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Cystic fibrosis transmembrane conductance regulator ion channel function testing in recurrent acute pancreatitis.
J Clin Gastroenterol 2008; 42(7):810-4JC

Abstract

GOALS

To understand the relationship between acute recurrent pancreatitis and cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction.

BACKGROUND

An emerging number of patients present with a nonclassic phenotype of cystic fibrosis (CF) with partial features or single-organ disease only. The association between the phenotype of recurrent pancreatitis CFTR dysfunction is unclear.

METHODS

Patients with idiopathic recurrent pancreatitis were referred for electrophysiologic investigation.

RESULTS

Thirty-three patients (18 males) aged 20+/-12 years with recurrent pancreatitis were studied. Three patients had mild asthma and 1 patient had mild ulcerative colitis. There was no family history of CF. All patients had normal imaging of the pancreatic duct by endoscopic retrograde cholangiopancreatography or magnetic resonance cholangiopancreatography. No patient was pancreatic insufficient. Mean sweat chloride values were 41+/-14 meq/L (range: 18 to 64). Nasal potential difference (NPD) measurement was pathologic in 7 patients. Mean basal potential difference in these 7 patients was -33+/-13 mV and there was an abnormal response to chloride-free and isoproterenol solutions. There was no difference in sweat chloride concentration between the 2 groups. Mutation analysis revealed W1282X/5T, D1152H/5T, and W1282X/- in 3 patients with abnormal NPD and 1 W1282X allele was found in 1 patient with normal NPD.

CONCLUSIONS

In this series, 21% of patients with recurrent pancreatitis have abnormalities of CFTR function. Patients presenting with recurrent, "idiopathic" pancreatitis require CFTR function testing.

Authors+Show Affiliations

CF Center, Hadassah University Hospital, Jerusalem, Israel.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

18360295

Citation

Segal, Idit, et al. "Cystic Fibrosis Transmembrane Conductance Regulator Ion Channel Function Testing in Recurrent Acute Pancreatitis." Journal of Clinical Gastroenterology, vol. 42, no. 7, 2008, pp. 810-4.
Segal I, Yaakov Y, Adler SN, et al. Cystic fibrosis transmembrane conductance regulator ion channel function testing in recurrent acute pancreatitis. J Clin Gastroenterol. 2008;42(7):810-4.
Segal, I., Yaakov, Y., Adler, S. N., Blau, H., Broide, E., Santo, M., ... Wilschanski, M. (2008). Cystic fibrosis transmembrane conductance regulator ion channel function testing in recurrent acute pancreatitis. Journal of Clinical Gastroenterology, 42(7), pp. 810-4. doi:10.1097/MCG.0b013e318156617c.
Segal I, et al. Cystic Fibrosis Transmembrane Conductance Regulator Ion Channel Function Testing in Recurrent Acute Pancreatitis. J Clin Gastroenterol. 2008;42(7):810-4. PubMed PMID: 18360295.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cystic fibrosis transmembrane conductance regulator ion channel function testing in recurrent acute pancreatitis. AU - Segal,Idit, AU - Yaakov,Yasmin, AU - Adler,Samuel N, AU - Blau,Hannah, AU - Broide,Efrat, AU - Santo,Moshe, AU - Yahav,Yaakov, AU - Klar,Aharon, AU - Lerner,Aaron, AU - Aviram,Micha, AU - Ellis,Ian, AU - Mountford,Roger, AU - Shteyer,Eyal, AU - Kerem,Eitan, AU - Wilschanski,Michael, PY - 2008/3/25/pubmed PY - 2008/10/16/medline PY - 2008/3/25/entrez SP - 810 EP - 4 JF - Journal of clinical gastroenterology JO - J. Clin. Gastroenterol. VL - 42 IS - 7 N2 - GOALS: To understand the relationship between acute recurrent pancreatitis and cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction. BACKGROUND: An emerging number of patients present with a nonclassic phenotype of cystic fibrosis (CF) with partial features or single-organ disease only. The association between the phenotype of recurrent pancreatitis CFTR dysfunction is unclear. METHODS: Patients with idiopathic recurrent pancreatitis were referred for electrophysiologic investigation. RESULTS: Thirty-three patients (18 males) aged 20+/-12 years with recurrent pancreatitis were studied. Three patients had mild asthma and 1 patient had mild ulcerative colitis. There was no family history of CF. All patients had normal imaging of the pancreatic duct by endoscopic retrograde cholangiopancreatography or magnetic resonance cholangiopancreatography. No patient was pancreatic insufficient. Mean sweat chloride values were 41+/-14 meq/L (range: 18 to 64). Nasal potential difference (NPD) measurement was pathologic in 7 patients. Mean basal potential difference in these 7 patients was -33+/-13 mV and there was an abnormal response to chloride-free and isoproterenol solutions. There was no difference in sweat chloride concentration between the 2 groups. Mutation analysis revealed W1282X/5T, D1152H/5T, and W1282X/- in 3 patients with abnormal NPD and 1 W1282X allele was found in 1 patient with normal NPD. CONCLUSIONS: In this series, 21% of patients with recurrent pancreatitis have abnormalities of CFTR function. Patients presenting with recurrent, "idiopathic" pancreatitis require CFTR function testing. SN - 1539-2031 UR - https://www.unboundmedicine.com/medline/citation/18360295/Cystic_fibrosis_transmembrane_conductance_regulator_ion_channel_function_testing_in_recurrent_acute_pancreatitis_ L2 - http://Insights.ovid.com/pubmed?pmid=18360295 DB - PRIME DP - Unbound Medicine ER -