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

Alexander Krauthammer, Avishay Lahad, Yifat Sarouk, Raz Somech, Andreea Nissenkorn, Dalit Modan-Moses, Hila Levi-Kidron, Tal Sadeh-Kon, Batia Weiss*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)48-52
Number of pages5
JournalNutrition
Volume46
DOIs
StatePublished - Feb 2018

Keywords

  • BMI-Z
  • Caloric intake
  • Percutaneous gastrostomy

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