Inflammatory aneurysm in the infrarenal portion of thoracoabdominal aneurysms: an uncommon variant

INTRODUCTION: We present an unusual variant of type IV thoracoabdominal aneurysm with inflammatory changes in part of the aortic wall. MATERIAL AND METHODS: Between January 1990 and December 2000, 5 male patients (mean age, 68 years) with inflammatory aneurysms of 29 with type IV thoracoabdominal aneurysms (17.2%) underwent surgery. All five had arterial hypertension. The diagnosis of inflammatory aneurysm was made on the basis of clinical suspicion supported by characteristic features on CT scans. Endoaneurysmorraphy was performed in all cases; a tube graft was inserted in three cases, and bifurcation was performed in 2. The macroscopic appearance of periaortic fibrosis was detected at the infrarrenal portion of the aneurysm in the 5 patients, but no retroperitoneal fibrosis was found at the level of the distal thoracic aorta in any case. RESULTS: There was no operative mortality. Paraparesis was not detected in any patient. One patient had acute kidney failure, and another required an extended stay in the intensive care unit because of respiratory insufficiency. Pathologic examination confirmed the diagnosis of inflammatory aneurysm in all 5 patients, with characteristic adventitial thickening caused by lymphoplasmacytic infiltrate and fibrosis around the ganglionic and nerve structures. CONCLUSIONS: Type IV thoracoabdominal aneurysm with inflammatory changes can be treated at surgery with a retroperitoneal approach. Usually the fibrotic response is confined to the infrarenal portion of the aneurysm.     

Serous cystadenoma of the pancreas in a child

Pancreatic tumors are very rare in children. Until now, approximately 150 cases have been reported in the English-language medical literature. Only 4 of them represented serous cystadenoma. Although the tumor is not potentially malignant, all patients required partial pancreatic resection. The authors present a 4-year-old girl with a short history of abdominal pain and vomiting. Sonography and computed tomography showed a pancreatic mass. The mass was proved to be a serous cystadenoma, and resection of the tumor was performed. Clinical discussion with review of the literature is presented.     

Inhibition of 5-lipoxygenase activating protein decreases proteinuria in diabetic rats

BACKGROUND: The binding of 5-lipoxygenase (5-LO) to the 5-LO activating protein (FLAP) is a prerequisite for subsequent formation of leukotrienes (LT) from arachidonic acid. We have shown that FLAP antagonist administration decreased proteinuria in glomerulonephritic patients. In this follow-up study, we assessed the role for FLAP in a rat model of streptozotocin-induced diabetic nephropathy. METHODS: Diabetic rats were treated for 4 weeks with FLAP (BAY X-1005, 200 mg/kg) or 5-LO (Zileuton, 80 mg/Kg) antagonists. Proteinuria, renal function and LT production was assessed. We also determined protein permeability of cultured glomerular endothelial cells (which possess no 5-LO) by measuring their permeability to radiolabeled albumin with and without FLAP antagonists. RESULTS: FLAP mRNA levels increased dramatically in glomeruli from diabetic animals compared to controls. Inhibition of FLAP (but not inhibition of 5-LO) reduced proteinuria, with no effect on estimated glomerular filtration rate. Interestingly, diabetes-induced rises in urinary excretion and glomerular production of leukotrienes were not modified by the inhibitors. Increased FLAP expression in glomerular endothelial cells in culture was associated with an increase in albumin permeability, and this increase was abolished by FLAP antagonists. On the other hand, addition of LTA(4) led to increases in leukotriene formation and in permeability. This increase in permeability was also reduced by co-incubation with FLAP antagonists, whereas the increase in leukotriene synthesis was not modified. CONCLUSIONS: These results suggest a role for FLAP other than the activation of 5-LO, possibly in protein handling, and point to FLAP antagonists as anti-proteinuric agents.     

Idebenone treatment in paediatric and adult patients with Friedreich ataxia: Long-term follow-up

BACKGROUND: Antioxidant therapy is a new therapeutical approach for patients with Friedreich ataxia. AIMS: To assess the effectiveness of long-term idebenone treatment in Friedreich ataxia patients. METHODS: An open-labelled prospective study. Ten paediatric patients (age range 8-18 years) and 14 adults (age range 18-46 years) with genetic diagnosis of Friedreich ataxia were treated with idebenone (5-20mg/kg/day) for 3-5 years. Neurological evolution was evaluated using the International Cooperative Ataxia Rating Scale (ICARS), and cardiological outcomes using echocardiography. RESULTS: In paediatric patients, no significant differences were observed in ICARS scores and echocardiographic measurements when comparing baseline status and after 5 years of follow-up. Concerning adult cases, ICARS scores showed a significant increase in neurological dysfunctions during 3 years of therapy (Wilcoxon test, p=0.005), while echocardiographic measurements remained unchanged. CONCLUSIONS: Our results indicate that longer-term idebenone treatment prevented progression of cardiomyopathy in both paediatric and adult patients, whereas its stabilizing effect on neurological dysfunction was present only in the paediatric population, mainly before puberty. This suggests that the age at which idebenone treatment is initiated may be an important factor in the effectiveness of the therapy.     

Hyperparathyroidism due to chronic renal failure. Analysis of 32 operated patients

We report our study of 32 patients diagnosed as hyperparathyroidism due to chronic renal failure (27 secondary forms and 5 tertiary ones), between June 1990 and 2003, that were sent from Nefrology Department for to surgical intervention. The following parameters have been analysed: Age, sex, clinical symptoms, kind of surgery performed, postoperative complications and anatomopathologic result (AP). On 64 nerves exposed, there were no recurrent nerve palsies. 12 patients suffered a symptomatic hypocalcemia (37,5%) although only one was permanent. The control of calcium, phosporus and calcitriol is essential to try to avoid a hyperplasia of the parathyroid glands.     

Sino-nasal undifferentiated carcinoma. Report of 1 case and literature review

Sinonasal undifferentiated carcinomas are rare neoplasms but highly malignant and aggressive. We are reporting the case of a 75 year-old female who had related recurrent epistaxis, facial pain and progressive nasal obstruction. The clinical data collected by the exploration joint to CT showed us the presence of a tumoral mass located in the right nasal fossa and homolateral maxillary sinus, eroding structures around. Its biopsy was informed as undifferentiated carcinoma and the patient was sent to Oncology Department, where she was treated by Radiotherapy (68 Gy) and weekly carboplatin during 2 months. After a short improvement of her symptoms and due to poor prognosis in this type of tumors, she died a few months later.     

Follicular carcinoma of the thyroid manifested initially as asymptomatic primary renal neoplasm

Metastases in the kidney are rare, evenmore if primary source is thyroid. We report the tenth case of metastases in the kidney from thyroid, and it is the first to be follicular type and absolutely asymptom. Sonography and computerized tomography with suspicion of renal tumour are showed in a asymtom female 75 years old. Left partial nephrectomy was perfomed, initially it has been pathologically diagnosed as renal clear cells tumour, however the definitive pathologic report showed follicular tumour of thyroid. Local and systemic stage was discovered with complementary techniques. Sources of metastases in kidney and diagnoses techniques are discussed.