OBJECTIVES/HYPOTHESIS: To determine the risk of chronic rhinosinusitis (CRS) in relatives of children with a diagnosis of CRS. STUDY DESIGN: Retrospective observational cohort study with population-based matched controls. METHODS: A unique genealogical database linked to medical records was used to identify subjects ≤12 years old with a diagnosis of CRS from 1996 to 2011. The familial recurrence risks of CRS in first- through fifth-degree relatives of probands were calculated using Cox models and compared to controls randomly selected from the Utah population and matched 10:1 on sex and birth year. RESULTS: We identified 496 pediatric patients with CRS. Siblings of patients with CRS demonstrated a 57.5-fold increased risk (P < 10(-8) ) of also having pediatric CRS. First cousins had a 9.0-fold increased risk (P < 10(-3) ) and second cousins had a 2.9-fold increased risk (P = .002) of pediatric CRS. First-degree relatives, second-degree relatives, and first cousins of pediatric cases demonstrated a significant increased risk of having adult CRS. Parents of probands demonstrated a 5.6-fold increased risk (P < 10(-15) ). Fifty-five probands had one affected parent versus three probands with two affected parents. CONCLUSIONS: In the largest population study to date of children with CRS, a significant familial risk is confirmed. Parents of probands were also at increased risk, although it was much more likely for one parent to be affected than both, suggesting a genetic component of the disease. Further understanding of the genetic basis of CRS and its interplay with environmental factors could clarify the etiology and lead to more effective targeted treatments. LEVEL OF EVIDENCE: 3b Laryngoscope, 126:739-745, 2016.
OBJECTIVES/HYPOTHESIS: To determine the risk of chronic rhinosinusitis (CRS) in relatives of children with a diagnosis of CRS. STUDY DESIGN: Retrospective observational cohort study with population-based matched controls. METHODS: A unique genealogical database linked to medical records was used to identify subjects ≤12 years old with a diagnosis of CRS from 1996 to 2011. The familial recurrence risks of CRS in first- through fifth-degree relatives of probands were calculated using Cox models and compared to controls randomly selected from the Utah population and matched 10:1 on sex and birth year. RESULTS: We identified 496 pediatric patients with CRS. Siblings of patients with CRS demonstrated a 57.5-fold increased risk (P < 10(-8) ) of also having pediatric CRS. First cousins had a 9.0-fold increased risk (P < 10(-3) ) and second cousins had a 2.9-fold increased risk (P = .002) of pediatric CRS. First-degree relatives, second-degree relatives, and first cousins of pediatric cases demonstrated a significant increased risk of having adult CRS. Parents of probands demonstrated a 5.6-fold increased risk (P < 10(-15) ). Fifty-five probands had one affected parent versus three probands with two affected parents. CONCLUSIONS: In the largest population study to date of children with CRS, a significant familial risk is confirmed. Parents of probands were also at increased risk, although it was much more likely for one parent to be affected than both, suggesting a genetic component of the disease. Further understanding of the genetic basis of CRS and its interplay with environmental factors could clarify the etiology and lead to more effective targeted treatments. LEVEL OF EVIDENCE: 3b Laryngoscope, 126:739-745, 2016.
Authors: Lechosław Paweł Chmielik; Grażyna Mielnik-Niedzielska; Anna Kasprzyk; Tomasz Stankiewicz; Artur Niedzielski Journal: Children (Basel) Date: 2021-12-04