Taylor Eddens1, Molly Mack2, Meghan McCormick3, Hey Chong4, Ramasubramanian Kalpatthi3. 1. Pediatric Scientist Development Program, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pa; Department of Allergy and Immunology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pa. Electronic address: eddenstj@upmc.edu. 2. Pediatric Residency Program, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pa. 3. Department of Hematology and Oncology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pa. 4. Department of Allergy and Immunology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pa.
Abstract
BACKGROUND: Primary immunodeficiency disorders (PIDDs) describe a myriad of diseases caused by inherited defects within the immune system. As the number of identified genetic defects associated with PIDDs increases, understanding the incidence and outcomes of PIDD patients becomes imperative. OBJECTIVE: To characterize the frequency of new diagnoses, patterns of health care utilization, rates of hematopoietic stem cell transplantation (HSCT), and mortality in pediatric patients with PIDDs. METHODS: A retrospective cohort analysis of the Pediatric Health Information System database from 2004 to 2018 for pediatric inpatients with an International Classification of Diseases, Ninth and 10th Revisions (ICD-9/ICD-10). code associated with PIDD. RESULTS: A total of 17,234 patients with a PIDD were hospitalized from 2004 to 2018. There were 2.8 new PIDD diagnoses and 6.3 PIDD hospitalizations per 1,000 discharges; these metrics were unchanged during the study period. The number of new diagnoses for B-cell and antibody defects significantly increased over time. The number of new PIDD diagnoses significantly increased in adolescents or adults and decreased in infants. T-cell disorders had the highest number of intensive care unit admissions. There were 747 PIDD patients who underwent HSCT; complications of HSCT significantly decreased over time. Mortality rates significantly decreased in all PIDD patients and in patients receiving HSCT. CONCLUSIONS: The total hospitalizations and incidence of PIDDs within the hospitalized pediatric population were unchanged. There were significant changes in the class of PIDD diagnosed, the age at diagnosis, and health care utilization metrics. Mortality significantly decreased over time within the PIDD cohort.
BACKGROUND: Primary immunodeficiency disorders (PIDDs) describe a myriad of diseases caused by inherited defects within the immune system. As the number of identified genetic defects associated with PIDDs increases, understanding the incidence and outcomes of PIDD patients becomes imperative. OBJECTIVE: To characterize the frequency of new diagnoses, patterns of health care utilization, rates of hematopoietic stem cell transplantation (HSCT), and mortality in pediatric patients with PIDDs. METHODS: A retrospective cohort analysis of the Pediatric Health Information System database from 2004 to 2018 for pediatric inpatients with an International Classification of Diseases, Ninth and 10th Revisions (ICD-9/ICD-10). code associated with PIDD. RESULTS: A total of 17,234 patients with a PIDD were hospitalized from 2004 to 2018. There were 2.8 new PIDD diagnoses and 6.3 PIDD hospitalizations per 1,000 discharges; these metrics were unchanged during the study period. The number of new diagnoses for B-cell and antibody defects significantly increased over time. The number of new PIDD diagnoses significantly increased in adolescents or adults and decreased in infants. T-cell disorders had the highest number of intensive care unit admissions. There were 747 PIDD patients who underwent HSCT; complications of HSCT significantly decreased over time. Mortality rates significantly decreased in all PIDD patients and in patients receiving HSCT. CONCLUSIONS: The total hospitalizations and incidence of PIDDs within the hospitalized pediatric population were unchanged. There were significant changes in the class of PIDD diagnosed, the age at diagnosis, and health care utilization metrics. Mortality significantly decreased over time within the PIDD cohort.
Authors: Suhag H Parikh; Prakash Satwani; Kwang Woo Ahn; Natasha A Sahr; Caitrin Fretham; Allistair A Abraham; Vaibhav Agrawal; Jeffery J Auletta; Hisham Abdel-Azim; Edward Copelan; Miguel-Angel Diaz; Christopher C Dvorak; Haydar A Frangoul; Cesar O Freytes; Shahinaz M Gadalla; Robert Peter Gale; Biju George; Usama Gergis; Shahrukh Hashmi; Peiman Hematti; Gerhard C Hildebrandt; Amy K Keating; Hillard M Lazarus; Kasiani C Myers; Richard F Olsson; Timothy Prestidge; Seth J Rotz; Bipin N Savani; Evan B Shereck; Kirsten M Williams; Baldeep Wirk; Marcelo C Pasquini; Alison W Loren Journal: JAMA Pediatr Date: 2019-05-06 Impact factor: 16.193
Authors: Lily E Leiva; Marta Zelazco; Matías Oleastro; Magda Carneiro-Sampaio; Antonio Condino-Neto; Beatriz Tavares Costa-Carvalho; Anete Sevciovic Grumach; Arnoldo Quezada; Pablo Patiño; José Luis Franco; Oscar Porras; Francisco Javier Rodríguez; Francisco Javier Espinosa-Rosales; Sara Elva Espinosa-Padilla; Diva Almillategui; Celia Martínez; Juan Rodríguez Tafur; Marilyn Valentín; Lorena Benarroch; Rosy Barroso; Ricardo U Sorensen Journal: J Clin Immunol Date: 2006-12-27 Impact factor: 8.317