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Small pituitary size in children with Fanconi Anemia
Author(s) -
SherafatKazemzadeh Rosa,
Mehta Sanjeev N.,
Care Marguerite M.,
Kim MiOk,
Williams David A.,
Rose Susan R.
Publication year - 2007
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.21148
Subject(s) - medicine , fanconi anemia , bone age , magnetic resonance imaging , thyroid , pituitary gland , endocrine system , growth hormone deficiency , endocrinology , pediatrics , hormone , gastroenterology , growth hormone , radiology , biochemistry , chemistry , dna repair , gene
Abstract Background Fanconi anemia (FA) is a genetic disorder associated with multiple congenital anomalies, bone marrow failure, and pituitary hypofunction including hypogonadism, thyroid dysfunction, and growth hormone (GH) deficiency. Procedure Among 44 patients with FA referred to Cincinnati Children's Hospital Medical Center (CCHMC) between 1975 and 2005, 33 had neuroimaging studies, including 11 cranial magnetic resonance imaging (MRIs). Two separate measurements per patient from these MRIs were used to evaluate pituitary height compared to on‐site control data of similar measurements of cranial MRIs on 22 age and gender‐matched children without any pathology involving the hypothalamic‐pituitary system. Growth pattern and endocrine studies were reviewed to assess potential correlation with pituitary size. Results When compared to the age‐gender matched on‐site control sample, the mean pituitary height of FA patients was significantly smaller ( P < 0.0001; mean ± SE from mixed effects model with age and gender as covariates: 3.96 ± 0.32 vs. 5.76 ± 0.24). Upon further adjusting for the effect of the small head size by including bi‐parietal diameter (BPD) as a covariate, the difference remained statistically significant ( P = 0.0013). Findings on the growth pattern and endocrinological measurements are as follows: 50% of patients with small pituitary gland were short. GH and adrenal function tests were normal in all tested patients. Thyroid, pubertal status, and glucose regulation were abnormal in 30, 50, and 75% of patients tested. Conclusions Children with FA tend to have unsuspected small pituitary glands beyond what is expected from the effects of their stunted growth. Further studies are required to reveal the clinical implications of this finding. Pediatr Blood Cancer 2007;49:166–170. © 2007 Wiley‐Liss, Inc.