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Heart transplant has become an accepted treatment for patients with end-stage (terminal) heart disease; survival rate is 79 percent at one year, and 66 percent at five years. Although graft rejection is the main concern in the postoperative period, many patients also develop abdominal problems. A review is presented of patients who developed abdominal complications following heart or heart-lung transplantation. Over a 10-year period, a total of 429 patients received transplants; of these, 41 patients (9.5 percent) developed abdominal complaints within one month after surgery. Twenty patients underwent abdominal surgery for these complications. Development of abdominal symptoms reduced survival, but not significantly. The most common complication was pancreatitis (inflammation of the pancreas usually due to damage to the biliary system) occurring in 10 patients. Other complications included peptic ulcer (loss of the lining of the stomach or small intestine); pseudo-obstruction of the intestine; perforation of the colon; and small bowel obstruction. Laparotomy (abdominal surgery), when necessary, was well tolerated. It is concluded that prompt diagnosis and treatment of abdominal complications following heart or heart-lung transplant is necessary. The risk associated with laparotomy used to establish a definite diagnosis is less than the risk major abdominal infection in a patient with a weakened immune system. (Treatment with drugs to suppress the immune system to avoid rejection of the transplanted organ is standard procedure in these patients.) (Consumer Summary produced by Reliance Medical Information, Inc.)

Objectives: Survey on the current medical approach to and the economic issues affecting infants with primary hyperoxaluria type 1. Methods: Questionnaire to specialized centers worldwide. Results: Seventy-eight infants were identified: 44% were of Muslim origin and 56% were not. The consanguinity rate was 76% and 0%, respectively. Thirty-three percent were treated in developing countries (group 1) and 67% in developed countries (group 2). Initial presentation (4.9 +/- 2.8 months) consisted of failure to thrive (22%), urinary tract infection (21%), and uremia (14%). Radiologic findings included nephrocalcinosis (91%), urolithiasis (44%), or both (22%). The diagnosis was based on family history, tissue biopsy, and urine oxalate level in most patients from group 1 and on urine oxalate and glycolate levels, alanine:glyoxalate aminotransferase activity, and DNA analysis in patients from group 2. Therapeutic withdrawal was the final option for 40% of children; financial reasons were given for 10 of 17 patients from group 1 and 0 of 9 from group 2. End-stage renal disease started at 3.2 +/- 6.4 years of age and was present in half of the patients at the time of diagnosis. Fifty-two percent of the patients died: 82% in group 1 versus 33% in group 2; 33% of patients who underwent transplantation died versus 71% of those who did not. Conclusion: The management of primary hyperoxaluria type 1 in infants is a major example of the ethical, epidemiologic, technical, and financial challenges that are raised by recessive inherited diseases with early life-threatening onset. In certain circumstances, oxalosis can be regarded as a condition for which therapeutic withdrawal may be an acceptable option. (J Pediatr 1999;135:746-50)