Spontaneous diabetes in the domestic pig, an animal suitable for metabolic and endocrine studies and for experimental surgery, is extremely rare. In this study we have compared the diabetogenic response of various doses of streptozotocin in comparison to surgically induced diabetes. Streptozotocin in a low dose, 35 mg/kg body weight did not influence glucose metabolism while an intermediate dose, 85 mg/kg, resulted in a transient diabetic reaction. Streptozotocin, 100-150 mg/kg body weight, caused a complete and permanent diabetes. Animals made diabetic by means of pancreatectomy did not survive more than 10 days due to their poor general condition and diabetes. Streptozotocin induced diabetic animals survived with insulin treatment up to seven months. The results show that juvenile pigs made diabetic with 100-150 mg/kg body weight of streptozotocin may be useful in experimental work on glucose-, insulin- and C-peptide-metabolism in a large animal. Therefore it is potentially useful in pancreatic transplantation research.

Twenty previously pancreatectomized dogs received a duct-ligated pancreatic allograft; 16 of the grafts were preserved for 24 h, 9 with continuous hypothermic albumin perfusion, 7 with simple hypothermic storage; 4 glands were transplanted immediately and served as controls. The outcome of preservation was assessed by studying the early endocrine function of the glands. Excluding five early failures due to bleeding, all grafts showed endocrine activity after transplantation and the glucose values were on a normal level. An initial hyperglucemia caused by pancreatectomy disappeared within 24 h. In the two groups with 24 h preservation, no differences in graft function were found. In the control group, there was a hypoglucemic tendency the first days after transplantation and a prolonged hyperinsulinemia during glucose load. It could be concluded that duct-ligated, isolated canine pancreas well tolerated 24 h of preservation both by continuous hypothermic albumin perfusion and by simple hypothermic storage in an extracellular type of fluid.

Heterotopic transplantation of a duct-ligated canine pancreas was performed in 20 previously pancreatectomized, non-related recipient dogs. A technical procedure was elaborated for this grafting. It was found that early, fatal postoperative bleeding in the recipient could be avoided when the interval between pancreatectomy and transplantation was reduced from 7 to 2–4 days. The early endocrine function was studied in 15 surviving animals by determination of blood glucose and serum insulin during fasting and during glucose load. 24 h after transplantation the blood glucose values were normal and 4–8 days after transplantation the glucose tolerance was within normal limits. The serum insulin was on a supranormal level, also during glucose load. The early endocrine capacity of the transplanted duct-ligated pancreas after transplantation was found to be satisfactory, restoring the blood glucose in the pancreatectomized recipients to normal levels.

The behaviour of eight duct-ligated canine pancreata was studied during 23 hours of continuous, hypothermic perfusion, with an albumin electrolyte solution. The organs developed a considerable oedema during perfusion. This did not seem to influence on the vascular resistance of the organs. A continuous insulin release, a moderate increase in LDH concentration and only small changes in the electrolyte concentrations were observed in the perfusate, indicating only a moderate degree of cellular damage.

Three patients with Fabry's disease with a similar clinical picture, including recurrent burning sensations in the extremities, hypohidrosis and slowly progressive renal insufficiency, have been investigated metabolically at different stages of renal impairment. One patient died after three unsuccessful renal transplantations in a 4-year period of intermittent haemodialysis with disabling pains. One successfully transplanted patient is still alive and well, 12 years after the start of therapy. Thermolabile a-galactosidase has been demonstrated in his urine. The third patient has slowly progressive renal impairment. No therapeutic enzyme replacement available today is ideal. Early diagnosis is therefore necessary to increase the possibilities of prenatal diagnosis and genetic counselling. Kpy Mwrds: Fabry's disease, diagnosis, renal failure, treatment. Acta Med Scand 21 I: 309-312. 1982.