Chronic Relapsing Pancreatitis Research Articles

Homoeopathic management of chronic replapsing pancreatitis in a paediatric patient: A case report

Chronic relapsing pancreatitis in the paediatric age group is a challenging case, especially when presenting in its acute exacerbation. This case report highlights the management of chronic relapsing pancreatitis in a 9-year-old female patient with homoeopathic treatment. The patient reported a year-long history of recurrent fever, abdominal pain, and raising titres of lipase and amylase; she had been admitted to a higher centre twice. The totality was constructed on day 1 and a homoeopathic remedy was prescribed. Detailed case taking, done after a week, confirmed the same remedy. Later, when the patient had an acute exacerbation, the same remedy-frequently repeated, helped settle the acute episode in a couple of days. The patient has been following up regularly for 3 years; the frequency and intensity of relapses reduced considerably over time and there have been no episodes for more than a year.

Endoscopic diagnosis and management of chronic relapsing pancreatitis due to eroded embolization coils.

Endoscopic diagnosis and management of chronic relapsing pancreatitis due to eroded embolization coils.

R117H mutation of the gene encoding the cystic fibrosis transmembrane regulatory protein in patients with chronic relapsing pancreatitis

R117H mutation of the gene encoding the cystic fibrosis transmembrane regulatory protein in patients with chronic relapsing pancreatitis

Pancreatitis crónica. Algunos aspectos históricos relevantes

Since ancient times the increase of size and hardness sometimes presented by the abdominal structure known as the pancreas has attracted attention. Portal was the first to describe the clinical signs of chronic pancreatitis in 1803. In 1815, Fleischman speculated about the potential role of excessive alcohol consumption. Comfort coined the term “chronic relapsing pancreatitis” in 1946 and described hereditary pancreatitis 6 years later. Zuidema defined tropical pancreatitis in 1959 and 2 years later Sarles described another form of pancreatitis to which Yoshida gave the name autoimmune pancreatitis in 1995. Groove pancreatitis was described by Potet in 1970. Obstructive pancreatitis was defined in 1984 and Ammann identified idiopathic pancreatitis 3 years later. This article gives a historical account of the pioneers who developed the knowledge of how to assess the characteristics that allowed the different forms of chronic pancreatitis to be defined.

Tu1395 LONG TERM FOLLOW-UP OF PATIENTS WITH A DISCONNECTED PANCREATIC DUCT FOLLOWING TREATMENT OF WALLED-OFF NECROSIS

The disconnected pancreatic duct syndrome (DPDS) is a common complication of necrotizing pancreatitis. Transmural plastic stents (PS) are often left in-situ in patients with DPDS even after resolution of walled-off necrosis (WON) to allow internal diversion of the orphan pancreatic tail. Data is scarce on the long-term outcomes in these patients. Patients from our tertiary referral center were identified from an IRB approved, prospectively maintained database of WON that underwent endoscopic therapy between 2008 and 2017. DPDS was diagnosed either during endoscopic retrograde cholangiopancreatography (ERCP) or by magnetic resonance cholangiopancreatography (MRCP). Transmural stents draining WON were placed in all cases. Patients with a minimal follow-up of 6 months from endoscopic therapy were included. Ninety-six patients (70M, 26F), with a median age of 56 years (20-77) and DPDS were followed for a median of 32 months (6-114 months). 13/96 (14%) patients were treated with a lumen-apposing metal stent (LAMS) and 83/96 (86%) patients were treated with transmural PS. Fourteen patients (15%) were diagnosed with recurrent pancreatic fluid collections (PFC). Three were asymptomatic with a median size of 37mm. Eleven symptomatic PFCs, of which 8 were treated endoscopically and 3 managed conservatively, were 40mm in median size and recurred after 9 months. In 4 patients the LAMS was removed with no stent left in-situ; 1 of these patients developed a recurrent symptomatic pancreatic fluid collection (PFC). Ninety-two patients (96%) were left with transmural PS after resolution of WON. Recurrent PFCs were diagnosed in 8/35 patients in whom the PS had migrated (21%) and 5/57 patients with still indwelling PS (9%), (p=0.06). Seven surgeries (7%) were performed on these 96 patients: 3 distal pancreatectomies for chronic relapsing pancreatitis, 1 hepaticoduodenostomy for a persistent biliary stricture, 1 small bowel resection for perforation from a migrated transgastric plastic stent and 1 Roux-en-Y hepaticojejunostomy for a duodenal ampullary adenocarcinoma,. One patient died of metastatic pancreatic cancer at 52 months. An external pancreatic fistula (EPF) was diagnosed in 8 patients (8%); 7 with inability to remove percutaneous drain despite resolution of fluid collection and 1 EPF after removal of the percutaneous drain. All 8 patients were treated successfully with fistula internalization by a combined endoscopic interventional radiology procedure. Recurrent PFC in DPDS is not uncommon and seems to be more frequent when associated with stent migration, suggesting a role to possibly leave indwelling transmural stents after drainage of WON and DPDS. Recurrent PFCs and fistulae can be managed endoscopically and surgery is infrequently needed even in this subset of patients. Prospective, larger, multicenter studies are needed.

