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Exocrine pancreatic insufficiency and muscular dystrophy in a father and daughter - a new syndrome
S132
Abstracts
Purpose: A 45–year– old man with history of chronic alcoholic pancreatitis presented with dyspnea and right–sided chest pain of ten days duration. He had an episode of acute pancreatitis 8 months back with accompanying ascites, which resolved with treatment. Physical examination revealed a right–sided effusion that was confirmed on chest radiography. Thoracentesis revealed rapidly refilling hemorrhagic pleural effusion requiring multiple aspirations. The pleural fluid amylase was 3200 IU with a corresponding serum amylase of 454 IU, fluid protein 3.4gm/dl and cytology was negative for malignant cells. CT scan showed right–sided pleural effusion, features of chronic pancreatitis and a pseudocyst in the head of the pancreas. There was no ascites and no primary pulmonary disease. Magnetic resonance pancreatography performed revealed a diffusely dilated pancreatic duct. Although no fistulous tract could be identified, based on a high index of clinical suspicion a diagnosis of pancreatico–pleural fistula due to pancreatic duct disruption or leak from pseudocyst was entertained. Patient was commenced on conservative management, which included no oral intake, total parenteral nutrition, repeated thoracentesis and octreotide administration. The rapid refilling of the effusion stopped and a repeat ultrasound after three weeks showed complete resolution of the pleural effusion. Discussion: Pancreatico–pleural fistula is a rare condition in which the pancreatic enzymes drain directly into the pleural cavity, most commonly from an enlarging pseudocyst. Pleural effusions are large, recurrent and usually left–sided. Isolated right–sided effusion without ascites as seen in our patient is very rare. Its presentation is often confusing with a predominance of pulmonary symptoms and a relative absence of abdominal complaints. Many patients undergo extensive pulmonary evaluation before pancreas is identified as the cause of the effusion. Pancreatico–pleural fistula should be suspected in a patient with history of alcoholism and recurrent pleural effusion. The key to the diagnosis is a dramatically increased pleural fluid amylase. Demonstration of a fistula tract between the pancreas and the pleural space by ERCP is the diagnostic standard, however, we feel that in the appropriate clinical situation conservative treatment for pancreatico–pleural fistula may be offered to patients prior to a more invasive procedure.
401 CELIAC DISEASE MIMICKING AS IMMUNOPROLIFERATIVE SMALL INTESTINAL DISEASE Mohinish Chhabra, M.D.* and Shailender Singh, M.D. Chhabra Gastroenterology & Endoscopy Clinic, Chandigarh, India and Department of Internal Medicine, Mercy Catholic Medical Center, Darby, PA. Purpose: A 24 –year– old Asian male presented with abdominal pain, small bowel diarrhea and forty–pound weight loss of 2 years duration. His past medical history was unremarkable and he was not on any medications. General physical exam was unremarkable except for pallor and clubbing. Abdominal examination revealed normal bowel sounds without any evidence of organomegaly or ascites. Initial laboaratory data showed iron deficiency anemia with a hemoglobin of 8 gm/dl. Other than serum albumin of 2.6 gm/dl the comprehensive metabolic panel was within normal limits. Stool analysis confirmed steatorrhea and microscopic examination was negative for giardiasis. Small bowel contrast radiography showed irregular and nodular mucosa. Upper endoscopy also revealed multiple small nodules of varying sizes in the second and third part of duodenum. The mucosal folds were decreased in size and number. Small bowel biopsy showed total villous atrophy with a lymphoplasmacytic infiltrate in lamina propria. In view of the symptoms, radiographic findings and plasmacytoid differentiation of the infiltrate, immunoproliferative small intestinal disease (IPSID) was considered. Further investigations revealed normal serum protein electrophoresis, however, serology for celiac disease was positive for antiendomysial antibodies. Patient was commenced on gluten–free diet which resulted in complete and dramatic resolution of his symptoms. Repeat endoscopy six months later demonstrated disappearance of the duodenal nodularity.
