[Hypoglycemia and endocrine effects of adults' inborn errors of metabolism]. / Hypoglycémies et manifestations endocriniennes des maladies héréditaires du métabolisme chez l'adulte.

Inborn errors of metabolism (IEM) are rare diseases, most often inherited as an autosomal recessive disorder. They may be associated with endocrine dysfunction, the most frequent of them being disorders of carbohydrate metabolism (hypoglycemia, diabetes). The endocrinologist might be led to screen these complications in a patient whose diagnosis has been done during childhood. In some rare cases, he should evoke the diagnosis in front of an endocrine disorder most often associated to a multisystemic involvement. This spreading field is new, not yet very well known in adulthood. Long-term consequences of IEM on fertility and bone metabolism are still poorly understood. Diagnosis orientation relies on a few specific lab investigations encompassing blood lactate, free fatty acids and 3-hydroxy-butyrate, ammoniemia, carnitine and acylcarnitines, aminoacid and urinary organic chromatography. Hyperinsulinism, glycogenosis, fatty acid ss-oxydation, carnitine cycle and glycosylation (CDG syndrome) disorders, fructose intolerance, tyrosinemia, organic aciduria may explain hypoglycemia. These diagnosis should be evoked in front of unexplained adult hypoglycemia. Diabetes is related to iron overload, mitochondriopathy and thiamine sensitive diabetes. Clinical spectrum of some forms of IEM switch from hypoglycemia in childhood to diabetes in adulthood. Mitochondriopathies can be associated to all types of endocrine disorders, the most frequent being diabetes and dysthyroidism. Hypothyroidism is encountered in mitochondriopathies, cystinosis and primary hyperoxaluria. Hypogonadism is almost constant in galactosemia, frequent in CDG syndromes, cystinosis and iron overload. Most of the time, a specialized advice is required, which is one of the mission of reference centres.

[Surgical management of duodenal ulcer with hemorrhage from the gastroduodenal artery: antrectomy versus conservative surgery?]. / Ulcère duodénal hémorragique par érosion de l'artère gastroduodénale nécessitant un geste chirurgical: antrectomie ou traitement conservateur?

When surgery is indicated for bleeding duodenal ulcer, the traditional standard of care has been "radical surgical treatment is preferable to conservative therapy since the risk of rebleeding is reduced without an augmentation in morbidity and mortality". This principle is based on two prospective studies published before 1995. Radical surgery at that time consisted of antrectomy, while conservative therapy included oversewing of the bleeding vessel in the ulcer bed and ligation of the gastroduodenal artery (Weinberg procedure). This strategy must be re-evaluated in 2008 in view of our better understanding of the role of Helicopacter pylori in the causation of duodenal ulceration and the decreased risk of post-operative re-bleeding with the use of proton pump inhibitors. The role of surgery has changed. Its aim is no longer to cure the ulcer diathesis but rather to urgently control bleeding in anticipation of ulcer cure with medical therapy.

[Not Available]. / Ulcère duodénal hémorragique par érosion de l'artère gastroduodénale nécessitant un geste chirurgical : antrectomie ou traitement conservateur ?

O. Brehant, D. Fuks, C. Sabbagh, A. Wouters, C. Mention, F. Dumont, JM. Regimbeau When surgery is indicated for bleeding duodenal ulcer, the traditional standard of care has been "radical surgical treatment is preferable to conservative therapy since the risk of rebleeding is reduced without an augmentation in morbidity and mortality". This principle is based on two prospective studies published before 1995. Radical surgery at that time consisted of antrectomy, while conservative therapy included oversewing of the bleeding vessel in the ulcer bed and ligation of the gastroduodenal artery (Weinberg procedure). This strategy must be re-evaluated in 2008 in view of our better understanding of the role of Helicopacter pylori in the causation of duodenal ulceration and the decreased risk of post-operative re-bleeding with the use of proton pump inhibitors. The role of surgery has changed. Its aim is no longer to cure the ulcer diathesis but rather to urgently control bleeding in anticipation of ulcer cure with medical therapy.