Progressive Pulmonary Hypertension and Lung Nodules Related to Intravenous Promethazine and Heparin Incompatibility.

SESSION TITLE: Miscellaneous SESSION TYPE: Global Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: This case describes a patient who had progressive embolic pulmonary hypertension and nodular lung disease related to intravenous heparin and promethazine drug incompatibility. CASE PRESENTATION: A 46-year-old woman with chronic relapsing pancreatitis under the supervision of a pain management specialist used self-administered promethazine injections through an implanted mediport at home. She presented to the hospital with abdominal pain, slowly progressive dyspnea on exertion, and a dry cough. Lab work demonstrated elevated liver function tests and CRP. Her chest x-ray showed borderline cardiomegaly and increased interstitial markings with reticular nodular opacities. CT angiography of the chest was negative for PE, but did confirm widespread poorly marginated small nodular densities as well as mild adenopathy. Bronchoscopy with BAL and wash was non-diagnostic. Her echo demonstrated an RVSP of 64 and a right heart catheter later confirmed moderate pulmonary HTN. Further lab workup for pulmonary HTN was negative. Pulmonary function testing showed restrictive disease with a reactive component. She required oxygen with exertion at discharge and was seen in close outpatient follow up for additional workup. Ten weeks later she was readmitted for symptom management of her severe abdominal pain. She denied any shortness of air or cough at that point in time. Repeat CT of the abdomen prompted chest imaging that demonstrated progressive widespread milliary nodular pattern of both lungs with progressive adenopathy. Echo demonstrated slight worsening of pulmonary hypertension. VATS was completed for surgical lung biopsy and pathology demonstrated an exuberant foreign body type reaction to polarizable amorphous foreign material associated with acute inflammation as well as focal microabscess formation. Cultures and stains for infectious organisms were negative. She denied illicit drug use and careful review of her outpatient medications revealed an in-line incompatibility of her IV heparin flushes and promethazine injections. MicroMedex IV compatibility listed the incompatibility as causing an increase in measured haze or turbidity, particulates and/or color change.1 Additional history was obtained and due to lack of readily available normal saline flushes, she had been using prefilled heparin syringes to flush her mediport after injecting promethazine. She was started on high dose prednisone that was slowly tapered over several months with normalization of her pulmonary hypertension on follow up echo. Follow up CT imaging demonstrated resolution of her adenopathy and improvement but incomplete resolution of her nodular airspace disease. DISCUSSION: The patient had been self-administering IV promethazine for nausea via an implanted port often several times per day. Her insurance company had only authorized a small allotment of prefilled saline syringes per month, but had provided a much larger allotment of prefilled heparin syringes for her to use for line maintenance. Due to this discrepancy, she would often use the more readily available heparin syringes as opposed to saline to flush her line after a promethazine dose. Incompatibility between IV promethazine and heparin led to precipitate formation that then embolized in the pulmonary circulation causing progressive pulmonary hypertension as well as diffuse micronodular airspace disease noted on CT. A progressive inflammatory reaction likely potentiated the progressive nodule growth. Treating the patient with steroids was successful in halting the inflammation and partial reversal of the disease process. Proper education about line maintenance and use of saline flushes prevented ongoing embolization. CONCLUSIONS: This case demonstrated the in-line incompatibility of IV promethazine and heparin had significant embolic effects on the pulmonary circulation. Obtaining a thorough history regarding utilization of home medications ultimately led to the diagnosis. Detailed attention as well as appropriate education and monitoring should be given to patients who self-administer IV medications via mediports. Reference #1: Truven Health Analytics MicroMedex IV compatability checker DISCLOSURE: The following authors have nothing to disclose: David Hasselbacher, Rebecca Parrish, Emma Coronel No Product/Research Disclosure Information

