We report the case of a 38-year-old woman with acute intermittent porphyria (AIP). Following the observation of an acute AIP attack in the patient's father, the diagnosis was established after genetic and biochemical examinations. At the age of 29, eight months after delivery of her first and only child, the patient was hospitalized due to a first proven attack of AIP. In the following years she suffered several premenstrual AIP attacks, with clinical symptoms ranging from abdominal pain to paralysis. One attack was accompanied by an increased urinary catecholamine output, strongly indicating adrenergic hyperactivity. The precipitation of acute episodes by secretion of gonadotrophins and a severe hyponatraemia due to a syndrome of inappropriate anti-diuretic hormone secretion indicated hypothalamic involvement in the pathogenesis of AIP. This patient has experienced an evolution of treatment regimens. At first, acute attacks were treated by i.v. hypertonic glucose. Afterwards propranolol was instituted as a maintenance therapy. Later on, i.v. injections of haem arginate were very successful in resolving acute AIP episodes. However, until therapy with an LHRH analogue was started, the patient continued to suffer premenstrual AIP attacks. These LHRH analogues cause hypothalamic inhibition of gonadotrophin secretion, with stabilization of endogenous ovarian steroid production at a low level, and therefore may be effective in preventing acute exacerbations of this disease. Since this patient went on a fixed regimen of an LHRH analogue combined with the lowest dose oestrogen patch her quality of life has improved substantially and she has not required hospitalization, now for over 3 years.

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Prediction of exocrine and endocrine insufficiency after pancreaticoduodenectomy using volumetry. ObjectivesThe aim of this study is to evaluate the use of pancreatic volumetric assessment to predict exocrine and endocrine insufficiency after pancreaticoduodenectomy. MethodsThirty-seven patients who underwent pancreaticoduodenectomy were included in the study. Endocrine function was assessed in all patients without a history of diabetes using an oral glucose tolerance test.A 13 C-labeled mixed triglyceride (MTG) breath test evaluated exocrine function before and after resection. Volumetric measurements were performed on CT or MRI. ResultsThe volumetric measurements could not predict pre-or postoperative diabetes. Moreover, the resected volume was significantly lower in patients who developed diabetes after resection. Comparing patients with a normal and a disturbed postoperative MTG, postoperative volumes and parenchymal thickness were significantly different. The parenchymal thickness om postoperative imaging is withheld as a predictive factor (OR = 0,85 [95%CI 0.71-1.01], p=0,049) and. The best cut-off value is a parenchymal thickness of less than 11,4 mm (AUC = 0,76, p =0,025, sensitivity= 88,9%, specificity = 70,0%). ConclusionsPancreatic remnant volumetry and parenchymal thickness measurement after pancreaticoduodenectomy are correlated with exocrine insufficiency, but with a limited predictive value. None of the preoperative measurements are withheld to predict postoperative exocrine function.Pre-and postoperative volumetry appear to have no use in predicting postoperative diabetes.

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