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P J Klinkhamer

Publications and source records attributed to P J Klinkhamer.

4 recordsLinked to original sources

Hereditary spastic dystonia with Leber's hereditary optic neuropathy: neuropathological findings.

Neuropathological findings in a 59-year-old male case of hereditary spastic dystonia with Leber's hereditary optic atrophy included: marked depletion of myelinated nerve fibres in the posterior funiculi, corticopontine tracts and striatum; practically complete neuronal depletion in the putamen and lateral part of the caudate, and mild cell loss in the substantia nigra. The putamina had changed into a spongy fibrillary scar, the pallidal fibres and laminae were practically all degenerated. Moreover, there was generalised mild fibre degeneration of the white matter. The optic nerve showed marked, predominantly central, loss of nerve fibres with demyelination.

Brain

Intraobserver and interobserver variability in the quality assessment of cervical smears.

Intraobserver and interobserver variability in assessing the quality of cervical smears, as measured by the presence or absence of endocervical columnar cells and squamous metaplastic cells, was evaluated. In total, 180 cervical smears representing the most important cytologic diagnoses were anonymously rescreened twice by 19 observers with an interval of six months. An absence of endocervical columnar cells was proven to correlate with a high percentage of false-negative diagnoses. Intraobserver agreement on the presence or absence of endocervical columnar cells was 85.7% between the two screenings. A predictive value of 57.7% was found for a negative scoring (absence of these cells) while the predictive value of a positive scoring (presence of endocervical cells) was 87.3%. Of the observer scorings, 83.9% concurred with the final diagnosis; there was no significant correlation between that concurrence and the number of years of experience in cytopathology of the observer. For squamous and squamous metaplastic cells in the cervical smear the predictive value of a negative scoring (absence) was only 20.6%. Compared to the final diagnosis, 69.5% of these scorings matched. A significant and relatively high correlation with the experience of the observer was found for the scoring for the presence of metaplastic cells. Even though the predictive values of these quality scorings were relatively low a significantly higher risk for false diagnoses was established when negative scorings were given. It is therefore advisable to have smears with negative scorings for endocervical columnar cells and squamous metaplastic cells always rescreened by another observer.

Cervix Uteri

Intraobserver and interobserver variability in the diagnosis of epithelial abnormalities in cervical smears.

In a study of variability in the diagnosis of epithelial abnormalities, cervical smears with abnormalities of different severity were rescreened twice by 19 observers with an interval of six months. The observers focused on grading atypicality of squamous, squamous metaplastic and endocervical columnar epithelial cells; their results were compared (1) for the two screenings to assess intraobserver variability and (2) to "review" (final) diagnoses to assess interobserver variability. When the same observer rescreened a smear, 83.3% of the diagnoses did not differ more than one grade between two screenings; however, average intraobserver variability differed considerably for individual observers. The intraobserver variability was only slightly (not significantly) influenced by the years of experience in cytopathology of the observers. Intraobserver variability proved to be an important factor in incorrect diagnoses: 49.1% of the smears with false-negative and 52.9% with false-positive diagnoses at the first rescreening were correctly assessed at the second rescreening. Of all diagnoses made at rescreening, 80.9% were in agreement with the review diagnosis. The interobserver variability also showed considerable differences between observers; however, there was a strong influence of the experience of the observer on the interobserver variability. Atypicality grading of endocervical columnar epithelium by the observers showed a low correlation with the review diagnoses. The relatively low accuracy in the evaluation of this kind of epithelial abnormality is likely to be attributable to the low incidence of abnormal changes of endocervical columnar epithelium. The results of this study point to intraobserver variability as the main cause of false diagnoses. When wrongly diagnosed, severe epithelial abnormalities are more often underestimated than completely overlooked. Apart from training in cytopathology, the establishment of laboratory protocols for multiple screening of even minor abnormalities seem to be the most effective means of reducing the number of false diagnoses.

Cervix Uteri