Abstract：
BACKGROUND:Achalasia in childhood is rare, also the etiology and the pathogenesis of the early onset ort he disease is practically unknown. Little is known about the neuropathological changes in structure of the esophageal wall in non-hereditary, sporadic achalasia in children and ist differentiation to that in adults. The aim of our study was to examine the morphological properties or high-pressure zone of the lower esophageal sphincter in children who had undergone a Heller myotomy because of achalasia as well as to compare them with the pathological findings in adults. METHODS:Muscle biopsies of the smooth musculature, a 20 x 10 mm long segment of the myenteric of the distal esophagus (lower boundary of the esophageal incision of the myotomy), were taken for histopathological and immunohistochemical studies. RESULTS:A conspicuous histomorphological finding was a marked reduction of the myenteric ganglion through to complete aganglionosis of the high-pressure zone of the lower esophageal sphincter. In contrast to achalasia in adults, neural inflammation was found only rarely. A pronounced fibrosis of the smooth muscle layers was found in all patients whereas muscular hypertrophy or visceral myopathy was not present. The interstitial Cajal cells were reduced, similar to those in adults. CONCLUSIONS:The variability of the clinical and pathological properties in cases of childhood achalasia are indicative of a complex pattern of varying etiologies and a comparison with the disease in adults does not, in principle, allow the assumption of a separate clinical entity. The present findings are compatible with the histopathological results of hereditary achalasia in children as described for Allgrove's syndrome.
journal_name
Z Gastroenteroljournal_title
Zeitschrift fur Gastroenterologieauthors
Bohl J,Gockel I,Sultanov F,Eckardt V,Junginger Tdoi
10.1055/s-2007-963649subject
Has Abstractpub_date
2007-12-01 00:00:00pages
1273-80issue
12eissn
0044-2771issn
1439-7803journal_volume
45pub_type
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journal_title：Zeitschrift fur Gastroenterologie
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journal_title：Zeitschrift fur Gastroenterologie
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journal_title：Zeitschrift fur Gastroenterologie
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journal_title：Zeitschrift fur Gastroenterologie
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journal_title：Zeitschrift fur Gastroenterologie
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journal_title：Zeitschrift fur Gastroenterologie
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doi：
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journal_title：Zeitschrift fur Gastroenterologie
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journal_title：Zeitschrift fur Gastroenterologie
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更新日期：1997-09-01 00:00:00
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journal_title：Zeitschrift fur Gastroenterologie
pub_type： 杂志文章
doi：
更新日期：1996-03-01 00:00:00
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journal_title：Zeitschrift fur Gastroenterologie
pub_type： 杂志文章
doi：
更新日期：1976-07-01 00:00:00
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journal_title：Zeitschrift fur Gastroenterologie
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journal_title：Zeitschrift fur Gastroenterologie
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journal_title：Zeitschrift fur Gastroenterologie
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doi：
更新日期：1976-05-01 00:00:00
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journal_title：Zeitschrift fur Gastroenterologie
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doi：
更新日期：1997-06-01 00:00:00
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pub_type： 杂志文章
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