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Beatriz E. Marciano, MD, Christa S. Zerbe, MD, E

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1 X-linked carriers of chronic granulomatous disease: Illness, lyonization, and stability 
Beatriz E. Marciano, MD, Christa S. Zerbe, MD, E. Liana Falcone, MD, PhD, Li Ding, MD, Suk See DeRavin, MD, PhD, Janine Daub, CRNP, Samantha Kreuzburg, RN, Lynne Yockey, RN, Sally Hunsberger, PhD, Ladan Foruraghi, CRNP, Lisa A. Barnhart, RN, Kabir Matharu, MD, Victoria Anderson, CRNP, Dirk N. Darnell, RN, Cathleen Frein, CRNP, Danielle L. Fink, MS, Karen P. Lau, MS, Debra A. Long Priel, MS, John I. Gallin, MD, Harry L. Malech, MD, Gulbu Uzel, MD, Alexandra F. Freeman, MD, Douglas B. Kuhns, PhD, Sergio D. Rosenzweig, MD, PhD, Steven M. Holland, MD  Journal of Allergy and Clinical Immunology  Volume 141, Issue 1, Pages (January 2018) DOI: /j.jaci Copyright © Terms and Conditions

2 Fig 1 %DHR+ values. A, Distribution according to %DHR+ values. Histogram of number of carriers according to %DHR+ values. B, %DHR+ values by age in female carriers of CGD. Distribution of %DHR+ values according to age by decade. Each point shows a single female subject at the last follow-up according to age. Gray boundaries show those highly skewed at a ratio 90:10; dotted line shows the slightly less skewed ratio of 80:20. Journal of Allergy and Clinical Immunology  , DOI: ( /j.jaci ) Copyright © Terms and Conditions

3 Fig 2 Clinical features by %DHR+ values. %DHR+ values according to clinical manifestations are shown, and values are presented as means with 95% CIs. Journal of Allergy and Clinical Immunology  , DOI: ( /j.jaci ) Copyright © Terms and Conditions

4 Fig 3 Risk of infection and AIMs. Probabilities are based on %DHR+ values. A logistic regression model was used to estimate the probability of infection or AIM as a function of %DHR+ value. P values tested whether the %DHR+ value is a significant predictor for infection or AIM. Journal of Allergy and Clinical Immunology  , DOI: ( /j.jaci ) Copyright © Terms and Conditions

5 Fig 4 Familial %DHR+ value correlations. A and B, %DHR+ values over time in 2 pair of twins: Fig 4, A, young twins; Fig 4, B, older twins. C, %DHR+ values between mothers and daughters independent of age. R values were calculated by using the Spearman rank test. D, Correlation of %DHR+ values between sisters who are both CGD carriers. Values are independent of age. R values were calculated by using the Spearman rank test. Journal of Allergy and Clinical Immunology  , DOI: ( /j.jaci ) Copyright © Terms and Conditions


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