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Acute pancreatitis in a patient with glutaric acidemia type I
The Journal of Pediatrics Volume 128, Number 4
Editorial correspondence
tained from children who had no H. pylori on the basis of biopsyrelated tests) and one had negative serologic results. Three patients still had symptoms and upper gastrointestinal endoscopy was offered. Two had H. pylori-associated gastritis and increased values of H. pylori antibodies, one had mild esophagitis but absence of gastritis and H. pylori infection. Our data demonstrate that a significant improvement of dyspeptic symptoms may occur in children affected by non-ulcer-related dyspepsia after a treatment that has not changed histologic and H. pylon status. These results are complementary to those of Gormally et al. 1 and emphasize the need for placebo-controlled therapeutic studies before children with H. pylori-associated gastritis, in the absence of peptic ulcer, undergo pharmacologic treatment designed to relieve dyspeptic symptoms.
Concetta Sferlazzas, MD Giovanni Tuccari, MD Mariangela Lombardo, MD Sergio Conti Nibali, MD Giusseppe Di Pasquale, MD Giuseppe Magazzt~, MD Departments of Pediatrics and Pathology University of Messina Messina, Italy
evaluating symptoms after treatment before we knew whether the infection had been eliminated in the children. Similarly, in the past we and our colleagues in Toronto have evaluated children who are sent for endoscopy in relation to their symptoms but without knowledge of their H. pylori infection status. There was no difference in the initial symptoms of children who were infected compared with those who were not infected.2 It is unlikely that placebo-controlled trials will be undertaken in children in relation to the treatment of H. pylori infection. Therefore we must rely on studies that evaluate symptoms in children at a community level to determine whether infected children have symptoms that differ from those with no H. pylori colonization. Studies to date suggest that there is no difference in terms of symptoms between these two groups. 3 This is consistent with the finding that up to 100% of children in some developing countries have H. pylori infection.
Brendan Drumm, MD Children's Research Centre Our Lady's Hospital for Sick Children Crumlin, Dublin 12, Ireland Siobhan M. Gormally, MD Department of Pediatrics Our Lady's Hospital for Sick Children Crumlin, Dublin 12, Ireland
9/35/71757
9/35/71760
REFERENCES
1. Gormally SM, Prakash N, Dumin MT, et al. Association of symptoms with Helicobacterpylori infection in children. J PEDIATR 1995;126:753-6. 2. Heidelberg D, Wagner Y, Heldenberg E, et al. The role of Helicobacterpylori in children with recurrent abdominal pain. Am J Gastroenterol 1995;90:906-9. 3. Tolla V. Helicobater pylori in pediatric nounlcer dyspepsia: pathogen or commensal. Am J Gastroenterol 1995;90:865-8. 4. Svediund J, Sjodin I, Dotevall G. GSRS-A clinical rating scale for gaslrointestinal symptoms in patients with irritable bowel syndrome and peptic ulcer disease. Dig Dis Sci 1988;33:12934.
Reply To the Editor." We thank Sferlazzas et al. for their interesting response to our article. They are correct in stating that relief of symptoms may occur after treatment of Helicobacter pylori infection, but that this improvement also is seen in individuals whose infection is not eliminated. We have also experienced this with adult patients. 1 Because treatment of H. pylori is now very effective in eradicating the infection, we must be very careful in interpretation of studies on the effect of treatment on symptoms. It is to be expected that many children will have improvement or relief of their symptoms after treatment. In fact, Sferlazzas et al. presumably would have concluded that treatment was effective in resolving symptoms except for the fact that their regimen did not succeed in eliminating the infection. This is the reason that we conducted our study by
589
REFERENCES
1. Patchett S, Beetle S, Leen E, Keane C, O'Morain C. Eradicating HelieobacterpyIori and symptoms of non-ulcer dyspepsia. Br Med J 1991;303:1238-40. 2. Reifen R, Rasool I, Drumm B, Millson ME, Murphy K, Sherman PM. Helicobacter pylori infection in children: Is there specific symptomatology? Dig Dis Sci 1994;39:1488-92. 3. Fiedorek SC, Malaty HM, Evans DL, et al. Factors influencing the epidemiology of Helicobacter pylori infection in children. Pediatrics 1991;88:578-82.
