Safety and long-term effects of renal denervation: Rationale and design of the Dutch registry.

BACKGROUND: Percutaneous renal denervation (RDN) has recently been introduced as a treatment for therapy-resistant hypertension. Also, it has been suggested that RDN may be beneficial for other conditions characterised by increased sympathetic nerve activity. There are still many uncertainties with regard to efficacy, safety, predictors for success and long-term effects. To answer these important questions, we initiated a Dutch RDN registry aiming to collect data from all RDN procedures performed in the Netherlands. METHODS: The Dutch RDN registry is an ongoing investigator-initiated, prospective, multicentre cohort study. Twenty-six Dutch hospitals agreed to participate in this registry. All patients who undergo RDN, regardless of the clinical indication or device that is used, will be included. Data are currently being collected on eligibility and screening, treatment and follow-up. RESULTS: Procedures have been performed since August 2010. At present, data from 306 patients have been entered into the database. The main indication for RDN was hypertension (n = 302, 99%). Patients had a mean office blood pressure of 177/100 (±29/16) mmHg with a median use of three (range 0-8) blood pressure lowering drugs. Mean 24-hour blood pressure before RDN was 157/93 (±18/13) mmHg. RDN was performed with different devices, with the Simplicity™ catheter currently used most frequently. CONCLUSION: Here we report on the rationale and design of the Dutch RDN registry. Enrolment in this investigator-initiated study is ongoing. We present baseline characteristics of the first 306 participants.

Ethmoid and upper nasal cavity carcinoma: treatment, results and complications.

From 1970 to 1985, 45 patients with carcinoma of the upper nasal cavity and ethmoid sinuses were radically treated. The tumor parameters, treatment strategy, radiotherapy technique and the results of treatment of these patients are retrospectively analysed in this study with particular reference to complete blindness as a major complication. In most cases tumor was removed by meticulous surgical dissection, and thereafter quality-controlled radiation therapy (external therapy: mean dose 65 Gy in about 7 weeks, or external therapy with brachytherapy boost: mean dose 82 Gy in about 10 weeks) was used for this group of patients. The results show 68% recurrence-free survival at 5 years, adjusted for intercurrent deaths. Complications of treatment were seen in seven patients (16%) who developed eye damage with some loss of visual acuity, none however leading per se to complete blindness. It may be concluded that blindness may be avoided to a major extent while treating carcinomas in the nose-ethmoid areas without compromising loco-regional control.

VCTS chart evaluation as a screening test.

Contrast sensitivity curves of 211 randomly selected patients were drawn using the VCTS chart. Depending on the type of curve obtained, patients were grouped in different diagnostic categories following the instructions supplied with the chart. Clinical examination revealed that the VCTS chart curves indicated a false diagnosis of cataract in 65.5% patients and in 54.2% patients a false diagnosis of glaucoma. In a number of additional patients, VCTS chart testing had failed to indicate the presence of cataract or glaucoma. Contrary to the claims of the manufacturer our results demonstrate that the VCTS chart has no value as a screening device in a clinical practice.

Lens implantation in children.

Two objections to lens implantation in children are answered. After the second year of life the size and the strength of the implanted lens does not have to be changed any more. Long-term follow-up (more than ten years) of Binkhorst 2-platinum loop lenses in traumatic cataract gives indication of slight decrease in visual function. In view of this the indication for lens implantation has still to be very strict, although fewer complications are to be expected in the posterior chamber lenses.

Radiation-retino and opticopathy.

This is an ad-interim report of a prospective study of irradiation pathology of retina and optic nerve following radiotherapy of tumors in the perinasal region. It is estimated that in approximately 20% of the cases after 1-3 years interval pathology of retina and optic nerve will occur.

Aland eye disease: no albino misrouting.

Electrophysiological studies showed that a patient with Aland eye disease had no misrouting of the optic pathways which is always found in all forms of albinism as a consequence of the retino-geniculate anomaly. Also the spontaneous and optokinetic nystagmus did not resemble that of the large majority of human albinos. The marked asymmetry found in this patient seems to be typical for humans with a defective development of foveal binocular vision. These findings are in agreement with clinical, nystagmographic and EM findings that Aland eye disease is distinct from the Nettleship-Falls type of X-linked ocular albinism. Furthermore, Aland eye disease is different from X-chromosomal congenital stationary night blindness with myopia by the fact that the scotopic functions are only moderately affected and there is no restriction of the peripheral photopic visual fields. In addition, there is latent nystagmus of extraocular type that appears also in female carriers. There is no ophthalmoplegia, there is a progression of the myopia and the dyschromatopsia is of secondary type.