AJG – Vol. 97, No. 9, Suppl., 2002
Discussion: The foremost cause of malabsorption with lymphoplasmacytic infiltration of the small bowel mucosa depends on the geographic area, IPSID being common in the developing world. Although, celiac disease is predominantly seen in the western hemisphere it should be considered in the differential diagnosis of malabsorption in developing countries as well. Common endoscopic findings in CD include scalloping, grooving, mosaic pattern along with decreased number and height of mucosal folds. However, unusual finding of duodenal mucosal nodularity, more common in IPSID, can also occur in CD as seen in our patient. The pathologic features of broadened or effaced villi with short crypts as opposed to total villous atrophy with long hyperplastic crypts along with serological tests help differentiate IPSID from celiac disease. It is essential to differentiate between these two conditions since treatment options are entirely different. 402 ASPERGILLUS ASSOCIATED WITH GASTRIC ULCERATION AND BLEEDING HERALDS A POOR OUTCOME Khalid Khan, M.B.Ch.B. and Sally Weisdorf–Schindele, M.D.*. Pediatrics, University of Minnesota, Minneapolis, MN. Background: Infection with Aspergillus in immunosuppressed individuals can be fatal complication. We report 4 children whose outcome was related to bleeding gastric ulcers caused by Aspergillus. Three of 4 died. Case 1. A 15 year– old male developed hematemesis 4 days after cadaver liver transplantation for acute fulminant hepatitis. At endoscopy an ulcer was seen in the fundus extending through the gastroesophageal junction. Brushings of the membranous black granulation tissue showed Aspergillus. He was treated aggressively but died of cerebral tonsillar herniation 2 days later. Multiple intracranial lesions were found at autopsy. Case 2. A 8 –year– old male, 78 days post cord blood transplantation for relapsed ALL was ventilated for respiratory failure and upper intestinal bleeding. At endoscopy large shallow areas of ulceration and bleeding were covered in black granulation tissue. Gastric brushings grew Aspergillus. Gastric bleeding areas were cauterized. The patient succumbed to multisystem infection within 2 days. Case 3. An 18 –year– old girl on chemotherapy for osteosarcoma was endoscoped after a hematemsis. Multiple shallow ulcers with dark granulation were seen throughout the stomach. Brushings revealed Aspergillus. Two days later she became acutely unwell, and at laparotomy necrotic gastric tissue was removed (histology showed Aspergillus. The liver, cerebral cortex and lungs were involved on CT. She made a gradual recovery. Case 4. A 2–year– old male developed a 2cm black skin lesion 6 days after cord blood transplantation for aplastic anemia. He had a craniotomy for fungal mass in the frontal lobes but made a gradual recovery. At 59 days he deteriorated with intestinal and pulmonary hemorrhage. Large bleeding ulcers with typical black appearance were evident in the stomach. Despite cautery the patient died of overwelming blood loss. Conclusion: Multisystem involvement is likely when there is endoscopic evidence of Aspergillus in immunosuppressed children. The typical findings are shallow ulcers with a dark ulcer base. Early endoscopy for upper intestinal symptoms should be considered in this group. 403 EXOCRINE PANCREATIC INSUFFICIENCY AND MUSCULAR DYSTROPHY IN A FATHER AND DAUGHTER – A NEW SYNDROME Khalid Khan, M.B.Ch.B., Steven Smith, M.D. and Nancy Mendelsohn, M.D.*. Pediatrics, University of Minnesota, Minneapolis, MN and Medicine, University of Minnesota, Minneapolis, MN. Purpose: There are few reports of primary exocrine pancreatic insufficiency outside of Cystic Fibrosis. None have been associated with muscle disease. We describe a young girl and her father with the exact same clinical phenotype. We believe this to be a new syndrome. Cases: The father was investigated at 13 years of age for orange oily stools for one year. A rash from infancy comprozed of telengectasia of the face and raised roughened, pigmented areas over the lateral areas of the trunk.