Chronic Pancreatitis: Landmark Papers, Management Decisions, and Future.

On May 16, 2015 at the invitation of the American Gastroenterological Association Institute Council E.P.D. presented a state-of-the-art lecture at Digestive Disease Week 2015. The aims were to discuss a selection of landmark papers in chronic pancreatitis (CP) that influence modern management and to conclude by suggesting some future directions. This is based on that presentation. We will specifically review the following: duct anatomy and pancreas divisum, description of chronic relapsing pancreatitis and its differentiation from recurrent acute pancreatitis and established CP (ECP), natural histories and gene discoveries of alcoholic, idiopathic and hereditary pancreatitis, development of pancreatic cancer in CP, exocrine pancreatic insufficiency and calculation of dose and delivery of enzymes, endoscopic ultrasonography, and autoimmune pancreatitis. With some exceptions, we exclude basic science and surgery.

Hereditary Pancreatitis Associated With the N29T Mutation of the PRSS1 Gene in a Brazilian Family

Hereditary pancreatitis (HP) is an autosomal-dominant disease with incomplete penetrance manifesting as early-onset chronic relapsing pancreatitis. A mutation in the PRSS1 gene is present in greater than 70% of HP kindreds and leads to a gain-of-function characterized by the increased autocatalytic conversion of trypsinogen to active trypsin, promoting autodigestion and damage to acinar cells. Other genetic defects observed in the pathogenic mechanism of pancreatitis include mutations in the genes encoding SPINK1, CTRC, and CPA1. There are few reports of HP in Latin America, and no families have been investigated in Brazil. A case-control observational study was conducted at Clementino Fraga Filho University Hospital in Brazil. Patients with suspected HP and healthy controls were enrolled in this study, and a detailed questionnaire was administered to patients with HP. PRSS1 and SPINK1 genes were analyzed by DNA sequencing, and a family that fit the HP diagnostic criteria was identified. The neutral polymorphism c.88-352A > G in the SPINK1 gene was found to be prevalent in the individuals studied, but no important alterations were found in this gene. Ten out of 16 individuals in this family carried the N29T mutation in the PRSS1 gene, with 2 clinically unaffected mutation carriers. The median age of HP onset was 6 years. Pancreatic exocrine failure occurred in 6 patients, 5 of whom also had diabetes mellitus. Surgical procedures were performed on 3 affected members, and no cases of pancreatic cancer have been reported thus far. This study identified the first PRSS1 gene mutation in a Brazilian family with HP.

Outcomes Following Laparoscopic Choledochoduodenostomy in the Management of Benign Biliary Obstruction

IntroductionWith the advent of endoscopic retrograde choledochoduodenostomy, the need for choledochoduodenostomy to treat common bile duct obstruction is less common, but occasionally required. MethodsPatients considered for laparoscopic choledochoduodenostomy secondary to benign conditions between 1999 and 2009 at a single institution were included. Charts were retrospectively reviewed for preoperative, operative, and long-term outcomes data. ResultsTwenty patients were identified; 15 with chronic choledocholithiasis causing benign biliary obstruction or chronic recurrent cholangitis, 3 with chronic relapsing pancreatitis, and 2 with distal common bile duct strictures. Mean operative time was 270 min. No major operative complications were reported. Five patients with severe adhesions or portal hypertension required conversion to laparotomy. Median hospital stay was 6 days (range, 2–32). Postoperatively, three (20%) patients completed laparoscopically and three (66.7%) patients converted to laparotomy had complications. In addition, one death due to myocardial infarction was reported 4 weeks after hospital discharge. With an average follow-up of 21 months, only one patient (5%) developed recurrent symptoms. ConclusionLaparoscopic choledochoduodenostomy is a useful technique in patients with benign, refractory common bile duct obstruction. This technically demanding procedure is feasible; however, the associated comorbidities in this complex group of patients result in a relatively high complication rate.

