Hereditary pancreatitis (HP)

Clinical characteristics, ERCP findings, outcome of endoscopic therapy and surgery

C. P. Choudari, N. J. Nickl, Evan Fogel, Stuart Sherman, Glen Lehman

Research output: Contribution to journalArticle

Abstract

There are limited data on the role of diagnostic/therapeutic ERCP and need for surgical intervention in patients (pts) with HP. We tallied clinical, radiography findings, and outcome of endoscopic and surgical therapy in a two-center series. Methods: The medical records and radiographs of 17 pts known to have HP who had undergone ERCP were reviewed along with telephone follow-up. Results: Seventeen pts (M:F 7:10; age range 9-62 yrs) were studied. The diagnosis was made by identifying a mutation in the cationic trypsinogen gene in 7 and strong family history in 10. The age of onset of symptoms was <5 yrs in 2; 6-15 yrs in 8; 16-20 yrs in 4; >20 yrs in 3. The age at diagnosis was <5 yrs in 1; 6-15 yrs in 4; 16-20 yrs in 1; and > 20 yrs in 11. All pts (100%) had abdominal pain and 53% had more than 5 hospitalizations prior to determining the correct hereditary etiology. Pancreatograms revealed Cambridge grade I chronic pancreatitis (CP) in 4; grade II in 4, grade III in 9; pancreatic duct (PD) strictures in 6 (head-4; body/tail 2); PD stone disease in 7 and pseudocyst in two. ERCP therapy was performed in 11 pts: biliary endoscopic sphincterotomy (ES) in 5 (with cystgastrostomy in 1); pancreatic stents in 4 (with stricture dilation in 1, combined biliary and pancreatic ES in 2, pancreatic ES alone in 1); pancreatic ES with PD stone extraction in 2. Four pts underwent repeat therapy. At a mean follow-up of 20 months after endoscopic therapy, 5 pts were pain free, 3 pts had >50% improvement in symptoms, and 3 pts underwent surgery (2 had cancer). Seven pts (3 following endo therapy) underwent surgery (duodenal-sparing pancreatectomy in 4; distal pancreatectomy in 2; gastric bypass in 1). At a mean surgical follow-up of 18 months, 4 pts remain asymptomatic, one has recurrent pain, one pt died (pancreatic cancer), and one is ill (pancreatic cancer). Summary: 1) 82% had their first attack of pancreatitis before their 20th yr, but only 35% were diagnosed as having HP by that age. 2) Chronic pancreatitis and its complications (strictures in 35%; stones in 41% and pseudocyst in 12%) are common findings at ERCP. 3) Forty-one percent (7/17) underwent surgery and 2 (12%) had pancreatic cancer. 4) Following therapy (endoscopic or surgery) around 50% remain asymptomatic. Conclusions: CP in HP tends to run an aggressive course with early age onset. Endoscopic/surgical therapy appear to benefit selected patients. Careful follow-up is necessary as pancreatic cancer may supervene.

Original languageEnglish
JournalGastrointestinal Endoscopy
Volume47
Issue number4
StatePublished - 1998
Externally publishedYes

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Endoscopic Retrograde Cholangiopancreatography
Endoscopic Sphincterotomy
Pancreatic Neoplasms
Pancreatic Ducts
Chronic Pancreatitis
Therapeutics
Pathologic Constriction
Pancreatectomy
Age of Onset
Hereditary pancreatitis
Trypsinogen
Gastric Bypass
Telephone
Radiography
Pancreatitis
Abdominal Pain
Stents
Medical Records
Dilatation
Hospitalization

