Recent reports of pancreatitis in patients with inborn errors of metabolism have appeared in the literature. Pancreatitis has been reported to be a complication primarily of the branched chain amino acid disorders.1Kahler SG Sherwood WG Woolf D et al.Pancreatitis in patients with organic acidemias.J Pediatr. 1994; 124: 239-243Abstract Full Text Full Text PDF PubMed Scopus (97) Google Scholar However, it also has been reported in several organic acid and fatty acid disorders such as carnitine palmitoyl-transferase II deficiency.2Tein I Christodoulou J Donner E McInnes RR Carnitine palmitoyl-transferase II deficiency: a new cause of recurrent pancreatitis.J Pediatr. 1994; 124: 938-940Abstract Full Text PDF PubMed Scopus (23) Google Scholar 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 (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 μkat/L) (normal, 23 to 120 U/L [0.38 to 2.00 μkat/L]). Serum amylase and lipase levels peaked at 1022 U/L (17.04 μkat/L) and 1280 U/L (12.34 μkat/L) (normal, 23 to 300 U/L [0.38 to 5.00 μkat/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 because 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 diets, 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 concentration of lipid.3Lashner BA Kirsner JB Hanauer SB Acute pancreatitis associated with high-concentration lipid emulsion during total parenteral nutrition therapy for Crohn's disease.Gastroenterology. 1986; 90: 1039-1041PubMed Google Scholar 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. 9/35/71613

The effect of whole bowel irrigation (WBI) with a polyethylene glycol electrolyte lavage solution (PEG-ELS; 27 children) was compared to the WBI with two electrolytic solutions: normal saline with added potassium chloride (NS; 25 children) and Ringer's injection (RI; 29 children). The PEG-ELS required less volume and time, and did not cause the weight gain, hemodilution, and hyperchloremia associated with use of the electrolytic solutions. A balanced PEG-ELS is preferred in WBI of children.

Pediatric general surgery should be included in the undergraduate medical curriculum for reasons of improving the total surgical care of infants and children, to enable teachers to serve as role models to students considering a career in pediatric surgery and to ensure survival of pediatric surgery in the medical school curriculum. A survey of recent medical literature and surgical textbooks revealed little or no discussion concerning the aims, objectives, content, and design of the pediatric surgical curriculum. A survey of 15 Canadian medical schools showed that students are assigned very little didactic time for pediatric surgery (average total seven hours) and only 25% of graduates proceed to a clerkship in pediatric surgery, usually as an elective. The Association hereby proposes an undergraduate medical education curriculum in pediatric surgery for Canadian medical schools in order to stimulate discussion and achieve uniform input of pediatric surgery in the undergraduate medical program.

Over a 30-year period (may 1955 to December 1985), day care surgery was performed on 39,654 patients at the Winnipeg Children's Hospital (WCH). Up to 51% of all pediatric surgical cases and 59% of elective pediatric cases, involving more than 50 different procedures, were performed annually on a day-care basis. The overall incidence of postoperative problems and admission to hospital was 1.5% and 1.1%, respectively. A unique preoperative home visiting program by nurses was demonstrably effective in reducing late cancellations by 75%. The WCH experience adds further evidence that day-care surgery in a pediatric hospital is safe and effective for a large proportion of infants and children requiring operation.

The cost-effectiveness for parents of day-care pediatric surgery was assessed by comparing time and financial costs associated with two surgical procedures, one (squint repair) performed exclusively as a day-care procedure, the other (adenoidectomy) performed exclusively as an inpatient procedure. All but 1 of 165 eligible families participated. The children underwent surgery between February and July 1981. The day-care surgery group (59 families) incurred average total time costs of 16.1 hours, compared with 37.1 hours for the inpatient surgery group (105 families), as parents in the latter group remained with their child during the longer hospital stay. Parents from out of town incurred the greater time and financial costs. In both groups parents of younger children tended to spend more time at the hospital than parents of older children. Type of surgical management was not a significant factor in out-of-pocket expenses. Loss of income was associated with employment of the mother as a professional or a manager and may reflect inequalities in access to compassionate leave between men and women in equivalent positions. Opening day-care surgery facilities on weekends might reduce the financial burden on working mothers. Overall, day-care surgery was found to be cost-effective for families.