AJG – September, Suppl., 2002
Abstracts
He had thin arms and was found to have weakness and wasting in the upper limbs, more pronounced distally. Muscle biopsy gave evidence of both a neurpathy and degeneration of muscle fibers and the EMG showed low amplitude muscle action potentials with normal nerve conduction. There was increased stool fat and absent trypsin in duodenal fluid. His sweat test was negative. He was treated with fat soluble vitamins and subsequently pancreatic enzymes. As an adult he had normal CT and USS of the pancreas. He was unable to discontinue enzymes because of symptoms. The skin condition remained unchanged. The muscle disease progressed to involve the lower limbs and speech and muscle biopsies showed increased fibrosis and fatty change. The gene probe (estimated to identify 60 –70% of cases) for Becker’s muscular dystrophy and Emery Drefus syndrome was negative. He died at 50 years from metatstatic adenocarcinoma arising from the distal pancreas. The daughter was assessed in early childhood to have the same skin condition and muscle disease, with muscle biopsy confirmation at 8 years of age. The genetic testing along with her father was negative. She had a gastrointestinal assessment at 15 years of age because of the mothers concern regarding the risk of pancreatic cancer. There were no gastrointestinal symptoms and her growth was at the 90th percentile. Sweat test and CF DNA mutational analysis was negative. There was marked fatty infiltration of the pancreas on CT scan. Stool fat was increased and she had an abnormal pancreatic stimulation test and normal small bowel histology. She was treated for vitamin E deficiency and considered for pancreatic enzyme supplementation. Conclusion: The clinical phenotype is indicative of Becker’s muscular dystrophy and the etiology of exocrine insufficiency in this syndrome may be related to a gene on the X chromosome. Other modes of inheritance, especially autosomal dominant inheritance cannot be excluded. 404 PERSISTENT NON–ACID REFLUX SYMPTOMS DIAGNOSED USING COMBINED MULTI–CHANNEL INTRALUMINAL IMPEDANCE AND PH METRY Sajid Jalil, M.D., Radu I. Tutuian, M.D. and Donald O. Castell, M.D., M.A.C.G.*. Gastroenterology and Hepatology, Medical University of South Carolina, Charleston, SC. Purpose: 24 – hour pH monitoring is the current standard for evaluation of gastroesophageal reflux. The pH monitoring records acid reflux only. However, recent studies have suggested that non–acid reflux may be an important component. Combined multichannel intraluminal impedance and pH (MII–pH) provides accurate recognition of all forms of reflux independent of the pH. The impedance catheter measures electric current between adjacent electrodes spaced at 2 cm. Movement of bolus or refluxate causes a change in impedance, depending on its conductivity. Hence non–acid reflux will cause change in impedance despite no change in pH. Both acid and non–acid reflux is identified and its proximal extent through the length of the esophagus is also noted. Case Report: This 42 year old white male had been experiencing symptoms of heartburn, acid taste and frequent regurgitation for the past 3 years. Medical history & physical examination were unremarkable. A barium esophagogram was performed. Diagnosis of gastroesophageal reflux disease (GERD) was made and he was treated initially with rabeprazole 20 mg daily. Because of persisting symptoms this was increased to BID and ranitidine 300 mg at bedtime was also added. Although heartburn and acid taste responded, he continued to have frequent regurgitation. Esophageal manometry and 24 hour pH study was performed. Manometry was normal. 24 hour pH study done on therapy (BID rabeprazole & HS ranitidine) using a distal esophageal and a gastric pH electrode showed excellent control of gastric and esophageal acid as shown below. No correlation with 13 episodes of regurgitation was noted. % Time pH ⬍4
Upright recumbent
Esophageal
Gastric
0.4 0
26.4 64.5
S133
An overnight (16 hr) ambulatory multichannel impedance and pH (MII– pH) recording was then performed (on continued therapy). The study showed frequent non–acid reflux with 100% positive association with all 19 symptoms of regurgitation. The patient was referred for a surgical anti– reflux procedure. Conclusions: This case highlights the utility of MII–pH technology in diagnosing non–acid reflux and its ability to help in making management decisions for patients with GERD. 405 PYLEPHLEBITIS: REPORT OF 2 CASES Sandhya R. Dasari, M.D., Samudyatha A. Kumar, M.D., Emad H. Elbadawy, M.D., K.V. Gopal, M.D. and Michael Springer, M.D.