Acute pancreatitis in a patient with glutaric acidemia type I To the Editor." Recent reports of pancreatitis in patients with inborn errors of metabolism have appeared in the literature. Pancreatitls has been reported to be a complication primarily of the branched chain amino acid disorders. 1 However, it also has been reported in several organic acid and fatty acid disorders such as camitine palmitoyltransferase II deficiency. 2 To our knowledge there have been no reports of pancreatitis in patients with glutaric acidemia type I. We describe one such patient, a 22-year-old severely disabled woman known to have glutaric acidemia type I. This patient, who resides in a chronic care facility, has severe dystonia, generalized joint contractures, severe scoliosis, and is unable to speak or communicate effectively. She was receiving a 1700 calorie, low-protein diet
590
Editorial correspondence
(15% protein, 40% carbohydrate, and 45% lipid) on our recommendation. The high-lipid diet was instituted to maintain a low recommended nutrient intake (RNI) for protein (1.7 gm/kg per day). Our patient came to the hospital in moderate distress with recurrent retching, intolerance of gastrostomy feedings, and copious bilious drainage from the gastrostomy tube. Her abdomen was soft and not distended; tenderness was difficult to elicit. Bowel sounds were absent. The serum amylase concentration was substantially elevated at 744 U/L (12.40 gkaffL) (normal, 23 to 120 U/L [0.38 to 2.00 gkat/L]). Serum amylase and lipase levels peaked at 1022 U/L (17.04 ~akat/L)mad 1280 U/L (12.34 gkat/L) (normal, 23 to 300 U/L [0.38 to 5.00 gkat/L]), respectively. The calcium level was normal at 8.8 mg/dl (2.2 mmol/L) (normal, 8.4 to 10.4 mg/dl [2.1 to 2.6 mmol/L]). The patient was kept in a fasting state and suction was applied to the gastrostomy tube. Antibiotic therapy was started hecause of the possibility of ascending cholangitis and intravenous fluids were administered. Her condition steadily improved and when the patient was discharged she was tolerating full feedings (total 1800 calories/day, 32% lipids). No viral or bacterial pathogens have been isolated and there has been no recurrence of the pancreatitis. The precise pathogenic mechanism for pancreatitis in our patient remains an enigma. A contributing factor may be the high lipid intake by patients receiving protein-restricted d!ets, as reported in the literature. A 17-year-old boy with Crohn disease had acute pancreatitis while receiving total parenteral nutrition with a 20% fat emulsion. A rechallenge after recovery resulted in the quick return of the signs and symptoms of acute pancreatitis. The authors concluded that the acute pancreatitis was caused by intolerance to the high
The Journal of Pediatrics April 1996
concentration of lipid. 3 The authors suggested that a high-lipid diet is inappropriate for patients wtih Crohn disease and that their caloric needs should not be met by increasing the proportion of lipids in their diets. We suggest that low-protein, high-lipid diets may also be associated with an increased risk for pancreatitis in patients with glutaric acidemia type I and in patients with other organic acidemias.
Edmond G. Lemire, MD, PhD Stan Moroz, MD, FRCPC Bradley Pollock, AID, FRCPC Ray Postuma, MD, FRCSC Cheryl R. Greenberg, AID, CM, FRCPC Departments of PediatriCs and Child Health Human Genetics and Surgery University of Manitoba and Health Sciences Centre Winnipeg, Manitoba R3E OZ3, Canada 9/35/71613
REFERENCES
1. Kahler SG, Sherwood WG, Woolf D, et al. Pancreatitis in patients with organic acidemias. J PEDIATR 1994;124:239-43. 2. Tein I, Christodoulou J, Donner E, McInnes RR. Carnitine palmitoyl-transferase II deficiency: a new cause of recurrent pancreatitis. J PZDIATR 1994;124:938-40. 3. Lashner BA, Kirsner JB, Hananer SB. Acute pancreatitis associated with high-concentration lipid emulsion during total parenteral nutrition therapy for Crohn's disease. Gastroenterolog}¢ 1986;90:1039-41.
