Prevalence and Predictors of Gastrointestinal Dysmotility in Patients with Hypermobile Ehlers-Danlos Syndrome: A Tertiary Care Center Experience

Al‐Akchar, Mohammad; Hitawala, Asif; Chadalavada, Pravallika; Covut, Fahrettin; Momani, Laith Al; Khazaaleh, Shrouq; Gosai, Falgun; Ashi, Suleiman Al; Abushahin, Ashraf; Schneider, Alison

doi:10.7759/cureus.7881

Cited by 29 publications

(55 citation statements)

References 29 publications

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“…Compared to men without EDS, men with EDS also had higher proportions of GI drug prescriptions, but the magnitude of difference was smaller. These findings support previous reports of a higher prevalence of gastrointestinal symptoms in women with EDS 26‐28 …”

Section: Discussionsupporting

confidence: 92%

“…These findings support previous reports of a higher prevalence of gastrointestinal symptoms in women with EDS. [26][27][28]…”

Section: Gender Differencesmentioning

confidence: 99%

“…Second, GI manifestations may reflect adverse drug effects from treatments, including opioids, prescribed for concurrent or previously occurring EDS-associated comorbidities, notably musculoskeletal dysfunction and pain. 39,[43][44][45][46][47][48] Third, the GI symptoms may be a manifestation of a non-GI comorbidity, such as dysautonomia 24,28,[49][50][51] or mast cell dysregulation. 42 We are focusing on the striking rise of GI prescription claims among adolescent females with EDS.…”

Section: Age Differencesmentioning

confidence: 99%

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Gastrointestinal medication burden among persons with the Ehlers‐Danlos syndromes

Dhingra

Bascom

Thompson

et al. 2021

Neurogastroenterology Motil

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The Ehlers-Danlos syndromes (EDSs) are a group of 14 1,2 rare, heritable, non-inflammatory disorders of connective tissue. 2 Joint hypermobility and skin involvement are common to all types, with variable involvement of other organs including fragile vasculature and laxity of internal organs. Dermal abnormalities include hyper-extensibility, velvety texture, extensive bruising, and abnormal scarring. However, every organ system in the body may become involved, leading to a myriad of clinical symptoms. 2 The hypermobile type of Ehlers-Danlos syndrome (hEDS), formerly known as hypermobility type (EDS-HT) or type III EDS, is the most common EDS type. The variability of the clinical presentation of hEDS and the lack of a defined laboratory or genetic test mandate

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Section: Discussionsupporting

confidence: 92%

“…These findings support previous reports of a higher prevalence of gastrointestinal symptoms in women with EDS. [26][27][28]…”

Section: Gender Differencesmentioning

confidence: 99%

Section: Age Differencesmentioning

confidence: 99%

See 1 more Smart Citation

Gastrointestinal medication burden among persons with the Ehlers‐Danlos syndromes

Dhingra

Bascom

Thompson

et al. 2021

Neurogastroenterology Motil

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show abstract

“…The modified barium swallowing test demonstrated slower initiation of airway closure in patients with PD [ 57 ]. The test is utilized in the diagnostics of esophageal dysmotility in other causes of autonomic dysfunction as well but the literature on this area is still scarce [ 59 ].…”

Section: Established Methods For Assessment Of Gastroenteropathymentioning

confidence: 99%

Assessment of Gastrointestinal Autonomic Dysfunction: Present and Future Perspectives

Kornum

Terkelsen

Bertoli

et al. 2021

JCM

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The autonomic nervous system delicately regulates the function of several target organs, including the gastrointestinal tract. Thus, nerve lesions or other nerve pathologies may cause autonomic dysfunction (AD). Some of the most common causes of AD are diabetes mellitus and α-synucleinopathies such as Parkinson’s disease. Widespread dysmotility throughout the gastrointestinal tract is a common finding in AD, but no commercially available method exists for direct verification of enteric dysfunction. Thus, assessing segmental enteric physiological function is recommended to aid diagnostics and guide treatment. Several established assessment methods exist, but disadvantages such as lack of standardization, exposure to radiation, advanced data interpretation, or high cost, limit their utility. Emerging methods, including high-resolution colonic manometry, 3D-transit, advanced imaging methods, analysis of gut biopsies, and microbiota, may all assist in the evaluation of gastroenteropathy related to AD. This review provides an overview of established and emerging assessment methods of physiological function within the gut and assessment methods of autonomic neuropathy outside the gut, especially in regards to clinical performance, strengths, and limitations for each method.

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“…These symptoms respond poorly to opioids and anti-emetics such as cyclizine, 33,34 which are best avoided in this situation due to the risks of dependency. Whilst associations between hEDS, postural orthostatic tachycardia syndrome and segmental dysmotility affecting different parts of the gastrointestinal tract have been demonstrated, 34,35 there is currently no evidence for any specific treatment approaches, or evidence to suggest a greater degree of small bowel dysmotility in this group of patients. 10 In the absence of an identified secondary cause, the BSG guidelines have recommended that a definitive diagnosis can only be given when a detailed history, symptoms and investigations including full thickness biopsy histology are indicative of gastro-intestinal dysmotility.…”

Section: Advances In the Diagnosis And Classification Of Gastrointestmentioning

confidence: 99%

Recent Advances in the Management of Severe Gastrointestinal Dysmotility

Vasant

Lal

2021

CEG

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Severe gastrointestinal motility disorders with small bowel involvement continue to pose a major clinical challenge to clinicians, particularly because of the limitations of diagnostic tests and the lack of efficacious treatment options. In this article, we review current understanding and the utility of diagnostic modalities and therapeutic approaches, and describe how their limitations may potentially exacerbate prolonged suffering with debilitating symptoms, diagnostic delays, the risk of iatrogenic harm and increased healthcare utilisation in this group of patients. Moreover, observations from intestinal failure units worldwide suggest that this problem could be set to increase in the future, with reported trends of increasing numbers of patients presenting with nutritional consequences. Unfortunately, until recently, there has been a lack of consensus recommendations and guidance to support clinicians with their management approach. The aim of this narrative review is to summarise recent developments in this field following publication of an international census of experts, and subsequent clinical guidelines, which have emphasized the importance of holistic, multidisciplinary care. This is particularly important in achieving good clinical outcomes and ensuring the appropriate use of artificial nutritional support, in order to prevent iatrogenic harm. We discuss how these recent developments may impact clinical practice by supporting the development of specialised clinical services to deliver optimal care, and highlight areas where further research is needed.

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Prevalence and Predictors of Gastrointestinal Dysmotility in Patients with Hypermobile Ehlers-Danlos Syndrome: A Tertiary Care Center Experience

Cited by 29 publications

References 29 publications

Gastrointestinal medication burden among persons with the Ehlers‐Danlos syndromes

Gastrointestinal medication burden among persons with the Ehlers‐Danlos syndromes

Assessment of Gastrointestinal Autonomic Dysfunction: Present and Future Perspectives

Recent Advances in the Management of Severe Gastrointestinal Dysmotility

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