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Biomedical subjects

L Feenstra

Publications and source records attributed to L Feenstra.

At least 37 records · Page 2Linked to original sources

Clinical features in 130 patients with the velo-cardio-facial syndrome. The Leuven experience.

The velo-cardio-facial syndrome (VCFS) is a leading cause of velopharyngeal dysfunction and cleft palate and caused by a submicroscopic deletion in the long arm of chromosome 22 (band 22q11). During the last 5 years, 130 patients with a 22q11 deletion were diagnosed in Leuven. Most patients presented a wide variety of the classical features of the velo-cardio-facial syndrome. Velopharyngeal dysfunction was almost always present whereas an isolated cleft lip/palate was observed in a minority of patients. The velopharyngeal function can be evaluated by the classic combination of indirect and direct techniques. Because of the frequent occurrence of the velo-cardio-facial syndrome, estimated at around 1/4000 live births, and given the extremely broad clinical spectrum which makes clinical diagnosis difficult, screening of patients with velopharyngeal dysfunction for a deletion 22q11 is indicated.

Abnormalities, Multiple↗

Ear witness.

A description is given of some aspects of the normal human auricle. The physiognomy of the auricle is different for every individual which leads to the possibility of identifying people based on their auricles. Indeed this has even led to the reading of earprints similar to fingerprints, a fact not generally known amongst ENT-specialists. This highly specialized knowledge has been developed within a special branch of forensic medicine and criminology, called 'earology' or 'otomorphology'. Two illustrative case histories are presented.

Ear, External↗

Mandibulo-acral dysplasia in a one-year-old boy.

We report on a 1-year-old boy with Mandibulo-acral dysplasia, a rare autosomal recessive syndrome (MIM 248370). He presented at the age of 6 months with short stature, scarce brittle hair and thin skin mainly on the skull with visible veins. The facial appearance was typical with micrognathia, prominent eyes and a thin nose. Hypoplastic terminal phalanges and acroosteolysis were present. Psychomotor development is normal.

Abnormalities, Multiple↗

Conductive hearing loss and multiple pre- and supra-auricular skin defects: a variant example of the Branchio-Oculo-Facial syndrome.

Conductive hearing loss and multiple pre- and supra-auricular skin defects: a variant example of the Branchio-Oculo-Facial Syndrome: We describe a 3-year-old girl with bilateral severe conductive hearing loss and multiple bilateral supra- and preauricular defects with unusual overlying thin skin. The severe conductive hearing loss is due to abnormal configuration of the ossicular chain. Multiple pre- and supra-auricular skin defects have been described in the Branchio-Oculo-Facial syndrome, and we propose that the findings in our present patient may be a variant manifestation of this syndrome.

Branchio-Oto-Renal Syndrome↗

The additional hospital costs of the LAURA cochlear implant in the department of otorhinolaryngology, head and neck surgery of the university hospital of Leuven.

From the start of 1994 until 1996 ten patients (eight adults and two children) received cochlear implants after careful preoperative selection in our department. Only the deaf adults implanted with the LAURA cochlear implant were included in this retrospective analysis. In this study, the additional hospital costs associated with cochlear implantations were estimated. In doing this, a differentiation was made between 'fixed' costs and 'variable' costs. In general, the average cost of cochlear implantation was 1,186,741 BF (29,418.54 EUR) per implanted adult and a direct fixed cost of 262,880 BF (6,516.62 EUR) was needed for the computer requisites. In general, the cochlear implant enhance speech-perception scores in the postlingually deafened patients as well as in the prelingually deafened adults. After intensive training, all implanted adults of the University Hospital Leuven could recognize the segmental aspects of speech with scores above the level of significance.

Adult↗

Effect of a logopedic instruction program after adenoidectomy on open mouth posture: a single-blind study.

The effect of adenoidectomy (controls) on habitual open mouth posture was compared with adenoidectomy in combination with a logopedic instruction program (cases). One hundred and fifty-nine children were included (aged 2-10 years, mean age 4.6 years). The instruction program consisted of three individual logopedic sessions at 1 week, 2 weeks and 4 weeks postoperatively. One week preoperatively, 2 months and 1 year postoperatively an estimation of open mouth posture was carried out. Two months postoperatively there was a significant difference between the two groups (p = 0.001), but 1 year postoperatively this difference had disappeared (p = 0.526). The logopedic instruction program was not effective for the youngest age-group (<3.6 years).