*. Internal Medicine, Fairview Hospital, Cleveland, OH. Introduction: Pylephlebitis is a rare and fatal complication of intra– abdominal suppurative infections. Early diagnosis is crucial because of the high mortality. We report two cases of pylephlebitis with no apparent source of infection on initial presentation. Case 1: A 44 –year old male with no significant past medical history presented with acute onset of abdominal pain and fever. Initial diagnostic evaluation including blood cultures, stool cultures, HIDA scan of the gallbladder and CT scan of the abdomen was unremarkable. Patient continued to have fever and abdominal pain. Repeat CT abdomen revealed branch like hypodensities within the liver consistent with portal vein thrombosis, which was confirmed by duplex ultrasound. Laboratory evaluation for hypercoagulable state was negative. Patient received heparin and was discharged home on warfarin. Few weeks later patient was readmitted with recurrent abdominal pain and fever. CT abdomen revealed a loculated abscess measuring 6x 6 cm within the right lobe of the liver, which was drained surgically. Blood cultures grew Streptococcus constellatus and patient was started on vancomycin because of penicillin allergy. Four months later, follow– up CT abdomen showed diverticulitis requiring admission for sigmoid colectomy. Case 2: A 49 –year– old male presented with three weeks history of fever and chills. Physical examination was remarkable for jaundice and epigastric tenderness. Diagnostic evaluation revealed leukocytosis and elevated liver enzymes. CT of the abdomen revealed small punctate collections of air within the portal venous system consistent with pylephlebitis. Blood cultures grew Streptococcus constellatus and Bacteroides fragilis. Patient was started on meropenem with significant improvement. Follow up CT of the abdomen showed resolution of the air in the portal system with new evidence of sigmoid diverticultis. Discussion: Pylephlebitis is an uncommon complication of inta–abdominal infections, most often diverticulitis. Diagnosis can be challenging because of non–specific clinical picture. Doppler ultrasound is important in establishing the diagnosis though CT scan can be superior in diagnosing and finding the primary focus of infection. Pylephlebitis can result in pyogenic liver abscesses, septicemia and complete thrombosis of the portal circulation. Recognition and early management of pylephlebitis is critical for optimal management and better outcome. 406 EOSINOPHILIC GASTROENTERITIS PRESENTING AS MASSIVE EOSINOPHILIC ASCITES Thomas Scileppi, M.D., Andrew Pastewski, M.D., Emanual Schiowitz, D.O., Avi Schiowitz, D.O. and Scott Tenner, M.D.,M.P.H., FACG*. Gastroenterology, Maimonides Medical Center, Brooklyn, NY and State University of New York Health Sciences Center, Brooklyn, NY. Purpose: Eosinophilic gastroenteritis is an idiopathic entity that is rarely seen, characterized by a triad of eosinophilic infiltration of the gut, peripheral eosinophilia and gastrointestinal symptoms. Few patients with eosinophilic gastroenteritis are found to have ascites as part of the evaluation. We present a patient with eosinophilic gastroenteritis who presented with massive eosinophilic ascites.
Abstracts
Purpose: A 45–year– old man with history of chronic alcoholic pancreatitis presented with dyspnea and right–sided chest pain of ten days duration. He had an episode of acute pancreatitis 8 months back with accompanying ascites, which resolved with treatment. Physical examination revealed a right–sided effusion that was confirmed on chest radiography. Thoracentesis revealed rapidly refilling hemorrhagic pleural effusion requiring multiple aspirations. The pleural fluid amylase was 3200 IU with a corresponding serum amylase of 454 IU, fluid protein 3.4gm/dl and cytology was negative for malignant cells. CT scan showed right–sided pleural effusion, features of chronic pancreatitis and a pseudocyst in the head of the pancreas. There was no ascites and no primary pulmonary disease. Magnetic resonance pancreatography performed revealed a diffusely dilated pancreatic duct. Although no fistulous tract could be identified, based on a high index of clinical suspicion a diagnosis of pancreatico–pleural fistula due to pancreatic duct disruption or leak from pseudocyst was entertained. Patient was commenced on conservative management, which included no oral intake, total parenteral nutrition, repeated thoracentesis and octreotide administration. The rapid refilling of the effusion stopped and a repeat ultrasound after three weeks showed complete resolution of the pleural effusion. Discussion: Pancreatico–pleural fistula is a rare condition in which the pancreatic enzymes drain directly into the pleural cavity, most commonly from an enlarging pseudocyst. Pleural effusions are large, recurrent and usually left–sided. Isolated right–sided effusion without ascites as seen in our patient is very rare. Its presentation is often confusing with a predominance of pulmonary symptoms and a relative absence of abdominal complaints. Many patients undergo extensive pulmonary evaluation before pancreas is identified as the cause of the effusion. Pancreatico–pleural fistula should be suspected in a patient with history of alcoholism and recurrent pleural effusion. The key to the diagnosis is a dramatically increased pleural fluid amylase. Demonstration of a fistula tract between the pancreas and the pleural space by ERCP is the diagnostic standard, however, we feel that in the appropriate clinical situation conservative treatment for pancreatico–pleural fistula may be offered to patients prior to a more invasive procedure.
