Literature DB >> 463823

Zollinger-Ellison syndrome with Marden-Walker syndrome. Association of two rare diseases in a 5-year-old girl.

K Abe, N Niikawa, H Sasaki.   

Abstract

A 5-year-old girl had the symptom of vomiting for four years. Gastric analysis showed elevated basal acidity unaffected by betazole stimulation. Roentgenographic examinations of the upper gastrointestinal tract showed two ulcers in the lesser curvature of the stomach. Fasting serum gastrin values were remarkably elevated and a calcium infusion test resulted in noticeable increases in serum gastrin levels. These data were consistent with the Zollinger-Ellison syndrome. In addition, the patient demonstrated such anomalies as retarded physical and mental development, kyphoscoliosis, median cleft palate, joint contracture and unusual facies due to blepharophimosis, and malformed low-set ears. These clinical features were in accord with the Marden-Walker syndrome. To our knowledge, the present case presents the first case of the combination of these two rare entities in the literature.

Entities:  

Mesh:

Year:  1979        PMID: 463823     DOI: 10.1001/archpedi.1979.02130070071015

Source DB:  PubMed          Journal:  Am J Dis Child        ISSN: 0002-922X


  3 in total

Review 1.  Pathophysiology of gastrointestinal hormones. Implications for paediatrics.

Authors:  I Henrichs; W M Teller
Journal:  Eur J Pediatr       Date:  1980-10       Impact factor: 3.183

2.  Two brothers with the Marden-Walker syndrome: case report and review.

Authors:  F M Howard; P Rowlandson
Journal:  J Med Genet       Date:  1981-02       Impact factor: 6.318

3.  A 26-month-old child with Marden-Walker syndrome and pyloric stenosis.

Authors:  D Gossage; J M Perrin; M G Butler
Journal:  Am J Med Genet       Date:  1987-04
  3 in total

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