Heterogeneity of Lower Esophageal Sphincter Function in Children With Achalasia

Morera, Claudio; Nurko, Samuel

doi:10.1097/mpg.0b013e3182293d8c

Cited by 29 publications

(11 citation statements)

References 31 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Typical symptoms include progressive solid and liquid dysphagia, regurgitation, chest pain, and weight loss. However, the symptoms and features are heterogeneous and not all “typical” features need to be present (Agrawal et al, 2008; Kushnir et al, 2012; Morera and Nurko, 2012). Esophageal manometry is the gold standard to establish the diagnosis for achalasia.…”

Section: Introductionmentioning

confidence: 99%

On the origin of rhythmic contractile activity of the esophagus in early achalasia, a clinical case study

et al. 2013

View full text Add to dashboard Cite

A patient with early achalasia presented spontaneous strong rhythmic non-propulsive contractions at ~7/min, independent of swallows. Our aim was to evaluate characteristics of the rhythmic contractions, provide data on the structure of pacemaker cells in the esophagus and discuss a potential role for interstitial cells of Cajal (ICC) in the origin of rhythmicity. We hypothesize that intramuscular ICC (ICC-IM) are the primary pacemaker cells. The frequency but not the amplitude of the rhythmic contractions was inhibited by the phosphodiesterase inhibitor drotaverine consistent with cAMP inhibiting pacemaker currents in ICC-IM. The frequency increased by wet swallows but not dry swallows, consistent with stretch causing increase in slow wave frequency in ICC-IM. New studies on archival material showed that ICC-IM were present throughout the human esophageal musculature and were not diminished in early achalasia. Although ICC-IM exhibited a low density, they were connected to PDGFRα-positive fibroblast-like cells with whom they formed a dense gap junction coupled network. Nitrergic innervation of ICC was strongly diminished in early achalasia because of the loss of nitrergic nerves. It therefore appears possibly that ICC-IM function as pacemaker cells in the esophagus and that the network of ICC and PDGFRα-positive cells allows for coupling and propagation of the pacemaker activity. Loss of nitrergic innervation to ICC in achalasia may render them more excitable such that its pacemaker activity is more easily expressed. Loss of propagation in achalasia may be due to loss of contraction-induced aboral nitrergic inhibition.

show abstract

Section: Introductionmentioning

confidence: 99%

On the origin of rhythmic contractile activity of the esophagus in early achalasia, a clinical case study

et al. 2013

View full text Add to dashboard Cite

show abstract

“…The manometric findings of aperistalsis and incomplete LES relaxation strongly sustains a diagnosis of achalasia .The presence of only one of these findings is rare in achalasia, but have also been described, mostly in pre-HRM era (24)(25)(26)(27). Aperistalsis can present with different pressure patterns, such as a quiescent esophageal body (type I), isobaric pan-esophageal pressurization (type II), and spastic contractions (type III) (26).…”

Section: Diagnosismentioning

confidence: 96%

“…Aperistalsis can present with different pressure patterns, such as a quiescent esophageal body (type I), isobaric pan-esophageal pressurization (type II), and spastic contractions (type III) (26).…”

Section: Diagnosismentioning

confidence: 99%

High Resolution Manometry - A Mandatory Examination in the Pre and Postoperative Assessment of Patients with Achalasia