401 CELIAC DISEASE MIMICKING AS IMMUNOPROLIFERATIVE SMALL INTESTINAL DISEASE Mohinish Chhabra, M.D.* and Shailender Singh, M.D. Chhabra Gastroenterology & Endoscopy Clinic, Chandigarh, India and Department of Internal Medicine, Mercy Catholic Medical Center, Darby, PA. Purpose: A 24 –year– old Asian male presented with abdominal pain, small bowel diarrhea and forty–pound weight loss of 2 years duration. His past medical history was unremarkable and he was not on any medications. General physical exam was unremarkable except for pallor and clubbing. Abdominal examination revealed normal bowel sounds without any evidence of organomegaly or ascites. Initial laboaratory data showed iron deficiency anemia with a hemoglobin of 8 gm/dl. Other than serum albumin of 2.6 gm/dl the comprehensive metabolic panel was within normal limits. Stool analysis confirmed steatorrhea and microscopic examination was negative for giardiasis. Small bowel contrast radiography showed irregular and nodular mucosa. Upper endoscopy also revealed multiple small nodules of varying sizes in the second and third part of duodenum. The mucosal folds were decreased in size and number. Small bowel biopsy showed total villous atrophy with a lymphoplasmacytic infiltrate in lamina propria. In view of the symptoms, radiographic findings and plasmacytoid differentiation of the infiltrate, immunoproliferative small intestinal disease (IPSID) was considered. Further investigations revealed normal serum protein electrophoresis, however, serology for celiac disease was positive for antiendomysial antibodies. Patient was commenced on gluten–free diet which resulted in complete and dramatic resolution of his symptoms. Repeat endoscopy six months later demonstrated disappearance of the duodenal nodularity.
AJG – Vol. 97, No. 9, Suppl., 2002
Discussion: The foremost cause of malabsorption with lymphoplasmacytic infiltration of the small bowel mucosa depends on the geographic area, IPSID being common in the developing world. Although, celiac disease is predominantly seen in the western hemisphere it should be considered in the differential diagnosis of malabsorption in developing countries as well. Common endoscopic findings in CD include scalloping, grooving, mosaic pattern along with decreased number and height of mucosal folds. However, unusual finding of duodenal mucosal nodularity, more common in IPSID, can also occur in CD as seen in our patient. The pathologic features of broadened or effaced villi with short crypts as opposed to total villous atrophy with long hyperplastic crypts along with serological tests help differentiate IPSID from celiac disease. It is essential to differentiate between these two conditions since treatment options are entirely different. 402 ASPERGILLUS ASSOCIATED WITH GASTRIC ULCERATION AND BLEEDING HERALDS A POOR OUTCOME Khalid Khan, M.B.Ch.B. and Sally Weisdorf–Schindele, M.D.*. Pediatrics, University of Minnesota, Minneapolis, MN. Background: Infection with Aspergillus in immunosuppressed individuals can be fatal complication. We report 4 children whose outcome was related to bleeding gastric ulcers caused by Aspergillus. Three of 4 died. Case 1. A 15 year– old male developed hematemesis 4 days after cadaver liver transplantation for acute fulminant hepatitis. At endoscopy an ulcer was seen in the fundus extending through the gastroesophageal junction. Brushings of the membranous black granulation tissue showed Aspergillus. He was treated aggressively but died of cerebral tonsillar herniation 2 days later. Multiple intracranial lesions were found at autopsy. Case 2. A 8 –year– old male, 78 days post cord blood transplantation for relapsed ALL was ventilated for respiratory failure and upper intestinal bleeding. At endoscopy large shallow areas of ulceration and bleeding were covered in black granulation tissue. Gastric brushings grew Aspergillus. Gastric bleeding areas were cauterized. The patient succumbed to multisystem infection within 2 days. Case 3. An 18 –year– old girl on chemotherapy for osteosarcoma was endoscoped after a hematemsis. Multiple shallow ulcers with dark granulation were seen throughout the stomach. Brushings revealed Aspergillus. Two days later she became acutely unwell, and at laparotomy necrotic gastric tissue was removed (histology showed Aspergillus. The liver, cerebral cortex and lungs were involved on CT. She made a gradual recovery. Case 4. A 2–year– old male developed a 2cm black skin lesion 6 days after cord blood transplantation for aplastic anemia. He had a craniotomy for fungal mass in the frontal lobes but made a gradual recovery. At 59 days he deteriorated with intestinal and pulmonary hemorrhage. Large bleeding ulcers with typical black appearance were evident in the stomach. Despite cautery the patient died of overwelming blood loss. Conclusion: Multisystem involvement is likely when there is endoscopic evidence of Aspergillus in immunosuppressed children. The typical findings are shallow ulcers with a dark ulcer base. Early endoscopy for upper intestinal symptoms should be considered in this group. 403 EXOCRINE PANCREATIC INSUFFICIENCY AND MUSCULAR DYSTROPHY IN A FATHER AND DAUGHTER – A NEW SYNDROME Khalid Khan, M.B.Ch.B., Steven Smith, M.D. and Nancy Mendelsohn, M.D.*. Pediatrics, University of Minnesota, Minneapolis, MN and Medicine, University of Minnesota, Minneapolis, MN. Purpose: There are few reports of primary exocrine pancreatic insufficiency outside of Cystic Fibrosis. None have been associated with muscle disease. We describe a young girl and her father with the exact same clinical phenotype. We believe this to be a new syndrome. Cases: The father was investigated at 13 years of age for orange oily stools for one year. A rash from infancy comprozed of telengectasia of the face and raised roughened, pigmented areas over the lateral areas of the trunk.
