the onset of disease in CVID, HIgM, SIgAD, and XLA was 2 (0-46), 0.91 (0-9), 1 (0-26), and 1 (0-10) years, respectively. Gastrointestinal infections were more prevalent in CVIDpatients, as were central nervous system infections in XLA patients. Autoimmune complications were more prevalent in HIgM patients, malignancies in CVIDpatients, and allergies in SIgAD patients. The mortality rate for CVID, HIgM, and XLA was 27.2%, 28.1%, and 25%, respectively. No deaths were reported in SIgAD patients. CONCLUSIONS: SIgAD patients had the best prognosis. While all PAD patients should be monitored for infectious complications, special attention should be paid to the finding of malignancy and autoimmune disorders in CVID and HIgM patients, respectively.
Authors: Cristiane J Nunes-Santos; Christopher Koh; Anjali Rai; Keith Sacco; Beatriz E Marciano; David E Kleiner; Jamie Marko; Jenna R E Bergerson; Michael Stack; Maria M Rivera; Gregory Constantine; Warren Strober; Gulbu Uzel; Ivan J Fuss; Luigi D Notarangelo; Steven M Holland; Sergio D Rosenzweig; Theo Heller Journal: J Allergy Clin Immunol Date: 2021-06-01 Impact factor: 10.793
Authors: R Shavit; R Maoz-Segal; S Frizinsky; S Haj-Yahia; I Offengenden; D Machnas-Mayan; Y Tunisky; M Iancovici-Kidon; N Agmon-Levin Journal: Clin Exp Immunol Date: 2021-02-25 Impact factor: 4.330
Authors: Zeinab A El-Sayed; Irina Abramova; Juan Carlos Aldave; Waleed Al-Herz; Liliana Bezrodnik; Rachida Boukari; Ahmed Aziz Bousfiha; Caterina Cancrini; Antonio Condino-Neto; Ghassan Dbaibo; Beata Derfalvi; Figen Dogu; J David M Edgar; Brian Eley; Rasha Hasan El-Owaidy; Sara Elva Espinosa-Padilla; Nermeen Galal; Filomeen Haerynck; Rima Hanna-Wakim; Elham Hossny; Aydan Ikinciogullari; Ebtihal Kamal; Hirokazu Kanegane; Nadia Kechout; Yu Lung Lau; Tomohiro Morio; Viviana Moschese; Joao Farela Neves; Monia Ouederni; Roberto Paganelli; Kenneth Paris; Claudio Pignata; Alessandro Plebani; Farah Naz Qamar; Sonia Qureshi; Nita Radhakrishnan; Nima Rezaei; Nelson Rosario; John Routes; Berta Sanchez; Anna Sediva; Mikko Rj Seppanen; Edith Gonzalez Serrano; Anna Shcherbina; Surjit Singh; Sangeetha Siniah; Guiseppe Spadaro; Mimi Tang; Ana Maria Vinet; Alla Volokha; Kathleen E Sullivan Journal: World Allergy Organ J Date: 2019-03-22 Impact factor: 4.084
Authors: Sampsa Pikkarainen; Timi Martelius; Ari Ristimäki; Sanna Siitonen; Mikko R J Seppänen; Martti Färkkilä Journal: Am J Gastroenterol Date: 2019-04 Impact factor: 10.864