ASJC Scopus subject areas

  • Gastroenterology

Cite this

@article{b918636d03f548039ec32021cfa8458a,
title = "Hereditary pancreatitis (HP): Clinical characteristics, ERCP findings, outcome of endoscopic therapy and surgery",
abstract = "There are limited data on the role of diagnostic/therapeutic ERCP and need for surgical intervention in patients (pts) with HP. We tallied clinical, radiography findings, and outcome of endoscopic and surgical therapy in a two-center series. Methods: The medical records and radiographs of 17 pts known to have HP who had undergone ERCP were reviewed along with telephone follow-up. Results: Seventeen pts (M:F 7:10; age range 9-62 yrs) were studied. The diagnosis was made by identifying a mutation in the cationic trypsinogen gene in 7 and strong family history in 10. The age of onset of symptoms was <5 yrs in 2; 6-15 yrs in 8; 16-20 yrs in 4; >20 yrs in 3. The age at diagnosis was <5 yrs in 1; 6-15 yrs in 4; 16-20 yrs in 1; and > 20 yrs in 11. All pts (100{\%}) had abdominal pain and 53{\%} had more than 5 hospitalizations prior to determining the correct hereditary etiology. Pancreatograms revealed Cambridge grade I chronic pancreatitis (CP) in 4; grade II in 4, grade III in 9; pancreatic duct (PD) strictures in 6 (head-4; body/tail 2); PD stone disease in 7 and pseudocyst in two. ERCP therapy was performed in 11 pts: biliary endoscopic sphincterotomy (ES) in 5 (with cystgastrostomy in 1); pancreatic stents in 4 (with stricture dilation in 1, combined biliary and pancreatic ES in 2, pancreatic ES alone in 1); pancreatic ES with PD stone extraction in 2. Four pts underwent repeat therapy. At a mean follow-up of 20 months after endoscopic therapy, 5 pts were pain free, 3 pts had >50{\%} improvement in symptoms, and 3 pts underwent surgery (2 had cancer). Seven pts (3 following endo therapy) underwent surgery (duodenal-sparing pancreatectomy in 4; distal pancreatectomy in 2; gastric bypass in 1). At a mean surgical follow-up of 18 months, 4 pts remain asymptomatic, one has recurrent pain, one pt died (pancreatic cancer), and one is ill (pancreatic cancer). Summary: 1) 82{\%} had their first attack of pancreatitis before their 20th yr, but only 35{\%} were diagnosed as having HP by that age. 2) Chronic pancreatitis and its complications (strictures in 35{\%}; stones in 41{\%} and pseudocyst in 12{\%}) are common findings at ERCP. 3) Forty-one percent (7/17) underwent surgery and 2 (12{\%}) had pancreatic cancer. 4) Following therapy (endoscopic or surgery) around 50{\%} remain asymptomatic. Conclusions: CP in HP tends to run an aggressive course with early age onset. Endoscopic/surgical therapy appear to benefit selected patients. Careful follow-up is necessary as pancreatic cancer may supervene.",
author = "Choudari, {C. P.} and Nickl, {N. J.} and Evan Fogel and Stuart Sherman and Glen Lehman",
year = "1998",
language = "English",
volume = "47",
journal = "Gastrointestinal Endoscopy",
issn = "0016-5107",
publisher = "Mosby Inc.",
number = "4",

}

TY - JOUR

T1 - Hereditary pancreatitis (HP)

T2 - Clinical characteristics, ERCP findings, outcome of endoscopic therapy and surgery

AU - Choudari, C. P.

AU - Nickl, N. J.