Adenoidectomy↗

Presenting symptoms and clinical features in 130 patients with the velo-cardio-facial syndrome. The Leuven experience.

During the last 5 years, we diagnosed in Leuven 130 patients with a 22q11 deletion. The deletion was familial in 14 out of 110 index patients (12%), which is significantly less compared to previous studies. In 10 patients, the deletion was maternal, in 4 patients paternal. A cardiac defect was the main presenting symptom in 49% of patients. The other patients were ascertained through developmental delay (16%), behavioural disturbances (7%), otorhinolaryngological manifestations (6%), psychiatric manifestations (3%) and mental retardation (2%). In one patient hypocalcemia was the presenting symptom. In another patient the severe immune deficiency led to diagnosis. Most patients presented a wide variety of the classical features of the Velo-Cardio-Facial syndrome. Velopharyngeal incompetence, learning difficulties or mostly mild mental retardation were almost always present, whereas clinical significant hypocalcemia or immune disturbances were rare. Previously un(der)recognised features include polyhydramnios, renal malformations and laryngotracheamalacia or laryngeal stenosis.

Abnormalities, Multiple↗

An inventory of patients' response to treatment at a multidisciplinary breath odor clinic.

OBJECTIVE: This study was undertaken to assess patients' response to their treatment at a multidisciplinary oral malodor clinic. METHOD AND MATERIALS: In 4 years, a multidisciplinary breath odor clinic in Belgium examined 406 patients. The team consisted of an ear, nose, and throat specialist, a periodontologist, occasionally a specialist in internal medicine, and, more recently, a psychiatrist. After the initial visit, each patient was scheduled for a follow-up appointment 2 to 6 months later; however, only 143 patients (35%) showed up for this control visit. The remaining 65% of the patients answered a mailed questionnaire. RESULTS: About half of the patients who returned no longer had complaints, while 17% reported no improvement. This group was characterized by imaginary bad breath and manifest psychologic problems. There was also disbelief of their cure, although clinical examination (organoleptic evaluation and volatile sulfide measurement by means of a portable monitor) did not reveal any oral malodor. Some also insufficiently performed the recommended oral hygiene measures (tongue brushing and interdental cleaning). Most of the patients who returned the questionnaire were disappointed by the suggestion that their halitosis was the result of insufficient oral hygiene. CONCLUSION: Better education of both the public and dental professionals as to the most frequent cause of halitosis, insufficient oral hygiene, might elevate the level of compliance by patients.

Adolescent↗

Middle ear disease in cystic fibrosis.

A prospective cross-sectional study was done to investigate the involvement of middle ear disease in cystic fibrosis. Eighty-eight patients with cystic fibrosis, aged 1-28 years, were examined during the winter period 1995-1996 at regular follow-up visits to detect history and prevalence of inflammatory and infectious middle ear disease by questionnaire, otoscopy and tympanometry. One third of the patients had a history of otitis media, but only in 9% did this occur more than once. Hearing problems were recorded in 8%, but this was severe in only one case. Otoscopy showed 141 normal tympanic membranes, 17 times retraction, 15 times otitis media with effusion, once an acute otitis media in both ears. Only one patient had a grommet in one ear. More than 70% of the tympanograms were normal. A flat curve, type B according to Jerger's classification, was found in 11 ears. The youngest age group had a higher prevalence of abnormal findings on otoscopy and tympanometry, while patients who had undergone paranasal sinus surgery had less frequently abnormal findings. In conclusion, no evidence that cystic fibrosis is associated with increased prevalence of middle ear disease has been found in this prospective cross-sectional study.

Acoustic Impedance Tests↗

Treatment of severe laryngeal papillomatosis with intralesional injections of cidofovir [(S)-1-(3-hydroxy-2-phosphonylmethoxypropyl)cytosine].

