BACKGROUND: Primary antibody deficiencies (PADs) are heterogeneous group of disorders, characterized by hypogammaglobulinemia and increased susceptibility to recurrent infections. To evaluate the diagnostic delay in Iranian PADs in association with their infections, we scored such manifestations to find an association between such delay and the scoring system. METHODS: Forty-eight patients with PADs, who were referred to our center during a 25-year period, were enrolled in this study. Each episode of infection, before making the PADs diagnosis, got a score of 5 or 10 based on the severity of infections. RESULTS: The diagnosis was made with median delay of 34.5 months, when the patients had mean score of 94.48 ± 52.89. There was a significant direct association between this scoring system and delay diagnosis. The score of 50 was considered as the cutoff point in our patient group. In this score, the suspicions to PADs in more than 90% of patients true positively lead to diagnosis of PADs. CONCLUSION: Although diagnosis delay significantly decreased over time, PADs still continue to be diagnosed late. Based on the results of this study, the assessment of immune system should be performed in the patients with 50 total score or about 25 score per year.
BACKGROUND:Primary antibody deficiencies (PADs) are heterogeneous group of disorders, characterized by hypogammaglobulinemia and increased susceptibility to recurrent infections. To evaluate the diagnostic delay in Iranian PADs in association with their infections, we scored such manifestations to find an association between such delay and the scoring system. METHODS: Forty-eight patients with PADs, who were referred to our center during a 25-year period, were enrolled in this study. Each episode of infection, before making the PADs diagnosis, got a score of 5 or 10 based on the severity of infections. RESULTS: The diagnosis was made with median delay of 34.5 months, when the patients had mean score of 94.48 ± 52.89. There was a significant direct association between this scoring system and delay diagnosis. The score of 50 was considered as the cutoff point in our patient group. In this score, the suspicions to PADs in more than 90% of patients true positively lead to diagnosis of PADs. CONCLUSION: Although diagnosis delay significantly decreased over time, PADs still continue to be diagnosed late. Based on the results of this study, the assessment of immune system should be performed in the patients with 50 total score or about 25 score per year.
Authors: Zeinab A El-Sayed; Dalia H El-Ghoneimy; José A Ortega-Martell; Nesrine Radwan; Juan C Aldave; Waleed Al-Herz; Maryam A Al-Nesf; Antonio Condino-Neto; Theresa Cole; Brian Eley; Nahla H H Erwa; Sara Espinosa-Padilla; Emilia Faria; Nelson A Rosario Filho; Ramsay Fuleihan; Nermeen Galal; Elizabeth Garabedian; Mary Hintermeyer; Kohsuke Imai; Carla Irani; Ebtihal Kamal; Nadia Kechout; Adam Klocperk; Michael Levin; Tomas Milota; Monia Ouederni; Roberto Paganelli; Claudio Pignata; Farah N Qamar; Isabella Quinti; Sonia Qureshi; Nita Radhakrishnan; Nima Rezaei; John Routes; Surjit Singh; Sangeetha Siniah; Intisar Abdel-Hakam Taha; Luciana K Tanno; Ben Van Dort; Alla Volokha; Kathleen Sullivan Journal: World Allergy Organ J Date: 2022-06-17 Impact factor: 5.516
Authors: Marcin Ziętkiewicz; Ewa Więsik-Szewczyk; Aleksandra Matyja-Bednarczyk; Katarzyna Napiórkowska-Baran; Zbigniew Zdrojewski; Karina Jahnz-Różyk Journal: Front Immunol Date: 2020-05-27 Impact factor: 7.561