The difficult and controversial diagnostic and therapeutic management of patients having gastrinoma or insulinoma with multiple endocrine neoplasia type I (MEN-I) has been discussed by reference to the literature and a personal series of 45 gastrinoma/MEN-I patients followed consecutively at Bichat Hospital. In both gastrinoma/ and insulinoma/MEN-I patients, anatomic distribution and morphology of tumoral process(es) are usually multiple, diffuse, of small size, and associated with endocrine cell hyperplasia and even nesidioblastosis. These features enhance the difficulty of tumor localization and eradication. Despite the dramatic development of modern medical imagery and surgical experience, the real possibility, on a long-term basis, of curing the patients from their disease remains limited, especially in the gastrinoma/MEN-I patients. In the latter group, according to our experience, persistence or recurrence of the disease after surgery is usual, and metachronous hepatic metastasis development is frequently observed when the follow-up is long enough. Patients with liver metastases, however, seem to undergo a more indolent course than sporadic gastrinoma cases. In insulinoma/MEN-I patients, removal of the functionally dominant islet cell area(s) is essential. Various preoperative and intraoperative localization techniques allow efficacious selective pancreatic surgery in many cases. The latter refinements and the promises of long-acting somatostatin analogs, if confirmed, might restrict to exceptional circumstances the indication of near-total or total pancreatectomy.

Gastrinoma↗

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Quantification of lipoprotein X and its relationship to plasma lipid profile during different types of parenteral nutrition.

An abnormal lipoprotein (LP), detected in plasma during total parenteral nutrition, has been shown to be similar to LPX observed in cholestasis and in familial lecithin-cholesterol-acyl-transferase (LCAT) deficiency. However, the conditions which facilitate the appearance of LPX during total parenteral nutrition are unclear; potential determining factors could be lipid input, plasma lipid levels, and/or inhibition of LCAT activity. An investigation was conducted on 12 patients receiving total parenteral nutrition for 3 wk by simultaneously evaluating plasma LPX (via a quantitative method) as well as total cholesterol, phospholipids (PL), triglycerides (TG), apolipoprotein B, and LCAT activity. Daily total nonprotein calories (40 kcal/kg body weight) and nitrogen input (250 mg/kg body weight) were fixed in this study. Three 7-day periods were defined: during periods 1 and 3, lipid emulsion (10 or 20% Intralipid) and glucose were given as nonprotein calories (glucose-lipid periods); in period 2, glucose was administered alone as the sole source of nonprotein energy (glucose period) so that the total energy input was not modified. During periods 1 and 3, the patients were randomly assigned to receive either 9 g (period 1) and 12 g (period 3) of PL/day for 7 days, or 12 and 9 g of PL/day. By infusing either 10 or 20% Intralipid, TG input was varied concomitantly so that the subjects received 75, 100, or 150 g/day in periods 1 and 3. During the glucose-lipid periods, plasma LPX was measurable from the 2nd day and increased progressively. Its increment was closely related to a rise in unesterified cholesterol and PL (r = 0.7; p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

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[Use of French as language for scientific publication. Warning of the "paper drain"].

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