Limits...
A 12 year old boy with recurrent episodes of pneumonia: triple A syndrome.

Parhizkar B, Maghsoodi N, Forootan M, Entezari AH - Gastroenterol Hepatol Bed Bench (2012)

Bottom Line: Triple A syndrome (Allgrove syndrome) is a rare inherited autosomal recessive disease with a typical triad including adrenocorticotrophic-hormone-resistant glucocorticoid insufficiency, reduced or absent tearing (alacrima) and achalasia and a wide range of symptoms can be detected due to multi organ involvement.This report describes the case of a Triple Asyndrome, a12 year-old boy with a history of recurrent episodes of pneumonia and growth retardation due to failure to timely diagnosis of his problem.

View Article: PubMed Central - PubMed

Affiliation: Research Institute for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

ABSTRACT
Triple A syndrome (Allgrove syndrome) is a rare inherited autosomal recessive disease with a typical triad including adrenocorticotrophic-hormone-resistant glucocorticoid insufficiency, reduced or absent tearing (alacrima) and achalasia and a wide range of symptoms can be detected due to multi organ involvement. This report describes the case of a Triple Asyndrome, a12 year-old boy with a history of recurrent episodes of pneumonia and growth retardation due to failure to timely diagnosis of his problem.

No MeSH data available.


Related in: MedlinePlus

Manometrical study (HRM) four years after previous ineffective myotomy.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4017457&req=5

Figure 0004: Manometrical study (HRM) four years after previous ineffective myotomy.

Mentions: We performed an esophageal Esophagogastroduodenoscopy (EGD), a barium swallow study and a high resolution esophageal manometry for evaluating the esophagus. EGD showed a dilated esophagus with lots of retained food and scope was passed with force via lower esophageal sphincter (LES). Barium swallow showed a dilated esophagus with distal narrowing and barium stasis (Figure 4). High resolution manometry showed normal LES resting pressure (23 mmHg) with incomplete relaxation (23%), prolonged IRP (integrated relaxation pressure) (18 mmHg) and raised intrabolus pressure in free drinking maneuver (Figure 4). These studies displayed ineffective response to previous treatment for ashalasia in our patient.


A 12 year old boy with recurrent episodes of pneumonia: triple A syndrome.

Parhizkar B, Maghsoodi N, Forootan M, Entezari AH - Gastroenterol Hepatol Bed Bench (2012)

Manometrical study (HRM) four years after previous ineffective myotomy.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4017457&req=5

Figure 0004: Manometrical study (HRM) four years after previous ineffective myotomy.
Mentions: We performed an esophageal Esophagogastroduodenoscopy (EGD), a barium swallow study and a high resolution esophageal manometry for evaluating the esophagus. EGD showed a dilated esophagus with lots of retained food and scope was passed with force via lower esophageal sphincter (LES). Barium swallow showed a dilated esophagus with distal narrowing and barium stasis (Figure 4). High resolution manometry showed normal LES resting pressure (23 mmHg) with incomplete relaxation (23%), prolonged IRP (integrated relaxation pressure) (18 mmHg) and raised intrabolus pressure in free drinking maneuver (Figure 4). These studies displayed ineffective response to previous treatment for ashalasia in our patient.

Bottom Line: Triple A syndrome (Allgrove syndrome) is a rare inherited autosomal recessive disease with a typical triad including adrenocorticotrophic-hormone-resistant glucocorticoid insufficiency, reduced or absent tearing (alacrima) and achalasia and a wide range of symptoms can be detected due to multi organ involvement.This report describes the case of a Triple Asyndrome, a12 year-old boy with a history of recurrent episodes of pneumonia and growth retardation due to failure to timely diagnosis of his problem.

View Article: PubMed Central - PubMed

Affiliation: Research Institute for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

ABSTRACT
Triple A syndrome (Allgrove syndrome) is a rare inherited autosomal recessive disease with a typical triad including adrenocorticotrophic-hormone-resistant glucocorticoid insufficiency, reduced or absent tearing (alacrima) and achalasia and a wide range of symptoms can be detected due to multi organ involvement. This report describes the case of a Triple Asyndrome, a12 year-old boy with a history of recurrent episodes of pneumonia and growth retardation due to failure to timely diagnosis of his problem.

No MeSH data available.


Related in: MedlinePlus