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Acute pancreatitis in children is a little known and poorly defined disease, and thus rarely considered in the diagnosis of pediatric abdominal pain. In the past 14 years, the authors treated 21 children who had acute pancreatitis. Trauma was the cause of the disease in 29% of the patients. One third (33%) had hypovolemic shock-related pancreatitis (mostly after either cardiopulmonary bypass or severe gastrointestinal bleeding). Furthermore, a major proportion (38%) had severe underlying organic disease. The clinical presentation was unremarkable; most patients (83%) had abdominal pain, especially in the epigastrium, and vomiting was the only other clinical sign exhibited by more than 50%. The Glasgow score (a severity grading system based on eight laboratory values and calculated within the first 48 hours after admission) had good specificity but poor sensitivity. Amylasemia had no predictive value. More than half our patients (57%) had complications, mainly pseudocysts (24%) and relapse (14%), and about one quarter (24%) had severe pancreatitis. There were two deaths (10%), and all surviving children (90%) eventually were symptom-free. Treatment was conservative in the majority of cases; eight patients (38%) required surgery. Hypovolemic shock and a severe underlying pathology were identified as risk factors for the occurrence of severe pancreatitis (P < .005) or death (P < .001), but not for the development of complications.

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To investigate the contribution of IL-11 and LIF to acute-phase protein (APP) production, we first analysed the effects of IL-11 and LIF on production of C-reactive protein (CRP), fibrinogen, and haptoglobin by human primary hepatocytes. We also measured the serum levels of IL-11, LIF, and CRP in serum from patients with inflammatory rheumatic diseases to assess the role of these cytokines in the APP response in vivo. We included patients with conditions associated with a high APP response such as rheumatoid arthritis (RA) or spondylarthropathy (SpA), and others usually associated with a weak APP response such as systemic lupus erythematosus (SLE), in order to investigate whether these cytokines could account for the differences in APP responses. Our results showed that IL-11 and LIF induced only minimal stimulation on production of APP by human primary hepatocytes compared with IL-6, known as the major inducer. Serum levels of CRP were elevated in RA and SpA, and significantly higher than in SLE patients. Despite the presence of a high APP response in some of our patients and despite the fact that we used sensitive assays to measure IL-11 and LIF, serum levels of both cytokines were not detected in any of the tested sera. In conclusion, our results show that circulating levels of IL-11 or LIF do not contribute significantly to the production of APP in vivo, and that they do not account for the difference in APP response between SLE and other inflammatory rheumatic diseases.

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The authors suggest that, prior to human liver transplantations, exhaustive training be undertaken on an animal model in order to acquire the expertise required for this delicate procedure. The pig has considerable anatomic similarities to man, as well as a comparable physiological and hemodynamic sensitivity, and has been used as a model by our experimental team to perfect our clinical liver transplantation technique. In particular, a surgical model representing the adult donor/child recipient situation has been developed. Anesthesia, extracorporeal bypass procedure and the full surgical protocol are described. This operative protocol was elaborated to mimic as closely as possible the clinical situation. Our model also allowed us to undertake several relevant physiological and pharmacological studies during the transplantation procedure.

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Hirschsprung's disease is characterized by a single aganglionic segment of colon extending distally to the anal margin. Well documented reports of segmental aganglionosis have been rare. We report a case of segmental aganglionosis in which there were two distinct aganglionic segments resected. The entire transverse colon between the two aganglionic segments was normally ganglionated, preserved, and utilized and functions in a normal fashion.

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Ninety-six renal transplants in 77 pediatric patients are reported with follow-up as long as 12 1/2 years. Thirteen of the first 14 patients are living with a functioning kidney after eight to 12 1/2 years. The patient survival for the entire group is 78%. Sixty-four percent are living with a functioning transplanted kidney. Splenectomy was initially performed at the time of transplant but has been discontinued because of concern that splenectomy in the immunosuppressed patient was related to an increased occurrence of septic complications. Anencephalic newborn infants have been found to be a satisfactory source of cadaver donor kidneys. Growth and development have been satisfactory when the transplant is performed prior to 12 years of age, if it functions well, and if an alternate-day regimen of steroid administration is followed. Both boys and girls have now passed through puberty with their transplanted kidneys, have married, become parents, and are leading essentially normal lives. A plea is made for earlier transplantation in small children with irreversible progressive renal failure before they develop severe stunting of growth and before the need for prolonged dialysis.

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Total colectomy, mucosal proctectomy, and ileoanal endorectal anastomosis have been successful in 17 of 19 children with chronic ulcerative colitis. No significant complications were encountered in our recent 9 cases. The technique of the operation as it is now performed in our institution is described.

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25 cases of post-operative small intestinal fistulae in children are described. 24 benefited by intensive feeding either exclusively parenterally (20 cases) or orally by constant infusion of an elemental diet (4 cases). In 13 cases the fistulae closed spontaneously but the other 11 needed further operations after an average of 41 days feeding. 2 children died; 1 was operated on early and the other had chronic granulomatous disease. Thus it is important to avoid early surgical intervention and to ensure adequate drainage of fistulae. Intensive feeding either parenterally or orally has an important role in this condition.

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During the past 5 yr, 25 children ranging in age from 10 days to 14 yr have been treated for single or multiple severe enterocutaneous fistulas. There were two deaths. In 24 cases out of 25, initial treatment was nonsurgical and consisted of nutritional support (by total parenteral nutrition in 20, and constant rate enteral feeding in 4) and was associated with local treatment. Successful closure was achieved without surgery in 13 cases, and 11 secondary operations were performed, with success in 9. The addition of nutritional methods has completely changed the prognosis of enterocutaneous fistula.

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Since ulcerative colitis is a mucosal disease, it would appear possible to remove the diseased rectal mucosa and preserve all anorectal musculature. When performed in conjunction with total colectomy, the terminal ileum could then be placed inside the retained muscular wall of the rectum and anastomosed to the anus. This would remove all of the disease and yet preserve anorectal continence. Seventeen patients with chronic ulcerative colitis have undergone this operation with satisfactory results in 15 and no deaths. Many details of preoperative, operative and postoperative management are presented which are imperative for a successful result. Sufficient experience has been gained that the operation can now be recommended.

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