Clinical analysis of short- and long-term complications after endoscopic Oddi's sphincterotomy in 95 patients

Objective To investigate the short- and long-term complications after endoscopic Oddi's sphincterotomy (EST) upon endoscopic retrograde cholangiopancreatography (ERCP) procedure and determine whether the size of EST correlates to the occurrence of EST complications.Methods 95 cases receiving EST in the process of ERCP in our hospital were studied and followed up. The patients were divided into large, moderate and small incision groups according to the size of EST and the states of short-term and long-term EST complications were statistically analyzed.Results The incidence of short-term complications of EST was 18. 94% (18/95). They included bleeding in the process of ERCP in 11 cases, delayed bleeding in 3, acute pancreatitis in 1, acute cholangitis in 2 and duodenal perforation in 1. All these patients but 1 with duodenal perforation were discharged after undergoing symptomatic treatments. Eleven out of the 95 patients had long-term complications (11.57 % ). These included biliary system infection in 5 cases, recurrent calculus of bile duct in 3, papilla stricture in 1 and chronic relapsing pancreatitis in 2. All 11 patients recovered after therapeutic ERCP again or symptomatic drug treatments. There was no significant difference in incidence of short-term (χ2 =2.433, P=0.296) or long-term complications (χ2 = 1.151, P=0.562) among the 3 groups. Furthermore, there was no statistical correlation between the incision size of Oddi and complications including operative bleeding (P=0.109), short-term complications (P=0.124) and longterm complications(P=0.402). Conclusion There are many short-term and long-term complications after EST, but there is no correlation between the complications and the incision size of Oddi. The occurring rate of complications might be reduced through accurate direction of papilla incision, avoidance from injury of blood vessel, keeping bile drainage unobstructed and protection of the function of Oddi sphincter as far as possible in the process of ERCP. Key words: Operative complication; Oddi's sphincter; Endoscopic sphincterotomy

Variations in trypsinogen expression may influence the protective effect of the p.G191R PRSS2 variant in chronic pancreatitis

The description of a family with inherited chronic relapsing pancreatitis by Comfort and Steinberg (1952) provided the first indication towards a possible genetic component in the aetiology of chronic pancreatitis...

Reduced inhibitory effect of somatostatin on the exocrine function of the pancreas and on serum insulin (IRI) levels in chronic relapsing pancreatitis.

In 3 healthy men and 4 healthy women, and in 5 men and 2 women with confirmed chronic relapsing pancreatitis (CRP) the inhibitory effect of somatostatin cn pancreatic exocrine function and insulin secretion stimulated with pancreozymin and secretin was determined. In all 7 patients with CRP the volume of pancreatic juice and the bicarbonate and amylase and protein output after stimulation were lower than in healthy subjects, and somatostatin had a reduced inhibitory effect on both the basal secretion and particularly on the pancreozymin-secretin-stimulated secretion. A similarly lower inhibitory effect of somatostatin on the secretin-pancreozymin induced increase in blood insulin (IRI) level was observed in these patients. The presented data suggest that the damaged pancreas reacts less effectively not only to the stimuli enhancing its exocrine and endocrine secretory activity, but also to the stimuli inhibiting this activity. This observation may be of practical value for the evaluation of the functional efficiency of the pancreas.

Relationship between insulin secretion and pancreas morphology in subjects with chronic pancreatitis.

