Long-term nutritional and gastrointestinal aspects in patients with ataxia telangiectasia.

Authors:
Avishay Lahad
Avishay Lahad
Edmond and Lily Safra Children's Hospital
Yifat Sarouk
Yifat Sarouk
Pediatric Pulmonology Unit and Ataxia Telangiectasia Center
Raz Somech
Raz Somech
Tel Aviv University
Israel
Andreea Nissenkorn
Andreea Nissenkorn
Tel Aviv University
Israel
Dalit Modan-Moses
Dalit Modan-Moses
Safra Children's Hospital
Israel

Nutrition 2018 Feb 24;46:48-52. Epub 2017 Aug 24.

Pediatric Gastroenterology and Nutrition Unit, The Edmond and Lily Safra Children's Faculty of Hospital, Chaim Sheba Medical Center, Tel-Hashomer, Israel; Sackler Medicine, Tel-Aviv University, Tel-Aviv, Israel. Electronic address:

Objective: Ataxia telangiectasia (A-T) is a rare genetic disease involving multiple organs, but, to our knowledge, data on long-term gastrointestinal and nutritional involvement are scarce. The aim of this study was to longitudinally review the nutritional and gastrointestinal aspects of A-T.

Methods: This was a retrospective chart review of patients followed from 1986 to 2015 at one center. Demographic, laboratory, and nutritional data were retrieved. Body mass index (BMI) values were converted to BMI Z-score (BMI-Z). Caloric intake was estimated by food diaries and compared with estimated energy requirements for sex and age with a physical activity level factor for light physical activity.

Results: The study included 53 patients (28 males [53%], ages 14.6 ± 5.2 y). BMI-Z was inversely correlated with age (r = 0.48; P < 0.004). A decline below minimal BMI percentiles was observed after the age of 4 y in boys and 7 y in girls. The relative percentage of caloric intake decreased with age (r = -0.5; P < 0.002), and was positively correlated with BMI-Z (r = 0.35; P < 0.05). Presence of cough during meals was associated with recurrent lower respiratory tract infections (Fisher exact test, P < 0.01). Gastrostomy tubes were inserted in 12 patients, leading to improvement in BMI-Z from -5.1 ± 2.4 to -4 ± 2.9 (P < 0.05).

Conclusions: There is a progressive growth failure and low nutritional intake with age in patients with A-T, starting in early childhood in males, and more prominent in patients with cough and choking during meals. A proactive approach and insertion of a percutaneous gastrostomy tube as soon as the BMI-Z starts to decrease should be considered.

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http://dx.doi.org/10.1016/j.nut.2017.08.008DOI Listing
February 2018
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