Dimitriu¹,

Gheorghe²

2018

chr

View full text Add to dashboard Cite

RezumatManometria esofagiană cu rezoluţie înaltă este actual investigaţia gold-standard în diagnosticul acalaziei şi a bolilor esofagiene funcţionale. Această examinare este întotdeauna precedată de endoscopia digestivă superioară, care are scopul de a face diagnosticul diferenţial al acalaziei cu pseudoacalazia (formaţiuni tumorale de fornix gastric/esofag distal). La momentul actual, nu există tratament curativ pentru acalazie. Însă, cele 3 subtipuri de acalazie (conform clasificării Chicago) au răspuns diferit în ceea ce priveşte eficacitatea în tratamentului ales. Astfel, comparativ cu tipurile I şi III, tipul II de acalazie are cel mai bun răspuns la intervenţiile terapeutice invazive (dilatare pneumatică sau cardiomiotomie Heller). În schimb, în tipul III cardiomiotomia Heller este de ales ca prima abordare terapeutică. Miotomia endoscopică perorală (POEM) este o tehnică relativ recentă, care necesită însă studii pe termen lung în ceea ce priveşte siguranţa şi eficacitatea. Articolul prezent îşi propune să prezinte variantele actuale de tratament ale acalaziei, comparându-le pe baza eficacităţii şi siguranţei pe termen scurt şi lung, subliniind de asemenea rolul manometriei cu rezoluţie înaltă în diagnosticul, alegerea metodei terapeutice şi prognosticul acestor pacienţi. Review ArticleAbstract High resolution manometry (HRM) is currently the gold standard for the diagnosis of achalasia and other functional esophageal disorders. All patients accusing dysphagia should be endoscopically evaluated prior to manometric investigations in order to rule out pseudoachalasia. The Chicago HRM classification has led to a subclassification of three manometric types of achalasia that seem to have different results to treatment. None of the actual achalasia treatment options are curative. Type II achalasia patients respond best to all treatment options compared to those with types I and III. Pneumatic dilation (PD) or Heller miotomy (LHM) can be both chosen as initial therapy in type I and II as they have good outcome , while type III achalasia patients respond better to LHM as a first therapeutic option. Peroral endoscopic myotomy (POEM) is a promising new technique but long-term follow-up studies for its safety and efficacy must be performed. This article reviews the current therapeutic options in achalasia and other functional esophageal disorders, based on the differences in safety and efficacy between approaches, highlighting the impact of HRM to predict the outcome but also the role of the techinque in guiding antireflux surgery.

show abstract

“…17,18 La aperistalsis esofágica es el hallazgo manométrico característico en los pacientes con acalasia, también puede observarse hipertensión y falta de relajación del esfínter esofágico inferior. Sin embargo, la función del esfínter esofágico inferior puede ser heterógenea encontrando relajaciones aparentemente normales después de la deglución en algunos pacientes, tal como fue informado por Morera y Nurko; 19 este hallazgo se pudo observar en dos de nuestros pacientes (Tabla 3).…”

Section: Tabla 4 Tratamiento Y Evolución De Los Pacientesunclassified

Sindrome de allgrove en niños. Reporte de 11 casos

López¹,

Ramírez²,

Rodríguez³

et al. 2020

Acta gastroenterol. latinoam.

View full text Add to dashboard Cite

Background. Allgrove Syndrome is a very rare genetic disease, which is inherited in an autosomal recessive way. The responsible gene is the AAAS, that encodes the protein ALADIN. It occurs most often in children of consanguineous parents. It is characterized by the classic triad of achalasia, alacrima, and adrenal insufficiency due to resistance to ACTH; the presence of two of the three previous manifestation events are required to establish the diagnosis. There is also a high frequency of the neurologic symptoms. Objective. Describe the clinical characteristics, age of presentation and evolution in 11 patients with Allgrove Syndrome. Methods. 11 clinical cases compatible with Allgrove Syndrome of presentation in childhood are retrospectively reviewed. Results. The average age at diagnosis was 5.9 years (range 1-16 years old). There was a predominance of the female sex (n = 7). The most common symptoms were postprandial vomiting and alacrima, present in 100% of the cases at the time of diagnosis. Adrenal insufficiency was not common; it was only documented in one patient. There was consanguinity between parents in 62.5% of the cases. Conclusions. Allgrove Syndrome is an uncommon cause of dysphagia, chronic vomiting and failure to grow in children. In case of any documented case of achalasia it is suggested to question in a directed way the presence of alacrima and adrenal insufficiency data such as seizures, hyperpigmentation of the skin and neurological alterations.

show abstract

Heterogeneity of Lower Esophageal Sphincter Function in Children With Achalasia

Abstract: The LES function in pediatric patients with achalasia is heterogeneous. The classic description of a nonrelaxing high-pressure LES in patients with achalasia is rarely found in children. Partial relaxations are common, and normal relaxations may be present.

Cited by 29 publications

References 31 publications

On the origin of rhythmic contractile activity of the esophagus in early achalasia, a clinical case study

On the origin of rhythmic contractile activity of the esophagus in early achalasia, a clinical case study

High Resolution Manometry - A Mandatory Examination in the Pre and Postoperative Assessment of Patients with Achalasia

Sindrome de allgrove en niños. Reporte de 11 casos

Contact Info

Product

Resources

About