Congenital corneal opacity (Peters' anomaly) combined with buphthalmos and aniridia.

A case history of a newborn infant is discussed. At birth he showed bilateral buphthalmos with aniridia and congenital corneal opacities, agreeing with Peters' anomaly. A keratoplasty was performed. The dysgenesis mesenchymalis classification is mentioned briefly. High resolution banding technique of chromosomes revealed no abnormality.

François' syndrome. An intermediate between mandibulofacial and craniofacial dysostoses.

Because of the presence of microphthalmos and cataract and the absence of auricular deformations the François syndrome belongs to neither the craniofacial nor the mandibulofacial dysostoses. TeleXray measurements revealed forward dislocation of the mandibular joint. A proposal to speak of basocraniofacial dysostosis in the cases of Hallermann-Streiff-François is made.

Fluorescein angiography in potential carriers for choroideremia. An additional aid for final diagnosis, when funduscopy shows equivocal symptoms.

As an extension of a large pedigree with choroideremia, described by Kurstjens in 1965, a family will be presented. A daughter of an affected male presented with her two carrier daughters and her three unaffected sons. In one daughter, funduscopy did not reveal characteristics specific for the diagnosis of the carrier state for choroideremia. Special attention will be paid to the diagnostic value of fluorescein angiography for final diagnosis and genetic counseling.

A retrospective study of ocular findings in patients treated with PUVA.

Photochemotherapy combined with psoralen, now widely used in the treatment of psoriasis, vitiligo, Andrews' disease and some other skin conditions, has been accepted as one of the potential factors that might contribute to the development of cataract. This view is based upon experimental animal studies. In the present study 408 patients receiving PUVA treatment in the period between 1977 and 1983 have been followed up for up to 6 years after the initiation of therapy. Repeated ophthalmological examinations were performed, particularly in order to detect any early signs of lens changes. In 20 patients development or increase of lens opacities could be observed. There is some evidence that in 3 of these 20 patients cataract formation was attributable to PUVA.

Cyclosporin A treatment in two cases of corneal peripheral melting syndrome.

One patient with the clinical diagnosis of Wegener's disease suffered from bilateral progressive uveitis and corneal melting which did not react to steroid and immunosuppressive therapy. Cyclosporin A produced dramatic improvement within 5 days, of both the uveitis and the corneal melting. The second patient had severe progressive corneal melting and scleral melting in one eye, which did not react to corticosteroid therapy or grafting of the defects. The corneal melting stopped completely on cyclosporin A therapy for two months. Gum hypertrophy was only seen as a side effect of cyclosporin A therapy in one patient, no severe side effects such as kidney or liver dysfunction were noted.

[Corneal pathology in Crohn's disease: electron microscopic study of a case]. / Kératopathie dans la maladie de Crohn. Etude d'un cas au microscope électronique.

Corneal pathology in Crohn's disease is relatively rare. We had the opportunity to take a biopsy of a bullous mass in the cornea of a man with Crohn's disease. Analysis of electronic pictures shows an aberrant structure of the nucleus, a very dense nuclear membrane and fragmented nucleoles. The cytoplasm contains many patches without membrane; the basal membrane is composed of filaments, and the stroma possesses figures of fingerprint-type. This unclassified description of the cornea might be typical for Crohn's disease.

Carrier detection in tapetoretinal degeneration in association with medullary cystic disease.

We conducted electro-oculographic and electroretinographic studies of six families with the autosomal recessively inherited Senior-Loken syndrome (juvenile familial nephronophthisis or medullary cystic disease in association with tapetoretinal degeneration). Sixteen patients had both renal and ocular involvement. In two families consanguinity between the patients was established. Routine ophthalmologic examination of relatives without renal involvement showed no retinal abnormalities, but electro-oculographic and electroretinographic studies were helpful in detecting 16 asymptomatic family members who carried the gene.