Editorial correspondence
tained from children who had no H. pylori on the basis of biopsyrelated tests) and one had negative serologic results. Three patients still had symptoms and upper gastrointestinal endoscopy was offered. Two had H. pylori-associated gastritis and increased values of H. pylori antibodies, one had mild esophagitis but absence of gastritis and H. pylori infection. Our data demonstrate that a significant improvement of dyspeptic symptoms may occur in children affected by non-ulcer-related dyspepsia after a treatment that has not changed histologic and H. pylon status. These results are complementary to those of Gormally et al. 1 and emphasize the need for placebo-controlled therapeutic studies before children with H. pylori-associated gastritis, in the absence of peptic ulcer, undergo pharmacologic treatment designed to relieve dyspeptic symptoms.
Concetta Sferlazzas, MD Giovanni Tuccari, MD Mariangela Lombardo, MD Sergio Conti Nibali, MD Giusseppe Di Pasquale, MD Giuseppe Magazzt~, MD Departments of Pediatrics and Pathology University of Messina Messina, Italy
evaluating symptoms after treatment before we knew whether the infection had been eliminated in the children. Similarly, in the past we and our colleagues in Toronto have evaluated children who are sent for endoscopy in relation to their symptoms but without knowledge of their H. pylori infection status. There was no difference in the initial symptoms of children who were infected compared with those who were not infected.2 It is unlikely that placebo-controlled trials will be undertaken in children in relation to the treatment of H. pylori infection. Therefore we must rely on studies that evaluate symptoms in children at a community level to determine whether infected children have symptoms that differ from those with no H. pylori colonization. Studies to date suggest that there is no difference in terms of symptoms between these two groups. 3 This is consistent with the finding that up to 100% of children in some developing countries have H. pylori infection.
Brendan Drumm, MD Children's Research Centre Our Lady's Hospital for Sick Children Crumlin, Dublin 12, Ireland Siobhan M. Gormally, MD Department of Pediatrics Our Lady's Hospital for Sick Children Crumlin, Dublin 12, Ireland
9/35/71757
9/35/71760
REFERENCES
1. Gormally SM, Prakash N, Dumin MT, et al. Association of symptoms with Helicobacterpylori infection in children. J PEDIATR 1995;126:753-6. 2. Heidelberg D, Wagner Y, Heldenberg E, et al. The role of Helicobacterpylori in children with recurrent abdominal pain. Am J Gastroenterol 1995;90:906-9. 3. Tolla V. Helicobater pylori in pediatric nounlcer dyspepsia: pathogen or commensal. Am J Gastroenterol 1995;90:865-8. 4. Svediund J, Sjodin I, Dotevall G. GSRS-A clinical rating scale for gaslrointestinal symptoms in patients with irritable bowel syndrome and peptic ulcer disease. Dig Dis Sci 1988;33:12934.
Reply To the Editor." We thank Sferlazzas et al. for their interesting response to our article. They are correct in stating that relief of symptoms may occur after treatment of Helicobacter pylori infection, but that this improvement also is seen in individuals whose infection is not eliminated. We have also experienced this with adult patients. 1 Because treatment of H. pylori is now very effective in eradicating the infection, we must be very careful in interpretation of studies on the effect of treatment on symptoms. It is to be expected that many children will have improvement or relief of their symptoms after treatment. In fact, Sferlazzas et al. presumably would have concluded that treatment was effective in resolving symptoms except for the fact that their regimen did not succeed in eliminating the infection. This is the reason that we conducted our study by
589
REFERENCES
1. Patchett S, Beetle S, Leen E, Keane C, O'Morain C. Eradicating HelieobacterpyIori and symptoms of non-ulcer dyspepsia. Br Med J 1991;303:1238-40. 2. Reifen R, Rasool I, Drumm B, Millson ME, Murphy K, Sherman PM. Helicobacter pylori infection in children: Is there specific symptomatology? Dig Dis Sci 1994;39:1488-92. 3. Fiedorek SC, Malaty HM, Evans DL, et al. Factors influencing the epidemiology of Helicobacter pylori infection in children. Pediatrics 1991;88:578-82.