AJG – September, Suppl., 2002
Abstracts
He had thin arms and was found to have weakness and wasting in the upper limbs, more pronounced distally. Muscle biopsy gave evidence of both a neurpathy and degeneration of muscle fibers and the EMG showed low amplitude muscle action potentials with normal nerve conduction. There was increased stool fat and absent trypsin in duodenal fluid. His sweat test was negative. He was treated with fat soluble vitamins and subsequently pancreatic enzymes. As an adult he had normal CT and USS of the pancreas. He was unable to discontinue enzymes because of symptoms. The skin condition remained unchanged. The muscle disease progressed to involve the lower limbs and speech and muscle biopsies showed increased fibrosis and fatty change. The gene probe (estimated to identify 60 –70% of cases) for Becker’s muscular dystrophy and Emery Drefus syndrome was negative. He died at 50 years from metatstatic adenocarcinoma arising from the distal pancreas. The daughter was assessed in early childhood to have the same skin condition and muscle disease, with muscle biopsy confirmation at 8 years of age. The genetic testing along with her father was negative. She had a gastrointestinal assessment at 15 years of age because of the mothers concern regarding the risk of pancreatic cancer. There were no gastrointestinal symptoms and her growth was at the 90th percentile. Sweat test and CF DNA mutational analysis was negative. There was marked fatty infiltration of the pancreas on CT scan. Stool fat was increased and she had an abnormal pancreatic stimulation test and normal small bowel histology. She was treated for vitamin E deficiency and considered for pancreatic enzyme supplementation. Conclusion: The clinical phenotype is indicative of Becker’s muscular dystrophy and the etiology of exocrine insufficiency in this syndrome may be related to a gene on the X chromosome. Other modes of inheritance, especially autosomal dominant inheritance cannot be excluded. 404 PERSISTENT NON–ACID REFLUX SYMPTOMS DIAGNOSED USING COMBINED MULTI–CHANNEL INTRALUMINAL IMPEDANCE AND PH METRY Sajid Jalil, M.D., Radu I. Tutuian, M.D. and Donald O. Castell, M.D., M.A.C.G.*. Gastroenterology and Hepatology, Medical University of South Carolina, Charleston, SC. Purpose: 24 – hour pH monitoring is the current standard for evaluation of gastroesophageal reflux. The pH monitoring records acid reflux only. However, recent studies have suggested that non–acid reflux may be an important component. Combined multichannel intraluminal impedance and pH (MII–pH) provides accurate recognition of all forms of reflux independent of the pH. The impedance catheter measures electric current between adjacent electrodes spaced at 2 cm. Movement of bolus or refluxate causes a change in impedance, depending on its conductivity. Hence non–acid reflux will cause change in impedance despite no change in pH. Both acid and non–acid reflux is identified and its proximal extent through the length of the esophagus is also noted. Case Report: This 42 year old white male had been experiencing symptoms of heartburn, acid taste and frequent regurgitation for the past 3 years. Medical history & physical examination were unremarkable. A barium esophagogram was performed. Diagnosis of gastroesophageal reflux disease (GERD) was made and he was treated initially with rabeprazole 20 mg daily. Because of persisting symptoms this was increased to BID and ranitidine 300 mg at bedtime was also added. Although heartburn and acid taste responded, he continued to have frequent regurgitation. Esophageal manometry and 24 hour pH study was performed. Manometry was normal. 24 hour pH study done on therapy (BID rabeprazole & HS ranitidine) using a distal esophageal and a gastric pH electrode showed excellent control of gastric and esophageal acid as shown below. No correlation with 13 episodes of regurgitation was noted. % Time pH ⬍4
