Pheochromocytoma revealed by acute heart failure. When should we operate? Presented at the ESES Congress, Gothenburg May 25-26, 2012.

PURPOSE: The aim of this study was to assess the safety and efficacy of adrenalectomy on patients with pheochromocytoma diagnosed at the time of an acute heart failure (AHF). METHODS: We reported cases of patients who presented an AHF secondary to a pheochromocytoma during a period of 10 years. The diagnosis of AHF was defined by a left ventricular ejection fraction of less than 30 % or the use of circulatory assistance. They had adrenalectomy as emergency surgery or later. Morbidity and mortality of surgery were studied. RESULTS: Thirteen patients required an adrenalectomy for AHF secondary to pheochromocytoma. Four patients (31 %) had an adrenalectomy in emergency. Nine patients (69 %) had a delayed surgery with a median delay of 25 days (7-180). Eight patients had circulatory assistance (61 %). Five of them had a circulatory assistance and a delayed surgery (38 %), two of them had a circulatory assistance followed by emergency surgery (at 1.5 and 3 days) and one had emergency surgery immediately followed by circulatory assistance. Emergency surgery was performed by laparotomy in all cases and delayed surgery by laparoscopy for seven patients (54 %). Perioperative complications consisted in: one circulatory arrest, two bleedings requiring transfusion, one intestinal ischaemia, one haemoperitoneum with re-operation (day 8). One patient died on day 5. Post-operative course of patients with delayed surgery was uneventful. CONCLUSIONS: AHF revealing a pheochromocytoma is a rare and serious event. Patients with emergency surgery have more complications than those with delayed surgery.

[Renal colic "ischemic" of renal infarction: A plea for routine helical CT in emergency]. / La colique néphrétique « ischémique ¼ de l'infarctus rénal : plaidoyer pour la tomodensitométrie hélicoïdale systématique en urgence.

OBJECTIVES: Through a retrospective cohort, to analyse causative factors of acute renal infarction and specific therapeutic. Recall the need to carry a computerised tomoraphy (CT) in any flank pain suggestive of renal colic can hide a renal infarct. METHOD: Over a period of 24 months (2008-2009), we have compiled six patients admitted for acute lumbar pain reported in renal colic and showing a renal infarction. We report the risk factors for cardiovascular disease, the clinical presentation, and the interest of heparin therapy started early in the recovery of renal function. We emphasise the need for abdominal-pelvic CT in emergency before a renal colic pain associated with injection of contrast medium if no stone obstacle is identified. RESULTS: Five patients had risk factors for cardiovascular disease including one personal history of deep vein thrombosis and pulmonary embolism and two, a family history of myocardial infarction. Five patients were treated early with LMWH, and for one the herapin was started at 72 hours of onset of symptoms. In all cases, no effect on renal function was noted. CONCLUSION: Renal infarction is a rare but probably underestimated, occurring on land known cardiovascular often. The abdominopelvic CT without injection is increasingly practiced in front of abdominal pain syndromes, it seems imperative to complete the review by the injection of contrast material if stone obstruction is unconfirmed. Early management by herapin appears to improve the complete recovery of renal function.

[Pheochromocytoma and cardiogenic failure: an indication for emergency adrenalectomy]. / Phéochromocytome et défaillance cardiaque : une indication exceptionnelle de surrénalectomie en urgence ou semi-urgence.

OBJECTIVE: To identify cardiogenic failure or cardiogenic shock associated with pheochromocytoma diagnosis and emergency adrenalectomy. Update this unusual presentation of pheochromocytoma. METHODS: Between 1998 and 2009, 119 adrenalectomies were performed in our department, among which 19 cases for pheochromocytoma. We reported three cases with cardiogenic failure or cardiogenic shock associated with emergency adrenalectomy. RESULTS: Patients were 36, 41 and 67 years old. The elapsed time between cardiogenic failure and surgery was 0, 7 and 19 days. The first diagnosis was a viral myocarditis in those three cases. The diagnosis of adrenal pheochromocytoma was done in a second step by the association of adrenal tumour on abdominal CT scan and detection of significantly elevated plasma/urine catecholamine. Severe systolic dysfunction with low ejection fraction was associated in all cases. Cardiac function was quickly restored after adrenalectomy. CONCLUSION: Cardiac emergency associated with pheochromocytoma is an unusual clinical presentation. When diagnosis fails to be performed, patients have a very poor prognosis. According to a review of the sparse literature, only early recognition and emergency adrenalectomy can improve the outcome.

[Adrenal hemorrhage acutised by adrenocorticotropin hormone]. / Hématomes surrénaliens potentialisés par le Synacthène (HSS).

OBJECTIVE: Many spontaneous adrenal hematomas have been observed in patients being treated by Synacthène. The purpose of this study is to define how to take those patients in charge on a short-, mid- and long-term. PATIENTS AND METHODS: From January 2000 to December 2008, five patients (four males and one female), mean age 47, were taken in charge in our service for spontaneous adrenal hematomas. All those patients had been treated with Synacthène for a mid-sciatic pain for 72 hours. We associated a clinical, endocrine and radiologic staging to treat those patients. RESULTS: Four patients underwent a watchful waiting, only one patient needed surgery. No adrenal tumor was ever found during the mean two years follow-up (one to four). Two patients suffered of the condition of the antiphospholipid syndrome. CONCLUSION: Spontaneous adrenal hematomas are a most uncommon pathology. The clinical attitude has thus to be defined clearly. The patient must be under close clinical evaluation. Biological and morphological parameters have to be often repeated. An adrenal tumor has to be excluded by the evaluation, as that tumor could be secreting or could not be secreting. Antiphospholipid syndrome must also be excluded.

Multicentric origin of hemochromatosis gene (HFE) mutations.

Genetic hemochromatosis (GH) is believed to be a disease restricted to those of European ancestry. In northwestern Europe, >80% of GH patients are homozygous for one mutation, the substitution of tyrosine for cysteine at position 282 (C282Y) in the unprocessed protein. In a proportion of GH patients, two mutations are present, C282Y and H63D. The clinical significance of this second mutation is such that it appears to predispose 1%-2% of compound heterozygotes to expression of the disease. The distribution of the two mutations differ, C282Y being limited to those of northwestern European ancestry and H63D being found at allele frequencies>5%, in Europe, in countries bordering the Mediterranean, in the Middle East, and in the Indian subcontinent. The C282Y mutation occurs on a haplotype that extends