In order to investigate whether a relationship exists between in vivo insulin secretion and islet mass, 8 patients suffering from severe chronic relapsing pancreatitis were studied before and after pancreatectomy by glucose-glucagon-test (per os 1.75 g glucose; i.v. glucagon 0.01 mg/kg b.w.) and by intravenous glucose-tolerance-test (iGTT) (i.v. glucose 0.33 g/kg b.w.). Postoperative in vitro assessments of pancreatic insulin and alpha-amylase content were performed, and morphometric studies were carried out. Patients were characterized by reduced c-peptide secretion when compared with healthy subjects. The c-peptide response to the glucose-glucagon-test correlated well with the morphometrically estimated exocrine and islet tissue mass (P less than 0.05) and with the content of insulin and amylase in the tissue. The findings suggest that in subjects suffering from severe chronic relapsing pancreatitis the maximal insulin response might represent a parameter for the patient's islet mass.

Resection vs Drainage in Treatment of Chronic Pancreatitis: Long-term Results of a Randomized Trial

Tailored organ-sparing procedures have been shown to alleviate pain and are potentially superior in terms of preservation of endocrine and exocrine function as compared with standard resection (Whipple) for chronic pancreatitis with inflammatory pancreatic head tumor. Long-term results comparing these 2 procedures have not been published so far. The aim of this study was to report on long-term results of a randomized trial comparing a classical resective procedure (pylorus-preserving Whipple) with an extended drainage procedure (Frey) for chronic pancreatitis. All patients who participated in a previously published randomized trial on the perioperative course comparing both procedures were contacted with a standardized, validated, quality of life and pain questionnaire. Additionally, patients were seen in the outpatient clinic to assess endocrine and exocrine pancreatic function by an oral glucose tolerance test and fecal chymotrypsin test. There were no differences between both groups regarding quality of life, pain control, or other somatic parameters after a median of 7 years postoperatively. Correlations among continuous alcohol consumption, endocrine or exocrine pancreatic function, and pain were not found. Both procedures provide adequate pain relief and quality of life after long-term follow-up with no differences regarding exocrine and endocrine function. However, short-term results favor the organ-sparing procedure.

Symposium 3. Chronic pancreatitis: current problems of the diagnostic criteria

criteria for chronic pancreatitis currently used in Japan were proposed by the Japan Pancreas Society (JPS) in 1995 (JPS Criteria 1995),5,6 with a small revision to include magnetic resonance cholangiopancreatography fi ndings in 2001 (JPS Criteria 2001).7,8 The fi rst Marseilles symposium of 1963 classifi ed pancreatitis into four main groups: (1) acute pancreatitis, (2) relapsing acute pancreatitis with clinical biological restitution of the gland, (3) chronic relapsing pancreatitis with acute exacerbation, and (4) chronic pancreatitis with anatomical and/or functional residual damage to the gland.9 The defi nition of chronic pancreatitis is based on the persistence of lesions. The Cambridge classifi cation of 1983 provided a grading and severity system using imaging tests.3,10 The second Marseilles symposium of 19842 clinically characterized chronic pancreatitis as recurrent or persisting abdominal pain along with pancreatic insuffi ciency indicated by steatorrhea or diabetes, and morphologically characterized it as irregular sclerosis with destruction and permanent loss of exocrine parenchyma and varying degrees of dilatation of the duct system. The most recent Marseilles–Rome classifi cation of 19884 classifi ed chronic pancreatitis into three groups; (1) chronic obstructive pancreatitis; (2) chronic calcifying pancreatitis, which is characterized by the presence of intraductal protein precipitates or plugs and calcifi ed precipitates (calculi or stones); and (3) chronic infl ammatory pancreatitis, which is characterized by the loss of exocrine parenchyma replaced by a dense fi brosis infi ltrated by mononuclear cells. Despite these international expert meetings in the past 40 years, there is no consensus on the classifi cation of chronic pancreatitis because many data on the diagnosis and treatment of chronic pancreatitis from different centers are hard to compare, except those data on patients with advanced chronic pancreatitis. The JPS Criteria of 1995 and 2001 classifi ed chronic pancreatitis into (1) defi nite, (2) probable, and (3) possible chronic pancreatitis.11 The criteria can be applied

The operative diagnosis of carcinoma of the pancreas.