Acute pancreatitis in a patient with glutaric acidemia type I To the Editor." Recent reports of pancreatitis in patients with inborn errors of metabolism have appeared in the literature. Pancreatitls has been reported to be a complication primarily of the branched chain amino acid disorders. 1 However, it also has been reported in several organic acid and fatty acid disorders such as camitine palmitoyltransferase II deficiency. 2 To our knowledge there have been no reports of pancreatitis in patients with glutaric acidemia type I. We describe one such patient, a 22-year-old severely disabled woman known to have glutaric acidemia type I. This patient, who resides in a chronic care facility, has severe dystonia, generalized joint contractures, severe scoliosis, and is unable to speak or communicate effectively. She was receiving a 1700 calorie, low-protein diet
590
Editorial correspondence
(15% protein, 40% carbohydrate, and 45% lipid) on our recommendation. The high-lipid diet was instituted to maintain a low recommended nutrient intake (RNI) for protein (1.7 gm/kg per day). Our patient came to the hospital in moderate distress with recurrent retching, intolerance of gastrostomy feedings, and copious bilious drainage from the gastrostomy tube. Her abdomen was soft and not distended; tenderness was difficult to elicit. Bowel sounds were absent. The serum amylase concentration was substantially elevated at 744 U/L (12.40 gkaffL) (normal, 23 to 120 U/L [0.38 to 2.00 gkat/L]). Serum amylase and lipase levels peaked at 1022 U/L (17.04 ~akat/L)mad 1280 U/L (12.34 gkat/L) (normal, 23 to 300 U/L [0.38 to 5.00 gkat/L]), respectively. The calcium level was normal at 8.8 mg/dl (2.2 mmol/L) (normal, 8.4 to 10.4 mg/dl [2.1 to 2.6 mmol/L]). The patient was kept in a fasting state and suction was applied to the gastrostomy tube. Antibiotic therapy was started hecause of the possibility of ascending cholangitis and intravenous fluids were administered. Her condition steadily improved and when the patient was discharged she was tolerating full feedings (total 1800 calories/day, 32% lipids). No viral or bacterial pathogens have been isolated and there has been no recurrence of the pancreatitis. The precise pathogenic mechanism for pancreatitis in our patient remains an enigma. A contributing factor may be the high lipid intake by patients receiving protein-restricted d!ets, as reported in the literature. A 17-year-old boy with Crohn disease had acute pancreatitis while receiving total parenteral nutrition with a 20% fat emulsion. A rechallenge after recovery resulted in the quick return of the signs and symptoms of acute pancreatitis. The authors concluded that the acute pancreatitis was caused by intolerance to the high
The Journal of Pediatrics April 1996
concentration of lipid. 3 The authors suggested that a high-lipid diet is inappropriate for patients wtih Crohn disease and that their caloric needs should not be met by increasing the proportion of lipids in their diets. We suggest that low-protein, high-lipid diets may also be associated with an increased risk for pancreatitis in patients with glutaric acidemia type I and in patients with other organic acidemias.
Edmond G. Lemire, MD, PhD Stan Moroz, MD, FRCPC Bradley Pollock, AID, FRCPC Ray Postuma, MD, FRCSC Cheryl R. Greenberg, AID, CM, FRCPC Departments of PediatriCs and Child Health Human Genetics and Surgery University of Manitoba and Health Sciences Centre Winnipeg, Manitoba R3E OZ3, Canada 9/35/71613
REFERENCES
1. Kahler SG, Sherwood WG, Woolf D, et al. Pancreatitis in patients with organic acidemias. J PEDIATR 1994;124:239-43. 2. Tein I, Christodoulou J, Donner E, McInnes RR. Carnitine palmitoyl-transferase II deficiency: a new cause of recurrent pancreatitis. J PZDIATR 1994;124:938-40. 3. Lashner BA, Kirsner JB, Hananer SB. Acute pancreatitis associated with high-concentration lipid emulsion during total parenteral nutrition therapy for Crohn's disease. Gastroenterolog}¢ 1986;90:1039-41.