Upright recumbent
Esophageal
Gastric
0.4 0
26.4 64.5
S133
An overnight (16 hr) ambulatory multichannel impedance and pH (MII– pH) recording was then performed (on continued therapy). The study showed frequent non–acid reflux with 100% positive association with all 19 symptoms of regurgitation. The patient was referred for a surgical anti– reflux procedure. Conclusions: This case highlights the utility of MII–pH technology in diagnosing non–acid reflux and its ability to help in making management decisions for patients with GERD. 405 PYLEPHLEBITIS: REPORT OF 2 CASES Sandhya R. Dasari, M.D., Samudyatha A. Kumar, M.D., Emad H. Elbadawy, M.D., K.V. Gopal, M.D. and Michael Springer, M.D.*. Internal Medicine, Fairview Hospital, Cleveland, OH. Introduction: Pylephlebitis is a rare and fatal complication of intra– abdominal suppurative infections. Early diagnosis is crucial because of the high mortality. We report two cases of pylephlebitis with no apparent source of infection on initial presentation. Case 1: A 44 –year old male with no significant past medical history presented with acute onset of abdominal pain and fever. Initial diagnostic evaluation including blood cultures, stool cultures, HIDA scan of the gallbladder and CT scan of the abdomen was unremarkable. Patient continued to have fever and abdominal pain. Repeat CT abdomen revealed branch like hypodensities within the liver consistent with portal vein thrombosis, which was confirmed by duplex ultrasound. Laboratory evaluation for hypercoagulable state was negative. Patient received heparin and was discharged home on warfarin. Few weeks later patient was readmitted with recurrent abdominal pain and fever. CT abdomen revealed a loculated abscess measuring 6x 6 cm within the right lobe of the liver, which was drained surgically. Blood cultures grew Streptococcus constellatus and patient was started on vancomycin because of penicillin allergy. Four months later, follow– up CT abdomen showed diverticulitis requiring admission for sigmoid colectomy. Case 2: A 49 –year– old male presented with three weeks history of fever and chills. Physical examination was remarkable for jaundice and epigastric tenderness. Diagnostic evaluation revealed leukocytosis and elevated liver enzymes. CT of the abdomen revealed small punctate collections of air within the portal venous system consistent with pylephlebitis. Blood cultures grew Streptococcus constellatus and Bacteroides fragilis. Patient was started on meropenem with significant improvement. Follow up CT of the abdomen showed resolution of the air in the portal system with new evidence of sigmoid diverticultis. Discussion: Pylephlebitis is an uncommon complication of inta–abdominal infections, most often diverticulitis. Diagnosis can be challenging because of non–specific clinical picture. Doppler ultrasound is important in establishing the diagnosis though CT scan can be superior in diagnosing and finding the primary focus of infection. Pylephlebitis can result in pyogenic liver abscesses, septicemia and complete thrombosis of the portal circulation. Recognition and early management of pylephlebitis is critical for optimal management and better outcome. 406 EOSINOPHILIC GASTROENTERITIS PRESENTING AS MASSIVE EOSINOPHILIC ASCITES Thomas Scileppi, M.D., Andrew Pastewski, M.D., Emanual Schiowitz, D.O., Avi Schiowitz, D.O. and Scott Tenner, M.D.,M.P.H., FACG*. Gastroenterology, Maimonides Medical Center, Brooklyn, NY and State University of New York Health Sciences Center, Brooklyn, NY. Purpose: Eosinophilic gastroenteritis is an idiopathic entity that is rarely seen, characterized by a triad of eosinophilic infiltration of the gut, peripheral eosinophilia and gastrointestinal symptoms. Few patients with eosinophilic gastroenteritis are found to have ascites as part of the evaluation. We present a patient with eosinophilic gastroenteritis who presented with massive eosinophilic ascites.