Abstract The radiological appearances of the main pancreatic duct in patients with carcinoma of the head of the pancreas is described. It is suggested that both a uniform dilatation of the duct system and a gap between the head of the dye and the duodenum may be taken as indicative of the presence of a carcinoma and hence allow an immediate distinction between this condition and chronic relapsing pancreatitis.

Chronic relapsing pancreatitis: a review of 64 cases.

A collected series of 64 cases of chronic relapsing pancreatitis is reported. Analysis of this material reveals several points of interest. There is a difference in the aetiological spectrum in Britain when compared with reports from France and the United States. In particular, nearly half the British cases were idiopathic. The clinical presentation and the age and sex ratios also varied with aetiology. Endoscopic retrograde cannulation of the pancreatic duct was of little value in confirming a diagnosis of pancreatitis in the problem case. This investigation did, however, demonstrate that a widespread dilatation of the pancreatic duct was a minority finding. In those patients with alcoholic pancreatitis follow-up studies have shown that, if the addiction can be broken, there is a 75 per cent chance that pain will diminish or disappear with the passage of time. The main indication for surgical intervention was severe pain and this study has shown that if strict criteria are observed, a worthwhile relief of symptoms can be achieved. In particular, subtotal pancreatectomy produced good results in up to 85 per cent of cases, although with an appreciable short term postoperative morbidity.

A Proinflammatory, Antiapoptotic Phenotype Underlies the Susceptibility to Acute Pancreatitis in Cystic Fibrosis Transmembrane Regulator (−/−) Mice

A Proinflammatory, Antiapoptotic Phenotype Underlies the Susceptibility to Acute Pancreatitis in Cystic Fibrosis Transmembrane Regulator (−/−) Mice

Percutaneous drainage of spontaneous subcapsular haematoma of the spleen complicating chronic pancreatitis

Subcapsular haematoma of the spleen is a rare complication of chronic pancreatitis. In the literature only a handful of cases have been documented. The exact aetiology and natural history of this complication remains speculative and its management controversial. A case of spontaneous subacute haematoma of the spleen in a patient with chronic relapsing alcohol-related pancreatitis was reported. A percutaneous drainage was performed with good outcome. A review of the literature has demonstrated only one previous similar report.

A follow-up study of annular pancreas in infants and children.

Fifteen pediatric patients undergoing surgery for annular pancreas from 1984 to 1996 were analyzed. Vomiting was the most common presenting symptom. Twelve patients (80%) had associated anomalies including malrotation (40%), intrinsic duodenal obstruction (33%), Down syndrome (27%) and duodenal bands (27%). Their ages at operation were between 5 hours and 8.5 years, with a median of 4 days. Surgical treatment included duodenojejunostomy in nine, duodenoduodenostomy in five and duodenotomy with duodenoplasty in one. The mean duration for reestablishment of bowel transit was 17.9 days, with 22.8 days for duodenojejunostomy and 12.3 days for duodenoduodenostomy. All cases received postoperative follow-up, but only 11 of them were long-term followed until April 1997, with a duration ranging from 1 year and 2 months to 11 years, with a median of 7 years and 5 months. The survival was 100%, but 12 cases (80%) developed postoperative complications including cholestatic jaundice (53%), upper gastrointestinal motility disorder (47%), failure to thrive (40%) and chronic diarrhea (33%). Annular pancreas divisum was noted in one case with chronic relapsing pancreatitis. At the end of follow-up (April 1997), when final ages ranged from 1 year and 2 months to 18 years and 9 months with a median of 7 years and 5 months, there were still problems: steatorrhea in 1, diarrhea after fatty diet in 3, malnutrition in 4, failure to thrive in 3 and lower concentration of stool trypsin in 3 cases. In conclusion, close long-term follow-up is essential for infants treated for annular pancreas because many of them can be expected to develop complications, even if the initial postoperative period is uncomplicated and survival is excellent.