Biomedical subjects
Publications and source records attributed to J Deroover.
Calcifications are uncommon in metastases. Adenocarcinoma, osteopenic, osteosarcoma, lung and breast carcinoma can be the origin. A rare case of calcified cystic brain metastasis deriving from papillary cystadenocarcinoma is reported.
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We report on two sibs with Dandy-Walker malformation and tetramelic postaxial polydactyly. We conclude that this is a new autosomal recessive syndrome.
In this report we summarize the results of a genetic-diagnostic survey of an institutionalized population of 158 severely mentally retarded patients. The etiological study was based on a clinical genetic approach with special attention to dysmorphology and neurological findings. In 72 patients a constitutional cause of their mental impairment was found: a chromosomal abnormality in 21, a Mendelian disorder in 36 (autosomal recessive disorder: 23; autosomal dominant: 12; and X-linked recessive: 1), a MCA/MR syndrome in 9, and a CNS malformation in 6 patients. In 33 patients, a pre- or perinatal cause was found, and 20 patients presented a pre- or perinatal infection of the CNS. Finally, no etiological diagnosis was detected in 28 patients; 6 of them presented a hitherto unclassifiable type of familial mental retardation.
A double-blind, placebo-controlled, crossover trial was carried out in 29 patients with Huntington's chorea to evaluate the effectiveness and tolerance of high doses of tiapride in the management of neurological symptoms. Patients were allocated at random into 3 groups to receive 3 g tiapride daily for two periods of 3 weeks either preceded, interrupted or followed by a 3-week period on placebo. Patients were assessed on entry and at the end of each treatment period using a battery of tests designed to measure choreatic movements, motor skills, recognition and reaction times, and mental state. Twenty-three patients completed the trial; only 2 of the 6 drop-outs withdrew because of drug-related side-effects (sedation and extrapyramidal signs). The results showed that tiapride treatment significantly improved choreatic movements and motor skills and these findings were supported by the objective psychometric measurements. Overall evaluation of effectiveness by the physician and patients indicated the significant superiority of tiapride over placebo. Treatment was generally well tolerated and although there were significantly more reports of sedation and extrapyramidal signs whilst patients were receiving tiapride, these effects were rated as mild in most cases and did not interfere with treatment.
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Triploid-diploid mosaicism in fibroblasts of a deeply mentally retarded 21-year-old female is reported. The relevant findings in 2n/3n mosaicism are reviewed and discussed.
A paracentric inversion of the short arm of chromosome 1 (inv(1)(p22p36)) is reported in a deeply mentally retarded 19-year-old girl and in her normal father.
A 21-year-old mentally retarded female with 10p12 trisomy is reported. She presented a slight craniofacial dysmorphism very similar to that found in the full 10p-trisomy syndrome. Other features usually found in full 10p trisomy, however, such as failure to thrive and internal malformations, were absent.
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A 10-year-old girl with partial deletion of the short arm of chromosome 9 is reported; karyotype: 46,XX,del(9)(p22). This syndrome results in a distinctive craniofacial dysmorphism with trigonocephaly and contrasting midfacial hypoplasia. Partial monosomy 9p was the result of a paternal de novo germinal deletion in this case.
A possibly new mental retardation syndrome is described in a large family. The major features of the syndrome are: short statue, craniofacial dysmorphism and dento-skeletal abnormalities. The mode of inheritance of this syndrome appears to be autosomal dominant with a variable degree of expressivity. The possible similarity to another autosomally dominant inherited mental retardation syndrome, "the K.B.G. syndrome" as described by Hermann et al. (1975), is discussed.
A large kindred is described in which 22 males and 3 females show non-specific mental retardation with impaired speech. An X-linked recessive is the most likely mode of inheritance of this condition. Similar families have been described in the literature, characteristic physical abnormalities are absent and performance I.Q. tends to be higher than verbal I.Q. This possible heterogenous condition may be a major individual cause of mental deficiency in males, and may account for the excess of male retardates in the population.
As elsewhere in Europe, the origins of Belgian obstetrics are related to the double rivalry between medical doctors and surgeons on the one hand and between surgeons and midwives on the other hand. Extension of obstetrical teaching in our regions has been hampered because of political turmoil resulting from the successive territorial dominations by Spain, Austria, France and Holland. In this evolution, the organisational sanitary model emerging from the French revolution exerted a prominent influence namely through creation of the subordinate grade of "Health Officer" and the suppression of local medical colleges as well as of the sole existing university faculty of medicine. These factors explain the poor quality of obstetrics which has prevailed on our soil until 1815.
The Napolean defeat at Waterloo entailed drastic changes of health services in our regions. Under the new Dutch rule, many different medical grades were put in use making the pertinent legal rules very difficult to apply. At Belgian independence, the Free University of Brussels was founded including a Faculty of Medicine of which the clinical infrastructure was mainly the Saint-Pierre Hospital located at the Hal Gate. In 1880, provincial schools for midwives were established while operative obstetrics remained in the hands of general surgeons. At Brussels, medical students rightly complained about the poor training of general practitioners in obstetrics. Until the first third of the XXth century, the vast majority of deliveries took place at home under the supervision of a midwife. A consultant surgeon was called upon only if deemed indispensable.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.