Sequential double lung transplant in Kartagener's syndrome

Very few cases of lung transplantation have been described for patients with Kartagener's syndrome. We report the first case to be published in Spain. A 15-year-old girl with complete Kartagener's syndrome underwent sequential transplantation of both lungs. Due to the unusual distribution of the organs in this syndrome, the bronchial stumps of donor and recipient had to be distributed differently. With the initial technical difficulties overcome, the patient now leads a normal life two years after transplantation.     

Evidence of otospongiosis obtained by computerized tomography. Does it compromise the post-stapedectomy auditory gain?

To study the influence of tomographic otospongiosis/otosclerosis on the audiometric gain after stapedectomy, we evaluated 34 patients (mean age 39.9 years, S.D. 9.8) with otosclerosis and mixed hearing loss. We performed Computed Tomography (CT) with densitometry before stapedectomy and audiometry before and 4 weeks after the surgery. CT results were classified as compatible or not for otospongiosis (< 1000 UH) or for otosclerosis (> 2000 UH). According to the affected turns of the cochlea, the studies were classified in 3 groups. In 43% of the patients the CT showed otospongiosis. After stapedectomy, air conduction thresholds of the low (125-500 Hz), middle (500-2000 Hz) and high frequency bands (2000-8000 Hz) and for the air/bone gap were similar for the ears with or without otospongiosis (p > 0.05, ANOVA). However, patients with otospongiosis in all the cochlea showed the lowest audiometric gain for the high frequency band (p < 0.05 ANOVA). Evidence of otospongiosis evaluated just by CT has a low impact on the audiometric outcome after stapedectomy.     

The effect of electrical stimulation of the subthalamic nucleus on seizures is frequency dependent

PURPOSE: Animal studies and anecdotal human case reports have indicated that the subthalamic nucleus (STN) may be a site of anticonvulsant action. METHODS: We tested the hypothesis that continuous electrical stimulation of the STN inhibits seizures acutely. We determined the effects of three stimulation frequencies, 130 Hz, 260 Hz, and 800 Hz, on generalized clonic and tonic-clonic flurothyl seizures. Adult male rats were implanted with concentric bipolar stimulating electrodes in the STN bilaterally. After recovery, rats underwent flurothyl seizures to compare the effects of each stimulation frequency on seizure threshold. Rats were tested 4 times, twice in the stimulated condition, and twice in the unstimulated condition. The order of trials was random, except that stimulation trials alternated with control trials. Flurothyl seizure thresholds under each stimulation condition were compared with control values from the same animal. RESULTS: Bilateral stimulation of the STN at 130 Hz produced a significant increase in the seizure threshold for clonic flurothyl seizures, whereas stimulation at 260 Hz did not appear to have any effect on seizures. STN stimulation at 800 Hz significantly lowered seizure threshold for tonic-clonic seizures. CONCLUSIONS: We conclude that electrical stimulation of the STN can be anticonvulsant, but the effects appear to depend on the stimulation frequency and the type of seizure.     

Retroperitoneal fibrosis: typical and atypical manifestations

Retroperitoneal fibrosis (RPF) is a well described clinical entity that is being diagnosed with increasing frequency. RPF is characterized by replacement of the normal tissue of the retroperitoneum with fibrosis and/or chronic inflammation. However, aetiology, clinical presentation and radiological appearance in many cases are protean. Up to 15% of patients have additional fibrotic processes outside the retroperitoneum. In the abdomen, RPF may spread contiguously to involve multiple structures in both the retroperitoneum and the peritoneal cavity or multiple non-contiguous sites may be involved. We retrospectively reviewed 30 patients (19 male, 11 female; age range 28-79 years) with biopsy proven RPF. Although we found RPF most commonly as an isolated fibrotic plaque in the lower lumbar region (18 patients), 12 patients (40%) presented with RPF in atypical locations (4 peripancreatic, 1 periduodenal, 7 pelvic). The RPF was non-malignant in 24 patients (21 idiopathic, 2 perianeurysmal, 1 ergot-derivative treatment) and malignant in six cases. We present a pictorial review of the varied appearances of RPF, concentrating on atypical sites. The radiological differential diagnosis and its appearance with various imaging modalities are discussed. Current concepts with respect to management, prognosis and treatment are summarized.