AU - Fogel, Evan

AU - Sherman, Stuart

AU - Lehman, Glen

PY - 1998

Y1 - 1998

N2 - There are limited data on the role of diagnostic/therapeutic ERCP and need for surgical intervention in patients (pts) with HP. We tallied clinical, radiography findings, and outcome of endoscopic and surgical therapy in a two-center series. Methods: The medical records and radiographs of 17 pts known to have HP who had undergone ERCP were reviewed along with telephone follow-up. Results: Seventeen pts (M:F 7:10; age range 9-62 yrs) were studied. The diagnosis was made by identifying a mutation in the cationic trypsinogen gene in 7 and strong family history in 10. The age of onset of symptoms was <5 yrs in 2; 6-15 yrs in 8; 16-20 yrs in 4; >20 yrs in 3. The age at diagnosis was <5 yrs in 1; 6-15 yrs in 4; 16-20 yrs in 1; and > 20 yrs in 11. All pts (100%) had abdominal pain and 53% had more than 5 hospitalizations prior to determining the correct hereditary etiology. Pancreatograms revealed Cambridge grade I chronic pancreatitis (CP) in 4; grade II in 4, grade III in 9; pancreatic duct (PD) strictures in 6 (head-4; body/tail 2); PD stone disease in 7 and pseudocyst in two. ERCP therapy was performed in 11 pts: biliary endoscopic sphincterotomy (ES) in 5 (with cystgastrostomy in 1); pancreatic stents in 4 (with stricture dilation in 1, combined biliary and pancreatic ES in 2, pancreatic ES alone in 1); pancreatic ES with PD stone extraction in 2. Four pts underwent repeat therapy. At a mean follow-up of 20 months after endoscopic therapy, 5 pts were pain free, 3 pts had >50% improvement in symptoms, and 3 pts underwent surgery (2 had cancer). Seven pts (3 following endo therapy) underwent surgery (duodenal-sparing pancreatectomy in 4; distal pancreatectomy in 2; gastric bypass in 1). At a mean surgical follow-up of 18 months, 4 pts remain asymptomatic, one has recurrent pain, one pt died (pancreatic cancer), and one is ill (pancreatic cancer). Summary: 1) 82% had their first attack of pancreatitis before their 20th yr, but only 35% were diagnosed as having HP by that age. 2) Chronic pancreatitis and its complications (strictures in 35%; stones in 41% and pseudocyst in 12%) are common findings at ERCP. 3) Forty-one percent (7/17) underwent surgery and 2 (12%) had pancreatic cancer. 4) Following therapy (endoscopic or surgery) around 50% remain asymptomatic. Conclusions: CP in HP tends to run an aggressive course with early age onset. Endoscopic/surgical therapy appear to benefit selected patients. Careful follow-up is necessary as pancreatic cancer may supervene.

AB - There are limited data on the role of diagnostic/therapeutic ERCP and need for surgical intervention in patients (pts) with HP. We tallied clinical, radiography findings, and outcome of endoscopic and surgical therapy in a two-center series. Methods: The medical records and radiographs of 17 pts known to have HP who had undergone ERCP were reviewed along with telephone follow-up. Results: Seventeen pts (M:F 7:10; age range 9-62 yrs) were studied. The diagnosis was made by identifying a mutation in the cationic trypsinogen gene in 7 and strong family history in 10. The age of onset of symptoms was <5 yrs in 2; 6-15 yrs in 8; 16-20 yrs in 4; >20 yrs in 3. The age at diagnosis was <5 yrs in 1; 6-15 yrs in 4; 16-20 yrs in 1; and > 20 yrs in 11. All pts (100%) had abdominal pain and 53% had more than 5 hospitalizations prior to determining the correct hereditary etiology. Pancreatograms revealed Cambridge grade I chronic pancreatitis (CP) in 4; grade II in 4, grade III in 9; pancreatic duct (PD) strictures in 6 (head-4; body/tail 2); PD stone disease in 7 and pseudocyst in two. ERCP therapy was performed in 11 pts: biliary endoscopic sphincterotomy (ES) in 5 (with cystgastrostomy in 1); pancreatic stents in 4 (with stricture dilation in 1, combined biliary and pancreatic ES in 2, pancreatic ES alone in 1); pancreatic ES with PD stone extraction in 2. Four pts underwent repeat therapy. At a mean follow-up of 20 months after endoscopic therapy, 5 pts were pain free, 3 pts had >50% improvement in symptoms, and 3 pts underwent surgery (2 had cancer). Seven pts (3 following endo therapy) underwent surgery (duodenal-sparing pancreatectomy in 4; distal pancreatectomy in 2; gastric bypass in 1). At a mean surgical follow-up of 18 months, 4 pts remain asymptomatic, one has recurrent pain, one pt died (pancreatic cancer), and one is ill (pancreatic cancer). Summary: 1) 82% had their first attack of pancreatitis before their 20th yr, but only 35% were diagnosed as having HP by that age. 2) Chronic pancreatitis and its complications (strictures in 35%; stones in 41% and pseudocyst in 12%) are common findings at ERCP. 3) Forty-one percent (7/17) underwent surgery and 2 (12%) had pancreatic cancer. 4) Following therapy (endoscopic or surgery) around 50% remain asymptomatic. Conclusions: CP in HP tends to run an aggressive course with early age onset. Endoscopic/surgical therapy appear to benefit selected patients. Careful follow-up is necessary as pancreatic cancer may supervene.

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