Respiratory papillomatosis is a rare and often severe disease, usually localized in the larynx. It may cause respiratory distress and even life-threatening obstruction of the airways. Treatment is generally based on the evaporation of the lesions with a CO2 laser, but microsurgery, cytotoxic and/or cytostatic drugs, interferons, and vaccines are also used. Cidofovir [(S)-1-(3-hydroxy-2-phosphonylmethoxypropyl)cytosine] (HPMPC) was shown to suppress the growth of tumors induced by rabbit papillomavirus as well as human papillomavirus (HPV). The efficacy of cidofovir was assessed in 17 patients with severe respiratory papillomatosis. Cidofovir at a concentration of 2.5 mg/ml was injected directly in the different laryngeal papillomatous lesions during microlaryngoscopy under general anesthesia. Biopsies were taken before the treatment was started both for anatomopathology and viral typing. HPMPC kinetics in serum was monitored in three patients, the drug levels being determined by high-performance liquid chromatography. Complete disappearance of the papillomatosis was observed in 14 patients. Four patients relapsed and were successfully treated again with cidofovir. Of the three remaining patients, one progressed while under treatment with cidofovir, after an initial marked response. One patient had a partial remission and remained stable for more than 1 year after the last injection. He had a very aggressive and extensive disease originally. Finally, one patient was lost to follow-up after four injections. Intratumoral injections of cidofovir for the treatment of severe laryngeal papillomatosis is a powerful new therapeutic approach for this disease. Treatment was well tolerated, and no significant side effects were noted.

Adolescent↗

[Halitosis, foetor ex ore].

OBJECTIVE: To review the results of a multidisciplinary approach on halitosis. DESIGN: Descriptive. SETTING: University Hospital Leuven, Belgium. METHODS: Review of the data on etiology, diagnosis and therapy of halitosis, obtained by a multidisciplinary team consisting of ENT specialists, periodontologists and a psychiatrist. Malodour was confirmed by a halimeter. RESULTS: 491 patients, equally males and females, with a concentration between 20-50 years of age were seen. Oral causes (87%) were due to tongue coating (51%), gingivitis (17%), periodontitis (15%) or combinations 17%). The other 13% causes were due to ENT-related problems (4%), both ENT and oral (3%), digestive tract (1%) and presumed psychic (5%). CONCLUSION: Halitosis is a rather unknown medical problem that often can be solved by a multidisciplinary approach.

Adult↗

Some reflections on distribution of centres for cochlear implants.

Belgian has about 10 million inhabitants. Flanders has five and a half million inhabitants and five "CI-implant centre". As the ENT-community is not able to find sensible solutions to regulate this absurdity of one CI-centre per one million inhabitants (in Antwerp even one per 150,000 inhabitants) some legal activity should be provided by the government.

Belgium↗

The additional hospital costs and outcome of the LAURA Cochlear Implant in the Department of Otorhinolaryngology, Head and Neck Surgery of the University Hospitals, Leuven.

The additional hospital costs and the effects of cochlear implantation are described. From the start in 1994 until 1996 ten patients, 8 adults and 2 children, received a cochlear implant after careful preoperative selection. Only the 8 deaf adults implanted with the LAURA cochlear implant in the department of Otorhinolaryngology, Head and Neck Surgery of the University Hospital Leuven will be included in this retrospective analysis. In this study, the additional hospital costs associated with cochlear implantations are estimated. In estimating the costs, a differentation is made between 'fixed' costs and 'variable' costs. In general the costs of cochlear implantation is high: an average cost of 1,186,741, -Bef per implanted adult and a direct fixed cost of 262,880, -Bef for the computer requisites. To evaluate the effect of cochlear implantation a standard test, the AN-test battery, is used. In general, the cochlear implant enhances the speech perception scores in the postlingually deafened as well as in the prelingually deafened adults. After intensive training, all implanted adults of the University hospital Leuven could recognize the segmental aspects of speech with scores above the level of significance. The cochlear implant has also a positive psychological and social impact.

Adult↗

[Contemplating a university clinical department].

University clinics combine teaching, research and patient care. Some think that no single person (head of department) can perform all three tasks. Also, the head has to have the political skills necessary to manage the faculty society. The guidelines for university clinics, and for their heads in particular, are: (a) conscious pursuit of optimal quality, (b) giving the highest priority to training house staff and teaching students, (c) providing patient care and (d) ensuring that clinical research should serve the purpose of inspiring scientific attitudes in house staff. The 'product' of a university clinic is neither the sum of its publications nor the cured patient, but the medical specialist delivered to society.

Academic Medical Centers↗