This paper reviews our 10 year clinical experience (1974 to 1983) with 33 patients with Crohn's disease; eight were diagnosed during the first five years and 25 during the second five years of the review. There were only 10 patients with ulcerative colitis during this period. The median age of diagnosis was 13 years, range 6 to 16 years. The main presenting clinical features were abdominal pain (29 patients), weight loss (26 patients), and diarrhea (23 patients). The method of diagnosis included radiological investigations and fiberoptic endoscopy with biopsy. The colon was involved in 20 patients. The therapy included Salazopyrine, steroids, parenteral nutrition (11 patients) including home parenteral nutrition (seven patients) and surgery (13 patients). Significant weight gain was observed in patients after intestinal resection. There were no deaths. We conclude that the incidence of pediatric Crohn's disease appears to be increasing, is more common than ulcerative colitis, and requires surgical treatment in a high proportion of patients. In our experience these patients respond well to aggressive nutritional therapy including home parenteral nutrition and carefully selected surgical treatment.

This full term male infant required a 90% enterectomy for congenital intestinal volvulus shortly after birth. The remaining small bowel consisted of 8 cm of proximal jejunum anastomosed to 5 cm of terminal ileum. The ileocecal valve was intact. Parenteral nutrition was administered for the first 21 months, including 17 months of home parenteral nutrition. The child's oral diet was mother's milk until 28 months of age when he was switched to an elemental diet. Solids were introduced at 6 months of age. Presently, at 36 months of age, the infant is off parenteral nutrition and is maintaining good nutritional status on a diet of 150 calories per kilo per day. His major problem has been an extreme allergy to cow's-milk protein. This infant is remarkable, since, despite record short bowel length, he maintained a completely normal growth and development, had remarkably few serious complications, spent relatively little time in hospital, and has documented improvement of G.I. absorption and radiographic contrast studies.

Whole bowel irrigation was used in 24 patients ages 8 to 17 yr, mean 13 yr, in preparation for colonoscopy (18), colon surgery (2), therapeutic irrigation for treatment of drug overdose (2), and constipation (2). An average of 9 liters warm, normal saline with added KCL (5 mEq/liter) was infused through a nasogastric tube over a period of 7 +/- 2 hr (mean +/- SD). This resulted in a weight gain of 2% and a mild hyperchloremia. The vital signs remained stable and there were no complications. The colonic preparation was complete in 10, adequate in 8 and unsatisfactory in 2. The irrigation was effective in the treatment of overdose ingestion and constipation. It is concluded that whole bowel irrigation is a satisfactory method of colonic preparation of pediatric patients.

Four hypotonic boys (aged 4 years and 11 months, 6 years and 9 months, 7 years and 4 months, and 8 years and 10 months, respectively), all of whom demonstrated a formal thought disorder, had been psychotic for more than six months, and met the DSM-III criteria for chronic undifferentiated schizophrenia, were studied with respect to skeletal muscle morphology. A significant difference between the mean fiber diameters of type I and type II muscle fibers was observed in the three boys with the most severe thought disorder, type II muscle fibers being consistently smaller. No significant difference in the mean fiber diameter between the two fiber types were seen in the fourth boy, who was also the only one who demonstrated any secondary thought process. The boys differed in their activity levels, but there was no correlation between type II muscle fiber atrophy and hypoactivity. It is hypothesized that a depressed cholinergic system is implicated in the pathogenesis of both the muscle fiber atrophy and the formal thought disorder.

Two hypotonic boys, aged 7 years, 3 months and 7 yers, 7 months, who possessed sufficient speech to demonstrate a severe thought disorder and who differed markedly in their activity levels, were subjected to a biopsy of the quadriceps muscle. The biopsies revealed atrophy of type 2 muscle fibers, as well as variability in the size of these fibers. The findings could be compatible with a denervation phenomenon.

Pyloric atresia, particularly the familial type, is rare. We present 2 cases where it was associated with epidermolysis bullosa dystrophica. To our knowledge, this association has not been reported elsewhere.The first patient presented with non-bile stained vomiting since birth. Multiple, fluid-filled, bullous lesions of the skin and mucous membranes appeared at 2 days. She underwent a gastro-jejunostomy for pyloric atresis but failed to thrive. The skin lesions spread and she developed severe hypoproteinemia, hyponatremia, anemia, septicemia and died at 9 weeks.Her first cousin also presented with vomiting and underwent gastrojejunestomy for pyloric atresia at 2 days. He developed similar skin lesions that day; biopsy was characteristic of epidermoiysis bullosa dystrophica. Persistent diarrhea contained casts of intestinal mucosa. He failed to respond to steroid, cholestyramine, antibiotic and parenteral nutrition therapy. Severe hypoproteinemia, anemia, and septicemia developed and he died at 16 weeks.Autopsy showed extensive epldermolysis bullosa lesions of skin, scalp, nails and esophagus; focal ulcerations were found in the small intestine. The pyloric atresia measured 1.7 cm.