Extraocular changes in congenital aniridia]

The authors draw attention to possible extraocular changes in children with congenital aniridia. Of 31 investigated patients they were found in 5 children. Among these changes, because of its serious character, Wilms tumour holds the first place; it was found by the authors in two children. To ensure its early detection, the authors emphasize the necessity to dispensarize all children with congenital aniridia. Collaboration with an experienced X-ray specialist and child oncologist is essential. In addition to Wilms' tumour congenital aniridia can be associated with serious somatic developmental changes. The authors observed in one child and AGR triad and in two patients deformities of the skeleton of the head and lower extremities.     

Central projections of trigeminal primary afferents innervating the nasal mucosa: a horseradish peroxidase study in the rat.

The respiratory region of the nasal mucosa is innervated by the ethmoidal nerve. Chemical nociceptive stimulation of this area leads to upper airway reflexes that prevent access of noxious substances to the respiratory tract and the lungs. In the present study we examined the localization of the cell bodies of the respective primary afferent fibres within the trigeminal ganglion, as well as their central projections. In 25 rats a horseradish peroxidase-wheat germ agglutinin gel was applied to the right nasal cavity. The animals were killed after 48-72 h. For visualization of the tracer the tissue was processed according to the tetramethylbenzidine method. In the trigeminal ganglion almost all labelled cell bodies were localized in a medial band immediately caudal to the entrance of the ophthalmomaxillary branch. Transganglionic projections to the trigeminal brainstem nuclear complex were only localized in the superficial laminae of the subnucleus caudalis and in the subnucleus interpolaris, areas known to be involved in processing of nociceptive information. An additional labelled terminal field was observed in the interstitial subnucleus of the nucleus tractus solitarius, which is involved in respiratory control. These results are in favour of the hypothesis that the ethmoidal nerve in rat constitutes the afferent limb of protective upper airway reflexes since it transmits mainly nociceptive information.     

Early complications of stenting in patients with congenital heart disease: a multicentre study.

AIMS: Stenting has become an established interventional cardiology procedure for congenital heart disease. Although most stent procedures are completed successfully, complications may occur. This multicentre study evaluated early complications after stenting in patients with congenital heart disease, including potential risk factors. METHODS AND RESULTS: In this combined Dutch-Belgian retrospective study, 309 consecutive patients had undergone 366 catheterizations and received 464 stents in 13 different anatomical positions (418 sites). Seventy-two stenting-related complications (19%) occurred, of which 24 (5.7%) were major. Seven procedure-related deaths were documented (2.3%). Stent malpositioning and embolization were most common (7.7%). The use of non-premounted stents tended to be associated with higher complication rates. Centre inexperience with stenting and stenting of native vs. post-surgical stenosis tended to be associated with increased major complication rates. CONCLUSION: After stenting, complications are common for congenital heart disease. The vast diversity of stenotic sites combined with relatively small patient populations makes these procedures sensitive to complications. Combining operator experience may reduce the risks of stenting in congenital heart disease. The availability of premounted stents for greater vessel diameters will likely reduce incidences of stent migration and embolization.     

The effect of quinidine on the analgesic effect of codeine

Summary We have studied the hypoalgesic effect of codeine (100 mg) after blocking the hepatic O-demethylation of codeine to morphine via the sparteine oxygenase (CYP2D6) by quinidine (200 mg). The study was performed in 16 extensive metabolizers of sparteine, using a double-blind, randomized, four-way, cross-over design. The treatments given at 3 h intervals during the four sessions were placebo/placebo, quinidine/placebo, placebo/codeine, and quinidine/codeine. We measured pin-prick pain and pain tolerance thresholds to high energy argon laser stimuli before and 1, 2, and 3 h after codeine or placebo.After codeine and placebo, the peak plasma concentration of morphine was 6–62 (median 18) nmol·.l^–1. When quinidine pre-treatment was given, no morphine could be detected (<4 nmol·l^–1) after codeine. The pin-prick pain thresholds were significantly increased after placebo/codeine, but not after quinidine/codeine compared with placebo/placebo. Both placebo/codeine and quinidine/codeine increased pain tolerance thresholds significantly. Quinidine/codeine and quinidine/placebo did not differ significantly for either pin-prick or tolerance pain thresholds.These results are compatible with local CYP2D6 mediated formation of morphine in the brain, not being blocked by quinidine. Alternatively, a hypoalgesic effect of quinidine might have confounded the results.     

Cerebral involvement in adult onset Still's disease

Still's disease was diagnosed in a 40-year-old patient as a cause of psycho-organic syndrome with complete disorientation and stupor, sensory and motor aphasia. There were no signs of a tumour, cerebrovascular accident, bacterial or viral infection. The patient recovered quickly from the cerebral disturbances under treatment with corticosteroids. Similar symptoms in adult patients with Still's disease are seldom found in the literature.     

Venous surgery in veno-occlusive dysfunction: long-time results after deep dorsal vein resection.

Follow-up of patients 1 year after deep dorsal vein resection gives evidence of an approximate 50-60% success rate. A careful selection of only this small percentage of patients, in whom abnormal drainage through the penile dorsum is obvious, is mandatory. Men with an arterial cofactor have to be excluded or to be subsequently treated by intracavernosal autoinjection of vasoactive substances. Late results from our study demonstrate a further loss of sufficient erection, also in men considered as persistent success by us, in the subjective view of the patient and/or his sexual partner.     

National survey on the management of primary hyperparathyroidism by Swiss endocrinologists

Background From the endocrine surgeon’s perspective, it is important to know how endocrinologists manage patients with primary hyperparathyroidism (pHPT). The aim of this survey was to evaluate the preoperative diagnostic workup and referral pattern for parathyroidectomy by Swiss endocrinologists. Materials and methods The survey was conducted by mailing a questionnaire to all members of the Swiss Society for Endocrinology and Diabetes in spring 2005. Results The questionnaire was sent back by 68 of 124 endocrinologists (55%). The median annual case volume of patients with pHPT was 6 (range 1–50). The mean fraction of these patients referred for surgery was 59 ± 24%. This fraction was significantly higher in the German-speaking part of Switzerland than in the French-speaking part (67 ± 21% vs 51 ± 27%). When considering surgery for asymptomatic pHPT, 62% of the endocrinologists rely routinely on the recommendations of the NIH consensus conference and 86% on the subsequent guidelines of the workshop in 2002. Sixty-seven percent of the endocrinologists routinely perform localization studies before possible referral for surgical exploration. Typically, they consisted of an ultrasonography of the neck (93%) and a ^99mTc-MIBI scintigraphy (80%). The impact of the availability of a minimally invasive surgical procedure on the number of patients referred for surgery seems to be considerable. Sixty-one percent of the participants would expand the indication for surgery if the operation could be done by a limited surgical approach. Conclusions In a relevant fraction of patients with pHPT, endocrinologists still do not regard curative therapy as mandatory. Surprisingly, there are significant cultural differences concerning referral patterns to surgery between the German-speaking and the French-speaking parts of Switzerland. Minimally invasive procedures seem to lower the threshold for referral for surgical therapy. This work was presented at the 2nd Biennial Congress of the ESES, May 2006, Krakow, Poland.