Tags

Type your tag names separated by a space and hit enter

Acute pancreatitis in a young girl with the Netherton syndrome.
J Pediatr Surg. 2005 Nov; 40(11):e69-72.JP

Abstract

Acute pancreatitis is uncommon in children younger than 15 years. We present the first report on the association of acute pancreatitis with the Netherton syndrome. The Netherton syndrome is an inherited skin disease characterized by ichthyosiform erythroderma, a pathognomonic hair shaft defect ("bamboo hair"), and atopic features. A 14-year-old girl with symptoms and signs of severe acute pancreatitis was admitted to our department. A diagnostic workup could not reveal any common known cause of pancreatitis, and the cause of pancreatitis would most likely be considered idiopathic. However, based on recent reports regarding various pathophysiological mechanisms for both acute pancreatitis and the Netherton syndrome (eg, shearing the 5q locus for the respective gene-associated defects in SPINK1 and SPINK5), we speculate if a possible association may exist. Investigations on pancreatitis and the Netherton syndrome may disclose factors closely involved in the pathomechanisms of both. This notion may be of clinical importance as it adds to the number of potential life-threatening events to patients with the Netherton syndrome.

Authors+Show Affiliations

Department of Surgery, Stavanger University Hospital, N-4068 Stavanger, Norway.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

16291148

Citation

Söreide, Kjetil, et al. "Acute Pancreatitis in a Young Girl With the Netherton Syndrome." Journal of Pediatric Surgery, vol. 40, no. 11, 2005, pp. e69-72.
Söreide K, Söiland H, Körner H, et al. Acute pancreatitis in a young girl with the Netherton syndrome. J Pediatr Surg. 2005;40(11):e69-72.
Söreide, K., Söiland, H., Körner, H., Haga, H., & Söreide, J. A. (2005). Acute pancreatitis in a young girl with the Netherton syndrome. Journal of Pediatric Surgery, 40(11), e69-72.
Söreide K, et al. Acute Pancreatitis in a Young Girl With the Netherton Syndrome. J Pediatr Surg. 2005;40(11):e69-72. PubMed PMID: 16291148.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Acute pancreatitis in a young girl with the Netherton syndrome. AU - Söreide,Kjetil, AU - Söiland,Håvard, AU - Körner,Hartwig, AU - Haga,Harald, AU - Söreide,Jon Arne, PY - 2005/11/18/pubmed PY - 2006/7/27/medline PY - 2005/11/18/entrez SP - e69 EP - 72 JF - Journal of pediatric surgery JO - J Pediatr Surg VL - 40 IS - 11 N2 - Acute pancreatitis is uncommon in children younger than 15 years. We present the first report on the association of acute pancreatitis with the Netherton syndrome. The Netherton syndrome is an inherited skin disease characterized by ichthyosiform erythroderma, a pathognomonic hair shaft defect ("bamboo hair"), and atopic features. A 14-year-old girl with symptoms and signs of severe acute pancreatitis was admitted to our department. A diagnostic workup could not reveal any common known cause of pancreatitis, and the cause of pancreatitis would most likely be considered idiopathic. However, based on recent reports regarding various pathophysiological mechanisms for both acute pancreatitis and the Netherton syndrome (eg, shearing the 5q locus for the respective gene-associated defects in SPINK1 and SPINK5), we speculate if a possible association may exist. Investigations on pancreatitis and the Netherton syndrome may disclose factors closely involved in the pathomechanisms of both. This notion may be of clinical importance as it adds to the number of potential life-threatening events to patients with the Netherton syndrome. SN - 1531-5037 UR - https://www.unboundmedicine.com/medline/citation/16291148/Acute_pancreatitis_in_a_young_girl_with_the_Netherton_syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0022-3468(05)00581-6 DB - PRIME DP - Unbound Medicine ER -