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Non-familial visceral myopathy: clinical and pathologic features of degenerative leiomyopathy.
Pediatr Surg Int. 2002 Jan; 18(1):6-12.PS

Abstract

Degenerative leiomyopathy (DL) is a distinctive form of acquired degenerative visceral myopathy of uncertain etiology that occurs largely in Africa and results in intestinal pseudo-obstruction (IP). In this review of 39 patients from the Western Cape region of South Africa, the mean age at presentation was 9.5 years (range 6 months to 16 years). Characteristic clinical features included a chronic, insidious history of repeated attacks of abdominal distension, abdominal pain, and vomiting. Marked gaseous distension with atony and IP, especially of the colon, was noted on X-ray films. Megacolon was the most common radiologic feature, but pseudo-obstruction extended proximally into the small intestine in some patients with advanced disease. In the majority of cases the condition was progressive and eventually affected the entire gastrointestinal (GI) tract.

Authors+Show Affiliations

Department of Paediatric Surgery, Faculty of Medicine, University of Stellenbosch, Tygerberg 7505, South Africa.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

11793055

Citation

Moore, S W., et al. "Non-familial Visceral Myopathy: Clinical and Pathologic Features of Degenerative Leiomyopathy." Pediatric Surgery International, vol. 18, no. 1, 2002, pp. 6-12.
Moore SW, Schneider JW, Kaschula RD. Non-familial visceral myopathy: clinical and pathologic features of degenerative leiomyopathy. Pediatr Surg Int. 2002;18(1):6-12.
Moore, S. W., Schneider, J. W., & Kaschula, R. D. (2002). Non-familial visceral myopathy: clinical and pathologic features of degenerative leiomyopathy. Pediatric Surgery International, 18(1), 6-12.
Moore SW, Schneider JW, Kaschula RD. Non-familial Visceral Myopathy: Clinical and Pathologic Features of Degenerative Leiomyopathy. Pediatr Surg Int. 2002;18(1):6-12. PubMed PMID: 11793055.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Non-familial visceral myopathy: clinical and pathologic features of degenerative leiomyopathy. AU - Moore,S W, AU - Schneider,J W, AU - Kaschula,R D C, PY - 2001/06/06/accepted PY - 2002/1/17/pubmed PY - 2002/3/27/medline PY - 2002/1/17/entrez SP - 6 EP - 12 JF - Pediatric surgery international JO - Pediatr Surg Int VL - 18 IS - 1 N2 - Degenerative leiomyopathy (DL) is a distinctive form of acquired degenerative visceral myopathy of uncertain etiology that occurs largely in Africa and results in intestinal pseudo-obstruction (IP). In this review of 39 patients from the Western Cape region of South Africa, the mean age at presentation was 9.5 years (range 6 months to 16 years). Characteristic clinical features included a chronic, insidious history of repeated attacks of abdominal distension, abdominal pain, and vomiting. Marked gaseous distension with atony and IP, especially of the colon, was noted on X-ray films. Megacolon was the most common radiologic feature, but pseudo-obstruction extended proximally into the small intestine in some patients with advanced disease. In the majority of cases the condition was progressive and eventually affected the entire gastrointestinal (GI) tract. SN - 0179-0358 UR - https://www.unboundmedicine.com/medline/citation/11793055/Non_familial_visceral_myopathy:_clinical_and_pathologic_features_of_degenerative_leiomyopathy_ L2 - https://doi.org/10.1007/s003830200002 DB - PRIME DP - Unbound Medicine ER -