Abstract
Metabolic bone disease (MBD) is a possible complication of intestinal failure (IF), with a multi-factorial pathogenesis. The reduction of bone density (BMD) may be radiologically evident before manifestation of clinical signs (bone pain, vertebral compression, and fractures). Diagnosis relies on dual-energy X-ray absorptiometry (DXA). Incidence and evolution of MBD are not homogeneously reported in children. The aim of this systematic review was to define the prevalence of MBD in IF children and to describe risk factors for its development. A comprehensive search of electronic bibliographic databases up to December 2021 was conducted. Randomized controlled trials; observational, cross-sectional, and retrospective studies; and case series published between 1970 and 2021 were included. Twenty observational studies (six case-control) were identified and mostly reported definitions of MBD based on DXA parameters. Although the prevalence and definition of MBD was largely heterogeneous, low BMD was found in up to 45% of IF children and correlated with age, growth failure, and specific IF etiologies. Data demonstrate that long-term follow-up with repeated DXA and calcium balance assessment is warranted in IF children even when PN dependence is resolved. Etiology and outcomes of MBD will be better defined by longitudinal prospective studies focused on prognosis and therapeutic perspectives.
Highlights
Intestinal failure (IF) is the consequence of several gastrointestinal conditions that determine the necessity of parenteral nutrition (PN) to maintain adequate growth and fluids/energy balance [1]
Patients with IF are at high risk of growth failure during PN [35,39,40] and after PN cessation [19,29]; an adjustment of bone density parameters is strongly recommended
Vitamin D requirement does not seem to be increased in patients with IF, and an adjustment based on serum levels is recommended
Summary
Intestinal failure (IF) is the consequence of several gastrointestinal conditions that determine the necessity of parenteral nutrition (PN) to maintain adequate growth and fluids/energy balance [1]. SBS can develop in older children because of a surgical resection for several conditions, including inflammatory bowel disease (IBD), volvulus, and ischemic events [2]. The reversibility of IF and the achievement of enteral autonomy depend on the underlying condition and the surgical outcomes but are related to both nutritional and medical treatments. Advancement in home PN (HPN) and enteral nutrition (EN), both in surgical techniques and in medical strategies and the development of specialized, multidisciplinary rehabilitation centers for the medical care of these vulnerable subjects, have notably improved the outcomes of IF children. Despite the progress in the field, children on HPN remain at risk of developing multiple serious complications, such as septic events, liver disease, renal dysfunction, and growth failure [3]
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