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Erschienen in: Clinical Autonomic Research 2/2023

03.02.2023 | Research Article

Gastrointestinal bleeding in children with familial dysautonomia: a case–control study

verfasst von: Chethan Ramprasad, Jose-Alberto Palma, Lucy Norcliffe-Kaufmann, Joseph Levy, Lea Ann Chen, Horacio Kaufmann

Erschienen in: Clinical Autonomic Research | Ausgabe 2/2023

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Abstract

Objective

Familial dysautonomia (FD) is a rare inherited autosomal recessive disorder with abnormal somatosensory, enteric, and afferent autonomic neurons. We aimed to define the incidence of gastrointestinal bleeding and its associated risk factors in patients with FD.

Methods

In this retrospective case–control study, we identified all episodes of gastrointestinal bleeding in patients with FD, occurring over four decades (January 1980–December 2017), using the New York University FD registry.

Results

We identified 104 episodes of gastrointestinal bleeding occurring in 60 patients with FD. The estimated incidence rate of gastrointestinal bleeds in the FD population rate was 4.20 episodes per 1000 person-years. We compared the 60 cases with 94 age-matched controls. Bleeding in the upper gastrointestinal tract from gastric and duodenal ulcers occurred most frequently (64 bleeds, 75.6%). Patients were more likely to have a gastrostomy (G)-tube and a Nissen fundoplication [odds ratio (OR) 3.73, 95% confidence interval (CI) 1.303–13.565] than controls. The mean time from G-tube placement to first gastrointestinal bleed was 7.01 years. The mean time from Nissen fundoplication to bleed was 7.01 years. Cases and controls had similar frequency of intake of nonsteroidal antiinflammatory drugs (NSAID) and selective serotonin reuptake inhibitors (SSRI).

Conclusion

The incidence of gastrointestinal bleeding in the pediatric FD population was estimated to be 4.20 per 1000 person-years, 21 times higher than in the general pediatric population (0.2 per 1000 person-years). Patients with FD with a G-tube and a Nissen fundoplication had a higher risk of a subsequent gastrointestinal bleeding.
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Metadaten
Titel
Gastrointestinal bleeding in children with familial dysautonomia: a case–control study
verfasst von
Chethan Ramprasad
Jose-Alberto Palma
Lucy Norcliffe-Kaufmann
Joseph Levy
Lea Ann Chen
Horacio Kaufmann
Publikationsdatum
03.02.2023
Verlag
Springer Berlin Heidelberg
Erschienen in
Clinical Autonomic Research / Ausgabe 2/2023
Print ISSN: 0959-9851
Elektronische ISSN: 1619-1560
DOI
https://doi.org/10.1007/s10286-023-00925-1

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