We report the case of a 6-year-old boy with X-linked adrenoleukodystrophy (ALD). In view of the acute onset of vomiting, fever, and coma, encephalitis was initially suspected. However, brain magnetic resonance imaging demonstrated a pattern of demyelination that was consistent with ALD; this diagnosis was confirmed by the finding of elevated plasma very long-chain fatty acids levels. At presentation, the patient was hyponatremic. That this metabolic disturbance and the coma resolved within hours of the initiation of corticosteroid therapy suggests that the presenting symptoms were secondary to adrenal cortical insufficiency. Primary adrenal failure was confirmed by endocrinologic evaluation. Thrombocytopenia, hepatic transaminase abnormalities, anemia and leukopenia developed during the subsequent course of therapy with oleic acid and erucic acid.

Acute Disease↗

PubMedDate not supplied

Magnetic resonance imaging using endoanal coil in anal canal tumors after radiochemotherapy or local excision.

The ideal method for evaluation of anal canal tumors after radiochemotherapy and/or local excision remains controversial. Endoanal magnetic resonance imaging (EMRI) is a new, promising technique. The effectiveness of EMRI is reported in a study of 24 patients. Axial SET1-weighted and TSET2-weighted, sagittal and coronal T2-weighted sequences using Fat-suppression were acquired. In 4 cases, the low signal/noise ratio did not allow a diagnosis. In 6 cases, the lesion was not detected. Parietal hypo-intense thickening was detected in 14 patients, but it was not diagnostic for disease recurrence. In this study, EMRI showed 58.3% sensitivity and 41.6% specificity, thus it was not useful in the follow-up of anal tumors.

Adult↗

PubMedDate not supplied

[Use of a trans-brachial access during percutaneous treatment of abdominal aortic aneurysms].

PURPOSE: To evaluate the utility of a trans-brachial access during percutaneous treatment of abdominal aortic aneurysm (AAA). MATERIAL AND METHODS: Since September 1998, 43 patients with AAA, have been treated using different types of endoprosthesis. A right trans-brachial access was performed in each patient to facilitate catheterization of the controlateral iliac limb and to make the advancement of the aortic device easier and safer. Moreover an angiographic catheter was positioned at renal arteries through the trans-brachial access. Then several angiographic controls were performed to check if the position of the endoprosthesis was correct. The catheterization of the iliac stump was performed using the retrograde technique in 31 cases (72%) and the anterograde one in 12 cases (27.9%). RESULTS: The percutaneous trans-brachial access was performed successfully in 42/43 patients. Only one case required surgical exposure. The procedure time for the trans-brachial access ranged from 2-12 min (mean 4.5). Trans-brachial access proved extremely useful in facilitating the aortic device advancement when the iliac-femoral axis was sharply angulated. This access allowed an easier catheterization of controlateral iliac limb. It also permitted several angiographic controls to check the correct position of the endoprosthesis. No pseudoaneurysms, no oculo-cerebral disease, no ischemic phenomena were observed during a 29 month follow-up. Only in 2 cases (4.6%) a small hematoma was noticed the day after the procedure. CONCLUSIONS: Trans-brachial access proved to be safe and useful during stent-graft positioning.

Aged↗

PubMedDate not supplied

Endorectal magnetic resonance imaging in the preoperative staging of rectal tumors.

A proper preoperative staging of rectal tumors is important for correct treatment planning. We included in our study 23 patients with rectal carcinoma in order to evaluate the diagnostic accuracy of endorectal MRI. This technique enabled us to show neoplasms as a hyperintense lesion as compared to the muscolaris. To better delineate the renal dimensions of the neoplasms and reduce chemical shift artifact we performed T2 weighted TSE sequences with and without fat suppression. The diagnostic accuracy in the evaluation of T and N factors as compared to surgery was respectively 78.2% and 78.9%. The major problem has been a slight tendency to overstage parietal infiltration and lymphnodal involvement. Endorectal MRI allows us to obtain an excellent anatomic detail of the three rectal wall layers and a very high spatial resolution which might make this technique the examination of choice in the evaluation of rectal carcinoma.

Humans↗